X-linked intellectual disability, Shashi type

Orpha code: 85286OMIM code: 300238

Definition

X-linked intellectual disability, Shashi type is characterised by moderate intellectual deficit, obesity, macroorchidism and a characteristic facies (large ears, a prominent lower lip and puffy eyelids). It has been described in nine boys from two families. Transmission is X-linked and the causative gene has been localised to the q21.3-q27 region of the X chromosome.

Disease data
Classification

Malformation syndrome

Synonyms
Syndromic X-linked intellectual disability type 11
Syndromiczna niepełnosprawność intelektualna sprzężona z chromosomem X typu 11
ORPHA code
85286
OMIM code
300238
ICD10 code
Q87.8
ICD11 code
-

No additional description.

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