Recurrent acute pancreatitis in a Wilson disease patient: an unusual association

Abstract

Abstract Background Wilson’s disease is a multisystem disorder with predominant clinical symptoms depending on the site of copper deposition in the body. Hepatic presentation is usually seen in the younger age group. And pancreatitis is rarely associated with Wilson’s disease. To the best of our knowledge, recurrent acute pancreatitis as a presenting manifestation in a WD patient has not been mentioned before in the literature. Case presentation We report a 17-year-old boy who presented with recurrent acute pancreatitis and subsequently developed deranged liver enzymes and ascites. Work up for the cause of recurrent acute pancreatitis was normal. Low ceruloplasmin (0.07 mg/dL), high 24-h urinary copper excretion (576 μg/day), and dry copper content in the liver (270 μg/g) clinched the diagnosis of Wilson’s disease. The patient was started on a low-copper diet and D-penicillamine therapy resulting in an improvement in symptoms and no further recurrence of pancreatitis. Conclusion The possibility of Wilson’s disease should be considered in young patients with recurrent acute pancreatitis, who have a protracted and obscure disease course.

Keywords

• Wilson’s disease
• Recurrent acute pancreatitis
• Copper metabolism
• Serum ceruloplasmin
• 24-h urinary copper
• D-Penicillamine