Jeune syndrome associated with pancreatic fibrosis

Jeune syndrome, a rare autosomal recessive disorder characterized by skeletal abnormalities of the thorax and extremities, is associated with respiratory distress in infancy, hepatic fibrosis in some instances, and renal failure that may develop later. We report here one additional feature, pancreatic fibrosis. The association of pancreatic fibrosis with Jeune syndrome has been previously described, but it has not been appreciated as a stable and important manifestation of this disorder.