UkuHlola kunye nokuPhunyezwa kwangaphambili kubalulekile
Zombili i- thalassemia enkulu kunye ne- thalassemia intermedia inokubangela okungaphezulu kwe-anemicia. Iingxaki ezinxulumene ne-thalassemia zinqunywe ngokukodwa ngobunzima belo hlobo oluthile lwe-thalassemia kunye nonyango olufunayo. Ngenxa yokuba i-thalassemia yinkinga yegazi, nayiphi na into inokuchaphazeleka.
Ukufunda ukuba usemngciphekweni weengxaki ezinkulu zezokwelapha ngenxa yakho ye-thalassemia iyangalisa.
Yazi ukuba ukunyamekela unyango rhoqo kubalulekile ekuhlolweni kwezi ngxaki kunye nokuqala unyango.
Iinguqu zeSkeletal
Ukuveliswa kwe-cell red (RBC) eyenzeka ngokuyinhloko kwintolongo yethambo. Kwimeko ye-thalassemia, lo mveliso weRBC awusebenzi. Enye indlela umzimba uzama ukuphucula imveliso kukunyusa indawo ekhoyo kwindawo yomnatha wethambo. Oku kuphawulekayo kwenzeka emathanjeni enkayi kunye nobuso. Abantu bangakwazi ukuhlakulela okubizwa ngokuba yi "thalassemic facies" -ipmunk njengezihlathi kunye nekhanda elivelele. Ukuqaliswa kwangaphambili kweyeza elingapheliyo lokuxilongwa kunokukhusela oku kungenzeki.
I-osteopenia (amathambo abuthathaka) kunye ne-osteoporosis (amathambo amancinci kunye nama-brittle) angenzeka kwiintsholongwane kunye nabantu abadala. Akuqondwa ukuba kutheni ezo zinguqu zenzeka kwi-thalassemia. I-osteoporosis inokuba yomelele ngokwaneleyo ukuba ibangele i-fractures, ingakumbi i-fracture ye-vertebral. Ukuguqulwa kwamathambo akubonakali kukuthintela le ngxaki.
Splenomegaly
I-spleen iyakwazi ukuvelisa iiseli ezibomvu zegazi (RBC); Ngokuqhelekileyo ilahleka lo msebenzi malunga nenyanga yesi-5 yokukhulelwa. Kwi-thalassemia, ukuveliswa kwe-RBC engenakwenzeka kwimnxeba wethambo kunokubangela ukuba ipenethi iqale ukuvelisa. Kwizame zokwenza oku, ipeni ikhula ngobukhulu (splenomegaly).
Lo mveliso weRBC awusebenzi kwaye awuphuculanga i-anemia. Ukuqaliswa kwangaphambili kweyeza lokumpontshelwa igazi kunokuthintela oku. Ukuba i-splenomegaly ibangela ukwanda kwevolumu yokumpontshelwa kunye / okanye ukuphindaphindiweyo, i-splenectomy (ukususwa kokutsalwa kwepen) kunokufuneka.
Gallstones
I-Thalassemia yi-anemia ye-hemolytic, echaza ukuba iiseli ezibomvu zegazi zonakaliswa ngokukhawuleza kunokuba ziveliswe. Ukutshatyalaliswa kweeseli ezibomvu zegazi kukhulula i-bilirubin, i-pigment, kwiiseli ezibomvu zegazi. Le bilirubin ngokweqile ingabangela ukuphuhliswa kwama-gallstones amaninzi. Enyanisweni, ngaphezu kwesiqingatha sabantu abane-beta thalassemia enkulu baya kuba nama-gallstones ngama-15 ubudala. Ukuba i-gallstones ibangela ubuhlungu obukhulu okanye ukuvuvukala, ukususwa kwe-gallbladder (cholecystectomy) kuyadingeka.
