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Pancreatic Lipomatosis Is a Structural Marker in Nondiabetic Children With Mutations in Carboxyl-Ester Lipase

¹ Section for Pediatrics, Department of Clinical Medicine, University of Bergen, Bergen, Norway
² Section for Radiology, Department of Surgery, University of Bergen, Bergen, Norway
³ Department of Clinical Engineering, Haukeland University Hospital, Bergen, Norway
⁴ Department of Biomedicine, University of Bergen, Bergen, Norway
⁵ Section for Pathology, the Gade Institute, University of Bergen, Norway
⁶ Department of Pathology, Haukeland University Hospital, Bergen, Norway
⁷ Department of Pediatrics, Haukeland University Hospital, Bergen, Norway

Address correspondence and reprint requests to Prof. Pål Rasmus Njølstad, MD PhD, Section for Pediatrics, Department of Clinical Medicine, University of Bergen, N-5020 Bergen, Norway. E-mail: pal.njolstad{at}uib.no

Abbreviations: MRI, magnetic resonance imaging; VIBE, volume interpolated breath-hold examination

Both pancreatic volume reduction and lipomatosis have been observed in subjects with diabetes. The underlying molecular and pathological mechanisms are, however, poorly known, and it has been speculated that both features are secondary to diabetes. We have recently described pancreatic atrophy and lipomatosis in diabetic subjects of two Norwegian families with a novel syndrome of diabetes and exocrine pancreatic dysfunction caused by heterozygous carboxyl-ester lipase (CEL) mutations. To explore the early pathological events in this syndrome, we performed radiological examinations of the pancreas in nondiabetic mutation carriers with signs of exocrine dysfunction. In a case series study at a tertiary hospital, we evaluated 11 nondiabetic and mutation-positive children with fecal elastase deficiency and 11 age- and sex-matched control subjects using ultrasound and magnetic resonance imaging (MRI) to estimate pancreatic fat content. The pancreata of nondiabetic mutation carriers exhibited increased reflectivity on ultrasound and had MRI findings indicative of lipomatosis. Apparently, carriers of heterozygous CEL mutations accumulate fat in their pancreas before the anticipated development of diabetes. Our findings suggest that lipomatosis of the pancreas reflects early events involved in the pathogenesis of diabetes and exocrine pancreatic dysfunction syndrome.

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