MCID: PST041
MIFTS: 46

Posterior Urethral Valves

Categories: Rare diseases, Nephrological diseases, Fetal diseases

Aliases & Classifications for Posterior Urethral Valves

MalaCards integrated aliases for Posterior Urethral Valves:

Name: Posterior Urethral Valves 50
Congenital Posterior Urethral Valves 69
Posterior Urethral Valve 56
Puv 56

Characteristics:

Orphanet epidemiological data:

56
posterior urethral valve
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe),1-9/100000 (Australia); Age of onset: Antenatal,Neonatal; Age of death: early childhood;

Classifications:



External Ids:

Orphanet 56 ORPHA93110
UMLS via Orphanet 70 C0238506 C0542520
ICD10 via Orphanet 34 Q64.2

Summaries for Posterior Urethral Valves

MalaCards based summary : Posterior Urethral Valves, also known as congenital posterior urethral valves, is related to congenital anomalies of kidney and urinary tract and sinusitis. An important gene associated with Posterior Urethral Valves is DSTYK (Dual Serine/Threonine And Tyrosine Protein Kinase), and among its related pathways/superpathways are TGF-Beta Pathway and Allograft rejection. The drugs Fentanyl and Pancuronium have been mentioned in the context of this disorder. Affiliated tissues include lung, kidney and prostate, and related phenotypes are Decreased substrate adherent cell growth and growth/size/body region

Wikipedia : 72 Posterior urethral valve (PUV) disorder is an obstructive developmental anomaly in the urethra and... more...

Related Diseases for Posterior Urethral Valves

Diseases in the Anterior Urethral Valve family:

Posterior Urethral Valves

Diseases related to Posterior Urethral Valves via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 139)
id Related Disease Score Top Affiliating Genes
1 congenital anomalies of kidney and urinary tract 32.9 DSTYK TBX18
2 sinusitis 29.4 ACE TGFB1 TNF
3 xanthogranulomatous pyelonephritis 27.3 ACE AGT AGTR1 AGTR2 IL6 TNF
4 urethritis 11.1
5 congenital anomalies of kidney and urinary tract 2 11.1
6 pelizaeus-merzbacher-like disease 10.6 ACE AGT
7 clitoris cancer 10.5 ACE AGTR1
8 familial isolated pituitary adenoma 10.4 AGT AGTR1
9 chronic diarrhea due to guanylate cyclase 2c overactivity 10.4 ACE IL6
10 mental retardation cataracts calcified pinnae myopathy 10.4 AGT AGTR1
11 vesicoureteral reflux 10.4
12 hypertrichosis of eyelid 10.3 ACE AGTR1 TGFB1
13 cataract, autosomal dominant congenital 4 10.3 ACE AGT AGTR1
14 adenocarcinoma 10.3 ACE AGT AGTR1
15 aortopulmonary window 10.3 ACE AGT AGTR1
16 trichinosis 10.3 ACE AGT AGTR1
17 chylocele of tunica vaginalis 10.3 ACE AGT AGTR1
18 cascade stomach 10.3 ACE AGT AGTR1
19 renal tubular dysgenesis 10.2 ACE AGT AGTR1
20 prostate neuroendocrine neoplasm 10.2 ACE AGTR1 TGFB1
21 laryngostenosis 10.2 ACE AGT AGTR1
22 joint disorders 10.2 ACE AGT AGTR1
23 congenital bilateral megacalycosis 10.2 ACE AGT AGTR1
24 thoracic spinal canal and spinal cord meningioma 10.2 ACE AGT AGTR1
25 prune belly syndrome 10.2
26 voyeurism 10.2 ACE AGTR2
27 12q15q21.1 microdeletion syndrome 10.2 ACE AGTR2 TGFB1
28 cryptogenic late-onset epileptic spasms 10.1 ACE TNF
29 down syndrome 10.1
30 cardiac sarcoidosis 10.1 ACE TNF
31 neuromuscular disease 10.1 ACE TNF
32 biemond syndrome 10.1 TGFB1 TNF
33 acrofrontofacionasal dysostosis 10.1 ACE AGT AGTR1
34 urethral stricture 10.0
35 renal dysplasia 10.0
36 malignant struma ovarii 10.0 AGTR1 AGTR2 TGFB1
37 smarca4-deficient sarcoma of thorax 10.0 IL6 TNF
38 plantar wart 10.0 TGFB1 TNF
39 aortic arch interruption 10.0 IL6 TNF
40 cutaneous leishmaniasis 10.0 IL6 TNF
41 spirillary rat-bite fever 10.0 IL6 TNF
42 imperforate anus 10.0
43 orchitis 10.0
44 ureterocele 10.0
45 urinary tract obstruction 10.0
46 kidney disease 10.0
47 epididymo-orchitis 10.0
48 anterior urethral valve 10.0
49 toxoplasmosis 10.0 IL6 TNF
50 dyslexia 5 10.0 IL6 TNF

