Ingabe unezinga eliphezulu lensimbi emzimbeni wakho, kodwa awuzizwa kahle? Ingabe kungaba yi-sideroblastic anemia?

Ingabe uvame ukuzizwa ukhathele futhi uphelelwa umoya? Ingabe ngezinye izikhathi uzizwa sengathi unomfutho wegazi ophansi? Lokhu kungaba izimpawu ze-anemia evamile. Kodwa-ke, ngezinye izikhathi kungaba nesizathu esiyinkimbinkimbi ngemuva kwalokhu. Namuhla sizokhuluma ngesifo segazi esingavamile kancane, kodwa okubalulekile ukwazi ngaso. Leso yi-sideroblastic anemia, noma njengoba odokotela besibiza `(Sideroblastic Anemia - SA)`.

Ngakho-ke iyini i-Sideroblastic Anemia?

Kalula nje, lesi yisimo esingavamile esithinta indlela imizimba yethu eyenza ngayo amangqamuzana abomvu egazi, amangqamuzana abomvu egazini lethu. Uma unalesi simo, unenani eliphansi lamangqamuzana abomvu egazi, okusho ukuthi une-anemia. Kodwa ngesikhathi esifanayo, ngokumangazayo, unenani elikhulu lensimbi. Kungani lokhu kwenzeka? Ngenxa yokuthi umzimba wakho awusebenzisi insimbi udinga ukwenza amangqamuzana abomvu egazi ngendlela efanele.

Ubani othinteka kakhulu yilesi simo?

Lesi simo esibizwa ngokuthi `(Sideroblastic Anemia)` singavela ngezindlela ezimbili. Esinye yi -congenital sideroblastic anemia noma i-CSA . Okusho ukuthi, ezinye izingane zizalwa nalesi sifo. Esinye yi-acquired sideroblastic anemia . Lolu uhlobo oluvame kakhulu. Lungabangelwa ezinye izifo ezihlobene negazi, ukusetshenziswa kwemithi ethile, noma ukuchayeka ngokweqile kumaminerali athile.

Cabanga nje, izinsana nezingane ezincane ezizalwa zinalesi simo ngezinye izikhathi zingaba nezinkinga zempilo ezisongela ukuphila ngenxa yensimbi eningi emizimbeni yazo, eyaziwa ngokuthi "i-iron overload." Uma abantu abadala beba nalesi sifo, bangaba nezimo ezifana nesifo senhliziyo kanye ne-cirrhosis yesibindi.

Kunezindaba ezinhle! Nakuba odokotela bengenakukwazi ukwelapha ngokuphelele isifo sofuba esizalwa naso (i-Sideroblastic Anemia), kunemithi engalawula izimpawu futhi ivimbele izinkinga ezinkulu. Kodwa-ke, ezinye izinhlobo ze-(Sideroblastic Anemia) ezikhula kamuva ekuphileni zingelapheka.

Lesi simo, okuwuhlobo olukhona lapho kuzalwa, singathinta izinsana, izingane ezincane, ngisho nabantu abadala abasebasha. Ngezinye izikhathi izimpawu ze-CSA zincane kakhulu. Ngakho-ke noma uzalwa unaso, abanye abantu bangase bangabi nezinkinga ezinkulu zempilo baze bakhule.

Uhlobo lwakamuva, olubizwa ngokuthi "i-CSA etholakale," luvame ukwenzeka kubantu abanesifo segazi esibizwa ngokuthi "i-myelodysplastic syndrome." Lungabangelwa futhi ukuchayeka kumaminerali afana ne-lead ne-zinc, ukusetshenziswa kwemithi ethile, noma yini ethinta ikhono lomzimba lokusebenzisa ithusi.

Lesi simo sithinta kanjani umzimba wami?

