Hemokromatosis

Ko te Hemochromatosis (he-moe-kroe-muh-TOE-sis) he āhuatanga e tino nui ai te tango i te rino i te kai e te tinana. Ka tāpirihia te rino nui ki roto i ngā whekau, ā, ko te ate, te ngākau me te pancreas ngā mea nui. Ka taea e te nui o te rino te arahi ki ngā āhuatanga kino e whakawehi ana i te ora, pēnei i te mate ate, ngā raruraru ngākau me te mate huka. E torutoru ngā momo hemochromatosis, engari ko te momo tino noa ko te mea i puta mai i te huringa o te ira e tukuna ana i roto i ngā whānau. He torutoru noa ngā tāngata e whai ira ana e whāiti ana ngā raruraru nui. Ka puta ngā tohu i waenganui o te ora. Ko te maimoatanga ko te tango tonu i te toto i te tinana. Nō te mea kei roto i ngā pūtau toto whero te nuinga o te rino o te tinana, ka whakaiti tēnei maimoatanga i ngā taumata rino.

Ko ētahi o te hunga e pangia ana e te hemochromatosis kaore rawa e whai tohu. Ko ngā tohu o te tīmatanga ka maha ngā wā e rite ana ki ngā tohu o ētahi atu āhuatanga noa. Āhei ngā tohu: Tōngā maripi. Tōngā puku. Ngoikore. Whakawero. Te huka. Ngaro o te hiahia mo te moe wahine/tāne. Te kore e taea te moe wahine/tāne. Te korenga o te ngakau. Te korenga o te ate. Te tae kiri parahi, pango rānei. Te ngaro o te mahara. Ko te momo hemochromatosis tino noa e puta mai ana i te whānau. Engari ko te nuinga o te iwi kaore e pāngia e ngā tohu tae noa ki te wā muri o te ora—he maha tonu i muri i te 40 tau mō ngā tāne, ā, i muri i te 60 tau mō ngā wāhine. He nui ake te tūponotanga o ngā wāhine ki te whanake i ngā tohu i muri i te menopause, ina kaore rātou e ngaro te rino i te wā o te pānga me te hapu. Tirohia tētahi kaimahi hauora mena ka pāngia koe e tētahi o ngā tohu o te hemochromatosis. Mena kei a koe tētahi mema whānau tata e pangia ana e te hemochromatosis, ui atu ki tō rōpū hauora mō te whakamātautau ira. Ka taea e te whakamātautau ira te tirotiro mena kei a koe te ira e whakapiki ake ana i tō tūponotanga ki te hemochromatosis.

Tena tirohia tō kaiwhakarato hauora mena ka pā atu koe ki tetahi o ngā tohu o te hemochromatosis. Ki te pēhea tētahi mema o tō whānau tata e hemochromatosis ana, ui atu ki tō kaiwhakarato mō te whakamātautau ira. Ka taea e te whakamātautau ira te tirotiro mena kei a koe te ira e whakapiki ake ana i tō tūponotanga ki te hemochromatosis.

