He kiʻekiʻe anei kou hao i loko o kou kino, akā ʻaʻole ʻoe maikaʻi? He anemia sideroblastic paha ia?

Ke luhi pinepine nei ʻoe a me ka hanu pōkole? Ke manaʻo nei ʻoe i kekahi manawa he haʻahaʻa kou koko? Hiki i kēia ke lilo i mau hōʻailona o ka anemia maʻamau. Eia nō naʻe, i kekahi manawa aia kekahi kumu paʻakikī ma hope o kēia. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi koko i kakaʻikahi iki, akā he mea nui e ʻike. ʻO ia ka anemia sideroblastic, a i ʻole e like me ka kapa ʻana o nā kauka he `(Sideroblastic Anemia - SA)`.

No laila he aha ka Anemia Sideroblastic?

I ka ʻōlelo maʻalahi, he ʻano laha ʻole kēia e hoʻopilikia ai i ke ʻano o ka hana ʻana o ko mākou kino i nā hunaola koko ʻulaʻula, nā hunaola koko ʻulaʻula i loko o ko mākou koko. Inā loaʻa iā ʻoe kēia ʻano, he haʻahaʻa kou helu o nā hunaola koko ʻulaʻula, ʻo ia hoʻi he anemia kou. Akā i ka manawa like, he mea kupanaha, he nui nō hoʻi ka hao i loaʻa iā ʻoe. No ke aha e hana nei kēia? No ka mea, ʻaʻole hoʻohana kou kino i ka hao e pono ai e hana pono i nā hunaola koko ʻulaʻula.

ʻO wai ka mea i hoʻopilikia nui ʻia e kēia kūlana?

Hiki ke loaʻa kēia maʻi i kapa ʻia ʻo `(Sideroblastic Anemia)` ma nā ʻano ʻelua. ʻO kekahi he congenital sideroblastic anemia a i ʻole CSA . ʻO ia hoʻi, ua hānau ʻia kekahi mau pēpē me kēia maʻi. ʻO kekahi he acquired sideroblastic anemia . ʻO kēia ke ʻano maʻamau. Hiki ke hana ʻia e nā maʻi ʻē aʻe e pili ana i ke koko, ka hoʻohana ʻana i kekahi mau lāʻau lapaʻau, a i ʻole ka nui o ka ʻike ʻana i kekahi mau minerala.

E noʻonoʻo wale, hiki i nā pēpē a me nā kamaliʻi ʻōpio i hānau ʻia me kēia ʻano ke loaʻa i kekahi manawa nā pilikia olakino e hoʻoweliweli ana i ke ola ma muli o ka hao nui i loko o ko lākou kino, i ʻike ʻia ʻo "iron overload." Inā loaʻa i nā pākeke kēia maʻi, hiki iā lākou ke loaʻa i nā maʻi e like me ka maʻi puʻuwai a me ka cirrhosis o ke ake.

He nūhou maikaʻi! ʻOiai ʻaʻole hiki i nā kauka ke ho'ōla loa i ka ʻAnemia hānau (Sideroblastic Anemia), aia nā lāʻau lapaʻau e hiki ke kāohi i nā hōʻailona a pale i nā pilikia koʻikoʻi. Eia nō naʻe, hiki ke ho'ōla ʻia kekahi mau ʻano o ka ʻAnemia (Sideroblastic Anemia) e ulu ana ma hope o ke ola.

ʻO kēia ʻano maʻi, ʻo ia ke ʻano i loaʻa i ka wā hānau, hiki ke hoʻopilikia i nā pēpē, nā keiki ʻōpio, a me nā mākua ʻōpio. I kekahi manawa he akahai loa nā hōʻailona o ka CSA. No laila, inā hānau ʻia ʻoe me ia, ʻaʻole paha e loaʻa i kekahi poʻe nā pilikia olakino nui a hiki i ko lākou wā ʻelemakule.

ʻO ke ʻano hope loa, i kapa ʻia ʻo "Acquired CSA," pinepine ia i nā poʻe me ka maʻi koko i kapa ʻia ʻo "myelodysplastic syndrome." Hiki ke hana ʻia e ka ʻike ʻana i nā minelala e like me ke kēpau a me ka zinc, ka hoʻohana ʻana i kekahi mau lāʻau lapaʻau, a i ʻole kekahi mea e hoʻopilikia ai i ka hiki o ke kino ke hoʻohana i ke keleawe.

Pehea ka hopena o kēia maʻi i koʻu kino?

