Duchenne akulu dystrophy ndi Becker: chithandizo

matenda majini a gulu la pathologies katundu mankhwala omwe lero ndi zovuta. Pakati kuvutika izi chromosomal zimapezeka kuyan'anila zosiyanasiyana. Ambiri a iwo zizindikiro neurologic. Zitsanzo ndi Duchenne akulu dystrophy, Becker. matenda amenewa kukhala ana ndipo ndi njira patsogolo. Ngakhale kupita patsogolo kwa neuroscience, pathologies amenewa ndi ovuta kuchiza. Ichi ndi chifukwa chromosomal kusintha amene anaika pa mapangidwe thupi.

Kufotokozera za Duchenne akulu dystrophy

Duchenne akulu dystrophy - matenda majini yodziwika ndi kupata matenda minofu unit. The kudwala ali osowa. Kuchuluka kwa anomaly pafupifupi anthu 3 pa 10 000 amuna. Disease pafupifupi milandu, zimakhudza anyamata. Komabe, kupanga Duchenne atsikana akulu si lilibe. kudwala kumeneku kumaonekera ana aang'ono.

matenda ena amene ali zimayambitsa chomwecho ndi zizindikiro ndi myodystrophy Becker. Iwo ali Inde yabwino kwambiri. minofu minofu kuwonongeka amapezeka patapita nthawi yaitali - mu unyamata. Pankhaniyi, zizindikiro kukhala pang'onopang'ono, ndipo wodwala angathe ntchito patangopita zaka zochepa. Mofanana ndi Duchenne akulu dystrophy, kudwala izi zinkachitika pakati anthu wamwamuna. Kuchuluka 1 munthu anyamata 20 sauzande.

Duchenne akulu dystrophy: matenda neuroimmunology

Chifukwa pathologies onse lagona kuphwanya X khromozomu. kusintha chibadwa kuti zimachitika Duchenne ndi Becker akulu dystrophy, anaphunzira 30 ntchentche Mu zaka zapitazo. Komabe mankhwala etiological silinakwaniritsidwe wapezeka. anomalies recessive akafuna cholowa. Izi zikutanthauza kuti ngati jini nthenda chili mwa kholo limodzi, Mwina kubadwa odwala mwana ndi 25%. The X khromozomu ndi Alevi thupi. Ndi mitundu onse dystrophies wakuswa amapezeka wina ndi locus yemweyo (p21). kuwonongeka umenewu umatipatsa kuchepa kaphatikizidwe mapuloteni, zomwe ndi mbali ya zomwe zimagwira ntchito zosiyanasiyana selo minofu minofu. Duchenne akulu dystrophy pamene palibe kwathunthu. Choncho, kuyan'anila zimachitika kale kwambiri. Becker akulu dystrophy pamene mapuloteni ndi apanga zochepa kapena pathological.

The matenda chithunzi cha akulu dystrophy

Duchenne akulu dystrophy amakhala ndi zotupa za dongosolo neuromuscular. Matendawa akhoza amaganiziridwa pa zaka 2-3. Nthawi imeneyi, izo zimaonekera kuti mwana lags kumbuyo kukula kwa anzawo akupita zoipa, akuthamanga ndi kudumpha. Choncho ana ovuta kukwera masitepe, nthawi zambiri kugwa. Kugonjetsedwa kwa minofu ikuyamba ndi malekezero m'munsi. Pambuyo pake, chimakwirira proximal akatumba onse. Alibe akupezeka lamba chapamwamba phewa, quadriceps. Mu malo awa pali kupatulira minofu. Kwa zaka zambiri, myodystrophy m'kati. Kugonjetsedwa kwa minofu ndi katundu nthawi zonse pa iwo kuyambitsa contractures - kakamirani kuwapotoza miyendo. Komanso, odwala ndi Duchenne akulu dystrophy anati matenda a mtima, omwe nthawi okha anamvera. Komanso matenda yodziwika ndi kuchepa kwa luso ozindikira (osati kwambiri kutchulidwa).

Becker akulu dystrophy ali ndi zizindikiro zomwezo, koma chokha pambuyo pake. Mawonetseredwe koyamba kudzachitika mu zaka 10-15. Pali kusintha pang'onopang'ono unsteadiness gait aonekera kenako anadzakhala contracture. Kupondelezedwa kwa dongosolo mtima wofatsa. Intelligence ku matenda zambiri osati zochepa.

Kodi matenda akulu dystrophy?

Matendawa "Duchenne akulu dystrophy" (kapena Becker) akhoza kuyika katswiriyu odziwa. Mu malo oyamba izo zinachokera pa chithunzi matenda a matenda amenewa. Akunena kuti zizindikiro monga kupatulira wa minofu proximal, ndi gastrocnemius minofu hypertrophy onyenga (kumachitika chifukwa fibrosis ndi madipoziti mafuta). zizindikiro pafupifupi zonse pamodzi pathologies mtima. The ECG Tingaone dysrhythmia, kumanzere yamitsempha yamagazi hypertrophy.

Komanso, odwala ndi Duchenne akulu dystrophy pang'ono kwanthawi kumbuyo anzawo kukula m'nzeru. Kudziwa ichi, ana ankagwira ntchito zamaganizo a. Pamene matenda ndi achifwamba anagwira myography (tanthauzo la lingathe minofu magetsi) ndi EhoKS - Kupenda zipinda za mtima. Pofuna molondola kudziwa pamaso pa matenda, matenda majini imagwiridwa. Pamene Duchenne ndi Becker odwala akulu dystrophy uyenera kuchitika pa akatswiri angapo. Pakati pawo - katswiriyu, ndi zamaganizo ndi cardiologist.

chithandizo

Mwatsoka, chithandizo etiological wa Duchenne akulu dystrophy ndi Becker osati zotere. Komabe, odwala kusonyeza symptomatic ndi wothandiza mankhwala. Mu magawo oyambirira a matenda ikuchitika maphunziro a physiotherapy, kutikita. Olumala kwambiri muyenera kuchita kayendedwe chabe nthambi. Kuti amachepetsa mphamvu ya contractures kutambasuka, amachita kukonza miyendo akagona. Yokonza mankhwala akhoza kutalikitsa moyo wa odwala ndi kuchepetsa zizindikiro. Ntchito kashiamu zowonjezera, mankhwala "galantamine" ndi "Neostigmine". Nthawi zina, mankhwala wothandizila m'thupi, makamaka "Prednisolone". Pamene matenda pang'onopang'ono kwa mtima anaika cardioprotectors.

Duchenne akulu dystrophy ndi Becker Mapa

Duchenne akulu dystrophy madokotala ananena zawo ndi zovuta. The woyambirira zizindikiro ndi kupitirira mofulumira matenda kumayambitsa chilema ana. Odwala matenda amafunika chisamaliro nthawi zonse. Wapakati moyo amayembekezeka odwala ndi zaka 20. Becker akulu dystrophy amakhala ndi njira yabwino. Moyang'aniridwa nthawi zonse madokotala ndi kukhazikitsa malangizo awo operability odwala ndi anasunga zaka 30-35.