3岁,缺氧,除氧外其他治疗

A. Porto, K. Levasseur
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引用次数: 0

摘要

背景:葡萄糖-6-磷酸酶脱氢酶(G6PD)缺乏症在暴露于氧化应激时引起急性溶血。高铁血红蛋白血症与G6PD缺乏症有关,认识到这一点对于避免进一步的并发症很重要。病例介绍:一个3岁的健康男孩出现在儿科急诊科,急性发作呼吸困难,口周发绀和缺氧对氧治疗无反应。输血填充红细胞后症状有所改善。实验室结果显著贫血和高铁血红蛋白血症。进一步的检查显示葡萄糖-6-磷酸酶脱氢酶缺乏。讨论:G6PD缺乏可引起急性溶血性贫血。高铁血红蛋白血症可加重紫绀。管理必须包括输血填充红细胞和避免亚甲基蓝,以防止进一步溶血。结论:G6PD缺乏症可在暴露于氧化应激后出现急性溶血性贫血和缺氧。偶尔高铁血红蛋白血症会加重症状。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A 3 Year Old with Hypoxia other Treatments Besides Oxygen
Background: Glucose-6-phosphatase Dehydrogenase (G6PD) deficiency causes acute hemolysis when exposed to oxidative stress. Methemoglobinemia has been associated with G6PD deficiency, and recognition is important to avoid further complications. Case Presentation: A 3-year-old healthy boy presents to the pediatric emergency department with acute onset of difficulty in breathing, perioral cyanosis and hypoxia not responding to oxygen therapy. Transfusion of packed red blood cells was given with improvement of symptoms. Laboratory results were significant for anemia and methemoglobinemia. Further tests revealed deficiency in glucose-6-phosphatase dehydrogenase enzyme. Discussion: G6PD deficiency can cause acute hemolytic anemia. Cyanosis can be exacerbated by the presence of methemoglobinemia. Management must include transfusion of packed red blood cells and avoidance of methylene blue to prevent further hemolysis. Conclusion: G6PD deficiency can manifest after exposure to oxidative stressors with acute onset hemolytic anemia and hypoxia. Occasionally methemoglobinemia could be present contributing to worsening symptoms.
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