Clinical Pharmacology Unit, Assaf Harofeh Medical Center, Zerifin, Israel.
Drug Saf. 2010 Sep 1;33(9):713-26. doi: 10.2165/11536520-000000000-00000.
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzyme defect and one of the most common genetic disorders worldwide, with an estimated 400 million people worldwide carrying a mutation in the G6PD gene that causes deficiency of the enzyme. Although drug-induced haemolysis is considered the most common adverse clinical consequence of G6PD deficiency, significant confusion exists regarding which drugs can cause haemolytic anaemia in patients with G6PD deficiency. In the absence of consensus among physicians, patients are subject to conflicting advice, causing uncertainty and distress. In the current review we aimed, by thorough search of the medical literature, to collect evidence on which to base decisions either to prohibit or allow the use of various medications in patients with G6PD deficiency. A literature search was conducted during May 2009 for studies and case reports on medication use and G6PD deficiency using the following sources: MEDLINE (1966-May 2009), PubMed (1950-May 2009), the Cochrane database of systematic reviews (2009), and major pharmacology, internal medicine, haematology and paediatric textbooks. After assessing the literature, we divided medications into one of three groups: medications that should be avoided in individuals with G6PD deficiency, medications that were considered unsafe by at least one source, but according to our review can probably be given safely in normal therapeutic dosages to individuals with G6PD deficiency as evidence does not contravene their use, and medications where no evidence at all was found to contravene their use in G6PD-deficient patients. It is reasonable to conclude that, over time, many compounds have been wrongly cited as causing haemolysis because they were administered to patients experiencing an infection-related haemolytic episode. We found solid evidence to prohibit only seven currently used medications: dapsone, methylthioninium chloride (methylene blue), nitrofurantoin, phenazopyridine, primaquine, rasburicase and tolonium chloride (toluidine blue). Regarding all other medications, our review found no evidence to contravene their use in normal therapeutic doses to G6PD-deficient patients. There is a need for evidence-based global consensus regarding medication use in G6PD-deficient patients.
葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症是最常见的人类酶缺陷之一，也是全球最常见的遗传疾病之一，估计全球有 4 亿人携带导致该酶缺乏的 G6PD 基因突变。尽管药物诱导的溶血性贫血被认为是 G6PD 缺乏症最常见的不良临床后果，但对于哪些药物会导致 G6PD 缺乏症患者发生溶血性贫血，仍存在很大的混淆。由于医生之间没有共识，患者会收到相互矛盾的建议，导致不确定性和困扰。在本次综述中，我们通过对医学文献的彻底搜索，旨在收集证据，以便在 G6PD 缺乏症患者中决定禁止或允许使用各种药物。在 2009 年 5 月期间，我们使用以下来源对药物使用和 G6PD 缺乏症的研究和病例报告进行了文献搜索：MEDLINE（1966 年-2009 年 5 月）、PubMed（1950 年-2009 年 5 月）、Cochrane 系统评价数据库（2009 年）以及主要的药理学、内科学、血液学和儿科学教科书。在评估文献后，我们将药物分为三类：应避免在 G6PD 缺乏症患者中使用的药物、至少有一种来源认为不安全但根据我们的综述可以在正常治疗剂量下安全给予 G6PD 缺乏症患者的药物，以及在 G6PD 缺乏症患者中没有发现任何证据表明药物会导致溶血性贫血的药物。可以合理地得出结论，随着时间的推移，许多化合物被错误地引用为引起溶血，因为它们被给予经历感染相关溶血性发作的患者。我们发现只有 7 种目前使用的药物有确凿的证据禁止使用：氨苯砜、亚甲蓝（美蓝）、呋喃妥因、匹美西林、伯氨喹、拉布立酶和甲苯胺蓝（甲苯胺蓝）。关于所有其他药物，我们的综述没有发现任何证据表明在正常治疗剂量下对 G6PD 缺乏症患者使用这些药物会有任何问题。在 G6PD 缺乏症患者中使用药物需要基于证据的全球共识。
