Malignant Hyperthermia ni iki? Ibimenyetso, Impamvu, n'Umuti

Malignant hyperthermia (MH) ni ikibazo gito cyane ariko gikomeye kiba mu gihe umuntu akoreshejwe imiti runaka yo kubyara ibitotsi mu gihe cy'ubuganga. Ibaho iyo imitsi yawe isubiramo nabi iyo miti, igahindura ubushyuhe bw'umubiri bwawe bugakira cyane kandi imitsi yawe igakomera.

Iki kibazo kiba kuri umwe kuri 5,000 kugeza kuri umwe kuri 50,000 mu bantu bakoreshejwe imiti yo kubyara ibitotsi. Nubwo byumvikana biteye ubwoba, bivurwa neza igihe byafashwe hakiri kare, kandi ibitaro bya none bifite ibikoresho bihagije byo kubikora neza.

Ibimenyetso bya Malignant Hyperthermia ni ibihe?

Ibimenyetso bya malignant hyperthermia bigaragara vuba mu gihe cyo kubyara ibitotsi cyangwa nyuma gato. Itsinda ry'abaganga bakurikirana ibi bimenyetso neza mu buvuzi bukoresha imiti ishobora kubitera.

Ibimenyetso bya mbere bikunze kugaragara birimo:

• Ubushyuhe bw'umubiri buzamuka cyane (rimwe na rimwe bugera kuri 41°C cyangwa hejuru)
• Ubukomere bw'imitsi, cyane cyane mu mitsi y'umunwa
• Umutima ukubita cyane kandi udakora neza
• Umuvuduko w'amaraso uhanitse
• Guhumeka cyane kandi guhumeka buhoro
• Kunyara cyane
• Ibara ry'uruhu rihinduka

Mu bihe bikomeye, ushobora no kugira ikibazo cyo kwangirika kw'imitsi, ibibazo by'impyiko, cyangwa impinduka z'amaraso ziteye akaga. Inkuru nziza ni uko abaganga bo mu bitaro bahuguwe mu kubona ibi bimenyetso vuba kandi bagafata ingamba zihuse.

Impamvu ziterwa na Malignant Hyperthermia ni izihe?

Malignant hyperthermia iterwa n'ikibazo cy'umuzuko kigira ingaruka ku buryo uturemangingo tw'imitsi dukoresha calcium. Iyo umuntu akoreshejwe imiti runaka yo kubyara ibitotsi, ubwo buryo butari bwo bw'umuzuko butera imitsi gusubiramo nabi.

Ibintu by'ingenzi bibitera ni imiti runaka yo kubyara ibitotsi:

• Gaz yo kubyara ibitotsi (nka sevoflurane, isoflurane, na halothane)
• Succinylcholine (umuti ufasha imitsi kureka gukora)

Urashobora kuzana ubwo buryo bw'umuzuko mu muryango. Bifitanye isano n'impinduka mu migeni igenzura uburyo calcium isohorwa mu mitsi, cyane cyane imigeni ya RYR1 na CACNA1S. Iyo iyo migeni idakora neza, gukoresha imiti itera iki kibazo bituma calcium yinjira mu mitsi yawe mu buryo buteye ubwoba.

Ubu buryo bw'umuzuko buri mu miryango, ariko kugira iyo gene ntibivuze ko uzagira icyo kibazo. Bamwe bafite iyo gene ariko ntibagire ibimenyetso, abandi bashobora kugira ibibazo bikomeye mu gihe cya mbere bakoreshejwe iyo miti.

Ni ryari ukwiye kubona muganga kubera Malignant Hyperthermia?

Niba utegereje kubagwa, ugomba kuvugana n'umuganga wawe mbere niba hari umuntu mu muryango wawe warigeze agira ibibazo kubera imiti yo kubyara ibitotsi. Icyo kiganiro gifasha itsinda ry'abaganga bawe gutegura uburyo bwiza bwo kubuvura.

Ugomba kuvuga by'umwihariko niba hari umuntu mu muryango wawe warigeze agira:

• Ubushyuhe bukabije mu gihe cy'ubuganga
• Gukira nabi nyuma yo kubyara ibitotsi
• Ibibazo by'imitsi cyangwa intege nke
• Kumenyekana ko ufite malignant hyperthermia

Mu gihe cy'ubuganga, itsinda ry'abaganga bakurikirana ubuzima bwawe buri gihe, ku buryo ntukeneye guhangayika kubona ibimenyetso ubwawe. Ariko, niba warigeze ukorerwa isuzuma ry'umuzuko ryerekana ko ufite ubwo buryo bw'umuzuko, buri gihe menyesha umuganga mbere yo gukoresha imiti yo kubyara ibitotsi cyangwa imiti imwe n'imwe.

Ibyago bya Malignant Hyperthermia ni ibihe?

