Raras
Buscar doenças, sintomas, genes...
Válvula uretral posterior
ORPHA:93110CID-10 · Q64.2CID-11 · LB31.2DOENÇA RARA

Válvula Uretral Posterior (VUP) é a causa mais comum de um bloqueio nas vias urinárias inferiores do feto (o bebê ainda na barriga da mãe). Ela ocorre devido a uma membrana anormal, presente desde o nascimento, que obstrui a uretra na sua parte posterior. Isso impede de forma significativa que a bexiga do bebê menino se esvazie normalmente.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Válvula Uretral Posterior (VUP) é a causa mais comum de um bloqueio nas vias urinárias inferiores do feto (o bebê ainda na barriga da mãe). Ela ocorre devido a uma membrana anormal, presente desde o nascimento, que obstrui a uretra na sua parte posterior. Isso impede de forma significativa que a bexiga do bebê menino se esvazie normalmente.

Pesquisas ativas
5 ensaios
13 total registrados no ClinicalTrials.gov
Publicações científicas
541 artigos
Último publicado: 2026 Apr 17

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
2.0
Europe
Início
All ages
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q64.2
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫘
Rins
12 sintomas
🦴
Ossos e articulações
1 sintomas
🫃
Digestivo
1 sintomas
❤️
Coração
1 sintomas
🫁
Pulmão
1 sintomas

+ 11 sintomas em outras categorias

Características mais comuns

90%prev.
Válvula uretral posterior congênita
Muito frequente (99-80%)
90%prev.
Infecções do trato urinário recorrentes
Muito frequente (99-80%)
90%prev.
Doença renal crônica
Muito frequente (99-80%)
55%prev.
Hidronefrose
Frequente (79-30%)
55%prev.
Refluxo vesicoureteral
Frequente (79-30%)
55%prev.
Obstrução da saída da bexiga
Frequente (79-30%)
27sintomas
Muito frequente (3)
Frequente (3)
Ocasional (16)
Muito raro (5)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 27 características clínicas mais associadas, ordenadas por frequência.

Válvula uretral posterior congênitaCongenital posterior urethral valve
Muito frequente (99-80%)90%
Infecções do trato urinário recorrentesRecurrent urinary tract infections
Muito frequente (99-80%)90%
Doença renal crônicaChronic kidney disease
Muito frequente (99-80%)90%
HidronefroseHydronephrosis
Frequente (79-30%)55%
Refluxo vesicoureteralVesicoureteral reflux
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico541PubMed
Últimos 10 anos200publicações
Pico202535 papers
Linha do tempo
2026Hoje · 2026🧪 2012Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

1 gene identificado com associação a esta condição. Padrão de herança: Autosomal recessive, Not applicable, X-linked recessive.

BNC2Zinc finger protein basonuclin-2Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Probable transcription factor specific for skin keratinocytes. May play a role in the differentiation of spermatozoa and oocytes (PubMed:14988505). May also play an important role in early urinary-tract development (PubMed:31051115)

LOCALIZAÇÃO

Nucleus

MECANISMO DE DOENÇA

Lower urinary tract obstruction, congenital

A disorder characterized by urinary bladder outflow obstruction, which can represent an anatomical blockage or a functional obstruction. The most common anatomical causes are posterior urethral valves at the level of the prostatic urethra, a lesion unique to males. Less common are anterior urethral valves, also called urethral atresia, that can occur in either sex. LUTO is an autosomal dominant disease with variable expression.

INTERAÇÕES PROTEICAS (1)
OUTRAS DOENÇAS (2)
lower urinary tract obstruction, congenitalposterior urethral valve
HGNC:30988UniProt:Q6ZN30

Variantes genéticas (ClinVar)

105 variantes patogênicas registradas no ClinVar.

🧬 BNC2: GRCh38/hg38 9p24.3-q21.13(chr9:208455-72054336)x3 ()
🧬 BNC2: GRCh38/hg38 9p24.3-22.2(chr9:449551-16713261)x3 ()
🧬 BNC2: GRCh38/hg38 9p24.3-13.1(chr9:208455-38787483)x3 ()
🧬 BNC2: GRCh37/hg19 9p24.3-22.2(chr9:2079051-17236505)x1 ()
🧬 BNC2: GRCh37/hg19 9p24.3-q21.11(chr9:203862-69977404)x3 ()
Ver todas no ClinVar

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 31
2Fase 21
1Fase 11
·Pré-clínico6
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 9 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Válvula uretral posterior

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

3 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

13 ensaios clínicos encontrados, 5 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
🧪 Está conduzindo uma pesquisa?
Divulgue para pacientes e familiares que acompanham esta doença.
Divulgar pesquisa →

Publicações mais relevantes

Timeline de publicações
276 papers (10 anos)
#1

Congenital Anomalies of Kidney and Urinary Tract in Children - Clinical Spectrum and Complications.

Indian journal of pediatrics2026 Jan 03

To study the spectrum of congenital anomalies of the kidney and urinary tract (CAKUT), and to examine the clinical presentation and potential complications. A total of 304 children were recruited (216 retrospectively and 88 prospectively). Clinical data were obtained from records in the retrospective cases, while clinical assessments, laboratory tests, and imaging were conducted as needed in the prospective cases. Primary vesicoureteric reflux (VUR) was the most common cause of CAKUT (34.2%), followed by posterior urethral valve (PUV) at 24% and pelvi-ureteric junction obstruction (PUJO) at 13.1%. Antenatal detection was the most common mode of identification (47.6%), followed by detection after urinary tract infection (UTI) in 29.5% of cases. Extrarenal manifestations occurred in 6.5% of cases. Recurrent UTI was the most common complication, seen in 42.3%. Additionally, 11.5% had hypertension, and 10% had a glomerular filtration rate (GFR) of less than 60 ml/min/1.73 m2. Primary VUR is the most common CAKUT, and the antenatal detection of anomalies is crucial. The prevalence of chronic kidney disease (CKD) stage 2 and above is quite high among CAKUT, particularly PUV, renal hypodysplasia, and VUR, warranting close nephrology follow-up.

