medigraphic.com
ISSN 1995-9427 (Print)

2016, Number 2

<< Back Next >>

Invest Medicoquir 2016; 8 (2)

Hepatic transplant in patients with Alagille syndrome

Bressler HN, Hernández RH, Moret VS, Domínguez CJ, Samada SM, Roque VA, Leal AG

Language: Spanish
References: 12
Page: 294-299
PDF size: 227.23 Kb.


ABSTRACT

The Alagille syndrome is a genetic dysfunction whose main manifestation is a chronic cholestasis caused by a hypoplasia of the intrahepatic bile ducts, also associated to renal, heart, skeletal congenital malformations and a peculiar phenotype. In the United States, it is assigned an incidence of 1:100000 in alive newborns. The histological lesions are described as a reduction in the relationship between the number of bile ducts and portal tracts. The indications for a transplant in patients with Alagille syndrome are reported as complex and polemic, located among the first five causes of hepatic transplant in children, and it is recommended when there is no medical control and treatment of the cholestasis and may appear an uncontrolled pruritus, osteopenia, spontaneous fractures, portal hypertension with varixes, cirrhosis or uncontrolled ascites.


REFERENCES

  1. Alagille D, Estrada A, Hadchouel M, Gautier M, Dommergues JP. Syndromic paucity of interlobular bile ductus (Alagille syndrome or arterihepatic dysplasia): Review of 80 cases. J Pediatr. 1987;110(2):195-200.

  2. Krantz ID, Piccoli DA, Spinner NB. Alagille syndrome. Med. Genet. 1997;34(2):152-57.

  3. Watson GH, Miller V. Arteriohepatic dysplasia: familiar pulmonary arterial stenosis with neonatal liver disease. Arch Dis Child. 1973;48(6):459-66.

  4. Schwartz R, Rehder K, Parsons DJ, Morrell DS. Intense pruritus and failure to thrive in Alagille síndrome. J. Am. Acad Dermatol. 2008;58(Suppl 2):9-11.

  5. Nagasaka H, Miida T, Hirano KI, Ota A, Murayamaa K, Yorifuji T, et al. Fluctuation of lipoprotein metabolism linked with bile acid-activated liver nuclear receptors in Alagille syndrome. Atherosclerosis. 2008;198(2):434-40.

  6. Benchimol EI, Walsh CM, Ling SC. Early diagnosis of neonatal cholestatic jaundice. Test at two weeks. Can Fam Physician. 2009;55(12):1184-92.

  7. Hermeziu B, Sanlaville D, Girard M, Léonard C. Heterozygous bile salt export pump deficiency: a possible genetic predisposition to transient neonatal cholestasis. J Pediatr Gastroenterol Nutr. 2009;42(1):114-6.

  8. Ciocca M, Álvarez F. Colestasis neonatal transitoria. Arch Argent Pediatr. 2011; 109(2):163-6.

  9. Arocena E, Machado K, Pírez M C, Montano A. Síndrome de Alagille. A propósito de un caso. Arch Pediatr Urug. 2010;81(3):158-62.

  10. Spinner N, Krantz I, Kamath B. Alagille Syndrome [homepage en Internet]. Gene reviews; may 19 2000 [citado 3 de marzo de 2011]. Disponible en: http://www.ncbi.nlm.nih.gov/sites/GeneTests

  11. Kamath BM, Schwarz KB, Hadzic N: Alagille syndrome and liver transplantation. J. Pediatr Gastroenterol. Nutr. 2010;50(1):11–15.

  12. Englert C, Grabborn E, Burdelski M, Ganschow R. Liver transplantation in children with Alagille syndrome: Indications and outcome. Pediatr Transplant. 2006; 10(2):154-58.




2020     |     www.medigraphic.com