Umlobi: Eugene Taylor
Usuku Lokudalwa: 16 Agasti 2021
Ukuvuselela Usuku: 13 Unovemba 2024
Anonim
Sickle cell anemia - causes, symptoms, diagnosis, treatment & pathology
Ividiyo: Sickle cell anemia - causes, symptoms, diagnosis, treatment & pathology

-Delile

Yini i-sickle cell anemia?

I-Sickle cell anemia, noma i-sickle cell disease (i-SCD), yisifo sofuzo samaseli abomvu egazi (RBCs). Imvamisa, ama-RBC abunjwe njengama-disc, okunikeza ukuguquguquka kokuhamba ngisho nemithambo yegazi emincane kakhulu. Kodwa-ke, ngalesi sifo, ama-RBCs anomumo wocezwana olungajwayelekile ofana nesikela. Lokhu kubenza banamathele futhi babe lukhuni futhi bathambekele ekubambekeni emithanjeni emincane, evimba igazi ekufinyeleleni ezingxenyeni ezihlukene zomzimba. Lokhu kungadala ubuhlungu kanye nokulimala kwezicubu.

I-SCD yisimo se-autosomal recessive. Udinga amakhophi amabili ofuzo ukuze ube nalesi sifo. Uma unekhophi eyodwa kuphela yofuzo, kuthiwa unesici se-sickle cell.

Ziyini izimpawu ze-sickle cell anemia?

Izimpawu ze-sickle cell anemia zivame ukuvela zisencane. Zingahle zivele ezinganeni zinezinyanga ezine ubudala, kepha ngokuvamile zenzeka lapho kunezinyanga eziyisithupha.

Ngenkathi kunezinhlobo eziningi ze-SCD, zonke zinezimpawu ezifanayo, ezihluka ngokuqina. Lokhu kufaka phakathi:


  • ukukhathala ngokweqile noma ukucasuka, kusuka ekushoneni kwegazi
  • ukuxokozela, ezinganeni
  • ukumanzisa umbhede, kusuka ezinkingeni zezinso ezihambisanayo
  • i-jaundice, ephuzi yamehlo nesikhumba
  • ukuvuvukala nobuhlungu ezandleni nasezinyaweni
  • ukutheleleka okuvamile
  • ubuhlungu esifubeni, emuva, ezingalweni, noma emilenzeni

Yiziphi izinhlobo zezifo ze-sickle cell?

IHemoglobin yiprotheni esemaseli abomvu egazi ethwala umoya-mpilo. Imvamisa inamaketanga amabili e-alpha namaketanga amabili we-beta. Izinhlobo ezine eziyinhloko ze-sickle cell anemia zibangelwa ukuguqulwa okuhlukile kulezi zofuzo.

Isifo se-Hemoglobin SS

Isifo i-Hemoglobin SS yisona sifo esijwayeleke kakhulu sesifo se-sickle cell. Kwenzeka lapho udla ifa lamakhophi wesakhi sofuzo i-hemoglobin S kubo bobabili abazali. Lokhu kwenza i-hemoglobin eyaziwa njenge-Hb SS. Njengefomu elibi kakhulu le-SCD, abantu abanaleli fomu nabo babhekana nezimpawu ezimbi kakhulu ngezinga eliphakeme.

Isifo seHemoglobin SC

Isifo seHemoglobin SC luhlobo lwesibili oluvame kakhulu lwesifo se-sickle cell. Kwenzeka lapho uthola ifa le-Hb C kumzali oyedwa kanye nohlobo lwe-Hb S komunye. Abantu abane-Hb SC banezimpawu ezifanayo kubantu abane-Hb SS. Kodwa-ke, i-anemia ayinzima kangako.


IHemoglobin SB + (beta) thalassemia

I-Hemoglobin SB + (beta) thalassemia ithinta ukukhiqizwa kofuzo lwe-beta globin. Usayizi weseli elibomvu wegazi uyancipha ngoba kwenziwa amaprotheni amancane we-beta. Uma uzuzwe ngofuzo lwe-Hb S, uzoba ne-hemoglobin S beta thalassemia. Izimpawu azinzima kangako.

IHemoglobin SB 0 (Beta-zero) thalassemia

I-Sickle beta-zero thalassemia uhlobo lwesine lwesifo se-sickle cell. Kubandakanya nohlobo lwe-beta globin. Inezimpawu ezifanayo ne-Hb SS anemia. Kodwa-ke, kwesinye isikhathi izimpawu ze-beta zero thalassemia ziba nzima kakhulu. Ihlotshaniswa nesibikezelo esimpofu.

