Ake sifunde ngalesi simo esiyingozi esivele sivele ngesikhathi sokuhlinzwa: I-Molignant Hyperthermia!

Uma wena noma othile omaziyo kufanele ahlinzwe, kungenzeka ukuthi uke wezwa igama elithi i-anesthesia. Lisho ukuthi silaliswa ngesikhathi sokuhlinzwa. Kodwa wake wacabanga ukuthi abanye abantu bangaba nokusabela okuyingozi kakhulu, okusongela ngisho nokuphila kule mithi yokubulala izinzwa? Leso yisimo esingavamile, kodwa esibi kakhulu esibizwa ngokuthi i-Malignant Hyperthermia. Igama lingase lizwakale liyesabeka kancane, kodwa uma unolwazi olufanele ngalo, izinkinga eziningi zingagwenywa. Ngakho-ke, ake sixoxe ngakho ngokuningiliziwe namuhla.

Kuyini i-Malignant Hyperthermia?

Kalula nje, i-'Malignant Hyperthermia' yisimo sofuzo . Okusho ukuthi, sibangelwa ukuguquka kwezakhi zofuzo zethu. Uma umuntu onalesi simo enikezwa imithi ethile yokubulala izinzwa, umzimba wakhe usabela kabi kakhulu. Lesi yisimo esiphuthumayo esingaba yingozi ngisho nokuphila.

Ingxenye engcono kakhulu ukuthi, nakuba lokhu kuvame ukudluliselwa kusuka esizukulwaneni kuya kwesinye, kunezindlela zokwelapha ngakho . Futhi, uma wazi ngale ngozi kusenesikhathi, ungazivikela kuyo.

Siyazi ukuthi uma sinikezwa i-anesthesia ejwayelekile, asizwa lutho ngesikhathi sokuhlinzwa. Kunezinhlobo ezahlukene zemithi yokunikeza le anesthesia. Kodwa-ke, zimbalwa kakhulu kulezo mithi ezibangela lesi simo esibizwa ngokuthi 'i-Malignant Hyperthermia'. Imithi eminingi yokubulala izinzwa ayibangeli lokhu.

Enye into ebalulekile ukuthi ngezinye izikhathi umuntu angase angazi ukuthi une-genetic mutation engabangela i-Malignant Hyperthermia, ngoba angase angakhombisi zimpawu aze athole imithi ethile eshiwoyo.

Ubani onamathuba amaningi okuthuthukisa lokhu?

Ake sibone ukuthi ubani osengozini enkulu yokuthola lesi simo 'se-Malignant Hyperthermia'.

Empeleni, uma othile emndenini wakho eke waba necala ngaphambilini le-'Malignant Hyperthermia' , khona-ke amanye amalungu alowo mndeni nawo angaba sengozini. Ikakhulukazi, uma isihlobo sezinga lokuqala (okungukuthi umama, ubaba, izingane zakubo, izingane) sibe nalesi simo, ubhekwa 'njengomuntu osengozini'.

Ngakho-ke, uma kwenzeka kudingeka uhlinzwe, kubaluleke kakhulu ukwazisa udokotela wezinzwa uma kukhona okufana nalokhu okwenzeke kunoma ubani emndenini wakho.

Ake sithi umndeni wakho awukaze ube nokusabela kwe-'Malignant Hyperthermia' kanje. Kodwa-ke, abantu abanezifo ezithile zemisipha nabo bangase babe sengozini enkulu. Ezinye zalezi zifo yilezi:

• `Isifo esiyinhloko`
• `Isifo se-Multiminicore`
• `I-King-Denborough syndrome`
• `Isifo se-STAC3`

Nakuba lokhu kungase kubonakale kuyinkimbinkimbi kancane, kuhle ngalabo abanalezi zimo ukuqaphela lokhu.

Sivame kangakanani lesi simo?

Kuvame kangakanani lokhu 'kwe-Mognant Hyperthermia'? Nakuba izibalo zixubene kancane, ngokuvamile kulinganiselwa ukuthi lesi simo singavela cishe kokuhlinzwa okukodwa kwabayi-100,000 kubantu abadala. Phakathi kwezingane, leli nani cishe liyi-1 kwabayi-30,000. Kuyaphawuleka futhi ukuthi ingxenye yeziguli ezitholakele zingaphansi kweminyaka eyi-19.

Kodwa ososayensi bacabanga ukuthi abantu abathambekele kulesi simo 'se-Malignant Hyperthermia' bangase babe semphakathini. Ngenxa yokuthi abantu abaningi abachayeke emithini ethile ebangela lokhu kukho konke ukuphila kwabo, bangase bangazi nokuthi basengozini.

