Inactivity of lipoprotein lipase (LPL) plays the predominant role in the development of<unk> . LPL plays a role in the metabolism of triglycerides within VLDL molecules. Inactivation mutations in LPL will create an environment with an increased concentration of VLDL molecules and therefore, triglycerides. The elevation of baseline triglyceride levels begins the cascade into other pathologies. The most common acute manifestation of<unk>  is the occurrence of pancreatitis. Pancreatitis is caused by the premature activation of exocrine pancreatic enzymes. Secreted zymogens are cleaved to active trypsin and play a central role in digestion of food in the duodenum. If there is premature activation of trypsin within the pancreatic tissues, there is an induction of autodigestion of local tissue which leads to the initial presentation of pancreatitis. Autodigestion of local tissues also leads to disruptions in pancreatic microvascular tissue which can cause an ischemia-reperfusion event at the pancreatic level. There are other varying secondary causes of pancreatitis that can further contribute to the primary scenario of pancreatitis related to<unk> .
Familial hypertriglyceridemia
Familial hypercholesterolemia
Combined hyperlipidemia
Lipoatrophic diabetes
Familial dysbetalipoproteinemia
Abetalipoproteinemia
Citrullinemia type I
HIDEA syndrome