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

Lezi zinhlobo zesifo se-sickle cell zivame kakhulu futhi imvamisa azinazo izimpawu ezinzima.

Isici sesiguli esigulayo

Abantu abazuza kuphela isakhi sofuzo esiguqukile (i-hemoglobin S) kusuka kumzali oyedwa kuthiwa banesici se-sickle cell. Kungenzeka bangabi nazimpawu noma izimpawu ezincishisiwe.

Ngubani osemngciphekweni we-sickle cell anemia?

Izingane zisengozini yesifo se-sickle cell uma bobabili abazali bephethe iseli. Ukuhlolwa kwegazi okubizwa nge-hemoglobin electrophoresis nakho kunganquma ukuthi yiluphi uhlobo ongaluthwala.


Abantu abavela ezifundeni ezinomalaleveva obusemathubeni amaningi okuba abathwali. Lokhu kufaka phakathi abantu abavela:

  • Afrika
  • India
  • iMedithera
  • I-Saudi Arabia

Yiziphi izinkinga ezingavela ku-sickle cell anemia?

I-SCD ingadala izinkinga ezinkulu, ezibonakala lapho amaseli amasikela evimba izitsha ezindaweni ezahlukahlukene zomzimba. Ukuvinjelwa okubuhlungu noma okulimazayo kubizwa ngezinkinga zeseli lesikela. Zingadalwa yizimo ezahlukahlukene, kufaka phakathi:

  • ukugula
  • izinguquko ekushiseni
  • ukucindezeleka
  • i-hydration engalungile
  • ukuphakama

Okulandelayo yizinhlobo zezinkinga ezingabangelwa yi-sickle cell anemia.

I-anemia enamandla

Ukushoda kwegazi kushoda ngama-RBC. Amangqamuzana agulayo agqashuka kalula. Lokhu kwehlukaniswa kwama-RBC kubizwa ngokuthi yi-hemolysis engapheli. Ama-RBCs ngokuvamile aphila cishe izinsuku eziyi-120. Amaseli agulayo aphila izinsuku ezingeqi kwezingu-10 kuya kwezingu-20.

Isifo sonyawo

I-hand-foot syndrome yenzeka lapho ama-RBC abunjwe ngesikela evimba imithambo yegazi ezandleni noma ezinyaweni. Lokhu kubangela ukuvuvukala kwezandla nezinyawo. Kungadala nezilonda zomlenze. Izandla nezinyawo ezivuvukile kuvame ukuba uphawu lokuqala lwe-sickle cell anemia ezinganeni.

Ukubuyiselwa kwe-Splenic

Ukwehlukaniswa kwe-Splenic ukuvimba kwemikhumbi ye-splenic ngamaseli erhengqa. Kubangela ukukhuliswa okungazelelwe, okubuhlungu kommese. Ubende kungadingeka ukuthi lususwe ngenxa yezinkinga zesifo se-sickle cell ekusebenzeni okwaziwa ngokuthi yi-splenectomy. Ezinye iziguli ze-sickle cell zizogcina umonakalo owanele ku-spleen yazo ukuthi iyancipha futhi iyeke ukusebenza nhlobo. Lokhu kubizwa nge-autosplenectomy. Iziguli ezingenayo ubende zisengozini enkulu yokutheleleka okuvela kubhaktheriya njenge I-Streptococcus, I-Haemophilus, futhi Salmonella izinhlobo.

Ukukhula okubambezelekile

Ukukhula okubambezelekile kuvame ukwenzeka kubantu abane-SCD. Izingane ngokuvamile zifushane kodwa ziphinde zikhule ukuphakama lapho sezikhulile. Ukuvuthwa ngokocansi nakho kungabambezeleka. Lokhu kwenzeka ngoba ama-RBCs amasikela erhengqa awakwazi ukunikela nge-oksijini nezakhi zomzimba ezanele.

Izinkinga zezinzwa

Ukuquleka, imivimbo, noma ngisho nokuquleka kungavela esifo se-sickle cell. Zibangelwa ukuvinjelwa kobuchopho. Kufanele kutholakale ukwelashwa ngokushesha.

Izinkinga zamehlo

Ubumpumputhe budalwa ukuvaleka ezitsheni ezinikeza amehlo. Lokhu kungalimaza i-retina.

Izilonda zesikhumba

Izilonda zesikhumba emilenzeni zingenzeka uma imikhumbi emincane lapho ivinjiwe.

