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):

Name Molecular formula Risk level For whom Notes
2-Naphthol/2-萘酚 C10H8O High All
Acetanilide (acetanilid)/乙酰苯胺 C8 H9 N O High Medit., Asian
Acetazolamide/乙酰唑胺 C4H6N4O3S2 Medium All
Acetophenetidin (phenacetin)/乙酰苯乙啶 (非那西丁) low All
Acetylphenylhydrazine (2-Phynylacetohydrazide)/乙酰苯肼 C8 H10 N2 O High All These substances taken in high quantities might cause hemolysis also with normal subjects.
Aldesulfone sodium (sulfoxone)/阿地砜钠 (亚砜) C14 H14 N2 Na2 O6 S3 High All
Aminophenazone (aminopyrine)/氨基非那宗 (氨基比林) C13 H17 N3 O Medium All
Aminopyrine (Pyramidon, amidopyrine)/氨基非那宗 (吡喃酮, 氨基吡啶) low All
Antazoline (antistine)/安他唑啉 (安替斯丁) C17 H19 N3 Medium All
Antipyrine/安替比林 low All
Arginine (2-Amino-5-guanidinopentanoic acid)/精氨酸 C6H14N4O2 Medium All
Arsine/砷化氢 As-H3 High All These substances taken in high quantities might cause hemolysis also with normal subjects.
Ascorbic Acid/抗坏血酸 C6H8O6 High All
Azathioprine (use with caution)/硫唑嘌呤 Low All Use with caution and not in combination with other drugs.
Baclofen (use with caution)/巴氯芬 Low All Use with caution and not in combination with other drugs.
Bean of St.Ignatius (Strychnos ignatii)/圣依纳爵豆 (吕宋果) Medium All
Benorilate/贝诺酯/扑炎痛 C17H15NO5 Medium All
Benzexolo/苯并唑 Low All
Beta-Naphthol (2-Naphthol)/β-萘酚 C10 H8 O High All
Brinzolamide/布林佐胺 High All See http://marineyes.com/glaucoma/medications.html
Bupivacaine/布比卡因 C18H28N2O High All
Calcium Carboxylate/羧酸钙 Medium All
Carbutamide/磺胺丁脲 C11H17N3O3S Medium All
Chloramphenicol/氯霉素 C11 H12 C12 N2 O5 High Medit., Asian
Chloroquine/氯奎 C18 H26 Cl N3 High Medit., Asian If required, this substance may be taken under medical supervision as a preventive treatment or cure of malaria.
Chloroquine + Proguanil/氯奎+氯胍 High Medit., Asian
Ciprofloxacin/环丙沙星 High All
Clorguanidina/氯胍 Low All
Colchicine/秋水仙碱 C22 H25 N O6 Medium All
Dapsone (diaphenylsulfone)/氨苯砜 (二苯砜) C12 H12 N2 O2 S High All These substances taken in high quantities might cause hemolysis also with normal subjects.
Diethylamine/二乙基胺 C4H11N High All
Dimenidrinato C24H28ClN5O3 High All
Dimercaprol/二巯基丙醇 C3 H8 O S2 High All
Diphenhydramine (difenilhydramine)/苯海拉明 C17 H21 N O Medium All
Dopamine/多巴胺 C8 H11 N O2 Medium All
Dorzolamide/杜塞酰胺 High All See http://marineyes.com/glaucoma/medications.html
Doxorubicin/多柔比星 C27 H29 N O11 High Medit., Asian
Enoxacin/依诺沙星 C15H17F4N4O3 High All
Epirubicin/表柔比星 C27H29NO11 Medium All
Flumequine/氟甲喹 C14H12FNO3 Medium All
Furazolidone/呋喃唑酮 C8 H7 N3 O5 High All
Glibenclamide/格列本脲 C32 H28 Cl N3 O5 S High Medit., Asian Hemolytic reactions to this substance have been reported only in few, isolated cases and no written reference exists as of this time
Glibornuride/格列波脲 C18H26N2O4S Medium All
Gliclazide/格列齐特 C15H21N3O3S High All
Glimepiride/格列美脲 C24H34N4O5S High All
Glipizide/格列吡嗪 C21H27N5O4S High All
Glucosulfone (glucosulfone sodium)/葡胺苯砜 (葡萄糖砜钠) C24 H34 N2 Na2 O18 S3 High All
Hydroxychloroquine/羟氯喹 C18H26ClN3O High All
Ibuprofen/布洛芬 CH3H18O2 Medium All
Indigofera Tinctoria/吲哚乙酸 Medium All
Isobutyl Nitrite/亚硝酸异丁酯 C4 H9 N O2 High Medit., Asian
Isoniazid/异烟肼 C6 H7 N3 O Medium All
Lawsone Inermis Medium All
Levodopa/左旋多巴 C9H11NO4 Medium All
Levofloxacin/左氧氟沙星 C18H20FN3O4 High Medit., Asian
Lomefloxacin/洛美沙星 C17H19F2N3O3 High All
Mefloquine/甲氟喹 C17H16F6N2O High All
Menadiol Sodium Sulfate (Vitamin k4 sodium sulfate)/甲萘氢醌硫酸钠 C11 H8 Na2 O8 S2 High All Synthetic substitutes of natural Vitamin K. Other substitutes non listed here are: menadiole diacetate, menadiole sodium phosphate (anhydrous salt). As treatment of hemorrhage in newborns, a single dose of this substance may be given (via parenteral) on the first day after birth and is well tolerated. The writers of these note (2,16) generally consider all Vitamin K substitutes as potentially hemolysis inducing substances. It is probable that natural Vitamin K1 (phyto-menadione) presents a lower risk.
