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Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September 22, 2010 Outline Case presentation: Cough, Hypoxia, and Down Syndrome Differential Diagnosis Multi-system complications of patients with Trisomy 21 Treatment and Monitoring Chief Complaint 9 month old female with Down Syndrome referred to pulmonary clinic for initial evaluation because of cough and persistent oxygen requirement History of Present Illness Daily cough for months - day and night No increased work of breathing Snoring Gags with jejunal feeds Treated with Ranitidine Severe oral aversion, tastes by mouth Past medical/surgical history Trisomy 21 Born at 36 weeks in Colorado Esophageal atresia without TEF GT placment DOL 1 Gastric pull-through at 4 months On and off oxygen Ventilated x 1 week after surgery Discharged at 5 months on ¼ lpm O2 via nasal cannula PMH/PSH PDA ligation at 5 months of age Recent echo revealed – “normal function, small left to right ASD, mild TR” Monthly esophageal dilations – tolerated well Meds at visit Ranitidine Spironolactone/hydrochlorothiazide ¼ lpm oxygen No inhaled medicines No steroids Review of Systems No fevers Adequate growth No hemoptysis No vomiting No steatorrhea Normal thyroid No hematuria Sitting with support Family and Social History No asthma, allergies, or lung disease in the family Lives with mom, “adopted grandparents” in Denver Parents are from Senegal No known TB exposures No pets No smokers Physical Exam Vitals HR 136 | RR 28 | Ht 65 cm (43%) | Wt 7.5 kg (39%) SaO2 88% RA | SaO2 95% ¼ lpm General: happy baby, + drooling HEENT: Down’s facies, small nares, +rhinorrhea Chest: Easy work of breathing, clear to auscultation, prolonged expiratory phase CVS: RRR, normal S1 and S2, no murmur Abd: Soft, non-tender, no hepatosplenomegaly Ext: No clubbing Neuro: Decreased truncal tone Chest Xray at 8 months of age What should we do? What should we do? Differential Diagnosis – cough, hypoxia Reflux / aspiration Pulmonary edema Airway anomaly / poor airway clearance Asthma Interstitial lung disease Pneumonia/infection What should we do? Differential Diagnosis – cough, hypoxia Reflux / aspiration Pulmonary edema Airway anomaly / poor airway clearance Asthma Interstitial lung disease Pneumonia/infection Because of her Down Syndrome, she is at risk for aspiration, heart disease, tracheal anomalies, obstructive sleep apnea, pulmonary hypertension Bronchoscopy Erythematous mucosa Copious clear secretions Mild-mod malacia of trachea and both mainstem bronchi COLOR CHARACTER NUCLEATED CELLS RBC RBC MORPH SEGS LYMPHS MONOCYTES MACROPHAGES COLORLESS HAZY 910 1705 NORMAL 55 18 7 17 LIPID INDEX = 0. IRON INDEX = 270. Bacterial and viral cultures: negative What should we do now? Cause of increased iron index? Treatment? Disorders with pulmonary capillaritis Disorders without pulmonary capillaritis Noncardiovascular Cardiovascular Chronic heart failure Pulmary Hypertension Pulmonary veno-occlusive disease Follow-up via phone New symptoms Rhinorrhea Increased cough, respiratory rate Sleeping more Requiring ½ - 1 lpm O2 Intervention 5 days of oral steroids Follow-up Outpatient echocardiogram scheduled Improved for 2 weeks, then symptoms returned Treated with 5 more days oral steroids by her PCP Symptoms do not resolve – present to ED Further history – taking liquids by mouth for 6 weeks as instructed by therapy Physical Exam in ED Vitals HR 180 | RR 62 | SaO2 94% 3 lpm General: Infant in moderate respiratory distress HEENT: Down’s facies, +rhinorrhea Chest: Subcostal retractions, tachypneic, coarse symmetric breath sounds CVS: Tachycardic, prominent S2, 2/6 systolic flow murmur at LLSB Abd: Soft, non-tender, Liver down 3 cm 8 months of age 12 months of age in ED What should we do? What should we do? Differential Diagnosis – cough, hypoxemia, prominent S2, hepatomegaly Pulmonary hypertension Aspiration Heart failure Infection Laboratory CBC 11.6 WBC 81% Segs, 8% Lymphs, 10% Monos Hb 16.3 g/dL / Hct 51.4 % Platelets 221 CBG pH 7.43 CO2 34 mm Hg Echocardiogram Small secundum ASD with bidirectional flow. Moderate right heart enlargement and moderate septal flattening. Normal left ventricular size and systolic function. Low normal RV systolic function. Systemic pulmonary hypertension (on 3 lpm NC O2). TV jet 4.48 m/second RV-RA grad 80 mm Hg (SBP 90/69) Pulmonary Hypertension Causes? Pulmonary Hypertension Causes of secondary PAH Cardiac/Vascular anomaly Chronic lung disease Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months) Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV Pulmonary Hypertension Causes of secondary PAH Cardiac/Vascular anomaly Chronic lung disease Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months) Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV Cardiac catheterization 30% FiO2 Qp/Qs = 1.25:1; Rp/Rs = 0.46 21% FiO2 Qp/Qs = 1.25:1; Rp/Rs = 0.64 100% FiO2 with 40 ppm iNO Qp/Qs = 1.14:1; Rp/Rs = 0.47 Pulmonary Hypertension Causes of secondary PAH Cardiac/Vascular anomaly Chronic lung disease Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months) Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV Chest CT Chest CT - prone Esophagram •Thread-like appearance of distal esophagus •Fundus of stomach superior to the diaphragm •No normal peristalsis – movement of feeds only with gravity Swallow study •Deep laryngeal penetration with thin liquids. •No aspiration with pureeds. Pulmonary Hypertension Causes of secondary PAH Cardiac/Vascular anomaly Chronic lung disease Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months) Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV Polysomnogram Mild sleep-disordered breathing Apnea-hypopnea index of 3 events/hour SpO2 in low 80s in RA SpO2 in mid 90s on ¼ lpm NC (≥92% for 99% of TST) Obstructive Sleep Apnea does not explain PAH Pulmonary Hypertension Causes of secondary PAH Cardiac/Vascular anomaly Chronic lung disease Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months) Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV Further laboratories Protein C and S Prothrombin Antithrombin III Lupus Anticoagulant Factor V Leiden Homocysteine Beta 2 GP1 (antiphospholipid) antibodies Cardiolipin IgG and IgM Pulmonary Hypertension Causes of secondary PAH Cardiac/Vascular anomaly Chronic lung disease Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months) Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV Further laboratories ANA, ESR, CRP TSH and free T4 HIV Treatment of PAH – Vasodilation and Diuretics Continuous oxygen Oral Sildenafil – started at 0.5 mg/kg/dose and titrated to 2 mg/kg/dose q6h Furosemide 1 mg/kg/dose TID Thromboembolic disease can contribute to PAH Elevated Beta 2 GP1 antibodies and low antithrombin III Discussed aspirin or coumadin therapy Patient’s Echo Changes with Therapy Date of Echo Oxygen use via nasal cannula RV-RA gradient (calculated from TR jet) Degree of septal flattening Right heart enlargement Week 0 3 lpm 80 mm Hg Moderate Moderate Week 1 1 lpm 60 mm Hg Moderate Moderate Week 2 ½ lpm / off 50 mm Hg / 70 mm Hg Mild / Moderate Mild Week 6 ½ lpm Unable to estimate; no TR jet Normal geometry None Conclusions Cause of Pulmonary Hypertension Chronic lung disease Primary Aspiration Trisomy 21 Overcirculation prior to PDA closure ?Thromboembolic disease Cause of increased iron index Pulmonary Hypertension Conclusions Cause of her hypoxia Pulmonary hypertension Chronic lung disease Cause of her cough Chronic lung disease Airway protection (aspiration/reflux) Airway Malacia Discussion Open lung biopsy? Repeat bronchoscopy and BAL? Outline Case presentation: Cough, Hypoxia, and Down Syndrome Differential Diagnosis of Cough and Hypoxia Multi-system complications of patients with Trisomy 21 Evaluation for Elevated Iron Index and Pulmonary Hypertension Treatment and Monitoring of PAH Thank you! 4 infants with acute pulmonnary hemorrhage stain first seen 50 hours – 5 days from event Clearance in 1-2 weeks Hemosiderin