Umbhali: Eugene Taylor
Umhla Wokudalwa: 16 Eyethupha 2021
Hlaziya Umhla: 5 Eyokwindla 2025
Anonim
Isifo seanemia - Zempilo
Isifo seanemia - Zempilo

Umxholo

Yintoni irhengiya yeseli?

I-Sickle cell anemia, okanye isifo se-sickle cell (SCD), sisifo semfuza seeseli ezibomvu zegazi (RBCs). Ngokwesiqhelo, ii-RBCs zimile okweediski, ezibanika ithuba lokuhamba nkqu nemithambo yegazi emincinci. Nangona kunjalo, kwesi sifo, ii-RBCs zinobume obungaqhelekanga be-crescent obufana nerhengqa. Oku kubenza babambelele kwaye babe ngqongqo kwaye bathambekele ekubambekeni kwimikhumbi emincinci, ethintela igazi ukuba lingafikeleli kwiindawo ezahlukeneyo zomzimba. Oku kunokubangela iintlungu kunye nomonakalo womzimba.

I-SCD yimeko ye-autosomal recessive. Ufuna iikopi ezimbini zemfuza ukuze ube nesi sifo. Ukuba unekopi enye yemfuza, kuthiwa uneselfowuni.

Ziintoni iimpawu zearomy cell anemia?

Iimpawu ze-sickle cell anemia zihlala zibonakalisa usemncinci. Banokubonakala kwiintsana kwinyanga ezine ubudala, kodwa ubukhulu becala zenzeka kwiinyanga ezi-6.

Nangona kukho iintlobo ezininzi ze-SCD, zonke zineempawu ezifanayo, ezahluka ngobunzima. Oku kubandakanya:


  • ukudinwa okugqithisileyo okanye ukucaphuka, ukusuka kwi-anemia
  • ukuxhamla, kwiintsana
  • ukondla umbhede, kwiingxaki ezinxulumene nezintso
  • i-jaundice, etyheli wamehlo nolusu
  • ukudumba kunye nentlungu ezandleni nasezinyaweni
  • usulelo rhoqo
  • iintlungu esifubeni, emqolo, kwiingalo, okanye emilenzeni

Zithini iintlobo zezifo zerhengqa?

IHemoglobin yiprotein ekwiiseli ezibomvu zegazi ezithwala ioksijini. Ngokwesiqhelo ineentambo ezimbini ze-alpha kunye neetyathanga ze-beta Iindidi ezine eziphambili ze-sickle cell anemia zibangelwa kukutshintsha okungafaniyo kwezi ntlobo zemfuza.

Isifo sehemoglobin SS

Isifo sehemoglobin SS sesona sifo sixhaphakileyo. Kwenzeka xa uzuza njengelifa iikopi zehemoglobin S gene kubo bobabini abazali. Ezi fom hemoglobin eyaziwa ngokuba yi-Hb SS. Njengeyona ndlela inzima kakhulu ye-SCD, abantu ngabanye kule fomu bafumana iimpawu ezimbi kakhulu kwizinga eliphezulu.

Isifo sehemoglobin SC

Isifo seHemoglobin SC sesona sifo sesibini sixhaphakileyo sesifo serhengqa. Kwenzeka xa uzuza njengelifa ufuzo lwe-Hb C ukusuka komnye umzali kunye nohlobo lwe-Hb S komnye. Abantu abane-Hb SC baneempawu ezifanayo kubantu abane-Hb SS. Nangona kunjalo, i-anemia ayinzima kangako.


IHemoglobin SB + (beta) thalassemia

IHemoglobin SB + (beta) thalassemia ichaphazela imveliso yemfuza ye-beta globin. Ubungakanani beeseli ezibomvu zegazi buyancitshiswa ngenxa yokuba kwenziwa iprotein encinci ye-beta. Ukuba uzuze njenge-Hb S gene, uya kuba ne-hemoglobin S beta thalassemia. Iimpawu azikho nzima kangako.

IHemoglobin SB 0 (Beta-zero) thalassemia

I-Sickle beta-zero thalassemia luhlobo lwesine lwesifo se-sickle cell. Ikwabandakanya uhlobo lwe-beta globin. Inempawu ezifanayo ne-Hb SS anemia. Nangona kunjalo, ngamanye amaxesha iimpawu ze-beta zero thalassemia ziba nzima kakhulu. Inxulunyaniswa nesi sifo.

