Pancreatic lipomatosis in an infant with cystic fibrosis

Excerpt

An 11-month-old boy presented with a history of passing bulky stools and foul-smelling oil droplets in his stool for the last 2 months. His birth history was unremarkable, with no perinatal/antenatal complications. There was no evidence of developmental delay. A bone-marrow examination revealed fragmented marrow spaces without any storage cells and adequate megakaryocytes. Laboratory tests revealed normal blood sugar levels and renal function. Sweat chloride levels were raised (63 mEq/l). A stool examination showed presence of 200 fat globules. Urine tests for fructose, galactose and mucopolysaccharides were negative. His chest radiograph was normal, and the patient had no respiratory problems. A CT scan of the abdomen revealed fatty replacement of the pancreatic head and tail region with relative sparing of the body of the pancreas (Fig. 1). The pancreas was normal in size, and there was no evidence of pancreatitis, pancreatic mass, atrophy, cysts, or biliary or pancreatic ductal dilatation. A genetic study revealed the patient was heterozygous for Δ F 508 deletion.

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