He aha ka hemahema o G6PD? E aʻo kākou me ka makaʻu ʻole!

Ua lohe paha ʻoe i ka G6PD Deficiency? Malia paha he ʻano ʻē iki ke kani ʻana o ka inoa. Akā, he ʻano hoʻoilina maoli ia i loaʻa i nā poʻe he nui o ka honua, akā ʻo ka hapa nui o ka manawa ʻaʻole ia e hoʻoulu i nā pilikia nui. No laila, i kēia lā e kamaʻilio mākou e pili ana i ke ʻano o kēia hemahema G6PD, no ke aha e hana ʻia ai, a me nā mea e pono ai mākou e ʻike. ʻAʻohe pono e makaʻu, e wehewehe maʻalahi kākou i nā mea āpau.

He aha maoli ka hemahema o ka G6PD?

I ka ʻōlelo maʻalahi, ʻo ka hemahema o ka G6PD he ʻano hoʻoilina kahi i loaʻa ai i kou kino nā pae haʻahaʻa loa o ka G6PD. ʻAe, ke nīnau nei ʻoe i kēia manawa he aha ka G6PD. He enzyme koʻikoʻi loa ka G6PD (Glucose-6-Phosphate Dehydrogenase) i loko o ko mākou kino. ʻO ka hana nui o kēia enzyme ka pale ʻana i ko mākou mau hunaola koko ʻulaʻula mai nā pōʻino like ʻole.

Hiki mai kēia hemahema ke hānau ʻia kekahi me kahi loli, a i ʻole mutation, i loko o ka gene e hana ana i ka enzyme G6PD. ʻO ka hopena, ʻaʻole lawa ka hana ʻana o kāu mau hunaola koko ʻulaʻula i ka enzyme G6PD.

Akā eia ka mea. ʻAʻohe o ka nui o ka poʻe me ka hemahema o ka G6PD i nā hōʻailona . Noho lākou i ke ola maʻamau. Eia nō naʻe, i kekahi manawa, hiki ke hoʻoulu ʻia nā pilikia e nā "mea hoʻoulu" e like me kekahi mau lāʻau lapaʻau, nā maʻi, a i ʻole kekahi mau meaʻai (e kamaʻilio mākou e pili ana i kēia mau mea ma hope). I ia mau hihia, hiki ke ulu kahi kūlana i kapa ʻia ʻo ka anemia hemolytic . ʻO ia hoʻi, ua wāwahi koke ʻia a luku ʻia nā hunaola koko ʻulaʻula. I kekahi manawa, hiki i nā pēpē hānau hou ke loaʻa i ka jaundice koʻikoʻi ma muli o ka hemahema o ka G6PD.

ʻOi aku ka maʻi maʻamau o ka hemahema o ka G6PD ma mua o kou manaʻo. Ua manaʻo ʻia aia ma waena o 400 a me 500 miliona mau kānaka ma ka honua holoʻokoʻa i loaʻa i ke ʻano. No laila, inā loaʻa iā ʻoe, hiki i kāu kauka ke kōkua iā ʻoe e mālama i kāu mau hunaola koko ʻulaʻula ma kahi pae palekana.

He aha nā hōʻailona o ka hemahema o ka G6PD?

E like me kā mākou i ʻōlelo ai ma mua, ʻo ka hapa nui o ka poʻe me ka hemahema o G6PD ʻaʻole lākou e loaʻa nā hōʻailona. Eia nō naʻe, ʻike wale ʻia nā hōʻailona ke kau kahi mea hoʻoulu i ke kaumaha ma luna o kāu mau ʻulaʻula koko a hoʻoulu iā lākou e haki (hemolysis). Ke hana ʻia kēlā, hiki ke hana ʻia nā mea e like me kēia:

• Manaʻo luhi loa
• Puʻuwai wikiwiki ( tachycardia )
• Ka paʻakikī o ka hanu ʻana ( Dyspnea )
• Melemele o ka ʻili a i ʻole nā ​​​​keʻokeʻo o nā maka (he mau hōʻailona kēia o ka jaundice )
• ʻIli keʻokeʻo (hiki i nā lehelehe a me ke alelo ke keʻokeʻo)
• Mimi melemele ʻeleʻele, ʻalani, a i ʻole ke kala tī
• ʻO ka spleen i hoʻonui ʻia

Inā loaʻa koke kēia mau hōʻailona a koʻikoʻi, ua kapa ʻia ia he pilikia hemolytic . Inā ʻike ʻoe i kēia , he mea nui loa ia e ʻimi koke i ke kōkua lapaʻau.

