Gcwalisa Umhlahlandlela Wezemfundo

Ukuqonda I-Mast Cell
I-Activation Syndrome
(MCAS)

Funda isayensi ngemuva kwama-mast cells, kungani esebenza ngokweqile, ukuthi i-MCAS iwuthinta kanjani umzimba, kanye nolwazi oluyisisekelo sonke isiguli, umnakekeli, umfundi, kanye nochwepheshe bezokunakekelwa kwezempilo okufanele baluqonde.

Isigaba 01

Yini I-MCAS?

I-Mast Cell Activation Syndrome isifo sokuzivikela komzimba ukuziphatha esikhundleni sokuzivikela ubuningi. Amaseli akhona ngezinombolo ezijwayelekile futhi abukeka ejwayelekile ngokwesakhiwo - okushintshile ukuthi adubula kalula kanjani.

I-Mast Cell Activation Syndrome (MCAS) yisimo lapho ama-mast cells - amaseli omzimba ahlala cishe kuzo zonke izicubu zomzimba - esebenza ngokungafanele nangokuphindaphindiwe, ekhulula abaxhumanisi bamakhemikhali ngobuningi futhi ngezinye izikhathi abangasebenzisi injongo yokuzivikela ewusizo. Umphumela uba iphethini yezimpawu eziphindaphindayo, zesiqephu ezithinta amasistimu wezitho ezimbili noma ngaphezulu ngesikhathi esisodwa.

Incazelo ibalulekile ngoba ihlukanisa i-MCAS nezimo ezifuze ngayo kancane. Akukho ukunqwabelana okungavamile kwamangqamuzana okutholakala ku-biopsy, akukho ukungezwani komzimba okukodwa ongakubona lapho kuhlolwa ukuhlaba esikhunjeni, futhi ngokuvamile akukho okungavamile nhlobo emsebenzini waselabhorethri ovamile. Lesi sifo sihlala embundwini lapho amangqamuzana abukeka evamile anquma ukuphendula.

Umqondo Oyinhloko

Ku-MCAS inkinga ayikho bangaki mast cells onawo. Kunjalo badubula kalula kanjani. Zonke ezinye izici zesimo - ukungaqiniseki, ukusabalala kwezinhlelo eziningi, ukuhlolwa okunzima - kulandela kulelo qiniso elilodwa.

Kungani ama-mast cells ekhona nhlobo

Umqondo Oyinhloko

Zibekwe ngendlela efanele - zigxile emingceleni, esikhunjeni, emzileni womoya, odongeni lwamathumbu, kanye nezicubu ezizungezile imithambo yegazi nezinzwa. Futhi zakhelwe isivinini: kunokuba zikhiqize impendulo yazo ngokufunwa, ziyibamba zenziwe ngaphambili kumagranule esitoreji, zilungele ukukhululwa phakathi nemizuzwana yesiginali. Lezo zakhiwo yikho okwenza ukungezwani komzimba kusheshe, futhi yikho futhi okwenza kusebenze okungalungile kube nzima ukuphila nakho.

Kungani ama-mast cells ekhona nhlobo

Ama-mast cell awawona iphutha lokuklama. Ziphakathi kwezingxenye ezindala kakhulu zesimiso somzimba sokuzivikela ezifweni, ezikhona ngandlela thize kuzo zonke izilwane ezinomgogodla, futhi zenza umsebenzi umzimba owudinga ngempela. Bafaka impendulo yokuqala kuma-parasites, basize ukuqeda ubuthi ngemva kokulunywa nokulunywa, babambe iqhaza ekwelapheni amanxeba nasekulungiseni kabusha izicubu, babambe iqhaza ekulawulweni kokungena kwemithambo yegazi, futhi basize ekugcineni izicubu ezivimbelayo ezihlukanisa umzimba nomhlaba wangaphandle.

Zibekwe ngendlela efanele - zigxile emingceleni, esikhunjeni, emzileni womoya, odongeni lwamathumbu, kanye nezicubu ezizungezile imithambo yegazi nezinzwa. Futhi zakhelwe isivinini: kunokuba zikhiqize impendulo yazo ngokufunwa, ziyibamba zenziwe ngaphambili kumagranule esitoreji, zilungele ukukhululwa phakathi nemizuzwana yesiginali. Lezo zakhiwo yikho okwenza ukungezwani komzimba kusheshe, futhi yikho futhi okwenza kusebenze okungalungile kube nzima ukuphila nakho.

Ibukeka kanjani imisebenzi ye-mast cell evamile

Ekuphenduleni okunempilo kokuzivikela komzimba, ingqamuzana le-mast lihlangabezana nosongo lwangempela - i-antigen ye-parasite, ubuthi, noma ukulimala kwezicubu - futhi liwohloke ngokulingana nalo. Imithambo yegazi iyanwebeka futhi ikwazi ukungena kalula, okwenza i-plasma namangqamuzana omzimba omzimba angene esicutshini. Iziphetho zezinzwa ziyashukunyiswa, zikhiqize ukulunywa noma ubuhlungu obudonsela ukunaka kusayithi. Amanye amangqamuzana omzimba omzimba ayabuthwa. Lapho usongo selusuliwe, impendulo iyaxazululeka.

Konke okungajabulisi ngaleyo nqubo kuyasebenza. Ukuvuvukala kuletha amasosha omzimba endaweni. Ukulunywa kukwenza ukuthi ususe noma yini edala umonakalo. Amafinyila abamba futhi axoshe izinto ezicasulayo. Ngempendulo elinganayo, ukungakhululeki kungokwesikhashana futhi inzuzo ingokoqobo.

Yini engahambi kahle ku-MCAS

Ku-MCAS, lowo mshini ofanayo usebenza ngaphandle kwesizathu esilinganayo. Amangqamuzana e-Mast avutha ngokuzenzekelayo, noma ephendula izisusa okufanele zingabonakali - igumbi elifudumele, iphunga emhubheni, ukudla, ukuma, ukuvivinya umzimba okuncane, usuku oluvamile olucindezelayo. Abalamuli abakhululiwe yibo abafanayo abasetshenziswa ekuzivikeleni okusemthethweni, kodwa bakhululwa esimweni esingalungile futhi ngokuvamile ngokuphindaphindiwe.

Ngenxa yokuthi ama-mast cell ahlala ezicutshini kuwo wonke umzimba, futhi ngenxa yokuthi abalamuli bawo basebenza emithanjeni yegazi, imisipha ebushelelezi, imizwa, namanye amangqamuzana omzimba omzimba, isiqephu esisodwa singaveza ukuguquguquka, inhliziyo egijimayo, ukuhlabeka kwesisu, ukuphelelwa umoya, kanye nenkungu yengqondo kanyekanye. Kudokotela ongacabangi ngama-mast cells, leyo nhlanganisela ingase ibukeke njengezinkinga ezimbalwa ezingahlobene ezifika ngesikhathi esisodwa - okuyingxenye enkulu yokuthi kungani isimo sigejwa.

Isifaniso esilula

I-Mastocytosis iqhathaniswa ne-MCAS
I-MCAS ayiyona i-mastocytosisLona umehluko owodwa obaluleke kakhulu ensimini, futhi ukudida kokubili kuholela ekudidekeni ngayo yonke into ezansi nomfula.
I-Mastocytosis iqhathaniswa ne-MCASI-MastocytosisI-MCAS
Ukungajwayelekile okuyinhlokoAma-mast cells amaningi kakhulu anqwabelana esicutshiniInombolo evamile yamaseli e-mast, asebenza ngokungafanele
Isibalo samaseliEphakeme - ukwanda clonalOkuvamile
I-tryptase eyisisekeloKIT Ukuguqulwa kwe-D816V ezimweni eziningi zesistimu yabantu abadalaNgokuvamile kujwayelekile ekuqaleni
Ukutholwa kofuzo okujwayelekileIzibalo ze-mast cell ezingavamile ziyaboniswaAkukho ukuguqulwa kwe-causative okukodwa okusunguliwe
Imiphumela ye-BiopsyIzibalo ze-mast cell ezingavamile ziyaboniswaAkukho ukuqoqwa kwesici
Uphawu lwesikhumbaIzilonda ze-maculopapular (urticaria pigmentosa) ezivamile kwezinye izinhloboAsikho isilonda esithile sesikhumba sokuxilonga

Kuqinisekiswa kanjani

Ubufakazi obuyinhloso bokweqisa kwamaseli

I-classical allergy yindlela yokusabela e-IgE entweni ethile, ekhonjwayo. Iyakwazi ukukhiqizwa kabusha - ukuchayeka okufanayo kukhiqiza ukusabela okufanayo - futhi ngokuvamile kungaboniswa ngokuhlolwa kwesikhumba noma ukuhlolwa kwegazi okukhethekile kwe-IgE. Ukungezwani namantongomane kuziphatha ngale ndlela; kanjalo ne-cat dander allergies.

Zombili lezi zimo zingaveza izimpawu ezigqagqene, ngoba kuzo zombili izimo abalamuli be-mast cell bafinyelela izicubu ngokweqile. Kodwa i-mastocytosis ingaboniswa ngokuthola amaseli; I-MCAS ayikwazi, okuyisizathu sokuthi kungani ukuhlonzwa kwayo kuncike kwinhlanganisela yemibandela esikhundleni sokuhlola okukodwa kokuqinisekisa.

Iziguli eziningi zifika ku-MCAS ngokuqondile ngoba ukuhlolwa kwe-allergies kwabuya kuhlanzekile kuyilapho izimpawu zabo zingabonakali. Iphaneli ye-negative yokungezwani komzimba akubona ubufakazi obumelene nokubandakanyeka kwe-mast cell; kulesi simo kuseduze nokulindelwe.

I-classical allergy yindlela yokusabela e-IgE entweni ethile, ekhonjwayo. Iyakwazi ukukhiqizwa kabusha - ukuchayeka okufanayo kukhiqiza ukusabela okufanayo - futhi ngokuvamile kungaboniswa ngokuhlolwa kwesikhumba noma ukuhlolwa kwegazi okuqondile kwe-IgE. Ukungezwani namantongomane kuziphatha ngale ndlela; kanjalo ne-cat dander allergies.

Ukusabela kwe-MCAS akuvamile. Eziningi aziyona i-IgE-mediated nakancane, kunalokho zivela ngamanye ama-receptors endaweni ye-mast cell noma ngokukhuthazwa okuqondile ngokomzimba. Izibangeli zivame ukuba ngokomzimba - ukushisa, amakhaza, ingcindezi, ukuzikhandla - kunokuba amaprotheni, futhi alikho iphaneli yokungezwani komzimba eklanyelwe ukuthola lokho. Ukusabela kungase kubambezeleke ngamahora, kunqamule ukuxhumana okunembile phakathi kokuchayeka kanye nomthelela, futhi umkhawulo ungashintsha ngokulala, ingcindezi, isimo sehomoni, noma ukugula kwakamuva, ngakho ukuchayeka okubekezelelwe isonto elilodwa kuvusa ukusabela ngokulandelayo.

Okubonakalayo okuphakanyisiwe

Umtholampilo wePearl

Isiguli esinokusabela okuphindaphindekayo, ukuhlolwa kwe-IgE engemihle, nezibangeli zomzimba ezifana nokushisa noma ukucindezela kuyisethulo esivamile se-MCAS kunesiguli esinokuhlolwa kokungezwani komzimba okuhle. Iphaneli enegethivu ivamise ukuba yingxenye yesithombe kunengxabano ephikisana nayo.
  • I-MCAS wukuphazamiseka kwe-mast cell engafanele ukwenza kusebenze, hhayi izinombolo ze-mast cell.
  • Amaseli e-Mast angamaseli omzimba awusizo ngokusemthethweni abekwe emigoqweni yomzimba futhi akhelwe ukuphendula ngemizuzwana.
  • Isifinyezo sesigaba
  • I-mastocytosis ihilela ukweqisa kwamaseli futhi ingaboniswa ngokuqondile; I-MCAS ayikwazi, yingakho ukuxilongwa kuyinhlanganisela.
  • Amaseli e-Mast angamaseli omzimba awusizo ngokusemthethweni abekwe emigoqweni yomzimba futhi akhelwe ukuphendula ngemizuzwana.
Isigaba 02

Ukuqonda Amaseli Ensika

Ukuhlolwa kokungezwani kahle komzimba okungalungile kuvamile ku-MCAS futhi akukhiphi.

Umsuka kanye nentuthuko

Ama-mast cell aqala emnkantsheni, avela kumaseli e-CD34-positive haematopoietic progenitor cell - inani elifanayo labantu elidala amanye amaseli egazi. Nokho, ngokungafani namangqamuzana amaningi amhlophe egazi, awaqedi ukukhula kwawo lapho. Okhokho abangavuthiwe bangena emgudwini wegazi, bajikeleze isikhashana, bese bethuthela esicubuni, lapho ukuvuthwa kokugcina kwenzeka khona.

Lokho kukhula kuncike kakhulu ekusayineni kwe-stem cell factor (SCF) ngesamukeli se-KIT endaweni ye-mast cell. Le ndlela imaphakathi ne-mast cell biology, futhi iyindlela efanayo ephazanyiswe yi- KIT Ukuguqulwa kwe-D816V okutholakala ezimweni eziningi zabantu abadala ze-systemic mastocytosis. Indawo yethishu yendawo ibumba i-phenotype yokugcina, yingakho ama-mast cells emathunjini ehluke ngokulinganiswa kulawo asesikhunjeni.

Umsuka kanye nentuthuko

Ukusabalalisa kuwo wonke umzimba

Amaseli e-Mast asakazwa ngokwecebo kunokuba afane, agxile lapho umzimba uhlangana khona nomhlaba wangaphandle futhi uzungeze izakhiwo ezithwala amasignali negazi. Lokhu kusatshalaliswa kuchaza uhlamvu lwesistimu eningi ye-MCAS ngokuqondile kunanoma yiliphi elinye iqiniso elilodwa.

Lapho ama-mast cell ahlala khona, nokuthi kukhiqizani lapho eqhuma kabi
Ukusabalalisa kuwo wonke umzimbaAmaseli e-Mast asatshalaliswa ngendlela ehlelekile kunokuba afane, agxile lapho umzimba uhlangana khona nomhlaba wangaphandle futhi uzungeze izakhiwo ezithwala amasignali negazi. Lokhu kusatshalaliswa kuchaza uhlamvu lwesistimu eningi ye-MCAS ngokuqondile kunanoma yiliphi elinye iqiniso elilodwa.Lapho ama-mast cell ahlala khona, nokuthi kukhiqizani lapho eqhuma kabi
IndawoIqhaza empilweniIzimpawu lapho ukwenza kusebenze kungafanele
Isikhumba kanye nesikhumbaUkuvikela isithiyo; ukuphendula ekulunyweni, ekulunyweni, nasekulimaleniUkuqunjelwa, ukulunywa, isifuba, idermographism, ukuvuvukala
Umgudu wamathumbuUkugcinwa kwezithiyo, ukulawulwa kwe-motility, ukuphendula ezinsongweni ezingenisiweUkuqunjelwa, isicanucanu, i-reflux, isifo sohudo, ukuqunjelwa, ukuqunjelwa
Airways kanye sinusesUkuzivikela kolwelwesi lokuphefumulaUkuqunjelwa, i-rhinitis, ukuqina komphimbo, ukukhwehlela, ukukhwehlela
Emithanjeni yegaziUkulawulwa kwethoni yomkhumbi kanye nokungenekaUmfutho wegazi uyashintsha, tachycardia, flushing, presyncope
Ubuchopho kanye ne-meningesKwethulwa nge-perivascularly naku-meninges; ukubonakaliswa kwe-neuroimmuneInkungu yengqondo, ikhanda elibuhlungu, isiyezi (izindlela zisacutshungulwa)
Izicubu ezixhumeneyoUkulungiswa kabusha nokulungiswa kwezicubuUbuhlungu obuhlangene nemisipha; ukuhlobana okunokwenzeka kokudlulana kwezicubu ezixhumeneyo

Umnkantsha

Indawo yomsuka wokhokho

Ama-mast cell aphila isikhathi eside ngokungavamile. Lapho i-neutrophil ingase iphile amahora ambalwa kuya ezinsukwini, amangqamuzana e-tissue mast angaqhubeka amasonto kuya ezinyangeni, futhi ubufakazi bubonisa ukuthi amanye aphila isikhathi eside kakhulu. Bayakwazi futhi ukwenza kabusha i-regranulation - ngemva kokuwohloka, i-mast cell ingakha kabusha izitolo zayo futhi iphendule futhi, kunokuba ife kunqubo.

