Ukuhlolwa nokuPhelela kokuqala kuyisihluthulelo
Kokubili i- thalassemia enkulu kanye ne- thalassemia intermedia ingabangela okungaphezu nje kwe-anemia. Izinkinga ezihlobene ne-thalassemia zihlukaniswe kancane ngokubaluleka kohlobo lwakho oluthile lwe-thalassemia kanye nokwelashwa okudingayo. Ngenxa yokuthi i-thalassemia iyinkinga yegazi, noma yiluphi uhlaka lungathinteka.
Ukufunda ukuthi usemngciphekweni wezinkinga ezinkulu zezokwelapha ngenxa ye-thalassemia yakho iyabuhlungu.
Yazi ukuthi ukugcina ukunakekelwa okwelashwa okuvamile kuyisihluthulelo sokuhlola lezi zinkinga nokuqala ukwelashwa.
Izinguquko Zamahlumela
Ukukhiqizwa kwegazi elibomvu (RBC) (i-RBC) kwenzeka ngokuyinhloko emnothweni wethambo. Endabeni ye-thalassemia, lokhu kukhiqizwa kwe-RBC akusebenzi. Enye indlela umzimba ozama ngayo ukuthuthukisa ukukhiqizwa ngokukhulisa isikhala esitholakalayo emnothweni wethambo. Lokhu okuphawulekayo kwenzeka emathanjeni e-skull nobuso. Abantu bangathuthukisa okuthiwa "ama-thalassemic facies" -i-chipmunk njengezihlathi kanye nekhanda elivelele. Ukusungulwa kokuqala kokwelapheka okungapheli kungavimbela lokhu ukuthi kwenzeke.
I-osteopenia (amathambo abuthakathaka) kanye ne-osteoporosis (amathambo amancane namaphutha) angenzeka kubantu abasha kanye nabantu abadala. Akuqondwa ukuthi kungani lezi zinguquko zenzeka ku-thalassemia. I-osteoporosis ingase ibe nzima ngokwanele ukuba ibangele ukuqhuma, ikakhulukazi ukuqhuma kwama-vertebral fractures. Ukwelashwa kwe-Transfusion akubonakali ukuvimbela le nkinga.
I-Splenomegaly
I-spleen iyakwazi ukukhiqiza amaseli obomvu egazi (RBC); Ngokuvamile ulahlekelwa lo msebenzi cishe ngenyanga yesihlanu yokukhulelwa. E-thalassemia, ukukhiqizwa kwe-RBC okungasebenziyo emnothweni wamathambo kungabangela uhlangothi ukuba luqale kabusha ukukhiqizwa. Emzamweni wokwenza lokhu, isilwane sikhula usayizi (splenomegaly).
Lokhu kukhiqizwa kwe-RBC akuphumelelanga futhi akusizi ngcono i-anemia. Ukusungulwa kokuqala kokwelashwa kwegazi kungavimbela lokhu. Uma i-splenomegaly ibangela ukwanda kwevolumu yokumpontshelwa kanye / noma imvamisa, i-splenectomy (ukususwa kokuhlinzwa kwesipere) kungadingeka.
Ama-gallstones
I-Thalassemia yi-anemia ye-hemolytic, okusho ukuthi amangqamuzana egazi abomvu abhujiswa ngokushesha kakhulu kunokuthi akhiqizwe. Ukubhujiswa kwamangqamuzana egazi abomvu kukhishwa i-bilirubin, i-pigment, kusuka kumaseli abomvu egazi. Le bilirubin ngokweqile ingabangela ukuthuthukiswa kwama-gallstones amaningi. Eqinisweni, abangaphezu kwesigamu abantu abane-beta thalassemia enkulu bayoba ne-gallstones uma beneminyaka engu-15. Uma lezi zitholampilo zenza ubuhlungu obukhulu noma ukuvuvukala, kungenzeka ukuthi ukususwa kwe-gallbladder (cholecystectomy) kuyadingeka.
