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Hemochromatosis

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Pfupiso

Hemochromatosis (he-moe-kroe-muh-TOE-sis) chirwere chinoita kuti muviri utore simbi yakawanda kubva mukudya. Simba yakawandisa inounganidzwa mumuviri, kunyanya muchiropa, mwoyo nepancreas. Simba yakawanda inogona kukonzera zvirwere zvinouraya, zvakadai sedenda rechiropa, matambudziko emwoyo nedenda reshuga. Kune marudzi mashoma e hemochromatosis, asi rudzi runowanzoitika rwakakonzerwa nechinja chejini chinopfuudzwa kubva kumhuri. Vanhu vashoma chete vane majini vanowana matambudziko chaiwo. Zviratidzo zvinowanzooneka pakati pehupenyu. Kurapa kunosanganisira kubvisa ropa mumuviri nguva dzose. Nekuti simbi yakawanda iri mumuviri iri mumasero matsvuku eropa, kurapa uku kunoderedza mazinga esimbi.

Zviratidzo

Vamwe vanhu vane hemochromatosis havambofi vaine zviratidzo. Zviratidzo zvepakutanga kazhinji zvinoenderana nezvimwe zvirwere zvakajairika. Zviratidzo zvinogona kusanganisira: Kurwadziwa kwemunwe. Kurwadziwa kwemudumbu. Kushaya simba. Kushaya simba. Chidhakwa. Kurasikirwa nechidimbu. Kusakwanisa pabonde. Kukundikana kwemoyo. Kukundikana kwechiropa. Ruvara rweganda rebhuruzi kana grey. Fog yekurangarira. Rudzi rwakajairika rweheochromatosis ruripo kubva pakuberekwa. Asi vanhu vazhinji havaoni zviratidzo kusvika gare gare muhupenyu - kazhinji mushure memakore makumi mana nemana muvarume uye mushure memakore makumi matanhatu muvakadzi. Vakadzi vane mukana mukuru wekuwana zviratidzo mushure memenopause, kana vasisisiri kurasikirwa ne simbi nekumwedzi nekumimba. Ona nyanzvi yehutano kana iwe ukawana chero chezviratidzo zve hemochromatosis. Kana uine nhengo yepedyo yemhuri ine hemochromatosis, bvunza timu yako yehutano nezvekutarisa kwemajini. Kutarisa kwemajini kunogona kutarisa kana une jini rinoita kuti uve nenjodzi yakawanda ye hemochromatosis.

Nguva yekunoona chiremba

Ona chiremba wako kana uine chero zviratidzo zvehemochromatosis. Kana uri nemumwe wemhuri yako ari nehemochromatosis, bvunza chiremba wako nezvekutarisa magene. Kutarisa magene kunogona kutarisa kana uine gene rinowedzera njodzi yako yehemochromatosis.

