Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Intersex medical interventions wikipedia , lookup
Kidney stone disease wikipedia , lookup
Kidney transplantation wikipedia , lookup
Urethroplasty wikipedia , lookup
Chronic kidney disease wikipedia , lookup
Urinary tract infection wikipedia , lookup
Autosomal dominant polycystic kidney disease wikipedia , lookup
CKD & OBSTRUCTIVE UROPATHY Jan23-24 2012@PNAN, Ile-Ife Felicia Eke, MD University of Port Harcourt Nigeria Outline 1. Definition CKD & Obstr Uropathy Mechanism Obstr.UropCKD Case presentations Quiz Management Definition-Obstructive Uropathy Obstruction anywhere down the Urinary tract Stages of CKD—KDOQI, 2002 Stage Description GFR ml/min/1.73m2 1 90 4 Kidney damage* with nl or GFR Kidney damage* with mild GFR Moderate GFR Severe GFR 5 Kidney failure 2 3 ** 60-89 ** 30-59** ** 15-29 <15 or dialysis *Kidney damage for >3 months, as defined by pathologic abnormalities or markers of kidney damage, including abnormalities in blood or urine or diagnostic imaging studies. A: MECHANICAL OBSTRUCTION B: NON-MECHANICAL- FUNCTIONAL Mech:Developmental Anomalies Posterior Urethral Valves Pelvi-ureteric Junction Obstruction Ureteric Stricture Ureterocoele TumoursRhabdomyosarcoma Ureteric & Bladder stones- Cystinuria Meatal stenosis Distal urethral stenosis Cystic malformations, Duplex Dysplastic Kidneys Mech: Acquired Anomalies Tumours Bladder & ureteric stones- Melamine stones from Infant formula Functional: Damaged kidneysCKD Vesico-Ureteric Reflux Spina Bifida Myelomeningocoele Neurogenic bladder eg from Prune belly etc PUV: Case 1-PO One of v. few success stories of PUV Noted at birth to have poor urine stream Big abdomen-bladder Both kidneys enlarged Mechanism by which ObstrCKD Posterior Urethral valves(PUV) Entire urinary tract develops in environment of high intraluminal pressure from Obstruction Cell apoptosis, inflammation Permanent defects - parenchymal dysplasia of kidneys, ureters, bladder despite adequate decompression of the urinary tract pre or post natally PATHOPHYSIOLOGY (cont) In some, renal tubules mainly affected poor urine concentrating ability, diuresis ureteral & bladder dysfunction Affected kidneys may function well initially, but have a reduced renal reserve Renal deterioration also frm chronic pyelonephritis from Vesicoureteric (V-U) reflux, urinary stasis PUV Clinical Presentation Extremely variable Prenatal US :Oligohydramnios hyroureters, hydronephrosis Respiratory distress-pulmonary hypoplasia Potter’s facies, ascites, and abdominal swelling Birth: Poor, dribbling urine stream So check urine stream of all males b/4 discharge from hospital PUV Clinical Presentation Toddlers - voiding dysfunction or UTI School-aged boys usually come to the clinician's attention because of urinary incontinence All enuretics should be screened at least with abdominal palpation &urine MCS Note Hypertrophied bladder neck and dilated posterior urethra proximal to valve narrowing. Anteroposterior view of the abdomen during a void cystourethrographic study. This image demonstrates a dilated bladder with trabeculation, diverticula, and massive reflux. Lateral view of a voiding cystourethrographic study during voiding after catheter removal. The dilated posterior urethra is highly suggestive of a posterior urethral valve, which is seen as the nonopacified line that separates the dilated posterior urethra from the normalcaliber distal urethra. Ectopic Ureter •Continuous urinarydribbling vs intermittent •Dry when supine with vaginal pooling Non-Mechanical Obstruction Non-Obstructed ‘Obstructed’ 22 Prune Belly Syndrome` Triad of 1. Weak abdominal muscles 2 Undescended testes (cryptorchidism) 3. Urinary tract problems- atonic ureters End Stage Renal Failure Formerly didn’t survive; Now do with EARLY SURGERY to correct