Hirschsprung disease-type D brachydactyly syndrome

Orpha code: 2150OMIM code: 306980

Definition

Hirschsprung disease-type D brachydactyly syndrome is characterized by Hirschsprung disease and absence or hypoplasia of the nails and distal phalanges of the thumbs and great toes (type D brachydactyly). It has been described in four males from one family (two brothers and two maternal uncles). Transmission appears to be X-linked recessive but autosomal dominant inheritance with incomplete penetrance in females can not be ruled out.

Disease data
Classification

Malformation syndrome

ORPHA code
2150
OMIM code
306980
ICD10 code
Q43.1
ICD11 code
-

No additional description.

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