Zvinokonzera

Hemochromatosis kazhinji inokonzerwa nechinja muchiitiko. Ichi chiitiko chinoongorora huwandu hwepfuma muviri unotora kubva mukudya. Chiitiko chakachinja chinopfuudzwa kubva kuvabereki kuenda kuvana. Rudzi urwu rweheochromatosis ndirwo rudzi runowanzoitika. Inonzi hereditary hemochromatosis. Chiitiko chinonzi HFE kazhinji ndicho chinokonzera hereditary hemochromatosis. Unogara uchigara neHFE gene kubva kune mumwe nemumwe wevabereki vako. HFE gene ine shanduko mbiri dzakajairika, C282Y neH63D. Kuongororwa kwemajini kunogona kuratidza kana uine shanduko idzi muchiitiko chako cheHFE. Kana wakagara nemajini maviri akachinja, unogona kukura hemochromatosis. Unogona zvakare kupfuudza gene yakachinja kuvana vako. Asi kwete munhu wese anogara nemajini maviri anokura matambudziko ane chekuita nekuwanda kwepfuma kweheochromatosis. Kana wakagara negene imwe yakachinja, hauna mukana wekukura hemochromatosis. Zvisinei, unofungwa semutakuri uye unogona kupfuudza gene yakachinja kuvana vako. Asi vana vako havazokuri chirwere kunze kwekunge vakagarawo negene imwe yakachinja kubva kune mumwe mubereki. Iron ine basa rinokosha mumabasa akawanda emuviri, kusanganisira kubatsira kugadzira ropa. Asi iron yakawanda ine chepfu. Hormone inobudiswa nechiropa, inonzi hepcidin, inodzora kuti iron inoshandiswa sei uye inotorwa sei mumuviri. Inodzorawo kuti iron yakawandisa inorambwa sei mune zvikamu zvakasiyana-siyana. Muheochromatosis, basa rehepchidin rinoonekwa, zvichikonzera muviri kutora iron yakawanda kupfuura yaunoda. Iyi iron yakawandisa inorambwa muzvikamu zvikuru, kunyanya chiropa. Kwemakore mazhinji, iron yakarambwa inogona kukonzera kukuvara kwakakomba kunogona kutungamira pakukundikana kwezvikamu. Inogona zvakare kutungamira kune zvirwere zvinogara kwenguva refu, senge cirrhosis, chirwere cheshuga uye kukundikana kwemoyo. Vanhu vazhinji vane shanduko dzemajini dzinoita hemochromatosis. Zvisinei, kwete munhu wese anokura iron yakawanda kusvika padanho rinoita kukuvara kumatishu nekuzvikamu. Hereditary hemochromatosis haisi rudzi rweheochromatosis chete. Mamwe marudzi anosanganisira: Juvenile hemochromatosis. Izvi zvinoita matambudziko akafanana muvechidiki ayo hereditary hemochromatosis inoita muvakuru. Asi kuunganidzwa kweiron kunotanga kare, uye zviratidzo kazhinji zvinooneka pakati pemakore 15 ne30. Iyi mamiriro inokonzerwa nechinja muchiitiko chehemojuvelin kana hepcidin. Neonatal hemochromatosis. Muchirwere ichi chakakomba, iron inoungana nekukurumidza muchiropa chemwana ari kukura mudumbu. Inofungwa kuva chirwere che autoimmune, umo muviri unozvirwisa. Secondary hemochromatosis. Iyi fomu yechirwere haina kuwanikwa uye kazhinji inonzi iron overload. Vanhu vane marudzi akati wandei eanemia kana chirwere chechiropa vanogona kazhinji kuda kuiswa ropa kakawanda. Izvi zvinogona kutungamira pakukura kweiron yakawanda.

Zvinhu zvinokonzera njodzi

Zvinhu zvinowedzera njodzi yehemochromatosis zvinosanganisira: Kuva nemaviri emhando dzakashandurwa dzejini reHFE. Iyi ndiyo njodzi huru yehemochromatosis inogarwa nhaka. Nhoroondo yemhuri. Kuva nemubereki kana hama ine hemochromatosis kunowedzera mukana wekuita chirwere ichi. Dzinza. Vanhu vanobva kuNorthern Europe vane mukana wakanyanya wekuita hemochromatosis inogarwa nhaka kupfuura vanhu vemamwe madzinza. Hemochromatosis haiwanzo kuwanikwa muvanhu vatema, veSpanish neveAsia. Zvepabonde. Varume vane mukana wakanyanya wekuita zviratidzo zvehemochromatosis pazera diki kupfuura vakadzi. Nekuti vakadzi vanorasikirwa nesimbi kuburikidza nekuenda kumwedzi uye nhumbu, vanowanzochengeta zvishoma zvemaminerari kupfuura varume. Mushure mekuenda kumwedzi kana kuvhiyiwa, njodzi inowedzera kuvakadzi.

Matambudziko
  • **Matambudziko ehuro.
Kuongorora

Hemochromatosis inogona kunge yakaoma kuziva. Zviratidzo zvepakutanga senge majoini akaoma uye kuneta zvinogona kunge zvichikonzerwa nemamwe mamiriro kunze kweheochromatosis.

Vanhu vazhinji vane chirwere havana zviratidzo kunze kwemazinga epamusoro e simbi muropa ravo. Hemochromatosis inogona kuzivikanwa nekuda kwezvibvumikiso zvisina kururama zve ropa mushure mekuyedzwa kwakaitwa nezvimwe zvikonzero. Inogonawo kuonekwa pakutarisa nhengo dzemhuri dzevanhu vakazivikanwa vane chirwere.

Kuongororwa kuri maviri kunokosha kwekutsvaga kuwanda kwesimbi ndeaya:

  • Kuwedzera kwe serum transferrin. Kuongororwa uku kunoyera huwandu hwesimbi yakabatana neprotein transferrin inotakura simbi muropa rako. Kukosha kwe transferrin saturation kunopfuura 45% kunofungwa kunge kwakanyanya.

Aya bvunzo dzeropa resitsi zviri nani kuita mushure mekunge watsanya. Kuwedzera mumwe kana vose bvunzo idzi kunogona kuwanikwa mune dzimwe chirwere. Ungada kuita bvunzo idzi zvakare kuti uwane mhinduro dzakanyatsojeka.