#2

Concomitant Bladder Neck Incision in Patients with Posterior Urethral Valve and Blad-der Neck Hypertrophy: Short-term Outcomes of a Randomized Controlled Trial.

International braz j urol : official journal of the Brazilian Society of Urology2026

Concomitant bladder neck incision (BNI) with posterior urethral valve ablation (VA) was proposed to mitigate the long-term sequela of posterior urethral valve (PUV) and reduce the reoperation rates. This study aimed to investigate the short-term outcomes of concomitant BNI and VA, particularly short-term reoperation rates. Patients with PUV and bladder neck hypertrophy on preoperative imaging were randomized to undergo VA only or VA with concomitant BNI. Surgical reoperation within one year was the primary endpoint. Renal function, UTI, hydronephrosis and VUR improvement at one year were secondary endpoints. Sixty-three patients were included in the final analysis, 33 in VA group (group A) and 30 in concomitant BNI and VA group (group B). After one year of follow-up, the reoperation rate was similar [5(15.2%) in group A and 3(10%) in group B, p=0.18]. The median (IQR) nadir serum creatinine was lower in group B [0.2 (0.1-0.3) vs. 0.2 (0.2-0.4) mg/dL in group A, p=0.049]. The last follow-up serum creatinine median (IQR) eGFR [107 (89.5-163) in group A vs. 139(102-165) mL/min/1.73 m2 in groups B, p=0.37], and febrile UTI rates were not different between the two groups. Hydronephrosis improved/ resolved in 27 (40.9%) renal units in group A vs. 33 (55%) renal units in group B (p=0.286). Vesicoureteral reflux improved/ resolved in 23(34.8%) and 12 (20%) renal units in group A and B, respectively (p=0.074). Concomitant BNI with VA does not confer a lower short-term reoperation rate or better upper urinary tract outcomes compared to VA only.

#3

Posterior urethral valve presenting as posterior urethral calculus in adult: A rare case scenario.

Urology case reports2026 May

Posterior urethral valve (PUV) rarely causes adult lower urinary tract obstruction and is rarely linked to posterior urethral calculi. A 21-year-old male with 5-year history of (LUTS) had a 19 × 22 × 39 mm posterior urethral stone removed via open cystolithotomy (35 mm stone). Persistent poor flow led to RUG/MCUG showing urethral dilatation and cystoscopy confirming PUV, ablated with holmium laser. Post-ablation uroflowmetry improved markedly. Adult PUV with impacted calculus is extremely rare, mimicking isolated stone disease. High suspicion for PUV in young males with posterior calculi and post-removal LUTS is vital; cystoscopy and voiding studies ensure accurate diagnosis and management.

#4

Long-term sexual function, fertility, and quality of life after childhood treatment for posterior urethral valves: a systematic review of clinical outcomes.

The journal of sexual medicine2026 Feb 06

Posterior urethral valves are the leading cause of congenital bladder outlet obstruction in boys and can result in long-term lower urinary tract dysfunction and reduced quality of life. Sexual and reproductive consequences in this population are poorly described and understood. The purpose of this systematic review (SR) was to assess the long-term sexual functions and fertility outcomes in patients treated in the pediatric age for posterior urethral valve (PUV). A SR was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. MEDLINE, Embase, and Scopus were searched for studies reporting sexual or reproductive outcomes in individuals with a history of PUV. The primary outcomes were prevalence and nature of sexual dysfunctions and fertility issues. Risk of bias was assessed using ROBINS-I or the Newcastle-Ottawa Scale, as appropriate. Eleven studies (2 non-randomized comparative studies and 9 case series) including 2723 individuals met the inclusion criteria. Most patients exhibited preserved erectile function, ejaculatory function was variably reported with most individuals described normal ejaculation. Semen analysis, reported in 7 studies, revealed normal parameters in approximately half of the cohorts, though abnormalities were noted in patients with renal impairment or complex urological histories. Paternity rates varied widely (6%-58%) and were lower in individuals with renal dysfunction or urinary incontinence. Only one study used validated tools to assess quality of life, showing negative impact in domains related to continence and renal status. Most males treated for PUV during childhood reach adulthood with preserved sexual and reproductive potential. Given the heterogeneity and methodological limitations of the current literature, prospective studies using standardized outcome measures are needed to better define long-term risks and inform clinical follow-up.

#5

Fulguration of Anterior Membrane by Endoscopy (FAME): A modified technique of posterior urethral valve fulguration reduces the incidence of urethral strictures.

