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OLD IRONSIDES NMCSD DEPARTMENT OF PEDIATRICS NORMAL VITAL SIGNS HR Beats/min RR Breaths/min BP Systolic BP Diastolic Newborn 0-1 mo 100-180 30-60 73-92 52-65 Infant 1-12 mo 80-150 30-60 90-109 53-67 Toddler 1-3 yrs 75-130 25-35 95-105 56-68 Preschool 3-5 yrs 75-120 22-32 99-110 55-70 School 6-11 yrs 70-110 20-30 97-118 60-76 Pre-adolescent 11-13 yrs 70-110 18-22 105-124 60-80 Adolescent 13-18 yrs 65-105 16-22 110-133 63-83 Adult 18+ yrs 50-90 12-20 113-136 65-84 VITAL SIGN MONITORING GUIDELINES FOR COMMON ADMISSIONS: • ROS: Q3 vitals with CRM/POX x24hrs, then Q6 w/o monitors (if stable) • Hyperbilirubinemia: Q6 vitals with temps Q1 x3 then Q3 USERNAMES & PASSWORDS Username Essentris CHCS/AHLTA DEA NPI AAP ACGME Athens Up to date My Evals Epic 2 Password Table of Contents Section 1: Nuts & Bolts........................................................................................... 4 Section 2: Radiology.............................................................................................10 Section 3: Cardiology........................................................................................... 14 Section 4: Emergency Medicine..........................................................................22 Section 5: Endocrinology.....................................................................................32 Section 6: FEN/Gastrointestinal...........................................................................38 Section 7: Hematology/Oncology....................................................................... 48 Section 8: Infectious Disease.............................................................................. 55 Section 9: Pediatric Neurology............................................................................ 72 Section 10: PICU.................................................................................................. 92 Section 11: Pulmonology.....................................................................................105 Section 12: Renal.................................................................................................. 117 Appendix.............................................................................................................. 136 3 SECTION 1 Nuts & Bolts Contact numbers.................................................................................................... 5 Important codes................................................................................................. 5 Important pharmacy/EEG numbers.................................................................. 5 Important laboratory numbers.......................................................................... 5 Pediatric subspecialist — attendings/consultants............................................. 6 Other important contact numbers......................................................................7 Type and Duration of Precautions Recommended for Select Infections........... 8 4 N U TS & B O LTS CONTACT NUMBERS HOSPITALISTS Dr. Andersen.................................... 619-218-5714 Dr. Ruff.............................................619-602-1483 Dr. Villarroel (Forensic Peds).........619-453-6081 IMPORTANT LABORATORY NUMBERS Laboratory Division Phone number Main Lab 2-9200 CDR Boamah..................................619-602-9240 Supervisor 2-8834 CHIEF RESIDENT Blood Bank 2-9356/9357/9353 Chemistry 2-7090 Hematology 2-6311 Microbiology 2-9234 Differential 2-9283 Mail Out 2-9271 PROGRAM DIRECTOR Ben Briggs....................................... 619-750-4528 NURSING DEPT. HEAD Deb Norton......................................619-532-6253 IMPORTANT CODES 2East Door Code 2-5-1 Outside Lab numbers Phone number Intern Call Room (4,5)-(1,2) PICU Call Room 3-2-1 Newborn screens 866-463-6436 ER 9911* HSV CSF PCR 858-966-5940 Resident’s Room 6-8-4-7 HSV Serum PCR 800-522-2787 Attending Office 25-31 UCSD Virology 619-543-5797 UCSD PCR 619-543-3798 CA Encephalitis 510-307-8608 Metabolic Clinic 619-543-7800 NHCP Lab 760-719-4088 IMPORTANT PHARMACY/ EEG NUMBERS EEG Phone number NHCP Micro 760-725-3410 Office number 2-7278/9757 NHCP Rads 760-719-4641 EEG Tech 619-665-3467 RCHSD Lab 858-966-5940 Sleep Lab 2-5620 RCHSD Micro 858-966-7725 Quest 800-848-4225 Pharmacy Division Phone number Discharge Pharmacy 2-5794 Wards/PICU/NICU 2-6310/8596 Compound 2-8406 NUTS & B OLTS 5 PEDIATRIC SUBSPECIALIST – ATTENDINGS/CONSULTANTS ***If there are multiple providers, please check call schedules before paging the subspecialists Specialty Physician Adolescent Medicine Duty Pager Office number Pager number 619-804-4398 (after hours) Allergy Clinic (NTC) 524-1519 Anesthesia Floor Walker Duty Pager 619-218-1692 Cardiology CDR Mao EKG fax (Mao) LCDR Kendall EKG fax (Kendall) 240-988-7742 888-415-7814 919-949-0909 Dental Duty Pager 619-453-6570 Dermatology Dr. Gibbs 619-218-4488 Dietitian Charis Ross Carly Hill ENT Duty Resident Endocrine Duty Pager CDR Kunz CDR Yates 2-6904 2-6920 619-804-4115 619-602-9454 619-804-4388 Gastroenterology CDR Boamah Dr. Yang 2-9720 619-602-9240 619-750-7895 2-9035 2-7540 2-8905 619-453-6955 General Surgery consult 619-453-7013 Genetics Dr. Willis Hematology/Oncology CAPT McManaman Dr. Pene 2-8861 619-218-9354 619-804-4328 Infectious Disease LCDR Arnold CDR Milder 2-7452 619-804-4807 619-453-7098 2-7745/5261 619-532-5261 Lactation Nephrology CAPT Ferrara Neurology Dr. Serena Dr. Zeldin Neurosurgery Duty Pager Dr. Klugh Occupational Therapy 619-804-4111 858-229-4301 2-9575 619-602-1739 619-804-4507 619-379-2604 619-665-3029 2-7100/7135 Ophthalmology Duty Resident 619-453-6302 Orthopedic Surgery Bone Phone Ortho Intern 619-954-6797 619-384-7173 Orthopedics (Hand) Ortho Cast Room 6 2-8473 24hr on call Cast Room 2-8454/8439 619-453-6883 N U TS & B O LTS Pain Service Duty Pager 619-677-7415 Physical Therapy 2-7100/7107 Plastics 2-6950 Psychiatry Duty Resident Pulmonary CDR Cleary CAPT Wojtczak Pediatric Surgery (call 2-North for Surg resident schedule and phone number) Margie Gabriel Dr. Ignacio Dr. Henry 619-384-7280 2-6883 519 532 6896 619-804-4757 619-453-6846 (Tues-Fri) 619-453-6956 502-938-5163 619-847-3541 202-257-6923 Peds Surg day pager 619-822-5495 Social Work Heather Ducksworth 2-9329 619-804-4008 OTHER IMPORTANT CONTACT NUMBERS Hospital/Area Phone Number Hospital/Area Phone Number 2East 2-6250 Fax: 2-5237 Alberta Agyemang 2-5081 619-886-7102 2East Backroom 2-5294 Lisa Burns (Pulm RN) 2-8819 2North Clinic 2-5953 Adolescent Clinic 2-6930 PICU 2-8153 Fax: 2-8668 Subspecialty Clinic (bldg 2) 2-6896 (parents) 2-5393 (staff) PICU Call Room 2-8979 NICU 2-8910 Audiology 2-9602 NICU Backroom 2-8913 619-453-6939 ER 2-8274 Melanie Kilgore (Peds audio) Gen Peds appt line (staff) 2-5767 or 2-5009 Med Photo 2-8051 619-279-0494 (ED) Gold Team Cell: 619-665-3903 RCHSD Main number 858-576-1700 RCHSD ER 858-966-8005 NHCP Quarter Deck 760-725-1288 NHCP ER 760-725-3258/1093 Green Team Cell: 619-847-7138 NHCP Clinic 760-725-1578 NTC 619-524-4947 Continuity Cell: 619-453-6841 TOC Clairemont 619-645-0155 TOC Chula Vista Gen Peds appt line (parents) 619-744-5365 (Dr. Ogena) 2-8225 Poison Control 800-411-8080 After Hours Pager 800-453-0491 Wound Care Nurse 619-453-6309 (LT Allison Redden) If you know the patient’s team (PCM) you can arrange f/u with the team nurse. NUTS & B OLTS Blue Team Cell: 619-379-6613 7 TYPE AND DURATION OF PRECAUTIONS RECOMMENDED FOR SELECT INFECTIONS Infection Type of Precautions Duration Abscess Contact Duration of illness Cellulitis Standard C. Diff Contact Until negative repeat x2 Enteroviral infections (i.e. Group A and B Coxsackie viruses) Standard EXCEPT use contact for diapered or incontinent children Duration of illness (if implementing contact precations) Gastroenteritis Standard EXCEPT use contact for diapered or incontinent children Duration of illness (if implementing contact precations) HSV - Encephalitis - Mucocutaneous, disseminated or primary, severe - Mucocutaneous, recurrent (skin, oral, genital) - Neonatal Standard Contact Until lesions dry & crusted Standard Contact Until lesions dry & crusted Influenza Droplet 5 days except immunocomp=duration of illness Measles (rubeola) Airborne 4 days after onset of rash; duration of illness in immunocompromised persons Meningitis - Asceptic - Bacterial, gram-neg enteric, in neonates - Hib, Neisseria, meningococcal disease (sepsis, PNA, meningitis) (known or suspected) Standard Standard Droplet Until 24hrs after initiation of treatment MDROs Contact Duration of illness Pertussis Droplet 5 days RSV Contact with mask Duration of illness Varicella Airborne, contact Until lesions dry and crusted * For infections not listed above, please refer to the Infection Control Manual under “references” on the intranet homepage Standard = Hand washing Contact = Gown and gloves Droplet = Mask and gloves Airborne = N95 respirator and gloves 8 N U TS & B O LTS NOTES NUTS & B OLTS 9 SECTION 2 Radiology Radiology phone directory...................................................................................10 Radiology (ordering studies).................................................................................11 Ordering additional studies.................................................................................. 12 NPO parameters.................................................................................................... 12 Arranging pediatric follow up from ER................................................................ 12 Drug levels............................................................................................................. 12 RADIOLOGY PHONE DIRECTORY Radiology Department Phone Number Front Desk 2-8666 Pediatric Reading Room 2-7382/6137 ER Reading Room 2-8684 (not available between 8am-noon) Fluoroscopy (UGI, VCUG, IVP, BE)/Nuc Med 2-8686/8775 CT Front Desk and Tech 2-8377 (Desk); 2-8731 (Tech) CT Scanner 1 & 2/ ER CT 1: 2-8771 2: 2-8772 ER: 2-8245 MRI Front Desk 2-7865 MRI Residents 2-7382/6137 US Desk/Techs/Duty Pager 2-8725 (Desk) 2-8746 (Techs) 619-453-6351 (Duty Phone) US Reading Room 2-5780/7820/5458 Interventional Radiology (IR) 2-8742 10 RA DI O LO G Y RADIOLOGY (ORDERING STUDIES) MRI CT scan Ultrasounds Fluoroscopy (UGI, VCUG, IVP, BE) Not sedated? Place STAT CHCS order Place order in Essentris (so nurses are aware of the study) Call Radiology (pediatric reading room) to inform them of the order and the indication for it – 2-6137 / 2-7382 Plain films (CXR, KUB, AAS) Place order in essentris only & include reason for study (i.e. r/o infiltrate) – clerk will fill out a chit and make the phone call to radiology *Do not need to call radiology Sedated? Follow guidelines for ordering nonsedated MRI/CT scan → Notify Anesthesia Floor Walker of need for sedation – (619) 218-1692 Coordinate study/sedation time between Anesthesia Floor Walker & Radiology *Routine Peds Anesthesia day is Tuesdays R ADIOLOGY 11 ORDERING ADDITIONAL STUDIES ECHOs: coordinated directly with the Pediatric Cardiologist EEG: order in CHCS stat, coordinated with EEG techs (2-7278; 619-665-3467) in conjunction with Pediatric Neurology Modified barium swallow studies: coordinate with June Carter, Speech Pathologist 2-5874 NPO PARAMETERS Clears: 2 hours Breast Milk: 4 hours Cow’s Milk: 6 hours Full Meal: 8 hours ARRANGING PEDIATRIC FOLLOW UP FROM ER 1. Open AHLTA 2. Select “ Telephone Consults” in Left-hand folder list column 3. Select “Actions” from top bar 4. Select “New Telcon” 5. Enter patient information and select “Find” 6. Select correct patient and press “Ok” 7. Select “Pediatrics General” . Verify phone number. 8. Select “Reyes, Edna” for Assigned Owner 9. Enter information and requested follow-up time frame and press ok DRUG LEVELS Vancomycin Trough only – 5th dose Peak if concern for gram + (ex. Staph aureus) after 4th dose Gentamicin Trough AND peak 3rd dose 12 RA DI O LO G Y NOTES R ADIOLOGY 13 SECTION 3 Cardiology EKG (mini).............................................................................................................. 14 EKG norms............................................................................................................. 16 Murmurs................................................................................................................ 17 Congenital heart defects....................................................................................... 17 SVT......................................................................................................................... 17 SVT management.................................................................................................. 18 Chest pain.............................................................................................................. 18 Syncope................................................................................................................. 19 Kawasaki Disease................................................................................................. 20 EKG (MINI) **Below are mini-EKGs – so boxes represent lead 1-V6, strip at bottom is II or V1 Where to Look What to Look For 1) Rate Paper speed- 25 mm/sec 1 small square= 1 mm = 0.04 sec 1 large square= 5 mm= 0.2 sec 2) Rhythm Sinus: P wave before every QRS; P wave for every QRS; normal PR interval, normal P-wave axis (up in lead 1 and aVF) 14 CA RDI O LO G Y 3) Axis up/up = normal down/up = RAD up/down = LAD 4) P wave Long width, biphasic V1 = LAE Tall height = RAE Height >2 small squares Width > 2.5 small squares 5) PR interval Long PR = 1st degree AVB Short PR w/ delta wave = WPW 6) QRS duration QRS > 120msec, > 3 small squares Rabbit ears in V1 = RBBB Rabbit ears in V6 = LBBB 7) QRS voltage Big R in V1 = RVH (Other criteria for RVH = upright T in V1; QR in V3R) Big R in V6 = LVH (R+S)(I) + (R+S)(II) + (R+S)(III) 8) ST segment Horizontal depress. or elev. > 1mm (use TP baseline) Downward sloping T wave axis: 9) T wave 10) QTc= QT/ square root(RR) Age V1, V2 AFV V5, V6 <1 day +/- + +/- 1-4 days +/- + + 4 days- adolesc - + + Adolesc- adult + + + Prolonged QTc: Female adol. >450 Male adol. >440 *Can calculate in any lead that the QTc is well visualized. If long QT, consider obtaining EKGs on primary family members. *If sinus arrhythmiacalculate range C AR DIOLOGY 15 EKG NORMS QRS Duration (sec)± RV1 amp (mm)± SV1 amp (mm)± RV6 amp (mm)± SV6 amp (mm)± +30 to 180 0.08-0.12 (+110) (0.10) 0.05 (0.07) 13.3 (25.5) 7.7 (18.8) 4.8 (11.8) 3.2 (9.6) 105-180 (145) +30 to 180 0.08-0.12 (+110) (0.10) 0.05 (0.07) 10.6 (20.8) 4.2 (10.8) 7.6 (16.4) 3.4 (9.8) 1-6 mos 110 - 180 (145) +10 to 125 (+70) 0.08-0.13 (0.11) 0.05 (0.07) 9.7 (19) 5.4 (15) 12.4 (22) 2.8 (8.3) 6-12 mos 110-170 (135) +10 to 125 (+60) 0.10-0.14 (0.12) 0.05 (0.07) 9.4 (20.3) 6.4 (18.1) 12.6 (22.7) 2.1 (7.2) 1-3 yrs 90-150 (120) +10 to 125 (+60) 0.10-0.14 (0.12) 0.06 (0.07) 8.5 (18) 9 (21) 14 (23.3) 1.7 (6) 4-5 yrs 65-135 (110) 0 to +110 (+60) 0.11-0.15 (0.13) 0.07 (0.08) 7.6 (16) 11 (22.5) 15.6 (25) 1.4 (4.7) 6-8 yrs 60-130 (100) -15 to +110 0.12-0.16 (+60) (0.14) 0.07 (0.08) 6 (13) 12 (24.5) 16.3 (26) 1.1 (3.9) 9-11 yrs 60-110 (85) -15 to +110 0.12-0.17 (+60) (0.14) 0.07 (0.09) 5.4 (12.1) 11.9 (25.4) 16.3 (25.4) 1.0 (3.9) 12-16 yrs 60-110 (85) -15 to +110 0.12-0.17 (+60) (0.15) 0.07 (0.10) 4.1 (9.9) 10.8 (21.2) 14.3 (23) 0.8 (3.7) >16 yrs 60-110 (80) -15 to +110 0.12-0.20 (+60) (0.15) 0.08 (0.10) 3 (9) 10 (20) 10 (20) Age Heart Rate* QRS axis* 0-7 days 95-160 (125) 1-3 wks PR interval (sec)* 0.8 (3.7) * Normal range and (mean) ± Mean and (98th percentile) 16 CA RDI O LO G Y MURMURS Benign heart murmurs: soft, systolic, ejection-type, vary with position •Still’s murmur- LLSB-LMSB, low- freq vibratory •Peripheral pulmonary stenosis of newborn- LUSB-transmits to back •Venous Hum-clavicular areas, inaudible in supine position Likely pathologic murmur: cyanosis, systolic murmur grade 3 or above, harsh, holosystolic, diastolic murmur, abnormal heart sounds, abnormally strong or weak pulses, family history of structural heart disease or sudden death •Holosystolic- VSD, MR, TR, PDA •Diastolic- AR, PR, mitral stenosis, tricuspid stenosis •Ejection-aortic stenosis, pulmonic stenosis, HOCM CONGENITAL HEART DEFECTS Occurs in 0.5-0.8% of live births. Acyanotic heart defects: volume vs. pressure overload •Volume overload: ↑ pulmonary to systemic blood flow (Qp :Qs). Eventually leads to left heart failure and resultant pulmonary edema (high-output failure). Examples: left-to-right shunts such as VSD or regurgitant lesions. •Pressure overload: obstruction to flow. Sx depend on location of obstruction (right vs. left heart failure). Examples: aortic valve stenosis or coarctation of the aorta. Cyanotic heart defects: ↑ or ↓ pulmonary blood flow •↑ Qp: no obstruction to pulmonary flow; involves abnormal flow patterns/connections (e.g. transposition of the great arteries) or mixing of venous and arterial blood (e.g. TAPVR or truncus arteriosus). •↓ Qp: obstruction to pulmonary flow with right-to-left shunt. •Examples: Tetralogy of Fallot or tricuspid atresia SVT SVT includes atrial tach, AV nodal tach, and reentrant tach (ex WPW) •No P waves or abnormal P wave axis or P after QRS •Regular, rapid – usually > 220 in infants, >180 in older C AR DIOLOGY 17 SVT MANAGEMENT Unstable patient (hypotensive, infant w severe CHF): IMMEDIATE CARDIOVERSION Stable: Vagal maneuvers •Infant: bag of ice to face for up to 10 seconds, PROTECT EYES •Older child: bearing down, blow through straw, headstand; avoid carotid massage If vagal maneuvers fail and still stable: Set up EKG and call PICU or cards • Adenosine: 100mcg/kg x1, if no effect rpt at 100mcg/kg, can go up to 200mcg/kg- Always admin with defib pads on •note: frequent sinus pause! scary for patients! •complicated to give – needs 3-way stopcock; set up prior to pushing adenosine •first stop-cock w/adenosine attached, second w/ saline flush •need to push adenosine FAST- adenosine has 10 sec half life → then immediately push 5-10 mls NS CHEST PAIN Rarely cardiac origin in kids. DDx: •Cardiac – hypertrophic CM, myo/pericarditis, arrhthymia, coronary artery anomaly, dissection (Marfan’s) •Chest wall – costochondritis, precordial catch (better w/ deep breath); •Pulm – PNA, asthma, PTX, acute chest in SS; •GI - GERD, esophageal spasms; •Psych – hyperventilation Dx: if positive family hx or past medical hx → EKG. If positive HPI or exam → EKG, monitors, consider card consult. Tx: mostly reassurance; NSAIDs for chest wall pain, etc Go to www.chop.edu/pathways/emergency-department/chest-pain/ for helpful chest pain pathway, specific questions to ask 18 CA RDI O LO G Y SYNCOPE Brief loss of consciousness due to lack of blood flow to brain. Note: syncope pts often have hypoxic jerks until the blood flow