Thumb stiffness-brachydactyly-intellectual disability syndrome

Orpha code: 1078OMIM code: 188201

Definition

A rare, genetic, congenital limb malformation syndrome characterized by bilateral thumb ankylosis, type A brachydactyly and mild to moderate intellectual disability. Patients present thumb stiffness and abnormalities of the metacarpal bones, frequently associated with mild facial dysmorphism and signs of obesity. There have been no further descriptions in the literature since 1990.

Disease data
Classification

Malformation syndrome

Synonyms
Piussan-Lenaerts-Mathieu syndrome
Zespół Piussana, Lenaertsa i Mathieu
ORPHA code
1078
OMIM code
188201
ICD10 code
Q87.2
ICD11 code
-

No additional description.

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