Marfan Syndrome: Inokonzera, Zviratidzo neMarapirwo Nzira

Marfan chirwere - nengozi chirwere, iro rine chokuita mutation zvimwe Gene boka. Vanhu vane chirwere chakadai vanotambura kuvhiringidzika nemamiriro connective tishu. Kokutanga hosha paakarondedzerwa 1876 kubudikidza Williams, asi chete muna 1896, asi chiremba wokuFrance Marfan A uwane Kudzidza zviratidzo uye zvinokonzera dambudziko iri uye akaitumidza zita.

Marfan urwere: Zvinokonzera

Sezvambotaurwa, chirwere nhaka, iyo chokuita mutation majini yeEncoding mapuroteni fibrin. Hazvina mapuroteni iri anokonzera kuipa mamiriro ose connective tishu. Murume ane mutation vakadaro vanoberekwa vaine urema. Zvisinei, Zviratidzo ringaratidzwa nenzira dzakasiyana. Somuenzaniso, kune varwere uyo chirwere kwatemwa netsaona, sezvo pasina zviratidzo zvinoonekwa havana. Asiwo, pane vanhu vanotambura zvikuru kubva chirwere, sezvo pamwe vakawanda deformities uye zvirwere.

Marfan Syndrome: Zviratidzo

Connective zvinyama - kunokosha chezvinonhuwira zvinenge zvose nhengo uye enyika ari mumuviri womunhu. Naizvozvo, Marfan chirwere kungazviratidza nenzira dzakasiyana - chirwere chinogona kukanganisa marangwanda, tsandanyama, huta uye kumwoyo:

• Kazhinji, munhu anorwara vangazivikanwa kubudikidza nemamiriro muviri. Varwere chirwere ichi kazhinji yakakwirira zvikuru uye dzakaonda, ane disproportionate elongated nemakumbo. Vakawanda vanotambura urema zvakadai scoliosis, flat tsoka, nenyonganiso marongerwo pechipfuva.
• Marfan chirwere kunowanzoguma maronda ziso - munhu, iya rimwe kana maviri lenses pakarepo. vanhu vakadaro vane kubatwa yemaziso, cataract uye retinal yokuzviparadzanisa.
• Kazhinji, chirwere achabatsirwa rwakavhunika marongerwo uye zvinoshanda kumwoyo - kunyanya remhosva kwakaderera rusvingo kutsemuka, iyo nengozi zvikuru muupenyu.
• Varwere Kazhinji tatamuka mavanga, kunyange pashure pakuguma nedanho kukura. Mukuwedzera, vari pasi inguinal uye ventral hernias.

Marfan chirwere: kuongorora chirwere uye kurapwa

To musi, hakuna chaiwo chirwere nzira. Pano hako vanoda misoro nzira. Chokutanga, chiremba anounganidza rakazara mishonga nhoroondo, uye ipapo ibasa miedzo uye zvidzidzo zviri zvetsandanyama nemapfupa uye kweropa enyika, uyewo ziso bvunzo.

Kana nzira kurapwa, ipapo kufanana nemagene zvirwere, Marfina chirwere anoramba nemunhu nekusingaperi. Asi norubatsiro ano mushonga mitoo anogona kuwedzera upenyu hupfupi uye nechokwadi murwere nyaradzo uye nyore. Murwere anofanira kugara kuongororwa navanachiremba. Somuenzaniso, kana matambudziko nokuona nyanzvi dzinogona basa akapfeka magirazi kana lenses. Achibatsirwa zvinodhaka anogona kubviswa chero zviratidzo kweropa ezvinhu, uye orthotics vanobatsira mune kunyatsogadzikana yacho marangwanda.

Nhasi, munyika yose uchipinda tsvakurudzo kupinda chirwere ichi. Scientific mudonzvo kuedza kufunga chii chinoitika kana zviratidzo mutation, uye kana zvichiita kumisa muitiro kana zvimwe kufurira nayo. Mukuwedzera, vemishonga vari kushanda pagadzirwe itsva, hubudirire mishonga kuti anogona kusimbisa zviratidzo uye kubvisa mukana rufu.