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X-linked microcephaly, microphthalmia, microcornea, congenital cataract, hypogenitalism, mental deficiency, growth retardation, spasticity: Possible new syndrome

✍ Scribed by Seemanová, Eva; Lesný, Ivan


Publisher
John Wiley and Sons
Year
1996
Tongue
English
Weight
415 KB
Volume
66
Category
Article
ISSN
0148-7299

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✦ Synopsis


We describe a male and his sister's son with microcephaly, microphthalmia, microcornea, congenital cataract, hypogenitalism, severe mental deficiency, progressive spasticity and growth retardation. Both affected males have brachycephaly, upslanting palpebral fissures, epicanthal folds, highly arched palate, small mouth, and retrognathia. Two maternal cousins of the propositus's mother may also have been affected. Chromosomal and metabolic findings in the propositus were normal. To our knowledge, this disorder had not been reported before as an X-linked syndrome.