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McKusick-Kaufman syndrome

ORPHA:2473· ICD-10 Q87.8

Definition

A rare genetic syndrome with multiple congenital anomalies characterized by urogenital malformations, mainly hydrometrocolpos (HMC) in females, postaxial polydactyly (PAP) and congenital heart disease (CHD). The disorder is allelic with Bardet-Biedl syndrome 6 (BBS6).

Prevalence
Unknown
Inheritance
Autosomal recessive
Age of onset
Antenatal, Neonatal