title: Liver disease is frequently observed in Down syndrome patients with transient abnormal myelopoiesis.
abstract: Transient abnormal myelopoiesis (TAM) in neonates with Down syndrome (DS) is characterized by the transient appearance of blast cells, which resolves spontaneously. Approximately 20 % of patients with TAM die at an early age due to organ failure, including liver disease. We studied 25 DS-TAM patients retrospectively to clarify the correlation between clinical and laboratory characteristics and liver diseases. Early death (<6 months of age) occurred in four of the 25 patients (16.0 %), and two of those four patients died due to liver failure. Although physiologic jaundice improved gradually after a week, all DS patients had elevated D-Bil levels during the clinical course of TAM, except one who suffered early death. The median peak day of the WBC count, total bilirubin (T-Bil) and D-Bil levels was: day 1 (range day 0-57), day 8 (range day 1-55), and day 17 (range 1-53), respectively. Our results reveal that all patients with DS-TAM may develop liver disease irrespective of the absence or presence of symptoms and risk factors for early death. In patients of DS-TAM, careful observation of the level of D-Bil is needed by at least 1 month of age for the detection of liver disease risk.
authors: Department of Hematology/Oncology, Gunma Children's Medical Center, 779 Shimohakoda, Hokkitsu, Shibukawa, Gunma, 377-8577, Japan, pakum-tky@umin.ac.jp.
authors: Myoung Ja
authors: MJ
authors: Park
authors: Manabu
authors: M
authors: Sotomatsu
authors: Kentaro
authors: K
authors: Ohki
authors: Kokoro
authors: K
authors: Arai
authors: Kenichi
authors: K
authors: Maruyama
authors: Tomio
authors: T
authors: Kobayashi
authors: Akira
authors: A
authors: Nishi
authors: Kiyoko
authors: K
authors: Sameshima
authors: Takeshi
authors: T
authors: Takagi
authors: Yasuhide
authors: Y
authors: Hayashi
mesh_terms: D018450:Disease Progression; D004314:Down Syndrome / Q000503:physiopathology*; D005260:Female; D048550:Hepatic Insufficiency / Q000151:congenital / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D006776:Hospitals, Pediatric; D006801:Humans; D007231:Infant, Newborn; D007564:Japan / Q000453:epidemiology; D007955:Leukemoid Reaction / Q000503:physiopathology*; D008099:Liver / Q000503:physiopathology*; D008297:Male; D009102:Multiple Organ Failure / Q000453:epidemiology / Q000209:etiology; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index