Chii Chinonzi Charcot-Marie-Tooth Disease?

Iboka Rematambudziko Rinobudirira Rinoita Mavhinga Ako

Chirwere cheCharcot-Marie-Tooth (CMT) chinomiririra boka rematambudziko anokonzera kukanganisa mumatambudziko emakumbo ako nemakumbo. Izvi zvinokonzerwa nehutachiona zvinogona kuenderera mberi zvichikonzera musimba kushaya simba, atrophy , uye kurasikirwa kwekunzwa.

Mhando dzeCharcot-Marie-Tooth Disease

Kune marudzi akawanda eCharcot-Marie-Tooth (CMT) chirwere. Mumatambudziko e1 CMT, chirwere chacho chinokanganisa myelin sheath, chifukidzo chekudzivirira chiropa.

Muchikamu cheCMT Type 2, marwadzo ivo pachavo akanganiswa. CMT Rudzi rwechitatu (Dejerine-Sottas chirwere), CMT Rudzi rwechina, uye CMT X, seChirungu 1, inokanganisa myelin sheath.

CMT inowanikwa munyika yose, ichibata vanhu vemarudzi ose. Munenge 150 000 vanhu muUnited States vane CMT uye 1 mu 2,500 munyika yose vanogara nemamiriro acho ezvinhu. CMT yakatanga kurondedzerwa nevatsvakurudzi Jean-Marie Charcot, Pierre Marie, naWoward Henry Tooth muna 1886, Charcot-Marie-Tooth inonzi ishoma. CMT pane imwe nguva yakanzi "peroneal muscular atrophy" kana kuti motokari inokonzerwa neuropathies.

Zviitiko zvakawanda zveCharcot-Marie-Tooth zvinopfuudzwa pane zvinyorwa, sechinhu chinokonzera chromosomal. Inenge 15 kubva muzana yematambudziko anoitika pasina mhuri yekare yeiyo. Chirwere ichi chinowanzoonekwa pakati pemakore gumi nemashanu.

Ndezvipi Zviratidzo?

Zviratidzo zvekutanga zveCMT zvinowanzofananidzirwa nezvinetso zvetsoka, izvi zvinogona kusanganisira zvikwangwani zvakaputika, kutsika, kana kuonekwa zvakashata.

Sezvo chirwere ichi chichipfuurira, rutsoka rutsoka runogona kuva rukuru uye zvigunwe zvakanyongedzwa. Zvinogona kuva zvakaoma kuvanhu vane CMT kusimudza tsoka dzavo. Vanhu vane CMT vanogona kufambisisa zvikuru, vachikotamisa mabvi kuti vasimudze tsoka dzavo pavanenge vachifamba.

Ruoko rwakatadza runogona kutanga nematambudziko nekunyora kana kushandisa zippers kana mabhatani.

Uturu uye musimba cramping zvinogona kuitika.

Kunyangwe pakati pemhuri yemhuri imwechete, kuoma kwe CMT kunogona kusiyana. Semuenzaniso, munhu mumwe chete angatomboona zviratidzo apo imwe yacho ine tsoka dzekutsvaira uye kunetseka kufamba. Vanhu vane CMT vangave vakapfupika mumamiriro uye vane maziso akavhara.

CMT haisi, pane dzimwe nguva, inopindira dzimwe nhengo dzomuviri seuropi kana mwoyo, mano ekufunga, uye kutarisira hupenyu zvose zvinowanzoitika. Mamiriro acho haasi kuuraya, asi hapana mushonga.

Inenge 15 kubva muzana yevanhu vane CMT vane chimiro chechirwere chakabatanidzwa ne X chromosome (inonzi CMTX). Zvidzidzo zvakaratidza kuti vanhu vane CMTX vanogona kuva nechepakati penheyo yezviratidzo zviratidzo kuwedzera kune zviratidzo zvinowanzoonekwa. Zviitiko zvakakomba zve CMT zvinogona kukonzera matambudziko ekufema zvakare.

CMT Inozivikanwa Sei?

Kana chiremba achifungira CMT nekuda kwesimba, gumbo, uye netsoka kushaya simba, kuongororwa kwakaitwa nemitsipa ( nerve conduction velocity , kana NCV) uye mishonga ( electromyogram , kana EMG) inogona kuitwa kusimbisa kuongororwa. Zvimwe zvirwere zvema genetic zvinogona kuona mamwe marudzi e CMT.

CMT Inotarirwa Sei?

Sezvo pasina chirwere kana nzira yekuderedza chirwere ichi, kurapwa kunowedzera pakuderedza zviratidzo.

Mhemberero dzegumbo uye shangu dzakakosha zvinogona kubatsira nekufamba, sezvinoitwa kurapa kwepanyama . Dzimwe nguva tsoka dzekuvhiya ( osteotomy kana arthrodesis ) inogona kudikanwa kururamisa tsoka dzakasvibiswa. Mishonga inogona kupiwa kugadzirisa kurwadziwa kwemasumbu nekupwanya.

Sources:

Muscular Dystrophy Association. Chokwadi Nezvechirwere cheCharcot-Marie-Tooth (CMT).

Kedlaya, D. (2002). Chirwere cheCharcot-Marie-Tooth. eMedicine.

National Institute for Neurological Disorders uye Stroke. Charcot-Marie-Tooth Disorder Information Page.

Zwipp, H., Rammelt, S., Dahlen, C., & Reichmann, H. (1999). Mutauro weCharcot. Orthopade, 28 (6), 550-558.