Kodi hemochromatosis ndi chiyani?

Zopweteka
Kutopa
Kusunthika kochepa
Kutupa kwa miyendo
Khungu lowonekera
Kuthamanga kwa magazi
Kuchepetsa thupi
Matenda a shuga
Kulephera kwa mtima
Zofooka
Anatsika libido
Kukula kwa chiwindi

Hemochromatosis ndi matenda omwe amatengera ndipo amabweretsa kuphwanya kwa kagayidwe kazitsulo m'thupi la munthu. Ndi matenda awa, ma pigment omwe amakhala ndi chitsulo amalowetsedwa m'matumbo ndipo amadziunjikira minofu ndi ziwalo.

Hemochromatosis inali ponseponse kumpoto kwa Europe - 5% ya anthu ali ndi nthenda ya homozygous kumeneko. Nthawi zambiri, amuna amadwala hemochromatosis (ziwerengero zimawonetsa kuchuluka kwa amuna odwala kwa amayi odwala monga 10: 1). Monga lamulo, zizindikiro zoyambirira za matendawa zimawonekera ali ndi zaka zapakati (kuyambira zaka 40 mpaka zaka zapenshoni). Nthawi zambiri, ndi hemochromatosis, chiwindi chimakhudzidwa, chifukwa zimatenga gawo mu kagayidwe kazitsulo.

Zizindikiro za matendawa

Hemochromatosis ali ndi izi:

kukhalapo kwa kufooka ndi kutopa kosalekeza,
kutsitsa magazi
kuwonda mwadzidzidzi
kuchuluka kwa pigment. Amapereka kusintha kwa khungu pakhungu loyipa, komanso kusintha kwa mtundu wa sclera kapena mucous membranes,
kukulitsa kwa matenda a shuga (matenda ophatikizira kuchuluka kwa shuga),
mawonekedwe a kulephera kwa mtima. Kuphatikizika kwamatenda awa kumaphatikiza ma pathologies onse omwe amakhudzidwa ndi kuthekera kwa minofu yamtima kuti agwirizane,
maonekedwe a cirrhosis a chiwindi (zimapereka kusintha kwa minyewa ya chiwindi mothandizidwa ndi ma cicatricial),
Kulephera kwa chiwindi (kulephera kuthana ndi ntchito pakugaya),
m'munsi libido
kukhalapo kwa edema komanso kusuntha pang'ono kwa miyendo.

Mitundu ndi magawo a matendawa

Mitundu yotsatayi yamatenda imasiyanitsidwa:

choyambirira. Zokhudzana ndi masinthidwe amtundu omwe amayambitsa kusinthana kwachitsulo mthupi,
neonatal. Zikuwoneka chifukwa chachitsulo chachikulu mwa akhanda. Zomwe zimayambitsa matendawa ndi madokotala sizinafotokozedwebe,
sekondale. Sekondale hemochromatosis, monga lamulo, limayamba motsutsana ndi kumbuyo kwa matenda ena omwe amaphatikizidwa ndi kufalikira kwa magazi, mavuto a khungu. Zimayambanso chifukwa chomwa mankhwala osokoneza bongo omwe amakhala ndi chitsulo chachikulu.

Hemochromatosis ili ndi magawo otsatirawa:

pa siteji yoyamba, pali zosokoneza mu kagayidwe kazitsulo, koma kuchuluka kwake kumatsalira povomerezeka,
pa siteji yachiwiri, wodwalayo amakhala ndi chitsulo chambiri, chomwe sichikhala ndi zizindikiro zapadera za kuchipatala, komabe, kuwunika kukuwonetsa kupatuka kuzizwitsa,
pa gawo 3, wodwalayo amakhala ndi zisonyezo zonse za matendawa chifukwa cha kuchuluka kwa chitsulo.

Zomwe zimayambitsa matendawa

Zomwe zimayambitsa chitukuko cha matendawa ndi monga:

cholowa m'malo mwake. Nthawi zambiri izi ndizomwe zimayambitsa kukula kwa mtundu woyambira wa matenda ndipo amawoneka chifukwa cha kuwonongeka kwa jini, komwe kumayambitsa kusinthana kwachitsulo,
kagayidwe kachakudya matenda. Nthawi zambiri zimawonetsedwa motsutsana ndi matenda amchiwindi chifukwa chotupa mkati mwake kuti magazi azithamanga.
matenda a chiwindi okhala ndi etiology. Izi zimaphatikizapo hepatitis B ndi C, zomwe zimawonedwa mwa wodwala koposa miyezi isanu ndi umodzi,
steatohepatitis (kukhathamiritsa kwa minyewa ya chiwindi ndi mafuta),
kuvula kwachikondwerero,
kupezeka kwa chotupa, mwachitsanzo, khansa kapena chotupa.

Kuzindikira matenda

Kuzindikira matenda monga sekondale hemochromatosis amachitika motengera:

kusanthula kwa mbiri yachipatala ndi madandaulo a odwala. Dokotalayo amaganizira nthawi yomwe matendawa akhazikika komanso zomwe wodwala angagwirizane ndi zomwe zimachitika,
kusanthula kwa mbiri yabanja. Zimatengera kuti matendawa adawonedwa pakati pa abale a wodwala,
zotsatira za mayeso amtundu. Zimathandizira kudziwa mtundu wopanda vuto,
kusanthula kwazitsulo posinthana ndi chitsulo m'magazi. Zimaphatikizapo mayeso angapo kuti muwone kukhalapo kwa chitsulo chochuluka,
zambiri zopezeka ndi biopsy (kusanthula, kuphatikizapo kuphatikiza minofu yaying'ono ya chiwindi ndi singano yopyapyala). Kuzindikira koteroko kumawonetsa ngati kuwonongeka kwa minofu ya ziwalo kulipo.

