MCID: PST041
MIFTS: 49

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 53
Congenital Posterior Urethral Valves 73
Posterior Urethral Valve 59
Puv 59

Characteristics:

Orphanet epidemiological data:

59
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 59 ORPHA93110
UMLS via Orphanet 74 C0238506 C0542520
ICD10 via Orphanet 34 Q64.2
UMLS 73 C0238506

Summaries for Posterior Urethral Valves

MalaCards based summary : Posterior Urethral Valves, also known as congenital posterior urethral valves, is related to renal dysplasia and vesicoureteral reflux 1. 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 p70S6K Signaling. The drugs Pancuronium and Fentanyl 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 : 76 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 149)
# Related Disease Score Top Affiliating Genes
1 renal dysplasia 30.5 ACE AGT AGTR1
2 vesicoureteral reflux 1 30.2 AGT AGTR1 AGTR2 DSTYK
3 anuria 30.1 ACE AGT AGTR1
4 kidney disease 29.7 ACE AGT AGTR1 TGFB1
5 prostatitis 29.4 IL6 TGFB1 TNF
6 pyelonephritis 29.2 ACE IL6 TNF
7 chronic kidney failure 29.0 ACE AGT AGTR1 IL6 TGFB1
8 end stage renal failure 27.2 ACE AGT AGTR1 IL6 TGFB1 TNF
9 congenital anomalies of kidney and urinary tract 1 11.3
10 urethritis 11.2
11 congenital anomalies of kidney and urinary tract 2 11.2
12 pediatric hypertension 10.7 ACE AGT
13 renal artery disease 10.6 ACE AGTR1
14 diabetic foot ulcers 10.5 IL6 TGFB1
15 hypertensive nephropathy 10.5 ACE AGTR1
16 familial hypertension 10.5 AGT AGTR1
17 angina pectoris 10.5 ACE IL6
18 meningococcal infection 10.4 AGT AGTR1
19 prune belly syndrome 10.3
20 malignant essential hypertension 10.3 ACE AGTR2
21 neurogenic hypertension 10.3 ACE2 AGTR1
22 nephrosclerosis 10.3 ACE AGTR1 TGFB1
23 aortic coarctation 10.2 ACE AGT AGTR1
24 diastolic heart failure 10.2 ACE AGT AGTR1
25 malignant hypertension 10.2 ACE AGT AGTR1
26 renal tubular dysgenesis 10.2 ACE AGT AGTR1
27 down syndrome 10.2
28 ischemic optic neuropathy 10.2 ACE AGT AGTR1
29 hypertensive heart disease 10.2 ACE AGT AGTR1
30 oligohydramnios 10.2 ACE AGT AGTR1
31 mitral valve disease 10.2 ACE AGT AGTR1
32 familial vesicoureteral reflux 10.1 ACE AGTR2 TGFB1
33 aging 10.1
34 urethral stricture 10.1
35 subacute cutaneous lupus erythematosus 10.1 ACE TNF
36 skin sarcoidosis 10.1 ACE TNF
37 granulomatous dermatitis 10.1 ACE TNF
38 anus, imperforate 10.1
39 epididymo-orchitis 10.1
40 orchitis 10.1
41 anterior urethral valve 10.1
42 ureterocele 10.1
43 urinary tract obstruction 10.1
44 atrial fibrillation 10.1 ACE AGT AGTR1
45 beryllium disease 10.1 TGFB1 TNF
46 clonorchiasis 10.0 TGFB1 TNF
47 ureteral obstruction 10.0 AGTR1 AGTR2 TGFB1
48 urethral diverticulum 10.0
49 epididymitis 10.0
50 bladder neck obstruction 10.0

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
# 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:

46 (show all 12)
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.16 AGTR2 ACE2 AGT IL6 AGTR1 ACE
2 cardiovascular system MP:0005385 10.13 AGT IL6 AGTR1 ACE AGTR2 ACE2
3 adipose tissue MP:0005375 10.11 AGT IL6 AGTR1 ACE AGTR2 TGFB1
4 behavior/neurological MP:0005386 10.1 AGTR2 DSTYK AGT AGTR1 ACE TGFB1
5 homeostasis/metabolism MP:0005376 10.09 AGTR2 ACE2 AGT IL6 AGTR1 ACE
6 mortality/aging MP:0010768 10.02 AGTR2 ACE2 AGT IL6 AGTR1 ACE
7 immune system MP:0005387 10.01 ACE2 AGT AGTR1 ACE TGFB1 IL6
8 integument MP:0010771 9.85 ACE2 AGTR2 TNF TBX18 TGFB1 IL6
9 muscle MP:0005369 9.8 AGTR2 ACE2 AGT IL6 TBX18 TGFB1
10 nervous system MP:0003631 9.56 AGTR2 DSTYK AGT AGTR1 TBX18 TGFB1
11 neoplasm MP:0002006 9.55 AGTR2 ACE TNF TGFB1 IL6
12 renal/urinary system MP:0005367 9.28 AGT IL6 AGTR1 ACE AGTR2 ACE2

