Progeria chirwere chisingawanzozivikanwa nekukura kwemwana.

Progeria, inozivikanwawo seHutchinson-Gilford syndrome, isingawanzo genetic disorder. Inoratidzwa nekuwedzera kukwegura kwemuviri. Vana vane chirwere ichi vanoratidza zviratidzo zvekutanga zvekukwegura.

Pakuzvarwa, mwana wacho haaratidzi chero zvisizvo. Zvinoratidzika "zvakajairika" kusvika paucheche. Zvishoma nezvishoma, morphology yake uye muviri wake unokura zvisina kujairika: anokura zvishoma nekukurumidza kupfuura zvakajairika uye haawane huremu hwemwana wezera rake. Kumeso, budiriro inononokawo. Anopa maziso akatanhamara (mukuzorodza, akasimba kwazvo), mhino yakatetepa uye yakakochekera, miromo mitete, chirebvu chidiki uye nzeve dzakabuda. Progeria ndiyo inokonzerawo kurasikirwa kukuru kwebvudzi (alopecia), ganda rakwegura, kukanganisa kwemajoini, kana kunyange kurasikirwa kwe subcutaneous fat mass (subcutaneous fat).

Kukura kwenjere nekuziva kwemwana kazhinji hakukanganisi. Izvo chaizvo kushomeka uye mhedzisiro pakukura kwemota, zvichikonzera kuomerwa nekugara, kumira kana kunyange kufamba.

Varwere vane Hutchinson-Gilford syndrome vanewo kuderera kweimwe tsinga, izvo zvinotungamirira mukugadzirwa kweatherosclerosis (kuvharwa kwetsinga). Zvisinei, arteriosclerosis ndiyo inonyanya kukonzera njodzi yekurwadziwa kwemoyo, kana kunyange Cerebral Vascular Accident (stroke).

Kuwanda kwechirwere ichi (nhamba yevanhu vanobatwa pakati pehuwandu hwevanhu) inokwana 1/4 mamiriyoni evana vachangoberekwa pasi rose. Naizvozvo chirwere chisingawanzoitiki.

Sezvo progeria chiri chirwere che autosomal chakanyanya kugarwa nhaka, vanhu vari panjodzi yekubatwa nechirwere chakadaro ndeavo vane vabereki vanewo progeria. Ngozi yekungoerekana yachinja genetic inogoneka. Progeria inogona ipapo kubata munhu upi noupi, kunyange kana hosha yacho isipo mumhuri.

Progeria chirwere chisingawanzo uye genetic. Kwakatangira iyi pathology imhaka yekuchinja mukati meLMNA gene. Iri geni rinokonzera kuumbwa kweprotein: Lamine A. Iyo yekupedzisira inoita basa rinokosha pakuumbwa kwesero nucleus. Chinhu chakakosha pakuumbwa kwehamvuropu yenyukireya (membrane yakatenderedza nucleus yemasero).

Kuchinja kweGenetic mune iyi gene kunotungamirira kune kuumbwa kusina kujairika kweLamine A. Iri puroteni isina kuumbwa iri pamavambo ekusagadzikana kwesero nucleus, pamwe nekufa kwekutanga kwemasero ehupenyu.

Masayendisiti parizvino ari kuzvibvunza nezvenyaya iyi, kuitira kuti awane rumwe ruzivo nezve kubatanidzwa kweprotein iyi mukuvandudza kwemuviri wemunhu.

Iyo genetic transmission yeHutchinson-Gilford syndrome inoitika kuburikidza ne autosomal inotonga nhaka. Zvichida kutapurirwa kweimwe chete yemakopi maviri ejini chairo (zvingava kubva kuna amai kana kuti kubva kuna baba) kunokwana kuti hosha ikure mumwana.

Uyezve, kuchinja kusina kujeka (kusingabvi kubva mukutapurirwa kwemajini evabereki) yeLMNA gene inogonawo kuva pamavambo echirwere chakadaro.

Zviratidzo zveprogeria zvinoratidzwa ne:

• kukwegura kwepakutanga kwemuviri (kubvira pauduku);
• kuderedza uremu pane zvakajairika;
• hukuru hudiki hwemwana;
• kukanganisa kwechiso: mhino yakaonda, yakasungirirwa, maziso akakurumbira, chirebvu chiduku, nzeve dzakaputika, nezvimwewo;
• kunonoka kwemotokari, zvichikonzera kuoma kumira, kugara kana kunyange kufamba;
• kutetepa kwetsinga, njodzi huru yearteriosclerosis.

Sezvo progeria isingawanzoitiki, genetic uye yakagarwa nhaka autosomal dominant chirwere, kuvapo kwechirwere mune mumwe wevabereki vaviri ndiyo inonyanya kukosha yengozi.

Zviratidzo zvine chekuita neprogeria zvinofambira mberi uye zvinogona kutungamirira kurufu rwemurwere.

Parizvino hapana mushonga wechirwere ichi. Iyo chete manejimendi yeprogeria ndeye zviratidzo.

Tsvagiridzo inozove mukutarisa, kuitira kuti uwane mushonga unobvumira kurapwa kwechirwere chakadaro.