ʻO ke kaumaha o ka hemoglobin
Hiki i ka hemoglobin mau keʻano o nā kaulahaoʻehā (globins), e pili ana i kēlā me kēia mea me kahi moleuma halihali oxygen - heme. ʻEluaʻano o ka honua - alpha and beta globins. ʻO ka huiʻana o nā huiʻelua, he mole lākou o ka hemoglobin. ʻO ka hanaʻana o nāʻano likeʻole o nā ao āpau, ua kaulikeʻia, no laila, aia lākou i ka likeʻana o ke ana o ke koko. Me ka thalassemia, ua hoʻopauʻia ka inoa o ka alpha-a me ka betaglobins, a hiki i ka hopena i ka hanaʻana o ka mole hemoglobin. ʻO ka nele o ka hemoglobin maʻamau i loko o ke koko e hiki ai i ka uluʻana o ka anemia a me nā mea'ē aʻe o thalassemia.
ʻEluaʻano nui o thalasmaemia:
- beta-thalassemia - pili i ka 'aʻe' ana i ka beta-gloss;
- alpha-thalassemia - keʻano o ka uhaʻiʻana i nā mele o nā'āpana alpha o ke ao. ʻO nā maʻiʻelua he mauʻano liʻiliʻi lākou, he likeʻole i ka paʻakikī. ʻO Alfa-thalassemia ma konaʻano a pau heʻoi aku ka māmā.
ʻEkolu mauʻano beta-thalassemia: ka thalassemia liʻiliʻi, ka thalassemia ma waena a me ka thalassemia nui. ʻO nā hōʻailona o ka liʻiliʻi liʻiliʻi liʻiliʻi ka liʻiliʻi, akāʻo nā mea halihali i nā maʻi kinoʻole e hoʻololi ana ia mea i kā lākou mau keiki ma kahiʻanoʻoi aku. Ua kākauʻia nā hihia o ka beta-thalassemia ma ka honua holoʻokoʻa, akā,ʻoi aku ka maʻalahi ma ka Mānāwaike a me nā'āina o ka Middle East. ʻO ka hapanui o 20% o ka heluna kanaka o kēia mau māhele (ʻoi aku ma mua o 100 miliona mau kānaka) ka nui o ka thalassemia. ʻO ia ka uluʻana o kēia maʻi i nā Mānālepa'āina e wehewehe ana i kona inoa ("thalassemia" i unuhiʻia he "anemia Mediterranean").
Nā ho'āʻo koko
ʻO ka poʻe maʻi me ka maʻi beta-thalassemia pinepine i ka noʻonoʻo aʻaʻole hoʻi e nānā i ka maʻi. ʻO ka hapanui o ka manawa, uaʻikeʻia ia ma ka hopena i ka hoʻonāʻana i ke koko. Hoʻomaopopoʻia ka anemia maʻamau i nā maʻi. ʻO keʻano o ke kokoʻeleʻele o ke koko, ua like loa me ka anemia o ka hao. Eia naʻe, hiki i nā noiʻi hou e hiki ke hoʻokuʻu i ka hikiʻole o kahi hapa o kēia māhele. I ka hoʻonuiʻiaʻana o ka hōʻailona,ʻikeʻia kaʻano kūponoʻano nui o nā hapa o ke koko.
Mākawā
Hoʻokumu ke keiki i loaʻa kahiʻano beta-thalassemia maʻamau aʻaʻole pono ka lāʻau lapaʻau, akā hiki paha iā ia ke predisposed i ka anemia ma kahi mamao loa, e like paha, i ka hāpaiʻana a i ka manawa o nā maʻi maʻi. Hoʻokumu ka beta-thalassemia liʻiliʻi ma keʻano he mea palekana e kū'ē i ka maʻi malaria. Hiki i kēia ke ho'ākāka i ka kiʻekiʻe o ka thalassemia i ka Middle East. Hanaʻia ka nui o ka beta-thalassemia i ka wā i loaʻa ai i nā keiki nā kupapaʻu kūlohelohe e pili ana i ka inoa o ka beta-globin mai nā mākuaʻelua, aʻaʻole i hāʻawi mai kona kino i ka beta-globin i kahi kūpono. I ka manawa hoʻokahiʻaʻole i uhaʻiʻia ke kahakahaʻana o nā kaula alpha; hana lākou i nā inclusions nonoluble i loko o nā erythrocytes, a ma muli o ia mau mea e emi iho ai kēia mauʻili o ke koko i ka nui a lilo i pale i keʻano. ʻO ka lōʻihi o kēlā me kēia erythrocytes i emi iho ka mea maʻamau, a ua emi loa ka hoʻonuiʻana i nā mea hou. Loaʻa kēia mau mea i ka anemiaʻeha. Akā naʻe,ʻaʻole i hōʻikeʻia kēiaʻano anemia, ma keʻano he kānāwai, a hiki i ka pihaʻana oʻeono mau mahina, no ka mea, i loko o nā malama mua o ka hemoglobin i kapaʻia he hemoglobin i loko o ke koko, i hoʻololiʻia e ka hemoglobin maʻamau.