Journal of pediatric urology2026 Feb

The standard technique of endoscopic fulguration of posterior urethral valves (PUV) involves fulguration at 5,7 and 12o'clock, with urethral stricture being a major complication. Since the anterior portion of the valve is the point of maximal obstruction, hence we modified the technique by fulgurating only the anterior part of the valve between 10 and 2 o'clock positions. We present the results of the standard and modified techniques of PUV fulguration. We hypothesised that the modified technique might reduce the risk of urethral strictures, while achieving adequate valve ablation. All PUV undergoing cystoscopic fulguration in our unit over a 6-year period were divided into 2 groups (group 1: standard 3-point fulguration; group 2: modified anterior fulguration). For valve fulguration, a 3F bugbee or hook electrode was used with appropriate sized compact cystoscope in all cases (6F in infants and 8/9.8F in older children). The two groups were compared for the adequacy of fulguration and urethral stricture formation during follow-up. Fifty-nine cases were included (22 group 1, 37 group 2). The mean age at fulguration was comparable between both groups. Follow-up voiding cystourethrogram and check cystoscopy revealed urethral stricture in 3 and residual valves in 4 children. The incidence of urethral stricture was significantly higher (p=0.047) in group 1 (3/22, 13.6 %) compared to group 2 (0/37, 0 %); there was no difference in the incidence of residual valves between both the groups (0/19, 0 % group 1 vs 4/37, 10.8 % group 2, p=0.288). We adopted a modified technique of PUV fulguration limiting diathermy application to the anterior part of PUV. There is no consensus on the best method of PUV ablation; commonly used techniques are cold knife and diathermy ablation. Incision with cold knife may cause bleeding, and may have a higher incidence of residual valves. Diathermy may predispose to more urethral strictures, especially when applied at 3 points circumferentially inside the small urethra, as in the standard technique of fulguration. Several autopsy and videocystoscopic studies, including our own observations, have documented that the valve is, in reality, a membrane with a posterior opening, the anterior part of the membrane being the major obstructing component. The results of the present study seem to be in agreement with this morphology of PUV. The modified technique of only anterior PUV fulguration resulted in significantly reduced incidence of urethral strictures with no increase in residual valves compared to the standard technique.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC207 artigos no totalmostrando 196

2026

Concomitant Bladder Neck Incision in Patients with Posterior Urethral Valve and Blad-der Neck Hypertrophy: Short-term Outcomes of a Randomized Controlled Trial.

International braz j urol : official journal of the Brazilian Society of Urology
2026

Posterior urethral valve presenting as posterior urethral calculus in adult: A rare case scenario.

Urology case reports
2026

Long-term sexual function, fertility, and quality of life after childhood treatment for posterior urethral valves: a systematic review of clinical outcomes.

The journal of sexual medicine
2026

Congenital Anomalies of Kidney and Urinary Tract in Children - Clinical Spectrum and Complications.

Indian journal of pediatrics
2026

Commentary to "Fulguration of Anterior Membrane by Endoscopy (FAME): A modified technique of posterior urethral valve fulguration reduces the incidence of urethral strictures".

Journal of pediatric urology
2025

Bladder Neck Incision in Posterior Urethral Valve Management: a Meta-Analysis with Insights into Adjunctive Bladder Interventions.

Current urology reports
2025

Impact of Preoperative Filling-Phase Vesicoureteral Reflux on Febrile Urinary Tract Infections After Vesicostomy in Children.

Cureus
2025

Coexisting congenital ano-urethral fistula with posterior urethral valve: a case report and literature review.

BMC surgery
2026

Fulguration of Anterior Membrane by Endoscopy (FAME): A modified technique of posterior urethral valve fulguration reduces the incidence of urethral strictures.

Journal of pediatric urology
2025

Atlas of predictive tools and scoring systems for outcomes in patients with posterior urethral valves: A comprehensive review of the literature.

Urology annals
2026

Comparative performance of cystatin C and creatinine eGFR equations in boys with posterior urethral valves: An exploratory study.

Journal of pediatric urology
2025

Segmental spinal dysgenesis - a rare type of spinal dysraphism.

Pediatric radiology
2025

Analysing Regional Variation in Referrals to the Children's Epilepsy Surgery Service: A Novel Methodology.

Child: care, health and development
2026

Impact of urinary extravasation in posterior urethral valves: clinical implications of urinomas and ascites on kidney function.

Pediatric nephrology (Berlin, Germany)
2025

[2024 EAU/ESPU paediatric urology guidelines: key updates on congenital lower urinary tract obstruction and clinical inter-pretation].

Zhejiang da xue xue bao. Yi xue ban = Journal of Zhejiang University. Medical sciences
2025

Impact of posterior urethral valve on patient quality of life and caregiver burden.

World journal of urology
2025

[Preliminary application of domestic single-port serpentine arm robotic surgical system in children's pyeloplasty].

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
2025

Short- and Long-Term Renal Outcomes in Clinically Matched Cohorts of Newborns With Posterior Urethral Valve (PUV) Managed by Primary Vesicostomy Versus Primary Valve Ablation.

Urology
2025

Predicting progression to renal replacement therapy in children with posterior urethral valves: international validation of the secondary use of the posterior urethral valve risk of chronic kidney disease (PURK) score.

Journal of pediatric urology
2025

Defining post-obstructive diuresis following posterior urethral valve ablation.

Frontiers in pediatrics
2025

A Comparative Study of Role of Holmium:YAG Laser Versus Electrocautery for Posterior Urethral Valve Fulguration in Paediatric Patients.

Journal of pediatric surgery
2025

Maternal Risk Factors for Infants Born with Congenital Renal and Urinary Tract (CAKUT) Anomalies: A Cross-Sectional Assessment.

Mymensingh medical journal : MMJ
2025

Voiding cystourethrography practices: experiences in a tertiary pediatric referral hospital.

Acta radiologica (Stockholm, Sweden : 1987)
2025

Teapot ureterocystoplasty in posterior urethral valve and chronic kidney disease: a case report.

The Canadian journal of urology
2025

Renal perfusion in dynamic scans: a key predictor of long-term outcomes in posterior urethral valves.

