Hemochromatosis da aka fara bayyana shi azaman cuta ce daban a shekarar 1889. Koyaya, zai iya yiwuwa a iya tabbatar da ainihin abubuwan da ke haifar da cutar kawai tare da haɓakar ilimin likita.
Irin wannan cutar da ba a makara ba ta inganta ta yanayin cutar da kuma rage yawan rarrabuwarta.
Don haka, bisa ga bayanan zamani, 0.33% na mazaunan duniya suna cikin haɗarin haemochromatosis. Me ke haifar da cutar kuma menene alamun ta?
Hemochromatosis - menene?
Wannan cuta tana gado ne kuma tana nunawa da yawaitar alamu da kuma babban haɗarin manyan rikice-rikice da alaƙa da alaƙa.
Nazarin ya nuna cewa haemochromatosis galibi ana haifar dashi ta maye gurbi ne a cikin hanyar HFE.
Sakamakon gazawar tarin kwayoyin halitta, ba a lalata hanyoyin samar da baƙin ƙarfe a cikin duodenum ba.. Wannan yana haifar da gaskiyar cewa jiki yana karɓar saƙo na karya game da ƙarancin ƙarfe a cikin jiki kuma ya fara aiki da ƙarfi kuma yalwace ɗimbin ƙwaƙwalwa suna haɗuwa da ƙwayar ta musamman.
Wannan yana haifar da zubar da jini mai yawa na haemosiderin (glandular pigment) a cikin gabobin ciki. Tare da haɓaka aikin furotin, kunnawar hanji yana faruwa, yana haifar da wuce kima baƙin ƙarfe daga abinci a cikin hanji.
Don haka ko da tare da abinci na yau da kullun, adadin baƙin ƙarfe da ke jikin mutum yana da yawa sau da yawa fiye da na al'ada. Wannan yana haifar da lalata kyallen ƙwayoyin gabobin ciki, matsaloli tare da tsarin endocrine, da rigakafi.
Rarrabuwa ta nau'ikan, tsari da matakai
A cikin aikin likita, an rarraba nau'ikan cuta da firamare. A wannan yanayin, na farko, wanda kuma ake kira gado, shine sakamakon ƙaddarar jini. Hemochromatosis na biyu shine sakamakon ci gaban karkatawa a cikin aikin tsarin samar da enzyme wanda ke tattare da metabolism na glandular.
An san nau'ikan nau'ikan nau'ikan cututtaka guda biyu (kwayoyin).
- na gargajiya
- matasa;
- nau'in HFE-hereditary;
- autosomal rinjaye.
Nau'in na farko yana da alaƙa da yanayin maye gurbi na yanki na shida na chromosome. Ana gano wannan nau'in a cikin mafi yawan lokuta - fiye da kashi 95 na marasa lafiya suna fama da hemochromatosis na gargajiya.
Nau'in cututtukan yarinyar na faruwa ne sakamakon maye gurbi a cikin wata hanyar kwayoyin, HAMP. A ƙarƙashin tasirin wannan canjin, aikin hepcidin, enzyme wanda ke da alhakin sanya baƙin ƙarfe a cikin gabobin, yana ƙaruwa sosai. Yawancin lokaci cutar tana bayyana kanta a cikin shekaru goma zuwa talatin.
Irin nau'in HFE-wanda ba a haɗa shi ba yana tasowa lokacin da ƙwayar HJV ta gaza. Wannan ilimin aikin likita ya hada da hanyar samar da hyperactivation na masu karɓar transrin-2. Sakamakon haka, ana haɓaka samar da hepcidin. Bambanci tare da nau'in cutar ita ce cewa a farkon lamari, gene ya kasa, wanda shine kai tsaye alhakin samar da enzyme baƙin ƙarfe.
Ganin cewa a cikin abu na biyu, jiki yana haifar da yanayin halayen yawan ƙarfe a cikin abinci, wanda ke haifar da samar da enzyme.
Nau'in nau'in hemochromatosis na hereditary yana da alaƙa da cutarwar ƙwayar cuta ta SLC40A1.
Cutar tana bayyana kanta a cikin tsufa kuma tana da alaƙa da rashin daidaituwa na furotin na ferroportin, wanda ke da alhakin ɗaukar mahadi baƙin ƙarfe cikin sel.
