Hyperlipoproteinemia Type IV (Type IV, Hyperlipoproteinemia)

A hypertriglyceridemia disorder, often with autosomal dominant inheritance. It is characterized by the persistent elevations of plasma TRIGLYCERIDES, endogenously synthesized and contained predominantly in VERY-LOW-DENSITY LIPOPROTEINS (pre-beta lipoproteins). In contrast, the plasma CHOLESTEROL and PHOSPHOLIPIDS usually remain within normal limits.

Also Known As:

Networked: 192 relevant articles (3 outcomes, 11 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Type 2 Diabetes Mellitus (MODY)
2.	Hypertriglyceridemia
3.	Pancreatitis
4.	Hypercholesterolemia
5.	Hyperuricemia

Experts

1.	Mateo-Gallego, Rocio: 2 articles (09/2010 - 02/2010)
2.	Burillo, Elena: 2 articles (09/2010 - 02/2010)
3.	Cenarro, Ana: 2 articles (09/2010 - 02/2010)
4.	Civeira, Fernando: 2 articles (09/2010 - 02/2010)
5.	Toumeh, Anis: 1 article (09/2014)
6.	Abuissa Qadan, Mahmoud A: 1 article (09/2014)
7.	Safi, Fadi: 1 article (09/2014)
8.	Karaz, Rana: 1 article (09/2014)
9.	Assaly, Ragheb: 1 article (09/2014)
10.	AlAkdar, Bassam: 1 article (09/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hyperlipoproteinemia Type IV:

1.	Lipoprotein Lipase (Diacylglycerol Lipase)IBA 11/01/1994 - "A newly identified heterozygous lipoprotein lipase gene mutation (Cys239-->stop/TGC972-->TGA; LPLobama) in a patient with primary type IV hyperlipoproteinemia." 09/01/1990 - "Thus heterozygous lipoprotein lipase deficiency, together with age-related influences, may account for a form of familial hypertriglyceridemia." 06/01/1998 - "We describe a 32-yr-old female with lipoprotein lipase-deficient familial hypertriglyceridemia who had recurrent episodes of acute pancreatitis. " 12/15/1996 - "Heterozygous lipoprotein lipase (LPL) deficiency has been associated with familial hypertriglyceridemia and familial combined hyperlipidemia. " 11/01/1994 - "We investigated measures for identification of heterozygous lipoprotein lipase (LPL) deficiency in unrelated subjects with primary type IV hyperlipoproteinemia in order to acquire a helpful clue for understanding the correlation between hypertriglyceridemia and the status of being a heterozygous carrier of an LPL gene variant. "
2.	DNA (Deoxyribonucleic Acid)IBA 06/01/1997 - "The purpose of this study was to develop an improved method of direct DNA sequencing, which makes it possible to identify heterozygous mutations of the lipoprotein lipase (LPL) gene in order to understand the underlying genetic disorder of type IV hyperlipoproteinemia. " 07/01/1987 - "DNA polymorphisms of apolipoprotein A-I/C-III and insulin genes in familial hypertriglyceridemia and coronary heart disease."
3.	ApolipoproteinsIBA 11/01/1997 - "Postprandial hemorrheology and apolipoprotein B metabolism in patients with familial hypertriglyceridemia." 03/20/2007 - "Apolipoprotein B is associated with metabolic syndrome in Chinese families with familial combined hyperlipidemia, familial hypertriglyceridemia and familial hypercholesterolemia." 05/01/2007 - "A novel gene encoding apolipoprotein AV, an activator of lipoprotein lipase, has also been linked to familial hypertriglyceridemia. " 07/01/2000 - "Platelet function in patients with familial hypertriglyceridemia: evidence that platelet reactivity is modulated by apolipoprotein E content of very-low-density lipoprotein particles." 07/01/1986 - "Plasma lipoproteins, apolipoproteins, and triglyceride metabolism in familial hypertriglyceridemia."
4.	HDL CholesterolIBA 06/01/1982 - "[Levels of high-density lipoprotein cholesterol in persons with hypertrigliceridemia-hyperlipoproteinemia type IV]." 10/16/1980 - "We therefore used dietary therapy to lower the triglyceride levels of 29 men with Type IV hyperlipoproteinemia and evaluated the effects on HDL-cholesterol levels. " 01/01/1997 - "Levels of total cholesterol were higher in patients with hyperlipoproteinemia type IV, while values of HDL cholesterol were decreased in both subgroups of patients in regard to the control group. " 05/01/1986 - "In addition, plasma triglyceride levels and HDL2 and HDL3 cholesterol were also determined in seven patients with Type IV hyperlipoproteinemia: three with normal LDL apo B levels and four with HyperapoB. " 10/16/1980 - "The persistently low HDL-cholesterol level in the presence of normalization of triglycerides suggests that depressed HDL cholesterol may be an independent metabolic abnormality in Type IV hyperlipoproteinemia."
5.	Glucose (Dextrose)FDA LinkGeneric 09/01/1974 - "Influence of triglyceridemia level on glucose tolerance and insulinemia in subjects with type IV hyperlipoproteinemia." 04/01/1998 - "Divergent responses of the liver to increased delivery of glucose or fatty acids: implications for the pathogenesis of type IV hyperlipoproteinemia." 06/01/1978 - "Interactions of obesity and glucose-stimulated insulin secretion in familial hypertriglyceridemia." 09/20/1985 - "The drug caused a significant reduction in very-low-density lipoprotein cholesterol in patients with non-insulin-dependent diabetes mellitus and type IV hyperlipoproteinemia, along with a variable change in high-density lipoprotein cholesterol, independent of its effect on plasma glucose." 01/01/1990 - "Blood glucose and insulin responses to oral glucose were measured in 33 men: 8 normal, 13 with borderline carbohydrate-lipid metabolism disturbance (BCLMD) (pre-beta-lipoproteinemia with normal cholesterol and triglyceride levels), and 12 with mild Type IV hyperlipoproteinemia. "
6.	VLDL CholesterolIBA 09/20/1985 - "The drug caused a significant reduction in very-low-density lipoprotein cholesterol in patients with non-insulin-dependent diabetes mellitus and type IV hyperlipoproteinemia, along with a variable change in high-density lipoprotein cholesterol, independent of its effect on plasma glucose."
7.	Oxandrolone (Anavar)FDA LinkGeneric 02/01/1979 - "In patients with type IV hyperlipoproteinemia, significant reduction of plasma apolipoproteinB and VLDL apolipoprotein B and inverse LDL apolipoprotein B increment was observed after treatment with oxandrolone. "
8.	Lipoproteins (Lipoprotein)IBA 11/01/1992 - "the HuCIIITg mouse appears to be a suitable animal model of primary familial hypertriglyceridemia, and these studies suggest a possible mechanism for this common lipoprotein disorder." 01/01/1991 - "Effect of dietary treatment on the lipid, lipoprotein and fatty acid compositions in type IV familial hypertriglyceridemia." 01/01/1985 - "A correct positive correlation (r = 0.75) between the triglyceride levels and low density lipoprotein (LDL)/very low density lipoprotein (VLDL) ratio and the VLDL increase observed by gel disk electrophoresis confirm that a type IV hyperlipoproteinemia is associated with AD. " 03/11/1983 - "Observation of differences between triglyceride structure of lipoproteins in type III and type IV hyperlipoproteinemia." 02/01/1983 - "There was no significant relationship between these two lipoprotein classes in familial hypertriglyceridemia or in normals. "
9.	VLDL LipoproteinsIBA 09/01/1984 - "Studies of triacyglycerol structure of very low density lipoproteins of normolipemic subjects and patients with type III and type IV hyperlipoproteinemia." 08/15/1978 - "Because this relatively time-consuming study cannot be envisaged currently in clinical services, it seems useful to analyse a storage procedure which does not affect the composition of very-low density lipoproteins, whose proportion is very important among subjects with type IV hyperlipoproteinemia. " 06/01/1999 - "Hypertriglyceridemic (HTG) very low density lipoproteins (VLDL) from subjects with type IV hyperlipoproteinemia induce both cholesteryl ester (CE) and triglyceride (TG) accumulation in cultured J774 macrophages. " 05/01/1998 - "We have previously shown that very low density lipoproteins (VLDL, Sf 60-400) from subjects with type IV hyperlipoproteinemia (HTG-VLDL) will induce appreciable cholesteryl ester accumulation in cultured macrophages (J774A.1). " 02/01/1977 - "P50 at in vivo pH in diabetics with moderately elevated very low density lipoproteins (VLDL)--Type IV hyperlipoproteinemia (HLP)--was likewise found to be slightly lower than normal (25.5 versus 26.6 mmHg, p less than 0.05). "
10.	Gemfibrozil (Lopid)FDA LinkGeneric 07/01/1988 - "A short term open study (90 days) was carried out with Gemfibrozil in patients (n = 10) suffering from type IV hyperlipoproteinemia. " 11/01/1997 - "We conclude that triglyceride-lowering therapy by gemfibrozil had no substantial beneficial effects with respect to hemorrheology in patients with familial hypertriglyceridemia." 01/01/1991 - "HDL metabolism in HDL deficiency associated with familial hypertriglyceridemia: effect of treatment with gemfibrozil." 07/01/1988 - "Changes induced by gemfibrozil on lipidic, coagulative and fibrinolytic pattern in patients with type IV hyperlipoproteinemia." 05/02/1989 - "These results suggest that subjects with low HDL cholesterol and type IIB hyperlipoproteinemia (and possibly type IV hyperlipoproteinemia) would benefit from treatment with gemfibrozil."

