Sickle Cell Anemia

Zvemukati

• Ndezvipi zviratidzo zve sickle cell anemia?
• Ndeapi marudzi echirwere chesero masero?
• Hemoglobin SS chirwere
• Hemoglobin SC chirwere
• Hemoglobin SB + (beta) thalassemia
• Hemoglobin SB 0 (Beta-zero) thalassemia
• Hemoglobin SD, hemoglobin SE, uye hemoglobin SO
• Sickle cell hunhu
• Ndiani ari panjodzi ye sickle cell anemia?
• Ndeapi matambudziko angamuka kubva ku sickle cell anemia?
• Yakanyanya kupererwa neropa
• Ruoko-tsoka tsoka
• Splenic kudzora
• Kunonoka kukura
• Neurological matambudziko
• Matambudziko eziso
• Maronda eganda
• Chirwere chemwoyo uye chifuva chirwere
• Chirwere chemapapu
• Kukoshesa
• Matombo
• Sickle chest chirwere
• Ko sickle cell anemia inoonekwa sei?
• Yakadzama nhoroondo yemurwere
• Kuongororwa kweropa
• Hb electrophoresis
• Ko sickle cell anemia inorapwa sei?
• Kuchengetwa kwemusha
• Ndeipi mamiriro ekureba kwenguva yechirwere chesero?
• Chinyorwa zvinyorwa

Chii chinonzi sickle cell anemia?

Sickle cell anemia, kana sickle cell chirwere (SCD), chirwere chemajini chemasero matsvuku eropa (RBCs). Kazhinji, maRBCs akaumbwa semadhisiki, izvo zvinovapa mukana wekufamba nemumidziyo midiki yeropa. Zvisinei, nechirwere ichi, maRBC ane chimiro chisina kujairika chakaita sekere. Izvi zvinovaita vanonamira uye kuomarara uye vanokombamira kubatwa mumidziyo midiki, inodzivirira ropa kuti risvike munzvimbo dzakasiyana dzemuviri. Izvi zvinogona kukonzera kurwadziwa uye kukuvara kwetishu.

SCD ndeye autosomal yakawandisa mamiriro. Unoda makopi maviri ejeni kuti uve nechirwere. Kana iwe uine kopi imwe chete yejeni, iwe unonzi une jeko sero maitiro.

Ndezvipi zviratidzo zve sickle cell anemia?

Zviratidzo zve sickle cell anemia zvinowanzo kuratidza pazera diki. Vanogona kuoneka muvacheche sekutanga semwedzi mina yekuberekwa, asi kazhinji kunoitika panotenderera mwedzi we6.

Kunyange paine mhando dzakawanda dzeSCD, dzese dzine zviratidzo zvakafanana, izvo zvinosiyana mukuomarara. Izvi zvinosanganisira:

• kuneta zvakanyanya kana kutsamwa, kubva kushomeka
• kukakavara, muvacheche
• kunyorova pamubhedha, kubva kune dzakabatana zvinetso zveitsvo
• jaundice, inova yero yeziso uye ganda
• kuzvimba uye kurwadziwa mumaoko nemakumbo
• kazhinji hutachiona
• kurwadziwa mubhokisi, musana, maoko, kana makumbo

Ndeapi marudzi echirwere chesero masero?

Hemoglobin ipuroteni iri mumasero matsvuku eropa anotakura oxygen. Inowanzo ine maviri alpha ngetani uye maviri beta macheni. Mhando ina dzeakakosha yesero anemia inokonzerwa nekuchinja kwakasiyana mumajeni aya.

Hemoglobin SS chirwere

Hemoglobin SS chirwere ndiyo yakajairika mhando yechirwere chesero masero. Zvinoitika kana iwe ukatora nhaka yemakopi ehemoglobin S gene kubva kuvabereki vaviri. Izvi zvinogadzira hemoglobin inozivikanwa seHb SS. Sechimiro chakanyanya kwazvo cheSCD, vanhu vane fomu iri vanowanawo zviratidzo zvakanyanya padanho repamusoro.

Hemoglobin SC chirwere

Hemoglobin SC chirwere ndiyo yechipiri rudzi rwechirwere chesero. Zvinoitika kana iwe uchigara nhaka iyo Hb C geni kubva kune mumwe mubereki uye iyo Hb S geni kubva kune mumwe. Vanhu vane Hb SC vane zviratidzo zvakafanana kune vanhu vane Hb SS. Zvisinei, chirwere chekushaya ropa hachina kunyanya kuoma.