Abubuwan maye gurbi suna haifar da dalilai masu haɗari
Kwayar halittar jini a cikin nau'in cuta mai gado shine sakamakon yanayin tunanin mutum.
Nazarin ya nuna cewa yawancin marasa lafiya fararen fata ne na Arewacin Amurka da Turai, tare da mafi yawan mutanen da ke fama da cutar haemochromatosis da aka lura tsakanin baƙi daga Ireland.
Bayan haka, yaduwar nau'ikan maye gurbi halaye ne ga sassa daban-daban na duniya. Maza suna saurin kamuwa da cutar sau da yawa fiye da mata. A karshen, bayyanar cututtuka bayyanar cututtuka bayan canje-canje na hormonal a cikin jiki sakamakon menopause.
A cikin marasa lafiyar da aka yiwa rajista, mata sun ninka sau 7-10 kasa da maza. Har yanzu ba a san dalilan canjin ba. Kawai yanayin gado na cutar an tabbatar da rashin tabbas, kuma alakar da ke tsakanin kasancewar hemochromatosis da fibrosis hanta kuma an gano.
Yayinda haɓakar ƙwayar haɗin haɗin kai ba zai iya bayanin kai tsaye ta hanyar tara baƙin ƙarfe a cikin jiki ba, har zuwa 70% na marasa lafiya da haemochromatosis suna da fibrosis hanta.
Haka kuma, yanayin gado ba lallai ya kai ga ci gaba da cutar ba.
Bugu da ƙari, akwai wani nau'in sakandare na hemochromatosis, wanda aka lura a cikin mutane tare da farawar ƙwayoyin jini na farko. Abubuwan da ke tattare da hadarin ma sun hada da wasu hanyoyin. Don haka, steatohepatitis da aka canza (ajiyar rashin shan giya na adipose nama), haɓakar hepatitis na cututtukan etiologies daban-daban, kazalika da toshewar hanji suna ba da gudummawa ga bayyanuwar cutar.
Kwayar cutar haemochromatosis a cikin mata da maza
A zamanin baya, kawai ci gaban bayyanannun alamun bayyanar cututtuka ne ya sanya aka sami damar gano wannan cutar.
Marasa lafiya tare da tara yawan ƙarfe yana jin gajiya mai rauni, rauni.
Wannan alamar dabi'a ce don 75% na marasa lafiya tare da bashinchromatosis. Haɓaka launin fata yana inganta, kuma wannan tsari bashi da alaƙa da samar da melanin. Fatar ta yi duhu saboda tara baƙin ƙarfe a wurin. Ana lura da duhu cikin fiye da 70% na marasa lafiya.
Sakamakon mummunan ƙwayar baƙin ƙarfe akan ƙwayoyin rigakafi yana haifar da rauni na rigakafi. Sabili da haka, tare da cutar, raunin haƙuri ga kamuwa da cuta yana ƙaruwa - daga ainihin tsanani zuwa banal kuma mara lahani a cikin yanayin al'ada.
Kimanin rabin marasa lafiya suna fama da cututtukan haɗin gwiwa waɗanda aka bayyana a cikin abin da ya faru na jin zafi.
Hakanan akwai tabarbarewa a cikin motsinsu. Wannan alamar na faruwa ne saboda yawan baƙin ƙarfe yana ɗaukar adadin alli a cikin gidajen abinci.
Arrhythmias da haɓakar bugun zuciya suma suna yiwuwa. Tasiri mara kyau akan cutar koda shine yakan haifar da ciwon suga. Arancin ƙarfe yana haifar da lalata gland shine yake. A cikin lokuta mafi ƙarancin yanayi, ana lura da ciwon kai.
Haɓakar cutar tana haifar da rashin ƙarfi a cikin maza. Rage aikin jima'i yana nuna alamun guba na jiki tare da kayan aikin ƙarfe. A cikin mata, zubar jini mai yawa yayin tsari yana yiwuwa.
Wani muhimmin alama shine haɓaka a hanta, da kuma raɗaɗin raunin ciki, yayin bayyanar da ba zai yiwu a tantance tsarin ba.
Kasancewar bayyanar cututtuka da yawa yana nuna buƙatar ingantaccen binciken dakin gwaje-gwaje na cutar.