Nthawi zina njira yozindikira ndi kufunsa wodwala ndi endocrinologist.

Kuchiza matenda

Chithandizo cha hemochromatosis ndichovuta ndipo amafuna kuti wodwalayo atenge njira zotsatirazi:

cholinga chakudya. Iyenera kuganizira kuchepetsedwa kwa zinthu zomwe zimakhala ndi chitsulo, komanso mapuloteni. Ndikofunikira kuti muchepetse zipatso ndi zinthu zina zomwe zili ndi mavitamini C ambiri, chifukwa mawonekedwe ake apamwamba amatsogolera kuyamwa kwa chitsulo. Zakudyazo zimapatsa kukana mowa, chifukwa zimathandizanso kuyamwa kwa khungu mu minyewa ya chiwindi ndikuwakhudza iwo. Wodwala ayenera kusiya kugwiritsa ntchito mkate wambiri kuchokera ku buckwheat, ufa wa rye, komanso zinthu zina za ufa. Simuyenera kudya impso ndi chiwindi, komanso musasankhe zakudya zam'madzi muzakudya (squid, shrimp, seaweed). Mutha kumwa tiyi wakuda, komanso khofi, chifukwa amachepetsa kuchuluka kwa kusinthana kwachitsulo chifukwa cha zomwe zili ma tannins,
kumwa mankhwalachomangira chitsulo. Amathandizira kuchotsa chitsulo champhamvu pazida za wodwalayo,
phlebotomy. Kukhetsa magazi kumaphatikizapo kuchotsa mthupi mpaka 400 ml ya magazi, omwe amakhala ndi chitsulo chachikulu, sabata lililonse. Amachepetsa zizindikiro (amachotsa pigmentation, amachepetsa kukula kwa chiwindi)
Chithandizo cha matenda okhudzana (matenda ashuga, zotupa, kulephera kwa mtima) komanso kuwunika kwawo kwakanthawi.

Mavuto omwe angakhalepo

Hemochromatosis imatha kuphatikizira zovuta za thupi:

maonekedwe a chiwindi kulephera. Nthawi yomweyo, thupi limaleka kuthana ndi ntchito zake (kutenga nawo gawo pakudya chakudya, kagayidwe kachakudya ndi kusaloledwa kwa zinthu zovulaza),
kuwoneka kwa arrhythmias ndi zolakwika zina pakugwira ntchito kwa minofu ya mtima,
myocardial infaration. Matendawa amapezeka chifukwa cha kusokonezeka kwakukulu kwa magazi ndipo kumafanana ndi kufa kwa gawo la mtima. Nthawi zambiri zimawoneka motsutsana ndi maziko osokonekera pamtima,
magazi ochokera m'mitsempha yomwe ili m'mero.
chikomokere (chiwindi kapena matenda ashuga). Vutoli limachitika chifukwa cha kuwonongeka kwa ubongo ndi zinthu zapoizoni zomwe zimadziunjika m'thupi chifukwa chakulephera kwa chiwindi.
mawonekedwe a zotupa za hepatic.

Kuti musatulutse zovuta zonsezi, ndikofunikira kuzindikira matendawa nthawi kuti dokotala athe kupereka chithandizo choyenera.

Chithandizo cha hemochromatosis chikuyenera kukhala chanthawi yake kupewa zoteteza ku ziwalo za wodwala. Kuti muchite izi, zizindikiro zoyambirira zikaonekera, ayenera kufunsa dokotala. Ponena za m'tsogolo monsemu matendawa atayamba, pakubwera kwa chithandizo kwakanthawi kwa zaka 10, oposa 80% odwala amakhalabe ndi moyo. Ngati mawonetseredwe a matendawo wodwala adayamba zaka 20 zapitazo, ndiye kuti kupulumuka kwake kumachepetsedwa mpaka 60-70%. Zoneneratu za madotolo pazotsatira zabwino zimadalira kuchuluka kwa mitundu yayikulu yazitsulo m'thupi la wodwalayo. Kwambiri, mwayi wocheperako. Ngati matendawa adapezeka musanayambike matenda a cirrhosis, ndiye kuti wodwalayo ali ndi mwayi wokhala ndi moyo wabwinobwino. Ndikofunika kudziwa kuti pafupifupi 30% ya odwala amafa chifukwa cha zovuta za matendawa, zomwe zimaphatikizapo kulephera kwa mtima kapena malabsorption syndrome.

Kupewa matenda

Hemochromatosis ndi matenda oopsa omwe amakhudza minofu yambiri ndi ziwalo. Kupewa kuyenera kutsatira kutsatira malamulo angapo nthawi imodzi. Choyamba, zimapereka chakudya (kuchepetsa kutsika kwa zakudya zomanga thupi, komanso ascorbic acid ndi zinthu zomwe zimakhala ndi chitsulo). Kachiwiri, kupewa kumaganizira kugwiritsa ntchito mankhwala apadera omwe amamanga chitsulo mthupi ndikuchichotsa mwachangu, moyang'aniridwa ndi dokotala. Chachitatu, ngakhale pakalibe zizindikiro zoonekeratu, kupewa ndi kugwiritsa ntchito mankhwala okhala ndi chitsulo, omwe adotolo amauza wodwala.

Zimayambitsa hemochromatosis

Chachikulu hemochromatosis ndi matenda obadwa nawo omwe amapanga mtundu wa masinthidwe a HFE. Matenda amtunduwu amapanga cholakwika cholanda chitsulo, pambuyo pake chizindikiro chabodza chimapangidwa ponena za kusowa kwa chitsulo. Mapeto ake, mumakhala mawonekedwe amtundu wambiri womwe umalowa ndi ziwalo zambiri zamkati, zomwe zimayambitsa kachitidwe ka sclerotic.

