Masifunde ngale meko iyingozi eyenzeka ngequbuliso ngexesha lotyando: I-Malignant Hyperthermia!

Ukuba wena okanye umntu omaziyo kufuneka enziwe utyando, mhlawumbi ukhe waliva igama elithi i-anesthesia. Lithetha ukuba siyalaliswa ngexesha lotyando. Kodwa ngaba wakha wacinga ukuba abanye abantu banokuba nempendulo eyingozi kakhulu, esongela ubomi kula mayeza e-anesthetic? Leyo yenye yeemeko ezingaqhelekanga, kodwa ezinzulu kakhulu ezibizwa ngokuba yi -Malignant Hyperthermia. Eli gama lisenokuvakala lisoyikisa kancinci, kodwa ukuba unolwazi olufanelekileyo ngalo, iingxaki ezininzi zinokuphetshwa. Ngoko ke, masithethe ngalo ngokweenkcukacha namhlanje.

Yintoni iMalignant Hyperthermia?

Ngamafutshane, 'iMalignant Hyperthermia' yimeko yemfuza . Oko kukuthi, ibangelwa kukuguquka kwezakhi zofuzo zethu. Xa umntu onesi sifo enikwa amayeza athile okuthomalalisa umzimba, umzimba wakhe usabela kakubi kakhulu. Le yimeko engxamisekileyo enokuba yingozi nakubomi.

Eyona nto ilungileyo kukuba, nangona oku kudla ngokuba yinto edluliselwa kwizizukulwana ngezizukulwana, kukho iindlela zokunyanga . Kwaye, ukuba uyazi ngale ngozi kwangethuba, ungazikhusela kuyo.

Siyazi ukuba xa sinikwa i-anesthesia eqhelekileyo, asiva nto ngexesha lotyando. Kukho iintlobo ngeentlobo zamayeza okubonelela ngolu hlobo lwe-anesthesia. Nangona kunjalo, zimbalwa kakhulu ezo ziyobisi ezibangela le meko ebizwa ngokuba yi-'Malignant Hyperthermia'. Uninzi lwamayeza e-anesthetic alubangeli oku.

Enye into ebalulekileyo kukuba ngamanye amaxesha umntu usenokungazi ukuba une-genetic mutation enokubangela i-Malignant Hyperthermia, kuba usenokungabonakalisi zimpawu de abe uchaphazeleke kumayeza athile akhankanyiweyo.

Ngubani onokuthi aphuhlise oku ngakumbi?

Makhe sibone ukuba ngubani osengozini enkulu yokufumana le meko 'ye-Malignant Hyperthermia'.

Ngokwenyani, ukuba kukho umntu kusapho lwakho okhe waba nesifo se-'Malignant Hyperthermia' ngaphambili , ngoko ke namanye amalungu alo sapho anokuba sengozini. Ngokukodwa, ukuba isalamane sakho esikwinqanaba lokuqala (umz. umama, utata, abantakwenu, abantwana) sibe nale meko, uthathwa njengomntu 'onokuchaphazeleka'.

Ngoko ke, ukuba kufuneka utyando, kubaluleke kakhulu ukwazisa i-anesthesiologist ukuba kukho umntu oye wehlelwa yinto efana nale kusapho lwakho.

Khawucinge nje, masithi usapho lwakho alukaze lube nempendulo ethi `Malignant Hyperthermia` ngolu hlobo. Nangona kunjalo, abantu abanezifo ezithile zemisipha nabo banokuba semngciphekweni omkhulu. Ezinye zezi zifo zezi:

• `Isifo esiphakathi`
• `Isifo se-Multiminicore`
• `Isifo sikaKing-Denborough`
• `Ingxaki ye-STAC3`

Nangona oku kungabonakala kuyinkimbinkimbi kancinci, kulungile ukuba abo banezi meko baqaphele oku.

Ixhaphake kangakanani le meko?

Ixhaphake kangakanani le 'Malignant Hyperthermia'? Nangona izibalo zixubene kancinci, ngokubanzi kuqikelelwa ukuba le meko inokwenzeka kutyando olunye kubantu abadala abayi-100,000. Phakathi kwabantwana, eli nani limalunga notyando olunye kubantu abayi-30,000. Kuyaphawuleka nokuba isiqingatha sezigulane ezichongiweyo zingaphantsi kweminyaka eli-19 ubudala.

Kodwa izazinzulu zicinga ukuba abantu abachaphazeleka yile meko 'ye-Malignant Hyperthermia' banokuba sengozini enkulu yokuba phakathi koluntu. Ngenxa yokuba abantu abaninzi abachatshazelwanga ngamayeza athile abangela le meko ubomi babo bonke, basenokungazi nokuba basengozini.

