Kulephera kwa hemoglobini
Kawirikawiri hemoglobin ili ndi mapuloteni anayi a mapulotini, omwe amapezeka ndi molekyu yotulutsa oxygen - heme. Pali mitundu iwiri ya ma globins - alpha ndi beta globins. Pogwirizanitsa awiriwa, amapanga molekyulu ya hemoglobin. Kupangidwa kwa mitundu yosiyanasiyana ya ma globins kuli koyenera, kotero iwo alipo mu ndalama zofanana m'magazi. Ndi thalassemia, kaphatikizidwe ka alpha-kapena betaglobini imasokonezeka, zomwe zimabweretsa chisokonezo pomanga makompyuta. Ndi kusowa kwa hemoglobini yeniyeni m'magazi omwe amachititsa kuti pakhale vuto la kuchepa kwa magazi ndi zizindikiro zina za thalassemia.
Pali mitundu iwiri ya thalassemia:
- beta-thalassemia - ikuphatikizidwa ndi kuphwanya kwa beta-chain production production;
- alpha-thalassemia - imadziwika ndi kuphwanya kufalikira kwa makina a alphabin a globin. Matenda onsewa ali ndi subtypes angapo, mosiyana kwambiri. Alfa-thalassemia mwa mitundu yonse ali ndi njira yopepuka kwambiri.
Pali mitundu itatu ya beta-thalassemia: yaing'ono thalassemia, pakati pa thalassemia ndi thalassemia yaikulu. Zizindikiro za thalassemia yazing'ono ndizochepa, koma onyamula zowonongeka za majeremusi amatha kuzifikitsa kwa ana awo mwakuya kwambiri. Matenda a beta-thalassemia amalembedwa padziko lonse lapansi, koma amapezeka ku Mediterranean komanso m'mayiko a ku Middle East. Pafupifupi 20 peresenti ya anthu a m'madera amenewa (anthu oposa 100 miliyoni) ali ndi thalassemia gene. Ndilikufalikira kwa matendawa m'mayiko a Mediterranean omwe amafotokoza dzina lake ("thalassemia" amatanthawuza kuti "Mankhwala a Mediterranean").
Mayeso a magazi
Odwala omwe ali ndi beta-thalassemia kawirikawiri amamva mwachibadwa ndipo samawoneka akudwala. Kawirikawiri, zimapezeka mwadzidzidzi kuyezetsa magazi. Kuchepetsa magazi m'thupi kumapezeka odwala. Chithunzi chochepa kwambiri cha magazi n'chofanana kwambiri ndi kuchepa kwa magazi m'thupi. Komabe, kafukufuku wowonjezera amachititsa kuti zitha kuthetsa kupezeka kwa kusowa kwa gawoli. Pa kafukufuku wowonjezereka, chiwerengero chosawerengeka cha zigawo zikuluzikulu za magazi chikuwululidwa.
Zolemba
Mwana yemwe ali ndi thalassemia yaing'ono imakula nthawi zambiri ndipo safuna chithandizo, koma nthawi zambiri amatha kutenga magazi m'thupi, mwachitsanzo, panthawi yoyembekezera kapena matenda opatsirana. Beta yaing'ono-thalassemia imakhala ndi chitetezo choletsa malungo. Izi zikhoza kufotokoza kukula kwa thalassemia ku Middle East. Matenda a beta-thalassemia amapezeka pamene mwana adzalandira zamoyo zopanda chilema zomwe zimayambitsa kugwiritsira ntchito beta-globin kuchokera kwa makolo onse awiri, ndipo thupi lake silitulutsa beta-globin muyeso yeniyeni. Pa nthawi yomweyi kaphatikizidwe ka alpha zamakono sikunathyoledwa; amapanga insoluble inclusions mu erythrocytes, chifukwa chazimene maselo a magazi ameneŵa amachepa ndi kukula ndipo amakhala otumbululuka mu mtundu. Nthawi yamoyo ya erythrocyte yotereyi ndi yochepa kuposa yachizolowezi, ndipo chitukuko cha atsopano chachepa kwambiri. Zonsezi zimayambitsa matenda ochepetsa magazi m'thupi. Komabe, mtundu uwu wamagazi, monga lamulo, suwonetseredwa mpaka usinkhu wa miyezi isanu ndi umodzi, kuyambira mu miyezi yoyamba ya moyo chomwe chimatchedwa fetal hemoglobin chimakhala m'magazi, ndipo kenako amalowetsedwa ndi hemoglobin wamba.
Zizindikiro
Mwana yemwe ali ndi thalassemia wamkulu amawoneka osagwira ntchito bwino, saganizira komanso akuthawa, akhoza kusiya pambuyo pa chitukuko. Ana otero angakhale atachepetsa chilakolako, samakhala wolemera, amayamba kuyenda mochedwa. Mwana wodwala amakhala ndi vuto la kuchepa kwa magazi m'thupi mwake limodzi ndi matenda omwe ali nawo:
- mawonekedwe a nkhope (omwe amatchedwa nkhope Mongoloid);
- chikhalidwe chimasintha m'mapfupa - chomwe chimatchedwa "fupa lachangu" chizindikiro (chingwe chopangidwa ndi singano) chomwe chimagwiritsidwa ntchito ndi fupa la hyperplasia ndizowunikira;
- kupatulira ndi kupasuka kwa mafupa.