Isifo senhliziyo nesifo sesifuba

Njengoba i-SCD iphazamisa ukunikezwa kwe-oksijeni yegazi, ingadala nezinkinga zenhliziyo ezingaholela ekuhlaselweni yinhliziyo, ukwehluleka kwenhliziyo, kanye nesigqi senhliziyo esingajwayelekile.

Isifo samaphaphu

Ukulimala kwamaphaphu ngokuhamba kwesikhathi okuhlobene nokwehla kokugeleza kwegazi kungadala umfutho wegazi ophezulu emaphashini (umfutho wegazi ophakeme wamaphaphu) kanye nezilonda emaphashini (pulmonary fibrosis). Lezi zinkinga zingenzeka ngokushesha ezigulini ezine-sickle chest syndrome. Ukulimala kwamaphaphu kwenza kube nzima kakhulu emaphashini ukudlulisela umoya-mpilo egazini, okungaholela ezinkingeni zeseli yesikela ejwayelekile.

Ukubheka phambili

I-Priapism yinto ehlala isikhathi eside, ebuhlungu engabonakala kwamanye amadoda anesifo se-sickle cell. Lokhu kwenzeka lapho imithambo yegazi epenis ivinjiwe. Kungadala ukungabi namandla uma kungalashwa.

Amatshe amatshe amade

Ama-Gallstones ayinkinga eyodwa engabangelwa ukuvinjelwa komkhumbi. Esikhundleni salokho, zibangelwa ukuwohloka kwama-RBC. Umkhiqizo walokhu kuchithwa yi-bilirubin. Izinga eliphakeme le-bilirubin lingaholela kuma-gallstones. Lawa abizwa nangokuthi amatshe e-pigment.

Isifo sesifuba sokugula

I-Sickle chest syndrome uhlobo olunzima lwenkinga yeseli yesikela. Kubanga ubuhlungu obukhulu besifuba futhi kuhlotshaniswa nezimpawu ezinjengokukhwehlela, umkhuhlane, ukukhiqizwa kwesikhwehlela, ukuphefumula okuncane, namazinga aphansi e-oxygen egazini. Ukungajwayelekile okubonwe esifubeni i-X-ray kungabonisa i-pneumonia noma ukufa kwezicubu zamaphaphu (i-infarction ye-pulmonary). Isibikezelo sesikhathi eside seziguli ezine-sickle chest syndrome sibi kakhulu kunalabo abangenaso.

Itholakala kanjani i-sickle cell anemia?

Zonke izingane ezisanda kuzalwa e-United States zihlolwe isifo se-sickle cell. Ukuhlolwa kokubeletha kubheka isakhi segciwane lesikela ku-amniotic fluid yakho.

Ezinganeni nakubantu abadala, eyodwa noma eziningi zalezi zinqubo ezilandelayo zingasetshenziswa futhi ukuthola isifo se-sickle cell.

Umlando wesiguli oningiliziwe

Lesi simo sivame ukuvela kuqala njengobuhlungu obukhulu ezandleni nasezinyaweni. Iziguli nazo zingaba nazo:

  • ubuhlungu obukhulu emathanjeni
  • i-anemia
  • ukwandiswa okubuhlungu kobende
  • izinkinga zokukhula
  • izifo zokuphefumula
  • izilonda zemilenze
  • izinkinga zenhliziyo

Udokotela wakho angafuna ukukuhlolela i-sickle cell anemia uma unezinye zezimpawu ezibalulwe ngenhla.

Ukuhlolwa kwegazi

Ukuhlolwa kwegazi okuningi kungasetshenziswa ukubheka i-SCD:

  • Ukubalwa kwegazi kungaveza izinga elingajwayelekile le-Hb ebangeni lama-6 kuye kwayi-8 amagremu nge-desilitha ngayinye.
  • Amafilimu egazi angakhombisa ama-RBC avela njengamaseli anenkontileka engajwayelekile.
  • Ukuhlolwa kokuncibilika kokugula kubheka ukutholakala kwe-Hb S.

Hb electrophoresis

I-Hb electrophoresis ihlala idingeka ukuqinisekisa ukutholakala kwesifo se-sickle cell. Ilinganisa izinhlobo ezahlukene ze-hemoglobin egazini.

Yelashwa kanjani i-sickle cell anemia?