Qaphela ukungena kobuchopho ngokucophelela. Ama-mast cell akhona ngempela kuma-meninges nasezimeni ze-perivascular ngaphakathi kwesistimu yezinzwa emaphakathi, futhi ukusebenzisana kwe-mast cell-nerve kuyindawo yocwaningo esebenzayo. Kodwa-ke, izindlela ezixhumanisa ukusebenza kwe-mast cell nezimpawu ezithile zemizwa nezingqondo zisalungiswa, futhi izimangalo zemishini eziqinisekayo kule ndawo zidlula ubufakazi bamanje.

Umjikelezo wokuphila

Ama-mast cell aphila isikhathi eside ngokungavamile. Lapho i-neutrophil ingase iphile amahora ambalwa kuya ezinsukwini, amangqamuzana e-tissue mast angaqhubeka amasonto kuya ezinyangeni, futhi ubufakazi bubonisa ukuthi amanye aphila isikhathi eside kakhulu. Bayakwazi futhi ukwenza kabusha i-regranulation - ngemva kokuwohloka, i-mast cell ingakha kabusha izitolo zayo futhi iphendule futhi, kunokuba ife kunqubo.

  • I-FceRI - i-high-affinity IgE receptor. Lena indlela ye-allergies yakudala: Amasosha omzimba e-IgE abopha isamukeli, futhi axhumaniswe ngokuphambana ngokwenza kusebenze ama-allergenic triggers.
  • I-MRGPRX2 - i-receptor mediating ivula i-IgE-independent ngezidakamizwa ezithile nama-peptide. Ukuhlukaniswa kwezinhlamvu zayo kusize ukuchaza ukusabela okwenzeka ngaphandle kokubandakanyeka kwe-IgE okubonakalayo.
  • KIT - i-receptor ye-stem cell factor; okuphakathi kokusinda kwe-mast cell, ukuvuthwa, kanye nokuhlala kwezicubu.
  • Ama-receptors ahambisanayo (C3a, C5a) - vumela ukuthi kusebenze ngengalo ehambisanayo yokugonywa okungokwemvelo.
  • Ama-receptors afana ne-toll - bona amaphethini ahlobene ne-pathogen, axhumanisa ama-mast cells nezimpendulo zokutheleleka.
  • KIT - i-receptor ye-stem cell factor; okuphakathi kokusinda kwe-mast cell, ukuvuthwa, kanye nokuhlala kwezicubu.
  • I-Adenosine, i-opioid, i-estrogen namanye ama-receptors - ukufaka isandla kumaphrofayili we-trigger ahlukahlukene kanye nawodwa abonwa ekusebenzeni.
Ingabe Bewazi?

Ukuba khona kwemizila eminingi engeyona ye-IgE yingakho ukuhlolwa okujwayelekile kokungezwani komzimba kuvame ukubuya ngokwejwayelekile ku-MCAS. Lezo zivivinyo zakhelwe ukuthola ukuzwela kwe-IgE-mediated — indlela eyodwa phakathi kwezimbalwa ezingamisa i-mast cell.

Okubonakalayo okuphakanyisiwe

Umfanekiso weseli ye-mast enelebula: ulwelwesi lweseli olugcwele ama-receptors aqanjwe igama (i-FcεRI, MRGPRX2, KIT, C3a/C5a, TLRs, CRH-R), ingaphakathi ligcwele ama-granules, elinesikwe elibonisa okuqukethwe yizinhlamvana. Isethi ehambisanayo ebonisa iseli ngaphambi nangemuva kokuwohloka.

Isiguli esinokusabela okuphindaphindekayo, ukuhlolwa kwe-IgE engemihle, nezibangeli zomzimba ezifana nokushisa noma ukucindezela kuyisethulo esivamile se-MCAS kunesiguli esinokuhlolwa kokungezwani komzimba okuhle. Iphaneli enegethivu ivamise ukuba yingxenye yesithombe kunengxabano ephikisana nayo.
  • Ama-mast cells asuka ku-CD34+ progenitors emnkantsheni kodwa avuthiwe esicutshini, ngakho izibalo zegazi aziwabonisi.
  • Ukuba khona kwemizila eminingi engeyona ye-IgE yingakho ukuhlolwa okujwayelekile kokungezwani komzimba kuvame ukubuya ngokwejwayelekile ku-MCAS. Lezo zivivinyo zakhelwe ukuthola ukuzwela kwe-IgE-mediated — indlela eyodwa phakathi kwezimbalwa ezingamisa i-mast cell.
  • Bagxila ezindaweni eziyisithiyo naseduze kwemithambo yegazi nezinzwa, yingakho i-MCAS inesistimu eminingi.
  • Ziphila isikhathi eside futhi zingakwazi ukuphindaphinda, ngakho-ke ukuphatha kuyaqina esikhundleni sesifundo esifushane.
  • Ukusayinda kwe-KIT/SCF kuyinhloko ekuthuthukisweni kwabo - indlela ephazamisekile kumastocytosis amaningi esistimu yabantu abadala.
Isahluko 03

Kwenzekani Ngesikhathi
Ukuvuselela Iseli Yezinsimbi

Izinhlobo eziningi zama-receptor ngale kwe-IgE zingazivula, yingakho amaphaneli okungezwani komzimba evame ukugeja izibangeli ze-MCAS.

1
Qalisa

Ukwenza kusebenze ukulandelana, futhi isinyathelo ngasinye sinegama. Ukukulandela kwenza ukuhlukahluka okudidayo kwezimpawu ze-MCAS kube lula kakhulu ukuzihumusha.

2
Qalisa

Isikhuthazo sifinyelela kungqamuzana - i-allergen, isidakamizwa, ukushisa, ingcindezi, ukuzikhandla, i-hormone yokucindezeleka, ukutheleleka, noma akukho nhlobo isisusa esibonakalayo.

3
Ukwenza kusebenze

Ama-Surface receptors abamba iqhaza futhi ama-Cascades wokusayina we-intracellular aqala. Ukungena kwe-calcium kuyisinyathelo sokuqala esibalulekile.

4
Ukuwohloka

Ama-granules esitoreji ahlangana nolwelwesi lweseli futhi akhiphe okuqukethwe kwawo okwenziwe ngaphambilini kuzicubu ezizungezile - ngemizuzwana.

5
Ukukhululwa komlamuli

Abalamuli abakhelwe ngaphambilini bakhululwa ngokushesha; abalamuli abasanda kuhlanganiswa njenge-prostaglandin kanye ne-leukotrienes balandela phakathi nemizuzu, nama-cytokines emahoreni amaningi.

Izici ezimbili zalokhu kulandelana zichaza okuningi kwalokho okutholwa yiziguli. Okokuqala, isigaba esisheshayo sidonsa ezitolo ezenziwe ngaphambilini, yingakho ukusabela kungaqala phakathi nemizuzwana. Okwesibili, abalamuli besigaba sakamuva bahlanganiswa ngemva kokuthi kuqale ukusebenza, yingakho ezinye izimpawu zifika kahle ngemva kwe-trigger nokuthi kungani ukusabela kungase kube nokubambezeleka kwamahora wesibili wamagagasi kamuva.

Umqondo Oyinhloko

Amaseli amakhulu akhulula abalamuli phakathi amagagasi, hhayi kanye kanye. Abalamuli abakhelwe ngaphambilini bayaphuma ngemizuzwana; abaxhumanisi be-lipid bakhiwa ngamaminithi; ama-cytokines aqoqana phakathi namahora. Ngakho-ke umcimbi owodwa wokwenza kusebenze ungaveza izimpawu usuku lonke.

Abalamuli basebenza emithanjeni yegazi, imisipha ebushelelezi, imizwa, namanye amangqamuzana omzimba omzimba, akhiqiza imiphumela kuwo wonke amasistimu wezitho eziningi.

Abalamuli abakhulu nokuthi ngamunye wenzani

Ama-mast cell ayakwazi ukukhulula inani elikhulu kakhulu lezinto. Okulandelayo yizona okuxoxwe ngazo kakhulu ngokomtholampilo, futhi ndawonye kubala iningi lesithombe esibonwayo sezimpawu.

Kuyafaneleka futhi ukuqaphela ukuthi ukwenza kusebenze akukhona konke-noma-lutho. Amaseli e-Mast angakhulula abalamuli abakhethiwe ngaphandle kokuwohloka okugcwele - ngezinye izikhathi okubizwa ngokuthi i-piecemeal noma ukukhululwa okuhlukile. Lokhu kusiza ukuchaza ukuthi kungani iziguli ziba nenhlanganisela enjalo yezimpawu, nokuthi kungani ukusabela kungesona isenzakalo esimangalisayo igama elithi 'ukwenza kusebenze' elisiphakamisayo.
Abalamuli abakhulu nokuthi ngamunye wenzaniAma-mast cell ayakwazi ukukhulula inani elikhulu kakhulu lezinto. Okulandelayo yizona okuxoxwe ngazo kakhulu ngokomtholampilo, futhi ndawonye kubala iningi lesithombe esibonwayo sezimpawu.Abalamuli be-mast cell abakhulu
I-HistamineUhloboUmlamuli owaziwa kakhulu. Ivula imithambo yegazi futhi ikhulise ukungena kwayo, ivuse iziphetho zezinzwa, futhi ikhulise ukukhishwa kwe-asidi yesisu. Ikhiqiza ukuguquguquka, ukulunywa, ukuqubuka, umfutho wegazi ophansi, i-tachycardia, ikhanda elibuhlungu, kanye ne-GI cramping. Isebenza ngama-subtypes ama-receptor amaningi - i-H1 esikhumbeni nasendleleni yomoya, i-H2 emathunjini nasenhliziyweni - yingakho ukuvimba womabili amakilasi kuvame ukusebenza kangcono kunokuvimba noma yikuphi.
I-HistamineUhloboKwakhiwe ngaphambilini
I-Prostaglandin D2Okusanda kuhlanganiswaIphrotheni eningi kakhulu kuma-mast cell granules kanye nomaka waselabhorethri osungulwe kakhulu wokwenza kusebenze. Inomthelela ekwakhiweni kabusha kwezicubu kanye nokuvuvukala, futhi ingakhulisa ukusabela kwe-mast cell. Inani layo lokuxilonga livela ekubeni licaciswe ngokuqhathaniswa namaseli amakhulu.
I-Leukotrienes (LTC4, LTD4, LTE4)Okusanda kuhlanganiswaAma-bronchoconstrictors anamandla, kakhulu kakhulu kune-histamine ngesisindo. Khulisa ukuvuthwa kwemithambo kanye nokukhiqizwa kwamafinyila, futhi kube nomthelela kukho kokubili izimpawu zendlela yokuphefumula neyomathumbu. I-LTE4 iyalinganiswa emchamweni.
Ama-Cytokines (IL-6, TNF-α, IL-1, IL-4 nabanye)Okusanda kuhlanganiswaAmaprotheni amasignali ashayela ukuvuvukala okubanzi. Iqala kancane futhi isebenza isikhathi eside kunabalamuli abangenhla, futhi kucatshangwa ukuthi ingaba nomthelela ekukhathaleni, ekuguleni komzimba, kanye nekhwalithi yesistimu 'efana nomkhuhlane' ichaza ngesikhathi nangemuva kokuqubuka.
AmakhemikhaliOkusanda kuhlanganiswaThola amaseli omzimba engeziwe esizeni, akhulise futhi andise impendulo yokuvuvukala ngale kokusabela kokuqala kwe-mast cell.
I-Platelet activating factor (PAF)Okusanda kuhlanganiswaUmxhumanisi we-lipid onamandla kakhulu okhuphula ukungeneka kwemithambo futhi ungabangela i-hypotension enzima. Ifakwe ebukhulwini bokusabela kwe-anaphylactic futhi ingavinjelwa ama-antihistamine.
I-HeparinUhloboI-anticoagulant eyenzeka ngokwemvelo. Ingase ibe nomthelela ekujuleni okungajwayelekile noma ekuthambekeni ukopha okubikwe ezinye iziguli, futhi ibambe iqhaza ekulawuleni ukuvuvukala kwendawo.
I-Chymase ne-carboxypeptidase A3UhloboAma-Granule protease abandakanyeka ekwenziweni kabusha kwezicubu, izindlela zokulawula umfutho wegazi, kanye nokuwohloka kobuthi obuthile nama-peptide.
I-classical allergy yindlela yokusabela e-IgE entweni ethile, ekhonjwayo. Iyakwazi ukukhiqizwa kabusha - ukuchayeka okufanayo kukhiqiza ukusabela okufanayo - futhi ngokuvamile kungaboniswa ngokuhlolwa kwesikhumba noma ukuhlolwa kwegazi okuqondile kwe-IgE. Ukungezwani namantongomane kuziphatha ngale ndlela; kanjalo ne-cat dander allergies.

I-anticoagulant eyenzeka ngokwemvelo. Ingase ibe nomthelela ekujuleni okungajwayelekile noma ekuthambekeni ukopha okubikwe ezinye iziguli, futhi ibambe iqhaza ekulawuleni ukuvuvukala kwendawo.

Okubonakalayo okuphakanyisiwe

Inqubo ye-flowchart egijima kwesokunxele iye kwesokudla: Qalisa → Ukwenza kusebenze → Ukwehliswa kwegranulation → Ukukhishwa komlamuli → Izimpawu, ezinomugqa wesikhathi ohlanganisa igatsha ngaphansi obonisa abalamuli abakheke ngaphambilini ngemizuzwana, abaxhumanisi be-lipid ngamaminithi, nama-cytokines ngamahora, igatsha ngalinye lichazwe ngezimpawu eliziqhubayo.

Isiguli esinokusabela okuphindaphindekayo, ukuhlolwa kwe-IgE engemihle, nezibangeli zomzimba ezifana nokushisa noma ukucindezela kuyisethulo esivamile se-MCAS kunesiguli esinokuhlolwa kokungezwani komzimba okuhle. Iphaneli enegethivu ivamise ukuba yingxenye yesithombe kunengxabano ephikisana nayo.
  • Ama-Granule protease abandakanyeka ekwenziweni kabusha kwezicubu, izindlela zokulawula umfutho wegazi, kanye nokuwohloka kobuthi obuthile nama-peptide.
  • Abalamuli abakhelwe ngaphambilini bakhululwa ngemizuzwana; abaxhumanisi be-lipid ngamaminithi; ama-cytokines amahora amaningi.
  • Amaseli e-Mast angakhulula abalamuli ngokukhetha ngaphandle kokuncipha okugcwele.
  • Ukwenza kusebenze kulandela ukulandelana: i-trigger, activation, degranulation, ukukhululwa komlamuli, izimpawu.
  • Abalamuli abakhelwe ngaphambilini bakhululwa ngemizuzwana; abaxhumanisi be-lipid ngamaminithi; ama-cytokines amahora amaningi.
Isigaba 04

Izimpawu ze I-MCAS

I-PAF kanye ne-prostaglandin ingashayela imiphumela enzima yenhliziyo nemithambo yegazi i-antihistamines engakhulumi ngayo.

Amathebula angezansi ahlela izimpawu ezivame ukubikwa ngohlelo lomzimba. Izixwayiso ezimbili ngaphambi kokuzifunda. Okokuqala, asikho isiguli esinazo zonke lezi, futhi uhlu olude akufanele lufundwe njengohlu lokuhlola oluzohambisana nalo. Okwesibili, zonke izimpawu lapha zenzeka kwezinye izimo eziningi; yini ephakamisa ukuthi i-MCAS iyona iphethini - i-episodic, i-recurrent, multisystem, ngokuvamile enezibangeli ezibonakalayo - kunanoma yikuphi okufakiwe ngakunye.