Abantu abane-sideroblastic anemia bavame ukubonisa izimpawu ze-anemia. Okusho ukuthi, bazizwa bekhathele kakhulu ngaphandle kwesizathu.Ukuzizwa ukhathele, unenkinga yokuphefumula, njll. Akukhona lokho kuphela, bangaba ne-"macrocytic anemia" (amangqamuzana abomvu egazi amakhulu kunokuvamile) kanye ne-"microcytic anemia" (amangqamuzana abomvu egazi amancane kunokuvamile). Zombili izinhlobo ze-anemia zingaba izimpawu zezinye izinhlobo ze-anemia.

Yiziphi izimbangela ze-sideroblastic anemia?

Njengoba sixoxile ngaphambili, lokhu kungaba yinto yokuzalwa, noma kungavela kamuva ngenxa yesinye isimo sezokwelapha, ukuchayeka ezinhlotsheni ezithile zensimbi, amaminerali, noma imithi.

Ukuze uqonde izimbangela ze-congenital sideroblastic anemia (congenital SA), kuyasiza ukwazi okuncane ngendlela amangqamuzana ethu abomvu egazi asebenza ngayo.

Isingeniso esifushane ngamaseli abomvu egazi

Amangqamuzana egazi abomvu ayilona uhlobo oluvame kakhulu lwamangqamuzana egazini lethu. Amangqamuzana egazi abomvu aphilile futhi avuthiwe abalulekile ekuthwaleni umoya-mpilo ezicutshini zomzimba wethu. Amangqamuzana egazi abomvu enza lokhu ngosizo lwephrotheni ebizwa ngokuthi i-hemoglobin. I-hemoglobin yilokho okunikeza amangqamuzana egazi abomvu umbala wawo obomvu.

Amangqamuzana egazi abomvu adinga insimbi ukuze enze i-hemoglobin. Ngokuvamile, umongo wethu wamathambo ukhiqiza amangqamuzana okuqala. Lawa maseli okuqala kamuva akhula abe amangqamuzana abomvu egazi angavuthiwe abizwa ngokuthi ama-erythrocyte. Lawa maseli angavuthiwe aqukethe inani elifanele le-hemoglobin kanye nensimbi edingekayo ukuze kwenziwe amangqamuzana abomvu egazi aphilile futhi avuthiwe.

Kodwa, uma une-anemia ye-sideroblastic, umzimba wakho awukwazi ukusebenzisa le iron. Lokhu kubangela izinkinga ezimbili. Ngaphandle kwe-iron, amangqamuzana akho abomvu egazi awakhi i-hemoglobin eyanele ukuthwala umoya-mpilo emzimbeni wakho wonke. Ngakolunye uhlangothi, i-iron engasetshenziswanga inqwabelana emnkantsheni wakho wamathambo. Lokhu kubangela uhlobo olungavamile lweseli olubizwa ngokuthi i-ringed sideroblast . I-sideroblast iyiseli legazi elibomvu elingakavuthwa elinendandatho yensimbi ezungeze i-nucleus yalo. Odokotela bangabona la macala uma bebheka amangqamuzana okuqala emnkantsheni wamathambo ngaphansi kwe-microscope. I-Sideroblastic anemia ithola igama layo kula macala ensimbi. La macala ensimbi abonisa ukuthi amangqamuzana agcina insimbi esikhundleni sokuyisebenzisa. Lokhu kuhlanganiswa kwensimbi kubizwa ngokuthi i-iron overload . Lokhu kuhlanganiswa kwensimbi overload kubangelwa izinkinga eziningi zempilo ezinkulu ezingase zenzeke ngenxa ye-sideroblastic anemia.

Yiziphi izinhlobo ze-Congenital Sideroblastic Anemia (CSA)?

