Le Isifo se-Ehlers-Danlos yiqembu lezifo zofuzo ezibonakala ngokuthi a ukungavamile kwezicubu ezixhumeneyo, okungukuthi, izicubu ezisekelayo.  

Kunezinhlobo ezahlukene zesifo¹, inengi linayo a hyperlaxity amalunga, lo isikhumba nokunwebeka kakhulu futhi imithambo yegazi entekenteke. I-syndrome ayithinti amakhono obuhlakani.

Izimbangela

I-Ehlers-Danlos syndrome iwukuphazamiseka kofuzo okuthinta ukukhiqizwa kwe-collagen, iphrotheni enikeza ukunwebeka namandla ezicutshini ezixhumene njengesikhumba, imisipha, imigqa, kanye nezindonga zezitho nezitho. imithambo yegazi. Ukuguqulwa kwezakhi zofuzo ezihlukene (isibonelo i-ADAMTS2, COL1A1, COL1A2, COL3A1) kungaba nesibopho sezimpawu ezihlukene ngokuya ngezinhlobo ezahlukene zesifo.

Izinhlobo eziningi ze-Ehlers-Danlos syndrome (EDS) zitholwa yizimo ezivelele ze-autosomal. Ngakho-ke umzali othwele uguquko olubangela lesi sifo unamathuba angama-50% okudlulisela lesi sifo kumntwana ngamunye wabo. Ezinye izimo zibuye zivele ngokushintshashintsha okuzenzakalelayo.

Izinkinga

Abantu abaningi abane-Ehlers-Danlos syndrome baphila ukuphila okuvamile, nakuba benemingcele emisebenzini engokomzimba. Izinkinga zincike ohlotsheni lwe-ADS oluhilelekile.

• izinzuzo izibazi kubalulekile.
• izinzuzo ubuhlungu obungapheli obuhlangene.
• I-arthritis yasekuqaleni.
• Un ukuguga ngaphambi kwesikhathi ngenxa yokuchayeka elangeni.
• I-osteoporosis.

Abantu abane-EDS yohlobo lwe-vascular (uhlobo IV SED) basengozini yezinkinga ezingathi sína, njengokuqhekeka kwemithambo yegazi ebalulekile noma izitho ezifana namathumbu noma isibeletho. Lezi zinkinga zingabulala.

Ukuvama

Ukusabalala kwazo zonke izinhlobo ze-Ehlers-Danlos syndrome emhlabeni wonke cishe ku-1 kubantu abangu-5000. thayipha i-hypermobile, evame kakhulu, ilinganiselwa ku-1 kwabayi-10, kuyilapho i- uhlobo lwe-vascular, ingavamile, ikhona ku-1 kwezingu-250. Lesi sifo sibonakala sihlasela abesifazane nabesilisa.