Abstract
Syndromic paucity of bile ducts or “Alagille syndrome” is characterized by peculiar facies, chronic cholestasis, posterior embryotoxon, butterfly-like vertebral arch defects and peripheral pulmonary artery hypopiasia or stenosis. We present a two-year-old female child with the ‘partial’ or ‘incomplete’ Alagille syndrome. The child had three of the five major features of the syndrome. A brief review of literature of the syndrome is presented.
References
Alagille D, Estrada A, Hadchouel M, Gautier M, Odievre M, Dommergues JP. Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): Review of 80 cases.J Pediatr 1987; 110:195–200.
Marodi L, Rigo G, Kaposzta R. Picture of the month. Alagille syndrome (arteriohepatic dysplasia).Arch Pediatr Adolesc Med 1994; 148:287–288.
Martin SR, Garel L, Alvarez F. Alagille’s syndrome associated with cystic renal disease.Arch Dis Child 1996; 74:232–235.
Gilboa N, Hopp L, Agostini RM. IgA nephritis in a patient with Alagille syndrome and a transplanted liver.Pediatr Nephrol 1992; 6:559–561.
Berard E, Sarles J, Triolo V, Gagnadoux M-F, Wernert F, Hadchouel Met al. Renovascular hypertension and vascular anomalies in Alagille syndrome.Pediatr Nephrol 1998; 12:121–124.
Quiros-Tejeira RE, Ament ME, Heyman MB, Martin MG, Rosenthal P, Hall TRet al. Variable morbidity in Alagille syndrome: A review of 43 cases.J Pediatr Gastroenterol Nutr 1999; 29:431–437.
Hingorani M, Nischal KK, Davies A, Bentley C, Vivian A, Baker AJet al. Ocular abnormalities in Alagille syndrome.Ophthalmology 1999; 106:330–337.
Emerick KM, Rand EB, Goldmuntz E, Krantz ID, Spinner NB, Piccoli DA. Features of Alagille syndrome in 92 patients: Frequency and relation to prognosis.Hepatology 1999; 29:822–829.
Krantz ID, Piccoli DA, Spinner NB. Alagille syndrome.J Med Genet 1997; 34:152–157.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Shendge, H., Tullu, M.S., Shenoy, A. et al. Alagille syndrome. Indian J Pediatr 69, 825–827 (2002). https://doi.org/10.1007/BF02723701
Issue Date:
DOI: https://doi.org/10.1007/BF02723701