ʻO ka poʻe me ka anemia sideroblastic e hōʻike pinepine i nā hōʻailona o ka anemia. ʻO ia hoʻi, manaʻo lākou ua luhi loa lākou me ke kumu ʻole.Manaʻo luhi, pilikia ka hanu ʻana, a pēlā aku. ʻAʻole wale ia, hiki iā lākou ke loaʻa i ka "anemia macrocytic" (nā ʻulaʻula koko nui aʻe ma mua o ka maʻamau) a me ka "anemia microcytic" (nā ʻulaʻula koko liʻiliʻi ma mua o ka maʻamau). Hiki i nā ʻano anemia ʻelua ke lilo i mau hōʻailona o nā ʻano anemia ʻē aʻe.

He aha nā kumu o ka anemia sideroblastic?

E like me kā mākou i kūkākūkā ai ma mua, hiki ke loaʻa kēia i ka wā hānau, a i ʻole hiki ke ulu aʻe ma hope ma muli o kekahi maʻi olakino ʻē aʻe, ka ʻike ʻana i kekahi mau metala, nā minelala, a i ʻole nā ​​​​lāʻau lapaʻau.

I mea e maopopo ai i nā kumu o ka anemia sideroblastic congenital (congenital SA), he mea kōkua ke ʻike iki e pili ana i ka hana ʻana o kā mākou mau hunaola koko ʻulaʻula.

He hoʻolauna pōkole i nā hunaola koko ʻulaʻula

ʻO nā hunaola koko ʻulaʻula ke ʻano maʻamau o nā hunaola i loko o ko kākou koko. He mea nui nā hunaola koko ʻulaʻula olakino a oʻo no ka lawe ʻana i ka oxygen i nā ʻiʻo o ko kākou kino. Hana nā hunaola koko ʻulaʻula i kēia me ke kōkua o kahi protein i kapa ʻia ʻo hemoglobin. ʻO ka hemoglobin ka mea e hāʻawi i nā hunaola koko ʻulaʻula i ko lākou kala ʻulaʻula.

Pono nā hunaola koko ʻulaʻula i ka hao e hana i ka hemoglobin. ʻO ka maʻamau, hana ko kākou iwi iwi i nā hunaola kumu. Ma hope aku, ulu kēia mau hunaola kumu i nā hunaola koko ʻulaʻula ʻōpiopio i kapa ʻia he erythrocytes. Loaʻa i kēia mau hunaola ʻōpiopio ka nui kūpono o ka hemoglobin a me ka hao e pono ai e hana i nā hunaola koko ʻulaʻula olakino a oʻo.

Akā, inā loaʻa iā ʻoe ka anemia sideroblastic, ʻaʻole hiki i kou kino ke hoʻohana i kēia hao. ʻElua mau pilikia kēia. Me ka ʻole o ka hao, ʻaʻole lawa ka hemoglobin o kāu mau ʻulaʻula koko e lawe i ka oxygen ma loko o kou kino. Ma ka ʻaoʻao ʻē aʻe, hōʻiliʻili ka hao i hoʻohana ʻole ʻia i loko o kou iwi iwi. Hoʻoulu kēia i kahi ʻano ʻano ʻē o ke kelepona i kapa ʻia he sideroblast apo . ʻO ka sideroblast kahi ʻulaʻula koko ʻōpiopio nona ke apo hao a puni kona nucleus. Hiki i nā kauka ke ʻike i kēia mau apo ke nānā lākou i nā cell kumu i loko o ka iwi iwi ma lalo o kahi microscope. Loaʻa ka inoa o ka anemia sideroblastic mai kēia mau apo hao. Hōʻike kēia mau apo hao e mālama ana nā cell i ka hao ma kahi o ka hoʻohana ʻana. Ua kapa ʻia kēia hōʻiliʻili hao he iron overload . ʻO kēia iron overload ke kuleana no nā pilikia olakino koʻikoʻi he nui e hiki ke hana ma muli o ka anemia sideroblastic.

He aha nā ʻano o ka Anemia Sideroblastic Congenital (CSA)?

