Thalassemias iboka rezvirwere zveropa zvinogara nhaka zvinokanganisa kugadzirwa kwehemoglobin (protein inotakura okisijeni). Dzinosiyana mukuomarara: zvimwe zvinokonzeresa zviratidzo nepo zvimwe zvichityisidzira hupenyu. Kugadziriswa kwepfupa kunofungidzirwa mumatambudziko akakomba.

Tsanangudzo yethalassemia

Thalassemia inoratidzwa nekukanganisa mukugadzirwa kwehemoglobin. Sechiyeuchidzo, hemoglobin ipuroteni hombe iripo mumasero matsvuku eropa (masero matsvuku eropa) ane basa rekuona kutakurwa kwe dixoygene kubva kuhurongwa hwekufema kuenda kumuviri wese.

Zvinonzi thalassemia chirwere cheropa. Basa rekutakura remasero matsvuku eropa rinokanganisa, izvo zvinogona kuva nemigumisiro yakakomba pamuviri. Panguva ino, zvakakosha kuziva kuti kune akati wandei marudzi ethalassemia asina hunhu hwakafanana kana chiyero chakafanana chekuomarara. Vamwe havana zviratidzo nepo vamwe vachiisa upenyu panjodzi.

Zvinokonzera thalassemia

Thalassemias chirwere chemajini. Izvo zvinokonzerwa nekushandurwa kwejini rimwe chete kana kupfuura rinobatanidzwa mukugadzirwa kwehemoglobin, uye zvakanyanya kunyanya kuchinjwa kwemajini anobatanidzwa mukugadzirwa kwehemoglobin protein chain. Kune zvina zveizvi: maviri alpha cheni uye maviri beta cheni.

Imwe neimwe yemaketani aya inogona kukanganiswa muthalassemia. Tinogona zvakare kusiyanisa:

• alpha-thalassemias inoratidzirwa nekushandurwa kweketani yealpha;
• beta-thalassemias inoratidzirwa nekushandurwa kweketani yebeta.

Kuoma kwealpha thalassemias uye beta thalassemias kunoenderana nehuwandu hwemajini akachinjwa. Iyo inonyanya kukosha, iyo yakakura chiyero chekuomarara.

Kuongororwa kwethalassemia

Kuongororwa kwethalassemia kunoitwa nekuongororwa ropa. Kuwanda kweropa kwakakwana kunoita kuti zvikwanise kuongorora chitarisiko uye nhamba yemasero matsvuku eropa, uye nokudaro kuziva huwandu hwehuwandu hwe hemoglobin. Ongororo yebiochemical yehemoglobin inoita kuti zvikwanise kusiyanisa alpha-thalassemias kubva beta-thalassemias. Pakupedzisira, kuongororwa kwemajini kunoita kuti zvikwanise kuongorora huwandu hwemajini akachinjwa uye nekudaro kutsanangura kuoma kwethalassemia.

Vanhu vane hanya

Thalassemias zvirwere zvakagarwa nhaka, ndiko kuti, zvinotapuriranwa kubva kuvabereki kuenda kuvana vavo. Vanonyanya kusvika kuvanhu vanobva kuMediterranean rim, Middle East, Asia uye sub-Saharan Africa.

MuFrance, kupararira kwealpha-thalassemia kunofungidzirwa kune 1 mu350 vanhu. Chiitiko chebeta-thalassemia chinofungidzirwa pakuzvarwa kwe000 pa1 pagore pasi rose.

Zviratidzo zvethalassemia zvinosiyana zvakanyanya kubva kune imwe nyaya, uye zvinoenderana zvakanyanya nehuwandu hwekushandurwa kwemajini anobatanidzwa mukugadzirwa kwehemoglobin mapuroteni cheni. Thalassemias inogona kunge isina-chiratidzo-isina zviratidzo mumhando dzadzo diki uye ichityisidzira hupenyu mumhando dzadzo dzakanyanya.

Zviratidzo zvataurwa pazasi zvinongobata zvepakati kusvika kumhando huru dzethalassemia. Izvi zvinongova zviratidzo zvikuru. Zvakanyanya kujeka zviratidzo dzimwe nguva zvinogona kuonekwa zvichienderana nerudzi rwethalassemia.

