Familial hypertriglyceridemia

Hypertriglyceridemia or significant family history of Aripiprazole, ziprasidone Risperidone, quetiapine... 

These effects are reversible after cessation of therapy. Patients who are at high risk of developing hypertriglyceridemia include those with diabetes, obesity, increased alcohol intake, a lipid metabolism disorder, and a familial history. 

Familial hypertriglyceridemia IV VLDL TG Overproduction of VLDL low LPL activity. Pancreatitis, CHD if HDL is low... 

The fibrates are mainly used to treat two hyperlipi-demias, familial hypertriglyceridemia (type IV) and dysbetalipoproteinemia (type III). They are also useful in the treatment of hypertriglyceridemia associated with type II diabetes (secondary hyperlipidemia). The fibrates are the drugs of choice in treating hypertriglyceridemias, particularly those associated with low levels of HDL cholesterol. The fibrates additionally appear to... 

It is indicated as an adjunct to diet to reduce elevated total cholesterol, LDL-cholesterol and TG levels in patients with primary hypercholesterolemia, diabetic dyslipidaemia or mixed hyperlipidemia, hypertriglyceridemia, dysbetalipo-proteinemia and familial hypercholesterolemia. 

Familial hypertriglyceridemia-Severe VLDL, chylomicrons increased Niacin, fibrate Niacin plus fibrate... 

For treatment of heterozygous familial hypercholesterolemia, most patients require 2-6 g of niacin daily more than this should not be given. For other types of hypercholesterolemia and for hypertriglyceridemia, 1.5-3.5 g daily is often sufficient. Crystalline niacin should be given in divided doses with meals, starting with 100 mg two or three times daily and increasing gradually. 

The consequences of hypertriglyceridemia are not well understood, but there may be an increased risk of cardiovascular disease and pancreatitis (SEDA-13, 123). Patients with an increased tendency to develop hypertriglyceridemia include those with diabetes mellitus, obesity, increased alcohol intake, and a positive family history. With a short course (16 weeks) of isotretinoin it is sufficient to ensure there is no hyperlipidemia before the start of therapy, and to determine the triglyceride response to therapy on one occasion after 4 weeks (1207). 

Patient Population. The proband of the B family, T.B., was referred to the Lipid Research Clinic at The Johns Hopkins Hospital at the age of five years because of hypercholesterolemia of 900 mg/100 ml. She had multiple planar xanthomas that had first appeared at three years of age. The patient was free of symptoms of ischemic heart disease. The index lipoprotein pattern was type lib (57), with marked hypercholesterolemia, hyperbeta-lipoproteinemia, a mild hyperprebetalipoproteinemia and hypertriglyceridemia. None of the relatives of T.B. had xanthomas or corneal arcus one (J.S.) developed signs of premature coronary atherosclerosis at the age of 43 years. Increased total plasma and LDL cholesterol levels were transmitted over three generations on both maternal and paternal sides of the family (Fig. I). The parents of the proband, S.B. and K.B., had endogenous hypertriglyceridemia as well. Two normolipidemic members of this family (S.B., Jr. and E.B.), were also studied. 

D. Familial hypertriglyceridemia (Type IV or V pattern). There is elevated VLDL, but the mechanism is unclear (possibly a defect in VLDL catabolism). There may be xanthomas, pancreatitis and premature atherosclerosis. 

The temporal relation in the second case suggests that lisinopril was causative. The authors raised the possibility that the concomitant use of an estrogen and simvastatin, as well as a history of familial hypertriglyceridemia, may have predisposed her to pancreatitis. 

Lipoprotein and hepatic lipases are important enzymes involved in the metabolism of chylomicrons and various fractions of lipoproteins. Both have been the subject of attention, as evidenced by numerous reviews (e.g., Garfinkel and Schotz, 1987 Wang eta/., 1992). This interest stems from the fact that abnormal lipoprotein metabolism has been linked to various disorders, including hyperchylomicronemia, hypercholesterolemia, hypertriglyceridemia, obesity, diabetes, and premature atherosclerosis. Genetic defects in both HL and LPL are now known to be the cause of at least some familial disorders of lipoprotein metabolism. 

Familial Combined Hyperlipidemia About 10% to 15% of patients with premature CHD actually have familial combined hyperlipidemia (FCHL). This disorder is recognized as a distinct phenotype by studying family members of survivors of myocardial infarction. Patients with FCHL can have increased plasma concentrations of total and LDL cholesterol (type Ila), or triglyceride (type IV), or both (type lib). In all cases, apo B-lOO concentrations are increased. The presentation of lipoprotein patterns can vary in an individual with time. Furthermore, patients with hypertriglyceridemia with normal partners tend to have offspring with hypercholesterolemia, and vice versa. 

The cause of the overproduction of VLDL triglyceride is unknown. The administration of estrogen and corticosteroids aggravates hypertriglyceridemia in these patients and can sometimes lead to acute pancreatitis. The diagnosis of FHTG requires study of other family members to differentiate this disorder from FCHL. This disorder appears to be inherited in an autosomal dominant pattern with a delayed expression and an estimated frequency in the population at about 1 500 persons. About one in five children born to affected parents manifest the phenotype early in life. ... 

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