Isikhumba kanye ne-mucosal
I-MCAS ichazwa ngokwengxenye ngobubanzi bayo. Ngenxa yokuthi ama-mast cell ahlala cishe kuzo zonke izicubu, izimpawu zingavela cishe noma kuphi - futhi imfuneko yezinhlelo zezitho ezimbili noma ngaphezulu ibalulekile ekuxilongweni.Amanothi
Isikhumba kanye ne-mucosalUphawu
AmanothiUkushaywa umoya
Ngokuvamile kungazelelwe, kuthinta ubuso, intamo, nesifuba; isici esibikwa njaloUkuluma (pruritus)
Kungenzeka noma ngaphandle kokuqhuma okubonakalayoI-Hives (i-urticaria)
Ama-welts aphakanyisiwe, alumayo; ingase ibe yesikhashana futhi ifudukeI-Dermographism
Ama-welts aphakanyiswe ukuphulula okuqinile kwesikhumba - uphawu olubonakalayo olubonakalayoI-Angioedema
Ukuvuvukala okujulile, ngokuvamile kwezindebe, amajwabu amehlo, izandla, noma umphimbo
I-MCAS ichazwa ngokwengxenye ngobubanzi bayo. Ngenxa yokuthi ama-mast cell ahlala cishe kuzo zonke izicubu, izimpawu zingavela cishe noma kuphi - futhi imfuneko yezinhlelo zezitho ezimbili noma ngaphezulu ibalulekile ekuxilongweni.Amanothi
Ukulibaziseka ukuphola kwesilondaKubikwe ezinye iziguli; izindlela ezingakasungulwa ngokugcwele
OkokuphefumulaUkucinana kwamakhala kanye ne-rhinitis
Ngokuvamile okungapheli futhi okungeyona isiziniUkuqina komphimbo
Idinga ukuhlolwa okuphuthumayo uma kuqhubeka - kungase kubonise i-anaphylaxisUkuhefuzela kanye nokuphelelwa umoya
I-bronchoconstriction eqhutshwa yi-leukotriene iyindlela eyaziwayoUkukhwehlela okungapheli
Ukungaphenduli ekwelashweni okujwayelekile kokukhwehlela
I-MCAS ichazwa ngokwengxenye ngobubanzi bayo. Ngenxa yokuthi ama-mast cell ahlala cishe kuzo zonke izicubu, izimpawu zingavela cishe noma kuphi - futhi imfuneko yezinhlelo zezitho ezimbili noma ngaphezulu ibalulekile ekuxilongweni.Amanothi
Ukuzwela amakha namakhemikhali asemoyeniUkumataniswa kwezimpawu zokuqalisa okubikwe kakhulu
Inhliziyo nemithambo yegaziI-Tachycardia
Inhliziyo egijimayo, ngokuvamile ngaphandle kokuzikhandlaUkungaqini komfutho wegazi
Ingashwibeka kunoma iyiphi indlela phakathi neziqephuI-presyncope ne-syncope
Ikhanda elikhanyayo noma ukuquleka, ngokuvamile lapho umileUkungakhululeki kwesifuba
Idinga ukuhlolwa ukuze ikhiphe izimbangela zenhliziyo
I-MCAS ichazwa ngokwengxenye ngobubanzi bayo. Ngenxa yokuthi ama-mast cell ahlala cishe kuzo zonke izicubu, izimpawu zingavela cishe noma kuphi - futhi imfuneko yezinhlelo zezitho ezimbili noma ngaphezulu ibalulekile ekuxilongweni.Amanothi
I-AnaphylaxisKunzima, kuyashesha, okungaba yingozi empilweni - bona isigaba sezimo eziphuthumayo
AmathumbuUkuqina kwesisu nobuhlungu
Phakathi kwezimpawu ezivame ukubikwa sezizonkeIsicanucanu nokuhlanza
Kungase kulandele ukudla noma kwenzeke ngokuzimelaUmthelela we-histamine H2 ku-asidi yesisu ungumhlanganyeli owaziwayo
I-Motility ingase ihluke phakathi kweziqephu kanye nesikhathiIvamise ukushayela imikhawulo yokudla - bona izexwayiso esigabeni 9
I-Neurological and cognitive
I-MCAS ichazwa ngokwengxenye ngobubanzi bayo. Ngenxa yokuthi ama-mast cell ahlala cishe kuzo zonke izicubu, izimpawu zingavela cishe noma kuphi - futhi imfuneko yezinhlelo zezitho ezimbili noma ngaphezulu ibalulekile ekuxilongweni.Amanothi
Ukuqunjelwa kanye nokusebenza kabusha kokudlaUbunzima bokugxilisa ingqondo, ukukhumbula, kanye nokuthola amagama
I-Neurological and cognitiveInkungu yengqondo
Ubunzima bokugxilisa ingqondo, ukukhumbula, kanye nokuthola amagamaIkhanda elibuhlungu kanye ne-migraine
I-Migraine ibikwa ngamanani aphezulu kulesi sibalo sabantuIsiyezi
Ivamise ukugqagqana nezici zenhliziyo nemithambo yegaziUkukhathala
Okuvame ukuba nzima futhi phakathi kwezimpawu ezibikiwe ezikhubaza kakhuluUkuzwela kwezinzwa
Ukukhanya, umsindo, kanye nephunga; Izindlela zisaphenywa
I-MCAS ichazwa ngokwengxenye ngobubanzi bayo. Ngenxa yokuthi ama-mast cell ahlala cishe kuzo zonke izicubu, izimpawu zingavela cishe noma kuphi - futhi imfuneko yezinhlelo zezitho ezimbili noma ngaphezulu ibalulekile ekuxilongweni.Amanothi
Imizwa ye-neuropathicIngase idlulelane nezimo zezicubu ezixhumeneyo okuxoxwe ngazo esigabeni 7
I-musculoskeletal, i-genitourinary, neyomqondoUbuhlungu obuhlangene nemisipha
Ukuphuthuma kwesinye kanye nobuningiUbuhlungu bethambo
Iqinisekisa ukuhlolwa, ikakhulukazi lapho i-mastocytosis icatshangwa khonaUkuphuthuma kwesinye kanye nobuningi
Izimpawu ezifana ne-Interstitial-cystitis ziyabikwaIzimpawu ziyahlukahluka ngomjikelezo wokuya esikhathini
Ithonya lamahomoni kuma-mast cells libhaliweIziqephu ezifana nokukhathazeka
I-classical allergy yindlela yokusabela e-IgE entweni ethile, ekhonjwayo. Iyakwazi ukukhiqizwa kabusha - ukuchayeka okufanayo kukhiqiza ukusabela okufanayo - futhi ngokuvamile kungaboniswa ngokuhlolwa kwesikhumba noma ukuhlolwa kwegazi okuqondile kwe-IgE. Ukungezwani namantongomane kuziphatha ngale ndlela; kanjalo ne-cat dander allergies.

Ukungena 'okufana nokukhathazeka' kufanele ukunakekelwa kuzo zombili izinkomba. Ukukhululwa komlamuli kungaveza i-physiology eqondile yokuhlasela kwe-panic, futhi iziguli zivame ukutholakala ngokungalungile ngaleyo sisekelo. Ngesikhathi esifanayo, ukukhathazeka nokucindezeleka kuhambisana ngempela nesifo esingelapheki futhi kufanele ukwelashwa ngokwako. Ukubona omunye akudingi ukuxosha omunye.

Ukushintsha kwemizwa nokucasuka

Kokubili kusuka emiphumeleni yomlamuleli nasekuphileni nesifo esingamahlalakhona, esingaziwa kahle

  • Ukusatshalaliswa kwesifunda. Ukuminyana kwe-mast cell kanye ne-phenotype kuyahlukahluka phakathi kwezicubu naphakathi kwabantu ngabanye, ngakho-ke izinhlelo zezitho ezithinteke kakhulu ziyehluka.
  • Ukukhishwa komlamuli ohlukile. Amaseli e-Mast angakhulula abalamuli abakhethiwe kunokuqukethwe kwabo okugcwele, futhi izingxube ezihlukene zokuxhumanisa ziveza izimpawu ezihlukene.
  • Iphrofayili yesamukeli. Ama-receptors avezwa kuma-mast cell omuntu abumba okuyizisusa ezisebenza njengezibangeli kuwo.
  • Ukusatshalaliswa kwesifunda. Ukuminyana kwe-mast cell kanye ne-phenotype kuyahlukahluka phakathi kwezicubu naphakathi kwabantu ngabanye, ngakho-ke izinhlelo zezitho ezithinteke kakhulu ziyehluka.
  • Ukukhishwa komlamuli ohlukile. Amaseli e-Mast angakhulula abalamuli abakhethiwe kunokuqukethwe kwabo okugcwele, futhi izingxube ezihlukene zokuxhumanisa ziveza izimpawu ezihlukene.
  • Iphrofayili yesamukeli. Ama-receptors avezwa kuma-mast cell omuntu abumba okuyizisusa ezisebenza njengezibangeli kuwo.
Okubonakalayo okuphakanyisiwe

Ibalazwe lomzimba wezimpawu: uhlaka lwe-anatomical olunama-callout anelebula endaweni ngayinye ethintekile - isikhumba, imigudu yomoya, inhliziyo, amathumbu, ubuchopho, amalunga, esinyeni - ngasinye siyanwebeka ukuze sibonise izimpawu ezihlobene nalolo hlelo. Ukuguqula kungagqamisa ukuthi yiziphi izinhlelo isiguli esithile esihlangabezana nazo.

Isiguli esinokusabela okuphindaphindekayo, ukuhlolwa kwe-IgE engemihle, nezibangeli zomzimba ezifana nokushisa noma ukucindezela kuyisethulo esivamile se-MCAS kunesiguli esinokuhlolwa kokungezwani komzimba okuhle. Iphaneli enegethivu ivamise ukuba yingxenye yesithombe kunengxabano ephikisana nayo.
  • Izimpawu zesikhumba, ukuphefumula, inhliziyo, i-GI, i-neurological, musculoskeletal, genitourinary, kanye nezizinda zengqondo.
  • Ukufakwa kwezinhlelo zezitho ezimbili noma ngaphezulu kuyingxenye yephethini yokuxilonga.
  • Alukho uphawu olulodwa oluqondile ku-MCAS - iphethini ye-episodic, multisystem yiyona ebalulekile.
  • Izimpawu zesikhumba, ukuphefumula, inhliziyo, i-GI, i-neurological, musculoskeletal, genitourinary, kanye nezizinda zengqondo.
  • Ukufakwa kwezinhlelo zezitho ezimbili noma ngaphezulu kuyingxenye yephethini yokuxilonga.
Isahluko 05

Okuvamile Izicupha

Iziqephu ezinjengokukhathazeka zingaqhutshwa ngumlamuleli; lokhu akufanele kusetshenziselwe ukususa lesi simo, noma ukulahla izidingo zangempela zempilo yengqondo.

Umqondo Oyinhloko

Ukwenza kusebenze okunqwabelanayo. Ukuchayeka okubekezelelwa ngakunye okuningana okufika ndawonye kungawela umkhawulo okungekho okungawela yedwa. Lona umbono owodwa osebenziseka kakhulu wokwenza umqondo wokusabela ngokusobala okungahleliwe.

Ukudla

Izimbangela zokudla ku-MCAS ngokuvamile zihlobene nokuqukethwe kwe-histamine noma ukuqondisa ukucasuka kwe-mast cell esikhundleni sokungavumelani nokudla kwakudala. I-histamine inqwabelana ekudleni njengoba iguga, ivubela, noma igcinwa, yingakho ubusha buvame ukuba nendaba ngaphezu kwesithako esithile - inhlanzi efanayo ingase ibekezelelwe ngosuku lokuthenga hhayi ezinsukwini ezintathu kamuva. Ushizi osekhulile, inyama eselashiwe negayiwe, ukudla okuvutshelwe, kanye nezinsalela kuvame ukuthinteka. Iqembu lesibili libonakala likhuthaza amaseli e-mast ukuthi akhiphe awawo i-histamine. Ukwehluka ngakunye kukhulu, futhi izinhlu ezishicilelwe ziphathwa kangcono njengezinto ezicatshangelwayo okufanele zihlolwe kunemithetho.

Ukushisa

Ukudla

Izimbangela zokudla ku-MCAS ngokuvamile zihlobene nokuqukethwe kwe-histamine noma ukuqondisa ukucasuka kwe-mast cell esikhundleni sokungavumelani nokudla kwakudala. I-histamine inqwabelana ekudleni njengoba iguga, ivubela, noma igcinwa, yingakho ubusha buvame ukuba nendaba ngaphezu kwesithako esithile - inhlanzi efanayo ingase ibekezelelwe ngosuku lokuthenga hhayi ezinsukwini ezintathu kamuva. Ushizi osekhulile, inyama eselashiwe negayiwe, ukudla okuvutshelwe, kanye nezinsalela kuvame ukuthinteka. Iqembu lesibili libonakala likhuthaza amaseli e-mast ukuthi akhiphe awawo i-histamine. Ukwehluka ngakunye kukhulu, futhi izinhlu ezishicilelwe ziphathwa kangcono njengezinto ezicatshangelwayo okufanele zihlolwe kunemithetho.

Umoya obandayo, amanzi abandayo, iziphuzo ezibandayo, nendawo efrijini kubikwa ukuthi kuyimbangela kwezinye iziguli. Ngokuphawulekayo, ngokushesha shintsha phakathi kwamazinga okushisa kuvame ukuchazwa njengokubi kakhulu kunanoma yiliphi izinga elibanjwayo eliqinile.

Phakathi kwezibangeli zomzimba ezibikwa kakhulu. Amashawa ashisayo namabhavu, ama-sauna, isimo sezulu esishisayo, amagumbi afudumele, neziphuzo ezishisayo konke kuvame ukucashunwa. Ukushisa akudingi lutho ukuze kungene emzimbeni, okuyingxenye yokuthi kungani ukuhlolwa okujwayelekile kwe-allergy kungakwazi ukukubona.

Kuyabanda

Ukucindezeleka

Ukuzivocavoca

Ukuzikhandla ngokomzimba kuyisisusa sangempela seziguli eziningi, futhi kunezindleko zangempela, njengoba umsebenzi uzuzisa impilo nesimo sengqondo. Ukuqina nezinga lokukhuphuka ngokuvamile kubaluleke ngaphezu kobude besikhathi esiphelele, yingakho izindlela ezihamba kancane, eziphansi kakhulu - kanye nomsebenzi osuselwe emanzini lapho ukuma kungabekezelelwa kahle - kuvame ukulawulwa kangcono.

Ukucindezeleka

Ukucindezeleka kuyisiqalo sokuphila, hhayi isifenqo. Amaseli e-Mast athwala ama-receptors e-hormone ekhulula i-corticotropin futhi ahlala eduze neziphetho zemizwa, anikeze umzila obhaliwe lapho isimiso sezinzwa sithonya ukusebenza kwawo. Leli phuzu lidinga ukugcizelelwa ngoba iziguli izimpawu zazo eziya ziba zimbi kakhulu ngenxa yokucindezeleka zivame ukutshelwa ukuthi isimo sazo singokwengqondo. Indlela ekhombisayo ye-neuroimmune ayiyona into efanayo nophawu olucatshangelwayo.

Amahomoni

Iziguli eziningi zibika amaphethini ezimpawu ze-cyclical, ngokuvamile ziba zimbi eduze nokuya esikhathini. I-Estrogen ithonya ukuziphatha kwe-mast cell, okungenye yesandla esihlongozwayo esilinganisweni esiphezulu sokuxilongwa kwabesifazane. Izinguquko ngesikhathi sokukhulelwa, emva kokubeletha, kanye ne-perimenopause nazo ziyachazwa, futhi ziyahlukahluka kakhulu phakathi kwabantu.

Amagciwane

Ukutheleleka kuphakamisa ngokuthembekile ukusebenza kabusha kwesisekelo ezigulini eziningi. Isifo esivamile esibangelwa amagciwane ngokuvamile sinweba i-trigger emisiwe amasonto ngemva kwalokho, ngakho ukuchayeka okwakubekezelelwe ngaphambili kuyavusa inkanuko phakathi nokululama. Ezinye iziguli ziqala ukuqala kwezimpawu zazo ekuguleni okuthelelanayo, nakuba ukusungula imbangela ezimweni ngazinye kunzima.

Ukuchayeka kwemvelo

Izimbangela ezibikiwe zemvelo zifaka isikhunta, uthuli, impova, intuthu, ukungcoliswa komoya, izinguquko zomswakama, kanye noshintsho lwengcindezi ye-barometric. Ukuzwela kuyahlukahluka kakhulu, futhi ukukhomba izibangeli zemvelo ngokuvamile kudinga ukugcinwa kwerekhodi okude kunokubhekwa okukodwa.

Imithi

Amakilasi athile emithi aqashelwa ezincwadini zamangqamuzana amakhulu njengezici ezingase zibe izishoshovu, okuhlanganisa ama-opioid, ezinye izidakamizwa ezingezona ukuvuvukala, imidiya ye-radiocontrast, ezinye izinto ezivimbela i-neuromuscular ezisetshenziswa ekubulaleni izinzwa, kanye ne-vancomycin. Izithako ezingasebenzi - odayi, izivimbelakuvunda, izigcwalisi - nakho kungaba yisici esicasulayo, yingakho isiguli singase sibekezelele ukwakheka komkhiqizi oyedwa futhi sisabele komunye.