abd. muscles DysplasticKidneys 3 Bladder Extrophy Bladder is turned "inside out“; Lr portion fails to form correctly Urethra & genitalia not formed completely (epispadias) Anus & vagina appear anteriorly displaced Pelvic bones widely separated –diastasis Rx: Series of Surgical Operations 8 Myelomeningocoele Myelomeningocele - backbone and spinal canal not close before birth. Associated : 1. Hydrocephalus in 90% 2. Big bladder- atonic bladder 3. Anus: Patulous: lack bowel control 4.Legs Partial or complete paralysis of legs & loss of sensation 28 5 Myelomeningocoele Rx Parents fully aware BEFORE BIRTH Surgery to repair defect at an early age 3.V-P shunt for hydrocephalus 4.Antibiotics 4 meningitis or UTI 5.Wheel chair 6. Bladder catheterization, Rx CRF- DT 30 Rhabdomyosarcoma-bladder masses 15 yr Survey of 45 CRF,PH Primary renal dis. No.(%) Glomerulopathies 23 (53.3) 9 14 Obstructive uropathy 13 (28.9) 13 0 Vascular,PN,Malig n,Malaria Nephropathy 9 (17.8) 4 Anochie I, Eke F.Ped Nephrol 2003;18:692-5 Male Female 5 Yearly Incidence ESRF UPTH,Nigeria Red X, Sth Africa Dysplasia 2.1 2.5 13 3 PUV 1985-91 PCKD 1986-91 REFLUX 1991-2000 GN 2007-2010 FSGS SLE Other EkeF.NigMedPract1992;35:37 EkeF,EkeN:PedNephrol1994;8:383-386 AnochieI,EkeF:PedNephrol2003:18:692-695 Approach to preventing Deterioration in CKD A: Surgical In Utero endoscopic valve ablation or vesicoamniotic shunting Endoscopic resection of valves 2-3 days of birth Operation should be done by the most experienced. Valvotomy in UCH-OPD. Catheter few hrs post Op Severe valves may need vesicostomy or ureterostomy; & Bladder augmentation later Mechanical Obstruction only one Aspect B: Medical Immediate catheterizaton-size 3 Foleys fluid & electrolyte Rx Long term monitoring of renal & bladder function Dysplastic kidneys, Ureters & Bladder These not often respond to the mechanical relief of Obstruction Bladder & Renal Care Vital Intermittent catheterization by patient or parents often an integral part of Rx Yearly U/S- continued or recurrent stasis Counselling re avoidance of UTI & stasis Yearly U&E & Cr, if renal function normaltests for Renal Osteodystrophy, bone mineral density, DMSA scan etc Pts have lost their Txpt. Kidney from poor bladder management Avoid constipation Recurrent UTIs VUR Bladder sphincter dysfunction Bowel dysfunction Bowel dysfunction is often part (Dysfunctional elimination synd Aims of CRF Management feel normal be normal - well - like your friends - intellectual development - school / other activities maintain normal growth preserving normal family functioning slow progression to ESRF prepare for ESRF treatment CRF Management Points nutrition fluid and electrolyte balance acid base status renal osteodystrophy infection anaemia growth hypertension preservation of renal function education and preparation social /psychological support Renal osteodystrophy Treatment hydroxylated vitamin D therapy - 1 hydroxycholecalciferol - 1-25dihydroxycholecalciferol by daily oral administration Counselling Surgery NOT A CURE Long term Follow Up Clearly told Paediatrician or Nephrol for Life Urological Follow Up may also be for Life Counsel against ‘It is not my portion’ Multidisciplinary Counselling best Despite all these, MOST PROGRESS to ESRF U/S PaedNephrologist Parents Obstetrician PaedUrologist Neonatologist PaedNurses Paed Nephrologist ±Urologist Vascular/Transplant Surgeon Dieticians Teachers Psychiatrists Social Workers Adult Nephrologist PlayTherapists Churches End Stage Renal Disease in PUV 100% 1919 90% 1990 45% 2011 Survival in PUV 25% 1919 60% 1990 90% 2011 QUIZ On presentation 8days after birth, this patient’s serum creatinine is 500 Umol The management should be 1. Urgent peritoneal dialysis 2.Urgent haemodialysis 3. Something else QUIZ Can a bladder rhbdomyosarcoma cause this anomaly? What