Severe liver dysfunction in an infant with cystic fibrosis masquerading as metabolic liver disease

Abstract

We present a rare presentation of cystic fibrosis with neonatal cholestasis. Histological features of mucoviscidosis were present in liver involving the biliary tract, intestinal mucosa, pancreas, and lung. Besides, there was a rare association with autosomal dominant type of polycystic renal disease.

Keywords

• Autosomal dominant polycystic kidney disease
• cholestatic liver disease
• cystic fibrosis
• mucoviscidosis