Isifo seLambert-Eaton
I-Lambert-Eaton syndrome (i-LES) sisifo esinqabileyo apho unxibelelwano oluphosakeleyo phakathi kwemithambo kunye nezihlunu lukhokelela kubuthathaka bemisipha.
I-LES kukuphazamiseka komzimba. Oku kuthetha ukuba amajoni akho omzimba ajonge ngempazamo iiseli kunye nezicubu emzimbeni. Nge-LES, ii-antibodies eziveliswa ngamajoni omzimba ahlasela iiseli zemithambo-luvo. Oku kwenza iiseli zemithambo-luvo zingakwazi ukukhupha ngokwaneleyo imichiza ebizwa ngokuba yi-acetylcholine. Le khemikhali ihambisa iimvakalelo phakathi kwemithambo-luvo kunye nezihlunu. Isiphumo kukuba buthathaka kwezihlunu.
I-LES inokuthi ivele ngomhlaza njengomhlaza omncinci wesifo somhlaza okanye ukuphazamiseka komzimba njenge-vitiligo, ekhokelela ekulahlekelweni ngumbala wesikhumba.
I-LES ichaphazela amadoda rhoqo kunabafazi. Ubudala obuqhelekileyo bokwenzeka kweminyaka engama-60. I-LES inqabile ebantwaneni.
Ubuthathaka okanye ukuphulukana nentshukumo enokuba ngaphezulu okanye ngaphantsi kakhulu, kubandakanya:
- Kunzima ukunyuka izinyuko, ukuhamba, okanye ukuphakamisa izinto
- Intlungu yezihlunu
- Ukujongwa kwentloko
- Isidingo sokusebenzisa izandla ukuvuka kwindawo ehleliyo okanye eleleyo
- Iingxaki ukuthetha
- Iingxaki zokuhlafuna okanye ukugwinya, ezinokubandakanya ukugxothwa okanye ukukrwitshwa
- Umbono utshintsha, njengombono ongacacanga, umbono ophindwe kabini, kunye nengxaki yokugcina ujongano oluzinzileyo
Ubuthathaka ngokubanzi buthathaka kwi-LES. Imisipha yomlenze ichaphazeleka kakhulu. Ubuthathaka bunokuphucuka emva kokuzilolonga, kodwa ukuzingisa okuqhubekayo kubangela ukudinwa kwezinye iimeko.
Iimpawu ezinxulumene namanye amalungu enkqubo ye-neva zihlala zenzeka, kwaye zibandakanya:
- Uxinzelelo lwegazi luyatshintsha
- Ukuba nesiyezi xa umi
- Umlomo owomileyo
- Ukungasebenzi kakuhle kwe-Erectile
- Amehlo omileyo
- Ukuqunjelwa
- Ukwehla kokubila
Umboneleli wezempilo uya kwenza uvavanyo lomzimba kwaye abuze malunga neempawu. Uviwo lunokubonisa:
- Ukuhla kwengqondo
- Ukuphulukana okunokwenzeka kwezihlunu zemisipha
- Ubuthathaka okanye ukukhubazeka okuba ngcono kancinci kunye nomsebenzi
Uvavanyo lokunceda ukufumanisa isifo kunye nokuqinisekisa i-LES inokubandakanya:
- Uvavanyo lwegazi ukukhangela izilwa-buhlungu ezihlasela imithambo-luvo
- I-Electromyography (EMG) yokuvavanya impilo yemicu yemisipha
- Ukuhamba kwesantya (NCV) ukuvavanya isantya sombane kunye ne-nerves
Ukuvavanywa kwe-CT kunye neMRI yesifuba kunye nesisu, kulandele i-bronchoscopy yabantu abatshayayo kunokwenziwa ngaphandle komhlaza. Ukuskena i-PET kungenziwa kwakhona ukuba kurhanelwa ithumba lemiphunga.
Iinjongo eziphambili zonyango kukuba:
- Chonga kwaye uphathe naziphi na iingxaki ezinje, njengomhlaza wemiphunga
- Nika unyango ukunceda ngobuthathaka
Ukutshintshiselana ngePlasma, okanye iplasmapheresis, lunyango olunceda ukususa emzimbeni naziphi na iiproteni ezinobungozi (ii-antibodies) eziphazamisa ukusebenza kwentliziyo. Oku kubandakanya ukususa i-plasma yegazi enee-antibodies. Ezinye iiproteni (ezinjengealbumin) okanye iplasma enikelweyo emva koko ifakwe emzimbeni.
Enye inkqubo ibandakanya ukusebenzisa i-intravenous immunoglobulin (IVIg) ukufaka isixa esikhulu sezinto zokulwa ngqo egazini.
Amayeza anokuzama kwakhona aquka:
- Iziyobisi ezicinezela impendulo yenkqubo yomzimba yokuzikhusela
- Amachiza e-anticholinesterase ukuphucula ithoni yemisipha (nangona ezi zingasebenzi kakuhle xa zinikwa zodwa)
- Iziyobisi ezonyusa ukukhutshwa kwe-acetylcholine kwiiseli zemithambo-luvo
Iimpawu ze-LES zinokuphucula ngokunyanga isifo esingaphantsi, ukucinezela amajoni omzimba, okanye ukususa ii-antibodies. Nangona kunjalo, i-paraneoplastic LES ayinakuphendula ngokunjalo kunyango. (Iimpawu ze-Paraneoplastic LES zibangelwa kukutshintsha kwenkqubo yomzimba yokuzikhusela kwisisu). Ukufa kubangelwa sisifo esibi.
Iingxaki ze-LES zingabandakanya:
- Ubunzima bokuphefumla, kubandakanya ukusilela kokuphefumla (okuqhelekileyo)
- Kunzima ukugwinya
- Usulelo, njenge-pneumonia
- Ukulimala kokuwa kunye neengxaki ngokulungelelanisa
Shayela umnikezeli wakho ukuba iimpawu ze-LES zikhula.
Isifo seMyasthenic; Isifo se-Eaton-Lambert; Isifo seLambert-Eaton myasthenic; IIMPAWU; LES
- Imisipha yangaphandle yangaphandle
U-Evoli A, uVincent A. Ukuphazamiseka kokuhanjiswa kwe-neuromuscular. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 394.
Moss HE. Ukuphazamiseka kwamehlo kunye nobuso. Ku: Liu GT, Volpe NJ, Galetta SL, ii-eds. Liu, Volpe, kunye neGaletta's Neuro-Ophthalmology. Ngomhla wesi-3. IPhiladelphia, PA: Elsevier; I-2019: isahluko 14.
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.