id	author	title	date	pages	extension	mime	words	sentences	flesch	summary	cache	txt
cord-349285-zmp7sw5q	Koh, Kyung‐Nam	Clinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in Korea: report of a nationwide survey from Korea Histiocytosis Working Party	2014-07-03		.txt	text/plain	3939	220	43	BACKGROUND: We analyzed a nationwide registry of pediatric patients with hemophagocytic lymphohistiocytosis (HLH) in Korea to assess the clinical and genetic features and treatment outcomes in pediatric HLH. CONCLUSION: Our study showed the unique predominance of a UNC13D mutation and vulnerability to Epstein–Barr virus infection in Korean children with HLH and emphasizes the prognostic significance of age, liver dysfunction, and treatment responses in this disease. The case report form included information on demographic characteristics, clinical, laboratory, and radiological findings at presentation, genetic mutation analysis, type of treatment and responses to treatment, allogeneic hematopoietic stem cell transplantation, and survival outcomes. In the familial HLH group, only a coagulation abnormality was a marginally significant prognostic factor, and CNS involvement and age were not found to be significantly associated with survival outcome. CNS involvement was found to be a significant prognostic factor in our presumed secondary HLH group but was not associated with a poor outcome in the familial group.	./cache/cord-349285-zmp7sw5q.txt	./txt/cord-349285-zmp7sw5q.txt