MCAS Dzidzo gwara. Kuti titsigirwe nevanhu vanogara nemamiriro akafanana, edu nyaya dzevarwere inzvimbo yakanaka yekutanga.">
Gwaro rakazara kuMCAS - mast cell biology, zviratidzo nehurongwa hwemuviri, zvinokonzeresa, kuongororwa, mamiriro anoenderana, manejimendi ezuva nezuva, 25 FAQs uye 91-temu glossary.

Kunzwisisa MCAS | Complete Guide

Mast cell biology, zviratidzo, zvinokonzeresa, kuongororwa, manejimendi, makumi maviri neshanu FAQs uye 91-temu glossary.

Gwaro Rakazara Redzidzo
Kunzwisisa Mast Cell
Activation Syndrome
(MCAS)
Dzidza sainzi kuseri kwemaseru maseru, nei vachinyanya kushanda, kuti MCAS inobata sei muviri, uye ruzivo rwekutanga murwere wese, mutarisiri, mudzidzi, uye nyanzvi yehutano inofanirwa kunzwisisa.
Nguva yekuverenga55 min
LevelNhanganyaya → Pakati
Yakapedzisira kuongororwaChikunguru 2026
Zvikamu13
Medical Disclaimer: Gwaro iri rinodzidzisa uye harisi zano rekurapa, kuongororwa, kana kurapwa. MCAS inopa zvakasiyana mumurwere wese, uye tsvagiridzo iri kubuda. Gara uchishanda nevarapi vanokwanisa vehutano vanoziva nhoroondo yako usati waita chero chinhu chaunoverenga pano. Kana urikusangana nedambudziko rakakura, tsvaga rubatsiro rwechimbichimbi nekukasika.

Zviri Mukati

01Chii chinonzi MCAS?02Kunzwisisa Mast Cells03Chii Chinoitika Panguva Mast Cell Activation04Zviratidzo zveMCAS05Common Triggers06Nei MCAS Yakaoma Kuongorora07Associated Conditions08Kunzwisisa Kwezvino Kwezvikonzero09Kugara neMCAS10Mibvunzo Inowanzo bvunzwa11Zvimwe Zvishandiso Zvekudzidza12Key Takeaways13Tsanangudzo yemashoko

Chikamu 01

Chii MCAS?

Mast Cell Activation Syndrome chirwere chekudzivirira muviri maitiro pane kudzivirira quantity. Iwo maseru aripo muhuwandu hwakajairwa uye anotaridzika zvakajairwa - chachinja ndechekuti vanopisa sei.

Mast Cell Activation Syndrome (MCAS) inzvimbo umo maseru emasero - maseru edziviriro anogara munharaunda yese yemuviri - anoshanda zvisina kufanira uye kakawanda, achiburitsa makemikari anoyananisa muhuwandu uye dzimwe nguva asingashande chinangwa chekudzivirira. Mhedzisiro iyi patani yekudzokororwa, episodic zviratidzo zvinobata maviri kana anopfuura enhengo masisitimu kamwechete.

Tsanangudzo yacho ine basa nekuti inoisa MCAS parutivi kubva pamamiriro ayo akafanana nepamusoro. Iko hakuna kuunganidzwa kusiri kujairika kwemaseru kuti awane pabiopsy, hapana imwe allergen yekuziva paganda-kubaya bvunzo, uye kazhinji hapana abnormality zvachose pabasa renguva yerabhoritari. Chirwere ichi chinogara pachikumbaridzo apo masero anotaridzika anosarudza kupindura.

Pfungwa Huru

MuMCAS dambudziko harisi zvingani mast cells aunawo. Zviri vanopfura zviri nyore sei. Chese chimwe chimiro chechimiro - kusafungidzirika, kupararira kwemultisystem, kuyedzwa kwakaoma - kunotevera kubva kune icho chimwe chokwadi.

Why mast cells aripo zvachose

Mast masero haasi dhizaini kukanganisa. Izvo zviri pakati pezvinhu zvekare zvekare zvemukati memuviri immune system, zviripo mune imwe fomu kune akawanda vertebrates, uye ivo vanoita basa iro muviri rinoda chaizvo.Vanoisa mhinduro yekutanga kune maparasite, vanobatsira kuderedza huturu mushure mekurumwa uye kurumwa, kutora chikamu mukuporeswa kwemaronda uye kugadzirisa matishu, vanobatsira pakudzorwa kwemidziyo yeropa, uye kubatsira kuchengetedza zvipingamupinyi zvinoparadzanisa muviri kubva kunze kwenyika.

Iwo akamiswa zvinoenderana - akaiswa pamiganhu, muganda, airway lining, gut madziro, uye tishu dzakatenderedza tsinga dzeropa netsinga. Uye ivo vakavakirwa kumhanya: pane kugadzira mhinduro yavo pane zvavanoda, vanoibata isati yagadzirwa mumagranules ekuchengetera, yakagadzirira kuburitsa mukati memasekondi echiratidzo.Iyo dhizaini ndiyo inoita kuti allergic reaction ikurumidze, uye ndizvo zvakare zvinoita kuti activation isina kufanira inetse kugara nayo.

Izvo zvakajairika mast cell function inotaridzika

Mune mhinduro ine hutano hwekudzivirira muviri, sero remast rinosangana netyisidziro yechokwadi - parasite antigen, uturu, kana kukuvara kwetishu - uye degranulate mukuenzana nayo. Mitsipa yeropa inodzika uye inowedzera kupinza, ichibvumira plasma uye masero ekudzivirira mumuviri kupinda munyama. Nhepfenyuro dzetsinga dzinokurudzirwa, dzichigadzira itch kana kurwadziwa kunokwevera kutarisa kunzvimbo. Mamwe masero anodzivirira zvirwere anotorwa. Kana kutyisidzira kwabviswa, mhinduro inogadziriswa.

Zvese zvisingafadzi nezve maitiro aya zvinoshanda. Kuzvimba kunoendesa immune components kune saiti. Kukwenya kunoita kuti ubvise chero chiri kukuvadza. Mucus anoteya uye anodzinga zvinogumbura. Mune mhinduro yakaenzana, kusagadzikana ndekwenguva pfupi uye kubatsirwa ndeyechokwadi.

Chii chinokanganisa muMCAS

MuMCAS, iwo muchina iwoyo anoshanda pasina chakaenzanirana chikonzero. Mast masero anodzima ega, kana kuti achipindura zvinokurudzira izvo zvinofanirwa kunge zvisingashamisi - imba inodziya, hwema mukoridho, chikafu, kusimuka, kurovedza muviri kwakapfava, zuva rakajairwa rekushushikana. Vayananisi vakasunungurwa ndivo vakafanana vanoshandiswa mukudzivirira zviri pamutemo, asi vanosunungurwa mumamiriro ezvinhu asina kunaka uye kazhinji kazhinji.

Nekuti mast masero anogara munyama mumuviri wese, uye nekuti avo vanoyananisira vanoita patsinga dzeropa, mhasuru dzakatsetseka, tsinga, uye mamwe maseru edziviriro, chikamu chimwe chete chinogona kuburitsa kuputika, moyo wekumhanya, dumbu rekurovera, kufema, uye mhute yekuziva panguva imwe chete.Kuna chiremba asiri kufunga nezve mast cell, musanganiswa iwoyo unogona kutaridzika senge matambudziko akati wandei asina hukama anosvika kamwechete - chinova chikamu chikuru chekuti sei mamiriro acho akapotsa.

Enzaniso iri nyore

Funga nezvemast cells sezvitsigiro zvehutsi zvinoparadzirwa mukamuri rega rega remba. Muchirongwa chekushanda, detector inonzwika kana pane moto, uye alarm - inonzwika uye inovhiringidza sezvairi - ndizvo chaizvo zvaunoda. MuMCAS ma detectors ave akawandisa. Ivo zvino vanotanga pamoto wakatsva, pautsi unobva mushower, masikati anodziya. Maalarm ndeyechokwadi, ane ruzha, uye imba inopindura zvechokwadi kwavari - asi hapana moto.Cherechedza zvinorehwa nekuenzanisa: dambudziko harisi nhamba yezvioni, uye haisi iyo maaramu ari kufungidzira. Ndiwo manzwisisiro ezvikumbaridzo pazvinonzwika.
MCAS haisi mastocytosisUyu ndiwo mutsauko umwechete unonyanya kukosha mumunda, uye kuvhiringa maviri kunotungamirira kunyonganiso pamusoro pezvinhu zvose zviri pasi perwizi.
Mastocytosis yakaenzaniswa neMCASMastocytosisMCAS
Kukanganisika kukuruMasero akawanda akawandisa anoungana munyamaNormal nhamba yemast cells, activate zvisina kufanira
Kuwanda kwemaseroYakasimudzwa - clonal kuwandaNormal
Tryptase yepakutangaKazhinji zvinoramba zvichikwidziridzwaKazhinji zvakajairwa pakutanga
Yakajairika genetic kuwanaKIT D816V shanduko mune dzakawanda dzevakuru systemic kesiHapana imwechete yakagadziriswa causative mutation
Biopsy zvakawanikwaAbnormal mast cell aggregates anoratidzwaHapana hunhu kuunganidza
Chiratidzo chegandaMaculopapular maronda (urticaria pigmentosa) anowanikwa mune mamwe maitiroHapana chaiyo yekuongorora ganda ronda

Kuti zvinosimbiswa sei

Uchapupu hwechinangwa chekuwedzera kwesero

Chiratidzo chechiratidzo + murevereri uchapupu + mhinduro yekurapa

Mamiriro ese ari maviri anogona kuburitsa zviratidzo zvinopindirana, nekuti mune ese maviri mast cell mediators ari kusvika munyama akawandisa. Asi mastocytosis inogona kuratidzwa nekuwana masero; MCAS haigone, izvo chaizvo chikonzero nei kuongororwa kwayo kuri pamusanganiswa wemaitiro kwete bvunzo imwe yekusimbisa.

MCAS haisi yakajairika allergies

A classical allergy ndeyeIgE-mediated reaction kune chaiyo, chinhu chinozivikanwa. Iyo inodzokororwa - kuratidzwa kwakafanana kunoburitsa maitiro akafanana - uye kazhinji inogona kuratidzwa neganda-prick kuongororwa kana chaiyo IgE kuongororwa ropa. Kusawirirana nenzungu kunoita seizvi; ndizvo zvinoita katsi dander allergies.

MCAS maitiro kazhinji haaite. Mazhinji haasi IgE-mediated zvachose, achisimuka pachinzvimbo kuburikidza nemamwe mareceptor pane mast cell sero kana kuburikidza nekukurudzira kwemuviri. Zvinokonzeresa zvinowanzove zvemuviri - kupisa, kutonhora, kudzvanywa, kushingaira - kwete mapuroteni, uye hapana allergy pani yakagadzirirwa kuona izvo.Kuita kunogona kunonoka nemaawa, zvichibvisa chinongedzo pakati pekufumurwa nemhedzisiro, uye chikumbaridzo chinogona kushanduka nekurara, kushushikana, mamiriro ehomoni, kana hurwere huchangobva kuitika, saka kuratidzwa kunobvumirwa vhiki imwe kunomutsa mhinduro inotevera.

Varwere vazhinji vanosvika paMCAS chaizvo nokuti kuongororwa kwe allergy kwakadzoka kwakachena nepo zviratidzo zvavo zvisina. A negative allergy panel haisi humbowo hunopesana nekubatanidzwa kwe mast cell; mumamiriro ezvinhu aya iri pedyo nekutarisirwa.

Zano rinokosha rekurapa

Murwere ane maitiro ekubereka, kuyedza IgE yakaipa, uye zvinokonzeresa senge kupisa kana kudzvanywa ndiyo yakajairika MCAS mharidzo pane murwere ane allergy bvunzo. The negative panel inowanzova chikamu chemufananidzo pane gakava pamusoro payo.
  • Inokurudzirwa yekuona
  • Side-ne-side graphic graphic: mapaneru matatu anoratidza akajairwa mast cell vanhu vachipindura zvakaenzanirana, mastocytosis pani inoratidza akawandisa maseru manhamba, uye MCAS pani inoratidza akajairwa maseru manhamba ane exaggerated muyananisi kusunungurwa. Masero akafanana anoverengerwa mumapanera ekutanga neatatu, akasiyana zvinoshamisa kubuda.
  • Chikamu chechidimbu
  • MCAS idambudziko remast cell isina kufanira activation, kwete nhamba dzemast cell.
  • Mast cells maseru edziviriro anobatsira zviri pamutemo akaiswa pamivharo yemuviri uye akavakwa kuti apindure mukati memasekonzi.
Zviratidzo ndezve episodic, zvinodzokororwa, uye zvinosanganisira maviri kana anopfuura enhengo masisitimu pamwechete.

Mastocytosis inosanganisira kuwanda kwemasero uye inogona kuratidzwa zvakananga; MCAS haigone, ndosaka kuongororwa kuri musanganiswa.

Negative allergy kuyedzwa kwakajairika muMCAS uye hazvibvire.

Chikamu 02

Kunzwisisa Mast Cells

Kutevera kuti sei MCAS ichiita sezvainoita, zvinobatsira kuziva kuti maseru aya anobva kupi, kwaanogara, anogara kwenguva yakareba sei, uye zvaanoteerera.

Origin uye kukura

Mast cells anotangira mumwongo, zvichibva kuCD34-positive haematopoietic progenitor cells - iwowo huwandu hunopa mamwe masero eropa. Kusiyana nemasero machena eropa akawanda, zvisinei, haapedzi kukura kwawo ikoko. Vasati vakura vanopinda muropa, votenderera kwenguva pfupi, vobva vatamira munyama, uko kunoitika pakupedzisira.

Iko kukura kunoenderana zvakanyanya ne stem cell factor (SCF) kusaina kuburikidza neKIT receptor pane mast cell surface. Iyi nzira iri pakati pemast cell biology, uye ndiyo nzira imwechete yakakanganiswa ne KIT D816V shanduko inowanikwa mune dzakawanda dzevakuru zviitiko zve systemic mastocytosis. Iyo yemuno tishu nharaunda inoumba yekupedzisira phenotype, ndosaka maseru mast ari mudumbu achisiyana kuyerwa kubva kune ari paganda.

