I-Hyperlipoproteinemia which clinical performance?

I-Hyperlipoproteinemia extremely rare, are genetic diseases, congenital defects of the lipoprotein lipase, triglycerides can not be derived from the hydrolysis, resulting in the accumulation of large number of chylomicrons in blood. The disease often in adolescence, and in less than 10 years old was found, but also reported in the week after the birth shall discoverer.
The main clinical manifestations are: (1) changes in the skin, for the symptoms first appeared. In the elbow, back and arm that measles-yellow tumor. But no eyelid tumors and muscle-related macular yellow tumor. (2) When the serum triglyceride> 22.6 cents mole / liter, fundus, there may be served hyperlipidemia retinopathy. (3) hepatosplenomegaly, with the degree of blood triglyceride content changes. (4) recurrent abdominal pain.
Its biochemical characteristics: Because chylomicrons increased plasma was like butter, at 4 ℃ refrigerator overnight, the upper deck was "butter"-like cover, lower clarify significantly higher triglyceride, and cholesterol is normal or only mildly increased, cholesterol / triglyceride ratio <0.2.