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Selective deficiency of hepatic triglyceride lipase and hypertriglyceridaemia in kwashiorkor
Insights
Kwashiorkor in children can lead to high triglyceride levels due to reduced hepatic postheparin lipolytic activity (PHLA). Treatment improved PHLA, suggesting a catabolic defect in very-low-density lipoprotein metabolism.
Area of Science:
- Biochemistry
- Pediatric Nutrition
- Metabolic Disorders
Background:
- Kwashiorkor is a severe form of malnutrition.
- Lipid metabolism is often disrupted in malnourished children.
Purpose of the Study:
- To investigate serum postheparin lipolytic activities (PHLA) and lipid profiles in children with kwashiorkor.
- To understand the role of PHLA in hypertriglyceridemia associated with kwashiorkor.
Main Methods:
- Measured serum PHLA, triglyceride, and free fatty acid concentrations in children with kwashiorkor before and after treatment.
- Compared measurements with healthy control children.
- Differentiated between hepatic and extrahepatic PHLA.
Main Results:
- Children with kwashiorkor exhibited varying triglyceride levels, with 28% showing hypertriglyceridemia.
- Hepatic PHLA was significantly lower in malnourished children compared to controls.
- Serum PHLA increased significantly after treatment, returning to normal levels.
Conclusions:
- A defect in hepatic PHLA may contribute to hypertriglyceridemia in kwashiorkor by impairing very-low-density lipoprotein catabolism.
- Treatment of kwashiorkor improves PHLA and lipid profiles.
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.