PUV
MCID: PST041
MIFTS: 47

Posterior Urethral Valves (PUV) malady

Categories: Rare diseases, Nephrological diseases, Fetal diseases

Aliases & Classifications for Posterior Urethral Valves

Aliases & Descriptions 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
ICD10 33 Q64.2

Summaries for Posterior Urethral Valves

MalaCards based summary : Posterior Urethral Valves, also known as congenital posterior urethral valves, is related to tuberculosis and xanthogranulomatous pyelonephritis. An important gene associated with Posterior Urethral Valves is ACE (Angiotensin I Converting Enzyme), 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 : 71 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 127)
id Related Disease Score Top Affiliating Genes
1 tuberculosis 29.9 ACE IL6 TNF
2 xanthogranulomatous pyelonephritis 29.5 ACE AGTR1 IL6 TNF
3 congenital anomalies of kidney and urinary tract 11.2
4 urethritis 11.1
5 pelviureteric junction obstruction 11.1
6 vesicoureteral reflux 10.3
7 vps11-related autosomal recessive hypomyelinating leukodystrophy 10.3 IL6 TNF
8 severe hemophilia a 10.3 ACE TNF
9 cardiac sarcoidosis 10.2 ACE TNF
10 dermatosis papulosa nigra 10.2 ACE TNF
11 penile cancer, childhood 10.2 ACE AGT
12 dyslexia 5 10.2 IL6 TNF
13 bilateral parasagittal parieto-occipital polymicrogyria 10.2 TGFB1 TNF
14 aortic atherosclerosis 10.2 IL6 TNF
15 degenerative disc disease 10.2 IL6 TNF
16 prune belly syndrome 10.2
17 distal trisomy 14q 10.2 TGFB1 TNF
18 unilateral multicystic dysplastic kidney 10.2 IL6 TNF
19 peripheral artery disease 10.2 ACE TGFB1 TNF
20 thymic neuroendocrine carcinoma 10.2 IL6 TNF
21 skin conditions 10.2 ACE TGFB1 TNF
22 anal paget's disease 10.2 IL6 TGFB1 TNF
23 hypoparathyroidism-retardation-dysmorphism syndrome 10.2 IL6 TGFB1 TNF
24 swine influenza 10.2 IL6 TNF
25 rheumatoid factor-negative juvenile idiopathic arthritis without anti-nuclear antibodies 10.2 IL6 TGFB1 TNF
26 alcoholic hepatitis 10.2 ACE IL6 TNF
27 hereditary night blindness 10.2 IL6 TGFB1 TNF
28 oppositional defiant disorder 10.2 ACE IL6 TNF
29 idiopathic panuveitis 10.2 ACE AGTR2 TGFB1
30 choroiditis 10.2 ACE IL6 TNF
31 labyrinthitis 10.2 IL6 TGFB1 TNF
32 immunoglobulin g deficiency 10.2 IL6 TGFB1 TNF
33 myiasis 10.2 IL6 TGFB1 TNF
34 drug dependence 10.2 IL6 TNF
35 biliary cirrhosis, primary, 2 10.2 ACE TGFB1 TNF
36 limb ischemia 10.2 ACE IL6 TNF
37 burns 10.2 IL6 TGFB1 TNF
38 diabetic encephalopathy 10.2 ACE IL6 TNF
39 baritosis 10.2 IL6 TGFB1 TNF
40 splenic marginal zone lymphoma 10.2 IL6 TGFB1 TNF
41 transient tic disorder 10.1 IL6 TNF
42 lichen disease 10.1 IL6 TGFB1 TNF
43 sclerosing adenosis of breast 10.1 IL6 TGFB1 TNF
44 pigmentation disease 10.1 IL6 TGFB1 TNF
45 bone angioendothelial sarcoma 10.1 IL6 TGFB1 TNF
46 down syndrome 10.1
47 peroneal neuropathy 10.1 ACE IL6 TNF
48 detrusor sphincter dyssynergia 10.1 IL6 TGFB1 TNF
49 nervous system cancer 10.1 IL6 TGFB1 TNF
50 body dysmorphic disorder 10.1 ACE 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 ACE ACE2 AGT AGTR1 AGTR2 IL6
2 cardiovascular system MP:0005385 10.11 ACE ACE2 AGT AGTR1 AGTR2 IL6
3 adipose tissue MP:0005375 10.08 TGFB1 TNF ACE AGT AGTR1 AGTR2
4 homeostasis/metabolism MP:0005376 10.06 AGTR1 AGTR2 IL6 SLC3A1 TGFB1 TNF
5 immune system MP:0005387 9.97 ACE ACE2 AGT AGTR1 IL6 SLC3A1
6 mortality/aging MP:0010768 9.96 ACE2 AGT AGTR1 AGTR2 IL6 SLC3A1
7 integument MP:0010771 9.8 ACE2 AGTR2 IL6 TBX18 TGFB1 TNF
8 muscle MP:0005369 9.7 ACE2 AGT AGTR2 IL6 TBX18 TGFB1
9 neoplasm MP:0002006 9.35 IL6 TGFB1 TNF ACE AGTR2
10 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)
id 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
4 Anti-Bacterial Agents
5 Anti-Infective Agents
6 Antibiotics, Antitubercular
7 Antitubercular Agents