Zithini iimpawu zoku?

Iimpawu ze-'Malignant Hyperthermia' zinokwahluka kancinci kumntu nomntu. Zinokuvela ngexesha lotyando okanye ngelixa usaphila emva kotyando. Umzekelo, ukuba uphantsi kwe-anesthesia kwaye ezi mpawu zibonakala ngexesha lotyando, awuyi kuziva. Nangona kunjalo, ingcali yakho ye-anesthesia okanye umongikazi we-anesthesia ulumkile kakhulu ngale nto. Bajonga wonke umntu ophantsi kwe-anesthesia ngononophelo. Nje ukuba baqaphele ezi mpawu, baya kuqala ngoko nangoko unyango lwe-'Malignant Hyperthermia'.

Iimpawu zokuqala

Ezi zizibonakaliso zokuqala onokuzibona:

• Ukunyuka kwesantya sentliziyo okungachazekiyo (i-tachycardia). Isantya sentliziyo siyanda ngequbuliso.
• Ukwanda ngequbuliso komthamo wekhabhoni dayokhsayidi ekhutshwa emzimbeni . Oku kunokunyangeka nangezixhobo zonyango.
• Ukunyuka kwesantya sokuphefumla (i-tachypnea). Uqala ukuphefumla ngokukhawuleza, ngokungathi uphefumla nzima.
• Ukuqina kwemisipha, ukuqina kwemisipha (`ukuqina kwemisipha`). Imisipha emzimbeni iba lukhuni ngokungathi ityunyuzwa lilitye.
• Ukunyuka ngequbuliso kobushushu bomzimba . Umzimba uqala ukufudumala.

Iimpawu ezibonakala kamva

Njengoko imeko isiya isiba mandundu, unokubona neempawu ezifana nezi:

• Ukunyuka kobushushu bomzimba ngaphezu komkhuhlane oqhelekileyo .
• Umchamo omnyama . Usenokude ube mdaka.
• Uvavanyo lwegazi lubonisa ukuwohloka kwemisipha .
• Ukungalingani kwesingqisho sentliziyo (arrhythmia). Intliziyo ibetha ngendlela engaqhelekanga.
• Ukopha .
• Iimeko ezinje ngokuxhuzula .

Kutheni kukho i-hyperthermia embi?

Ngoku makhe sibone ukuba kutheni le meko ibizwa ngokuba yi-'Malignant Hyperthermia' isenzeka.

Abantu abanale meko baneproteni engaqhelekanga kwiiseli zabo zemisipha. Oku kungenxa yotshintsho kwiijini zabo, olubizwa ngokuba yi-genetic mutation .

Ngamafutshane, i-DNA yethu ifana "nencwadi yemiyalelo" equlethe ulwazi olufunekayo kwiiseli zomzimba wethu ukuze zenze imisebenzi yazo. Ukuba kukho ulwazi olungekhoyo ndaweni ithile kule DNA, okanye ukuba kukho umonakalo kwinto ethile, kulapho ke kuvela khona izifo zemfuza ezifana nezi.

Olu tshintsho lwemfuza alubangeli zimpawu ebomini obuqhelekileyo. Nangona kunjalo, ukuba umntu onolu tshintsho uchaphazeleka ngamayeza athile okuthomalalisa iintlungu akhankanyiweyo apha ngasentla - okanye, kunqabile kakhulu, ukuba uchaphazeleka ngamaqondo obushushu aphezulu okanye ukuba wenza umthambo onzima - iiseli zakhe zemisipha ziqala ukukhupha i-calcium ngendlela engaqhelekanga.

Ngenxa yoko, izihlunu ziyaqhubeka zincipha kwaye ziqine okanye ziqine, imetabolism yomzimba kunye nobushushu bomzimba buyanda ngendlela engaqhelekanga. Ekugqibeleni, iiseli zemisipha ziyafa, kwaye inani elikhulu le-potassium liqokelelana egazini. Oku kunokukhokelela kwiimpawu ezingaphezulu kunye neengxaki.

Amayeza okubulala iintlungu angabangela le meko.

Umntu onesifo se-'Malignant Hyperthermia' ngokwemfuza unokufumana le mpendulo ukuba unikwa la mayeza alandelayo e-anesthetic:

Amayeza afunxwayo:

• `IHalothane`
• `Desflurane`
• `Sevoflurane`
• `I-Isoflurane`

Amayeza afakwa kwimithambo yegazi:

• I-Succinylcholine – Le yi-relaxative yemisipha esebenza ngokukhawuleza.