Alamar cutar itace babban haemoglobin cikin jini, tare da karancin abun ciki a lokaci guda a cikin kwayoyin jini. Ana nuna alamun satirin canja wuri tare da baƙin ƙarfe a ƙasa da 50% ana ɗaukar alamar alamar dakin gwajin jini na hemochromatosis.
Babban haɓaka a hanta tare da ɗimbin yawa daga kyallen takarda ma alama ce ta cutar. Bugu da ƙari, tare da hemochromatosis, ana lura da canji a cikin launi na ƙwayar hanta.
Yaya bayyaninsa a cikin yaro?
Hemochromatosis na farko yana da fasali da yawa - daga maye gurbi wanda ya haddasa shi zuwa ga jerin sassan chromosome zuwa hoton halayyar halayyar da kuma bayyane.
Da farko dai, alamun cutar a farkon shekarun su polymorphic ne.
Yara suna halin haɓaka alamun bayyanar alama dake nuna hauhawar jini. Ya haifar da take hakkin abinci, yawan karuwa a hanta da hanta.
Tare da haɓakar ƙwaƙwalwar ƙwaƙwalwa, mai nauyi da tsayayya ga cututtukan cututtukan cututtukan ascites yana farawa - saukad da cewa yana fitowa a yankin na ciki. Haɓaka jijiyoyin varicose na esophagus halayen ne.
Waɗanne gwaje-gwaje da kuma hanyoyin bincike na taimaka wajan gano cutar?
Don gano cutar, ana amfani da hanyoyi daban-daban na gwaje-gwaje.
Da farko, ana yin gwajin jini don nazarin matakin haemoglobin a cikin sel jini da jini.
Hakanan ana yin aikin tantance baƙin ƙarfe.
Jarrabawar son zuciya ta taimaka wajan tabbatar da cutar. Don yin wannan, ana gudanar da allura ta glandular magani, kuma bayan awa biyar ana ɗaukar samfurin fitsari. Bugu da kari, CT da MRI na gabobin ciki ana yin su ne domin sanin canjin yanayinsu - karuwa da girman jiki, canza launi, da kuma canji a cikin tsarin jijiyar.
Ana bincika ƙwayar ƙwayar cuta ta jiki ta ba ka damar sanin kasancewar wani ɓangaren lalacewar kwayar halitta. Wannan binciken, wanda aka gudanar a tsakanin dangin mai haƙuri, kuma yana ba mu damar tantance yiwuwar faruwar cutar koda a farkon bayyanannun alamun asibiti wanda ke rikitar da mai haƙuri.
Ka'idojin jiyya
Babban hanyoyin maganin sune daidaituwar alamomi na abubuwan da ke cikin baƙin ƙarfe a cikin jiki da kuma hana lalacewar gabobin ciki da tsarin. Abin takaici, maganin zamani bai san yadda za a daidaita tsarin aikin kwayoyin ba.
Zubar jini
Hanyar magani daya gama gari shine zubar da jini. Tare da farawar farko, ana cire 500 MG na jini mako-mako. Bayan al'ada na baƙin ƙarfe, suna canzawa zuwa aikin kulawa, lokacin da samin jini ke faruwa kowane watanni uku.
Hakanan ana amfani da gudanar da maganin hana daukar ƙarfe. Don haka, masu kwalliyar suna ba ku damar cire abubuwa masu wucewa tare da fitsari ko feces. Koyaya, wani ɗan gajeren lokacin aiki yana sa allurar subcutaneous na yau da kullun tare da taimakon famfo na musamman.
Matsaloli da ka iya yiwuwa da kuma hangen nesa
Tare da gano asali, cutar za a iya sarrafawa yadda ya kamata.Tsawon lokaci da ingancin rayuwar marasa lafiyar da suke samun kulawa ta yau da kullun basu bambanta da na mutanen da ke da lafiya.
Haka kuma, rashin kulawa ya kan haifar da matsaloli sosai. Waɗannan sun haɗa da haɓakar cirrhosis da gazawar hanta, ciwon sukari, lalacewar jijiya har zuwa zub da jini.
Akwai babban hadarin kamuwa da cututtukan zuciya da cututtukan hanta, ana kuma lura da cututtukan cikin zuciya.
Bidiyo masu alaƙa
Game da abin da hemochromatosis yake da yadda za a bi da shi, a cikin telecast “Ku kasance lafiya!” tare da Elena Malysheva:
