This rare autosomal recessive disorder results from the absence or deficiency of lipoprotein lipase, which in turn impairs the metabolism of chylomicrons. Accumulation of chylomicrons in plasma causes recurrent bouts of pancreatitis, usually beginning in childhood, and hepatosplenomegaly is present. Accelerated atherosclerosis is not a feature.
Section 13. Endocrinology and Metabolism