is the likely cause? QUIZ True or False All children with Posterior Urethral valves have Chronic Kidney Disease MEMBERSHIP AFPNA Learn more about afpna & its members by visiting the website www.afpna.com PNAN integral part of AFPNA & her Mtgs are advertised there Officers Secretary-General Felicia Eke, Nigeria Secretary-Treasurer Bashir Admani, Kenya Member & IPNA Councillor Udai Kala, South Africa Member & IPNA Councillor Safaa Medani, Sudan (North africa) Member Priya Gajjar, South Africa Member Francis Lalya, Benin Congress President Elect Sampson Antwi, Ghana Ex-Officio Mignon Mc Culloch Dates of Annual Meetings 3yrly Mtg – 5th Congress Ghana Feb 20-23, 2013 4th Nairobi March19-21, 2010 3rd Cape Town, March 2006 2nd Port Harcourt, Nigeria 2002 1st Cairo, Egypt 2000 IPNA Fellows Dr Franca Ikimalo DrAmos Odit Port Harcourt, Nigeria Uganda CT, SA CT, SA 1.9 03 – 29.2.04 1.3.04 –31.8.04 Dr.Godson Achugwo DrFrancis Lalya Owerri,Nigeria Cotonou,Benin CT, SA CT, SA 05-10.05 14.10.05–30.4.06 Again 12.08-12.09 Dr Hemed Twahir Mombasa,Kenya CT, SA 1.4.06 –5.3.07 & again in 2009 Dr.Bashir Admani Nairobi,Kenya CT, SA 8.06-8.08 Dr.Isaac Ocheke Jos, Nigeria CT,SA 7.3.07 – 2.08 Dr Sampson Antwi Kumasi,Ghana CT, SA 4/09-4.10 Dr Seyi Oniyangi Dr Chris Esezobor Dr David Galgallo Abuja,Nigeria Lagos, Nigeria Kenya Joburg,SA 9.08-2.09 Dr Elena Naicker Dr Odetunde Dr NosaIdurionye Durbar, SA Enugu, Nigeria Benin, Nigeria Joburg,SA CT, SA CT, SA 5-12.09 Total 14 3.11 6.11- IPNA Discount Membership Fee for Regional Societies IPNA at its recent Council Meeting in Sao Paulo, Brazil (October 25-26th 2011) offers discounted membership fee to members of its affiliated Regional Societies, namely the ASPN, ESPN, African Pediatric Nephrology Association (AFPNA), AsPNA, JSPN,ANZPNA &ALANEPE IPNA Discount Membership Fee for Regional Societies The current membership fee is US$175/yr The discounted membership fee for Regional Society members is US$50/yr privileges 1. IPNA newsletter 2. On-line access to Ped Nephrol Journal 3. On-line access to Ped Nephrol textbook 4. IPNA Congress member discount 5. Print version of Pediatric Nephrology at an additional rate of US$65 annually (total IPNA Discount Membership Fee for Regional Societies Individual paid-up members of the Regional Soc are eligible 4 discounted membership. Combined fee administered either by IPNA or the Regional Soc accordingly. thus: ASPN: US$300+US$50 (USA) US$150+US$50 (Canada) ESPN: US$70 (Euro$50) + US$50 JSPN: US$135 (Y10,000) + US$50 AsPNA: US$10 + US$50 IPNA Discount Membership Fee for Regional Societies AFPNA: US$25 + US$50 For fees paid through IPNA, the currency will be in USD. IPNA will then transfer the Reg Soc dues accordingly at the end of each financial year. The fee for wire transfer will be charged to each Reg Soc accordingly. The IPNA secretariat will only deal with the payment of full fees for Reg Soc. Discounted fees for Reg Soc should be dealt with by the respective Reg Soc. IPNA Discount Membership Fee for Regional Societies For fees paid through the Reg Soc the Regional Societies can charge in the appropriate currency, but the corresponding total sum should be paid to IPNA in USD. The Regional Society must send the appropriate membership information to IPNA in order to effect the membership privileges. The discounted membership fee will be implemented from January 1st 2012 Management of neuropathic bladder Aims: Preserve renal function Improve continence Methods: Conservative/ medical management Surgical Failure of above methods to prevent upper tract dilation/ongoing renal damage Unacceptable level of incontinence using conservative means Cornerstone of management Clean intermittent catheterisation (CIC) Anticholinergics (counteract detrusor overactivity) 4 X daily Oxybutynin Tolterodine Early intervention. Prevents