Manifestaciones renales en pacientes con síndrome de Alagille: Reporte de casos y revisión de la literatura.

Abstract

Alagille syndrome is an autosomal dominant condition defined clinically by changes in five major systems: lack of bile ducts with chronic cholestasis, cardiac involvement (pulmonary stenosis), bone (butterfly vertebrae), ophthalmic (posterior embryotoxon) and the face (slight but recognizable dysmorphic facies). Renal involvement is common in these patients, but not part of the criteria for the syndrome. We report the cases of 3 patients with Alagille syndrome and renal involvement and conducted a literature review to establish the importance of including the evaluation of this system in the diagnosis of the syndrome. We conclude that renal involvement is common, and therefore suggest that in all cases to evaluate the possibility of renal involvement at both structural and glomerular and tubular function