ʻO nā mea āpau āu e ʻike ai e pili ana iā Thalassemia

Anter

• Nā ʻōuli o ka thalassemia
• Nā kumu o ka thalassemia
• Nā ʻano like ʻole o thalassemia
• Kānāwai thalassemia
• Nā koho lapaʻau no ka thalassemia
• ʻO Thalassemia beta
• Thalassemia nui
• Thalassemia intermedia
• Thalassemia alpha
• Hemoglobin H
• ʻO Hydrops fetalis
• Thalassemia a me ka anemia
• Thalassemia a me nā genetics
• ʻO Thalassemia ʻōpio
• ʻO Thalassemia i nā keiki
• Meaʻai no ka thalassemia
• Hōʻikeʻike
• Manaʻo ola
• Pehea e pili ai ka thalassemia i ka hāpai ʻana?
• Outlook

He aha ka thalassemia?

ʻO Thalassemia kahi maʻi koko hoʻoilina i hana ʻia ai ke kino i kahi ʻano hemoglobin. ʻO Hemoglobin ka mole protein i loko o nā hunaola ʻulaʻula e lawe i ka oxygen.

ʻO ka hopena ka hopena i ka luku nui ʻia o nā hunaola ʻulaʻula, e alakaʻi ai i ka anemia. ʻO ke Anemia kahi ʻano i lawa ʻole ai ke kino o nā kino ʻulaʻula olakino.

Ua hoʻoilina ʻia ʻo Thalassemia, ʻo ia hoʻi ma ka liʻiliʻi o hoʻokahi o kou mau mākua e pono ke lawe i ka maʻi. Hoʻokumu ʻia ia e ka mutation mutation a i ʻole ka holoi ʻia ʻana o kekahi mau ʻāpana gene kī.

ʻO Thalassemia ʻōpio kahi ʻano koʻikoʻi o ka maʻi. ʻElua ʻano nui o ka thalassemia i ʻoi aku ke koʻikoʻi. I ka alpha thalassemia, ma ka liʻiliʻi o kekahi o nā ʻāpana alpha globin he hoʻololi a he pono ʻole paha. I ka beta thalassemia, hoʻopili ʻia nā genes beta globin.

ʻO kēlā me kēia ʻano o ka thalassemia he mau subtypes ʻokoʻa. ʻO ke ʻano kikoʻī āu e hoʻopili ai i ka paʻakikī o kāu mau ʻōuli a me kou nānā.

Nā ʻōuli o ka thalassemia

Hiki ke loli nā ʻōuli o ka thalassemia. ʻO kekahi o nā mea maʻamau e hoʻopili pū me:

• deformities iwi, o ka oi aku i ka maka
• mimi ʻeleʻele
• lohi ka ulu ʻana a me ka hoʻomohala ʻana
• luhi nui loa a luhi hoʻi
• melemele a ʻili ʻili

ʻAʻole ʻike ʻia nā kānaka āpau o nā thalassemia. Hōʻike pū nā hōʻailona o ka maʻi i hope ma ka wā kamaliʻi a ʻōpio paha.

Nā kumu o ka thalassemia

Hana ʻia ʻo Thalassemia ke loaʻa kahi abnormality a i ʻole hoʻololi i kekahi o nā gen i pili i ka hana hemoglobin. Loaʻa iā ʻoe kēia hana ʻino mai nā mākua mai.

Inā hoʻokahi o kou mau mākua kahi lawe no thalassemia, hiki iā ʻoe ke hoʻomohala i kahi ʻano o ka maʻi i kapa ʻia ʻo thalassemia minor. Inā kū kēia, ʻaʻole paha ʻoe e loaʻa i nā ʻōuli, akā e lawe ʻoe. ʻO kekahi poʻe me ka thalassemia ʻōpio e hoʻomohala i nā ʻōuli liʻiliʻi.

Inā ʻo nā mākua ʻelua nā mea lawe i ka thalassemia, loaʻa iā ʻoe kahi manawa ʻoi aku ka nui o ka hoʻoili ʻana i kahi ʻano koʻikoʻi o ka maʻi.

i ka poʻe mai ʻAsia, ka Hikina Waena, ʻApelika, a me nā ʻāina Mediterranean e like me Helene a me Turkey.

