一名镰状细胞性贫血和 G6PD 缺乏症共同遗传患者因蜡质引起的发作性溶血

IF 0.2 Q4 MEDICINE, GENERAL & INTERNAL Nigerian Journal of Basic and Clinical Sciences Pub Date : 2023-07-01 DOI:10.4103/njbcs.njbcs_25_22
Abdulwahab Baba, Aminu Yusuf, Dalha H Gwarzo, Ibrahim Idris
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引用次数: 0

摘要

镰状细胞性贫血(SCA)和葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症都是与溶血性贫血有关的常见遗传性疾病,在撒哈拉以南非洲地区发病率很高。这两种疾病的共同遗传在尼日利亚都有报道,而尼日利亚的每种疾病的发病率都很高。然而,诊断同一人同时患有这两种疾病需要高度怀疑。在此,我们介绍一例不寻常的病例,患者是一名 32 岁的牙科学生,自一岁起就患有 SCA。他在皮肤和吸入用于牙科整形实践课程的蜡(多环芳烃)后出现黄疸阵发性加重和排出深色尿液。经过检查,发现他患有 G6PD 缺乏症。该报告强调了对出现偶发性溶血症状的男性 SCA 患者进行 G6PD 缺乏症筛查的重要性。
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Wax-induced episodic hemolysis in a patient with co-inheritance of sickle cell anemia and G6PD deficiency
Both sickle cell anemia (SCA) and glucose-6-phosphate dehydrogenase (G6PD) deficiency are common genetically transmitted disorders associated with hemolytic anemia, with a high prevalence in sub-Saharan Africa. Co-inheritance of both diseases has been reported in Nigeria where the prevalence of each disease is high. However, a high index of suspicion is needed to diagnose the co-existence of both disorders in the same individual. Here, we present an unusual case of a 32-year-old dentistry student who was known to have SCA since the age of one year. He presented with episodic exacerbation of jaundice and passage of dark-colored urine following skin and inhalational exposure to wax (polycyclic aromatic hydrocarbons) used for reconstructive dental practical sessions. After investigations, he was found to have G6PD deficiency. This report underscores the importance of screening for G6PD deficiency in male patients with SCA presenting with episodic symptoms of hemolysis.
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来源期刊
Nigerian Journal of Basic and Clinical Sciences
Nigerian Journal of Basic and Clinical Sciences MEDICINE, GENERAL & INTERNAL-
CiteScore
0.20
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0.00%
发文量
8
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