to the brain improves – this is not a seizure! Workup: •Good history, exam including neuro (and pupils), orthostatics and EKG. •CT head is NOT needed unless focal neuro signs or significant head injury from fall. Vasovagal Syncope Vasovagal syncope (most common): sympathetic burst followed by parasympathetic activation leading to profound bradycardia, hypotension. Dx: History – can have premonitory symptoms. Ex: feeling hot, sweating, lightheadedness, nausea, vision going black •Associated with emotional states, prolonged standing, dehydration, hair brushing, going to the bathroom •Post-syncope pallor, re-syncope on standing too quickly afterward, hypoxic jerks occasionally occur •pt wakes up rapidly upon lying down, can feel dizzy and “not usual self” for several hours following Tx: hydration, lie down when feeling lightheaded, nml dietary salt intake, avoidance of stimulants Cardiac Syncope Cardiac syncope: due to hypertrophic CM, tachy or bradycardia, aortic stenosis, CHF, etc •Few preceding sx; usually abrupt onset/offset; occ w/ palpitations but not always! •May occur during exercise – always worrisome! Dx: hx, exam, EKG, Holter, echo Tx: depends on cause; beta blocker for HCM and SVT, etc SEIZURES Seizures: urine incontinence (can happen w/vasovagal as well), Postictal confusion, tongue biting, etc Dx: hx, exam, EEG if convincing hx Tx: see neuro section C AR DIOLOGY 19 KAWASAKI DISEASE Dx: Fever lasting 5 days, plus 4 of the following criteria: •Bilateral conjunctival injection without exudate Incomplete Kawasaki: Fever x 5 days plus 2-3 above criteria, plus supplemental lab criteria •Labs: •Elevated inflammatory markers: CRP, ESR •Red mouth and pharynx, strawberry tongue, or red, cracked lips •Leukocytosis (WBC> 15) •Rash (may be morbiliform, maculopapular, or scarlatiniform) •Thrombocytosis (plts >500K) •Albumin < 3 •Swelling of hands and feet, redness of palms and soles •Elevated AST, ALT •U/A: sterile pyuria •Cervical lymphadenopathy- usually single and unilateral •LP: aseptic meningitis Tx: •IVIG- within first 10 days of illness, 2 g/kg over 10-12 hours •Aspirin: 80-100 mg/kg/day div over 4 doses •Echo (r/o coronary involvement Evaluation of Suspected incomplete Kawasaki Disease (KD) 20 CA RDI O LO G Y NOTES C AR DIOLOGY 21 SECTION 4 Emergency Medicine Wound management............................................................................................23 Acetaminophen toxicity........................................................................................23 Chemical exposures..............................................................................................24 Toxidromes............................................................................................................25 Antidotes............................................................................................................... 26 Burns......................................................................................................................27 Bite Management..................................................................................................28 Sutures...................................................................................................................28 Suturing................................................................................................................. 29 Fractures............................................................................................................... 30 22 E M E RGE N CY M E D IC IN E WOUND MANAGEMENT Clean and Minor Wounds All Other Wounds (1) Tetanus toxoid TIG (2) Tetanus toxoid TIG (3) <3 or unknown Yes No Yes Yes ≥3 Only if last dose given ≥ 10 years ago No Only if last dose given ≥ 5 years ago No Previous Doses of Tetanus toxoid (1) Such as wounds contaminated with dirt, feces, soil, and saliva; puncture wounds, avulsions and wounds resulting from missles, crushing, burns, and frostbite. (2) The preferred vaccine preparation depends upon the age of the child or adolescent: < 7 years: DTaP > 7 years: Tdap (3) Intravenous immune globulin should be administered if TIG is not available. ACETAMINOPHEN TOXICITY E ME R GE NCY MEDICINE 23 CHEMICAL EXPOSURES Table 1. Features of Selected Major Chemical Exposures Feature Asphyxiants Cholinesterase Inhibitors Respiratory Tract Irritants Most likely agent in accidental release Carbon monoxide Organic phosphorus pesticides Chlorine and its deriv——— atives, ammonia Most likely agent in act of terrorism Cyanide Sarin and VX Chlorine, phosgene Sulfur mustard Hallmark Tissue hypoxia in cardiovascular system and central nervous system; usually, absence of respiratory tract irritation; no increase in secretions Cholinergic syndrom with pupil constriction (miosis) and increased exocrine secretions, with or without fasciculations; increasing effects on central nervous system with increasing exposure Respiratory tract irritation and symptoms, usually more prominent than irritation of eyes and skin Eye injuries and skin burns with vesicle formation, followed by respiratory irritation and, in the case of exposure to high concentrations, systemic effects Headache, fatigue, anxiety, irritability, dizziness, nausea Miosis, dim vision, eye pain, rhinorrhea, irritability, headache, chest tightness, sweating Nose and throat irritation, sore throat, cough, chest tightness, eye irritation Conjunctivitis, limited erythema, epistaxis, sore throat, cough Moderate to severe symptoms Dyspnea, altered mental status, cardiac ischemia, syncope, coma, seizure Silivation, lacrimation, urination, defectation, gastrointestinal cramping, and emesis (SLUDGE); wheezing, muscle weakness, fasciculations, cognitive impairment, incontinence, coma, seizure Corneal damage, Laryngitis, wheezing, visicles and bullae, stridor, laryngeal ede- nausea, wheezing, ma, acute lung injury stridor, laryngeal edema, acute lung injury Hyperacute onset — sudden collapse High concentrations of cyanide or hydrogen sulfide and oxygen deficiency within a confined space Exposure to VX or high-vapor concen——— trations of other nerve agents Acute onset — typically within minutes to hours after exposure Most exposures to asphyxiant gases (carbon monoxide, cyanide) or oxygen deficiency Vapor exposure, ingestion of liquid form, or moderate-to-large dermal exposure Riot-control agents, irritants highly and intermediately water soluble (ammonia, hydrochloric acid, chlorine) Lewisite, phosgene oxime, high concentrations of sulfur mustard Delayed onset — typically 4 to 6 hr after exposure Low to moderate concentrations of substances that metabolize to primary asphyxiant — methylene chloride (carbon monoxide), acrylonitrile, and propionitrile (cyanide) Limited exposure of skin to droplets but not vapor Poorly soluble gases (phosgene, nitrogen dioxide) Sulfur mustard Vesicants Typical presentations Mild symptoms 24 ——— E M E RGE N CY M E D IC IN E TOXIDROMES E ME R GE NCY MEDICINE 25 ANTIDOTES Drug Antidote Acetaminophen N-acetylcysteine (NAC, Mucomyst) Alcohols Calcium gluconate Alcohols (ethylene glycol, methanol) Ethanol 10% or Fomepizole (4— methylpyrazole, Antizol) Anticholinesterase Atropine Antihistamines Other anticholinergic agents Physostigmine [Antilirium) Benzodiazepines Flumazenil (Romazicon) ß-Blockers Glucagon Calcium-channel blockers Calcium or Glucagon Carbon monoxide Hyperbaric oxygen Cyanide Cyanide antidote kit Digitalis Digoxin—specific Fab antibodies (Digibind) Iron Desferoxamine (Desferal) Methemoglobinemia Methylene blue Opioids Naloxone (Narcan): serum half—life is 1 hr. Duration of action is 1-4 hr. Pesticides (carbamate, organophosphate) Atropine or Pralidoxime (2—PAM, Protopam) Phenothiazines Diphenhydramine (Benadryl) Phenothiazines — acute dystonic reaction Benztrapine (Cogentin) Warfarin Vitamin K 26 E M E RGE N CY M E D IC IN E BURNS BURN CENTER TRANSFER CRITERIA: TBSA >10%, involvement of face, hands feet, genitalia, perineum or major joints. Chemical or electrical burns, inhalation injury, or 3rd Degree burns. E ME R GE NCY MEDICINE 27 BITE MANAGEMENT • Irrigate!!! Leave open: bites > 24 hours old or deep puncture wounds of hand/foot — esp. human or cat bites. • Steri-strips OK. • Never close an infected wound. • Antibiotics: (augmentin, cefuroxime, or doxycycline) for 5 days with HIGH RISK wounds (hand/ foot bites, puncture wounds, deep or extensive wounds, immunosuppressed or diabetic patients). • Re-examine within 24-48 hours. SUTURES SUTURE TYPES Absorbable (time) Non-absorbable Chromic (3-4 weeks) Nylon Vicryl (2-3 weeks) Ethilon Fast gut (3-5 days) Prolene • Removal: face in 3-5 days, extremities in 10-14 days • Use 5-0 or 6-0 for faces • Use 3-0 or 4-0 for ext/chest/ abd SUTURE MATERIAL, SIZE AND REMOVAL Body Region Monofilament * Absorbable † (Superficial Lacerations) (Deep Lacerations) Days Scalp 5-0 or 4-0 4-0 5-7 Face 6-0 5-0 3-5 Eylid 7-0 or 6-0 ——— 3-5 Eyebrow 6-0 or 5-0 5-0 3-5 Trunk 5-0 or 4-0 3-0 5-7 Extremities 5-0 or 4-0 4-0 7 Joint Surface 4-0 ——— 10-14 Hand 5-0 5-0 7 Foot/Sole 4-0 or 3-0 4-0 7-10 * Examples of monofilament nonabsorbable sutures: nylon, polypropylene. † Examples of absorbable sutures: polyglycolic acid and polyglactin 910 (Vicryl). 28 E M E RGE N CY M E D IC IN E SUTURING Irrigation • NS >250cc + 60 cc syringe + 16g angiocath. • Look for and document any foreign bodies. • Avoid betadine — may cause tissue necrosis. Numbing • Topical LET (lidocaine, epinephrine, tetracaine): Max LET 0.5 ml/10 kg. • Do not use on distal extremities, nose, or penis! • Onset: 15 min. • Duration: 20 min. Buffered Lidocaine • Use at room temp. • Mix: lidocaine w/or without epinephrine (5cc of 2%) + sterile H20/NS (4 cc) + Sodium bicarb (1cc of 8.4%) • Onset: Rapid • Duration: 20 min. • The max dose of this 1% lidocaine is 4 mg/kg or 0.4cc/kg. • No epinephrine on digits, nose, or penis! Needle and Suture Selection • Consider using steri-strips, Dermabond, or staples. • Do buried stitches with Dexon (absorbable — takes two weeks to dissolve). • Use Ethilon (nonabsorbable monofilament) on a cutting needle for most superficial suturing: 4-0 to 5-0 for most lacs. • Plain gut is absorbable and lasts 7 days max, so it is usually only used on face and scalp. • Do not use chromic gut on the face — it scars! • Consider 6-0 absorbable plain gut on little kids’ faces; it won’t need to be removed. • May staple scalp lacerations under hair. Discharge Advice • Keep wound clean and dry, covered with antibiotic ointment to prevent scab formation. • OK to shower, but do not comb hair if scalp laceration. • Return to MD if signs of infection. • Later: sunscreen to avoid hyperpigmentation. E ME R GE NCY MEDICINE 29 FRACTURES 30 E M E RGE N CY M E D IC IN E NOTES E ME R GE NCY MEDICINE 31 SECTION 5 Endocrinology Diabetes.................................................................................................................33 Hypoglycemia.......................................................................................................33 Adrenal Insufficiency.............................................................................................33 Short Stature.........................................................................................................34 Endocrine Disorders.............................................................................................34 Hyperparathyroidism............................................................................................35 Tanner Stages....................................................................................................... 36 Puberty................................................................................................................. 36 32 E N DOCRI N O LO G Y DIABETES Must meet 1 of 4 criteria. • Symptoms of diabetes (polydipsia, polyuria, weight loss) AND random BG>200 • Fasting BG >126 • 2 hour post prandial (OGTT) >200 • HbA1C >6.5% New onset labs: chem panel, HbA1C, islet cell antibodies, insulin antibodies, thyroid antibodies, TFTs, endomesial antibody, TTG, IgA, b-hydroxybutyrate, serum insulin and C peptide, Call ENDO ON CALL for recommendations for starting subcutaneous insulin Record carbs, insulin, and BG on blank diabetes chart found on the ward HYPOGLYCEMIA BG<40-50mg/dl Draw critical labs at the time of hypoglycemia: BG, insulin, GH, cortisol, beta hydroxybutyrate, UA, AST, ALT, electrolytes, VBG Treatment: • If patient is conscious and can take food by mouth, administer 40% Glucose Gel. Single dose equal to 0.5g/kg to max of 15 grams (1 tube) . • If patient is NPO, administer 5ml/kg of D10% to max of 100 ml. Dose is given IV push and can be repeated in severe cases. • Check finger stick glucose every 15 minutes and repeat treatment until blood glucose is >80 mg/dl. ADRENAL INSUFFICIENCY • Stress Dose for Illness: 25-50 mg/m2/day of hydrocortisone IV/IM as a continuous drip or divided q3-6hr • Stress Dose for Surgery or Severe illness: hydrocortisone 50-125 mg/m2/day IV Use a med calc to get BSA (mg/m2) E NDOC R INOLOGY 33 SHORT STATURE Midparental Height Bone Age Growth Velocity Idiopathic Normal Normal Normal Familial ↓ Normal Normal Constitutional Delay Normal ↓ Normal Endocrine* Normal ↓ ↓ Chronic illness malnutrition Normal ↓ ↓ * e.g. hypothyroidism, Cushings, GH deficiency ENDOCRINE DISORDERS Disorder Diagnostic Studies and Lab Findings Diabetes Insipidus CMP, UA, serum and urine osm, possible H2O deprivation test (consult: endo) • Urine SG <1.005 • UR Osm 50-200 • Urine output > 4 ml/kg/hr CMP, UA, serum and urine osm, urine Na • Hyponatremia • Low serum osm SIADH • High urine osm • High urine Na > 40 Hypoparathyroidism ↓ serum Ca2+ ↑ serum phos Normal or ↓ alk phos ↓ 1,25-hydroxy-vit. D3 ↓ PTH (nl or ↑ in pseudohypoparathyroidism) Hyperparathyroidism ↑ serum Ca2+ ↓ serum phos Normal or ↓ alk phos ↑ 1,25-hydroxy-vit. D3 ↑ PTH (nl or ↑ in pseudohypoparathyroidism) CAH (21-hydroxylase deficiency) ↓ ↑ ↑ ↓ ↑ ↓ 34 serum Na+ & Clserum K+ renin cortisol androgen/cortisol precursors glucose E N DOCRI N O LO G Y HYPERPARATHYROIDISM Serum Primary Secondary Tertiary Calcium ↑ ←→ or ↓ ↑ Phosphorous ↓ ↑ ↑ Alkaline Phosphatase ↑ or ←→ ↑ or ←→ ↑ or ←→ Calcium ↑ ↓ Phosphorous ↑ ↓ Urine E NDOC R INOLOGY 35 TANNER STAGES Female Male Pubic Hair 1 Pre-pubertal. No breast tissue Pre-pubertal Pre-pubertal 2 Areolar enlargement, breast bud Testes enlarge (4ml); scrotum larger, skin reddened and coarser Sparse, downy hair 3 Enlargement of breast and areola as single mound Penis elongates, continued growth of testes Sparse, coarse hair and scrotum 4 Projection of areola above breast as double mound Growth of testes, penis length & breadth. Adult type hair, not on thighs Scrotum has increased pigmentation 5 Adult. Areola is a part Testes, scrotum, penis of breast contour, only adult size nipple projects Adult type hair, spread to medial thighs PUBERTY Thelarche Precocious Delayed Work up < 8 yr >13 yr LH, FSH, estradiol, testosterone, bone age, growth chart Testicular enlargement < 9 yr 36 > 14 yr E N DOCRI N O LO G Y NOTES E NDOC R INOLOGY 37 SECTION 6 Gastroenterology Diarrhea/stool replacement (for short gut or age)..............................................39 Oral rehydration therapy......................................................................................39 Elevated AST/ALT..................................................................................................39 H. Pylori.................................................................................................................39 Metabolic workup.................................................................................................39 Infant nutrition...................................................................................................... 40 Failure to thrive...................................................................................................... 41 TPN guidelines...................................................................................................... 41 TPN and re-feeding...............................................................................................42 TPN advancements...............................................................................................43 TPN monitoring.....................................................................................................43 Re-feeding syndrome...........................................................................................43 Inflammatory bowel disease/bloody diarrhea (initial workup)...........................43 Constipation......................................................................................................... 44 GI bleed.................................................................................................................45 Hyperbilirubenemia............................................................................................. 