Sekondale hemochromatosis imapangidwa ndi kudya kwambiri chitsulo mthupi. Izi zitha kuwoneka. ndimakonda kupakidwa magazi pafupipafupi, kudya kosayenera kwa kukonzekera kwazitsulo, mitundu ina ya kuchepa magazi, kuledzera, hepatitis B kapena C, khansa, kutsatira zakudya zochepa za mapuloteni komanso matenda ena.

Zizindikiro

Choyambirira chomwe chimaphatikizapo kuzindikira kwa hemochromatosis ndi mbiri ya banja, kuphunzira za kubadwa kwa wodwala.

Gawo lotsatira lidzakhala zasayansi yoyeserera magazi. Kudziwa mulingo wachitsulo mu seramu, transerrin ndi ferritin ndikofunikira kuyesedwa kwa magazi kuti mupeze matenda otere.

Pakufufuza kwamkodzo wa mkodzo, chizindikiro chodziwikiratu cha hemochromatosis ndichinsinsi chambiri (kuyambira mamiligalamu 10 patsiku).

Kubooletsa sternum komanso kukopeka kwa khungu lanu ndizotheka. Kuboola wowuma kumawonetsa hemochromatosis okhala ndi milingo yayitali kwambiri. Ndipo pakhungu la khungu, kuwunika kwake kudzatsimikiziridwa ndikuwonetsa kwakukulu kwa pigment melanin.

Njira ina yothandiza yopezera matendawa ndi mayeso onyansa. Desferal imayambitsidwa (imatha kumangiriza ndi mitundu yayikulu yazitsulo), yomwe pambuyo pake imachoka m'thupi. Chizindikiro cha matendawa pakuyesedwa uku ndi kugawa kwa chitsulo ndi mkodzo pamwamba pazowoneka bwino (pamilingo yoposa 2 milligram).

Ithandizira kuzindikira hemochromatosis ndi ultrasound ya chiwindi ndi kapamba.

Zizindikiro za matendawa

Matendawa amagawidwa m'magawo atatu panthawi ya chitukuko:

Thupi silodzaza ndi chitsulo, koma pamakhala matendawo amtunduwu.
Thupi limadzaza ndi chitsulo, koma zizindikiro zamankhwala sizinawonekerebe.
Gawo la mawonetseredwe a matendawa.

Matendawa amayamba kuonekera pa ukalamba. Pofika nthawi imeneyi, kuchuluka kwa ayironi m'thupi kumatha kufika magalamu 40, ndipo nthawi zina ochulukirapo.

Zizindikiro zoyambirira nthawi zambiri zimakhala zowonda, kutopa, kuchepa thupi, kuchepa kwa libido mwa amuna - onse Zizindikirozi zitha kuonekera kwa chaka chimodzia. Nthawi zambiri pamakhala kupweteka mu hypochondrium yoyenera, mafupa, kuuma komanso kusintha kwa khungu.

Pa nthawi yodziwira matendawa komanso kuwonekera kwathunthu, wodwalayo amakhala ndi khungu, khungu komanso matenda ashuga.

Kugonana ndi chimodzi mwazizindikiro zoyambirira za matendawa.. Kutalika kwake kumatengera kutalika kwa matendawa. Khungu lamunthu losakhala lachilengedwe limakhala lodziwikirika m'malo owonekera a khungu.
M'magulu ambiri odwala, chitsulo chimayikidwa mu chiwindi. Izi zikuchitika chifukwa cha kuchuluka kwa chiwindi, komwe kumatsimikizidwa pafupifupi odwala onse.
Ambiri mwa odwala, zovuta za endocrine zimapezeka. Matenda ofala kwambiri ndi matenda ashuga.
Gawo lachitatu la odwala ali ndi mawonetseredwe azachipatalaNdili kuwonongeka kwa mtima.

Chifukwa chake, zizindikiro zazikulu za matendawa ndi:

Kupweteka kwapakati ndi nyamakazi,
Kupatsirana khungu
Kufooka pafupipafupi ndi kutopa,
Kuthamanga kwa magazi
Matenda a shuga
Matenda a Endocrine
Kukula kwa chiwindi.

Zizindikiro zofananazi zikamapezeka m'mbale za wodwalayo, ndikofunikira kukaikira kupezeka kwa matendawa.

Mavuto

Cirrhosis. Ndi cirrhosis, ntchito zofunika za chiwindi zimachepetsedwa kwambiri, mwachitsanzo, kupatsirana, kuphatikiza mapuloteni ndi zinthu zina zomwe zimayambitsa khungu, zomwe zimapangitsa ngozi yaumoyo wa anthu.
Sepsis - matenda opatsirana a thupi okhala ndi mabakiteriya owopsa komanso poizoni omwe amalowa m'magazi. Sepsis imayenderana ndi kuledzera kwambiri komanso kukulira vuto la wodwalayo. Ndi sepsis, matendawa amatha kulowa ziwalo ndi machitidwe a thupi.
Hypogonadism - matenda omwe akuwoneka ndi kuchepa pakupanga mahomoni ogonana, omwe angayambitse kusabala, kubereka.
Arthropathy (matenda ogwirizana) - matenda angapo mu zovuta, kuphatikiza kuphwanya kagayidwe kachakudya mafupa.
Zosintha mu chithokomirozikubweretsa kusokonekera kagayidwe ka mapuloteni ndi chakudya.

Zambiri za maphunziro a hemochromatosis

kuchuluka kotenga kachilombo ka matenda a bacteria.
macrocytosis ndi kuchuluka kwa ma ESR (osati chifukwa cha cirrhosis) adadziwika ndi cholowa cha hemochromatosis.
mukazindikira chibadwa cha hemochromatosis, ndikofunikira kupenda abale am'banja. Pazotsatira zosakhala bwino za kukhalapo kwa matendawa, kuunikiridwa kuyenera kubwerezedwa zaka zisanu zilizonse.