ʻEkolu ʻano o ka SA hānau ʻana. Hoʻokumu ʻia kēia e nā mutations i loko o kekahi mau genes e hoʻopilikia i ka hana o nā hunaola koko ʻulaʻula. E nānā kākou i ko lākou ʻano:

• ʻO ka anemia sideroblastic i hoʻopili ʻia e X:ʻO kēia ke ʻano maʻamau o ka anemia hānau. Hoʻokumu ʻia ia e kahi mutation i loko o ka gene e hana ana i kahi enzyme kikoʻī i kapa ʻia ʻo "ALAS2", e hana ana i ka hemahema o ka hemoglobin. ʻO ka poʻe me kēia maʻi he mau hunaola koko ʻulaʻula palupalu, liʻiliʻi ma mua o ka maʻamau (microcytic). Loaʻa iā lākou ka hao keu i loko o ko lākou kino.
• ʻO ka anemia sideroblastic autosomal recessive (ARCSA): ʻO kēia ka lua o ke ʻano maʻamau. Hoʻopilikia ka ARCSA i nā pēpē a me nā keiki ʻuʻuku loa. He kūlana koʻikoʻi ia i kekahi manawa hiki ke hoʻoweliweli i ke ola. Hana ʻia ia ke piʻi nui ka nui o ka hao i loko o ke koko.
• Mākuahine: Ma kahi o 20% o nā maʻi CSA āpau e hāʻule ana i loko o kēia māhele. ʻO kēia no ka mea ua loli ke gene MT-ATP6, e hoʻopilikia ana i ka hana o kāu mitochondria. (ʻO Mitochondria nā organelles i loko o kā mākou mau cell. ʻO lākou ke kuleana no ke kumu ikehu nui o nā cell. Hoʻololi ʻo Mitochondria i ka ikehu mai ka meaʻai i loko o kahi ʻano e hiki ai i nā cell ke hoʻohana.)

He aha nā ʻano o ka anemia sideroblastic i loaʻa e kū mai ana ma hope o ke ola?

ʻElua ʻano o ka anemia sideroblastic e ulu aʻe ma hope o ke ola - mua a me ke lua. ʻO ka SA mua e pili ana i ka SA e pili ana i kahi kūlana i kapa ʻia ʻo myelodysplastic syndrome. ʻO ka SA lua e pili ana i ka SA hiki ke ulu ma hope o ka hōʻike nui ʻana i kekahi mau metala, nā kemika, a me nā lāʻau lapaʻau. ʻO nā mea e hiki ai ke hoʻoulu i ka anemia sideroblastic lua:

• ʻOna: ʻO ka hoʻohana ʻana i ka waiʻona ke kumu nui o ka anemia sideroblastic ma hope.
• Ka lāʻau make metala kaumaha: Hoʻokomo pū kēia i ka lāʻau make kēpau a me ka lāʻau make arsenic.
• Ka nele o ka wikamina B: Kōkua ka wikamina B i ka hana ʻana i kahi ʻāpana i kapa ʻia ʻo heme, ka mea i hoʻololi ʻia i hemoglobin. ʻO ka Heme ka mea e kōkua ai i ka lawe ʻana i ka oxygen.
• Ka nele o ke keleawe: Kōkua ke keleawe i ka hana ʻana i kahi enzyme e pale ai i ka hao keu.
• ʻO ka nui o ka zinc: ʻO ka lawe ʻana i ka nui o ka zinc hiki ke hoʻopilikia i ke ʻano o ka hoʻohana ʻana o ke kino i ka hao a me ke komo ʻana o ke keleawe. Lawe kekahi poʻe i nā mea hoʻohui zinc a hiki ke hana ʻia kēia.
• Nā lāʻau lapaʻau: Hiki i kekahi mau antibiotics, chemotherapy, hormones, a me nā lāʻau lapaʻau e hoʻopau ana i ke keleawe ke hana i ka anemia sideroblastic lua. Hoʻōla nā kauka i kēia ʻano SA ma ka hoʻāʻo ʻana i nā lāʻau lapaʻau āu e lawe nei a me ka wehe ʻana i nā lāʻau lapaʻau i manaʻo ʻia e hoʻoulu ai i ka SA.

He aha nā hōʻailona o ka anemia sideroblastic?

E like me nā ʻano anemia he nui, ʻo nā hōʻailona o ka anemia sideroblastic:

• Luhi nui:ʻO ke ʻano kēia o ka luhi loa a hiki ʻole iā ʻoe ke hana i nā hana o kēlā me kēia lā.
• Dyspnea: He manaʻo kēia o ka ʻūmiʻi ʻana a i ʻole ka paʻakikī o ka hanu ʻana.
• Nā palpitations o ka puʻuwai: He manaʻo kēia e kuʻi wikiwiki ana ka puʻuwai, a i ʻole ma ke ʻano ʻē.
• ʻEha poʻo.

