Sulphated lithocholic acid conjugates in serum from children with hepatic and intestinal diseases.

Sulphated lithocholic acid conjugates (SGLC) were measured in the sera of 268 children with various hepatic and intestinal disorders. Two groups were distinguished: (I) SGLC concentration less than or equal to 1.2 mumol/l, n = 198, and (II) SGLC concentration greater than 1.2 mumol/l, n = 70. In 28 patients of the latter group the SGLC concentration was less than 25% of the concentration of glycocholic acid (GC) in the same serum sample. This group (IIA) consisted predominantly of patients with cholestasis, as characterized by high serum bile acid levels and deviating liver function tests. The rest of the group (IIB), with SGLC levels exceeding 25% of the GC concentration and relatively low serum bile acid concentrations, showed no clear cholestatic symptoms. A postprandial increase in serum SGLC (delta SGLC) greater than 1.0 mumol/l was found in only 1 of 32 patients of group I (3%), in 1 of 6 patients of group IIA (17%), but in 9 of 11 patients of group IIB (81%). delta SGLC did not correlate with delta GC in the same test, which indicated that a general hepatic bile acid clearance defect was not responsible. In two patients with intermittent cholestasis, the distinct postprandial rise in serum SGLC that was always found during anicteric periods could be prevented by adding cholestyramine to the test meal. We conclude that elevated serum concentrations of SGLC develop during the course of cholestasis but may also be caused by influx of this bile acid from the intestine. Because of its hepatotoxic properties, SGLC may be involved in the initiation or perpetuation of specific cholestatic phenomena.