MCID: PST041
MIFTS: 50

Posterior Urethral Valves

Categories: Fetal diseases, Nephrological diseases, Rare diseases

Aliases & Classifications for Posterior Urethral Valves

MalaCards integrated aliases for Posterior Urethral Valves:

Name: Posterior Urethral Valves 54
Congenital Posterior Urethral Valves 74
Posterior Urethral Valve 60
Puv 60

Characteristics:

Orphanet epidemiological data:

60
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:

ICD10 34 Q64.2
ICD10 via Orphanet 35 Q64.2
UMLS via Orphanet 75 C0238506 C0542520
Orphanet 60 ORPHA93110
UMLS 74 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 NFAT and Cardiac Hypertrophy. The drugs Pancuronium and Fentanyl have been mentioned in the context of this disorder. Affiliated tissues include kidney, heart and spinal cord, and related phenotypes are Decreased substrate adherent cell growth and growth/size/body region

Wikipedia : 77 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 32.4 TBX18 TGFB1
2 vesicoureteral reflux 1 30.8 AGT AGTR1 AGTR2
3 renal dysplasia 30.5 ACE AGT AGTR1
4 anuria 30.1 ACE AGT AGTR1
5 obstructive nephropathy 29.7 ACE AGT AGTR2 TGFB1
6 pyelonephritis 29.6 ACE IL6 TNF
7 chronic kidney failure 29.6 ACE AGT AGTR1 IL6 TGFB1
8 end stage renal failure 29.1 ACE AGT AGTR1 IL6 TGFB1 TNF
9 kidney disease 29.1 ACE AGT AGTR1 AGTR2 IL6 TGFB1
10 congenital anomalies of kidney and urinary tract 1 11.5
11 congenital anomalies of kidney and urinary tract 3 11.3
12 prune belly syndrome 10.4
13 down syndrome 10.4
14 pediatric hypertension 10.3 ACE AGT
15 urinary tract obstruction 10.3
16 hypospadias 10.3
17 hypertensive nephropathy 10.2 ACE AGTR1
18 uveoparotid fever 10.2 ACE TNF
19 neurogenic hypertension 10.2 ACE2 AGTR1
20 vaccinia 10.2
21 subacute cutaneous lupus erythematosus 10.2 ACE TNF
22 skin sarcoidosis 10.2 ACE TNF
23 meningococcal infection 10.2 AGT AGTR1
24 granulomatous dermatitis 10.2 ACE TNF
25 anus, imperforate 10.2
26 hydronephrosis 10.2
27 orchitis 10.2
28 epididymo-orchitis 10.2
29 duplication of urethra 10.2
30 anterior urethral valve 10.2
31 malignant essential hypertension 10.2 ACE AGTR2
32 clonorchiasis 10.2 TGFB1 TNF
33 nephrosclerosis 10.1 ACE AGTR1 TGFB1
34 cryptorchidism, unilateral or bilateral 10.1
35 renal tubular acidosis 10.1
36 bladder neck obstruction 10.1
37 urethral diverticulum 10.1
38 renal tubular acidosis, distal 10.1
39 renal artery disease 10.1 ACE AGT AGTR1
40 microvascular complications of diabetes 3 10.1 ACE AGT AGTR1
41 aortic coarctation 10.1 ACE AGT AGTR1
42 malignant hypertension 10.1 ACE AGT AGTR1
43 diastolic heart failure 10.1 ACE AGT AGTR1
44 renal tubular dysgenesis 10.1 ACE AGT AGTR1
45 ischemic optic neuropathy 10.1 ACE AGT AGTR1
46 hypertensive heart disease 10.1 ACE AGT AGTR1
47 mitral valve disease 10.1 ACE AGT AGTR1
48 oligohydramnios 10.1 ACE AGT AGTR1
49 myocardial stunning 10.1 AGTR1 AGTR2
50 glioma 10.1

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:

27
# 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:

47 (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 16974-53-1, 15500-66-0 441289 27350
2
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 2 437-38-7 3345
3 Anesthetics Phase 2,Not Applicable
4 Antitubercular Agents Not Applicable
5 Antibiotics, Antitubercular Not Applicable
6 Anti-Bacterial Agents Not Applicable
7 Anti-Infective 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:

42
Kidney, Heart, Spinal Cord, Prostate, Skin, Eye

Publications for Posterior Urethral Valves

Articles related to Posterior Urethral Valves:

(show top 50) (show all 524)
# Title Authors Year
1
Consensus on the Management of Posterior Urethral Valves from Antenatal Period to Puberty. ( 30686881 )
2019
2
Current epidemiology and antenatal presentation of posterior urethral valves: Outcome of BAPS CASS National Audit. ( 30528204 )
2019
3
Does Voiding Cystourethrogram Exclude Posterior Urethral Valves in Late Presenting Cases? ( 30267391 )
2019
4
No single reason behind adult lower urinary tract symptoms in patients with posterior urethral valves. ( 31006310 )
2019
5
Prenatal extravasation of urine seems to preserve renal function in boys with posterior urethral valves. ( 30982696 )
2019
6
Long-term growth in children with posterior urethral valves. ( 30948260 )
2019
7
Re: Caring for Infants with Posterior Urethral Valves: . ( 30759650 )
2019
8
Correlation between the estimated GFR and SWRD score in patients with posterior urethral valves at King Abdul-Aziz University Hospital. ( 30755266 )
2019
9
Renal Replacement Therapy and Intermittent Catheterization Risk in Posterior Urethral Valves. ( 30709926 )
2019
10
A clinical predictive model of chronic kidney disease in children with posterior urethral valves. ( 30196383 )
2019
11
Improvise, Adapt, Overcome: mobile phone LED used as a light source for cystoscopy and resection of posterior urethral valves in a low-cost setting. ( 30591408 )
2018
12
Rare association of the anterior and posterior urethral valves. ( 30585406 )
2018
13
Posterior urethral valves: comparison of clinical outcomes between postnatal and antenatal cohorts. ( 30554921 )
2018
14
Urine biochemistry to predict long-term outcomes in fetuses with posterior urethral valves. ( 30207389 )
2018
15
Posterior urethral valves in South African boys: Outcomes and challenges. ( 30182883 )
2018
16
Long-term outcome of pediatric renal transplantation in boys with posterior urethral valves. ( 30037657 )
2018
17
Management of posterior urethral valves in fetuses and neonates: a critical point of view. ( 29795074 )
2018
18
Posterior urethral valves: 10 years audit of epidemiologic, diagnostic and therapeutic aspects in Yaoundé gynaeco-obstetric and paediatric hospital. ( 29783971 )
2018
19
Predicting and Modifying Risk for Development of Renal Failure in Boys with Posterior Urethral Valves. ( 29774481 )
2018
20
All grown up: A transitional care perspective on the patient with posterior urethral valves. ( 29681268 )
2018
21
The protective arm of the renin-angiotensin system may counteract the intense inflammatory process in fetuses with posterior urethral valves. ( 29534877 )
2018
22
Prediction of need for intervention in posterior urethral valves: Use of urine osmolality. ( 29223674 )
2018
23
Current strategies to predict and manage sequelae of posterior urethral valves in children. ( 29159472 )
2018
24
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 )
2018
25
Neonate with urinary ascites but no hydronephrosis: unusual presentation of posterior urethral valves. ( 30317193 )
2018
26
Caring for infants with posterior urethral valves: A qualitative study of parents' experiences. ( 29110519 )
2017
27
Posterior urethral valves: Impact of low birth weight and preterm delivery on the final renal outcome. ( 29071146 )
2017
28
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
29
Can quantity of amniotic fluid reliably predict postnatal renal function in boys with posterior urethral valves: a decision curve analysis. ( 28753736 )
2017
30
Predicting postnatal renal function of prenatally detected posterior urethral valves using fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient determination. ( 28453880 )
2017
31
Outcome of Antenatally Presenting Posterior Urethral Valves (PUV) in Children. ( 28031548 )
2017
32
Posterior urethral valves: are neonatal imaging findings predictive of renal function during early childhood? ( 27401742 )
2016
33
Posterior urethral valves and vesicoureteral reflux: can prenatal ultrasonography distinguish between these two conditions in male fetuses? ( 27374758 )
2016
34
Posterior urethral valves: how the study of fetal micturition can help to reach the diagnosis? ( 27351949 )
2016
35
Challenges in Predicting Renal Outcomes in Boys with Posterior Urethral Valves. ( 27311411 )
2016
36
Genetic Basis of Posterior Urethral Valves Inheritance. ( 27261184 )
2016
37
DNA copy number variants: A potentially useful predictor of early onset renal failure in boys with posterior urethral valves. ( 27160979 )
2016
38
Predicting Risk of Chronic Kidney Disease in Infants and Young Children at Diagnosis of Posterior Urethral Valves: Initial Ultrasound Kidney Characteristics and Validation of Parenchymal Area as Forecasters of Renal Reserve. ( 27017936 )
2016
39
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
40
Controlled Outcomes for Achievement of Urinary Continence among Boys Treated for Posterior Urethral Valves. ( 26964916 )
2016
41
Posterior urethral valves (PUV) in pediatric practice--Promoting methods to understand how to diagnose and incise (PUV). ( 26921022 )
2016
42
Re: Combined Creatinine Velocity and Nadir Creatinine: A Reliable Predictor of Renal Outcome in Neonatally Diagnosed Posterior Urethral Valves. ( 26887751 )
2016
43
Re: Nadir Creatinine in Posterior Urethral Valves: How High is Low Enough? ( 26853033 )
2016
44
Posterior urethral valves: Risk factors for progression to renal failure. ( 26705688 )
2016
45
Rare copy number variants implicated in posterior urethral valves. ( 26663319 )
2016
46
Antenatal Determinants of Long-Term Kidney Outcome in Boys with Posterior Urethral Valves. ( 26375276 )
2016
47
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
48
Urological fistulas after fetal cystoscopic laser ablation of posterior urethral valves: surgical technical aspects. ( 24817027 )
2015
49
Population-specific serum creatinine centiles in neonates with posterior urethral valves already predict long-term renal outcome. ( 25000449 )
2015
50
Posterior Urethral Valves in Children: Pattern of Presentation and Outcome of Initial Treatment in Ile-Ife, Nigeria. ( 26425072 )
2015