Okubalulekile

Ungayeki, ugweme, noma uguqule umuthi owunikeziwe ngesisekelo sohlu olujwayelekile. Letha uhlu kumtholampilo owazi umlando wakho. Ingozi yokuyeka ukwelashwa okudingekayo ingadlula ingozi egwenywayo, futhi lesi sinqumo sidinga ukuhlolwa komuntu ngamunye.

Amakha nephunga

Amakha, izinto zokuhlanza umoya, imikhiqizo yokuhlanza, imikhiqizo yokuwasha, izincibilikisi, upende omusha, nefenisha entsha kuphakathi kwezimbangela ezibikwa njalo, futhi phakathi kwezinto okunzima kakhulu ukuzigwema ezindaweni ezihlanganyelwe. Izindawo ezingenawo amakha ziyisicelo sokuhlala esijwayelekile ezikoleni nasezindaweni zokusebenza ngenxa yalesi sizathu.

Utshwala

Utshwala buvame ukubikwa njengesiqalo emzileni ongaphezu kowodwa: iziphuzo eziningi ezidakayo zine-histamine ngokuqondile, ikakhulukazi iwayini nobhiya; utshwala bungaphazamisa ama-enzyme aphula i-histamine; futhi kubangela i-vasodilation ngokuzimela. Ukusabela kumanani amancane kuvame ukuchazwa.

Amasethi e-trigger ayahluka phakathi kwabantu ngabanye ngenxa yokuthi amaphrofayili ama-receptor, ukusatshalaliswa kwezicubu, izimo ezikhona kanye, kanye nokusebenza kabusha kwesisekelo konke kuyahluka. Nabo bayashintsha ngaphakathi umuntu ngokuhamba kwesikhathi - kuvame ukwanda ngezikhathi zokulawula okungekuhle kanye nokuncipha futhi uma ukwelashwa kwehlisa ukusebenza kabusha kwesisekelo. Ukusabela kungase kubambezeleke ngamahora, okusitha isixhumanisi phakathi kwembangela nomphumela. Kuzo zonke lezi zizathu, ukukhomba izibangeli kudinga ukugcinwa kwerekhodi okuhlelekile emavikini amaningi kuneziphetho ezithathwe esehlakalweni esisodwa.

Okubonakalayo okuphakanyisiwe

Ukungalali kahle noma okunganele kwehlisa ukubekezelelana ngokubanzi futhi kuhlanganyele nazo zonke ezinye izinto kulolu hlu. Akuvamile ukuba kube ukuphela kwembangela yokuqubuka, kodwa kungenye yabanikelayo abathintekayo, futhi iziguli zivame ukubika ukuthi ukuvikela ukulala kuphakamisa umkhawulo wazo kukho konke okunye.

Isiguli esinokusabela okuphindaphindekayo, ukuhlolwa kwe-IgE engemihle, nezibangeli zomzimba ezifana nokushisa noma ukucindezela kuyisethulo esivamile se-MCAS kunesiguli esinokuhlolwa kokungezwani komzimba okuhle. Iphaneli enegethivu ivamise ukuba yingxenye yesithombe kunengxabano ephikisana nayo.
  • Kungani ama-trigger ahluka kakhulu
  • Izinto ezibangela umzimba (ukushisa, ukubanda, ingcindezi, ukuzikhandla) azidingi into engenisiwe futhi azibonakali ekuhlolweni kokungezwani komzimba.
  • Ukucindezeleka kusebenza ngendlela ebhaliwe ye-neuroimmune; lokhu akusenzi isimo sibe ngokwengqondo.
  • Izitaki zokucupha — umthwalo oqongelelekayo ubaluleke ngaphezu kwanoma yikuphi ukuchayeka okukodwa.
  • Izinto ezibangela umzimba (ukushisa, ukubanda, ingcindezi, ukuzikhandla) azidingi into engenisiwe futhi azibonakali ekuhlolweni kokungezwani komzimba.
Isahluko 06

Kungani i-MCAS injalo
Kunzima uku Ukuxilonga

Amasethi e-trigger ayahluka phakathi kwabantu futhi agudluke ngokuhamba kwesikhathi, ngakho ukugcinwa kwerekhodi kubalulekile.

Izimpawu eziguquguqukayo

I-MCAS ikhiqiza inhlanganisela yezimpawu ezihlukene ezigulini ezihlukene, nezinhlanganisela ezahlukene esigulini esifanayo ngokuhamba kwesikhathi. Ayikho iphrezentheshini eyodwa yokufanisa iphethini emelene nayo. Ngenxa yokuthi izimpawu aziqondile ngazinye, ngasinye singabalelwa esimeni esivame kakhulu uma sicatshangelwa sodwa.

Ukubambezeleka kokuxilonga ku-MCAS akukhona ngokuyinhloko ukwehluleka kodokotela abangabodwana. Kulandela ezicini ezithile, ezikhonjwayo zesimo kanye nokuhlolwa okusetshenziselwa ukusiphenya.

Izimpawu eziguquguqukayo

Ukukhululwa komlamuli ngezikhathi ezithile

Lena inkinga yezobuchwepheshe emaphakathi. Abalamuli abalinganisiwe banesigamu sempilo emifushane. I-serum tryptase ifinyelela isiqongo phakathi namahora ambalwa okusabela bese iyawa; ama-metabolites omchamo aphikelela isikhathi eside kodwa asabonisa iwindi elilinganiselwe. Ukusampula phakathi kweziqephu kungase kuthole lutho oluphakeme - hhayi ngoba ukuqalisa akuzange kwenzeke, kodwa ngoba akusalinganiseki.

Umsebenzi wegazi ojwayelekile ngokuvamile awuphawuleki ku-MCAS. Isibalo segazi eligcwele, izimpawu zokuvuvukala, kanye ne-biochemistry evamile kuvame ukujwayelekile, njengamaphaneli okungezwani komzimba nezinto ezithile. Ukuhlolwa komlamuli okukhethekile kuyadingeka, futhi nalokho kuvame ukubuyisela imiphumela evamile ngezizathu zobuchwepheshe ezivezwe ngezansi. Ngakho-ke isiguli singaqongelela ifayela elibanzi lophenyo oluvamile kuyilapho sihlala singaphilile ngempela - iphethini ngeshwa eyandisa amathuba okuba sitshelwe ukuthi akukho okungalungile.

I-classical allergy yindlela yokusabela e-IgE entweni ethile, ekhonjwayo. Iyakwazi ukukhiqizwa kabusha - ukuchayeka okufanayo kukhiqiza ukusabela okufanayo - futhi ngokuvamile kungaboniswa ngokuhlolwa kwesikhumba noma ukuhlolwa kwegazi okuqondile kwe-IgE. Ukungezwani namantongomane kuziphatha ngale ndlela; kanjalo ne-cat dander allergies.

Isabelo esikhulu semiphumela yomlamuli ongemuhle siyabonisa isikhathi nokuphatha ngaphandle kokungabikho kwezifo. Ukuqinisekisa iphrothokholi yokuqoqwa nodokotela o-odayo kanye nelabhorethri ngaphambi kokuthatha amasampula kubaluleke kakhulu kunokuphinda ukuhlolwa okungaphathwanga kahle.

Hlangana nezinye izifo

Izimfuneko zokuphatha zihlanganisa lokhu. Abalamuli abaningana abazinzile ekamelweni lokushisa, futhi amasampula e-prostaglandin antekenteke kakhulu. Amasampuli ngokuvamile adinga ukugodola ngokushesha, ukuphatha okubandayo kulo lonke iqoqo, nokucutshungulwa ngokushesha. Isifanekiso esishiywe ekamelweni lokushisa noma esibambezelekile ekuhambeni singaveza umphumela ojwayelekile ovela esigulini ebesisabela ngokweqiniso.

Ukuntuleka kokuqwashisa udokotela

I-MCAS yabonakala ngokusemthethweni kuphela ngo-2007, ngemibandela yokuvumelana eyalandela ngo-2010 nango-2012. Odokotela abaningi abasebenzayo baphothula ukuqeqeshwa kwabo ngaphambi kokuba isimo singene ezinhlelweni ezijwayelekile. Ukujwayelana kuyahluka kakhulu ngisho nakuma-allergist kanye nodokotela bamasosha omzimba, futhi ukufinyelela kochwepheshe abanolwazi lwe-mast cell akulingani ngokwendawo.

Izimpawu ze-MCAS zigqagqana kakhulu nesifo samathumbu esicasulayo, i-urticaria engapheli, isifuba somoya, i-fibromyalgia, isifo sokukhathala okungamahlalakhona, ukuphazamiseka kokukhathazeka, i-dysautonomia, nezinye ezimbalwa. Ngayinye yalezi zijwayele kakhulu odokotela abaningi futhi kuvame ukuxilonga. Lapho isiguli sibonisa izimpawu ezilingana ezimbalwa zalezi ngokwengxenye, umphumela ovamile uwuchungechunge lokuxilonga ingxenye kunokuba kube okuhlanganisayo.

Kufanelekile ukusho ngokusobala ukuthi indlela yokuxilonga ye-MCAS ihlala iyisihloko senkulumompikiswano yangempela ngaphakathi kwezincwadi zezokwelapha. Ngokubanzi, isikhundla esisodwa sibambelele kumbandela oqinile odinga ubufakazi bomlamuli obunenhloso, ngezizathu zokuthi ngaphandle kwakho ubungozi bokuxilonga busetshenziswa kabanzi. Esinye isikhundla sithi imibandela eqinile igeja iziguli ezinesimo sangempela kodwa ukuhlolwa komlamuli wazo okuphindelelayo kwehluleka ngenxa yezizathu zobuchwepheshe ezichazwe ngenhla.

Lokhu akukona ukungaboni ngaso linye okwenziwe yiziguli, futhi akuxazululeki. Umphumela wayo ongokoqobo ukuthi ochwepheshe ababili abanekhono bangase bafinyelele iziphetho ezihlukene ngesiguli esifanayo. Ukuqonda ukuthi impikiswano ikhona kusiza ukuchaza imibono engqubuzanayo ngaphandle kokudinga ukucabanga ukuthi udokotela oyedwa wayenephutha.

Ingxabano yokuxilonga

Kufanelekile ukusho ngokusobala ukuthi indlela yokuxilonga ye-MCAS ihlala iyisihloko senkulumompikiswano yangempela ngaphakathi kwezincwadi zezokwelapha. Ngokubanzi, isikhundla esisodwa sibambelele kumbandela oqinile odinga ubufakazi bomlamuli obunenhloso, ngezizathu zokuthi ngaphandle kwakho ubungozi bokuxilonga busetshenziswa kabanzi. Esinye isikhundla sithi imibandela eqinile igeja iziguli ezinesimo sangempela kodwa ukuhlolwa komlamuli wazo okuphindelelayo kwehluleka ngenxa yezizathu zobuchwepheshe ezichazwe ngenhla.

Okubonakalayo okuphakanyisiwe

Lokhu akukona ukungaboni ngaso linye okwenziwe yiziguli, futhi akuxazululeki. Umphumela wayo ongokoqobo ukuthi ochwepheshe ababili abanekhono bangase bafinyelele iziphetho ezihlukene ngesiguli esifanayo. Ukuqonda ukuthi impikiswano ikhona kusiza ukuchaza imibono engqubuzanayo ngaphandle kokudinga ukucabanga ukuthi udokotela oyedwa wayenephutha.

Isiguli esinokusabela okuphindaphindekayo, ukuhlolwa kwe-IgE engemihle, nezibangeli zomzimba ezifana nokushisa noma ukucindezela kuyisethulo esivamile se-MCAS kunesiguli esinokuhlolwa kokungezwani komzimba okuhle. Iphaneli enegethivu ivamise ukuba yingxenye yesithombe kunengxabano ephikisana nayo.
  • Ukuhlolwa kwaselabhorethri okujwayelekile kuvame ukujwayelekile ku-MCAS; ukuhlolwa komlamuli okukhethekile kuyadingeka.
  • Abalamuli baphila isikhashana futhi abazinzile, ngakho-ke isikhathi nokuphatha amasampula kushayela amagethivu amaningi angamanga.
  • Izimpawu ziyedlulana nezimo ezimbalwa ezijwayelekile, okukhiqiza ukuxilonga okungaphelele.
  • Ukuhlolwa kwaselabhorethri okujwayelekile kuvame ukujwayelekile ku-MCAS; ukuhlolwa komlamuli okukhethekile kuyadingeka.
  • Abalamuli baphila isikhashana futhi abazinzile, ngakho-ke isikhathi nokuphatha amasampula kushayela amagethivu amaningi angamanga.
Isahluko 07

Okuhlobene Izimo

Indlela yokuxilonga isalokhu kuphikiswana ngayo, ngakho imibono yochwepheshe ingahluka ngokusemthethweni.

Funda lokhu kuqala

Ukuhlangana akuyona imbangela. Izimo ezimbalwa ezingezansi ziyenzeka kanye ne-MCAS kaningi kunalokho okungabikezelwa ithuba, kodwa noma ngabe zabelana ngendlela ethile, noma ngabe enye idala enye, noma izindlela zokudlulisela ezabiwe zikhulisa ukugqagqana okusobala akusungulwa kokuningi kwalokhu kumataniswa.

I-Postural Orthostatic Tachycardia Syndrome (POTS)

Funda lokhu kuqala

I-Hypermobile Ehlers-Danlos Syndrome (hEDS) kanye nokuphazamiseka kwe-hypermobility spectrum

Ukwenzeka ngokubambisana kwe-hEDS, POTS, ne-MCAS kuxoxwa kaningi ngokwanele ukuze kuchazwe ngokungakahleleki njengokuthathu. Inhlangano ibikwa ngokungaguquki, kodwa indlela eyisisekelo isalokhu iyinkoleloze. Amaseli e-Mast ahlala kuzicubu ezixhumeneyo futhi abambe iqhaza ekwenziweni kabusha kwezicubu, okunikeza umugqa ozwakalayo wophenyo, kodwa ukuba nokwenzeka akubona ubufakazi. Amaphethini okudluliselwa nawokuxilonga nawo angase abe nomthelela ekutheni ukugqagqana kunamandla kangakanani.

I-Hereditary alpha tryptasemia (HaT)

I-HaT iyisici sofuzo esibandakanya amakhophi engeziwe ofuzo lwe-alpha-tryptase I-TPSAB1, itholwa njengefa kuphethini ebusayo ye-autosomal, futhi ikhiqiza i-serum tryptase ye-serum eqhubekayo eqhubekayo. Kuvamile uma kuqhathaniswa nomphakathi. Ukuhlobana kwayo nezimpawu kusacaciswa: kuhlotshaniswa nokusabela okubucayi kwezinye izimo, kodwa abantu abaningi abanalesi sici abanazimpawu.

I-HaT ibalulekile ekutolikeni. Ngenxa yokuthi iphakamisa i-tryptase eyisisekelo, inani eliphakeme elilodwa le-tryptase eliphakanyisiwe kumuntu one-HaT alibonisi ngokwalo ukusebenza kwe-mast cell - okuqinisa ukuthi kungani umkhawulo wokuxilonga uchazwa ngokuthi ukuphakama ngaphezu kwesisekelo somuntu kunenombolo ephelele.

I-Hereditary alpha tryptasemia (HaT)

Izimpawu zamathumbu ku-MCAS zidlulana cishe ngokuphelele nenqubo ye-IBS, futhi ezinye iziguli ziphethe womabili amalebula. Ucwaningo luhlole ukuminyana kwe-mast cell kanye nokusebenza ku-gut mucosa yeziguli ze-IBS ngokutholwa okuhlukile. Ukuthi isethi engaphansi ye-IBS imele ukubandakanyeka kwe-mast cell okungaziwa kuwumbuzo ovulekile esikhundleni seqiniso eliqinisekisiwe.

I-Migraine

I-Migraine ibikwa ngamanani aphakeme phakathi kweziguli ze-MCAS. I-Histamine iyisithako se-vasoactive esisunguliwe esinokubaluleka okubonakalayo ekhanda, futhi ama-mast cell akhona kuma-meninges. Isixhumanisi semishini siyabonakala ngokwebhayoloji futhi singaphansi kophenyo, kodwa asixazululiwe.

Isifuba somoya kanye ne-urticaria engapheli

I-Migraine

I-Migraine ibikwa ngamanani aphakeme phakathi kweziguli ze-MCAS. I-Histamine iyisithako se-vasoactive esisunguliwe esinokubaluleka okubonakalayo ekhanda, futhi ama-mast cell akhona kuma-meninges. Isixhumanisi semishini siyabonakala ngokwebhayoloji futhi singaphansi kophenyo, kodwa asixazululiwe.