Nā ʻano like ʻole o thalassemia

ʻEkolu ʻano nui o thalassemia (a ʻehā mau subtypes):

• beta thalassemia, i komo pū me nā subtypes nui a me intermedia
• alpha thalassemia, i komo pū me nā subtypes hemoglobin H a me nā hydrops fetalis
• thalassemia ʻōpio

Hoʻololi kēia mau ʻano āpau a me nā subtypes i nā ʻōuli a me ke koʻikoʻi. Hiki ke loli iki ka hoʻomaka ʻana.

Kānāwai thalassemia

Inā ke hoʻāʻo nei kāu kauka e hōʻoia i ka thalassemia, e lawe paha lākou i kahi hōʻailona koko. E hoʻouna lākou i kēia hāpana i kahi lab e hoʻāʻo no ka anemia a me ka hemoglobin maʻamau. E nānā pū kekahi kauka loea i ke koko ma lalo o kahi microscope e ʻike inā ʻano ʻokoʻa ke ʻano o nā hunaola ʻulaʻula.

ʻO nā kinona koko ʻulaʻula ke ʻano o ka thalassemia. E hana paha ka mea loea hana i kahi hoʻokolohua i kapa ʻia ʻo hemoglobin electrophoresis. Hoʻokaʻawale kēia hoʻokolohua i nā mole ʻokoʻa i nā hunaola ʻulaʻula, e ʻae iā lākou e ʻike i ka ʻano ʻokoʻa.

Kaukaʻi ʻia i ke ʻano a me ke koʻikoʻi o ka thalassemia, kōkua paha kahi hoʻokolokolo kino i kāu kauka e hana i kahi hōʻailona. ʻO kahi laʻana, ʻōlelo paha kahi ʻāpala hoʻonui nui ʻia i kāu kauka ua loaʻa ʻoe i ka maʻi hemoglobin H.

Nā koho lapaʻau no ka thalassemia

Pili ka lāʻau no thalassemia i ke ʻano a me ke koʻikoʻi o nā maʻi e pili ana. E hāʻawi aku kāu kauka iā ʻoe i kahi papa hana e ʻoi aku ka maikaʻi no kāu hihia ponoʻī.

ʻO kekahi o nā ʻano hana me:

• hoʻoliʻiliʻi koko
• hoʻoiho iwi
• nā lāʻau a me nā mea hoʻohui
• hiki ke ʻoki kino e hemo i ka spleen a i ʻole ka gallbladder

Kauoha paha kāu kauka iā ʻoe mai lawe i nā huaora a i ʻole nā ​​mea hoʻopili i piha i ka hao. Heʻoiaʻiʻo loa kēia inā pono ʻoe i ke koko no ka mea ʻo ka poʻe e loaʻa iā lākou e hōʻiliʻili i ka hao keu i hiki ʻole i ke kino ke hemo maʻalahi. Hiki i ka hao ke kūkulu i loko o nāʻaʻaʻa, kahi hiki ke make.

Inā loaʻa iā ʻoe kahi transfusion o ke koko, pono paha ʻoe i ka chelation therapy. Pili kēia i ka loaʻa ʻana o ka pahu o kahi kemika e hoʻopili me ka hao a me nā metala kaumaha ʻē aʻe. Kōkua kēia i ka hoʻohemo ʻana i ka hao keu mai kou kino.

ʻO Thalassemia beta

Kū ka beta thalassemia ke hiki ʻole i kou kino ke hana i ka beta globin. ʻElua mau gen, hoʻokahi mai kēlā me kēia makua, hoʻoilina e hana i ka beta globin. Hele mai kēia ʻano thalassemia i loko o ʻelua subtypes koʻikoʻi: thalassemia nui (Cooley's anemia) a me thalassemia intermedia.

Thalassemia nui

ʻO Thalassemia nui ka mana nui loa o ka beta thalassemia. Kūkulu ia ke nalowale nā ​​genes beta globin.

Hōʻike maʻamau nā ʻōuli o ka thalassemia nui ma mua o ka hānau hānau ʻana o ke keiki. ʻO ka anemia koʻikoʻi e pili ana i kēia ʻano hiki ke hoʻoweliweli i ke ola. ʻO nā hōʻailona ʻē aʻe a me nā ʻōuli e like me:

• fussiness
• paleness
• nā maʻi pinepine
• he ʻai ʻilihune
• holomua ʻole
• jaundice, kahi melemele o ka ʻili a i ʻole nā ​​keʻokeʻo o nā maka
• nā mea hoʻonui i hoʻonui ʻia

ʻO kēia ʻano thalassemia ka mea maʻa mau loa e koi ai i nā transfusions koko maʻamau.

