Progressive intrahepatic cholestasis (Byler's disease): case report

Abstract

Description of a child in whom the clinical and laboratory data indicated a progressive intrahepatic cholestasis of the type described as Byler's disease. The histologic and histochemical findings suggest intrahepatic cholestasis. Electron microscopy revealed interruptions of the bile canalicular membrane, which have been described as characteristic of this disease. A striking feature in the present case was the remarkable increase of microfilamentous structures in the pericanalicular ectoplasm and in the hepatocytic cytoplasm. The findings suggest a primary disturbance in bile acid secretion as the cause of cholestasis, entailing a hypertrophy of pericanalicular microfilaments which supposedly play a role in the final step of bile secretion.