Myasthenia Gravis
Uhlolojikelele
I-Myasthenia gravis (my-us-THEE-nee-uh GRAY-vis) ibangela ukuba imisipha ephilile ngokuzithandela ibe buthakathaka futhi ikhathale ngokushesha. Lokhu kwenzeka lapho ukuxhumana phakathi kwama-nerve namisipha kuwohloka.
Ayikho imithi yokwelapha i-myasthenia gravis. Ukwelashwa kungasiza ngezimpawu. Lezi zimpawu zingafaka ubuthakathaka bemisipha yesandla noma olunye uhlangothi, ukubona okukabili, amehlo awohlokayo, nezingqinamba zokukhuluma, ukukoma, ukugwinya nokuphefumula.
Lesi sifo singathinta abantu banoma iyiphi iminyaka, kodwa sivame kakhulu kubesifazane abaneminyaka engaphansi kuka-40 kanye namadoda aneminyaka engaphezu kuka-60.
Izimpawu
Ubuthakathaka bemisipha obubangelwa yi-myasthenia gravis buya bube kubi uma umsipha othombekile usetshenziswa. Ngenxa yokuthi izimpawu zivame ukuthuthuka ngokuphumula, ubuthakathaka bemisipha bungafika futhi buhambe. Noma kunjalo, izimpawu zithambekele ekukhuleni ngokuhamba kwesikhathi. Zivame ukufinyelela okubi kakhulu phakathi neminyaka embalwa ngemuva kokuba isifo siqale. I-Myasthenia gravis ingathinta noma yimiphi imisipha ongayilawula. Amaqembu athile emisipha athinteka kakhulu kunabanye. Kubantu abangaphezu kwengxenye abathuthukisa i-myasthenia gravis, izimpawu zabo zokuqala zithinta amehlo. Izimpawu zihlanganisa: Ukwehlela kweliso elilodwa noma womabili, okuthiwa yi-ptosis. Ukubona okubili, okuthiwa yi-diplopia, okungaba ubude noma ukuphakama, futhi kuthuthuka noma kuphele uma iliso elilodwa livaliwe. Cishe ku-15% yabantu abane-myasthenia gravis, izimpawu zokuqala zihilela imisipha yobuso nomlomo. Lezi zimpawu zingenza: Ukwenza ukukhuluma kube nzima. Inkulumo yakho ingase izwakale ithobekile noma ibe ngaphansi, kuye ngokuthi yimiphi imisipha ethintekayo. Ukubangela izinkinga zokugwinya. Ungase ukhwehle kakhulu, okwenza kube nzima ukudla, ukuphuza noma ukuthatha amaphilisi. Ngezinye izikhathi, izinto eziphuzwayo ozimathisayo ziphuma ekhaleni lakho. Ukuthinta ukukoma. Imisipha esetshenziswa ekukomeni ingase ikhathale phakathi kokudla. Lokhu kuyiqiniso ikakhulukazi uma udle into enzima ukuyikhoma, njenge-steak. Ushintsho lobuso. Isibonelo, ukuhleka kwakho kungase kubukeke njengokubhonga. I-Myasthenia gravis ingabangela nobuthakathaka entanyeni, ezingalweni nasezinzwaneni. Ubuthakathaka ezinzwaneni bungathinta indlela ohamba ngayo. Imisipha yentanyeni ebuthakathaka yenza kube nzima ukuthwala ikhanda. Xhumana nodokotela wakho uma unezinkinga: Zokuphefumula. Zokubona. Zokugwinya. Zokukoma. Zokuhamba. Zokusebenzisa izingalo noma izandla. Zokuphakamisa ikhanda lakho.
Isikhathi sokubona udokotela
Xhumana nodokotela wakho uma unezinkinga:
- Ukuthandaza.
- Ukubona.
- Ukugwinya.
- Ukukoma.
- Ukuhamba.
- Ukusebenzisa izandla zakho noma izingalo.
- Ukuphakamisa ikhanda lakho.
