IHemchromatosis, eyaziwa nangokuthi isifo sikashukela se-bronze, yisifo esiyinzalo esiyivelakancane kakhulu esihambisana nokuphulwa okukhulu kwe-iron metabolism emzimbeni womuntu.
Ngalesi sifo, lokhu okulandela umkhondo kudonswa ngokweqile emathunjini bese kuqongelelwa ezithweni nasezithanjeni, kubangele ukweqisa.
Ngenxa yalokhu, isibindi siyalimala, izinhlungu ezijoyineni ziyavela, umbala wesikhumba uyashintsha, inhlala-kahle yomguli iyaqina. Lesi sifo asikwazi ukubizwa ngokuthi sande kakhulu, ngakho-ke, abantu abaqale ukuzwa ukutholakala kwe-hemochromatosis abaqondi ngokushesha ukuthi siyini. Lo mbhalo uchaza kabanzi izimbangela, izimpawu nezindlela ezisetshenziswa kakhulu zokuxilongwa, ukwelashwa kwalesi sifo.
Izici zesifo
Hlukanisa phakathi kwe-hemochromatosis eyisisekelo neyesibili. Esokuqala sihambisana nokuguqulwa kofuzo okuku-chromosome wesithupha, okungukuthi, kuyifa. Kwenzeka cishe kubantu abathathu kwabayinkulungwane, kaningi kumadoda. I-heeredch hemochromatosis ibonakaliswa isivele ikhulile, imvamisa eminyakeni engama-40-60, kwabesifazane ngisho nangemva kwalokho.
I-Hemochromatosis yesibindi
I-hemochromatosis yesibili ibangelwa izimbangela zangaphandle futhi ingaxilongwa ngokudla ngokweqile kwe-iron emzimbeni wethu, ngokwesibonelo, ngokuhlukunyezwa kwamalungiselelo wensimbi wokwenza noma ngenxa yokumpontshelwa igazi njalo. Kwesinye isikhathi kungakhula njengokuxakaniseka kwezifo zegazi lapho imetabolism yensimbi iphazamiseka khona.
Imvamisa, umzimba womuntu wesifazane uqukethe i-300-1000 mg yensimbi, amadoda - cishe u-500-1500 mg. Iningi lazo liyingxenye ye-hemoglobin, kanti into etholakalayo nayo iyatholakala kuma-enzymes nakuma-pigment. Kuma-iron ahlinzekwe ngokudla, kuphela ingxenye yeshumi, i-1-1.5 mg, imvamisa, futhi nge-hemochromatosis leli nani lenyuka lifinyelela ku-3-4 mg ngosuku.
Kuyavela ukuthi isikhathi esingaphezu konyaka igramu yonke yensimbi iqongelela emzimbeni wesiguli, ifakwa esibindini, enhliziyweni, pituitary, pancreas, njll., Okuholela ekuphazamiseni ukusebenza kwabo.
Ngokushesha, isiguli siba ne-cirrhosis, bese ama-30% eba nomdlavuza wesibindi. Ama-pancreas nawo ayahlupheka, okuthi emuva kwalokho kuholele ekukhuleni kwesifo sikashukela.
Ngenxa yokungasebenzi kahle kwe-pituitary gland, izinsizakusebenza ekusebenzeni kohlelo lokuzala ziyabonakala: ukungabi namandla kokukhula kumadoda, ama-testicles atrophy, amabele angaqala ukukhula, futhi kwabesifazane umjikelezo uyaphazamiseka, i-libido iyancipha. Ukulimala enhliziyweni kuholela ekuphazamisweni kwesigqi futhi, kwezinye izikhathi, ekuhlulekeni kwenhliziyo.
Izimpawu
Izimpawu eziphambili ze-hemochromatosis:
- ukukhathala, ubuthakathaka;
- ukunciphisa umzimba
- ingcindezi ephansi
- ukucwilisa ulwelwesi lwama-mucous, i-sclera;
- ukuvuvukala kwamalunga, ukwanda kokuhamba kwawo;
- kwehla i-libido;
- ukumnyama kwesikhumba (kuthola i-bronze noma i-brown-brown hue).
Futhi, ukuqala okungazelelwe kwesifo sikashukela, i-cirrhosis yesibindi, ukuthuthuka kwenhliziyo nokuhluleka kwesibindi kungabangelwa yizimpawu zesifo.
Isibonakaliso esidalulwe kakhulu, kusobala, ukubukeka kwethoni yesikhumba ethile. Ubulongwe buvame ukwenziwa endaweni nasezandleni, ebusweni, emilenzeni, entanyeni nasezinsini zangasese. Imikhawulo yesikhumba nezintende ihlala ilula.
Izizathu
Isizathu esiyinhloko ubuhlakani, yikuthi kubangela i-hemochromatosis eyisisekelo.
Ngokuqondene nesekondari, ochwepheshe bakhomba lezi zinto ezilandelayo ezishukumisayo:
- i-hepatitis B ye-viral engapheli
- ukubekwa kwezicubu ze-adipose esibindini - i-nonato-steatohepatitis;
- isimila sesibindi;
- ukuphuma kwe-pancreatic duct;
- ukuhlinzwa kwe-portocaval bypass, okubangele ukwephulwa kwe-iron metabolism;
- ukudla okungalawulwa kwezidakamizwa eziqukethe i-iron;
- i-leukemia.
Ukuxilonga
Udokotela we-gastroenterologist noma i-endocrinologist anganquma nge-hemochromatosis (ikhodi yokuxilonga ngokuya ngokuhlukaniswa kwezifo emhlabeni jikelele ngezifo i-E83.1) ngokususelwa kulezi zinto ezilandelayo:
- izimpawu ezikhona kulesi sifo;
- umlando womndeni, okungukuthi, ukuba khona kwezihlobo eziseduzane ezihlangabezane nenkinga enjalo;
- imiphumela yokuhlaziywa okuphelele kokuqukethwe kwensimbi nezindlela zayo ezithile egazini;
- Imininingwane ye-punop biopsy ethathwe esibindini, okukhombisa ukufakwa kwensimbi emangqamuzaneni alesi sitho.