Ikintu gikomeye cyane cy'ibyago ni ukugira amateka y'umuryango wa malignant hyperthermia cyangwa ibibazo bitasobanuwe mu gihe cyo kubyara ibitotsi. Kubera ko ari ikibazo cy'umuzuko, gikunda kuba mu miryango.

Ibindi bintu bishobora kongera ibyago birimo:

• Kuba umugabo (abagabo bafite ibyago byinshi)
• Kuba muto (abana n'urubyiruko bafite ibyago byinshi)
• Kugira ibibazo bimwe by'imitsi nka central core disease
• Ibibazo bitasobanuwe byabayeho mbere kubera imiti yo kubyara ibitotsi

Ibibazo bimwe na bimwe bito cyane by'imitsi bifitanye isano n'ibyago byinshi. Ibyo birimo congenital myopathies, muscular dystrophies, na periodic paralysis syndromes. Niba ufite ikibazo cy'imitsi cyamenyekanye, itsinda ry'abaganga bawe bazagufasha cyane.

Ni byiza kuzirikana ko umunaniro, ubushyuhe, cyangwa imyitozo ngororamubiri bisanzwe biterwa na malignant hyperthermia. Icyo kibazo gikunda kubaho iyo umuntu akoreshejwe imiti runaka yo kubyara ibitotsi kandi afite ubwo buryo bw'umuzuko.

Ingaruka zishoboka za Malignant Hyperthermia ni izihe?

Iyo bivuwe vuba, abantu benshi barakira neza malignant hyperthermia nta ngaruka zirambye. Ariko, niba icyo kibazo kitamenyekanye kandi kikitavuwe vuba, gishobora gutera ingaruka zikomeye.

Ingaruka ziteye impungenge cyane harimo:

• Kwangirikwa kw'imitsi (rhabdomyolysis) bishobora kwangiza impyiko zawe
• Ibibazo by'umutima bishobora kuba bibi
• Kwangirikwa kw'ubwonko kubera ubushyuhe bukabije bw'umubiri
• Ibibazo byo gukama kw'amaraso
• Kumara amazi cyane no kutagira imyunyu ngugu ihagije
• Kwanga kw'imikorere y'imigongo

Ikintu cy'ingenzi cyo kwirinda izi ngaruka ni ukumenya icyo kibazo hakiri kare no kuvurwa vuba. Ibitaro bya none bifite ibikoresho byo gupima ubushyuhe n'imiti yo gutabara, bituma ingaruka zikomeye ziba nke ugereranije n'uko byari bimeze mu bihe byashize.

Iyo havuwe neza, umubare w'abarokoka urenga 95%. Abantu benshi bagize malignant hyperthermia bakomeza kubaho ubuzima busanzwe, nubwo bagomba kwirinda imiti itera icyo kibazo mu gihe cy'ubuganga buzaba buza.

Malignant Hyperthermia imenyekanwa gute?

Malignant hyperthermia imenyekanwa ahanini hashingiwe ku bimenyetso byawe n'uburyo usubiramo mu gihe cyo kubyara ibitotsi. Itsinda ry'abaganga bawe rigena icyo kibazo rikurikije ibimenyetso byagaragaye n'uburyo usubiramo imiti yo gutabara.

Mu gihe icyo kibazo kiriho, abaganga bashaka ibimenyetso byihariye by'ubushyuhe bukabije, ubukomere bw'imitsi, n'impinduka zihariye mu maraso. Bakurikirana kandi uburyo usubiramo dantrolene, imiti yo gutabara.

Nyuma yo gukira, isuzuma ry'umuzuko rishobora gufasha kwemeza ko ufite ubwo buryo bw'umuzuko no kuyobora ubuvuzi buzaba buza. Iryo suzuma rirebana n'impinduka mu migeni ikunze kuba ifitanye isano na malignant hyperthermia. Ariko, isuzuma ry'umuzuko ntirigaragaza ibintu byose, ku buryo ibisubizo bisanzwe bitavuze ko nta kibazo ufite.

Ku bantu bo mu muryango, gupima imitsi byari bimenyerewe cyane mu kumenya icyo kibazo. Ibyo bisobanura gufata agace gato k'imitsi no kuyishyira mu miti itera icyo kibazo mu ikusanyirizo. Ariko, icyo gipimo ubu kiraboneka mu bigo bike byihariye kandi gikoreshwa gake cyane kuva isuzuma ry'umuzuko ryabonetse.

Umuti wa Malignant Hyperthermia ni uwuhe?

Umuti wa malignant hyperthermia ugamije guhagarika vuba imiti itera icyo kibazo no guha umuti wo gutabara witwa dantrolene. Uwo muti ukora mu kuburizamo calcium kwinjira mu mitsi yawe, ugahagarika icyo kibazo.