Abanye abacwaningi bahlongoze ukwenziwa kusebenze kwe-mast cell njengomnikelo ku-Long COVID, bephawula ukuthi izimpawu zinqwabelane ngokukhathala, ubunzima bokuqonda, kanye ne-dysautonomia. Lena indawo yophenyo olusebenzayo futhi ubufakazi bukhona okwandulelayo. Kufanele kuthathwe njengokucatshangelwa okungaphansi kocwaningo kunobudlelwano obumisiwe, kanye nezimangalo zokuxhumana okuxazululiwe - kunoma iyiphi indlela - okwamanje zidlula idatha.

Lawa anobudlelwane obucace kakhulu bemishini kumaseli amakhulu anoma yini kulesi sigaba. Amaseli e-Mast ayingxenye ye-pathophysiology yakho kokubili - i-leukotrienes ne-histamine drive bronchoconstriction kanye nokwakheka kwamasondo ngokulandelana. I-urticaria ezenzakalelayo ehlala njalo ikakhulukazi ihilela ukusebenza kwe-mast cell ngqo. Lezi akuzona izinhlangano eziqagelayo; ziyizimo lapho ukubandakanyeka kwe-mast cell kumiswa khona, nakuba zihlala ziyizifo ezihlukile ezivela ku-MCAS.

I-COVID ende

Amandla obufakazi ngokubuka nje
Izimo ze-AutoimmuneUbunjalo bobudlelwanoAmandla obufakazi ngokubuka nje
IsimoUbunjalo bobudlelwanoIsimo sobufakazi
I-urticaria engapheliUkusebenza kwe-Mast cell kuwumgogodla wesifo ngokwasoIsimo sobufakazi
Indlela esunguliweIsifuba somoyaAma-mast cell mediators ashayela i-bronchoconstriction
I-Hereditary alpha tryptasemiaIsici sofuzo esikhulisa i-tryptase yokuqala; kuthinta incazelo yokuhlolwaIsici esimisiwe; ubudlelwano bezimpawu busacaciswa
AMAPHOSTHIOkuvame ukwenzeka ngokubambisana; imiphumela yomlamuli kuthoni yemithambo ehlongozwayoInhlangano ibikiwe; indlela engaxazululiwe
I-MigraineI-hEDS / hypermobilityOkuvame ukwenzeka ngokubambisana; ukubandakanyeka kwezicubu ezixhumeneyo kuhlongozwayo
Inhlangano ibikiwe; indlela yokucabangaAmazinga aphezulu abikiwe; abaxhumanisi be-vasoactive abazwakalayoInhlangano ibikiwe; indlela esacwaningwayo
I-IBSUkugqagqana kwezimpawu; ukutholakala kwe-gut mast cell okuguquguqukayoUkugqagqana kucacile; ubudlelwano abuzange buxazululwe
I-Migraine ibikwa ngamanani aphakeme phakathi kweziguli ze-MCAS. I-Histamine iyisithako se-vasoactive esisunguliwe esinokubaluleka okubonakalayo ekhanda, futhi ama-mast cell akhona kuma-meninges. Isixhumanisi semishini siyabonakala ngokwebhayoloji futhi singaphansi kophenyo, kodwa asixazululiwe.Isifo se-AutoimmuneKubikiwe ukuhlangana; ukwabiwa kwe-immune dysregulation okuhlongozwayo
Isiguli esinokusabela okuphindaphindekayo, ukuhlolwa kwe-IgE engemihle, nezibangeli zomzimba ezifana nokushisa noma ukucindezela kuyisethulo esivamile se-MCAS kunesiguli esinokuhlolwa kokungezwani komzimba okuhle. Iphaneli enegethivu ivamise ukuba yingxenye yesithombe kunengxabano ephikisana nayo.
  • Inhlangano ibikiwe; indlela ayikasungulwa
  • Ukugqagqana kwezimpawu; ukubandakanyeka kwe-mast cell kucatshangelwe
  • Okwandulelayo - ngaphansi kophenyo olusebenzayo
  • Ukubandakanyeka kwe-mast cell kusungulwa ku-urticaria engapheli kanye nesifuba somoya - lezi akuzona izixhumanisi zokuqagela.
  • I-HaT iwuphawu lwangempela lofuzo oluphakamisa i-tryptase eyisisekelo futhi lushintshe indlela imiphumela yokuhlolwa okufanele ifundwe ngayo.
Isahluko 08

Ukuqonda Kwamanje
kwe Izimbangela

Kuyo yonke le ndawo, ukuhlobana kuye kwenziwa iphutha ngokuphindaphindiwe njengembangela - funda izimangalo ngokucophelela.

Amandla obufakazi ngokubuka nje

Ayikho imbangela eyodwa ye-MCAS esunguliwe. Amafektha angezansi yizindawo zophenyo ezinamazinga ahlukene okusekela. Noma yimuphi umthombo owethula i-akhawunti ethembekile, ehlanganisiwe ye-MCAS okwamanje uyabudlula ubufakazi.

I-Genetics

Yini esunguliwe: I-alpha tryptasemia yofuzo iyisici sofuzo sangempela, esinezici ezinhle ezibandakanya amakhophi engeziwe I-TPSAB1, futhi iphakamisa i-tryptase eyisisekelo. Ngokwehlukana, i KIT Ukuguqulwa kwe-D816V kusungulwa njengomshayeli kuningi labantu abadala be-systemic mastocytosis - isimo esihlukile, kodwa esibonisa ukuthi ukuguqulwa okukodwa kungaphazamisa kanjani i-mast cell biology.

Yini engekho: Akukho ukuguqulwa kwe-causative okufanayo okuhlonziwe ku-MCAS. Ukuhlangana komndeni kuyabikwa, futhi ezinye iziguli zichaza izihlobo eziningi ezithintekile, kodwa iphethini yefa echaziwe ye-MCAS ngokwayo ayikasungulwa. Ukuthi i-MCAS imele isimo esisodwa esinesisekelo sofuzo esingakatholakali, noma izimo ezimbalwa ezihlukene eziqoqwe ngaphansi kwelebula yomtholampilo eyodwa, akuxazululwa.

I-Genetics

I-Broad immune dysregulation iyindawo ehamba phambili yophenyo. Izindlela ezihlongozwayo zifaka phakathi ukuguquguquka kokuvezwa kwesamukeli noma ukuzwela kuma-mast cell, ukusayinda okuphazamisayo okulawula okungavamise ukuvimba ukuqalisa, nokusebenzisana okungavamile phakathi kwamaseli e-mast nezinye izakhamuzi zokuzivikela ezifweni. Lezi zinengqondo futhi zicwaningwa ngenkuthalo, kodwa zichaza isigaba sencazelo kunendlela ebonisiwe.

Ukucindezeleka kuyisiqalo sokuphila, hhayi isifenqo. Amaseli e-Mast athwala ama-receptors e-hormone ekhulula i-corticotropin futhi ahlala eduze neziphetho zemizwa, anikeze umzila obhaliwe lapho isimiso sezinzwa sithonya ukusebenza kwawo. Leli phuzu lidinga ukugcizelelwa ngoba iziguli izimpawu zazo eziya ziba zimbi kakhulu ngenxa yokucindezeleka zivame ukutshelwa ukuthi isimo sazo singokwengqondo. Indlela ekhombisayo ye-neuroimmune ayiyona into efanayo nophawu olucatshangelwayo.

Iziguli eziningi zibonisa izimpawu zokuqala zesifo esithathelwanayo. Ukutheleleka kwaziwa ngokwenza amaseli e-mast asebenze ngama-receptors afana ne-Toll, ahlinzeka ngomzila ozwakalayo, futhi ukuqala kwangemva kokutheleleka kuyiphethini eyaziwayo ezimeni ezimbalwa ezingelapheki. Nokho, ukusungula ukuthi ukutheleleka kubangelwa I-MCAS esimweni ngasinye inzima kakhulu ngokwendlela, futhi ukukhumbula ukuchema kuthinta ama-akhawunti abheka emuva. Lokhu kuseyinhlangano esacwaningwayo.

Ukungasebenzi kahle kwamasosha omzimba

I-Broad immune dysregulation iyindawo ehamba phambili yophenyo. Izindlela ezihlongozwayo zifaka phakathi ukuguquguquka kokuvezwa kwesamukeli noma ukuzwela kuma-mast cell, ukusayinda okuphazamisayo okulawula okungavamise ukuvimba ukuqalisa, nokusebenzisana okungavamile phakathi kwamaseli e-mast nezinye izakhamuzi zokuzivikela ezifweni. Lokhu kunengqondo futhi kufundwe ngenkuthalo, kodwa kuchaza isigaba sencazelo kunendlela ebonisiwe.

Ukuvuvukala okungapheli

Izici zemvelo

Abahlanganyeli bezemvelo abahlongozwayo bahlanganisa ukuchayeka kwamakhemikhali, isikhunta, ukungcoliswa komoya, kanye nezinguquko zokudla ezingeni labantu. Ubufakazi lapha bubuthakathaka kakhulu kunobezindawo ezingenhla, futhi obuningi babo abuqondile. Lena indawo ezwakalayo yophenyo esikhundleni sokuba negalelo elisunguliwe, futhi kuyindawo lapho izimangalo zezohwebo ezizethembayo zivamile futhi zisekelwa kabi.

Ukuvuvukala okungapheli

Amaseli e-Mast womabili asabela futhi anomthelela ekuvuvukeni, okuphakamisa amathuba okuba khona kwemijikelezo yokuziqinisa lapho ukwenza kusebenze kuzala indawo evuvukalayo eyehlisa umkhawulo wokuqhubeka kusebenze. Lokhu kuhambisana ngokomshini futhi kuhambisana nokubonwa komtholampilo ukuthi ukuphinda kusebenze kuyakhula phakathi nokulawula okungeyikho. Ihlala iyimodeli esikhundleni sesizathu esibonisiwe.

I-Epigenetics

Okubonakalayo okuphakanyisiwe

Umdwebo wesigaba sobufakazi: amabhande agxilile ahambela ngaphandle asuka 'Kumisiwe' (isici se-HaT; i-KIT D816V ku-mastocytosis) ngokusebenzisa 'Ukuphenywa okusebenzayo ngobufakazi obusekelayo' (ukungasebenzi kahle kwamasosha omzimba, ukuqala kwangemva kokutheleleka) ukuya 'ku-Hypothesised, ubufakazi obuqondile obulinganiselwe' (i-epigenetics, ukuchayeka okubonakalayo kwezemvelo.

Isiguli esinokusabela okuphindaphindekayo, ukuhlolwa kwe-IgE engemihle, nezibangeli zomzimba ezifana nokushisa noma ukucindezela kuyisethulo esivamile se-MCAS kunesiguli esinokuhlolwa kokungezwani komzimba okuhle. Iphaneli enegethivu ivamise ukuba yingxenye yesithombe kunengxabano ephikisana nayo.
  • Lapho lokhu kushiya izinto
  • I-HaT kanye nokuguqulwa kwe-KIT D816V ku-mastocytosis kuwufuzo olumisiwe - futhi akuyona imbangela eqinisekisiwe ye-MCAS.
  • Ukungasebenzi kahle kwamasosha omzimba kanye nokuqala kwangemva kokutheleleka kuyaphenywa ngobufakazi obuthile obusekelayo.
  • Ayikho imbangela ye-MCAS esunguliwe.
  • Phatha izimangalo 'eziwumsuka' ngokuzethemba ngokungabaza - umphakathi wocwaningo awukazihlonzi esisodwa.
Isahluko 09

Ukuhlala Na I-MCAS

Phatha izimangalo 'eziwumsuka' ngokuzethemba ngokungabaza - umphakathi wocwaningo awukazihlonzi esisodwa.

Ukuphathwa kwansuku zonke kanye nezinqubo

Iziguli eziningi zithola ukuthi ukungaguquguquki kuphakamisa umkhawulo wazo ngokuthembekile kunanoma yisiphi isilinganiso somuntu ngamunye. Ukulala okuvamile nezikhathi zokuvuka, isikhathi sokudla esibikezelwayo, izinga lokushisa lendawo elizinzile lapho kufinyeleleka khona, kanye nesikhathi semithi esingaguquki konke kunciphisa ukuhlukahluka. Imijikelezo iphinde yenze ukuhlonza i-trigger kwenzeke: uma okuhlukile okuningi kuzinzile, lezo ezishintshile ziyabonakala.

Ukuphatha kwakhiwe kakhulu ngokusebenza kwansuku zonke kunanoma yikuphi ukungenelela okukodwa. Okulandelayo kuqondiswe ekusebenzeni - inhloso impilo ebanzi kunazo zonke ezosekelwa yi-physiology yakho.

Ukuphathwa kwansuku zonke kanye nezinqubo

Iziguli eziningi zithola ukuthi ukungaguquguquki kuphakamisa umkhawulo wazo ngokuthembekile kunanoma yisiphi isilinganiso somuntu ngamunye. Ukulala okuvamile nezikhathi zokuvuka, isikhathi sokudla esibikezelwayo, izinga lokushisa lendawo elizinzile lapho kufinyeleleka khona, kanye nesikhathi semithi esingaguquki konke kunciphisa ukuhlukahluka. Imijikelezo iphinde yenze ukuhlonza i-trigger kwenzeke: uma okuhlukile okuningi kuzinzile, lezo ezishintshile ziyabonakala.

I-Pacing itimu ephindaphindayo kuma-akhawunti esiguli — ukusabalalisa umsebenzi usuku lonke noma iviki kunokugxilisa kuwo, nokwakha ngesikhathi sokuthola kabusha ngemva kwezidingo ezaziwayo. Lokhu akufani nokungasebenzi, futhi ukungasebenzi isikhathi eside kunezindleko zakho ezibhalwe kahle.

I-classical allergy yindlela yokusabela e-IgE entweni ethile, ekhonjwayo. Iyakwazi ukukhiqizwa kabusha - ukuchayeka okufanayo kukhiqiza ukusabela okufanayo - futhi ngokuvamile kungaboniswa ngokuhlolwa kwesikhumba noma ukuhlolwa kwegazi okuqondile kwe-IgE. Ukungezwani namantongomane kuziphatha ngale ndlela; kanjalo ne-cat dander allergies.

Idayari yezimpawu ingenye yezinto eziwusizo kakhulu isiguli esingaziletha ku-aphoyintimenti: ukhonkolo, i-longitudinal, futhi ethize kubo ngendlela okungekho hlu lwezicupho ezishicilelwe olungaba khona. Kuyasiza futhi ukuhlukanisa izibangeli zangempela kokuzenzekela, okuyinto inkumbulo iyodwa eyenza kabi.

Irekhodi elihlelekile liwumzila othembeke kakhulu wokuhlonza izibangeli zomuntu siqu. Okufakiwe okuwusizo kudlula kakhulu ukudla: isikhathi sosuku, izinga lokushisa lendawo, okudliwe nokuthi bekukusha kangakanani, ukuvivinya umzimba, ingcindezi nokulala, ukuchayeka kwephunga, imithi ephuziwe, kanye - neziguli ezisesikhathini - ukuma komjikelezo. Izimpawu kufanele zirekhodwe ngesikhathi kanye nokuqina.

Ukwethula izimpawu ezihleliwe ngohlelo lwesitho kunokuba ngokulandelana kwezikhathi kwenza iphethini yesistimu eningi ibonakale ngokushesha, lapho i-akhawunti elandisayo ivame ukuyifihla. Ukuletha imiphumela yangaphambili - okuhlanganisa nejwayelekile - kufundisa ngempela futhi kugwema ukuphinda uphenyo oluqediwe.

Idayari yezimpawu ingenye yezinto eziwusizo kakhulu isiguli esingaziletha ku-aphoyintimenti: ukhonkolo, i-longitudinal, futhi ethize kubo ngendlela okungekho hlu lwezicupho ezishicilelwe olungaba khona. Kuyasiza futhi ukuhlukanisa izibangeli zangempela kokuzenzekela, okuyinto inkumbulo iyodwa eyenza kabi.

Ukusebenza nodokotela

Izindawo zokuhlala ezivamile zihlanganisa izinqubomgomo zekilasi ezingenawo amakha, imvume yokuthwala nokuzinikeza imithi, ukufinyelela emanzini nekhefu lokuphumula, ukucabangela izinga lokushisa lapho uhlezi, ukuguquguquka lapho ufika ngesikhathi sokuvutha, kanye nokulungiswa kwemfundo yokuzivocavoca. Lapho kunezinhlelo zokulala ezisemthethweni ohlelweni lwakho lwezemfundo, ukubhala ukuthi ukutholakala kwezifo kubhalwe phansi ngokuvamile kwenza ukufinyelela kuzo kube lula kakhulu.

Ukubuza ngokuqondile mayelana nesikhathi sokuhlola umlamuli kanye nokuphathwa kwesampula kunengqondo futhi kuthuthukisa ngokubonakalayo ithuba lomphumela osebenzisekayo. Uma ungezwakali, ukufuna umbono wesibili kusemthethweni; isifo se-mast cell siyindawo ye-niche futhi ukujwayela kuyahluka kakhulu. Ukuxoshwa kanye nje kuvamile futhi akusona isinqumo.

Isikole

Izindawo zokuhlala ezivamile zihlanganisa izinqubomgomo zekilasi ezingenawo amakha, imvume yokuthwala nokuzinikeza imithi, ukufinyelela emanzini nekhefu lokuphumula, ukucabangela izinga lokushisa lapho uhlezi, ukuguquguquka lapho ufika ngesikhathi sokuvutha, kanye nokulungiswa kwemfundo yokuzivocavoca. Lapho kunezinhlelo zokulala ezisemthethweni ohlelweni lwakho lwezemfundo, ukubhala ukuthi ukutholakala kwezifo kubhalwe phansi ngokuvamile kwenza ukufinyelela kuzo kube lula kakhulu.

Sebenza

Ukucatshangelwa kwendawo yokusebenza kufana nalezo zesikole: izinqubomgomo zephunga, ukulawula izinga lokushisa, ukuhlela okuguquguqukayo noma okukude ngesikhathi sokuvutha, nokulungiswa kweshejuli lapho ukukhathala kulandela iphethini ebikezelwayo. Ukuthi noma kungakanani okumele kudalulwe kuyisinqumo somuntu siqu esinokuhwebelana kwangempela, futhi sisebenzisana nokuvikelwa kokuqashwa okuhluke kakhulu phakathi kwezindawo ezithile.

Ukuhamba

Izinyathelo ezisebenzayo ezivame ukushiwo iziguli zihlanganisa ukuphatha imithi emithwalweni yesandla enemibhalo, ukuthola incwadi kadokotela yemithi ejovwayo, ukucwaninga izikhungo zezokwelapha lapho uya khona, ukuhlela ukudla kusenesikhathi lapho ukusabela kubalulekile, kanye nokuvumela isikhathi sokululama lapho ufika. Ukushintsha kwezinga lokushisa nokuphakama kufanele kulindelwe.

Impilo yengqondo

Ukuphila nesifo esishintshashintshayo, esingaqashelwanga kahle, futhi esivame ukuxoshwa kunezindleko zangempela zengqondo, futhi lokho kubiza akukona ubuthakathaka bomlingisi. Iziguli eziningi zichaza isikhathi eside sokungakholwa ngaphambi kokuxilongwa, okushiya uphawu lwazo. Ukusekela - kungakhathaliseki ukuthi uchwepheshe, ontanga, noma kokubili - kuyingxenye enengqondo yokuphatha esikhundleni sokuvuma ukuthi izimpawu zingokwengqondo.

  • Umehluko okufanele ubanjwe yilokhu: ukukhululwa komlamuli kungaveza isayensi yengqondo efana nokukhathazeka, futhi lokho kufanele ukuqashelwa esikhundleni sokuphinda kubhalwe kabusha ngokwengqondo kuso sonke isimo. Ngesikhathi esifanayo, ukukhathazeka nokucindezeleka kujwayelekile, kuyalapheka, futhi kufanelekile ukubhekana nakho ngokwako uma kukhona.
  • I-epinephrine iwumugqa wokuqala wokwelashwa kwe-anaphylaxis. Ama-Antihistamine awathathi indawo futhi akufanele kuthenjelwe kuwo ekuphenduleni okunzima kakhulu.
  • Ezinye iziguli ezinesifo se-mast cell zisengozini ye-anaphylaxis - ukusabela okusheshayo, okunzima, okungase kube yingozi empilweni. Lapho lokho kuba sengozini, ukuhlela akuyona into yokuzikhethela, futhi kuyingxoxo ongaba nayo nodokotela ngaphambi kokuba kudingeke.
  • Iziguli ezibhekwa njengezisengozini ngokuvamile zinikezwa ama-epinephrine auto-injection futhi zelulekwa ukuthi ziwathwale njalo.
  • I-epinephrine iwumugqa wokuqala wokwelashwa kwe-anaphylaxis. Ama-Antihistamine awathathi indawo futhi akufanele kuthenjelwe kuwo ekuphenduleni okunzima kakhulu.
  • Uhlelo olubhaliwe lwesenzo sezimo eziphuthumayo okuvunyelenwe ngalo nodokotela wakho kufanele luveze izimpawu eziyisixwayiso, lokho okumelwe ukwenze, nokuthi kufanele ushayele nini abezimo eziphuthumayo.
Izimbangela ezibikiwe zemvelo zifaka isikhunta, uthuli, impova, intuthu, ukungcoliswa komoya, izinguquko zomswakama, kanye noshintsho lwengcindezi ye-barometric. Ukuzwela kuyahlukahluka kakhulu, futhi ukukhomba izibangeli zemvelo ngokuvamile kudinga ukugcinwa kwerekhodi okude kunokubhekwa okukodwa.

Iziguli ezibhekwa njengezisengozini ngokuvamile zinikezwa ama-epinephrine auto-injection futhi ziyalelwa ukuthi ziwathwale njalo. I-epinephrine iwumugqa wokuqala wokwelapha we-anaphylaxis; ama-antihistamine awathathi indawo yawo futhi akufanele kuthenjelwe kuwo ekuphenduleni okunzima kakhulu.

Inothi lokuvinjelwa kokudla

Umazisi wezokwelapha oqaphela ukuxilongwa kanye nezimbangela zemithi ezaziwayo zisiza abasebenzi bezimo eziphuthumayo okungenzeka abasazi lesi simo.

Ukuhlinzwa nokubulala izinzwa kuqinisekisa ukuxoxisana kusengaphambili ukuze ama-agent akwazi ukukhethwa kucatshangwa ngesimo.

Isiguli esinokusabela okuphindaphindekayo, ukuhlolwa kwe-IgE engemihle, nezibangeli zomzimba ezifana nokushisa noma ukucindezela kuyisethulo esivamile se-MCAS kunesiguli esinokuhlolwa kokungezwani komzimba okuhle. Iphaneli enegethivu ivamise ukuba yingxenye yesithombe kunengxabano ephikisana nayo.
  • Inothi lokuvinjelwa kokudla
  • Idayari yezimpawu ehleliwe emavikini amaningi iyindlela ethembeke kakhulu yokuhlonza izibangeli zomuntu siqu.
  • Ukwethula izimpawu ngohlelo lwesitho kwenza iphethini ye-multisystem ibonakale kodokotela.
  • Izinqubo ezingaguquguquki ziphakamisa umkhawulo futhi zenze ukuhlonzwa kwembangeli kwenzeke.
  • Idayari yezimpawu ehleliwe emavikini amaningi iyindlela ethembeke kakhulu yokuhlonza izibangeli zomuntu siqu.
  • Ukwethula izimpawu ngohlelo lwesitho kwenza iphethini ye-multisystem ibonakale kodokotela.
Isigaba 10

Okuvame Ukubuzwa Imibuzo

Imibuzo engu-25 iziguli, imindeni, kanye nabelaphi abavame ukuyibuza. Khetha noma yimuphi umbuzo ukuze uwunwebe.

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Ingabe i-MCAS iwufuzo?

Izimpawu zingathuthuka kakhulu, kwesinye isikhathi kuze kufike ezingeni lapho iziguli zizichaza khona njengezingabonakali kahle - ikakhulukazi uma ukwelashwa kwehlisa ukusebenza kabusha kwesisekelo futhi kukhonjwe izibangeli ezinkulu. Ukulungiswa okuzenzakalelayo, okuhlala njalo ngaphandle kwanoma yikuphi ukuphatha akubhaliwe kahle. Izikhathi zokuxolelwa ezilandelwa ukuphindeka zivame ukuchazwa.

Izinto ezimbalwa ziyashintsha ngokuhamba kwesikhathi: ukuphinda kusebenze kabusha kwesisekelo kuhamba nokulala, ingcindezi, isimo sehomoni, kanye nokutheleleka kwakamuva; ama-mast cell angakhulula inhlanganisela yomlamuli ehlukene ngezikhathi ezahlukene; futhi ama-trigger azibeka ngokwawo ayanda ngesikhathi sokulawula okungalungile futhi ngokuvamile anciphise futhi ngokwelashwa. Ukushintsha izimpawu kuyisici sesimo kunokuba kube uphawu lokuthi ukuxilongwa akulungile.

Ngokuvamile ngenxa yezizathu zobuchwepheshe. Abalamuli abalinganisiwe banesigamu sokuphila esifushane, ngakho ukusampula phakathi kokusabela kungase kuthole lutho oluphakeme. Eziningi nazo azizinzile ezingeni lokushisa legumbi futhi zidinga ukugodola ngokushesha nokucutshungulwa ngokushesha - isampula ehlezi noma elibaziseke endleleni yokuthutha ingafunda ngokwejwayelekile esigulini ebesisabela ngokweqiniso. Umsebenzi wegazi ojwayelekile kanye namaphaneli okungezwani komzimba nakho kulindeleke ukuthi kube okuvamile ku-MCAS, njengoba ayakhelwe ukukubona.

Yebo, ngomzila wokuphila obhaliwe. Amaseli e-Mast athwala ama-receptors e-hormone ekhulula i-corticotropin futhi ahlale eduze neziphetho zemizwa, enikeza i-stress physiology ukufinyelela okuqondile kuwo. Lokhu kubalulekile ukukusho ngokucacile, ngoba iziguli izimpawu zazo eziba zimbi kakhulu ngaphansi kwengcindezi zivame ukutshelwa ukuthi lesi simo singokwengqondo. Indlela ye-neuroimmune ayifani nophawu olucatshangelwayo.

Cha. Ukushintsha ukudla kunganciphisa umthwalo wezimpawu kwezinye iziguli ngokunciphisa inani le-trigger load, futhi leyo nzuzo kuwufanele ukuphishekela ngokucabangisisa ngayo - kodwa ayiselisi isimo. Ubufakazi bokudla okune-histamine ephansi kunomkhawulo futhi bekhwalithi enesizotha. Ukuvinjelwa isikhathi eside okungagadiwe kuthwala izingozi zangempela zokuntuleka komsoco kanye nokudla okungenamsoco, ngakho-ke ukuhlola okuhlelekile, okunomkhawulo wesikhathi nokwethulwa kabusha kuphephe kakhulu kunokugwema okuvulekile.

Ku-mastocytosis umzimba uqongelela amangqamuzana ensika amaningi kakhulu, futhi lokho okweqile kungaboniswa - nge-biopsy yomnkantsha, izilonda zesikhumba eziyisici, noma i-tryptase eyisisekelo eqhubekayo eqhubekayo, ngokuvamile ngokuguqulwa kwe-KIT D816V kusifo sesistimu yabantu abadala. Ku-MCAS inani lamaseli ensika livamile; okungavamile ukuthi zisebenza kalula kangakanani. Kungakho i-mastocytosis ingaqinisekiswa ngokuthola amaseli futhi i-MCAS ayikwazi.

Cha, nakuba bevame ukudideka. Ukungabekezelelani kwe-histamine ngokuvamile kuchazwa njengobunzima bokwephula i-histamine engenisiwe, ngokuvamile okubangelwa umsebenzi oncishisiwe we-enzyme diamine oxidase. I-MCAS ibandakanya amaseli e-mast omzimba akhipha i-histamine nabanye abaxhumanisi abaningi ngokungafanele. Zingaveza izimpawu ezeqile futhi zingase zibe khona ndawonye, ​​kodwa izindlela ziyahlukahluka.

Yilapho kanye ochwepheshe bengavumelani khona. Imibandela yokuvumelana idinga ubufakazi obunenjongo bokukhululwa komlamuli, futhi odokotela abaningi babambelela kulokho ngokuqinile. Abanye baphikisa ngokuthi isicelo esiqinile sigeja iziguli ezivivinywa zazo ezihluleka ngokuphindaphindiwe ngenxa yezizathu zesikhathi nokusingatha. Inkulumo-mpikiswano ayixazululiwe ezincwadini, yingakho ochwepheshe ababili abanekhono bengafinyelela iziphetho ezihlukene ngesiguli esifanayo.

Ama-Allergists kanye ne-immunologists ngokuvamile, kanye ne-hematologists lapho i-mastocytosis ibhekwa khona. Empeleni, ukujwayela kuyahluka kakhulu ngisho nangaphakathi kwalezo zici, futhi ukufinyelela kubahlengikazi abanolwazi lwe-mast cell akulingani ngokwendawo. Iziguli eziningi zithola ukuthi udokotela ojwayelekile onolwazi oluphelele ozimisele ukuhlanganisa ukunakekelwa kubaluleke njengokuqokwa kochwepheshe.

Ngokuvamile zisiza kodwa azivamile ukuxazulula yonke into, futhi leyo mpendulo eyingxenye ilindelekile kunophawu lokuthi ukuxilongwa akulungile. Ama-Antihistamine avimba i-histamine kuphela; i-prostaglandin, i-leukotrienes, i-platelet activating factor, nama-cytokines awathinteki. Yingakho nje ukwelashwa kukhula ngezinga, okwengeza ama-ejenti akhuluma nabaxazululi abaphuthelwa ama-antihistamine.

Izincazelo ezimbili ezivamile. Ezinye izigaba zezidakamizwa zingenza ama-mast cells asebenze ngokuqondile, ngaphandle kwanoma iyiphi indlela yokungezwani nomzimba. Ngokuhlukana, izithako ezingasebenzi - odayi, izigcwalisi, izilondolozi - kungaba ingxenye ecasulayo kunokuba umuthi osebenzayo, yingakho isiguli singase sibekezelele inguqulo yomkhiqizi oyedwa futhi sisabele komunye. Amalungiselelo ahlanganisiwe ngezinye izikhathi asetshenziswa lapho kusolwa khona lokhu. Xoxa nganoma yikuphi ukusabela okusolisayo nodokotela wakho kunokuba ume ucele.

Kungaba, kuye ngobunzima kanye nohlaka lomthetho lapho uhlala khona. Ezinye iziguli zisebenza futhi zifunda ngaphandle kokulungiswa; ezinye zilinganiselwe kakhulu. Ukunqunywa kokukhubazeka kuqondene nendawo ethile futhi ngokuvamile kudinga imibhalo yomthelela wokusebenza kunokuxilongwa kuphela. I-akhawunti ebhaliwe kadokotela yemikhawulo ivamise ukuba usizo kakhulu kunelebula yokuxilonga iyodwa.

Yebo. Ezinye iziguli ezinesifo se-mast cell zisengozini ye-anaphylaxis, futhi lapho leyo ngozi isebenza khona, ama-epinephrine auto-injection kanye nohlelo lwesenzo oluphuthumayo olubhaliwe lujwayelekile. I-epinephrine ingumugqa wokuqala; i-antihistamine ayithathi indawo yokusabela okunzima kakhulu. Ukuthi le ngozi iyasebenza yini kuwe ukuhlolwa komtholampilo komuntu ngamunye.

Okuhlangenwe nakho okubikiwe kuyehluka kakhulu - ezinye iziguli zichaza ukuthuthukiswa ngesikhathi sokukhulelwa, ezinye ziba zimbi kakhulu, kanti ezinye azikho izinguquko ezicacile. Ithonya lamahomoni kuma-mast cells libhaliwe, okwenza ushintsho kunoma iyiphi indlela ibonakale. Ukukhulelwa nge-MCAS kuqinisekisa ukuhlela kusengaphambili nabo bobabili udokotela wakho we-mast cell kanye nethimba lakho lokubelethisa, ikakhulukazi mayelana nokuthi yimiphi imithi ezoqhubeka.

Ngoba ama-mast cells aphendula ngokuqondile ekushukunyisweni komzimba, hhayi kuphela ezintweni ezingezwani nomzimba. Ukushisa, amakhaza, ingcindezi, ukungqubuzana, nokuzikhandla konke kungakwenza kusebenze ngaphandle kokungena emzimbeni. Ukuhlolwa kokungezwani komzimba kuklanyelwe ukuthola ukuzwela kwe-IgE-mediated kumaphrotheni athile, ngakho-ke akukwazi ukubona lezi zimbangela - yingakho iphaneli yokungezwani komzimba ehlanzekile ihambisana ngokuphelele nokusabela kwangempela ekushiseni.

