MCID: UTR057
MIFTS: 28

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

Aliases & Classifications for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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 56
Obstructed Hemivagina and Ipsilateral Renal Anomaly 58
Double Uterus and Obstructed Hemivagina Syndrome 58
Double Uterus-Hemivagina-Renal Agenesis Syndrome 58
Herlyn-Werner Syndrome 58
Wunderlich Syndrome 58
Ohvira Syndrome 58

Characteristics:

Orphanet epidemiological data:

58
double uterus-hemivagina-renal agenesis syndrome
Prevalence: <1/1000000 (Worldwide); Age of onset: Infancy,Neonatal;

OMIM:

56
Inheritance:
autosomal dominant


HPO:

31
uterus bicornis bicollis with partial vaginal septum and unilateral hematocolpos with ipsilateral renal agenesis:
Inheritance autosomal dominant inheritance


Classifications:

Orphanet: 58  
Rare renal diseases
Rare gynaecological and obstetric diseases
Developmental anomalies during embryogenesis


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

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 double uterus-hemivagina-renal agenesis and renal hypodysplasia/aplasia 1. Affiliated tissues include uterus, kidney and cervix, and related phenotypes are fever and premature birth

More information from OMIM: 192050

Related Diseases for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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 75)
# Related Disease Score Top Affiliating Genes
1 double uterus-hemivagina-renal agenesis 11.7
2 renal hypodysplasia/aplasia 1 10.8
3 endometriosis 10.6
4 angiomyolipoma 10.5
5 pelvic inflammatory disease 10.5
6 vaginal discharge 10.5
7 infertility 10.5
8 septate uterus 10.5
9 longitudinal vaginal septum 10.5
10 kidney angiomyolipoma 10.3
11 renal cell carcinoma, nonpapillary 10.3
12 werner syndrome 10.3
13 pelvic organ prolapse 10.3
14 ureterocele 10.3
15 uterine anomalies 10.3
16 vesicoureteral reflux 1 10.3
17 hydronephrosis 10.3
18 disseminated intravascular coagulation 10.3
19 acute cystitis 10.3
20 toxic shock syndrome 10.3
21 azoospermia 10.3
22 cervical incompetence 10.3
23 hemometra 10.3
24 multicystic dysplastic kidney 10.3
25 didelphys uterus 10.3
26 bicornuate uterus 10.3
27 complete septate uterus 10.3
28 transverse vaginal septum 10.3
29 renal dysplasia 10.3
30 cystic kidney disease 10.2
31 pyosalpinx 10.2
32 polyarteritis nodosa 10.2
33 cervical cancer 10.2
34 endocarditis 10.1
35 sarcoma 10.1
36 choriocarcinoma 10.1
37 spindle cell sarcoma 10.1
38 appendicitis 10.1
39 vasculitis 10.1
40 vaginal atresia 10.1
41 suppressor of tumorigenicity 3 10.0
42 unilateral multicystic dysplastic kidney 10.0
43 anorectal anomalies 9.9
44 colorectal cancer 9.9
45 split-hand/foot malformation 1 9.9
46 thrombophilia due to thrombin defect 9.9
47 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 9.9
48 testicular germ cell tumor 9.9
49 adenomyosis 9.9
50 anorexia nervosa 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

Symptoms & Phenotypes for Uterus Bicornis Bicollis with Partial Vaginal Septum and...