Izimbangela
Izithunywa zekhemikhali, ebizwa ngokuthi i-neurotransmitters, zifanelana ngokuqondile ezindaweni zokwamukela kumaseli akho emisipha. Ku-myasthenia gravis, ezinye izindawo zokwamukela zivaliwe noma zibhujiswe, okubangela ubuthakathaka bemisipha.\n\nAma-nerve akho axhumana nemisipha yakho ngokukhulula amakhemikhali, abizwa ngokuthi yi-neurotransmitters, afaneleka ezindaweni kumaseli emisipha, ebizwa ngokuthi izindawo zokwamukela, ekuhlanganeni kwemisipha yezinzwa.\n\nKu-myasthenia gravis, uhlelo lokukhulula lwenza ama-antibodies avimba noma abhubhise izindawo eziningi zokwamukela imisipha yakho ye-neurotransmitter ebizwa ngokuthi i-acetylcholine (as-uh-teel-KOH-leen). Ngezindawo ezimbalwa zokwamukela ezitholakalayo, imisipha yakho ithola izibonakaliso zezinzwa ezimbalwa. Lokhu kubangela ubuthakathaka.\n\nAma-antibodies angavimba iprotheni ebizwa ngokuthi i-muscle-specific receptor tyrosine kinase (TIE-roh-seen KIE-nays), ngezinye izikhathi ibhekwa njenge-MuSK. Le protheni iyasiza ukwakha i-nerve-muscle junction. Ama-antibodies aphikisana nale protheni angholela ku-myasthenia gravis.\n\nAma-antibodies aphikisana nenye iprotheni, ebizwa ngokuthi i-lipoprotein-related protein 4 (LRP4), angadlala indima kule simo. Izifundo zocwaningo zithole amanye ama-antibodies nenani lama-antibodies elihilelekile cishe lizokhula ngokuhamba kwesikhathi.\n\nabantu abanye bane-myasthenia gravis engabangelwa ama-antibodies avimba i-acetylcholine, i-MuSK noma i-LRP4. Lolu hlobo lwe-myasthenia gravis lubizwa ngokuthi yi-seronegative myasthenia gravis, okuyaziwa nangokuthi yi-antibody-negative myasthenia gravis. Ngokuvamile, abacwaningi bakholelwa ukuthi lolu hlobo lwe-myasthenia gravis lusavela enkingeni yokungaziphathi kahle komzimba, kodwa ama-antibodies ahilelekile awatholakali okwamanje.\n\nIglands ye-thymus, ingxenye yohlelo lwakho lokukhulula olukhona esifubeni esiphezulu ngaphansi kwesifuba, ingaqalisa noma igcine ukukhiqizwa kwama-antibodies okuholela ebuthakathakeni bemisipha.\n\nIglands ye-thymus ingxenye yohlelo lwakho lokukhulula. Le glands itholakala esifubeni esiphezulu ngaphansi kwesifuba. Abacwaningi bakholelwa ukuthi i-thymus gland yenza noma iyasisiza ukukhiqiza ama-antibodies avimba i-acetylcholine.\n\nIglands ye-thymus inkulu kubantwana futhi incinyane kubantu abadala abaphilile. Kubantu abadala abane-myasthenia gravis, noma kunjalo, i-thymus gland inkulu kunokujwayelekile. Abanye abantu abane-myasthenia gravis banemikhuhlane ye-thymus gland, ebizwa ngokuthi yi-thymomas. Ngokuvamile, i-thymomas ayiyona umdlavuza, okuyaziwa nangokuthi yi-malignant. Kodwa i-thymomas ingaba umdlavuza.\n\nNgokungavamile, omama abane-myasthenia gravis banezingane ezizalwa ne-myasthenia gravis. Lokhu kubizwa ngokuthi yi-neonatal myasthenia gravis. Uma kuphathwa ngokushesha, izingane ngokuvamile ziyaphila phakathi nezinyanga ezimbili ngemva kokuzalwa.\n\nEzinye izingane zizalwa ne-myasthenia gravis ehlukile, elidluliselwa ezizukulwaneni, ebizwa ngokuthi yi-congenital myasthenic syndrome.\n\Izinto ezingenza i-myasthenia gravis ibe mbi kakhulu zihlanganisa:\n\n- Ukukhathala.\n- Ukugula noma ukutheleleka.\n- Ukuhlinzwa.\n- Ukuqina kwengqondo.\n- Eminye imithi — njenge-beta blockers, i-quinidine gluconate, i-quinidine sulfate, i-quinine (Qualaquin), i-phenytoin (Dilantin), ezinye izidakamizwa kanye nezinye izidakamizwa.\n- Ukukhulelwa.\n- Izikhathi zenyanga.
Izingqinamba
Izinkinga ze-myasthenia gravis zingathathwa, kodwa ezinye zingaba yingozi eyingozi. Ukuphahlazeka kwe-myasthenic kuyisimo esiyingozi eyingozi. Kwenzeka lapho imisipha elawula ukuphefumula ihluleka ukusebenza. Ukwelashwa okusheshayo nokusiza ngomshini ekuphefumuleni kuyadingeka. Imithi nezinye izindlela zokwelapha ezihlanza igazi zisiza abantu ukuba baphefumule bodwa. Abanye abantu abane-myasthenia gravis bane-tumor e-thymus gland. I-thymus yigland engaphansi kwesifuba esiyinqobo yesistimu yokuvimba. Iningi lalezi tumors, ebizwa ngokuthi yi-thymomas, azinawo umdlavuza. Abantu abane-myasthenia gravis banesihlobo esikhulu sokuba nalezi zimo:
- I-thyroid ehambayo noma esebenza kakhulu. I-thyroid gland ebusweni ikhipha ama-hormone alawula ukuguqulwa kwemvelo. Uma i-thyroid ingasebenzi kahle, ungaba nezinkinga zokubhekana nobusika, ukwanda kwesisindo nezinye izinkinga. I-thyroid esebenza kakhulu ingabangela izinkinga zokubhekana nokushisa, ukulahlekelwa isisindo nezinye izinkinga.
