Microcephaly-congenital cataract-psoriasiform dermatitis syndrome

Orpha code: 488168OMIM code: 616834

Definition

A rare sterol biosynthesis disorder characterized by microcephaly, bilateral congenital cataract, mild developmental delay, growth delay with short stature, psoriasiform dermatitis of variable severity, and immune dysregulation. Behavioral disorder, joint contractures, and arthralgia have also been described.

Disease data
Classification

Malformation syndrome

Synonyms
SMO deficiency
Niedobór sterol-C4-metylo oksydazy
Sterol-C4-methyl oxidase deficiency
ORPHA code
488168
OMIM code
616834
ICD10 code
E88.8
ICD11 code
-

No additional description.

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