Kunezinhlobo ezintathu ze-SA yokuzalwa. Lezi zibangelwa izinguquko ezakhiweni ezithile ezithinta ukusebenza kwamaseli abomvu egazi. Ake sibheke ukuthi ziyini:

• I-anemia ye-sideroblastic exhunywe ku-X:Lolu uhlobo oluvame kakhulu lwe-anemia yokuzalwa. Lubangelwa ukuguquka kwezakhi zofuzo okwenza i-enzyme ethile ebizwa ngokuthi ``ALAS2``, okubangela ukuntuleka kwe-hemoglobin. Abantu abanalesi simo banamaseli egazi abomvu akhanyayo, amancane kunejwayelekile (i-microcytic). Baphinde babe nensimbi eningi emizimbeni yabo.
• I-Autosomal recessive sideroblastic anemia (ARCSA): Lolu uhlobo lwesibili oluvame kakhulu. I-ARCSA ithinta izinsana nezingane ezincane kakhulu. Kuyisimo esibi esingaba yingozi empilweni ngezinye izikhathi. Senzeka lapho inani lensimbi egazini liphezulu kakhulu.
• Omama: Cishe u-20% wabo bonke abaguli be-CSA bawela kulesi sigaba. Lokhu kungenxa yokuthi i-gene MT-ATP6 iyashintshashintsha, ithinta ukusebenza kwe-mitochondria yakho. (I-Mitochondria yi-organelles ngaphakathi kwamaseli ethu. Yiyo enesibopho somthombo wamandla oyinhloko wamaseli. I-Mitochondria iguqula amandla avela ekudleni abe uhlobo olungasetshenziswa amaseli.)

Yiziphi izinhlobo ze-anemia ye-sideroblastic etholakala kamuva ekuphileni?

Kunezinhlobo ezimbili ze-sideroblastic anemia ezikhula kamuva empilweni - eyinhloko neyesibili. I-Primary SA ibhekisela ku-SA ehlotshaniswa nesimo esibizwa ngokuthi i-myelodysplastic syndrome. I-Secondary SA ibhekisela ku-SA engakhula ngemva kokuchayeka kakhulu ezintweni ezithile zensimbi, amakhemikhali, kanye nemithi. Izinto ezingabangela i-secondary sideroblastic anemia zifaka:

• Utshwala: Ukuphazamiseka kokusebenzisa utshwala kuyimbangela eyinhloko ye-sideroblastic anemia eqala kamuva.
• Ubuthi bensimbi enamandla: Lokhu kuhlanganisa ubuthi be-lead kanye nobuthi be-arsenic.
• Ukuntuleka kwevithamini B: Ivithamini B isiza ekwakheni isakhi esibizwa ngokuthi i-heme, esiguqulwa sibe yi-hemoglobin. I-heme yilokho okusiza ukuthwala umoya-mpilo.
• Ukuntuleka kwe-Copper: I-Copper isiza ekwakheni i-enzyme evikela i-iron eningi.
• Ukweqisa i-zinc: Ukuthatha i-zinc eningi kakhulu kungathinta indlela umzimba osebenzisa ngayo i-iron futhi kumunce ithusi. Abanye abantu bathatha izithasiselo ze-zinc futhi lokhu kungenzeka.
• Imithi: Eminye imithi elwa namagciwane, i-chemotherapy, ama-hormone, kanye nemithi eqeda ithusi ingabangela i-secondary sideroblastic anemia. Odokotela belapha lolu hlobo lwe-SA ngokuhlola imithi oyithathayo nokususa imithi okucatshangwa ukuthi ibangela i-SA.

Ziyini izimpawu ze-sideroblastic anemia?

Njengezinhlobo eziningi ze-anemia, izimpawu ze-sideroblastic anemia zifaka:

• Ukukhathala okukhulu:Lokhu kusho ukuzizwa ukhathele kakhulu kangangokuthi awukwazi ukwenza imisebenzi yansuku zonke.
• Ukuphelelwa umoya: Lokhu kuwumuzwa wokuminyana noma ubunzima bokuphefumula.
• Ukushaya kwenhliziyo: Lona umuzwa wokuthi inhliziyo ishaya ngokushesha kakhulu, noma ngendlela engavamile.
• Ikhanda elibuhlungu.