Pediatric nephrology (Berlin, Germany)
2025

Impact of a standardized care pathway and a dedicated multidisciplinary posterior urethral valve clinic on 5-year kidney and bladder outcomes.

Pediatric nephrology (Berlin, Germany)
2025

Urethral ratio in predicting residual valve following ablation of posterior urethral valve.

Journal of pediatric urology
2025

The effect of fecal microbial transplantation in a pediatric patient after 28 episodes of febrile urinary tract infection.

Pediatric nephrology (Berlin, Germany)
2025

Bladder Stone Incarceration in Posterior Urethral Valve Leading to Urinary Retention: A Case Report.

Case reports in nephrology and dialysis
2025

CONGENITAL MEGALOURETHRA: Lower Urinary Tract Obstruction With "The Sandwich Sign".

Journal of clinical ultrasound : JCU
2025

Does unilateral reflux have a protective effect in posterior urethral valve patients?

Pediatric surgery international
2025

Urinary Bladder and Posterior Urethral Morphology in Voiding Cystourethrogram and Its Implications in Management and Prognosis of Posterior Urethral Valves.

Journal of Indian Association of Pediatric Surgeons
2025

Fetal Urinoma Secondary to Posterior Urethral Valve and Its Association to Postnatal Renal Function: A Multicenter Retrospective Study.

Prenatal diagnosis
2025

Late Presentation of Posterior Urethral Valve in a Four-Year-Old Child With Subtle Voiding Symptoms: A Case Report.

Cureus
2025

Development and international validation of a novel multivariate prognostic tool: posterior urethral valve risk of chronic kidney disease (PURK) score.

Pediatric nephrology (Berlin, Germany)
2025

Posterior urethral valve complicated by bilateral urinoma and vesicoureteral reflux.

BMJ case reports
2025

Posterior urethral valves: Examining the relationship of socioeconomic factors in disease presentation and progression.

Journal of pediatric urology
2025

A quality assessment of YouTube content on posterior urethral valve.

Journal of pediatric urology
2024

A case of posterior urethral valve diagnosed by ultrasound in antenatal and pathology: A case report.

Medicine
2024

Outcomes of children with isolated antenatal hydronephrosis.

Pediatrics international : official journal of the Japan Pediatric Society
2025

Effect of overnight bladder drainage on posterior urethral valve sequelae: A randomized controlled trial.

Journal of pediatric urology
2024

[Cobb's collar … seek and you shall find!].

Aktuelle Urologie
2025

The effect of urinary diversion on kidney function in posterior urethral valves and ureterovesical obstruction.

International urology and nephrology
2024

Posterior urethral valves: risk factors of progression to end-stage chronic renal disease after 10 years of follow-up.

Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia Pediatrica
2025

Renal Transplantation with Triple Ureter Implantation into the Urinary Bladder after Dissolving a Bilateral Ureterocutaneostomy for Posterior Urethral Valves.

Urologia internationalis
2024

A Precious Puzzle: Unveiling a Suspected VACTERL (Vertebral, Anal, Cardiovascular Malformations, Tracheo-esophageal Fistula, Renal Anomalies, and Limb Defects) Association in a 28-Day-Old Neonate.

Cureus
2024

Hidden in Plain Sight: Ralstonia mannitolilytica as a Rare Cause of Urinary Tract Infections.

Cureus
2025

Posterior urethral valves: Clinical audits of presentation, diagnostic and therapeutic intervention in a nigerian teaching hospital.

International urology and nephrology
2025

Comparison of the Role of Anticholinergics and α-1 Adrenergic Blockers in Bladder Management in Posterior Urethral Valves: A Pilot Randomized Control Trial.

Urologia internationalis
2024

Utilizing artificial neural network system to predict the residual valve after endoscopic posterior urethral valve ablation.

Archivio italiano di urologia, andrologia : organo ufficiale [di] Societa italiana di ecografia urologica e nefrologica
2024

Placement of transurethral urinary drainage catheter using microwire in a newborn with posterior urethral valve: A better alternative to suprapubic catheterization.

Radiology case reports
2024

The effect of use of alpha-blockers in posterior urethral valve pediatric patients postvalve ablation in the absence of further outlet obstruction.

Urology annals
2024

Posterior Urethral Valves in a Healthy-Appearing Athletic Adult: A Case Report.

Cureus
2024

Fetal Urinary Ascites From Bladder Rupture: A Rare Complication of Posterior Urethral Valve.

Cureus
2024

Long-Term Kidney Outcomes in Children with Posterior Urethral Valves: A Population-Based Cohort Study.

Journal of the American Society of Nephrology : JASN
2024

Noonan syndrome and Noonan-like syndrome with loose anagen hair: rare phenotypes may emerge during follow-up.

Translational pediatrics
2024

Fetal Lower Urinary Tract Obstruction: Current Diagnostic and Therapeutic Strategies and Future Directions.

Fetal diagnosis and therapy
2024

Right perirenal urinoma and urinothorax in an infant after neonatal ablation of posterior urethral valve: A rare complication diagnosed by pleural aspiration and treated with perinephric drainage: a case report.

Journal of medical case reports
2024

Role of Urinary Biomarkers (Transforming Growth Factor β1, Neutrophil Gelatinase-Associated Lipocalin, and Cystatin C) as a Prognostic Factor of Renal Outcome in the Posterior Urethral Valve.

Journal of Indian Association of Pediatric Surgeons
2024

Rectourethral Fistula after Posterior Urethral Valves Ablation in an Infant: A Rare Complication.

Journal of Indian Association of Pediatric Surgeons
2024

Lack of consensus in atypical congenital obstructive urethral lesions in children: Snapshot of the web-based ObsCUre (obstruction to the child urethra) study.

