ACUTE LIVER FAILURE WITH ASSOCIATED APLASTIC ANEMIA IN CHILDREN
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Tipo de produção
conferenceObject
Data de publicação
2013
Título da Revista
ISSN da Revista
Título do Volume
Editora
WILEY-BLACKWELL
Citação
PEDIATRIC TRANSPLANTATION, v.17, n.2, p.194-194, 2013
Resumo
OBJECTIVES: Herein we describe the experience of six cases of acute liver failure with associated aplastic anemia treated at ICR – HCFMUSP. MATERIAL AND METHODS: We reviewed the records of patients undergoing liver transplantation for acute liver failure with associated aplastic anemia treated at ICR–HCFMUSP between 2007 and 2012. We collected information regarding the type of treatment offered, diagnosis, bone marrow recovery time, interval between onset of symptoms until liver transplantation and the diagnosis of bone marrow aplasia. RESULTS: In this period we found five patients who met the criteria established, four boys and a girl. The mean age was 9.6 yrs (9–12). The time between onset of symptoms and liver transplantation was 25.2 days (17–30) and until the appearance of pancytopenia 35 days (20–45). Two patients were treated with thymoglobulin and median time to recovery of bone marrow function after treatment was 17.5 days. One patient died from sepsis after treatment and another from bleeding without taking thymoglobulin. CONCLUSION: Aplastic anemia is a potentially fatal complication associated with acute liver failure. There is no standardized treatment in the literature. Reports show cases of liver and bone marrow recovery after immunosuppressive therapy with anti-thymocyte globulin and anti-lymphocytes despite the risk inherent in the use of such substances in critically ill patients the presence of large numbers of activated T lymphocytes in the marrow and liver biopsies from these patients corroborates this therapeutic. In severe cases of aplastic anemia with HLA compatible sibling hematopoietic stem cell transplantation has been performed successfully in the treatment of this condition.