Cholestasis syndrome in mucoviscidosis
Popa, I.; Potencz, E.; Moga, D.; Cucuruz, M.; Gaza, S.; Cordeş, E.
Revista de Pediatrie Obstetrica Si Ginecologie. Pediatria 34(1): 45-51
1985
ISSN/ISBN: 0303-8416 PMID: 3925529 Document Number: 260045
On the basis of 15 yr experience, with > 40 cases of mucoviscidosis, the problem of cholostatic syndrome in the course of this disease is discussed. Two cases are reported one characterized by prolonged neonatal jaundice and the other by hemorrhagic diathesis. Of the cases followed up for several years only 1 presented portal cirrhosis. The liver is often affected in mucoviscidosis but the clinical signs of the disease pass unobserved and discordant cholestasis tests are obtained. The pathophysiology of the cholestatic syndrome in mucoviscidosis is complex as the mucosis process is similar to that of the pancreas, with altered metabolism of the bile salts and malnutrition.