Differential clinical characteristics of acute liver failure caused by hemophagocytic lymphohistiocytosis in children.

Ryu, Jeong‐Min; Kim, Kyung Mo; Oh, Seak Hee; Koh, Kyung Nam; Im, Ho Joon; Park, Chan‐Jeoung; Chi, Hyun‐Sook; Seo, Jong Jin

Background Children with acute liver failure ( ALF) caused by hemophagocytic lymphohistiocytosis ( HLH) may be at risk of undergoing unnecessary liver transplantation ( LT). The aim of this study was to compare the characteristics of ALF caused by HLH with those of ALF of unknown etiology in children. Methods The clinical features and laboratory findings for eight children with ALF caused by HLH ( ALF-HLH group) and 27 children with ALF of unknown etiology ( ALF-UK group) were retrospectively compared by reviewing medical records. Results The ALF-HLH group had a higher incidence of pleural effusion, C-reactive protein elevation (especially >5 mg/dL), thrombocytopenia, anemia, fever, splenomegaly, and hypoalbuminemia (<2.5 mg/dL), and a higher in-hospital mortality rate. No significant differences were found in the white blood cell count, liver enzymes, coagulation profile, or incidence of hepatomegaly. Conclusions LT should be performed only after it is proven that ALF is not caused by HLH, if a child with ALF shows the differential clinical features of ALF caused by HLH. Further research with larger sample sizes, however, is needed.

SOUTH Korea; ANEMIA; C-reactive protein; CHI-squared test; CONFIDENCE intervals; FEVER; HOSPITALS; LIVER failure; LIVER transplantation; DEATH rate; PLEURAL effusions; HEMOPHAGOCYTIC lymphohistiocytosis; SPLEEN diseases; UNNECESSARY surgery; THROMBOCYTOPENIA; U-statistics; DISEASE incidence; DATA analysis software; DESCRIPTIVE statistics; DISEASE complications; SYMPTOMS; CHILDREN

Pediatrics International, 2013, Vol 55, Issue 6, p748

1328-8067

Academic Journal

10.1111/ped.12181