Dormann Sabine, Krüger Marcus, Hentschel Roland, Rasenack Regina, Strahm Brigitte, Kontny Udo, Niemeyer Charlotte
Department of Paediatrics and Adolescent Medicine, University of Freiburg, Mathildenstrasse 1, 79106 Freiburg, Germany.
Eur J Pediatr. 2004 Jul;163(7):374-7. doi: 10.1007/s00431-004-1452-7. Epub 2004 Apr 28.
Neonates with Down syndrome can present with a haematological disorder called transient abnormal myelopoiesis (TAM). While TAM is usually a self-limiting disease, patients with severe complications such as hydrops fetalis, cardiorespiratory failure and liver fibrosis have been described. Here, we present five consecutive neonates with trisomy 21 and TAM, four of whom were critically ill and were therefore treated with cytosine-arabinoside. All five patients survived.
severely affected neonates with Down syndrome and transient abnormal myelopoiesis might benefit from early cytostatic treatment with cytosine-arabinoside.
患有唐氏综合征的新生儿可能会出现一种名为短暂异常髓系造血（TAM）的血液系统疾病。虽然TAM通常是一种自限性疾病，但已有出现诸如胎儿水肿、心肺衰竭和肝纤维化等严重并发症的患者的报道。在此，我们报告了5例连续的21三体综合征合并TAM的新生儿，其中4例病情危急，因此接受了阿糖胞苷治疗。所有5例患者均存活。
患有唐氏综合征且有短暂异常髓系造血的严重受影响新生儿可能受益于早期使用阿糖胞苷进行的细胞抑制治疗。
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