Interventional clinical trials:


id Name Status NCT ID Phase
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 547)
id Title Authors Year
1
Posterior urethral valves (PUV) in pediatric practice - Promoting methods to understand how to diagnose and incise (PUV). ( 26921022 )
2016
2
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
3
Outcome of Antenatally Presenting Posterior Urethral Valves (PUV) in Children. ( 28031548 )
2016
4
DNA copy number variants: A potentially useful predictor of early onset renal failure in boys with posterior urethral valves. ( 27160979 )
2016
5
Posterior urethral valves: how the study of fetal micturition can help to reach the diagnosis? ( 27351949 )
2016
6
Posterior urethral valves and vesicoureteral reflux: can prenatal ultrasonography distinguish between these two conditions in male fetuses? ( 27374758 )
2016
7
Genetic Basis of Posterior Urethral Valves Inheritance. ( 27261184 )
2016
8
Posterior urethral valves: are neonatal imaging findings predictive of renal function during early childhood? ( 27401742 )
2016
9
Challenges in Predicting Renal Outcomes in Boys with Posterior Urethral Valves. ( 27311411 )
2016
10
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
11
Re: Combined Creatinine Velocity and Nadir Creatinine: A Reliable Predictor of Renal Outcome in Neonatally Diagnosed Posterior Urethral Valves. ( 26887751 )
2016
12
Rare copy number variants implicated in posterior urethral valves. ( 26663319 )
2016
13
Controlled Outcomes for Achievement of Urinary Continence among Boys Treated for Posterior Urethral Valves. ( 26964916 )
2016
14
Re: Nadir Creatinine in Posterior Urethral Valves: How High is Low Enough? ( 26853033 )
2016
15
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
16
Posterior urethral valves: Metabolic consequences in a cohort of patients. ( 26096436 )
2015
17
Antenatal Determinants of Long-Term Kidney Outcome in Boys with Posterior Urethral Valves. ( 26375276 )
2015
18
Posterior urethral valve with unilateral vesicoureteral reflux and patent urachus: A rare combination of urinary tract anomaliess. ( 25836363 )
2015
19
Posterior Urethral Valves in Children: Pattern of Presentation and Outcome of Initial Treatment in Ile-Ife, Nigeria. ( 26425072 )
2015
20
Concomitant anterior and posterior urethral valves in pediatrics: A single center experience over 12a88years and long-term follow up after endoscopic treatment. ( 25689730 )
2015
21
Presentation, management, and outcome of posterior urethral valves in a Nigerian tertiary hospital. ( 25659544 )
2015
22
Late diagnosis of posterior urethral valves. ( 26112258 )
2015
23
Concomitant Anterior and Posterior Urethral Valves: A Comprehensive Review of Literature. ( 25863834 )
2015
24
Nadir creatinine in posterior urethral valves: How high is low enough? ( 26292912 )
2015
25
Posterior urethral valves: Risk factors for progression to renal failure. ( 26705688 )
2015
26
Study of prognostic significance of antenatal ultrasonography and renin angiotensin system activation in predicting disease severity in posterior urethral valves. ( 25829668 )
2015
27
Split-cuff nipple technique of ureteral reimplantation in children with thick-walled bladders due to posterior urethral valves. ( 25444631 )
2015
28
Combined creatinine velocity and nadir creatinine: A reliable predictor of renal outcome in neonatally diagnosed posterior urethral valves. ( 26062970 )
2015
29
Laser Ablation of Posterior Urethral Valves by Fetal Cystoscopy. ( 25614247 )
2015
30
