Chii chinonzi Zellweger syndrome uye maitiro ekurapa

Zvemukati

• Syndrome maficha
• Chii chinokonzera chirwere
• Kurapwa kunoitwa sei

Zellweger syndrome chirwere chisingawanzoitika chinokonzera shanduko mumasaga uye kumeso, pamwe nekukuvara kwakanyanya kunhengo dzakakosha senge moyo, chiropa neitsvo. Uye zvakare, kushomeka kwesimba, kunetseka kunzwa uye kubatwa zvakare zvakajairika.

Vacheche vane chirwere ichi vanowanzoratidza zviratidzo nezviratidzo mumaawa mashoma ekutanga kana mazuva mushure mekuzvarwa, saka chiremba wevana angabvunze kuti ropa nemurini zviongororwe kuti zvionekwe kuongororwa.

Kunyangwe pasina mushonga weichi chirwere, kurapwa kunobatsira kugadzirisa zvimwe zvekuchinja, kunowedzera mikana yekurarama uye kunotendera kuvandudzwa kwehupenyu hwehupenyu. Nekudaro, zvinoenderana nerudzi rwechimiro chinoshanduka, vamwe vacheche vane avhareji tarisiro yeupenyu isingasviki mwedzi mitanhatu.

Syndrome maficha

Hunhu hukuru hwehunhu hweZellweger syndrome hunosanganisira:

• Flat kumeso;
• Mhino yakafara uye yakati sandara;
• Huma pahuma;
• Warhead mukanwa;
• Maziso akatarira kumusoro;
• Musoro wakanyanya kukura kana kudiki;
• Mapfupa ehenya akapatsanurwa;
• Rurimi rwakakura kudarika zvakajairwa;
• Ganda rinopeta mumutsipa.

Uye zvakare, shanduko dzinoverengeka dzinogona kuitika munhengo dzakakosha senge chiropa, itsvo, huropi nemoyo, izvo, zvinoenderana nekuomarara kwekukanganisa, zvinogona kutyisidzira hupenyu.

Zvakajairikawo kuti mumazuva ekutanga ehupenyu, mwana anoshaya simba mumhasuru, kunetseka kuyamwisa, kugwinha uye kunetseka kunzwa nekuona.

Chii chinokonzera chirwere

Iyo syndrome inokonzerwa ne autosomal recessive genetic shanduko mumaPEX magene, zvinoreva kuti kana paine zviitiko zvechirwere mumhuri dzevabereki vese, kunyangwe kana vabereki vasina chirwere, pane mukana ungangoita 25% wekuve mwana ane Zellweger's syndrome.

Kurapwa kunoitwa sei

Iko hakuna chaiwo maitiro ekurapwa kweZellweger syndrome, uye mune imwe neimwe kesi, chiremba wevana anofanirwa kuongorora shanduko dzinokonzerwa nechirwere mumwana uye kukurudzira kurapwa kwakanyanya. Dzimwe sarudzo dzinosanganisira:

• Zvakaoma kuyamwisa: kuisa chubhu diki yakananga mudumbu kubvumira chikafu kupinda;
• Shanduko mumoyo, itsvo kana chiropa: chiremba angasarudza kuvhiyiwa kuti aedze kugadzirisa kusagadzikana kana kushandisa mishonga inoderedza zviratidzo;

Zvisinei, muzviitiko zvakawanda, shanduko munhengo dzakakosha, dzakadai sechiropa, moyo uye huropi, haigone kugadziriswa mushure mekuzvarwa, saka vana vazhinji vanopedzisira vaine kutadza kwechiropa, kubuda ropa kana kutyisidzira hupenyu matambudziko ekufema. Mumwedzi mishoma yekutanga.

Kazhinji, zvikwata zvekurapa zverudzi urwu zve syndromes zvinoumbwa nevanoverengeka vezvehutano mukuwedzera kune vanachiremba vevana, vakadai sevana cardiologist, maurosurgeon, ophthalmologists uye orthopedists, semuenzaniso.