- Izimo ze-autoimmune. Abantu abane-myasthenia gravis bangaba nesihlobo esikhulu sokuba nezimo ze-autoimmune, njenge-rheumatoid arthritis noma i-lupus.
Ukuxilongwa
Umhlinzeki wakho wezempilo uzobheka izimpawu zakho kanye nomlando wakho wezokwelapha futhi enze ukuhlolwa komzimba. Umhlinzeki wakho angase asebenzise ukuhlolwa okuningi, okuhlanganisa:
Umhlinzeki wakho angase ahlole impilo yakho yezinzwa ngokuhlola:
- Ukubonakaliswa kwezinzwa.
- Amandla ezinzwa.
- Ukuma kwezinzwa.
- Izinzwa zokuthinta nokubona.
- Ukuhlanganiswa.
- Ukulinganisela.
Ukuhlolwa okuzokusiza ukuqinisekisa ukuxilongwa kwe-myasthenia gravis kungafaka:
Uma unelephu eliqandayo, umhlinzeki wakho angase afake isikhwama esigcwele uqhwa elephwini lakho. Ngemuva kwemizuzu emibili, umhlinzeki wakho ususa isikhwama bese ehlola ilehphu lakho eliqandayo ukuze abone ukuthuthuka.
Ukuhlolwa kwegazi kungase kubonise ama-antibodies angavamile ahlanganisa izindawo zokwamukela lapho izinzwa zibika khona izicubu zakho ukuthi zihambe.
Kuloluvivinyo lokuqhuba kwezinzwa, abahlinzeki banamathela ama-electrodes ezikhwameni zakho phezu kwezinzwa ezizohlolwa. Ama-pulses amancane kagesi aqhubeka ngama-electrodes. La ma-pulses alawula ukuthi inzwa ingathumela isignali esicubwini yini.
Phakathi nalesi sivivinyo, inzwa ihlolwa izikhathi eziningi ukuze kubonwe ukuthi ikhono layo lokuthumela iziginali libi kakhulu ngokukhathala yini. Imiphumela yalesi sivivinyo ikusiza ukwazisa ukuxilongwa kwe-myasthenia gravis.
Lesi sivivinyo silinganisa umsebenzi kagesi ohamba phakathi kobuchopho bakho kanye nezinzwa zakho. Kuhilela ukufaka i-electrode yentambo enhle ngesikhumba sakho nangaphakathi kwesicubu ukuze uhlole uboya olulodwa lwesicubu.
Umhlinzeki wakho angase ayalele ukuhlolwa kwe-CT noma i-MRI ukuze ahlole ukuthi kukhona i-tumor noma enye inkinga nge-thymus yakho yini.
Lezi zivivinyo zilinganisa ukuthi isimo sakho siyaphazamisa ukuphefumula kwakho yini.
Ukwelashwa
Izindlela zokwelapha ezihlukahlukene, zodwa noma ndawonye, zingasiza ngezimpawu ze-myasthenia gravis. Ukwelashwa kwakho kuzoxhomeka ebudaleni bakho, ukuthi isifo sakho sibukhali kangakanani nokuthi siqhubeka ngokushesha kangakanani.
- Abaphikisi be-Cholinesterase. Imithi enjenge-pyridostigmine (Mestinon, Regonal) ilungisa ukuxhumana phakathi kwezinzwa nemisipha. Le mithi ayilona ikhambi, kodwa ingathuthukisa ukuncika kwemisipha namandla emisipha kwabanye abantu.
Imiphumela emibi engenzeka ihlanganisa ukuphazamiseka kokugaya ukudla, isifo sohudo, isicanucanu, nokukhuluphala kokukhipha umalume nokwehla.
- I-Corticosteroids. I-Corticosteroids enjenge-prednisone (Rayos) ivala isimiso somzimba, imenze angakwazi ukukhiqiza ama-antibodies. Nokho, ukusetshenziswa kwe-corticosteroids isikhathi eside, kungabangela imiphumela emibi enzima. Lokhu kuhlanganisa ukuncipha kwama-bone, ukwanda kobunzima, isifo sikashukela kanye nenkinga enkulu yokutheleleka okuthile.