Mankhwala a hemochromatosis, amalembedwa:

Zakudya Zitsulo. Zakudya za tsiku ndi tsiku ziyenera kuphatikizapo zakudya zomanga thupi. Komanso, odwala saloledwa kumwa zakumwa zoledzeretsa.
Mankhwala opepera chitsulo chochokera m'thupi.
Kukhetsa magazi. Njirayi imathandizira kuchotsa chitsulo mthupi ndipo imabweretsa zotsatira zabwino pamlingo wa wodwalayo, kuchepetsa khungu ndi kukula kwa chiwindi.
Cytophoresis - njira yomwe magazi amadutsidwa kudzera m'chiuno chotsekedwa ndikubwerera. Kuphatikiza apo, gawo lina la magazi limachotsedwa (nthawi zambiri ili ndi gawo lake lama cellular).

Ndi matenda amodzimodzi omwe amayamba chifukwa cha kuchuluka kwazitsulo mu ziwalo ndi minofu, chithandizo chothandizirachi chingafunike. Mwachitsanzo, mukazindikira matenda a shuga, ndikofunikira kuchita chithandizo choyenera ndikuwunikira ndikuwongolera matendawa.

Pakuzindikiritsa matenda ndi kuwonongeka kwa chiwindi, kuyang'anira pafupipafupi ndikuwunikanso bwino momwe thupilo limafunikira. Izi ndichifukwa choti odwala ali ndi chiopsezo chachikulu chotupa chotupa cha chiwindi.

Hemochromatosis ndimatenda ambiri a mtundu wakubadwa. Palibe chithandizo chomwe chidapezeka kuti chithana ndi omwe amayambitsa matendawa. Mpaka pano, alipo ndipo akugwiritsa ntchito njira zapadera zochepetsera mawonetseredwe azachipatala, kuchepetsa mwayi wamavuto ndikuchepetsa mkhalidwe wa wodwalayo.

Kuchita bwino kwa mankhwalawa komanso kutha kwa kuchira kwa wodwala zimatengera "zaka" zamatendawa. Munthawi yoyenera komanso moyenera chithandizo imatenga zaka zambiri pakadutsa zaka makumi angapo. Popanda chithandizo chonse, chiyembekezo chokhala ndi moyo wa odwala, atazindikira matendawa m'magawo omaliza, nthawi zambiri sichikhala choposa zaka 5.

Malangizo popewa

Popeza hemochromatosis nthawi zambiri imakhala matenda obadwa nawo, pali kuthekera kwakukulu kwa kupezeka kwa abale apafupi (pafupifupi 25%). Dziwani ndi kuwunika kwa achibale azaka za 18-30. Pankhani yoyambitsa matendawa m'magawo oyambira, ndizotheka kupewa zovuta zina zazikulu.

Popewa kuchitika kwa sekondale hemochromatosis, tikulimbikitsidwa kutsatira zakudya zoyenera, kudya moyenera mankhwala okhala ndi chitsulo, kuchititsa kuyang'anira ndikuwongolera matenda a chiwindi ndi magazi.

Ngati ndi kotheka, ndi hemochromatosis, ndizotheka kukhala wopereka magazi. Zigawo za magazi zimakhalabe zolimba, ndipo matendawa satumizidwa mwachindunji kudzera pakuikidwa magazi.

Ngati mayi ali ndi hemochromatosis, ndipo wapeza kapena akukonzekera kutenga pakati, ayenera kudziwitsa adokotala za izi. Koma pa kukula kwabwinobwino kwa pakati, izi sizowopsa.

Kodi hemochromatosis ndi chiyani?

Hemochromatosis ndimatenda a chiwindi omwe amadziwika ndi kupuwala kwachitsulo. Izi zimakwiyitsa kudzikundikira kwazinthu zokhala ndi chitsulo ndi ma pigment mu ziwalo. Mtsogolomo, izi zimapangitsa kuti ziwalo zingapo zizituluka. Matendawa adatenga dzina chifukwa cha mawonekedwe a khungu ndi ziwalo zamkati.

Nthawi zambiri ndi chibadwa cha hemochromatosis chomwe chimachitika. Pafupipafupi pamakhala zochitika pafupifupi 3-4 pa anthu 1000. Komanso, hemochromatosis ndi yofala kwambiri mwa amuna kuposa akazi. Kukula kwachangu, ndipo zizindikiro zoyambirira za matendawa zimayamba kudziwonetsa ali ndi zaka 40-50. Popeza hemochromatosis imakhudza pafupifupi machitidwe ndi ziwalo zonse, madokotala m'magawo osiyanasiyana amatenga nawo mankhwalawa matenda: mtima, gastroenterology, rheumatology, endocrinology.

Akatswiri amasiyanitsa mitundu iwiri yayikulu yamatenda: yoyamba ndi yachiwiri. Hemochromatosis ya pulayimale ndi chilema m'machitidwe a enzyme. Kusweka kumeneku kumakwiyitsa kuchuluka kwa chitsulo mkati mwa ziwalo zamkati. Nawonso, hemochromatosis yoyamba imagawidwa m'mitundu inayi, kutengera mtundu wosalimba:

Autosomal recessive classic,
Mwana
Cholowa chodzipatula,
Autosomal wamkulu.

Kukula kwa sekondale hemochromatosis kumachitika motsutsana ndi maziko omwe adapezeka a enzyme machitidwe omwe amatenga nawo gawo pazitsulo zachitsulo. Second hemochromatosis imagawikidwanso m'mitundu yosiyanasiyana: alimentary, kutumiza magazi, metabolic, neonatal, kusakanikirana. Kukula kwa mtundu uliwonse wa hemochromatosis kumachitika m'magawo atatu - popanda chitsulo chowonjezera, ndi chitsulo chowonjezera (chopanda zizindikiro), ndi chitsulo chowonjezera (ndi mawonekedwe awonetsero).

