FATTY-ACID CONTENT IN LYMPHOCYTES FROM CHILDREN WITH SYNDROMIC PAUCITY OF INTERLOBULAR BILE-DUCTS, ALAGILLE SYNDROME

Fatty acid (FA) concentrations were studied in lymphocytes isolated fr om children with syndromic paucity of interlobular bile ducts (PILED), Alagille syndrome. The aim of this study was to assess whether the sp ecific FA changes previously observed in fibroblast cultures from such patients were also present in other tissues. Lymphocyte FA, obtained both from controls and patients were studied under two experimental co nditions, either after separation of the mononuclear cells or after 48 hours of culture. Freshly isolated lymphocytes from patients presente d few FA changes compared to the controls. However, when patient lymph ocytes were placed in culture medium for 48 hours, FA changes were amp lified compared to those observed in controls; the decrease in the sum of saturated and n-6 polyunsaturated FA of total lipids was significa nt only in patients, and the n-3 FA of phospholipids was strikingly in creased in patients (p less than or equal to 0.001), compared to contr ols. These results are related to those previously observed in fibrobl ast cultures and suggest that placing cells in culture could reveal a pre-existing cellular abnormality in patients with PILED.