Lehlers-Danlos syndrome
Le Ehlers-Danlos chirwere iboka rezvirwere zvemajini zvinoratidzwa nea connective tissue abnormality, kureva, kutsigira matishu.
Kune zvakasiyana-siyana zvechirwere1, vazhinji vane a hyperlaxity yemajoini, ivo chaizvo elastic ganda uye tsinga dzeropa dzisina kusimba. Iyo syndrome haina kukanganisa kugona kwenjere.
Ehlers-Danlos syndrome yakatumidzwa zita remadermatologists maviri ekurapa, mumwe weDanish, Edvard Ehlers uye mumwe wechiFrench, Henri-Alexandre Danlos. Vakatsanangura chirwere ichi zvakare muna 1899 na1908. |
Zvinokonzera
Ehlers-Danlos syndrome is a genetic disorder inokanganisa kugadzirwa kwe collagen, puroteni inopa elasticity uye simba kune inobatana tishu seganda, tendon, ligaments, pamwe nemadziro enhengo uye nhengo. tsinga. Shanduko dzemajini akasiyana (semuenzaniso ADAMTS2, COL1A1, COL1A2, COL3A1) ndiwo angakonzeresa zviratidzo zvakasiyana zvichienderana nemhando dzakasiyana dzechirwere.
Mazhinji mafomu eEhlers-Danlos syndrome (EDS) anogarwa nhaka ne autosomal inotonga mamiriro. Mubereki akatakura shanduko yechirwere ichi nokudaro ane mukana we50% wekutapurira chirwere ichi kumwana mumwe nemumwe wavo. Zvimwe zviitiko zvinoonekwawo nezvinongoerekana zvachinja.
Matambudziko
Vanhu vazhinji vane Ehlers-Danlos syndrome vanorarama hupenyu hwakajairika, kunyangwe vaine zvirambidzo pamabasa emuviri. Matambudziko anoenderana nerudzi rweADS inobatanidzwa.
- zvinobatsira mavanga zvinokosha.
- zvinobatsira kusingaperi kurwadziwa kwemajoini.
- Early arthritis.
- Un aging nguva isati yakwana nekuda kwekuva nezuva.
- Osteoporosis.
Vanhu vane vascular-type EDS (Type IV SED) vari panjodzi yemamwe matambudziko akakomba, akadai sekuputika kwetsinga dzeropa dzakakosha kana nhengo dzakaita seura kana chibereko. Matambudziko aya anogona kuuraya.
Kuwanda
Kuwanda kwemarudzi ese eEhlers-Danlos syndrome pasi rese angangoita 1 mu5000 vanhu. nyora hypermobile, inonyanyozivikanwa, inofungidzirwa pa1 mu10, nepo iyo vascular type, isingawanzoitiki, inowanikwa mu1 mu250 kesi. Chirwere ichi chinoita sechinobata vese vakadzi nevarume.