Graphical network of the top 20 diseases related to Posterior Urethral Valves:



Diseases related to Posterior Urethral Valves

Symptoms & Phenotypes for Posterior Urethral Valves

GenomeRNAi Phenotypes related to Posterior Urethral Valves according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased substrate adherent cell growth GR00193-A-1 8.8 AGTR1 AGTR2 DSTYK

MGI Mouse Phenotypes related to Posterior Urethral Valves:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.13 TNF TBX18 TGFB1 ACE AGTR2 ACE2
2 cardiovascular system MP:0005385 10.11 TNF TBX18 TGFB1 ACE AGTR2 ACE2
3 adipose tissue MP:0005375 10.08 TNF TGFB1 AGTR2 IL6 AGT SLC3A1
4 homeostasis/metabolism MP:0005376 10.06 TGFB1 TNF ACE AGTR2 ACE2 IL6
5 immune system MP:0005387 9.97 TGFB1 TNF ACE ACE2 IL6 AGT
6 mortality/aging MP:0010768 9.96 TGFB1 TNF TBX18 ACE AGTR2 ACE2
7 integument MP:0010771 9.8 TGFB1 TNF TBX18 AGTR2 ACE2 IL6
8 muscle MP:0005369 9.7 TNF TBX18 TGFB1 AGTR2 ACE2 IL6
9 neoplasm MP:0002006 9.35 TGFB1 TNF ACE AGTR2 IL6
10 renal/urinary system MP:0005367 9.28 TBX18 TGFB1 AGTR2 ACE2 IL6 AGT

Drugs & Therapeutics for Posterior Urethral Valves

Drugs for Posterior Urethral Valves (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 7)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 2 437-38-7 3345
2
Pancuronium Approved Phase 2 15500-66-0 27350 441289
3 Anesthetics Phase 2
4 Anti-Bacterial Agents
5 Antibiotics, Antitubercular
6 Anti-Infective Agents
7 Antitubercular Agents

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 Fetal Cystoscopy Versus Vesico-amniotic Shunting in Severe Lower Urinary Tract Obstructions Not yet recruiting NCT01552824 Phase 2
2 CIRCumcision and Urinary Tract Infections in Boys With Posterior Urethral Valves Recruiting NCT01537601
3 Standardized Prenatal Clinical Care for LUTO Recruiting NCT02315521
4 Validation of a Fetal Urine Peptidome-based Classifier to Predict Post-natal Renal Function in Posterior Urethral Valves Not yet recruiting NCT03116217
5 Fetal Cystoscopy for Lower Urinary Tract Obstruction Terminated NCT02446184

Search NIH Clinical Center for Posterior Urethral Valves

Genetic Tests for Posterior Urethral Valves

Anatomical Context for Posterior Urethral Valves

MalaCards organs/tissues related to Posterior Urethral Valves:

39
Lung, Kidney, Prostate, Testes, Spinal Cord

Publications for Posterior Urethral Valves

Articles related to Posterior Urethral Valves:

(show top 50) (show all 551)
id Title Authors Year
1
Predicting postnatal renal function of prenatally detected posterior urethral valves using fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient determination. ( 28453880 )
2017
2
Aggressive diagnosis and treatment for posterior urethral valve as an etiology for vesicoureteral reflux or urge incontinence in children. ( 28612060 )
2017
3
Comment on "Predicting postnatal renal function of prenatally detected posterior urethral valves using fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient determination". ( 28777461 )
2017
4
Can quantity of amniotic fluid reliably predict postnatal renal function in boys with posterior urethral valves: a decision curve analysis. ( 28753736 )
2017
5
Posterior urethral valves and vesicoureteral reflux: can prenatal ultrasonography distinguish between these two conditions in male fetuses? ( 27374758 )
2016
6
Posterior urethral valves: are neonatal imaging findings predictive of renal function during early childhood? ( 27401742 )
2016
7
Vesicostomy and Colostomy in a Premature Neonate With Posterior Urethral Valves, Bilateral Dysplastic Kidneys, and High Imperforate Anus: The Challenge of Stoma Placement. ( 27015939 )
2016
8
Challenges in Predicting Renal Outcomes in Boys with Posterior Urethral Valves. ( 27311411 )
2016
9
Predicting risk of chronic kidney disease in infants and young children with posterior urethral valves at time of diagnosis: Objective analysis of initial ultrasound kidney characteristics and validation of parenchyma area as forecasters of renal reserve. ( 27017936 )
2016
10
DNA copy number variants: A potentially useful predictor of early onset renal failure in boys with posterior urethral valves. ( 27160979 )
2016
11
Genetic Basis of Posterior Urethral Valves Inheritance. ( 27261184 )
2016
12
Re: Combined Creatinine Velocity and Nadir Creatinine: A Reliable Predictor of Renal Outcome in Neonatally Diagnosed Posterior Urethral Valves. ( 26887751 )
2016
13
Posterior urethral valves (PUV) in pediatric practice - Promoting methods to understand how to diagnose and incise (PUV). ( 26921022 )
2016
14
Rare copy number variants implicated in posterior urethral valves. ( 26663319 )
2016
15
Controlled Outcomes for Achievement of Urinary Continence among Boys Treated for Posterior Urethral Valves. ( 26964916 )
2016
16
Re: Nadir Creatinine in Posterior Urethral Valves: How High is Low Enough? ( 26853033 )
2016
17
Outcome of Antenatally Presenting Posterior Urethral Valves (PUV) in Children. ( 28031548 )
2016
18
Posterior urethral valves: how the study of fetal micturition can help to reach the diagnosis? ( 27351949 )
2016
19
Posterior urethral valve with unilateral vesicoureteral reflux and patent urachus: A rare combination of urinary tract anomaliess. ( 25836363 )
2015
20
Study of prognostic significance of antenatal ultrasonography and renin angiotensin system activation in predicting disease severity in posterior urethral valves. ( 25829668 )
2015
21
Presentation, management, and outcome of posterior urethral valves in a Nigerian tertiary hospital. ( 25659544 )
2015
22
Concomitant anterior and posterior urethral valves in pediatrics: A single center experience over 12a88years and long-term follow up after endoscopic treatment. ( 25689730 )
2015
23
Combined creatinine velocity and nadir creatinine: A reliable predictor of renal outcome in neonatally diagnosed posterior urethral valves. ( 26062970 )
2015
24
Concomitant Anterior and Posterior Urethral Valves: A Comprehensive Review of Literature. ( 25863834 )
2015
25
Laser Ablation of Posterior Urethral Valves by Fetal Cystoscopy. ( 25614247 )
2015
26
Posterior Urethral Valves in Children: Pattern of Presentation and Outcome of Initial Treatment in Ile-Ife, Nigeria. ( 26425072 )
2015
27
Re: Tran et al.: anterior urethral valve associated with posterior urethral valves: report of 2 cases and review of the literature (Urology 2014;84:469-471). ( 25733298 )
2015
28
Split-cuff nipple technique of ureteral reimplantation in children with thick-walled bladders due to posterior urethral valves. ( 25444631 )
2015
29
Nadir creatinine in posterior urethral valves: How high is low enough? ( 26292912 )
2015
30
Antenatal Determinants of Long-Term Kidney Outcome in Boys with Posterior Urethral Valves. ( 26375276 )
2015
31
Posterior urethral valves: Metabolic consequences in a cohort of patients. ( 26096436 )
2015
32
Posterior urethral valves: Risk factors for progression to renal failure. ( 26705688 )
2015
33
Late diagnosis of posterior urethral valves. ( 26112258 )
2015
34
The management of vesicoureteral reflux in the setting of posterior urethral valve with emphasis on bladder function and renal outcome: a single center cohort study. ( 24149109 )
2014
35
Posterior urethral polyp with type I posterior urethral valves: a rare association in a neonate. ( 24767515 )
2014
36
Anterior urethral valve associated with posterior urethral valves: report of 2 cases and review of the literature. ( 24958476 )
2014
37
Late presentation of posterior urethral valves. ( 24906275 )
2014
38
Neonatal presentation of posterior urethral valves and tethered spinal cord. ( 25073498 )
2014
39
Epidemiology and demography of recently diagnosed cases of posterior urethral valves. ( 25198372 )
2014
40
Urinary tract tuberculosis in a child with late presentation posterior urethral valves. ( 24529021 )
2014
41
Re: long-term bladder function, fertility and sexual function in patients with posterior urethral valves treated in infancy. ( 24331507 )
2014
42
Urological fistulas after fetal cystoscopic laser ablation of posterior urethral valves - surgical technical aspects. ( 24817027 )
2014
43
Population-specific serum creatinine centiles in neonates with posterior urethral valves already predict long-term renal outcome. ( 25000449 )
2014
44
Residual valve and stricture after posterior urethral valve ablation: how to evaluate? ( 22364895 )
2013
45
Long-term consequences of posterior urethral valves. ( 23871421 )
2013
46
Renal impairment in children with posterior urethral valves. ( 23263712 )
2013
47
Long-term bladder function, fertility and sexual function in patients with posterior urethral valves treated in infancy. ( 22154080 )
2013
48
Multiple urethral anomalies: anterior urethral diverticulum, posterior urethral valves, and distal hypospadias. ( 23331841 )
2013
49
Posterior urethral valves: Persistent renin angiotensin system activation after valve ablation and role of pre-emptive therapy with angiotensin converting enzyme-inhibitors on renal recovery. ( 23798811 )
2013
50
Use of holmium:YAG laser in posterior urethral valves: another method of fulguration. ( 23660492 )
2013