Zoyambitsa zazikulu za hemochromatosis

Hereditary hemochromatosis (pulayimale) ndi mtundu wofatsa wamatenda otha kufala. Chifukwa chachikulu chomwe chimapangidwira kuti chimatha kutchedwa kusintha kwamtundu wotchedwa HFE. Ili pa phewa lalifupi mu chromosome yachisanu ndi chimodzi. Kusintha kwamtunduwu kumapangitsa kuti chitsulo cham'mimba chisagwidwe. Zotsatira zake, chizindikiro chabodza chimakhazikitsidwa pa kusowa kwachitsulo mthupi ndi magazi. Vutoli limayambitsa kutulutsa kowonjezereka kwa mapuloteni a DCT-1, omwe amamanga chitsulo. Chifukwa chake, mayamwidwe a chinthucho m'matumbo amathandizidwa.

Kupitilira apo, matenda am'mimba amatsogolera pakuwonjezereka kwa mitundu yazitsulo. Mota ikangowonjezereka, kufa kwa zinthu zambiri zogwirika kumawonedwa, zomwe zimayamba chifukwa cha njira zoyipa. Chomwe chikuwoneka ngati yachiwiri hemochromatosis ndichakudya chambiri chakunja kuchokera kunja. Izi nthawi zambiri zimachitika motsutsana ndi maziko a mavuto awa:

Mafuta okwanira omwe amamwa mankhwala achitsulo,
Thalassemia
Anemia
Cutaneous porphyria,
Mankhwala osokoneza bongo a chiwindi
Virusi chiwindi B, C,
Zotupa zoyipa,
Kutsatira zakudya zochepa zama protein.

Zizindikiro za matendawa

Hemochromatosis ya chiwindi imadziwika ndi zizindikiro zowoneka bwino. Koma, zizindikiro zoyambirira za matenda zimayamba kudziwonetsa akadzakula - atatha zaka 40. Kufikira nthawi yino ya moyo, mpaka magalamu 40 a chitsulo amadziunjikira m'thupi, zomwe zimaposa kwambiri zovomerezeka zonse. Kutengera ndi gawo la chitukuko cha hemochromatosis, zizindikiro za matendawa zimasiyanitsidwa. M'pofunika kuwaganizira mwatsatanetsatane.

Zizindikiro za gawo loyamba lachitukuko

Matendawa amakula pang'onopang'ono. Pa gawo loyamba, zizindikirozi sizifotokozedwa. Kwa zaka zambiri, wodwalayo amatha kudandaula za ambiri: malaise, kufooka, kutopa, kuchepa thupi, kuchepa kwa potency mwa amuna. Kupitilira apo, zizindikiritso zochulukirapo zimayamba kulumikizana ndi izi: kupweteka kwa chiwindi, kupweteka kwa pakhungu, khungu louma, kusintha kwa ma testicles mwa amuna. Pambuyo pa izi, kukula kwamphamvu kwa hemochromatosis kumachitika.

Zizindikiro zakutha kwa hemochromatosis

Zizindikiro zazikulu za tsambali ndi izi:

Makulidwe a khungu,
Kutulutsa kwamkati mucous,
Cirrhosis
Matenda a shuga.

Herederal hemochromatosis, monga mtundu wina uliwonse, amadziwika ndi pigmentation. Ichi ndiye chizindikiritso chofala kwambiri komanso chofunikira kwambiri chosinthira matendawa kukhala gawo lotukuka. Kuwala kwa chizindikirocho kumatengera kutalika kwa matendawa. Nyimbo zakuda zautsi ndi zamkuwa, nthawi zambiri zimawonekera m'malo otseguka khungu - nkhope, manja, khosi. Komanso, mawonekedwe a mawonekedwe amtundu wamtunduwu amadziwika bwino.

Chitsulo chowonjezera chimayikidwa makamaka mu chiwindi. Chifukwa chake, pafupifupi wodwala aliyense amapezeka kuti akuwonjezeka m'matumbo. Kapangidwe ka chiwindi kamasinthanso - kamakhala kowonda kwambiri, kupweteka palpation. Odwala 80% amakhala ndi matenda a shuga, ndipo nthawi zambiri amadalira insulin. Zosintha za Endocrine zimawonetsedwa muzizindikiro zotere:

Kusowa kwanyengo,
Kupanikizika kwa pineal gland,
Kuphwanya ndulu za adrenal,
Kutha kwa gonads, chithokomiro England.

Kuchuluka kwambiri kwazitsulo mu ziwalo za mtima zamtima pazoyambira chiberekero cha hemochromatosis kumachitika mu 95% ya milandu. Koma, zizindikiro zochokera kumbali ya mtima zimawonekera mu 30% yokha yamatenda onse. Chifukwa chake, kukulitsa kwa mtima, arrhythmia, kukanika kwa mtima kumadziwika. Pali zodziwika kutengera ndi jenda. Chifukwa chake, amuna ali ndi testicular atrophy, kusabala kwathunthu, gynecomastia. Akazi nthawi zambiri amakhala ndi vuto losabala, amenorrhea.

Zizindikiro zamagetsi a hemochromatosis

Munthawi imeneyi, akatswiri amawona momwe ziwalo zimagwirira ntchito. Izi zimawonekera mu mawonekedwe a chitukuko cha matenda a portal, kulephera kwa chiwindi, kupsinjika kwamtima, kutopa, dystrophy, ndi matenda a shuga. Zikatero ,imfa imakonda kupezeka chifukwa cha magazi a mitsempha ya varicose yam'minyewa, peritonitis, matenda ashuga ndi chiwindi. Chiwopsezo chotukula chotupa neoplasms chimachuluka. Juvenile hemochromatosis, yemwe amakula kwambiri ali ndi zaka 20-30, amawerengedwa ngati mawonekedwe osowa. Kwenikweni, chiwindi ndi dongosolo lamtima limakhudzidwa.