progressive detrusor damage with loss of compliance and later requirement for cystoplasty. Urodynamics Indications for urodynamics Spinal cord pathology/features of neuropathic bladder Anorectal malformations Infravesical obstruction (PUVs) Unresolving VUR with renal scarring Voiding dysfunction/incontinence failing to respond conventional measures NB** Should only be undertaken with a clinical implication in mind. URODYNAMICS vesical abdominal detrusor-= ves - abd Urodynamic Features associated with renal deterioration Detrusor sphincter dyssynergia High filling/voiding pressures Decreased bladder compliance Leak point pressure>40 cm H2O The Multidisciplinary Team PD Nursing Team Ward Nursing Team Administratio n of fluid deliveries Medical Team PATIENT Dietitian Social Worker Counsellor COMPLICATIONS In the newborn Pulmonary hypoplasia secondary to intrauterine renal dysfunction and Oligohydramnios is the primary cause of patients death. Other complications of PUV are generally secondary to chronic bladder changes, leading to elevated detrusor pressures. This, in turn, leads to progressive renal damage, infection, and incontinence. Renal insufficiency Due to renal dysplasia Elevated detrusor pressure and back flow of urine. COMPLICATIONS (cont) Vesicoureteral reflux Commonly associated with PUV and is present in as many as one third of patients Vesicoureteral reflux in most children is believed to be due to an abnormal insertion of the ureter into the bladder. When associated with PUV, reflux is generally secondary to elevated intravesical pressures. Urinary tract infections Recurrent UTIs are common in patients with PUV. Elevated intravesical pressures predispose patients to infection, possibly by altering urothelial blood flow. Additionally, patients with PUV may have elevated post void residual urine volumes, leading to stasis of urine. Dilated upper urinary tracts, with or without vesicoureteral reflux, further elevate UTI risk. COMPLICATIONS (cont) Bladder dysfunction Thought to be caused by alterations in collagen deposition and the development of detrusor smooth muscle cells. In mild cases, incontinence may be present; In severe cases, ongoing deterioration of renal function occurs. Bladder dysfunction often improves over time after definitive treatment of the obstruction. End Stage Renal Disease( ESRD) Progression of ESRD is accelerated at the time of puberty due to the increased metabolic workload placed on the kidneys. PROGNOSIS . Over the last 30 years, the prognosis of children with PUV has steadily improved. In the past, most children were found to have PUV only after presenting with urosepsis or progressive renal insufficiency. Older series demonstrated mortality rates approaching 50% by late adolescence. Today, most individuals with PUV are discovered when prenatal ultrasonography reveals hydronephrosis. Historically, of patients with adequate pulmonary function, approximately 25% died of renal insufficiency in the first year of life, 25% died later in childhood, and 50% survived to adulthood with varying degrees of renal function. Prompt resolution of bladder obstruction, aggressive treatment of bladder dysfunction, and improved surgical techniques have lowered the neonatal mortality rate to less than 3%. Approximately one third of patients progress to renal insufficiency in their lifetimes. Improved dialysis and transplantation techniques have significantly improved not only the mortality rate for these children but also their quality of life. PROGNOSIS Additionally, medical and surgical management can achieve urinary continence in nearly all patients An interesting group of patients are those with vesicoureteral reflux dysplasia (VURD) syndrome. In these patients, one kidney is hydronephrotic, nonfunctioning, and has high-grade