Kunzwisisa Mast Cell Activation Syndrome (MCAS)

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.

Key Concept

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.

Sei mast cells aripo zvachose

Pfungwa Huru

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.

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.

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

Mastocytosis yakaenzaniswa neMCAS
	MCAS haisi mastocytosis	Uyu ndiwo mutsauko umwechete unonyanya kukosha mumunda, uye kuvhiringa maviri kunotungamirira kunyonganiso pamusoro pezvinhu zvose zviri pasi perwizi.
Mastocytosis yakaenzaniswa neMCAS	Mastocytosis	MCAS
Kukanganisika kukuru	Masero akawanda akawandisa anoungana munyama	Normal nhamba yemast cells, activate zvisina kufanira
Kuwanda kwemasero	Yakasimudzwa - clonal kuwanda	Normal
Tryptase yepakutanga	KIT D816V shanduko mune dzakawanda dzevakuru systemic kesi	Kazhinji zvakajairwa pakutanga
Yakajairika genetic kuwana	Abnormal mast cell aggregates anoratidzwa	Hapana imwechete yakagadziriswa causative mutation
Biopsy zvakawanikwa	Abnormal mast cell aggregates anoratidzwa	Hapana hunhu kuunganidza
Chiratidzo cheganda	Maculopapular maronda (urticaria pigmentosa) anowanikwa mune mamwe maitiro	Hapana chaiyo yekuongorora ganda ronda

Kuti zvinosimbiswa sei

Uchapupu hwechinangwa chekuwedzera kwesero

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.

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.

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.

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.

Inokurudzirwa yekuona

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.

MCAS idambudziko remast cell isina kufanira activation, kwete nhamba dzemast cell.
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
Mastocytosis inosanganisira kuwanda kwemasero uye inogona kuratidzwa zvakananga; MCAS haigone, ndosaka kuongororwa kuri musanganiswa.
Mast cells maseru edziviriro anobatsira zviri pamutemo akaiswa pamivharo yemuviri uye akavakwa kuti apindure mukati memasekonzi.

Chikamu 02

Kunzwisisa Mast Cells

Negative allergy kuyedzwa kwakajairika muMCAS uye hazvibvire.

Origin uye kukura

Mast cells anotangira mumwongo, zvichibva kuCD34-positive haematopoietic progenitor cells - uwandu humwe chetehwo 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.

Origin uye kukura

Kugovera muviri wose

Mast cells anogovaniswa zvine hungwaru kwete zvakafanana, achitarisa uko muviri unosangana nenyika yekunze 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
Kugovera muviri wose	Mast 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
Nzvimbo	Basa muhutano	Zviratidzo kana activation isina kufanira
Ganda uye dermis	Barrier defense; mhinduro pakurumwa, kurumwa, nekukuvara	Kuputika, kuputika, mikoko, dermographism, kuzvimba
Gastrointestinal turakiti	Kugadziriswa kwezvipingamupinyi, motility regulation, kupindura kune kutyisidzira kwakapinzwa	Kupwanya, kurutsa, reflux, manyoka, kuvimbiswa, kuputika
Airways uye sinuses	Kudzivirirwa kwetambo yekufema	Congestion, rhinitis, kuoma kwehuro, kufema, kukosora
Around tsinga dzeropa	Kudzora kwechikepe toni uye permeability	Kuchinja kweropa, tachycardia, flushing, presyncope
Brain uye meninges	Ipa perivascularly uye mune meninges; neuroimmune chiratidzo	Kuziva mhute, kutemwa nemusoro, dzungu (maitiro achiri pasi pekudzidza)
Connective tishu	Kugadziridza nekugadzirisa matishu	Majoini uye tsandanyama kurwadziwa; zvinokwanisika kuenderana nekubatanidza tishu kupindirana

Mwongo

Nzvimbo panobva masero ekutanga

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.

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

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.
KIT - receptor ye stem cell factor; pakati pemast cell kupona, maturation, uye kugara kwematishu.
Adenosine, opioid, estrogen uye mamwe receptors - kubatsira kune akasiyana uye ega ega anokonzeresa profiles anoonekwa mukuita.