Variations for Posterior Urethral Valves

Expression for Posterior Urethral Valves

Search GEO for disease gene expression data for Posterior Urethral Valves.

Pathways for Posterior Urethral Valves

Pathways related to Posterior Urethral Valves according to GeneCards Suite gene sharing:

(show all 26)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
13.33 AGT AGTR1 AGTR2 IL6 TGFB1 TNF
2
Show member pathways
12.66 AGTR1 IL6 TGFB1 TNF
3
Show member pathways
12.65 ACE IL6 TGFB1 TNF
4
Show member pathways
12.61 ACE AGT AGTR1 AGTR2 IL6 TGFB1
5
Show member pathways
12.06 ACE ACE2 AGT
6 12.05 IL6 TGFB1 TNF
7 12 IL6 TGFB1 TNF
8
Show member pathways
11.97 AGT TGFB1 TNF
9 11.81 IL6 TGFB1 TNF
10 11.77 IL6 TGFB1 TNF
11
Show member pathways
11.72 IL6 TGFB1 TNF
12 11.65 IL6 TGFB1 TNF
13 11.61 IL6 TGFB1 TNF
14 11.51 IL6 TGFB1 TNF
15 11.45 IL6 TGFB1 TNF
16 11.39 ACE AGT AGTR1
17 11.38 AGT IL6 TGFB1 TNF
18 11.34 IL6 TGFB1 TNF
19 11.19 IL6 TGFB1 TNF
20 11.01 ACE ACE2
21 10.93 IL6 TNF
22 10.92 AGTR1 IL6 TGFB1 TNF
23 10.85 IL6 TNF
24 10.81 IL6 TGFB1 TNF
25 10.7 IL6 TGFB1
26 10.69 TGFB1 TNF

GO Terms for Posterior Urethral Valves

Cellular components related to Posterior Urethral Valves according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.43 ACE ACE2 AGT IL6 TGFB1 TNF
2 extracellular region GO:0005576 9.17 ACE ACE2 AGT AGTR2 IL6 TGFB1

Biological processes related to Posterior Urethral Valves according to GeneCards Suite gene sharing:

(show top 50) (show all 61)
id Name GO ID Score Top Affiliating Genes
1 regulation of apoptotic process GO:0042981 9.96 AGT AGTR2 IL6 TGFB1
2 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.91 DSTYK IL6 TGFB1 TNF
3 aging GO:0007568 9.9 AGT IL6 TGFB1
4 negative regulation of cell growth GO:0030308 9.87 AGT AGTR2 TGFB1
5 kidney development GO:0001822 9.86 ACE AGT AGTR1
6 positive regulation of protein kinase B signaling GO:0051897 9.85 IL6 TGFB1 TNF
7 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.85 AGT IL6 TGFB1
8 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.83 IL6 TGFB1 TNF
9 regulation of blood pressure GO:0008217 9.82 ACE AGT AGTR2
10 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.8 AGT IL6 TGFB1 TNF
11 negative regulation of fat cell differentiation GO:0045599 9.78 IL6 TGFB1 TNF
12 positive regulation of cytokine secretion GO:0050715 9.73 AGTR2 TNF
13 protein import into nucleus, translocation GO:0000060 9.73 TGFB1 TNF
14 positive regulation of cytokine production GO:0001819 9.73 AGT TNF
15 cellular response to dexamethasone stimulus GO:0071549 9.73 AGTR2 IL6 TGFB1
16 regulation of cell proliferation GO:0042127 9.73 ACE2 AGT AGTR1 IL6 TGFB1 TNF
17 positive regulation of extrinsic apoptotic signaling pathway GO:2001238 9.72 AGT AGTR2
18 positive regulation of protein complex assembly GO:0031334 9.72 TGFB1 TNF
19 negative regulation of myoblast differentiation GO:0045662 9.72 TGFB1 TNF
20 blood vessel remodeling GO:0001974 9.72 ACE AGT AGTR2
21 vasodilation GO:0042311 9.71 AGT AGTR2
22 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.71 AGT TNF
23 negative regulation of blood vessel endothelial cell migration GO:0043537 9.71 AGTR2 TGFB1
24 nitric oxide mediated signal transduction GO:0007263 9.71 AGT AGTR2
25 positive regulation of chemokine production GO:0032722 9.71 IL6 TNF
26 positive regulation of macrophage derived foam cell differentiation GO:0010744 9.7 AGT AGTR1
27 cell growth involved in cardiac muscle cell development GO:0061049 9.7 AGT AGTR2
28 low-density lipoprotein particle remodeling GO:0034374 9.69 AGT AGTR1
29 positive regulation of renal sodium excretion GO:0035815 9.69 AGT AGTR2
30 cellular sodium ion homeostasis GO:0006883 9.69 AGT AGTR2
31 positive regulation of superoxide anion generation GO:0032930 9.68 AGT TGFB1
32 neutrophil mediated immunity GO:0002446 9.68 ACE IL6
33 negative regulation of lipid storage GO:0010888 9.67 IL6 TNF
34 positive regulation of cholesterol esterification GO:0010873 9.67 AGT AGTR1
35 positive regulation of nitric oxide biosynthetic process GO:0045429 9.67 AGT AGTR2 IL6 TNF
36 angiotensin-activated signaling pathway GO:0038166 9.66 AGTR1 AGTR2
37 positive regulation of reactive oxygen species metabolic process GO:2000379 9.65 ACE2 AGT AGTR1
38 receptor-mediated virion attachment to host cell GO:0046813 9.64 ACE ACE2
39 epithelial cell proliferation involved in salivary gland morphogenesis GO:0060664 9.64 IL6 TNF
40 positive regulation of NAD(P)H oxidase activity GO:0033864 9.63 AGT AGTR1
41 negative regulation of neurotrophin TRK receptor signaling pathway GO:0051387 9.63 AGT AGTR2
42 positive regulation of branching involved in ureteric bud morphogenesis GO:0090190 9.63 AGT AGTR2 TGFB1
43 regulation of renal sodium excretion GO:0035813 9.62 AGT AGTR1
44 positive regulation of mononuclear cell migration GO:0071677 9.62 TGFB1 TNF
45 positive regulation of cellular protein metabolic process GO:0032270 9.61 AGT AGTR1 TGFB1
46 angiotensin catabolic process in blood GO:0002005 9.6 ACE ACE2
47 receptor biosynthetic process GO:0032800 9.59 ACE2 TNF
48 positive regulation of gap junction assembly GO:1903598 9.58 ACE2 AGT
49 angiotensin-mediated drinking behavior GO:0003051 9.58 ACE2 AGT
50 angiotensin maturation GO:0002003 9.58 ACE ACE2 AGT

Molecular functions related to Posterior Urethral Valves according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 protein heterodimerization activity GO:0046982 9.67 AGTR1 SLC3A1 TBX18 TGFB1
2 growth factor activity GO:0008083 9.5 AGT IL6 TGFB1
3 metallocarboxypeptidase activity GO:0004181 9.4 ACE ACE2
4 carboxypeptidase activity GO:0004180 9.26 ACE ACE2
5 peptidyl-dipeptidase activity GO:0008241 9.16 ACE ACE2
6 angiotensin type II receptor activity GO:0004945 8.96 AGTR1 AGTR2
7 bradykinin receptor binding GO:0031711 8.62 ACE AGTR1

Sources for Posterior Urethral Valves

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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