Kuya nge-ejenti. Ama-Antihistamine ngokuvamile abonisa ukusebenza phakathi nezinsuku. Izinzinzisi ze-mast cell ezifana ne-cromolyn ne-ketotifen ngokuvamile zidinga amasonto okusetshenziswa okungashintshi ngaphambi kokuba kubonakale inzuzo yazo egcwele, futhi ukuma kusenesikhathi kuyindlela evamile yokulahla okuthile obekungasebenza. Ukuhlolwa okungamaviki amane kuya kwayisithupha ngoshintsho kuyisimiso esivame ukucashunwa.

Ngokujwayelekile cha, futhi leli elinye lamaphutha ajwayelekile okuzilawula. Ukuqala ama-ejenti amaningana ndawonye kwenza umphumela ungachazeki: uma izinto ziba ngcono, awukwazi ukusho ukuthi yiluphi ushintsho olube nesibopho, futhi uma usabela, awukwazi ukusho ukuthi yikuphi okumele uyeke. Ushintsho olulodwa ngesikhathi luhamba kancane kodwa yilo elenza inqubo ifundeke.

Iziguli eziningi zidethi izimpawu zokuqala zesifo esithathelwanayo, futhi ukutheleleka kwaziwa ngokwenza ama-mast cells asebenze ngama-Toll-like receptors, ngakho umzila ohlongozwayo unengqondo. Ukuthi ukutheleleka kubangela i-MCAS akukasungulwa, futhi ukubonisa imbangela ezimeni ezingazodwana kunzima ngokwendlela yokusebenza. Mayelana ne-Long COVID ngokuqondile, ukubandakanyeka kwe-mast cell kuwumbono osebenzayo onobufakazi bokuqala, hhayi ukutholwa okuxazululiwe.

Itholakala kaningi kwabesifazane. I-Estrogen ithonya ukuziphatha kwe-mast cell, okuyincazelo eyodwa ehlongozwayo, futhi iziguli eziningi zibika amaphethini ezimpawu zomjikelezo. Ukuthi umehluko ubonisa ukusabalala kwangempela noma umehluko ekuxilongweni nasekudluliseleni akuxazululwa ngokugcwele - i-caveat esebenza eziningana zezimo ezixilongwa kakhulu kwabesifazane.

Izibalo zokuvama okuthembekile azikabi khona. Lesi simo sabonakala ngokusemthethweni kuphela ngo-2007, izindlela zokuxilonga zisaxoxwa, futhi ukusebenza kuyehluka phakathi kwamazwe. Izilinganiso ezishicilelwe ziyahlukahluka kakhulu, futhi ububanzi balobo bubanzi yibona ngokwabo okutholakele. Yiba nokungabaza ngezibalo zokuvama okunembe ngokuzethemba ezivela kunoma yimuphi umthombo.

Cha, futhi ukuzama ukwenza ngokuvamile akuzuzisi. Uhlu olushicilelwe luqala ukucabanga ukuze luhlolwe ngokumelene nerekhodi lakho siqu, hhayi imithetho esebenza ngokufanayo - ukuhlukahluka komuntu ngamunye kukhulu, futhi iziguli eziningi zibekezelela izinto ezivela kulo lonke uhlu. Ngoba izitaki ezicuphayo, ubusha kanye nomthwalo ophelele kuvame ukubaluleka ngaphezu kwanoma yikuphi ukudla okukodwa. Sebenzela emikhawulweni emincane efinyelela ukulawula.

Umlando wezimpawu ohlelwa yisistimu yesitho kunokulandelana kwezikhathi, okwenza iphethini yesistimu eningi ibonakale ngokushesha. Idayari yezimpawu kanye ne-trigger ehlanganisa okungenani amasonto amabili. Yonke imiphumela yokuhlolwa yangaphambilini, kuhlanganise nevamile, njengoba lena iyingxenye yenqubo yokukhishwa. Uhlu lwamanje lwemithi kanye nesengezo. Futhi uhlu olufushane olubhaliwe lwemibuzo yakho - ama-aphoyintimenti mafushane futhi kulula ukuhamba ngaphandle kokubuza ukuthi yini ebaluleke kakhulu.

Isigaba 11

Ukufunda Okwengeziwe Izinsiza

Amaphuzu okuqala akhethiwe ukuze afundwe ngokujulile. Izixhumanisi ezithile ziyahlanganiswa futhi zizokwengezwa lapha.

Abaqondisi Besiguli

Izingeniso ezifinyelelekayo ezibhalelwe iziguli nemindeni, ezilungele ukwabelana nabantu abasha kulesi simo.

  • Umhlahlandlela wesiguli oyisingeniso - isixhumanisi esizongezwa
  • Iphakethe le-orientation elisanda kutholwa - isixhumanisi esizongezwa
  • Uphawu oluphrintekayo kanye nesifanekiso sedayari - isixhumanisi esizongezwa
  • Umhlahlandlela wesiguli oyisingeniso - isixhumanisi esizongezwa

Iziqondiso Zokwelashwa

Izitatimende zokuvumelana neziqondiso zomtholampilo ezihloselwe ochwepheshe bezokunakekelwa kwempilo. Kuyasiza ukuletha kuma-aphoyintimenti.

  • Umhlahlandlela wamalungu omndeni nabanakekeli — isixhumanisi esizongezwa
  • Isiqondiso somtholampilo somphakathi ochwepheshe - isixhumanisi esizongezwa
  • Ukuhlolwa komlamuli kanye nesampula yokuphatha iphrothokholi - isixhumanisi esizongezwa
  • I-Consensus diagnostic criteria - ingcaphuno nesixhumanisi okufanele kwengezwe

Amaphepha Ocwaningo

Izincwadi eziyisisekelo. Lapho kungenzeka khona, ncamela imithombo ebuyekezwe ngontanga kanye nezinsuku zokushicilelwa kwenothi, njengoba le nkambu ihamba.

  • Amaphepha okulinganisa okuyisisekelo (2007 kuya phambili) - izingcaphuno ezizokwengezwa
  • Amaphepha enqubo yokuvumelana (2010, 2012) - izingcaphuno ezizokwengezwa
  • Ucwaningo lwe-alpha tryptasemia - izingcaphuno ezizokwengezwa
  • Ukubuyekezwa kwezimo ezihambisanayo - izingcaphuno ezizokwengezwa

Izinhlangano Zochwepheshe

Imiphakathi yochwepheshe kanye nezinhlangano zezemfundo ezisebenza ngesifo se-mast cell.

  • Ukubuyekezwa kwezimo ezihambisanayo - izingcaphuno ezizokwengezwa
  • Amanethiwekhi ocwaningo lwesifo se-Mast cell - izixhumanisi ezizongezwa
  • Izinhlangano zezambulela zezifo ezingavamile - izixhumanisi ezizongezwa

Izincwadi

Ukufundwa kwefomu elide kweziguli nabelaphi.

  • Izinhlangano zezambulela zezifo ezingavamile - izixhumanisi ezizongezwa
  • Imibhalo eyinkomba yomtholampilo — ezongezwa

Ukufundwa kwefomu elide kweziguli nabelaphi.

Izinkulumo ezirekhodiwe, izichazi, nezinto zenkomfa.

  • Imibhalo eyinkomba yomtholampilo — ezongezwa
  • Izinkulumo zengqungquthela eziyisipesheli - izixhumanisi ezizongezwa
  • Imininingwane yokuqukethwe kwezemfundo kwe-MCAS - izixhumanisi ezizongezwa

Izinhlangano Ezisekelayo

Ukwesekwa kontanga, ukumela, nomphakathi.

  • Imininingwane yokuqukethwe kwezemfundo kwe-MCAS - izixhumanisi ezizongezwa
  • Imiphakathi yokusekela ontanga - izixhumanisi ezizongezwa
  • Amaqembu asekelayo ezifunda kanye nakazwelonke - izixhumanisi ezizongezwa

Izilingo Zomtholampilo

Izifundo eziqashwayo njengamanje. Ukutholakala kwesilingo kushintsha njalo, ngakho hlola uhlu lokubhalisa ngokuqondile.

  • Amaqembu asekelayo ezifunda kanye nakazwelonke - izixhumanisi ezizongezwa
  • Okwamanje kuqashwa izifundo ze-mast cell - izixhumanisi ezizongezwa
  • Isiqondiso mayelana nokuthi ukuhlanganyela kocwaningo kuhilelani— isixhumanisi esizongezwa
Isigaba 12

Njengamanje kuqashwa ama-mast cell studies - izixhumanisi ezizongezwa

Imiqondo ebaluleke kakhulu evela kulo mhlahlandlela, ifingqiwe.

Ngamafuphi
  • I-MCAS wukuphazamiseka kwe-mast cell engafanele ukwenza kusebenze, hhayi izinombolo ze-mast cell - amaseli ajwayelekile, umkhawulo wabo awukho.
  • Imiqondo ebaluleke kakhulu evela kulo mhlahlandlela, ifingqiwe.
  • Ngamafuphi
  • Abalamuli bakhululwa ngamagagasi: bakheke ngaphambili ngemizuzwana, abalamuli be-lipid ngamaminithi, ama-cytokines ngaphezu kwamahora. Umcimbi owodwa wokwenza kusebenze ungadonsa usuku.
  • Amaseli e-Mast angamaseli omzimba awusizo ngokusemthethweni abekwe emigoqweni yomzimba, abambe abalamuli enziwe ngaphambilini balungele ukukhululwa phakathi nemizuzwana.
  • Izimpawu ziyi-episodic, ziyaphindaphindeka, futhi zibandakanya amasistimu wezitho ezimbili noma ngaphezulu - lelo phethini, hhayi noma yiluphi uphawu olulodwa, yilo eliphakamisa ukuxilongwa.
  • Abalamuli bakhululwa ngamagagasi: bakheke ngaphambili ngemizuzwana, abalamuli be-lipid ngamaminithi, ama-cytokines ngaphezu kwamahora. Umcimbi owodwa wokwenza kusebenze ungadonsa usuku.
  • I-Histamine ingumlamuleli oyedwa kuphela phakathi kwabaningi, yingakho ama-antihistamine ngokuvamile enikeza impumuzo ngokwengxenye esikhundleni sokukhululeka okuphelele.
  • Icupha isitaki. Umthwalo oqoqiwe uchaza ukusabela okubukeka kungahleliwe, futhi kuwumbono owodwa owusizo kakhulu wokuphatha kwansuku zonke.
  • Izinto ezibangela umzimba - ukushisa, amakhaza, ingcindezi, ukuzikhandla - azidingi into engenisiwe futhi azibonakali ekuhlolweni okujwayelekile kokungezwani komzimba.
  • Ukubandakanyeka kwe-Mast cell emisiwe ku-urticaria engapheli kanye ne-asthma; izinhlangano ne-POTS, i-hEDS, i-IBS kanye ne-Long COVID zibikwa kodwa azixazululwanga.
  • Ukuhlolwa okuningi komlamuli okungalungile kubonisa izinkinga zesikhathi kanye nesampula yokuphatha esikhundleni sokungabikho kwesifo.
  • Indlela yokuxilonga isalokhu kuphikiswana ngayo, ngakho ochwepheshe abanekhono bangafinyelela iziphetho ezihlukene ngesiguli esifanayo.
  • Lapho ubungozi be-anaphylaxis busebenza, i-epinephrine kanye nohlelo lwesenzo olubhaliwe kubalulekile; ama-antihistamine awangeni esikhundleni.
  • Ayikho imbangela ye-MCAS esunguliwe - phatha 'izimbangela eziyinhloko' ngokuzethemba ngokungabaza.
Isigaba 13

Uhlu lwamagama

Amagama angama-91 asetshenziswa ezincwadini ze-MCAS nengxoxo yomtholampilo, achazwe ngokucacile.