Ko te Hemochromatosis te nuinga o te wa e puta mai ana i te huringa i roto i te ira. Ko tenei ira te mana o te nui o te rino e tangohia ana e te tinana mai i te kai. Ka tukuna te ira i whakarereketia mai i nga matua ki nga tamariki. Ko tenei momo hemochromatosis te momo tino noa. Ka kiia ko te hemochromatosis tūturu. Ko te ira e kiia nei ko HFE te nuinga o te take o te hemochromatosis tūturu. Ka whiwhi koe i tetahi ira HFE mai i ia o ou matua. E rua nga rereketanga noa o te ira HFE, ko C282Y me H63D. Ka taea e te whakamātautau ira te whakakite mena kei a koe enei huringa i roto i to ira HFE. Ki te whiwhi koe i nga ira e rua kua whakarereketia, ka taea e koe te whanake i te hemochromatosis. Ka taea hoki e koe te tuku i te ira kua whakarereketia ki ou tamariki. Engari ehara i te katoa o te hunga e whiwhi ana i nga ira e rua e whanake ana i nga raruraru e hono ana ki te nui o te rino o te hemochromatosis. Ki te whiwhi koe i tetahi ira kua whakarereketia, ka iti te tupono ka whanake koe i te hemochromatosis. Heoi, ka kiia koe he kaimau ka taea te tuku i te ira kua whakarereketia ki ou tamariki. Engari e kore o tamariki e whanake i te mate ki te kore e whiwhi ano ratou i tetahi atu ira kua whakarereketia mai i tetahi atu matua. He mahi nui te rino i roto i etahi mahi o te tinana, tae atu ki te awhina i te whakaputa toto. Engari he kino te nui o te rino. Ko te homoni e tukuna ana e te ate, e kiia nei ko te hepcidin, te mana o te whakamahinga me te tango o te rino i roto i te tinana. Ka whakahaeretia ano e ia te nui o te rino e penapena ana i roto i nga okana maha. I roto i te hemochromatosis, ka pa te mahi a te hepcidin, ka nui ake te tango rino e te tinana i te mea e hiahiatia ana. Ka penapena tenei rino nui i roto i nga okana nui, ina koa te ate. I roto i nga tau, ka taea e te rino e penapena ana te kino nui ka arahi pea ki te korenga o nga okana. Ka taea hoki te arahi ki nga mate roa, penei i te cirrhosis, te mate huka me te korenga o te ngakau. He tokomaha nga tangata kei a ratou nga huringa ira e puea ai te hemochromatosis. Heoi, ehara i te katoa e whanake ana i te nui o te rino ki te taumata e puea ai te kino o nga kiko me nga okana. Ehara i te hemochromatosis tūturu anake te momo hemochromatosis. Ko etahi atu momo ko: Te hemochromatosis taitamariki. Ko tenei te take o nga raruraru ano i roto i nga taitamariki e puea ai e te hemochromatosis tūturu i roto i nga pakeke. Engari ka timata te hanga rino i mua atu, a ka puta mai nga tohu i waenga i nga tau 15 me te 30. Ko tenei ahuatanga e puta mai ana i nga huringa i roto i nga ira hemojuvelin, hepcidin ranei. Te hemochromatosis neonatal. I roto i tenei mate kino, ka tere te hanga rino i roto i te ate o te pepeha e whanake ana i roto i te kopu. E whakaarohia ana he mate autoimmune, i reira ka whakaeke te tinana i a ia ano. Te hemochromatosis tuarua. Kaore tenei ahua o te mate e tukuna ana, a ka kiia nei ko te nui o te rino. Ko nga tangata kei a ratou etahi momo mate toto iti, mate ate ranei, ka maha pea te hiahia ki nga whakawhitinga toto. Ka taea e tenei te arahi ki te nui o te hanga rino.

'Factors that increase the risk of hemochromatosis include:': 'Ko ētahi o ngā take e piki ai te tūponotanga o te hemochromatosis ko: ', 'Having two copies of an altered HFE gene. This is the greatest risk factor for hereditary hemochromatosis.': 'Te whai i ngā kape e rua o te hēne HFE kua whakarerekē. Koinei te take tino nui o te tūponotanga o te hemochromatosis tūturu.', 'Family history. Having a parent or sibling with hemochromatosis increases the likelihood of developing the disease.': 'Te hītori whānau. Mēnā he mātua, he teina rānei kei a rātou te hemochromatosis, ka piki ake te tūponotanga o te mate.', 'Ethnicity. People of Northern European descent are more prone to hereditary hemochromatosis than are people of other ethnic backgrounds. Hemochromatosis is less common in people of Black, Hispanic and Asian ancestry.': 'Iwi. Ko te hunga o te whenua o te Raki o Ūropi te tino tūponotanga o te hemochromatosis tūturu atu i ētahi atu iwi. Ka iti ake te hemochromatosis i roto i te hunga pango, Hīpānika me Āhia.', 'Sex. Men are more likely than women to develop symptoms of hemochromatosis at an earlier age. Because women lose iron through menstruation and pregnancy, they tend to store less of the mineral than men do. After menopause or a hysterectomy, the risk increases for women.': 'Ira tangata. Ko ngā tāne te tino tūponotanga ki te whanake i ngā tohu o te hemochromatosis i te wā taitamariki. Nō te mea ka ngaro te rino i ngā wāhine mā te uterine me te hapu, ka iti ake te rokiroki o te kohuke i ngā tāne. I muri i te menopause, te hysterectomy rānei, ka piki ake te tūponotanga mō ngā wāhine.'

