G6PD

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G6PD Deficiency (Kekurangan Enzim G6PD)

Pengenalan

G6PD ialah singkatan nama enzim Glucose-6-Phosphate Dehydrogenase, yang terdapat di dalam sel darah merah. Ia bertindak dalam melindungi sel darah merah daripada pecah (hemolisis) bila terdedah kepada ubat-ubat tertentu, makanan atau jangkitan.

Diagnosa

Kekurangan enzim G6PD sangat ketara di kalangan masyarakat Asia. Di negara kita semua bayi baru lahir akan disaring untuk kekurangan enzim G6PD dengan menggunakan darah dari tali pusat. Kekurangan enzim ini adalah keadaan yang diwarisi secara keturunan. Ia ditentukan oleh bahan baka yang dibawa oleh kromosom X. Oleh itu kebanyakan mereka yang mempunyai kekurangan enzim G6PD adalah kaum lelaki, manakala kaum perempuan biasanya adalah pembawa.

Ujian untuk mengesan kehadiran enzim ini adalah ujian darah yang mudah dilakukan. Pastikan anda tahu status enzim anda dan keluarga anda.


Gejala/Tanda-tanda

Kebanyakan mereka yang kekurangan enzim G6PD ini tidak mempunyai tanda-tanda khusus. Bayi baru lahir perlu diperhatikan di dalam wad untuk suatu jangka masa kerana mereka boleh mendapat penyakit kuning (jaundice) yang tinggi dengan cepat, akibat dari hemolisis berlebihan. Bayi yang kuning (jaundice) perlu diletakkan di bawah lampu (fototerapi) untuk menurunkan tahap bilirubin dalam darah.

Tanda-tanda yang terjadi sekiranya sel darah merah pecah (hemolisis) termasuk :

Akibat dari kurang darah

  • kelihatan pucat
  • tidak bermaya (atau keletihan yang teramat)
  • pening
  • degupan jantung yang laju
  • sukar bernafas
  • sakit dada

Akibat dari hemolisis

  • jaundis (kekuningan)- boleh dilihat pada mata putih atau kulit, warna air kencing seperti air teh O atau Coca-Cola.

Jika ini berlaku, dapatkan rawatan hospital dengan segera.

Kekurangan enzim G6PD adalah untuk seumur hidup. Walaupun tiada rawatan yang boleh menghilangkannya, ia tidak membawa kesan negatif yang ketara dan tidak menjejaskan perkembangan dan tumbesaran kanak-kanak.

Ubat-ubat dan bahan-bahan yang patut dielakkan

Bagi kanak- kanak dan orang dewasa yang kekurangan enzim G6PD, mereka perlu tahu mendalami mengenai keadaan ini agar dapat memahaminya dengan lebih jelas. Mereka perlu menjauhi dari terdedah kepada ubat-ubat serta bahan-bahan tertentu yang boleh menyebabkan hemolisis (senarai ubatubat serta bahan-bahan seperti di Rajah 1).

Hemolisis juga akan berlaku jika beliau termakan kacang parang atau menggunakan ubat gegat pada pakaian. Bahan-bahan ini patut dijauhi.

Ibu-ibu yang menyusu bayi yang kekurangan enzim G6PD digalakkan meneruskan penyusuan tetapi mereka perlu mengelakkan bahan-bahan/ubat-ubat seperti di Rajah 1 agar tidak termasuk di dalam susu ibu.

Jangkitan kuman juga boleh menyebabkan hemolisis berlaku.

Pastikan anda beritahu doktor anda sekiranya anda/anak anda mempunyai kekurangan enzim G6PD untuk memastikan ubat yang diterima selamat untuk anda dan tidak akan menyebabkan hemolisis berlebihan.

Rajah 1 : Bahan-bahan / Ubat-ubat Tertentu Yang Boleh Menyebabkan Hemolisis

Makanan dan Herba yang mesti dielakkan

  • Kacang parang (fava beans)
  • Ubat China
    • Chuen Lin
    • San Chi
    • 13 herbs
    • 12 herbs
  • Ubat tradisi lain (dapatkan nasihat doktor)

Bahan lain yang mesti dielakkan

  • Ubat gegat (moth balls)
  • Ubat nyamuk yang mengandungi pyrethium

Ubat yang mesti dielakkan

  • Acetanilide
  • Doxorubicin
  • Furazolidene
  • Methylene Blue
  • Nalidixic acid
  • Niridazole
  • Nitrofurantoin
  • Phenozopyridine
  • Primaquine
  • Sulfamethoxazole
  • Bactrim

Ubat yang boleh diberi dalam dos terapeutik

  • Paracetamol
  • Ascorbic acid
  • Aspirin
  • Chloramphenicol
  • Chloroquine
  • Colchicine
  • Diphenhydramine
  • Isoniazid
  • Phenacetin
  • Phenylbutazone
  • Phenytoin
  • Probenecid
  • Procainamide
  • Pyrimethamine
  • Quinidine
  • Streptomycin
  • Sulfisoxazole
  • Trimethoprim
  • Tripelennamine
  • Vitamin K
  • Mefloquine

*credit to myhealth

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20 comments
  1. 3 org adik lelaki ayu ada G6PD..
    waktu dpat Ian dulu, takutr gak..tapi dia ok..next next (kalu ada) tak tau la…sakit nie tak susah, but remeh sikit

  2. bagus betul wat entri pasal ni kan..at least dpt info..eli dulu tahu la jgk.skang dh lupa..bila baca balik entri, igt balik..esok2 lupa balik ehehhe

  3. wah sungguh detail sekali bagus2. elakkan kacang parang ya

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