Abantu abane-anemia ye-sideroblastic exhunywe ku-X bangase babe nezimpawu ezengeziwe ngenxa yokuntuleka kwe-hemoglobin kanye namazinga ensimbi akhuphukile emzimbeni. Lezi zimpawu zifaka:

• Isibindi esikhulu: Isibindi sakho senza imisebenzi eminingi ebalulekile emzimbeni.
• Ubende obukhulu: Ubende bakho buyisitho esilingana nenqindi ecijile esitholakala esiswini esingaphezulu. Ungase ungaboni ukuthi ubende bakho bukhulu kunokuvamile, noma ungase ube nobuhlungu obungazwakali esiswini sakho esingaphezulu.
• Isikhumba esinombala wethusi: Uma umzimba wakho ugcina insimbi eningi kakhulu, isikhumba sakho singase sibe nombala onsundu wethusi.
• Isifo sikashukela esingalawulwa.

Odokotela bakuthola kanjani ukuthi i-sideroblastic anemia ibangelwa yini?

Ngenxa yokuthi i-sideroblastic anemia ingaba khona lapho uzalwa noma ivele kamuva, udokotela wakho uzoqala akuhlole ukuze aqinisekise noma akhiphe i-acquired sideroblastic anemia. Uma benquma ukuthi akuyona i-acquired sideroblastic anemia, bazokwenza izivivinyo ezengeziwe ukuthola ukuthi hlobo luni lwe-congenital sideroblastic anemia onayo. Nazi ezinye zezivivinyo abangase bazenze:

• Ukubalwa kwegazi okuphelele (i-CBC): Imiphumela ye-`(CBC)` ikhombisa usayizi nesimo samaseli egazini lakho, ukuthi mangaki amaseli egazi amasha akhiqizwa umzimba wakho, futhi, esimweni se-`(SA)`, mangaki amaseli egazi abomvu avuthiwe futhi angavuthiwe onawo.
• I-peripheral blood smear: Lena inqubo esetshenziswa odokotela ukuhlola amangqamuzana egazi. Nakuba ezinye izivivinyo zegazi zisebenzisa umshini ukuzihlaziya, kulokhu, odokotela bahlaziya amangqamuzana egazi ngaphansi kwe-microscope.
• Izifundo zensimbi: Lolu vivinyo lubonisa amazinga akho ensimbi.
• Ukufuthwa komnkantsha: Lena inqubo yokuthatha isampula yengxenye ewuketshezi yomnkantsha wakho.
• I-Bone marrow biopsy: Kulolu vivinyo, odokotela bathatha isampula encane yomnkantsha wakho bese behlola amangqamuzana omnkantsha.
• Ukuhlolwa kofuzo:Ukuhlolwa kwezakhi zofuzo kungasiza ekuqinisekiseni noma ekususeni izimo ezithile zofuzo. Kungakusiza futhi ukuthi uqonde amathuba okudlulisela lesi simo ezinganeni zakho.

Odokotela baphatha kanjani i-sideroblastic anemia?

Odokotela basebenzisa izindlela zokwelapha ezahlukene ze-anemia yokuzalwa kanye ne-accounted sideroblastic. Lezi zindlela zokwelapha ziyahlukahluka kuye ngesimo sakho, ngakho udokotela wakho uzoxoxa ngokwelashwa okungcono kakhulu kuwe. Isibonelo, umuntu one-X-linked SA (CSA) anganikwa i-vitamin B6 kanye nemithi yokwehlisa insimbi. Uma i-SA ibangelwa ubuthi be-lead, udokotela wakho uzokwelapha ubuthi be-lead. Kungcono ukukhuluma nodokotela wakho ngalokhu.

Ngingakuvimbela kanjani lokhu?

I-anemia ye-Sideroblastic ivame ukubangelwa ukuguquka kwezakhi zofuzo. Nakuba lolu hlobo lwe-anemia ye-sideroblastic yokuzalwa lungenakuvinjelwa, lungelashwa. Uma une-X-linked `(CSA)` futhi izimpawu zakho zincane, noma uma othile emndenini wakho (abazali, abafowenu nodadewenu, noma izingane) ene-`(CSA)`, kungumqondo omuhle ukubuza udokotela wakho mayelana nokuhlolwa kwezakhi zofuzo.