Imwe mhedzisiro inofanirwa kusimbiswa: nekuti mast masero anokura munyama kwete muropa, kuverenga kweropa kwakajairwa hakukuudze chero chinhu nezvazvo. Mature mast cells haawanzo kuwanikwa achitenderera muhuwandu hwakakosha. Ichi ndicho chimwe chikonzero basa rakajairwa reropa muMCAS rinowanzo verenga seisingashamisi.
Kugovera muviri woseMast cells anogovaniswa zvine hungwaru kwete zvakafanana, achitarisa uko muviri unosangana nekunze uye nekutenderedza zvimiro zvinotakura zviratidzo neropa. Uku kugovera kunotsanangura iyo multisystem hunhu hweMCAS zvakananga kupfuura chero chimwe chokwadi chimwe.Panogara mast masero, uye izvo zvinoburitsa kana vakanganisa moto
NzvimboBasa muhutanoZviratidzo kana activation isina kufanira
Ganda uye dermisBarrier defense; mhinduro pakurumwa, kurumwa, nekukuvaraKuputika, kuputika, mikoko, dermographism, kuzvimba
Gastrointestinal turakitiKugadziriswa kwezvipingamupinyi, motility regulation, kupindura kune kutyisidzira kwakapinzwaKupwanya, kurutsa, reflux, manyoka, kuvimbiswa, kuputika
Airways uye sinusesKudzivirirwa kwetambo yekufemaCongestion, rhinitis, kuoma kwehuro, kufema, kukosora
Around tsinga dzeropaKudzora kwechikepe toni uye permeabilityKuchinja kweropa, tachycardia, flushing, presyncope
Brain uye meningesIpa perivascularly uye mune meninges; neuroimmune chiratidzoKuziva mhute, kutemwa nemusoro, dzungu (maitiro achiri pasi pekudzidza)
Connective tishuKugadziridza nekugadzirisa matishuMajoini uye tsandanyama kurwadziwa; zvinokwanisika kuenderana nekubatanidza tishu kupindirana

Mwongo

Nzvimbo panobva masero ekutanga

Iko chirwere checlonal chinoongororwa kana mastocytosis ichifungidzirwa

Nyatsocherechedza kupinda muuropi. Mast cells aripo zvechokwadi mumameninges uye munzvimbo dzeperivascular mukati me central nervous system, uye mast cell-nerve interaction inzvimbo yetsvakurudzo inoshanda. Nekudaro, nzira dzekubatanidza mast cell activation kune chaiyo tsinga uye yekuziva zviratidzo zvichiri kugadziridzwa, uye zvine chivimbo mechanistic zvirevo munzvimbo ino zvinopfuura humbowo hwazvino.

Hupenyu kutenderera

Mast cell anogara kwenguva refu zvisingaite. Iko neutrophil inogona kurarama kwemaawa mashoma kusvika kumazuva, masero ematishu anogona kuramba kwemavhiki kusvika kumwedzi, uye humbowo hunoratidza kuti vamwe vanorarama kwenguva yakareba. Ivo zvakare vanokwanisa regranulation - mushure mekudonha, sero remast rinogona kuvakazve zvitoro zvaro uye kupindura zvakare, pane kufa mukuita.

  • Izvi zvinokosha kliniki nenzira mbiri. Zvinoreva kuti zviratidzo zvekutyaira kwesero zvakagadzikana pane kukurumidza kutendeuka, saka hapana kukurumidza kugadzirisa kwechisikigo kumirira. Uye zvinoreva marapirwo anodzikamisa maseru mast anofanirwa kutorwa nguva dzose nekufamba kwenguva - uri kushandura hunhu hwevagari vemo, kwete kudzima imwe yenguva pfupi.
  • Surface receptors: izvo mast cell inoteerera
  • Masero eMast anotakura huwandu hwakasiyana hwepamusoro receptors, uye hupamhi hweiyo dhizaini ndicho chikonzero chakawanda chakawanda chakaita zvinhu zvinokonzeresa. Zvimwe zvezvinonyanya kuzivikanwa zvinosanganisira:
  • FcεRI - iyo yakakwirira-yakabatana IgE receptor. Iyi ndiyo classical allergic nzira: masoja ekudzivirira chirwere eIgE anosunga receptor, uye kuyambuka-kubatanidza neallergic trigger activation.
  • MRGPRX2 - a receptor mediating IgE-yakasununguka activation nemimwe mishonga uye peptides. Hunhu hwayo hwakabatsira kutsanangura maitiro anoitika pasina inotaridza IgE kubatanidzwa.
  • KIT - receptor ye stem cell factor; pakati pemast cell kupona, maturation, uye kugara kwematishu.
  • Kuzadzisa zvinogamuchirwa (C3a, C5a) - bvumidza activation kuburikidza neruwoko runogonesa rwe innate immunity.
Toll-like receptors - cherechedza maitiro akabatana nehutachiona, kubatanidza mast cells kune mhinduro dzehutachiona.

CRH receptors - pindura kune corticotropin-releasing hormone, ichipa nzira yakanyorwa iyo kushungurudzika kwefiziology kunosvika mast cells.

Varwere vazhinji vanosvika paMCAS chaizvo nokuti kuongororwa kwe allergy kwakadzoka kwakachena nepo zviratidzo zvavo zvisina. A negative allergy panel haisi humbowo hunopesana nekubatanidzwa kwe mast cell; mumamiriro ezvinhu aya iri pedyo nekutarisirwa.

Adenosine, opioid, estrogen uye mamwe receptors - kubatsira kune akasiyana uye ega ega anokonzeresa profiles anoonekwa mukuita.

Murwere ane maitiro ekubereka, kuyedza IgE yakaipa, uye zvinokonzeresa senge kupisa kana kudzvanywa ndiyo yakajairika MCAS mharidzo pane murwere ane allergy bvunzo. The negative panel inowanzova chikamu chemufananidzo pane gakava pamusoro payo.
  • Waizviziva Here?
  • Kuvapo kweakawanda asiri-IgE activation nzira ndosaka akajairwa allergy kuyedzwa kazhinji achidzoka zvakajairika muMCAS. Iwo maedzo anovakwa kuti aone IgE-mediated sensitization - imwe nzira pakati akati wandei inogona kuseta mast cell.
  • Yakanyorwa mast cell illustration: cell membrane yakazara nemazita mareceptor (FcεRI, MRGPRX2, KIT, C3a/C5a, TLRs, CRH-R), yemukati yakazara yakazara nemagranules, ine cutaway inoratidza granule zviri mukati. Inoperekedza inset inoratidza sero isati yasvika uye mushure mekudzikisa.
  • Mast cells anobva kuCD34+ progenitors mumwongo asi akakura munyama, saka kuwanda kweropa hakuaratidzi.
  • Kuratidzira kweKIT/SCF kuri pakati pekukura kwavo - nzira yakakanganiswa mune yakawanda yevakuru systemic mastocytosis.
Ivo vanotarisisa pane zvipingamupinyi uye zvakatenderedza tsinga dzeropa uye tsinga, ndosaka MCAS iri multisystem.

Ivo vanogara kwenguva refu uye vanogona kudzoreredza, saka manejimendi anotsigirwa kwete kwenguva pfupi-kosi.

Mazhinji marudzi ekugamuchira anopfuura IgE anogona kuvamisa, ndosaka allergy mapaneru achiwanzopotsa MCAS zvinokonzeresa.

Chikamu 03
Chii Chinoitika Panguva
Mast Cell Activation

Activation inotevedzana, uye nhanho imwe neimwe ine zita. Kuitevera kunoita kuti zvimwe zvinokatyamadza zvakasiyana-siyana zveMCAS zviratidzo zvive nyore kududzira.

1
Trigger

Kukurudzira kunosvika kuchitokisi - allergen, mushonga, kupisa, kudzvanywa, kushingaira, kushushikana kwehomoni, kutapukirwa, kana kusazivikanwa kwekukurudzira zvachose.

2
Activation

Surface receptors inobata uye intracellular signing cascades inotanga. Calcium influx inhanho yakakosha yekutanga.

3
Degranulation

Magranules ekuchengeta anosanganidzana nesero membrane uye anoburitsa izvo zvakafanogadzirwa mukati mematishu akatenderedza - mukati memasekonzi.

4
Kusunungurwa kwemurevereri

Varevereri vakagadzirwa kare vanosunungurwa pakarepo; vamiriri vachangobva kugadzirwa senge prostaglandins uye leukotrienes vanotevera mukati memaminitsi, uye macytokines kwemaawa.

5

Mast Cell Activation Syndrome chirwere chekudzivirira muviri maitiro pane kudzivirira quantity. Iwo maseru aripo muhuwandu hwakajairwa uye anotaridzika zvakajairwa - chachinja ndechekuti vanopisa sei.

Zviratidzo

Vayananisi vanoita patsinga dzeropa, tsandanyama dzakatsetseka, tsinga, uye mamwe masero edziviriro, achigadzira mhedzisiro mumasystem akawanda enhengo.

Zvinhu zviviri zvekutevedzana uku zvinotsanangura zvakawanda zvinosangana nevarwere. Chekutanga, chikamu chekare chinokwevera pazvitoro zvakafanogadzirwa, ndosaka maitiro achigona kutanga mukati memasekondi. Chechipiri, varevereri vekupedzisira-chikamu vanogadzirwa mushure mekunge activation yatanga, ndosaka zvimwe zviratidzo zvinosvika zvakanaka mushure mekutanga uye nei kuita kungave nekunonoka kwechipiri wave maawa gare gare.

Mast cell anoburitsa vayananisi mukati masaisai, kwete zvose panguva imwe chete. Pre-formed mediators inobuda mumasekondi; lipid mediators inovakwa mumaminitsi; cytokines inounganidza kwemaawa. Chiitiko chekuita chimwe chete chinogona kuburitsa zviratidzo mukati mezuva rose.

Izvo zvakakoshawo kuziva kuti activation haisi yese-kana-hapana. Mast masero anogona kusunungura vamiriri vakasarudzwa pasina kuzara kwakazara - dzimwe nguva kunonzi piecemeal kana musiyano kusunungurwa. Izvi zvinobatsira kutsanangura kuti sei varwere vachiona zviratidzo zvakasiyana-siyana zvakasanganiswa, uye nei kuita kusiri chiitiko chinoshamisa icho izwi rekuti 'activation' rinoratidza.
Varevereri vakuru uye zvinoitwa nemumwe nemumweMast cells anokwanisa kuburitsa huwandu hwakakura kwazvo hwezvinhu. Aya anotevera ndiwo anonyanya kukurukurwa nekiriniki, uye pamwe chete vanoverengera mazhinji echiratidzo chechiratidzo chechiratidzo.Zvinhu zvikuru zvinobudiswa nemast cell
MurevereriRudziMigumisiro mikuru
HistamineRudziPre-formed
Murevereri anonyanyozivikanwa. Inoderedza tsinga dzeropa uye inowedzera kupinza kwayo, inomutsa magumo etsinga, uye inowedzera gastric acid secretion. Inogadzira kuputika, kuputika, mikoko, kuderera kweropa, tachycardia, musoro, uye GI cramping. Inoshanda kuburikidza akati wandei receptor subtypes - H1 muganda uye mhepo, H2 muura uye pamwoyo - ndosaka kuvhara makirasi ese ari maviri kunowanzo shanda zvirinani pane kuvharira chero.TryptaseMapuroteni akanyanya kuwanda mune mast cell granules uye yakanyanya kusimbiswa murabhoritari chiratidzo che activation. Inobatsira pakugadziridza matishu uye kuzvimba, uye inogona kuwedzera mamwe mast cell mhinduro. Kukosha kwayo kwekuongorora kunobva pakuve yakananga kune mast masero.
Prostaglandin D2TryptaseIchangobva synthesized
A lipid murevereri anogadzirwa mushure mekuita activation. Zvinokonzera kuratidzwa kwevasodilation uye flushing, bronchoconstriction, uye manyoka, uye zvinogona kukonzera kudonha kwakanyanya kweBP. Kazhinji inobatanidzwa apo kutsvaira ndicho chinhu chikuru.TryptaseLeukotrienes (LTC4, LTD4, LTE4)
Yakasimba bronchoconstrictor, zvakanyanya zvakanyanya kupfuura histamine nehuremu. Kuwedzera vascular permeability uye mucus kugadzirwa, uye kubatsira kune zvose mhepo uye gastrointestinal zviratidzo. LTE4 inoyerwa muweti.TryptaseCytokines (IL-6, TNF-α, IL-1, IL-4 nevamwe)
Kuratidzira mapuroteni anofambisa kuzvimba kwakakura. Inononoka mukutanga uye yakareba mukuita kupfuura varevereri vari pamusoro, uye inofungidzirwa kuti inobatsira kune kuneta, malaise, uye systemic 'flu-like' varwere vanotsanangura panguva uye mushure mekupisa.TryptaseChemokines
Tora mamwe maseru edziviriro kune saiti, achikudza uye kurebesa mhinduro yekuzvimba kupfuura yekutanga mast cell reaction.RudziPlatelet activating factor (PAF)
Iyo yakasimba kwazvo lipid murevereri iyo inowedzera vascular permeability uye inogona kukonzera yakanyanya hypotension. Inobatanidzwa mukuoma kweanaphylactic reactions uye isina kuvharwa ne antihistamines.RudziHeparin
A classical allergy ndeyeIgE-mediated reaction kune chaiyo, chinhu chinozivikanwa. Iyo inodzokororwa - kuratidzwa kwakafanana kunoburitsa maitiro akafanana - uye kazhinji inogona kuratidzwa neganda-prick kuongororwa kana chaiyo IgE kuongororwa ropa. Kusawirirana nenzungu kunoita seizvi; ndizvo zvinoita katsi dander allergies.

Anticoagulant inowanzoitika. Inogona kubatsira mukukuvadzwa kusingajairike kana kubuda ropa kunotaurwa nevamwe varwere, uye zvakare kutora chikamu mumutemo wemuno wekuzvimba.

Varwere vazhinji vanosvika paMCAS chaizvo nokuti kuongororwa kwe allergy kwakadzoka kwakachena nepo zviratidzo zvavo zvisina. A negative allergy panel haisi humbowo hunopesana nekubatanidzwa kwe mast cell; mumamiriro ezvinhu aya iri pedyo nekutarisirwa.

Chymase & carboxypeptidase A3

Murwere ane maitiro ekubereka, kuyedza IgE yakaipa, uye zvinokonzeresa senge kupisa kana kudzvanywa ndiyo yakajairika MCAS mharidzo pane murwere ane allergy bvunzo. The negative panel inowanzova chikamu chemufananidzo pane gakava pamusoro payo.
  • Granule proteases inosanganisirwa mukugadzirisa matishu, nzira dzekudzora BP, uye kushatiswa kwehumwe huturu nemapeptide.
  • Nekuti antihistamines inovhara histamine chete, mhinduro isina kukwana kune antihistamines inopindirana zvachose neMCAS pane humbowo hunopesana nazvo. Prostaglandins, leukotrienes, PAF, uye cytokines hazvina kubatwa nevamiririri - inova chikonzero chekuwedzera kwekuwedzera kwemamwe makirasi ezvinodhaka.
  • Maitiro ekuyerera achimhanya kubva kuruboshwe kuenda kurudyi: Trigger → Activation → Degranulation → Mediator kuburitswa → Zviratidzo, zvine danho remapazi pazasi rinoratidza vayananisi vakamboumbwa pamasekonzi, lipid mediators pamaminetsi, uye cytokines pamaawa, bazi rega rega rinotsanangurwa nezviratidzo zvarinotyaira.
  • Kushanda kunotevera kutevedzana: trigger, activation, degranulation, murevereri kusunungurwa, zviratidzo.
  • Pre-formed varevereri vanosunungurwa mumasekondi; lipid varevereri mumaminitsi; cytokines kwemaawa akawanda.
Mast masero anogona kusunungura vayananisi zvakasarudzwa pasina degranulation yakazara.

