IMyasthenia gravis

IMyasthenia gravis sisifo se-neuromuscular disorder. Iingxaki ze-Neuromuscular zibandakanya imisipha kunye neentsholongwane ezilawulayo.

I-Myasthenia gravis ikholelwa ukuba luhlobo lwesifo sokuzimela. Ukuphazamiseka komzimba kwenzeka xa amajoni omzimba ehlasela ngempazamo izicwili ezisempilweni. Ii-antibodies ziiprotein ezenziwe ngamajoni omzimba xa zibhaqa izinto ezinobungozi. Ii-antibodies zinokuveliswa xa amajoni omzimba ngokungalunganga ejonga izicwili ezisempilweni njengezinto eziyingozi, njengakwimeko ye-myasthenia gravis. Kubantu abane-myasthenia gravis, umzimba uvelisa izilwa-buhlungu ezithintela iiseli zemisipha ekufumaneni imiyalezo (ii-neurotransmitters) ezivela kwiiseli zemithambo-luvo.

Ngamanye amaxesha, i-myasthenia gravis inxulunyaniswa nezihlunu ze-thymus (ilungu lomzimba).

I-Myasthenia gravis inokuchaphazela abantu nangaliphi na ubudala. Kuqheleke kakhulu kubafazi abancinci nakumadoda amadala.

I-Myasthenia gravis ibangela ubuthathaka bemisipha yokuzithandela. Ezi zihlunu onokuzilawula. Izihlunu ezizimeleyo zentliziyo kunye neendlela zokugaya ukutya zihlala zingachaphazeleki. Ubuthathaka bemisipha ye-myasthenia gravis buba mandundu ngomsebenzi kwaye buphucule ngokuphumla.

Oku buthathaka kwezihlunu kungakhokelela kwiimpawu ezahlukeneyo, kubandakanya:

• Ukuphefumla ubunzima ngenxa yobuthathaka bemisipha yodonga lwesifuba
• Ukuhlafuna okanye ukugwinya ubunzima, kubangele ukugcagca rhoqo, ukukrwitsha okanye ukuhlahlela amathe
• Kunzima ukunyuka izitepsi, ukuphakamisa izinto, okanye ukunyuka kwindawo ohleli kuyo
• Kunzima ukuthetha
• Intloko ewileyo kunye neenkophe
• Ukukhubazeka ebusweni okanye ubuthathaka bemisipha yobuso
• Ukudinwa
• Ukugquma okanye ukutshintsha kwelizwi
• Umbono kabini
• Kunzima ukugcina ujongano oluzinzileyo

Umboneleli wezempilo uya kwenza uvavanyo lomzimba. Oku kubandakanya inkqubo yoviwo lweemvakalelo (luvo). Oku kungabonisa:

• Ubuthathaka bemisipha, izihlunu zamehlo zihlala zichaphazeleka kuqala
• Ukucinga okuqhelekileyo kunye nemvakalelo (imvakalelo)

Uvavanyo olunokwenziwa lunokubandakanya:

• Iintsholongwane ze-Acetylcholine receptor antibodies ezinxulumene nesi sifo
• Ukuxilongwa kwe-CT okanye iMRI kwesifuba ukukhangela ithumba
• Izifundo zokuqhuba ngemithambo ukuvavanya ukuba zikhawuleza kangakanani iimpawu zombane kwi-nerve
• I-Electromyography (EMG) yokuvavanya impilo yezihlunu kunye neovalo ezilawula izihlunu
• Uvavanyo lomsebenzi wePulmonary ukulinganisa ukuphefumla kunye nendlela esebenza kakuhle ngayo imiphunga
• Uvavanyo lwe-Edrophonium ukubona ukuba eli yeza liyazibuyisa iimpawu okwexeshana

Akukho lunyango lonyango lwe-myasthenia gravis. Unyango lunokuvumela ukuba ube namaxesha ngaphandle kweempawu (uxolelo).

Ukutshintsha indlela yokuphila kunokukunceda uqhubeke nemisebenzi yakho yemihla ngemihla. Kunokucetyiswa oku kulandelayo:

• Ukuphumla kulo lonke usuku
• Sebenzisa isikhewu wamehlo ukuba umbono ophindwe kabini uyasokolisa
• Ukuphepha uxinzelelo kunye nokuvezwa kobushushu, okunokwenza ukuba iimpawu zibembi

Amayeza anokumiselwa abandakanya:

• Neostigmine okanye pyridostigmine ukuphucula unxibelelwano phakathi kwemithambo-luvo kunye nezihlunu
• I-Prednisone kunye nezinye iziyobisi (ezinje nge-azathioprine, cyclosporine, okanye mycophenolate mofetil) ukucinezela impendulo yomzimba xa uneempawu ezinzima kwaye amanye amayeza engakhange asebenze kakuhle.

