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Abnormal lipoprotein pattern in patients with Alagille syndrome depends on Icterus severity

A Davit-Spraul1, M L Pourci, V Atger

  • 1Laboratoire de Biochimie I, Hôpital Bicêtre, France.

Gastroenterology
|October 1, 1996
PubMed

Insights

Alagille syndrome in children causes lipid abnormalities linked to jaundice severity. Lecithin/cholesterol acyltransferase (LCAT) activity variations due to jaundice impact abnormal lipid profiles in these patients.

Area of Science:

  • Biochemistry
  • Pediatric Gastroenterology
  • Clinical Lipidology

Background:

  • Alagille syndrome is characterized by lipid abnormalities that vary with the severity of icteric periods.
  • Understanding these lipid differences is crucial for managing the condition in pediatric patients.

Purpose of the Study:

  • To determine the lipoprotein profiles of 22 Alagille syndrome patients.
  • To compare these profiles with the severity of jaundice to identify distinct lipid abnormalities.

Main Methods:

  • Analysis of plasma lipids, apolipoproteins (apos), isolated lipoprotein composition, and lecithin/cholesterol acyltransferase (LCAT) activity.
  • Classification of patients into two groups based on total bilirubin levels (<100 mumol/L and >100 mumol/L).

Main Results:

  • Patients with lower bilirubin levels (Group II) showed hypercholesterolemia with increased high-density lipoprotein (HDL) and apoAI/apoAII.
  • Patients with higher bilirubin levels (Group I) exhibited very low HDL cholesterol and apoAI/apoAII, with lipoprotein X contributing to hypercholesterolemia.
  • Group I patients had decreased LCAT activity, high unesterified cholesterol, and emergent lipoprotein X; both groups had elevated apoE, apoCII, apoCIII, and phospholipid-enriched lipoproteins.

Conclusions:

  • Variations in lecithin/cholesterol acyltransferase (LCAT) activity, influenced by jaundice severity, are implicated in the abnormal lipid profiles observed in Alagille syndrome.
  • These findings highlight the critical role of jaundice in shaping lipid metabolism disturbances in pediatric Alagille syndrome.
Abstract

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