• Ngā raru o te ate. Ko te cirrhosis—arā, te whākākino ā-mau o te ate—he kotahi noa te take pea e puta mai ai. Ka piki ake te tūponotanga o te mate pukupuku ate me ētahi atu raruraru kino e whakawehi ana i te ora.
• Te mate huka. Ka taea e te pakaru o te pancreas te arahi ki te mate huka.
• Ngā raru o te ngakau. Ko te nui o te rino kei roto i tō ngakau ka pā ki te kaha o te ngakau ki te whakaheke i te toto hei whakatutuki i ngā hiahia o tō tinana. Ka kiia tēnei ko te ngoikore o te ngakau. Ka taea hoki e te hemochromatosis te whakaeke i ngā āwangawanga o te ngakau, e kiia nei ko ngā arrhythmias.
• Ngā raru o te whakapaparinga. Ka taea e te nui o te rino te arahi ki te ngoikore o te taangata me te ngaro o te hiahia taangata i ngā tāne. Ka taea e ia te whakaeke i te korenga o te huringa marama i ngā wāhine.
• Ngā huringa o te tae kiri. Ka taea e ngā whakato rino i roto i ngā pūtau kiri te whakaatu i tō kiri he parakore, he hina rānei te tae.

Kaore he uauatanga

Ka uaua pea te whakatau i te Hemochromatosis. Ko ngā tohu o mua, pēnei i ngā hononga kōtiti me te ngenge, ka puta pea i ngā āhuatanga kē atu i te hemochromatosis.

He tokomaha ngā tāngata e pangia ana e te mate, kāore hoki he tohu atu i te nui o te rino i roto i o rātou toto. Ka taea pea te kite i te Hemochromatosis nā te koretake o ngā hua whakamātau toto i muri i te whakamātau i mahia mō ētahi take kē. Ka kitea hoki pea i te whakamātautau i ngā whānau o ngā tāngata kua whakatauhia te mate.

Ko ngā whakamātautau matua e rua hei kimi i te nui o te rino ko:

• Te whakamātautau saturation transferrin serum. Ka ine tenei whakamātautau i te nui o te rino e hono ana ki te pūmua transferrin e kawe ana i te rino i roto i ō toto. Ko ngā uara saturation transferrin neke atu i te 45% ka whakaarohia he nui rawa.

He pai te mahi i ēnei whakamātautau toto mō te rino i muri i tō nohopuku. Ka kitea ngā pikinga i tētahi, i ngā whakamātautau katoa rānei i roto i ētahi atu mate. Me whakahoki pea ngā whakamātautau kia tika ai ngā hua.

Ka taea e tō kaiwhakarato hauora te kōrero atu i ētahi atu whakamātautau hei whakau i te whakatau, ā, hei kimi hoki i ētahi atu raruraru:

• Ngā whakamātautau mahi ate. Ka āwhina ēnei whakamātautau ki te kimi i te kino o te ate.
• MRI. He huarahi tere, ā, kore-whakawehi te MRI hei ine i te nui o te rino i roto i tō ate.
• Te whakamātautau mō ngā huringa ira. E tūtohutia ana te whakamātautau i tō DNA mō ngā huringa i roto i te ira HFE mēnā he nui te rino i roto i ō toto. Mēnā kei te whakaaro koe ki te whakamātautau ira mō te hemochromatosis, kōrerohia ngā take mō, me ngā take e kore ai e pai ki tō kaiwhakarato, ki tētahi kaitohutohu ira rānei.
• Te tango i te tauira o te kiko ate hei whakamātautau. Mēnā kei te whakaaro tō kaiwhakarato he kino tō ate, ka taea e te kaiwhakarato te tono i te biopsy ate. I te wā o te biopsy ate, ka tangohia he tauira o te kiko mai i tō ate mā te ngira angiangi. Ka haere te tauira ki tētahi rūma hei tirotiro i te rino. Ka titiro hoki te rūma ki ngā tohu o te kino o te ate, ina koa te tohe, te cirrhosis rānei. Ko ngā tūponotanga o te biopsy ko te whē, te toto, me te mate.