Current urology
2024

Obstructing urethral membrane in a girl: a rare cause of female bladder outlet obstruction.

BMJ case reports
2024

Medullary pyramids opacification in high-grade vesicoureteral reflux associated with posterior urethral valve.

Indian journal of urology : IJU : journal of the Urological Society of India
2024

Pathways to paediatric urology subspecialisation: a study of casemix, incumbent attitudes and opinions.

World journal of urology
2024

External validation and reliability assessment of posterior urethral morphology on initial voiding cystourethrogram as a predictor for infants with posterior urethral valves.

Journal of pediatric urology
2024

Clinical considerations in adults with history of posterior urethral valves.

Journal of pediatric urology
2023

Mechanical Ablation of the Posterior Urethral Valve Omitting Thermal Energy: A Brief Report.

Iranian journal of medical sciences
2023

Thulium Laser for the Treatment of Posterior Urethral Valves in Infants.

Journal of endourology
2023

Parental perception of contrast enhanced voiding ultrasonography urodynamics vs fluoroscopic urodynamics.

Journal of pediatric urology
2023

Posterior urethral valve in thai boys.

BMC pediatrics
2024

Post-obstructive diuresis after posterior urethral valve treatment in neonates: a retrospective cohort study.

Pediatric nephrology (Berlin, Germany)
2023

Comparing Estimated and Measured Glomerular Filtration Rate in Children with Posterior Urethral Valve.

Journal of Indian Association of Pediatric Surgeons
2023

A grading system for evaluation of bladder trabeculation.

World journal of urology
2023

Is posterior urethral valve a chronic disease, not merely a chronic kidney disease? Reflections on models of (multidisciplinary) care.

Pediatric nephrology (Berlin, Germany)
2023

Infantile versus childhood posterior urethral valve diagnosis: management patterns and clinical outcomes at opposite ends of the spectrum.

Journal of pediatric urology
2023

Posterior urethral valves, pressure pop-offs, and kidney function: systematic review and meta-analysis.

World journal of urology
2023

Does VURD syndrome impact voiding efficiency in posterior urethral valves?

Journal of pediatric urology
2022

Clinico-epidemiological Profile of Children with Chronic Kidney Disease in Tertiary Care Referral Center in Nepal: Prospective observational study.

Kathmandu University medical journal (KUMJ)
2023

Re: Primary ablation versus urinary diversion in posterior urethral valve: Systematic review and meta-analysis.

Journal of pediatric urology
2023

Nadir creatinine as a predictor of renal outcomes in PUVs: A systematic review and meta-analysis.

Frontiers in pediatrics
2023

A novel endoscopic approach for management of hutch diverticulum concomitant vesicoureteral reflux with dextranomer/hyaluronic acid copolymer injection.

Journal of pediatric urology
2023

Overlooked Factors Causing Renal Damage in Vesicoureteric Reflux!

Journal of Indian Association of Pediatric Surgeons
2023

Primary ablation versus urinary diversion in posterior urethral valve: Systematic review and meta-analysis.

Journal of pediatric urology
2023

Posterior urethral valve: A case report in an older Nigerian child.

Radiology case reports
2023

Urodynamic changes before and after endoscopic valve ablation in boys diagnosed with the posterior urethral valve without chronic renal failure.

BMC urology
2024

Single-center outcome analysis of 46 fetuses with megacystis after intrauterine vesico-amniotic shunting with the Somatex®intrauterine shunt.

Archives of gynecology and obstetrics
2023

Relation between blood levels of heavy metals and some markers of oxidative stress among boys with neuropathic bladder and posterior urethral valve.

Journal of trace elements in medicine and biology : organ of the Society for Minerals and Trace Elements (GMS)
2023

Outcomes of intermediate-risk hydronephrosis in pediatric patients.

Journal of pediatric urology
2023

Simultaneous Renal Transplantation With Bilateral Nephrectomy and Ureterocystoplasty.

Urology
2023

The effect of primary urinary diversion on kidney function in posterior urethral valve: A matched comparison.

Urology
2023

Circumcision reduces urinary tract infection in children with antenatal hydronephrosis: Systematic review and meta-analysis.

Journal of pediatric urology
2022

Epidemiological Patterns of Cannabis- and Substance- Related Congenital Uronephrological Anomalies in Europe: Geospatiotemporal and Causal Inferential Study.

International journal of environmental research and public health
2022

Clinical profile of children with posterior urethral valve at Tertiary Care Center.

Pakistan journal of medical sciences
2022

Pop-off mechanisms as protective factors against chronic renal disease in children with posterior urethral valves.

Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia Pediatrica
2022

Publication landscape of Posterior urethral valve- A scientometric study.

Journal of pediatric urology
2022

Does Early Upper Tract Diversion and Delayed Undiversion in Megaureters Secondary to Severe Posterior Urethral Valves Lead to Better Renal Outcomes?

Journal of Indian Association of Pediatric Surgeons
2023

Novel Biomarkers for Posterior Urethral Valve.

Current medicinal chemistry
2022

Health Worker (HW) Factors in Delayed Access to Pediatric Surgery in Low- and Middle-Income Countries (LMICs).

Nigerian journal of clinical practice
2022

Risk factors for end stage renal disease in children with anorectal malformation and outcome comparison to children with isolated urological anomalies.

Journal of pediatric urology
2022

Long-term outcomes of augmentation cystoplasty: A retrospective view of 54 children.