46 38 GA ST ROE NT E RO LO G Y DIARRHEA/STOOL REPLACEMENT (FOR SHORT GUT OR AGE) • Initial Mgmt: bowel rest initially, then restart with eliminating excessive sugar from diet(juice, fruit, soda) and add yogurt to reg. diet. Milk is okay. • Report stool output in mL/kg/day. If > 30 mL/kg/d, consider replacing, • For stool output > 5gm/kg/4 hours, replace 1ml fluid : 1gm stool with ½ NS + 20 meq/L K Acetate (Or NaCO3 if K elevation). Send stool studies, stool electrolytes, osm •*Do not exceed maintenance fluids or risk hyperK (max dose of K = 0.15 meq/kg/hr). Calculate and write a max dose/rate with your order! ORAL REHYDRATION THERAPY • 1 Liter Water + 6 tsp sugar + ½ tsp salt • Add ½ cup OJ or 1 mashed banana for added potassium • Or Pedialyte ELEVATED AST/ALT • DDX: idiopathic, trauma, structural anomalies, infectious, inflammatory, autoimmune, medication related, and familial or genetic disorders, toxic ingestion • Labs: • Initial: CMP, Dbili, GGT, Coags, EBV and CMV titers, acute viral hepatitis panel, urine tox, acetaminophen level • Extended: ferritin (hemochromatosis), ANA, total IgG, anti-smooth muscle Ab, Anti-LKM1 Ab (autoimmune hepatitis), celiac panel, serum copper, ceruloplasmin (Wilson’s), alpha-1 antitrypsin phenotype. • Imaging: Abdominal ultrasound with Doppler (NPO x 8 hrs) Consider liver biopsy H. PYLORI • Lab: stool H.pylori antigen (NOT the serum antibody); falsely negative if on PPI or bismuth • Treatment: PPI up to 20 mg BID, Clarithromycin 15 mg/kg/day up to 500 mg BID plus amox 50 mg/kg/day up to1g BID OR metronidazole 20 mg/kg/day up to 500 mg BID. • Refer for EGD/Breath urea test if necessary (depending on symptoms, regardless of Lab results) GA ST R O E NTEROLOGY 39 METABOLIC WORKUP • Initial Labs: VBG, CBC, Chem18, NH4, Serum amino acids, UA, Urine organic acids, acylcarnitine profile lactate, ESR, CRP, CK, Coags • Check Newborn screen (If not in military call see http://www.babysfirsttest.org/newborn-screening/ states) and call appropriate stated. Give them mothers last name, birth hospital and date of birth INFANT NUTRITION WEIGHT GAIN EXPECTATIONS Small Premie 15 gm/day 0-3 months 15-30 gm/day 3-6 months 15-20 gm/day 6-12 months 5-8 gm/day 1-6 years 5-8 gm/day 7-10 years 5-11 gm/day MIXING INFANT FORMULAS 20 cal/oz 1 scoop 2 oz 24 cal/oz 3 scoops 5 oz 26 cal/oz 2 scoops 3 oz 28 cal/oz 7 scoops 10 oz 30 cal/oz 3 scoops 4 oz CALORIC REQUIREMENTS BY AGE Age Calories/kg Protein 1-6 months 108 kcal/kg/day 2.2 gm/kg/day 6-12 months 98 kcal/kg/day 2.0 gm/kg/day 1-3 years 102 kcal/kg/day 1.2 gm/kg/day 4-6 years 90 kcal/kg/day 1.1 gm/kg/day 7-10 years 70 kcal/kg/day 1.0 gm/kg/day 40 GA ST ROE NT E RO LO G Y FAILURE TO THRIVE • Definition: Wt < 2%tile for correct gest. age and sex more than once, crossing more than 2 major weight %tiles, etc • Etiology: Insufficient caloric intake (GERD, underfeeding, improper formula mixing, oral/motor discoordination/aspiration, etc); increased metabolic demand (cardiac dz, metabolic/genetic, RTA, other chronic illness), increased output (malabsorption, milk protein allergy, chronic diarrhea, CF, etc) • Labs: CMP, CBCD, UA , TFTs (>1 yo), Celiac panel (>1 yo), IGF-1 (After failure of dietary counseling with increased calories) • Extended, pre-albumin, zinc, selenium, celiac panel; consider metabolic/genetic • Stool studies: stool fecal fat, reducing substance, alpha-fetal protein, fecal elastase. • Imaging: KUB, UGI if persistent emesis to r/o malro, CT Head for possible increased ICP • Daily weights, strict I/O, Nutrition (calorie count), possible NG placement, monitor for re-feeding, follow up newborn screen TPN GUIDELINES Neonates Per 24 hours Preterms less than 3 kg Infants & Children Full term 10 kg 10-20 kg greater than 20 kg 1000 mL - Add 50 mL/kg for each extra kg greater than 10 kg Adolescents 1500 mL - Add 2025 mL/kg for each extra kg greater than 20 kg Fluids (mL/kg) 100-200 100-150 100125 Calories (Kcal/kg) 70-120 greater than 100 75-90 75-90 greater than 40 30-50 Protein (g/kg) Max Perip: 2 g/kg/day Max Central: 3.5 g/kg/ day 2.5-3.5 2-2.5 2-2.5 1.5-2.5 1.5-2.5 1-2 Dextrose (%) Max Periph: 12.5% Max Central (30%) 5-25 5-25 5-30 5-30 5-30 5-30 Fat: (g/kg/day) 0.5-3 1-3 1-3 1-3 1-3 1-3 Neonates Vitamins (mL/day) MVI - Peds (Contains Vitamin K = 0.2 mg/5 mL) Infants/Children less than 1 kg 1-2.5 kg greater than 2.5 kg & less than 11 years 1.5 mL 3.25 mL/ day 5 mL/day GA ST R O E NTEROLOGY Children/Adult greater than or equal to 11 years 41 TPN AND RE-FEEDING Pediatrics Standard Adult Solution (3L) *Sodium 2-5 mEq/kg/day 100 mEq/day Potassium 2-5 mEq/kg/day 100 mEq/day Chloride 2-5 mEq/kg/day 100 mEq/day Phosphate (as K+) 1-4 mEq/kg/day 45 mEq/day Calcium Gluconate (Maximum peripheral conc = 2 mg/mL) 100-500 mg/kg/day 3000 mg/day Magnesium 0.25-0.5 mEq/kg/day 24 mEq/day * by Definition NS = 154 mEq/L; 1/4 NS = 38.5 mEq/L Trace Elements Infants greater than 3 months and children Neonates Preterm Peds Multi-Trace 0.2 mL/kg Additional Zinc (mcg/kg) *300 0-3 months Average 0.2 mL/kg (max 10 mL/day) 150 * (Premature infants weighing less than or equal to 3 kg require an additional 300 mcg/kg/day of zinc for total of 400 mcg/kg/day) PEDIATRIC MULTI-TRACE ELEMENTS — 0.2 ML/KG/DAY PROVIDES: Element Dose Normal Range Zinc 100 mcg/kg/day 100-300 mcg/kg/day Copper 20 mcg/kg/day 15-30 mcg/kg/day Chromium 0.17 mcg/kg/day 0.14-0.2 mcg/kg/day Manganese 42 5 mcg/kg/day 2-10 mcg/kg/day GA ST ROE NT E RO LO G Y TPN ADVANCEMENT • TPN should be calculate based on IBW not admit weight • Increase dextrose 2.5 - 5% per 24 hours. • Increase protein and fats 0.5 - 1g / kg / day, over 3 - 4 days. GOAL: Maximum of 60% total calories from fat. • If pt losing wt on TPN needs more calories even if at goal TPN MONITORING • Initiation/unstable: daily Chem10, TG; Twice/week: LFTs, Daily weights • Monitor TG as the dose increases and keep below 100 - 250 mg / dL • Check TG after lipids are off for 4 hours • Stable: Twice/week BMP; weekly Mg, Phos, TG, LFTs RE-FEEDING SYNDROME • Severe fluid/electrolyte shifts in malnourished patients undergoing oral, enteral, or parenteral refeeding after prolonged fasting/poor feeding • HypoPhos, hypoK, hypoMg, hyperglycemia • Monitor lytes BID x 3d when initiating nutrition • Correct electrolyte abnormalities and progress diet slowly until goal protein/energy intakes achieved INFLAMMATORY BOWEL DISEASE/BLOODY DIARRHEA (INITIAL WORKUP) • Initial workup: CXR, KUB, CBCD, ESR, CRP, CMP, DBili, GGT; consider type/cross with retic count if significant hematochezia • Stool labs: FOBT, CDiff, culture w/Yersinia, WBC • Other: PPD, CXR, Quantiferon • Initial management: NPO + IVF; may need clean out for endoscopy (see constipation section GA ST R O E NTEROLOGY 43 CONSTIPATION Mild: Functional constipation without impaction 1. Increase fluids (consider prune /pear juice, 2-4 oz/d) 2. Regular toilet time (BID x 5-10 min after meals) 3. Benefiber: - 6 – 11 yrs 1 t s p TID ( 4.5 gm/day) OR ½ - 1½ chewable tabs 3-5 times/day > 12 yrs 2 tsp TID (9 gm/day) OR 1 -3 chewable tabs 3 -5 times/day Moderate 1. Miralax (polyethylene glycol): Most ages ½ - 1 cap, 1-3 times/day – titrate for effect 2. Milk of Magnesia: teens- 1 mL/kg/ day (conc. 400mg/5mL) - maximum dose = 30 mL/day OR 0.5 mL/kg /day (conc. 800mg/5mL) - maximum dose = 15 mL/day 3. Senokot: - 2-6 yrs: 2.5-7.5 mL/day - 6-12 yrs: 5-15 mL/day (syrup 8.8 mg/5 ml) 4. Dulcolax: 3-12 yrs: 5-10 mg or 0.3 mg/kg/day PO >12yrs: 5-15 mg/day single dose PO; 39 mg max 5. Mineral Oil: over 1 yr: 5-30 mL/day BID *DO NOT USE with children with neurological impairments or seizure disorder due to risk of aspiration pneumonia 6. Lactulose: Infants-1ml/kg/day in divided doses Severe: Need for Disimpaction or clean out 1. Miralax Clean-out: - Mix 238 g in 64 oz non-red fluid - Give 8 oz every 20-30 min until complete - Follow with 5-10 mg Dulcolax po x 1 2. Golytely Clean-out: - Place NG, get KUB to confirm placement - Start 50ml/hr x1 hr then advance to 100ml/hr x1 hr then to max rate of 200ml/hr as tolerated. Stop after max volume given. May consider additional dose if lytes stable and stools not yet clear. - Max Doses: 10-20kg 1.5L; 20-30kg 2L; 30-40kg 3L; 40-50kg 3.5L; >50 kg 4L - Check BMP, Mg, Phos qday while getting GoLytely - Clears ad lib + MIVF during clean out - Continue until clear effluent 1. Soap suds enema: 100ml/year of life up to 1 liter 44 GA ST ROE NT E RO LO G Y GI BLEED History and Physical Exam Essential History: History of Bleeding/Coagulopathy/Gastroenteropathy/Ulcer/Liver Disease, Frequency of Melena/Hematemesis Current Medications Physical Exam: Vital Signs (Orthostatics), Pallor, Jaundice, Hepatosplenomegaly Labs: CBC Including including platelet counts, PT/PTT, Type and Screen, Liver Function Tests NG/Gastric Aspiration to determine site of bleed (verify upper location) & lavage STABLE UNSTABLE Discuss with Attending Consider EGD Admt for observation? Admit to ICU CArdiopulmonary Monitor NPO/IV access x 2 Fluid/blood product resuscitation Octreotide bolus and drip IV pantoprazole Frequent Hemoglobin/Hct monitoring q4 Unstable: If requiring > 85 cc/kg of transfusion, CONSULT SURGEON. Set up for EGD with surgery present GA ST R O E NTEROLOGY Stable: EGD 45 HYPERBILIRUBENEMIA Guidelines for phototherapy in hospitalized infants of 35 or more weeks gestation. Guidelines for exchange transfusion in hospitalized infants of 35 or more weeks gestation. 46 GA ST ROE NT E RO LO G Y NOTES GA ST R O E NTEROLOGY 47 SECTION 7 Heme/Onc Anemia.................................................................................................................. 49 Pancytopenia work-up........................................................................................ 49 Transfusion guidelines......................................................................................... 50 Sickle cell care...................................................................................................... 51 Fever and Neutropenia.........................................................................................52 Tumor lysis syndrome..........................................................................................53 48 HE M E/ O N C ANEMIA Corrected reticulocyte index = % retics x (patient Hct / normal Hct) >1.5 suggests increased RBC production from hemolysis or blood loss CLASSIFICATION OF ANEMIA Retic count microcytic normocytic macrocytic low Iron deficiency, lead poisoning, anemia of chronic disease, aluminum toxicity, copper deficiency, protein malnutrition, inflammation Chronic disease, RBC aplasia(TEC, infection, drug induced), Malignancy, juvenile rheumatoid arthritis, endocrinopathies, renal failure, splenomegaly Folate deficiency, vitamin B12 deficiency, aplastic anemia, congenital bone marrow dysfunction, drug induced, trisomy 21, hypothyroidism normal Thalassemia trait, sideroblastic anemia Thalassemia trait, sideroblastic anemia, acute bleed Normal newborn, trisomy 21, drug induced Thalassemia syndromes, hemoglobin C disorders Anti-body mediated hemolysis, hypersplenism, microangiopathy (HUS,TTP, DIC, Kasabch-Merrit), membranopathies (spherocytosis, elliptocytosis), enzyme disorders (G6PD, pyruvate kinase) hemoglobinopathies Dyserythropoietic anemia I,III; active hemolysis high PANCYTOPENIA WORK-UP 1. CBC W/ manual diff, retic panel, chem 18 (CMP, LFT), LDH, DIC panel, CXR, other labs as clinically indicated. 2. CALL HONC ATTENDING. HE ME /O NC 49 TRANSFUSION GUIDELINES Cryoprecipitate •Dose: 1 unit: approx.. 250 mg fibrinogen. Rate: As fast as tolerated •Indications: raise fibrinogen when a small volume is needed. 3.To achieve desired HCT: volume of pRBC’s (ml) = EBV (ml) X (desired HCT- actual HCT)/HCT of pRBC’s EBV- estimated blood volume ( see harriet lane for values) Fresh Frozen Plasma Ordering Packed Red Blood Cells: •Dose: 10-30 mL/kg •Leukoreduced •Contains all factors and albumin, but no platelets •Rate: as indicated by clinical situation •Indications: see sharepoint Single Donor Platelet Pheresis (SDPP) •Dose: 5-10 mL/kg, raises platelet count by 40,000 – 60,000 (for 10ml/kg) •Rate: as fast as tolerated (30 minutes to 1 hour) •Transfusion goals: 1. Placing lines > 20, 000 2.Lumbar puncture > 50,000 3.DIC > 20, 000 4.Normal maintenance > 10,000 5.Brain lesion or major surgery > 100,000 6.ITP- NEVER GIVE PLATELETS WITHOUT DISCUSSING CASE WITH ATTENDING Packed Red Blood Cells •Dose: 10 mL/kg ( if hgb is less than 6, use numerical Hgb value as starting dose. Ex. If hgb is 4, first transfusion dose should be 4ml/ kg); 10 mL/kg raises Hgb by 3 g/dl •Rate: 2-5 mL/kg/hr, or mainly 10 mg/kg over 2-4 hours •Indications: see sharepoint 1. If hGb < 4.0 call HONC attending and transfer to PICU 2.Goal of transfusion > 7.0 or as clinically indicated 50 •All blood products need to be leukoreduced and CMV safe. •Irradiated •All neonates < 4 months •Severe known or suspected immunodeficiency or immunosuppression(chemo, radiation, stem cell transplant) •Donor-directed blood products •CMV Seronegative •Infants < 1000gm •CMV negative/pending patient: •receiving stem cells from CMV negative donor •AML or aplastic anemia •high-risk neuroblastoma •congenital or acquired immunodeficiency syndrome •CMV+ patient with low or undetectable viral load receiving stem cells from a CMV neg donor •Phenotype Matched •Sickle cell, Thalassemia, other chronic anemias (dicuss with heme/onc first) •Washed RBCs •Cardiac patients < 4 kg (non-pump surgery or “push” transfusion) •GI surgery patients < 5kg •History of serious febrile or allergic transfusion reaction •IgA deficiency •Kidney failure, Liver failure HE M E/ O N C SICKLE CELL CARE ACUTE PAIN CRISIS •Labs on admission: CBC w/ manual diff, retic panel, chem 18, blood culture, consider ordering type and cross for 2-4 units and possible post-transfusion electrophoresis •Orders on admission: •Ketorolac q6h or other NSAID •Oral opioid, consider PCA pump if pain is not well controlled •1.5 X IVMF •CXR •Heating pad •Incentive spirometry •Miralax if pt will be receiving heavy opioids •Antihistamine for pruritis •ALWAYS CHECK FOR PRIAPISM: SURGICAL EMERGENCY IF PRESENT FOR > 4 HOURS ACUTE CHEST SYNDROME Most common cause of death in SCD. The development of a new pulmonary infiltrate associated with fever, chest pain, hypoxia, tachypnea, and cough. Generally caused by infection, infarction, or fat embolization. •Acute Chest Syndrome Orders: •STAT CXR- repeat CXR with any with any respiratory status changes •Ketorolac Q6H or other NSAID; also consider opioid if pain is not well controlled •1.5 X IVMF •Transfuse- 10-20 ml/kg of pRBC’s (see transfusion guidelines) and recheck Hgb 2-4 hours post-transfusion. Post transfusion should not exceed Hgb > 12 (see formula in transfusion guidelines) •Start broad spectrum (cefepime) and a macrolide (azithromycin) •Give bronchodilators if wheezing •Incentive spirometry •Miralax if giving lots of opiates •Antihistamine for pruritis •Consider steroids •ALWAYS CHECK FOR PRIAPISM: SURGICAL EMERGENCY IF PRESENT FOR > 4 HOURS HE ME /O NC 51 FEVER AND NEUTROPENIA Fever and neutropenia is the most common admission for heme/onc that you will get overnight. Be aware that a neutropenic patient may not have a normal inflammatory response and could become septic without signs of fever or other classical signs of infection. PE should include dental, nail beds, perirectal examination, line site visualization. No rectal exams or temperatures. Generally things that you should do: •CXR •Urine culture, blood culture (from port- each lumen) •Cefepime unless there is a specific indication for another antibiotic- add vancomycin for cellulitis, PNA, or ill-appearing upon presentation •Chem10, LFT’s, and CRP •Tylenol for fever/pain- No motrin until platelets return to greater than 150,000 •IVMF •Consider LP if any signs of mental status changes (not routine) •Q24H Bcx for continued fever relapse 52 HE M E/ O N C TUMOR LYSIS SYNDROME Etiology: Lysis of tumor cells before or during early stages of chemotherapy. Most commonly seen in lymphomas and leukemias. Worry about renal failure. Check chem 10, LDH and uric acid q6h. Labs abnormalities Start treatment Management hyperurecemia ≥ 8.0 Or Any 25% increase from baseline - Alkalinization: D5 ½NS + 40mEq/L NaHCO3 at 1.5- 2.0 xMIVF - Allopurinol 200mg/m2/day divided Q8 IV - If Uric Acid still rising: Rasburicase 0.2mg/kg/day IV infusion over 30 mins (no alkalinization required) – Call attending before starting! hypocalcemia <8.0 Or Any 25% decrease from baseline 1. Correct for albumin 2. Check ionized calcium 3. Calcium Carbonate (Tums) 2-3 tabs QID (max 15 tabs/day) 4. If emergent: Calcium Gluconate 100mg/kg IV hyperkalemia >7.0 Or Any 25% increase from baseline 1. Stop K in fluids 2. EKG – if no changes, go to Kayexalate; if changes or K >7.0: 3. Calcium Gluconate (heart protection) 100mg/kg IV 4. Insulin (0.1U/kg IV) + Glucose (D25W 2ml/kg) over 30 min 5. Beta-agonist: Albuterol nebs 6. Kayexalate 1g/kg/dose PO Q6 hyperphosphatemia >6.0 Consider NS bolus and mannitol >8.0 Or Any 25% increase from baseline If not responding to fluidsRenagel 120mg/kg/day HE ME /O NC 53 NOTES 54 HE M E/ O N C SECTION 8 Infectious Disease Croup pathway..................................................................................................... 55 Admission for croup.............................................................................................57 Rule out serious bacterial infection.................................................................... 58 Community acquired pneumonia pathway........................................................ 59 Influenza................................................................................................................ 60 Endocarditis.......................................................................................................... 61 Urinary tract infection........................................................................................... 61 Meningitis............................................................................................................. 63 Intrauterine & perinatal infections (TORCH)........................................................67 Zoonotic exposure............................................................................................... 68 Septic joint............................................................................................................ 