vitalwiki

Синдром Йохансона-Блізард

ORPHA:2315· ICD-10 Q87.8· Johanson-Blizzard syndrome

Визначення(English summary)

Johanson-Blizzard syndrome (JBS) is a multiple congenital anomaly characterized by exocrine pancreatic insufficiency, hypoplasia/aplasia of the nasal alae, hypodontia, sensorineural hearing loss, growth retardation, anal and urogenital malformations, and variable intellectual disability.

Поширеність
1-9 / 1 000 000
Успадкування
Autosomal recessive
Вік початку
Antenatal, Infancy, Neonatal