Histamine inongova imwe chete yevarevereri vakawanda, ndosaka antihistamine chete inowanzopa zororo.

PAF uye prostaglandin zvinogona kutyaira zvakakomba zvemoyo mhedzisiro iyo antihistamine isingagadzirise.

Chikamu 04

Zviratidzo zve MCAS
MCAS inotsanangurwa chikamu nehupamhi hwayo. Nekuti mast masero anogara munenge matishu ese, zviratidzo zvinogona kuoneka pese pese - uye chinodiwa chenhengo mbiri kana kupfuura chiri pakati pekuongororwa.Matafura ari pasi apa anoronga zviratidzo zvinowanzotaurwa nemuviri system. Yambiro mbiri tisati taverenga. Chekutanga, hapana murwere ane zvese izvi, uye runyoro rurefu harwufanirwe kuverengerwa sechinyorwa chekuenderana nacho. Chechipiri, chiratidzo chese pano chinoitika mune mamwe mamiriro mazhinji; chii chinoratidza MCAS ndiyo muenzaniso - episodic, recurrent, multisystem, kazhinji iine zvinokonzeresa zvinokonzeresa - pane chero munhu apinda.
Ganda uye mucosalChiratidzo
NotesFlushing
Kazhinji kamwe kamwe, kukanganisa chiso, mutsipa, uye chipfuva; chinhu chinogara chichitaurwaKuvava (pruritus)
Inogona kuitika kana pasina kuputika kunoonekwaMikoko (urticaria)
Yakasimudzwa, inovava mawisi; inogona kuva yenguva pfupi uye inotamaDermographism
Welts inosimudzwa nekupuruzira kwakasimba kweganda - chiratidzo chemuviri chinozivikanwaAngioedema
Kuzvimba kwakadzika, kazhinji kwemiromo, maziso, maoko, kana huro
MCAS inotsanangurwa chikamu nehupamhi hwayo. Nekuti mast masero anogara munenge matishu ese, zviratidzo zvinogona kuoneka pese pese - uye chinodiwa chenhengo mbiri kana kupfuura chiri pakati pekuongororwa.Matafura ari pasi apa anoronga zviratidzo zvinowanzotaurwa nemuviri system. Yambiro mbiri tisati taverenga. Chekutanga, hapana murwere ane zvese izvi, uye runyoro rurefu harwufanirwe kuverengerwa sechinyorwa chekuenderana nacho. Chechipiri, chiratidzo chese pano chinoitika mune mamwe mamiriro mazhinji; chii chinoratidza MCAS ndiyo muenzaniso - episodic, recurrent, multisystem, kazhinji iine zvinokonzeresa zvinokonzeresa - pane chero munhu apinda.
Kunonoka kupora rondaZvakataurwa nevamwe varwere; nzira dzisina kunyatsogadzirwa
KufemaNasal congestion uye rhinitis
Kazhinji zvisingaperi uye zvisiri zvemwakaKuoma kwehuro
Inoda kukurumidza kuongororwa kana ichifambira mberi - inogona kuratidza anaphylaxisWheeze uye kupfupika kwekufema
Leukotriene-driven bronchoconstriction inzira inozivikanwaChikosoro chisingaperi
Kakawanda kusadaira kurapwa kwechikosoro
MCAS inotsanangurwa chikamu nehupamhi hwayo. Nekuti mast masero anogara munenge matishu ese, zviratidzo zvinogona kuoneka pese pese - uye chinodiwa chenhengo mbiri kana kupfuura chiri pakati pekuongororwa.Matafura ari pasi apa anoronga zviratidzo zvinowanzotaurwa nemuviri system. Yambiro mbiri tisati taverenga. Chekutanga, hapana murwere ane zvese izvi, uye runyoro rurefu harwufanirwe kuverengerwa sechinyorwa chekuenderana nacho. Chechipiri, chiratidzo chese pano chinoitika mune mamwe mamiriro mazhinji; chii chinoratidza MCAS ndiyo muenzaniso - episodic, recurrent, multisystem, kazhinji iine zvinokonzeresa zvinokonzeresa - pane chero munhu apinda.
Kunzwa kunhuhwirira uye makemikari emhepoA anowanzo kushumwa trigger-symptom pairing
CardiovascularTachycardia
Kumhanya mwoyo, kazhinji pasina kushanda nesimbaKusagadzikana kweropa
Inogona kutenderera mune chero nzira panguva yezvikamuPresyncope uye syncope
Kuchena musoro kana kupera simba, kazhinji pakumiraChifuva kusagadzikana
Inoda kuongororwa kusabvisa zvikonzero zvemoyo
MCAS inotsanangurwa chikamu nehupamhi hwayo. Nekuti mast masero anogara munenge matishu ese, zviratidzo zvinogona kuoneka pese pese - uye chinodiwa chenhengo mbiri kana kupfuura chiri pakati pekuongororwa.Matafura ari pasi apa anoronga zviratidzo zvinowanzotaurwa nemuviri system. Yambiro mbiri tisati taverenga. Chekutanga, hapana murwere ane zvese izvi, uye runyoro rurefu harwufanirwe kuverengerwa sechinyorwa chekuenderana nacho. Chechipiri, chiratidzo chese pano chinoitika mune mamwe mamiriro mazhinji; chii chinoratidza MCAS ndiyo muenzaniso - episodic, recurrent, multisystem, kazhinji iine zvinokonzeresa zvinokonzeresa - pane chero munhu apinda.
AnaphylaxisZvakaoma, nekukurumidza, zvinogona kuisa hupenyu panjodzi - ona chikamu chekukurumidzira
GastrointestinalAbdominal cramping uye kurwadziwa
Pakati pezvinonyanya kutaurwa zviratidzo zvakazaraNausea uye kurutsa
Inogona kutevera kudya kana kuitika yakazvimiriraDiarrhea uye / kana kuvimbiswa
Motility inogona kusiyana pakati pezvikamu uye nekufamba kwenguvaReflux
Histamine's H2 mhedzisiro pagastric acid ndiyo inozivikanwa inopa
MCAS inotsanangurwa chikamu nehupamhi hwayo. Nekuti mast masero anogara munenge matishu ese, zviratidzo zvinogona kuoneka pese pese - uye chinodiwa chenhengo mbiri kana kupfuura chiri pakati pekuongororwa.Matafura ari pasi apa anoronga zviratidzo zvinowanzotaurwa nemuviri system. Yambiro mbiri tisati taverenga. Chekutanga, hapana murwere ane zvese izvi, uye runyoro rurefu harwufanirwe kuverengerwa sechinyorwa chekuenderana nacho. Chechipiri, chiratidzo chese pano chinoitika mune mamwe mamiriro mazhinji; chii chinoratidza MCAS ndiyo muenzaniso - episodic, recurrent, multisystem, kazhinji iine zvinokonzeresa zvinokonzeresa - pane chero munhu apinda.
Kuputika uye reactivity yezvokudyaInowanzo fambisa kurambidzwa kwekudya - ona yambiro muchikamu 9
Neurological uye cognitiveKuvhiringidzika kwepfungwa
Kunetseka nekuisa pfungwa, kurangarira, uye kutsvaga mazwiMusoro uye migraine
Migraine inoshumwa pamwero wepamusoro muhuwandu uhuDzungu
Kazhinji inopindirana nemoyo uye autonomic featuresKuneta
Inowanzo kuomarara uye pakati pezvinonyanya kuremara zvinoshumwa zviratidzoKunzwa zvakanyanya
Kuvhenekera, inzwi, uye hwema; nzira dzinoramba dzichiongororwa
MCAS inotsanangurwa chikamu nehupamhi hwayo. Nekuti mast masero anogara munenge matishu ese, zviratidzo zvinogona kuoneka pese pese - uye chinodiwa chenhengo mbiri kana kupfuura chiri pakati pekuongororwa.Matafura ari pasi apa anoronga zviratidzo zvinowanzotaurwa nemuviri system. Yambiro mbiri tisati taverenga. Chekutanga, hapana murwere ane zvese izvi, uye runyoro rurefu harwufanirwe kuverengerwa sechinyorwa chekuenderana nacho. Chechipiri, chiratidzo chese pano chinoitika mune mamwe mamiriro mazhinji; chii chinoratidza MCAS ndiyo muenzaniso - episodic, recurrent, multisystem, kazhinji iine zvinokonzeresa zvinokonzeresa - pane chero munhu apinda.
Manzwiro etsinga asina kujairikaKudzvova, kutsva, kana kuti chiveve kunotaurwa nevamwe varwere
Musculoskeletal, genitourinary, uye zvepfungwaMajoini uye tsandanyama kurwadziwa
Inogona kupindirana neakabatana tishu mamiriro akakurukurwa muchikamu 7Kurwadza kwebhonzo
Waranti yekuongorora, kunyanya uko mastocytosis iri kutariswaKukurumidzira dundira uye kuwanda
Interstitial-cystitis-kufanana zviratidzo zvinoshumwaZviratidzo zvinosiyana nekuenda kumwedzi
Hormonal pesvedzero pamasero mast inonyorwaKuzvidya mwoyo-sezvikamu
A classical allergy ndeyeIgE-mediated reaction kune chaiyo, chinhu chinozivikanwa. Iyo inodzokororwa - kuratidzwa kwakafanana kunoburitsa maitiro akafanana - uye kazhinji inogona kuratidzwa neganda-prick kuongororwa kana chaiyo IgE kuongororwa ropa. Kusawirirana nenzungu kunoita seizvi; ndizvo zvinoita katsi dander allergies.

Kusunungurwa kwemurevereri kunogona kuburitsa palpitations zvechokwadi, kufema, uye pfungwa yekurangwa

Kuchinja kwemaitiro uye kushatirwa

Zvose kubva pamigumisiro yemuyananisi uye kubva pakurarama nechirwere chisingaperi, chisingazivikanwi

  • Iyo 'kufunganya-kufanana' yekupinda inofanirwa kutarisirwa mumativi ese. Kuburitswa kwemurevereri kunogona kuburitsa iyo chaiyo physiology yekuvhunduka kurwiswa, uye varwere vanowanzo zivikanwa zvisizvo pachikonzero ichocho. Panguva imwecheteyo, kuzvidya mwoyo uye kuora mwoyo kunoitika pamwe chete nechirwere chisingaperi uye zvakakodzera kurapwa pachavo. Kuziva mumwe hakudi kudzinga mumwe.
  • Sei zviratidzo zvakasiyana zvakanyanya pakati pevarwere
  • Vanhu vaviri vane chirwere chakafanana vanogona kuratidza zvisingazivikanwe zvakasiyana. Zvinhu zvakati wandei zvinopa, uye mazhinji achiri asina kunyatsonzwisiswa:
  • Kugoverwa kwenharaunda. Mast cell density uye phenotype inosiyana pakati pematishu uye pakati pevanhu, saka nhengo dzenhengo dzakanyanya kubatwa dzinosiyana.
  • Kusunungurwa kwemuyananisi kwakasiyana. Mast masero anogona kusunungura vamiriri vakasarudzwa kwete zviri mukati mavo zvizere, uye musanganiswa wemuyananisi wakasiyana unoburitsa zviratidzo zvakasiyana.
  • Receptor profile. Mareceptor anoratidzwa pamaseru emunhu anoumbwa izvo zvinokurudzira zvinoshanda sezvinokonzeresa kwavari.
Varwere vazhinji vanosvika paMCAS chaizvo nokuti kuongororwa kwe allergy kwakadzoka kwakachena nepo zviratidzo zvavo zvisina. A negative allergy panel haisi humbowo hunopesana nekubatanidzwa kwe mast cell; mumamiriro ezvinhu aya iri pedyo nekutarisirwa.

Mamiriro ezvinhu aripo. Kupindirana kwezvirwere zvakadai sePOTS kana hypermobility inoshandura mufananidzo wekiriniki zvakanyanya.

Murwere ane maitiro ekubereka, kuyedza IgE yakaipa, uye zvinokonzeresa senge kupisa kana kudzvanywa ndiyo yakajairika MCAS mharidzo pane murwere ane allergy bvunzo. The negative panel inowanzova chikamu chemufananidzo pane gakava pamusoro payo.
  • Threshold uye cumulative load. Kurara, kushushikana, mamiriro ehomoni, uye hutachiona huchangobva kuitika zvese zvinoshanduka zvekutanga, saka munhu mumwechete anosiyana nekufamba kwenguva.
  • Genetic factors. Hereditary alpha tryptasemia uye kumwe kusiyanisa kwemajini kunogona kushandura mharidzo - nzvimbo yekutsvagisa inoshingaira kwete ruzivo rwakagadzika.
  • Mepu yemuviri wechiratidzo: rondedzero yeanatomical ine yakanyorwa callouts kudunhu rega rega rakakanganisika - ganda, nzira dzemhepo, moyo, ura, huropi, majoini, dundira - imwe neimwe inokwidziridzwa kuratidza zviratidzo zvine chekuita neiyo system. Toggle inogona kuratidza kuti ndeapi masisitimu ari kusangana nemurwere.
  • Zviratidzo zvinotora ganda, kufema, moyo, GI, neurological, musculoskeletal, genitourinary, uye psychological domains.
  • Kubatanidzwa kwemaviri kana anopfuura organ system chikamu cheiyo diagnostic pateni.
Hapana chiratidzo chemunhu chakanangana neMCAS - iyo episodic, multisystem pateni ndiyo yakakosha.

Mharidzo inosiyana zvakanyanya pakati pevarwere uye nekufamba kwenguva mukati memurwere mumwe chete.

Kuzvidya mwoyo-kufanana zvikamu zvinogona kutungamirirwa nemurevereri; izvi hazvifanirwe kushandiswa kudzinga mamiriro acho, kana kubvisa zvinodiwa zvehutano hwepfungwa zvechokwadi.

Mast Cell Activation Syndrome chirwere chekudzivirira muviri maitiro pane kudzivirira quantity. Iwo maseru aripo muhuwandu hwakajairwa uye anotaridzika zvakajairwa - chachinja ndechekuti vanopisa sei.

