Ka nele o Glucose-6-phosphate dehydrogenase

ʻO ka Glucose-6-phosphate dehydrogenase (G6PD) ka hemahema kahi kūlana kahi e wāwahi ai nā hunaola ʻulaʻula ke hōʻike ʻia ke kino i kekahi mau lāʻau a i ʻole ke koʻikoʻi o ka maʻi. He hoʻoilina ia, ʻo ia hoʻi ua hoʻoili ʻia i loko o nā ʻohana.

Kū ka hemahema G6PD ke nalowale kahi kanaka a ʻaʻohe lawa paha o kahi enzyme i kapa ʻia ʻo glucose-6-phosphate dehydrogenase. Kōkua kēia enzyme i nā hunaola ʻulaʻula e hana pono.

ʻO G6PD liʻiliʻi loa ke alakaʻi i ka luku ʻia ʻana o nā hunaola ʻulaʻula. Kapa ʻia kēia hana he hemolysis. Ke hana ʻiʻo kēia hana, ua kapa ʻia ia he hanana hemolytic. Pōkole pinepine nā māhele. ʻO kēia ke hoʻomau nei ke kino e hana i nā hunaola ʻulaʻula hou, i loaʻa ka hana maʻamau.

Hiki ke hoʻomaka ʻia ka luku ʻana o ke koko ʻulaʻula e nā maʻi, kekahi mau meaʻai (e like me nā pī fava), a me kekahi mau lāʻau, e like me:

• Nā lāʻau Antimalarial e like me ka quinine
• Aspirin (nā koina kiʻekiʻe)
• Nā lāʻau anti-inflammatory nonsteroidal (NSAIDs)
• Quinidine
• Nā lāʻau Sulfa
• Nā Antibiotics e like me quinolones, nitrofurantoin
  

ʻO nā kemika ʻē aʻe, e like me nā mea i ka mothballs, hiki ke hoʻomaka i kahi ʻāpana.

Ma ʻAmelika Hui Pū ʻIa, ʻoi aku ka nui o ka hemahema G6PD i waena o nā poʻe ʻeleʻele ma mua o nā keʻokeʻo. ʻOi aku ka maʻi o nā kāne ma mua o ka wahine.

E hoʻomohala paha ʻoe i kēia ʻano inā ʻoe:

• ʻAmelika ʻAmelika
• No waena o ka Hikina Hikina kūpono, ʻo ia hoʻi nā Iudaio Kurdish a i ʻole Sephardic Iudaio
• He kāne
• Loaʻa i kahi moʻolelo ʻohana o ka hemahema

ʻO kahi ʻano o kēia maʻi ka mea maʻamau i ka poʻe keʻokeʻo o ka moʻokūʻauhau. Hoʻopili pū ʻia kēia palapala me nā ʻāpana koʻikoʻi o hemolysis. ʻOi aku ka lōʻihi o nā Episodes ma mua o nā ʻano ʻē aʻe o ka maʻi.

ʻAʻole hōʻike ka poʻe me kēia ʻano i nā hōʻailona o ka maʻi a hiki i ka wā e hōʻike ʻia ai nā hunaola koko ʻulaʻula i kekahi mau kemika i ka meaʻai a lāʻau paha.

ʻOi aku ka maʻamau o nā ʻōuli ma nā kāne a komo pū paha me:

• Mimi ʻeleʻele
• Fiva
• ʻEha ma ka ʻōpū
• Hoʻonui ākea a me ke ake
• Ka luhi
• Pallor
• Helu puʻuwai wikiwiki
• Pokole o ka hanu
• Waihoʻoluʻu melemele (jaundice)

Hiki ke hana i kahi hōʻike koko e nānā i ka pae o G6PD.

Nā hoʻokolohua ʻē aʻe i hana ʻia e hoʻopili ʻia:

• Pae Bilirubin
• Helu piha koko
• Hemoglobin - mimi
• Pae Haptoglobin
• Hoʻāʻo LDH
• Hōʻike hoʻoliʻiliʻi Methemoglobin
• Helu helu Retikulosit

Hoʻopili paha ka mālama ʻana:

• Nā lāʻau lapaʻau e mālama ai i kahi maʻi, inā aia
• Ke kū nei i nā lāʻau āpau e hōʻino ana i ka lula koko
• Transfusions, i kekahi mau hihia

I ka hapanui o nā hihia, hele nā ​​mana hemolytic iā lākou iho.

I ka hihia maʻamau, hiki i ke kōpaʻa ʻole a make paha ma hope o kahi hanana hemolytic koʻikoʻi.

Kāhea i kāu mea mālama ola inā loaʻa nā ʻōuli o kēia ʻano.

Kāhea i kāu mea hāʻawi inā ua hōʻoia ʻia ʻoe me ka hemahema G6PD a ʻaʻole nalowale nā ​​ʻōuli ma hope o ka lapaʻau ʻana.

Pono ka poʻe me ka hemahema G6PD e hōʻalo loa i nā mea i hiki ke hoʻoulu i kahi ʻāpana. E kamaʻilio i kāu mea hoʻolako e pili ana i kāu lāʻau.

Loaʻa paha nā ʻōlelo aʻoaʻo kūmole a i ʻole nā ​​hoʻāʻo ʻana i ka poʻe i loaʻa ka moʻolelo ʻohana o kēia ʻano.

Hemahema G6PD; Hemolytic anemia ma muli o ka hemahema G6PD; Anemia - hemolytic ma muli o ka hemahema G6PD

• Nā pūnaewele koko

ʻO Gregg XT, Prchal JT. ʻO nā enzymopathies o ke koko ʻulaʻula. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 44.

Lissauer T, Carroll W. Nā maʻi Haematological. I: Lissauer T, Carroll W, eds. ʻO ka puke kākau moʻolelo no Paediatrics. 5th lā. Piladelapia, PA: Elsevier; 2018: chap 23.

ʻO Michel M. Autoimmune a me nā anemias hemolytic intravascular. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 151.