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.

Inokurudzirwa yekuona

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.
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.
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.
Kuratidzira kweKIT/SCF kuri pakati pekukura kwavo - nzira yakakanganiswa mune yakawanda yevakuru systemic mastocytosis.

Chikamu 03

Chii Chinoitika Panguva
Mast Cell Activation

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

Trigger

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

Trigger

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

Activation

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

Degranulation

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

Kusunungurwa kwemurevereri

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

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.

Key Concept

Mast masero anoburitsa varevereri 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.

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

Varevereri vakuru uye zvinoitwa nemumwe nemumwe

Mast cells anokwanisa kuburitsa huwandu hwakakura kwazvo hwezvinhu. Aya anotevera ndiwo anonyanya kukurukurwa nekiriniki, uye pamwe chete vanoverengera mazhinji echiratidzo chechiratidzo chechiratidzo.

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 nemumwe	Mast 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
Histamine	Rudzi	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.
Histamine	Rudzi	Pre-formed
Prostaglandin D2	Ichangobva synthesized	Mapuroteni 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.
Leukotrienes (LTC4, LTD4, LTE4)	Ichangobva synthesized	Yakasimba bronchoconstrictor, zvakanyanya zvakanyanya kupfuura histamine nehuremu. Kuwedzera vascular permeability uye mucus kugadzirwa, uye kubatsira kune zvose mhepo uye gastrointestinal zviratidzo. LTE4 inoyerwa muweti.
Cytokines (IL-6, TNF-α, IL-1, IL-4 nevamwe)	Ichangobva synthesized	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.
Chemokines	Ichangobva synthesized	Tora mamwe maseru edziviriro kune saiti, achikudza uye kurebesa mhinduro yekuzvimba kupfuura yekutanga mast cell reaction.
Platelet activating factor (PAF)	Ichangobva synthesized	Iyo yakasimba kwazvo lipid murevereri iyo inowedzera vascular permeability uye inogona kukonzera yakanyanya hypotension. Inobatanidzwa mukuoma kweanaphylactic reactions uye isina kuvharwa ne antihistamines.
Heparin	Rudzi	Anticoagulant inowanzoitika. Inogona kubatsira mukukuvadzwa kusingajairike kana kubuda ropa kunotaurwa nevamwe varwere, uye zvakare kutora chikamu mumutemo wemuno wekuzvimba.
Chymase & carboxypeptidase A3	Rudzi	Granule proteases inosanganisirwa mukugadzirisa matishu, nzira dzekudzora BP, uye kushatiswa kwehumwe huturu nemapeptide.

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

Inokurudzirwa yekuona

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.

Granule proteases inosanganisirwa mukugadzirisa matishu, nzira dzekudzora BP, uye kushatiswa kwehumwe huturu nemapeptide.
Pre-formed varevereri vanosunungurwa mumasekondi; lipid varevereri mumaminitsi; cytokines kwemaawa akawanda.
Mast masero anogona kusunungura vayananisi zvakasarudzwa pasina degranulation yakazara.
Kushanda kunotevera kutevedzana: trigger, activation, degranulation, murevereri kusunungurwa, zviratidzo.
Pre-formed varevereri vanosunungurwa mumasekondi; lipid varevereri mumaminitsi; cytokines kwemaawa akawanda.

Chikamu 04

Zviratidzo zve MCAS

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

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 mucosal
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.	Notes
Ganda uye mucosal	Chiratidzo
Notes	Flushing
Kazhinji kamwe kamwe, kukanganisa chiso, mutsipa, uye chipfuva; chinhu chinogara chichitaurwa	Kuvava (pruritus)
Inogona kuitika kana pasina kuputika kunoonekwa	Mikoko (urticaria)
Yakasimudzwa, inovava mawisi; inogona kuva yenguva pfupi uye inotama	Dermographism
Welts inosimudzwa nekupuruzira kwakasimba kweganda - chiratidzo chemuviri chinozivikanwa	Angioedema