Chikamu 05

Common Zvikonzero

Zvikonzero hazviwanzoita zvega. Mast cell anopindura kune akawedzera mutoro, ndosaka chikafu chimwechete, kamuri, kana chiitiko chinogona kusakuvadza rimwe zuva uye kusashiviririka kune rinotevera.

Activation ndiyo cumulative. Zviitiko zvakasiyana-siyana zvinoshiviririka zvakasvika pamwe chete zvinogona kuyambuka chikumbaridzo hapana angayambuka ari ega. Iri ndiro zano rimwechete rinonyanya kubatsira pakuita pfungwa yezvinongoerekana zvaitika.

Zvokudya

Zvekudya zvinokonzeresa muMCAS zvinowanzoenderana nezvehistamine zvirimo kana kutungamira mast cell kutsamwisa kwete kune yekirasi yechikafu allergy. Histamine inoungana muchikafu kana ichikura, ichivirisa, kana kuchengetwa, ndosaka kutsva kuchiwanzokosha kupfuura chinhu chaicho - hove imwe chete inogona kuregererwa pazuva rekutenga uye kwete mazuva matatu gare gare.Chizi dzachembera, nyama dzakaporeswa uye dzakagadzirwa, chikafu chakaviriswa, uye zvakasara zvinowanzobatanidzwa. Boka rechipiri rinoita serikuchidzira mast cell kuburitsa yavo histamine. Musiyano wega wega wakakura, uye zvinyorwa zvakaburitswa zvinobatwa zvakanyanya sefungidziro yekuyedza kwete mitemo.

Kupisa

Pakati pezvinonyanya kushumwa zvinokonzeresa zvemuviri. Mvura inopisa uye yekugezera, saunas, mamiriro okunze anopisa, makamuri anodziya, uye zvinwiwa zvinopisa zvese zvinowanzotaurwa. Kupisa hakudi chinhu kuti chipinde mumuviri, icho chiri chikamu chechikonzero nei standard allergy yekuongorora isingazvione.

Kutonhora

Mhepo inotonhora, mvura inotonhora, zvinwiwa zvechando, uye nharaunda dzine firiji zvinonzi zviri kukonzera vamwe varwere. Zvikurukuru, nekukurumidza shanduko pakati petembiricha inowanzotsanangurwa seyakaipisisa kupfuura chero yakanyanyisa yakabatwa yakatsiga.

Exercise

Kushanda nesimba kwemuviri ndicho chikonzero chechokwadi chevarwere vazhinji, uye chimwe chine mutengo chaiwo, sezvo chiitiko chinobatsira kune hutano uye manzwiro. Kusimba uye mwero wekuwedzera kazhinji zvine basa kupfuura nguva yakazara, ndosaka nzira zvishoma nezvishoma, yakaderera-yakanyanya - uye recumbent kana yemvura-yakavakirwa chiitiko apo kumira kusingatenderwe zvakanaka - kunowanzo tarisirwa zvirinani.

Stress

Kushushikana mupfungwa chinhu chinokonzeresa chemuviri, kwete chirevo chekutaura. Mast cell anotakura mareceptor e-corticotropin-releasing hormone uye anogara padyo nemagumo etsinga, achipa nzira yakanyorwa iyo iyo tsinga inopesvedzera kuita kwavo. Pfungwa iyi inofanirwa kusimbiswa nekuti varwere vane zviratidzo zvinowedzera nekushushikana vanowanzoudzwa kuti mamiriro avo ndeepfungwa.A demonstrable neuroimmune nzira haisi chinhu chakafanana nechiratidzo chiri kufungidzira.

Mahomoni

Varwere vazhinji vanorondedzera cyclical zviratidzo maitiro, kazhinji kuwedzera kutenderera kwekutevera. Estrogen inopesvedzera mast cell behaviour, inova imwe inokurudzirwa kupa kune mwero wepamusoro wekuongororwa kwevakadzi. Shanduko panguva yekuzvitakura, postpartum, uye perimenopause zvinotsanangurwa zvakare, uye zvinosiyana zvakanyanya pakati pevanhu.

Utachiona

Utachiona hwakavimbika hunosimudza yekutanga reactivity kune varwere vazhinji. Chirwere chevhairasi chenguva dzose chinowanzowedzera chinokonzeresa chinoiswa kwemavhiki anotevera, saka kufumurwa kwaimbobvumirwa kunova kunotsamwisa panguva yekupora. Vamwe varwere vanotanga kutanga kwezviratidzo zvavo kuchirwere chinotapukira, kunyangwe kumisa causation mune imwe nyaya kwakaoma.

Kuratidzwa kwezvakatipoteredza

Zvinonzi zvinokonzeresa nharaunda zvinosanganisira mold, guruva, mukume, utsi, kusvibiswa kwemhepo, shanduko yemwando, uye barometric pressure shifts. Sensitivity inosiyana zvakanyanya, uye kuziva zvinokonzeresa kwezvakatipoteredza kazhinji zvinoda kureba kurekodha-kuchengeta kwete kungoona chete.

Mishonga

Mamwe makirasi emishonga anozivikanwa muzvinyorwa zvemast cell seanogona kuita activator, kusanganisira maopioid, mamwe asiri-steroidal anti-inflammatory drugs, radiocontrast media, mamwe neuromuscular blocking agents anoshandiswa muanesthesia, uye vancomycin. Zvisizvo zvisingashande - dhayi, zvinochengetedza, zvigadziriso - zvinogona zvakare kuve chikamu chinotsamwisa, ndicho chikonzero murwere anogona kushivirira kuumbwa kwemumwe mugadziri uye kuita kune imwe.

Zvakakosha

Usamira, kudzivisa, kana kuti kushandura mushonga waunenge wapiwa pamusana perondedzero yezvakawanda. Uya nerondedzero kumurapi anoziva nhoroondo yako. Ngozi yekurega kurapwa kunodiwa inogona kudarika njodzi inodziviswa, uye iyi sarudzo inoda kuongororwa kwega.

Kunhuwirira uye kunhuhwirira

Perfumes, air fresheners, zvinhu zvekuchenesa, zvinhu zvekuwachira, zvinonyungudutsa, pendi itsva, uye midziyo mitsva zviri pakati pezvinokonzeresa zvinogara zvichishumwa, uye pakati pezvakanyanya kuoma kudzivirira munzvimbo dzakagoverwa. Nzvimbo dzisina kunhuhwirira chikumbiro chekugara muzvikoro nenzvimbo dzebasa nekuda kweichi chikonzero.

Doro

Doro rinowanzotaurwa sechinhu chinokonzera kuburikidza nenzira inopfuura imwe: zvinwiwa zvakawanda zvinodhaka zvine histamine zvakananga, kunyanya waini uye doro; doro rinogona kukanganisa ma enzymes anoputsa histamine; uye inokonzera vasodilation yakazvimirira. Kuita kune zvishoma kunowanzotsanangurwa.

Kushaya hope

Varwere vazhinji vanosvika paMCAS chaizvo nokuti kuongororwa kwe allergy kwakadzoka kwakachena nepo zviratidzo zvavo zvisina. A negative allergy panel haisi humbowo hunopesana nekubatanidzwa kwe mast cell; mumamiriro ezvinhu aya iri pedyo nekutarisirwa.

Kurara zvisina kunaka kana kusakwana kunodzikisira kushivirira zvakanyanya uye kunopindirana nechimwe chinhu chiri pachirongwa ichi. Haisi kashoma ndiyo inokonzera moto, asi ndeimwe yevanonyanya kubatsira, uye varwere vanowanzotaura kuti kuchengetedza kurara kunosimudza chikumbaridzo chavo kune zvimwe zvese.

Murwere ane maitiro ekubereka, kuyedza IgE yakaipa, uye zvinokonzeresa senge kupisa kana kudzvanywa ndiyo yakajairika MCAS mharidzo pane murwere ane allergy bvunzo. The negative panel inowanzova chikamu chemufananidzo pane gakava pamusoro payo.
  • Sei zvinokonzeresa zvakasiyana zvakanyanya
  • Trigger seti inosiyana pakati pevanhu nekuti ma receptor profiles, kugoverwa kwetishu, mamiriro aripo-aripo, uye yekutanga reactivity zvese zvinosiyana. Vanoshandukawo mukati munhu nekufamba kwenguva - kunowanzo wedzera panguva dzekutadza kutonga uye kudzikisira zvakare kana kurapwa kwadzikisira kwekutanga reactivity. Kuita kunogona kunonoka nemaawa, izvo zvinovharidzira kubatana pakati pechikonzero nemhedzisiro. Nekuda kwezvikonzero zvese izvi, kuziva zvinokonzeresa nekuvimbika kunoda kurongeka-kuchengetwa kwemavhiki pane mhedziso dzakatorwa kubva muchiitiko chimwe chete.
  • Threshold infographic: bhaa yakachinjika inomiririra kuita chikumbaridzo, ine zvidhinha zvakaturikidzana pasi pekupisa kwakanyorwa, kusarara zvakanaka, kushushikana, chikafu chechembere, uye kushanda nesimba. Ratidza chiitiko chekutanga apo mabhuroko matatu anogara pazasi pemutsara, uye chechipiri apo chechina bhuroko inosundira stack pamusoro payo - zvimwe chete zvikamu, zvakasiyana.
  • Inokonzeresa stack - kuwedzera mutoro unokosha kupfuura chero kuratidzwa kumwe chete.
  • Zvikonzero zvemuviri (kupisa, kutonhora, kudzvanywa, kushingaira) hazvidi chinhu chakamwiswa uye hazvionekwe pakuongororwa kweallergy.
Kushungurudzika kunoshanda kuburikidza neyakanyorwa neuroimmune nzira; izvi hazviiti kuti mamiriro acho ave mupfungwa.

Mishonga inokonzera mishonga inofanira kukurukurwa neanonyora, asingambozvigadziri kubva pane rondedzero.

Trigger seti inosiyana pakati pevanhu uye inochinja nekufamba kwenguva, saka kuchengetedza rekodhi kwakakosha.

Chikamu 06

Nei MCAS iri
Zvakaoma ku Kuongorora

Diagnostic kunonoka muMCAS hakusi kutadza kwevarapi vega. Izvi zvinotevera kubva kune chaiwo, zvinozivikanwa maficha emamiriro uye bvunzo dzinoshandiswa kuiongorora.

Variable zviratidzo

MCAS inogadzira zviratidzo zvakasiyana-siyana zvakasanganiswa muvarwere vakasiyana, uye musanganiswa wakasiyana mumurwere mumwechete nekufamba kwenguva. Iko hakuna mharidzo imwe chete yekufananidza-match inopesana. Nekuda kwekuti zviratidzo zvacho hazvina kujeka, chimwe nechimwe chinogona kuverengerwa kune chimwe chiitiko chakajairika kana ichifungwa yakazvimiririra.

Normal laboratory kuongororwa

Kushanda kweropa nguva dzose hakushamisi muMCAS. Kuwanda kweropa rakazara, zviratidzo zvekuzvimba, uye zvakajairwa biochemistry zvinowanzojairika, sezvakaita allergy mapaneru. Kuongororwa kwakasarudzika kwemuyananisi kunodiwa, uye kunyangwe izvo zvinowanzo dzosera zvakajairika zvikonzero zvehunyanzvi zvakafukidzwa pazasi.Murwere anogona saka kuunganidza faira rakawanda rekuferefeta kwakajairika asi achiramba asina kugadzikana - maitiro ayo zvinosuruvarisa anowedzera mukana wekuudzwa kuti hapana chakaipa.

A classical allergy ndeyeIgE-mediated reaction kune chaiyo, chinhu chinozivikanwa. Iyo inodzokororwa - kuratidzwa kwakafanana kunoburitsa maitiro akafanana - uye kazhinji inogona kuratidzwa neganda-prick kuongororwa kana chaiyo IgE kuongororwa ropa. Kusawirirana nenzungu kunoita seizvi; ndizvo zvinoita katsi dander allergies.

Kusunungurwa kwemuyananisi

Iri ndiro dambudziko repakati pehunyanzvi. Vayananisi vakayerwa vane hafu yeupenyu hupfupi. Serum tryptase inokwira mukati memaawa mashoma ekuita uye yobva yadonha; urinary metabolites inopfuurira kwenguva refu asi ichiri kuratidza hwindo shoma. Sampling pakati pezvikamu zvinogona kuwana chinhu chakakwira - kwete nekuti activation haina kumboitika, asi nekuti haichayereki.

Kubata zvinodiwa zvinosanganisa izvi. Varevereri vakati wandei havana kugadzikana patembiricha yekamuri, uye prostaglandin samples inonyanya kusimba. Samples dzinowanzoda kutonhora nekukurumidza, kubata kutonhora mukati mekuunganidza, uye nekukasira kugadzirisa. Muenzaniso wakasiiwa patembiricha yemumba kana kunonoka kufamba unogona kuburitsa mhedzisiro kubva kumurwere ainyatso pindura.

Mugove wakakura wemhedzisiro yakaipa yemuyananisi inoratidza nguva uye kubata pane kusavapo kwechirwere. Kusimbisa kuunganidzwa kweprotocol nemurapi anohodha uye murabhoritari tisati tatorwa kwakakosha pane kudzokorora bvunzo isina kubatwa zvakanaka.

Kubatana nezvimwe zvirwere

Zviratidzo zveMCAS zvinopindirana zvakanyanya nekugumbuka bowel syndrome, chisingaperi urticaria, asima, fibromyalgia, chisingaperi kuneta chirwere, kusagadzikana kusagadzikana, dysautonomia, uye zvimwe zvakati wandei. Chimwe nechimwe cheizvi chinozivikanwa kune vazhinji varapi uye chinowanzoonekwa. Kana murwere auya nezviratidzo zvinokwana zvakati wandei zveizvi zvishoma, zvinowanzoitika ndezvekuongororwa zvishoma pane kusanganisa.

Kushaikwa kwekuziva kwachiremba

MCAS yakaitwa zviri pamutemo muna 2007 chete, paine nzira dzekubvumirana dzinotevera mu2010 uye 2012. Vazhinji varapi vaidzidzira vakapedza kudzidziswa kwavo chirwere chisati chapinda muzvidzidzo zvekare. Kuzivana kunosiyana zvakanyanya kunyangwe pakati pevanorwara nevanorwara nezvirwere uye vanodzivirira zvirwere, uye kuwana nyanzvi dzine ruzivo rwemast cell hakuna kuenzana nenzvimbo.

