MCID: FMR007
MIFTS: 14

Femur Bifid with Monodactylous Ectrodactyly

Categories: Rare diseases

Aliases & Classifications for Femur Bifid with Monodactylous Ectrodactyly

MalaCards integrated aliases for Femur Bifid with Monodactylous Ectrodactyly:

Name: Femur Bifid with Monodactylous Ectrodactyly 54 74
Gollop-Wolfgang Complex 54
Gwc 54

Classifications:



External Ids:

UMLS 74 C1856789

Summaries for Femur Bifid with Monodactylous Ectrodactyly

NIH Rare Diseases : 54 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.Orpha Number: 1986Disease definitionGollop-Wolfgang complex is a very rare malformation characterized by ectrodactyly of the hand and ipsilateral bifurcation of the femur.EpidemiologyApproximately 200 cases have been reported worldwide.Clinical descriptionCongenital aplasia/hypoplasia of the tibia, accompanied by pre-axial oligodactyly or monodactyly of the feet, may also be present. In most cases, the bifurcation of the distal femur is unilateral. Around 80% of patients present with major aplasia without metacarpophalangeal bones, which is sometimes associated with an abnormality of the ulna. Patients are often small.EtiologyThe etiology remains unknown.Diagnostic methodsDiagnosis is based on clinical and radiological evidence.Differential diagnosisHypoplastic tibia-polydactylysyndrome (see this term) is the main differential diagnosis.Antenatal diagnosisPrenatal diagnosis is possible by ultrasound.Genetic counselingAutosomal dominant and autosomal recessive modes of transmission have been suggested.Management and treatmentPatients should be offered orthopedic and reconstructive surgery (involving prosthetics) and regular monitoring.PrognosisLife expectancy is not reduced but, in the absence of treatment, the functional prognosis is poor.Visit the Orphanet disease page for more resources.

MalaCards based summary : Femur Bifid with Monodactylous Ectrodactyly, also known as gollop-wolfgang complex, is related to femur, unilateral bifid, with monodactylous ectrodactyly and split-hand/foot malformation 1. Affiliated tissues include bone and heart.

Related Diseases for Femur Bifid with Monodactylous Ectrodactyly

Diseases related to Femur Bifid with Monodactylous Ectrodactyly via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 femur, unilateral bifid, with monodactylous ectrodactyly 11.7
2 split-hand/foot malformation 1 10.6
3 isolated split hand-split foot malformation 10.6
4 cleft palate, isolated 10.4
5 tibial hemimelia 10.4
6 split hand-foot malformation 10.4
7 heart disease 10.4
8 cleft lip 10.4
9 tracheoesophageal fistula 10.4

Graphical network of the top 20 diseases related to Femur Bifid with Monodactylous Ectrodactyly:



Diseases related to Femur Bifid with Monodactylous Ectrodactyly

Symptoms & Phenotypes for Femur Bifid with Monodactylous Ectrodactyly

Drugs & Therapeutics for Femur Bifid with Monodactylous Ectrodactyly

Search Clinical Trials , NIH Clinical Center for Femur Bifid with Monodactylous Ectrodactyly

Genetic Tests for Femur Bifid with Monodactylous Ectrodactyly

Anatomical Context for Femur Bifid with Monodactylous Ectrodactyly

MalaCards organs/tissues related to Femur Bifid with Monodactylous Ectrodactyly:

42
Bone, Heart

Publications for Femur Bifid with Monodactylous Ectrodactyly

Articles related to Femur Bifid with Monodactylous Ectrodactyly:

(show all 16)
# Title Authors Year
1
Gollop-Wolfgang Complex. ( 28083505 )
2017
2
Gollop Wolfgang Complex: Can it be a Teratogenic Condition? ( 26922797 )
2016
3
Prenatal diagnosis of Gollop-Wolfgang complex. ( 25302853 )
2015
4
Japanese founder duplications/triplications involving BHLHA9 are associated with split-hand/foot malformation with or without long bone deficiency and Gollop-Wolfgang complex. ( 25351291 )
2014
5
Case Report: Gollop-Wolfgang Complex in a 5 month old baby. ( 26064472 )
2014
6
Limb salvage treatment for Gollop-Wolfgang complex (femoral bifurcation, complete tibial hemimelia, and hand ectrodactyly). ( 23660549 )
2013
7
Gollop-Wolfgang complex: an alternative to amputation. ( 23821979 )
2013
8
Prenatal diagnosis of Gollop-Wolfgang complex (tibial agenesis and femoral bifurcation). ( 19180625 )
2009
9
Prenatal diagnosis of Gollop-Wolfgang Complex. ( 19353531 )
2009
10
Tibial agenesis and Gollop-Wolfgang complex. ( 17853458 )
2007
11
A 5-year follow-up study after knee disarticulation in two cases of Gollop-Wolfgang complex. ( 17909338 )
2007
12
Agenesis of tibia with bifid femur, congenital heart disease, and cleft lip with cleft palate or tracheoesophageal fistula: possible variants of Gollop-Wolfgang complex. ( 15732065 )
2005
13
Agenesis of tibia with ectrodactyly/Gollop-Wolfgang complex associated with congenital heart malformations and additional skeletal abnormalities. ( 10340652 )
1999
14
Bilateral involvement of hands and legs in the Gollop-Wolfgang complex. ( 9880223 )
1998
15
Tibial aplasia-ectrodactyly as variant expression of the Gollop-Wolfgang complex: report of a Brazilian family. ( 3688036 )
1987
16
Gollop-Wolfgang complex in a 3-month-old girl. ( 3146290 )
1986

Variations for Femur Bifid with Monodactylous Ectrodactyly

Expression for Femur Bifid with Monodactylous Ectrodactyly

Search GEO for disease gene expression data for Femur Bifid with Monodactylous Ectrodactyly.

Pathways for Femur Bifid with Monodactylous Ectrodactyly

GO Terms for Femur Bifid with Monodactylous Ectrodactyly

Sources for Femur Bifid with Monodactylous Ectrodactyly

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70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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