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.	Notes
Kunonoka kupora ronda	Zvakataurwa nevamwe varwere; nzira dzisina kunyatsogadzirwa
Kufema	Nasal congestion uye rhinitis
Kazhinji zvisingaperi uye zvisiri zvemwaka	Kuoma kwehuro
Inoda kukurumidza kuongororwa kana ichifambira mberi - inogona kuratidza anaphylaxis	Wheeze uye kupfupika kwekufema
Leukotriene-driven bronchoconstriction inzira inozivikanwa	Chikosoro 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.	Notes
Kunzwa kunhuhwirira uye makemikari emhepo	A anowanzo kushumwa trigger-symptom pairing
Cardiovascular	Tachycardia
Kumhanya mwoyo, kazhinji pasina kushanda nesimba	Kusagadzikana kweropa
Inogona kutenderera mune chero nzira panguva yezvikamu	Presyncope uye syncope
Kuchena musoro kana kupera simba, kazhinji pakumira	Chifuva 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.	Notes
Anaphylaxis	Zvakaoma, nekukurumidza, zvinogona kuisa hupenyu panjodzi - ona chikamu chekukurumidzira
Gastrointestinal	Abdominal cramping uye kurwadziwa
Pakati pezvinonyanya kutaurwa zviratidzo zvakazara	Nausea uye kurutsa
Inogona kutevera kudya kana kuitika yakazvimirira	Histamine's H2 mhedzisiro pagastric acid ndiyo inozivikanwa inopa
Motility inogona kusiyana pakati pezvikamu uye nekufamba kwenguva	Inowanzo fambisa kurambidzwa kwekudya - ona yambiro muchikamu 9

Neurological uye cognitive
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.	Notes
Kuputika uye reactivity yezvokudya	Kunetseka nekuisa pfungwa, kurangarira, uye kutsvaga mazwi
Neurological uye cognitive	Kuvhiringidzika kwepfungwa
Kunetseka nekuisa pfungwa, kurangarira, uye kutsvaga mazwi	Musoro uye migraine
Migraine inoshumwa pamwero wepamusoro muhuwandu uhu	Dzungu
Kazhinji inopindirana nemoyo uye autonomic features	Kuneta
Inowanzo kuomarara uye pakati pezvinonyanya kuremara zvinoshumwa zviratidzo	Kunzwa 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.	Notes
Manzwiro etsinga asina kujairika	Inogona kupindirana neakabatana tishu mamiriro akakurukurwa muchikamu 7
Musculoskeletal, genitourinary, uye zvepfungwa	Majoini uye tsandanyama kurwadziwa
Kukurumidzira dundira uye kuwanda	Kurwadza kwebhonzo
Waranti yekuongorora, kunyanya uko mastocytosis iri kutariswa	Kukurumidzira dundira uye kuwanda
Interstitial-cystitis-kufanana zviratidzo zvinoshumwa	Zviratidzo zvinosiyana nekuenda kumwedzi
Hormonal pesvedzero pamasero mast inonyorwa	Kuzvidya mwoyo-sezvikamu

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.

Kuchinja kwemaitiro uye kushatirwa

Zvose kubva pamigumisiro yemuyananisi uye kubva pakurarama nechirwere chisingaperi, chisingazivikanwi

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.
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.

Inokurudzirwa yekuona

Mepu yemuviri wechiratidzo: ratidziro 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.
Zviratidzo zvinotora ganda, kufema, moyo, GI, neurological, musculoskeletal, genitourinary, uye psychological domains.
Kubatanidzwa kwemaviri kana anopfuura organ system chikamu cheiyo diagnostic pateni.

Chikamu 05

Common Zvikonzero

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

Key Concept

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

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.

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

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

Stress

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.

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.

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.

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.

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.

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.

Inokurudzirwa yekuona

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.