Uyibona njani le nto?

Ukuba utyando usebenzisa i-anesthetic eyingozi ungazi ukuba unesifo se-'Malignant Hyperthermia', wena okanye i-anesthesiologist aniyi kwazi ukuba unayo de kube emva kokuba kwenzeke ukusabela.

Nangona kunjalo, xa wena okanye omnye umntu enikwa i-anesthesia, i-anesthesiologist okanye umongikazi uya kujonga iimpawu zakho ezibalulekileyo nempilo yakho ngexesha nasemva kotyando. Ukuba une-'Malignant Hyperthermia', baya kuyiqonda ngeempawu ezibalulekileyo ezibalulekileyo kunye neempendulo ezichazwe ngaphambili. Oko kukuthi:

• Ukunyuka kwesantya sentliziyo ngaphandle kwesizathu.
• Ukunyuka ngokukhawuleza kobushushu bomzimba.
• Ukwanda ngequbuliso komthamo wekhabhoni diokside ephuma emzimbeni.
• Ukuqina kwemisipha.

Iingcali zokubulala iintlungu ziqeqeshwe ukuba ziqaphele olu tshintsho kwangethuba . Emva koko zinokusabela ngokukhawuleza kule meko ingxamisekileyo. Okukhona ukuxilongwa ngokukhawuleza, kokukhona unyango nolawulo olungakumbi lunokufezekiswa.

Ungazi njani kwangaphambili ukuba unalo mngcipheko?

Ukuba wena okanye ugqirha wakho nicinga ukuba nisesichengeni seMalignant Hyperthermia, nokuba kungenxa yembali yosapho lwakho okanye ngenxa yezinye izizathu, kukho iimvavanyo ezikhethekileyo ezinokwenziwa ukuqinisekisa oku:

• Uvavanyo lwe-caffeine halothane contracture (CHCT) : Kolu vavanyo, kuthathwa iqhekeza elincinci lemisipha yakho (i-muscle biopsy) kwaye isampuli yezicubu ivezwe kwizisombululo ze-halothane kunye ne-caffeine ukuze kubonwe ukuba iyasabela na kwigesi ye-anesthetic.
• Uvavanyo lwemfuza : Olu vavanyo lunokuchonga utshintsho kwiijini kwi-DNA ebizwa ngokuba yi-RYR1, STAC3, kunye ne-CACNA1S. Kuye kwafunyaniswa utshintsho olungaphezu kwama-45 olunokubangela i-Malignant Hyperthermia.

Nangona kunjalo, ezi mvavanyo zokufumanisa ukuba umntu unesifo se-'Malignant Hyperthermia' zibiza kakhulu, kwaye zingenziwa kuphela kwiilabhoratri ezimbalwa ezikhethekileyo. Umzekelo, uvavanyo lwe-'CHCT' lufumaneka kuphela kwiindawo ezine eMelika. Ke ngoko, ukuba ufuna utyando olungxamisekileyo kwaye isalamane esisondeleyo sikhe saba ne-'Malignant Hyperthermia' ngaphambili, iingcali ze-anesthesiologists zihlala zisebenzisa ii-anesthetics ezikhuselekileyo endaweni yezo ziyobisi ziyingozi.

Zithini iindlela zonyango zoku?

Unyango oluphambili lwe-malignant hyperthermia yi-dantrolene (igama lophawu iDantrium®).

Kwakamsinya nje xa iingcali ze-anesthesiologists zikrokrela imeko ebizwa ngokuba yi-'Malignant Hyperthermia', zinika eli yeza le-'dantrolene'. Kwangaxeshanye, ziyayeka ukunika eli yeza le-anesthetic elibangele ukusabela, kwaye ugqirha otyandayo ugqiba utyando ngokukhawuleza.

Ukongeza, iqela le-anesthesia kunye neqela lotyando likwanyanga ezinye iimpawu kunye neengxaki ezibangelwa yi-'Malignant Hyperthermia'. Ezinye zezi ndlela zonyango zezi:

• I-Hyperthermia inyangwa ngeendlela zokupholisa , ezinje ngokunika ulwelo olubandayo kunye nokusebenzisa iipakethe zomkhenkce.
• Ioksijini inikwa ngemaski yobuso okanye ngetyhubhu ebekwe kwi-trachea.
• Ukuba kukho ukubetha kwentliziyo okungahambelaniyo (arrhythmia), kunikwa amayeza ngenxa yoko.

