No abstract available.
Hyperlipoproteinemia Type IV* ; Hyperlipoproteinemias ; Xanthomatosis*

Tibric acid is a new oral hypolipidemic agent with the chemical name of 2-chloro-5(3,5-dimethyl piperidinosulfonyl) benzoic acid. The results of tibric acid administration in 22 patients with primary hyperlipidemia were as follows. 1) Serum triglyceride level was significantly decreased in 84.6% of 22 cases with an average 43.9% decrease in serum triglyceride. 2) Serum cholesterol level was decreased in 59.1% of 22 cases with an average 15.8% decrease in serum cholesterol. The decrease in serum cholesterol level was not significant. 3) It seemed that there were significant falls in the serum triglyceride of the hyperlipoproteinemia type IV and IIb patietns and in the serum cholesterol of type IIa patients 4) The side effects of tibric acid were indigestion, loose stool and anorexia. There was no side effects in 63.6% of cases.
Anorexia ; Benzoic Acid ; Cholesterol ; Dyspepsia ; Humans ; Hyperlipidemias* ; Hyperlipoproteinemia Type IV ; Triglycerides

We experienced a case of a 30 year-old male with hyperlipoproteinemia associated with xanthoma tuberosum and xanthoma striatum palmare. The laboratory findings showed increased serum cholesterol and triglyceride on lipid profile, and widening of prep band on elec1rophoresis of lipoprotein. The biopsy specimens of orange yellow colored nodules on the elbow and yellow streaks on the palrnar creases showed characteristic findings of xanthoma. He had been treated with gempibrozil(lopid) for 1 month.
Adult ; Biopsy ; Cholesterol ; Citrus sinensis ; Elbow ; Humans ; Hyperlipoproteinemia Type IV* ; Hyperlipoproteinemias ; Lipoproteins ; Male ; Triglycerides ; Xanthomatosis*

Type IV hyperlipoproteinemia is characterized by increased blood levels of the triglyceride form of the fat that makes up very low-density lipoproteins (VLDL). Eruptive xanthomas have been frequently observed in type I and V hyperlipoproteinemias but rarely observed in patients with type IV hyperlipoproteinemia. The Koebner phenomenon is the development of the isomorphic skin lesions in persons with certain skin diseases after an injury has occurred on skin of normal appearance. Although this response can develop in psoriasis, lichen planus, verruca etc., it has seldom been associated with eruptive xanthoma. We report a case of eruptive xanthoma with type IV hyperlipoproteinemia and Koebner phenomenon in a 28-year-old Korean male.
Adult ; Humans ; Hyperlipoproteinemia Type IV ; Hyperlipoproteinemias ; Lichen Planus ; Lipoproteins, LDL ; Male ; Psoriasis ; Skin ; Skin Diseases ; Warts ; Xanthomatosis

We report a case of type IV hyperlipoproteinemia associated with xanthoma tuberosum and diabets mellitus in a 48 year old female patient. She had several yellowish-brown papules on the knees and palms. The blood glucose level was elevated and serum lipid and lipoprotein analysis revealed patterns of type IV hyperlipoproteinemia. Histopathologic findings of yellowish-brown papules on the knee showed the aggregation of foam cells in the dermis. She has been treated with diet control and hypolipidemic drug(lovastatin and procetofen) and is under our continuing supervision.
Blood Glucose ; Dermis ; Diabetes Mellitus* ; Diet ; Female ; Foam Cells ; Humans ; Hyperlipoproteinemia Type IV* ; Knee ; Lipoproteins ; Middle Aged ; Organization and Administration ; Xanthomatosis*

Most cases of hypertriglyceridemia (HTG)-induced gestational pancreatitis occur when a person with hyperlipidemia is overweight due to pregnancy or has secondary triggers associated with triglycerides (TGs). In Korea, 6 cases of HTG-induced gestational pancreatitis have been reported, but none of the affected patients had TG levels below 1,000 mg/dL. A 36-year-old female at 30 weeks of gestation was admitted due to pain in her upper abdomen. Initial biochemical analysis revealed a TG level of 260 mg/dL, an amylase level of 2,951 U/L and a lipase level of 3,500 U/L. Abdominal ultrasonography showed pancreatic swelling with a hypoechogenic rim. After several days, the patient was discharged and had a normal delivery at 38 weeks of gestation. This case report is the first to describe acute pancreatitis occurring in the presence of type IV hyperlipoproteinemia even though the TG level was less than 500 mg/dL, contrary to findings in previously reported cases.
Abdomen ; Adult ; Amylases ; Female ; Humans ; Hyperlipidemias ; Hyperlipoproteinemia Type IV ; Hypertriglyceridemia ; Korea ; Lipase ; Overweight ; Pancreatitis ; Pregnancy ; Triglycerides ; Ultrasonography