Nā hōʻailona o ka hemahema o G6PD i nā keiki hānau hou

ʻAʻole paha e ʻike ʻia nā hōʻailona maopopo o ka hemahema o ka G6PD i nā pēpē hānau hou. ʻO ka hōʻailona maʻamau ka jaundice . ʻIke pinepine ʻia kēia i loko o kekahi mau lā o ka hānau ʻana. Inā ʻaʻole mālama pono ʻia, hiki i ka jaundice koʻikoʻi ke hōʻeha i ka lolo o ka pēpē. Ua kapa ʻia kēia ʻo kernicterus . ʻO ia ke kumu e hoʻāʻo ai nā kauka i nā pēpē hānau hou no ka hemahema o ka G6PD inā manaʻo lākou he hemahema ia.

He aha ke kumu o ka hemahema o ka G6PD?

ʻO ka hemahema o ka G6PD ke kumu o kahi ʻokoʻa (variant) i loko o kāu gene G6PD. Pale kēia ʻokoʻa i kou kino mai ke kuhikuhi pono ʻana iā ʻoe e hana i ka enzyme G6PD. Loaʻa iā ʻoe kēia ʻokoʻa genetic mai kou mau mākua, ma o ka chromosome X.

ʻO ka loaʻa ʻana o kēia ʻano hoʻololi genetic, ʻo ia hoʻi, he haʻahaʻa nā pae o G6PD i loko o kāu mau hunaola koko ʻulaʻula. He mea nui loa ka enzyme G6PD no ka mea e pale ana i nā hunaola koko ʻulaʻula mai ka hōʻiliʻili ʻana i nā " radicals free" he nui. ʻO nā radicals free he mau mea ʻino ʻole ia. Hiki ke loaʻa iā lākou ma ke kaiapuni, ma kekahi mau meaʻai (nā pī fava, no ka laʻana), a ma nā lāʻau lapaʻau.

Eia nō naʻe, inā ʻaʻole lawa kāu G6PD, hiki i kekahi mau mea hoʻonāukiuki (e laʻa me ka ʻai ʻana i nā pīni fava) ke hana i ka nui o kēia mau radical manuahi e kūkulu i loko o kāu mau cell. Hoʻoulu kēia i kahi kūlana i kapa ʻia ʻo oxidative stress , kahi e kau ai i ke kaumaha ma luna o kāu mau cell koko ʻulaʻula. I ka hopena, haki kēia mau cell a luku ʻia. Hoʻemi kēia i ka helu o nā cell koko ʻulaʻula, e hana ana i ka anemia hemolytic .

He aha nā kumu no ka anemia o ka hemolysis e pili ana i kēia maʻi?

Wahi a kekahi poʻe noiʻi, ʻo ka ʻai ʻana i nā pīni fava ka mea hoʻoulu maʻamau . Inā loaʻa i kahi kanaka me ka hemahema G6PD ka anemia ma hope o ka ʻai ʻana i nā pīni fava, ua kapa ʻia ʻo ia he favism . ʻO nā mea hoʻoulu maʻamau ʻē aʻe:

• Nā maʻi: Nā maʻi e like me ka Hepatitis A a me ka Hepatitis B , ka Typhoid Fever , a me ka Pneumonia .
• ʻO kekahi mau lāʻau lapaʻau i hoʻohana ʻia no ka malaria: No ka laʻana , ʻo Primaquine .
• ʻO kekahi mau lāʻau antibiotic : e like me ka nitrofurantoin , dapsone, a me nā lāʻau sulfa .
• Nā NSAID (nā lāʻau hoʻopau ʻeha): E like me ka aspirin a me ka ibuprofen .
• Ka puhipaka a me ka inu nui ʻana i ka waiʻona.
• Ka pilikia nui o ka noʻonoʻo.
• Hoʻoikaika kino ikaika.

He aha nā kumu pilikia?

Aia kekahi mau kumu e hoʻonui ai i ka pilikia o ka hoʻoilina ʻana i ka hemahema G6PD:

• Kāne/Wahine:ʻOi aku ka nui o ka loaʻa ʻana o ka hemahema o ka G6PD i nā kāne. ʻO kēia no ka mea hoʻokahi wale nō chromosome X a nā kāne e lawe nei i kēia hoʻololi genetic. (ʻOiai he ʻelua chromosome X a nā wahine, aia kekahi mau manawa e hiki ai ke hoʻoponopono ʻia kahi pilikia me kekahi e kekahi.)
• Kahi honua: He mea maʻamau kēia ʻano i waena o ka poʻe e noho ana ma ʻApelika hema o ka Sahara, ka ʻāina Mediterranean, a me Asia Hikina Hema.
• Lāhui: Manaʻo nā kānaka noiʻi he ʻoi aku ma mua o 10% o nā kāne o ka hanauna ʻApelika ma ʻAmelika Hui Pū ʻIa i nele i ka G6PD.