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
13.29 AGT AGTR1 AGTR2 IL6 TGFB1 TNF
2
Show member pathways
12.66 AGT AGTR2 IL6 TGFB1
3
Show member pathways
12.62 ACE AGT AGTR1 AGTR2 IL6 TGFB1
4
Show member pathways
12.05 ACE ACE2 AGT
5 12.04 IL6 TGFB1 TNF
6 11.99 IL6 TGFB1 TNF
7
Show member pathways
11.96 AGT TGFB1 TNF
8
Show member pathways
11.87 ACE IL6 TGFB1 TNF
9
Show member pathways
11.85 IL6 TGFB1 TNF
10 11.8 IL6 TGFB1 TNF
11 11.76 IL6 TGFB1 TNF
12 11.67 IL6 TGFB1 TNF
13 11.63 IL6 TGFB1 TNF
14 11.52 IL6 TGFB1 TNF
15 11.48 IL6 TGFB1 TNF
16 11.45 ACE AGT AGTR1
17 11.38 IL6 TGFB1 TNF
18 11.38 AGT IL6 TGFB1 TNF
19 11.23 IL6 TGFB1 TNF
20 11.12 IL6 TNF
21 10.97 ACE ACE2
22 10.93 IL6 TNF
23 10.92 AGTR1 IL6 TGFB1 TNF
24 10.85 IL6 TNF
25 10.79 IL6 TGFB1 TNF
26 10.68 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 9.1 ACE ACE2 AGT IL6 TGFB1 TNF

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

(show top 50) (show all 60)
# 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 regulation of cell proliferation GO:0042127 9.72 ACE2 AGT AGTR1 TGFB1 TNF
19 positive regulation of protein complex assembly GO:0031334 9.71 TGFB1 TNF
20 positive regulation of blood vessel diameter GO:0097755 9.71 AGT AGTR2
21 positive regulation of blood pressure GO:0045777 9.71 ACE AGT
22 positive regulation of chemokine production GO:0032722 9.71 IL6 TNF
23 vasodilation GO:0042311 9.71 AGT AGTR2
24 nitric oxide mediated signal transduction GO:0007263 9.7 AGT AGTR2
25 cellular sodium ion homeostasis GO:0006883 9.7 AGT AGTR2
26 positive regulation of glial cell proliferation GO:0060252 9.7 IL6 TNF
27 positive regulation of macrophage derived foam cell differentiation GO:0010744 9.69 AGT AGTR1
28 cell growth involved in cardiac muscle cell development GO:0061049 9.69 AGT AGTR2
29 positive regulation of superoxide anion generation GO:0032930 9.69 AGT TGFB1
30 low-density lipoprotein particle remodeling GO:0034374 9.68 AGT AGTR1
31 positive regulation of renal sodium excretion GO:0035815 9.68 AGT AGTR2
32 angiotensin maturation GO:0002003 9.67 ACE ACE2
33 angiotensin-activated signaling pathway GO:0038166 9.66 AGTR1 AGTR2
34 negative regulation of lipid storage GO:0010888 9.66 IL6 TNF
35 positive regulation of cholesterol esterification GO:0010873 9.65 AGT AGTR1
36 neutrophil mediated immunity GO:0002446 9.65 ACE IL6
37 regulation of protein import into nucleus GO:0042306 9.64 AGTR2 TGFB1
38 positive regulation of NAD(P)H oxidase activity GO:0033864 9.63 AGT AGTR1
39 blood vessel remodeling GO:0001974 9.63 ACE AGT AGTR2
40 positive regulation of neuroinflammatory response GO:0150078 9.62 IL6 TNF
41 negative regulation of neurotrophin TRK receptor signaling pathway GO:0051387 9.61 AGT AGTR2
42 positive regulation of reactive oxygen species metabolic process GO:2000379 9.61 ACE2 AGT AGTR1
43 regulation of renal sodium excretion GO:0035813 9.6 AGT AGTR1
44 positive regulation of gap junction assembly GO:1903598 9.58 ACE2 AGT
45 positive regulation of branching involved in ureteric bud morphogenesis GO:0090190 9.58 AGT AGTR2 TGFB1
46 positive regulation of mononuclear cell migration GO:0071677 9.57 TGFB1 TNF
47 receptor biosynthetic process GO:0032800 9.56 ACE2 TNF
48 regulation of blood vessel diameter by renin-angiotensin GO:0002034 9.55 AGT AGTR1
49 positive regulation of cellular protein metabolic process GO:0032270 9.54 AGT AGTR1 TGFB1
50 angiotensin-mediated drinking behavior GO:0003051 9.52 ACE2 AGT

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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
Content
Loading form....