Therapies and Procedures

1.	Aftercare (After-Treatment) 02/01/1979 - "In patients with type IV hyperlipoproteinemia, significant reduction of plasma apolipoproteinB and VLDL apolipoprotein B and inverse LDL apolipoprotein B increment was observed after treatment with oxandrolone. "
2.	Fat-Restricted Diet (Diet, Fat Restricted) 03/01/1996 - "Treatment of severe familial hypertriglyceridemia during pregnancy with very-low-fat diet and n-3 fatty acids." 06/01/2003 - "After study at entry, in 56 women (median age, 52 years), 36 with familial hypertriglyceridemia, to lower triglycerides, estrogens and SERMs (hormone treatment, HT) were stopped; a very low fat diet (<15% of calories), gemfibrozil (1.2-1.5 mg/day), and omega-3-fatty acid (4-12 g/day) were started, with restudy 2-4 weeks later. "
3.	Renal Dialysis (Hemodialysis) 05/01/1980 - "[Hyperprebetalipoproteinemia in patients treated by hemodialysis: clinico-experimental study]." 05/01/1985 - "[Effect of pantethine therapy in chronic uremic patients undergoing hemodialysis with type IV hyperlipoproteinemia]."
4.	Diet Therapy (Therapy, Diet) 01/01/1979 - "[Study of lipid metabolic coefficient K2 in patients with hyperlipoproteinemia type IV before and after reduction of triglyceride level by adapted diet therapy]."
5.	Plasma Exchange 01/01/2009 - "Management of familial hypertriglyceridemia during pregnancy with plasma exchange." 09/01/2014 - "Management of familial hypertriglyceridemia-induced pancreatitis during pregnancy with therapeutic plasma exchange: a case report and review of literature."