Benign recurrent intrahepatic cholestasis progressing to progressive familial intrahepatic cholestasis: low GGT cholestasis is a clinical continuum.

[benign recurrent intrahepatic cholestasis]

Benign recurrent intrahepatic cholestasis (BRIC ) is an autosomal recessive liver disease , characterised by intermittent attacks of cholestasis , which can start at any age and last for several weeks to months . Characteristically serum GGT activity is low and normal liver structure is preserved . Progressive familial intrahepatic cholestasis (PFIC ) is another liver disease , characterised by severe cholestasis , starting almost invariably before 6 months of age . All patients progress to cirrhosis , liver failure and death , unless a liver transplantation is performed . We now identified four patients who presented in childhood with recurrent attacks of cholestasis , while in the course of the disease the cholestasis gradually became permanent . Although liver biopsies performed in the early stages of the disease showed normal liver architecture , late stage biopsies revealed evident fibrosis with porto -portal septa formation . In conclusion , the disease of these patients started with the clinical and histopathological characteristics of BRIC but progressed to PFIC .

Diseases
Validation

Diseases presenting "recurrent attacks" symptom

allergic bronchopulmonary aspergillosis

benign recurrent intrahepatic cholestasis

erythropoietic protoporphyria

familial mediterranean fever

neuralgic amyotrophy

systemic capillary leak syndrome

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