Menadione (menaphtone)/甲萘醌/VK3 C11 H8 O2 High All 同上
Menadione sodium Bisulfite (Vitamin K3 sodium bisulfite)/甲萘醌亚硫酸氢钠 C11 H8 O2 NaHSO3 High All 同上
Mepacrine (Quinacrine)/米帕林 (奎纳克林) C23 H3O Cl N3 O High Medit., Asian
Mesalazine – 5-Aminosalicylic Acid (paraminosalicylic acid)/美沙拉嗪 – 5-氨基水杨酸(对氨基水杨酸) C7 H7 N O3 High Medit., Asian
Metamizole/安乃近 C13H16N3NaO4S High Medit., Asian
Methyltioninium Chloride (methylene blue)/亚甲基蓝 C16 H18 Cl N3 S High All
Morpholine/吗啡啉 C4H9NO Medium All
Moxifloxacin/莫西沙星 High Medit., Asian
Nalidixic Acid/萘啶酸 C12 H12 N2 O3 High Medit., Asian Hemolytic reactions to this substance have been reported only in few, isolated cases and no written reference exists as of this time.
Naphtalene, Pure (naphtalin)/双二甲氨基萘(拿弗他林) C10 H8 High All
Niridazole/尼立达唑 C6 H6 N4 O3 S High All
Nitric Oxide/一氧化氮 NO High All
Nitrofural (nitrofurazone)/硝基呋喃(呋喃西林) C6 H6 N4 O4 High All
Nitrofurantoin/呋喃妥因 C8 H6 N4 O5 High All
Nitroglycerin/硝酸甘油 C3H5N3O9 High All
Noramidopyrine/去甲氨基比林 C13H16N3NaO4S High All
Norfloxacin/诺氟沙星 C16 H18 F N3 O3 Medium All
O-Acetylsalicylic Acid (acetylsalicylic acid)/阿司匹林 C9 H8 O4 High Medit., Asian Among the alternatives are Paracetamol, which is generally considered safe, and flurbiprophene.
O-Acetylsalicylic Acid + Acetanilide/阿司匹林+乙酰苯胺 High Medit., Asian
O-Acetylsalicylic Acid + Ascorbic Acid/阿司匹林+Vc High All
O-Acetylsalicylic Acid + Paracetamol/阿司匹林+对乙酰氨基酚 High All
Ofloxacin/氧氟沙星 High Medit., Asian
Oxidase, Urate (urate oxidase)/尿酸氧化酶 High Medit., Asian
Pamaquine/帕马奎宁 C42 H45 N3 O7 High All
Para-Aminobenzoic Acid (4-Aminobenzoic Acid)/对氨基苯甲酸 C7 H7 N O2 Medium All
Paracetamol (acetaminophen)/扑热息痛(对乙酰氨基酚) C8 H9 N O2 Medium All
Paracetamol + Propyphenazone/扑热息痛+异丙安替比林 Medium All
Pefloxacin/培氟沙星 C17H20N3O3 High All
Pentaquine/戊奎宁 C18 H27 N3 O High All
Phenacetin (acetophenetidin)/非那西丁 (乙酰苯乙啶) C10 H13 N O2 High Medit., Asian Probably safe in moderate doses.
Phenazone (antipyrine)/非那宗 (安替必灵) C11 H12 N2 O Medium All
Phenazopyridine/非那吡啶 C11 H11 N5 High Medit., Asian
Phenylbutazone/苯丁腙 C19 H20 N2 O2 Medium All
Phenytoin/苯妥英钠 C19 H20 N2 O2 Medium All
Phynylhydrazine/苯肼 C6 H8 N2 High All
Phytomenadione (Vitamin K1)/植物甲萘醌 C31 H46 O2 Medium All
Pipemidic Acid/吡哌酸 C14H17N5O3 High All
Pregabalin (use with caution)/普瑞巴林 Low All Use with caution and not in combination with other drugs.
