Ingaba une-iron eninzi emzimbeni wakho, kodwa awuziva kakuhle? Ngaba kungenxa ye-sideroblastic anemia?

Ngaba uhlala uziva udiniwe kwaye uphelelwa ngumphefumlo? Ngaba ngamanye amaxesha uziva ngathi unegazi eliphantsi? Ezi zinokuba ziimpawu ze-anemia eqhelekileyo. Nangona kunjalo, ngamanye amaxesha kunokubakho isizathu esintsonkothileyo emva koku. Namhlanje siza kuthetha ngesifo segazi esingaqhelekanga, kodwa kubalulekile ukwazi ngaso. Leyo yi-sideroblastic anemia, okanye njengoko oogqirha beyibiza `(Sideroblastic Anemia - SA)`.

Ngoko ke yintoni iSideroblastic Anemia?

Ngamafutshane, le yimeko engaqhelekanga echaphazela indlela imizimba yethu eyenza ngayo iiseli ezibomvu zegazi, iiseli ezibomvu egazini lethu. Ukuba unale meko, unenani eliphantsi leeseli ezibomvu zegazi, oko kuthetha ukuba une-anemia. Kodwa kwangaxeshanye, ngokumangalisayo, une-iron eninzi. Kutheni oku kusenzeka? Ngenxa yokuba umzimba wakho awusebenzisi i-iron kufuneka wenze iiseli ezibomvu zegazi ngokufanelekileyo.

Ngubani ochaphazeleka kakhulu yile meko?

Le meko ibizwa ngokuba yi-`(Sideroblastic Anemia)` inokwenzeka ngeendlela ezimbini. Enye yi -congenital sideroblastic anemia okanye i-CSA . Oko kukuthi, abanye abantwana bazalwa benesi sifo. Enye yi-acquired sideroblastic anemia . Olu lolona hlobo luqhelekileyo. Ingabangelwa zezinye izifo ezinxulumene negazi, ukusetyenziswa kwamayeza athile, okanye ukuvezwa kakhulu kwiiminerali ezithile.

Khawucinge nje, iintsana nabantwana abancinci abazalwa benale meko ngamanye amaxesha banokuba neengxaki zempilo ezisongela ubomi ngenxa ye-iron egqithisileyo emizimbeni yabo, eyaziwa ngokuba yi-"iron overload." Ukuba abantu abadala banesifo esi, banokuba nesifo sentliziyo kunye ne-cirrhosis yesibindi.

Kukho iindaba ezimnandi! Nangona oogqirha bengenakuyinyanga ngokupheleleyo i-'(Sideroblastic Anemia) yokuzalwa, kukho amayeza anokulawula iimpawu kwaye athintele iingxaki ezinkulu. Nangona kunjalo, ezinye iintlobo ze-'(Sideroblastic Anemia)' ezikhula kamva ebomini zinokunyangwa.

Le meko, eluhlobo olufumaneka xa umntu ezalwa, inokuchaphazela iintsana, abantwana abancinci, kwanabantu abadala abancinci. Ngamanye amaxesha iimpawu ze-CSA azibalulekanga kangako. Ngoko ke nokuba uzalwe unayo, abanye abantu basenokungabi nazo naziphi na iingxaki zempilo ezinkulu de babe badala.

Uhlobo lwamva nje, olubizwa ngokuba yi-"Acquired CSA," ludla ngokuvela kubantu abanengxaki yegazi ebizwa ngokuba yi-"myelodysplastic syndrome." Lunokubangelwa kukuvezwa kwiiminerali ezifana ne-lead kunye ne-zinc, ukusetyenziswa kwamayeza athile, okanye nantoni na echaphazela amandla omzimba okusebenzisa i-copper.

Le meko ichaphazela njani umzimba wam?