Ziyini izimpawu zalokhu?

Izimpawu ze-'Malignant Hyperthermia' zingahluka kancane kumuntu nomuntu. Zingavela ngesikhathi sokuhlinzwa noma ngenkathi ululama ekuhlinzweni. Isibonelo, uma ungaphansi kwe-anesthesia futhi lezi zimpawu zivela ngesikhathi sokuhlinzwa, ngeke uzizwe. Kodwa-ke, udokotela wakho wezinzwa noma umhlengikazi wezinzwa uqaphile kakhulu ngalokhu. Baqapha wonke umuntu ongaphansi kwe-anesthesia ngokucophelela. Lapho nje bebona lezi zimpawu, bazoqala ngokushesha ukwelashwa kwe-'Malignant Hyperthermia'.

Izimpawu zokuqala

Lezi yizibonakaliso zokuqala ongase uzibone:

• Ukwanda kwesilinganiso senhliziyo okungachazeki (i-tachycardia). Isilinganiso senhliziyo sikhuphuka ngokuzumayo.
• Ukwanda okungazelelwe kwenani le-carbon dioxide elikhishwa emzimbeni . Lokhu kungelashwa nangemishini yezokwelapha.
• Isivinini sokuphefumula esikhulayo (i-tachypnea). Uqala ukuphefumula ngokushesha, njengokungathi uphefumula kanzima.
• Ukuqina kwemisipha, ukuqina (`ukuqina kwemisipha`). Imisipha emzimbeni iba lukhuni njengokungathi ichotshozwa yidwala.
• Ukwanda okuzumayo kwezinga lokushisa lomzimba . Umzimba uqala ukufudumala.

Izimpawu ezibonakala kamuva

Njengoba isimo siba sibi kakhulu, ungase ubone nezimpawu ezifana nalezi:

• Ukwenyuka kwezinga lokushisa lomzimba ngaphezu komkhuhlane ojwayelekile .
• Umchamo omnyama . Ungaze uphenduke ube nsundu.
• Ukuhlolwa kwegazi kubonisa ukuwohloka kwemisipha .
• Ukungalingani kwesigqi senhliziyo (i-arrhythmia). Inhliziyo ishaya ngendlela engavamile.
• Ukopha .
• Izimo ezifana nokuquleka .

Kungani kwenzeka i-malignant hyperthermia?

Manje ake sibone ukuthi kungani lesi simo esibizwa ngokuthi 'i-Malignant Hyperthermia' senzeka.

Abantu abanalesi simo baneprotheni engajwayelekile emaseli emisipha yabo. Lokhu kungenxa yoshintsho ezizakhini zabo zofuzo, olubizwa ngokuthi ukuguqulwa kwezakhi zofuzo .

Kalula nje, i-DNA yethu ifana "nencwadi yemiyalelo" equkethe ulwazi oludingwa amaseli emzimbeni wethu ukuze enze imisebenzi yawo. Uma kukhona ulwazi oluthile olungekho ndawana thize kule DNA, noma uma kukhona umonakalo kokuthile, yilapho kuvela khona izifo zofuzo ezinjengalezi.

Lokhu kuguquka kwezakhi zofuzo akubanga zimpawu empilweni evamile. Kodwa-ke, uma umuntu onalolu guquko evezwa emithini ethile yokubulala izinzwa okukhulunywe ngayo ngenhla - noma, ngokungavamile kakhulu, uma evezwa emazingeni okushisa aphezulu noma uma ehlanganyela ekuzivocavoceni okunzima - amangqamuzana emisipha yakhe aqala ukukhulula i-calcium ngendlela engavamile.

Ngenxa yalokho, imisipha iyaqhubeka nokufinyela futhi iqine noma iqine, i-metabolism yomzimba kanye nokushisa komzimba kuyanda ngendlela engavamile. Ekugcineni, amangqamuzana emisipha ayafa, futhi inani elikhulu le-potassium liqongelela egazini. Lokhu kungaholela ezimpawini ezengeziwe kanye nezinkinga.

Imithi yokubulala izinzwa ingabangela lesi simo.

Umuntu othambekele ekuguleni ngokwezakhi zofuzo kulesi simo esithi `Malignant Hyperthermia` angase abhekane nalokhu kusabela uma enikezwa imithi elandelayo yokubulala izinzwa:

Imithi ephefumulwayo:

• `I-Halothane`
• `I-Desflurane`
• `I-Sevoflurane`
• `I-Isoflurane`

Imithi efakwa ngemithambo yegazi:

• I-Succinylcholine - Lesi isitho esiphumuzayo semisipha esisebenza ngokushesha.

