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Kuzamuka vuba Intege nke, Syndrome ya Guillain-Barre, na Myasthenic Crisis

Imibare yihutirwa itandukanye nigihugu. Uru rupapuro rukoresha 911, umubare kuri Amerika na Kanada. Ibindi bihugu byinshi bikoresha 112, 999, cyangwa undi mubare. Shakisha umubare wigihugu cyawe.

Intege nke zigenda ziyongera ziva hejuru y'ibirenge n'amaguru yerekeza mu gituza (** kuzamuka paralise ** muri ** Guillain-Barre Syndrome, cyangwa GBS ) cyangwa bigahita bigabanya intege amaso y'amaso, urwasaya, umuhogo, ijosi, n'imitsi ihumeka ( Myasthenic Crisis ** muri ** Myasthenia Gravis **) ni ibintu byihutirwa byangiza ubuzima. Bitandukanye n'indwara y'ubwonko cyangwa umutima, kunanirwa k'ubuhumekero bwa neuromuscular ntibikunze gutera umwuka mubi cyangwa umubare wa ogisijeni muke; Ahubwo, diaphragm n'imitsi y'urukuta rw'igituza bigabanuka gahoro gahoro kugeza igihe umuntu adashobora gukuraho amacandwe cyangwa guhumeka dioxyde de carbone, bigatuma umuntu ahumeka atunguranye. Ibigo byambere byita kumfashanyo kumenyekanisha hakiri kare, gutwara ambilansi yihuse kubuvuzi bukomeye (ICU), guhagarara neza kugirango bipakurure diafragma, kwirinda cyane ibiryo cyangwa amazi, no gukora uburiri ** Ikizamini cyo guhumeka kimwe gusa ** kugirango bakurikirane imitsi ihumeka mbere yuko inkeragutabara zihagera.

Ntutegereze ko pulse oximeter igabanuka mbere yo guhamagara serivisi zubutabazi

Muri syndrome ya Guillain-Barre hamwe nikibazo cya myasthenic, ** oximeter y'urutoki ($ SpO_2 $) nikimenyetso cyo gutinda kubi . Kubera ko ibihaha ubwabyo ari byiza, umuntu ufite intege nke za neuromuscular arashobora gukomeza gusoma ogisijeni isanzwe ya $ 96 \% inyandiko {kugeza} 99 \% $ ku mwuka w’icyumba mu gihe diafragma yabo yananiwe kandi urugero rwica dioxyde de carbone ($ CO_2 $) irimo kwiyongera mu maraso yabo ( hypercapnic respiratory failure **). Mugihe impiswi ya oxyde amaherezo igabanutse munsi ya $ 92 \% $, ifatwa ryubuhumekero akenshi iba isegonda cyangwa iminota mike. Buri gihe ujye ucira imanza imbaraga zo guhumeka ukoresheje ** Ikigeragezo cyo Guhumeka Kubara **, ijwi ryijwi, hamwe nubushobozi bwo kumira amacandwe, ntanarimwe na pulse oximetry yonyine.

Kora ubu

  1. ** Hamagara nimero yawe yihutirwa ako kanya niba intege nke zigenda zijyana no guhumeka, kumira, cyangwa guhindura ijwi. ** Hamagara 911 muri Amerika na Kanada, 112 hirya no hino mubumwe bw’ibihugu by’Uburayi, 999 mu Bwongereza, cyangwa urebe byose nimero yihutirwa. Bwira mu buryo bweruye uwabohereje: * "Umurwayi afite umuvuduko ukabije w'intege nke za neuromuscula [ukekwaho syndrome ya Guillain-Barre / Myasthenic Crisis] afite ibyago byo guhumeka neza." * Saba ambulanse ishigikira ubuzima bwihuse.
  2. ** Saba umuntu ugororotse kuri dogere 45 kugeza kuri dogere 90 (ntuzigere urambika umugongo) keretse niba nta ubwenge afite. Komeza wicare neza n'umutwe n'ijosi bishyigikiwe.
  3. ** Kora ku gitanda Ikizamini cyo Kubara Umwuka umwe (SBCT) ubungubu hanyuma wandike umubare. Umuntu mukuru ukuze abara $ 30 inyandiko {kugeza} 50 $; ** kutabasha kubara $ 20 $ byashize kumyuka imwe byerekana ko imitsi ihumeka ikabije yubuhumekero, hamwe no kubara $ 15 $ cyangwa munsi yibimenyetso byerekana ko hakenewe intubation ya ICU no guhumeka imashini **.
  4. ** Ntugumane umuntu rwose kumunwa (NPO) kandi ubafashe gucira amacandwe yuzuye. ** Ntutange amazi, ibiryo, cyangwa ibinini byo munwa (harimo ibinini bya pyridostigmine / Mestinon mugihe cyibibazo). Intege nke zo mu muhogo no mu mitsi (** intege nke za bulbar **) zitera amazi guhita amanuka mu muyaga uhuha mu bihaha, kandi pyridostigmine irenze urugero itera ururenda rwinshi rwa bronchial rwuzuza umwuka mubi.
  5. ** Kurikirana umuvuduko wumutima hamwe numuvuduko wamaraso kugirango uhindurwe gitunguranye (Autonomic Dysfunction). Ntureke ngo umuntu ahagarare vuba.
  6. ** Kusanya amacupa yimiti yose hamwe namateka yanduye kumurwi wibitaro. beta-blokers).