Hemoglobin SB + (beta) thalassemia

Hemoglobin SB + (beta) thalassemia inobata beta globin gene kugadzirwa. Ukuru hwesero tsvuku reropa hwaderedzwa nekuti shoma beta protein inogadzirwa. Kana ukagarwa nhaka neiyo Hb S geni, unenge uine hemoglobin S beta thalassemia. Zviratidzo hazvina kunyanyisa.

Hemoglobin SB 0 (Beta-zero) thalassemia

Sickle beta-zero thalassemia ndiyo yechina mhando yechirwere chesero masero. Inosanganisirawo beta globin geni. Iine zviratidzo zvakafanana neHb SS anemia. Zvisinei, dzimwe nguva zviratidzo zve beta zero thalassemia zvakanyanya kuoma. Iyo inosanganisirwa nehurombo kufungidzira.

Hemoglobin SD, hemoglobin SE, uye hemoglobin SO

Aya marudzi echirwere chesero masero anonyanya kushomeka uye kazhinji haana zviratidzo zvakakomba.

Sickle cell hunhu

Vanhu vanongogara nhaka yejeni rakachinjika (hemoglobin S) kubva kune mumwe mubereki vanonzi vane jeko masero maitiro. Vanogona kunge vasina zviratidzo kana kuderedzwa zviratidzo.

Ndiani ari panjodzi ye sickle cell anemia?

Vana vanongova panjodzi yechirwere chesero chemasero kana vabereki vese vakatakura jeko masero maitiro. Kuongororwa kweropa kunonzi hemoglobin electrophoresis kunogona zvakare kuona kuti ndeupi rudzi waungatakure.

Vanhu vanobva kumatunhu ane malaria anogara aripo vanogona kutakura. Izvi zvinosanganisira vanhu vanobva:

• Africa
• India
• kuMediterranean
• Saudhi Arabhiya

Ndeapi matambudziko angamuka kubva ku sickle cell anemia?

SCD inogona kukonzera matambudziko makukutu, ayo anoonekwa kana masero ejeko achivhara midziyo munzvimbo dzakasiyana dzemuviri. Zvinorwadza kana zvinokuvadza mabhureki anonzi makwikwi masero matambudziko. Zvinogona kukonzerwa nemamiriro ezvinhu akasiyana siyana, anosanganisira:

• urwere
• shanduko mukushisa
• kunetseka
• hydrate yakashata
• kukwirira

Izvi zvinotevera mhando dzematambudziko anogona kukonzerwa nejeko cell anemia.

Yakanyanya kupererwa neropa

Anemia kushomeka kweRBCs. Masero anorwara anotyoka zviri nyore. Uku kupatsanurwa kweRBCs kunonzi kusingaperi hemolysis. MaRBC anowanzo rarama kwemazuva angangoita zana nemakumi maviri. Masero anorwara anorarama kwemazuva anokwana gumi kusvika makumi maviri.

Ruoko-tsoka tsoka

Hand-foot syndrome inoitika kana maRBC akafanana nejeko akavhara tsinga dzeropa mumaoko kana mutsoka. Izvi zvinokonzera maoko nemakumbo kuzvimba. Inogona zvakare kukonzera maronda emakumbo. Akazvimba maoko netsoka kazhinji chiratidzo chekutanga chejeko cell anemia muvacheche.

Splenic kudzora

Splenic sequestration iko kuvhara kwemidziyo ye splenic nemasero esero. Inokonzera kuwedzera kamwe kamwe, kunorwadza kwepleen. Iyo spleen inogona kubviswa nekuda kwematambudziko echirwere chesero yesero mukuvhiya kunozivikanwa se splenectomy. Vamwe vanorwara nemasero evarwere vanochengetedza zvakakwana kukuvara kune yavo spleen iyo inova shrunken uye inomira kushanda zvachose. Izvi zvinonzi autosplenectomy. Varwere vasina spleen vari panjodzi huru yekutapukirwa kubva kubhakitiriya senge Streptococcus, Haemophilus, uye Salmonella mhando.

Kunonoka kukura

Kukura kunonoka kunowanzoitika kune vanhu vane SCD. Vana kazhinji vakapfupika asi vanowana zvakare kukwirira kwavo nekukura. Kukura pabonde kunogona zvakare kunonoka. Izvi zvinoitika nekuti jeko masero eRBC haakwanise kupa oxygen yakakwana uye zvinovaka muviri.