Thalassemia intermedia

ʻO Thalassemia intermedia kahi ʻano ʻoi aku ka paʻakikī. Hoʻolālā ia ma muli o nā loli i nā ʻaoʻao beta globin ʻelua. ʻAʻole pono ka poʻe me thalassemia intermedia i ke kuhi koko.

Thalassemia alpha

Kū ka Alpha thalassemia ke hiki ʻole i ke kino ke hana i ka alpha globin. I mea e hana ai i ka alpha globin, pono ʻoe i ʻehā mau gen, ʻelua mai kēlā me kēia makua.

ʻO kēia ʻano thalassemia he ʻelua ʻano koʻikoʻi kona: hemoglobin H maʻi a me hydrops fetalis.

Hemoglobin H

Hoʻokumu ʻo Hemoglobin H e like me ka nalowale ʻana o kekahi kanaka i ʻekolu mau ʻāpana alpha globin a i ʻole nā ​​ʻike e loli i kēia mau ʻaoʻao. Hiki i kēia maʻi ke alakaʻi i nā pilikia iwi. Paha paha nā papalina, ka lae, a me ka iwi āpau. Hoʻohui ʻia, hiki i ka maʻi hemoglobin H ke kumu:

• ʻōmaʻomaʻo ʻōiwi
• he ʻāpala hoʻonui nunui
• hana ʻino ʻole

ʻO Hydrops fetalis

ʻO Hydrops fetalis kahi ʻano koʻikoʻi loa o ka thalassemia i kū ma mua o ka hānau ʻana. ʻO ka hapa nui o nā pēpē me kēia ʻano hanau hānau a make paha ma hope koke o ka hānau ʻana. Kūkulu kēia ʻano ke hoʻololi a nalowale paha nā ʻāpana alpha globin ʻehā.

Thalassemia a me ka anemia

Hiki i ka Thalassemia ke alakaʻi koke i ka anemia. Kuhi ʻia kēia ʻano e ka nele o ka oxygen e lawe ʻia i nā pūnaewele. Ma muli o ke kuleana o nā hunaola ʻulaʻula no ka lawe ʻana i ka oxygen, hoʻemi ʻia kahi helu o kēia mau hunaʻa ʻaʻole lawa ʻoe i ka oxygen i loko o ke kino.

Māmā a paʻakikī paha kou anemia. ʻO nā ʻōuli o ka anemia me:

• ʻūpā
• luhi
• ukiuki
• pokole o ka hanu
• nāwaliwali

Hiki i ka Anemia ke hoʻolilo iā ʻoe i waho. Hiki i nā hihia koʻikoʻi ke alakaʻi i ka hōʻino ʻana o nā meaola, a he mea make ia.

Thalassemia a me nā genetics

He ʻano kūlohelohe ʻo Thalassemia. E hoʻomohala i ka thalassemia piha, lāua ʻelua o kou mau mākua pono e halihali i ka maʻi. A ʻo kahi hopena, e loaʻa iā ʻoe ʻelua mau ʻaoʻao mutated.

Hiki nō hoʻi ke lilo i mea lawe i ka thalassemia, kahi āu i hoʻokahi mutated gen a ʻaʻole ʻelua mai nā mākua ʻelua. Pono i hoʻokahi a ʻelua paha o kou mau mākua ke ʻano a i ʻole a lawe halihali o ia. ʻO kēia ka hoʻoilina iā ʻoe i kahi ʻāpana mutated mai kekahi o kou mau mākua.

He mea nui e hoʻāʻo inā loaʻa kekahi o kou mau mākua a i ʻole ka hoahānau i kekahi ʻano o ka maʻi.

ʻO Thalassemia ʻōpio

I nā hihia liʻiliʻi alpha, ua nalowale ʻelua mau gen. I ka beta minor, ua nalowale hoʻokahi moʻokūʻauhau. ʻAʻole loaʻa nā ʻōuli o ka poʻe me ka thalassemia minor.Inā lākou e hana, he anemia liʻiliʻi paha ia. Hoʻokaʻawale ʻia kēia ʻano ma ke ʻano he alpha a i ʻole beta thalassemia ʻōpio.