I-Iron Overload
Abantu abanesifo se-thalassemia basengozini yokuthuthukisa inqwaba ye- iron , ebizwa ngokuthi i-hemochromatosis. Insimbi enamandla ngokweqile ivela emithonjeni emibili: ukuphindaphindiwe kwegazi obomvu nokuphindaphindiwe kwe-iron kusuka ekudleni. Ukwehla kwe-iron kungabangela izinkinga ezibalulekile zezokwelapha enhliziyweni, esibindi, nase-pancreas. Imithi ebizwa ngokuthi i- iron chelators ingasetshenziselwa ukususa insimbi emzimbeni.
Inkinga ye-Aplastiki
Abantu abane-thalassemia (kanye namanye ama-anemia e-hemolytic) badinga izinga eliphakeme lokukhiqizwa kwegazi elisha elibomvu.
I-Parvovirus B19 igciwane elibangela ukugula okuvamile ezinganeni ezibizwa ngokuthi yiFifth Disease. I-Parvovirus ihlasela ama-stem amaseli emnothweni wamathambo avimbela ukukhiqizwa kwe-RBC izinsuku ezingu-7 kuya kwezingu-10. Lokhu kunciphisa ukukhiqizwa kwe-RBC kumuntu onesifo se-thalassemia kuholela ekuthuthukiseni i-anemia enkulu futhi ngokuvamile isidingo sokumpontshelwa kwe-RBC.
Izinkinga ze-Endocrine
Ukugqithisa ngokweqile kwensimbi ku-thalassemia kungaholela ekutheni insimbi ibekwe ezingxenyeni ze-endocrine ezifana ne-pancreas, i-thyroid, nezitho zobulili. I-iron ema-pancreas ingabangela ukuthuthukiswa kwesifo sikashukela. I-iron ekhwameni ingabangela i-hypothyroidism (amazinga e-hormone aphansi e-thyroid) angabangela ukukhathala, ukuzuza isisindo, ukubekezelelana okubandayo (ukuzwa kubanda lapho abanye bengaboni), nezinwele ezibomvu.
I-iron ezithombeni zobulili (i-hypogonadism) ibangela ukunciphisa i-libido nokungabi namandla emadodeni nokuntuleka kwemijikelezo yokuya esikhathini kwabesifazane.
Izinkinga Zengqondo Nezinhliziyo
Izinkinga zenhliziyo azivamile kumuntu omkhulu we-beta thalassemia. Ukwandiswa kwenhliziyo kwenzeka esikhathini esithile empilweni ngenxa ye-anemia. Ngegazi elingaphansi, inhliziyo ifuna ukuthemisa kanzima ukudala ukwandisa. Ukwelashwa kwe-Transfusion kungasiza ukuvimbela lokhu kungenzeki. Insimbi ende yesikhathi eside enesisindo senhliziyo iyinkinga enkulu. Insimbi enhliziyweni ingabangela ukushaya kwenhliziyo engavamile (arrhythmias) nokuhluleka kwenhliziyo. Ukuqala ukwelashwa kwe-iron chelation kusencane kubalulekile ukuvimbela lezi zinkinga zokusongela impilo.
Nakuba kungaqondakali ngokuphelele, abantu abane-thalassemia babonakala bengengozini yokuthuthukisa ukucindezeleka komfutho wegazi noma ukucindezeleka kwegazi emaphashini. Lapho umfutho wegazi ukhuphuka emaphashini, kwenza kube nzima ngenhliziyo ukupompa igazi emaphashini, okungaholela ezinkingeni zenhliziyo. Izimpawu zingaba izivivinyo zokuhlola ukucabangela ezicashile zibalulekile ukuze ukwelashwa kungaqaliswa kusenesikhathi.
> Imithombo:
> Benz EJ. Ukubonakaliswa kwemitholampilo nokuxilongwa kwe-thalassemia. Ku: Phezulu, I-post TW (Ed), UpToDate, Waltham, MA.
> Izilinganiso Zemihlahlandlela Yokunakekela Thalassemia-2012. http://thalassemia.com/SOC/index.aspx