I-Hemoglobin SD, i-hemoglobin SE, kunye ne-hemoglobin SO

Ezi ntlobo zezifo zerhengqa zinqabile kwaye zihlala zingenazo iimpawu ezinzima.

Uphawu lweseli lokugula

Abantu abazuza njengelifa kuphela i-gene eguqulweyo (i-hemoglobin S) evela kumzali omnye kuthiwa baneempawu zerhengqa. Basenokungabi nazimpawu okanye bancitshiswe.

Ngubani osemngciphekweni we-sickle cell anemia?

Abantwana basemngciphekweni kuphela wesifo se-sickle cell ukuba bobabini abazali baphethe iiseli zerhengqa. Uvavanyo lwegazi olubizwa ngokuba yihemoglobin electrophoresis lunokumisela ukuba loluphi uhlobo onokuthi uluphathe.


Abantu abavela kwimimandla eneengcongconi ezixhaphakileyo kunokwenzeka ukuba babe ngabathwali. Oku kubandakanya abantu abavela:

  • Afrika
  • Indiya
  • iMeditera
  • Sawudi Arabhiya

Zeziphi iingxaki ezinokuvela kwi-sickle cell anemia?

I-SCD inokubangela iingxaki ezinzima, ezibonakala xa iiseli zerheng zivimba iinqanawa kwiindawo ezahlukeneyo zomzimba. Iibhloko ezibuhlungu okanye ezonakalisayo zibizwa ngokuba ziingxaki zeseli zerhengqa. Zingabangelwa ziimeko ezahlukeneyo, kubandakanya:

  • isigulo
  • utshintsho kubushushu
  • uxinzelelo
  • i-hydration engalunganga
  • ukuphakama

Ezi zilandelayo ziindidi zeengxaki ezinokubangelwa yi-sickle cell anemia.

Anemia kakhulu

I-anemia kukusilela kwee-RBCs. Iiseli zemilwelwe zaphuka ngokulula. Oku kwahlukana kwee-RBCs kubizwa ngokuba yi-hemolysis engapheliyo. Ii-RBCs zihlala zihlala malunga neentsuku ezili-120. Iiseli ezigulayo ziphila kangangeentsuku ezili-10 ukuya kwezi-20.

Isifo seenyawo zenyawo

Isifo seenyawo zenyawo senzeka xa ii-RBC ezimile okwe rhengqa zivimba imithambo yegazi ezandleni okanye ezinyaweni. Oku kubangela ukudumba kwezandla neenyawo. Inokubangela nezilonda zomlenze. Izandla neenyawo ezidumbileyo zihlala zingumqondiso wokuqala we-sickle cell anemia kwiintsana.

Ukulandelwa kwe-Splenic

Ukutshatyalaliswa kwe-Splenic kukucinywa kweenqanawa ze-splenic ziiseli zerhengqa. Kubangela ukwandiswa ngokukhawuleza, okubuhlungu kwepeni. I-spleen inokuthi isuswe ngenxa yeengxaki zesifo se-sickle cell ekusebenzeni okubizwa ngokuba yi-splenectomy. Abanye abaguli beeseli zerheng baya kugcina umonakalo owoneleyo kwipeni yabo yokuba iye iyancipha kwaye iyeke ukusebenza kwaphela. Oku kubizwa ngokuba yi-autosplenectomy. Izigulana ezingenalo udakada zisemngciphekweni omkhulu wokosulelwa ziintsholongwane ezifana IStreptococcus, Haemophilus, kunye Salmonella iintlobo.

Ukukhula okulibazisekileyo

Ukukhula okulibazisekayo kuhlala kwenzeka kubantu abane-SCD. Ngokuqhelekileyo abantwana bafutshane kodwa babuyisele ukuphakama kwabo ebudaleni. Ukuvuthwa ngokwesondo nako kunokubambezeleka. Oku kwenzeka ngenxa yokuba ii-RBCs zeerhengqa azikwazi ukubonelela ngeoksijini eyaneleyo kunye nezondlo.

Iingxaki ze-Neurological

Ukuxhuzula, ukubetha, okanye nokudideka kunokubangelwa sisifo serhengqa. Zibangelwa ziibhlokhi zobuchopho. Unyango kwangoko kufuneka lufunwe.

Iingxaki zamehlo

Ubumfama bubangelwa kukuvaleka kwimithambo enikezela ngamehlo. Oku kungonakalisa i-retina.

Izilonda zolusu

Izilonda zolusu emilenzeni zinokubakho ukuba iinqanawa ezincinci zivaliwe.

