Chii chinonzi kuberekwa myasthenia, zviratidzo uye kurapwa

Zvemukati

• Zviratidzo zveKuberekwa Myasthenia
• Maitiro ekuongororwa kunoitwa
• Kurapa kweKuberekwa Myasthenia
• Inogona kuberekwa myasthenia inogona kurapwa?

Congenital Myasthenia chirwere chinosanganisira kusangana kwemitsipa uye nekudaro chinokonzeresa kushomeka kwemhasuru kushomeka, kazhinji zvichitungamira munhu kuti afambe newiricheya. Chirwere ichi chinogona kuwanikwa mukuyaruka kana mukukura uye zvichienderana nerudzi rwekuchinja kwemajini munhu wacho, hunogona kurapwa nekushandisa mishonga.

Pamusoro pemishonga inoratidzirwa nachiremba wezveuropi, physiotherapy inodikanwa zvakare kuti iwane simba remhasuru uye kurongedza mafambiro, asi munhu anogona kufamba zvakajairazve, pasina chinodiwa chewiricheya kana madondoro.

Congenital myasthenia haina kunyatso kufanana ne myasthenia gravis nekuti mune nyaya yaMyasthenia Gravis chikonzero chinokanganisa immune system yemunhu, nepo pakuzvarwa myasthenia chikonzero chiri chekuchinja kwemajini, icho chinowanzoitika muvanhu mumhuri imwe chete.

Zviratidzo zveKuberekwa Myasthenia

Zviratidzo zveConitalital Myasthenia zvinowanzoonekwa muvacheche kana pakati pemakore matatu neanomwe ekuberekwa, asi mamwe marudzi anoonekwa ari pakati pemakore makumi maviri nemakumi mana, angave ari:

Mucheche:

• Kuoma mukuyamwisa kana kuyamwisa-bhodhoro, kudzipwa nyore uye kushoma simba rekuyamwa;
• Hypotonia inozviratidza kuburikidza nekushaya simba kwemaoko nemakumbo;
• Kudonhedza eyelid;
• Chibvumirano chakabatana (congenital arthrogryposis);
• Kuderera kwechimiro chechiso;
• Kunetseka kufema uye purplish zvigunwe nemiromo;
• Kubudirira kunonoka kugara, kukambaira uye kufamba;
• Vana vakura vanogona kuzviwana kwakaoma kukwira masitepisi.

Muvana, vachiri kuyaruka kana vakuru:

• Kushaya simba mumakumbo kana maoko nekunzwa kunzwa;
• Zvakaoma kufamba pamwe nekudiwa kwekugara pasi kuti uzorore;
• Panogona kuve nehutera mumhasuru dzemaziso dzinodonhedza eyelid;
• Kuneta paunenge uchiedza kushoma;
• Panogona kunge paine scoliosis mumuzongoza.

Kune mana mana akasiyana mhando yekuzvarwa myasthenia: inononoka chiteshi, yakaderera kudyidzana inokurumidza chiteshi, yakaoma AChR kushomeka kana AChE kushomeka. Sezvo kuberekwa kunononoka-chiteshi myasthenia kunogona kuoneka pakati pemakore makumi maviri kusvika makumi matatu ekuberekwa. Mhando yega yega ine hunhu hwayo uye marapirwo anogona kusiyanisa kubva pamunhu kuenda kune mumwe nekuti havazi vese vane zviratidzo zvakafanana.

Maitiro ekuongororwa kunoitwa

Kuongororwa kweCongenital Myasthenia kunofanirwa kuitwa zvichienderana nezviratidzo zvinoratidzwa uye zvinogona kusimbiswa nemiyedzo yakadai seCK bvunzo yeropa uye kuongororwa kwemajini, bvunzo dzeantibody kuti dzisimbise kuti haisi Myasthenia Gravis, uye electromyography inoongorora mhando yechibvumirano. mhasuru, semuenzaniso.