Sei zvinokonzeresa zvakasiyana zvakanyanya
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.
Inokonzeresa stack - kuwedzera mutoro unokosha kupfuura chero kuratidzwa kumwe chete.
Zvikonzero zvemuviri (kupisa, kutonhora, kudzvanywa, kushingaira) hazvidi chinhu chakamwiswa uye hazvionekwe pakuongororwa kweallergy.

Chikamu 06

Nei MCAS iri
Zvakaoma ku Kuongorora

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

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.

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

Variable zviratidzo

Intermittent mediator release

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.

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.

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

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.

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.

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.

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.

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.

Diagnostic gakava

Inokurudzirwa yekuona

Diagnostic kunonoka
Vayananisi ndevenguva pfupi uye havana kugadzikana, saka nguva uye sampuli yekubata inotyaira akawanda enhema.
Zviratidzo zvinopindirana nemamwe akawanda akajairika mamiriro, zvichigadzira chidimbu chechirwere.
Kuongororwa marabhoritari kunowanzojairika muMCAS; nyanzvi yekuongorora muyananisi inodiwa.
Vayananisi ndevenguva pfupi uye havana kugadzikana, saka nguva uye sampuli yekubata inotyaira akawanda enhema.

Chikamu 07

Associated Conditions

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

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)

Verenga izvi kutanga

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.

Hereditary alpha tryptasemia (HaT)

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

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.

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.

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

Simba rehumbowo nekungotarisa
Autoimmune mamiriro	Mamiriro ehukama	Simba rehumbowo nekungotarisa
Condition	Mamiriro ehukama	Mamiriro ehumbowo
Chisingaperi urticaria	Mast cell activation iri pakati pehosha pachayo	Mamiriro ehumbowo
Yakagadzirwa michina	Asthma	Mast cell mediators anodzinga bronchoconstriction
Hereditary alpha tryptasemia	Genetic hunhu hunosimudza baseline tryptase; kunokanganisa kududzira kwebvunzo	Yakasimbiswa hunhu; hukama hwechiratidzo huchiri kujekeswa
POTS	Zvinowanzoitika pamwe chete; murevereri mhedzisiro pane vascular toni inokurudzirwa	Sangano rakashuma; nzira isina kugadziriswa
Migraine	hEDS / hypermobility	Zvinowanzoitika pamwe chete; connective tishu kubatanidzwa kunokurudzirwa
Sangano rakashuma; mechanic hypothetical	Mitengo yakakwira yakashumwa; vasoactive mediators zvinonzwisisika	Sangano rakashuma; nzira iri kudzidza
IBS	Chiratidzo chinopindirana; gut mast cell zvakawanikwa zvakasiyana	Kupindirana 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 chirwere	Co-occurrence yakashumwa; yakagoverwa immune dysregulation yakarongwa

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.

Chikamu 08

Kunzwisisa Kwazvino
ye Zvinokonzera

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

Simba rehumbowo nekungotarisa

Hapana chikonzero chimwe cheMCAS chakagadzwa. Zvinhu zviri pasi apa inzvimbo dzekuferefeta dzine matanho akasiyana erutsigiro. 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 inosimudza 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.

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.

Genetics

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.

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.

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.

Kusashanda zvakanaka kweimmune system

Kuzvimba kwenguva refu

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

Inokurudzirwa yekuona

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.

Apa panosiya zvinhu
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.
Hapana chikonzero cheMCAS chakagadzwa.
Bata zvichemo zve 'mudzi wechikonzero' nekukahadzika - nharaunda yekutsvagisa haisati yazivisa imwe.

Chikamu 09

Kugara Na MCAS

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

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.

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

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.

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.

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.

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.

Kushanda nevarapi

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.

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.
Epinephrine ndiyo yekutanga-mutsara kurapwa kweanaphylaxis. Antihistamines haisi inotsiva uye haifanirwe kuvimbwa nayo mukuitika kwakanyanya.
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.

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.

Chirevo pamusoro pekurambidzwa kwekudya

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.

Chirevo pamusoro pekurambidzwa kwekudya
Dhayari yechiratidzo yakarongeka kwemavhiki ndiyo nzira yakavimbika yekuziva zvinokonzeresa.
Kupa zviratidzo neorgan system inoita kuti multisystem pateni ionekwe kuvarapi.
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.