Abantu abane-sideroblastic anemia badla ngokubonisa iimpawu ze-anemia. Oko kukuthi, baziva bediniwe kakhulu ngaphandle kwesizathu.Ukudinwa, ubunzima bokuphefumla, njl. Akupheleli apho, banokuba ne-"macrocytic anemia" (iiseli zegazi ezibomvu ezinkulu kuneziqhelekileyo) kunye ne-"microcytic anemia" (iiseli zegazi ezibomvu ezincinci kuneziqhelekileyo). Zombini ezi ntlobo ze-anemia zinokuba ziimpawu zezinye iintlobo ze-anemia.

Zithini izizathu ze-sideroblastic anemia?

Njengoko besitshilo ngaphambili, oku kungabakho xa umntwana ezalwa, okanye kungavela kamva ngenxa yesinye isifo, ukuchatshazelwa zizinto ezithile zesinyithi, iiminerali, okanye amayeza.

Ukuze uqonde izizathu ze-congenital sideroblastic anemia (congenital SA), kuyanceda ukwazi okuncinci malunga nendlela iiseli zethu ezibomvu zegazi ezisebenza ngayo.

Intshayelelo emfutshane ngeeseli ezibomvu zegazi

Iiseli ezibomvu zegazi zezona ziqhelekileyo kwiiseli egazini lethu. Iiseli ezibomvu zegazi eziphilileyo nezivuthiweyo zibalulekile ekuthwaleni ioksijini kwiithishu zomzimba wethu. Iiseli ezibomvu zegazi zenza oku ngoncedo lweproteni ebizwa ngokuba yihemoglobin. Ihemoglobin yiyo enika iiseli ezibomvu zegazi umbala wazo obomvu.

Iiseli ezibomvu zegazi zifuna i-iron ukuze zenze i-hemoglobin. Ngokwesiqhelo, umongo wethu wethambo uvelisa iiseli ezisisiseko. Ezi seli ezisisiseko kamva zikhula zibe ziiseli ezibomvu zegazi ezingavuthwanga ezibizwa ngokuba zii-erythrocytes. Ezi seli zingavuthwanga ziqulathe umlinganiselo ofanelekileyo we-hemoglobin kunye ne-iron efunekayo ukwenza iiseli ezibomvu zegazi eziphilileyo nezivuthiweyo.

Kodwa, ukuba une-sideroblastic anemia, umzimba wakho awunakuyisebenzisa le iron. Oku kubangela iingxaki ezimbini. Ngaphandle kwe-iron, iiseli zakho ezibomvu zegazi azenzi i-hemoglobin eyaneleyo yokuthwala ioksijini emzimbeni wakho wonke. Kwelinye icala, i-iron engasetyenziswanga iqokelelana kwi-bone marrow yakho. Oku kubangela uhlobo olungaqhelekanga lweseli olubizwa ngokuba yi-ringed sideroblast . I-sideroblast yiseli ebomvu yegazi engavuthwanga ene-iron ring ejikeleze i-nucleus yayo. Oogqirha banokuzibona ezi rings xa bejonga ii-stem cells kwi-bone marrow phantsi kwe-microscope. I-Sideroblastic anemia ifumana igama layo kwezi rings ze-iron. Ezi rings ze-iron zibonisa ukuba iiseli zigcina i-iron endaweni yokuyisebenzisa. Oku kuqokelelana kwe-iron kubizwa ngokuba yi-iron overload . Oku kugqithisa kwe-iron kunoxanduva lweengxaki ezininzi zempilo ezinokubakho ngenxa ye-sideroblastic anemia.

Ziziphi iintlobo zeCongenital Sideroblastic Anemia (CSA)?

