Спадковий хронічний панкреатит
ORPHA:676· ICD-10 K86.1· Autosomal dominant hereditary chronic pancreatitis
Визначення(English summary)
A rare gastroenterologic disease characterized by recurrent acute pancreatitis and/or chronic pancreatitis in at least 2 first-degree relatives, or 3 or more second-degree relatives in 2 or more generations, for which no predisposing factors are identified. This rare inherited form of pancreatitis leads to irreversible damage to both exocrine and endocrine components of the pancreas.
- Поширеність
- 1-9 / 1 000 000
- Успадкування
- Autosomal dominant
- Вік початку
- Adolescent, Childhood