Features of Alagille syndrome in 92 patients: frequency and relation to prognosis.

@article{Emerick1999FeaturesOA,
  title={Features of Alagille syndrome in 92 patients: frequency and relation to prognosis.},
  author={Karan McBride Emerick and Elizabeth B. Rand and Elizabeth Goldmuntz and Ian D. Krantz and Nancy B. Spinner and David A. Piccoli},
  journal={Hepatology},
  year={1999},
  volume={29 3},
  pages={822-9}
}
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… CONTINUE READING
Highly Influential
This paper has highly influenced 17 other papers. REVIEW HIGHLY INFLUENTIAL CITATIONS