Kukho iintlobo ezintathu ze-congenital SA. Ezi zibangelwa kukuguquka kwezakhi zofuzo ezithile ezichaphazela ukusebenza kweeseli ezibomvu zegazi. Makhe sijonge ukuba zeziphi:

• I-anemia ye-sideroblastic edityaniswe ne-X:Olu lolona hlobo luqhelekileyo lwe-anemia yokuzalwa. Ibangelwa kukuguquka kwejini okwenza i-enzyme ethile ebizwa ngokuba yi-``ALAS2```, okubangela ukunqongophala kwe-hemoglobin. Abantu abanale meko baneeseli zegazi ezibomvu ezimthubi, ezincinci kuneziqhelekileyo (microcytic). Bakwanayo ne-iron egqithisileyo emizimbeni yabo.
• I-Autosomal recessive sideroblastic anemia (ARCSA): Olu lolona hlobo lwesibini oluxhaphakileyo. I-ARCSA ichaphazela iintsana kunye nabantwana abancinci kakhulu. Yimeko enzima enokuba yingozi ebomini ngamanye amaxesha. Yenzeka xa ubungakanani bentsimbi egazini buphezulu kakhulu.
• Oomama: Malunga ne-20% yazo zonke izigulana ze-CSA ziwela kolu didi. Oku kungenxa yokuba i-gene MT-ATP6 iyatshintshatshintsha, ichaphazela ukusebenza kwe-mitochondria yakho. (I-Mitochondria zii-organelles ezingaphakathi kwiiseli zethu. Zinoxanduva lomthombo wamandla oyintloko weeseli. I-Mitochondria iguqula amandla avela ekutyeni abe yifom enokusetyenziswa ziiseli.)

Ziziphi iintlobo ze-anemia ye-sideroblastic efunyenweyo eyenzeka kamva ebomini?

Kukho iintlobo ezimbini ze-sideroblastic anemia ezikhula kamva ebomini - i-primary kunye ne-secondary. I-Primary SA ibhekisa kwi-SA enxulunyaniswa nesifo esibizwa ngokuba yi-myelodysplastic syndrome. I-Secondary SA ibhekisa kwi-SA enokuvela emva kokuchaphazeleka kakhulu kwiintsimbi ezithile, iikhemikhali kunye namayeza. Izinto ezinokubangela i-secondary sideroblastic anemia ziquka:

• Utywala: Ingxaki yokusebenzisa utywala yeyona nto iphambili ebangela i-sideroblastic anemia eqala kamva.
• Ityhefu yesinyithi esinzima: Oku kuquka ityhefu yelothe kunye netyhefu ye-arsenic.
• Ukunqongophala kweVithamin B: IVithamin B inceda ekwenzeni into ebizwa ngokuba yi-heme, eguqulwa ibe yi-hemoglobin. I-Heme yiyo enceda ekuthwaleni ioksijini.
• Ukunqongophala kweCopper: ICopper inceda ekwenzeni i-enzyme ekhusela kwi-iron egqithisileyo.
• Ukugqithisa i-zinc: Ukusela i-zinc eninzi kunokuchaphazela indlela umzimba osebenzisa ngayo i-iron kwaye kufunxe i-copper. Abanye abantu bathatha izongezo ze-zinc kwaye oku kunokwenzeka.
• Amayeza: Amanye amayeza okubulala iintsholongwane, i-chemotherapy, iihomoni, kunye namayeza anciphisa ubhedu anokubangela i-anemia yesibini ye-sideroblastic. Oogqirha banyanga olu hlobo lwe-SA ngokuvavanya amayeza owasebenzisayo kunye nokususa amayeza ekucingelwa ukuba abangela i-SA.

Zithini iimpawu ze-sideroblastic anemia?

Njengeentlobo ezininzi ze-anemia, iimpawu ze-sideroblastic anemia ziquka:

• Ukudinwa kakhulu:Oku kuthetha ukuziva udiniwe kangangokuba awukwazi ukwenza imisebenzi yemihla ngemihla.
• Ukuphelelwa ngamandla: Oku kukuziva ufuthanisekile okanye ubunzima bokuphefumla.
• Ukubetha kwentliziyo: Olu luvakalelo lokuba intliziyo ibetha ngokukhawuleza kakhulu, okanye ngendlela engaqhelekanga.
• Intloko ebuhlungu.