Nā hōʻailona
ʻO ke keiki me kahi maʻilala nui e nānāʻole i ka maikaʻi, hikiʻole iā ia ke kūponoʻole a me ka pale, hiki ke waiho i hope i ka uluʻana. Hiki paha i nā keiki ke emi iho i ka makemake,ʻaʻole lākou e lawe i ka kaumaha, hoʻomaka lākou e hele lōʻihi. Hoʻokumu kekahi keiki maʻi i ka anemia maʻamau i hele pū me nā maʻi iʻikeʻia:
- he helehelena hiʻohiʻona (kahi iʻikeʻiaʻo Mongoloid);
- ʻO nā hoʻololi kūikawā i nā iwi - ka mea i kapaʻia he "poʻoleo lauoho" (ʻano periostosis a me ke ahe) e pili ana i ka hyperplasia e ka iwi he radioio;
- ka lahilahi a me ka haʻi o nā iwi.
ʻO Thalassemia he maʻi maʻiʻole. ʻO nā maʻi i loaʻa i nā maʻi pili i kēlā me kēia ola. Hiki ke hoʻopau i ka ola o ka mea maʻi ma keʻano o ka hana lāʻau. ʻO ke kumu no ka hoʻomaʻamaʻaʻana i ka nui o ka beta-thalassemia,ʻo ia ka hoʻokahe koko i nā manawa. Ma hope o ka hōʻailona, hāʻawiʻia ka mea maʻi i ke kaheʻana i ke koko,ʻo ia keʻano o ke kani i 4-6 mau wiki. ʻO ke kumu o ia hanaʻana,ʻo ia ka hoʻonuiʻana i nā kālā; nā pūpū o ke koko (ke kaulike o keʻano toto). Me ke kōkuaʻana i ke kalapiliʻana i ke koko, ua hoʻolakoʻia ka anemia, e hiki ai i ke keiki ke hoʻolālā i keʻano a pale i nā weheweheʻoi o nā iwi. ʻO ka pilikia nui e pili ana i ka hoʻokala liʻiliʻiʻana o ke koko he mea nui ka hao, a he hopena maʻino ka kino. Hiki i ka hao ke hao i ke ake, i ka naʻau a me nā mea'ē aʻe.
