G6Pd缺乏伴严重溶血性贫血1例

Zahoor Hussain Daraz, Berkheez Shabir, Rehana Afshan, Pamelle Yadav, Mohamed Rashwan Meselhy shady
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摘要

一名3岁男孩在我们位于H.A Alif Dhidhoo的环礁医院就诊,过去3天出现严重苍白、黄疸、易疲劳和反复出现深色尿。他在怀孕39周时出生成熟,过去没有明显的病史。最近的历史显示,食用了2罐蚕豆和使用了一些草药。入院时,体格检查显示发烧101华氏度,严重苍白,黄疸,颈部淋巴结病变和轻度肝肿大。实验室检查结果显示血红蛋白水平为5.4 g/dl,伴有溶血图像,血清胆红素为6mg/dl。测定患者G6PD水平,显示明显不足。排除其他原因的溶血性贫血。病人需要紧急输血和抗生素治疗感染。他对治疗反应良好,出院时情况稳定。家长们得到了适当的建议,了解了这种情况以及避免某些食物和药物的重要性。叶酸用于维持正常血红蛋白浓度。这是马尔代夫北部报告的首例G6PD病例,表现为需要输血的严重溶血性贫血。
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G6Pd deficiency with severe hemolytic anemia: a case report
A 3-year-old boy presented to our atoll hospital in H.A Alif Dhidhoo, with severe pallor, jaundice, easy fatigability and recurrent episodes of passage of dark-colored urine for past 3 days. He was born mature at 39 weeks of gestation with no past significant medical history. Recent history revealed the consumption of 2 cans of fava beans and application of some medicinal herbs. On admission, physical examination revealed fever of 101 degree Fahrenheit, severe pallor, jaundice, cervical lymphadenopathy and mild hepatomegaly. Laboratory investigation results showed a hemoglobin level of 5.4 g/dl with a hemolytic blood picture and serum Bilirubin of 6mg/dl. The patient's G6PD level was measured which showed marked deficiency. Other causes of hemolytic anemia were excluded. Patient required urgent packed RBC transfusion and antibiotics for infection. He responded well to the treatment and was discharged in a stable condition. Parents were appropriately advised on the condition and the importance of avoiding certain foods and medication. Folic acid was prescribed for maintaining normal hemoglobin concentration. This is a first case report in North Maldives of G6PD presenting with severe hemolytic anemia requiring blood transfusion.
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