Nje ukuba imeko yakho izinzile, uza kugcinwa kwigumbi lokhathalelo olunzulu (ICU) ubuncinane usuku lonke ukuze ujongwe . Iqela lezonyango liza kwenza olunye uvavanyo (umz. uvavanyo lwegazi) kwaye libonelele ngonyango olongezelelweyo ukuba kuyimfuneko.

Ngaba oku kungathintelwa?

I-Molignant Hyperthermia kunzima ukuyithintela, ngaphandle kokuba uyazi ukuba une-genetic mutation ebangela loo nto , okanye umntu kusapho lwakho unale meko kwaye uxelele i-anesthesiologist yakho. Kwimeko enjalo, i-anesthesiologist ayizukusebenzisa loo mayeza anobungozi kwisicwangciso sakho se-anesthesia.

Kodwa, into ebuhlungu kukuba, uninzi lwexesha abantu bafumanisa kuphela ukuba basengozini yeMalignant Hyperthermia emva kokuba yenzeke kubo.

Kwenzeka ntoni emva kwale meko? Ngaba inganyangeka?

Ukuba iimpawu ze-'Malignant Hyperthermia' zibonwa kwangethuba kwaye zinyangwa ngokufanelekileyo, kunokwenzeka ukuba umntu aphile ngokupheleleyo . Isenzo esikhawulezileyo se-anesthesia kunye neqela lotyando sibaluleke kakhulu apha.

Nangona kunjalo, simele sikhumbule ukuba ngamanye amaxesha, nokuba kunyango olukhawulezileyo, amalungu amaninzi angasebenzi kakuhle aze afe.

Ziziphi ezinye iingxaki ezinokubakho ngenxa ye-malignant hyperthermia?

I-Hyperthermia eyingozi inokubangela iingxaki ezinkulu ezifana nezi:

• Ukonakala kwemisipha
• Ukungasebenzi kakuhle kwezintso
• Ukungasebenzi kakuhle kwesibindi
• Ukopha
• Ukubanjwa kwe-cardiac
• Ukuxhuzula
• I-Coma
• Ukufa

Ngaba oku kuyabulala?

Ukuba ayinyangwa, i-'Malignant Hyperthermia' yimeko enokubangela ukufa . Nokuba unyango olufanelekileyo lufanelekile, izinga lokufa elixeliweyo liphakathi kwe-3% kunye ne-5%. Ke ngoko, oku akuyonto ifanele ithathwe lula.

Ndifanele ndimbone nini ugqirha ngale nto?

Ukuba ulungiselela utyando, kubaluleke kakhulu ukuxelela oogqirha ukuba kukho umntu kusapho lwakho okhe waba neengxaki zokubulala iintlungu ngaphambili .

Kwakhona, ukuba usandul’ ukufunyanwa une-genetic mutation ebangela i-Malignant Hyperthermia kumntu ozalana naye ngegazi (isalamane sebhayoloji), okanye ukuba ufumanisa ukuba loo mntu unesifo esibizwa ngokuba yi-Malignant Hyperthermia, kubaluleke kakhulu ukwazisa ugqirha wakho kwaye ubhale oku kwiirekhodi zakho zonyango.

Ezona zinto zibalulekileyo ekufuneka uzikhumbule kweli nqaku

I-Molignant Hyperthermia yindlela enobungozi, enokubangela ukufa xa kuthe kwasetyenziswa amayeza athile okubulala iintlungu.

Iindaba ezimnandi zezokuba inokunyangeka kwaye, ukuba uyazi ukuba usemngciphekweni, inokuthintelwa.

Ukuba kunokwenzeka, yazi zonke iinkcukacha malunga nembali yezonyango zezalamane zakho zegazi. Yabelana ngolo lwazi nogqirha wakho. Ekubeni i-'Malignant Hyperthermia' ibangelwa kukuguquka kwezakhi zofuzo okudlulela kwizizukulwana ngezizukulwana, ukuba kukho umntu kusapho lwakho onalo, nawe usengozini enkulu.

Ukuba unemibuzo okanye iinkxalabo malunga notyando kunye ne-anesthesia oza kuyifumana, ungaze ungathandabuzi ukubuza ugqirha wakho. Bakhona ukukunceda. Ukwazi kwakho kunye nokunxibelelana ngokukhululekileyo neqela lakho lezonyango zezona zikhuselo zilungileyo kuwe.

i- malignant hyperthermia, i-anesthesia, utyando, izifo zemfuza, imbali yosapho, iingxaki ze-anesthesia, ubushushu obuphezulu bomzimba