Ukuhlaziywa kwe-Non-Transfusion exhomekeke kwiThalassemia
I-Thalassemia yiqela leengxaki ezichaphazela i-hemoglobin, iprotheni, ngaphakathi kweeseli zegazi ezimnyama (RBC). Abantu abadla ilifa le-thalassemia abakwazi ukuvelisa i-hemoglobin ngokuqhelekileyo ekukhokelela ku-anemia (ubuncinane be-RBC count) kunye nezinye iingxaki.
I-Thalassemia ingaphulwa kwiindidi ezintathu ezinkulu:
- Umxholo: Umntu uphatha umthamo wokuguquka kodwa akanalo isifo. Ngamanye amaxesha kuthiwa yi-thalassemia encinci.
- I-Intermedia: Umntu onethalamia kwenye indawo phakathi kwendlela kunye neyona nto.
- Mkhulu: Umntu onomdla we-thalassemia ofuna i-RBC igazi.
I-Thalassemia Intermedia ichongwa njani?
Nangona, ininzi yexesha elikhulu le-thalassemia liya kuchongwa kwiskrini esandula kusana , abantu abane-thalassemia intermedia abanako ukuchongwa de kube yiminyaka kamva. Laba bantu baqatshelwa ngokubanzi kwibala elipheleleyo legazi (CBC). I-CBC iya kubonakalisa i-anemia elula kunye neyomodareyitha kunye neeseli ezincinci zegazi ezibomvu. Oku kungadideka ngentswelo ye-anemia.
Ukuxilongwa kuqinisekiswa yiprofayili ye-hemoglobin (ebizwa ngokuba yi-electrophoresis). Kwi-beta thalassemia intermedia kunye nomxholo lo vavanyo lubonisa ukuphakama kwe-hemoglobin A2 (ifom ye-2 ye-hemoglobin yabantu abadala) ngamanye amaxesha i-F (fetal). I-Alpha thalassemia intermedia ngokuqhelekileyo ibizwa ngokuba yi-hemoglobin H njengesifo esiyi-hemoglobin ebonakalayo kwiprofayili.
Ziziphi iingxaki zeThalassemia Intermedia?
Iingxaki ze-thalassemia intermedia ziquka:
- Ukugqithiswa kweensimbi
- I-Osteoporosis: Ukuphuphuma kwamathambo
- I-hematopoiesis ye-Extramedullary: Oku kubhekisela ekwandiseni i-spleen, isibindi kunye / okanye umongo weethambo ukwenzela ukwandisa ukuveliswa kweeseli zegazi. Amathambo amaninzi achaphazelekayo asemakhanda - ebunzini nasemathambo amathambo.
- I-Hypogonadism: Ukuphuculwa kwemveliso yezitho zesini. Oku kunokuthintela ukukhulelwa komntwana kungenzeki ngokwemvelo.
- Gallstones:
- Amacwecwe egazi
Kutheni abantu Abantu abaneThalassemia Intermedia bezama ukukhupha i-Iron?
Kukho izizathu ezibini ukuba kutheni abantu abane-thalassemia phakathi kwabo bahlakulela i-iron overload.
- Phinda ubethelwe igazi elibomvu: Nangona abantwana abane-thalassemia phakathi kweemfuno abaqhelekanga ukuxilongwa nganye kwiiveki ezi-3 ukuya kwe-4 ezifana nezingane ezine- thalassemia eziphambili , zisenokufuna igazi eliqela rhoqo ngonyaka. Igazi ngalinye elibomvu elifunyenweyo lifana ne-intravenous (IV) idosi yesinyithi. Umzimba awunayo indlela enkulu yokususa le ntsimbi emzimbeni. Ngoko ngokugqithisa kwexesha ukuxilongwa ngokuphindaphindiweyo kunokubangela ukuphuhliswa kwengqimba yesinyithi, nangona ngokubanzi kamva ebomini (abadala) kunabantu abane-thalassemia enkulu (ebuntwaneni).
- Ukwandiswa kwentsimbi ekudleni: Umzimba uyaqaphela ukuba umongo wethambo awusebenzi umsebenzi ovelisa i-hemoglobin kunye neeseli ezibomvu zegazi. I-Hepcidin yiprothini evimbela ukutsalwa kwesinyithi. Kwi-thalassemia, amazinga e-hepcidin aphantsi avumela ukuba insimbi eninzi ithathwe ngaphezu kokufunekayo. Kunconywa ukuba abantu abane-thalassemia intermedia balandele ukutya okunesitya esincinci basebenzise itiye kunye nokutya njengemibhobho yetiyi ukutyunjwa kwensimbi.
Ziziphi iindlela zokwelapha kwiThalassemia Intermedia?
- Unonophelo lwangonyango oluqhelekileyo: Akunjalo wonke umntu ophakathi ne-thalassemia intermedia uya kufuna unyango, kodwa kubalulekile ukuqhubeka nokunyamekela unyango lwezonyango ukujongana neengxaki.
- Ukutshintshiselwa: Abantu abane-thalassemia intermedia bangadinga ukumpontshelwa, kodwa ngokuqhelekileyo abahlali rhoqo njengoko abantu abane-thalassemia enkulu bade badala. Ukutshatyalaliswa kungadinga ngexesha lokukhula okukhulayo kunye nophuhliso (ukukhulelwa), izifo ikakhulukazi ezinomkhuhlane, ukukhulelwa, okanye ukulungiselela ukuhlinzwa.
- I- aclic Folic: Abanye amagqirha banokuncoma i-folic acid imihla ngemihla ukuxhasa ukuveliswa kweRBC.
- I-Splenectomy: Kwi-thalassemia, i-spleen inokwandisa (i-splenomegaly) ngenzame yokuphucula ukuveliswa kwe-RBC. Oku kudla ngokungafezeki kwaye kungabangela ukonakalisa i-anemia kunye / okanye ukwanda kweemfuno zokumpontshelwa. Kule meko, u-splenectomy unokuqwalaselwa.
- I-Hydroxyurea: I-Hydroxyurea yimithi yansuku zonke eyenziwa ngomlomo ekuzameni ukwandisa i-hemoglobin, ngaloo ndlela iyanciphisa imfuneko yokumpontshelwa kwe-RBC.
- Ukutya kwesinyithi esiphantsi: Ngenxa yokuba abantu abane-thalassemia intermedia basemngciphekweni wokwenza i-iron overload ukusuka ekunyuseni kwe-iron ekunyuseni, kunokunconywa ukutya okunesitya. I-Tea, ingakumbi itiye emnyama, inciphisa ukutyunjwa kwesinyithi kwaye iyakunconywa ngokutya.
Ukufunda ukuba une-thalassemia intermedia inokutshatyalaza njengoko ungenayo nayiphi na impawu. Qinisekisa ukuba ulandele ugqirha wakho njengeshedyuli ukwenzela ukuba unokubekwa iliso malunga neengxaki ezinokwenzeka.
> Imithombo:
> Benz EJ. I-molecular pathology ye-thalassemia syndromes, ukubonakaliswa kwezonyango kunye nokuxilongwa kwe-thalassemia, kunye noTyango lwe-beta thalassemia. Ku: U-UpToDate, i-Post TW (Ed), UpToDate, i-Waltham, MA.