Iron Overload
Abantu abane-thalassemia basemngciphekweni wokuphuhlisa intsimbi , ebizwa ngokuba yi-hemochromatosis. Isinyithi esinyanisiweyo sisuka kwimithombo emibini: ukuphindwa kwegazi ebomvu ngokuphindaphindiweyo kunye / okanye ukunyuka kwe-iron kwi-foods. I-Iron overload ingabangela iingxaki ezibalulekileyo zezokwelapha entliziyweni, isibindi kunye ne-pancreas. Imithi ebizwa ngokuthi i- iron chelators ingasetyenziselwa ukususa insimbi emzimbeni.
Inkcazo yeAlastiki
Abantu abane-thalassemia (kunye nezinye i-hemolytic anemias) zidinga izinga eliphezulu lokuveliswa kweeseli zebomvu.
I-Parvovirus B19 yintsholongwane ebangela ukugula kancinci kubantwana ekuthiwa yiFifth Disease. I-Parvovirus iyathintela iiseli ezinamahlathi emathambo avimbela ukuveliswa kwe-RBC iintsuku ezi-7 ukuya kwe-10. Oku kwehla kwimveliso ye-RBC kumntu onothalamia kukukhokelela ekuphuhliseni i-anemia enkulu kwaye ngokuqhelekileyo imfuno yokumpontshelwa kwe-RBC.
Iingxaki ze-Endocrine
Ukugqithisa insimbi ngokweqile kwi-thalassemia kunokubangela ukuba isinyithi ifakwe kwiimpawu ze-endocrine ezifana ne-pancreas, thyroid kunye nezitho zesini. Intsimbi kwi-pancreas inokubangela ukuphuhliswa kwesifo sikashukela. Intsimbi kwi-thyroid ingabangela i-hypothyroidism (amanqanaba aphantsi e-hormone e-thyroid) engabangela ukukhathala, ukufumana ubunzima, ukungabi nanyameko (ukuziva kubanda xa abanye bengaboni), kunye neenwele ezinqabileyo.
Intsimbi kwizitho zesondo (i-hypogonadism) ibangela ukunciphisa i-libido kunye nokungabi namandla kumadoda kunye nokungabikho kwimizi yokuya esikhathini kubafazi.
Iintliziyo kunye neengxaki zokuLungisa
Imiba yeenhliziyo ayiqhelekanga kubantu abakhulu be-beta thalassemia. Ukwandiswa kwentliziyo kwenzeka kusenokwenzeka ebomini ngenxa yokugula kwegazi. Ngegazi elingaphantsi, intliziyo ifuna ukupompa kanzima kubangela ukwandiswa. Ulwaphulo lwe-Transfusion lunokukunceda ukukhusela oku kungenzeki. I-long term iron overload kwi-heart muscle yinkxalabo enkulu. Intsimbi entliziyweni inokubangela ukubetha kwentliziyo engaqhelekanga (arrhythmias) kunye nokuhluleka kwentliziyo. Ukuqala unyango lwe-chelation yonyango kubalulekile kubalulekile ukukhusela ubunzima bokubandezeleka kokuphila.
Nangona kungenakuqondwa ngokupheleleyo, abantu abane-thalassemia kubonakala bengengozini yokuphucula umfutho wexinzelelo lwe-pulmonary okanye uxinzelelo lwegazi oluphezulu kwimiphunga. Xa uxinzelelo lwegazi luphakanyisiwe emiphakeni, yenza kube nzima kwinhliziyo ukupompa igazi kumaphaphu, oku kunokukhokelela kwiengxaki zeentliziyo. Iimpawu ziyakwazi ukucima ukuhlola iimvavanyo kubalulekile ukwenzela ukuba unyango luqaliswe ekuqaleni.
> Imithombo:
> Benz EJ. Ukubonakaliswa kwezonyango kunye nokuxilongwa kwe-thalassemia. Ku: U-UpToDate, i-Post TW (Ed), UpToDate, i-Waltham, MA.
> ImiGangatho yeZikhokelo zoKhathalelo zeThalassemia-2012. http://thalassemia.com/SOC/index.aspx