Hanaʻia o kaʻona
No ka hōʻokiʻana i ka pīpī, hoʻohanaʻole i ka lāʻau lapaxamine maʻamau. Hoʻokau pinepineʻia iʻewalu hora i infusions deferoxamine 5-6 mau manawa o ka pule. No ka wikiwikiʻana o ka haoʻana o ka hao nui, ua'ōleloʻia nō hoʻi ka nohoʻana o ka lāʻauʻo Camin C. He mea kaumaha loa kēia hana no ka mea maʻi a me nā mea i alohaʻia, no laila,ʻaʻole ia he mea kānalua no ka meaʻaʻole mau nā mea maʻi e mālama i nā kūʻai maʻamau. ʻO keʻano o ka hōʻailona e hoʻonui loa i ka manaʻo ola o nā poʻe maʻi me ka beta-thalassemia nui, akāʻaʻole lākou e ola. ʻO kahi'āpana liʻiliʻi wale nō o nā maʻi i ola i ka 20 makahiki,ʻaʻohe kūponoʻole ka hōʻailona no lākou. Me ka hoʻomauʻana i nā maʻi, hikiʻole i nā keiki me kēiaʻano thalassemia ke hoʻokō i ka pākeke. ʻO ka hōʻike pinepine o thalassemia,ʻo ia ka hoʻonuiʻiaʻana o ka'ōpū, me ka hui pūʻana i nā kele kokoʻulaʻula i loko o ia mea, e waiho ana i ke koko, e hoʻonui ai i ka anemia. No ka mālamaʻana i nā poʻe maʻi me ka thalassemia, i kekahi manawa lākou e kiʻi i ka weheʻana i ka splenectomy. Eia nō naʻe, i nā maʻi i hala i ka splenectomy, hiki i ka poʻe maʻi ke piʻi i ka maʻi pneumococcal. I nāʻano o ia mau mea, ua'ōleloʻia ka lāʻau lapaʻau a me nā lāʻau āpau o ke ola. Ma waho aʻe o nāʻano beta-thalassemia liʻiliʻi a me ka nui, aia kekahi mau beta-thalassemia a me ka alpha-thalassemia. ʻO ka hōʻoiaʻana o nā wāhine hāpai me nā hōʻailona liʻiliʻi liʻiliʻi e hōʻike ana i keʻano o nā maʻi kūpilikiʻi o ka maʻi ma ka'ōpiopio. Aia ke kolu o ka beta-thalassemia, i kapaʻiaʻo mideta beta-thalassemia. ʻO kēia maʻi i kaʻino ma waena o nāʻano liʻiliʻi a me nāʻano nui. I nā maʻi me ka thalassemia ma waena, ua emi iho ka liʻiliʻi o ka hemoglobin i loko o ke koko, akā lawa nō ia e hiki i ka mea maʻi ke alakaʻi i ke ola maʻamau.
Mākawā
Nui nāʻano alpha-thalassemia. I ka manawa hoʻokahi,ʻaʻohe puka mahele i loko o nāʻano nui a me nāʻano liʻiliʻi. ʻO ke kumu kēia o nā genesʻokoʻaʻehā no ka hōʻailona o nā'āpana alpha alpha e hakuʻia hemoglobin.
Paʻi ka maʻi
ʻO ka paʻakikī o ka maʻi e pili ana i ka nui o nāʻehāʻehā hikiʻole. Inā pākahi ka pahu hoʻokahi, aʻo nā mea'ē aʻeʻekolu, he mea maʻamau,ʻaʻole hōʻike ka mea maʻi i nā meaʻino i loko o ke koko. Eia naʻe, me ka hoʻomauʻana o ka hinaʻana o nā gen aʻelua pahaʻekolu. E like me ka beta-thalassemia,ʻo alpha thalassemia ka mea nui ma nā wahi i hopena no ka maʻi malaria. ʻO kēia maʻi heʻano o ka Hikina Hemaʻo Asia, akā, heʻole ia ma ka Middle East a me ka Mediterranean.
Nā diagnostics
ʻO ka hōʻailona o ka alpha-thalassemia e kauʻia ma luna o nā hopena o ka hoʻokolohuaʻana i ke koko. Hoʻokomoʻia ka maʻi me ka anemiaʻeha. I ka hoʻohālike i ka beta-thalassemia, me ka alpha-thalassemiaʻaʻole he piʻi i ka hemoglobin HA2. Hiki keʻikeʻia he nui beta-thalassemia ma mua o ka hānauʻana. Inā, ma ke kumu o ka moʻolelo a iʻole nā hopena o ka hoʻokolokoloʻana i ka hoʻolālāʻana,ʻo ka wahine ka mea halihali o ka gene no ka liʻiliʻi liʻiliʻi, e nānāʻia hoʻi ka makua kāne e hiki mai ana no ka thalassemia. Keʻikeʻia kahi'āpana maʻalialamia ma nā mākuaʻelua, hiki ke hoʻomaopopo i ka hōʻailona pololei i ka lā mua mua o ka hāpaiʻana ma ke kumu o ke koko o ka hānal. Keʻikeʻia kekahi pēpē me nā hōʻailona o ka beta-thalassemia nui, hōʻikeʻia ka'ōpū.