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高铁血红蛋白血症合并 G6PD 缺乏症:新生儿持续低饱和度的罕见病因。

Methaemoglobinaemia with G6PD deficiency: rare cause of persistently low saturations in neonates.

机构信息

Department of Paediatrics, Sandwell & West Birmingham NHS Hospitals Trust, Birmingham, UK.

出版信息

Acta Paediatr. 2011 Jul;100(7):e47-8. doi: 10.1111/j.1651-2227.2011.02278.x.

Abstract

UNLABELLED

We report a very rare case of methaemoglobinaemia associated with glucose 6 phosphate dehydrogenase (G6PD) deficiency, complicating a respiratory illness in a preterm neonate. This neonate had consistently low saturation readings despite being ventilated at moderately high pressures in 100% oxygen. An arterial blood gas confirmed a high methaemoglobin level and a high pO2, inconsistent with the saturations. In addition, the bilirubin increased to exchange levels and was difficult to control with quadruple phototherapy. A double volume exchange transfusion was performed, which reduced both bilirubin and methaemoglobin. The pulse oximetry then started to correlate well with pO2. G6PD deficiency was confirmed.

CONCLUSION

Paediatricians should remember that methaemoglobinaemia is a rare but important cause of persistently low saturations, and exchange transfusion is a reliable treatment for this condition.

摘要

未标注

我们报告了一例非常罕见的葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症相关的高铁血红蛋白血症,并发早产儿呼吸疾病。尽管在 100%氧气中以中等高压进行通气,但该新生儿的饱和度读数一直较低。动脉血气证实高铁血红蛋白水平高,pO2 高,与饱和度不一致。此外,胆红素增加到交换水平,并用四倍光疗难以控制。进行了双容量交换输血,降低了胆红素和高铁血红蛋白。脉搏血氧饱和度计随后开始与 pO2 良好相关。证实存在 G6PD 缺乏症。

结论

儿科医生应记住,高铁血红蛋白血症是持续低饱和度的罕见但重要原因,而交换输血是该病症的可靠治疗方法。

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