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MCID: UTR057
MIFTS: 27

Uterus Bicornis Bicollis with Partial Vaginal Septum and Unilateral Hematocolpos with Ipsilateral Renal Agenesis

Categories: Blood diseases, Fetal diseases, Nephrological diseases, Rare diseases, Reproductive diseases
Tissues Related diseases Publications Symptoms & Phenotypes
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Aliases & Classifications for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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MalaCards integrated aliases for Uterus Bicornis Bicollis with Partial Vaginal Septum and Unilateral Hematocolpos with Ipsilateral Renal Agenesis:

Name: Uterus Bicornis Bicollis with Partial Vaginal Septum and Unilateral Hematocolpos with Ipsilateral Renal Agenesis 57
Obstructed Hemivagina and Ipsilateral Renal Anomaly 59
Double Uterus-Hemivagina-Renal Agenesis Syndrome 59
Double Uterus and Obstructed Hemivagina Syndrome 59
Herlyn-Werner Syndrome 59
Wunderlich Syndrome 59
Ohvira Syndrome 59

Characteristics:

Orphanet epidemiological data:

59
double uterus-hemivagina-renal agenesis syndrome
Prevalence: <1/1000000 (Worldwide); Age of onset: Infancy,Neonatal;

OMIM:

57
Inheritance:
autosomal dominant


HPO:

32
uterus bicornis bicollis with partial vaginal septum and unilateral hematocolpos with ipsilateral renal agenesis:
Inheritance autosomal dominant inheritance


Classifications:

MalaCards categories:
Global: Fetal diseases Rare diseases
Anatomical: Nephrological diseases Reproductive diseases Blood diseases
See all MalaCards categories (disease lists)
Orphanet: 59  
Rare renal diseases
Rare gynaecological and obstetric diseases
Developmental anomalies during embryogenesis


External Ids:

OMIM 57 192050
UMLS via Orphanet 73 C1860549
Orphanet 59 ORPHA3411
MedGen 42 C1860549
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Summaries for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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MalaCards based summary : Uterus Bicornis Bicollis with Partial Vaginal Septum and Unilateral Hematocolpos with Ipsilateral Renal Agenesis, also known as obstructed hemivagina and ipsilateral renal anomaly, is related to renal hypodysplasia/aplasia 1 and angiomyolipoma. Affiliated tissues include uterus, kidney and cervix, and related phenotypes are fever and premature birth

More information from OMIM: 192050
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Related Diseases for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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Diseases related to Uterus Bicornis Bicollis with Partial Vaginal Septum and Unilateral Hematocolpos with Ipsilateral Renal Agenesis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 64)
# Related Disease Score Top Affiliating Genes
1 renal hypodysplasia/aplasia 1 10.5
2 angiomyolipoma 10.5
3 pelvic inflammatory disease 10.5
4 renal cell carcinoma, nonpapillary 10.3
5 kidney angiomyolipoma 10.3
6 werner syndrome 10.3
7 hydronephrosis 10.3
8 azoospermia 10.3
9 cervical incompetence 10.3
10 multicystic dysplastic kidney 10.3
11 complete septate uterus 10.3
12 cystic kidney disease 10.2
13 pyosalpinx 10.2
14 polyarteritis nodosa 10.2
15 uterine anomalies 10.2
16 cervical cancer 10.2
17 endometriosis 10.2
18 infertility 10.2
19 didelphys uterus 10.2
20 septate uterus 10.2
21 renal dysplasia 10.2
22 endocarditis 10.1
23 sarcoma 10.1
24 spindle cell sarcoma 10.1
25 appendicitis 10.1
26 vasculitis 10.1
27 vaginal atresia 10.1
28 pelvic organ prolapse 10.0
29 suppressor of tumorigenicity 3 10.0
30 ureterocele 10.0
31 vesicoureteral reflux 1 10.0
32 disseminated intravascular coagulation 10.0
33 acute cystitis 10.0
34 toxic shock syndrome 10.0
35 vaginal discharge 10.0
36 hemometra 10.0
37 transverse vaginal septum 10.0
38 longitudinal vaginal septum 10.0
39 anorectal anomalies 9.9
40 colorectal cancer 9.9
41 split-hand/foot malformation 1 9.9
42 thrombophilia due to thrombin defect 9.9
43 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 9.9
44 anorexia nervosa 9.9
45 ewing sarcoma 9.9
46 infective endocarditis 9.9
47 scoliosis 9.9
48 pre-eclampsia 9.9
49 chronic pyelonephritis 9.9
50 arteriovenous malformation 9.9

Graphical network of the top 20 diseases related to Uterus Bicornis Bicollis with Partial Vaginal Septum and Unilateral Hematocolpos with Ipsilateral Renal Agenesis:



Diseases related to Uterus Bicornis Bicollis with Partial Vaginal Septum and Unilateral Hematocolpos with Ipsilateral Renal Agenesis
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Symptoms & Phenotypes for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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Human phenotypes related to Uterus Bicornis Bicollis with Partial Vaginal Septum and Unilateral Hematocolpos with Ipsilateral Renal Agenesis:

59 32 (show all 13)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 fever 59 32 hallmark (90%) Very frequent (99-80%) HP:0001945
2 premature birth 59 32 hallmark (90%) Very frequent (99-80%) HP:0001622
3 breech presentation 59 32 hallmark (90%) Very frequent (99-80%) HP:0001623
4 dyspareunia 59 32 hallmark (90%) Very frequent (99-80%) HP:0030016
5 metrorrhagia 59 32 hallmark (90%) Very frequent (99-80%) HP:0100608
6 dysmenorrhea 59 32 hallmark (90%) Very frequent (99-80%) HP:0100607
7 renal agenesis 59 32 hallmark (90%) Very frequent (99-80%) HP:0000104
8 chronic pain 59 32 hallmark (90%) Very frequent (99-80%) HP:0012532
9 hydrocolpos 59 32 hallmark (90%) Very frequent (99-80%) HP:0030711
10 uterus didelphys 59 32 hallmark (90%) Very frequent (99-80%) HP:0003762
11 partial vaginal septum 59 32 hallmark (90%) Very frequent (99-80%) HP:0008670
12 abnormality of the uterine cervix 59 32 hallmark (90%) Very frequent (99-80%) HP:0012888
13 hematocolpos 32 HP:0031923

Symptoms via clinical synopsis from OMIM:

57
G U:
renal agenesis
partial vaginal septum
double uterus
two cervices
unilateral hematocolpos

Clinical features from OMIM:

192050
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Drugs & Therapeutics for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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Search Clinical Trials , NIH Clinical Center for Uterus Bicornis Bicollis with Partial Vaginal Septum and Unilateral Hematocolpos with Ipsilateral Renal Agenesis

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Genetic Tests for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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Anatomical Context for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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MalaCards organs/tissues related to Uterus Bicornis Bicollis with Partial Vaginal Septum and Unilateral Hematocolpos with Ipsilateral Renal Agenesis:

41
Uterus, Kidney, Cervix
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Publications for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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Articles related to Uterus Bicornis Bicollis with Partial Vaginal Septum and Unilateral Hematocolpos with Ipsilateral Renal Agenesis:

(show top 50) (show all 178)
# Title Authors PMID Year
1
Uterine anomalies associated with unilateral renal agenesis. 8
934555 1976
2
Minimally Invasive Endoscopic Approach for Management of OHVIRA Syndrome. 38
31391743 2019
3
Life-threatening Wunderlich Syndrome in a Patient with Solitary Kidney Successfully Managed by Nephron-sparing Approach. 38
31423068 2019
4
[Acute urine retention due to herlyn-Werner-Wunderlinch syndrome.] 38
31223131 2019
5
Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): Early Diagnosis and Management of Asymptomatic Herlyn-Werner-Wunderlich Syndrome. 38
31266634 2019
6
Wunderlich syndrome, spontaneous ruptured renal angiomyolipoma and tuberous sclerosis. 38
30629244 2019
7
Herlyn-Werner-Wunderlich Syndrome: Report of a Prenatally Recognised Case and Review of the Literature. 38
30597168 2019
8
Spontaneous perirenal hemorrhage (Wunderlich syndrome): An analysis of 28 cases. 38
29779678 2019
9
Case Report: Partial nephrectomy in primary renal sarcoma presenting as Wunderlich syndrome; a rare tumour with rare presentation managed atypically. 38
31354947 2019
10
Prenatal Diagnosis of Hydro(metro)colpos: A Series of 20 Cases. 38
29478043 2019
11
Successful preterm pregnancy in a rare variation of Herlyn-Werner-Wunderlich syndrome: a case report. 38
30558561 2018
12
Spontaneous Rupture of Renal Cell Carcinoma in Pregnancy, Surgical Management With Fetal Preservation:A Case Report. 38
29682721 2018
13
An unusual case of Herlyn-Werner-Wunderlich syndrome with a complete septate uterus and complicated urinary tract with rare ectopic ureteral opening. 38
29959772 2018
14
Late presentation, MR imaging features and surgical treatment of Herlyn-Werner-Wunderlich syndrome (classification 2.2); a case report. 38
30285830 2018
15
A case report on Herlyn-Werner-Wunderlich syndrome with spontaneous abortion. 38
30200079 2018
16
Wunderlich syndrome. 38
30214791 2018
17
Spontaneous perirenal hemorrhage in hemodialysis patient treated with selective embolization: A case series and review of the literature. 38
29152881 2018
18
The Herlyn-Werner-Wunderlich triad (OHVIRA syndrome) with good pregnancy outcome - two cases and literature review. 38
30845309 2018
19
[Chinese expert consensus on the diagnosis and treatment of Herlyn-Werner-Wunderlich syndrome, Mayer-Rokitansky-Küster-Hauser syndrome and vaginal atresia]. 38
29374884 2018
20
A rare case of pelvic pain caused by Herlyn-Werner-Wunderlich Syndrome in an adult: A case report. 38
29980030 2018
21
Clinical and radiological findings for early diagnosis of Herlyn-Werner-Wunderlich syndrome in pediatric age: experience of a single center. 38
28562147 2018
22
A Variant of Herlyn-Werner-Wunderlich Syndrome Presenting With Acute Abdomen: A Case Report and Review of Literature. 38
29761137 2018
23
Herlyn-Werner-Wunderlich syndrome - a rare genitourinary anomaly in females: a series of four cases. 38
30627251 2018
24
Point-of-care Ultrasound Aiding in the Diagnosis of Herlyn-Werner-Wunderlich Syndrome. 38
29849371 2017
25
Uncommon presentations of an uncommon entity: OHVIRA syndrome with hematosalpinx and pyocolpos. 38
29479403 2017
26
Herlyn-Werner-Wunderlich syndrome: An unusual presentation with pyocolpos. 38
28791270 2017
27
Wunderlich Syndrome due to Ruptured Pseudoaneurysm of Angiomyolipoma in First Trimester: A Rare Case Report. 38
28764248 2017
28
Page Kidney in Wunderlich Syndrome Causing Acute Renal Failure and Urosepsis: Successful Timely Minimally Invasive Management of a Devastating Clinical Entity. 38
28764242 2017
29
Polyarteritis nodosa presenting as bilateral Wunderlich syndrome: Rare cause of flank pain in a young woman. 38
27865573 2017
30
Wunderlich Syndrome. 38
28159184 2017
31
Herlyn-Werner-Wunderlich Syndrome with Ureteric Remnant Abscess Managed Laparoscopically: A Case Report. 38
28439388 2017
32
Herlyn-Werner-Wunderlich Syndrome: Sonographic and Magnetic Resonance (MR) Imaging Findings of This Rare Urogenital Anomaly. 38
28469738 2017
33
Gossypiboma Following Emergency Nephrectomy for Wunderlich Syndrome. 38
27720974 2017
34
Wunderlich's syndrome as a manifestation of polyarteritis nodosa. 38
28137905 2017
35
True management of Obstructed Hemi-vagina and Ipsilateral Renal Anomaly syndrome. 38
28913123 2016
36
Pyometra and Pregnancy with Herlyn-Werner-Wunderlich Syndrome. 38
28002847 2016
37
Urinary Incontinence in Puberty: A Rare Clinical Presentation of the Herlyn-Werner-Wunderlich Syndrome. 38
27519169 2016
38
Case Report: A Rare Cause of Complicated Urinary Tract Infection in a Woman with Herlyn-Werner-Wunderlich Syndrome. 38
28203451 2016
39
Laparoscopic Hemi-hysterectomy and Trachelectomy in a Case of Herlyn-Werner-Wunderlich Syndrome. 38
27466877 2016
40
Use of imaging in diagnosis of Herlyn-Werner-Wunderlich syndrome, a case report. 38
27616278 2016
41
Renal Angiomyolipoma in Pregnancy: Surgical Management with Fetal Preservation - Approach in a Developing Setting. 38
28176962 2016
42
Pelvic cystic mass and ipsilateral renal agenesis detected by ultrasound in a young girl: early onset of Herlyn-Werner-Wunderlich Syndrome. 38
27622422 2016
43
Embryogenesis of vagina and embryopathogenesis of Herlyn-Werner-Wunderlich syndrome. 38
27857474 2016
44
Late presentation of a variant of Herlyn-Werner-Wunderlich syndrome. 38
26861887 2016
45
Herlyn-Werner-Wunderlich Syndrome. 38
27046969 2016
46
Herlyn Werner Wunderlich Syndrome with Hematocolpos: An Unusual Case Report of Full Diagnostic Approach and Treatment. 38
27123211 2016
47
An eventful journey from menarche to successful motherhood. 38
27382240 2016
48
The Herlyn-Werner-Wunderlich Syndrome - A Case Report with Radiological Review. 38
28058067 2016
49
Complete septate uterus with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) in a young woman-a rare variant of Herlyn-Werner-Wunderlich syndrome. 38
30363628 2016
50
Herlyn-Werner-Wunderlich Syndrome: A Rare Cause of Pelvic Pain and High CA 19-9 Levels in an Adolescent Girl. 38
26816677 2016
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Variations for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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Expression for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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Search GEO for disease gene expression data for Uterus Bicornis Bicollis with Partial Vaginal Septum and Unilateral Hematocolpos with Ipsilateral Renal Agenesis.
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Pathways for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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GO Terms for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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Sources for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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