A
I-Adrenaline
Bheka i-Epinephrine. Igama laseBrithani nelamazwe ngamazwe okungelona elobunikazi behomoni efanayo nemithi.
Ikhanda elikhanyayo noma ukuquleka, ngokuvamile lapho umile
A
Ama-welts aphakanyisiwe, alumayo; ingase ibe yesikhashana futhi ifuduke
I-Adrenaline
Bheka i-Epinephrine. Igama laseBrithani nelamazwe ngamazwe okungelona elobunikazi behomoni efanayo nemithi.
Umuthi ovimba ama-histamine receptors. Ama-H1 blockers asebenza ikakhulukazi esikhumbeni nasezimpawu zomoya; Ama-H2 blockers asebenza ikakhulukazi emgudwini wamathumbu.
Ukuvuvukala kwezingqimba ezijulile zesikhumba noma ulwelwesi lwamafinyila, okuvamise ukuthinta izindebe, amajwabu amehlo, izandla, noma umphimbo. Ukubandakanyeka komphimbo kuyisimo esiphuthumayo sezokwelapha.
I-Antihistamine
B
Isibalo samaseli
I-Serum tryptase ilinganiswa lapho izimpawu zithulile. Ihlinzeka ngephuzu lokuqhathanisa okwahlulelwa ngalo ukwenyuka okukhulu.
Ingxenye yesistimu yezinzwa elawula imisebenzi ezenzakalelayo njengokushaya kwenhliziyo, umfutho wegazi, nokugaya ukudla. Ukungasebenzi kwayo kubizwa nge-dysautonomia.
B
I-serum tryptase ilinganiswa lapho izimpawu zithulile. Ihlinzeka ngephuzu lokuqhathanisa okwahlulelwa ngalo ukwenyuka okukhulu.
I-Basophil
Iseli elimhlophe legazi elizungezayo elabelana ngezici ezithile namaseli e-mast, okuhlanganisa nezinhlayiyana ze-histamine, kodwa liyahluka ngendawo nomjikelezo wokuphila.
I-Biomarker
Inkomba yebhayoloji elinganisekayo esetshenziselwa ukuthola noma ukuqapha isimo. I-Tryptase iyi-biomarker esungulwe kakhulu ku-mast cell disease.
I-Carboxypeptidase A3
Ukuncipha kwemigudu yokuphefumula ngenxa yokuqina kwemisipha ebushelelezi ezungezile, okukhiqiza ukuhefuzela kanye nokuphefumula. Ama-leukotrienes angabashayeli abanamandla.
C
Iphrotheni esayinayo ebuthela amanye amaseli omzimba kusayithi, akhulise futhi andise impendulo yokuvuvukala.
I-protease egcinwe kuma-mast cell granules, ebandakanyeka ekwakhiweni kabusha kwezicubu kanye nasekuqhekekeni kwama-peptide athile nobuthi.
I-Chemokine
Iphrotheni esayinayo ebuthela amanye amaseli omzimba kusayithi, akhulise futhi andise impendulo yokuvuvukala.
Chymase
I-protease etholakala kuma-mast cell granules abamba iqhaza ekwenziweni kabusha kwezicubu kanye nasezindleleni zokulawula umfutho wegazi.
Inqwaba yamaphrotheni egazi enza ingxenye yokugoma okungokwemvelo. Izingxenye zayo i-C3a ne-C5a zingenza amaseli e-mast asebenze ngokuqondile.
I-Corticotropin-releasing hormone (CRH)
Isistimu yokugcwalisa
I-Cromolyn sodium
I-mast cell stabilizer enciphisa ukukhululwa komlamuli kunokuvimbela imiphumela yomlamuli. Ukwakheka komlomo kusebenza ikakhulukazi emgudwini wamathumbu.
Ihomoni yokucindezeleka lapho ama-mast cell athwala ama-receptors, ehlinzeka ngomzila obhaliwe lapho i-stress physiology ithonya ukusebenza kwe-mast cell.
I-Cromolyn sodium
I-mast cell stabilizer enciphisa ukukhululwa komlamuli kunokuvimbela imiphumela yomlamuli. Ukwakheka komlomo kusebenza ikakhulukazi emgudwini wamathumbu.
Isikhumba
Okuhlobene nesikhumba.
Ukwenza kusebenze
I-Cytokine
Kungenzeka noma ngaphandle kokuqhuma okubonakalayo
Iphrotheni ekhombisayo eshayela ukuvuvukala nokuxhumana kwamasosha omzimba. Ama-Cytokines asebenza kancane futhi aphikelela isikhathi eside kunabalamuli abakhelwe ngaphambilini.
D
Inqubo lapho ama-granules esitoreji se-mast cell ahlangana ngayo nolwelwesi lweseli futhi akhiphe okuqukethwe kwawo kuzicubu ezizungezile, ngokuvamile phakathi kwamasekhondi.
I-welt ephakanyisiwe ekhiqizwa ukuphulula okuqinile kwesikhumba. Uphawu olubonakalayo olubonakalayo oluhlotshaniswa nokusebenza kabusha kwe-mast cell.
I-Diamine oxidase (DAO)
I-enzyme ephula i-histamine engenisiwe. Umsebenzi owehlisiwe uyindlela evame ukuphakanyiswa yokungabekezelelani kwe-histamine.
Ukukhishwa okuhlukile
Ukukhishwa okukhethiwe kwabanye abalamuli ngaphandle kokuncipha okugcwele. Ebizwa nangokuthi i-piecemeal degranulation; isiza ukuchaza amaphethini ezimpawu ahlukahlukene.
I-Dysautonomia
Ukungasebenzi kahle kwesistimu yezinzwa ezizimele, okukhiqiza izimpawu ezinjengokushaya kwenhliziyo nokungaqini komfutho wegazi. I-POTS iyifomu elilodwa.
E
Indlela ehlelekile yokususa ukudla okusolwa ukuthi yi-trigger futhi iphinde iphinde iphinde ifakwe ngendlela ehlelekile. Ithwala ingozi yokondleka komzimba uma inde ngaphandle kokugadwa.
Iqembu lezinkinga zezicubu ezixhumene ezifuywayo. Uhlobo lwe-hypermobile luvame ukubikwa eduze kwe-MCAS ne-POTS.
Ukuqeda ukudla
Abahlanganyeli bezemvelo abahlongozwayo bahlanganisa ukuchayeka kwamakhemikhali, isikhunta, ukungcoliswa komoya, kanye nezinguquko zokudla ezingeni labantu. Ubufakazi lapha bubuthakathaka kakhulu kunobezindawo ezingenhla, futhi obuningi babo abuqondile. Lena indawo ezwakalayo yophenyo esikhundleni sokuba negalelo elisunguliwe, futhi kuyindawo lapho izimangalo zezohwebo ezizethembayo zivamile futhi zisekelwa kabi.
Indlela ehlelekile yokususa ukudla okusolwa ukuthi yi-trigger futhi iphinde iphinde iphinde ifakwe ngendlela ehlelekile. Ithwala ingozi yokondleka komzimba uma inde ngaphandle kokugadwa.
I-Eosinophil
Ingqamuzana elimhlophe legazi elibandakanyeka ekuphenduleni kokungezwani komzimba kanye nezimuncagazi. Ihlukile kuma-mast cells kodwa iyasebenza ezimeni ezigqagqene.
Izinguquko ekukhulumeni kofuzo ezingaguquli ukulandelana kwe-DNA engaphansi. Kuphakanyiswe njengendlela engenzeka ku-MCAS, enobufakazi obuqondile obunomkhawulo.
I-Epinephrine
Ukwelashwa komugqa wokuqala we-anaphylaxis, ngokuvamile okulethwa ngomjovo ozenzakalelayo. Ama-Antihistamine awayona indawo yokusabela okunamandla.
I-Erythema
Ukubomvu kwesikhumba okubangelwa ukwanda kwegazi, njengoba kwenzeka ngesikhathi sokushaywa.
F
I-FceRI
Isikhumba kanye ne-mucosal
I-high-affinity IgE receptor endaweni ye-mast cell. Ukuxhumanisa i-IgE eboshwe yi-allergen kubangela ukuqaliswa kwe-allergic yakudala.
Ukuvutha
Inkathi yezimpawu eziba zimbi kakhulu, ezingase zilandele inhlamvu ekhonjwayo noma ziphakame ngaphandle kwayo. Ubude besikhathi buhluka kusuka emahoreni kuya emavikini.
Ukuba bomvu nokufudumala okungazelelwe, ngokuvamile kobuso, intamo, nesifuba. Enye yezimpawu ze-mast cell.
G
I-H1 receptor
I-subtype ye-histamine receptor egxile esikhunjeni, emigudwini yomoya, nasemithanjeni yegazi. Ithagethi ye-H1 antihistamines.
I-H2 receptor
I-subtype ye-histamine receptor egxile esiswini kanye nezicubu zenhliziyo. Ithagethi ye-H2 blockers, esiza izimpawu zesisu.
I-Hematopoietic progenitor
Iseli lomnkantsha wethambo elingavuthiwe elikwazi ukuthuthuka libe izinhlobo zamangqamuzana egazi. Ama-mast cells avela kubazali be-CD34-positive.
I-Heparin
I-anticoagulant eyenzeka ngokwemvelo egcinwe kuma-mast cell granules. Kungase kubangele imihuzuko noma ukopha okungajwayelekile kwezinye iziguli.
I-Hereditary alpha tryptasemia (HaT)
Isici sofuzo esibandakanya amakhophi engeziwe wofuzo lwe-TPSAB1, okhiqiza i-tryptase yesisekelo eqhubekayo eqhubekayo. Kuzuzwe njengefa kuphethini ebusayo ye-autosomal.
I-Heparin
I-anticoagulant eyenzeka ngokwemvelo egcinwe kuma-mast cell granules. Kungase kubangele imihuzuko noma ukopha okungajwayelekile kwezinye iziguli.
Ukungabekezelelani kwe-Histamine
I-Histamine
Umlamuli we-mast cell owaziwa kakhulu. Ivula imithambo yegazi, ikhulise ukungena kwayo, ivuse imizwa, futhi ikhuphule i-asidi yesisu, ikhiqize ukuguquguquka, ukulunywa, ukuqunjelwa, nokuqunjelwa.
Ukungabekezelelani kwe-Histamine
Ubunzima bokwephula i-histamine engenisiwe, ngokuvamile okubangelwa umsebenzi owehlisiwe we-diamine oxidase. Ihlukile ku-MCAS, noma izimpawu ziyeqana.
Izidleke
Bheka i-Urticaria. Ama-welts aphakanyisiwe, alumayo esikhumbeni, ngokuvamile adlulayo futhi afudukayo.
I-Hypotension
Umfutho wegazi ophansi ngokungavamile. Kungenzeka ngesikhathi sokusabela okunzima kwe-mast cell futhi kuyisici se-anaphylaxis.
I
IgE
I-Immunoglobulin E, isigaba se-antibody esimaphakathi ne-classical allergy. Ibopha i-FcERI kumast cell; ukusabela okuningi kwe-MCAS ku-IgE-independent.
Immunoglobulin
I-antibody - iphrotheni ekhiqizwa amasosha omzimba abopha izinhloso ezithile. I-IgE yikilasi elifaneleka kakhulu ekusebenzeni kwe-mast cell.
Ukuvuvukala
Ukusabela kwamasosha omzimba ekulimaleni noma ekusongelwa, okubandakanya ukugeleza kwegazi okwandisiwe, ukubuthwa kwamangqamuzana omzimba omzimba, kanye noshintsho lwezicubu. Amaseli e-Mast aqala futhi aphendule kuwo.
Isigaba se-cytokine esisetshenziselwa ukuxhumana phakathi kwamaseli omzimba. I-IL-6 ne-IL-4 ziphakathi kwalezo ezikhishwe ama-mast cells.
Ingalo esheshayo, engaqondile yamasosha omzimba ikhona kusukela ekuzalweni. Amaseli e-Mast ayingxenye.
I-Interleukin
Umuthi onazo zombili izakhiwo ze-antihistamine kanye ne-mast cell stabilizing. Ngokuvamile kudinga amaviki okusetshenziswa okungashintshi ngaphambi komphumela ogcwele.
K
I-Ketotifen
I-KIT D816V
KIT
I-receptor endaweni ye-mast cell ehlanganisa i-stem cell factor. Okubalulekile ekuthuthukisweni kwe-mast cell, ukusinda, nokuhlala kwezicubu.
I-Leukotriene
Uguquko oluthile kufuzo lwe-KIT olutholakala kumastocytosis amaningi esistimu yabantu abadala. Akuyona imbangela eqinisekisiwe ye-MCAS.
I-Leukotriene E4 (LTE4)
I-Leukotriene
Umxhumanisi we-lipid owenziwe ngemuva kokusebenza. Ama-bronchoconstrictors anamandla abuye akhulise ukuvuthwa kwemithambo kanye nokukhiqizwa kwamafinyila.
Umlamuli owakhiwe ngama-lipids e-membrane yeseli ngemva kokuqaliswa kusebenze esikhundleni sokugcinwa kusenesikhathi. I-Prostaglandin kanye ne-leukotrienes yizibonelo eziyinhloko.
I-leukotriene metabolite engalinganiswa emchamweni, esetshenziswa njengengxenye yokuhlolwa komlamuleli.
Umlamuli we-lipid
Umlamuli owakhiwe ngama-lipids e-membrane yeseli ngemva kokuqaliswa kusebenze esikhundleni sokugcinwa kusenesikhathi. I-Prostaglandin kanye ne-leukotrienes yizibonelo eziyinhloko.
M
Iseli ye-Mast
I-MCAS ayiyona i-mastocytosis
Isimo esibandakanya ukunqwabelana kwamaseli e-mast ngokweqile esicutshini, ngokuvamile eqhutshwa ukuguqulwa kwe-KIT D816V kusifo sesistimu yabantu abadala. Ihlukile ku-MCAS.
Abalamuli abakhulu nokuthi ngamunye wenzani
I-stabilizer ye-Mast cell
Umuthi owehlisa ukukhululwa kwe-mast cell mediator kunokuvimbela imiphumela yomlamuli. I-Cromolyn ne-ketotifen yizibonelo ezivamile.
Ulwelwesi oluzungeze ubuchopho nomgogodla. Ama-mast cell akhona lapha, abalulekile ocwaningweni ngezimpawu zemizwa.
Noma iyiphi ikhemikhali ekhishwa yi-mast cell ekhiqiza imiphumela ezicutshini ezizungezile. Kuhlanganisa i-histamine, i-tryptase, i-prostaglandin, nabanye abaningi.
I-Menings
I-MRGPRX2
I-Montelukast
Umphikisi we-leukotriene receptor osetshenziselwa ukubhekana nezimpawu zokuphefumula nezamathumbu ezingafinyeleli ama-antihistamine.
Izicubu ezimanzi ezihlanganisa imigudu yangaphakathi njengamathumbu, imigudu yomoya, nekhala. Kugcwele abantu abaningi ngamaseli e-mast.
I-mast cell receptor mediating ukusebenza okuzimele kwe-IgE ngezidakamizwa ezithile nama-peptide. Isiza ukuchaza ukusabela ngaphandle kokubandakanyeka kwe-IgE okubonakala.
Mucosa
I-histamine metabolite elinganiswa emaqoqweni womchamo wamahora angu-24 njengobufakazi bokukhululwa komlamuli weseli enkulukazi.
N
I-N-methylhistamine
O
I-Neuroimmune
Okuhlobene nokusebenzisana phakathi kwamasistimu wezinzwa kanye namasosha omzimba. Ukusebenzisana kwe-mast cell-nerve kuyisisekelo sengcindezi esebenza njengesiqalo sokuphila.
O
Omalizumab
Ukwelashwa kwe-biologic okuqondiswe ku-IgE, okusetshenziswa kwezinye izimo eziphikisayo ngaphansi kokugadwa kochwepheshe.
P
Ukushaya kwenhliziyo
Ukuqaphela ukushaya kwenhliziyo, okuvame ukuchazwa njengokugijima, ukushaya ngamandla, noma okungajwayelekile.
I-Perivascular
I-Platelet activating factor (PAF)
Umxhumanisi we-lipid onamandla kakhulu okhuphula ukungeneka kwemithambo futhi ungabangela i-hypotension enzima. Ayivinjelwe ama-antihistamine.
I-Hereditary alpha tryptasemia
Bona ukukhishwa Okuhlukile. Ukukhishwa komlamuli okhethiwe ngaphandle kokukhishwa kwesamba sokuqukethwe kwe-granule.
I-Platelet activating factor (PAF)
Umxhumanisi we-lipid onamandla kakhulu okhuphula ukungeneka kwemithambo futhi ungabangela i-hypotension enzima. Ayivinjelwe ama-antihistamine.
I-Prostaglandin D2
I-Presyncope
Umuzwa wokuthi usuzoquleka, ngaphandle kokulahlekelwa umqondo.
Igama lezokwelapha lokulunywa.
Umxhumanisi we-lipid obangela i-vasodilation ephawulekayo, ukushaywa kwamanzi, i-bronchoconstriction, kanye nesifo sohudo. Okuvame ukugqama lapho ukushaywa kubusa khona.
I-Pruritus
Ukungaphenduli ngokwanele ekwelashweni okujwayelekile. Amacala aphikisayo angase abangele ukucatshangelwa kwama-ejenti akhethekile.
R
I-Refractory
Ukungaphenduli ngokwanele ekwelashweni okujwayelekile. Amacala aphikisayo angase abangele ukucatshangelwa kwama-ejenti akhethekile.
Regranulation
Inqubo lapho iseli ye-mast lakha kabusha izitolo zayo zezinhlayiya ngemva kokuwohloka, ukuyivumela ukuthi iphendule futhi kunokuba ife.
I-Rhinitis
Ukuvuvukala kolwelwesi lwamakhaleni okukhiqiza ukuminyana, ikhala eligelezayo, nokuthimula. Imvamisa okungapheli futhi okungeyona eyesizini ku-MCAS.
S
I-serum tryptase
I-Tryptase ilinganiswa egazini. Umaka waselabhorethri osungulwe kakhulu wokwenza kusebenze iseli ye-mast, odinga womabili amasampuli ashubile nayisisekelo.
I-Stem cell factor (SCF)
I-molecule esayinayo ehlanganisa i-KIT, ishayela ukuthuthukiswa kwe-mast cell, ukusinda, nokuhlala kwezicubu.
I-syncope
Ukuquleka - ukulahlekelwa ukwazi okwesikhashana, ngokuvamile kusukela ekuncipheni kwegazi ukuya ebuchosheni.
Uhlobo lwe-mastocytosis lapho amaseli e-mast engeziwe anqwabelana khona ezithweni zangaphakathi, ngokuvamile ngokuguqulwa kwe-KIT D816V.
Ukuthinta umzimba wonke esikhundleni sendawo eyodwa yendawo.
Ukuzwela amakha namakhemikhali asemoyeni
I-mastocytosis yesistimu
Umkhawulo
T
Izinga lokushaya kwenhliziyo elisheshayo ngokungavamile, elivame ukubikwa ngesikhathi sokusabela kwe-mast cell.
Umkhawulo
I-TPSAB1
I-toll-like receptor
Qalisa
I-receptor eqaphela amaphethini ahlotshaniswa namagciwane, axhumanisa ukusebenza kwe-mast cell nokutheleleka.
I-Tryptase
I-gene efaka ikhodi ye-alpha-tryptase. Amakhophi engeziwe akhiqiza i-alpha tryptasemia yofuzo kanye ne-tryptase eyisisekelo ephakanyisiwe.
Noma isiphi isisusa esivusa ama-mast cell. Kungaba ngamakhemikhali, ngokomzimba, amahomoni, okuthathelwanayo, noma okungokomzwelo; kuvame ukunqwabelana esikhundleni sobunye.
I-Tryptase
Iphrotheni eningi kakhulu kuma-mast cell granules kanye nomaka osungulwe kakhulu wokwenza kusebenze, yenaniselwa ukucaciswa ngokuqhathaniswa ne-mast cell-specific.
U
Urticaria
Ama-Hives - aphakanyisiwe, ama-welts alumayo abangelwa ukukhululwa komlamuli esikhumbeni. I-urticaria ezenzakalelayo ehlala njalo ihilela ukusebenza kwe-mast cell ngqo.
I-Urticaria pigmentosa
Ukuba nomthelela kububanzi bomkhumbi wegazi noma ekungeneni. I-histamine ne-prostaglandin D2 zisebenza ngokunamandla.
V
I-Vasoactive
W
I-Vasodilation
Ukunwetshwa kwemithambo yegazi, ukwandisa ukugeleza kwegazi nokwehlisa umfutho wegazi. Ikhiqiza ukuguquguquka futhi ingabangela ukukhanya kwekhanda.

Awuyena Ngedwa

Imininingwane ye-MCAS ikhona ukuze kuqinisekiswe ukuthi sonke isiguli siyakwazi ukuthola ukwesekwa, umphakathi, kanye nolwazi lokuzikhulumela. Joyina inethiwekhi yethu yomhlaba wonke.

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