PUV
MCID: PST041
MIFTS: 51

Posterior Urethral Valves (PUV)

Categories: Fetal diseases, Nephrological diseases, Rare 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 congenital anomalies of kidney and urinary tract 2 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 spinal cord, 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 157)
# Related Disease Score Top Affiliating Genes
1 congenital anomalies of kidney and urinary tract 2 32.2 TBX18 TGFB1
2 vesicoureteral reflux 1 30.8 AGT AGTR1 AGTR2
3 renal dysplasia 30.4 ACE AGT AGTR1
4 anuria 30.0 ACE AGT AGTR1
5 chronic kidney failure 29.9 ACE AGT AGTR1 IL6 TGFB1
6 pyelonephritis 29.8 ACE IL6 TNF
7 obstructive nephropathy 29.8 ACE AGT AGTR2 TGFB1
8 kidney disease 29.6 ACE AGT AGTR1 AGTR2 IL6 TGFB1
9 end stage renal failure 29.4 ACE AGT AGTR1 IL6 TGFB1 TNF
10 congenital anomalies of kidney and urinary tract 1 11.5
11 prune belly syndrome 10.4
12 down syndrome 10.3
13 hypospadias 10.2
14 pediatric hypertension 10.2 ACE AGT
15 anus, imperforate 10.2
16 orchitis 10.2
17 urinary tract obstruction 10.2
18 epididymo-orchitis 10.2
19 anterior urethral valve 10.2
20 neurogenic hypertension 10.2 ACE2 AGTR1
21 uveoparotid fever 10.2 ACE TNF
22 subacute cutaneous lupus erythematosus 10.2 ACE TNF
23 skin sarcoidosis 10.1 ACE TNF
24 hypertensive nephropathy 10.1 ACE AGTR1
25 granulomatous dermatitis 10.1 ACE TNF
26 malignant essential hypertension 10.1 ACE AGTR2
27 clonorchiasis 10.1 TGFB1 TNF
28 meningococcal infection 10.1 AGT AGTR1
29 diabetic foot ulcers 10.1 IL6 TGFB1
30 renal tubular acidosis 10.1
31 hydronephrosis 10.1
32 bladder neck obstruction 10.1
33 urethral diverticulum 10.1
34 renal tubular acidosis, distal 10.1
35 nephrosclerosis 10.1 ACE AGTR1 TGFB1
36 familial vesicoureteral reflux 10.0 ACE AGTR2 TGFB1
37 renal artery disease 10.0 ACE AGT AGTR1
38 scorpion envenomation 10.0 IL6 TNF
39 myocardial stunning 10.0 AGTR1 AGTR2
40 microvascular complications of diabetes 3 10.0 ACE AGT AGTR1
41 aortic coarctation 10.0 ACE AGT AGTR1
42 malignant hypertension 10.0 ACE AGT AGTR1
43 diastolic heart failure 10.0 ACE AGT AGTR1
44 renal tubular dysgenesis 10.0 ACE AGT AGTR1
45 leukomalacia 10.0 IL6 TNF
46 ischemic optic neuropathy 10.0 ACE AGT AGTR1
47 angioimmunoblastic lymphadenopathy with dysproteinemia 10.0 IL6 TNF
48 hypertensive heart disease 10.0 ACE AGT AGTR1
49 mitral valve disease 10.0 ACE AGT AGTR1
50 silicosis 10.0 ACE TGFB1 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
# 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 ACE ACE2 AGT AGTR1 AGTR2 IL6
2 cardiovascular system MP:0005385 10.13 ACE ACE2 AGT AGTR1 AGTR2 IL6
3 adipose tissue MP:0005375 10.11 ACE AGT AGTR1 AGTR2 IL6 SLC3A1
4 behavior/neurological MP:0005386 10.1 ACE AGT AGTR1 AGTR2 DSTYK IL6
5 homeostasis/metabolism MP:0005376 10.09 ACE ACE2 AGT AGTR1 AGTR2 IL6
6 mortality/aging MP:0010768 10.02 ACE ACE2 AGT AGTR1 AGTR2 IL6
7 immune system MP:0005387 10.01 ACE ACE2 AGT AGTR1 IL6 SLC3A1
8 muscle MP:0005369 9.86 ACE2 AGT AGTR2 IL6 SLC3A1 TBX18
9 integument MP:0010771 9.85 ACE2 AGTR2 IL6 TBX18 TGFB1 TNF
10 nervous system MP:0003631 9.56 AGT AGTR1 AGTR2 DSTYK IL6 TBX18
11 neoplasm MP:0002006 9.55 ACE AGTR2 IL6 TGFB1 TNF
12 renal/urinary system MP:0005367 9.28 ACE ACE2 AGT AGTR1 AGTR2 IL6

