唐氏综合征、短暂性骨髓增殖性疾病和婴儿肝纤维化。
Down syndrome, transient myeloproliferative disorder, and infantile liver fibrosis.
作者信息
Schwab M, Niemeyer C, Schwarzer U
机构信息
Dr. Margarete Fischer-Bosch Institute of Clinical Pharmacology, Stuttgart, Germany.
出版信息
Med Pediatr Oncol. 1998 Sep;31(3):159-65. doi: 10.1002/(sici)1096-911x(199809)31:3<159::aid-mpo6>3.0.co;2-a.
BACKGROUND
In neonates, Down syndrome is rarely accompanied by the leukemoid reaction called transient myeloproliferative disorder.
PROCEDURES AND RESULTS
We present clinical and histopathologic data of another Down syndrome neonate with transient myeloproliferative disorder and severe infantile liver fibrosis. These findings in our patients are compared in detail with the 20 cases published previously. Similar clinical and laboratory findings were present in all 21.
CONCLUSIONS
Knowing the cellular mechanism of hepatic fibrosis and its modulation by growth factors (e.g, platelet-derived growth factor), a pathogenetic link between transient myeloproliferative disorder and the development of liver fibrosis in Down syndrome neonates seems probable. An association of this triad of findings no longer appears to be accidental.
背景
在新生儿中，唐氏综合征很少伴有称为暂时性骨髓增殖性疾病的类白血病反应。
过程与结果
我们展示了另一例患有暂时性骨髓增殖性疾病和严重婴儿肝纤维化的唐氏综合征新生儿的临床和组织病理学数据。我们将患者的这些发现与之前发表的20例病例进行了详细比较。所有21例病例都有相似的临床和实验室检查结果。
结论
了解肝纤维化的细胞机制及其受生长因子（如血小板衍生生长因子）的调节后，唐氏综合征新生儿的暂时性骨髓增殖性疾病与肝纤维化发展之间的发病机制联系似乎是可能的。这三项发现之间的关联似乎不再是偶然的。
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