Prilocaine/丙胺卡因 C13H20N2O Medium All
Primaquine/伯氨喹 C15 H21 N3 O High All May be given in reduces doses under medical supervision.
Probenecid/丙磺舒 C13 H19 NO4 S High All
Procainamide/普鲁卡因酰胺 C13 H21 N3 O Medium All
Procainamide Hydrochloride/盐酸普鲁卡因酰胺 Low All
Proguanil (chlorguanidine)/氯胍 C11 H16 Cl N5 Medium All
Propylene Glycol/丙二醇 C3H8O2 Medium All
Propyphenazone/异丙安替比林 Medium All
Pyrimethamine/乙胺嘧啶 C12 H13 Cl N4 Medium All
Quinidine C20 H24 N2 O2 Medium All
Quinidine + Phynylhydrazine/奎尼丁+ 苯肼 High All
Quinidine + Quinine Medium All
Quinine/奎宁 C20 H24 N2 O2 Medium All
Rasburicase/拉布立酶 High Medit., Asian Helps clear uric acid from the blood.
Sodium nitroprusside/硝普钠 Na2[Fe(CN)5NO] High All
Spiramycin/螺旋霉素 C43H74N2O14 High All
Stibophen (2-(2-Oxido-3,5-Disulphonatophenoxy)-1,3,2,Benzodioxastibole-4-6-Disulphonate)/锑波芬 C12 H4 Na5 O16 S4 Sb High All
Streptomycin/链霉素 C21 H39 N7 O12 Medium All
Succimer/二巯基丁二酸 C4H6O4S2 Medium All
Sulfacetamide/磺胺醋酰胺 C8 H10 N2 O3 S High All
Sulfacytine/磺胺西汀 C12 H14 N4 O3 S Medium All
Sulfadiazine/磺胺嘧啶 C10 H10 N4 O2 S Medium All
Sulfadiazine Medium Medit., Asian
Sulfadiazine + Trimethoprim/~ + 甲氧苄啶 Medium All
Sulfadimidine/磺胺二甲嘧啶 C12 H14 N4 O2 S High All
Sulfadoxine/磺胺多辛 C12H14N4O4S Medium All
Sulfafurazole (sulfafurazone, sulfisoxazole)/磺胺异恶唑 C11 H13 N3 O3 S High Medit., Asian
Sulfaguanidine/磺胺脒 C7 H10 N4 O2 S Medium All
Sulfamerazine/磺胺甲密啶 C11 H12 N4 O2 S Medium All
Sulfamethizole/磺胺甲二唑 C9H10N4O2S2 Medium All
Sulfamethoxazole/磺胺甲恶唑 C10 H11 N3 O3 S High All A component, together with Trimetroprim, of Co-trimoxazole.
Sulfamethoxypyridazine/磺胺甲氧哒嗪 C11 H12 N4 O3 S Medium All
Sulfanilamide (Sulphanilamide)/氨苯磺胺 C6 H8 N2 O2 S High All
Sulfapyridine/磺胺吡啶 C11 H11 N3 O2 S High All
Sulfasalazine, Salazosulfapyridine (salazopyrin)/柳氮磺胺吡啶 C18 H14 N4 O5 S High All For treating Ulcerative Colitis, Crohn’s Disease, Rheumatoid Arthritis, Fibromyalgia.
Sulfisoxazole (Gantrisin)/磺胺异恶唑 (甘替新) Low All
Sulfoxone/阿地砜钠 C14H16N2O6S3 Medium All
Thiamphenicol/甲砜霉素 C12H15Cl2NO5S High All
Thiazosulfone (thiazolesulfone)/噻唑砜 C9 H9 N3 O2 S2 High Medit., Asian
Tiaprofenic Acid/噻洛芬酸 C14 H12 O3 S Medium All
Tolonium Chloride, Tolonium Chloride (toluidine blue)/甲苯胺蓝 C15 H16 Cl N3 S High All
Trihexyphynidyl (benzhexol)/三己芬迪 (苯海索) C20 H31 N O Medium All
Trimethoprim/甲氧苄啶 C14 H18 N4 O3 Medium All Component, together with Sulfametossazole, of Co-trimoxazolo.
Trimethoprim + Sulfamethoxazole/~ + 新诺明 High All
Trinitrotoluene (2,4,6-Trinitrotoluene)/三硝基甲苯 C7 H5 N3 O6 High Medit., Asian
Tripelennamine/曲吡那敏 C16 H21 N3 Medium Medit., Asian

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/

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