ABSTRACT
Congenital generalized lipodystrophy (CGL) is a rare autosomal recessive disorder characterized by near complete absence of adipose tissue since birth and insulin resistance. The diagnosis is made on the basis of lack of body fat, muscular hypertrophy, acanthosis nigricans, hirsutism, hepatomegaly with fatty liver, hyperlipidemia and hyperglycemia with insulin resistance. We describe a 4-year-old Chinese girl with the clinical features of CGL.
Subject(s)
Child, Preschool , China , Diabetes Mellitus, Lipoatrophic/diagnosis , Female , Humans , Hypertriglyceridemia/diet therapyABSTRACT
En un estudio prospectivo, longitudinal y abierto se evaluó la modificación en el perfil de lípidos de pacientes hepercolesterolémicos. Se incluyeron 22 individuos (7 H y 15 M) dislipidémicos (colesterol mayor de 6.20 mmoles), se agregaron 100 gramos de nuez cáscara de papel cada tercer día y se siguieron con determinaciones de colesterol cada mes durante tres meses; no se dio otro tipo de dieta que la habitual del paciente, para evitar confusiones sobre la causa de la modificación del perfil de lípidos (42 por ciento del peso de este tipo de nuez es grasa monoinsaturada y 18 por ciento grasa poliinsaturada). La prueba estadística empleada fue la t de Student. Los 22 pacientes tuvieron una respuesta satisfactoria tanto en la reducción de colesterol -inicial 7.39 mmoles, final 7.03 mmoles (32 por ciento)- como en la de triglicéridos -inicial 2.05 mmoles, final 1-62 mmoles- p < 0.05. Siete pacientes eran además hipertensos y cinco diabéticos; sin embargo, no hubo diferencia significativa en su respuesta. Nuestros resultados muetran un claro efecto benéfico del uso de este tipo de alimentos sobre el perfil de lípidos del paciente con dislipidemia, reforzando el que su manejo se base en modificaciones en la dieta más que en el uso de fármacos hipocolesterolemiantes. Una ventaja adicional de esta variedad de nuez es su alto contenido en L-arginina, aminoácido precursor del óxido nítrico, el cual ha demostrado en animales hipercolesterolémicos un marcado efecto antiaterogénico
Subject(s)
Atherosclerosis/diet therapy , Atherosclerosis/prevention & control , Fats, Unsaturated , Hypercholesterolemia/blood , Hypercholesterolemia/diet therapy , Hyperlipidemias/diet therapy , Hypertriglyceridemia/diet therapy , SeedsABSTRACT
Indian studies on lipid profile abnormalities in chronic renal failure (CRF) have varied from no abnormalities at all to significant abnormality (hypertriglyceridemia and reduced HDL) as described in the Western literature. Moreover, there is no Indian study on the effect of renal transplantation on the abnormal lipid profile of CRF. The aim of our study was to determine the lipid profile of CRF patients on conservative treatment, end stage renal disease (ESRD) patients on maintenance hemodialysis (HD) treatment and renal transplant patients. We also looked at the effect of fish oil rich in polyunsaturated fatty acids (Max-EPA) on hypertriglyceridemia of CRF. The study included 4 groups; Gp I: control subjects (n = 9, age = 30 +/- 5 yrs), Gp II: CRF patients on conservative treatment (n = 9, age = 49 +/- 17 yrs), Gp III: ESRD patients on HD for at least 3 months (n = 19, age = 53 +/- 9 yrs), Gp IV: 3 months post-renal transplant patients (n = 9, age 31 +/- 11 yrs). The lipids and lipoproteins analysed include total cholesterol, HDL, LDL, triglycerides, Apo A1 and Apo B. It was observed that in Gp II patients triglycerides were significantly elevated (p < .05) and Apo A1/Apo B significantly abnormally lower (p < .001) compared to Gp I. In Gp IV patients, there was no significant difference in lipid profile compared to Gp I. With the use of Max-EPA in 5 patients with hypertriglyceridemia, there was a significant improvement in hypertriglyceridemia (p < .05). Our study suggests: 1) significant hypertriglyceridemia does develop in a majority of CRF patients. The abnormality probably improves with dialysis treatment and renal transplantation. 2) A lower Apo A1/Apo B ratio in CRF patients may account for higher risk of atherosclerosis. 3) Fish oil rich in polyunsaturated fatty acids improves hypertriglyceridemia of CRF.