Chikamu 10

Inowanzo bvunzwa Mibvunzo

25 mibvunzo varwere, mhuri, uye varapi vanobvunza kazhinji. Sarudza chero mubvunzo kuti uwedzere.

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MCAS inhaka here?

Zviratidzo zvinogona kuvandudza zvakanyanya, dzimwe nguva kusvika apo varwere vanozvitsanangura sezvisina symptomatic - kunyanya kana kurapwa kwadzikisira kwekutanga reactivity uye zvinokonzeresa zvakaonekwa. Kuzviitira, kugadzirisa zvachose pasina manejimendi hakuna kunyorwa zvakanaka. Nguva dzekuregererwa dzinoteverwa nekudzoka dzinowanzotsanangurwa.

Zvinhu zvakawanda zvinoshanduka nekufamba kwenguva: yekutanga reactivity inofamba nekurara, kushushikana, mamiriro ehomoni, uye hutachiona huchangobva kuitika; mast masero anogona kuburitsa musanganiswa wakasiyana wemurevereri panguva dzakasiyana; uye trigger inoisa pachayo kuwedzera panguva yekutadza kutonga uye kazhinji kutetepa zvakare nekurapa. Kuchinja kwezviratidzo kunoratidza mamiriro acho pane chiratidzo chokuti kuongororwa kwakaipa.

Kazhinji nekuda kwehunyanzvi zvikonzero. Vayananisi vakayerwa vane hafu yeupenyu hupfupi, saka sampling pakati pemapinduriro inogona kuwana hapana chakakwira. Dzakawanda zvakare hadzina kugadzikana patembiricha yekamuri uye dzinoda kutonhora nekukurumidza uye nekukasira kugadzirisa - sampuli yakagara kunze kana yakanonoka mukufamba inogona kuverenga zvakajairwa kubva kumurwere ainyatsoita.Basa reropa renguva dzose uye allergy mapaneru anotarisirwawo kuve akajairwa muMCAS, sezvo asina kugadzirwa kuti azvione.

Hongu, kuburikidza nenzira yakanyorwa yefiziology. Mast cells anotakura mareceptor ecorticotropin-releasing hormone uye anogara pedyo nemagumo etsinga, achipa kushushikana kwefiziology kuwana kwakananga kwavari. Izvi zvakakosha kutaura zvakajeka, nekuti varwere vane zviratidzo zvinowedzera pasi pekunetseka vanowanzoudzwa mamiriro acho epfungwa. A neuroimmune nzira haina kufanana nechiratidzo chiri kufungidzira.

Kwete. Kuchinja kwezvokudya kunogona kuderedza mutoro wezviratidzo kune vamwe varwere nekuderedza mutoro wose we trigger, uye iyo inobatsira inofanirwa kutevera nekufungisisa - asi haiporesi mamiriro acho ezvinhu. Humbowo hwekudya kwakaderera-histamine hushoma uye hwemhando ine mwero. Kurebesa kurambidzwa kusingatarisirwe kunotakura njodzi chaidzo dzekushaikwa kwekudya uye kusagadzikana kwekudya, saka yakarongeka, yenguva-inogumira muyedzo ine kudzoreredzwa yakachengeteka pane yakavhurika-yakapera kudzivirira.

Mu mastocytosis muviri unounganidza masero akawanda akawandisa, uye kuti kuwandisa kunogona kuratidzwa - kuburikidza nemwongo biopsy, maitiro eganda maronda, kana anoramba akakwidziridzwa baseline tryptase, kazhinji neKIT D816V mutation muvakuru systemic chirwere. MuMCAS nhamba yemasero mast ndiyo yakajairika; chiri abnormal ndechekuti vanokurumidza kuita activate. Ichi ndicho chikonzero mastocytosis inogona kusimbiswa nekutsvaga maseru uye MCAS haigone.