Drugs & Therapeutics for Posterior Urethral Valves

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

(show all 7)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Pancuronium Approved Phase 2 15500-66-0 27350 441289
2
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 2 437-38-7 3345
3 Anesthetics Phase 2,Not Applicable
4 Anti-Bacterial Agents Not Applicable
5 Antibiotics, Antitubercular Not Applicable
6 Anti-Infective Agents Not Applicable
7 Antitubercular Agents Not Applicable

Interventional clinical trials:


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

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:

41
Lung, Kidney, Prostate, Testes, Spinal Cord

Publications for Posterior Urethral Valves

Articles related to Posterior Urethral Valves:

(show top 50) (show all 561)
# Title Authors Year
1
Posterior urethral valves: 10 years audit of epidemiologic, diagnostic and therapeutic aspects in YaoundAc gynaeco-obstetric and paediatric hospital. ( 29783971 )
2018
2
All grown up: A transitional care perspective on the patient with posterior urethral valves. ( 29681268 )
2018
3
Predicting and Modifying Risk for Development of Renal Failure in Boys with Posterior Urethral Valves. ( 29774481 )
2018
4
The protective arm of the renin-angiotensin system may counteract the intense inflammatory process in fetuses with posterior urethral valves. ( 29534877 )
2018
5
Management of posterior urethral valves in fetuses and neonates: a critical point of view. ( 29795074 )
2018
6
Predicting postnatal renal function of prenatally detected posterior urethral valves using fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient determination. ( 28453880 )
2017
7
Current strategies to predict and manage sequelae of posterior urethral valves in children. ( 29159472 )
2017
8
Posterior urethral valves: Impact of low birth weight and preterm delivery on the final renal outcome. ( 29071146 )
2017
9
Aggressive diagnosis and treatment for posterior urethral valve as an etiology for vesicoureteral reflux or urge incontinence in children. ( 28612060 )
2017
10
Prediction of need for intervention in posterior urethral valves: Use of urine osmolality. ( 29223674 )
2017
11
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
12
Caring for infants with posterior urethral valves: A qualitative study of parents' experiences. ( 29110519 )
2017
13
Can quantity of amniotic fluid reliably predict postnatal renal function in boys with posterior urethral valves: a decision curve analysis. ( 28753736 )
2017
14
Impact of Adjuvant Urinary Diversion versus Valve Ablation Alone on Progression from Chronic to End Stage Renal Disease in Posterior Urethral Valves: A Single Institution 15-Year Time-to-Event Analysis. ( 29061539 )
2017
15
Posterior urethral valves: how the study of fetal micturition can help to reach the diagnosis? ( 27351949 )
2016
16
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
17
Posterior urethral valves: are neonatal imaging findings predictive of renal function during early childhood? ( 27401742 )
2016
18
DNA copy number variants: A potentially useful predictor of early onset renal failure in boys with posterior urethral valves. ( 27160979 )
2016
19
Re: Nadir Creatinine in Posterior Urethral Valves: How High is Low Enough? ( 26853033 )
2016
20
Re: Combined Creatinine Velocity and Nadir Creatinine: A Reliable Predictor of Renal Outcome in Neonatally Diagnosed Posterior Urethral Valves. ( 26887751 )
2016
21
Genetic Basis of Posterior Urethral Valves Inheritance. ( 27261184 )
2016
22
Posterior urethral valves and vesicoureteral reflux: can prenatal ultrasonography distinguish between these two conditions in male fetuses? ( 27374758 )
2016
23
Posterior urethral valves (PUV) in pediatric practice - Promoting methods to understand how to diagnose and incise (PUV). ( 26921022 )
2016
24
Challenges in Predicting Renal Outcomes in Boys with Posterior Urethral Valves. ( 27311411 )
2016
25
Rare copy number variants implicated in posterior urethral valves. ( 26663319 )
2016
26
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
27
Controlled Outcomes for Achievement of Urinary Continence among Boys Treated for Posterior Urethral Valves. ( 26964916 )
2016
28
Outcome of Antenatally Presenting Posterior Urethral Valves (PUV) in Children. ( 28031548 )
2016
29
Laser Ablation of Posterior Urethral Valves by Fetal Cystoscopy. ( 25614247 )
2015
30
Concomitant anterior and posterior urethral valves in pediatrics: A single center experience over 12a88years and long-term follow up after endoscopic treatment. ( 25689730 )
2015
31
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
32
Posterior urethral valve with unilateral vesicoureteral reflux and patent urachus: A rare combination of urinary tract anomaliess. ( 25836363 )
2015
33
Posterior Urethral Valves in Children: Pattern of Presentation and Outcome of Initial Treatment in Ile-Ife, Nigeria. ( 26425072 )
2015
34
Concomitant Anterior and Posterior Urethral Valves: A Comprehensive Review of Literature. ( 25863834 )
2015
35
Posterior urethral valves: Metabolic consequences in a cohort of patients. ( 26096436 )
2015
36
Posterior urethral valves: Risk factors for progression to renal failure. ( 26705688 )
2015
37
Combined creatinine velocity and nadir creatinine: A reliable predictor of renal outcome in neonatally diagnosed posterior urethral valves. ( 26062970 )
2015
38
Study of prognostic significance of antenatal ultrasonography and renin angiotensin system activation in predicting disease severity in posterior urethral valves. ( 25829668 )
2015
39
Split-cuff nipple technique of ureteral reimplantation in children with thick-walled bladders due to posterior urethral valves. ( 25444631 )
2015
40
Presentation, management, and outcome of posterior urethral valves in a Nigerian tertiary hospital. ( 25659544 )
2015
41
Antenatal Determinants of Long-Term Kidney Outcome in Boys with Posterior Urethral Valves. ( 26375276 )
2015
42
Late diagnosis of posterior urethral valves. ( 26112258 )
2015
43
Nadir creatinine in posterior urethral valves: How high is low enough? ( 26292912 )
2015
44
Posterior urethral polyp with type I posterior urethral valves: a rare association in a neonate. ( 24767515 )
2014
45
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
46
Urinary tract tuberculosis in a child with late presentation posterior urethral valves. ( 24529021 )
2014
47
Population-specific serum creatinine centiles in neonates with posterior urethral valves already predict long-term renal outcome. ( 25000449 )
2014
48
Re: long-term bladder function, fertility and sexual function in patients with posterior urethral valves treated in infancy. ( 24331507 )
2014
49
Urological fistulas after fetal cystoscopic laser ablation of posterior urethral valves - surgical technical aspects. ( 24817027 )
2014
50
Neonatal presentation of posterior urethral valves and tethered spinal cord. ( 25073498 )
2014

