2014, Number 4
<< Back Next >>
Rev Mex Cir Pediatr 2014; 18 (4)
Prognostic Factors Related With Mortality In Children With Biliary Atresia
Monroy-Teniza ZA, Flores-Calderón J, Villasis-Keever MA, Ortiz-Galvan R, Oliver-García EF, Jean-Aureliusa P, Ayala-Hernández VI
Language: Spanish
References: 15
Page: 183-192
PDF size: 393.32 Kb.
ABSTRACT
Background. Biliary atresia (BA) is a destructive process, idiophatic and inflamatory, that affects the bile ducts intra and extrahepatic, resulting in
fibrosis, obliteration of biliary tract and biliary cirrhosis. It has high rates of mortality. It is the principal cause of liver transplantation in pediatrics.
Methods. Observational, longitudinal, analytic and retrospective study; retrospective cohort. Included newborns and infants with BA of 2008 to 2012,
excluded patients with acute liver failure or secondary cholestasis to other causes.
Results. Included 66 patients with BA, Kasai was performed in 47, and they had decreased bilirubin levels. Also, in the other group they had lower
levels of serum albumin and greater extension of PPT and INR. The survival in the whole group was 5.6 years; for transplant patients, of 1.9 years. The
rate of patients with Kasai that died, was lower than those that lived (36.4% vs 78.2%).
Conclusions. The most important factor related with the survival of these patients was to perform the Kasai surgery. The early acknowledgement of
this illness, and the timely surgical management, decreases the mortality and improves their quality of life.
REFERENCES
Balistreri WF, Bezerra Ja, Ryckman FC. Biliary atresia and other disorders of the extrahepatic bile ducts, En: Suchy FJ, Sokol RJ, Balistreri WF, Liver Disease in Children 3rd ed. USA 2007;247-269.
Balistreri WF, Grand R, Hoofnagle JH. Biliary atreisa: current concepts and research directions. Summary of a symposium. Hepatology. 1996;23: 1682-1692.
Guideline for the evaluation of cholestatic jaundice in infants: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatologý and Nutrition. J. Pediatr Gastroenterol Nutr. 2004; 39: 115-128.
Strickland AD, Shannon K. Studies in the etiology of extrahepatic biliary atresia: time-space clustering. J. Pediat. 1982;100:749-753.
Yoon PW, Bresse JS, Olney RS, James LM, Khoury MJ. Epidemiology of biliary atresia: a population-based study. Pediatrics. 1997;99:376-382.
Ayas MF, Hillemeler AC, Olson AD. Lack of evidence for seasonal variation in extrahepatic biliary atresia during infancy. Clin J Gastroenterol. 1996; 22: 292-294.
Silveira TH, Salzano FM, Donaldson PT. Association between HLA and extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr. 1993; 16: 114-117.
Jurado A, Jara P, Camarena C. Is extrahepatic biliary atresia an HLA-associated disease? J Pediatr Gastroenterol Nutr. 1997; 25: 557-558.
Ulrich B, Ure B. Biliary atresia. Clin Res Hepatol Gastroenterol. 2012;36:257-259.
Davenport M, Savage M, Mowat AP, Howard ER. Biliary atresia splenic malformation syndrome: an etiologic and prognostic subgroup. Surgery. 1993;113:662-668.
Ibrahim M, Miyano T, Ohi R. Japanese Biliary atresia Registry, 1989 to 1994. Tohoku J Exp Med. 1997;181: 85-95.
Sokol RJ, Mack C, Narkewicz. Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr. 2003;37:4-21.
Moreira RK1, Cabral R, Cowles RA, Lobritto SJ. Biliary atresia: a multidisciplinary approach to diagnosis and management. Arch Pathol Lab Med. 2012;136:746-760.
Guo C, Zhu J, Pu CL. Combinatory effects of hepatic CD8* and NK lymphocytes in bile duct injury from biliary atresia. Pediatr Res. 2012;1-7.
Wildhaber BE. Screening for biliary atresia: Swiss stool color card. Hepatology 2011;54:367-368.