Actas urologicas espanolas
2022

Role of progressive urethral dilation and primary valve ablation in the long-term renal outcomes of small, preterm infants with posterior urethral valve.

Journal of pediatric urology
2022

Mid-short-term risk factors for chronic renal failure in children with posterior urethral valve.

Pediatric surgery international
2022

Experience with the use of mohan's valvotome for posterior urethral valve ablation at a centre in North-Eastern Nigeria.

African journal of paediatric surgery : AJPS
2022

New challenges of fetal therapy in Japan.

The journal of obstetrics and gynaecology research
2022

Surgical management of the posterior urethral valve in children.

Asian journal of surgery
2022

Use of Eculizumab in Pediatric Patients with Late Antibody-Mediated Rejection After Kidney Transplantation.

Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation
2022

The risk factors of influencing postoperative bladder function in children with posterior urethral valve: A retrospective cohort study of 23 children.

Asian journal of surgery
2022

Catheter test: A reliable alternative to assess adequacy of bladder management in patients of PUV in developing countries.

Journal of pediatric urology
2022

Bladder height width ratio on voiding cystourethrogram as a predictor of future valve bladder in children with posterior urethral valve.

Pediatric surgery international
2022

Rare case of inguinal ureteral hernia in a child diagnosed by drip infusion pyelography-computed tomography.

International journal of surgery case reports
2022

Tubular and glomerular biomarkers of renal tissue function in the urine of fetuses with posterior urethral valves.

Journal of pediatric urology
2022

Concomitant anterior urethral valve, distal urethral diverticulum and posterior urethral valve with five-year follow up; A case report and literature review.

Urology case reports
2022

Correlation of Urethral Ratio and Bladder Wall Thickness with Cystoscopic Findings in Posterior Urethral Valve Patients to Assess Residual Valves.

Journal of Indian Association of Pediatric Surgeons
2022

Biallelic pathogenic variants in roundabout guidance receptor 1 associate with syndromic congenital anomalies of the kidney and urinary tract.

Kidney international
2022

Can Concomitant Bladder Neck Incision and Primary Valve Ablation Reduce Early Re-admission Rate and Secondary Intervention?

International braz j urol : official journal of the Brazilian Society of Urology
2022

Outcome of boys with posterior urethral valves from a single tertiary hospital in Singapore.

ANZ journal of surgery
2022

Unusual case of ascites secondary to unilateral distal ureteric obstruction in an infant.

BMJ case reports
2022

Deciphering the mutation spectrum in south Indian children with congenital anomalies of the kidney and urinary tract.

BMC nephrology
2021

Effects of oxybutynin in children with posterior urethral valves post fulguration: Report in a select cohort of children.

Journal of family medicine and primary care
2021

Impact of surgical intervention on progression to end-stage renal disease in patients with posterior urethral valve.

African journal of urology : the official journal of the Pan African Urological Surgeons' Association (PAUSA)
2021

Evolution of Management of Posterior Urethral Valve.

Journal of Indian Association of Pediatric Surgeons
2022

On-table urodynamic with ureteric occlusion: A resource for measuring bladder capacity and compliance in the management of patients with gross vesico-ureteric reflux.

Neurourology and urodynamics
2022

Computational fluid dynamics modeling approaches to assess lower urinary tract hydraulic dynamics in posterior urethral valve before and after endoscopic valve ablation: a pilot study.

World journal of urology
2021

Pediatric urology surgical practice in the time of COVID-19: Results from tertiary Saudi Arabia hospitals.

Urology annals
2022

Predicting childhood chronic kidney disease severity in infants with posterior urethral valve: a critical analysis of creatinine values in the first year of life.

Pediatric nephrology (Berlin, Germany)
2022

A case of posterior urethral valve identified in an older child by straining to void.

Urology case reports
2022

Posterior Urethral Valves Outcomes Prediction (PUVOP): a machine learning tool to predict clinically relevant outcomes in boys with posterior urethral valves.

Pediatric nephrology (Berlin, Germany)
2021

Temporary vesicostomy in pediatrics: What are the potential predictors of functional and morphological improvement of the upper urinary tract?

Journal of pediatric urology
2021

Spontaneous bladder rupture secondary to posterior urethral valves in a boy with Down syndrome.

BMJ case reports
2021

Outcome of PUV Patients Following Ablation in a Tertiary Teaching Hospital in Addis Ababa, Ethiopia.

Research and reports in urology
2020

INITIAL EXPERIENCE WITH 3D-ULTRASOUND AS AN ADJUNCT TO 2DULTRASOUND IN FETAL ANOMALY DIAGNOSIS IN A NIGERIAN DIAGNOSTIC FACILITY.

Annals of Ibadan postgraduate medicine
2021

CDH12 as a Candidate Gene for Kidney Injury in Posterior Urethral Valve Cases: A Genome-wide Association Study Among Patients with Obstructive Uropathies.

European urology open science
2021

Insight into Posterior Urethral Valve Management: My Two Cents.

Journal of Indian Association of Pediatric Surgeons
2021

Posterior urethral valves and the risk of neurodevelopmental disorders in two FINNISH cohorts.

Journal of pediatric urology
2021

Prenatally diagnosed lower urinary tract obstruction: A 15-year experience at two tertiary centers in Japan.

The journal of obstetrics and gynaecology research
2021

Insight into Posterior Urethral Valve Management.

Journal of Indian Association of Pediatric Surgeons
2021

Case Report: Severe Hyponatremia in Infants With Urinary Tract Infection.

Frontiers in pediatrics
2021

Fetal lower urinary tract obstruction: a current overview of intrauterine diagnosis and treatment.

