Werdnig-HOFFMANN: mawonekedwe ndi zizindikiro zazikulu

Werdnig-HOFFMANN - kwambiri aakulu kudwala amene anatsagana ndi amyotrophy, pang'onopang'ono chiwonongeko cha waukulu mitsempha ulusi chingwe msana, ndipo, chotero, minofu ya manja. Mwakucimbidza Dziwani kuti ali ndi anatengera chibadwa matenda, zomwe imafalikira mu autosomal unsembe recessive.

Werdnig-HOFFMANN ndi chiyani?

Ndi matenda a ubongo limodzi ndi chiwonongeko mwapang'onopang'ono nyumba zofunika mantha dongosolo innervating. Mwachitsanzo, zambiri pali demyelination a mizu zapambuyo wa chingwe msana. Komanso, matendawa amakhudza ena misempha cranial.

Inde, kuwonongeka kwa ulusi mitsempha amakhudza boma minofu. Komabe, matenda yodziwika ndi otchedwa mtengo minofu imfa, imene mbali ya minofu kuti angathe kutenga, pamene osiyana "mitolo" manja.

Msana manja Verdniga-HOFFMANN akuwonetseredwa mu mwana. Today lagawidwa njira zitatu za matendawa.

Kobadwa nako matenda Verdniga-HOFFMANN ndi zizindikiro zake

Childs, zizindikiro woyamba wa mawonekedwe a matenda kale noticeable mu masiku oyambirira angapo pambuyo pa kubadwa. The mwana azipereka waulesi paresis wa malekezero. Kulira kwa ana odwala ndi ofooka ndipo sakonda lomveka, kuwonjezera, iwo ndi ndondomeko mphamvu wathyoka.

Monga mwana chanu chikula, mukhoza zindikirani kwanthawi mu kukula. ana odwala sasunga mutu, sakhoza kukhala kapena kuima. Only mu nthawi zina, mwanayo akhoza kugwira thupi ndi malo olungama, koma luso limeneli Mwamsanga mwachilungamo mwamsanga chiwonongeko ulusi mitsempha.

Komanso pali ena kuvutika mu chitukuko chigoba, makamaka m'chiuno dysplasia, kupindika msana, mapangidwe nyuzi pachifuwa, clubfoot, hydrocephalus.

Pa matenda oopsa, ndipo alibe wa molekezera mitsempha akuchita mofulumira. Manja tinganene osati minofu chigoba, komanso ulusi ziwalo mkati. Nthawi zambiri bwanji zakulera, imbaenda kulephera kupuma. Mwatsoka, ana ndi matenda ofanana moyo (pafupifupi) kwa zaka zisanu ndi zinayi.

matenda oyambirira Verdniga-HOFFMANN

Zizindikiro zazikulu za matenda zimapezeka mu theka lachiwiri la moyo. Miyezi ingapo ya kukula kwa mwana akudutsa zabwinobwino - mwana amaphunzira agwire mutu ndi kukhala, nthawi zina angathe kukhala tsonga okha. Koma luso onsewa anatayika pambuyo kutsegula kwa matendawa. Mwa njira, nthawi zambiri amakwiya matenda syndrome.

Kugwedeza zala ndi tendon contracture - zizindikiro loyamba la chiwonongeko ulusi mitsempha. Kenako akufotokozera manja ndi ziwalo minofu. Kutalika avareji moyo odwala ndi pakati pa zaka 14 mpaka 16.

Mochedwa matenda Verdniga-HOFFMANN

matenda amenewa ndi bata. Monga ulamuliro, mwana akufotokozera ndithu bwinobwino mpaka zaka ziwiri. Mwana amaphunzira kukhala, kuima ndi kuyenda. Only ndi nthawi makolo ndinayamba kuzindikira kupatuka ena.

Choyamba, kusintha gait a mwana wodwala - amapita, zambiri kupinda mawondo, kazinji kene mbagwa n'kudawa, samachititsa muyezo. Monga kudwala ndi kuzindikira kusintha mafupa, monga mapindikidwe a chifuwa. syndrome Verdniga-Hoffman amakhala ndi kugwedeza amphamvu, utachepa minofu kamvekedwe, kutha kwa waukulu unconditioned bongo.

Nthawi zambiri zaka 10-12 mwana kwathunthu wotaya luso kuyenda palokha. Komabe, mu nkhani iyi odwala adzapulumuke 20, ndipo nthawi zina zaka 30.