Features of Alagille syndrome in 92 patients: frequency and relation to prognosis
KM Emerick, EB Rand, E Goldmuntz, ID Krantz… - Hepatology, 1999 - journals.lww.com
We have studied 92 patients with Alagille syndrome (AGS) to determine the frequency of
clinical manifestations and to correlate the clinical findings with outcome. Liver biopsy
specimens showed paucity of the interlobular ducts in 85% of patients. Cholestasis was
seen in 96%, cardiac murmur in 97%, butterfly vertebrae in 51%, posterior embryotoxon in
78%, and characteristic facies in 96% of patients. Renal disease was present in 40% and
intracranial bleeding or stroke occurred in 14% of patients. The presence of intracardiac …
clinical manifestations and to correlate the clinical findings with outcome. Liver biopsy
specimens showed paucity of the interlobular ducts in 85% of patients. Cholestasis was
seen in 96%, cardiac murmur in 97%, butterfly vertebrae in 51%, posterior embryotoxon in
78%, and characteristic facies in 96% of patients. Renal disease was present in 40% and
intracranial bleeding or stroke occurred in 14% of patients. The presence of intracardiac …