Chiremba wako anogona kukurudzira mamwe bvunzo ekutsigira chirwere uye kutsvaga mamwe matambudziko:

  • Bvunzo dzebasa rechiropa. Aya bvunzo anogona kubatsira kuziva kukuvara kwechiropa.
  • MRI. MRI inzira inokurumidza uye isina kuipisisa yekuyera huwandu hwesimbi yakawanda muchiropa chako.
  • Kuongororwa kwekuchinja kwemajini. Kuongorora DNA yako kuti uone kuchinja mu HFE gene kunokurudzirwa kana uine mazinga epamusoro esimbi muropa rako. Kana uri kufunga nezvekuongororwa kwemajini kweheochromatosis, kurukurai zvikonzero zvekuita uye kusaita nechiroko chako kana mushandi wemajini.
  • Kubvisa sampuli yetsinga yechiropa kuti iongororwe. Kana chiremba wako achifungidzira kukuvara kwechiropa, chiremba anogona kuraira biopsy yechiropa. Panguva yebiopsy yechiropa, sampuli yetsinga inobviswa muchiropa chako uchishandisa tsono yakasimba. Sampuli inoenda ku laboratori kuti iongororwe kuvapo kwesimbi. Laboratori inotarisawo humbowo hwe kukuvara kwechiropa, kunyanya kuita mavara kana cirrhosis. Njodzi dze biopsy dzinosanganisira kuzvimba, kubuda ropa uye hutachiona.

Kuongororwa kwemajini kunokurudzirwa kune vabereki vose, hama nevana vemunhu wese akazivikanwa ane hemochromatosis. Kana kuchinja kwe gene kuchiwonekwa mubaba mumwe chete, ipapo vana havafaniri kuongororwa.

Kurapa

Vachipatara vezvehutano vanogona kurapa hemochromatosis zvakachengeteka uye zvakanaka nekubvisa ropa mumuviri nguva dzose. Izvi zvakafanana nekupa ropa. Maitiro acho anozivikanwa se phlebotomy.

Chinangwa che phlebotomy ndechekudzikisa mazinga ako e iron. Huwandu hweropa hunobviswa uye kuti huno bviswa kakawanda sei zvinoenderana nezera rako, hutano hwako hwese uye kukomba kwe iron overload.

  • Chirongwa chekurapa chekutanga. Pakutanga, ungatora pini (anenge mamiririta mazana mana nemakumi manomwe) eropa kamwe kana kaviri pavhiki - kazhinji muchipatara kana muhofisi yemupi wako wehutano. Paunenge wakatarisa kumashure muchigaro, tsono inoiswa mumutsinga weruoko rwako. Ropa rinoyerera kubva kutsono kuenda kumuchina wakabatanidzwa kubhegi reropa. Maitiro ekubvisa ropa anozivikanwa se therapeutic blood removal.
  • Chirongwa chekurapa chekuchengetedza. Kana mazinga ako e iron adzika, ropa rinogona kubviswa zvishoma, kazhinji mwedzi miviri kusvika mitatu. Vamwe vanhu vanogona kuchengetedza mazinga e iron asingabviswe ropa. Vamwe vanogona kuda kubviswa ropa pamwedzi. Chirongwa chinoenderana nekuti iron inokura sei mumuviri mako.

Kurapa hemochromatosis kunogona kubatsira kudzikamisa zviratidzo zvekuneta, kurwadziwa kwemudumbu uye kusimba kweganda. Inogona kubatsira kudzivirira matambudziko akakomba akadai sedenda rechiropa, chirwere chemwoyo uye chirwere cheshuga. Kana iwe uchitova nechimwe chezvipatara izvi, phlebotomy inogona kunonoka kufambira mberi kwechirwere. Muzviitiko zvimwe, zvinogona kutochinja.

Phlebotomy haigone kuchinja cirrhosis kana kurwadziwa kwemukoko, asi inogona kunonoka kufambira mberi.

Kana uine cirrhosis, mupi wako wehutano anogona kukurudzira kuongororwa kwekenza yechiropa. Izvi kazhinji zvinosanganisira ultrasound ye abdominal uye CT scan.

Phlebotomy ingave isiri sarudzo kana uine zvimwe zvirwere, senge anemia kana matambudziko emwoyo. Panzvimbo iyoyo, mupi wako wehutano anogona kukurudzira mushonga wekubvisa iron yakawandisa. Mushonga unogona kuiswa mumuviri mako, kana unogona kunwa se piritsi. Mushonga unobatanidza iron yakawandisa, uchibvumira muviri wako kubudisa iron kuburikidza ne weewee yako kana tsvina muitiro unonzi chelation (KEE-lay-shun). Chelation haishandiswe kazhinji mu hemochromatosis.

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