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夸休可尔症患者肝脏甘油三酯脂肪酶选择性缺乏与高甘油三酯血症

Selective deficiency of hepatic triglyceride lipase and hypertriglyceridaemia in kwashiorkor.

作者信息

Agbedana E O, Johnson A O, Taylor G O

出版信息

Br J Nutr. 1979 Nov;42(3):351-6. doi: 10.1079/bjn19790124.

Abstract
  1. Serum postheparin lipolytic activities (PHLA), triglyceride and free fatty acid concentrations were determined in children with kwashiorkor before and after treatment and also in normal control children. 2. Using the range (571-1650 mumol/l) of serum triglyceride of the control children as normal, five (20%) of the twenty-five children with kwashiorkor had low (less than 570 mumol/l), thirteen (52%) had normal (571-1650 mumol/l) and seven (28%) had high (more than 1650 mumol/l) serum triglyceride levels. 3. The serum PHLA did not show any definite correlation with the level of circulating triglycerides, although the lowest levels of PHLA were found in the malnourished children with highest triglyceride level. 4. While the hepatic PHLA in the malnourished children was significantly less than control value, the extrahepatic PHLA did not differ significantly. 5. After treatment, serum PHLA rose significantly and the mean levels were within normal range. 6. Our findings suggest that a defect in catabolism of very-low-density lipoprotein caused by a low hepatic PHLA may cause hypertriglyceridaemia in children with kwashiorkor.
摘要
  1. 测定了夸休可尔症患儿治疗前后以及正常对照儿童的血清肝素后脂解活性(PHLA)、甘油三酯和游离脂肪酸浓度。2. 将对照儿童血清甘油三酯范围(571 - 1650 μmol/l)视为正常范围,25例夸休可尔症患儿中,5例(20%)血清甘油三酯水平低(低于570 μmol/l),13例(52%)正常(571 - 1650 μmol/l),7例(28%)高(高于1650 μmol/l)。3. 血清PHLA与循环甘油三酯水平未显示出任何明确的相关性,尽管在甘油三酯水平最高的营养不良儿童中发现了最低的PHLA水平。4. 营养不良儿童的肝脏PHLA显著低于对照值,而肝外PHLA无显著差异。5. 治疗后,血清PHLA显著升高,平均水平在正常范围内。6. 我们的研究结果表明,肝脏PHLA降低导致极低密度脂蛋白分解代谢缺陷可能是夸休可尔症患儿高甘油三酯血症的原因。

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