Urological fistulas after fetal cystoscopic laser ablation of posterior urethral valves - surgical technical aspects. ( 24817027 )
2014
31
Posterior urethral polyp with type I posterior urethral valves: a rare association in a neonate. ( 24767515 )
2014
32
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
33
Neonatal presentation of posterior urethral valves and tethered spinal cord. ( 25073498 )
2014
34
Re: long-term bladder function, fertility and sexual function in patients with posterior urethral valves treated in infancy. ( 24331507 )
2014
35
Epidemiology and demography of recently diagnosed cases of posterior urethral valves. ( 25198372 )
2014
36
Late presentation of posterior urethral valves. ( 24906275 )
2014
37
Urinary tract tuberculosis in a child with late presentation posterior urethral valves. ( 24529021 )
2014
38
Population-specific serum creatinine centiles in neonates with posterior urethral valves already predict long-term renal outcome. ( 25000449 )
2014
39
Anterior urethral valve associated with posterior urethral valves: report of 2 cases and review of the literature. ( 24958476 )
2014
40
Residual valve and stricture after posterior urethral valve ablation: how to evaluate? ( 22364895 )
2013
41
Long-term consequences of posterior urethral valves. ( 23871421 )
2013
42
Posterior Urethral Valves; A single Center Experience. ( 24800012 )
2013
43
Utilization of computer enhanced visual learning (CEVL) method improves endoscopic diagnosis of posterior urethral valves (PUV). ( 22981142 )
2013
44
Bilateral epididymitis in a child with undiagnosed posterior urethral valves. ( 23477755 )
2013
45
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
46
The impact of posterior urethral valves on adult quality of life. ( 22878058 )
2013
47
Newborn radiographic clues for undiagnosed posterior urethral valves. ( 23043682 )
2013
48
Spontaneous rupture of kidney due to posterior urethral valve-diagnostic difficulties. ( 23795264 )
2013
49
Early outcome following diathermy versus cold knife ablation of posterior urethral valves. ( 22417679 )
2013
50
Long-term bladder function, fertility and sexual function in patients with posterior urethral valves treated in infancy. ( 22154080 )
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 27)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
13.33 AGT AGTR1 AGTR2 IL6 TGFB1 TNF
2
Show member pathways
12.68 ACE AGT AGTR1 AGTR2 IL6 TGFB1
3
Show member pathways
12.65 AGTR1 IL6 TGFB1 TNF
4
Show member pathways
12.07 ACE ACE2 AGT
5 12.06 IL6 TGFB1 TNF
6 12 IL6 TGFB1 TNF
7
Show member pathways
11.98 AGT TGFB1 TNF
8
Show member pathways
11.91 ACE IL6 TGFB1 TNF
9 11.82 IL6 TGFB1 TNF
10 11.78 IL6 TGFB1 TNF
11
Show member pathways
11.74 IL6 TGFB1 TNF
12 11.68 IL6 TGFB1 TNF
13 11.64 IL6 TGFB1 TNF
14 11.58 AGT IL6 TGFB1 TNF
15 11.55 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 11.04 ACE ACE2
22
Show member pathways
11 ACE ACE2 AGT AGTR1 AGTR2 TGFB1
23 10.93 IL6 TNF
24 10.91 IL6 TGFB1 TNF
25 10.86 IL6 TNF
26 10.75 IL6 TGFB1
27 10.7 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 region GO:0005576 9.5 ACE ACE2 AGT AGTR2 IL6 TGFB1
2 extracellular space GO:0005615 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 65)
id Name GO ID Score Top Affiliating Genes
1 regulation of apoptotic process GO:0042981 9.94 AGT AGTR2 IL6 TGFB1
2 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.92 DSTYK IL6 TGFB1 TNF
3 aging GO:0007568 9.9 AGT IL6 TGFB1
4 negative regulation of cell growth GO:0030308 9.88 AGT AGTR2 TGFB1