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 Antibiotics, Antitubercular Not Applicable
5 Anti-Infective Agents Not Applicable
6 Antitubercular Agents Not Applicable
7 Anti-Bacterial 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, Spinal Cord, Prostate, Heart, Lymph Node, Eye

Publications for Posterior Urethral Valves

Articles related to Posterior Urethral Valves:

(show top 50) (show all 516)
# 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
Neonate with urinary ascites but no hydronephrosis: unusual presentation of posterior urethral valves. ( 30317193 )
2018
7
A clinical predictive model of chronic kidney disease in children with posterior urethral valves. ( 30196383 )
2018
8
Does Voiding Cystourethrogram Exclude Posterior Urethral Valves in Late Presenting Cases? ( 30267391 )
2018
9
Current epidemiology and antenatal presentation of posterior urethral valves: Outcome of BAPS CASS National Audit. ( 30528204 )
2018
10
Posterior urethral valves: comparison of clinical outcomes between postnatal and antenatal cohorts. ( 30554921 )
2018
11
Long-term outcome of pediatric renal transplantation in boys with posterior urethral valves. ( 30037657 )
2018
12
Posterior urethral valves in South African boys: Outcomes and challenges. ( 30182883 )
2018
13
Urine biochemistry to predict long-term outcomes in fetuses with posterior urethral valves. ( 30207389 )
2018
14
Predicting postnatal renal function of prenatally detected posterior urethral valves using fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient determination. ( 28453880 )
2017
15
Current strategies to predict and manage sequelae of posterior urethral valves in children. ( 29159472 )
2017
16
Posterior urethral valves: Impact of low birth weight and preterm delivery on the final renal outcome. ( 29071146 )
2017
17
Prediction of need for intervention in posterior urethral valves: Use of urine osmolality. ( 29223674 )
2017
18
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
19
Caring for infants with posterior urethral valves: A qualitative study of parents' experiences. ( 29110519 )
2017
20
Can quantity of amniotic fluid reliably predict postnatal renal function in boys with posterior urethral valves: a decision curve analysis. ( 28753736 )
2017
21
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
22
Posterior urethral valves: how the study of fetal micturition can help to reach the diagnosis? ( 27351949 )
2016
23
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
24
Posterior urethral valves: are neonatal imaging findings predictive of renal function during early childhood? ( 27401742 )
2016
25
DNA copy number variants: A potentially useful predictor of early onset renal failure in boys with posterior urethral valves. ( 27160979 )
2016
26
Re: Nadir Creatinine in Posterior Urethral Valves: How High is Low Enough? ( 26853033 )
2016
27
Re: Combined Creatinine Velocity and Nadir Creatinine: A Reliable Predictor of Renal Outcome in Neonatally Diagnosed Posterior Urethral Valves. ( 26887751 )
2016
28
Genetic Basis of Posterior Urethral Valves Inheritance. ( 27261184 )
2016
29
Posterior urethral valves and vesicoureteral reflux: can prenatal ultrasonography distinguish between these two conditions in male fetuses? ( 27374758 )
2016
30
Posterior urethral valves (PUV) in pediatric practice - Promoting methods to understand how to diagnose and incise (PUV). ( 26921022 )
2016
31
Challenges in Predicting Renal Outcomes in Boys with Posterior Urethral Valves. ( 27311411 )
2016
32
Rare copy number variants implicated in posterior urethral valves. ( 26663319 )