Ceska gynekologie
2021

Signal Detection in EUROmediCAT: Identification and Evaluation of Medication-Congenital Anomaly Associations and Use of VigiBase as a Complementary Source of Reference.

Drug safety
2021

Renal outcome among children with posterior urethral valve: When to worry?

Urology annals
2021

Comparison of curative effect between cold knife resection and electrosurgical resection of posterior urethral valve (PUV) in children.

Asian journal of surgery
2021

Posterior Urethral Valve and Prenataly Resolved Multicystic Dysplastic Kidney.

Prilozi (Makedonska akademija na naukite i umetnostite. Oddelenie za medicinski nauki)
2021

Renal Transplant in Pediatric Patients With Congenital Abnormalities of the Lower Urinary Tract.

Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation
2021

Incidence of urinary tract infection in neonates with significant indirect Hyperbilirubinemia of unknown etiology: case-control study.

Italian journal of pediatrics
2020

A Rare Association of Posterior Urethral Valve with Anterior Urethral Valve, Large Anterior Urethral Diverticula, and Glanular Hypospadias.

Journal of endourology case reports
2020

Insight into Posterior Urethral Valve from Our Experience: Paradigm Appended to Abate Renal Failure.

Journal of Indian Association of Pediatric Surgeons
2021

Urodynamics in Posterior Urethral Valve: Pursuit of prognostication or optimisation.

Journal of pediatric urology
2021

Are anterior urethral valve and anterior urethral diverticulum two separate entities: A radiological and endoscopic review.

Journal of pediatric urology
2021

Non-obstructive, non-refluxing uretero-colonic anastomosis by colonic sero-muscular excision and extra mural peri-ureteric wrap - A new technique in 17 children.

Journal of pediatric urology
2021

Renal, Bladder and Sexual Outcomes in Adult Men with History of Posterior Urethral Valves Treated in Childhood.

Urology
2019

Congenital megalourethra and posterior urethral valve in a patient with Down syndrome.

Turkish journal of urology
2020

Clinical characteristics of children with congenital anomalies of the kidney and urinary tract and predictive factors of chronic kidney disease.

The Turkish journal of pediatrics
2021

Congenital anterior urethral diverticula with posterior urethral valve: A rare combination, case report.

Urology case reports
2021

An Unusual Urological Manifestation of Williams-Beuren Syndrome: Posterior Urethral Valve.

Urologia internationalis
2020

"When ablation goes wrong"- urethral strictures after ablation of posterior urethral valves-characteristics, management and outcomes".

Journal of pediatric urology
2020

Risk Factors Associated with Chronic Kidney Disease in Infants With Posterior Urethral Valve: A Single Center Study of 110 Patients Managed By Valve Ablation And Bladder Neck Incision.

Urology journal
2020

Role of Urinary Transforming Growth Factor Beta-B1 and Monocyte Chemotactic Protein-1 as Prognostic Biomarkers in Posterior Urethral Valve.

Journal of Indian Association of Pediatric Surgeons
2020

Spinal Cavernoma in an Infant: A Rare Pathological Entity with an Atypical Presentation.

Pediatric neurosurgery
2020

Primary infertility secondary to a hidden posterior urethral valve representing a diagnostic challenge in the fifth decade.

Saudi medical journal
2020

Is Endoscopic Transurethral Incision Really Effective for Boys with Refractory Daytime Incontinence?

Research and reports in urology
2020

Comparative outcome analysis of pediatric kidney transplant in posterior urethral valves children with or without pretransplant Mitrofanoff procedure.

Pediatric transplantation
2020

Can the posterior:anterior urethral ratio on voiding cystourethrogram be used as a reliable predictor of successful posterior urethral valve ablation in male children?

SA journal of radiology
2020

Vesicoamniotic shunting for fetal megacystis in the first trimester with a Somatex® intrauterine shunt.

Archives of gynecology and obstetrics
2020

Serum creatinine levels in cases of posterior urethral valve: 29 years experience of a pediatric urology reference center.

Lower urinary tract symptoms
2019

Short-Term Outcomes of Treatment of Boys with Posterior Urethral Valves.

Nigerian medical journal : journal of the Nigeria Medical Association
2020

Serial voiding urosonography in posterior urethral valve diagnosis and management in pediatric patients.

Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia Pediatrica
2020

Primary Infertility Secondary to Posterior Urethral Valve.

Nigerian journal of surgery : official publication of the Nigerian Surgical Research Society
2020

Urinary Tract Infection in Pediatric Patients on Clean Intermittent Catheterization via a Mitrofanoff port with Reused Catheters - Any Association with Catheter Sterility?

Journal of Indian Association of Pediatric Surgeons
2020

Can rectal catheters be avoided during paediatric urodynamic studies?

Arab journal of urology
2020

Gross Hematuria as a Presenting Feature of Posterior Urethral Valves in a Neonate with Normal Antenatal Sonograms.

Medicines (Basel, Switzerland)
2020

Effect of Early Oxybutynin Treatment on Posterior Urethral Valve Outcomes in Infants: A Randomized Controlled Trial.

The Journal of urology
2020

The perineal midsagittal view in male fetuses - pivotal for assessing genitourinary disorders.

Pediatric radiology
2019

Renal Arterial Doppler Resistive Index in Differentiating Obstructive and Non-Obstructive Hydronephrosis in Children at Tikur Anbessa Specialized Hospital, Ethiopia, October, 2018.

Ethiopian journal of health sciences
2019

Survival and Kidney Outcomes of Children with an Early Diagnosis of Posterior Urethral Valves.

