Prenatal sonographic findings and hematological abnormalities in fetuses with transient abnormal myelopoiesis with Down syndrome
Prenatal sonographic findings and hematological abnormalities in fetuses with transient abnormal myelopoiesis with Down syndrome
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DOI：
10.1002/pd.1718
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发表时间：
2007-06-01
影响因子：
3
通讯作者：
Wake, Norio
中科院分区：
文献类型：
--
作者：
Hojo, Satoshi;Tsukimori, Kiyomi;Wake, Norio
目的探讨短暂性骨髓增生异常（TAM）的相关产前表现对预后的影响。方法回顾性分析所有确诊TAM合并唐氏综合征患者的产前和产后医疗记录，重点分析产前超声检查、胎儿血液分析、新生儿结局和死亡原因。结果1992年1月至2005年12月，7例出生后确诊TAM合并唐氏综合征。超声检查显示4例水肿伴肝肿大，7例中2例孤立性肝肿大。没有发现提示心力衰竭的病例积液。胎儿血液分析显示6例肝酶水平升高，4例低白蛋白血症。超声检查结果与胎儿血液检查结果的比较显示了水肿和低白蛋白血症之间的联系。7例中有4例死亡。所有死亡病例都与水肿有关，死亡的主要原因是肝衰竭引起的凝血功能障碍，这可能是由胚细胞浸润肝脏引起的。结论胎儿TAM与肝肿大及肝酶水平升高有关。具有预后意义的产前发现是水肿，这可能是由于肝功能衰竭引起的低白蛋白血症。版权所有(c) 2007约翰威利父子有限公司
Objectives To determine relevant prenatal findings of transient abnormal myelopoiesis (TAM) that have important prognostic implications.Methods The prenatal and postnatal medical records of all cases with confirmed TAM associated with Down syndrome were reviewed retrospectively, with emphasis on prenatal sonographic findings, fetal blood analysis, neonatal outcomes, and causes of death.Results From January 1992 to December 2005, seven cases were confirmed postnatally as having TAM associated with Down syndrome. Sonography demonstrated hydrops with hepatomegaly in four, and isolated hepatomegaly in two of these seven cases. There were no findings suggestive of cardiac failure in cases of hydrops. Fetal blood analysis revealed elevated liver enzyme levels in six cases and hypoalbuminemia in four cases. Comparison of sonographic findings with fetal blood findings demonstrated an association between hydrops and hypoalbuminemia. Four of the seven cases were fatal. All fatal cases were associated with hydrops and the main cause of death was coagulopathy due to liver failure, which may have resulted from infiltration of the liver by blast cells.Conclusions Fetal TAM is associated with hepatomegaly and elevated liver enzyme levels. The prenatal finding with prognostic implications is hydrops, which may result from hypoalbuminemia due to liver failure. Copyright (c) 2007 John Wiley & Sons, Ltd.