Diagnostic gakava

Zvakakodzera kutaura pachena kuti nzira yekuongorora yeMCAS inoramba iri nyaya yegakava rechokwadi mukati memabhuku ekurapa. Zvakawanda, chinzvimbo chimwe chinobatirira kumaitiro akasimba anoda humbowo hwemuyananisi, nechikonzero chekuti pasina iyo njodzi yekuongorora inogona kushandiswa zvakanyanya.Imwe chigaro inobata kuti maitiro akasimba anopotsa varwere vane chirwere chechokwadi asi avo vanoyedza murevereri vanoramba vachikundikana nekuda kwezvikonzero zvehunyanzvi zvinotsanangurwa pamusoro.

Varwere vazhinji vanosvika paMCAS chaizvo nokuti kuongororwa kwe allergy kwakadzoka kwakachena nepo zviratidzo zvavo zvisina. A negative allergy panel haisi humbowo hunopesana nekubatanidzwa kwe mast cell; mumamiriro ezvinhu aya iri pedyo nekutarisirwa.

Uku hakusi kusawirirana kwevarwere kwakagadzirwa, uye hakuna kugadziriswa. Mhedzisiro yaro inoshanda ndeyekuti nyanzvi mbiri dzinogona kusvika pamhedzisiro dzakasiyana nezvemurwere mumwechete. Kunzwisisa kuti gakava riripo kunobatsira kutsanangura maonero anopokana pasina kufungidzira kuti mumwe murapi akanga asina kururama.

Murwere ane maitiro ekubereka, kuyedza IgE yakaipa, uye zvinokonzeresa senge kupisa kana kudzvanywa ndiyo yakajairika MCAS mharidzo pane murwere ane allergy bvunzo. The negative panel inowanzova chikamu chemufananidzo pane gakava pamusoro payo.
  • Diagnostic kunonoka
  • Iko kusanganiswa kuri pamusoro kunoburitsa kunonoka kwenguva refu. Varwere vanowanzofambiswa kuburikidza nehunyanzvi hwakawanda - gastroenterology, dermatology, cardiology, allergy, neurology, psychiatry - imwe neimwe ichiongorora dunhu rimwe redambudziko rehurongwa.Huwandu hwakatsikiswa uye ongororo dzevarwere dzinowanzo tsanangura kunonoka kuyerwa mumakore, uye fungidziro dzinotenderedza makore gumi dzinowanzotaurwa, kunyangwe manhamba akadaro achiuya nemaitiro chaiwo ekusakwanisa uye anofanirwa kuverengerwa sechiratidzo pane kunyatso.
  • Timeline graphic yemumiriri rwendo rwekuongorora: zviratidzo zvekutanga pagore zero; kutanga kushanya kwaChiremba; kutumirwa kune gastroenterology ine yakajairika endoscopy; dermatology nechiratidzo-kurapa mhedzisiro; cardiology ine yakajairika Holter monitoring; kutumirwa kwepfungwa; ipapo allergy/immunology uye pakupedzisira mast cell evaluation.Imwe neimwe nodi inotsanangurwa nekufamba kwenguva uye 'zvakajairwa mhedzisiro' mamaki, zvichiita kuti kuunganidzwa kwebvunzo dzisina kunaka kuonekwe sepateni pane kutevedzana kwezviitiko zvega.
  • Kuongororwa marabhoritari kunowanzojairika muMCAS; nyanzvi yekuongorora muyananisi inodiwa.
  • Vayananisi ndevenguva pfupi uye havana kugadzikana, saka nguva uye sampuli yekubata inotyaira akawanda enhema.
Zviratidzo zvinopindirana nemamwe akawanda akajairika mamiriro, zvichigadzira chidimbu chechirwere.

Kujairana kwekiriniki kunosiyana; mamiriro acho akapinda mucurricula nguva pfupi yapfuura.

Nzira dzekuongorora dzinoramba dzichipikiswa, saka maonero enyanzvi anogona kusiyanisa zviri pamutemo.

Chikamu 07

Zvirwere Zvinowirirana

MCAS inowanzo kutaurwa pamwe nemamwe akati wandei mamiriro. Simba rehumbowo rinosiyana zvakanyanya pakati pezvibatanidzwa izvi, uye musiyano unobata nyaya.

Verenga izvi kutanga

Kushamwaridzana hakusi chikonzero. Mamiriro akati wandei ari pazasi anoitika pamwe chete neMCAS kazhinji kupfuura mukana waungafungidzira, asi kunyangwe ivo vachigovana michina, ingave imwe ichikonzeresa imwe, kana yekugovana nzira dzekutumira dzinowedzera kupindirana kuri pachena. isati yasimbiswa kwezvizhinji zvezvibatanidzwa izvi.

Postural Orthostatic Tachycardia Syndrome (POTS)

POTS imhando ye dysautonomia inoratidzwa nekuwedzera kurova kwemoyo pakumira, kazhinji nekureruka-musoro uye kuneta. Kubatana-kuitika neMCAS kunotsanangurwa zvakanaka mumabhuku. Tsanangudzo dzakarongwa dzinosanganisira mast cell mediators inokanganisa vascular tone uye autonomic regulation, asi kutungamira kwehukama hwese hunokonzera hahuna kusimbiswa.Mamiriro ese ari maviri anogona kuburitsa tachycardia, dzungu, uye kuneta, izvo zvinokanganisa kupa zviratidzo kune imwe kana imwe.

Hypermobile Ehlers-Danlos Syndrome (hEDS) uye hypermobility spectrum disorders

Iko-kuitika kwehEDS, POTS, uye MCAS kunokurukurwa kakawanda zvakakwana kuti zvitsanangurwe zvisina kurongwa semutatu. Kushamwaridzana kunoshumwa nguva dzose, asi nzira yepasi inoramba iine fungidziro. Mast masero anogara mumatishu akabatana uye anotora chikamu mukugadziridza matishu, izvo zvinopa mutsara unonzwisisika wekubvunza, asi kuvepo hakusi humbowo. Referral uye diagnostic maitiro anogonawo kubatsira kune kuti kupindirana kwakasimba kunoratidzika sei.

Hereditary alpha tryptasemia (HaT)

HaT ihwo hunhu hunosanganisira mamwe makopi eiyo alpha-tryptase gene TPSAB1, inogarwa nhaka mune autosomal inotonga pateni, uye inogadzira inoenderera yakasimudzwa baseline serum tryptase. Inowanikwa muhuwandu hwevanhu. Hukama hwayo nezviratidzo huchiri kujekeswa: hunobatanidzwa nekunyanya kuita kwakasimba mune mamwe mamiriro, asi vanhu vazhinji vane hunhu hwacho havana symptomatic.

HaT inokosha pakududzira. Nekuti inosimudza yekutanga tryptase, imwechete yakakwira tryptase kukosha mumunhu ane HaT haingoratidze mast cell activation - iyo inosimbisa kuti nei chikumbaridzo chekuongorora chichitsanangurwa se kukwira pamusoro pekutanga kwemunhu kwete nhamba yakakwana.

Irritable bowel syndrome (IBS)

Zviratidzo zvemudumbu muMCAS zvinopindirana zvachose neBSS maitiro, uye vamwe varwere vanotakura mavara ese. Tsvagiridzo yakaongorora mast cell density uye activation mugut mucosa yevarwere veIBS vane zvakasiyana zvakawanikwa. Kunyangwe iyo subset yeIBS inomiririra isingazivikanwe mast cell kubatanidzwa mubvunzo wakavhurika kwete chokwadi chakasimbiswa.

Migraine

Migraine inoshumwa pamwero wakakwira pakati pevarwere veMCAS. Histamine chinhu chakasimbiswa vasoactive chinhu chinozivikanwa kukosha kune musoro, uye mast masero aripo mumeninges. Iyo mechanistic link ine biologically inogoneka uye iri kuferefetwa, asi haina kugadziriswa.

Asthma uye chisingaperi urticaria

Aya ane hukama hwakajeka hwemakanika kune maseru makuru echero chinhu chiri muchikamu chino. Mast masero ari pakati pepathophysiology yezvose zviri zviviri - leukotrienes uye histamine drive bronchoconstriction uye wheal formation zvichiteerana. Chronic spontaneous urticaria kunyanya inosanganisira mast cell activation zvakananga.Aya haasi masangano ekufungidzira; iwo mamiriro umo mast cell kubatanidzwa kunotangwa, kunyangwe ivo vachiramba vakasiyana kuongororwa kubva kuMCAS.

Yakareba COVID

Vamwe vaongorori vakakurudzira mast cell activation semupiro kuLong COVID, vachiona chiratidzo chinopindirana mukuneta, kuoma kwekuziva, uye dysautonomia. Iyi inzvimbo yekuongorora inoshanda uye humbowo huripo preliminary. Inofanirwa kubatwa sefungidziro iri pasi pechidzidzo kwete hukama hwakasimba, uye zvirevo zvekubatana kwakagadziriswa - mune chero nzira - parizvino inodarika data.
Autoimmune mamiriroYakasiyana-siyana autoimmune mamiriro anoshumwa padivi peMCAS, kusanganisira thyroid autoimmunity. Mast masero anotora chikamu mukutonga kwekudzivirira uye kupindirana nemhinduro dzekudzivirira muviri, zvichipa hwaro hunonzwisisika hwekugovana dysregulation, asi nzira dzakananga dzinobatanidza MCAS kune zvimwe zvirwere zve autoimmune hadzina kusimbiswa.Simba rehumbowo nekungotarisa
ConditionMamiriro ehukamaMamiriro ehumbowo
Chisingaperi urticariaMast cell activation iri pakati pehosha pachayoMamiriro ehumbowo
Yakagadzirwa michinaAsthmaMast cell mediators anodzinga bronchoconstriction
Hereditary alpha tryptasemiaGenetic hunhu hunosimudza baseline tryptase; kunokanganisa kududzira kwebvunzoYakasimbiswa hunhu; hukama hwechiratidzo huchiri kujekeswa
POTSZvinowanzoitika pamwe chete; murevereri mhedzisiro pane vascular toni inokurudzirwaSangano rakashuma; nzira isina kugadziriswa
HaT inokosha pakududzira. Nekuti inosimudza yekutanga tryptase, imwechete yakakwira tryptase kukosha mumunhu ane HaT haingoratidze mast cell activation - iyo inosimbisa kuti nei chikumbaridzo chekuongorora chichitsanangurwa se kukwira pamusoro pekutanga kwemunhu kwete nhamba yakakwana.hEDS / hypermobilityZvinowanzoitika pamwe chete; connective tishu kubatanidzwa kunokurudzirwa
Sangano rakashuma; mechanic hypotheticalMitengo yakakwira yakashumwa; vasoactive mediators zvinonzwisisikaSangano rakashuma; nzira iri kudzidza
IBSChiratidzo chinopindirana; gut mast cell zvakawanikwa zvakasiyanaKupindirana kwakajeka; hukama husina kugadziriswa
Migraine inoshumwa pamwero wakakwira pakati pevarwere veMCAS. Histamine chinhu chakasimbiswa vasoactive chinhu chinozivikanwa kukosha kune musoro, uye mast masero aripo mumeninges. Iyo mechanistic link ine biologically inogoneka uye iri kuferefetwa, asi haina kugadziriswa.Autoimmune chirwereCo-occurrence yakashumwa; yakagoverwa immune dysregulation yakarongwa
Murwere ane maitiro ekubereka, kuyedza IgE yakaipa, uye zvinokonzeresa senge kupisa kana kudzvanywa ndiyo yakajairika MCAS mharidzo pane murwere ane allergy bvunzo. The negative panel inowanzova chikamu chemufananidzo pane gakava pamusoro payo.
  • Sangano rakashuma; nzira haina kusimbiswa
  • Chiratidzo chinopindirana; mast cell kubatanidzwa kunofungidzirwa
  • Preliminary - pasi pekuongorora kwakasimba
  • Mast cell kubatanidzwa inotangwa mune isingaperi urticaria uye asthma - izvi hazvisi zvekufungidzira zvinongedzo.
  • HaT ihwo hunhu chaihwo hunosimudza tryptase yekutanga uye inoshandura kuti mhinduro dzebvunzo dzinofanira kuverengwa sei.
POTS uye hEDS zvinowanzoitika neMCAS kazhinji, asi magadzirirwo ari seri kwekupindirana haana kugadziriswa.

Iyo Yakareba COVID yekubatanidza ndeyekutanga uye haifanirwe kuratidzwa sezvakamiswa.

Munzvimbo yese iyi, kushamwaridzana kwagara kuchikanganisika nekuda kwekukonzeresa - verenga zvirevo zvakanyatsonaka.

Simba rehumbowo nekungotarisa

Chikamu 08

Kunzwisisa Kwazvino
ye Zvinokonzera

Pfupiso yakatendeseka ndeyekuti chikonzero cheMCAS hachizivikanwe. Zvinotevera zvinosiyanisa izvo zvakasimbiswa kubva pane zvinosara fungidziro.

Hapana chikonzero chimwe cheMCAS chakagadzwa. Zvinhu zviri pasi apa inzvimbo dzekuferefeta dzine mwero wakasiyana werutsigiro. Chero sosi inopa chivimbo, yakabatana causal account yeMCAS parizvino irikupfuura humbowo.

Genetics

Chii chinogadzirwa: Hereditary alpha tryptasemia ndeyechokwadi, inonyatso hunhu hunhu hunosanganisira mamwe makopi e TPSAB1, uye inomutsa yekutanga tryptase. Kusiyana, iyo KIT D816V mutation inogadzwa semutyairi mune yakawanda yevakuru systemic mastocytosis - imwe mamiriro, asi iyo inoratidza kuti imwe shanduko inogona kukanganisa sei mast cell biology.

Kushushikana mupfungwa chinhu chinokonzeresa chemuviri, kwete chirevo chekutaura. Mast cell anotakura mareceptor e-corticotropin-releasing hormone uye anogara padyo nemagumo etsinga, achipa nzira yakanyorwa iyo iyo tsinga inopesvedzera kuita kwavo. Pfungwa iyi inofanirwa kusimbiswa nekuti varwere vane zviratidzo zvinowedzera nekushushikana vanowanzoudzwa kuti mamiriro avo ndeepfungwa.A demonstrable neuroimmune nzira haisi chinhu chakafanana nechiratidzo chiri kufungidzira.

Chii chisiri: Hapana yakaenzana causative mutation yakaonekwa yeMCAS. Kuunganidzwa kwemhuri kunoshumwa, uye vamwe varwere vanotsanangura hama dzakawanda dzakakanganisika, asi nzira yakatsanangurwa yenhaka yeMCAS pachayo haisati yatangwa. Kunyangwe MCAS inomiririra imwe mamiriro ane genetic hwaro husati hwawanikwa, kana akati wandei akasiyana akaiswa pasi peimwe kiriniki label, haina kugadziriswa.

