Синдром гострої дитячої печінкової недостатності, повязаний з лихоманкою
ORPHA:464724· ICD-10 K72.0· Fever-associated acute infantile liver failure syndrome
Визначення(English summary)
A rare genetic parenchymatous liver disease characterized by infantile or early childhood onset of recurrent episodes of acute liver failure precipitated by a febrile illness. During the life-threatening episodes, patients present with vomiting, lethargy, jaundice, as well as elevated levels of liver enzymes and coagulopathy. There is usually complete recovery between the episodes with conservative treatment.
- Поширеність
- <1 / 1 000 000
- Успадкування
- Autosomal recessive
- Вік початку
- Infancy