Familial chylomicronemia syndrome.

Familial chylomicronemia syndrome is a group of rare genetic disorders characterized by deficient activity of an enzyme lipoprotein lipase or apo-protein C-II deficiency. Incidence is 1 out of 1,000,000. Alternative names to this syndrome are Type I hyper lipoproteinemia and familial lipoprotein lipase deficiency.

Ausência de efeito do captopril no metabolismo de uma emulsão lipídica artificial semelhante aos quilomícrons em pacientes hipertensos e hipercolesterolêmicos / Lack of effect of captopril on the metabolism of an artificial lipid emulsion similar to chylomicrons in hypertensive hypercholesterolemic patients

OBJETIVO: Avaliar o efeito do captopril, sobre o metabolismo dos quilomícrons e de seus remanescentes e as possíveis alterações nas concentrações dos lípides plasmáticos em hipertensos e hipercolesterolêmicos. MÉTODOS: O metabolismo dos quilomícrons foi testado pelo método da emulsão lipídica artificial de quilomícrons marcada com 3H-oleato de colesterol, foi injetada intravenosamente em 10 pacientes com hipertensão arterial leve-moderada antes e após 45 dias de tratamento com captopril (50 mg/dia). Após injeção, foram coletadas amostras de sangue durante 60min em intervalos de tempo pré-estabelecidos para determinar a curva de decaimento e a taxa fracional de remoção (TFR em min-1), bem como o tempo de residência no plasma, da emulsão lipídica artificial, por análise compartimental. As concentrações dos lípides do plasma também foram avaliadas antes e após o tratamento. RESULTADOS: A taxa fracional de remoção (em min-1) da emulsão lipídica antes e após o tratamento com captopril (0,012±0,003 e 0,011±0,003, respectivamente; p=0,85, n.s.) ou o tempo de permanência da emulsão no plasma (83,3±20,8 e 90,9± 22,5 min, n.s.) não se alteraram, mas os níveis de colesterol total e de LDL-c reduziram-se em 7 por cento e 10 por cento respectivamente (p=0,02). As concentrações de HDL-c, triglicérides, Lp(a) e apolipoproteínas AI e B não se modificaram. CONCLUSÃO: O tratamento com captopril, avaliado pelo método da emulsão lipídica artificial, não provoca alterações deletérias no metabolismo dos quilomícrons e seus remanescentes.

Comparação morfológica e bioquímica entre ratos envelhecidos alimentados com dieta hiperlipídica e com óleo de canola / Morphological and Biochemical Comparison among Aged Rats Fed with Hyperlipidic and Canola Oil Diet

PURPOSE: To observe cardiovascular and metabolic changes due to canola oil diet (rich in n-3 fatty acid). METHODS: Forty five rats were followed during 15 months separated in three groups. In group aged (A), they received a regular diet. In group H, animals received a hyperlipidic diet and in group O they received a canola oil diet. The rats were fed for 15 months after weaning. At the end of the experience a blood analysis was performed and heart and aorta were analyzed. The total lipids were extracted and the LDL, VLDL and HDL were determined. RESULTS: Total cholesterol and triglyceride levels were not different among groups. Differences were found in body and cardiac weight, the thickness of the right and left ventricular wall, aorta and pulmonary diameters, HDL and LDL (p < 0.05). Smallest values of the cardiac weight and thickness of the ventricular walls were found in group O. The aorta and pulmonary internal diameters were smaller in group H. The HDL was 40greater in group O than in groups A and H while the LDL was more than 80lower in group O than in groups A and H. Differences in survival curves between groups H x A and between H x O were significant (p < 0.05) but not between groups A x O (p = 0.48). CONCLUSION: Aged rats fed with canola oil diet (rich in n-3 fatty acid) presented morphological cardiovascular and metabolic changes less important in magnitude than old animals and, mainly, the same age animals under hyperlipidic diet.

Clearance of a chylomicron-like emulsion from plasma is delayed in patients with coronary artery disease

To determine whether the metabolism of chylomicrons and their remmants is related to atherogenesis, a triglyceride-rich emulsion known to mimic chylomicron behavior in plasma and labeled with 1-14C-cholesteryl oleate was injected intravenously into 11 normolipidemic patients (triglycerides: 133 + or - 60 mg/dl; total cholesterol: 207 + or - 20 mg/dl; LDL: 143 + or - 20 mg/dl; VLDL: 27 + or - 13 mg/dl; and HDL cholesterol: 41 + or - 11 mg/dl; apo AI: 1.27 + or - 0.54 g/l and apo B: 1.42 + or - 0.31 gl) with atherosclerotic coronary artery disease and into 11 control subjects (triglycerides: 109 + or - 32 mg/dl; total cholesterol: 190 + or - 38 mg/dl; LDL: 125 + or - 29 mg/dl; VLDL: 22 + or - 6 mg/dl; and HDL cholesterol: 44 + or - 9 mg/dl; apo AI: 1.09 + or - 0.28 g/land apo B: 1.09 + or - 028 g/l) without the disease. The emulsion (69 percent triolein, 23 percent lecithin, 6 percent cholesteryl oleate and 2 percent cholesterol) was injected intravenously at approximately 9:00 a.m., after a 12-h fast. The clearance of the emulsion label from plasma, expressed as fractional clearance rate (FCR), was lower in coronary artery disease compared to controls (0.019 + or - 0.005/min vs 0.036 + or - 0.018/min, respectively, P<0.05). These results indicate that the rate of removal of chylomicron remmants is slower in patients with coronary artery disease