Raunin haemoglobin
Haliglobin na al'ada ya ƙunshi sassan fannoni hudu (duniya), kowannensu yana da alaƙa da kwayoyin oxygen-transport - heme. Akwai nau'i biyu na duniya - alpha da beta globins. Haɗuwa da nau'i-nau'i, sun kafa kwayoyin hemoglobin. Samar da nau'o'in jinsunan duniya daidai ne, saboda haka suna cikin kimanin daidai da jini. Tare da thalassemia, an rusa kira na alpha- ko betaglobins, wanda zai haifar da rushewa a gina tsarin kwayar hemoglobin. Wannan rashin rashin lafiya ne a cikin jini wanda zai haifar da ciwon anemia da sauran alamun alamun cutar thalassemia.
Akwai manyan nau'o'i biyu na thalassemia:
- beta-thalassemia - an hade da cin zarafin beta-sarkar samarwa;
- alpha-thalassemia - yana haifar da cin zarafin haɗin jerin haruffa na katako. Dukkanin cututtuka suna da nau'o'in subtypes, bambanta da tsanani. Alfa-thalassemia a cikin dukkan siffofinsa yana da hanya mai yawa.
Akwai nau'o'i uku na beta-thalassemia: ƙananan ƙwayoyin cuta, matsakaiciyar magani da kuma manyan ƙwayoyin cuta. Kwayoyin cututtukan ƙananan ƙwayoyin cutar ba su da kima, amma masu ɗaukan nauyin kwayoyin halitta suna iya kawo hadari zuwa ga 'ya'yansu a cikin wata ƙari. Ana yin rajistar beta-thalassemia a duk faɗin duniya, amma ya fi kowa a cikin Rumunan da ƙasashen Gabas ta Tsakiya. Kusan kashi 20 cikin 100 na yawanin wadannan yankuna (fiye da mutane miliyan 100) suna da gurguntaccen cutar. Wannan shi ne cutar da wannan cuta a kasashen Rumunan da ke bayyana sunansa ("thalassemia" wanda aka fassara a matsayin "anemia Ruman").
Yin gwajin jini
Marasa lafiya tare da kananan beta-thalassemia yakan ji al'ada kuma basuyi rashin lafiya ba. Mafi sau da yawa, an gano shi ta hanyar haɗari a gwada jini. Ana lura da cutar anemia a cikin marasa lafiya. Hoton hoto na jini yana kama da nauyin anemia ta ƙarfe. Duk da haka, ƙarin bincike ya sa ya yiwu ya ware gaban ɓangaren wannan ɓangaren alama. A binciken da aka ƙaddamar da aka ƙaddamar da ƙarancin ma'auni mai yawa na ɓangarorin jini.
Hasashen
Yarin da ke da karamin beta-thalassemia yana tasowa kullum kuma baya buƙatar magani, amma yana iya zama predisposed zuwa anemia a gaba mai zuwa, misali, a lokacin ciki ko a lokacin cututtuka. Ƙananan beta-thalassemia suna taka muhimmiyar rawa a matsayin wani abu mai mahimmanci ga cutar malaria. Wannan na iya bayyana yadda yawancin maganin thalassemia a ƙasashen Gabas ta Tsakiya. Babban beta-thalassemia yana faruwa a lokacin da yaro ya gaji kwayoyin halitta da ke da alhakin kira na beta-globin daga iyaye biyu, jikinsa kuma ba ya samar da beta-globin a cikin adadin al'ada. A lokaci guda kira na haruffan haruffan ba karya ba; suna haifar da ƙananan haɗari a cikin erythrocytes, wanda sakamakon wannan yaduwar jini ya karu cikin girman kuma ya zama kodadde cikin launi. Yau da irin wannan erythrocytes ya kasa da saba, kuma cigaba da sababbin abubuwa yana ragewa sosai. Duk wannan yana haifar da mummunan anemia. Duk da haka, wannan nau'i na anemia, a matsayin mai mulkin, ba a nuna shi ba har sai tsawon watanni shida, tun a cikin farkon watanni na rayuwa abin da ake kira tarin hemoglobin tayi rinjaye a cikin jini, wadda aka maye gurbin baya bayan haɓakar haɓakar haɓakar jini.
Cutar cututtuka
Yarin da ke dauke da babban rubutunsa yana da rashin lafiya, yana da rashin tausayi da kodadde, yana iya bar baya a ci gaba. Irin waɗannan yara na iya rage yawan ci abinci, ba su da nauyi, sun fara yin tafiya da marigayi. Yarin da yaron yaron ya tasowa mummunan anemia tare da halayyar halayen:
- tsarin al'ada (wanda ake kira Mongoloid fuska);
- halayyar halayen kasusuwa cikin kasusuwa - alamar da ake kira "gashi skull" (tsohuwar tsintsiya mai siffa) wanda ke hade da kasusuwan kasusuwan hyperplasia shine radiyo;
- thinning da fractures na kasusuwa.