2016
33
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
34
Controlled Outcomes for Achievement of Urinary Continence among Boys Treated for Posterior Urethral Valves. ( 26964916 )
2016
35
Outcome of Antenatally Presenting Posterior Urethral Valves (PUV) in Children. ( 28031548 )
2016
36
Laser Ablation of Posterior Urethral Valves by Fetal Cystoscopy. ( 25614247 )
2015
37
Concomitant anterior and posterior urethral valves in pediatrics: A single center experience over 12a88years and long-term follow up after endoscopic treatment. ( 25689730 )
2015
38
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
39
Posterior Urethral Valves in Children: Pattern of Presentation and Outcome of Initial Treatment in Ile-Ife, Nigeria. ( 26425072 )
2015
40
Concomitant Anterior and Posterior Urethral Valves: A Comprehensive Review of Literature. ( 25863834 )
2015
41
Posterior urethral valves: Metabolic consequences in a cohort of patients. ( 26096436 )
2015
42
Posterior urethral valves: Risk factors for progression to renal failure. ( 26705688 )
2015
43
Combined creatinine velocity and nadir creatinine: A reliable predictor of renal outcome in neonatally diagnosed posterior urethral valves. ( 26062970 )
2015
44
Study of prognostic significance of antenatal ultrasonography and renin angiotensin system activation in predicting disease severity in posterior urethral valves. ( 25829668 )
2015
45
Split-cuff nipple technique of ureteral reimplantation in children with thick-walled bladders due to posterior urethral valves. ( 25444631 )
2015
46
Presentation, management, and outcome of posterior urethral valves in a Nigerian tertiary hospital. ( 25659544 )
2015
47
Antenatal Determinants of Long-Term Kidney Outcome in Boys with Posterior Urethral Valves. ( 26375276 )
2015
48
Late diagnosis of posterior urethral valves. ( 26112258 )
2015
49
Nadir creatinine in posterior urethral valves: How high is low enough? ( 26292912 )
2015
50
Posterior urethral polyp with type I posterior urethral valves: a rare association in a neonate. ( 24767515 )
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 27)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.3 AGT AGTR1 AGTR2 IL6 TGFB1 TNF
2
Show member pathways
12.68 ACE AGT AGTR1 AGTR2 IL6 TGFB1
3
Show member pathways
12.67 AGT AGTR2 IL6 TGFB1
4
Show member pathways
12.06 ACE ACE2 AGT
5 12.05 IL6 TGFB1 TNF
6 12 IL6 TGFB1 TNF
7
Show member pathways
11.97 AGT TGFB1 TNF
8
Show member pathways
11.91 ACE IL6 TGFB1 TNF
9
Show member pathways
11.86 IL6 TGFB1 TNF
10 11.81 IL6 TGFB1 TNF
11 11.77 IL6 TGFB1 TNF
12 11.68 IL6 TGFB1 TNF
13 11.64 IL6 TGFB1 TNF
14 11.58 AGT IL6 TGFB1 TNF
15 11.54 IL6 TGFB1 TNF
16 11.5 IL6 TGFB1 TNF
17 11.45 ACE AGT AGTR1
18 11.41 IL6 TGFB1 TNF
19 11.27 IL6 TGFB1 TNF
20 11.25 AGTR1 IL6 TGFB1 TNF
21 11.12 IL6 TNF
22
Show member pathways
11 ACE ACE2 AGT AGTR1 AGTR2 TGFB1
23 10.97 ACE ACE2
24 10.94 IL6 TNF
25 10.9 IL6 TGFB1 TNF
26 10.85 IL6 TNF
27 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 61)
# 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 9.86 AGT AGTR2 TGFB1
3 kidney development GO:0001822 9.85 ACE AGT AGTR1
4 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.84 AGT IL6 TGFB1
5 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.84 AGT IL6 TGFB1 TNF
6 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.83 IL6 TGFB1 TNF
7 positive regulation of inflammatory response GO:0050729 9.81 AGT AGTR1 TNF
8 regulation of inflammatory response GO:0050727 9.8 ACE2 AGTR1 TNF
9 regulation of blood pressure GO:0008217 9.79 ACE AGT AGTR2