Ukubona kanjani lokhu?

Uma uhlinzwa usebenzisa umuthi wokubulala izinzwa oyingozi ngaphandle kokwazi ukuthi unesifo 'se-Malignant Hyperthermia', wena noma udokotela wezinzwa ngeke nazi ukuthi unaso kuze kube yilapho ukusabela sekwenzekile.

Kodwa-ke, uma wena noma omunye umuntu enikezwa i-anesthesia, udokotela wezinzwa noma umhlengikazi uzoqapha izimpawu zakho ezibalulekile kanye nempilo yakho ngokucophelela ngesikhathi nangemva kokuhlinzwa. Uma une-'Malignant Hyperthermia', bazoyibona ngezibonakaliso ezibalulekile ezibalulekile kanye nokusabela okukhulunywe ngakho ekuqaleni. Okungukuthi:

• Ukwanda kwesilinganiso senhliziyo ngaphandle kwesizathu.
• Ukwenyuka okusheshayo kwezinga lokushisa lomzimba.
• Ukwanda okungazelelwe kwenani le-carbon dioxide elikhishwa emzimbeni.
• Ukuqina kwemisipha.

Odokotela ababulala izinzwa baqeqeshwe ukuqaphela lezi zinguquko kusenesikhathi . Bese bekwazi ukusabela ngokushesha kulesi simo esiphuthumayo. Uma ukuxilongwa kushesha, kulapho ukwelashwa nokulawula isimo kungatholakala khona.

Wazi kanjani kusengaphambili ukuthi unale ngozi?

Uma wena noma udokotela wakho nicabanga ukuthi ungase ube sengozini ye-Malignant Hyperthermia, kungaba ngenxa yomlando womndeni wakho noma ngezinye izizathu, kunezivivinyo ezikhethekile ezingenziwa ukuqinisekisa lokhu:

• Ukuhlolwa kwe-caffeine halothane contracture (CHCT) : Kulolu vivinyo, kuthathwa ingxenye encane yemisipha yakho (i-muscle biopsy) bese isampula yezicubu ivezwa ezixazululweni ze-halothane ne-caffeine ukuze kubonakale ukuthi iyasabela yini kugesi yokubulala izinzwa.
• Ukuhlolwa kwezakhi zofuzo : Lezi zivivinyo zingabona izinguquko ezakhiweni zezakhi zofuzo eziku-DNA ezibizwa ngokuthi i-RYR1, STAC3, kanye ne-CACNA1S. Kutholakale izinguquko ezingaphezu kuka-45 ezingabangela i-Malignant Hyperthermia.

Kodwa-ke, lezi zivivinyo zokuthola ukuthambekela kwe-'Malignant Hyperthermia' zibiza kakhulu, futhi zingenziwa kuphela emalabhorethri ambalwa akhethekile. Isibonelo, ukuhlolwa kwe-'CHCT' kutholakala kuphela ezindaweni ezine e-United States. Ngakho-ke, uma udinga ukuhlinzwa okuphuthumayo futhi isihlobo esiseduze sike saba ne-'Malignant Hyperthermia' ngaphambilini, odokotela be-anesthesiologists bavame ukusebenzisa ama-anesthetics aphephile esikhundleni salawo mithi ayingozi.

Yiziphi izindlela zokwelapha lokhu?

Ukwelashwa okuyinhloko kwe-'Malignant Hyperthermia' umuthi i-'dantrolene' (igama lomkhiqizo elithi 'Dantrium®').

Lapho nje odokotela bezinzwa besola isimo esibizwa ngokuthi 'i-Malignant Hyperthermia', banikeza lo muthi 'we-dantrolene'. Ngesikhathi esifanayo, bayayeka ukunikeza umuthi wezinzwa obangele ukusabela, bese udokotela ohlinzayo eqeda ukuhlinzwa ngokushesha okukhulu.

Ngaphezu kwalokho, ithimba lokubulala izinzwa kanye nodokotela abahlinzayo liphinde lelaphe ezinye izimpawu nezinkinga ezibangelwa yi-'Malignant Hyperthermia'. Ezinye zalezi zindlela zokwelapha yilezi:

• I-Hyperthermia iphathwa ngezindlela zokupholisa , njengokunikeza uketshezi olubandayo lwe-IV kanye nokusebenzisa amaphakethe eqhwa.
• I-oksijini inikezwa nge-face mask noma ngepayipi elibekwe ku-trachea.
• Uma kukhona ukushaya kwenhliziyo okungajwayelekile (i-arrhythmia), kunikezwa imithi yalokho.