Pehea e ʻike ʻia ai ka hemahema o ka G6PD?

ʻO ka maʻamau, e nīnau mua nā kauka e pili ana i kāu mōʻaukala olakino piha a hana i kahi hoʻokolokolo kino. Hiki iā lākou ke nīnau inā ua hoʻololi koke ʻoe i nā lāʻau lapaʻau a i ʻole ua loaʻa i kekahi mau maʻi. E nānā pū lākou e ʻike inā loaʻa i kekahi o kou ʻohana ka hemahema o G6PD.

ʻO nā hoʻokolohua i hoʻohana ʻia no ka ʻike ʻana i ka hemahema o G6PD:

• Helu Koko Piha (CBC): Nānā i ka helu o kāu mau hunaola koko ʻulaʻula.
• ʻO ka Peripheral Blood Smear: Nānā i nā hōʻailona o ka anemia i loko o kahi paʻi koko, ʻo ia hoʻi, no nā hunaola koko ʻulaʻula i ke ʻano a i ʻole ka nui maʻamau ʻole.
• Hoʻāʻo Bilirubin: E nānā i ka pae o ka bilirubin, kahi huahana ʻōpala i hana ʻia mai nā hunaola koko ʻulaʻula i wāwahi ʻia.
• Hoʻāʻo G6PD: E nānā i kāu mau pae enzyme G6PD.

Pehea e mālama ʻia ai ka hemahema o ka G6PD?

Hoʻōla nā kauka i ke kūlana ma muli o ke kūlana. No ka laʻana, inā he jaundice iki kou a ʻike ʻoe he hemahema G6PD kou, e mālama mua lākou i nā hōʻailona o ka jaundice. A laila, e haʻi lākou iā ʻoe i nā mea hoʻonāukiuki āu e pono ai e pale aku.

Eia nō naʻe, inā koʻikoʻi nā hōʻailona, ​​ʻokoʻa ka lāʻau lapaʻau. Inā loaʻa iā ʻoe ka anemia hemolytic koʻikoʻi, pono paha ʻoe i ka hoʻoili koko .

Inā loaʻa i kāu pēpē hānau hou ka jaundice, hiki i kāu kauka ke mālama iā ia me ka phototherapy (kahi lāʻau e hoʻohana ana i ka mālamalama kūlohelohe a i ʻole ke kukui hana). I nā hihia koʻikoʻi, pono paha i kāu pēpē ke loaʻa kahi hoʻololi koko . Pili kēia i ka wehe ʻana i ke koko maikaʻi ʻole o ka pēpē a hoʻololi iā ia me ke koko olakino i hāʻawi ʻia.

Hiki ke pale ʻia ka hemahema o ka G6PD?

ʻOiai he maʻi hoʻoilina kēia, ʻaʻole hiki ke pale aku iā ia mai ka hanana ʻana. Eia nō naʻe, aia nā ala e ʻike ai ma mua o kona hoʻoulu ʻana i nā pilikia olakino nui. ʻO kēia mau mea:

• Ke nānā ʻana i nā pēpē hānau hou: Ma nā ʻāina he nui kahi i maʻamau ai kēia ʻano, ua hoʻokumu ʻia nā papahana nānā e ʻike i ka hemahema o ka G6PD i nā pēpē hānau hou.
• Ka hoʻāʻo ʻana i ka hoʻoilina: Inā loaʻa i kekahi o kou ʻohana ka hemahema o ka G6PD, pono paha ʻoe i kahi hoʻāʻo DNA.Hiki i kāu kauka ke aʻo aku iā ʻoe e hana pēlā.

He aha kaʻu e manaʻo ai inā loaʻa iaʻu kēia maʻi?

ʻAʻohe lāʻau lapaʻau mau loa no ka hemahema o ka G6PD. Eia nō naʻe, hiki i ka hapa nui o ka poʻe ke ola me ka ʻole o nā pilikia inā pale lākou i nā mea hoʻonāukiuki. A ʻaʻole ia e hoʻopilikia i ke ola.