Kusashanda zvakanaka kweimmune system

Broad immune dysregulation inzvimbo inotungamira yekuferefeta. Nzira dzakarongwa dzinosanganisira kuchinjwa kwekutaura kwekugamuchira kana kunzwisiswa pamasero emasero, kuvhiringidza zviratidzo zvekugadzirisa izvo zvinowanzodzivisa kushandiswa, uye kushamwaridzana kusina kunaka pakati pemasero emasero uye mamwe masoja ekudzivirira zvirwere. Izvi zvinonzwisisika uye zvinonyatsodzidza, asi zvinotsanangura chikamu chetsanangudzo kwete nzira yakaratidzwa.

Varwere vakawanda vanotanga zviratidzo zvechirwere chinotapukira. Utachiona hunozivikanwa kumutsa mast cells kuburikidza neToll-like receptors, zvichipa nzira inonzwisisika, uye post-infectious mavambo inzira inozivikanwa mune akati wandei asingaperi. Zvisinei, kutanga kuti utachiona zvakakonzera MCAS mune imwe nyaya yakaoma kwazvo, uye rangarira kurerekera kunokanganisa maakaundi ekudzoreredza. Iri rinoramba riri sangano riri pasi pekudzidza.

Zvinhu zvakatipoteredza

Zvinofungidzirwa zvinopa nharaunda zvinosanganisira kufumurwa kwemakemikari, mold, kusvibiswa kwemhepo, uye shanduko yekudya pahuwandu hwevanhu. Humbowo huri pano husina kusimba zvakanyanya pane hwenzvimbo dziri pamusoro, uye zhinji dzacho hadzina kunanga. Iyi inzvimbo inonzwisisika yekubvunza kwete iyo yakasimbiswa mupiro, uye zvakare inzvimbo iyo inovimbika zvichemo zvekutengesa zvakajairika uye zvisingatsigirwe.

Kuzvimba kwenguva refu

Mast masero anopindura uye anobatsira mukuzvimba, izvo zvinosimudza mukana wekuzvisimbisa wega denderedzwa umo activation inobereka nharaunda yekuzvimba inodzikisira chikumbaridzo chekuwedzera activation. Izvi zvinoenderana nemakanika uye zvinoenderana nekiriniki yekutarisa iyo reactivity inowedzera panguva yekutonga kwakashata. Inoramba iri muenzaniso kwete chikonzero chakaratidzwa.

Epigenetics

Varwere vazhinji vanosvika paMCAS chaizvo nokuti kuongororwa kwe allergy kwakadzoka kwakachena nepo zviratidzo zvavo zvisina. A negative allergy panel haisi humbowo hunopesana nekubatanidzwa kwe mast cell; mumamiriro ezvinhu aya iri pedyo nekutarisirwa.

Epigenetic modification - shanduko mukutaura kwemajini pasina shanduko kune yakadzika kutevedzana - yakatsanangurwa senzira iyo kuratidzwa kwezvakatipoteredza kunogona kuunza shanduko yekusingaperi mumast cell maitiro. Iyi ndiyo yekutanga-danho hypothesis. Zvine musoro uye zvakakodzera kuferefetwa, uye parizvino kune humbowo hushoma hwakananga kuMCAS.

Murwere ane maitiro ekubereka, kuyedza IgE yakaipa, uye zvinokonzeresa senge kupisa kana kudzvanywa ndiyo yakajairika MCAS mharidzo pane murwere ane allergy bvunzo. The negative panel inowanzova chikamu chemufananidzo pane gakava pamusoro payo.
  • Apa panosiya zvinhu
  • Zvingangoitika kuti MCAS sekutsanangurwa kwazvino kunosanganisira zvinopfuura imwe nzira, uye kuti kiriniki yakanyorwa inobatanidza varwere vanozopedzisira vaparadzaniswa nemaitiro. Tsvakurudzo irikuenderera mberi munzvimbo dzese dziri pamusoro.Kune varwere, chirevo chinoshanda ndechekuti manejimendi anonangana nevayananisi uye zvinokonzeresa kwete chikonzero chikuru - uye kuti kusava nechokwadi kunotenderwa kune chero chigadzirwa kana protocol inotaura kugadzirisa chikonzero icho nharaunda yekutsvagisa haisati yazivisa.
  • Evidence-tier diagram: concentric bands inofamba ichienda kunze kubva 'Yakasimbiswa' (HaT trait; KIT D816V in mastocytosis) kuburikidza 'Kunyatsoongororwa neuchapupu hunotsigira' (immune dysregulation, post-infectious kutanga) kune 'Hypothesised, shoma yakananga humbowo' (epigenetics, kuratidzwa kwezvakatipoteredza kuratidzwa.
  • Hapana chikonzero cheMCAS chakagadzwa.
  • HaT uye iyo KIT D816V shanduko mune mastocytosis inogadzika genetics - kana isiri iyo yakasimbiswa chikonzero cheMCAS.
Immune dysregulation uye post-infectious kutanga zvinoferefetwa zvine humbowo hunotsigira.

Zvakatipoteredza uye epigenetic mipiro ifungidziro ine humbowo hushoma hwakananga.

Bata zvichemo zve 'mudzi wechikonzero' nekukahadzika - nharaunda yekutsvagisa haisati yazivisa imwe.

Chikamu 09

Kugara Na MCAS

Kutungamira kunovakwa zvakanyanya kubva kumaitiro ezuva nezuva kwete kubva kune chero kupindira kumwe chete. Izvo zvinotevera zvakanangidzirwa kune basa - chinangwa ndihwo hupenyu hwakakura kwazvo huchatsigira physiology yako.

Kutarisira kwezuva nezuva uye maitiro

Varwere vazhinji vanoona kuti kuenderana kunosimudza chikumbaridzo chavo zvakavimbika kupfuura chero chiyero chemunhu. Nguva dzenguva dzose dzekurara nekumuka, nguva yekudya inofanotaurwa, tembiricha yakagadzikana pazvinogoneka, uye nguva yemishonga isingachinji zvinoderedza kushanduka. Maitiro anoitawo kuti trigger izivikanwe: kana mazhinji akasiyana akagadzikana, iwo akachinja anoonekwa.

Pacing idingindira rinodzokororwa mumaakaundi evarwere - kugovera chiitiko mukati mezuva kana vhiki pane kungoisa pfungwa pazviri, uye kuvaka munguva yekudzoreredza mushure mezvinodiwa zvinozivikanwa. Izvi hazvina kufanana nekusaita basa, uye kusaita basa kwenguva refu kunotakura mari dzayo dzakanyorwa zvakanaka.

A classical allergy ndeyeIgE-mediated reaction kune chaiyo, chinhu chinozivikanwa. Iyo inodzokororwa - kuratidzwa kwakafanana kunoburitsa maitiro akafanana - uye kazhinji inogona kuratidzwa neganda-prick kuongororwa kana chaiyo IgE kuongororwa ropa. Kusawirirana nenzungu kunoita seizvi; ndizvo zvinoita katsi dander allergies.

Kutsvaga zviratidzo uye kutsvaga zvinokonzeresa

Rekodhi yakarongeka ndiyo nzira yakavimbika yekuziva zvinokonzeresa. Manyorero anobatsira anodarika chikafu: nguva yezuva, tembiricha yepakati, chii chakadyiwa uye chaive chitsva sei, kuita zvemuviri, kushushikana uye kurara, kuratidzwa hwema, mishonga inotorwa, uye - kune varwere vari kumwedzi - kutenderera chinzvimbo. Zviratidzo zvinofanirwa kunyorwa nenguva uye kuomarara.

Vhiki mbiri dzinenge dzave shoma mapatani asati anyorwa, uye kureba kuri nani. Nemhaka yokuti maitiro anogona kunonotswa nemaawa, kuratidzwa kwechinhu kungagara zvakanaka chiratidzo chacho chisati chavapo. Tarisa kudzokorora pane zviitiko pane tsananguro yechiitiko chimwe chete - chinangwa ifungidziro yekuyedza, kwete mutongo.

Dhayari yechiratidzo ndechimwe chezvinhu zvinobatsira zvakanyanya izvo murwere anogona kuunza kumusangano: kongiri, longitudinal, uye zvakananga kwavari nenzira isina yakadhindwa trigger runyorwa inogona kuve. Inobatsirawo kusiyanisa zvinokonzeresa kubva kune zvakangoitika, izvo ndangariro chete inoita zvisina kunaka.

Kushanda nevarapi

Kupa zviratidzo zvakarongeka neorgan system pane kutevedzana kwenguva inoita kuti multisystem pateni ionekwe nekukasika, uko nhoroondo yenyaya inoda kuivharisa. Kuunza mhedzisiro - kusanganisira yakajairika - inodzidzisa zvechokwadi uye inodzivirira kudzokorora ongororo dzakapedzwa.

Kubvunza zvakananga nezve muyananisi yekuyedza nguva uye sampuli kubata zvine musoro uye zvine hunyoro hunovandudza mukana wemugumisiro unoshanda. Kana usiri kunzwikwa, kutsvaga maonero echipiri zviri pamutemo; mast cell chirwere inzvimbo niche uye kujairika kunosiyana zvakanyanya. Kudzingwa kamwe chete kwakajairika uye hakusi mutongo.

Chikoro

Nzvimbo dzekugara dzakajairika dzinosanganisira mitemo yekirasi isina kunhuhwirira, mvumo yekutakura uye kuzvipa mushonga, kuwana mvura nekuzorora, kutarisisa tembiricha pazvigaro, kuchinjika pakupinda panguva yekupisa, uye kugadzirisa kwedzidzo yemuviri. Iko kune zvirongwa zvekugara zviri pamutemo muhurongwa hwako hwedzidzo, kuve nekunyorwa kwechirwere ichi mukunyora kunowanzoita kuti kuwana kwavari kuve nyore.

Basa

Mafungiro epabasa anoratidza ayo ekuchikoro: mitemo yekunhuhwirira, kudzora tembiricha, kuchinjika kana kure kure kuronga panguva yekupisa, uye kuronga kugadzirisa uko kuneta kunotevera nzira inofanotaurwa. Kana uye zvakadii zvekuzivisa isarudzo yemunhu ane kutengeserana kwechokwadi, uye inodyidzana nedziviriro yebasa inosiyana zvakanyanya pakati penzvimbo.

Travel

Matanho anoshanda anowanzo kutaurwa nevarwere anosanganisira kutakura mishonga mumikwende yemaoko ine zvinyorwa, kuwana tsamba yachiremba yemishonga inobayiwa, kuferefeta nzvimbo dzekurapa kwaunoenda, kuronga chikafu pachine nguva apo reactivity yakakosha, uye kubvumira nguva yekupora pakusvika. Tembiricha uye altitude shanduko dzakakodzera kutarisira.

Utano hwepfungwa

Kurarama nechirwere chinochinja-chinja, chisinga zivikanwe, uye chinowanzo dzingwa kune mutengo chaiwo wepfungwa, uye mutengo iwoyo hausi hutera hwehunhu. Varwere vazhinji vanotsanangura nguva yakareba yekusatenda vasati vaongororwa, izvo zvinosiya chiratidzo chayo. Tsigiro - ingave nyanzvi, vezera, kana vese - chikamu chinonzwisisika chekutungamira kwete kubvuma kuti zviratidzo ndezvepfungwa.

  • Musiyano wakakodzera kubata ndeuyu: kusunungurwa kwemuyananisi kunogona kuburitsa chokwadi-senge physiology, uye iyo inofanirwa kucherechedzwa kwete kudzoreredzwa kwepfungwa kwemamiriro ese. Panguva imwecheteyo, kuzvidya mwoyo uye kuora mwoyo zvakajairika, zvinorapika, uye zvakakodzera kugadzirisa pachavo kana zviripo.
  • Kugadzirira kwechimbichimbi
  • Vamwe varwere vane chirwere che mast cell vari panjodzi yeanaphylaxis - kukurumidza, kwakakomba, kunogona kuisa upenyu panjodzi. Kana njodzi iyoyo inoshanda, kuronga hakusi kwekusarudza, uye inhaurirano yekuva nayo nachiremba isati yadiwa.
  • Varwere vanoongororwa sevari panjodzi vanowanzonyorerwa epinephrine auto-injection uye vanorayirwa kuti vatakure nguva dzose.
  • Epinephrine ndiyo yekutanga-mutsara kurapwa kweanaphylaxis. Antihistamines haisi inotsiva uye haifanirwe kuvimbwa nayo mukuitika kwakanyanya.
  • Hurongwa hwechimbichimbi hwakanyorwa hwakabvumiranwa nanachiremba wako hunofanira kuratidza zviratidzo zveyambiro, zvekushandisa, uye nguva yekufonera rubatsiro rwechimbichimbi.
Zvinonzi zvinokonzeresa nharaunda zvinosanganisira mold, guruva, mukume, utsi, kusvibiswa kwemhepo, shanduko yemwando, uye barometric pressure shifts. Sensitivity inosiyana zvakanyanya, uye kuziva zvinokonzeresa kwezvakatipoteredza kazhinji zvinoda kureba kurekodha-kuchengeta kwete kungoona chete.

Varwere vanoongororwa sevari panjodzi vanowanzonyorerwa epinephrine auto-injection uye vanorairwa kuti vatakure nguva dzose. Epinephrine ndiyo yekutanga-mutsara kurapwa kweanaphylaxis; antihistamines haisi inotsiva iyo uye haifanirwe kuvimbwa nayo mune yakanyanya kuomarara kuita.

Makopi akapihwa mhuri, vaunoshanda navo, kana vashandi vekuchikoro zvinoreva kuti vamwe vanogona kuita kana iwe usingakwanise.

Chiziviso chekurapa chinocherekedza kuongororwa uye inozivikanwa mishonga inokonzeresa inobatsira vashandi vekukurumidzira vanogona kunge vasina kujairana nemamiriro acho.

Kuvhiya uye anesthesia inobvumidza kukurukurwa kwepamberi kuitira kuti vamiririri vagone kusarudzwa nemamiriro acho mupfungwa.

