Postaxial polydactyly type B

General Information (adopted from Orphanet):

Synonyms, Signs: PAPB, INCLUDED
POLYDACTYLY, POSTAXIAL POSTAXIAL POLYDACTYLY, TYPE B, INCLUDED
POSTAXIAL POLYDACTYLY, TYPE A
PAPA
PAPA1
Number of Symptoms 3
OrphanetNr: 93335
OMIM Id: 174200
ICD-10: Q69.0
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance:
Age of onset:

Disease classification (adopted from Orphanet):

Parent Diseases: Postaxial polydactyly of fingers
 -Postaxial polydactyly of fingers

Symptom Information: Sort by abundance 

1
(HPO:0100259) Postaxial polydactyly 85 / 7739
2
(OMIM) Well formed articulating extra digit in Type A 1 / 7739
3
(OMIM) Pedunculated postminimi in Type B 1 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Description: (OMIM) Polydactyly refers to the occurrence of supernumerary digits and is the most frequent of congenital hand and foot deformities. Based on the location of the extra digits, polydactyly can be classified into preaxial, involving the thumb or great ...
Clinical Description OMIM Studies of various pedigrees with postaxial polydactyly indicate that there are 2 phenotypically different forms. In postaxial polydactyly type A, the extra digit is well formed and articulates with the fifth or an extra metacarpal. This type is ...
Molecular genetics OMIM In an Indian family with PAPA1 linked to chromosome 7p, Radhakrishna et al. (1997) identified a heterozygous in the GLI3 gene (165240.0004).

In a patient with postaxial polydactyly type B of the hands, Furniss et al. ...

Population genetics OMIM Postaxial polydactyly is about 10 times more frequent in blacks than in Caucasians (Frazier, 1960).

In Nigeria, Scott-Emuakpor and Madueke (1976) found frequencies of 17.92 and 27.08 per thousand for females and males, respectively. They concluded ...