Abstract

ROMAN CASTELLINI, Estela V; UMPIERREZ GARCIA, Ibis; PONCE RODRIGUEZ, Félix M  and  LOPEZ ZAYAS, María de los Ángeles. Evolution of a pediatric patient with Alagille’s Syndrome. Report of a case. Rev. Med. Electrón. [online]. 2012, vol.34, n.2, pp. 231-240. ISSN 1684-1824.

The Alagille’s syndrome is a few frequent congenital disease; it is transmitted in a dominant autosomal way, with variable expressivity. It characterizes for presenting cholestasis, vertebral and ocular anomalies, congenital cardiopathies and facial dysmorphias. The prognosis of this syndrome is variable, mainly depending of the hepatic injury and the associated cardiovascular defects. We presented the case of a patient with the diagnosis of Alagille’s Syndrome and stable evolution.

Keywords : hepatic damage; autosomal dominant; cardiovascular defects; congenital disease; child; Alagille’s Syndrome.

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