Xq21 microdeletion syndrome

Orpha code: 1435OMIM code: 303110

Definition

An X-linked retinal dystrophy characterized by choroideremia, causing in affected males progressive nyctalopia and eventual central blindness. Obesity, moderate intellectual disability and congenital mixed (sensorineural and conductive) deafness are also observed. Female carriers show typical retinal changes indicative of the choroideremia carrier state.

Disease data
Classification

Malformation syndrome

Synonyms
Ayazi syndrome
Zespół Ayazi
Del(X)(q21)
Monosomy Xq21
ORPHA code
1435
OMIM code
303110
ICD10 code
Q93.5
ICD11 code
-

No additional description.

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