E tūtohutia ana te whakamātautau ira mō ngā mātua katoa, ngā teina, me ngā tamariki o te tangata kua whakatauhia te hemochromatosis. Mēnā ka kitea he huringa ira i tētahi mātua anake, kāore e hiahiatia te whakamātautau i ngā tamariki.

Ka taea e ngā kaiwhakarato hauora te whakaora i te hemochromatosis i te huarahi haumaru me te whai hua mā te tango i te toto i roto i te tinana i ngā wā maha. He rite tēnei ki te tuku toto. Ko te tikanga nei e kiia ana ko te phlebotomy.

Ko te whāinga o te phlebotomy ko te whakaiti i ō taumata rino. Ko te nui o te toto e tangohia ana, me te auau o te tango, ka whakawhirinaki ki tō tau, ki tō hauora whānui, me te kaha o te nui o te rino.

• Kaupapa whakaora tuatahi. I te tīmatanga, ka tangohia pea tētahi paini (tau 470 milliliters) o te toto kotahi, rua rānei i te wiki – i te whare hauora, i te tari rānei o tō kaiwhakarato. I a koe e tūroro ana ki te nohoanga, ka werohia he ngira ki roto i tētahi uaua i tō ringa. Ka rere te toto i te ngira ki roto i tētahi pūnaha e hono ana ki tētahi putea toto. Ko te tikanga o te tango toto e kiia ana ko te tango toto whakaora.
• Kaupapa whakaora tiaki. Kia heke iho ō taumata rino, ka taea te tango toto i ngā wā iti ake, i ia marama e rua, e toru rānei. Ka taea e ētahi te pupuri i ngā taumata rino noa kaore he toto e tangohia. Ka hiahia pea ētahi ki te tango toto i ia marama. Ko te kaupapa ka whakawhirinaki ki te tere o te hanga o te rino ki roto i tō tinana.

Ka taea e te whakaora i te hemochromatosis te awhina i te whakaora i ngā tohu o te ngenge, te mamae o te kopu, me te pōuri o te kiri. Ka taea te āwhina i te aukati i ngā raruraru kino penei i te mate ate, te mate ngakau, me te mate huka. Ki te pēhea kē koe i tētahi o ēnei āhuatanga, ka taea e te phlebotomy te whakaheke i te tere o te mate. I ētahi wā, ka taea e ia te whakahoki mai.

Kāore e taea e te Phlebotomy te whakahoki mai i te cirrhosis, i te mamae o ngā hononga rānei, engari ka taea te whakaheke i te tere o te whanaketanga.

Ki te pēhea koe i te cirrhosis, ka tūtohu pea tō kaiwhakarato hauora ki te tirotiro i ngā wā maha mō te mate kōpū ate. Ko te tikanga tenei he ultrasound o te kopu me te CT scan.

Pea kāore te phlebotomy e wātea ki a koe ki te pēhea koe i ētahi āhuatanga, penei i te mate toto iti, i ngā raruraru ngakau rānei. Engari, ka tūtohu pea tō kaiwhakarato i te rongoā hei tango i te rino nui. Ka taea te wero i te rongoā ki roto i tō tinana, ka taea rānei te tango hei rongoā. Ka herea e te rongoā te rino nui, ka taea e tō tinana te peia atu te rino mā tō mimi, mā tō paru rānei i roto i tētahi tikanga e kiia ana ko te chelation (KEE-lay-shun). Kāore te chelation e whakamahia whānuitia i te hemochromatosis.

Kaore he uauatanga