Abaphikisi be-Cholinesterase. Imithi enjenge-pyridostigmine (Mestinon, Regonal) ilungisa ukuxhumana phakathi kwezinzwa nemisipha. Le mithi ayilona ikhambi, kodwa ingathuthukisa ukuncika kwemisipha namandla emisipha kwabanye abantu.
Imiphumela emibi engenzeka ihlanganisa ukuphazamiseka kokugaya ukudla, isifo sohudo, isicanucanu, nokukhuluphala kokukhipha umalume nokwehla.
Lezi zindlela zokwelapha zivame ukusetshenziswa isikhathi esifushane sokwelapha izimpawu eziqala ngokuzumayo noma ngaphambi kokuthi kwenziwe ubuyekezo noma ezinye izindlela zokwelapha.
- I-Intravenous immunoglobulin (IVIg). Lolu lwelo lwemfundo lunikeza umzimba wakho ama-antibodies ajwayelekile, okuthinta impendulo yesistimu yakho yokuvimba. Izinzuzo zivame ukubonwa ngaphansi kweviki futhi zingagcina amasonto angu-3 kuya kwangu-6.
- I-Monoclonal antibody. I-Rituximab (Rituxan) ne-eculizumab (Soliris) yimithi enikezwa ngomqondo we-myasthenia gravis. Le mithi ivame ukusetshenziswa lapho ezinye izindlela zokwelapha zingasebenzi. Zingaba nemiphumela emibi enzima.
I-Plasmapheresis (plaz-muh-fuh-REE-sis). Le nqubo isebenzisa inqubo yokucwecwa efana ne-dialysis. Igazi lakho libekwa emishini esusa ama-antibodies avimba ukudluliswa kwemizwa evela ezipheleni zezinzwa zakho kuya emisipha yakho. Kodwa-ke, imiphumela emihle yale nqubo ivame ukugcina amasonto ambalwa nje. Ukuba nezinqubo eziningi kungabangela izinkinga zokuthola ama-veins wokwelashwa.
I-Intravenous immunoglobulin (IVIg). Lolu lwelo lwemfundo lunikeza umzimba wakho ama-antibodies ajwayelekile, okuthinta impendulo yesistimu yakho yokuvimba. Izinzuzo zivame ukubonwa ngaphansi kweviki futhi zingagcina amasonto angu-3 kuya kwangu-6.
Imiphumela emibi, evame ukuba mncane, ingaba yizikhathi zokubanda, ukukhungatheka, izikhathi zokukhathazeka nokugcinwa kwamanzi.
Abanye abantu abane-myasthenia gravis bane-tumor e-thymus gland. Uma une-tumor, ebizwa nge-thymoma, uzodinga ukuhlinzwa ukuze ususe i-thymus gland, ebizwa nge-thymectomy.
Ngisho noma ungenayo i-tumor e-thymus gland, ukususa i-gland kungathuthukisa izimpawu zakho. Kodwa-ke, izinzuzo zale nqubo zingathatha iminyaka ukuze zithuthuke.
I-thymectomy ingenziwa njengokuhlinzwa okuvulekile noma njengokuhlinzwa okuncane. Ekuhlinzweni okuvulekile, udokotela uhlukanisa i-breastbone ephakathi, ebizwa nge-sternum,) ukuvula isifuba bese ususa i-thymus gland.
Ukuhlinzwa okuncane ukususa i-thymus gland kusebenzisa izikhafu ezincane, ebizwa ngokuthi yi-incisions. Kungase futhi kuhlanganise:
- I-Video-assisted thymectomy. Kwenye indlela yale nqubo, ochwepheshe bokuhlinzwa benza ukuvulwa okuncane entanyeni noma ukuvulwa okuncane eceleni kwesifuba. Bese basebenzisa ikhamera ende, encane, ebizwa nge-video endoscope, namathuluzi amancane ukubona nokususa i-thymus gland.
- I-Robot-assisted thymectomy. Kule ndlela ye-thymectomy, ochwepheshe bokuhlinzwa benza ukuvulwa okuncane eceleni kwesifuba. Basebenzisa uhlelo lwe-robotic ukususa i-thymus gland. Lolu hlelo luhlanganisa i-camera arm nama-arms omishini.
Umshwana wokuzihlangula: I-August iyinkundla yolwazi lwezempilo futhi izimpendulo zayo azihlanganisi iseluleko sezokwelapha. Njalo thintana nochwepheshe bezokwelapha onelayisensi eduze nawe ngaphambi kokwenza noma yiluphi ushintsho.