Chithandizo cha hematchatatosis

Chithandizo cha hemochromatosis ndichachidziwikire. Cholinga chachikulu cha mankhwalawa ndikuchotsa chitsulo m'thupi. Koma, ndikofunikira kuti chidziwitsochi chipangidwe molondola. Pambuyo pokhapokha chithandizo akalandira. Kudzichitira nokha mankhwala nkoletsedwa. Chifukwa chake, gawo loyamba la chithandizo ndikumwa mankhwala omangira chitsulo.

Mankhwalawa, akamwetsa, amayamba kumangiratu ma mamolekyulu azitsulo, ndi kutulutsa kwawo kwina. Pachifukwa ichi, yankho la 10% la desferal limagwiritsidwa ntchito nthawi zambiri. Amapangidwira makina amkati. Njira ya mankhwalawa mankhwala dokotala yekha, kutengera kuopsa kwa mapangidwe a hemochromatosis. Pafupifupi, maphunzirowa amatenga milungu iwiri kapena itatu.

Chofunikira pakuchiritsa kwa hemochromatosis ndi phlebotomy. Njirayi imadziwikanso kuti magazi. Kuyambira kale, kuthira magazi kuchiritsa matenda osiyanasiyana. Ndipo hemochromatosis imabwereketsa bwino mtundu uwu wa chithandizo. Chifukwa chololeza, kuchuluka kwa maselo ofiira m'magazi athunthu kumatsika. Chifukwa chake, milingo yazitsulo imachepetsedwa. Kuphatikiza apo, phlebotomy imachotsa mwachangu ma pigmentation, kukanika kwa chiwindi. Koma, ndikofunikira kutsatira mitundu yonse ndi malamulo a njirayi. Chifukwa chake, mtunduwo umadziwika kuti ndi 300-400 ml wamagazi nthawi. Koma ndi kutayika kwa 500 ml ya magazi, wodwalayo angamve bwino kwambiri. Ndikokwanira kuchita njirayi 1-2 pa sabata.

Panthawi yamankhwala, ndikofunikira kuyang'ana zinthu izi:

Kupatula mowa,
Kukana kudya zakudya zowonjezera,
Kukana kumwa vitamini C, ma multivitamin complexes,
Kuchotsedwa kwa zakudya zazitsulo zambiri pazakudya
Kukana kugwiritsa ntchito zopangika zam'mimba mosavuta.

Kuti ayeretse magazi, akatswiri amatha kugwiritsa ntchito plasmapheresis, cytapheresis kapena hemosorption. Munthawi yomweyo ndikuchotsa chitsulo, chithandizo cha chiwindi, kulephera kwa mtima, komanso matenda osokoneza bongo ndikofunikira. Kusamalira matendawa moyenera kumaphatikizapo kutsatira zakudya zina.

Zakudya hemochromatosis

Kudya ndi matenda oterewa kumathandizira pakuthandizira. Chifukwa chake, zogulitsa zomwe zimapangitsa chitsulo chochuluka zimaphatikizidwa kwathunthu kuchokera kuzakudya za wodwalayo. Izi zikuphatikiza ndi izi:

Nkhumba, ng'ombe,
Buckwheat groats
Pistachios
Maapulo
Nyemba
Chimanga
Sipinachi
Parsley

Ndikofunika kukumbukira kuti nyama ikada, ndiye kuti michere yambiri imakhalamo. Ndi hemochromatosis, ndizoletsedwa kumwa zakumwa zilizonse. Kuchulukitsidwa kwachitsulo kumabweretsa kuti munthu azidya Vitamini C. Chifukwa chake, ascorbic acid iyeneranso kuyikidwa pambali. Akatswiri amati sikofunikira kusiya zonse zomwe zimakhala ndi chitsulo. Mumangofunika kuchepetsa kuchuluka kwaomwe amamwa.

Kupatula apo, hemochromatosis ndi matenda amchere wachuma. Ndikofunika kusintha mulingo wake. Koma kuchepa kwachitsulo kumayambitsa matenda owopsa a m'magazi. Chilichonse chikuyenera kukhala chopumira. Mukamakonza menyu yazakudya, muyenera kusintha nyama yakuda ndi kuwala, phala la buckwheat ndi tirigu. Kugwirizana ndi zakudya zotere kumathandizira kuchira, kukonza mkhalidwe wa wodwala.

Kodi ukuneneratu chiyani?

Pankhani ya hemochromatosis yodziwikiratu, moyo wa wodwalayo umawonjezedwa kwa zaka makumi ambiri. Kwakukulukulu, kudalirika kumayesedwa kuganiziranso za kuchuluka kwa ziwalo. Kuphatikiza apo, hemochromatosis imachitika munthu akadzakula, matendawo amakumana nthawi zambiri. Ngati simukuchita nawo mankhwala a hemochromatosis, nthawi yayitali ingakhale zaka 3-5. Kupeza bwino kosavomerezeka kumawonedwanso pakuwonongeka kwa chiwindi, mtima ndi endocrine dongosolo lomwe limayambitsa matendawa.

Popewa kukula kwa hemochromatosis yachiwiri, ndikofunikira kutsatira malamulo a kupewa. Zakudya zikuluzikulu ndizakudya zopatsa thanzi, zomwe zimatenga kukonzekera kwachitsulo kokha moyang'aniridwa ndi dokotala, kuikidwa magazi pafupipafupi, kupatula mowa, komanso kuyang'anira kusanza pamaso pa matenda a mtima ndi chiwindi. Hemochromatosis yoyamba imafunika kuwunika kwa mabanja. Pambuyo pa izi, chithandizo chothandiza kwambiri chimayamba.