Ibihe byihutirwa hamwe na neuromuscular ibyemezo byubuhumekero igiti

Koresha iyi clinique clinique kugirango utandukanye syndrome ya Guillain-Barre, ikibazo cya myasthenique, kwikuramo uruti rwumugongo, hamwe na neurotoxine imanuka (nka botulisme cyangwa paralise), no kumenya kunanirwa guhumeka hakiri kare.

flowchart TD
    Start["Intege nke cyangwa Iterambere Byihuse Intege nke Zimitsi <br/> mumaguru yombi / Intwaro CYANGWA Amaso / Umuhogo / Ijosi"] --> AirwayScreen{"Akanya Bulbar & Diaphragm Mugaragaza: <br/> 1. Kubara Umwuka umwe <= 20 (cyangwa <= 15 bikomeye)? <br/> 2. Ijwi ryizuru, kuvuga nabi, cyangwa gukorora iyo umira? <br/> 3. Ijosi ridakomeye (umutwe ugwa imbere cyangwa ntushobora gukuramo umusego)? <br/> 4. Ntushobora kuryama neza udahumeka (Orthopnea)? <br/> 5. Kurohama munda mugihe cyo guhumeka (Guhumeka Paradoxique)?"}

    AirwayScreen -->|"YEGO: Umuntu wese uhari"| RespEmerg["KUNANIRA KUBIKORWA BIKURIKIRA: <br/> 1. Hamagara 911/112/999 Ako kanya kuri ALS Ambulance <br/> 2. Icara wihangane UBURENGANZIRA kuri dogere 45-90 (shyigikira ijosi ridakomeye) <br/> 3. NPO rwose (ntakintu nakimwe kumunwa; amacandwe mumacupa / igitambaro) <br/> 4. NTIWITANZE pyridostigmine (Mestinon) cyangwa imiti igabanya ubukana"]

    AirwayScreen -->|"OYA: Guhumeka no kumira muri iki gihe"| PatternCheck{"Ni ubuhe buryo bwa anatomique <br/> nuburyo bw'intege nke?"}

    PatternCheck -->|"KUGARAGARA (Hasi-Hejuru): <br/> Bitangirana no gutitira amano / intoki -> intege nke zamaguru zamaguru <br/> -> kuzamuka mukibuno / amaboko; gutakaza refleks ya tendon; <br/> ibyumweru 1-4 nyuma yimpiswi cyangwa uburwayi bwa virusi yubuhumekero"| SuspectGBS["SYNDROME YAKOREWE GUILLAIN-BARRE (GBS): <br/> - Kwimura ishami ryihutirwa ryihutirwa uyumunsi"]

    PatternCheck -->|"FLUCTUATING OCULO-BULBAR: <br/> Kunama amaso (ptose), kureba kabiri, urwasaya ruke / guhekenya, <br/> ijwi ryizuru, guta ijosi; bibi hamwe nimbaraga cyangwa nimugoroba; <br/> bizwi na Myasthenia Gravis cyangwa antibiotic / infection nshya"| SuspectMG["BITEKEREZO MYASTHENIYA GRAVIS YAKORESHEJWE / CRISIS: <br/> - Isuzuma ryihutirwa ryihutirwa ryihutirwa <br/> - Reba urutonde rwimiti ya fluoroquinolone, macrolide, beta-blokers <br/> - Witegure guhana ICU IVIG cyangwa Plasma (PLEX)"]

    PatternCheck -->|"DESCENDING (Hejuru-Hasi) hamwe nabanyeshuri bahamye: <br/> Kubona kabiri, abanyeshuri bagutse, umunwa wumye -> umuhogo -> amaboko -> amaguru; <br/> nta kunanirwa kumva; ibiryo biherutse murugo / ibiryo byabitswe"| SuspectBot["BOTULISM YEMEJWE CYANGWA TICK PARALYSIS: <br/> - Shakisha igihanga / umusatsi / imitsi yumubiri kumatiku yihishe <br/> - Reba Botulism & Neurotoxin Guide; Hamagara 911/112/999"]

    PatternCheck -->|"URWEGO RWA SENSORY SHARP + KUGARUKA K'UMUKARA:"| SuspectCord["BITEKEREZO BIKORESHWA BIKURIKIRA / CAUDA EQUINA: <br/> - Reba Cauda Equina & Spinal Cord Compression Guide <br/> - Hamagara 911/112/999 kugirango byihutirwa umugongo MRI"]

Gusobanukirwa ibintu bibiri byihutirwa byubwonko

1. Indwara ya Guillain-Barre (GBS): Igitero Cyiza Cyizamuka Cyimitsi

** Indwara ya Guillain-Barre (GBS) ** (izwi kandi nka ** Acute Inflammatory Demyelinating Polyradiculoneuropathie, cyangwa AIDP **, hamwe na variant axon ** AMAN ** na ** AMSAN **) ni igitero gikaze cya autoimmune yibasira imitsi ya peripheri n'imizi y'imitsi isohoka mu ruti rw'umugongo. - ** Uburyo bikururwa ("Molecular Mimicry"): ** Mubice 70% byabarwayi, GBS itangira ** ibyumweru 1 kugeza kuri 4 nyuma yo kwandura bisanzwe **, impiswi izwi cyane yibiryo byatewe na *** Campylobacter jejuni *** (akenshi ituruka ku nkoko zidatetse), cyangwa indwara zubuhumekero nka grippe, cytomegalovirus (CMV) umusonga *, virusi ya Zika, cyangwa SARS-CoV-2. Antibodies zakozwe na sisitemu yubudahangarwa kugirango zirwanye mikorobe ku buryo butunguranye zikorana na lipide ya ganglioside ku mitsi ya peripheri y’abantu, ikuraho icyuma cyitwa myelin ** (nko kwambura insina insinga z'amashanyarazi) cyangwa kwangiza imitsi ya nervice. - ** Uburyo GBS itera imbere: ** 1. ** Icyiciro cya mbere cya Sensory & Pain Phase (Amasaha Yumunsi): ** Abarwayi benshi babanje kubona ** guhuza "pin na inshinge" (paresthesiya) cyangwa kunanirwa mu mano no ku rutoki **, bikunze guherekezwa no kubabara cyane, kubabara, kubabara mu mugongo wo hepfo, mu kibuno, no ku bibero (biterwa no gutwika imizi yumugongo ya disiki ya disiki). 2. ** Kuzamuka kw'ibimuga (Iminsi kugeza ku byumweru 2 kugeza kuri 4): ** Intege nke zidasanzwe zitangirira mumaguru yombi (gukandagira hejuru yumuhanda, ingorane zo kuzamuka ingazi cyangwa guhagarara hejuru yintebe yumusarani) hanyuma ikazamuka hejuru hejuru yamasaha kugeza kumunsi mumatako, umutiba, amaboko, imitsi yo mumaso (akenshi bitera ** ubumuga bwo mumaso mumaso **, aho umuntu adashobora kumwenyura cyangwa gufunga ijisho **) diaphragm . 3. ** Gutakaza Reflexes ya Deep Tendon (Areflexia): ** Iyo umuganga akubise ikivi cyangwa ikirenge akoresheje inyundo ya refleks, reflex irahari cyangwa iragabanuka cyane. 4. ** Autonomic Nervous Sisitemu Ihungabana: ** Abagera kuri bibiri bya gatatu by'abarwayi ba GBS bafite ihungabana ribi rya sisitemu y'imitsi itabishaka, itera umuvuduko ukabije w'amaraso, umuvuduko ukabije w'umutima ( bradycardia / asystole **), kugumana inkari, cyangwa ileus (guhagarika amara). - ** Indwara ya Miller Fisher Syndrome (MFS) Impinduka: ** Impinduka yihariye ya GBS yimura ** hejuru-hasi ** aho kuba hasi-hejuru, irangwa na triad classique ya ** ophthalmoplegia ** (kugenda mumaso yamugaye itera iyerekwa rikabije), ** ataxia ** (kudahagarara bikabije no gutakaza guhuza), na ** areflexia ** (reflexes idahari).

2. Ikibazo cya Myasthenic: Kunanirwa kwa Neuromuscular

** Myasthenia Gravis (MG) ** ni indwara ya autoimmune aho antibodies (cyane cyane ** anti-acetylcholine reseptor [AChR] antibodies ** cyangwa ** anti-MuSK antibodies **) zifunga cyangwa zisenya imiti ya mikorosikopi ya fibre yimitsi aho imitsi irekura neurotransmitter ** acetylcholine . - ** Kugaragaza intege nke zinaniza: ** Kuberako imitsi ifite reseptor nke zakazi, kugabanuka kwimitsi ya mbere irashobora kumva ko ari ibisanzwe, ariko gukoresha inshuro nyinshi bigabanya vuba ihuriro ( umunaniro **). Ibimenyetso nibisanzwe byoroheje mugitondo nyuma yo kuruhuka bikarushaho kwiyongera nyuma yumunsi cyangwa mugihe cyo kuganira, guhekenya, cyangwa kureba hejuru. - ** Ikibazo cya Myasthenic ni iki: ** A ** myasthenic crise ** ni ubukana bwangiza ubuzima aho intege nke zikwirakwira kumitsi ya ** bulbar (umuhogo, ururimi, umunwa, urwasaya) ** na ** imitsi yubuhumekero (diaphragm nurukuta rwigituza) ** bihagije kugirango bibangamire uburinzi bwumuyaga cyangwa bisaba guhumeka neza. Abagera kuri 15 kugeza kuri 20 ku ijana by'abarwayi bafite Myasthenia Gravis bahura byibura n'ikibazo kimwe cya myasthenique, kandi ku barwayi bamwe na bamwe, ikibazo ni ubwa mbere basuzumye Myasthenia Gravis. - ** Imbarutso isanzwe yibibazo bya Myasthenic: ** - Indwara zikomeye z'ubuhumekero cyangwa inkari n'indwara. - Kubaga, ihungabana ry'amarangamutima, gutwita, cyangwa kubyara. - Gutangira corticosteroide ikabije (prednisone irashobora gutera paradox yinzibacyuho gukomera kwintege nke za MG muminsi 5 kugeza 14). - ** Ubuyobozi bwimiti yanduye ** ibuza kwanduza imitsi (reba Imbonerahamwe Yumuti Yangiza).

Kugereranya kwuzuye: Indwara ya Guillain-Barre na Myasthenic Crisis na Botulism na Spinal Cord Compression

Kubera ko intege nke zikomeye zishobora guturuka mu ruti rwumugongo, imitsi ya periferique, cyangwa ihuriro ry’imitsi, abaganga bakoresha ibi bimenyetso bitandukanya uburiri:

Ikiranga ivuriro Indwara ya Guillain-Barre (GBS) Ikibazo cya Myasthenic (Myasthenia Gravis) Ibiribwa cyangwa ibikomere Botulism Gukomeretsa Uruti rw'umugongo / Guhindura Myelitis Indwara yamugaye
** Icyerekezo cyintege nke ziterambere ** ** Kuzamuka (Hasi-Hejuru) **: Bitangirira mu birenge / amaguru hanyuma bikazamuka ku gihimba, amaboko, mu maso, na diaphragm (usibye variant ya Miller Fisher). ** Oculo-Bulbar kuri Rusange **: Itangirira mumaso / amaso, urwasaya, umuhogo, nijosi, hanyuma ikwirakwira mubitugu, diaphragm, no mumaguru. ** Kumanuka (Hejuru-Hasi) **: Itangirira mumaso, abanyeshuri, no mu muhogo, hanyuma ikamanuka ijosi, amaboko, diaphragm, n'amaguru. ** Munsi y'urwego rwihariye rw'umugongo **: Ubumuga butunguranye bw'amaguru yombi (cyangwa ingingo zose uko ari 4 niba inkondo y'umura) munsi ya vertebra ikomye. ** Kuzamuka (Hasi-Hejuru) **: Bitangirana no kugenda no kudahagarara ukuguru, kuzamuka kumutwe, amaboko, no guhumeka mumasaha 24-48.
** Ibimenyetso Byumviro (Kunanirwa / Gutitira) ** ** Kugeza ubu: Kunyeganyeza amano n'intoki + ububabare bwimbitse bwinyuma / ikibero cyububabare bwumuzi. ** Kubura rwose (0%) **: Kumva no gukoraho nibisanzwe 100%; nta gucogora cyangwa gutitira. ** Ntibihari rwose (0%) **: Kumva ni ibisanzwe 100% (keretse ububabare butandukanye). ** Ikarishye "Urwego Rukomeye" **: Umurongo utambitse utambitse hejuru yigituza, inda, cyangwa igituba munsi aho gukoraho / pinprick byatakaye. ** Ntoya cyangwa idahari **: Mubisanzwe nta gutakaza ibyiyumvo (bishobora kuba byoroheje); nta muriro cyangwa kubabara umugongo.
** Ijisho n'ibimenyetso by'abanyeshuri ** Abanyeshuri basanzwe; irashobora gukura ** intege nke zo mumaso ** (ntishobora gufunga amaso) cyangwa ubumuga bwimitsi yijisho muburyo bwa Miller Fisher. ** Kunama hejuru y'amaso (ptose) ** no kubona kabiri bikabije iyo ureba hejuru amasegonda 30; ** abanyeshuri bitabira bisanzwe kumuri **. ** Abanyeshuri baragutse, batinda / batuje (mydriasis) ** + ijisho ryunamye, iyerekwa kabiri, numunwa wumye cyane. Amaso asanzwe hamwe nabanyeshuri (keretse niba ijosi rirerire ritera syndrome ntoya ya Horner). Abanyeshuri mubisanzwe; irashobora guteza imbere iyerekwa kabiri cyangwa intege nke zo mumaso bitinze.
** Impinduka zimbitse za Tendon (Amavi / Amaguru) ** ** Kubura cyangwa kugabanuka cyane (Areflexia) ** hakiri kare uburwayi. ** Ibisanzwe (Byuzuye) ** refleks ya tendon. ** Ubusanzwe kare **, hanyuma bigabanuke uko imitsi ihinduka ubumuga rwose. ** Kugabanuka hakiri kare (uruti rw'umugongo) **, hanyuma ** hyperactive (brisk) ** hamwe n'ikimenyetso cy'amano ya Babinski. ** Kubura (Areflexia) **, kwigana hafi syndrome ya Guillain-Barre.
** Uruhago n'imikorere y'amara ** Mubisanzwe muburyo bwambere (nubwo kugumana inkari byigenga bishobora gukura nyuma mubihe bikomeye). ** Ntibisanzwe **: Uruhago rusanzwe no kugenzura amara. Kuribwa mu nda no kugumana inkari birasanzwe kubera guhagarika imitsi ya autonomic. ** Kera, kugaragara cyane kwinkari cyangwa kutigomeka ** + gutakaza ijwi ryurukiramende (reba Indwara ya cauda). Uruhago rusanzwe no kugenzura amara.
** Ako kanya Imfashanyo Yambere & Igisobanuro Cyuzuye ** ** Hamagara 911/112/999 **; icara neza; Kubara Umwuka umwe; ICU kuri ** IVIG cyangwa Plasma Guhana (PLEX) **. ** Hamagara 911/112/999 **; icara neza; NPO; kwima Mestinon y'inyongera; ICU kuri ** IVIG cyangwa PLEX **. ** Hamagara 911/112/999 **; ICU ihumeka + ako kanya ** igereranya heptavalent botulinum antitoxine ** (reba botulism). ** Hamagara 911/112/999 **; komeza umugongo; byihutirwa ** MRI + kubaga decompression cyangwa IV steroid **. ** Shakisha igihanga cyose, umusatsi, inyuma yamatwi, hamwe nuruhu rwuruhu rwa tike yashizwemo **: gukuramo amatiku bikiza ubumuga mumasaha.

Nigute ushobora kubona kunanirwa guhumeka neuromuscular murugo (Mbere ya Oximeter Igitonyanga)

Ku muntu urwaye asima cyangwa umusonga, ikibazo cyo guhumeka kiragaragara: bahumeka, bakorora cyane, kandi umwuka wa ogisijeni ugabanuka. Mu muntu urwaye syndrome ya Guillain-Barre cyangwa Myasthenia Gravis, ** imitsi itera umwuka cyangwa inkorora ikabije ni imitsi ubwayo iba ikimuga **. Reba kuri ibi bimenyetso bitanu byihariye bya ** bulbar na diaphragmatic gutsindwa **:

1. Ikizamini cyo kuryama kimwe-Guhumeka Ikizamini (SBCT): Proxy yemewe kubushobozi bwibihaha (FVC)

Mu bitaro ICU, abaganga bapima ** Ubushobozi bwibihaha ku gahato (FVC) ** na ** Negative Inspiratory Force (NIF) ** bakoresheje spirometero (gukoresha itegeko rya ** "20/30/40" : intubation irakenewe mugihe Ubushobozi bwibihaha bugabanutse munsi ya $ 20 inyandiko {mL / kg} $, Umuvuduko mwinshi wa Expiratory uri munsi ya $ 40 inyandiko {cmH} _2 inyandiko {O} $). Murugo, ** Ikizamini cyo Guhumeka Umwuka umwe ** gifitanye isano itaziguye nubushobozi bwibihaha ku gahato (buri mubare ubarwa uhwanye n $ 100 inyandiko {kugeza} 115 inyandiko {mililitiro} $ yubushobozi bwibihaha): - ** Uburyo bwo kubikora: ** Saba umuntu yicare neza, ahumeke neza cyane, kandi abare hejuru ($ 1, 2, 3, 4, 5 ... $) ku muvuduko wa nimero ** 2 ku isegonda ** (guhuza ukuboko kwa kabiri kw'isaha) kugeza igihe bagomba guhumeka. - ** Nigute wasobanura amanota: ** - ** Kubara $> 30 $: ** Ubusanzwe imitsi yubuhumekero isanzwe (komeza usuzume buri masaha 1 kugeza kuri 2 niba GBS itera imbere). - ** Kubara $ 20 inyandiko {kugeza} 25 $: ** Intege nke zimitsi yubuhumekero ( Ishami ryihutirwa ryihutirwa risabwa ako kanya ). - ** Kubara $ le 15 $ kugeza $ 20 $: ** Bihuye nubushobozi bwibihaha ku gahato (FVC) munsi y $ 15 inyandiko {kugeza} 20 inyandiko {mL / kg} $ ( Akaga gakomeye: Hamagara 911/112/999 ako kanya; umurwayi birashoboka ko bisaba kurinda ICU inzira yo guhumeka **). - ** Icyerekezo cyo kumanuka: ** Umurwayi ufite ibara ryamanutse kuva $ 35 $ saa sita kugeza 22 $ saa tatu za mugitondo aragenda yangirika vuba kandi agomba kuba muri ICU mbere yijoro.

2. Intege nke zo mu ijosi (Ikimenyetso "Umutwe Wumutwe / Kuzamura umusego")

Imitsi izamura umutwe ku musego cyangwa ifata urushyi hejuru (** flexors cervical flexors na extensors **, itangwa nu mizi yumutima winkondo y'umura C2 kugeza kuri C5) isangiye neza na neza imizi yumugongo yumutima wumugongo nka ** frenic nervice (C3, C4, C5) ** itanga diafragma ("C3, 4, 5 ituma diafragma ibaho"). - ** Ikizamini: ** Niba umuntu aryamye kuri dogere 30 kandi ** ntashobora kuzamura umutwe kumusego kumasegonda 5 , cyangwa niba umunwa wabo ukomeje kumanuka imbere yigituza wicaye ( umutwe wumutwe **), diafragma yabo iracika intege rwose kuruhande rwimitsi yijosi.

3. Intege nke za Bulbar (Ibimenyetso byo Kunanirwa Kurinda Umuyaga)

Mbere yuko diafragma inanirwa burundu, intege nke zo mu muhogo, ururimi, no mu magage yoroshye (** bulbar palsy **) byangiza ubushobozi bwumuntu bwo kwirinda amacandwe mu bihaha: - ** Guhindura amajwi: ** Ijwi ryabo ryumvikana gitunguranye ** hypernasal ("kuvuga mumazuru yabo") **, yoroshye, gutontoma, cyangwa gutitira, cyane cyane nyuma yo kuvuga amasegonda 30. - ** Kugarura amazuru: ** Iyo bagerageje kunywa amazi, amazi asubira mumazuru yabo kuko umunwa woroshye udashobora gufunga inzira yizuru. - ** Inkorora idakomeye "bovine": ** Iyo usabwe gukorora ku gahato, inkorora yumvikana neza, ihumeka, kandi ifite intege nke (idashobora gukuramo flegime mu muhogo). - ** Guhekenya umunaniro (muri Myasthenia Gravis): ** Urwasaya rwabo runaniza ifunguro rya saa sita, bibahatira gufunga urushyi n'intoki.

4. Guhumeka mu nda ya paradoxique na orthopnea

  • ** Orthopnea: ** Umuntu yahise agira ubwoba cyangwa yumva adashobora guhumeka mugihe bicaye ku buriri, kuko uburemere butagikura ingingo zo munda ziva munzira ya diafragma yabo idakomeye.
  • ** Guhumeka Paradoxical (Paradomical Abdominal): ** Mubisanzwe, iyo uhumeka neza, diafragma yawe irasunika hasi kandi inda yawe igenda ** hanze **. Iyo diafragma yamugaye, imitsi yigituza igerageza kunyunyuza umwuka kandi mubyukuri ikurura diafragma ya floppy hejuru mu gituza, bigatuma inda yo hejuru ** irohama INWARD mugihe cyo guhumeka **. Nibintu byihutirwa bisaba gutwara ambulance byihuse.

5. Ibimenyetso byatinze byo kubika Dioxyde de Carbone (Hypercapnia)

Iyo guhumeka bibaye bike cyane kugirango uhoshe karuboni ya dioxyde ($ CO_2 $), reba ** ibyuya byo mu ruhanga (diaphoresis), impiswi yihuse, umuvuduko ukabije wamaraso, kubabara umutwe mugitondo, guhangayika, guhagarika umutima, hanyuma amaherezo gusinzira cyangwa kwitiranya **. Ntuzigere utanga ibinini cyangwa ibinini byo kuryama kumurwayi utuje ufite GBS cyangwa Myasthenia Gravis; ntibaruhuka kuko barimo guhumeka buhoro buhoro bitewe na dioxyde de carbone izamuka.

Ibyago bidasanzwe bya Myasthenia Gravis: Ibiyobyabwenge byanduye hamwe na Cholinergic Crisis

1. Imiti ishobora gukurura ikibazo gikomeye cya Myasthenic

Niba wowe cyangwa uwo ukunda ufite Myasthenia Gravis, imiti myinshi yandikirwa burimunsi hamwe nibiyobyabwenge birenze imiti irashobora guhagarika imiyoboro isigaye ya neuromuscula kandi bigashobora gufatwa nubuhumekero butunguranye mumasaha make. Buri gihe ugenzure ibiyobyabwenge byatangiye kurwanya urutonde rwibyago byinshi byashyizweho na ** Myasthenia Gravis Foundation of America (MGFA) ** hamwe nubuyobozi mpuzamahanga bw’imitsi:

Icyiciro Cyinshi Cyibiyobyabwenge Imiti yihariye yo kwirinda cyane cyangwa gukoresha gusa muri ICU Impamvu Bitera Ikibazo muri Myasthenia Gravis
** Antibiyotike ya Fluoroquinolone (Iburira ry'agasanduku k'umukara) ** ** Ciprofloxacin, levofloxacin, moxifloxacin, ofloxacin ** Ihagarika mu buryo butaziguye kwanduza imitsi; itwara US FDA Boxe Yiburira Kubuza gukoreshwa muri Myasthenia Gravis.
** Antibiyotike ya Macrolide & Ketolide ** ** Azithromycin (Z-Pak), Clarithromycine, erythromycine, telithromycine ** Kubangamira irekurwa rya presynaptic acetylcholine kandi bikarushaho gukomera no guhumeka vuba.
** Antibiyotike ya Aminoglycoside ** ** Gentamicin, tobramycin, amikacin, neomycine, streptomycine ** Irabuza calcium kwinjira muri nervinal nervice, ikabuza kurekura acetyloline.
** Imitsi yinjira-nini cyane-Magnesium ** ** IV magnesium sulfate ** (yatanzwe kuri preeclampsia cyangwa arththmias), ** dosiye nini ya magnesium laxatives / antacide (amata ya magnesia, umunyu wa Epsom) ** Magnesium irushanwa na calcium mu masangano ya neuromuscular, itera ubumuga bwihuse ku barwayi ba MG.
** Ibiyobyabwenge byumutima (Beta-Blockers & Kalisiyumu) ​​** ** Propranolol, metoprolol, atenolol, labetalol, ijisho rya timolol **, ** verapamil, diltiazem **, na ** procainamide / quinidine ** Kugabanya uburyo bwo kwanduza ubwonko bwa neuromuscular kandi burashobora guhishura cyangwa gukomera intege nke za myasthenic.
** Inshinge za Botulineum Uburozi ** ** OnabotulinumtoxinA (Botox) nuburozi bwose bwo kwisiga cyangwa migraine botulineum ** Kurandura burundu poroteyine SNARE ikenewe kugirango irekure acetyloline, itera intege nke za sisitemu kubarwayi ba MG.
** Inzitizi za Kanseri Ikingira ** ** Pembrolizumab, nivolumab, ipilimumab, atezolizumab ** Irashobora gukurura * de novo * autoimmune Myasthenia Gravis ikunze guhuzwa na myocarditis yica (imitsi yumutima yumutima) na myosite.
** Sedatives, Opioide, hamwe norohereza imitsi ** ** Benzodiazepine (lorazepam, diazepam, alprazolam), opioide (morphine, oxycodone, fentanyl), cyclobenzaprine, baclofen ** Kurwanya imiyoboro yubuhumekero hagati kandi ikoroshya imitsi yo mu kirere yo hejuru, igabanya intege nke zumupaka muburyo bwo guhumeka neza.

2. Impamvu utagomba gutanga Pyridostigmine yinyongera (Mestinon) mugihe cyibibazo

Abarwayi benshi barwaye Myasthenia Gravis bafata umunwa wa buri munsi ** pyridostigmine (Mestinon) , inhibitor ya acetylcholinesterase yongerera by'agateganyo urugero rwa acetyloline ku mitsi yakira imitsi. Iyo umurwayi atangiye guhangana no kumira cyangwa guhumeka, imiryango ikunze kumva ishaka kumenagura no gutanga ibinini byinyongera bya Mestinon. ** Ntuzigere ukora ibi mugihe gikomeye: ** 1. ** Aspiration Hazard: ** Niba imitsi yo mu muhogo idakomeye, kumira ibinini n'amazi bitera guhita uniga no guhaha umusonga. 2. ** Umwuzure wa Mucus (Kurenza Muscarinic): ** Pyridostigmine itera glande y'amacandwe na glande ya mucus ya bronchial gusuka imyunyu ngugu, amazi ( salivation na bronchorrhea ) mugihe nayo igabanya imiyoboro ya bronchial. Umurwayi ufite inkorora idakomeye ntashobora gukuraho uyu mwuzure wamacandwe na mucus kandi arashobora kurohama mumyanya yabo. 3. ** Ikibazo cya Cholinergic: ** Gufata urugero rwinshi rwa pyridostigmine bikangura cyane kandi bigatandukanya ibyakirwa byimitsi kugeza bifunze bikananirana burundu ( cholinergic crisis **), bigatera ubumuga bukabije hamwe nibimenyetso bya ** SLUDGE **: profuse ** S ** alivation, ** L ** acrimation (kurira), ** U ** rination, ** D ** iarrhe ** E.

Intambwe ku yindi ubufasha bwambere mugihe utegereje ambulance

Intambwe ya 1: Inzira nziza nziza na Diaphragm

  • ** Niba ukangutse kandi uhumeka wenyine: ** Wicare umuntu ** ugororotse kuri dogere 45 kugeza kuri dogere 90 ya dogere , cyangwa ubasunike imbere gato hamwe namaboko yabo ahagarara kumeza cyangwa umusego ( imyanya itatu ya trode **) bityo uburemere bukurura ibibyimba byo munda hasi kandi bigatanga diaphragm icyumba kinini cyo kwaguka.
  • ** Shyigikira ijosi ridakomeye: ** Niba umuntu afite intege nke zijosi (umutwe ugana imbere), shyira witonze igitambaro kizungurutse inyuma y ijosi cyangwa ushyigikire uruhanga rwabo hamwe numusaya kugirango umuyaga udahita ufunga.
  • ** Guhangana n'amacandwe yuzuye: ** Guha umuntu igitambaro, imyenda, cyangwa igikombe hanyuma ubategeke ** kwunama imbere gato hanyuma ucire amacandwe hanze ** aho kugerageza kuyamira.
  • ** Niba utazi ubwenge cyangwa kuruka ushishikaye: ** Uzunguruke ako kanya kuruhande rwabo mumwanya wa ** wo gukira kuruhande ** hanyuma uhanagure ibirutsi byose bigaragara cyangwa urusenda rwinshi uhereye imbere yumunwa.

Intambwe ya 2: Koresha "Ikizamini cya Pack Pack"

Niba umuntu afite uburibwe butunguranye bw'ijisho rimwe cyangwa byombi hejuru (** ptose ) ukaba utegereje isuzuma ry'ubuvuzi (udatinze guhamagara byihutirwa niba guhumeka cyangwa kumira bigira ingaruka): - Wizike ipaki cyangwa umufuka wamashaza yakonjeshejwe mumyenda yoroheje hanyuma uyifate witonze hejuru yumutwe ufunze kuminota ** 2, hanyuma uyikureho uhite ureba mumaso. - ** Icyo bivuze: ** Ubushyuhe bukonje butinda enzyme ( acetylcholinesterase ) isenya acetylcholine. Niba ijisho ryunamye rifunguye ku buryo bugaragara ( itezimbere $ 2 inyandiko {milimetero} $ cyangwa irenga **) mu minota mike ukimara gukuramo ipaki, ni ikimenyetso cyiza cya ** Myasthenia Gravis ** (hafi 80% sensitivite). Bwira umuganga wibyumba byihutirwa ibyo wabonye.

Intambwe ya 3: Ibimenyesha Ibitaro Byibanze kubatabazi n'abaganga (Iburira rya Succinylcholine)

Niba umurwayi urwaye Syndrome ya ** Guillain-Barre ** (cyangwa subacute iyo ari yo yose yerekana ubumuga bumara amasaha arenga 48 kugeza kuri 72) bisaba intubation yihutirwa muri ambulance cyangwa ibitaro, ** abaganga byihutirwa ntibagomba na rimwe gukoresha imitsi ya depolarizing imitsi iruhura succinylcholine (suxamethonium) **: - ** Impamvu: ** Imitsi yanduye muri syndrome ya Guillain-Barre igenga reseptor ya acetylcholine mumitsi yose. Gutanga succinylcholine bitera umuyoboro wa potasiyumu muri iyo mitsi guta potasiyumu mumaraso icyarimwe, bigatuma ** ako kanya, hyperkalemic yumutima ifata ** mumasegonda 60. Ahubwo, abaganga bakoresha imitsi itagabanya imitsi (nka rocuronium kuri dosiye yagenwe). Kuvuga * "Gukekwaho syndrome ya Guillain-Barre" * biragaragara ko ari ukuboko kurinda umurwayi iyi miti yica.

Inyandiko itumanaho neza kuri 911/112/999 hamwe no Gutwara Ibitaro

Kubera ko umurwayi uri muri syndrome ya Guillain-Barre cyangwa Myasthenic Crisis akenshi aba afite okisimetero isanzwe ya pulse ($ 98 \% $) kandi akavuga atuje, abakozi ba triage yihutirwa barashobora gupfobya uburyo begereye ifatwa ryubuhumekero. Koresha iyi mvugo nyayo yubuvuzi:

  • ** Kubakekwa Syndrome ya Guillain-Barre: ** . [Umubare], kandi [afite] ikibazo cyo kumira / inkorora idakomeye / kudashobora gukura umutwe ku musego]. Turakeka ko Syndrome ya Guillain-Barre ifite ikibazo cyo gutsindwa kwa diaphragmatique. "*

  • ** Kubikekwa Ikibazo cya Myasthenic: **

    • Gukurikirana ibihaha ku gahato (FVC), kugenzura ubuhumekero, na IVIG cyangwa plasmapheresi. "*

Urutonde rwo gutegura ibitaro mugihe utegereje EMS

  • ** Igitabo cyanditse cyerekana amanota yikizamini kimwe cyo guhumeka hamwe nigihe cyamasaha ** (urugero, * "1:00 PM kubara = 26; 2:30 PM kubara = 17" *).
  • ** Igihe ntarengwa cyintege nke ziterambere ** (mugihe gutitira byatangiye, mugihe kugenda byananiranye, iyo kumira cyangwa imvugo yahindutse).
  • ** Amateka yanduye vuba aha, uburozi bwibiryo, cyangwa inkingo ** mubyumweru 6 bishize.
  • ** Amacupa yimiti yose yubu ** (cyane cyane antibiotique nshya yatangiriye muminsi 7 ishize, pyridostigmine / Mestinon, prednisone, azathioprine, mycophenolate, efgartigimod, cyangwa eculizumab).
  • ** Kugenzura byihuse igihanga n'umusatsi kuri tike ifatanye ** (cyane cyane mubana cyangwa ba mukerarugendo bafite ubumuga bwihuta kandi nta gutakaza amarangamutima).

Fata inzira

Gufata ibyemezo byambere byemejwe hamwe na CPR bivuye muri Croix-Rouge, Ishyirahamwe ry’umutima ry’Abanyamerika, St John Ambulance, cyangwa umutanga w’ibanze wihutirwa biguha ubumenyi bufatika bwo gukumira inzitizi zo mu kirere, gushyira umurwayi utitabira neza, no gukora guhumeka gutabara no kwikomeretsa mu gatuza iyo habaye guhumeka.

Ibibazo bikunze kubazwa

Kuki oximeter y'urutoki ishobora gusoma 98% ogisijeni mugihe umuntu urwaye syndrome ya Guillain-Barre cyangwa Myasthenia Gravis ari hafi gufatwa n'ubuhumekero?

Imisemburo ya pulse irapima umwuka wa ogisijeni mu maraso, uguma usanzwe igihe cyose imifuka yo mu kirere iri mu bihaha iba isobanutse. Muri syndrome ya Guillain-Barre hamwe na myasthenique, ibihaha ni byiza, ariko diaphragm n'imitsi yo mu gatuza biba intege nke kuburyo bidashobora guhumeka neza (** hypoventilation **). Ibi bitera uburozi bwa dioxyde de carbone ($ CO_2 $) kwiyubaka bucece mumaraso mugihe umubare wa ogisijeni ukomeje gusoma 96% kugeza 98%; iyo diafragma imaze kurangira burundu, ogisijeni igabanuka no gufatwa k'umutima bikurikira muminota mike.

Nigute nakora Ikizamini cyo Kubara Umwuka umwe murugo, kandi numero niyihe yihutirwa?

Saba umuntu kwicara neza, ahumeka neza cyane bishoboka, kandi abare hejuru cyane kuva 1 hejuru (1, 2, 3, 4 ...) kumuvuduko uhamye wimibare ibiri kumasegonda kugeza igihe agomba guhumeka. Umuntu mukuru ukuze arashobora kubara kugeza hagati ya 30 na 50; kutabasha kugera kuri 20 byerekana intege nke zimitsi yubuhumekero isaba ubuvuzi bwihutirwa, kandi kubara 15 cyangwa munsi byerekana kunanirwa guhumeka byihuse bisaba ambulance yihuse hamwe nubufasha bwumuhanda wa ICU.

Kuki umuntu utarwaye Myasthenia Gravis adakwiye gufata ibinini bya Mestinon (pyridostigmine) mugihe atangiye kugira ikibazo cyo kumira cyangwa guhumeka?

Kumira ibinini n'imitsi yo mu muhogo bidakomeye birashobora guhita biniga no kwifuza mu bihaha. Byongeye kandi, pyridostigmine irenze itera glande y'amacandwe hamwe nibihaha gusuka ururenda rwijimye umurwayi ufite intege nke adashobora gukorora, kandi bishobora gutera "cholinergic crise" mubyukuri bikomeretsa imitsi.

Nigute abaganga bashobora gutandukanya syndrome ya Guillain-Barre no gukomeretsa umugongo niba byombi bitera ubumuga bwamaguru?

Indwara ya Guillain-Barre yibasira imitsi ya periferique, itera intege nke ziva kumano hejuru, gutakaza ivi n'amaguru ya refleks, no gutitira ibirenge n'amaboko nta murongo utyaye utambitse wo gutitira ku mubiri. Ibinyuranye na byo, kwikuramo uruti rw'umugongo mubisanzwe bitera "urwego rwo kumva" rutambitse ku gituza cyangwa mu nda munsi aho ibyiyumvo byose bitakara, kutabasha kwihagarika hakiri kare (kugumana uruhago), hamwe n'ububabare bukabije bw'umugongo, ibyo bikaba byemezwa ako kanya n'umugongo wihutirwa MRI.

Inkomoko yagenzuwe

  • ** Ishuri Rikuru ry’Uburayi (EAN) na Sosiyete Sivile ya Periferique (PNS): ** * 2023/2024 Amabwiriza ya EAN / PNS yerekeye gusuzuma no kuvura indwara ya Guillain-Barré * (* Ikinyamakuru cya Periferique Nervous System * / * Ikinyamakuru cyo mu Burayi cya Neurologiya *, van Doorn n'abandi).
  • ** Fondasiyo ya Myasthenia Gravis yo muri Amerika (MGFA, Amerika) & Amabwiriza mpuzamahanga y’ubwumvikane: ** * Amabwiriza mpuzamahanga y’ubwumvikane mu micungire ya Myasthenia Gravis * (Sanders et al. / Narayanaswami n'abandi.
  • ** Sosiyete ishinzwe ubuvuzi bwa Neurocritical (NCS): ** * Amabwiriza yo gucunga neza ikibazo cyo guhumeka neza kwa Neuromuscular muri Syndrome ya Guillain-Barré na Myasthenic Crisis * (kwemeza itegeko rya 20/30/40 hamwe nigitanda Ikizamini cyo Kubara Umwuka umwe).
  • ** Ikigo cy'igihugu gishinzwe ubuzima no kwita ku ndashyikirwa (NICE, Ubwongereza) & Ishyirahamwe ry’aba Neurologiste b'Abongereza (ABN): ** * Intege nke za Neuromuscular Intege nke hamwe na Guillain-Barré Syndrome Syndrome Amabwiriza Yihutirwa Yihutirwa *.
  • ** Ishami ry'Umuryango w'Abibumbye ryita ku Buzima (OMS) & Ibigo bishinzwe kurwanya no gukumira indwara (CDC, Amerika): ** * Kumenyekanisha ivuriro no kugenzura indwara ya Guillain-Barré na Botulism Isuzuma ritandukanye *.

Kuyobora