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)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.24 AGT AGTR1 AGTR2 IL6 TGFB1 TNF
2
Show member pathways
12.68 ACE AGT AGTR1 AGTR2 IL6 TGFB1
3
Show member pathways
12.66 AGT AGTR2 IL6 TGFB1
4
Show member pathways
12.07 IL6 TGFB1 TNF
5
Show member pathways
12.05 ACE ACE2 AGT
6 12.03 IL6 TGFB1 TNF
7 11.98 IL6 TGFB1 TNF
8
Show member pathways
11.95 AGT TGFB1 TNF
9
Show member pathways
11.91 ACE IL6 TGFB1 TNF
10 11.81 IL6 TGFB1 TNF
11 11.77 IL6 TGFB1 TNF
12 11.67 IL6 TGFB1 TNF
13 11.63 IL6 TGFB1 TNF
14 11.58 AGT IL6 TGFB1 TNF
15 11.52 IL6 TGFB1 TNF
16 11.45 ACE AGT AGTR1
17 11.41 IL6 TGFB1 TNF
18 11.27 IL6 TGFB1 TNF
19 11.25 AGTR1 IL6 TGFB1 TNF
20 11.12 IL6 TNF
21
Show member pathways
11 ACE ACE2 AGT AGTR1 AGTR2 TGFB1
22 10.96 ACE ACE2
23 10.93 IL6 TNF
24 10.91 IL6 TGFB1 TNF
25 10.85 IL6 TNF
26 10.69 TGFB1 TNF