Thalassemia ndi matenda osachiritsika. Odwala amavutika ndi zovuta zomwe zimachitika pamoyo wawo wonse. Njira zamakono zamankhwala zingapitirizire moyo wa wodwalayo. Maziko a chithandizo chachikulu cha beta-thalassemia ndi kuikidwa magazi nthaŵi zonse. Pambuyo pa matendawa, wodwalayo amapatsidwa magazi, kawirikawiri amakhala ndi masabata 4-6. Cholinga cha chithandizo chotero ndi kuwonjezera ndalama; maselo a magazi (normalization of blood formula). Mothandizidwa ndi kuikidwa magazi, kumathandiza kuti magazi apewe magazi, zomwe zimathandiza kuti mwanayo azikhala bwino komanso amalepheretsa kuti mafupa asinthe. Vuto lalikulu lomwe limagwirizanitsidwa ndi kuika magazi ambiri ndilokudya kwambiri, komwe kumawononga thupi. Chitsulo chowonjezera chikhoza kuwononga chiwindi, mtima ndi ziwalo zina.
Kuchiza kwauchidakwa
Kuti musamamwe mowa kwambiri, deferxamine yajeremusi injected injected. Kawirikawiri amapereka maola eyiti infusions deferoxamine kasanu ndi kamodzi pa sabata. Pofuna kuthamangitsa kuchotsa zitsulo zochulukirapo, kumwa mavitamini C kumalimbikitsanso. Mchitidwe umenewu ndi wolemetsa kwambiri kwa wodwala komanso okondedwa ake, kotero n'zosadabwitsa kuti odwala nthawi zambiri satsatira ndondomeko za mankhwala oyenera. Mankhwalawa amachititsa kuti odwala ali ndi beta-thalassemia, koma asachiritse. Ndi ochepa okha odwala omwe amakhala ndi zaka 20, zomwe zimawavutitsa. Ngakhale kuti akudwala chithandizo, ana omwe ali ndi thalassemia otere safika msinkhu. Kawirikawiri mawonekedwe a thalassemia ndikutambasula kwa nthata, kuphatikizapo kusonkhanitsa maselo ofiira m'magazi, kumasiya magazi, omwe amachititsa kuti magazi asapitirire kuchepa kwa magazi. Pofuna kuchiza odwala ndi thalassemia, nthawi zina amachititsa opaleshoni kuchotsa opaleshoni (splenectomy). Komabe, kwa odwala omwe adzizidwa ndi splenectomy, chiwopsezo chikuwonjezeka ku matenda a pneumococcal. Zikatero, katemera kapena moyo wonse wa antibiotic akulimbikitsidwa. Kuphatikiza pa thalassemia yaing'ono ndi yayikulu, palinso beta-thalassemia ndi alpha-thalassemia. Kufufuza kwa amayi apakati omwe ali ndi zizindikiro za thalassemia amasonyeza kuti pali mitundu yambiri ya matendawa m'mimba. Pali mtundu wachitatu wa beta-thalassemia, wotchedwa intermediate beta-thalassemia. Matendawa mwakhama ali pakati pa mawonekedwe aang'ono ndi aakulu. Odwala omwe ali ndi pakati pa thalassemia, hemoglobin m'magazi amachepa pang'ono, koma amatha kulola wodwalayo kukhala ndi moyo wamba. Odwalawa safuna kuika magazi, choncho ali pangozi yaikulu kwambiri yowonjezera yowonjezera m'thupi
Zolemba
Pali mitundu yambiri ya alpha-thalassemia. Pa nthawi yomweyi, palibe kusiyana kwakukulu mu mawonekedwe akulu ndi ang'onoang'ono. Izi zimakhalapo chifukwa cha kukhalapo kwa majini anayi osiyana omwe amachititsa kuti magulu a alpha globin apange hemoglobin.
Matenda owopsa
Kuwopsa kwa matendawa kumadalira kuti zingati zamoyo zinayi zomwe zingatheke. Ngati mliri umodzi umakhudzidwa, ndipo ena atatuwo ndi achilendo, wodwala nthawi zambiri samasonyeza kusayenerera kwakukulu m'magazi. Komabe, kugonjetsedwa kwa majini awiri kapena atatu kumapitirirabe. Monga beta-thalassemia, alpha thalassemia imapezeka m'madera omwe ali ndi malungo ambiri. Matendawa amapezeka kum'mwera chakum'mawa kwa Asia, koma ndi ochepa ku Middle East ndi Mediterranean.
Zosokoneza
Chidziwitso cha alpha-thalassemia chimachokera ku zotsatira za kuyesedwa kwa magazi. Matendawa amaphatikizidwa ndi kuchepa kwa magazi m'thupi. Mosiyana ndi beta-thalassemia, ndi alpha-thalassemia palibe kuwonjezeka kwa haloglobin HA2. Matenda akuluakulu a thalassemia angapezeke asanabadwe. Ngati, malinga ndi mbiri kapena zotsatira za kafukufuku wokonzedweratu, mkaziyo ndiye wonyamulira wa jini ya thalassemia yaing'ono, abambo amtsogolo amayang'aniranso thalassemia. Pamene jini la thalassemia laling'ono likupezeka mwa makolo onse awiri, kudziŵa molondola kungapangidwe m'miyezi itatu yoyamba ya mimba chifukwa cha magazi a fetus. Pamene mwana amapezeka ndi zizindikiro za beta-thalassemia, kuchotsa mimba kumasonyezedwa.