Murwere ane maitiro ekubereka, kuyedza IgE yakaipa, uye zvinokonzeresa senge kupisa kana kudzvanywa ndiyo yakajairika MCAS mharidzo pane murwere ane allergy bvunzo. The negative panel inowanzova chikamu chemufananidzo pane gakava pamusoro payo.
  • Chirevo pamusoro pekurambidzwa kwekudya
  • Kudzikira-histamine uye kudya kwekudya kunoedzwa zvakanyanya, uye humbowo hunotsigira hushoma uye hune mwero mhando. Kuwedzerwa kurambidzwa kusingatarisirwe kunotakura njodzi yechokwadi yekushomeka kwekudya uye yekudya zvisina tsarukano, uye njodzi iyi yakakura muvachiri kuyaruka. Muedzo wakarongeka, wenguva-mushoma uine kudzoreredzwa kwakarongeka - zvine chekuita nekudya kwekudya - ndiyo nzira yakachengeteka.
  • Zvakakodzera kutumidza musungo zvakananga: zvinokwanisika kuderedza zvinokonzeresa zvine hukasha zvekuti hupenyu hunoita zvibvumirano kune chero chinhu. Chirongwa chinobvisa maitiro nokubvisa basa, chikoro, zvokudya zvakasiyana-siyana, uye kuonana nevamwe chakatsinhanisa imwe nzira yokukuvadza neimwe.
  • Maitiro anowirirana anosimudza chikumbaridzo uye anoita kuti chiratidziro chizivikanwe.
  • Dhayari yechiratidzo yakarongeka kwemavhiki ndiyo nzira yakavimbika yekuziva zvinokonzeresa.
  • Kupa zviratidzo neorgan system inoita kuti multisystem pateni ionekwe kuvarapi.
Chikoro, basa, uye pekugara pekugara zvinoshanda uye zvinowanzo kupihwa nemagwaro.

Apo ngozi yeanaphylaxis inoshanda, epinephrine uye chirongwa chekuita chakanyorwa zvakakosha - antihistamines haisi inotsiva.

Aggressive diet restriction inotakura njodzi chaidzo uye inofanirwa kurongeka, ine nguva-shoma, uye inotariswa.

?

MCAS inhaka here?

Vana vanogona kugadzira MCAS?

MCAS inogona kunyangarika yega here?

Sei zviratidzo zvangu zvichiramba zvichichinja?

Sei mhinduro dzebvunzo dzangu dzichigara dziri normal?

Kushushikana kunogona kukonzeresa moto here?

Kudya kunogona kurapa MCAS?

Ndeupi musiyano uripo pakati peMCAS uye mastocytosis?

Ko MCAS yakafanana nehistamine kusashivirira?

Ndinoda kuongororwa here kuti ndionekwe?

Ndeupi chiremba anobata MCAS?

Ko antihistamine ichagadzirisa zvese here?

Sei ndichiita kune mishonga inofanirwa kubatsira?

MCAS hurema here?

Ko MCAS inogona kukonzera anaphylaxis?

Mimba inokanganisa MCAS here?

Sei kupisa kuchiita kuti ndiite chimwe chinhu kana ndisiri allergic kune chero chinhu?

Zvinotora nguva yakareba sei kuti kurapwa kushande?

Ndinofanira kuchinja mishonga yakawanda panguva imwe chete kuti ndiite zviri nani nekukurumidza?

COVID-19 kana humwe hutachiona hunogona kukonzera MCAS?

Ko MCAS inonyanya kuwanda muvakadzi?

Zvakawanda sei MCAS?

Ini ndinofanira kudzivirira chikafu chese pane yakakwira-histamine zvinyorwa?

Aiwa, uye kuedza kudaro kazhinji kunopesana. Zvinyorwa zvakadhindwa zviri kutanga kufungidzira kuti uedze maererano nerekodhi yako, kwete mitemo inoshanda zvakafanana - kusiyana kwega kwega kwakanyanya, uye varwere vakawanda vanobvumira zvinhu zvinoonekwa pane zvese. Nekuti zvinokonzeresa stack, kutsva uye mutoro wakazara zvinowanzokosha kupfuura chero chikafu chimwe chete. Shandira kuganhuriro diki inowana kutonga.

Chii chandinofanira kuuya nacho kumusangano wangu wekutanga wenyanzvi?

Nhoroondo yechiratidzo yakarongwa neorgan system pane kutevedzana, izvo zvinoita kuti multisystem pateni ionekwe nekukasika. Chiratidzo uye chinokonzeresa diary chinofukidza angangoita mavhiki maviri. Zvese zvakamboitika bvunzo zvabuda, kusanganisira zvakajairwa, nekuti izvi zvinoumba chikamu chemaitiro ekusabatanidzwa. A yazvino mishonga uye supplement list.Uye rondedzero pfupi yakanyorwa yemibvunzo yako - kugadzwa kupfupi uye zviri nyore kusiya usina kubvunza zvakakosha.

Chikamu 11

Kuwedzera Kudzidza Zviwanikwa

  • Mapoinzi ekutanga ekuverenga zvakadzama. Manongedzo chaiwo ari kuunganidzwa uye achawedzerwa pano.
  • Nhungamiro dzevarwere
  • Sumo dzinowanikwa dzakanyorerwa varwere nemhuri, dzakakodzera kugovana nevanhu vatsva kune mamiriro.
  • Nhanganyaya murwere gwara - link kuti iwewedzerwe

Ichangobva kuwanikwa yekutarisisa pakiti - link kuti iwewedzerwe

Chiratidzo chinodhinda uye chinokonzeresa diary template - link kuti iwewedzerwe

  • Nhungamiro yenhengo dzemhuri nevachengeti - link kuti iwewedzerwe
  • Nhungamiro dzekurapa
  • Mazwi ekubvumirana uye gwara rekiriniki rakaitirwa vashandi vehutano. Inobatsira kuunza kumaaphoyintimenti.
  • Consensus diagnostic maitiro - citation uye link kuti zviwedzerwe

Specialist society clinical guide - link kuti iwewedzerwe

Muyananisi kuyedzwa uye muenzaniso wekubata protocol - link kuti iwewedzerwe

  • Perioperative uye anesthesia kufunga - link kuti iwewedzerwe
  • Mapepa Okutsvakurudza
  • Mabhuku ekutanga. Pazvinogoneka, sarudza zvakaongororwa nevezera rako uye nemazuva ekuburitswa, sezvo ndima iyi inofamba.
  • Mapepa ekutanga maitiro (2007 zvichienda mberi) - zvinyorwa zvinofanira kuwedzerwa

Consensus criteria mapepa (2010, 2012) - zvinyorwa zvinofanira kuwedzerwa

Hereditary alpha tryptasemia tsvakurudzo - zvinyorwa zvinofanira kuwedzerwa

  • Ongororo yemamiriro akabatana - zvinyorwa zvinofanira kuwedzerwa
  • Professional Masangano
  • Nyanzvi nzanga uye masangano ezvidzidzo anoshanda pa mast cell chirwere.

Nyika uye yepasirese allergy/immunology societies - links kuti zviwedzerwe

Mast cell chirwere chekutsvaga network - links kuti zviwedzerwe

  • Zvirwere zvisingawanzo masangano - links kuti zviwedzerwe
  • Mabhuku

Kuverengera kwenguva refu kwevarwere nevarapi.

Mazita anotungamirwa nemurwere - kuwedzerwa

  • Clinical reference zvinyorwa - kuwedzerwa
  • Mavhidhiyo Edzidzo
  • Hurukuro dzakarekodhwa, vatsananguri, uye zvinyorwa zvemusangano.

Mavhidhiyo ekutsanangura - links kuti zviwedzerwe

Nyanzvi dzemusangano hurukuro - links kuti zviwedzerwe

  • Tsanangudzo yeMCAS yezvidzidzo zvemukati - links kuti zviwedzerwe
  • Masangano Anotsigira
  • Rutsigiro rwevezera, kumiririra, uye nharaunda.

Masangano anomirira varwere - links kuti zviwedzerwe

Nharaunda dzinotsigira vezera - links kuti zviwedzerwe

  • Mapoka ekutsigira mudunhu nemunyika - links kuti zviwedzerwe
  • Clinical Miedzo
  • Zvidzidzo zvino tsvaga. Kuwanikwa kwemuyedzo kunochinja kazhinji, saka tarisa registry zvinyorwa zvakananga.
Public clinical trial registries - links kuti zviwedzerwe

Parizvino kunyorera mast cell zvidzidzo - links kuti zviwedzerwe

Nhungamiro pane izvo kutora chikamu muyedzo - link kuti iwewedzerwe

Chikamu 12
  • Pfungwa huru dzokurangarira
  • Mafungiro akanyanya kukosha kubva kugwaro iri, akapfupikiswa.
  • Muchidimbu
  • MCAS idambudziko remast cell isina kufanira activation, kwete ye mast cell number - masero akajairika, chikumbaridzo chavo hachisi.
  • Mast cells maseru edziviriro anobatsira zviri pamutemo akaiswa pazvivhariso zvemuviri, akabata vayananisi vakagadzirwa kare vakagadzirira kuburitsa mukati memasekonzi.
  • Zviratidzo ndezve episodic, zvinodzokororwa, uye zvinosanganisira maviri kana anopfuura enhengo masisitimu - iyo pateni, kwete chero chiratidzo chemunhu, ndiyo inoratidza kuongororwa.
  • Varevereri vanoburitswa mumafungu: pre-yakaumbwa mumasekonzi, lipid mediators mumaminitsi, cytokines kwemaawa. Imwe activation chiitiko inogona kutora zuva.
  • Histamine ingori murevereri mumwe chete pakati pevakawanda, ndosaka ma antihistamines achiwanzopa kusarura pane kuzorodza kwakakwana.
  • Zvinokonzera stack. Cumulative mutoro unotsanangura maitiro ayo anotaridzika zvisina tsarukano, uye ndiyo inonyanya kubatsira pfungwa imwe chete yekutarisira zuva nezuva.
  • Zvinokonzeresa zvemuviri - kupisa, kutonhora, kudzvanywa, kushanda nesimba - hazvidi chinhu chakamwiswa uye hazvionekwe kune yakajairwa allergy kuyedzwa.
  • Negative allergy mapaneru uye zvakajairwa basa reropa zvinotarisirwa muMCAS uye usazvibvise.
  • Yakawanda yakaipa muyananisi bvunzo inoratidza nguva uye sampuli-kubata matambudziko pane kusavapo kwechirwere.
  • Nzira dzekuongorora dzinoramba dzichipikiswa, saka nyanzvi dzinokwanisa dzinogona kusvika pamhedziso dzakasiyana nezvemurwere mumwechete.
  • Mast cell kubatanidzwa ndiko yakasimbiswa mune chisingaperi urticaria uye asima; kushamwaridzana nePOTS, hEDS, IBS uye Long COVID zvinotaurwa asi mechanistic isina kugadziriswa.
  • Hapana chikonzero cheMCAS chakavambwa - chengetedza 'mudzi wechikonzero' zvikumbiro nekufungira.
Kutungamira kune nhanho: shandura chinhu chimwe panguva, uye ipa vanodzikama mavhiki usati wavatonga.

Apo ngozi yeanaphylaxis inoshanda, epinephrine uye chirongwa chekuita chakanyorwa zvakakosha; antihistamines haisi chinotsiva.

Chinangwa ndihwo hupenyu hwakakura kwazvo huchatsigira physiology - kwete hupenyu hudiki hunodzivirira zviratidzo zvese.

Chikamu 13
Tsanangudzo yemashoko
91 mazwi anoshandiswa muMCAS mabhuku uye hurukuro yekiriniki, inotsanangurwa zvakajeka.
Kuchena musoro kana kupera simba, kazhinji pakumira
A
Yakasimudzwa, inovava mawisi; inogona kuva yenguva pfupi uye inotama
Adrenaline
Ona Epinephrine. Iro zita reBritish uye repasirese risiri-risiri remuridzi wehomoni imwechete nemishonga.
Kukurumidza, kuomarara, kunogona kuuraya hupenyu hwemhando yeallergiki inosanganisira maorgan system akawanda. Inoda epinephrine nekukurumidza uye kurapwa kwekukurumidzira.
Kuzvimba munzvimbo dzakadzika dzeganda kana mucosa, kunowanzo kanganisa miromo, maziso emaziso, maoko, kana huro. Kubatanidzwa kwehuro idambudziko rezvokurapa.
Antihistamine
Mushonga unovhara histamine receptors. H1 blockers vanoita zvakanyanya paganda uye zviratidzo zvemhepo; H2 blockers inoshanda zvakanyanya padumbu remudumbu.
Kuwanda kwemasero
Autonomic nervous system
Chikamu chetsinga dzetsinga dzinodzora mabasa asingadiwe akadai sekurova kwemoyo, BP, uye kugaya. Kusashanda kwayo kunonzi dysautonomia.
B
Serum tryptase inoyerwa kana zviratidzo zvanyarara. Inopa poindi yekuenzanisa iyo kusimuka kwakanyanya kunotongwa.
Basophil
Sero jena reropa rinotenderera rinogovera zvimwe zvinhu nemasero mast, kusanganisira histamine granules, asi rinosiyana munzvimbo uye kutenderera kwehupenyu.
Biomarker
Chiratidzo chinoyerika chebiological chinoshandiswa kuona kana kuongorora mamiriro. Tryptase ndiyo yakanyanya kusimbiswa biomarker mune mast cell chirwere.
Bronchoconstriction
Kutetepa kwenzira dzemhepo nekuda kwekuomesera kwemhasuru yakatsvedzerera yakatenderedza, ichiburitsa kuhwihwidza uye kufema. Leukotrienes vatyairi vane simba.
C
Carboxypeptidase A3
Protease yakachengetwa mumast cell granules, inobatanidzwa mukugadzirisa matishu uye mukuparara kwemamwe mapeptide nehuturu.
Chemokine
Iprotein inosaina iyo inotora mamwe maseru edziviriro kune saiti, inosimudzira uye nekurebesa mhinduro yekuzvimba.
Chymase
A protease inowanikwa mumast cell granules anotora chikamu mukugadziridza matishu uye muropa rekutonga nzira.
Clonal
Zvakadzika kubva musero rimwechete risina kujairika. Clonal mast cell proliferation inoratidza mastocytosis; MCAS kazhinji isiri-clonal.
Complement system
Kuyerera kwemapuroteni eropa kuumba chikamu chekudzivirira kwekuzvarwa. Zvikamu zvaro C3a uye C5a zvinogona kumisa mast masero zvakananga.
Corticotropin-releasing hormone (CRH)
Ihomoni yekushushikana iyo mast masero anotakura ma receptors, achipa nzira yakanyorwa iyo kushushikana kwefiziology kunokanganisa mast cell activation.
Cromolyn sodium
Iyo mast cell stabilizer inoderedza kusunungurwa kwemurevereri pane kuvharira muyananisi mhedzisiro. Oral formulations inoshanda zvakanyanya pane gastrointestinal turakiti.
Cutaneous
Inoenderana neganda.
Activation
Cytokine
Inogona kuitika kana pasina kuputika kunoonekwa
Chiratidzo cheprotein chinotyaira kuzvimba uye kurongeka kwe immune. MaCytokines anoita zvishoma nezvishoma uye anoramba akareba kupfuura varevereri vakamboumbwa.
D
Maitiro ekuti mast cell's ekuchengetedza magranules anosanganidzana nesero membrane uye oburitsa zvirimo munyama yakatenderedza, kazhinji mukati memasekonzi.
Welt yakasimudzwa inogadzirwa nekupuruzira kwakasimba kweganda. Chiratidzo chemuviri chinozivikanwa chine chekuita nemast cell reactivity.
Diamine oxidase (DAO)
Iyo enzyme inoputsa yakamwisa histamine. Kuderedzwa basa ndiyo nzira inowanzo kurongedzerwa yehistamine kusashivirira.
Kusunungurwa kwakasiyana
Kusarudzwa kusunungurwa kwevamwe varevereri pasina degranulation yakazara. Uyewo inonzi piecemeal degranulation; inobatsira kutsanangura zviratidzo zvakasiyana-siyana zvezviratidzo.
Dysautonomia
Kusashanda zvakanaka kweautonomic nervous system, kuburitsa zviratidzo senge kurova kwemoyo uye kusagadzikana kweropa. POTS imwe fomu.
E
Ehlers-Danlos syndrome (EDS)
Boka reanogadzika connective tissue disorders. Iyo hypermobile mhando inowanzotaurwa padivi peMCAS nePOTS.
Kubvisa kudya
Zvinofungidzirwa zvinopa nharaunda zvinosanganisira kufumurwa kwemakemikari, mold, kusvibiswa kwemhepo, uye shanduko yekudya pahuwandu hwevanhu. Humbowo huri pano husina kusimba zvakanyanya pane hwenzvimbo dziri pamusoro, uye zhinji dzacho hadzina kunanga. Iyi inzvimbo inonzwisisika yekubvunza kwete iyo yakasimbiswa mupiro, uye zvakare inzvimbo iyo inovimbika zvichemo zvekutengesa zvakajairika uye zvisingatsigirwe.
Nzira yakarongeka yekubvisa zvinofungidzirwa kuti zvinokonzeresa zvikafu uye kuzviunza zvakare zvakarongeka. Inotakura njodzi yekudya kana ikarebeswa pasina kutarisirwa.
Eosinophil
Sero jena reropa rinosanganisirwa neallergic uye parasitic mhinduro. Yakasiyana kubva kumaseru mast asi inoshanda mukupindirana.
Shanduko mukutaura kwemajini izvo zvisingachinji kutevedzana kweDNA. Inokurudzirwa senzira inogoneka muMCAS, ine humbowo hushoma hwakananga.
Epinephrine
Mutsara wekutanga kurapwa kweanaphylaxis, kazhinji inounzwa neauto-injector. Antihistamines haisi inotsiva mune yakanyanya acute reaction.
Erythema
Kutsvuka kweganda kunokonzerwa nekuwedzera kweropa, sezvinoitika panguva yekutsvaira.
F
FcεRI
Ganda uye mucosal
Iyo yakakwirira-yakabatana IgE receptor pane mast cell pamusoro. Muchinjikwa-kubatanidza weakasungwa IgE nealeji inokonzeresa classical allergic activation.
Flare
Nguva yezviratidzo zvakaipisisa, izvo zvinogona kutevera chinokonzeresa chinozivikanwa kana kusimuka pasina chimwe. Nguva yenguva inosiyana kubva pamaawa kusvika kumavhiki.
Kamwe kamwe kutsvuka nekudziya, kazhinji kwechiso, mutsipa, uye chipfuva. Imwe yeakanyanya hunhu mast cell zviratidzo.
G
Granule
Iyo vesicle yekuchengetera mukati mesero remast rine pre-yakaumbwa vayananisi senge histamine, tryptase, uye heparin, yakagadzirira kuburitswa nekukurumidza.
H
H1 receptor
A histamine receptor subtype yakanangidzirwa muganda, nzira dzemhepo, uye tsinga dzeropa. Chinangwa cheH1 antihistamines.
H2 receptor
A histamine receptor subtype yakanangidzirwa mudumbu uye pamatishu emwoyo. Chinangwa cheH2 blockers, iyo inobatsira zviratidzo zvedumbu.
Hematopoietic progenitor
POTS imhando ye dysautonomia inoratidzwa nekuwedzera kurova kwemoyo pakumira, kazhinji nekureruka-musoro uye kuneta. Kubatana-kuitika neMCAS kunotsanangurwa zvakanaka mumabhuku. Tsanangudzo dzakarongwa dzinosanganisira mast cell mediators inokanganisa vascular tone uye autonomic regulation, asi kutungamira kwehukama hwese hunokonzera hahuna kusimbiswa.Mamiriro ese ari maviri anogona kuburitsa tachycardia, dzungu, uye kuneta, izvo zvinokanganisa kupa zviratidzo kune imwe kana imwe.
Sero remwongo risati rakura rinokwanisa kukura kuva marudzi emasero eropa. Mast cells anosimuka kubva kuCD34-positive progenitors.
Heparin
Anticoagulant inowanzoitika yakachengetwa mumast cell granules. Inogona kukonzera maronda asina kujairika kana kubuda ropa mune vamwe varwere.
Chimiro chemajini chinosanganisira mamwe makopi eiyo TPSAB1 gene, ichigadzira inoenderera yakasimudzwa yekutanga tryptase. Kugara nhaka mune autosomal dominant pateni.
Histamine
Iyo inonyanyozivikanwa mast cell mediator. Inodiridza tsinga dzeropa, inowedzera kupinda kwayo, inomutsa tsinga, uye inosimudza gastric acid, inogadzira kuputika, kuputika, mikoko, uye cramping.
Histamine kusashivirira
Kuomerwa kuputsa kumwisa histamine, kazhinji kunokonzeresa kuderedzwa kwediamine oxidase chiitiko. Yakasiyana neMCAS, kunyange zviratidzo zvinopindirana.
Mikoko
Ona Urticaria. Yakasimudzwa, inotsvedza inonyungudika paganda, kazhinji inopera uye inotama.
Hypotension
Kuderera kweBP kwakaderera. Inogona kuitika panguva yakaoma mast cell reaction uye chinhu cheanaphylaxis.
I
IgE
Immunoglobulin E, iyo antibody kirasi pakati peiyo classical allergy. Inosunga FcεRI pamasero mast; akawanda MCAS maitiro ari IgE-yakazvimirira.
Immunoglobulin
Antibody - puroteni inogadzirwa neiyo immune system inosunga zvakanangwa. IgE ndiyo kirasi inonyanya kukosha kune mast cell activation.
Kuzvimba
Kupindura kwemuviri kune kukuvara kana kutyisidzira, kunosanganisira kuwedzera kuyerera kweropa, immune cell kuunganidza, uye shanduko yetishu. Mast cell ose anokonzeresa uye anopindura kwairi.
Innate immunity
Ruoko rwekukurumidza, rusina-chaiyo rwemasoja edziviriro aripo kubva pakuzvarwa. Mast cells inhengo.
Interleukin
Chikamu che cytokine chinoshandiswa kutaurirana pakati pemasero ekudzivirira. IL-6 uye IL-4 ndedzimwe dzakaburitswa nemaseru maseru.
K
Ketotifen
Mushonga une zvese antihistamine uye mast cell inodzikamisa zvivakwa. Kazhinji zvinoda mavhiki ekushandiswa kwakafanana kusati kwazara.
KIT
A receptor pane mast cell surface inosunga stem cell factor. Pakati pemast cell kukura, kurarama, uye kugara kwematishu.
KIT D816V
Iyo chaiyo shanduko muKIT gene inowanikwa mune yakawanda yevakuru systemic mastocytosis. Haisi chikonzero chakasimbiswa cheMCAS.
L
Leukotriene
A lipid murevereri akagadzirwa mushure mekuita activation. Yakasimba bronchoconstrictor iyo inowedzerawo vascular permeability uye mucus kugadzirwa.
Leukotriene E4 (LTE4)
Leukotriene metabolite inoyerwa muweti, inoshandiswa sechikamu chekuyedzwa kwemuyananisi.
Lipid murevereri
Muyananisi akavakwa kubva musero membrane lipids mushure mekuita activation pane kuchengetwa pamberi. Prostaglandins uye leukotrienes ndiyo mienzaniso mikuru.
M
Mast cell
MCAS haisi mastocytosis
Sero redziviriro rekugara kwenguva refu rinogara munyama mumuviri wese, rine magranules evamiriri vekare vakaumbwa. Pakati peallergic uye innate immune immune.
Varevereri vakuru uye zvinoitwa nemumwe nemumwe
Mast cell stabilizer
Mushonga unoderedza mast cell mediator kusunungurwa pane kuvharira murevereri mhedzisiro. Cromolyn uye ketotifen ndiyo mienzaniso yakajairika.
Chimiro chinosanganisira kuunganidzwa kweakawandisa mast masero munyama, kazhinji inofambiswa neKIT D816V shanduko mune yevakuru systemic chirwere. Yakasiyana kubva kuMCAS.
Chero kemikari inoburitswa nesero remast inoburitsa mhedzisiro pane yakatenderedza tishu. Inosanganisira histamine, tryptase, prostaglandins, uye vamwe vakawanda.
Menings
Ma membranes akakomberedza uropi uye musana. Mast masero aripo pano, izvo zvinoenderana nekutsvaga nezvezviratidzo zvetsinga.
Montelukast
A leukotriene receptor antagonist anoshandiswa kugadzirisa kufema uye egastrointestinal zviratidzo izvo antihistamines hazvisviki.
MRGPRX2
A mast cell receptor mediating IgE-yakasununguka activation nemimwe mishonga uye peptides. Inobatsira kutsanangura maitiro pasina inotaridza IgE kubatanidzwa.
Mucosa
Iwo matishu akanyorova anonamira mukati memukati senge ura, mhepo, uye mhino. Izere nevanhu vane mast cells.
N
N-methylhistamine
Histamine metabolite yakayerwa mumaawa makumi maviri nemana ekuunganidza weti sehumbowo hwekuburitswa kwemast cell mediator.
Neuroimmune
Zvinoenderana nekudyidzana pakati penheyo uye immune system. Kudyidzana kweMast cell-nerve ndiyo hwaro hwekushushikana kuchiita senge physiological trigger.
O
Omalizumab
A biologic therapy yakanangana neIgE, inoshandiswa mune dzimwe nyaya dzinoramba dziri pasi pekutariswa kwenyanzvi.
P
Palpitations
Kuziva kurova kwemwoyo, kunowanzotsanangurwa sekumhanya, kurova, kana kusajairika.
Perivascular
Iri pedyo netsinga dzeropa. Nzvimbo huru yekugara mast cell, inoenderana neBP uye zviratidzo zvekuyerera.
Piecemeal degranulation
Hereditary alpha tryptasemia
Ona Differential release. Sarudzo yemurevereri kuburitswa pasina kuburitswa kwehuwandu hwemukati megranule.
Platelet activating factor (PAF)
Iyo yakanyanya simba lipid murevereri iyo inowedzera vascular permeability uye inogona kukonzera yakanyanya hypotension. Haina kuvharwa ne antihistamines.
Postural Orthostatic Tachycardia Syndrome. Chimiro che dysautonomia chinosanganisira kuwandisa kwemwoyo kunowedzera pakumira; inowanzotaurwa pamwe neMCAS.
Presyncope
Kunzwa kwekuda kufenda, pasina kurasikirwa nekuziva.
Prostaglandin D2
A lipid murevereri anokonzera kuoneka vasodilation, flushing, bronchoconstriction, uye manyoka. Kakawanda kwakakurumbira uko kutsvaira kunotonga.
Pruritus
Izwi rekurapa rekukwenya.
R
Refractory
Kusapindura zvakakwana kurapwa kwakajairika. Refractory kesi dzinogona kukurudzira kutariswa kwevamiriri vehunyanzvi.
Regranulation
Maitiro ekuti sero remast rinovakazve zvitoro zvaro zvegranule mushure mekudonha, zvichibvumira kuti ipindure zvakare pane kufa.
Rhinitis
Kuzvimba kwenasal lining kunokonzera kusangana, mhino inomhanya, uye kuhotsira. Kazhinji isingaperi uye isiri-mwaka muMCAS.
S
Serum tryptase
Trypase inoyerwa muropa. Iyo yakanyanya kusimbiswa murabhoritari chiratidzo che mast cell activation, inoda ese acute uye ekutanga samples.
Stem cell factor (SCF)
Iyo inosaina mamorekuru inosunga KIT, inotyaira mast cell kusimudzira, kupona, uye kugara kwematishu.
Syncope
Kufenda - kurasikirwa kwekanguva kwekuziva, kazhinji kubva pakuderera kwekuyerera kweropa kuuropi.
Systemic
Kukanganisa muviri wose pane imwe nzvimbo yenzvimbo.
Kunzwa kunhuhwirira uye makemikari emhepo
Systemic mastocytosis
Chimiro che mastocytosis umo masero akawandisa anoungana munhengo dzemukati, kazhinji neKIT D816V shanduko.
T
Kurova kwemoyo kusingaite, kunowanzo kutaurwa panguva yemast cell reaction.
Chikumbaridzo
The cumulative level of stimulation panoita mast cells. Pakati pekuti nei zvinokonzeresa stack pane kuita wega.
Toll-like receptor
Chii Chinoitika Panguva
Mast Cell Activation
A receptor inoziva mapatani ane hukama nehutachiona, inobatanidza mast cell activation kune hutachiona.
TPSAB1
Iyo geni encoding alpha-tryptase. Mamwe makopi anogadzira nhaka alpha tryptasemia uye yakakwira yekutanga tryptase.
Chero kukurudzira kunomutsa mast cell activation. Inogona kunge iri kemikari, yemuviri, yehomoni, inotapukira, kana manzwiro; kazhinji inounganidzwa pane imwe chete.
Tryptase
Maprotein akanyanya kuwanda mu mast cell granules uye chicherechedzo chakanyanya kusimbiswa che activation, chinokosheswa kuve chine mast-cell-specific.
U
Urticaria
Mikoko - yakasimudzwa, yakafunuka welts inokonzerwa nemurevereri kusunungurwa muganda. Chronic spontaneous urticaria inosanganisira mast cell activation zvakananga.
Urticaria pigmentosa
Chimiro cheganda rinowanikwa mune mamwe marudzi ecutaneous mastocytosis, anoratidza semaculopapular maronda.
V
Vasoactive
Kuva nemhedzisiro pamudziyo weropa dhayamita kana kupera. Histamine uye prostaglandin D2 zvine vasoactive zvakasimba.
Vasodilation
Kuwedzera kwetsinga, kuwedzera kuyerera kweropa uye kuderedza BP. Inogadzira flushing uye inogona kukonzera light-headedness.

Iwe Hausi Ndega

Tsanangudzo yeMCAS iripo kuitira kuti murwere wese awane rutsigiro, nharaunda, uye ruzivo rwekuzvimiririra. Joinha network yedu yepasi rose.

Patient Stories Batanidzwa →