Aiwa, kunyange zvazvo vachiwanzovhiringidzika. Histamine kusashivirira kunowanzo kutsanangurwa sekunetseka kuputsa kumedzwa histamine, kazhinji kunokonzerwa nekudzikiswa kwekuita kwe enzyme diamine oxidase. MCAS inosanganisira masero emuviri wega mast anoburitsa histamine uye mamwe akawanda anoyananisa zvisina kufanira. Zvinogona kuburitsa zviratidzo zvinopindirana uye zvinogona kuvapo, asi maitiro anosiyana.

Apa ndipo chaipo apo nyanzvi dzinopokana. Maitiro ekubvumirana anoda humbowo hwechinangwa chekusunungurwa kwemuyananisi, uye varapi vazhinji vanobatirira pane izvo chaizvo. Vamwe vanopokana kuti kunyorera kwakasimba kunopotsa varwere vane bvunzo dzinoramba dzichikundikana nekuda kwenguva uye kubata zvikonzero. Iyo gakava harina kugadziriswa mumabhuku, ndosaka nyanzvi mbiri dzinogona kusvika pamhedziso dzakasiyana nezvemurwere mumwechete.

Allergists uye immunologists kazhinji, uye hematologists uko mastocytosis iri kufunga. Mukuita, kujairana kunosiyana zvakanyanya kunyangwe mukati meiyo hunyanzvi, uye kuwana kune varapi vane mast cell ruzivo hakuna kuenzana nenzvimbo. Varwere vazhinji vanoona kuti murapi ane ruzivo rwakakwana anoda kurongedza kutarisirwa akakosha sekugadzwa kwenyanzvi.

Vanowanzo batsira asi kashoma kugadzirisa zvese, uye kuti mhinduro isina kukwana inotarisirwa pane chiratidzo chekuti kuongororwa kwakaipa. Antihistamines inodzivirira histamine chete; prostaglandins, leukotrienes, platelet activating factor, uye cytokines hazvibatsiri. Ichi ndicho chikonzero nei kurapwa kuchikwira mumatiyeri, kuwedzera vamiririri vanogadzirisa vanoyananisa antihistamines vanopotsa.

Tsananguro mbiri dzakajairika. Mamwe makirasi emishonga anogona kushandura mast masero zvakananga, akazvimirira pane chero allergic mechanism. Zvakaparadzana, zvisingashande zvinosanganisa - madhayi, mafirita, zvinodzivirira - zvinogona kuve chikamu chinotsamwisa kwete chemushonga unoshanda, ndicho chikonzero murwere anogona kushivirira shanduro yemugadziri uye kuita kune yemumwe. Kugadzirira kwakasanganiswa dzimwe nguva kunoshandiswa apo izvi zvinofungidzirwa.Kurukura chero zvaunofungira kuita neanokupa mushonga pane kungomira unilaterally.

Zvinogona kudaro, zvichienderana nekuomarara uye nehurongwa hwemutemo kwaunogara. Vamwe varwere vanoshanda uye vanodzidza pasina kugadzirisa; dzimwe dzakaganhurirwa zvikuru. Zvisungo zvekuremara ndezvekutonga-zvakananga uye zvinowanzoda zvinyorwa zvekushanda kwemaitiro kwete kuongororwa chete. Nyaya yakanyorwa nanachiremba yezvisingakwanisi kazhinji inobatsira kudarika magwaro ekuongorora ari ega.

MCAS hurema here?

Zviitiko zvakashumwa zvinosiyana zvakanyanya - vamwe varwere vanotsanangura kuvandudzwa panguva yekuzvitakura, vamwe vachiwedzera, uye vamwe hapana shanduko yakajeka. Hormonal pesvedzero pamasero mast inonyorwa, izvo zvinoita kuti shanduko mune chero nzira ive inonzwisisika. Kubata pamuviri neMCAS kunopa mvumo yekuronga kumberi nevese mast cell kiriniki yako uye timu yako yekuzvara, kunyanya maererano nemishonga ichaenderera mberi.