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

58 31 (show all 13)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 fever 58 31 hallmark (90%) Very frequent (99-80%) HP:0001945
2 premature birth 58 31 hallmark (90%) Very frequent (99-80%) HP:0001622
3 dysmenorrhea 58 31 hallmark (90%) Very frequent (99-80%) HP:0100607
4 dyspareunia 58 31 hallmark (90%) Very frequent (99-80%) HP:0030016
5 renal agenesis 58 31 hallmark (90%) Very frequent (99-80%) HP:0000104
6 uterus didelphys 58 31 hallmark (90%) Very frequent (99-80%) HP:0003762
7 metrorrhagia 58 31 hallmark (90%) Very frequent (99-80%) HP:0100608
8 breech presentation 58 31 hallmark (90%) Very frequent (99-80%) HP:0001623
9 partial vaginal septum 58 31 hallmark (90%) Very frequent (99-80%) HP:0008670
10 chronic pain 58 31 hallmark (90%) Very frequent (99-80%) HP:0012532
11 abnormality of the uterine cervix 58 31 hallmark (90%) Very frequent (99-80%) HP:0012888
12 hydrocolpos 58 31 hallmark (90%) Very frequent (99-80%) HP:0030711
13 hematocolpos 31 HP:0031923

Symptoms via clinical synopsis from OMIM:

56
G U:
renal agenesis
partial vaginal septum
double uterus
two cervices
unilateral hematocolpos

Clinical features from OMIM:

192050

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

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

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

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

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

40
Uterus, Kidney, Cervix

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

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

(show top 50) (show all 194)
# Title Authors PMID Year
1
Uterine anomalies associated with unilateral renal agenesis. 56
934555 1976
2
Herlyn-Werner-Wunderlich syndrome: diverse presentations and diagnosis on MRI. 61
32171579 2020
3
Wunderlich syndrome secondary to cyst rupture and concurrent anticoagulation. 61
32544052 2020
4
MRI image features and differential diagnoses of Herlyn-Werner-Wunderlich syndrome. 61
31657238 2020
5
In-office Hysteroscopic Treatment of Herlyn-Werner-Wunderlich Syndrome: A Case Series. 61
32320799 2020
6
Wunderlich syndrome and regression of angiomyolipoma. 61
32229794 2020
7
Herlyn-Werner-Wunderlich syndrome with cervical atresia complicated by ovarian endometrioma: A case report. 61
31814219 2020
8
Bilateral wunderlich syndrome secondary to synchronous bilateral testicular germ cell tumor. A case report. 61
31641608 2020
9
An unusual appearance of the post-pubertal Herlyn-Werner-Wunderlich syndrome with acute abdominal pain: A case report. 61
31911967 2019
10
Diagnosis of Wunderlich syndrome in a patient with flank pain. 61
31761752 2019
11
Herlyn-Werner-Wunderlich syndrome presenting with dysmenorrhea: a case report. 61
31666132 2019
12
Herlyn-Werner-Wunderlich syndrome: A rare cause of abdominal pain and dyspareunia. 61
31462954 2019
13
Minimally Invasive Endoscopic Approach for Management of OHVIRA Syndrome. 61
31391743 2019
14
Herlyn Werner Wunderlich Syndrome with Hematocolpos Symptom. 61
31777633 2019
15
Herlyn-Werner-Wunderlich syndrome; laparoscopic treatment of obstructing longitudinal vaginal septum in patients with hematocolpos - a different technique for virgin patients 61
31450882 2019
16
An Unusual Cause of Recurrent Urinary Retention in an Adolescent Female. 61
31523565 2019
17
Life-threatening Wunderlich Syndrome in a Patient with Solitary Kidney Successfully Managed by Nephron-sparing Approach. 61
31423068 2019
18
[Acute urine retention due to herlyn-Werner-Wunderlinch syndrome.] 61
31223131 2019
19
Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): Early Diagnosis and Management of Asymptomatic Herlyn-Werner-Wunderlich Syndrome. 61
31266634 2019
20
An interesting case of Herlyn-Werner-Wunderlich syndrome. 61
32269958 2019
21
Wunderlich syndrome, spontaneous ruptured renal angiomyolipoma and tuberous sclerosis. 61
30629244 2019
22
Herlyn-Werner-Wunderlich Syndrome: Report of a Prenatally Recognised Case and Review of the Literature. 61
30597168 2019
23
Spontaneous perirenal hemorrhage (Wunderlich syndrome): An analysis of 28 cases. 61
29779678 2019
24
Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature. 61
31586891 2019
25
Case Report: Partial nephrectomy in primary renal sarcoma presenting as Wunderlich syndrome; a rare tumour with rare presentation managed atypically. 61
31354947 2019
26
Prenatal Diagnosis of Hydro(metro)colpos: A Series of 20 Cases. 61
29478043 2019
27
Successful preterm pregnancy in a rare variation of Herlyn-Werner-Wunderlich syndrome: a case report. 61
30558561 2018
28
Spontaneous Rupture of Renal Cell Carcinoma in Pregnancy, Surgical Management With Fetal Preservation:A Case Report. 61
29682721 2018
29
An unusual case of Herlyn-Werner-Wunderlich syndrome with a complete septate uterus and complicated urinary tract with rare ectopic ureteral opening. 61
29959772 2018
30
Late presentation, MR imaging features and surgical treatment of Herlyn-Werner-Wunderlich syndrome (classification 2.2); a case report. 61
30285830 2018
31
A case report on Herlyn-Werner-Wunderlich syndrome with spontaneous abortion. 61
30200079 2018
32
Wunderlich syndrome. 61
30214791 2018
33
Spontaneous perirenal hemorrhage in hemodialysis patient treated with selective embolization: A case series and review of the literature. 61
29152881 2018
34
The Herlyn-Werner-Wunderlich triad (OHVIRA syndrome) with good pregnancy outcome - two cases and literature review. 61
30845309 2018
35
Clinical and radiological findings for early diagnosis of Herlyn-Werner-Wunderlich syndrome in pediatric age: experience of a single center. 61
28562147 2018
36
A Variant of Herlyn-Werner-Wunderlich Syndrome Presenting With Acute Abdomen: A Case Report and Review of Literature. 61
29761137 2018
37
[Chinese expert consensus on the diagnosis and treatment of Herlyn-Werner-Wunderlich syndrome, Mayer-Rokitansky-Küster-Hauser syndrome and vaginal atresia]. 61
29374884 2018
38
A rare case of pelvic pain caused by Herlyn-Werner-Wunderlich Syndrome in an adult: A case report. 61
29980030 2018
39
Herlyn-Werner-Wunderlich syndrome - a rare genitourinary anomaly in females: a series of four cases. 61
30627251 2018
40
Point-of-care Ultrasound Aiding in the Diagnosis of Herlyn-Werner-Wunderlich Syndrome. 61
29849371 2017
41
Uncommon presentations of an uncommon entity: OHVIRA syndrome with hematosalpinx and pyocolpos. 61
29479403 2017
42
Herlyn-Werner-Wunderlich syndrome: An unusual presentation with pyocolpos. 61
28791270 2017
43
Wunderlich Syndrome due to Ruptured Pseudoaneurysm of Angiomyolipoma in First Trimester: A Rare Case Report. 61
28764248 2017
44
Page Kidney in Wunderlich Syndrome Causing Acute Renal Failure and Urosepsis: Successful Timely Minimally Invasive Management of a Devastating Clinical Entity. 61
28764242 2017
45
Polyarteritis nodosa presenting as bilateral Wunderlich syndrome: Rare cause of flank pain in a young woman. 61
27865573 2017
46
Wunderlich Syndrome. 61
28159184 2017
47
Herlyn-Werner-Wunderlich Syndrome with Ureteric Remnant Abscess Managed Laparoscopically: A Case Report. 61
28439388 2017
48
Herlyn-Werner-Wunderlich Syndrome: Sonographic and Magnetic Resonance (MR) Imaging Findings of This Rare Urogenital Anomaly. 61
28469738 2017
49
Gossypiboma Following Emergency Nephrectomy for Wunderlich Syndrome. 61
27720974 2017
50
Wunderlich's syndrome as a manifestation of polyarteritis nodosa. 61
28137905 2017

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

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

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

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

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

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

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32 ICD10
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72 UMLS via Orphanet
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