ʻOiai ke kumu ʻole o ka thalassemia minor i kekahi mau hōʻailona ʻike ʻia, hiki nō iā ʻoe ke lawe i nā maʻi. ʻO kēia ke kumu, inā he mau keiki kāu, hiki iā lākou ke hoʻomohala i kekahi ʻano o ka hoʻohuli gen.

ʻO Thalassemia i nā keiki

ʻO nā pēpē āpau i hānau ʻia me ka thalassemia i kēlā me kēia makahiki, ua manaʻo ʻia he 100,000 ka hānau ʻia ʻana me nā ʻano koʻikoʻi a puni ka honua.

Hiki i nā keiki ke hoʻomaka i ka hōʻike ʻana i nā ʻōuli o ka thalassemia i ko lākou mau makahiki ʻelua i ke ola. ʻO kekahi o nā hōʻailona ʻike nui loa ʻia e:

• luhi
• ʻōmaʻomaʻo ʻōiwi
• ʻili mae
• ʻai ʻilihune
• ulu lohi

He mea nui e hōʻoia koke i ka thalassemia i nā keiki. Inā ʻo ʻoe a ʻo ka makua ʻē aʻe paha o kāu keiki he mau halihali, pono ʻoe e hana i ka hoʻokolohua i ka wanaʻao.

Ke waiho maikaʻi ʻole ʻia, hiki i kēia ʻano ke alakaʻi i nā pilikia i ke ake, ka puʻuwai, a me ka pūpū. ʻO nā maʻi āpau a me ka puʻuwai puʻuwai ka pōpilikia weliweli ola o ka thalassemia i nā keiki.

E like me nā mākua, pono i nā keiki me ka thalassemia koʻikoʻi ke hoʻololi i ke koko e hoʻopau i ka nui o ka hao i ke kino.

Meaʻai no ka thalassemia

ʻO kahi momona momona, nā mea kanu i nā mea kanu ke koho maikaʻi loa no ka hapanui o ka poʻe, me nā mea me ka thalassemia. Eia nō naʻe, pono paha ʻoe e kaohi i nā meaʻai waiwai hao inā ʻoe i kiʻekiʻe nā kiʻekiʻe o ka hao i kou koko. Nui ka iʻa a me nā iʻa i ka hao, no laila pono ʻoe e kaohi i kēia mau mea i kāu papaʻai.

E noʻonoʻo paha ʻoe i ka hōʻalo ʻana i nā cereala paʻa, nā berena, a me nā wai momona. Loaʻa iā lākou nā pae hao kiʻekiʻe.

Hiki i ka Thalassemia ke kumu i nā hemahema folic (folate). ʻIke maoli ʻia i nā meaʻai e like me nā lau ʻōmaʻomaʻo a me nā legume, pono kēia vitamin B no ka pale ʻana i nā hopena o nā kiʻekiʻe hao a me ka pale ʻana i nā hunaola ʻulaʻula. Inā ʻaʻole lawa ʻoe i ka waikawa folic i kāu papaʻai, hiki i kāu kauka ke koi aku i kahi waihona 1 mg i lawe ʻia i kēlā me kēia lā.

ʻAʻohe meaʻai i hiki ke hoʻōla i ka thalassemia, akā e hōʻoia e ʻai i nā meaʻai kūpono hiki ke kōkua. E kūkā kamaʻilio e pili ana i nā loli dietary me kāu kauka i mua o ka manawa.

Hōʻikeʻike

ʻOiai ʻo ka thalassemia kahi maʻi maʻi, ʻaʻohe ala e pale aku ai. Eia nō naʻe, aia kekahi mau ala e hiki ai iā ʻoe ke mālama i ka maʻi e pale aku ai i nā pilikia.

Ma waho aʻe o ka mālama olakino hoʻomau ʻana, pale nā ​​kānaka āpau me nā maʻi iā lākou iho i nā maʻi ma ka mālama ʻana i nā lāʻau āpau:

• haemophilus influenza ʻano b
• maʻi hepa
• meningococcal
• pneumococcal

Ma waho aʻe o ka papaʻai olakino, hiki i ka hoʻoikaika kino mau ke kōkua i ka mālama ʻana i kāu mau ʻōuli a alakaʻi i kahi wānana maikaʻi aʻe. Paipai pinepine ʻia ka hoʻoikaika kino ikaika, ʻoiai ka hoʻoikaika kino hiki ke hōʻino i kāu mau ʻōuli.