Kodwa-ke, kunezinto ongazenza ukuvimbela ezinye izinhlobo ze-CSA ezitholwe ukuthi zingakhuli kamuva. Lezi zinyathelo zifaka:

• Ukugwema ubuthi be-lead noma i-arsenic ngengozi.
• Uma uthatha izithasiselo ze-zinc ezintweni ezifana nomkhuhlane ovamile, buza udokotela wakho ngesilinganiso esifanele.
• Nciphisa ukuphuza kwakho utshwala. Ukuphuza utshwala ngokweqile kungaholela ekulahlekelweni yigazi okubangelwa yi-sideroblastic anemia. Zama ukunciphisa inani lotshwala oluphuzayo nsuku zonke. Uma udinga usizo, cela udokotela wakho ukuthi akuncomele izinhlelo ezifanele.

Yini engingayilindela ngalesi simo?

Ukubikezela kwakho kuncike ohlotsheni lwe-anemia ye-sideroblastic onayo. Isibonelo, uma une-anemia ye-sideroblastic yokuzalwa exhunywe ku-X, udokotela wakho angase elaphe isimo sakho nge-vitamin B6 kanye nemithi yokwehlisa insimbi. Uma une-anemia ye-sideroblastic, ebangelwa ubuthi be-lead, udokotela wakho angase elaphe ubuthi be-lead.

Ngizinakekela kanjani?

Abantu abaningi abane-sideroblastic anemia badinga ukubhekwa njalo amazinga abo e-iron ukuze kuvinjelwe ukugcwala kwe-iron okwengeziwe. Uma uke waba ne-sideroblastic anemia esikhathini esidlule, buza udokotela wakho ukuthi yini ongayenza ukuze uvimbele i-SA entsha.

I-Sideroblastic anemia iyisifo segazi esingavamile. Abanye abantu bayazi ukuthi kungani bene-sideroblastic anemia, kodwa abanye bangase bangabi nayo. Uma une-sideroblastic anemia, ungase ufune ukubuza udokotela wakho le mibuzo:

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* Hlobo luni lwe-`(SA)` enginalo?

* Kungani ngithole `(SA)`?

* Yiziphi izindlela zami zokwelapha?

* Iyini imiphumela emibi yokwelashwa?

* Yini engingayilindela ngemva kokwelashwa?

* Ngingadlulisela `(SA)` kubantwana bami?

Ngakho-ke, yiziphi izinto ezibaluleke kakhulu okudingeka sizikhumbule kule ndaba?

I-Sideroblastic anemia iyisifo segazi esingavamile kodwa esingaba yingozi. I-SA ibangelwa okuthile okuphazamisa ikhono lomzimba lokukhiqiza amangqamuzana abomvu egazi anempilo. Abantu abane-SA bavame ukuba ne-iron eningi egazini labo. Ezimweni eziningi, lokhu kugcwala kwe-iron kulula ukuyelapha. Kodwa-ke, ezinsaneni nasezinganeni ezincane ezizalwa ne-SA, ukugcwala kwe-iron kungabangela izimo zempilo ezisongela ukuphila.

Cabanga nje, uma ingane yakho encane izalwa ine-sideroblastic anemia, odokotela bayo bazoqala ukwelapha lesi simo ngokushesha. Ukuba nomntwana ogula kakhulu noma ingane encane kungaba yinto eyesabekayo kakhulu futhi ekhungathekisayo. Odokotela bakho bayakuqonda lokhu futhi bazokwenza konke okusemandleni abo ukusiza wena nomntwana wakho ngesikhathi sokwelashwa nangemva kwalokho. Ngakho-ke, uma wena noma othile emndenini wakho enalezi zimpawu, kubalulekile ukubona udokotela ngokushesha ukuze uthole iseluleko.

I - iron, i-anemia, ama-sideroblast, umnkantsha wamathambo, i-hemoglobin, izakhi zofuzo, izifo zegazi