Hiki i nā poʻe me ka anemia sideroblastic X-linked ke ʻike i nā hōʻailona hou aʻe ma muli o ka lawa ʻole o ka hemoglobin a me ka piʻi ʻana o nā pae hao i loko o ke kino. ʻO kēia mau hōʻailona:

• Ake i hoʻonui ʻia: Hana kou ake i nā hana koʻikoʻi he nui i loko o ke kino.
• ʻO ka spleen i hoʻonui ʻia: ʻO kou spleen kahi ʻōkana e like me ka nui o kahi lima i ʻūlū ʻia ma ka ʻōpū o luna. ʻAʻole paha ʻoe e ʻike he nui aku kou spleen ma mua o ka maʻamau, a i ʻole e ʻike paha ʻoe i kahi ʻeha palupalu ma kou ʻōpū o luna.
• ʻIli kala keleawe: Inā mālama kou kino i ka hao he nui, e lilo paha kou ʻili i kala keleawe-ʻeleʻele.
• ʻO ka maʻi diabetes mellitus i kāohi ʻole ʻia.

Pehea e ʻike ai nā kauka i ka anemia sideroblastic?

No ka mea hiki ke loaʻa ka anemia sideroblastic i ka wā hānau a i ʻole e ulu ma hope, e hoʻāʻo mua kāu kauka iā ʻoe e hōʻoia a kāpae paha i ka anemia sideroblastic i loaʻa. Inā hoʻoholo lākou ʻaʻole ia he anemia sideroblastic i loaʻa, e hana lākou i nā hoʻokolohua hou aʻe e ʻike ai i ke ʻano o ka anemia sideroblastic congenital āu. Eia kekahi o nā hoʻokolohua a lākou e hana ai:

• Helu koko piha (CBC): Hōʻike nā hopena o ka `(CBC)` i ka nui a me ke ʻano o nā ʻāpana i loko o kou koko, ehia mau ʻāpana koko hou e hana nei kou kino, a, ma ka hihia `(SA)`, ehia mau ʻāpana koko ʻulaʻula makua a me nā ʻōpiopio i loaʻa iā ʻoe.
• ʻO ka palaki koko peripheral: He kaʻina hana kēia a nā kauka e hoʻohana ai e nānā i nā hunaola koko. ʻOiai kekahi mau hoʻokolohua koko e hoʻohana i kahi mīkini e kālailai iā lākou, i kēia hihia, kālailai nā kauka i nā hunaola koko ma lalo o kahi microscope.
• Nā haʻawina hao: Hōʻike kēia hoʻokolohua i kāu mau pae hao.
• Ka ʻimi ʻana i ka iwi iwi: He kaʻina hana kēia e lawe ai i kahi hāpana o ka ʻāpana wai o kou iwi iwi.
• ʻO ka biopsy iwi iwi: Ma kēia hoʻokolohua, lawe nā kauka i kahi ʻāpana liʻiliʻi o kou iwi iwi a nānā i nā ʻāpana iwi iwi.
• Hoʻāʻo hoʻoilina:Hiki i ka hoʻāʻo ʻana i ka genetic ke kōkua i ka hōʻoia a i ʻole ke kāpae ʻana i nā kūlana genetic kikoʻī. Hiki iā ia ke kōkua iā ʻoe e hoʻomaopopo i ka hiki ke hoʻoili i ke kūlana i kāu mau keiki.

Pehea e mālama ai nā kauka i ka anemia sideroblastic?

Hoʻohana nā kauka i nā ʻano lapaʻau like ʻole no ka anemia sideroblastic hānau a me ka anemia i loaʻa. ʻOkoʻa kēia mau lapaʻau ma muli o kou kūlana, no laila e kūkākūkā kāu kauka i ka lāʻau lapaʻau maikaʻi loa nāu. No ka laʻana, hiki ke hāʻawi ʻia i kahi kanaka me ka X-linked SA (CSA) i ka huaora B6 a me nā lāʻau hoʻohaʻahaʻa hao. Inā hoʻokumu ʻia ka SA e ka lāʻau make kēpau, e mālama kāu kauka i ka lāʻau make kēpau. ʻOi aku ka maikaʻi o ke kamaʻilio ʻana me kāu kauka e pili ana i kēia.

Pehea e hiki ai iaʻu ke pale aku i kēia?

Hoʻokumu pinepine ʻia ka anemia sideroblastic e kahi hoʻololi genetic. ʻOiai ʻaʻole hiki ke pale ʻia kēia ʻano anemia sideroblastic congenital, hiki ke mālama ʻia. Inā loaʻa iā ʻoe ka X-linked `(CSA)` a he akahai kāu mau hōʻailona, ​​​​a i ʻole inā loaʻa i kekahi o kou ʻohana (nā mākua, nā kaikaina, a i ʻole nā ​​​​keiki) ka `(CSA)`, he manaʻo maikaʻi e nīnau i kāu kauka e pili ana i ka hoʻāʻo genetic.