69 Acute otitis media treatment algorithm for children 6 months-12 years of age.................................................................................. 70 CROUP PATHWAY Croup occurs most commonly in children 6 months to 3 years of age but may occur in children as young as 3 months and as old as 12 to 15 years of age. Inclusion criteria •Age 3 months to 6 years old •Able to control secretions INF E C T IO U S DISEASE 55 •Moderate stridor with mild to moderate respiratory symptoms and upper respiratory symptoms compatible with croup •Alternate diagnosis not suspected (FB, anatomical abnormaility, etc.) Croup Symptoms •Barky cough •Inspiratory stridor •Hoarseness •No to moderately high fever •Symptoms usually improve during the day, exacerbate at night •Majority of children resolve croup symptoms within 48 hours •May or may not have antecedent cough, rhinorrhea or fever Impending respiratory failure suggested by •Changes in mental status such as lethargy, fatigue or listlessness •Pallor, dusky appearance, or cyanosis •Decreasing retractions or decreasing breath sounds with decreased stridor Radiologic Pearls •Croup - “steepling” of the subglottic area instead of normal square shoulder appearance on AP neck radiograph •Bacterial tracheitis – ragged edge or membrane spanning the trachea •Epiglottitis - thickening of epiglottis and aryepiglottic folds “thumbprint” appearance •Retropharyngeal abscess – bulging of the posterior pharynx soft tissues Differential diagnosis •Epiglottitis •Foreign body •Retropharyngeal abscess (before 3 to 4 years old) or peritonsillar abscess •Hereditary angioedema •Subglottic stenosis •Infectious mononucleosis •Diphtheria •Extrinisic (hematoma) or intrinsic (tumor or cyst) obstruction of airway •Trauma 56 I N F E CT I OU S DI S E AS E ADMISSION FOR CROUP INF E C T IO U S DISEASE 57 RULE OUT SERIOUS BACTERIAL INFECTION 58 I N F E CT I OU S DI S E AS E COMMUNITY ACQUIRED PNEUMONIA PATHWAY Diagnostic Studies: •Order studies only as anticipated to contribute to therapeutic decisions •For outpatient, no need for CXR or BCx •CXR (PA/Lat) for In-Pt management to document the presence, size, and character of parenchymal infiltrates and identify complications of pneumonia •A chest radiograph interpretation cannot be relied upon to distinguish between viral and bacterial etiologies •Blood cultures should be obtained in children requiring hospitalization for presumed bacterial CAP that is moderate to severe, particularly those with complicated pneumonia •CBC, CRP or ESR not necessary to empirically treat, nor to differentiate viral vs bacterial •In selected older children, sputum Gram stain and culture might be helpful •Nasopharyngeal bacterial cultures are not usually helpful •If you are considering pertussis, obtain a nasopharyngeal swab or aspirate of the posterior nasopharynx •Laboratory confirmation of a viral etiology (e.g., RSV) can occasionally be helpful •The incidence of bacterial co-infection with RSV pneumonia is low, so when the RSV test is positive, generally antibiotics are not indicated •Remember to consider TB and the intradermal TB skin test, or interferon gamma release assay (Quantiferon) if >4y old •PCR, serologies and acute phase reactant studies are not usually helpful Admission Factors: •Age < 6 months •SpO2 <92% or marked respiratory distress •Dehydration with inability to take PO fluids/antibiotics •Toxic appearance •complications (empyema/effusions) •Failed oral antibiotics (48-72hrs) •Comorbid conditions/ immumocompromised •Unstable social situation; non-adherence Outpatient Antibiotic Therapy for suspected Bacterial PNA (Community Acquired) •3 mo - 5 years: Amoxicillin 90mg/kg/day BID x 7-10 days •≥ 5 years: Azithromycin 10mg/kg/day (Day 1), then 5mg/kg/day (Day 2-5) •+/- Amoxicillin as above INF E C T IO U S DISEASE 59 Parenteral empiric antibiotics for inpatient treatment of pediatric pneumonia •Bacterial and fully immunized-Ampicillin (150-200mg/kg/day div q6hr) •Bacterial and partially immunized-Ceftriaxone (50-100mg/kg/day div q12-24hr) •If atypical is suspected- Azithromycin (10mg/kg/day, then 5mg/kg/day ) •If complicated or suspect abscess- Clindamycin (30-40mg/kg/day div q6-8hr) , or Vancomycin (60mg/kg/day div q6hr) if severe disease INFLUENZA 60 I N F E CT I OU S DI S E AS E ENDOCARDITIS Diagnostic criteria 2 major criteria, or 1 major criteria and 3 minor criteria, or 5 minor criteria Major criteria •2 separate positive blood cultures positive for endocarditis organisms. Cultures must be drawn >12 hours apart: Staph aureus,Viridans strep, Strep bovid, HACEK group, enterococci in absence of primary focus •Single positive blood culture for Coxiella burnetti •Echocardiogram evidence of vegetations on valves or implanted devices Minor criteria •Predisposing heart condition •Fever •Vascular phenomena: arterial emboli, septic pulmonary infarcts, mycotic aneurysm, intracranial hemorrhage, conjunctival hemorrhage, Janeway’s lesions •Immunologic phenomena: glomerulonephritis, Osler’s nodes, Roth’s spots, rheumatoid factor •Micro evidence: positive blood culture for organism not associated with endocarditis URINARY TRACT INFECTION Clinical scenario: Consider UTI in any child < 2 years with fever; symptoms non-specific include abdominal or flank pain, strong-smelling urine, new urinary incontinence, dysuria, urgency, nausea/vomiting, diarrhea Additional History: stooling pattern, prenatal ultrasound, potty-training and wiping, sexual intercourse/abuse, circumcision, recent antibiotics; FHx VUR, recurrent UTI, CKD Diagnosis: requires positive pure culture; pyelonephritis vs cystitis is clinical (fever, systemic symptoms, CVA tenderness); febrile infants assumed to have pyelonephritis UA: positive LE suggestive of WBCs; positive nitrite consistent with urease producing gramnegative bacteria (E. Coli, Klebsiella, Proteus), but poor sensitivity in infants who empty bladder frequently INF E C T IO U S DISEASE 61 Collection Method Colony Count Probability of Infection Suprapubic Gram-negative: any number >99% Aspiration Gram-positive: >1,000 Transurethral >100,000 95% Catheterization 10,000-100,000 Infection likely 1,000-10,000 Suspicious, repeat <1,000 Infection unlikely Clean-voided (boy) >10,000 Infection likely Clean-voided (girl) > 100,000 80-95% 50,000100,000 Suspicious, repeat 10,000-50,000 Infection unlikely if no symptoms <10,000 Infection unlikely Common Pathogens E. coli (75-90%) Klebsiella Proteus Staph saprophyticus Staph aureus *Consider enterococcus and pseudomonas in abnormal host. *Consider GBS or other blood-borne pathogens in neonates Admission criteria: Require IV fluids, Those who require Iv antibiotic due to severe illness or failure of po antibiotics, <30 days, 31-60 days and high risk 62 I N F E CT I OU S DI S E AS E Treatment: * Prophylaxis may not prevent recurrent febrile UTI; no specific recommendation in current AAP guideline, RCT (Randomized Intervention for Children with Vesicoureteral Reflux) in progress. Consider prophylaxis for VUR Grade 3 or higher: < 2mo: Amoxicillin 20mg/kg daily, > 2mo: TMP/ SMX 2mg/kg daily or Nitrofurantoin 2mg/kg daily (depending on allergies) * VCUG should not be performed routinely after first febrile UTI; VCUG indicated if RBUS reveals hydronephrosis, scarring, or other findings suggestive of high-grade VUR or obstructive uropathy Urinary Tract Infection: Clinical Practice Guideline for the Diagnosis and Management of the Initial UTI in Febrile Infants and Children 2 to 24 Months (Pediatrics 2011) MENINGITIS Hx & Sx •Infants: change in temp, irritability, lethargy, poor feeding, high-pitched cry, vomiting, bulging fontanelles, seizure •Children: neck pain, HA, N/V, photophobia Risk Factors •Assess recent sinusitis, AOM, antibiotic use, sick contacts, recent trauma, h/o neurosurg or VP shunt. •Functional asplenia, sickle cell disease, nephrotic syndrome, IgG •Deficiency: INC risk of encapsulated S. pneumo, Hib, N. meningitidis •Complement deficiency - N. meningitidis risk •CSF leak / head trauma- risk of strep or staph •VP shunt: increased risk of Staph. epidermidis (coag neg) or P. acnes INF E C T IO U S DISEASE 63 PE findings •Check fontanelle, FOC, papilledema, CN palsy, signs of head trauma, e/o otitis media, sinus infection, neck pain/stiffness •Petechial / purpuric rash assoc with meningococcus. •Kernig sign: inability to straighten the knee with hip flexed due to pain •Brudzinski sign: passive neck flexion that leads to hip flexion due to pain Treatment •Based on clinical suspicion and patient age •If HSV is possible, give Acyclovir until HSV PCR is neg •Prophylaxis needed for N. meningitidis or Hib with rifampin, Cipro or CTX Complications •Hypovolemia or hyponatremia 2/2 SIADH, sepsis, DIC, seizures, cerebral edema, herniation, stroke, subdural effusions •Sensorineural hearing lost is most common COMMON ORGANISMS Viral: Enterovirus (90%), HSV, EBV, CMV, VZV, arbovirus (EEE, West Nile), Influenza A/B Bacterial: by age Neonate 1-3mo 3mo-12yr >12yr GBS GBS S. pneumonia N. meningitidis E. coli S. pneumonia N. meningitidis S. pneumonia GNR Listeria Listeria N. meningitidis Salmonella Tuberculous: Often indolent HA, low grade fever, may have CN palsy. *In unvaccinated children, think of uncommon organisms as well* 64 I N F E CT I OU S DI S E AS E RECOMMENDATIONS FOR ANTIMICROBIAL THERAPY IN ADULT PATIENTS WITH PRESUMPTIVE PATHOGEN IDENTIFICATION BY POSITIVE GRAM STAIN Organism Recommended Treatment Alternative S. pneumoniae Vanco+ CTX or Cefotax Mero, Fluoroquinolone N. meningitidis CTX or Cefotax PCN G, Amp Listeria Amp TMP-SMX, Mero S. agalactiae Amp CTX or Cefotax H. influenza CTX or Cefotax Cefepime, Mero E. coli CTX or Cefotax Cefepime, Mero, TMP-SMX In children, ampicillin is added to the standard therapeutic regimen of cefotaxime or ceftriaxone plus vancomycin when Listeria is considered, and to an aminoglycoside if a gram-negative enteric pathogen is of concern EVALUATION OF CEREBROSPINAL FLUID Age WBC count (median) 95%ile 0-28 d 0-12 (3) 19 29-56 d 0-6 (2) 9 Child 0-7 n/a Glucose mg/dl mmol/L Preterm 24-63 1.3-3.5 Term 34-119 1.9-6.6 Child 40-80 2.2-404 Protein mg/dL g/L Preterm 65-150 0.65-1.5 0-14d 56-102 0.56-1.02 15-28d 49-89 0.49-0.89 29-42d 41-75 0.41-0.75 43-56d 36-70 0.36-0.70 Child 5-40 0.05-0.4 Opening Pressure Newborn 8-11 cm H2O 1-18yrs 11.5-28 cm H2O INF E C T IO U S DISEASE 65 HSV DISEASE IN NEONATES Disseminated Disease •25 percent of neonatal disase •CNS involvement in 60-75% disseminated cases •Sepsis-like state around day 7 to 14 of life •Hepatitis and pneumonitis are common findings •Death from severe coagulopathy, liver dysfunction, and pulmonary involvement CNS Disease •One-third of all neonates with HSV infection •Seizures (both focal and generalized), lethargy, irritability, tremors, poor feeding, temperature instability, and bulging fontanelle •Presentation is usually around day 14 to 21 of life •Can be indistinguishable from other viral and bacterial meningitis •Should be considered in young infants evaluated for sepsis when bacterial cultures are negative at 48 to 72 hours and the infant has experienced a worsening or lack of improvement clinically •Mortality usually is the product of devastating brain destruction neonatal HSV can involve any and often multiple parts of the brain SEM Disease •Infection localized to the skin, eye, and/or mouth •20-45% of all cases of neonatal HSV disease •Presentation usually around day 7 to 14 of life •By definition, limited infection •80-85% have vesicular lesions on physical examination Risk Factors •Type of maternal infection (primary > recurrent) •Maternal antibody status (pos Hx = Abs) •Duration of rupture of membranes •Integrity of mucocutaneous barriers (fetal scalp electrodes) •Mode of delivery (vaginal > cesarean) Work Up & Treatment •Surface cultures & PCR •Single swab (Eyes, Nares, Mouth, Lesions, Rectum) •General Viral Culture-red top, pink media (Mailout) •Serum labs •LFTs (in house) and Serum HSV PCR (Mailout) 66 I N F E CT I OU S DI S E AS E •Alternative is Plasma HSV PCR (Local mailout, ~24hr turnaround time) •CSF Labs •CSF HSV PCR (Mailout) •Acyclovir 60mg/kg/day div q8hr, 21days for disseminated and CNS disease (CSF PCR negative prior to stopping), 14 days for SEM. •Oral acyclovir suppression for 6 months after IV therapy completed INTRAUTERINE & PERINATAL INFECTIONS (TORCH) Organism Characteristics Prenatal Dx Postnatal Dx Treatment CMV IUGR, jaundice, HSmegaly, microcephaly, TCP, intracranial calcifications, deafness n/a Ucx/CSF Cx within 2-4wks Gancyclovir Enterovirus hepatitis, myocarditis, encephalitis, NEC, DIC n/a RNA PCR IVIG Hep B jaundice, HS-megaly, Chronic HBV Maternal HBsAg HBsAg & anti-HBs @ 9-18mo HBIG + HepB Vacc; IFN-a Hep C asymptomatic Maternal Abs IgG @ 18mo IFN-a-2b + ribarvirn HIV goal maternal viral load <1000; elective C/S at 38wks; avoid breastfeeding HIV screening rapid HIV-Ab; HIV DNA PCR Zidovudine; HSV CNS, Disseminated or SEM disease Maternal screening Culture; PCR after 24hrs Acyclovir Parvo B19 hydrops, IUGR, pleural/ pericardial effusions n/a Serum IgM; PCR Supportive, IU transfusion Rubella IUGR, cataracts, cardiac anomalies, blueberry muffin rash Maternal Abs infant IgM or nasal culture Supportive Syphilis HS-megaly, abnml bone, rash, LAD VDRL or RPR; FTA-Abs VDRL or RPR PCN G Toxoplasmosis chorioretinitis, meningitis, cerebral calcifications Parasite DNA Baby & Maternal Abs; PCR Pyrimethamine + sulfadiazine VZV rash, pneominitis, encephalitis, purpura fulinans, hypotension n/a DFA; PCR Acyclovir; VariZIG INF E C T IO U S DISEASE 67 ZOONOTIC EXPOSURE COMMON AND UNCOMMON ZOONOTIC ORGANISMS Host Common Uncommon Humans GAS, MSSA, Fusobacterium, Peptostreptococcus Eikenella, Prevotella, Porphyromonas Dog Rabies, Pasteurella Campylobacter, Eccinococcus Cat Bartonella, Pasteurella Giardia, Toxoplasma Horse Salmonella, Campylobacter, Cryptosporidium Giardia, Brucella, Coxiella Rabbit Tularemia, Babesia, Pasteurella, Ringworm Salmonella, Yersinia, Cryptosporidium Cattle/Sheep Brucella, Coxiella , M. bovis Parapoxyvirus Rodents Streptobacillus moniliformis, Spirillum minus, Leptospira Salmonella, Tularemia, Hantavirus, Yersinia Birds C. psittaci, M. avium, Salmonella, Avian Flu Cryptococcus, Histoplasma, West Nile Fish M. marinum, Aeromonas Erysipelothrix Reptiles Salmonella, Campylobacter, Yersinia Aeromonas, Pentastomiasis Exotic animals Salmonella, Campylobacter, Cryptosporidium Giardia, M. microti, Toxoplasma 68 I N F E CT I OU S DI S E AS E SEPTIC JOINT Evaluation/Treatment of the Child with Suspected Septic Arthritis *used for children, with no known trauma, who have one or more of the following •Significant joint pain with or without swelling •Significant limp or refusal to bear weight •Fever (absence of fever does not rule out septic arthritis or osteomyelitis) INF E C T IO U S DISEASE 69 ACUTE OTITIS MEDIA TREATMENT ALGORITHM FOR CHILDREN 6 MONTHS-12 YEARS OF AGE Based on the 2013 AAP Treatment Guidelines •Do not diagnose AOM if there is no middle ear effusion •Mgmt of AOM should include assessment for pain and treatment if pain is present First line treatment of choice is Amoxicillin (80-90 mg/kg/day divided BID). •Use Amox/Clav first line if: •Antibiotics within 30 days or history of recurrent AOM unresponsive to Amox •Child has AOM with conjunctivitis (likely to be H.influenzae) Treatment failures (48-72 hours after appropriate treatment): Amox/Clav (if amox used) or Ceftriaxone (3 doses 50mg/kg). Could consider Clinda with or without 3rd generation cephalosporin. Tympanocentesis for multiple treatment failures (including Ceftriaxone x 3) Alternatives for penicillin allergy include Cefdinir and Ceftriaxone. There are no official recommendations for Type I PCN allergy (could consider Clindamycin with or without Septra). Azithromycin is not recommended for any scenario in the guidelines 70 I N F E CT I OU S DI S E AS E NOTES INF E C T IO U S DISEASE 71 SECTION 9 Neurology Before Calling the Pediatric Neurologists............................................................ 73 Altered Mental Status........................................................................................... 73 Ataxia..................................................................................................................... 74 Concussion and RTP (Return to Play) Guidelines ..............................................76 Headaches and Headache medications.............................................................. 77 Seizure and AEDs................................................................................................. 80 Weakness/Peripheral neuropathy........................................................................83 Pain....................................................................................................................... 84 Head abnormalities.............................................................................................. 85 Developmental Milestones.................................................................................. 86 Neuro-imaging......................................................................................................87 Cranial Nerves...................................................................................................... 88 Reflexes................................................................................................................. 89 Motor.................................................................................................................... 90 Sensation/Dermatomes....................................................................................... 90 72 P E DI AT RI C N E U RO LO G Y BEFORE CALLING THE PEDIATRIC NEUROLOGISTS As a part of your excellent H&P, don’t forget to obtain •Birth history •Family history (seizure and use of medication and duration of treatment, early wheelchair, family hx of developmental delays, neuro-genetic syndromes etc) •Developmental history (when did they meet their milestones, regression) •Head circumference in children <2yo EEG’s Consult Peds Neuro if you think you need an EEG. Order the EEG in CHCS (and Essentris). EEGs will be approved and scheduled by Peds Neuro (or EEG tech). Read within 24-48 business hours of the test, and the report documented as an encounter in AHLTA. See phone list for EEG Tech. You do not need to consult neuro in the case of first time afebrile unprovoked sz. However, let neurology know about any inpatient EEG so that they can look out for it. ALTERED MENTAL STATUS Definitions/Clinical Findings: •Normal: awake, easy to arouse and maintain alertness •Lethargic: difficult to maintain alertness •Obtunded: decreased alertness, responsive to pain, other stimuli •Stuporous: decreased alertness, responsive to pain only •Comatose: unresponsive even to pain PE DIAT R IC NEUROLOGY 73 DIFFERENTIAL DIAGNOSIS OF ALTERED MENTAL STATUS T Trauma Tumor Infection Increased ICP I Intussusception - concussion, contusion, hemorrhage - meningitis, encephalitis, CNS abscess - space-occupying lesion, obstructed VP shunt, cerebral edema, subarachnoid, subdural, intracerebral hemorrhage P Poisons - CO, cyanide, acetaminophen, benzodiazepines, barbituates S Sepsis Shock Seizure - nonconvulsive seizures or post-ictal state A Abuse Alcohol Encephalopathy E Endocrine Electrolytes I Insulin/ Hypoglycemia Inborn error of metabolism O Opiates U Uremia - thyroid dysfunction, adrenal insufficiency - low or high Na+, or Ca2+, low Mg2+, hyperammonemia, hypoxia, hypercarbia - Low or high glucose Diagnostics: •Consider: CMP, ammonia, ABG, CBC, TFT, tox screen, BCx, UCx, LP (strongly consider CT prior to LP) •Head CT, EKG, EEG Management: •Treat reversible causes (electrolytes/acid-base/glucose disturbances, maintain normal body temp), abx (consider acyclovir), anticonvulsants, consider naloxone or specific antidotes ATAXIA Definitions/Clinical Findings: •Dysmetria: disturbance of metric/rhythmic aspect of movement, incoordination of voluntary movement •Dysdiadochokinesia: dysfunction of rapid alternating movements •Cerebellar ataxia: wide-based swerving gait, unchanged with eyes opened/closed, pendular DTRs, dysmetria, dysdiadochokinesia, over/undershoot FTN test, scanning speech, intention tremor 74 P E DI AT RI C N E U RO LO G Y •Sensory ataxia: broad-based/high-step gait, worse with eyes closed, +Romberg DIFFERENTIAL DIAGNOSIS OF ACUTE ATAXIA Postinfectious cerebellitis Most common cause of acute ataxia in children, typically 1-3yo, often 1-3wks after illness or immunizations: Varicella (up to 26%), influenza, mycoplasma, coxsackie etc. Maximum symptoms at onset, trunk>extremity, head titubation, truncal ataxia, dysmetria, nystagmus, intention tremor, opsoclonus and horizontal ocular flutter, no fever, typically no altered mental status Infectious Usually viral, meningitis, labrynthitis Drug ingestion Alcohol, phenytoin, carbamazepine, sedatives, hypnotics, PCP, thallium, antihistamine, mercury, lead, ethylene glycol, TCAs, insecticides Other Head trauma, cerebellar hemorrhage, neuroblastoma (opsoclonusmyoclonus-ataxia), ADEM, hydrocephalus, Miller-Fisher variant of GuillainBarré, seizure, post-ictal, basilar migraine, posterior circulation stroke, conversion, mass DIFFERENTIAL DIAGNOSIS OF INTERMITTENT ATAXIA Metabolic disorder (eg. pyruvate dehydrogenase deficiency), acute paroxysmal vertigo, basilar migraine, non-convulsive seizure, genetic episodic ataxia Other DIFFERENTIAL DIAGNOSIS OF SUBACUTE, CHRONIC, OR PROGRESSIVE ATAXIA Other tumor, congenital anomaly, degenerative spinocerebellar disease (Friedreich ataxia, Ataxia-telangectasia), A-beta-lipoproteinemia Diagnostics: •Consider: CBC, P2, tox screen, LP, urine VMA/HVA, metabolic/genetics evaluation (VBG, Chem18, NH4, Serum amino acids, UA, Urine organic acids, acylcarnitine profile lactate, ESR, CRP, CK, CBC, Coags), •Head CT or MRI, body CT/scintigraphy (mIBG) if suspect neuroblastoma, EEG (positive in postinfectious cases and non-convulsive seizures) Management: •Treat underlying etiology. •Post-infectious cerebellitis is self-limited (begins to resolve in 1-4wks), steroids not indicated, limited case studies show IVIG and/or plasma exchange to be effective, PT/OT. •ADEM high-dose steroids/IVIG PE DIAT R IC NEUROLOGY 75 CONCUSSION/HEAD TRAUMA Clinical Findings: Acute: LOC, headache, dizziness, emesis, incoordination, impaired physical skills, amnesia, slurred speech, seizure, confusion, unsteady gait Late: persistent headache, depression, disorganization, poor concentration, frustration, emotionality, personality & behavior changes, memory problems Diagnostics: Pecarn Criteria . <2 yo . ≥ 2 yo 76 P E DI AT RI C N E U RO LO G Y Severe mech of injury: MVC with patient ejection, death of another passenger, rollover, pedestrian or bicyclist w/o helmet in MVC, falls more than 3ft (<2yrs) or 5 feet (>2yrs), or injury from high impact object Return to Play: Each stage should last no less than 24 hours with a minimum of 5 days required to full return. If symptoms recur, the athlete should stop immediately. Once asymptomatic after at least another 24 hours, resume at the previous asymptomatic level and try to progress again. Multiple concussions or prolonged symptoms may require a longer rehabilitation. RETURN TO PLAY GUIDELINES Rehabilitation Stage Functional Exercise 1. No activity Complete physical and cognitive rest. 2. Light aerobic activity Raise heart rate. Walking, swimming, stationary cycling at 70% maximum heart rate, no resistance exercises, 5 to 10 minutes. Absolutely no weightlifting, jumping or hard running. 