MCID: UTR057
MIFTS: 29

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 57
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®:

57 (Updated 05-Apr-2021)
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, cervix and kidney, 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 79)
# Related Disease Score Top Affiliating Genes
1 double uterus-hemivagina-renal agenesis 11.6
2 renal hypodysplasia/aplasia 1 10.8
3 endometriosis 10.6
4 pelvic inflammatory disease 10.6
5 vaginal discharge 10.6
6 septate uterus 10.6
7 longitudinal vaginal septum 10.6
8 angiomyolipoma 10.5
9 pelvic organ prolapse 10.3
10 ureterocele 10.3
11 uterine anomalies 10.3
12 vesicoureteral reflux 1 10.3
13 urinary tract infection 10.3
14 hydronephrosis 10.3
15 disseminated intravascular coagulation 10.3
16 toxic shock syndrome 10.3
17 azoospermia 10.3
18 infertility 10.3
19 cervical incompetence 10.3
20 hemometra 10.3
21 multicystic dysplastic kidney 10.3
22 didelphys uterus 10.3
23 bicornuate uterus 10.3
24 complete septate uterus 10.3
25 transverse vaginal septum 10.3
26 renal dysplasia 10.3
27 kidney angiomyolipoma 10.3
28 cervical cancer 10.2
29 sarcoma 10.1
30 cystic kidney disease 10.1
31 spindle cell sarcoma 10.1
32 pyosalpinx 10.1
33 vasculitis 10.1
34 polyarteritis nodosa 10.1
35 werner syndrome 10.1
36 renal cell carcinoma, nonpapillary 10.0
37 endocarditis 10.0
38 adenocarcinoma 10.0
39 choriocarcinoma 10.0
40 appendicitis 10.0
41 vaginal atresia 10.0
42 suppressor of tumorigenicity 3 9.9
43 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 9.9
44 urolithiasis 9.9
45 unilateral multicystic dysplastic kidney 9.9
46 anorectal anomalies 9.9
47 colorectal cancer 9.9
48 split-hand/foot malformation 1 9.9
49 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 9.9
50 testicular germ cell tumor 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®:

57 (Updated 05-Apr-2021)
G U:
renal agenesis
partial vaginal septum
double uterus
two cervices
unilateral hematocolpos

Clinical features from OMIM®:

192050 (Updated 05-Apr-2021)

