<prompt> Steatorrhoea with increased triglycerides seen in deficiency of: PGI Dec 2006Pancreatic lipaseSerum lipaseLipoprotein lipaseAcetyl CoA carboxylase  <response>: Ans; C Lipoprotein lipase deficiency Lipoprotein lipase deficiency leads to hypertriglyceridemia and hyperchyiomicronemia.Hypertriglyceridemia can cause pancreatistis that leads to steatorrhea.Familial Chylomi crone mia Syndrome Type Hyperlipoproteinemia ; Lipoprotein Lipase and Apo C - II Deficiency Harrison 17th2421In these disorders, called familial chylomicronemia syndromes, fasting triglyceride levels are almost invariably 1000 mg dL. Fasting cholesterol levels are also usually elevated but to a much lesser degree. LPL deficiency has autosomal recessive inheritance and has a frequency of approximately 1 in 1 million in the population. Apo C-II deficiency is also recessive in inheritance pattern and is even less common than LPL deficiencyBoth LPL and apo C-II deficiency usually present in childhood with recurrent episodes of severe abdominal pain due to acute pancreatitisQuantitatively, steatorrhea is defined as stool fat exceeding the normal 7 gd; rapid-transit diarrhea may result in fecal fat up to 14 gd; daily fecal fat averages 15-25 g with small intestinal diseases and is often 32 g with pancreatic exocrine insufficiency. Intraluminal maldigestion, mucosal malabsorption, or lymphatic obstruction may produce steatorrhea-:Harrison 17th250Hepatic Lipase Deficiency Harrison 17th2421HL is a member of the same gene family as LPL and hydrolyzes triglycerides and phospholipids in remnant lipoproteins and HDLs. HL deficiency is a very rare autosomal recessive disorder characterized by elevated plasma levels of cholesterol and triglycerides mixed hyperlipidemia Table : Harrison 17th2419 Frederickson Classification of HyperlipoproteinemiasPhenotypeIIIaIIbIIIVVLipoprotein, elevatedChylomicronsLDLLDL and VLDLChylomicron and VLDL remnantsVLDLChylomicrons and VLDLTriglycerides-- to Cholesterol to to to -- to to LDL-cholesterol HDL-cholesterolPlasma appearanceLactescentClearClearTurbidTurbidLactescentXanthomasEruptiveTendon, tuberousNonePalmar, tuberoerup- tiveNoneEruptivePancreatitis0000Coronary athe rosclerosis0--Peripheral atherosclerosis0 --Molecular defectsLPL and apoC-IILDL receptor, ApoB- 100, PCSK9, ARH, ABCG5 and ABCG8UnknownApoEApoA-V and UnknownApoA-V and UnknownGenetic nomenclatureFCSFH, FDB. ADH, ARH, sitosteroiemiaFCHLFDBLFHTGFHTGNote: LPL, lipoprotein lipase; apo. apolipoprotein; PCS, familial chylomicrortemia syndrome. FH. familial hypercholesterolemia: FDB. familial defective apoB: ARH. aulosomai recessive hypercholesterolemia; ADH, autosomal dominant hypercholesterolemia; FCHL. familial combined HLAlso Remember:Triacylglycerol undergoes hydrolysis by a hormone-sensitive lipase to form free fatty acids and glycerol. This lipase is distinct from lipoprotein lipase, which catalyzes lipoprotein triacylglycerol hydrolysis before its uptake into extrahepatic tissues.Hormone-sensitive lipase is activated by ACTH, TSH, glucagon, epinephrine, norepinephrine, and vasopressin and inhibited by insulin, prostaglandin PS, and nicotinic acid,Lingual lipase in saliva,Gastric lipase in gastric juice.Pancreatic lipase in pancreatic juice.Intestinal lipase in intestinal epithelial cell,Adipolytic lipase in adipose tissue, andSerum lipase.Pancreatic LipasePancreatic lipase is essential for absorption of dietary fats in small intestine-Warper 28th460It deficiency definciency cause Steatorrhea, but hypertriglyuceridemia is unlikelyAcetyl Co A carboxylase is enzyme for lipogenesisQ- Harper 28th196Succinate thiokinase is a enzyme of TCA cycle- Harper 28th145Serum Lipase is marker of acute pancreatitis- Harper 28th59 