ʻO ka hele wāwae a me ka holo kaʻa kekahi mau laʻana o ka hoʻoikaika kino waena. ʻO kaʻauʻau a me ka yoga kekahi koho ʻē aʻe, a maikaʻi nō hoʻi ia no kou mau ami. ʻO ke kī e ʻimi i kahi mea āu e hauʻoli ai a neʻe mau i ka neʻe.

Manaʻo ola

He maʻi nui ʻo Thalassemia i hiki ke alakaʻi i nā pōpilikia hoʻoweliweli ke waiho ʻia a mālama ʻole ʻia. ʻOiai paʻakikī e kuhikuhi i kahi manaolana ola, ʻo ka lula maʻamau ʻoi aku ke koʻikoʻi o ke kūlana, hiki i ka thalassemia wikiwiki ke lilo i mea make.

Wahi a kekahi mau koho, ʻo ka poʻe me ka beta thalassemia - ke ʻano ʻoi loa - make pinepine i ka makahiki 30. Pili ka lōʻihi o ke ola me ka hao o ka hao, a laila hiki ke hoʻololi i kāu mau lālā.

Ke hoʻomau nei nā mea noiʻi e ʻimi i ka hoʻokolohua kūlohelohe a me ka hiki o ka gen therapy. Kuhi ʻia ka thalassemia ma mua, ʻo ka hiki iā ʻoe ke loaʻa i ka lāʻau. I ka wā e hiki mai ana, hiki i ka gen therapy ke hoʻoulu hou i ka hemoglobin a hoʻopau i ka hoʻololi ʻana o nā genetus abnormal i loko o ke kino.

Pehea e pili ai ka thalassemia i ka hāpai ʻana?

Hāpai pū ʻo Thalassemia i nā hopohopo ʻokoʻa e pili ana i ka hāpai keiki. Hoʻopilikia ka maʻi i ka ulu ʻana o nā mea hoʻohua. Ma muli o kēia, hiki i nā wahine me ka thalassemia ke kū i nā pilikia momona.

I mea e hōʻoia ai i ke olakino o ʻolua a me kāu pēpē, he mea nui e hoʻolālā ma mua o ka manawa e like me ka hiki. Inā makemake ʻoe e loaʻa kahi pēpē, e kūkākūkā me kāu kauka e hōʻoia ai aia ʻoe i ke olakino maikaʻi loa.

Pono e nānā pono ʻia kāu pae hao. Nānā pū ʻia nā pilikia preexisting me nā mea nui.

ʻO ka hoʻāʻo Prenatal no thalassemia e hana ʻia ma 11 a 16 mau pule. Hana ʻia kēia i ka lawe ʻana i nā mea wai mai ka placenta a i ʻole ka fetus.

Hāpai ka hāpai i nā mea pilikia i ka wahine me ka thalassemia:

• kahi kiʻekiʻe kiʻekiʻe no nā maʻi
• mimikō hāpai
• pilikia puʻuwai
• hypothyroidism, a i ʻole thyroid haʻahaʻa
• mahuahua ka helu o ke kuhi koko
• haʻahaʻa iwi haʻahaʻa

Outlook

Inā loaʻa iā ʻoe ka thalassemia, pili kāu nānā i ke ʻano o ka maʻi. ʻO ka poʻe nona nā ʻano akahai a liʻiliʻi paha o ka thalassemia hiki ke alakaʻi maʻamau i nā ola maʻamau.

I nā hihia koʻikoʻi, hiki i ka puʻuwai puʻuwai ke hiki. ʻO nā pilikia ʻē aʻe e pili ana i ka maʻi akepaʻa, ka ulu ʻana o nā iwi kino ʻole, a me nā pilikia endocrine.

Hiki i kāu kauka ke hāʻawi iā ʻoe i ka ʻike hou aku e pili ana i kāu kuana. E wehewehe lākou i ka hiki i kāu mau hōʻailona ke kōkua i ka hoʻomaikaʻi ʻana i kou ola o ke ola a hoʻonui i kou ola.