Neurological matambudziko

Kugwinha, kurohwa, kana kunyangwe coma zvinogona kukonzerwa nechirwere chesero. Izvo zvinokonzerwa nehuropi hwekuvhara. Kurapa kwekukurumidza kunofanirwa kutsvaga.

Matambudziko eziso

Hupofu hunokonzerwa nekuvharika mumidziyo inopa maziso. Izvi zvinogona kukuvadza iro retina.

Maronda eganda

Maronda eganda mumakumbo anogona kuitika kana midziyo midiki iripo yakavharwa.

Chirwere chemwoyo uye chifuva chirwere

Sezvo SCD ichipindira neropa reokisijeni, inogona zvakare kukonzera matambudziko emoyo ayo anogona kukonzeresa pakurwara kwemoyo, kukundikana kwemoyo, uye zvisina kujairika moyo zvitaira.

Chirwere chemapapu

Kukuvara kwemapapu nekufamba kwenguva kwakabatana nekuderera kweropa kuyerera kunogona kukonzera kukwira kweropa kwakakwira mumapapu (pulmonary hypertension) uye kupora kwemapapu (pulmonary fibrosis). Aya matambudziko anogona kuitika nekukurumidza kune varwere vane sickle chest syndrome. Kukanganiswa kwemapapu kunoita kuti zviwedzere kuoma kuti mapapu aendese okisijeni muropa, izvo zvinogona kukonzera kuwanda kwesero maseru.

Kukoshesa

Priapism inononoka, inorwadza erection iyo inogona kuoneka mune vamwe varume vane chirwere chesero rechirwere. Izvi zvinoitika kana tsinga dzeropa muurume dzakavharwa. Inogona kutungamira mukushaya simba kana ikasiiwa isina kurapwa.

Matombo

Matombo emabwe ndomumwe dambudziko risingakonzerwe nekuvharika kwemudziyo. Pane kudaro, zvinokonzerwa nekuparara kweRBCs. Chibereko chekuparara uku bilirubin. Nzvimbo dzakakwirira dzebilirubin dzinogona kutungamira kune nduru. Aya anonziwo pigment matombo.

Sickle chest chirwere

Sickle chest syndrome rudzi rwakakomba rwesero dambudziko.Inokonzera kurwadziwa kwakanyanya muchipfuva uye inosanganisirwa nezviratidzo sekukosora, kupisa muviri, kugadzirwa kwesvutugadzike, kufema nekufema, uye nemazinga akaderera eropa reokisijeni. Kusagadzikana kunoonekwa pachifuva X-rays kunogona kumiririra pneumonia kana kufa kwemapapu tishu (pulmonary infarction). Kufungidzira kwenguva refu kwevarwere vane chirwere chekachefu chirwere kwakanyanya kupfuura kune avo vasina.

Ko sickle cell anemia inoonekwa sei?

Vese vachangozvarwa muUnited States vanovhenekwa chirwere chemajeko. Kuzvarwa kwepamberi kunoongorora jeko remagenera mune yako amniotic fluid.

Muvana nevakuru, imwe kana yakawanda yeanotevera maitiro anogona zvakare kushandiswa kuongorora chirwere chesero.

Yakadzama nhoroondo yemurwere

Aya mamiriro anowanzo kutanga kuoneka sekurwadziwa kwakanyanya mumaoko nemakumbo. Varwere vanogona zvakare kuva ne:

• kurwadziwa kwakanyanya mumapfupa
• kupererwa neropa
• kuwedzera kunorwadza kwepleen
• matambudziko ekukura
• mweya wekufema
• maronda emakumbo
• matambudziko emwoyo

Chiremba wako angangoda kukuedza iwe for sickle cell anemia kana iwe uine chero zviratidzo zvataurwa pamusoro apa.

Kuongororwa kweropa

Kuongororwa kweropa kwakawanda kunogona kushandiswa kutsvaga SCD:

• Kuverenga kweropa kunogona kuburitsa huwandu hweHb husingaenzanisike mumatanho e6 kusvika ku8 magiramu padhisita imwe.
• Ropa mafirimu anogona kuratidza maRBCs anoita semasero asina kubvumirana.
• Sickle solubility bvunzo tarisa kuvapo kweHb S.

Hb electrophoresis

Hb electrophoresis inogara ichidikanwa kuti isimbise kuongororwa kwechirwere chesero. Inoyera mhando dzakasiyana dzehemoglobin muropa.