Futhi, ukuze kutholakale i-hemochromatosis, ukuba khona kwezinguquko ezivame kakhulu kuhlobo lwe-HFE obhekene nalesi sifo esizuzwayo kunganqunywa ngokuhlaziywa kwezakhi zofuzo.
Uma usola ukuba khona kwe-hemochromatosis, udokotela uzokuqondisa ukuthi uthathe ukuhlolwa kwegazi okujwayelekile, okune-biochemical, kanye nokuhlolwa kwegazi kokuqukethwe ushukela, uzodinga ukuhlolwa kwe-ultrasound kwezitho ezahlukahlukene zesibeleko, i-scintigraphy yesibindi.
Ngaphezu kwalokho, kuyadingeka ukuthola ukuthi kwaqhamuka kuphi i-iron ngesikhathi se-hemochromatosis nokuthi iziphi izitho ezithinteke kakhulu, ngakho-ke odokotela batusa ukuthi kwenziwe ukuhlolwa kwe-echocardiography ne-electrocardiography, i-biopsy yesibindi, kanye nokuhlolwa kwama-radiographic.
Ukwelashwa
Ukwelashwa kwalesi sifo kwenziwa ngokuphelele. Okokuqala, isiguli sinqunywa izidakamizwa ezibopha i-iron (Deferoxamine, Desferal, Desferin), ezibandakanya izinto ezisebenzayo ezakha izingqimba zensimbi ezikhishwe emzimbeni. Isikhathi inkambo yokuphatha cishe izinsuku ezingama-20- 40.
Ukuchithwa
Kuyadingeka futhi ukulandela ukudla okhethekile: ukunciphisa ukusetshenziswa kwayo yonke imikhiqizo equkethe i-iron ngamanani aphezulu (inyama yenkomo, ihalananda, isibindi, i-buckwheat, njll), ukuqeda utshwala, ukunciphisa umkhawulo wokudla kwe-ascorbic acid emzimbeni wesiguli.
Imvamisa ukuphuma kwegazi kuchazwa yi-phlebotomy - udonga lwe-ulnar noma enye i-vein efanelekile ihlakazwa okwesikhashana ukukhipha inani elithile legazi (200-400 ml), ngenxa yalokho okuqukethwe yensimbi emzimbeni kuncipha, isimo sesiguli siyathuthuka, bese isikhumba sokwehla kwesikhumba sinciphe.
Le nqubo yenziwa izikhathi ezingama-1-2 ngesonto iminyaka engu-2-3, khona-ke imvamisa iyancipha, ukuphuma kwegazi kwenziwa kuphela kwesinye isikhathi ukuze kugcinwe inani elanele lezinto zokulandela emzimbeni.
Ngesikhathi sokusebenza kwalezi zinqubo, izinga le-hemoglobin, i-ferritin, i-hematocrit yegazi, kanye nenani le-iron okweqile kuqashelwa njalo. Kuvunyelwe ukwenza ukuphuma kwegazi kuze kube yilapho isiguli sikhula i-anemia emnene.
Kwezinye izimo, i-cytapheresis isetshenziswa - ukususwa kwengxenye yegazi legazi ngokubuya okuphelele kwe-autoplasma. Ngasikhathi sinye, ingxenye yamangqamuzana egazi abomvu, aqukethe iningi lensimbi, isuswa egazini, umphumela wokuqina uyabonakala futhi, ngenxa yokuthi ubukhulu bezinqubo ezahlukahlukene zokuwohloka nokuvuvukala kuncishiswa.
Izinkinga
Uma ukwelashwa kwe-hemochromatosis kungaqalwa ngendlela yesikhathi, ukwehluleka kwesibindi kwenzeka - lo mzimba awuhambelani nemisebenzi yawo, okungukuthi, izinqubo zokugaya ukudla, ukungathathi hlangothi kobuthi obungena emzimbeni kuyaphazamiseka.I-Arrhasmia nayo ivela, imvamisa ukwehluleka kwenhliziyo, okuwukuthi, ukwephulwa okukhulu kokuqina kwesicubu senhliziyo, ngenxa yokonakala kokumelana komzimba, izinkinga ezithathelwanayo ziba kaningi.
Imiphumela emibi kakhulu ye-hemochromatosis ukufakwa kwe-myocardial infarction, ukopha kulwelwesi lwe-mucous lwe-esophagus, lukashukela nesifo sikashukela kanye nesimila sesibindi.
Ukuvimbela Izinkinga
Lapho kukhona i-hemochromatosis eyisisekelo, kuyadingeka ukulandela ukudla okuvinjelwe imikhiqizo equkethe i-iron, i-ascorbic acid, kanye nokudla okunama-protein. Ngaphezu kwalokho, izidakamizwa ezibopha i-iron kumele zithathwe ngezifundo.
Amavidiyo ahlobene
Kucashunwe kumdlalo we-TV othi "Live Healthy!" mayelana nokuthi iyini i-hemochromatosis nokuthi ungabhekana kanjani nayo:
Njengoba ubona, lesi sifo ngeke sithathwe kalula, ukwelashwa kufanele kuqalwe ngokushesha. Izinga eliphakeme lensimbi emzimbeni linomphumela olimazayo ezinhlakeni nasezitho zomzimba ezahlukahlukene. Uma uhlangabezana nezimpawu zokukhathazeka, thintana nodokotela oyi-endocrinologist noma i-gastroenterologist ngokushesha.