vesicoureteral reflux. The high-grade reflux is thought to act as a pop-off valve, leading to reduced overall bladder pressures and preservation of contralateral renal function. In the past, these patients were thought to have a better outcome due to preserved renal function in one kidney at the sacrifice of the other. More recent data suggests that, although short-term serum creatinine levels may be favorable, these patients may suffer long-term adverse renal function with hypertension, proteinuria, and renal failure In the long. CONCLUSION Posterior urethral valve though not a common congenital anomaly can be fatal Early detection, prompt management and routine follow up of patients is needed for a good life expectancy. INVESTIGATIONS (cont) Nuclear Renography Detects upper-tract consequences of obstruction Absent or dysplastic kidney photopenic area in renal fossa Altered renal function Delayed visualization of renal unit with slow rise to peak activity TREATMENT (cont) Vesicostomy When urethral size precludes safe valve ablation, a communicating channel between the bladder and lower abdominal wall (ie, vesicostomy) can be created to provide bladder drainage Generally, an 18-20F stoma is created approximately midway between the pubis and umbilicus in the midline Take care to bring the dome of the bladder to the skin and to limit the stomal size to prevent prolapse of bladder urothelium through the vesicostomy Its use has decreased because most patients TREATMENT (cont) Cutaneous ureterostomies: Bilateral cutaneous ureterostomies can also be placed to provide for urinary drainage Techniques for cutaneous ureterostomy include: -End stomal ureterostomy, -loop ureterostomy, -Y-ureterostomy (in which the ureter is divided and one end is brought to the skin and the other is reanastomosed in a ureteroureterostomy), and -Ring ureterostomy techniques Potential complications of cutaneous ureterostomies include ureteral TREATMENT (cont) Secondary bladder surgery Augmentation cystoplasty Indications for bladder augmentation include : -Inadequately low bladder storage volumes -High bladder pressures despite anticholinergic medication and clean intermittent catheterization. The ileum is most commonly used; however, large bowel, stomach, and ureter are also used, depending on clinical conditions and surgeon preference. Augmentation should only be offered to patients willing to commit to lifelong intermittent catheterization. TREATMENT (cont) Potential complications include -Bladder rupture (approximately 10% of patients); -Electrolyte disturbances, which may be worsened by the placement of intestinal mucosa in contact with urine. -Several cases of malignant degeneration in augmented bladder have been reported. Despite these risks, augmentation can significantly improve patient lifestyle in those who have intractable incontinence due to poor compliance and bladder over activity. Continent Appendicovesicostomy: Also called the Mitrofanoff technique, Involves placement of a non refluxing tubular conduit for catheterization between the bladder and skin to provide an alternative channel for catheterization. TREATMENT (cont) Potential complications include -Bladder rupture (approximately 10% of patients); -Electrolyte disturbances, which may be worsened by the placement of intestinal mucosa in contact with urine. -Several cases of malignant degeneration in augmented bladder have been reported. Despite these risks, augmentation can significantly improve patient lifestyle in those who have intractable incontinence due to poor compliance and bladder over activity. Continent Appendicovesicostomy: Also called the Mitrofanoff technique, Involves placement of a non refluxing tubular conduit for catheterization between the bladder and skin to provide an alternative channel for catheterization.