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Correctable plasma lipoprotein abnormalities in infants with choledochal cysts
Insights
Children with biliary obstruction exhibit abnormal plasma lipoproteins, similar to adults. Surgical correction rapidly reverses these lipid alterations, indicating potential prognostic value for hepatic function outcomes.
Area of Science:
- Biochemistry
- Pediatric Gastroenterology
- Hepatology
Background:
- Biliary obstruction in children, often due to choledochal cysts, can lead to significant hepatic dysfunction.
- Lipoprotein abnormalities are characteristic of cholestatic liver disease in adults, but pediatric data are less defined.
Observation:
- Two pediatric patients with choledochal cyst-related biliary obstruction were studied pre- and post-surgical correction.
- Plasma lipid profiles, including cholesterol, phospholipids, triglycerides, and specific lipoprotein levels (HDL, ApoA-I), were analyzed.
- Hepatic function was monitored using standard biochemical markers (SGPT, GGTP, bilirubin, etc.).
Findings:
- Pre-surgery, both patients showed elevated plasma lipids, low cholesterol esters, subnormal HDL cholesterol and ApoA-I, and the presence of lipoprotein-X.
- Post-surgery, patient B (less hepatic injury) normalized lipid profiles and improved hepatic function within one week.
- Patient A (severe hepatic injury) showed minimal improvement at one week, with near-normal levels and function by five weeks.
Implications:
- Pediatric biliary obstruction causes lipoprotein abnormalities analogous to those in adults.
- Lipoprotein profile normalization post-surgery correlates with hepatic recovery.
- Lipoprotein analysis may serve as a prognostic indicator for children undergoing surgical correction for biliary obstruction.
Abstract:
Plasma lipoproteins from two female patients--patient A, 4 wk and patient B, 19 months--were examined prior to and at 1 and 5 wk after surgical correction of biliary obstruction due to choledochal cyst. The findings were correlated with standard indices of hepatic function, namely SGPT, GGTP, 5'nucleotidase, serum bile salts, and total and conjugated bilirubin. Prior to surgery in both patients plasma cholesterol, phospholipid, and triglyceride were elevated; cholesterol esters were low; high-density lipoprotein (HDL) cholesterol and apolipoprotein A-I, the major protein constituent of HDL, were subnormal; most of the plasma lipids were contained in the low-density lipoprotein density region; lipoprotein-X was present. Patient B had had a relatively brief obstruction and suffered little secondary hepatic injury. One week after surgery, plasma lipid concentrations returned to normal; apolipoprotein A-I increased in the HDL density region and a concomitant rise in cholesterol esters to near normal, 65%, was observed; plasma lipids were contained predominantly in HDL; hepatic function improved markedly. Patient A had had intrauterine obstruction and suffered major hepatic injury with cirrhosis. One week after surgery, plasma lipid concentrations, cholesterol esters, low-density lipoprotein lipid predominance, and hepatic function remained essentially unchanged. Five weeks after surgery, the lipoprotein levels and composition and hepatic function were near normal. In conclusion, children with biliary obstruction have lipoprotein abnormalities similar to those seen in adult patients. These alterations are rapidly reversible with surgical relief and may be used as prognostic indicators of outcome.