G-6-P-D Deficiency
紅血球內葡萄糖-六-磷酸鹽去氫酶缺乏症
紅血球內葡萄糖-六-磷酸鹽去氫酶缺乏症
蠶豆症患者（G-6-PD Deficiency）應注意事項
葡萄糖-6-磷酸鹽脫氫酶缺乏症，俗稱蠶豆症，患者若服用某些中西藥物或食品，如苦瓜(含有抗瘧疾的奎寧成分)、蠶豆酥或薄荷相關產品，容易發生溶血性貧血。
蠶豆症患者(葡萄糖-6-磷酸鹽脫氫酶缺乏症)，日常生活方面要注意下列事項：
不要隨意服用中西藥物，所有中、西藥物均需經由醫師處方。
不要在衣櫥及廁所放置萘丸（樟腦丸Camphor）、薄荷腦（Menthol）、
臭丸。
不要使用龍膽紫藥品（紫藥水）、冬青油、綠油精、萬金油、白花油、香茅精油。
假若發現有黃疸或貧血（臉色蒼白）或尿液變成暗紅色或茶色等現象，
請儘速帶往醫院診治，看病時應主動告訴醫護人員，病患為蠶豆症患者。
蠶豆症患者，下列中西藥物請避免使用（僅供父母、醫師參考）：
抗瘧疾藥劑：
奎寧類藥物 Qunine (苦瓜含有抗瘧疾的奎寧成分)
Primaquine、Pentaquine、Pamaquine (Plasmoquinum)
磺胺劑：
常用於泌尿感染或皮膚科的抗生素（以Sulfa為字首的藥品名）
Sulfanilamide、Sulfapyridine、Sulfacetamide (Sulamyd)、Thiazolesulfone
Sulfamethoxazole (Gontanol)、Salicylazosulfapyridine (Azulfidine)
硝基夫喃類
(Nitrofurans)：用於泌尿感染的抗生素
Nitrofurantoin (Furadantin)、Nitrofurazone (Furacin)、Furazolidone (Furoxone)
解熱劑及鎮痛劑：
Acetanilid、Antipyrine、Aminopyrine（Pyramidon）
其他類：
心血管疾病或解熱鎮痛用的阿斯匹靈類藥物（Aspirin）
Methylene Blue、Naphthalene (Moth Balls)、Nalidixic Acid (Negacide)
Phenylhydrazine、大量維生素C、維生素K3、維生素K4
中藥類：
中藥及中成藥產品 (Traditional Chinese Herbal Medicine/Formulas)
大黃、黃連、梔子、柴胡、虎杖、金銀花、牛蒡子、牡丹皮、生地黃、
番瀉葉、冰片(薄荷腦) 、臘梅花、牛黃、珍珠粉末、熊膽末等。
銀翹散、小柴胡湯、半夏瀉心湯、龍膽瀉肝湯、六味地黃丸、導赤散、
紫雲膏、安宮牛黃丸、曼秀雷敦(綠)、西瓜霜、廣東目藥粉、京都念慈庵川貝枇杷膏等。
G6PD Deficiency
G6PD deficiency is a genetic disorder in which the body doesn’t have enough of an enzyme (chemical) called glucose-6-phosphate dehydrogenase (G6PD). G6PD is important in protecting red blood cells.
The lack of G6PD can lead to red blood cells breaking down too easily (haemolysis) when the person is exposed to certain triggers, which are usually certain foods, viruses or medications. The red blood cells carry oxygen around the body, and the excessive breakdown of red blood cells can lead to anaemia, which can cause pale skin and lethargy. The excessive breakdown of red blood cells may also cause a build up of their waste products, resulting in jaundice (yellow skin/eye discolouration) in some children.
The condition is more common in males and is usually passed on to male children from their mother, even though she probably had no symptoms herself. G6PD cannot be spread from one person to another.
There is no cure for G6PD deficiency, and it is a lifelong condition. However, most people with G6PD deficiency have a completely normal life as long as they avoid the triggers.
Signs and symptoms of G6PD deficiency
Your child will only show symptoms of G6PD deficiency when their red blood cells are being broken down in excess. They may have:
l pale skin (pallor) / persistent and/or severe tiredness
l dark coloured urine (wee) / jaundice (yellow skin or eyes).
It is common for babies to have jaundice in the first week of life, but some babies with G6PD deficiency have jaundice for longer than usual.
When to see a doctor
Newborns with persistent jaundice may have a blood test to look for G6PD deficiency. If your child is showing the signs of G6PD deficiency, take them to your GP so that the symptoms can be investigated.
If your child has been diagnosed with G6PD deficiency, take them to be checked by a GP whenever any of the following symptoms develop: If your child has been diagnosed with G6PD deficiency, take them to be checked by a GP whenever any of the following symptoms develop:
l jaundice (yellow skin and eyes)
l dark coloured urine
l anaemia (pale skin and lethargy).
Care at home
Make sure people who care for your child (school, kindergarten, child care, babysitters, health professionals) know your child has G6PD deficiency and provide them with a printout of this fact sheet.
Always check with your doctor or pharmacist before giving any medication to your child.
To prevent the symptoms of G6PD deficiency, a child with G6PD should avoid the triggers on the following lists:
Antibiotics
l Sulphonamides (check with your doctor) / Nitrofurantoin / Nalidixic acid
l Co-trimoxazole (Bactrim, Septrin) / Dapsone / Chloramphenicol
Antimalarials
l Chloroquine / Hydroxychloroquine / Primaquine / Quinine / Mepacrine
Chemicals
l Moth balls (naphthalene) / Methylene blue
Foods
Fava beans (also called broad beans) Fava beans (also called broad beans)
Other drugs
l Sulphasalazine / Methyldopa Large doses of vitamin C
l Hydralazine / Procainamide / Quinidine / Some anti-cancer drugs
Some children with G6PD must not take aspirin – check with your doctor first.
Always check the labels of any medications you buy without a prescription, and be careful about using herbal, naturopathic or other alternative/complementary therapies.
Key points to remember
G6PD is an inherited condition and cannot be spread from one person to another. Most children with G6PD deficiency have a completely normal life as long as they avoid certain foods and drugs. Some children with the condition will get anaemia or jaundice, especially after taking medicine or eating food they should avoid, or after an infection.
Common questions for G6PD deficiency
How did my child get G6PD deficiency?
G6PD deficiency is an inherited disease (passed on from previous generations), and cannot be caught by being in contact with someone else. G6PD deficiency is more common in some racial groups, for example, people with African, Asian or Mediterranean heritage. Scientists think there are about 400 million people in the world with G6PD deficiency.
Should I give my child iron tablets to prevent anaemia?
Never give a child with G6PD deficiency any medicine (including complementary medicines) or tablets without consulting with your doctor first. You doctor will advise which medications are safe. It is safe for children with G6PD deficiency to take iron, but iron should only be prescribed when the anaemia is confirmed as being due to iron deficiency.
Can I give my child paracetamol or ibuprofen?
Yes – paracetamol and NSAIDS (Nonsteroidal anti-inflammatory drugs) are safe for children with G6PD deficiency.
Should I give my child iron tablets to prevent anaemia?
Never give a child with G6PD deficiency any medicine (including complementary medicines) or tablets without consulting with your doctor first. You doctor will advise which medications are safe. It is safe for children with G6PD deficiency to take iron, but iron should only be prescribed when the anaemia is confirmed as being due to iron deficiency.
Can I give my child paracetamol or ibuprofen?
Yes – paracetamol and NSAIDS (Nonsteroidal anti-inflammatory drugs) are safe for children with G6PD deficiency.