I-Ehlers-Danlos syndrome: kuyini, izimpawu nokwelashwa

-Delile

• Ziyini izimpawu nezimpawu
• Izimbangela ezingaba khona
• Yiziphi izinhlobo
• Yini ukuxilongwa
• Yelashwa kanjani

Isifo i-Ehlers-Danlos syndrome, esaziwa kangcono njengesifo esinwebeka sowesilisa, sibonakala ngeqembu lokuphazamiseka kofuzo okuthinta izicubu zesikhumba, amalunga nezindonga zemithambo yegazi.

Ngokuvamile, abantu abanalesi sifo banamalunga, izindonga zemithambo yegazi nesikhumba esikwazi ukwelula kakhulu kunokujwayelekile futhi futhi sintekenteke kakhudlwana, ngoba yizicubu ezixhumene nomsebenzi wokubanika ukumelana nezimo, futhi kwezinye izimo, umonakalo omkhulu wemithambo yegazi kwenzeka.

Lesi sifo esidlula sisuka kubazali siye ezinganeni asinalo ikhambi kepha singelashwa ukunciphisa ubungozi bezinkinga. Ukwelashwa kuqukethe ukuphathwa kwemithi ye-analgesic ne-antihypertensive, ukwelashwa ngokomzimba futhi, kwezinye izimo, ukuhlinzwa.

Ziyini izimpawu nezimpawu

Izimpawu nezimpawu ezingenzeka kubantu abane-Ehlers-Danlos syndrome zinwebeka kakhulu amalunga, okuholela ekwenzekeni kokunyakaza okubanzi kunezinhlungu ezivamile nezinomphumela wendawo nokwenzeka kokulimala, ukuqina okukhulu kwesikhumba okusenza sithambe kakhudlwana futhi okuthe xaxa futhi kunesibazi esingajwayelekile.

Ngaphezu kwalokho, ezimweni ezinzima kakhulu, lapho i-Ehlers-Danlos syndrome inemithambo yegazi, abantu bangaba nekhala elizacile nomlomo ongaphezulu, amehlo agqamile nesikhumba esincanyana esilimele kalula. Imithambo yegazi emzimbeni nayo intekenteke kakhulu kanti kwabanye abantu umthambo i-aortic nawo ungaba buthakathaka kakhulu, ongahle uqhume futhi uholele ekufeni. Izindonga zesibeletho namathumbu nazo zincane kakhulu futhi zingaphuka kalula.

Ezinye izimpawu ezingavela yilezi:

• Ukuhlukaniswa kaningi nokuxinwa, ngenxa yokungazinzi kwamalunga;
• Ukuxubana kwemisipha;
• Ukukhathala okungapheli;
• I-arthrosis ne-arthritis ngenkathi isencane;
• Ubuthakathaka bemisipha;
• Ubuhlungu emisipheni nasemalungeni;
• Ukumelana okukhulu nobuhlungu.

Ngokuvamile, lesi sifo sitholakala ebuntwaneni noma ebusheni ngenxa yokuhlukaniswa kaningi, ukuqina nokuqina kwehaba iziguli ezinakho, okugcina kubize ukunakekelwa komndeni nodokotela wezingane.

Izimbangela ezingaba khona

I-Ehlers-Danlos syndrome yisifo sofuzo esizuzwe njengefa esenzeka ngenxa yokuguquka kwezakhi zofuzo ezifaka izinhlobo ezahlukahlukene ze-collagen noma ama-enzyme aguqula i-collagen, futhi angadluliselwa kusuka kubazali iye ezinganeni.

Yiziphi izinhlobo

I-Ehlers-Danlos syndrome ihlukaniswa ngezinhlobo eziyisithupha eziyinhloko, ngokuba uhlobo 3, noma ukungabi namandla, okuvame kakhulu, okubonakala ngokunyakaza okukhulu kakhulu, kulandelwe uhlobo 1 no-2, noma okudala, okuguquguqukayo kunika uhlobo lwe-collagen I no uhlobo IV noluthinta ukwakheka kwesikhumba kakhulu.

Uhlobo lwe-4 noma lwe-vascular luyivelakancane kunalolo lwangaphambilini futhi lwenzeka ngenxa yezinguquko kuhlobo III collagen oluthinta imithambo yegazi nezitho zomzimba, ezingahle ziqhume kalula.

Yini ukuxilongwa

Ukwenza ukuxilongwa, vele wenze ukuhlolwa ngokomzimba futhi uhlole isimo samalunga. Ngaphezu kwalokho, udokotela angenza futhi ukuhlolwa kwezofuzo kanye ne-biopsy yesikhumba ukuthola ukutholakala kwemicu ye-collagen eshintshiwe.

Yelashwa kanjani

I-Ehlers-Danlos syndrome ayinalo ikhambi, kepha ukwelashwa kungasiza ekunciphiseni izimpawu futhi kuvimbele izinkinga kulesi sifo. Udokotela anganikeza amaphilisi ezinhlungu, njenge-paracetamol, ibuprofen noma naproxen, ngokwesibonelo, ukudambisa izinhlungu nemithi yokwehlisa umfutho wegazi, ngoba izindonga zemithambo yegazi zibuthakathaka futhi kuyadingeka ukunciphisa amandla egazi elidlula ngalo.

Ngaphezu kwalokho, i-physiotherapy nayo ibaluleke kakhulu, njengoba isiza ukuqinisa imisipha nokuqinisa amalunga.

Ezimweni ezinzima kakhulu, lapho kudingeka khona ukulungisa ilunga elonakele, lapho kuqhuma khona umthambo wegazi noma isitho, ukuhlinzwa kungadingeka.