3. Sport-specific exercise Specific sport-related drills but no head impact. Moderate jogging, brief running, moderate-intensity stationary biking, and moderateintensity weightlifting. 4. Non-contact training drills More complex drills. Running, high-intensity stationary biking, the player’s regular weightlifting routine, and non-contact sport-specific drills. This stage may add some cognitive component to practice in addition to the aerobic and movement components introduced above. 5. Full-contact practice After medical clearance, participate in normal training. 6. Return to play Normal game play. HEADACHES Definitions/Clinical Findings: HA Red Flags (consider non-con head CT or MRI) •Papilledema, worse supine, with valsalva or cough, awakes at night or early in the morning, a/w N/V •Abnormal neuro exam, vision change (not aura a/w migraine), AMS or behavior change or LOC •Sudden or recent onset of severe headache •Progressive pattern •Specific associated symptoms, including FTT or weight loss •Headache a/w high risk condition •<3yo PE DIAT R IC NEUROLOGY 77 DIFFERENTIAL DIAGNOSIS Types of Acute Headache: Migraine without aura > or = 5 attacks, lasts 1-72 hrs, at least 2 of: frontal/temporal, unilateral/bilateral (non-occipital), pulsating/throbbing, moderate – severe pain, increases with activity, light, noise, at least 1 of: photophobia/phonophobia, N/V Migraine with aura Kids may present with irritability, malaise, anorexia, pallor, sensory, motor, or visual change (scotoma, visual field defect), lasts minutes to hours, includes basilar and hemiplegic Tension Bitemporal, tightening/pressure, worse with stress, noise, afternoon, lasts min – days, no pulsating/throbbing Cluster Intense unilat periorbital/temporal, wks- mo, typically adult Exertional Occurs with exertion/exercise, migraine variant Acute Localized Sinusitis, myopia/eye strain, TMJ, trauma Acute Generalized Systemic infection or disease (SLE), CNS infection, CO toxicity, lead poisoning, HTN, LP, hypoglycemia, trauma, CVA, venous sinus thrombosis, substance use or withdrawal, infection Types of Chronic Headache: Chronic, non-progressive <6mo post-concussion, stress, chronic daily HA (>5/wk), medication overuse, obesity, chronic illness, mental health disorder, facial pain, vascular disorder, disorder of head/neck Chronic progressive Brain tumor, pseudotumor cerebri, abscess, hydrocephalus, subdural hematoma, SAH/ICH, arachnoid cysts, HTN, vasculitis, vasospasm, refractive vision error, PRES Diagnostics: •BP, head circumference, fundoscopic exam •Consider P2 and UA if elevated BP, CBC and BCX, LP (get opening pressure) •Non-con head CT or MRI for Red Flags above Management: HEADACHE MEDICATIONS ED Migraine Treatment Orders: NS bolus 20mg/kg x1-2; Benadryl 1mg/kg IV (max 50mg); Ketorolac 0.5mg/kg IV (max 15mg); Reglan (see dosing below); if needed, Morphine 0.05-0.1mg/kg (max 15mg). Reglan Dosing: <6 yo: 0.1mg/kg IV x1, 6-14 yo: 2.5-5mg IV x1, >14yo: 10mg IV x1. 78 P E DI AT RI C N E U RO LO G Y Drug name mg/kg/dose Max dose HA types Side effects Acetaminophen 15 PO/PR Q4-6 1g/dose, 4g/day T Hepatotoxicity, nausea Ibuprofen 10 PO Q6-8 40mg/kg/day T, MA Bleeding, renal insufficiency Naproxen 5-7 PO Q8-12 400mg dose, 1250mg/day T, MA Black Box: fatal CV thrombotic event Ketorolac 0.5 IM/IV Q6 30mg/dose or 120 mg/day MA GI bleed, nephritis Sumatriptan (Imitrex) <12 yo: 0.06 SQ Max 6mg 200mg/day PO 12mg/day SQ MA Cluster Vasospasm Ergotamine 1mg/dose Q30min SL/PO 3mg/HA child. 6mg/HA adult. Cluster GI, rebound HA Propranolol <35kg 10-20mg PO TID >35kg 20-40mg PO TID NA MP, PTC Asthma attacks, depression, exacerbates DM, hypotension Amitriptyline 0.1-0.25 QHS 2mg/kg/day or 75mg/day MP Minimal to mild sedation, anticholinergic MP Sedation, increased appetite, anticholinergic. Contraindicated in asthma. Cyproheptadine (Periactin) 0.25-0.4 BID 2-6yr: 12mg/day 7-14yr: 16mg/day Promethazine (Phenergan) 0.25-1 PO Q4-6 25mg dose Antiemetic Black box: respiratory depression Metoclopramide (Reglan) 1-2 Q2-6 IV/IM/PO 10mg dose Antiemetic EPS, tardive dyskinesia Prochlorperazine (Compazine) 0.1-0.15 IM Q8 10-14kg: 2.5mg QD-BID 15-39 kg: 2.5mg BID-TID 10-14kg: 7.5mg/ day 15-39kg: 10-15mg/day Adult: 40mg/day Antiemetic EPS, orthostatic hypotension T=tension; MA = Migraine, abortive; MP= Migraine Consider topiramate: Children 6 to <12 years; weight: ≥ 20 kg: Initial: 15 mg/day for one week; then increase to 15 mg twice daily for 1 week; then increase to 25 mg twice daily for 7 days; continue to gradually titrate to effect up to target dose of 2-3 mg/kg/day divided twice daily; maximum daily dose: 200 mg/day for migraine prophylaxis PE DIAT R IC NEUROLOGY 79 FEBRILE SEIZURE Definitions/Clinical Findings: >6mo of age with febrile illness not associated with previous seizures and not meeting criteria for other acute symptomatic seizure. 50% of children who present with febrile seizures will not have any identified risk factors (history of fever is first-degree relative, neurodevelopmental delays, HHV6, MMRV or DTaP or influenza vaccines) TYPES OF FEBRILE SEIZURES Simple febrile seizure (75%) <15min, generalized, 1 episode within 24hr period Complex febrile seizure (25%) >15min, focal component, and/or >1 episode within 24hr period Diagnostics: •First simple febrile seizure: no work up unless otherwise clinically indicated. •Complex febrile seizure: CBC w/ diff, P2, may consider EEG, LP if suspect meningitis/intracranial infection, neuroimaging if focal •Routine EEG and neuroimaging are not indicated for first simple febrile seizures. Neuroimaging is recommended for neurologic deficits, prolonged post-ictal state, and increased ICP. Management: •Benzodiazepines/rectal diazepam for prolonged seizure >5min. •Parent Education: Febrile seizures are the most common seizure disorder in children, affecting 2-5%, between 6-60mo. Recurrence usually occurs within the initial 1-2yrs after initial seizure. Recurrence risk ~30% if >12mo and ~50% if <12mo at time of initial febrile seizure. Epilepsy risk ~1% if >12mo and ~2.4% if <12mo after initial febrile seizure. Antipyretics have not shown to decreased risk of recurrence of simple febrile seizures. 80 P E DI AT RI C N E U RO LO G Y FIRST AFEBRILE SEIZURE DIFFERENTIAL DIAGNOSIS OF FIRST AFEBRILE SEIZURE Seizure •Partial paroxysmal change in motor or behavior caused by abnormal electrical discharges in the brain one cerebral hemisphere involved motor signs, sensory, autonomic or psychic experience •Simple partial - awareness preserved •Complex partial - awareness impaired, often staring •Generalized both cerebral hemispheres involved •Tonic-clonic, tonic, clonic, atonic, myoclonic, or absence Epilepsy 2 or more unprovoked seizures >24hrs apart, usually stereotyped Status epilepticus single prolonged >30min or series of seizures >30min without full recovery between events, see PICU section Neonatal seizure seizure in first 30DOL, most common cause in first 24HOL is HIE Infantile spasm 90% present <1yo, hypsarrhythmia on EEG, developmental regression Seizure-like breath-holding, syncope, GERD/Sandifer syndrome, pseudoseizure, panic attacks, TIA, vestibular disorder, paroxysmal choreoathetosis, atypical migraine, tic, benign myoclonus of infancy, cardiac dysrhythmia with collapse, syncope, behavioral event (eg. staring), conversion disorder, acute dystonic reaction, medication withdrawal, night terrors, hypoxia, ischemia, hyperammonemia, toxin, electrolyte disturbance Diagnostics: •consider: POC glucose, P2, VBG, CBC, UA, ammonia, lactate, tox screen, LP. Routine labs in >6mo who returns to baseline and whose history is non-suggestive is generally not necessary. •EEG on all first afebrile seizure (most sensitive within 24hrs of seizure). Urgent neuroimaging if prolonged focal postictal deficit or not back to baseline within few hours. •Strongly consider non-urgent imaging if cognitive or motor impairment of unknown etiology, abnormal neuro exam, focal seizure, abnormal/non-benign EEG, or <1yo. Management: •AEDs not generally indicated after a first non-febrile seizure. AEDs are recommended after 2 or more recurrent afebrile seizures. •Parent Education: Majority of children with first unprovoked afebrile seizure have no or few recurrences. Recurrence risk ~24% in 1yr, and ~45% over 14yrs. PE DIAT R IC NEUROLOGY 81 SEIZURE Min Intervention • Stabilize the patient • Assess airway, breathing, circulation 0-5 • Check vitals. Administer oxygen. Pulse ox. IV or IO access. CR monitors. Check Accucheck and electrolytes. (If hypoglycemic: check critical labs prior to correction—glucose, insulin, cortisol, growth hormone, IGFPB1, C-peptide, lactate, pyruvate, beta hydroxybutyrate) • Correct Hypoglycemia: dextrose 25% 2–4 mL/kg. In adolescents, give thiamine (100 mg) first. • Labs: lytes, Ca, Mg, Phos, BUN, Cr, glucose, LFTs, CBC, ammonia, toxicology (serum + urine), anticonvulsant levels, BCx, UCx, ABG if arterial access. Begin pharmacotherapy •Lorazepam (Ativan) 0.1mg/kg IV or IM (max 4 mg). Rate of 2mg/min. Repeated Q 2-5 minutes x3. Watch for ↓BP, respiratory depression, ↓HR. 5-15 OR • PR Diazepam (Valium) - Dosing by age: 2-5yo: 0.5mg/kg, 6-11 yo 0.3mg/kg, ≥ 11 yo 0.2mg/kg. Max 20 mg. OR • Midazolam IM, IN or buccal if no IV access AND If infectious cause suspected: Ceftriaxone 100mg/kg once / Acyclovir 20mg/kg Q8 if <12 yo, 10mg/kg if >12yo 15 Fosphenytoin 20 mg/kg IV at 3mg/kg/min (max dose 1250mg, max rate 150 mg/ min). Watch for arrhythmias & hypotension. IM okay if no IV access. (ALWAYS CHECK FOR PHENYTOIN ALLERGY) CALL PICU after fosphenytoin administration if seizure continues. 20 Phenobarbitol 20mg/kg IV slow push (1mg/kg/min), max 300mg. Watch for hypotension & respiratory depression. May take 10 minutes for effect. Fosphenytoin 20 mg/kg IV at 3mg/kg/min over 8 minutes. ICU Management may include: continuous EEG Midazolam 0.3mg/kg bolus with drip 10 μg/kg/min. Watch for hypotension & apnea. Possible coma induction using penbarbital, propofol, or thiopental. PICU AFTER INITIAL IMAGING COMPLETED •Continuous EEG if patient has prolonged altered mental status or was paralyzed during resuscitation ef- forts to monitor for nonconvulsive status epilepticus. •Head CT: Indications: new onset unexplained status epilepticus, or if no prior imaging for patient with h/o status epilepticus, focal deficits, or suspected ↑ICP. •LP: Concern for infection or SAH. Head CT first if concerned about ↑ICP. 82 P E DI AT RI C N E U RO LO G Y WEAKNESS DIFFERENTIAL DIAGNOSIS OF ACUTE WEAKNESS CNS Unilateral or bilateral stroke CNS-spinal cord Cord infarction, cord compression, trauma, contusion, ingestion, transverse myelitis, spinal epidural abscess, syringomyelia Peripheral nerve spinal root Guillain Barré Peripheral nerve ICU neuropathy, HIV or zidovudine therapy, hereditary tyrinosemia, acute intermittent porphyria, medication-related (phenytoin, vincristine, nitrofurantoin, INH), toxins (heavy metals, glue), metabolic (uremia), autoimmune, chronic JRA, demyelinating diseases (CIS, ADEM, NMO, MS) NMJ Myasthenia gravis, botulism, tic paralysis, pharmacologic blockade, aminoglycoside toxicity Muscle Myositis (infectious, dermatomyositis, polymyositis), metabolic (hypocalcemia, hypokalemia, hypothyroid), medication- related, ICU myopathy, familial periodic paralysis (hypo/hyperkalemia), Duchenne/ Becker Muscular Dystrophy Diagnostics: •Consider: CK, LP, lyme titers, amino-levulinic acid, muscle bx •CT head, MRI brain if suspect stroke, MRI brain and spinal cord, EMG and nerve conduction studies if after 1wk of symptoms Central Anterior horn Peripheral nerve NMJ Muscle Distribution Distal Proximal Distal Proximal, symmetric, wax/wane Proximal, symmetric DTR ↑ ↓, Absent Absent WNL in MG, ↓ in LE botulism ↓ Fasciculation Absent Yes Variable No No Atrophy Not acutely Yes Severe No Variable, Pseudohypertrophy Sensation Often impaired WNL Impaired WNL WNL Nerve conduction WNL ↓30-50% Decreased velocity Abnl repetitive stimulation WNL CK WNL WNL / mild ↑ WNL ↑ PE DIAT R IC NEUROLOGY 83 PAIN MANAGEMENT NON-OPIOID ANALGESICS Drug Route Dose Comments Acetaminophen PO/IV 15 mg/kg q6h Weak analgesic, excellent antipyretic, no antiinflammatory properties Ibuprofen PO 10 mg/kg q6-8h For age > 6 mo. Contraindicated in IBD pts and thrombocytopenia Ketorolac IV/IM/PO 0.5 mg/kg q6 to max 20 doses (72 hours) 1 mg/kg comparable to 0.1 mg/kg morphine. Only IV NSAID. Contraindicated in pts with thrombocytopenia or risk of bleeding. Naproxen PO 5-7mg/kg/dose q12h Long acting NSAID COMMON OPIATES Drug Starting Dose (IV) Fentanyl Infants: 3 mcg/kg/dose q2-4 prn Child: 1-2 mcg/kg/dose; may repeat 30-60 min intervals Hydromorphone (Dilaudid) 0.015mg/kg/dose q3-6h Hydrocodone w/ Tylenol (Lortab) 0.1-0.2mg/kg q4 PO Meperidine (Demerol) 1 – 1.5 mg/kg/dose q3-4 prn (max 100 mg/ dose) Morphine 0.05-0.1 mg/kg/dose Q21 mg IV = 3mg PO 4prn (max 15 mg/dose) Oxycodone (oxycotin) PO: 0.05–0.15 mg/kg/ dose q4-6 (max 5mg/ dose) Methadone 0.1 mg/kg/dose q6-8 prn (max 10 mg/dose) 84 Equianalgesic Comments Chest wall rigidity with doses >5 mcg/kg. Tx naloxone or Neuromuscular blockade. 1 mg IV = 5 mg PO Decreased sedation, nausea, pruritus than morphine. 1 mg IV = 1.5 mg PO More euphoria than morphine. Not recommended for PCA. Less nausea than codeine 1 mg IV = 1 mg PO P E DI AT RI C N E U RO LO G Y RELATIVE POTENCY ( IV) STARTING A PCA Children ≥5 years and Adolescents, weighing <50 kg: •Meperidine 0.1 •Methadone 1 Usual concentration: 1 mg/mL Demand dose: Usual initial: 0.02 mg/kg/dose; usual range: 0.01-0.03 mg/kg/dose Lockout: Usual initial: 5 doses/hour Lockout interval: Range: 6-8 minutes Usual basal rate: 0-0.03 mg/kg/hour •Morphine 1 •Dilaudid 7 •Fentanyl 100 NAMES •Percocet = oxycodone + acetaminophen •Vicodin = hydrocodone + acetaminophen •Lortab = hydrocodone + acetaminophen •Norco = hydrocodone + acetaminophen Children and Adolescents, weighing ≥50 kg: Usual concentration: 1 mg/mL Demand dose: Usual initial: 1 mg; usual range: 0.5-2.5 mg Lockout interval: Usual initial: 6 minutes; usual range: 5-10 minutes Usual basal rate: 0-0.03 mg/kg/hour HEAD ABNORMALITIES Craniosynostosis Abnormal head shape secondary to premature fusion of sutures. X-ray shows increased density on closed suture. Head CT is indicated if ≥ 2 sutures involved or ↑ICP is suspected. NSG manages these patients. In the non- syndromic subtype abnormal head shape is the only feature. Definitions •Acrocephaly: high, tower-like head with vertical forehead, premature coronal + sagittal + lambdoid closure •Brachycephaly: wide head, premature coronal suture closure •Trigonocephaly: narrow triangle shaped forehead, premature fusion of metopic suture •Plagiocephaly: flattened on one side, premature fusion of one coronal or one lambdoid suture •Scaphocephaly/Dolichocephaly: long and narrow, premature sagittal suture closure Normocephaly Dolichocephaly PE DIAT R IC NEUROLOGY Triganocephaly Plagiocephaly Plagiocephaly Brachycephaly 85 DEVELOPMENTAL MILESTONES Age Gross Motor Fine Motor Language Social 1 mo Raises head when prone Tight grasp, tracks to midline Alerts to sounds Regards face 2 mo Holds head up, lifts chest when prone Tracks past midline Social smile Recognizes parent 3 mo On forearms when prone Hands open at rest Laughs, orients to voice Reaches for people, objects 6 mo Sits unsupported, Raking grasp, held horizontal w/ transfers objects head above plane Babbles Recognizes strangers 9 mo Pulls to stand, cruises Immature pincer, throws Dada/mama indiscriminant Understands no, plays pat-a-cake 12 mo Walks Mature pincer grip 1-2 words, follows Imitates, comes commands w/ when name is gesture called 15 mo Creeps up stairs, walks backwards Scribbles, stacks 2 blocks 4-6 words, one step commands 18 mo Runs, throws Scribbles, 3 7-10 words, blocks, turn book knows 5 body pages. Hand prefparts erence develops. 