Anatomy ndi physiology ya chiwindi

Ndi hemochromatosis, chiwindi, chomwe chimakhudzidwa ndi kagayidwe kachitsulo, nthawi zambiri chimakhudzidwa.

Chiwindi chimakhala pansi pa phokoso lamanja la diaphragm. Pamwambapa, chiwindi chimayandikana ndi diaphragm. Malire otsika a chiwindi ali pamlingo wa nthiti 12. Pansi pa chiwindi pali ndulu. Kulemera kwa chiwindi mwa munthu wamkulu kumakhala pafupifupi 3% ya kulemera kwa thupi.

Chiwindi ndi chiwalo cha mtundu wofiirira, mawonekedwe osakhazikika komanso kusasinthasintha. Imasiyanitsa pakati pa malo okhala kumanja ndi kumanzere. Gawo la lobe lamanja, lomwe lili pakati pa fossa ya ndulu (kama kama gallbladder) ndi chipata cha chiwindi (komwe miziwalo, mitsempha imadutsa), limatchedwa mraba lobe.

Chiwindi chimakutidwa ndi kapisozi pamwamba. Mu kapisozi muli mitsempha yomwe imalowetsa chiwindi. Chiwindi chimapangidwa ndi maselo otchedwa hepatocytes. Maselo amenewa amatenga nawo gawo pama protein angapo amchere, amchere, komanso amathandizira kupanga mapangidwe a bile (njira zovuta, chifukwa cha momwe bile limapangidwira).

Ntchito za chiwindi:
1. Kusasinthika kwa zinthu zosiyanasiyana zovulaza thupi. Chiwindi chimachotsa poizoni wambiri (ammonia, acetone, phenol, ethanol), ziphe, allergen (zinthu zingapo zomwe zimayambitsa thupi kupewa).

2. Ntchito ya Depot. Chiwindi ndimasungidwe a glycogen (chakudya chosungirako chopangidwa kuchokera ku glucose), potero amatenga nawo gawo la metabolism (metabolism) ya glucose.
Glycogen imapangidwa mutatha kudya, kuchuluka kwa glucose m'magazi kukwera kwambiri. Kuchuluka kwa shuga m'magazi kumabweretsa kupangika kwa insulin, ndipo, nawonso, amatenga nawo gawo pakusintha kwa glucose kukhala glycogen. Mwazi wamagazi ukatsika, glycogen imasiya chiwindi, ndipo mothandizidwa ndi glucagon amasinthidwa kukhala glucose.

3. Chiwindi chimapanga bile acid ndi bilirubin. Pambuyo pake, bile acid, bilirubin, ndi zinthu zina zambiri zomwe chiwindi chimagwiritsa ntchito kupanga bile. Bile ndi viscous greenish chikasu chamadzimadzi. Ndikofunikira kuti chimbudzi chikhale bwino.
Bile, yotulutsidwa mu lumen ya duodenum, imayendetsa ma enzymes ambiri (lipase, trypsin, chymotrypsin), komanso imachita nawo mwachindunji pakuwonongeka kwa mafuta.

4. Kutaya mahomoni owonjezera, oyimira pakati (mankhwala omwe amaphatikizidwa ndi mchitidwe wamanjenje). Ngati mahomoni owonjezera sakusankhika pakapita nthawi, vuto lalikulu la kagayidwe kachakudya ndi moyo wathupi lathunthu zimachitika.

5. Kusunga ndi kudzikundikira kwa mavitamini, makamaka magulu A, D, B₁₂. Ndikufuna kudziwa kuti chiwindi chimakhudzidwa ndi metabolism ya mavitamini E, K, PP ndi folic acid (yofunikira kuphatikizira kwa DNA).

6. Chiwindi chokhacho cha mwana wosabadwayo chimagwira hematopoiesis. Mwa munthu wamkulu, imathandizira kupanga magazi m'magazi (amatulutsa fibrinogen, prothrombin). Chiwindi chimapanganso albumin (mapuloteni onyamula omwe amapezeka m'madzi a m'magazi).

7. Chiwindi chimapanga mahomoni ena okhudzana ndi chimbudzi.

Udindo wazitsulo mthupi

Iron amadziwika kuti ndi chinthu chofunikira kwambiri chotsatira. Kuchuluka kwachitsulo muzakudya za tsiku ndi tsiku kumakhala pafupifupi mamililita 10 mpaka 10, omwe ndi 10% yokha omwe amamwe. Munthu wathanzi amakhala ndi pafupifupi 4-5 magalamu achitsulo. Zambiri mwa izo ndi gawo la hemoglobin (yofunikira kuperekera minofu ndi mpweya), myoglobin, ma enzyme osiyanasiyana - catalase, cytochromes. Iron, yomwe ndi gawo la hemoglobin, imapanga pafupifupi 2.7-2.8% pazitsulo zonse mthupi.

Gwero lalikulu la chitsulo kwa anthu ndi chakudya, monga:

nyama
chiwindi
impso.

Zogulitsa zotere zimakhala ndi chitsulo mumapangidwe osavuta kugaya.

Iron kudziunjikira (aikamo) mu chiwindi, ndulu, ndi mafuta m'mafupa mu mawonekedwe a ferritin (mapuloteni okhala ndi chitsulo). Ngati ndi kotheka, chitsulo chimachoka m'malo ogulitsira ndipo chimagwiritsidwa ntchito.

