El síndrome de Alagille en Cuba. Informe de 9 casos.

Translated title of the contribution: Alagille's syndrome in Cuba. A report of 9 cases

C. Castañeda, T. Fragoso, B. Gra, L. Guerra, O. Castellanos, M. E. Trujillo

Research output: Contribution to journalReview articlepeer-review

6 Scopus citations


Alagille's syndrome or arteriohepatic dysplasia has been described in Cuba in nine patients between nine months and 12 years of age (8 males and one female). Among the clinical features we found five major abnormalities: chronic cholestasis with neonatal jaundice (9/9), peculiar facies (9/9), peripheral pulmonary artery hypoplasia associated with cardiac murmur (6/9), butter-fly-like arch defects (4/9), and posterior embryotoxon (6/7). Two children had a severe xanthomatosis. Laparoscopy showed green hepatomegaly depending on the degree of cholestasis, and only one patient had incipient signs of micronodular cirrhosis. Liver histology showed a paucity of interlobular bile ducts. Survival was of 60%. One patient survived more than 30 years. Four patients died of liver carcinoma (unique report in infants), broncho-pneumonia, acute renal failure, and sudden death respectively. Among the minor features were mental retardation (5/9), a peculiar voice (3/9), growth retardation observed in some of our patients. This is the first report on Alagille's syndrome in Latin America, because so far reports have come only from Europe and North America.

Translated title of the contributionAlagille's syndrome in Cuba. A report of 9 cases
Original languageSpanish
Pages (from-to)341-346
Number of pages6
Issue number4
StatePublished - 1992
Externally publishedYes


Dive into the research topics of 'Alagille's syndrome in Cuba. A report of 9 cases'. Together they form a unique fingerprint.

Cite this