Hemophagocytic Syndrome Presenting as Severe Acute Hepatitis / 대한소아소화기영양학회지
Korean Journal of Pediatric Gastroenterology and Nutrition
;
: 213-221, 2005.
Artículo
en Coreano
| WPRIM
| ID: wpr-27834
ABSTRACT
PURPOSE:
Hemophagocytic syndrome (HPS) is characterized by persistent high fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, and/or hypofibrinogenemia. Hepatic manifestations including overt hepatic failure and fulminant hepatitis are common in HPS. Liver transplantation (LT) should be considered in a case of fulminant hepatitis by other than HPS, but LT is contraindicated and complete cure is possible by chemotherapy in HPS. Therefore, we conducted this study to define the characteristics of HPS presenting as severe acute hepatitis.METHODS:
Among the total of 23 patients diagnosed as HPS by bone marrow examination between 1994 and 2005 in Asan Medical Center, 11 cases presented as severe acute hepatitis were enrolled in this study. We analyzed the clinical features, laboratory findings and outcome retrospectively.RESULTS:
Seven (64%) of the 11 children with HPS and hepatitis were referred to pediatric gastroenterologist at first. The mean age of onset was 50 months. There was no case with family history of primary HPS. Epstein-Barr virus was positive in 4, and herpes Simplex virus was positive simultaneously in 1 case. As the presenting symptoms and signs, fever was present in 10, hepatosplenomagaly was noted in all and jaundice in 10. Anemia was observed in 10, thrombocytopenia in 10, leukopenia in 8, hypertriglyceridemia in 9, hypofibrinogenemia in 8 and hyperferritinemia in 7 cases, respectively. Nine children received chemotherapy including etopside. The overall mortality rate was 72% (8/11).CONCLUSION:
HPS, which needs chemotherapy, should be considered as a cause of severe acute hepatitis especially when accompanied with prolonged high fever and cytopenias.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Trombocitopenia
/
Examen de la Médula Ósea
/
Hipertrigliceridemia
/
Estudios Retrospectivos
/
Mortalidad
/
Trasplante de Hígado
/
Fallo Hepático
/
Simplexvirus
/
Edad de Inicio
/
Herpesvirus Humano 4
Tipo de estudio:
Estudio observacional
/
Estudio pronóstico
Límite:
Niño
/
Humanos
Idioma:
Coreano
Revista:
Korean Journal of Pediatric Gastroenterology and Nutrition
Año:
2005
Tipo del documento:
Artículo
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