Ntchito zachitsulo m'thupi la munthu:

Chitsulo ndichofunikira pakupanga maselo ofiira am'magazi (ma cell ofiira a magazi) ndi hemoglobin (puloteni yomwe imanyamula oxygen),
Imagwira ntchito yofunika kwambiri pakuphatikizidwa kwa maselo a chitetezo cha m'thupi (maselo oyera am'magazi, macrophages),
imagwira ntchito pakupanga mphamvu mu minofu,
amatenga nawo gawo pakusinthana ndi cholesterol,
amalimbikitsa kukonzanso thupi m'zinthu zovulaza,
imalepheretsa kudzikundikira m'thupi la zinthu zamagetsi (mwachitsanzo plutonium),
gawo la michere yambiri (catalase, cytochromes), mapuloteni m'magazi,
amatenga nawo gawo mu DNA.

Zizindikiro za hemochromatosis

Kuzikongoletsa kwa khungu (nkhope, kutsogolo kwa mkono, mbali yakumanja ya dzanja, navel, nipple ndi genitalia yakunja). Chizindikiro ichi chimapezeka mu 90% ya milandu.
Khungu lowoneka khungu limachitika chifukwa cha hemosiderin, komanso mbali ina ya melanin.
Hemosiderin ndi utoto wachikasu wakuda wokhala ndi chitsulo oxide. Amapangidwa pambuyo pa kuphulika kwa hemoglobin komanso kuwonongeka kwa mapuloteni pambuyo pake.
Ndi kudzikundikira kwa hemosiderin yambiri, khungu limavala zovala zofiirira kapena zamkuwa.
Kuperewera kwa tsitsi pankhope ndi thupi.
Ululu m'mimba yamphamvu kwambiri, osakhala ndi kutengera kwina.
Chizindikiro ichi chimapezeka mu 30-40% ya milandu. Kupweteka kwam'mimba nthawi zambiri kumayendetsedwa ndi zovuta za dyspeptic.
Dyspeptic syndrome zimaphatikizira zizindikiro zingapo: nseru, kusanza, kutsegula m'mimba, kusowa kwa chakudya.
Khansa ya m'mimba imakhala yosangalatsa m'mimba kapena m'mphepete. Khansa ya m'mimba nthawi zambiri imayendera limodzi ndi chizungulire, kufooka.
Kusuntha ndi chinthu chowoneka bwino komwe kumayamba kuphulika kwam'mimba kudzera mkamwa. Kusintha kumachitika chifukwa cha kupindika kwamkati m'mimba.
Kutsekula m'mimba ndi vuto lomwe limakhalapo kangapo (kawiri pa tsiku). Zidutswa zokhala ndi m'mimba zimakhala zamadzimadzi (zamadzimadzi).
Kupezeka kwa wodwala matenda ashuga. Matenda a shuga ndi matenda amtundu wa endocrine momwe mumakhala kuchuluka kwa shuga (glucose) m'magazi. Pali zifukwa zambiri zomwe zimayambitsa matenda ashuga. Chimodzi mwa izo ndi kusakwanira katemera wa insulin. Ndi hemochromatosis chifukwa cha kuchuluka kwachitsulo mu kapamba, maselo abwinobwino amawonongeka. Pambuyo pake, mitundu ya fibrosis - maselo abwinobwino a chimbudzi amasinthidwa ndi ma cell olumikizana, ntchito yake imachepa (satulutsa insulin).
Matenda a shuga amapezeka 60-80% ya milandu.
Hepatomegaly - kuchuluka kwa chiwindi. Poterepa, ndi chifukwa cha kuchuluka kwachitsulo. Zimachitika mu 65-70% ya milandu.
Splenomegaly - kukulitsa kwa kukula kwa ndulu. Zimachitika mu 50-65% ya milandu.
Cirrhosis Ndi matenda ochitika mosadukiza momwe maselo amoyo wathanzi amasinthidwa ndi minyewa yam'mimba. Cirrhosis imachitika mu 30-50% ya milandu.
Arthralgia - zilonda m'malumikizidwe. Nthawi zambiri ndi hemochromatosis, zolumikizana zolumikizana za zala ziwiri ndi zitatu zimakhudzidwa. Pang'onopang'ono, mafupa ena (ulnar, bondo, phewa komanso kawirikawiri m'chiuno) amayamba kukhudzidwa. Pakati pa madandaulo, pali zoletsa kuyenda kwa malo olumikizirana, ndipo nthawi zina kuwonongeka kwawo.
Arthralgia imapezeka mu 44% ya milandu. Kufunsidwa kwa rheumatologist ndikulimbikitsidwa.
Kugwiriridwa. Nthawi zambiri, kusowa pogonana ndi kupanda mphamvu - zimachitika mu 45% ya milandu.
Kusabala ndi matenda omwe mwamuna sangathe kugona nawo mwanjira yovomerezeka, kapena sangakwaniritse. Kufunsidwa kwa katswiri wazogonana kumalimbikitsidwa.
Mwa akazi, amenorrhea imatheka mu 5-15% ya milandu.
Amorrhea - kusowa kwa msambo kwa miyezi 6 kapena kupitirira. Kufunsira kwa gynecologist ndikulimbikitsidwa.
Matenda monga hypopituitarism (kusowa kwa mahomoni amodzi kapena zingapo), hypogonadism (kuchuluka kosakwanira kwa mahomoni ogonana) ndi osowa.
Matenda a mtima (arrhythmias, cardiomyopathies) amapezeka 20-50% ya milandu.
Arrhythmia ndi mkhalidwe womwe kuphwanya mzere wamtima kumachitika.
Cardiomyopathy ndimatenda amtima omwe myocardium imakhudzidwira.
Pakadandaula izi, ndikofunikira kufunsa katswiri wamtima.

Pali wotchedwa classical triad wa hemochromatosis. Izi ndi: cirrhosis, matenda a shuga komanso khungu lotupa. Kutumphuka kotereku kumawonekera, monga lamulo, pamene kuchuluka kwazitsulo kumafikira 20 magalamu, omwe amapitilira muyeso yachilengedwe ka 5.