Dab tsi yog myasthenia congenital, cov tsos mob thiab kev kho mob

Zoo Siab

• Cov tsos mob ntawm Congenital Myasthenia
• Yuav kuaj li cas
• Kev Kho Mob Rau Congenital Myasthenia
• Kho puas tau myasthenia los kho?

Congenital Myasthenia yog ib yam kab mob uas cuam tshuam rau ntawm lub hauv siab neuromuscular thiab yog li ua rau cov leeg muaj zog nce mus ntev, feem ntau ua rau tus neeg yuav tsum taug kev hauv lub laub muaj log. Tus kab mob no tuaj yeem tshawb pom nyob rau thaum tiav hluas lossis neeg laus thiab nyob ntawm seb hom kev hloov caj ces ntawm tus neeg muaj, nws tuaj yeem kho tau nrog kev siv tshuaj kho mob.

Ntxiv rau cov tshuaj uas tau qhia los ntawm tus kws kho mob hlwb, kev tawm dag zog kuj tseem yuav tsum tau rov qab los ua kom cov leeg muaj zog thiab sib koom ua ke, tab sis tus neeg tuaj yeem taug kev ib txwm nyob dua, tsis tas yuav thawb lub laub lossis laub.

Congenital myasthenia tsis yog tib yam li Myasthenia gravis vim tias qhov tshwm sim ntawm Myasthenia Gravis qhov ua rau muaj kev hloov pauv hauv tib neeg lub cev tiv thaiv kab mob, thaum nyob rau hauv myasthenia congenital ua rau muaj kev hloov caj ces, uas nquag tau hauv tib neeg hauv tib tsev neeg.

Cov tsos mob ntawm Congenital Myasthenia

Cov tsos mob ntawm Congenital Myasthenia feem ntau tshwm sim hauv menyuam yaus lossis nruab nrab ntawm 3 txog 7 xyoo, tab sis qee yam tshwm sim ntawm 20 txog 40 xyoo, uas tuaj yeem yog:

Hauv tus menyuam:

• Nyuaj nyob hauv kev pub niam mis lossis pub mis pub mis, txhawm chim yooj yim thiab dag zog yuam kom nqus;
• Hypotonia uas ua tau nws tus kheej los ntawm qhov tsis muaj zog ntawm caj npab thiab ceg;
• Dhuav cov tawv muag;
• Cov ntawv cog lus sib koom (kev sib daj sib deev hnub tim);
• Txo qhov ntsej muag zoo nkauj;
• Ua pa nyuaj thiab ntxuav cov rau tes thiab daim di ncauj;
• Kev loj hlob qeeb rau zaum, nkag thiab taug kev;
• Cov menyuam loj dua yuav pom tias nyuaj nce ntaiv.

Hauv cov menyuam yaus, cov hluas lossis cov neeg laus:

• Kev tsis muaj zog ntawm txhais ceg lossis caj npab nrog tingling ncus;
• Nyuaj taug kev nrog qhov xav tau los zaum kom so;
• Tej zaum yuav muaj qhov tsis muaj zog hauv cov leeg qhov muag uas thim tawv muag;
• Nkees thaum sib zog me me;
• Tej zaum muaj scoliosis hauv nqaj qaum.

Muaj 4 yam sib txawv ntawm qhov ua tiav myasthenia: qeeb channel, tsis tshua muaj affinity ceev channel, AChR tsis txaus lossis AChE tsis txaus. Txij li thaum yug me nyuam qeeb-channel myasthenia tuaj yeem tshwm sim nruab nrab ntawm 20 thiab 30 xyoo. Txhua yam nws muaj nws tus yam ntxwv thiab txoj kev kho kuj tuaj yeem sib txawv ntawm ib tus neeg mus rau lwm tus vim tias txhua tus tsis muaj cov tsos mob tshwm sim.

Yuav kuaj li cas

Kev kuaj mob ntawm Congenital Myasthenia yuav tsum tau ua raws li cov tsos mob tau nthuav tawm thiab tuaj yeem lees paub los ntawm kev kuaj mob xws li kuaj ntshav CK thiab kev kuaj mob caj ces, tshuaj tiv thaiv kab mob kom paub meej tias nws tsis yog Myasthenia Gravis, thiab electromyography uas ntsuas qhov zoo ntawm qhov kev cais tawm. nqaij, piv txwv li.