24 mo Walks up/down stairs Takes off clothes 50 words, 2 word phases, 2 step commands Parallel play 3 yr Tricycle, alternates steps on stairs Copies circle 250 words, 3 Word phrases, uses pronouns/ plural Knows name, age, sex. Group play 4 yr Hop, skips Copies square, catches ball Asks questions, knows colors / songs Imaginative play, tells stories 5 yr Jumps over things Copies triangle, ties shoes Prints first name, asks what words mean Plays games 86 Uses spoon & cup Copies tasks P E DI AT RI C N E U RO LO G Y NEURO-IMAGING Type DWI Uses Bright Find dead (aka ischemic) tissue Dead tissue (aka where there is no H20) note airbrain and bonebrain interfaces will be bright ADC Acute ischemia SWI Look for bleed T1 Anatomy – like the “real” brain would be Edema, gliosis, CSF Dark Comments CSF To check if artifact, look at ADC – if bright on MRI and dark on ADC = REAL. If bright both = artifact Acute ischemia Use to distinguish acute ischemia on DWI from T2 shine through artifact Bleed! Will be circular White matter > gray matter, cancer, subacute bleed CSF, bone, acute bleed Look for open space below midbrain to r/o herniation. T1 w/ gad good to eval highly perfused lesions – gad is bright T2 Infarcts, inflammation, tumors CSF, fluid, bone, Gray matter > edema – opposite white matter T1 Good if white matter lesions present. Can also look at CNs 7-8 as will be bright at CPA FLAIR Edema, gliosis, small white matter lesions Edema, gliosis, old strokes, subacute bleed Hyperacute bleed, CSF Same as T2 but with dark CSF MR spect Tumors, metabolic disorder NA NA R → L: lipid, lactate, NAA, creatinine, choline, myoinositol CT Acute hemorrhagic stroke, skull fx, hydrocephalus, trauma New blood, mets, Tumor, old blood, posterior fossa meningioma, edema, air poorly visualized cancer, bone PE DIAT R IC NEUROLOGY 87 CRANIAL NERVES Function/ Region CN Test/Observation Olfactory I Smell (e.g., coffee, vanilla, peppermint) Vision II Acuity, fields, fundus Pupils II, III Pupil size, reaction to light and accommodation Range and quality of eye movements, saccades, pursuits, nystagmus, ptosis. Eye movements and eyelids III, IV, VI Frontal eye field cortical lesion causes deviation toward lesion with intact doll’s eye. Cortical sz causes deviation away from foci. Brainstem lesion causes ipsilateral deviation away from lesion side with impaired doll’s eye. Sensation V Corneal reflexes, facial sensation Muscles of mastication V Clench teeth Facial strength VII Observe degree of expression of emotions, eye closure strength, smile, puff out cheeks, asymmetry forehead and lower face Hearing VIII Localize sound, audiologic testing: finger rub, Rhinne, Weber Mouth, pharynx VII, IX, X, XII Swallowing, speech quality (labial, lingual, or palatal articulation deficits), symmetrical palatal elevation, tongue protrusion Head control XI Lateral head movement, shoulder shrug Tongue XII Tongue protrusion, push out cheeks with tongue. Deviation to side of lesion. Test CN tested Cover eyes: baby opens eyes, direct gaze to you II, III, IV, VI Facial symmetry VII Check rooting reflex on both sides V (sensory), XI Suck on finger, extend finger back to gag V (motor), IX, X, XII 88 P E DI AT RI C N E U RO LO G Y REFLEXES Primitive reflexes Appears by Gone by Gag 32 wk GA Persists in 90% Suck 34 wk GA 4 mo Palmar grasp 34 wk GA 6 mo Plantar grasp 34 wk GA 10 mo Tonic neck - fencer’s 2-3 wks 7 mo Moro 34 wk 3 mo (rarely 4-5 mo) Stepping 35 wk 2 mo Crossed adductor 35 wk 7 mo Babinski- extensor birth 9-12 mo Grasp 32 wk 2 mo Reflex Site Biceps C5, C6 Brachioradialis C5, C6 Triceps C7, C8 Knee L2-L4 Achilles S1–S2 Grade 5 Normal power Grade 4 Active movement against gravity with resistance Grade 3 Active movement against gravity without resistance Grade 2 Active movement with gravity eliminated Grade 1 Only a trace or flicker of movement Grade 0 No movement PE DIAT R IC NEUROLOGY 89 UPPER AND LOWER MOTOR NEURON General: brisk reflexes and weakness often suggests CNS problem, absent reflexes may suggest lesion distal to CNS (nerve, NMJ, muscle). Asymmetries are always abnormal. On Exam UMN LMN Power Decreased Decreased Reflexes Increased Decreased Tone Increased Normal or decreased Babinski Toes up-going Toes down-going Exception: Acute upper motor neuron and spinal cord injury produces decreased tone and absent/depressed reflexes. DERMATOMES 90 P E DI AT RI C N E U RO LO G Y NOTES PE DIAT R IC NEUROLOGY 91 SECTION 10 PICU PALS algorithms....................................................................................................93 Common medications and drips........................................................................ 96 Code meds........................................................................................................ 96 Vasoactive meds................................................................................................97 Sedation............................................................................................................ 98 Intubation.............................................................................................................. 99 Ventilator basics................................................................................................. 100 Status asthmaticus.............................................................................................. 101 DKA......................................................................................................................102 Status epilepticus................................................................................................ 103 92 P IC U PIC U 93 94 P IC U PIC U 95 CODE MEDS Adenosine SVT 0.1mg/kg (max 6mg) IV/IO rapid push Second dose 0.2mg/kg (max 12mg) IV/IO Amiodarone Pulseless arrest 5mg/kg (max 300mg) IV/IO bolus May repeat x2 up to 15mg/kg (max 2.2g/day) Atropine Severe bradycardia 0.02mg/kg (min 0.1mg, max 1mg) IV/IO May repeat dose x1 Calcium chloride 10% 20mg/kg (max 2g) IV/IO slowly, CVL strongly preferred Dextrose (D10W) 5-10 mL/kg Epinephrine Pulseless arrest/severe bradycardia IV/IO: 0.01mg/kg (0.1mL/kg of 1:10,000) Q3-5 min (max 1mg) ET: 0.1mg/kg (0.1mL/kg of 1:1,000) Q3-5 min (max 10mg) Lidocaine VF/pulseless VT/wide-complex tachycardia with pulse 1mg/kg (max 100mg) IV/IO bolus, 2-3mg/kg ET Magnesium sulfate Torsades de pointes 25-50mg/kg (max 2g) IV/IO Procainamide SVT, atrial flutter, VT with pulse 15mg/kg (max 500mg) IV/IO over 30 min ProstaglandinE1 Ductal dependent congenital heart lesion 0.05-0.1mcg/kg/min IV/IO Sodium bicarbonate 1mEq/kg IV/IO, give slowly 96 P IC U VASOACTIVE MEDS Med Infusion Rate Inotropy Chronotropy Pressor Effects Dopamine 1-10mcg/kg/min 10-20mcg/kg/min ↑↑↑↑ ↑↑↑↑ ↑↑ ↑↑ ↑↑ Epinephrine 0.1-0.5mcg/kg/ min 0.5-1mcg/kg/min ↑↑↑↑ ↑↑↑↑ ↑↑ ↑↑ ↑↑ Norepinephrine 0.05-2mcg/kg/ min ↑↑ ↑ ↑↑↑↑ Phenylephrine 0.1-0.5mcg/kg/ min ↑↑↑ Vasopressin 0.2-10mUnits/kg/ min ↑↑↑ Dobutamine 2-20mcg/kg/min ↑↑↑↑ Milrinone 0.25-0.75mcg/kg/ min ↑↑↑↑ Isoproterenol 0.05-2mcg/kg/ min ↑↑ Nitroprusside 0.3-10mcg/kg/min ↓* PIC U ↑ ↓↓* ↓↓* ↑↑↑↑ ↓↓↓* ↓↓↓↓* 97 SEDATION Adjuncts Atropine Bolus: 0.02mg/kg (min 0.1mg, Prevents bradycardia max 1mg) Miscellaneous Dexmedetomidine Bolus: 1mcg/kg Drip: 0.5-1mcg/kg/hr Causes bradycardia Etomidate Bolus: 0.2-0.6mg/kg (max 20mg) Drip: 5-20mcg/kg/min Lowers ICP, few CV effects. Can cause adrenal suppression (caution use with sepsis) Ketamine Bolus: 1-2mg/kg Drip: 5-20mcg/kg/min Raises BP, ICP, and HR. Mild bronchodilator. Thiopental Bolus: 2-5mg/kg Lowers BP, ICP, and CPP. Anticonvulsant. Propofol Bolus: 1mg/kg Drip: 50-300mcg/kg/min Lowers BP Benzodiazepines (Sedatives) Midazolam Bolus: 0.05-0.1mg/kg Drip: 20-200mcg/kg/hr Lowers BP and HR Lorazepam Bolus: 0.05-0.1mg/kg Lowers BP and HR Opiates (Analgesics) Fentanyl Bolus: 1-5mcg/kg Drip: 1-5mcg/kg/hr Lowers BP. Can cause chest wall rigidity if pushed rapidly. Morphine Bolus: 0.05-0.1mg/kg Drip: 20-200mcg/kg/hr Lowers BP Cisatracurium Bolus: 0.15mg/kg Drip: 1-4mcg/kg/min Good choice for patients w/ hepatic or renal dysfunction Rocuronium Bolus: 0.6-1mg/kg Lasts 15-20min Paralytics Succinylcholine Bolus: 2mg/kg Vecuronium Bolus: 0.1mg/kg Drip: 20-200mcg/kg/hr Can cause muscle fasciculation (depolarizing agent). Contraindicated in many patients. May cause hyperkalemic cardiac arrest. Do not use without discussing with PICU Attending. Defasciculation dose prior to succinylcholine: 0.01mg/kg Reversal Naloxone Partial reversal: 5mcg/kg Full reversal: 0.1mg/kg Flumazenil 0.01mg/kg (max 0.2mg) Neostigmine 0.07mg/kg (max 5mg) Glycopyrrolate 0.2mg for each 1mg of Neostigmine 98 ***Pre-medicate first with glycopyrrolate P IC U INTUBATION Age Preterm Newborn Infant 1yr 3yr 6yr 10yr Adolescent Adult Weight 1.5kg 3kg 5kg 10kg 15kg 20kg 30kg 50kg 10kg ETT size (uncuffed) 2.5-3.0 3.0-3.5 3.5-4.0 4.04.5 4.55.0 5.0-5.5 6.06.5 6.5-7.0 7.0 Blade size 0 1 1 1-2 2 2 2-3 3>3 >3 1 1-1.5 1.5-2 2 2.5 3 3 4 Newborn NewbornInfant Infant Child Child Adult Adult Adult LMA size Mask Newborn INTUBATION ESTIMATES Blade size: •Preemie/newborn = Size 0 •Older newborn – 6 months = Size 1 •>6 months – 2 years = Size 1-2 •>2 years – 8 years = Size 2 ETT size: •Uncuffed ETT size = (age/4) + 4 [max 7.0] •*Chose ½ size smaller for cuffed ETTs •*<1kg: 2.5, 1-2kg: 3.0, 2-3kg: 3.5, >3kg: 4.0 ETT depth: • = age in years + 10 –OR- 3x ETT size • *Max depth = 23cm for males or 20cm for females PIC U 99 VENTILATOR BASICS Non-Invasive Ventilation (via Mask or Nasal Cannula) •BiPAP (Bi-level Positive Airway Pressure) – set rate and/or augment spontaneous breaths; provides inspiratory pressure support and PEEP •CPAP (Continuous Positive Airway Pressure) – provides PEEP Liter Flow % O2 Delivered Nasal Cannula 1-4 L/min 21-50% Simple Mask 5-10 L/min 21-50% Partial-rebreather 6-10 L/min Up to 80% Non-rebreather >10 L/min Up to 80-100% Vapotherm 8-40 L/min Up to 100% INVASIVE VENTILATION Control of rate: •AC (Assist Control) – minimum rate synchronized to patient triggered breaths; patient triggered breaths above minimum rate delivers a breath identical to set breaths (see control of volume/ pressure below) •SIMV (Synchronous Intermittent Mandatory Ventilation) – minimum rate synchronized to patient triggered breaths; patient triggered breaths above min rate are supported with Pressure Support •CPAP/PS (Continuous Positive Pressure with Pressure Support Ventilation) – no set rate, all breaths patient triggered and pressure supported Control of Volume/Pressure: •Volume Control (may be AC or SIMV): Breaths supported to provide a pre-determined volume per breath. •Pressure Control (may be AC or SIMV): Breaths supported at a pre-determined pressure above PEEP. •PRVC (Pressure-Regulated Volume Control; may be AC or SIMV): Provider pre-determined a volume per breath. Computer adjusts the pressure control to obtain volumes at the target volume. Basic ventilator settings Basic ventilator changes Infant Child Goal Rate Rate 30-40 15-25 ↑ CO2 ↓ Tidal Volume 5-7cc/kg 5-7cc/kg ↓ CO2 ↑ PEEP 4-5 3-5 ↑ O2 I-time 0.4-0.6s 0.8-1s ↓ O2 10 0 PEEP FiO2 ↑ ↑ ↓ P IC U STATUS ASTHMATICUS Step 1: Immediately upon arrival in ED or PICU •Oxygen •Steroids: SoluMedrol 2 mg/kg IV (max dose 150 mg) then 1 mg/kg IV q6hr •Ranitidine 1 mg/kg IV q12hr •Albuterol: Continuous Albuterol 1 mg/kg/hr neb; max dose 40 mg/hr •Do not wean Albuterol unless severe tachycardia defined as: •Sustained HR > 220 for children under 2 years of age •Sustained HR > 200 for children aged 2 – 10 years •Sustained HR > 190 for children aged 10 – 20 years •If patient develops sustained tachycardia, wean Albuterol by 20% q1hr until HR < maximum rates listed above. No role for Levalbuterol. •Atrovent 1 unit dose (500 mcg) neb q6hr only for children > 6 years of age •IV fluids: NPO. NS 20 ml/kg IV x 1-3 if signs of depressed cardiac output then D5 ½NS + 20 mEq/kg KCl at 1-1.5x maintenance. •Labs: Consider chest X-ray to rule-out pneumonia or pneumothorax. Blood gas, chemistry studies, CBC, and other labs are generally not indicated. May consider EKG prior to starting Terbutaline. Step 2: If insufficient response to step 1 (consider one of more of the following) •Terbutaline IV drip •Start at 1 mcg/kg/min •MgSO4 40 mg/kg (max 2 g) IV push over 20 minutes •HeliOx •Start with 80:20 (80% Helium: 20% Oxygen) •Use regulator to increase FiO2 as needed to maintain SaO2 > 88% •Use the least amount of O2 required to maximize Helium concentration •Ensure mask is tight fitting with high gas flow rate to ensure pt is not entraining Nitrogen from the room air •Ensure that the continuous Albuterol is running off of HeliOx to maximize FiHelium. Step 3: BiPAP via face mask: PEEP 10, PS 15, no rate; adjust setting as needed Step 4: Ketamine 1-2 mg/kg IV push followed by 1-2.5 mg/kg/hr drip Step 5: Endotracheal intubation with mechanical ventilation •Use rate similar to patient’s spontaneous breathing rate, and attempt to mimic the patient’s spontaneous inspiratory and expiratory times. Rapidly transition patient to Pressure Support mode (i.e., no set rate, no set inspiratory time) as soon as paralysis is resolved. Step 6: Inhaled anesthetics Step 7: Extra-Corporeal Membrane Oxygenation (ECMO) •Double dose q30min to desired effect or severe tachycardia •Maximum dose 10 mcg/kg/min •Check Troponin I levels q6hr PIC U 1 01 DKA DKA = Hyperglycemia, presence of ketones (serum or urine), acidosis pH < 7.30 or HCO3 < 15 mM Orders under “PICU Diabetic Ketoacidosis” order set 1. Initial laboratory studies: •EG7, CBC w/diff, Chem 10, Urinalysis, HgbA1c, Anti-insulin antibody, Anti-GAD antibody, Islet cell 512 antibody, Beta hydroxybutyrate, Serum insulin, C-peptide, Thyroid panel, Anti-thyroid antibodies, Anti-tissue transglutaminase (tTG), total IgA, Vitamin D 25-hydroxy 2. NPO status 3. Initial fluid resuscitation: •0.9% normal saline 10-20 cc/kg over 1 hour or faster if in shock 4. Insulin drip: Do not give a bolus. •Begin with a continuous insulin infusion of 0.1 unit/kg/hr. Do not adjust insulin rate without discussing with PICU Attending. 5. Fluid therapy: •Order both standard PICU DKA fluids: •Bag #1: NS + 20mEq/L of KPhos + 20mEq/L of KAcetate. •Bag #2: D12.5-NS + 20mEq/L of KPhos + 20mEq/L of KAcetate. •Total IVF Rate: 1.5 x maintenance •If glucose is >300, run Bag #1 at 1.5 x maintenance •Once glucose falls below 300, adjust rate of Bag #1 and Bag #2 to keep glucose 150 – 250. Maintain total rate (Bag #1 + Bag #2) at 1.5 x maintenance 6. Laboratory studies: Order labs per DKA order set •Glucose every hour •Chem 10 q 2 hrs for first 6 hours. If stable after 6 hours then q4 hours. •*Must correct Na for Glc •EG7 with lytes until HCO3 > 10mM •Calculate effective osmolality •OSMeff = 2(sodium + potassium) + glucose/18 (normal range = 280-295 mosm) •Phosphate and ionized calcium at 6 and 12 hour lab draw. 7. Monitor for complications: •Cerebral edema, hypophosphatemia, hyponatremia, hypoglycemia, hypokalemia, hypocalcemia 8. Switch to subcutaneous insulin when pH> 7.30 or HCO3 > 15 and patient is able to tolerate PO. •Immediately after giving the first subcutaneous insulin injection, discontinue IV fluids and continuous insulin infusion and allow the patient to eat their meal. 10 2 P IC U STATUS EPILEPTICUS Min Intervention •Stabilize the patient •Assess ABCs •Check vitals: give O2, pulse ox, IV or IO access, monitor 0-5 •Check Accucheck and electrolytes •Correct hypoglycemia if present: D10W 5-10cc/kg •Other labs: Gas, chem 10, LFTs, CBC w/diff, ammonia, toxicology (serum + urine), AED levels, BCx, UCx •Begin pharmacotherapy: •Lorazepam (Ativan) 0.1mg/kg (max 4mg) IV/IO/IM Repeat Q 2-5 min x3 5-15 •Rectal Diazepam (Valium) – for patient in ED when no IV access available 2-5yo: 0.5mg/kg 6-11yo: 0.3mg/kg >11yo: 0.2mg/kg (max 20mg) •If infectious cause suspected: Ceftriaxone 100mg/kg +/- Acyclovir 10mg/kg •If not responsive to benzodiazepines: •Levetiracetam (Keppra) 30mg/kg IV load Maintenance of 10-20mg/kg IV Q12 hours 15-45 •Phenobarbital 20mg/kg IV over 20min May repeat additional 10mg/kg x 2 or more. May require doses above 60mg/kg •Fosphenytoin 20mg/kg IV over 10min May repeat additional 10mg/kg •If still not responsive to pharmacotherapy: •Pentobarbital coma 5mg/kg IV over 20min, then 0.5-1mg/kg/hr titrated for burst suppression •Midazolam coma 150-200mcg/kg IV, then 1-10mcg/kg/min •Consider: >60 •Continuous EEG •Head CT •Lumbar puncture •Patients in induced coma will likely require intubation and mechanical ventilation. Intubation is not a treatment for status epilepticus and should only be performed if necessary. Paralytics used for intubation will mask physical signs of seizures, however the seizures will continue throughout paralysis and lead to ongoing neurological damage. PIC U 103 NOTES 10 4 P IC U SECTION 11 Pulmonology ALTE.....................................................................................................................106 NMCSD ALTE Pathway....................................................................................... 107 Asthma.................................................................................................................108 Bronchiolitis.........................................................................................................112 PULMO NO LOGY 1 05 ALTE Relationship to gastroesophageal reflux (GER) •GER is normal event in infants. •Possible to have evidence of GER and another disorder. •Reflux induced apnea may be central or related to laryngospasm from local acid effect Relationship to SIDS •ALTE may be risk factor for SIDS, especially if required resuscitation •ALTE peak 1 to 3 months of age •SIDS peak 3 to 5 months of age •Apnea of prematurity not a risk factor for SIDS Conditions not requiring ALTE workup •Normal child (overreaction to simple choking, gagging, vomiting episodeeyes open and “bulging”, increased tone) if not sure, consider admission and workup •Periodic breathing: respiratory pauses 320 seconds. Normal in preterm, may persist in term infants. Clues to diagnosis •Nasal congestion/cough consider bronchiolitis •Stridor/hoarsenss consider airway evaluation •Recurrent ALTEconsider NAT •History involving foreign bodyconsider FB aspiration •Snoring/stridor at baseline consider obstructive sleep apnea/ airway eval •Pallor consider anemia/intracranial hemorrhage •Blood in infant’s mouth or nose consider NAT •Visible hemangioma, especially on face/neckconsider airway eval •History of altered consciousness during or following eventconsider seizure •Low tone, constipationconsider infant botulism 10 6 P U LMON O LO G Y NMCSD ALTE PATHWAY PULMO NO LOGY 107 ASTHMA SEVERITY OF ASTHMA EXACERBATION Mild Moderate Severe Respiratory Arrest Imminent Activity Level: Walks briskly Walks slowly Walks with assistance Unable to walk Feeding (infant): Normal Difficulty feeding Unable to feed Unable to suck Talks in: Sentences Phrases Words Too dyspneic to speak; perspiring Sounds (infant): Normal cry, cooing Short, clipped cry Faint cry, grunting Alertness: May be agitated Usually agitated Usually agitated Respiratory rate: Increased Increased Often > 30/min SIGNS / SYMPTOMS Drowsy or confused Retractions & accessory muscle use: Usually not Usually Usually Paradoxical thoraco-abdominal movement (see-saw breathing) Wheeze: Moderate, often only end expiratory Loud expiratory Usually loud, may be biphasic Absence of wheeze SaO2% (on RA) > 95% 91-95% < 90% PEF after initial bronchodilator treatment Over 80% Approx. 