Just as hyperbetalipoproteinemia is the most common kind of familiar hyperlipoproteinemia, hyperprebetalipoproteinemia or increased very low density lipoprotein (VLDL) and its associated hypertriglyceridemia is an abnormality frequently found in association with several metabolic and nutritional disorders. This VLDL abnormality is demonstrable in high percentage of insulin dependent diabetic children before they receive treatment. It is also observed in many older, overweight, insulin resistant diabetic, with poor control of diabetes. An elevation of VLDL was also observed after ethanol intake. A mild to moderate degree of VLDL elevation is the most frequently observed from of clinical hyperlipidemia. Many investigators reported that hyperprebetalipoproteinemia (hypertriglyceridemia) is associated with an increasing incidence of coronary artery disease. As opposed to hyperbetalipoproteinemia, hyperprebetalipoproteinemia is prone to influence on the development of atherosclerosis in the middle and older aged persons which indicates the importance of triglyceride determination in these aged groups. There has been relatively rare study reported on the triglyceride metabolism in patients with congestive heart failure, essential hypertension and liver disease in Korea. This study was designed to determine serum triglyceride, free fatty acid and glycerol in the disease mentioned in Busan University Hospital between jan. 1975 and December 1976 and analized the change of lipid profile in each different condition. The results were as follows; 1. The mean of serum FFA, triglyceride and glycerol in the 37 normal were as follows 502.32+/-146.54microEq/L, 111.84+/-40.53mg% and 432.00+/-212.13microM/ml. 2. 23 patients with congestive heart failure showed a significant reduction in serum triglycerides (91.96+/-27.80mg%) and a significant increase in serum free fatty acid (576.77+/-129.67microEqL) and glycerol (432.50+/-212.13microM/ml). 3. In 15 patients with essential hypertension a significant elevation of serum triglycerides (149.38+/-42.28mg%) was noted. 4. In 13 patients with liver cirrhosis, a reduction in serum triglycerides (80.50+/-34.27mg%) was noted.
Atherosclerosis ; Busan ; Child ; Coronary Artery Disease ; Estrogens, Conjugated (USP)* ; Ethanol ; Glycerol* ; Heart Failure* ; Humans ; Hyperlipidemias ; Hyperlipoproteinemia Type II ; Hyperlipoproteinemia Type IV ; Hyperlipoproteinemias ; Hypertension* ; Hypertriglyceridemia ; Incidence ; Insulin ; Korea ; Lipoproteins ; Liver Cirrhosis* ; Liver Diseases ; Liver* ; Metabolism ; Nutrition Disorders ; Overweight ; Research Personnel ; Triglycerides*

OBJECTIVETo perform linkage analysis and mutation screening in a Chinese family with familial hpertriglyceridemia (FHTG).

METHODSThirty-two family members including 12 hypertriglyceridemia patients participated in the study. Genotyping and haplotype analysis for 22 subjects were performed using short tandem repeat (STR) microsatellite polymorphism markers on 16 candidate genes and/or loci related to lipid metabolism. Two of the sixteen known candidate genes, APOA2 and USF1 were screened for mutation by direct DNA sequencing.

RESULTSNo linkage was found between the candidate genes/loci of APOA5, LIPI, RP1, APOC2, ABC1, LMF1, APOA1-APOC3-APOA4, LPL, APOB, CETP, LCAT, LDLR, APOE and the phenotype in this family. The two-point Lod scores (theta =0) were all less than-1.0 for all the markers tested. Linkage analysis suggested linkage to chromosome 1q23.3-24.2 between the disease phenotype and STR marker D1S194 with a two-point maximum Lod score of 2.44 at theta =0. Fine mapping indicated that the disease gene was localized to a 5.87 cM interval between D1S104 and D1S196. No disease-causing mutation was detected in the APOA2 and USF1 genes.

CONCLUSIONThe above mentioned candidate genes were excluded as the disease causing genes for this family. The results implied that there might be a novel gene/locus for FHTG on chromosome 1q23.3-1q24.2.

Adult ; Chromosome Mapping ; Chromosomes, Human, Pair 1 ; genetics ; Genetic Linkage ; Genotype ; Haplotypes ; Humans ; Hyperlipoproteinemia Type IV ; genetics ; Lod Score ; Male ; Middle Aged ; Pedigree

We report a case of type IV hyperlipoproteinemia associated with xanthoma tuberosum and xanthoma eruptivum, which improved by treatment with gernfibrozil and procetofene. A 46-year-old male patient visited our clinic for evaluation of multiple orange-yellow colored nodules on the elbows and multiple papules on the knees, buttocks and back. Laboratory examination showed an abnormal elevation of serun cholesterol and triglyceride. Lipoprotein electrophoresis showed an increase of pre-b band and plasma standing test showed turbid plasma. Analysis of lipoprotein revealed increase of VLDL level in his plasma. The biopsy specimens from the nodule on the elbow and the papule on the back revealed characteristic findings of xanthoma. We diagnosed the patient as type IV hyperlipoproteinemia. After 5 months of treatment with diet restriction, gemfibrozil, and procetofene, the serum levels of cholesterol and triglyceride were reduced to normal level. The skin lesions of the both back and buttock showed marked improvement and the skin lesions on the elbows and knee were much improved.
Biopsy ; Buttocks ; Cholesterol ; Diet ; Elbow ; Electrophoresis ; Fenofibrate ; Gemfibrozil ; Humans ; Hyperlipoproteinemia Type IV* ; Knee ; Lipoproteins ; Male ; Middle Aged ; Plasma ; Skin ; Triglycerides ; Xanthomatosis*

A case of plane xanthoma arising in type IV hyperlipoproteinemia in a 53-year old woman is reported. The patient had well-defined yellowish to orange colored slightly elevated plaques on both upper eyelids, cheeks, neck, and upper chest. The laboratory findings showed increased serum triglyceride on lipid profile, and widening of pre-beta band on electrophoresis lipoprotein. She had a fatty liver, mild hepatomegaly, but other systemic diseases such as multiple myeloma, leukemia and lymphoma were not found.
Cheek ; Citrus sinensis ; Electrophoresis ; Eyelids ; Fatty Liver ; Female ; Hepatomegaly ; Humans ; Hyperlipoproteinemia Type IV* ; Leukemia ; Lipoproteins ; Lymphoma ; Multiple Myeloma ; Neck ; Thorax ; Triglycerides ; Xanthomatosis*