10 negative regulation of fat cell differentiation GO:0045599 9.77 IL6 TGFB1 TNF
11 positive regulation of nitric oxide biosynthetic process GO:0045429 9.76 AGT AGTR2 TNF
12 osteoclast differentiation GO:0030316 9.73 TGFB1 TNF
13 positive regulation of cytokine production GO:0001819 9.73 AGT TNF
14 positive regulation of cytokine secretion GO:0050715 9.72 AGTR2 TNF
15 positive regulation of extrinsic apoptotic signaling pathway GO:2001238 9.72 AGT AGTR2
16 negative regulation of myoblast differentiation GO:0045662 9.72 TGFB1 TNF
17 negative regulation of neurogenesis GO:0050768 9.72 IL6 TNF
18 positive regulation of protein complex assembly GO:0031334 9.72 TGFB1 TNF
19 regulation of cell proliferation GO:0042127 9.72 ACE2 AGT AGTR1 TGFB1 TNF
20 positive regulation of blood vessel diameter GO:0097755 9.71 AGT AGTR2
21 protein import into nucleus, translocation GO:0000060 9.71 TGFB1 TNF
22 positive regulation of blood pressure GO:0045777 9.71 ACE AGT
23 positive regulation of chemokine production GO:0032722 9.71 IL6 TNF
24 vasodilation GO:0042311 9.71 AGT AGTR2
25 nitric oxide mediated signal transduction GO:0007263 9.7 AGT AGTR2
26 cellular sodium ion homeostasis GO:0006883 9.7 AGT AGTR2
27 positive regulation of glial cell proliferation GO:0060252 9.7 IL6 TNF
28 positive regulation of macrophage derived foam cell differentiation GO:0010744 9.69 AGT AGTR1
29 cell growth involved in cardiac muscle cell development GO:0061049 9.69 AGT AGTR2
30 positive regulation of superoxide anion generation GO:0032930 9.69 AGT TGFB1
31 low-density lipoprotein particle remodeling GO:0034374 9.68 AGT AGTR1
32 positive regulation of renal sodium excretion GO:0035815 9.68 AGT AGTR2
33 angiotensin maturation GO:0002003 9.67 ACE ACE2
34 angiotensin-activated signaling pathway GO:0038166 9.66 AGTR1 AGTR2
35 negative regulation of lipid storage GO:0010888 9.66 IL6 TNF
36 positive regulation of cholesterol esterification GO:0010873 9.65 AGT AGTR1
37 neutrophil mediated immunity GO:0002446 9.65 ACE IL6
38 regulation of protein import into nucleus GO:0042306 9.64 AGTR2 TGFB1
39 positive regulation of NAD(P)H oxidase activity GO:0033864 9.63 AGT AGTR1
40 blood vessel remodeling GO:0001974 9.63 ACE AGT AGTR2
41 positive regulation of neuroinflammatory response GO:0150078 9.62 IL6 TNF
42 negative regulation of neurotrophin TRK receptor signaling pathway GO:0051387 9.61 AGT AGTR2
43 positive regulation of reactive oxygen species metabolic process GO:2000379 9.61 ACE2 AGT AGTR1
44 regulation of renal sodium excretion GO:0035813 9.6 AGT AGTR1
45 positive regulation of gap junction assembly GO:1903598 9.58 ACE2 AGT
46 positive regulation of branching involved in ureteric bud morphogenesis GO:0090190 9.58 AGT AGTR2 TGFB1
47 positive regulation of mononuclear cell migration GO:0071677 9.57 TGFB1 TNF
48 receptor biosynthetic process GO:0032800 9.56 ACE2 TNF
49 regulation of blood vessel diameter by renin-angiotensin GO:0002034 9.55 AGT AGTR1
50 positive regulation of cellular protein metabolic process GO:0032270 9.54 AGT AGTR1 TGFB1

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 9.71 AGTR1 SLC3A1 TBX18 TGFB1
2 growth factor activity GO:0008083 9.54 AGT IL6 TGFB1
3 carboxypeptidase activity GO:0004180 9.32 ACE ACE2
4 exopeptidase activity GO:0008238 9.26 ACE ACE2
5 angiotensin type II receptor activity GO:0004945 9.16 AGTR1 AGTR2
6 peptidyl-dipeptidase activity GO:0008241 8.96 ACE ACE2
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
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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