60-80% < 60% predicted PaO2 (on RA) Normal Test not usually necessary > 60 mm Hg < 60 mm Hg Possible cyanosis Cyanosis PaCO2 < 45 mm Hg < 45 mm Hg > 45 mm Hg >50 mm Hg Partial relief after multiple treatments. Requires continuous inhaled SABA Minimal or no relief from inhaled SABA. Requires systemic bronchodilator (subcutaneous epinephrine, terbutaline) Emergency department; possible hospitalization Hospitalization following stabilization in emergency department TESTS INTERVENTION Response to inhaled Short-Acting Bronchodilator (SABA) Prompt relief Complete relief after multiple treatments Location of care Home Management Office or emergency department 10 8 P U LMON O LO G Y DIFFERENTIAL DIAGNOSES Diagnosis Symptoms Test • Trial of antihistamines Allergic Rhinitis • Seasonal or chronic rhinorrhea/nasal obstruction • Daytime and/or morning cough • Nasal steroids • Heartburn • Irritable after feeding Gastro-esophageal Reflux [children] (GERD) • Commonly asymptomatic • Allergy testing • Trial of H2-blocker or proton pump inhibitors • Consider GI referral for pH probe: reflux Radiographic Findings (CT, CXR) N/A N/A • Poor response to asthma Rx Vocal cord dysfunction (VCD) • Inspiratory wheeze/ stridor • Episodic dyspnea • Laryngoscopy: inspiratory vocal cord closure • Normal • Rapid onset/relief • Emotional trigger Allergic bronchopulmonary aspergillosis (ABPA) • Brownish sputum, wheezing, SOB, fever, malaise • Blood: eosinophilia • Serum precipitins to aspergillus • Very elevated IgE • Recurrent fleeting • infiltrates, bronchiectasis • Stage 0 — None Sarcoidosis – Multisystem inflammatory disorder; granulomatous changes primarily found in lung • Asymptomatic, SOB, • wheezing, cough • ACE level: Elevated hypercalcemia • Stage 1 — Hilaradenopathy • Non-caseating granulomas on biopsy • Stage II — Adenopathy + infiltrates • Stage III — Infiltrates Bronchiectasis – Airway enlargement due to previous infections • Chronic productive cough, wheezing, SOB None • Unresponsive to bronchodilator Pulmonary embolus (PE) • High Resolution CT: Localized infiltrates, airway enlargement • CT: chest PE protocol • Hemodynamic compromise • D-dimer: elevated • Sudden chest pain • ABG: hypoxemia • Presence of risk factors • Ventilation/ • Perfusion (V/Q) mismatch • CXR normal • Tachycardia CHART CONTINUES ON NEXT PAGE PULMO NO LOGY 1 09 Cystic Fibrosis • Recurrent productive cough • Sweat chloride test: abnormal • Unilateral wheeze Foreign Body • Sudden onset • Choking history • Bronchoscopy • Age: 6 months-6 years • Hyperinflation, cystic changes • CXR – Unilateral hyperinflation or atelectasis • Failure to deflate on expiratory or decubitus CXR • Premature birth: Bronchopulmonary dysplasia (BPD) • Hx prolonged mechanical ventilation/ oxygen requirement in neonatal period. If responsive to bronchodilators and steroids, treat as asthma N/A • CXR: May appear identical to asthma patients • Laryngoscopy N/A • Bronchoscopy N/A • Inspiratory or expiratory monophonic wheeze • Bronchoscopy • No bronchodilator response N/A • Inspiratory wheeze Laryngomalacia • Onset prior to 6 weeks of age • Improves when prone • No bronchodilator response • Hx of intubation Subglottic stenosis • Biphasic wheeze, loudest in neck • No bronchodilator response Tracheo/ bronchomalacia Bronchiolitis (asthma exacerbation caused by viruses) Recurrent upper respiratory infection 110 • No response to beta-2 agonist • Diffused wheeze and/or bronchi • Respiratory Syncytial N/A • Virus testing • Common cold symptoms • Reduction of respiratory symptoms after bulb N/A suction or decongestion P U LMON O LO G Y PULMO NO LOGY Minor limitation Minor limitation Some limitations Extremely limited Extremely limited > 2 days/ week, not daily Not more than once a day Daily Several times a day Several times a day Step 2 Mild Step 3 Moderate Step 4 Severe Step 5 Severe Step 6 Severe Several times a day [a] Throughout the day [a] Daily [a] > 2 days/ week, not daily [a] > 2 days/ week, not daily [a] < 2 days/ week Day FEV1 Nightly Nightly Nightly < 60% < 60% < 60% > 1x/week, 60not nightly 80% > 2x/month > 80% < 2x/month > 80% Night Symptoms Age ≥ 5 to Adult: High-dose ICS + LABA + oral corticosteroids SABA: Short acting beta agonist ICS: Inhaled corticosteroid LTRA: Leukotriene receptor antagonist LABA: Long acting beta agonist High-dose ICS + LABA + LTRA Refer to specialist Refer to specialist Refer to specialist Age 0-4: Medium-dose ICS + LABA + LTRA Age 0-4: High-dose ICS + LABA + LTRA (Consider 5-10 day course of oral corticosteroids) Medium-dose ICS + LTRA Age ≥ 5 to Adult: Medium-dose ICS + LABA Medium-dose ICS + LABA + LTRA Consider referral to specialist Consider referral to specialist Age 0-4: Medium-dose ICS + LTRA Age ≥ 5 to Adult: High-dose ICS + LABA Consider oral corticosteroids Low-dose ICS + LTRA -- Age 0-4: Medium-dose ICS or Low-dose ICS +LTRA Alternative Age ≥ 5 to Adult: Low-dose ICS + LABA or Medium-dose ICS -- -- Low-dose ICS SABA PRN Preferred Daily Medication [a] More than 2 exacerbations per year (requiring oral systemic steroids) should prompt step up in therapy NONE < 2 days/ week Step 1 Intermittent Activity limits Use of Quick relief Initial Severity LONG TERM CONTROL STEP-WISE APPROACH 111 BRONCHIOLITIS Mild Moderate Severe Wheeze None or end expiratory Entire expiration Inspiratory & Expiratory Feeding Normal Less than usual. Frequently stops feeding. More than ½ normal feed volumes. Not interested. Gasping / coughing. Less than ½ normal feeds. Oxygen No oxygen requirement May require oxygen Requires oxygen Indrawing No / mild indrawing Intercostal and / or tracheosternal Severe with nasal flaring Behaviour Normal Some / intermittent irritability Irritability and / or lethargy Prevention Hand Washing Respiratory-Isolation Contact Precautions Assessment/Diagnosis Clinical history and PE Influenza is endemic – test for flu when considering anti-viral therapy Monitoring Repeated clinical assessment Cardiac & Respiratory rate monitoring – acute stage: risk of apnea &/or bradycardia O2/Medication O2 when <92% (awake Consider wean when>95% Single trial inhalation racemic epi or albuterol (FHx: allergy,asthma,atopy) Continuous pulse Ox – initial assessment and to determine stability Stable: spot check pulse ox with vitals Education care of child with bronchiolitis Prevention http://patiented.aap.org/content.aspx?aid=6347 Admission Criteria Individual assessment Indications for inpatient monitoring and assessments. Inhalations Scheduled or serial inhalation therapies not to be used routinely Do not repeat if no improvement after trial 112 P U LMON O LO G Y Hypertonic Saline Inhalations Studies have not been able to universally validate this therapy Clinical Decision to use therapy 3% hypertonic Used with bronchodilator therapy Hypertonic saline continue to be used for duration of nebulization therapies. Corticosteroids NOT RECOMMENDED Antibiotics Not be used in ABSENCE of identified bacterial focus. Other medications Do NOT use IVIG Montelukast rhDNAase inhaled furosemide OTC remedies DO NOT USE antihistamines, oral decongestants, & nasal vasoconstrictors. Other Therapies Not be used routinely aerosol with saline chest PT (unless used with neb therapies) Diagnostic Testing Do NOT perform routinely CXR Cultures CBG, ABG RSV/Influenza testing will not influence management but may be useful for COHORT, or ascertain need for antiviral PULMO NO LOGY 113 114 P U LMON O LO G Y Pancrelipase (Creon, Pancreaze) Pancreatic Enzyme Replacement Therapy (PERT) – can cause fibrosing colonopathy, diaper dermatitis, oral lesions (if chewed) Vitamins A, D, E, K (AquaDEKs) Fat-soluble vitamin replacement Dornase alfa (Pulmozyme) Mucolytic (nebulized) Hypertonic (7%) saline Mucolytic (nebulized) – can cause bronchospasm N-acetylcysteine (Mucomyst) Azithromycin Ibuprofen PULMO NO LOGY Mucolytic (nebulized) Anti-inflammatory Anti-inflammatory 115 NOTES 116 P U LMON O LO G Y SECTION 12 Renal Calculations: GFR and FeNa............................................................................... 117 Urinalysis Interpretation.......................................................................................118 Hematuria............................................................................................................120 Proteinuria............................................................................................................121 Acute Kidney Injury..............................................................................................121 Indications for Acute Dialysis............................................................................. 123 Hyperkalemia, Hypernatremia, Hyponatremia................................................. 123 Hypertension....................................................................................................... 124 Nephrotic Syndrome.......................................................................................... 127 Glomerulonephritis............................................................................................. 128 Renal Tubular Acidosis (RTA).............................................................................. 129 Hemolytic Uremic Syndrome (HUS) vs Henoch-Schonlein Purpura (HSP).... 130 Chronic Kidney Disease...................................................................................... 131 Prenatal hydronephrosis algorithm................................................................... 132 Electrolytes.......................................................................................................... 134 GFR CALCULATION Modified Schwartz: GFR = 0.413 x height (cm) Plasma creatinine If Creatinine is doubling daily → GFR is 0 *Patients with CrCl < 50 may need dose adjustments on medications RE NA L 117 FRACTIONAL EXCRETION CALCULATION * Only useful in oliguria or hypo/hypernatremia, unreliable if recent Lasix administration Fractional Excretion of Sodium (FENa) = (PCr*UNa)/PNA x UCr) % Prerenal Intrinsic Renal Postrenal FENa <1% >1% >4% UNa (mmol/L) <20 >40 >40 Prerenal: Anything that causes decreased effective renal perfusion: Hypovolema, CHF, Renal Artery Stenosis, Sepsis, etc. Remember, contrast-induced nephropathy will often look pre-renal. Intrinsic Renal: ATN, AIN, Glomerulonephritides, etc. Postrenal: Obtrusion (BPH, bladder stone, bilateral ureter obstruction) URINALYSIS INTERPRETATION Dipstick •Color: pink, green, white urine likely secondary to medications (green = propofol), consider UTI or metabolic errors. Grossly red urine = hematuria only if microscopy reports RBC too numerous to count (otherwise think rhabdomyolysis or hemolytic anemic) 118 RE N AL •Glucose: positive suggests systemic glucose > 180 or proximal tubule defect •Glucose in the urine does not always mean diabetes (steroids given?) •Isolated glycosuria with normal serum glucose is commonly due to a different set point for glucose reabsorption in the proximal tubule, but requires Fanconi’s eval. •Fanconi syndrome: spill glucose, phosphate, uric acid, bicarb, and amino acids (work up: chem 10 and VBG checked simultaneously with urine phosphate and urine creatine; urine amino acids any time) •Bilirubin: reflects elevated serum conjugated bili levels, consider liver or gallbladder disease •Unconjugated bilirubin is not water soluble, so not excreted in urine •Ketones: suggests ketosis (using fat instead of glucose for fuel) •consider serum beta-hydroxybuterate levels •anorexia, prolonged vomiting, high protein low carb diet, burns, fever, severe illness, hyperthyroidism, diabetes •Specific Gravity: depends on number and size of particles, varies with osmolality • Normal findings: 1.003-1.030 • 1.008: isotonic with plasma (280 mOsm/kg) •< 1.003: maximally dilute •After IV contrast given, urine spec grav will go way up for 24-48 hours •Blood: hematuria, free hemoglobin, or free myoglobin (ie rhabdomyolysis) •See flowsheet above, hematuria section below •False positive: semen present in urine •pH: 4.5-8, reflects systemic acid-base status •In metabolic acidosis, urine pH should be < 5. If not, consider distal RTA (with proximal RTA, you can still bring your urine pH < 5) •pH > 7.5, consider UTI (urease producing, ex: proteus mirabilis) or hypokalemia •Protein: negative to trace is normal. Trace proteinuria does not need aggressive work up. •Urobilinogen: formed in intestines by bacteria acting on bilirubin •High: hemolytic anemia, liver disease •Low: obstructive jaundice, broad-spectrum antibiotics (loss of intestinal flora) •Interpret with urine bilirubin (high bilirubin with low urobilinogen suggests obstructive gallbladder disease, because bili didn’t go into intestines) •Nitrite: suggests bacteriuria; positive when bacteria able to convert urinary nitrate to nitrite •False negative: bacteria with minimal nitrate reductase (ex: enterococcus), or minimal dwell time in the bladder •Leuko Est: released by WBC and macrophages; dilute urine favors cell lysis and may cause falsepositives (compare to WBC count on microscopy) •Sterile pyuria (culture negative): consider interstitial nephriits, renal tuberculosis, nephrolithiasis, Kawasaki, urethritis, non-cath sample •Clinitest: presence of reducing substance (glucose, lactose, fructose, galactose, pentose) •If no glucose, consider galactosemia or other inborn error of metabolism •May be secondary to beta-lactam antibiotics •Confirm normal newborn screen RE NA L 119 HEMATURIA Definition >5 RBC per high power field in 3 consecutive fresh, centrifuged specimens obtained over the span of several weeks or gross hematuria on single urine specimen Red Flags: •Elevated or rising creatinine •Hypertension •Proteinuria Causes: Renal (glomerular) Extrarenal / Urologic Color Tea, Cola, Smoky Red, Pink RBC Morphology Dysmorphic Normal Casts RBC --- Clots ---- +/- Proteinuria >2+ Usually <2+ Stream Throughout Partial Extrarenal / Urologic: Renal: Urinary tract infection, irritation of meatus/ perineum, trauma (catheterized sample?), nephrolithiasis, coagulaopathy, renal vessel thrombosis, nutrcracker syndrome, neoplasm glomerulonephritis Work-up: *Carefully weigh risk of IV contrast for CT if concerned for poor renal perfusion or obstruction >5 RBC per high power field Yes - Abd/Pelvis Imaging (CT, US, KUB) - Consider cystoscopy 12 0 No Trauma? (Clots?) Yes - Urine Ca/Cr - Consider UTI (+ nitrite, LE, WBC?) - Image for stones (US if possible intrarenal vs CT stone protocol) - US with Doppler r/o RVT Pain? No - PMHx (sickle? Coagulopathy?) - Meds (ASA, NSAIDs, PCN, Lasix?) - Family history? / family UAs - Serum Cr, Urine Ca/Cr, Urine Protein /Cr - Renal US +/- dopplers, consider CT - Work up glomerulonephritis: ASO, ANA, C3/C4, dsDNA, IgA, electrolytes, albumin - Consider biopsy (rarely needed) RE N AL PROTEINURIA Definition: Protein : Creatinine 24 hour sample m2/hr Abnormal > 0.2 (>0.5 if <2yo) > 150mg (varies by age/BSA) >4mg Nephrotic Range* >2 > 3.5g >40mg *Nephrotic Syndrome: proteinuria, hypoalbuminemia, hyperlipidemia, +/- edema Causes To determine glomerular vs tubular proteinuria: microalbumin vs beta-2-microglobulin 1.Glomerular (increased filtration): nephrotic syndrome, glomerulonephritis •non-pathological: secondary to fever, exercise, seizure, orthostasis 2.Tubular (decreased proximal tubular reabsorption): low molecular weight proteins, Fanconi syndrome 3.Overflow: very rare in children (multiple myeloma in adults) Work-up: •To rule out transient/orthostatic proteinuria, compare first morning sample to QHS sample •Assess for other findings supportive of renal disease: change in urine volume or color, edema, hypertension, recent streptococcal infection (pharyngitis in past 2 weeks/dermatitis in past 3 weeks), recent URI/AGE in past 24 hours with new onset gross hematuria (suggests IgAN), family history of high frequency sensorineural hearing loss or anterior lenticonus (suggests Alport disease) •Chem 10, cholesterol, albumin; consider C3/C4, ANA, ASO, Hep B, Hep C, HIV •Consider renal ultrasound, referral for possible renal biopsy •See Nephrotic Syndome for more information ACUTE KIDNEY INJURY Definition: •Declining GFR, decreased UOP retention of nitrogenous waste, volume & electrolyte dysregulation •No single pediatric definition, especially difficult if baseline creatinine unknown Calculate eCCl by Schwartz equation RE NA L 121 Pediatric RIFLE Classification of acute kidney injury pRIFLE stage Estimated creatinine clearance (eCCl) Urine output R = Risk for renal dysfunction eCCl decreased by 25% <0.5 mL/kg per hour for 8 hours I = Injury to the kidney eCCl decreased by 50% <0.5 mL/kg per hour for 16 hours F = Failure of kidney function eCCl decreased by 75% or eCCl <35 mL/min per 1.73m2 <0.3 mL/kg per hour for 24 hours or anuria for 12 hours L = Loss of kidney function Persistent failure > 4 weeks E = End-stage renal disease Persistent failure > 3 months Causes: Mechanism Etiology Decreased Intravasacular Volume Dehydration, hemorrhage, diuretics, burns, shock, hypoalbuminemia Decreased cardiac function Heart failure, arrhythmia Peripheral vasodilation Sepsis, anaphylaxis, antihypertensive meds Renal vasoconstriction Sepsis, NSAIDS, ACE-inhibitor Tubular injury (ATN) Prolonged ischemia, nephrotoxins, hypotension, sepsis Renal vascular disease Hemolytic uremic syndrome, vasculitidies, thrombosis Interstitial disease Interstitial nephritis, infection, malignant infiltration Glomerulonephritides Post-infectious glomerulonephritis, rapidly progressive glomerulonephritis, HenochSchonlein purpura Obstruction: bilateral urinary tract obstruction, or obstruction of urinary tract of solitary kidney Renal calculi, clots, urinary retention secondary to neurogenic bladder or medications Pre-Renal BUN: CR FeNa > 20:1 < 1% Intrinsic Renal BUN: CR FeNa < 20:1 > 1% Post-Renal BUN: CR FeNa 12 2 < 20:1 > 4% RE N AL INDICATIONS FOR ACUTE DIALYSIS •Needed when metabolic/fluid derangements are not controlled by aggressive medical management, or for some toxic ingestions •Requires transfer to Rady Children’s Hospital (pediatric dialysis not currently available at NMCSD) Indications *Creatinine level alone is not an indication for dialysis Mnemonic: A – E – I – O – U Acidosis – Electrolytes – Intoxications – Overload – Uremia (see list on following page for details) •Volume overload with pulmonary edema or hypertension refractory to diuretics •Hyperkalemia > 6 (if hypercatabolic) or > 6.5 •Metabolic acidosis (pH < 7.2, HCO3 < 10) •BUN > 150 or rising rapidly •Neurological symptoms secondary to uremia and/or electrolyte imbalances •Dialyzable toxin or poison (lactate, ammonia, ethanol, barbiturate, ethylene glycol, isopropanolol, methanol, salicylates, theophylline) •Tumor lysis syndrome with hyperuricemia