Eia nō naʻe, aia kekahi mau mea hiki iā ʻoe ke hana e pale aku i kekahi mau ʻano CSA i loaʻa mai ka ulu ʻana ma hope. Eia nā ʻanuʻu hiki iā ʻoe ke hana:

• Ke pale nei i ka lāʻau make alakaʻi a i ʻole ka arsenic.
• Inā ʻoe e lawe ana i nā mea hoʻohui zinc no nā mea e like me ke anu maʻamau, e nīnau i kāu kauka e pili ana i ka nui kūpono.
• E hōʻemi i kāu inu ʻona. Hiki i ka ʻona ke alakaʻi i ka anemia sideroblastic. E ho'āʻo e hōʻemi i ka nui o ka waiʻona āu e inu ai i kēlā me kēia lā. Inā makemake ʻoe i ke kōkua, e noi i kāu kauka e ʻōlelo i nā papahana kūpono.

He aha kaʻu e manaʻo ai me kēia kūlana?

Aia kou wānana i ke ʻano o ka anemia sideroblastic āu e loaʻa nei. No ka laʻana, inā loaʻa iā ʻoe ka anemia sideroblastic congenital X-linked, hiki i kāu kauka ke mālama i kou kūlana me ka huaora B6 a me nā lāʻau hoʻohaʻahaʻa hao. Inā ua loaʻa iā ʻoe ka anemia sideroblastic, i hoʻokumu ʻia e ka lāʻau make kēpau, hiki i kāu kauka ke mālama i ka lāʻau make kēpau.

Pehea wau e mālama ai iaʻu iho?

Pono ka nui o ka poʻe me ka anemia sideroblastic e nānā mau ʻia ko lākou pae hao e pale aku i ka nui o ka hao. Inā ua loaʻa iā ʻoe ka anemia sideroblastic ma mua, e nīnau i kāu kauka i ka mea hiki iā ʻoe ke hana e pale aku ai i kahi SA hou.

He maʻi koko laha ʻole ka anemia sideroblastic. Ua ʻike kekahi poʻe i ke kumu o ko lākou anemia sideroblastic, akā ʻaʻole paha kekahi. Inā loaʻa iā ʻoe ka anemia sideroblastic, makemake paha ʻoe e nīnau i kāu kauka i kēia mau nīnau:

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* He aha ke ʻano o ka `(SA)` iaʻu?

* No ke aha i loaʻa ai iaʻu ka `(SA)`?

* He aha kaʻu mau koho lapaʻau?

* He aha nā hopena ʻaoʻao o ka lāʻau lapaʻau?

* He aha kaʻu e manaʻo ai ma hope o ka mālama ʻana?

* Hiki iaʻu ke hoʻoili i ka `(SA)` i kaʻu mau keiki?

No laila, he aha nā mea nui e pono ai kākou e hoʻomanaʻo mai kēia moʻolelo?

He maʻi koko laha ʻole ka anemia sideroblastic akā he maʻi koko koʻikoʻi paha. Hoʻokumu ʻia ka SA e kahi mea e hoʻopilikia ai i ka hiki o ke kino ke hana i nā hunaola koko ʻulaʻula olakino. Loaʻa pinepine i ka poʻe me SA ka nui o ka hao i loko o ko lākou koko. I ka hapanui o nā hihia, hiki ke mālama maʻalahi ʻia kēia hao nui. Eia nō naʻe, i nā pēpē a me nā kamaliʻi ʻōpio i hānau ʻia me SA, hiki i ka hao nui ke hoʻoulu i nā kūlana olakino e hoʻoweliweli i ke ola.

E noʻonoʻo, inā ua hānau ʻia kāu keiki liʻiliʻi me ka anemia sideroblastic, e hoʻomaka koke kā lākou mau kauka e mālama i ke kūlana. ʻO ka loaʻa ʻana o kahi pēpē maʻi loa a keiki liʻiliʻi paha he mea weliweli loa a hoʻoluhi. Hoʻomaopopo kāu mau kauka i kēia a e hana i nā mea āpau e hiki ai iā lākou ke kōkua iā ʻoe a me kāu keiki i ka wā a ma hope o ka mālama ʻana. No laila, inā loaʻa iā ʻoe a i ʻole kekahi o kou ʻohana kēia mau hōʻailona, ​​​​he mea nui e ʻike koke i ke kauka no ka ʻōlelo aʻo.

Hao , anemia, sideroblasts, iwi ʻiʻo, hemoglobin, genes, nā maʻi koko