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唐氏综合征中由羧基血红蛋白浓度反映的新生儿胆红素生成情况。

Neonatal bilirubin production, reflected by carboxyhaemoglobin concentrations, in Down's syndrome.

作者信息

Kaplan M, Vreman H J, Hammerman C, Stevenson D K

机构信息

Department of Neonatology, Shaare Zedek Medical Center, Jerusalem, Israel.

出版信息

Arch Dis Child Fetal Neonatal Ed. 1999 Jul;81(1):F56-60. doi: 10.1136/fn.81.1.f56.

Abstract

AIM

To determine whether increased bilirubin production, reflected by blood carboxyhaemoglobin (COHb) values, is responsible for hyperbilirubinaemia in cases of Down's syndrome with no obvious cause for excessive jaundice.

METHODS

Blood was sampled on the third day of life for COHb, total haemoglobin (tHb), and serum total bilirubin, from 19 consecutively born neonates with Down's syndrome (a subset of 34 term babies), who had developed hyperbilirubinaemia (serum bilirubin >/= 256 micromol), and from 32 term controls. COHb, measured by gas chromatography, was corrected for inspired CO (COHbc) and expressed as a percentage of tHb.

RESULTS

Significantly more of the Down's syndrome subset developed hyperbilirubinaemia than the controls (10/19 (52%) vs 7/32 (22%), relative risk 2.4, 95% confidence intervals (CI) 1.10 to 5.26). Third day serum bilirubin values (mean (SD)) were higher in the Down's syndrome neonates than in controls (214 +- 63 micromol/l vs 172 +- 54 micromol/l, respectively, p=0.015). Mean (SD) COHbc values were significantly higher in the Down's syndrome neonates than in controls (0.92 +- 0. 24% vs 0.63 +- 0.17%; p<0.0001). However, Down's syndrome neonates who became hyperbilirubinaemic had similar COHbc values to those who did not (0.87 +- 0.26% and 0.95 +- 0.23%, respectively). These values contrast with those of the controls, in whom a significant increase in COHbc was associated with hyperbilirubinaemia (0.74 +- 0. 15% vs 0.60 +- 0.16%, respectively; p<0.05). tHb values were similar in both groups.

CONCLUSIONS

Down's syndrome neonates had a greater risk of hyperbilirubinaemia, and higher COHbc values, than controls. However, excessive bilirubin production could not be exclusively responsible for the hyperbilirubinaemia. By inference, decreased bilirubin elimination probably plays a greater part in its pathogenesis than in controls. Down's syndrome neonates may have abnormal erythropoiesis, leading to increased haem turnover.

摘要

目的

通过血液中碳氧血红蛋白(COHb)值反映的胆红素生成增加,是否是唐氏综合征中无明显过度黄疸原因的高胆红素血症的病因。

方法

对19例连续出生的患有高胆红素血症(血清胆红素≥256微摩尔)的唐氏综合征新生儿(34例足月儿的一个子集)和32例足月儿对照在出生后第3天采集血样,检测COHb、总血红蛋白(tHb)和血清总胆红素。通过气相色谱法测量的COHb校正吸入的一氧化碳(COHbc),并表示为tHb的百分比。

结果

唐氏综合征子集发生高胆红素血症的比例明显高于对照组(10/19(52%)对7/32(22%),相对风险2.4,95%置信区间(CI)1.10至5.26)。唐氏综合征新生儿出生后第3天的血清胆红素值(均值(标准差))高于对照组(分别为214±63微摩尔/升对172±54微摩尔/升,p = 0.015)。唐氏综合征新生儿的平均(标准差)COHbc值显著高于对照组(0.92±0.24%对0.63±0.17%;p<0.0001)。然而,发生高胆红素血症的唐氏综合征新生儿的COHbc值与未发生者相似(分别为0.87±0.26%和0.95±0.23%)。这些值与对照组形成对比,对照组中COHbc的显著增加与高胆红素血症相关(分别为0.74±0.15%对0.60±0.16%;p<0.05)。两组的tHb值相似。

结论

唐氏综合征新生儿比对照组有更高的高胆红素血症风险和更高的COHbc值。然而,胆红素生成过多并非高胆红素血症的唯一原因。由此推断,胆红素清除减少在其发病机制中可能比对照组起更大作用。唐氏综合征新生儿可能存在异常的红细胞生成,导致血红素周转率增加。

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