Harshen Thalassemia wani cuta ne wanda ba zai iya warkewa ba. Marasa lafiya na fama da cuta a cikin rayuwa. Hanyar zamani na jiyya na iya tsawanta rayuwar mai haƙuri. Dalili don kula da manyan beta-thalassemia shine jini na jini na yau da kullum. Bayan ganewar asali, an ba masu haƙuri karuwan jini, yawanci tsagi a cikin makonni 4-6. Dalilin wannan magani shine ƙara yawan kuɗi; Kwayoyin jini (ƙayyadewa na jini tsari). Tare da taimakon yaduwar jini, ana ba da kula da anemia, wanda ya ba da damar yaron ya ci gaba da al'ada kuma ya hana halayen kasusuwan kasusuwa. Babban matsalar da ke tattare da yaduwa da jini mai yawa shine cin nama mai yawa, wanda yana da mummunan tasiri akan jiki. Rashin ƙarfe na ƙarfe zai iya lalata hanta, zuciya da sauran gabobin.
Jiyya na maye
Don kawar da shan giya, ƙwayar magungunan ƙwayar cuta ta intravenously. Yawancin lokaci an rubuta jita-jita guda takwas a cikin mako guda. Don saurin cire cire baƙin ƙarfe, ana bada shawarar shawarar cin abinci na bitamin C.A wannan yanayin yana da wuyar gaske ga mai haƙuri da ƙaunatattunsa, don haka ba abin mamaki bane cewa marasa lafiya ba sa bi ka'idodin kulawa daidai. Irin wannan farfadowa yana ƙaruwa da rai ga marasa lafiya tare da manyan beta-thalassemia, amma ba ya warkar da shi. Kadan ƙananan yawan marasa lafiya sun tsira har zuwa shekaru 20, rashin ganewa a gare su ba shi da kyau. Duk da maganin da ke gudana, yara da irin wannan magani basu iya isa balaga. Saurin bayyanuwar thalassemia shine kara girma, tare da tara jinsin jinin jini a ciki, yana barin jinin jini, wanda ya kara yawan anemia. Don magance marasa lafiya da cutar shan magani, wasu lokuta sukan yi amfani da ƙwayar motsa jiki (splenectomy). Duk da haka, a cikin marasa lafiya wadanda suka sami lalacewa, akwai ƙwayar cuta mai tsanani ga kamuwa da cutar pneumococcal. A irin waɗannan lokuta, an riga an bada maganin alurar rigakafin ko maganin rigakafi. Baya ga kananan da manyan beta-thalassemia, akwai kuma matsakaici na beta-thalassemia da alpha-thalassemia. Yin jarrabawa ga mata masu juna biyu da alamun ƙananan ƙwayar cutar ta nuna cewa akwai mummunar cututtuka na cutar a tayin. Akwai nau'i na uku na beta-thalassemia, wanda aka sani da matsakaici na beta-thalassemia. Wannan cututtukan da ke cikin ƙananan shine tsakanin kananan da manyan siffofin. A cikin marasa lafiya tare da matsakaicin matsakaiciyar jini, matakin hemoglobin a cikin jini yana da ɗan ƙasa kaɗan, amma ya isa ya ba da damar mai haƙuri ya jagoranci rayuwa marar kyau. Wadannan marasa lafiya ba su buƙatar yaduwar jini, saboda haka suna cikin haɗari da ƙananan matsalolin da suka haɗa da ƙwayar ƙarfe a jiki
Hasashen
Akwai nau'o'in alpha-thalassemia da dama. A lokaci guda kuma, babu rarrabuwar rarraba cikin manyan ƙananan siffofin. Wannan shi ne saboda kasancewar nau'o'in jinsuna hudu da ke da alhakin kira na sarkar haɗin alpha wanda ke haɓakar hemoglobin.
Cutar cututtuka
Mawuyacin cutar ya danganta da yawancin kwayoyin halittar hudu da suke da nakasa. Idan har guda daya ya kamu, kuma sauran uku na al'ada, mai haƙuri bazai nuna rashin hasara a cikin jini ba. Duk da haka, tare da shan kashi kashi biyu ko uku na gwangwadon suna ciwo. Kamar beta-thalassemia, alpha thalassemia yana ci gaba a yankunan da ke fama da cutar malaria. Wannan cuta ta hankulan kudu maso gabashin Asia, amma yana da wuya a Gabas ta Tsakiya da Rum.
Diagnostics
Sakamakon ganewar asali na alpha-thalassemia ya dogara akan sakamakon gwajin jini. Haka kuma cutar ta kasance tare da anemia mai tsanani. Ya bambanta da beta-thalassemia, tare da alpha-thalassemia babu karuwa a hemoglobin HA2. Za a iya gano beta-thalassemia kafin haihuwa. Idan, bisa ga tarihin ko sakamakon binciken da aka shirya, mace ita ce mai ɗaukar kwayar halitta don ƙananan ƙwayoyin cuta, an kuma bincika uban gaba a matsayin likita. Lokacin da aka gano kwayar cutar ƙanana a cikin iyayen biyu, za'a iya gane cikakkiyar ganewar asali a farkon farkon shekaru uku na ciki akan jinin tayi. Lokacin da aka gano tayin tare da alamun beta-thalassemia, an nuna zubar da ciki.