Itsinda ry'abaganga bawe rizakora ibintu byihuse:

1. Guhagarika imiti yose itera icyo kibazo
2. Guha dantrolene binyuze mu mitsi (akenshi 2.5 mg kuri kg imwe y'umubiri)
3. Kugabanya ubushyuhe bw'umubiri hakoreshejwe ubukonje, amazi akonje, n'ibikoresho byo kugabanya ubushyuhe
4. Guha ogisijeni no gufasha guhumeka
5. Gukurikirana no gukosora ibibazo by'umutima
6. Gusuzuma no kubungabunga imyunyu ngugu mu maraso

Umuti wa dantrolene ugomba gusubirwamo buri masaha make kugeza igihe ibimenyetso byawe byashize burundu. Abantu benshi bakenera umuti ugereranije mu masaha 24 kugeza kuri 48 kugira ngo birinde ko icyo kibazo gisubira.

Itsinda ry'abaganga bawe rizakurikirana kandi imikorere y'impyiko zawe hafi kandi baguhe amazi ahagije kugira ngo bafashe gukuraho ibintu byangiza imitsi. Mu bihe bikomeye, ushobora gukenera kuvurwa hakoreshejwe imashini kugira ngo impyiko zawe zikomeze gukora mu gihe zikira.

Uburyo bwo gucunga ubuvuzi bwa Malignant Hyperthermia?

Gukira malignant hyperthermia bisanzwe bibaho mu cyumba cy'ubuvuzi buhambaye aho itsinda ry'abaganga bawe bashobora kukukurikirana hafi. Abantu benshi batangira kumva neza mu masaha make nyuma yo guhabwa dantrolene, nubwo gukira burundu bishobora gutwara iminsi myinshi.

Mu gihe cyo gukira, itsinda ry'abaganga bawe rizakomeza kukugira dantrolene kandi bakurikirane ibimenyetso byawe by'ubuzima, imikorere y'impyiko, n'ibintu byangiza imitsi. Uzahora mu bitaro byibuze amasaha 24 kugeza kuri 48 kugira ngo wirinde ko icyo kibazo gisubira.

Iyo ugeze murugo, ugomba kuruhuka no kureka umubiri wawe gukira neza. Bamwe bagira ububabare bw'imitsi cyangwa intege nke iminsi mike, ibyo ni ibisanzwe. Kunywa amazi menshi bifasha impyiko zawe gukuraho ibintu byangiza imitsi.

Ikintu cy'ingenzi cyo gukira ni ukubona inyandiko z'icyo kibazo n'inama ku migeni niba bikenewe. Ibyo bintu ni ingenzi mu buvuzi buzaba buza no kumenyesha abagize umuryango bashobora kuba bafite ibyago.

Malignant Hyperthermia ishobora kwirindwa gute?

Uburyo bwiza bwo kwirinda malignant hyperthermia ni ukwirinda imiti itera icyo kibazo. Niba uzi ko ufite ubwo buryo bw'umuzuko cyangwa ufite amateka y'umuryango, itsinda ry'abaganga bawe bazakoresha imiti itari mbi kuri wewe.

Imiti itari mbi irimo:

• Propofol n'izindi miti yo kubyara ibitotsi zitera icyo kibazo
• Imiti yo kubyara ibitotsi yo mu mitsi nka lidocaine
• Imiti yo kubyara ibitotsi mu mugongo
• Imiti idatera icyo kibazo
• Nitrous oxide (gaz iseka)

Niba ufite amateka y'umuryango wa malignant hyperthermia, tekereza gukora isuzuma ry'umuzuko mbere y'ubuganga ubwo aribwo bwose. Ibyo bifasha itsinda ry'abaganga bawe gufata ibyemezo byiza ku bijyanye no kubyara ibitotsi.

Buri gihe bambara ikimenyetso cy'ubuvuzi cyangwa ufite ikarita ivuga ko ufite malignant hyperthermia. Mu bihe by'amahoro, ibyo bintu bishobora gukiza ubuzima kandi bifasha abaganga guhitamo imiti ikwiye vuba.

Kubijyanye no kubyara abana, inama ku migeni ishobora kugufasha kumva ibyago byo guha icyo kibazo abana bawe no kuganira ku buryo bwo gupima abagize umuryango.

Ugomba gutegura gute uruzinduko rwawe kwa muganga?

Niba uhangayikishijwe n'ibyago bya malignant hyperthermia, tanga ukusanya amakuru arambuye ku mateka y'ubuzima bw'umuryango wawe. Ibaze cyane cyane ibibazo abagize umuryango wawe bagize kubera imiti yo kubyara ibitotsi cyangwa ibibazo bitasobanuwe mu gihe cy'ubuganga.

Mbere y'uruzinduko rwawe, andika:

• Amateka y'umuryango w'ibibazo byo kubyara ibitotsi cyangwa malignant hyperthermia
• Ubuganga warigeze ukora n'uburyo wakize
• Ibibazo by'imitsi cyangwa intege nke mu muryango wawe
• Ibibazo ku bijyanye no gupima umuzuko cyangwa umutekano wo kubyara ibitotsi
• Imiti ukoresha ubu n'ibintu ufite allergy

Niba witegura kubagwa, tegura inama yo kubagwa mbere n'umuganga wawe. Ibyo biguha umwanya wo kuganira ku byo uhangayikishijwe n'ibyo bikaba byamufasha gutegura uburyo bwiza bwo kubyara ibitotsi mu gihe cy'ubuganga.

Zana inyandiko z'ubuvuzi zabanje zijyanye n'ibibazo byo kubyara ibitotsi, ibisubizo by'isuzuma ry'umuzuko, cyangwa raporo z'isuzuma ry'imitsi niba ubifite. Ibyo bintu bifasha itsinda ry'abaganga bawe gufata ibyemezo byiza ku bijyanye no kubitaho.

Icy'ingenzi cyo kuzirikana kuri Malignant Hyperthermia ni iki?

Malignant hyperthermia ni ikibazo gikomeye ariko kivurwa neza kiba mu bantu bafite ubwo buryo bw'umuzuko ku miti runaka yo kubyara ibitotsi. Nubwo bishobora kuba bibi ntibyamenyekanye vuba, ubuvuzi bwa none bumaze kubikora neza iyo bivuwe neza.

Ikintu cy'ingenzi cyo kuzirikana ni uko ubumenyi ari bwo burinda cyane. Niba ufite amateka y'umuryango w'ibibazo byo kubyara ibitotsi, vugana n'abaganga bawe mbere y'ubuganga ubwo aribwo bwose. Bashobora gukoresha imiti itari mbi kandi biteguye imiti yo gutabara niba bikenewe.

Hamwe n'amabwiriza meza n'ubumenyi bw'abaganga, abantu bafite ubwo buryo bw'umuzuko bashobora kubagwa neza kandi bagakomeza kubaho ubuzima busanzwe. Ikintu cy'ingenzi ni ukumenyesha itsinda ry'abaganga bawe ibyago byawe kugira ngo bafate ingamba zikwiye.

Ibibazo bikunze kubaho kuri Malignant Hyperthermia

Q1: Malignant hyperthermia ishobora kubaho hatabayeho kubyara ibitotsi?

Malignant hyperthermia hafi ya buri gihe isaba gukoresha imiti itera icyo kibazo ikoreshwa mu kubyara ibitotsi. Nubwo hari ibintu bike cyane byavuzwe ku bushyuhe bukabije cyangwa imiti imwe n'imwe, ibintu byinshi bibaho mu gihe cy'ubuganga hakoreshejwe imiti yo kubyara ibitotsi cyangwa succinylcholine.

Q2: Niba umubyeyi wanjye yagize malignant hyperthermia, nanjye nzayigira?

Oya ntabwo ari ngombwa. Umuntu arashobora kuzana ubwo buryo bw'umuzuko, ariko ntibikurikira uburyo bumwe. Ufite amahirwe 50% yo kuzana ubwo buryo bw'umuzuko niba umubyeyi wawe abufite, ariko kugira iyo gene ntibivuze ko uzagira icyo kibazo. Bamwe bafite iyo gene ariko ntibagire ibimenyetso.

Q3: Malignant hyperthermia itera gute vuba mu gihe cy'ubuganga?

Malignant hyperthermia ishobora kubaho mu minota mike nyuma yo gukoresha imiti itera icyo kibazo, nubwo rimwe na rimwe bitwara igihe kinini kugira ngo bigaragara. Icyo kibazo gikunda kugaragara mu isaha ya mbere yo kubyara ibitotsi, niyo mpamvu itsinda ry'abaganga bawe bakukurikirana hafi muri icyo gihe.

Q4: Nshobora gukorerwa ubuganga bw'amenyo niba mfite malignant hyperthermia?

Yego, ushobora gukorerwa ubuganga bw'amenyo neza. Imiti yo kubyara ibitotsi yo mu mitsi nka lidocaine na novocaine ni nziza ku bantu bafite malignant hyperthermia. Gusa menyesha umuganga wawe icyo kibazo kugira ngo birinde imiti itera icyo kibazo niba hakenewe kubyara ibitotsi byinshi.

Q5: Nzagomba kwirinda ubuganga bwose buzaba buza niba naragize malignant hyperthermia?

Oya rwose. Ushobora kubagwa neza hakoreshejwe imiti yo kubyara ibitotsi itari mbi. Itsinda ry'abaganga bawe bazakoresha imiti itari mbi kuri wewe. Abantu benshi bafite malignant hyperthermia bagize ubuganga bwinshi neza hakoreshejwe amabwiriza meza.