Abantu abane-anemia ye-sideroblastic edityaniswe ne-X banokufumana iimpawu ezongezelelweyo ngenxa yokungabikho kwe-hemoglobin eyaneleyo kunye namanqanaba aphezulu esinyithi emzimbeni. Ezi mpawu ziquka:

• Isibindi esikhulu: Isibindi sakho senza imisebenzi emininzi ebalulekileyo emzimbeni.
• I-spleen ekhulisiweyo: I-spleen yakho sisitho esilingana nenqindi ecinezelweyo esikwisisu esingaphezulu. Usenokungaqapheli ukuba i-spleen yakho inkulu kunesiqhelo, okanye unokuba neentlungu ezingathandekiyo kwisisu sakho esingaphezulu.
• Ulusu olunombala webronze: Ukuba umzimba wakho ugcina i-iron eninzi, ulusu lwakho lunokuba nombala webronze-brown.
• Isifo seswekile esingalawulekiyo.

Oogqirha bayixilonga njani i-sideroblastic anemia?

Ngenxa yokuba i-sideroblastic anemia inokubakho xa uzalwa okanye ivele kamva, ugqirha wakho uya kuqala akuvavanye ukuqinisekisa okanye akuthintele i-acquired sideroblastic anemia. Ukuba bagqiba kwelokuba ayiyo-acquired sideroblastic anemia, baya kwenza uvavanyo olongezelelekileyo ukuze bafumane ukuba loluphi uhlobo lwe-congenital sideroblastic anemia onayo. Nazi ezinye zeemvavanyo abanokuzenza:

• Ubalo lwegazi olupheleleyo (CBC): Iziphumo ze-`(CBC)` zibonisa ubungakanani kunye nokuma kweeseli egazini lakho, zingaphi iiseli zegazi ezintsha ezenziwa ngumzimba wakho, kwaye, kwimeko ye-`(SA)`, zingaphi iiseli zegazi ezibomvu ezivuthiweyo nezingekavuthwa onazo.
• I-smear yegazi engaphandle: Le yinkqubo esetyenziswa ngoogqirha ukuhlola iiseli zegazi. Nangona ezinye iimvavanyo zegazi zisebenzisa umatshini ukuzihlalutya, kule meko, oogqirha bahlalutya iiseli zegazi phantsi kwemakroskopu.
• Izifundo zesinyithi: Olu vavanyo lubonisa amanqanaba esinyithi sakho.
• Ukufunxwa komongo wethambo: Le yinkqubo yokuthatha isampuli yenxalenye yolwelo yomongo wethambo lakho.
• I-Bone Marrow Biopsy: Kolu vavanyo, oogqirha bathatha isampuli encinci yomongo wakho baze bahlole iiseli zomongo.
• Uvavanyo lwemfuza:Uvavanyo lwemfuza lunokunceda ukuqinisekisa okanye ukuthintela iimeko ezithile zemfuza. Lunokukunceda uqonde amathuba okudlulisela esi sifo kubantwana bakho.

Oogqirha banyanga njani i-sideroblastic anemia?

Oogqirha basebenzisa iindlela ezahlukeneyo zonyango lwe-anemia yokuzalwa kunye ne-acquired sideroblastic. Olu nyango luyahluka ngokuxhomekeke kwimeko yakho, ngoko ke ugqirha wakho uza kuxoxa ngonyango olungcono kuwe. Umzekelo, umntu one-X-linked SA (CSA) unokunikwa i-vitamin B6 kunye namayeza okwehlisa isinyithi. Ukuba i-SA ibangelwa yityhefu yelothe, ugqirha wakho uza kunyanga ityhefu yelothe. Kungcono ukuthetha nogqirha wakho ngale nto.

Ndingayithintela njani le nto?

I-Sideroblastic anemia idla ngokubangelwa kukuguquka kwezakhi zofuzo. Nangona olu hlobo lwe-congenital sideroblastic anemia lungenakuthintelwa, lunokunyangeka. Ukuba une-X-linked `(CSA)` kwaye iimpawu zakho zincinci, okanye ukuba umntu kusapho lwakho (abazali, abantakwenu, okanye abantwana) une-`(CSA)`, ngumbono olungileyo ukubuza ugqirha wakho malunga novavanyo lwezakhi zofuzo.