Muvana vakura, vachiri kuyaruka nevakuru, chiremba kana physiotherapist vanogona zvakare kuita bvunzo muhofisi kuti vaone kushomeka kwemhasuru, senge:

• Tarisa padenga kwemaminetsi maviri, wakagadzika uye uone kana paine kuwedzera kwekuoma kwekuchengeta maziso eziso akavhurika;
• Simudza maoko ako kumberi, kusvika pakureba kwepafudzi, wakabata chinzvimbo ichi kwemaminetsi maviri uye uone kana zvichikwanisika kuchengetedza chibvumirano ichi kana maoko ako akadonha;
• Simudza mubhedha usingabatsirwe nemaoko ako kanopfuura ka1 kana kusimudza kubva pachigaro kanopfuura ka2 kuti uone kana paine kuwanda uye kuoma kwekuita kufamba uku.

Kana kushomeka kwemhasuru kuchionekwa uye zvichinetsa kuita bvunzo idzi, zvinowanzoitika kuti kune hurema hwesimba, zvichiratidza chirwere chakadai semyasthenia.

Kuti uone kana kutaura kwakakanganiswawo, unogona kukumbira munhu kuti ataure manhamba kubva pa1 kusvika 100 uye uone kana paine shanduko yezwi, kutadza kwenzwi kana kuwedzerwa kwenguva pakati pekutaura kwenhamba yega yega.

Kurapa kweKuberekwa Myasthenia

Marapirwo anosiyana zvichienderana nerudzi rwekuberekwa myasthenia iyo munhu anayo asi mune dzimwe nguva mishonga yakadai seacetylcholinesterase inhibitors, Quinidine, Fluoxetine, Ephedrine uye Salbutamol inogona kuratidzwa pasi pekurudziro yachiremba neuropediatrician kana neurologist. Physiotherapy inoratidzwa uye inogona kubatsira munhu kuti anzwe zvirinani, kurwisa kushaya simba kwemhasuru uye kugadzirisa kufema, asi hazvizobudirire pasina mishonga.

Vana vanogona kurara nemasikisi eoksijeni anonzi CPAP uye vabereki vanofanira kudzidza kupa rubatsiro rwekutanga kana paine kusungwa kwekufema.

Mune physiotherapy maekisesaizi anofanirwa kuve isometric uye ane mashoma ekudzokorora asi iwo anofanira kuvhara akawanda mhasuru mapoka, kusanganisira iwo ekufema uye anobatsira kwazvo kuwedzera huwandu hwemitochondria, mhasuru, capillaries uye kudzikisa iyo lactate yevasungwa, iine mashoma cramp.

Inogona kuberekwa myasthenia inogona kurapwa?

Muzviitiko zvakawanda, congenital myasthenia haigone kurapwa, inoda kurapwa kwehupenyu hwese. Nekudaro, mishonga uye physiotherapy zvinobatsira kusimudzira hupenyu hwemunhu, kurwisa kuneta uye kushomeka kwemhasuru uye kudzivirira kunetseka senge atrophy yemaoko nemakumbo uye kudzipwa kunogona kumuka kana kufema kukanganisika, ndosaka, hupenyu hwakakosha.

Vanhu vane Congenital Myasthenia inokonzerwa nehurema muDOK7 geni vanogona kuve nekuvandudzika kukuru mumamiriro avo, uye vanogona kunge 'vachipora' nekushandiswa kwemushonga unowanzoshandiswa kurwisa asthma, salbutamol, asi muchimiro chemapiritsi kana lozenges. Zvisinei, iwe unogona kunge uchiri kuda kuita kurapwa kwepanyama zvishoma nezvishoma.

Kana munhu aine Congenital Myasthenia uye akasarapwa, vanozopedzisira vashaya simba mumhasuru, voita mweya, vachida kuramba vakarara pamubhedha uye vanogona kufa nekufema kutadza uye ndosaka kurapwa uye kurapwa kwemuviri kuchikosha nekuti vese vanogona kuvandudza hupenyu hwemunhu uye hurebese hupenyu.

Mimwe mishonga inokanganisa mamiriro eCongenital Myasthenia ndeye Ciprofloxacin, Chloroquine, Procaine, Lithium, Phenytoin, Beta-blockers, Procainamide neQuinidine uye nekudaro mishonga yese inofanirwa kushandiswa chete pasi pezano rekurapa mushure mekuzivikanwa kwerudzi rwune munhu wacho.