Etiopathogenesis and management of bladder dysfunction in patients with posterior urethral valves

Thomas, Joseph

doi:10.4103/0970-1591.74434

Cited by 31 publications

(30 citation statements)

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“…34 Filling and emptying of the bladder up until the third trimester lead to remodeling of the bladder wall. 35 Obstruction by PUVs impairs this process, contributing to long-term bladder dysfunction. 35…”

Section: Phases Of Kidney and Urinary Tract Developmentmentioning

confidence: 99%

Developmental Genetics and Congenital Anomalies of the Kidney and Urinary Tract

Reidy

2015

J Pediatr Genet

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Congenital anomalies of the kidney and urinary tract (CAKUT) are common birth defects and the leading cause of end-stage renal disease in children. There is a wide spectrum of renal abnormalities, from mild hydronephrosis to more severe cases, such as bilateral renal dysplasia. The etiology of the majority of cases of CAKUT remains unknown, but there is increasing evidence that genomic imbalance contributes to the pathogenesis of CAKUT. Advances in human and mouse genetics have contributed to increased understanding of the pathophysiology of CAKUT. Mutations in genes involved in both transcription factors and signal transduction pathways involved in renal development are associated with CAKUT. Large cohort studies suggest that copy number variants, genomic, or de novo mutations may explain up to one-third of all cases of CAKUT. One of the major challenges to the use of genetic information in the clinical setting remains the lack of strict genotype-phenotype correlation. However, identifying genetic causes of CAKUT may lead to improved diagnosis of extrarenal complications. With the advent of decreasing costs for whole genome and exome sequencing, future studies focused on genotype-phenotype correlations, gene modifiers, and animal models of gene mutations will be needed to translate genetic advances into improved clinical care.

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Section: Phases Of Kidney and Urinary Tract Developmentmentioning

confidence: 99%

Developmental Genetics and Congenital Anomalies of the Kidney and Urinary Tract

Reidy

2015

J Pediatr Genet

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“…Posterior urethral valves (PUV) are the most common cause of congenital lower urinary tract outflow obstruction in male infants with an incidence of 1:5,000 male infants and 1:25,000 live births. [ 1 2 3 ] The incidence is unknown in our sub-region. Uba et al [ 1 ] in Jos, Nigeria reported 3–8 cases per annum.…”

Section: Introductionmentioning

confidence: 99%

“…In fact, PUV are now commonly diagnosed by the postnatal evaluation of infants who had prenatal hydronephrosis. [ 3 ]…”

Section: Introductionmentioning

confidence: 99%

Posterior urethral valves in children: Pattern of presentation and outcome of initial treatment in Ile-Ife, Nigeria

et al. 2015

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Background:The management of posterior urethral valves (PUV) and its sequelae is still a challenge to most pediatric surgeons in our environment due to late presentation and inadequate facilities for long-term evaluation and treatment. Despite initial successful treatment about 40% would develop chronic renal failure. The aim is to describe the presentation, management and outcome of the initial treatment in boys with PUV.Materials and Methods:It is a retrospective analysis of PUV in boys 8 years and below over a 17 years period. Demographic characteristics, clinical features, investigations, and treatment outcome were reviewed.Results:Thirty-seven cases were analyzed. The median age was 5 months (range from birth to 8 years). Three (8.1%) patients had prenatal ultrasound diagnosis. The most common presentation was voiding dysfunction 37 (100%). Part of the preoperative investigation included micturating cystourethrogram (n = 31: 83.8%) and abdomino-pelvic ultrasonography (n = 37:(100%). The mean serum creatinine value of those who presented within the first 30 days of life and those who presented afterwards were 325 (±251) µmol/L and 141 (±100) µmol/L respectively, P = 0.003. Surgical interventions included trans-vesical excision of valves (n = 9: 28.1%), valvotomy (n = 10: 31.3%), balloon avulsion (n = 8: 25.0%), vesicostomy (n = 4: 12.5%) and endoscopic valve avulsion (n = 1: 3.1%). Seventeen (56.7%) patients had serum creatinine >70.4 µmol/L after 1-month of valve excision. Five (13.5%) patients had postrelief complications and 5 (13.5%) died on admission. Ninety percentage (27/30) of patients had poor prognostic indices.Conclusions:The initial treatment outcome was good but most had poor prognostic factors.

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“…3 Due to the obstructive nature of PUV, development of detrusor hypertrophy, low bladder compliance, and high pressure are some of the subsequent changes that take place in the urinary bladder. 1,4 Such changes in the detrusor muscle must be considered in the context of any future surgical intervention, as they can greatly impact placement and drainage of urinary catheters. Casale forewarns that the use of a Foley catheter may cause irritation and subsequent bladder spasms in children with PUV.…”

Section: Discussionmentioning

confidence: 99%