Isifo sentliziyo kunye nesifo sesifuba

Kuba i-SCD iphazamisana nokunikezelwa kweoksijini yegazi, inokubangela iingxaki zentliziyo ezinokuthi zikhokelele ekuhlaselweni sisifo sentliziyo, ukusilela kwentliziyo, kunye nesingqisho sentliziyo esingaqhelekanga.

Isifo semiphunga

Ukonakaliswa kwemiphunga ngokuhamba kwexesha okunxulumene nokuhla kwegazi kungakhokelela kuxinzelelo lwegazi oluphezulu emiphungeni (uxinzelelo lwegazi) kunye nokunqaba kwemiphunga (pulmonary fibrosis). Ezi ngxaki zinokwenzeka kwangoko kwizigulana ezine-sickle chest syndrome. Umonakalo wemiphunga wenza ukuba kube nzima kwimiphunga ukuba igqithise ioksijini egazini, enokubangela iingxaki zeesele zerhengqa ezixhaphakileyo.

Ukubaluleka

Ukukhetha phambili kukuhlala ixesha elide, kubuhlungu ukwakheka kunokubonwa kwamanye amadoda anesifo seerhengqa. Oku kwenzeka xa imithambo yegazi kwilungu lobudoda ivaliwe. Ingakhokelela ekungafezekini ukuba ishiywe inganyangwa.

Amatye egall

Amatye egall yinto enye engabangelwa kukuvaleka kwenqanawa. Endaweni yoko, zibangelwa kukwaphuka kwee-RBCs. Imveliso yolu lwaphulo yi-bilirubin. Amanqanaba aphezulu e-bilirubin anokukhokelela kumatye enyongo. Ezi zibizwa ngokuba ngamatye e-pigment.

Isifo sesifuba sokugula

Isifo sesifuba sokugula luhlobo olunzima lwengxaki yeseli.Ibangela iintlungu zesifuba kwaye inxulunyaniswa neempawu ezinjengokukhohlela, umkhuhlane, ukuvelisa isikhohlela, ukuphefumla kancinci, kunye neqondo eliphantsi leoksijini yegazi. Ukungaqheleki okujongwa esifubeni iX-reyi kunokubonisa inyumoniya okanye ukufa kwethishu yemiphunga (pulmonary infarction). Ukuxela kwangaphambili kwexesha elide kwizigulana ezinesifo esifana nesigulo sesifuba sibi kakhulu kunabo bangenaso.

Ichongwa njani i-sickle cell anemia?

Bonke abantwana abasandul 'ukuzalwa eUnited States bavavanyelwa izifo zerhengqa. Ukuvavanywa kwangaphambi kokuzalwa kujonga iseli yemfuza yeseli kwi-amniotic fluid yakho.

Kubantwana nakubantu abadala, enye okanye ezingaphezulu kwezi nkqubo zilandelayo zinokusetyenziselwa ukufumanisa isifo se-sickle cell.

Imbali yezigulana eneenkcukacha

Le meko ihlala iqala ukubonakala njengeentlungu ezisezandleni nasezinyaweni. Izigulana zisenokuba nazo:

  • iintlungu eziqatha emathanjeni
  • ianemia
  • ukwandiswa kabuhlungu kwepeni
  • iingxaki zokukhula
  • izifo zokuphefumla
  • izilonda emilenzeni
  • iingxaki zentliziyo

Ugqirha wakho unokufuna ukuvavanya ukuba une-sickle cell anemia ukuba unayo nayiphi na impawu ekhankanywe apha ngasentla.

Uvavanyo lwegazi

Iimvavanyo ezininzi zegazi zingasetyenziselwa ukukhangela i-SCD:

  • Ubalo lwegazi lunokutyhila inqanaba le-Hb elingaqhelekanga kuluhlu lwe-6 ukuya kwi-8 yeegramu kwidesilitha nganye.
  • Iifilimu zegazi zingabonisa ii-RBCs ezibonakala njengeeseli ezingaqhelekanga.
  • Uvavanyo lokunyibilika lokugula lukhangela ubukho be-Hb S.

I-Hb electrophoresis

I-Hb electrophoresis ihlala ifuneka ukuze kuqinisekiswe ukuxilongwa kwesifo se-sickle cell. Ilinganisa iintlobo ezahlukeneyo zehemoglobin egazini.

Inyangwa njani irhengqa yeseli?

