[Recurrent benign familial intrahepatic cholestasis].
Benign recurrent intrahepatic cholestasis is a disorder described 3 decades ago. The literature describes at least 60 cases. This syndrome is characterized by attacks of jaundice with obstructive features recurring over a number of years. Though the etiology remains, obscure the coincidence in members of a family or brothers suggests that this may be a constitutional form of jaundice. We describe the case of a young man who presented two episodes of obstructive jaundice. The serologic tests were negative for hepatitis and the biopsy revealed a severe intrahepatic cholestasis without histologic inflammatory changes and preserved lobulillar architecture. Inquest of the family were negative, but parents were possibly related, this factor may be important if a genetic defect is implicated. We conclude that from the clinical biochemical and histological findings this case of jaundice is due to intermittent intrahepatic cholestasis. The most common causes of intrahepatic cholestasis--viral hepatitis and certain drugs--; can be ruled out in this patient. Finally we have to suspect this syndrome, even though very rare, when we have a patient as we described.
Ortíz de Soto
M
M
D'Abenzio
M
M
Nevado
H
H
Inciarte
A E
AE
Fereira
E
E
Martínez
C
C
D000293:Adolescent; D001706:Biopsy; D002780:Cholestasis, Intrahepatic / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D003937:Diagnosis, Differential; D006801:Humans; D008099:Liver / Q000473:pathology*; D008297:Male; D011241:Prednisone / Q000627:therapeutic use
D011241:Prednisone
Colestasis intrahepática benigna familiar recurrente.