Iimeko ezinengxaki kukuhlaselwa kobuthathaka bemisipha yokuphefumla. Olu hlaselo lunokwenzeka ngaphandle kwesilumkiso xa kusetyenziswe iyeza elininzi okanye elincinci kakhulu. Olu hlaselo luhlala lungekho ngaphezulu kweeveki ezimbalwa. Kuya kufuneka ungeniswe esibhedlele, apho unokufuna uncedo lokuphefumla ngomatshini wokuphefumla.

Inkqubo ebizwa ngokuba yi-plasmapheresis inokusetyenziselwa ukunceda ukuphelisa ingxaki. Le nkqubo ibandakanya ukususwa kwenxalenye esegazini (iplasma), equlethe izilwa-buhlungu. Oku kuthatyathelw 'indawo yiplasma engenikelwayo engenazintsholongwane, okanye ezinye iziselo. I-Plasmapheresis inokunceda ekunciphiseni iimpawu kwiiveki ezi-4 ukuya kwezi-6 kwaye ihlala isetyenziswa ngaphambi kotyando.

Iyeza elibizwa ngokuba yi-intravenous immunoglobulin (IVIg) linokusetyenziswa

Ugqirha lokususa i-thymus (thymectomy) kunokubangela ukuxolelwa ngokusisigxina okanye isidingo esincinci samayeza, ngakumbi xa kukho ithumba elikhoyo.

Ukuba unengxaki yamehlo, ugqirha wakho unokucebisa iilensi prism ukuphucula umbono. Unyango lunokucetyiswa ukuba unyange izihlunu zamehlo akho.

Unyango lomzimba lunokunceda ukugcina amandla akho emisipha. Oku kubaluleke ngakumbi kwimisipha exhasa ukuphefumla.

Amanye amayeza anokuzenza mandundu iimpawu kwaye kufuneka kuthintelwe. Ngaphambi kokuba uthathe naliphi na iyeza, buza ugqirha wakho ukuba kulungile na ukuba uwasele.

Ungaluphelisa uxinzelelo lokugula ngokujoyina iqela lenkxaso ye-myasthenia gravis. Ukwabelana nabanye abanamava afanayo kunye neengxaki kunokukunceda ungaziva ulilolo.

Akukho lunyango, kodwa uxolelo lwexesha elide lunokwenzeka. Kuya kufuneka uthintele imisebenzi ethile yemihla ngemihla. Abantu abaneempawu zamehlo kuphela (i-ocular myasthenia gravis), banokuphuhlisa i-myasthenia ngokubanzi ngokuhamba kwexesha.

Umfazi one-myasthenia gravis unokukhulelwa, kodwa ukhathalelo lwangaphambi kokubeleka kubalulekile. Usana lunokuba buthathaka kwaye lufuna amayeza iiveki ezimbalwa emva kokuzalwa, kodwa ngesiqhelo aluyi kuba nesi sifo.

Le meko inokubangela iingxaki zokuphefumla ezisongela ubomi. Oku kubizwa ngokuba yingxaki ye-myasthenic.

Abantu abane-myasthenia gravis basemngciphekweni ophezulu wokunye ukuphazamiseka komzimba, njenge-thyrotoxicosis, i-rheumatoid arthritis, kunye ne-systemic lupus erythematosus (lupus).

Fowunela umboneleli wakho wokhathalelo lwempilo ukuba uvelisa iimpawu ze-myasthenia gravis.

Yiya kwigumbi likaxakeka okanye fowunela inombolo yongxamiseko yendawo (enje nge-911) ukuba unengxaki yokuphefumla okanye unengxaki yokuginya.

Ingxaki ye-Neuromuscular - myasthenia gravis

• Imisipha yangaphandle yangaphandle
• I-Ptosis-ukugoba kwenkophe
• Inkqubo ye-nervous central kunye ne-peripheral system

Utshintsho CWJ. IMyasthenia gravis kunye nesifo sikaGuillain-Barré. Ku: Parrillo JE, Dellinger RP, ii-eds. Unyango oluKhathalelayo oluBalulekileyo: Imigaqo yokuchongwa kunye noLawulo kuLuntu oluDala. Umhla we-5. IPhiladelphia, PA: Elsevier; I-2019: isahluko 61.

ISanders DB, iGuptill JT. Ukuphazamiseka kokuhanjiswa kwe-neuromuscular. Ku: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, ii-eds. I-Neurology kaBradley kwiKlinikhi yokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; Ngo-2016: isahluko 109.

ISanders DB, iWolfe GI, uBenatar M, et al. Isikhokelo semvumelwano yamazwe aphesheya kulawulo lwe-myasthenia gravis: isishwankathelo sesigqeba. Neurology. Ngo-2016; 87 (4): 419-425. IINKCUKACHA: 27358333 www.ncbi.nlm.nih.gov/pubmed/27358333.