Ko sickle cell anemia inorapwa sei?

Mimwe mishonga yakasiyana-siyana inowanikwa yeSDCD:

• Kudzoreredza nemvura inopinza kunobatsira masero matsvuku eropa kuti adzokere kune yakajairika mamiriro. Masero matsvuku eropa anowanzoita deform uye anotora iyo jeko chimiro kana iwe uri kupera mvura.
• Kurapa hutachiona hwepasi kana hwakabatana chikamu chakakosha chekugadzirisa dambudziko, sezvo kushushikana kwehutachiona kunogona kukonzera dambudziko rekeresa. Utachiona hunogona zvakare kukonzeresa senge dambudziko rematambudziko.
• Kuwedzerwa ropa kunovandudza kutakura kweoxygen uye zvinovaka muviri pazvinenge zvichidikanwa. Masero matsvuku akarongedzwa anobviswa muropa rakapihwa opihwa varwere.
• Yekuwedzera okisijeni inopihwa kuburikidza nemask. Inoita kuti kufema kuve nyore uye inovandudza mazinga eoksijeni muropa.
• Mishonga yemarwadzo inoshandiswa kudzikamisa marwadzo panguva yedambudziko rejeko. Iwe ungangoda pamusoro-e-pikisi zvinodhaka kana yakasimba mishonga yekurapa yekurapa se morphine.
• (Droxia, Hydrea) inobatsira kuwedzera kugadzirwa kwe fetus hemoglobin. Zvinogona kuderedza kuwanda kweropa.
• Kubaya majekiseni ekudzivirira zvirwere kunogona kubatsira kudzivirira kutapukirwa. Varwere vanowanzove nehutachiona hwakaderera.

Bone marrow transplant yakashandiswa kurapa sickle cell anemia. Vana vari pasi pemakore gumi nematanhatu vane matambudziko akakomba uye vane unoenderana mupi ndivo vakanyanya kukwikwidza.

Kuchengetwa kwemusha

Pane zvinhu zvaunogona kuita kumba kubatsira ako masero esero zviratidzo:

• Shandisa kudziyisa mapedhi ekurwadziwa kwekurwadziwa.
• Tora folic acid zvinowedzera, sezvakakurudzirwa nachiremba wako.
• Idya huwandu hwakaringana hwemichero, miriwo, uye tsanga dzese dzegorosi. Kuita izvi kunogona kubatsira muviri wako kugadzira mamwe maRBC.
• Inwa mvura yakawanda kuti uderedze mikana yematambudziko esero.
• Rovedza muviri nguva dzose uye udzikise kushushikana kuti udzikise matambudziko, futi.
• Bata chiremba wako nekukurumidza kana iwe uchifunga kuti une chero rudzi rwehutachiona. Kurapa kwekutanga kwehutachiona kunogona kudzivirira dambudziko rakazara.

Mapoka ekutsigira anogona zvakare kukubatsira iwe kubata nemamiriro aya.

Ndeipi mamiriro ekureba kwenguva yechirwere chesero?

Kufungidzira kwechirwere kunosiyana. Vamwe varwere vane kakawanda uye inorwadza siki maseru matambudziko. Vamwe chete havawanzo kurwiswa.

Sickle cell anemia chirwere chakagara nhaka. Taura nemudzidzisi wezvechibereko kana iwe uchinetseka kuti iwe unogona kunge uri mutakuri. Izvi zvinogona kukubatsira kuti unzwisise zvinogona kurapwa, matanho ekudzivirira, uye sarudzo dzekubereka.

• Chokwadi nezve sickle cell chirwere. (2016, Mbudzi 17). Kudzorerwa kubva
• López, C., Saravia, C., Gomez, A., Hoebeke, J., & Patarroyo, M. A. (2010, Mbudzi 1) Maitiro ezvemageneti-akavakirwa nemalaria. Gene, 467(1-2), 1-12 Kudzorerwa kubva
• Vashandi veMayo Clinic. (2016, Zvita 29). Sickle cell anemia. Kudzorerwa kubva ku http://www.mayoclinic.com/health/sickle-cell-anemia/DS00324
• Sickle cell anemia. (2016, Kukadzi 1). Kudzorerwa kubva ku http://www.umm.edu/ency/article/000527.htm

Chinyorwa zvinyorwa

Ndezvipi zviratidzo nezviratidzo zvechirwere chesero? (2016, Nyamavhuvhu 2). Kudzorerwa kubva