not controlled medically (rasburicase, urinary alkalinization, induction of high UOP). HYPERKALEMIA •Recheck K; hemolysis unlikely to have K > 6.5 •d/c K in IVFs and check EKG, place on CRM •If K 6 - 7 and no EKG changes- Kayexelate 1g/kg/dose PO Q6h •If K >7 or EKG changes •Calcium gluconate 100 mg/kg/dose over 3-5 mins (may repeat in 10 mins) •Sodium bicarb 1-2 meq/kg IV over 5-10 mins •Insulin ( 0.1-0.3 U/kg) + glucose (1 g/kg) over 2h •Furosemide RE NA L 1 23 HYPERNATREMIA Symptoms neurologic (lethargy, weakness, altered mental status, irritability, seizure, decreased DTRs); muscle cramps, respiratory failure Treat Replace free water losses, treat cause, consider natriuretic Free Water Deficit (mL) = 4mL/kg x weight (kg) x [serum Na concentration – 140] HYPONATREMIA Symptoms nausea, headache, lethargy, seizure, coma Secondary causes Na decreased by predictable amount in: •Hyperlipidemia: 0.002 x lipid (mg/dL) •Hyperproteinemia: 0.25 x [protein (g/dL) – 8] •Hyperglycemia: 1.6mEq/L for each 100-mg/dL rise in glucose Treat Replace Na losses or restrict fluids, pending etiology ACUTE HYPERTENSION Definition Normal BP depends on age, sex, and height; see Harriet Lane pg 156-163 for data •Mean Arterial Pressure (MAP): 1/3 systolic + 2/3 diastolic BP CONTINUES ON NEXT PAGE WITH CHART 124 RE N AL Hypertensive Urgency Hypertensive Emergency Definition Significant elevation in BP without end-organ damage Elevation of systolic and diastolic BP with endorgan damage Symptoms headache, blurry vision, nausea cerebral infarction or hemorrhage, pulmonary edema, renal failure, encephalopathy, seizure Etiology increased ICP (must be ruled out before lowering BP), cardiovascular, renovascular, renal parenchymal, endocrine, CNS dysautonomia, medications, ingestions Physical Exam Simultaneous RUE and RLE BP, fundoscopy (papilledema, hemorrhage, exudate), visual acuity, thyroid exam, congestive heart failure signs (tachycardia, gallop, hepatomegaly, edema, JVD), abdominal mass, bruit, detailed neuro exam, virilization, cushingoid Labs Chem 10, TSH/FT4, renin, aldosterone, UA, tox screen Add if pheochromocytoma suspected: Urine catecholamines, plasma fractionated metanephrines Imaging EKG, CXR, Renal ultrasound Abdominal ultrasound r/o mass Echocardiogram CT Head Treatment Goal Lower MAP by 20% over 1 hour then return to baseline over 24-48 hours Lower BP promptly but gradually to preserve cerebral autoregulation; lower MAP by 1/3 of goal over first 6 hours, next 1/3 over 24-3 hours, then final 1/3 over next 48 hours Transfer to PICU for continous BP monitoring Drug Mechanism Onset Duration Increase Dose Comments Diazoxide Arteriole vasodilator 1-5min (IV) 2-12h 15-30min May cause edema, hyperglycemia Hydralazine Arteriole vasodilator 5-20min (IV) 2-6h 4-6h May cause reflex tachycardia, prolonged low BP, nausea Nitroprusside (drip) Arteriole Venous vasodilator < 30sec (IV) Very short 30-60min ICU, follow thiocyanate levels Labetalol (drip) Alpha-, betablocker 1-5min (IV) ~6h 10min ICU Nicardipine (drip) Ca channel blocker 1min (IV) 3h 15min May cause edema, headache, nausea, vomiting Enalapril ACE-inhibitor 15min (IV) 12-24h 8-24h May cause hyperkalemia, hypoglycemia, contraindicated in renal artery stenosis Minoxidil Arteriole vasodilator 30min (PO) 2-5 days 4-8h Contraindicated in pheochromocytoma RE NA L 125 CHRONIC HYPERTENSION Definition Normal BP depends on age, sex, and height; see Harriet Lane pg 156-163 for data Etiologies •Factitious: improper cuff size or measurement technique (ask for manual) •- Appropriate cuff size: 2/3 upper arm length with bladder cuff 80-100% of arm circumference •Non-pathologic: fever, pain, anxiety, muscle spasm •Iatrogenic: medication side effect, excessive fluid resuscitation Age Most common Less Common Neonate/Infant - Renal artery thrombosis after umbilical artery catheterization - Coarctation - Renal artery stenosis - Bronchopulmonary dysplasia Medications PDA (patent ductus arteriosus) grade 4 IVH (intraventricular hemorrhage) 1-10y - Renal parenchymal disease (VURN) - Coarctation - Renal artery stenosis Hypercalcemia Neurofibromatosis Neurogenic tumor, pheochromocytoma Mineralcorticoid increase Hyper/hypothyroid Obstructive sleep apnea Essential hypertension Medications 11-21y - Renal parenchymal disease - Essential hypertension All diagnoses above Evaluation: •See acute hypertension for physical exam, labs, and imaging recommendations •Consider assessing co-morbidities: polysomnography, fasting lipids, fasting glucose Treatment: •Non-pharmacologic: aerobic exercise, salt restriction, smoking cessation, weight loss x 6 months •Pharmacologic: for BP >95-99%ile, secondary hypertension (if operative management not recommended), end-organ damage (echocardiogram, retinal exam), comorbid diabetes mellitus, or failed life-style modification after 6 months •See Harriet Lane for options 12 6 RE N AL NEPHROTIC SYNDROME Definition •renal diseases that increase the permeability across the glomerular filtration barrier •Nephrotic range proteinuria (>3.5g/day, protein:cr ratio > 2), hypoalbumenia (serum < 3), hyperlipidemia, +/- edema Etiologies determine if active urine sediment (red cell casts), which would suggest glomerular inflamation and be more consistent with nephritic syndrome. Primary Nephrotic Syndrome (no identifiable systemic disease) Minimal change disease (MCD) 77% 2-6yo, no HTN when normovolemic, normal complement, normal renal function; steroid trial before biopsy Membranoproliferative glomerulonephritis (MPGN) 8% Immune complex (low C4) vs complement mediated (low C3) Focal segmental glomerulosclerosis (FSGS) 7% Membranous nephropathy 2% (most common cause in non-DM adults) Secondary Nephrotic Syndrome Systemic lupus, HSP, vasculitides, HUS Infectious Hep B, Hep C, HIV Drugs NSAID, Lithium, Pamidronate Malignancies lymphoma, leukemia Inherited Genetic Disorders Sickle cell disease Associated with secondary FSGS Alport syndrome abnormal type IV-alpha 5 collagen, hearing loss , x-linked, anterior lenticonus (pathognomonic for Alport’s in absence of penetrating eye trauma) Rare genetic disorders 85% of all nephrotic syndrome in infants < 3mo of age, poor outcomes (ex: Finnish CNS or Denys-Drash) Evaluation •urine protein:creatinine ratio, lipid panel, chem 10; renal ultrasound •Evaluate for trigger of nephrotic flare: infection, medication/toxin •Evaluate for secondary causes: ANA, dsDNA, ANCA, Hep B, Hep C, C3, C4 Treatment: •Fluid overload: albumin/diuretic therapy: 25% albumin 1gram/kg (max 25g) IV over 1 hour if not hypertensive or 2 hours if hypertensive with lasix 1-2mg/kg IV given immediately after albumin infusion RE NA L 127 •- Salt restrict (1.5-2g/day) and fluid restrict (variable, approximately 400 mls/m2 + 750 mls/day) •- Place PPD in anticipation of steroids for immunosuppression; 2 mg/kg/day divided BID for 4 weeks followed by taper; consider solumedrol 60mg/m2 IV only if concerned that gut edema is preventing oral steroid absorption •- Only 10% of kids < 10yo fail steroids; if non-responsive, needs renal biopsy GLOMERULONEPHRITIS Definition glomerular injury due to inflammation, produces active urine sediment •Hematuria (microscopic or macroscopic, dysmorphic RBC and RBC casts pathognomonic), proteinuria (may be up to nephrotic range), hypertension, acute kidney injury Etiologies narrow differential based on low or normal complement Primary Glomerulonephritis Low complement levels Normal complement levels Membranoproliferative Glomerulonephritis (MPGN) Type 1 vs Type 2 (dense deposit disease) Berger’s disease IgA nephropathy Goodpasture’s disease Anti-glomerular basement membrane disease Idiopathic crescentic glomerulonephritis Secondary Glomerulonephritis Low complement levels Post-streptococal GN Skin or throat, Group A strep Other post-infectious GN Endocarditis, visceral abscess, shunt nephritis, pneumococcal infection, typhoid, EBV, parvo B19, CMV, coxsackie, rubella, mumps, Hep B, malaria, toxo, filaria, schistosoma Lupus Median age onset 12y, about 50% with renal disease at time of diagnosis Henoch-Schonlein Purpura Normal complement levels ANCA+ glomerulonephritis (granulomatosis with polyangiitis, microscopic polyangiitis) Evaluation serology for recent strep infection (ASO titers), ANA, dsDNA, C3, C4, IgA level; consider CH50, 12 8 RE N AL ANCA; consider serologic testing for EBV, Hep B, and Hep C, anti-GBM (if pulmonary symptoms) Rapidly Progressive Glomerulonephritis (RPGN) rare in children; may be caused by any immune mediated process, though anti-GBM, ANCA, and HSP most common •Elevated creatinine which continues to climb •50% of glomeruli affected by crescents on renal biopsy histology •Treat with steroids RENAL TUBULAR ACIDOSIS (RTA) Definition renal abnormality causing loss of bicarb in urine; consider in differential of non-anion gap hyperchloremic metabolic acidosis Evaluation blood gas, urinalysis, urine electrolytes (to calculate urine anion gap), chem panel Type One (distal) Type Two (proximal) Primary defect Impaired distal acidification; can not secret H+ Reduced proximal bicarb absorption; also spills Decreased aldosterone phos, glucose, other secretion or effect electrolytes Plasma HCO3 Variable, possible < 10 Usually 12-20mEq/L Greater than 17mEq/L Urinary pH Greater than 5.3 Variable, above 5.3 if above bicarb resorption threshold Usually less than 5.3 Plasma K Usually low, may correct with bicarb rx Usually low, worsens with bicarb rx Increased Causes Familial (AD or AR), hypercalciuria, SLE, obstructive uropathy, amphotericin B, hyperglobulinemia, sickle cell, lithium, renal transplant Cystinosis, tyrosinemia, hereditary fructose intolerance, galactosemia, glucogen storage disease type 1, Vit D deficiency, renal transplant, paroxysmal nocturnal hemoglobinuria, heavy metals, Fanconi’s syndrome Primary adrenal insufficiency, CAH (esp 21-OH), heparin, ACEinhibitors, NSAIDS, aldosterone resistence (aldactone, triamterene, amiloride, trimethoprim), tubulointerstitial disease Treatment 1-2mEq/kg/day bicarb, decrease Na intake to decrease Ca exchange at distal tubule 10-15mEq/kg/day bicarb (bicarb ingestion often leads to bicarb diuresis and excess K losses) 1-5mEq/kg/day bicarb; consider diuretics and fludrocortisone; restrict dietary K Nephrocalcinosis? Common Not common Absent RE NA L Type 4 (hypo-aldo) 129 HUS & HSP Features Differential Causes Prodrome Evaluation Hemolytic Uremic Syndrome Henoch-Schonlein Purpura = IgA vasculitis 1. Microangiopathic hemolytic anemia 2. Thrombocytopenia 3. Acute Kidney Injury 1. Palpable purpura (normal plt & coag) 2. Arthritis/Arthralgia 3. Abdominal pain / Intussusception 4. Renal disease DIC, TTP, systemic vasculitis Varies pending initial complaint (purpura, joint pain, abdominal pain; renal disease late) Typical (diarrhea): Shiga toxin- producing E. coli (STEC) or Shigella Atypical (no diarrhea): strep pneumoniae, HIV, H1N1 influenza; genetic disorders of complement or cobalamin C, chemotherapy, post-transplant immunosuppression Immune-mediated vasculitis associated with IgA deposition; variety of infectious and chemical triggers; undetermined pathogenesis with immune, genetic, and environmental factors Typical HUS (>90%): abdominal pain, vomiting, bloody diarrhea May follow bacterial or viral infection Pneumococcal: 70% complicated pneumonia, 20-30% meningitis; consider bactermia, sinusitis, otitis CBC with peripheral smear (anemia , thrombocytopenia, schistocytes, helmet cells), renal function panel, urinalysis; coag panel (to differentiate from DIC); infectious studies (stool and blood culture, CXR, HIV); for atypical, C3, C4, ANA, factor H mutations Clinical diagnosis (classic: purpura on legs and buttocks); consider skin biopsy if needed (diagnostic if leukocytoclastic vasculitis with IgA predominance), serum IgA elevated in 50-70% Urinalysis; confirm normal plt and coag; consider serum creatinine if abnormal UA Supportive: PRBC for Hgb < 6, platelets for clinical bleeding or invasive procedures, fluid/electrolyte monitoring and management; watch for indications for acute dialysis Treatment Consider Eclulizumab (C5 antibody) for familial HUS, consider plasmapheresis or plasma infusions Avoid abx if HUS thought to be due to shiga toxin Pneumococcal: broad double coverage (cefotaxime, vancomycin) Supportive: hospitalization if inability to orally hydrate, severe abdominal pain, GI bleeding, altered mental status, unable to ambulate due to joint pain; elevated creatinine, hypertension, or proteinuria Monitor: blood pressure, urine output, abdominal exam, stool guaiac Consider: NSAIDs, steroids, abdominal imaging, surgical consult CHART CONTINUES ON NEXT PAGE 13 0 RE N AL Outcome Follow-up Typical: <5% mortality; hematologic lines recover in 1-2 weeks, followed by renal function recovery; 5-25% permanent renal disease Short- and long-term prognosis excellent, resolves within 1 month; recurrence in 1/3 (usually less severe episode), usually within first 4 months Pneumococcal: more severe initial disease, longer duration oliguria, requires more transfusions; 12% mortality, 10% ESRD, 16% CKD Short-term morbidity from surgical GI complications (intussception, usually ileoileal so best diagnosed by US not by barium enema); longterm from renal disease (20-54% with renal disease, 90% within first 2 months, more likely if >8yo) Annual: blood pressure, urinalysis, renal function panel Weekly or bi-weekly UA and BP for 4 months, then at subsequent well checks CHRONIC KIDNEY DISEASE Classification guides management by stratifying the risk of progression and complications GFR Category GFR (mL/min/1.73 m2) Terms G1 >90 Normal or high G2 60-89 Mild decrease G3a 45-59 Mild to moderate decrease G3b 30-44 Moderate to severe decrease G4 15-29 Severe decrease G5 <15 Kidney failure Selected Associated Problems Cause Diagnosis Intervention Fluid and Electrolyte shifts, Edema Oliguria, anuria, or high urine output History, urine output Diuretic therapy, fluid restriction, dialysis Anemia Poor EPO production, low iron CBC, retic, ferritin, TIBC, serum Fe Epogen, iron supplement Osteodystrophy Failure to convert Vit D to active form, acidosis, nutritional deficiency, hyperparathyroid Ca, Phos, iPTH, Vit D 25-OH Vit D (cholecalciferol); if Vit D deficient usually need 1,25-OH Vit D (calcitriol = Rocaltrol or paricalcitol = Zemplar) CHART CONTINUES ON NEXT PAGE RE NA L 1 31 Phos Phosphate binders (PhosLo = CaAcetate, TUMS = CaCarbonate, Sevelamer with meals, low phos diet Growth curve Improve electrolytes and nutritional intake, consider growth hormone Hyperphosphatemia Poor excretion Poor nutrition and growth Acidosis, anorexia, growth hormone binding protein irregularities Hypertension Fluid status, vasoactive Blood pressures, LVH substances from on echo kidney Low Na diet, antihypertensives * Hemodialysis patients often get their Epopoetin and 1,25-OH Vit D as part of dialysis prescription PRENATAL HYDRONEPHROSIS ALGORITHM 13 2 RE N AL RE NA L 133 ELECTROLYTES 13 4 RE N AL NOTES RE NA L 135 SECTION 13 Appendix Crawford’s AED Table......................................................................................... 125 Cystic Fibrosis Pathway..................................................................................... 128 Spirometry Interpretations in Adults.................................................................. 129 Evaluate spirometry and response to bronchodilator...................................... 130 Sickle Cell Disease and Pulmonary Complications........................................... 131 Urinary Casts....................................................................................................... 132 13 6 A P P E N D IX APPE NDIX 137 13 8 A P P E N D IX APPE NDIX 1 39 CYSTIC FIBROSIS PATHWAY 140 A P P E N D IX APPE NDIX 1 41 142 A P P E N D IX SICKLE CELL DISEASE AND PULMONARY COMPLICATIONS Test or Therapy Frequency Rationale Review of Systems for atopy and asthma. (All children with SCD) Annually, starting at one year of age. If history is positive, refer to allergy or asthma specialist. Atopy is a risk factor for asthma. Children with asthma and SCD are at increased risk of vaso-occlusive episodes (VOE) and ACS. Assessment of lung function by spirometry (All children with SCD) Annually in all children with SCD starting at 6 years of age. With every visit for children with SCD and asthma, max 4 times per year. Bronchodilator challenge should be done in children with obstruction (FEV1/FVC less than 95% CI). Children with SCD may have obstructive and restrictive defects. Starting at 6 years of age: Every 5 years in children with no asthma or ACS episodes. Every 2-3 years in children with asthma or ACS episodes. Children with SCD may have obstructive and restrictive defects. Assessment of defects in lung function by lung volumes with plethysmography (All children with SCD) Treatment of children with SCD and asthma per NHLBI guidelines (All children with SCD) Review of Systems for Sleep Disturbed Breathing (Sleep Apnea) (All children with SCD and asthma) Indefinitely. Once diagnosed with asthma, children should be assessed at least every 6 months for persistent symptoms. Treatment of persistent asthma in children without SCD with daily inhaled corticosteroids is effective in reducing asthma hospitalizations and symptom days. Annually by history. If history is positive, refer to pulmonologist for evaluation. Children with nocturnal hypoxemia have higher rates of pain (VOE). At least annually for children Appointment with with SCD and mild asthma. Pulmonologist, Allergist or At least every 6 months Asthma Specialist (All children for children with SCD and with SCD and asthma) moderate to severe asthma. Children with asthma and SCD are at increased risk of vasoocclusive episodes (VOE) and ACS. Assessment for elevated TR Jet velocity and pulmonary hypertension by 2-D Echocardiography (All children with SCD) A tricuspid jet velocity greater than 2.5 m/s is associated with an increased risk of death in adults. Effective intervention not well defined. APPE NDIX At least once between ages 16-18 years. 143 URINARY CASTS •Formed in tubular lumen (ie diagnostic of intrarenal origin); matrix = Tamm-Horsfall mucoprotein Red Blood Cell Glomerulonephritis Interstitial Nephritis White Blood Cell Pyelonephritis Interstitial Nephritis Proliferative Glomerulonephritis Renal Tubular Epithelial Cells Acute Tubular Necrosis (ATN) Interstitial Nephritis Proliferative Glomerulonephritis Granular Acute Tubular Necrosis (ATN) Broad Formed in large dilated tubules with little flow = advanced CKD * Waxy casts: nonspecific, seen in chronic and acute kidney disease * Hyaline casts: nonspecific, seen in small volumes of concentrated urine or with diuretic use 144 A P P E N D IX NOTES OLD IRONSIDES NMCSD Pediatric Ward Handbook Last updated 08/2014