5 kidney development GO:0001822 9.87 ACE AGT AGTR1
6 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.86 AGT IL6 TGFB1
7 positive regulation of protein kinase B signaling GO:0051897 9.85 IL6 TGFB1 TNF
8 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.84 IL6 TGFB1 TNF
9 regulation of blood pressure GO:0008217 9.83 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 9.79 IL6 TGFB1 TNF
12 positive regulation of protein tyrosine kinase activity GO:0061098 9.74 ACE AGT
13 cellular response to dexamethasone stimulus GO:0071549 9.74 AGTR2 IL6 TGFB1
14 positive regulation of cytokine secretion GO:0050715 9.73 AGTR2 TNF
15 protein import into nucleus, translocation GO:0000060 9.73 TGFB1 TNF
16 positive regulation of cytokine production GO:0001819 9.73 AGT TNF
17 negative regulation of mitotic cell cycle GO:0045930 9.73 TGFB1 TNF
18 positive regulation of vasodilation GO:0045909 9.73 AGT AGTR2
19 blood vessel remodeling GO:0001974 9.73 ACE AGT AGTR2
20 regulation of cell proliferation GO:0042127 9.73 ACE2 AGT AGTR1 IL6 TGFB1 TNF
21 positive regulation of extrinsic apoptotic signaling pathway GO:2001238 9.72 AGT AGTR2
22 positive regulation of protein complex assembly GO:0031334 9.72 TGFB1 TNF
23 vasodilation GO:0042311 9.72 AGT AGTR2
24 negative regulation of myoblast differentiation GO:0045662 9.72 TGFB1 TNF
25 positive regulation of reactive oxygen species metabolic process GO:2000379 9.72 ACE2 AGT AGTR1
26 nitric oxide mediated signal transduction GO:0007263 9.71 AGT AGTR2
27 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.71 AGT TNF
28 positive regulation of chemokine production GO:0032722 9.71 IL6 TNF
29 positive regulation of macrophage derived foam cell differentiation GO:0010744 9.71 AGT AGTR1
30 negative regulation of blood vessel endothelial cell migration GO:0043537 9.71 AGTR2 TGFB1
31 low-density lipoprotein particle remodeling GO:0034374 9.7 AGT AGTR1
32 positive regulation of renal sodium excretion GO:0035815 9.7 AGT AGTR2
33 cellular sodium ion homeostasis GO:0006883 9.69 AGT AGTR2
34 neutrophil mediated immunity GO:0002446 9.69 ACE IL6
35 positive regulation of superoxide anion generation GO:0032930 9.68 AGT TGFB1
36 negative regulation of lipid storage GO:0010888 9.68 IL6 TNF
37 positive regulation of cholesterol esterification GO:0010873 9.68 AGT AGTR1
38 cell growth involved in cardiac muscle cell development GO:0061049 9.67 AGT AGTR2
39 positive regulation of nitric oxide biosynthetic process GO:0045429 9.67 AGT AGTR2 IL6 TNF
40 angiotensin-activated signaling pathway GO:0038166 9.66 AGTR1 AGTR2
41 receptor-mediated virion attachment to host cell GO:0046813 9.65 ACE ACE2
42 epithelial cell proliferation involved in salivary gland morphogenesis GO:0060664 9.65 IL6 TNF
43 positive regulation of branching involved in ureteric bud morphogenesis GO:0090190 9.65 AGT AGTR2 TGFB1
44 positive regulation of NAD(P)H oxidase activity GO:0033864 9.64 AGT AGTR1
45 negative regulation of neurotrophin TRK receptor signaling pathway GO:0051387 9.63 AGT AGTR2
46 regulation of renal sodium excretion GO:0035813 9.63 AGT AGTR1
47 positive regulation of cellular protein metabolic process GO:0032270 9.63 AGT AGTR1 TGFB1
48 positive regulation of mononuclear cell migration GO:0071677 9.62 TGFB1 TNF
49 angiotensin catabolic process in blood GO:0002005 9.61 ACE ACE2
50 angiotensin maturation GO:0002003 9.61 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 Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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