Eia nō naʻe, ʻaʻole like ka hopena o kēia maʻi i nā kānaka a pau. Nui ka poʻe me ka hemahema o G6PD ʻaʻole paha e ʻike he loaʻa iā lākou ia mea no ka mea ʻaʻohe o lākou hōʻailona. ʻO ka hapa nui o ka poʻe i loaʻa nā hōʻailona he akahai loa lākou. Eia nō naʻe, no kekahi poʻe, i ka wā e hōʻike ʻia ai i kahi mea hoʻonāukiuki, hiki iā lākou ke hoʻomohala i kahi pilikia hemolytic, kahi hiki ke hoʻoweliweli i ke ola.

Hiki i kāu kauka ke wehewehe pono i ka mea hiki iā ʻoe ke manaʻo ma muli o kāu ʻike.

Pehea e hiki ai iaʻu ke mālama iaʻu iho?

E nīnau i kāu kauka i nā meaʻai a me nā lāʻau lapaʻau āu e pale aku ai. Eia kekahi mau manaʻo ʻē aʻe:

• E hōʻemi i ka inu ʻona: ʻO ka inu ʻana i ka waiʻona nui e hiki ke kau i ke kaomi ma kāu mau ʻula koko ʻulaʻula.
• Hōʻalo i ka puhipaka: Hiki i ka puhipaka ke hoʻoulu i nā radical manuahi e hōʻiliʻili i loko o kāu mau ʻula koko ʻulaʻula.
• Hoʻomaʻamaʻa kaulike: ʻO ka hoʻomaʻamaʻa ikaika loa hiki ke hoʻonui i ke kaumaha oxidative.
• E ho'āʻo e loaʻa ka hoʻomaha lawa: Hoʻoikaika ka hiamoe i kāu ʻōnaehana pale, kahi e kōkua ai i ka hakakā ʻana i nā maʻi e hiki ai ke hoʻoulu i ka anemia i loko o ka hoʻoili koko.
• E hoʻokele i ke kaumaha: Inā ʻoe e manaʻo nei i ka nui o ke kaumaha, e noi i kāu kauka no ke kōkua i ka hoʻokele ʻana i kou kaumaha.

He mea nui: Inā loaʻa i kāu keiki hānau hou ka hemahema o ka G6PD, pono ʻoe e pale i ka ʻai ʻana i nā pīni fava i ka wā e hānai waiū ana . Hiki i nā mea i hiki ke hoʻoulu i ka anemia pernicious ke hele i ka pēpē ma o ka waiū umauma.

Āhea wau e ʻike ai i kaʻu kauka?

E ʻike i kāu kauka i kēlā me kēia manawa inā loaʻa iā ʻoe nā hōʻailona o ka hemahema o G6PD. Inā koʻikoʻi nā hōʻailona a ulu koke (nā hōʻailona o ka pilikia hemorrhagic), e ʻimi koke i ke kōkua lapaʻau .

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

Eia kekahi mau nīnau e nīnau ai inā loaʻa iā ʻoe ka hemahema o G6PD:

• Pehea ke koʻikoʻi o koʻu kūlana?
• He aha nā meaʻai a me nā lāʻau lapaʻau e pono iaʻu ke pale aku?
• Aia kekahi mau mea hoʻonāukiuki o ke kaiapuni e pono iaʻu e pale aku?
• He aha nā manawa kūpono e loaʻa ai i kaʻu keiki ka hemahema G6PD?

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai (Leka Lawe-Home)

Hiki i ka ʻike ʻana o ka hemahema G6PD ke hoʻopilikia i kēlā me kēia kanaka i nā ʻano like ʻole. ʻOiai ʻaʻole hiki ke ho'ōla ʻia, he kūlana maʻamau ia e hiki ke hoʻokele ʻia. ʻO ke kī, ʻo ia ka ʻike a me ka pale ʻana i nā mea hoʻoulu e hoʻonui ai i ka pilikia o ka loaʻa ʻana o ka anemia pernicious.ʻO ia hoʻi paha ke pale ʻana i nā pīni fava a me nā mea hoʻonāukiuki ʻē aʻe. He mea nui hoʻi e hoʻomaʻamaʻa i nā ʻano olakino, e like me ka hoʻomaha nui a me ka puhi ʻole ʻana. E noi i kāu kauka no ka ʻike e kōkua iā ʻoe e hoʻokele pehea e pili ai ka hemahema o G6PD i kou ola. Mai hopohopo, ʻo ka ʻike ka pale maikaʻi loa!

Ka hemahema o ka G6PD, ka anemia hemolytic, ka jaundice, nā maʻi genetic, nā hunaola koko ʻulaʻula, nā pīni fava, nā enzymes