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, Cervix, Kidney, Ovary

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 213)
# Title Authors PMID Year
1
Uterine anomalies associated with unilateral renal agenesis. 57
934555 1976
2
Herlyn-Werner-Wunderlich syndrome: clinical considerations and management. 61
33664029 2021
3
Herlyn-Werner-Wunderlich (HWW) syndrome with kyphoscoliosis: a rare urogenital anomaly in a teenage girl. 61
33753378 2021
4
Herlyn-Werner-Wunderlich syndrome: Report of a delayed diagnosed case with video presentation of the operative technique of vaginal septum resection. 61
33723897 2021
5
Herlyn-Werner-Wunderlich syndrome with borderline serous cystadenoma of the ovary: case report and literature review. 61
33532015 2021
6
Hysteroscopic management of an oblique vaginal septum with diverticulum in Herlyn-Werner-Wunderlich syndrome. 61
33775924 2021
7
A case report of first hearing loss, then painful menarche: a young girl with Herlyn-Werner-Wunderlich syndrome (OHVIRA syndrome) and concomitant inner ear anomalies. 61
33650926 2021
8
Mayer-Rokitansky-Küster-Hauser syndrome with rare findings of inferior crossed-fused renal ectopia and Gartner's duct cyst: a video case report. 61
33069370 2021
9
Herlyn-Werner-Wunderlich syndrome in a multiparous female. 61
33614122 2021
10
Wunderlich Syndrome: Wonder What It Is. 61
33483188 2021
11
[Herlyn Werner Wunderlich syndrome: Müllerian malformation]. 61
33611261 2021
12
Herlyn-Werner-Wunderlich Syndrome; laparoscopic treatment of obstructing longitudinal vaginal septum in patients with hematocolpos - a different technique for virgin patients 61
31450882 2020
13
Pathognomonic Presentation of Herlyn-Werner-Wunderlich Syndrome with Significant Hematometrocolpos. 61
33326863 2020
14
Wunderlich syndrome and regression of angiomyolipoma. 61
32229794 2020
15
Successful pregnancy outcome in Herlyn-Werner-Wunderlich syndrome with pyocolpos: A case report and literature review. 61
33235762 2020
16
New Consideration of Herlyn-Werner-Wunderlich Syndrome Diagnosed by Ultrasound. 61
33236794 2020
17
In-office Hysteroscopic Treatment of Herlyn-Werner-Wunderlich Syndrome: A Case Series. 61
32320799 2020
18
Wunderlich syndrome with spontaneous renal hemorrhage into renal angiomyolipoma. 61
33776340 2020
19
Complex Abdominal Pain in an Adolescent Female with a Mullerian Duct Anomaly: A Case Report. 61
33264521 2020
20
A Renal Colic Mimic - Wunderlich Syndrome: A Case Report. 61
33274125 2020
21
Herlyn-Werner-Wunderlich Syndrome: Comparison of Two Cases. 61
33007989 2020
22
Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): Early Diagnosis and Management of Asymptomatic Herlyn-Werner-Wunderlich Syndrome. 61
31266634 2020
23
Herlyn-Werner-Wunderlich syndrome: diverse presentations and diagnosis on MRI. 61
32171579 2020
24
Wunderlich syndrome secondary to cyst rupture and concurrent anticoagulation. 61
32544052 2020
25
MRI image features and differential diagnoses of Herlyn-Werner-Wunderlich syndrome. 61
31657238 2020
26
An interesting case of Herlyn-Werner-Wunderlich syndrome. 61
32269958 2020
27
Herlyn-Werner-Wunderlich syndrome with cervical atresia complicated by ovarian endometrioma: A case report. 61
31814219 2020
28
Does spontaneous renal hemorrhage mandate close surveillance for impending renal cell carcinoma? A case report and literature review. 61
32634616 2020
29
Bilateral wunderlich syndrome secondary to synchronous bilateral testicular germ cell tumor. A case report. 61
31641608 2020
30
Primary vaginal clear cell adenocarcinoma accompanied by Herlyn-Werner-Wunderlich syndrome without prenatal diethylstilbestrol exposure: a case report. 61
33284870 2020
31
An unusual appearance of the post-pubertal Herlyn-Werner-Wunderlich syndrome with acute abdominal pain: A case report. 61
31911967 2019
32
Diagnosis of Wunderlich syndrome in a patient with flank pain. 61
31761752 2019
33
Herlyn-Werner-Wunderlich syndrome presenting with dysmenorrhea: a case report. 61
31666132 2019
34
Herlyn-Werner-Wunderlich syndrome: A rare cause of abdominal pain and dyspareunia. 61
31462954 2019
35
Herlyn Werner Wunderlich Syndrome with Hematocolpos Symptom. 61
31777633 2019
36
Minimally Invasive Endoscopic Approach for Management of OHVIRA Syndrome. 61
31391743 2019
37
An Unusual Cause of Recurrent Urinary Retention in an Adolescent Female. 61
31523565 2019
38
Life-threatening Wunderlich Syndrome in a Patient with Solitary Kidney Successfully Managed by Nephron-sparing Approach. 61
31423068 2019
39
[Acute urine retention due to herlyn-Werner-Wunderlinch syndrome.] 61
31223131 2019
40
Wunderlich syndrome, spontaneous ruptured renal angiomyolipoma and tuberous sclerosis. 61
30629244 2019
41
Herlyn-Werner-Wunderlich Syndrome: Report of a Prenatally Recognised Case and Review of the Literature. 61
30597168 2019
42
Prenatal Diagnosis of Hydro(metro)colpos: A Series of 20 Cases. 61
29478043 2019
43
Spontaneous perirenal hemorrhage (Wunderlich syndrome): An analysis of 28 cases. 61
29779678 2019
44
Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature. 61
31586891 2019
45
Case Report: Partial nephrectomy in primary renal sarcoma presenting as Wunderlich syndrome; a rare tumour with rare presentation managed atypically. 61
31354947 2019
46
Successful preterm pregnancy in a rare variation of Herlyn-Werner-Wunderlich syndrome: a case report. 61
30558561 2018
47
Spontaneous Rupture of Renal Cell Carcinoma in Pregnancy, Surgical Management With Fetal Preservation:A Case Report. 61
29682721 2018
48
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
49
Late presentation, MR imaging features and surgical treatment of Herlyn-Werner-Wunderlich syndrome (classification 2.2); a case report. 61
30285830 2018
50
A case report on Herlyn-Werner-Wunderlich syndrome with spontaneous abortion. 61
30200079 2018

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...

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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