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唐氏综合征婴儿新生儿期的血清血小板生成素水平和血小板减少症。

Serum thrombopoietin level and thrombocytopenia during the neonatal period in infants with Down's syndrome.

机构信息

Department of Pediatrics, Nishi-Kobe Medical Center, Kobe, Hyogo, Japan.

出版信息

J Perinatol. 2010 Feb;30(2):98-102. doi: 10.1038/jp.2009.120. Epub 2009 Aug 13.

Abstract

BACKGROUND

The pathogenesis of thrombocytopenia during the neonatal period in Down's syndrome (DS) infants remains unclear.

OBJECTIVE

To elucidate kinetic changes of serum thrombopoietin (TPO) level and platelet count, and their correlation in DS neonates.

STUDY DESIGN

Twelve DS infants (male/female: 7/5, term/late preterm: 10/2) born between 1997 and 2007 were included. Blood samples were serially collected during the neonatal period and serum TPO levels were determined in 44 sera using an enzyme-linked immunosorbent assay.

RESULTS

Thrombocytopenia <150 x 10(9) per liter was observed in seven (58%) patients. In 12 DS patients, the median TPO value showed 2.86 fmol ml(-1) on day 0, rose to 4.64 fmol ml(-1) on day 2, and thereafter decreased to 4.30 fmol ml(-1) on day 5, 2.40 fmol ml(-1) on days 11-15, and 1.75 fmol ml(-1) on days 28-30. This kinetics parallels that in historical non-DS controls. In 35 pair sample analysis from 11 patients without transient myeloproliferative disease, TPO level inversely correlated with platelet count (r=-0.38, P=0.023). However, there was no significant difference in TPO concentrations between thrombocytopenic and non-thrombocytopenic DS individuals.

CONCLUSIONS

This is the first study to describe the relationship between TPO level and platelet count in neonates with DS. Median TPO levels and their kinetic changes in DS neonates are comparable to those in non-DS controls. In contrast to earlier findings in several studies showing higher TPO concentrations in thrombocytopenic non-DS newborns than those in non-thrombocytopenic counterparts, the response of the TPO system to thrombocytopenia in DS during the neonatal period seems suboptimal.

摘要

背景

唐氏综合征(DS)新生儿期血小板减少症的发病机制尚不清楚。

目的

阐明 DS 新生儿血清促血小板生成素(TPO)水平和血小板计数的动态变化及其相关性。

研究设计

纳入 1997 年至 2007 年间出生的 12 例 DS 婴儿(男/女:7/5,足月/晚期早产儿:10/2)。在新生儿期连续采集血样,用酶联免疫吸附法检测 44 例血清中 TPO 水平。

结果

7 例(58%)患者血小板计数<150×109/L。在 12 例 DS 患儿中,TPO 值中位数在第 0 天为 2.86 fmol/ml,第 2 天升至 4.64 fmol/ml,第 5 天降至 4.30 fmol/ml,第 11-15 天降至 2.40 fmol/ml,第 28-30 天降至 1.75 fmol/ml。这种动力学与非 DS 对照组的动力学相似。在 11 例无短暂性骨髓增生性疾病的患者中,35 对样本分析显示 TPO 水平与血小板计数呈负相关(r=-0.38,P=0.023)。然而,血小板减少性和非血小板减少性 DS 个体之间的 TPO 浓度无显著差异。

结论

这是第一项描述 DS 新生儿 TPO 水平与血小板计数之间关系的研究。DS 新生儿的 TPO 水平中位数及其变化规律与非 DS 对照组相似。与几项研究的早期发现相反,即血小板减少性非 DS 新生儿的 TPO 浓度高于非血小板减少性新生儿,DS 新生儿在新生儿期对血小板减少的 TPO 系统反应似乎不理想。

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