Clinical journal of the American Society of Nephrology : CJASN
2020

Fetal Cystoscopy and Vesicoamniotic Shunting in Lower Urinary Tract Obstruction: Long-Term Outcome and Current Technical Limitations.

Fetal diagnosis and therapy
2019

Posterior urethral valve and anterior rectal duplication: a new combination.

BMJ case reports
2019

Urinary Ascites and Transient Intestinal Obstruction in a Preterm Infant: An Interesting Case of Posterior Urethral Valve.

AJP reports
2019

Laparoscopy-assisted ureterostomy- technique.

Journal of pediatric urology
2019

Double trouble: A rare case of posterior urethral valve and Cobb's collar.

Urology case reports
2019

Nonsecretory intestinocystoplasty: postoperative outcomes of 25 years.

International braz j urol : official journal of the Brazilian Society of Urology
2019

Can transient resting of the bladder with vesicostomy reduce the need for a major surgery in some patients?

Journal of pediatric urology
2019

Rare Variants in BNC2 Are Implicated in Autosomal-Dominant Congenital Lower Urinary-Tract Obstruction.

American journal of human genetics
2020

Biometric and morphological features on magnetic resonance imaging of fetal bladder in lower urinary tract obstruction: new perspectives for fetal cystoscopy.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2019

No single reason behind adult lower urinary tract symptoms in patients with posterior urethral valves.

Scandinavian journal of urology
2019

Prenatal extravasation of urine seems to preserve renal function in boys with posterior urethral valves.

Journal of pediatric urology
2019

Prognostic significance of maternal urinary carbohydrate antigen 19-9 for antenatal diagnosis of posterior urethral valve associated with fetal hydronephrosis.

International urology and nephrology
2019

Clinical Profile and Outcome of Children with Congenital Obstructive Uropathy.

Indian journal of pediatrics
2019

Outcome of posterior urethral valve in 64 children: a single center's 22-year experience.

Minerva urologica e nefrologica = The Italian journal of urology and nephrology
2019

Re: Bladder Contractility Index in Posterior Urethral Valve: A New Marker for Early Prediction of Progression to Renal Failure.

The Journal of urology
2021

Graft Survival in Patients With Lower Urinary Tract Dysfunction.

Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation
2019

Urodynamic Changes after Valve Fulguration Alone and Valve Fulguration with Bladder Neck Incision.

Journal of Indian Association of Pediatric Surgeons
2019

Ureteric implantation into the bowel portion of augmented bladders during kidney transplantation: a review of urological complications and outcomes.

ANZ journal of surgery
2018

Paediatric nephrectomy: Patterns, indications and outcome in a developing country.

Malawi medical journal : the journal of Medical Association of Malawi
2018

A single-center 4-year experience with 47 pediatric renal transplants: Evolving trends.

Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia
2018

Rare association of the anterior and posterior urethral valves.

Pediatrics international : official journal of the Japan Pediatric Society
2019

Predicting posterior urethral obstruction in boys with lower urinary tract symptoms using deep artificial neural network.

World journal of urology
Ver todos os 207 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Válvula uretral posterior.

É de uma associação que acompanha esta doença? Fale com a gente →

Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Válvula uretral posterior

Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.

Tire suas dúvidas

Perguntas, dicas e experiências compartilhadas aqui na página

Participe da discussão

Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.

Fazer login

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Congenital Anomalies of Kidney and Urinary Tract in Children - Clinical Spectrum and Complications.
    Indian journal of pediatrics· 2026· PMID 41484534mais citado
  2. Concomitant Bladder Neck Incision in Patients with Posterior Urethral Valve and Blad-der Neck Hypertrophy: Short-term Outcomes of a Randomized Controlled Trial.
    International braz j urol : official journal of the Brazilian Society of Urology· 2026· PMID 41811016mais citado
  3. Posterior urethral valve presenting as posterior urethral calculus in adult: A rare case scenario.
    Urology case reports· 2026· PMID 41809426mais citado
  4. Long-term sexual function, fertility, and quality of life after childhood treatment for posterior urethral valves: a systematic review of clinical outcomes.
    The journal of sexual medicine· 2026· PMID 41729776mais citado
  5. Fulguration of Anterior Membrane by Endoscopy (FAME): A modified technique of posterior urethral valve fulguration reduces the incidence of urethral strictures.
    Journal of pediatric urology· 2026· PMID 41249069mais citado
  6. Clinical characteristics and kidney outcomes in children with posterior urethral valve: a single-center experience.
    Int Urol Nephrol· 2026· PMID 41995987recente
  7. Preoperative cystourethroscopic evaluation of children with posterior hypospadias.
    Bioinformation· 2025· PMID 41907977recente
  8. Delayed Diagnosis of Posterior Urethral Valves in a 14-Year-Old Adolescent.
    Reports (MDPI)· 2026· PMID 41893437recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:93110(Orphanet)
  2. MONDO:0019640(MONDO)
  3. GARD:7439(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Artigo Wikipedia(Wikipedia)
  7. Q2500550(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Válvula uretral posterior
Compêndio · Raras BR

Válvula uretral posterior

ORPHA:93110 · MONDO:0019640
Prevalência
1-9 / 100 000
Herança
Autosomal recessive, Not applicable, X-linked recessive
CID-10
Q64.2 · Válvulas uretrais posteriores congênitas
CID-11
Ensaios
5 ativos
Início
All ages
Prevalência
2.0 (Europe)
MedGen
UMLS
C0238506
EuropePMC
Wikidata
Wikipedia
Papers 10a
DiscussaoAtiva

Nenhuma novidade ainda. O agente esta monitorando.

0membros
0novidades