Izindlela zokwelashwa ezahlukahlukene ziyatholakala nge-SCD:

  • Ukuvuselelwa komzimba ngamanzi afakwa emithanjeni kusiza amangqamuzana abomvu egazi abuyele esimweni esejwayelekile. Amaseli abomvu egazi maningi amathuba okuthi akhubazeke futhi acabange ukwakheka kwesikela uma uphelelwa ngamanzi emzimbeni.
  • Ukwelapha ukungenwa yizifo okuyisisekelo noma okuhambisana nakho kuyingxenye ebalulekile yokulawula le nkinga, ngoba ingcindezi yokutheleleka ingaholela enkingeni yamaseli erhengqa. Ukutheleleka nakho kungaholela ekubeni inkinga yenkinga.
  • Ukumpontshelwa igazi kuthuthukisa ukuthuthwa komoya-mpilo nezakhi zomzimba njengoba kudingeka.Amaseli abomvu apakishiwe ayasuswa egazini elinikelwe futhi anikezwe iziguli.
  • I-oxygen eyengeziwe inikezwa ngemaski. Kwenza ukuphefumula kube lula futhi kuthuthukise amazinga e-oxygen egazini.
  • Imithi yezinhlungu isetshenziselwa ukudambisa izinhlungu ngesikhathi senhlekelele yesikela. Ungahle udinge izidakamizwa ezithengiswa ekhawunteni noma imishanguzo enamandla yezinhlungu kadokotela efana ne-morphine.
  • (I-Droxia, i-Hydrea) isiza ukukhulisa ukukhiqizwa kwe-hemoglobin yombungu. Kunganciphisa inani lokumpontshelwa igazi.
  • Ukugonywa kungasiza ekuvikeleni izifo. Iziguli zivame ukuba nokuzivikela okuncane.

Ukufakelwa komnkantsha we-Bone kusetshenziselwe ukwelapha i-sickle cell anemia. Izingane ezingaphansi kweminyaka engu-16 ubudala ezinezinkinga ezinzima futhi ezinomnikeli ofanayo yibo abazongenela ukhetho.

Ukunakekelwa kwasekhaya

Kunezinto ongazenza ekhaya ukusiza izimpawu zeseli lakho lesikela:

  • Sebenzisa amapayipi wokufudumeza ukukhululeka kobuhlungu.
  • Thatha izengezo ze-folic acid, njengoba kunconywe ngudokotela wakho.
  • Yidla inani elanele lezithelo, imifino, nezinhlamvu zikakolweni ogcwele. Ukwenza kanjalo kungasiza umzimba wakho ukwenza ama-RBC amaningi.
  • Phuza amanzi amaningi ukunciphisa amathuba okuba nezinkinga zeseli lesikela.
  • Ukuzivocavoca umzimba njalo futhi unciphise ukucindezeleka ukunciphisa izingqinamba, futhi.
  • Xhumana nodokotela wakho ngokushesha uma ucabanga ukuthi unanoma yiluphi uhlobo lokutheleleka. Ukwelashwa kusenesikhathi kokutheleleka kungavimbela inkinga egcwele ngokugcwele.

Amaqembu okusekela angakusiza futhi ukubhekana nalesi simo.

Uyini umbono wesikhathi eside wesifo se-sickle cell?

Ukubikezelwa kwalesi sifo kuyahlukahluka. Ezinye iziguli zinezinkinga zeseli yesikela ejwayelekile futhi ebuhlungu. Abanye kuyaqabukela behlaselwa.

I-Sickle cell anemia yisifo esizuzwe njengefa. Khuluma nomeluleki wezofuzo uma ukhathazekile ngokuthi ungaba yisithwali. Lokhu kungakusiza uqonde ukwelashwa okungenzeka, izindlela zokuvimbela, kanye nezinketho zokuzala.

  • Amaqiniso ngesifo se-sickle cell. (2016, Novemba 17). Ibuyiswe kusuka ku-
  • ULópez, C., Saravia, C., Gomez, A., Hoebeke, J., & Patarroyo, M. A. (2010, Novemba 1) Izindlela zokumelana nomalaleveva okususelwa kwizakhi zofuzo. UGene, 467(1-2), 1-12 Ibuyisiwe kusuka ku-
  • Abasebenzi baseMayo Clinic. (2016, Disemba 29). I-Sickle cell anemia. Ibuyiswe kusuka ku-http: //www.mayoclinic.com/health/sickle-cell-anemia/DS00324
  • I-Sickle cell anemia. (2016, Februwari 1). Ibuyiswe kusuka ku-http: //www.umm.edu/ency/article/000527.htm

    • Imithombo ye-athikili

    Ziyini izimpawu nezimpawu zesifo se-sickle cell? (2016, Agasti 2). Ibuyiswe kusuka ku-

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