A fetal case of transient abnormal myelopoiesis with severe liver failure in down syndrome: Prognostic value of serum markers
A fetal case of transient abnormal myelopoiesis with severe liver failure in down syndrome: Prognostic value of serum markers
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DOI：
10.1080/08880010490277088
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发表时间：
2004-04-01
影响因子：
1.7
通讯作者：
Yamashiro, Y
中科院分区：
文献类型：
--
作者：
Shiozawa, Y;Fujita, H;Yamashiro, Y
作者最近遇到了一个致命的唐氏综合征伴有短暂性骨髓增生异常（TAM）的病例。虽然在没有特异性治疗的情况下，患者外周血白细胞计数和母细胞计数有所改善，但患者在5岁时死于肝纤维化引起的严重凝血功能障碍。TAM合并肝纤维化预后较差。患者III型前胶原蛋白、IV型胶原蛋白n端肽、透明质酸含量高。这些血清生成物是肝纤维化的无创指标，可作为唐氏综合征TAM的预后指标。
The authors recently encountered a lethal case of Down syndrome with transient abnormal myelopoiesis (TAM). Although the peripheral white blood cell count and blast cells had improved without specific treatment, the patient died of severe coagulopathy due to liver fibrosis when he was 5 years old. The prognosis of TAM with liver fibrosis was poor. The patient had high levels of N-terminal peptide of III procollagen, type IV collagen, and hyaluronic acid. These serum makers are noninvasive indicators of liver fibrosis and may be useful as prognostic indicators of TAM in Down syndrome.