Uma isimo sakho sesizinzile, uzogcinwa egunjini lokunakekelwa okujulile (ICU) okungenani usuku olulodwa ukuze uhlolwe . Ithimba lezokwelapha lizokwenza ezinye izivivinyo (isib. ukuhlolwa kwegazi) futhi linikeze ukwelashwa okwengeziwe uma kudingeka.

Ingabe lokhu kungavinjelwa?

I-Hyperthermia Eyingozi Kunzima ukuyivimbela, ngaphandle kokuthi wazi ukuthi une-genetic mutation eyibangela , noma othile emndenini wakho unalesi simo futhi utshele udokotela wakho we-anesthesiologist. Uma kunjalo, udokotela we-anesthesiologist ngeke asebenzise leyo mithi eyingozi ohlelweni lwakho lwe-anesthesia.

Kodwa, into edabukisayo ukuthi, isikhathi esiningi abantu bathola kuphela ukuthi basengozini yokuthola i-Malignant Hyperthermia ngemva kokuba senzeke kubo.

Kwenzekani ngemva kwalesi simo? Ingabe singelapheka?

Uma izimpawu ze-'Malignant Hyperthermia' zibonakala kusenesikhathi futhi zelashwa kahle, kungenzeka ukuthi umuntu alulame ngokugcwele . Isinyathelo esisheshayo sethimba lokubulala izinzwa kanye nodokotela abahlinzayo sibaluleke kakhulu lapha.

Kodwa-ke, kumelwe sikhumbule nokuthi ngezinye izikhathi, ngisho nangokwelashwa okusheshayo, ukwehluleka kwezitho eziningi kanye nokufa kungenzeka.

Yiziphi ezinye izinkinga ezingaba khona ngenxa ye-malignant hyperthermia?

I-Hyperthermia eyingozi ingabangela izinkinga ezinkulu ezifana nalezi:

• Ukulimala kwemisipha
• Ukwehluleka kwezinso
• Ukwehluleka kwesibindi
• Ukopha
• Ukuboshwa kwenhliziyo
• Ukuquleka
• I-Coma
• Ukufa

Ingabe lokhu kuyabulala?

Uma ingelashwa, i-'Malignant Hyperthermia' iyisimo esingaba yingozi . Ngisho noma ukwelashwa okufanele, izinga lokufa elibikiwe liphakathi kuka-3% no-5%. Ngakho-ke, lokhu akuyona into okufanele ithathwe kalula.

Kufanele ngimbone nini udokotela ngalokhu?

Uma ulungiselela ukuhlinzwa, kubaluleke kakhulu ukutshela odokotela uma kukhona emndenini wakho oke waba nezinkinga zokubulala izinzwa ngaphambilini .

Futhi, uma usanda kutholwa une-genetic mutation ebangela i-Malignant Hyperthermia esihlotsheni segazi (isihlobo segazi), noma uma uthola ukuthi lowo muntu unesifo esibizwa ngokuthi i-Malignant Hyperthermia, kubaluleke kakhulu ukwazisa udokotela wakho futhi ukubhale phansi lokhu kumarekhodi akho ezokwelapha.

Izinto ezibaluleke kakhulu okudingeka uzikhumbule kulesi sihloko

I-Molignant Hyperthermia iyindlela engathi sína, esongela ukuphila emithini ethile yokubulala izinzwa.

Izindaba ezinhle ukuthi ingelashwa futhi, uma wazi ukuthi usengozini, ingavinjelwa.

Uma kungenzeka, yazi yonke imininingwane ngomlando wezokwelapha wezihlobo zakho zegazi. Yabelana ngalolo lwazi nodokotela wakho. Njengoba i-'Malignant Hyperthermia' ibangelwa ukuguquka kwezakhi zofuzo okudluliselwa ezizukulwaneni ngezizukulwane, uma othile emndenini wakho enayo, nawe usengozini enkulu.

Uma unemibuzo noma ukukhathazeka mayelana nokuhlinzwa kanye nokuhlinzwa kokubulala izinzwa ozokuthola, ungalokothi unqikaze ukubuza udokotela wakho. Bakhona ukukusiza. Ukwazi kwakho kanye nokukhulumisana okuvulekile nethimba lakho lezokwelapha kuyisivikelo esingcono kakhulu kuwe.

i- hyperthermia embi, i-anesthesia, ukuhlinzwa, izifo zofuzo, umlando womndeni, izinkinga ze-anesthesia, ukushisa komzimba okuphezulu