title: Retrospective analysis of risk factors for development of liver dysfunction in transient leukemia of Down syndrome.
abstract: About 20% of patients with transient leukemia (TL), which is a disease noted in Down syndrome, are reported to develop hepatic fibrosis, which has a poor prognosis. The clinical factors related to the poor prognosis of TL were retrospectively analyzed in 25 patients, and criteria for starting chemotherapy were established. The initiation of chemotherapy was recommended when two or more of the following categories were fulfilled in the process of the disease: (1) a reduced hepatic functional reserve estimated by direct bilirubin, prothrombin time, and the presence of ascites, (2) an elevated level of hyaluronic acid (>500 U/mL), (3) respiratory failure or poor sucking associated with hepatosplenomegaly, and (4) demonstration of fibrosis by liver biopsy. When these criteria were applied to our cases, all patients who received chemotherapy remained alive. Our criteria are useful for selecting patients with TL at high risk of developing hepatic fibrosis and for starting chemotherapy.
authors.affiliation: Department of Pediatric Interdisciplinary Medicine, Nagano Children's Hospital, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Hirabayashi
authors.forename: Masaaki
authors.initials: M
authors.lastname: Shiohara
authors.forename: Daijiro
authors.initials: D
authors.lastname: Takahashi
authors.forename: Shoji
authors.initials: S
authors.lastname: Saito
authors.forename: Miyuki
authors.initials: M
authors.lastname: Tanaka
authors.forename: Ryu
authors.initials: R
authors.lastname: Yanagisawa
authors.forename: Kazuo
authors.initials: K
authors.lastname: Sakashita
authors.forename: Tomohiko
authors.initials: T
authors.lastname: Nakamura
authors.forename: Eizaburo
authors.initials: E
authors.lastname: Ishii
authors.forename: Kenichi
authors.initials: K
authors.lastname: Koike
mesh_terms: D003561:Cytarabine / Q000627:therapeutic use; D018450:Disease Progression; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006529:Hepatomegaly / Q000150:complications; D006801:Humans; D006820:Hyaluronic Acid / Q000097:blood; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D007938:Leukemia / Q000150:complications*; D008103:Liver Cirrhosis / Q000150:complications; D008107:Liver Diseases / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D008297:Male; D012120:Respiration Disorders / Q000150:complications; D012189:Retrospective Studies; D012307:Risk Factors; D013163:Splenomegaly / Q000150:complications
chemical_list: D007166:Immunosuppressive Agents; D003561:Cytarabine; D006820:Hyaluronic Acid