蚕豆病患者的用药-更新

这是一篇更新的文章，旧版在此

蚕豆病是葡萄糖-6-磷酸脱氢酶缺乏症（glucose-6-phosphate dehydrogenase deficiency, G6PD）的一个类型¹：

Class	Levels of deficiency	Enzyme activity	Prevalence
I	Severe	Chronic nonspherocytic hemolytic anemia in the presence of normal erythrocyte function	Uncommon; occurs across populations
II	Severe	Less than 10 percent of normal	Varies; more common in Asian and Mediterranean populations
III	Moderate	10 to 60 percent of normal	10 percent of black males in the United States
IV	Mild to none	60 to 150 percent of normal	Rare
V	None	Greater than 150 percent of normal	Rare

G6PD是一种X染色体连锁遗传病，G6PD变异型的临床表现包括一系列溶血综合征。几乎所有的患者都是完全无症状的。在大多数类型的G6PD中，儿童和成人发生溶血的原因为较老且缺陷较严重的红细胞暴露于具有高氧化还原潜能的药物(包括抗疟药伯氨喹和某些磺胺类药物)、蚕豆、特定感染或者代谢异常后被突然破坏²。

一般认为食物和感染是导致发生急性溶血症的两个主要原因。

这类患者需要避免的食物包括³：

• 蚕豆/Fava beans。蚕豆的同义词：Bell beans,Broad beans, English dwarf beans, Fever beans, Haba beans, Horse beans, Pigeon beans, Silkworm beans, Tick beans
• 红酒/Red wine
• 所有的豆（荚）类/All legumes
• 蓝莓/Blueberries
• 大豆制品/Soya products
• 汤力水（奎宁水）/Tonic water

但是具体需要避免的食物种类取决于患者体内的G6PD缺乏程度和基因突变类型。

对于这类患者的药物使用禁忌方面，有多个版本，但大多引用了https://www.g6pd.org/en/G6PDDeficiency/SafeUnsafe/DaEvitare_ISS-it网站上的资料。摘录于下(2023-05-20)：

注:
1. Medium risk means that for common G6PD deficiency variants these may be considered safe in most cases, when taken in normal therapeutic doses. However, they might provoke hemolysis when taken in large doses (accidental ingestion, poisoning, special treatments) or in the period preceding birth, or in presence of other pathologies.
2. Drug names in red are of high risk to all deficiency types.
3. 中文译名采用`Bing词典`翻译，可能不准确，以英文名为准。

和口腔相关的一些药物主要是抗炎镇痛药物、局部麻醉药物以及抗生素。

• 解热镇痛抗炎药物中，阿司匹林、对乙酰氨基酚、双氯芬酸钠、布洛芬等都有导致急性溶血症的情况，在上表中归为中风险，而阿斯匹林+Vc/对乙酰氨基酚等等连用的情况归为高风险，需要注意；也有认为、对乙酰氨基酚双氯芬酸钠以及布洛芬是安全的⁴，可能和G6PD的种类有关系。
• 局麻药，上表中出现的有布比卡因、丙胺卡因、普鲁卡因。因为对氨基苯甲酸被归为中风险，而酯类局麻药在血浆内被假胆碱酯酶水解成对氨基苯甲酸和乙二胺乙酸，所以酯类局麻药可能也存在相应的风险。
• 抗生素主要有硝基呋喃类、喹诺酮类、氯霉素以及磺胺类，抗分枝杆菌药氨苯砜、对氨基水杨酸和砜类药物。

Further reading:

1. https://www.uptodate.com/contents/image/print?imageKey=HEME%2F74254&topicKey=PEDS%2F5927&source=see_link
2. https://www.ihtc.org/G6PD
3. https://www.science.gov/topicpages/h/human+glucose-6-phosphate+dehydrogenase
4. Frank JE. Diagnosis and management of G6PD deficiency. Am Fam Physician. 2005 Oct 1;72(7):1277-82. PMID: 16225031.
5. Youngster I, Arcavi L, Schechmaster R, Akayzen Y, Popliski H, Shimonov J, Beig S, Berkovitch M. Medications and glucose-6-phosphate dehydrogenase deficiency: an evidence-based review. Drug Saf. 2010 Sep 1;33(9):713-26. doi: 10.2165/11536520-000000000-00000. PMID: 20701405.
6. Hagag AA, Badraia IM, Elfarargy MS, Abd Elmageed MM, Abo-Ali EA. Study of Glucose-6-Phosphate Dehydrogenase Deficiency: 5 Years Retrospective Egyptian Study. Endocr Metab Immune Disord Drug Targets. 2018 Feb 13;18(2):155-162. doi: 10.2174/1871530317666171003160350. PMID: 28982343.
7. Bubp J, Jen M, Matuszewski K. Caring for Glucose-6-Phosphate Dehydrogenase (G6PD)-Deficient Patients: Implications for Pharmacy. P T. 2015;40(9):572-574.

1. Frank JE. Diagnosis and management of G6PD deficiency. Am Fam Physician. 2005 Oct 1;72(7):1277-82. PMID: 16225031. ↩

2. https://www.uptodate.com/contents/zh-Hans/genetics-and-pathophysiology-of-glucose-6-phosphate-dehydrogenase-g6pd-deficiency#subscribeMessage ↩

3. Drugs that should be avoided – Official List. G6PD Deficiency Association. Available at https://www.g6pd.org/en/G6PDDeficiency/SafeUnsafe/DaEvitare_ISS-it. ↩

4. https://www.rch.org.au/kidsinfo/fact_sheets/G6PD_deficiency/ ↩