Hauv cov menyuam loj dua, cov hluas thiab cov laus, tus kws kho mob lossis kws kho mob hauv lub cev kuj tseem tuaj yeem ua qee qhov kev ntsuas hauv chav ua haujlwm kom paub txog cov leeg tsis muaj zog, xws li:

• Saib saum qab nthab li 2 feeb, kho thiab soj ntsuam yog tias muaj qhov ntsuas tsis zoo ntawm qhov nyuaj los khaws lub tawv muag;
• Tsa koj ob sab caj npab rau pem hauv ntej, txog ntawm lub xub pwg kom siab, tuav txoj haujlwm no rau 2 feeb thiab saib seb nws puas tuaj yeem tswj txoj kev cog lus no lossis yog tias koj ob txhais npab poob;
• Tsa lub ncab dua yog tias tsis muaj kev pab ntawm koj ob txhais caj npab ntau dua 1 lub sijhawm lossis nqa los ntawm lub rooj zaum ntau dua 2 zaug saib seb puas muaj ntau thiab nyuaj rau ua cov haujlwm no.

Yog tias pom tias cov leeg tsis muaj zog thiab nws yog qhov nyuaj rau kev sim no, nws yog qhov muaj feem ntau muaj qhov ua rau cov leeg tsis muaj zog, qhia tias muaj kab mob xws li myasthenia.

Txheeb xyuas seb puas muaj kev cuam tshuam nrog kev hais lus, koj tuaj yeem nug tus neeg kom hais tus lej ntawm 1 txog 100 thiab saib yog muaj kev hloov ntawm lub suab ntawm lub suab, lub suab tsis ua haujlwm lossis nce sijhawm hauv kev suav ntawm txhua tus lej.

Kev Kho Mob Rau Congenital Myasthenia

Cov kev kho mob sib txawv raws li cov kab mob qog nqaij hlav myasthenia uas tus neeg muaj tab sis qee qhov kev kho mob xws li acetylcholinesterase inhibitors, Quinidine, Fluoxetine, Ephedrine thiab Salbutamol tuaj yeem qhia nyob rau hauv kev pom zoo ntawm neuropediatrician lossis neurologist. Kev qhia tawm dag zog lub cev tau qhia thiab tuaj yeem pab tus neeg kom zoo siab dua, ntaus cov leeg tsis muaj zog thiab txhim kho kev ua pa, tab sis nws yuav tsis muaj txiaj ntsig yam tsis muaj tshuaj.

Cov menyuam yaus tuaj yeem pw nrog lub npog ntsej muag oxygen hu ua CPAP thiab niam txiv yuav tsum kawm paub muab kev pabcuam ua ntej thaum raug ntes ua pa.

Hauv kev tawm dag zog rau kev tawm dag zog yuav tsum yog isometric thiab muaj ob peb qhov rov ua dua tab sis lawv yuav tsum them rau ntau pawg leeg, nrog rau txoj kev ua pa thiab muaj txiaj ntsig zoo los txhawm rau nce mitochondria, cov leeg, capillaries thiab txo qis lactate concentration, nrog cramps tsawg dua.

Kho puas tau myasthenia los kho?

Feem ntau, mob qog nqaij hlav ntshav qab zib tsis tuaj yeem kho zoo, uas yuav tsum tau kho mus tas sim neej. Txawm li cas los xij, cov tshuaj kho mob thiab qoj ib ce pab txhawm rau txhim kho tus neeg lub neej zoo, tiv thaiv kev qaug zog thiab cov leeg lub cev tsis muaj zog thiab zam kev mob uas yog mob caj dab thiab caj npab thiab txhawm chim uas tuaj yeem tshwm sim thaum ua pa tsis txaus, uas yog vim li cas, lub neej yog qhov tseem ceeb.

Cov neeg uas muaj Congenital Myasthenia tshwm sim los ntawm qhov tsis zoo hauv lub noob DOK7 tuaj yeem muaj kev txhim kho zoo rau lawv tus mob, thiab tuaj yeem pom tias 'kho tau' nrog kev siv tshuaj uas feem ntau siv los tiv thaiv mob hawb pob, salbutamol, tab sis nyob rau hauv daim ntawv ntawm cov ntsiav tshuaj lossis lozenges. Txawm li cas los xij, koj kuj tseem yuav tsum ua kev kho mob lub cev ib puag ncig.

Thaum tus neeg muaj Congenital Myasthenia thiab tsis tau txais kev kho mob, lawv yuav maj mam tawm dag zog hauv cov leeg, ua kom mob plab, yuav tsum tau mus pw thiab yuav tuag los ntawm kev ua pa tsis ua haujlwm thiab vim li ntawd kev kho mob thiab kho lub cev yog ib qho tseem ceeb vim tias ob leeg tuaj yeem txhim kho tus neeg lub neej zoo thiab lub neej ntev.

Qee cov kev kho mob uas ua rau cov mob ntawm Congenital Myasthenia yog Ciprofloxacin, Chloroquine, Procaine, Lithium, Phenytoin, Beta-blockers, Procainamide thiab Quinidine thiab yog li ntawd txhua yam tshuaj yuav tsum tsuas yog siv cov lus qhia kho mob tom qab kev txheeb xyuas ntawm hom uas tus neeg muaj.