GO Terms for Posterior Urethral Valves

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

# 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 56)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 9.98 AGTR2 IL6 TGFB1 TNF
2 negative regulation of cell growth GO:0030308 9.85 AGT AGTR2 TGFB1
3 kidney development GO:0001822 9.84 ACE AGT AGTR1
4 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.83 AGT IL6 TGFB1
5 regulation of inflammatory response GO:0050727 9.82 ACE2 AGTR1 TNF
6 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.81 IL6 TGFB1 TNF
7 regulation of blood pressure GO:0008217 9.79 ACE AGT AGTR2
8 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.78 AGT IL6 TGFB1 TNF
9 negative regulation of fat cell differentiation GO:0045599 9.77 IL6 TGFB1 TNF
10 regulation of cell proliferation GO:0042127 9.77 ACE2 AGT AGTR1 TGFB1 TNF
11 osteoclast differentiation GO:0030316 9.72 TGFB1 TNF
12 positive regulation of cytokine production GO:0001819 9.72 AGT TNF
13 positive regulation of cytokine secretion GO:0050715 9.72 AGTR2 TNF
14 positive regulation of nitric oxide biosynthetic process GO:0045429 9.72 AGT AGTR2 TNF
15 protein import into nucleus, translocation GO:0000060 9.71 TGFB1 TNF
16 positive regulation of extrinsic apoptotic signaling pathway GO:2001238 9.71 AGT AGTR2
17 positive regulation of blood vessel diameter GO:0097755 9.71 AGT AGTR2
18 positive regulation of protein complex assembly GO:0031334 9.71 TGFB1 TNF
19 negative regulation of myoblast differentiation GO:0045662 9.7 TGFB1 TNF
20 vasodilation GO:0042311 9.7 AGT AGTR2
21 positive regulation of chemokine production GO:0032722 9.7 IL6 TNF
22 nitric oxide mediated signal transduction GO:0007263 9.69 AGT AGTR2
23 cellular sodium ion homeostasis GO:0006883 9.69 AGT AGTR2
24 cell growth involved in cardiac muscle cell development GO:0061049 9.69 AGT AGTR2
25 positive regulation of macrophage derived foam cell differentiation GO:0010744 9.68 AGT AGTR1
26 positive regulation of superoxide anion generation GO:0032930 9.68 AGT TGFB1
27 positive regulation of renal sodium excretion GO:0035815 9.68 AGT AGTR2
28 low-density lipoprotein particle remodeling GO:0034374 9.67 AGT AGTR1
29 blood vessel remodeling GO:0001974 9.67 ACE AGT AGTR2
30 neutrophil mediated immunity GO:0002446 9.66 ACE IL6
31 negative regulation of lipid storage GO:0010888 9.65 IL6 TNF
32 positive regulation of cholesterol esterification GO:0010873 9.65 AGT AGTR1
33 angiotensin-activated signaling pathway GO:0038166 9.65 AGTR1 AGTR2
34 regulation of protein import into nucleus GO:0042306 9.63 AGTR2 TGFB1
35 positive regulation of NAD(P)H oxidase activity GO:0033864 9.63 AGT AGTR1
36 positive regulation of reactive oxygen species metabolic process GO:2000379 9.63 ACE2 AGT AGTR1
37 regulation of renal sodium excretion GO:0035813 9.61 AGT AGTR1
38 negative regulation of neurotrophin TRK receptor signaling pathway GO:0051387 9.61 AGT AGTR2
39 positive regulation of branching involved in ureteric bud morphogenesis GO:0090190 9.61 AGT AGTR2 TGFB1
40 receptor biosynthetic process GO:0032800 9.59 ACE2 TNF
41 regulation of blood vessel diameter by renin-angiotensin GO:0002034 9.58 AGT AGTR1
42 positive regulation of gap junction assembly GO:1903598 9.58 ACE2 AGT
43 positive regulation of cellular protein metabolic process GO:0032270 9.58 AGT AGTR1 TGFB1
44 angiotensin-mediated drinking behavior GO:0003051 9.57 ACE2 AGT
45 positive regulation of mononuclear cell migration GO:0071677 9.56 TGFB1 TNF
46 regulation of renal output by angiotensin GO:0002019 9.54 ACE AGT
47 mononuclear cell proliferation GO:0032943 9.52 ACE TGFB1
48 G-protein coupled receptor signaling pathway coupled to cGMP nucleotide second messenger GO:0007199 9.51 AGT AGTR2
49 angiotensin maturation GO:0002003 9.5 ACE ACE2 AGT
50 regulation of vasoconstriction GO:0019229 9.46 ACE ACE2 AGT AGTR1

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

# 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 carboxypeptidase activity GO:0004180 9.26 ACE ACE2
4 peptidyl-dipeptidase activity GO:0008241 9.16 ACE ACE2
5 angiotensin type II receptor activity GO:0004945 8.96 AGTR1 AGTR2
6 bradykinin receptor binding GO:0031711 8.62 ACE AGTR1

Sources for Posterior Urethral Valves

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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