Proximal Xq28 duplication syndrome

Orpha code: 1762OMIM code: 300260

Definition

A rare X-linked genomic disorder associated with interstitial chromosomal duplications at Xq28 encompassing the <i>MECP2</i> gene. It is characterized in males by infantile onset hypotonia, severe global developmental delay, intellectual disability, progressive spasticity, seizures, gastrointestinal symptoms and recurrent respiratory infections. In females, the phenotype is more variable.

Disease data
Classification

Malformation syndrome

Synonyms
MECP2 duplication syndrome
Dystalna duplikacja Xq
Telomerowa duplikacja Xq
X-linked intellectual disability syndrome, Lubs type
ORPHA code
1762
OMIM code
300260
ICD10 code
Q99.8
ICD11 code
-

No additional description.

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