Nekuti mast masero anopindura kurudziro yemuviri zvakananga, kwete chete kune allergens. Kupisa, kutonhora, kudzvanywa, kupokana, uye kushanda nesimba zvese zvinogona kuzvimutsa pasina chinhu chinopinda mumuviri. Kuongororwa kweAllergy kwakagadzirirwa kuona IgE-mediated sensitization kune chaiwo mapuroteni, saka haigone kuona izvi zvinokonzeresa - ndosaka yakachena allergy pani ichinyatsoenderana nekuita kwechokwadi pakupisa.

Zvinoenderana nemumiririri. Antihistamines inowanzoratidza kushanda mukati memazuva. Mast cell stabilizers akadai secromolyn neketotifen anowanzoda mavhiki ekushandiswa nguva dzose kusati kwanyatsobuda pachena, uye kumira nekukurumidza inzira yakajairika yekurasa chimwe chinhu chingadai chashanda. Muedzo wemavhiki mana kusvika matanhatu pakuchinja kwega kwega mutemo unowanzo taurwa wegunwe.

Kazhinji aiwa, uye iyi ndeimwe yezvikanganiso zvinowanzoitika pakuzvitonga. Kutanga vamiririri vakati wandei pamwechete kunoita kuti mhedzisiro isadudzirke: kana zvinhu zvikafamba zvakanaka, haugone kuziva kuti ndeipi shanduko yakakonzeresa, uye kana ukaita, haugone kutaura yekumisa. Imwe shanduko panguva inononoka asi ndiyo inoita kuti nzira yacho iverengeke.

Varwere vazhinji vanosangana nechiratidzo chekutanga kwechirwere chinotapukira, uye hutachiona hunozivikanwa kumutsa maseru mast kuburikidza neToll-like receptors, saka nzira yakarongwa inonzwisisika. Kunyangwe hutachiona huchikonzera MCAS haina kusimbiswa, uye kuratidza causation mune imwe neimwe nyaya kwakaoma nzira. Nezve Yakareba COVID chaizvo, mast cell kubatanidzwa ndeye inoshanda hypothesis ine humbowo hwekutanga, kwete yakagadziriswa kuwanikwa.

Inowanikwa kakawanda muvakadzi. Estrogen inokanganisa mast cell maitiro, inova imwe tsananguro inotsanangurwa, uye varwere vazhinji vanorondedzera cyclical zviratidzo maitiro. Kunyangwe mutsauko unoratidza kuwanda kwechokwadi kana mutsauko mukuongororwa uye kutumirwa hakuna kugadziriswa zvizere - caveat inoshanda kune akati wandei mamiriro anoonekwa zvakanyanya muvakadzi.

Huwandu hwakatendeseka hwekupararira hausati hwavapo. Mamiriro acho akaratidzirwa zviri pamutemo muna 2007 chete, maitiro ekuongorora anoramba achikakavadzana, uye maitiro anosiyana pakati penyika. Zvakadhindwa fungidziro dzinosiyana zvakanyanya, uye hupamhi hwehuwandu ihwohwo ndihwo hutsvagiro. Iva nekukahadzika nezvehuwandu hwakasimba hwehuwandu kubva kune chero kupi.

Zvakawanda sei MCAS?

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.

Patient Guides

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
Nhanganyaya murwere gwara - link kuti iwewedzerwe

Medical Guidelines

Mazwi ekubvumirana uye gwara rekiriniki rakaitirwa vashandi vehutano. Inobatsira kuunza kumaaphoyintimenti.

Nhungamiro yenhengo dzemhuri nevachengeti - link kuti iwewedzerwe
Nyanzvi munzanga yekiriniki kutungamira - link kuti iwewedzerwe
Muyananisi kuyedzwa uye muenzaniso wekubata protocol - link kuti iwewedzerwe
Consensus diagnostic maitiro - citation uye link kuti zviwedzerwe

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.

