Matambudziko eThalassemia

Kuongorora nekupindira kwekutanga kunokosha

Zvose zviviri thalassemia chikuru uye thalassemia intermedia zvinogona kukonzera zvinopfuura kungoita ropa. Izvo zvinetso zvinosanganiswa ne thalassemia zvinonzwisiswa nechisimba chemarudzi ako chaiwo e thalassemia uye kurapwa kwaunoda. Nokuti thalassemia chirwere cheropa, chero nhengo inogona kuchinjwa.

Kudzidza kuti uri pangozi yekutambudzika kwakakomba kwehutano nekuda kwethalassemia yako inotyisa.

Ziva kuti kuchengetedza kurapwa nguva dzose ndiyo chinhu chinokosha pakuongorora maitiro aya uye kutanga chirwere chekare.

Skeletal Changes

Rudzi rutsvuku rwesero (RBC) (RBC) runowanzoitika mumapfupa emapfupa. Munyaya ye thalassemia, iri ribhinzi RBC harina maturo. Imwe nzira iyo muviri unoedza kuvandudza michina ndeyokuwedzera nzvimbo inowanikwa mumapfupa emapfupa. Izvi zvinonyanya kuzivikanwa mumapfupa ehenya nechiso. Vanhu vanogona kukura izvo zvinonzi "thalassemic facies" -chipmunk sematama uye pfumbu yakakurumbira. Kutanga kutanga kwechirwere chisingaperi chine ropa rinogona kudzivisa izvi kuti zvirege kuitika.

Osteopenia (mafupa asina simba) uye osteoporosis (mapfupa mapfupa uye ane simba) anogona kuitika kune vechidiki uye vechidiki. Hazvanzwisisi kuti nei kuchinja uku kuchiitika mu thalassemia. Iosteoporosis inogona kuva yakawanda zvakakwana kukonzera kuputsika, kunyanya kuputika kwemafupa. Kurapa kwechirwere hakuratidzi kunodzivirira kusangana uku.

Splenomegaly

Iyo spleen inokwanisa kubudisa masero matsvuku eropa (RBC); inowanzorasikirwa nebasa iri mumwedzi wechishanu wekudzivirira. MuThalassemia, kukosha kweRBC kubasa mumapfupa emapfupa kunogona kuita kuti chirwere chidzoke zvakare. Mukuedza kuita izvi, chirwere chinokura muhukuru (splenomegaly).

Izvi zvinogadzirwa neRBC hazvibudi uye hazvivandudzi anemia. Kutanga kwekutanga kwokurapa ropa kunogona kudzivirira izvi. Kana iyo splenomegaly inokonzera kuwedzerwa kwehuwandu hwekuwedzerwa ropa uye / kana nguva yakawanda, splenectomy (kubviswa kushandiswa kwepleen) kunogona kudiwa.

Gallstones

Thalassemia inonzi hemolytic anemia, zvinoreva kuti masero matsvuku eropa anoparadzwa nokukurumidza kupfuura zvaangagona kubudiswa. Kuparadzwa kwemasero matsvuku eropa kunobudisa bilirubin, pigment, kubva mumasero matsvuku eropa. Izvi zvakanyanyisa bilirubin zvinogona kukonzera kukura kwezvinyorwa zvakawanda. Zvechokwadi, inopfuura hafu yevanhu vane beta thalassemia makuru vachave nemaronda emakore nemakore gumi nemashanu. Kana mazamu aya achikonzera kurwadziwa kukuru kana kuputika, kubuda kwegorofu (cholecystectomy) inogona kudiwa.

Iron Overload

Vanhu vane thalassemia vari pangozi yekugadzira simbi yakawanda , inonziwo hemochromatosis. Simba rakawandisa rinouya kubva kumadziviri maviri: rinodzokorora ropa red cell transfusions uye / kana kuwedzerwa kushandiswa kwesimbi kubva kune zvokudya. Uremu hunorema hunogona kukonzera matambudziko makuru ezvoutano mumwoyo, chiropa, uye pancreas. Mishonga inonzi iron chelators inogona kushandiswa kubvisa simbi kubva mumuviri.

Aplastic Crisis

Vanhu vane thalassemia (pamwe chete nemamwe hemolytic anemias) vanoda huwandu hwekutsva kwemasero matsvuku ekuisira ropa.

Parvovirus B19 chirwere chinokonzera chirwere chechidiki muvana chinonzi Fifth Disease. Parvovirus inokonzera masero emadziro mumapfupa emapfupa achidzivirira RBC kukosha kwemazuva manomwe kusvika kune gumi. Izvi zvishoma nezvishoma muRBC kuburitsa kune munhu ane thalassemia inotungamirira kukurudzirwa kwehutachiwana hwekushaya ropa uye kazhinji kudiwa kweRBC kuwedzerwa.

Endocrine Matambudziko

Iyo yakawanda yesimbi yakawandisa mu thalassemia inogona kuita kuti iron iiswe muzvikamu zve endocrine zvakadai sepancreas, thyroid, uye nhengo dzepabonde. Iron in the pancreas inogona kukonzera kukura kwechirwere cheshuga mellitus. Utachi muhutachiona hunogona kukonzera hypothyroidism (inotapira mahara hormone mazinga) izvo zvingaguma nokutsamwa, kuwedzera kuwedzera, kusagadzikana kwechando (kunzwa kunotonhora kana vamwe vasingaiti), uye bvudzi rakabhururuka.

Iron inhengo dzepabonde (hypogonadism) inokonzera kuderedza libido uye kusava nesimba mune varume uye kushaya kwekuenda kumwedzi kune vakadzi.

Mwoyo uye Mhemberero Matambudziko

Nyaya dzemwoyo hazvishamisi muvanhu beta thalassemia zvikuru. Kuwedzera kwemoyo kunoitika pakutanga kwehupenyu nekuda kwekushaya ropa. Neine ropa shoma, mwoyo unoda kupomesa zvakaoma zvichiita kuti kuwedzerwa. Kurapa kwechirwere kunogona kubatsira kudzivirira izvi kuti zvirege kuitika. Nguva yakareba yesimbi yakanyanya kuwanda mukati memwoyo musungo chinhu chikuru chekuita. Indarira mumoyo inogona kukonzera kusagadzikana kwemoyo mwoyo (arrhythmias) uye mwoyo kusakona. Kutanga iron chelation therapy mangwanani-kare kunokosha pakudzivirira hupenyu huno hutambudza matambudziko.

Kunyange zvazvo isinganzwisiswi zvakakwana, vanhu vane thalassemia vanoita sevari mungozi yekuvandudza kupemha yeupemenia kana kuwedzerwa kweropa mumapapu. Apo kuwedzera kweropa kunokwidziridzwa mumapapu, zvinoita kuti zvive zvakanyanya kuoma mwoyo kuputira ropa mumapapu, izvo zvinogona kutungamirira kumatambudziko emwoyo. Zviratidzo zvinogona kuva zvinyengeri saka kuongororwa kuongororwa zvakakosha kuitira kuti kurapwa kunogona kutangwa kare.

> Sources:

> Benz EJ. Chipatara zviratidzo uye kuongororwa kwethalassemia. Mu: UpToDate, Post TW (Ed), UpToDate, Waltham, MA.

> Maitiro Ekutarisira Mirayiridzo yeThalassemia-2012. http://thalassemia.com/SOC/index.aspx