Nangona kunjalo, kukho izinto onokuzenza ukuthintela ezinye iintlobo ze-CSA ezifunyenweyo ukuba zingaphuhli kamva. La manyathelo aquka:

• Ukuphepha ityhefu yelothe ngengozi okanye i-arsenic.
• Ukuba uthatha izongezo ze-zinc kwizinto ezifana nomkhuhlane oqhelekileyo, buza ugqirha wakho malunga nomlinganiselo ofanelekileyo.
• Nciphisa ukusela kwakho utywala. Utywala bunokukhokelela kwi-sideroblastic anemia. Zama ukunciphisa utywala obusela yonke imihla. Ukuba ufuna uncedo, cela ugqirha wakho akucebise ngeenkqubo ezifanelekileyo.

Ndingalindela ntoni kule meko?

Ingqikelelo yakho ixhomekeke kuhlobo lwe-sideroblastic anemia onayo. Umzekelo, ukuba une-X-linked congenital sideroblastic anemia, ugqirha wakho unokunyanga imeko yakho nge-vitamin B6 kunye namayeza okwehlisa isinyithi. Ukuba ufumene i-sideroblastic anemia, ebangelwa yityhefu yelothe, ugqirha wakho unokunyanga ityhefu yelothe.

Ndingazinyamekela njani?

Abantu abaninzi abane-sideroblastic anemia kufuneka bahlolwe rhoqo amanqanaba abo e-iron ukuthintela ukugcwala kwe-iron ngaphezulu. Ukuba ukhe waba ne-sideroblastic anemia ngaphambili, buza ugqirha wakho ukuba ungenza ntoni ukuthintela i-SA entsha.

I-Sideroblastic anemia sisifo segazi esingaqhelekanga. Abanye abantu bayazi ukuba kutheni bene-sideroblastic anemia, kodwa abanye basenokungazi. Ukuba une-sideroblastic anemia, ungabuza ugqirha wakho le mibuzo:

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* Luhlobo luni lwe-`(SA)` endinalo?

* Kutheni ndifumene `(SA)`?

* Ziziphi iindlela zonyango endinokukhetha kuzo?

* Zithini iziphumo ebezingalindelekanga zonyango?

* Yintoni endinokuyilindela emva konyango?

* Ndingadlulisela `(SA)` ebantwaneni bam?

Ngoko ke, zeziphi izinto ezibalulekileyo ekufuneka sizikhumbule kweli bali?

I-Sideroblastic anemia sisifo segazi esingaqhelekanga kodwa esinokuba yingozi kakhulu. I-SA ibangelwa yinto ephazamisa amandla omzimba okuvelisa iiseli ezibomvu zegazi ezisempilweni. Abantu abane-SA badla ngokuba ne-iron eninzi egazini labo. Kwiimeko ezininzi, oku kugcwala kwe-iron kulula ukunyangwa. Nangona kunjalo, kwiintsana nakubantwana abancinci abazelwe ne-SA, ukugcwala kwe-iron kunokubangela iimeko zempilo ezisongela ubomi.

Khawucinge nje, ukuba umntwana wakho omncinci wazalwa ene-sideroblastic anemia, oogqirha bakhe bebeza kuqala ukunyanga le meko ngoko nangoko. Ukuba nomntwana okanye umntwana omncinci ogula kakhulu kunokuba yinto eyoyikisayo neyoyikisayo. Oogqirha bakho bayakuqonda oku kwaye baya kwenza konke okusemandleni abo ukunceda wena nomntwana wakho ngexesha nasemva konyango. Ngoko ke, ukuba wena okanye umntu kusapho lwakho unezi mpawu, kubalulekile ukubona ugqirha ngoko nangoko ukuze ufumane ingcebiso.

I- iron, i-anemia, i-sideroblasts, umongo wethambo, i-hemoglobin, iijini, izifo zegazi