Inani leendlela ezahlukeneyo zonyango ziyafumaneka kwi-SCD:

  • Ukulungiswa kwakhona kwamanzi afakwa ngaphakathi kufakela iiseli ezibomvu zegazi ukubuyela kwimeko yesiqhelo. Iiseli ezibomvu zegazi kunokwenzeka ukuba zikhubazeke kwaye zithathe imilo yerhengqa ukuba unamanzi emzimbeni.
  • Ukunyanga usulelo olusisiseko okanye olunxulumene noko yinxalenye ebalulekileyo yokulawula ingxaki, njengoko uxinzelelo losulelo lunokubangela ingxaki yeseli erhengayo. Usulelo lunokubangela njengengxaki yengxaki.
  • Ukutofelwa igazi kuphucula ukuthuthwa kweoksijini kunye nezondlo njengoko kufuneka. Iiseli ezibomvu ezipakishiweyo ziyasuswa kwigazi elinikelweyo kwaye zinikwe abaguli.
  • Ioksijini eyongezelelweyo inikwa ngemaski. Yenza ukuphefumla kube lula kwaye kuphucule amanqanaba eoksijini egazini.
  • Amayeza eentlungu asetyenziselwa ukuphelisa iintlungu ngexesha lokurhoxa. Unokufuna iziyobisi ezingaphaya kwekhawuntareni okanye amayeza anamandla amayeza eentlungu afana ne-morphine.
  • (Droxia, Hydrea) iyanceda ukonyusa imveliso yehemoglobin engekazalwa. Inokunciphisa inani lotofelo-gazi.
  • Ugonyo lunokunceda ukuthintela usulelo. Izigulana zihlala zinomkhuhlane omncinci.

Ukufakelwa komongo wethambo kuye kwasetyenziselwa ukunyanga i-sickle cell anemia. Abantwana abangaphantsi kweminyaka eli-16 ubudala abaneengxaki ezinzima kwaye banomxhasi ofanayo ngabona bagqatswa bangcono.

Unonophelo lwasekhaya

Kukho izinto onokuzenza ekhaya ukunceda iimpawu zeseli yakho irhengqa:

  • Sebenzisa iiphedi zokufudumeza iintlungu.
  • Thatha izongezo ze-folic acid, njengoko ugqirha wakho ekucebisa.
  • Yitya izixa ezaneleyo, imifuno, kunye neenkozo zengqolowa. Ukwenza njalo kunokunceda umzimba wakho wenze ii-RBCs ezingaphezulu.
  • Sela amanzi amaninzi ukunciphisa amathuba eengxaki zeseli zerhengqa.
  • Ukuzivocavoca rhoqo kunye nokunciphisa uxinzelelo ukunciphisa iingxaki, nazo.
  • Nxibelelana nogqirha wakho ngokukhawuleza ukuba ucinga ukuba unayo nayiphi na intsholongwane. Unyango lwakwangoko losulelo lunokuthintela ingxaki egcweleyo.

Amaqela enkxaso anokukunceda ujongane nale meko.

Liyintoni imbonakalo yexesha elide yesifo serhengqa?

Ukuxelwa kwesi sifo kuyahluka. Abanye abaguli baneengxaki zeseli zerhengqa ezibuhlungu rhoqo. Abanye kunqabile ukuba babe nohlaselo.

I-Sickle cell anemia sisifo esizuzwe njengelifa. Thetha nomcebisi ngemfuzo ukuba ukhathazekile ukuba ungangumphathi. Oku kunokukunceda uqonde unyango olunokwenzeka, amanyathelo othintelo, kunye nokuzala.

  • Iinyani malunga nesifo serhengqa. (2016, Novemba 17). Kubuyiselwa kwi
  • López, C., Saravia, C., Gomez, A., Hoebeke, J., & Patarroyo, M. A. (2010, Novemba 1) Iindlela zokumelana nemfuza esekwe kwimfuza. Gene, 467(1-2), 1-12 Ibuyisiwe kwi
  • Abasebenzi bekliniki yaseMayo. (2016, Disemba 29). Isifo seanemia. Ukufunyanwa kwi-http: //www.mayoclinic.com/health/sickle-cell-anemia/DS00324
  • Isifo seanemia. (Ngo-2016, ngoFebruwari 1). Ukufunyanwa kwi-http: //www.umm.edu/ency/article/000527.htm

    • Imithombo yenqaku

    Zeziphi iimpawu zesifo serhengqa? (2016, Agasti 2). Kubuyiselwa kwi

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