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Синдром лімфедеми-задньої атрезії хоан

ORPHA:99141· ICD-10 Q82.0· Lymphedema-posterior choanal atresia syndrome

Визначення(English summary)

A rare genetic disease characterized by choanal atresia and early onset of lymphedema of the lower extremities. Additional reported features include facial dysmorphism (hypertelorism, broad forehead, smooth philtrum, unilateral low-set ear, and high-arched palate), hypoplastic nipples, and pectus excavatum.

Поширеність
<1 / 1 000 000
Успадкування
Autosomal recessive
Вік початку
Neonatal