Anemia

Chiratidzo chethalassemia ndeye anemia. Uku kushaikwa kwehemoglobin kunogona kukonzera kutaridzika kwezviratidzo zvakasiyana:

• kuneta;
• mweya wekufema;
• pallor;
• kusagadzikana;
• palpitations.

Kusimba kwezviratidzo izvi kunosiyana zvichienderana nekuoma kwethalassemia.

Jaundice

Vanhu vane thalassemia vanogona kuva nejaundice (jaundice) inoonekwa paganda kana machena emaziso. 

Gallstones

Kuumbwa kwematombo mukati me gallbladder kunogonawo kuonekwa. Maverengero akaita se“hurungudo diki”.

Splenomegaly

Splenomegaly kuwedzera kwespleen. Rimwe rebasa renhengo iyi nderekusefa ropa nekusefa zvinhu zvinokuvadza zvinosanganisira masero matsvuku eropa asina kujairika. Muthalassemia, spleen inobatanidzwa zvakasimba uye zvishoma nezvishoma inowedzera muhukuru. Marwadzo anogona kunzwika.

Zvimwe, zvisingawanzo zviratidzo

Zvizhinji kashoma, maitiro akaomarara ethalassemia anogona kutungamirira kune zvimwe zvisiri izvo. Somuenzaniso, inogona kuonekwa:

• hepatomegaly, ndiko kuti, kuwedzera kwekukura kwechiropa;
• kuremara kwemapfupa;
• kunonoka kukura kwemwana;
• maronda.

Kutarisira thalassemia kwakakosha kudzikamisa kuitika kweaya matambudziko.

Kutarisirwa kwethalassemia kunoenderana nezvakawanda zvimiro zvinosanganisira mhando yethalassemia, kuoma kwayo uye mamiriro emunhu ane hanya. Mafomu madiki haadi kurapwa nepo mafomu akakomba achida kutariswa kwekurapa nguva dzose.

Mishonga yadudzwa pazasi inongobata yepakati kusvika kumhando huru dzethalassemia

Kugadziriswa kweanemia

Kana kushayikwa kwehemoglobin kwakanyanyisa, kuisirwa ropa nguva dzose kunodiwa. Zvinosanganisira kubaya munhu ane hanya neropa kana kuti masero matsvuku eropa anotorwa kumupi kuti arambe aine mwero unogamuchirika wemasero matsvuku eropa.

Vitamin B9 kuwedzera

Zvinogona kukurudzirwa kutanga zuva nezuva vhitamini B9 yekuwedzera nekuti kudiwa kwevhitamini iyi kunowedzerwa mune zviitiko zve thalassemia. Vitamin B9 inobatanidzwa mukugadzirwa kwemasero matsvuku eropa.

Splenectomy

A splenectomy ndiko kuvhiyiwa kubviswa kwespleen. Kuvhiya uku kunogona kutariswa kana anemia yakakosha.

Kurapa kwesimbi yakawandisa

Vanhu vane thalassemia vane iron yakawandisa mumiviri yavo. Kuunganidza uku kunogona kukonzera matambudziko akasiyana. Ichi ndicho chikonzero iron chelators inopiwa kubvisa simbi yakawandisa.

Bone marongo kutapurirana

Kuisirwa mwongo ndiko chete kurapwa kunogona kurapa zvachose thalassemia. Uku ndiko kurapa kunorema kunongopihwa mumhando dzakanyanya dzechirwere.

Thalassemia chirwere chinogarwa nhaka. Hapana chiyero chekudzivirira.

Kune rumwe rutivi, kuongororwa kwemajini kunoita kuti zvikwanisike kuona vatakuri vane hutano (vanhu vane rimwe kana kupfuura rakashandurwa gene (s) asi vasingarware). Vaviri vevatakuri vane hutano vanofanirwa kuziviswa nezve njodzi yekuzvara mwana ane thalassemia. Mune zvimwe zviitiko, njodzi iyi inogona kuongororwa ne geneticist. Prenatal diagnosis inogonawo kutariswa mune mamwe mamiriro. Inofanira kukurukurwa nachiremba wako.