Ongororo yemamiriro akabatana - zvinyorwa zvinofanira kuwedzerwa
Mast cell chirwere chekutsvaga network - links kuti zviwedzerwe
Zvirwere zvisingawanzo masangano - links kuti zviwedzerwe

Mabhuku

Kuverengera kwenguva refu kwevarwere nevarapi.

Zvirwere zvisingawanzo masangano - links kuti zviwedzerwe
Clinical reference zvinyorwa - kuwedzerwa

Kuverengera kwenguva refu kwevarwere nevarapi.

Hurukuro dzakarekodhwa, vatsananguri, uye zvinyorwa zvemusangano.

Clinical reference zvinyorwa - kuwedzerwa
Nyanzvi dzemusangano hurukuro - links kuti zviwedzerwe
Tsanangudzo yeMCAS yezvidzidzo zvemukati - links kuti zviwedzerwe

Masangano Anotsigira

Rutsigiro rwevezera, kumiririra, uye nharaunda.

Tsanangudzo yeMCAS yezvidzidzo zvemukati - 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.

Mapoka ekutsigira mudunhu nemunyika - links kuti zviwedzerwe
Parizvino kunyorera mast cell zvidzidzo - links kuti zviwedzerwe
Nhungamiro pane izvo kutora chikamu muyedzo - link kuti iwewedzerwe

Chikamu 12

Parizvino kunyorera mast cell zvidzidzo - links kuti zviwedzerwe

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.
Mafungiro akanyanya kukosha kubva kugwaro iri, akapfupikiswa.
Muchidimbu
Varevereri vanoburitswa mumafungu: pre-yakaumbwa mumasekonzi, lipid mediators mumaminitsi, cytokines kwemaawa. Imwe activation chiitiko inogona kutora zuva.
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.
Mast cell kubatanidzwa ndiko yakasimbiswa mune chisingaperi urticaria uye asima; kushamwaridzana nePOTS, hEDS, IBS uye Long COVID zvinotaurwa asi mechanistic isina kugadziriswa.
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.
Apo ngozi yeanaphylaxis inoshanda, epinephrine uye chirongwa chekuita chakanyorwa zvakakosha; antihistamines haisi chinotsiva.
Hapana chikonzero cheMCAS chakavambwa - chengetedza 'mudzi wechikonzero' zvikumbiro nekufungira.

Chikamu 13

Tsanangudzo yemashoko

91 mazwi anoshandiswa muMCAS mabhuku uye hurukuro yekiriniki, inotsanangurwa zvakajeka.

A B C D E F G H I K L M N O P R S T U V W

Adrenaline

Ona Epinephrine. Iro zita reBritish uye repasirese risiri-risiri remuridzi wehomoni imwechete nemishonga.

Kuchena musoro kana kupera simba, kazhinji pakumira

Yakasimudzwa, inovava mawisi; inogona kuva yenguva pfupi uye inotama

Adrenaline

Ona Epinephrine. Iro zita reBritish uye repasirese risiri-risiri remuridzi wehomoni imwechete nemishonga.

Mushonga unovhara histamine receptors. H1 blockers vanoita zvakanyanya paganda uye zviratidzo zvemhepo; H2 blockers inoshanda zvakanyanya padumbu remudumbu.

Kuzvimba munzvimbo dzakadzika dzeganda kana mucosa, kunowanzo kanganisa miromo, maziso emaziso, maoko, kana huro. Kubatanidzwa kwehuro idambudziko rezvokurapa.

Antihistamine

Kuwanda kwemasero

Autonomic nervous system

Chikamu chetsinga dzetsinga dzinodzora mabasa asingadiwe akadai sekurova kwemoyo, BP, uye kugaya. Kusashanda kwayo kunonzi dysautonomia.

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.

Carboxypeptidase A3

Kutetepa kwenzira dzemhepo nekuda kwekuomesera kwemhasuru yakatsvedzerera yakatenderedza, ichiburitsa kuhwihwidza uye kufema. Leukotrienes vatyairi vane simba.

Iprotein inosaina iyo inotora mamwe maseru edziviriro kune saiti, inosimudzira uye nekurebesa mhinduro yekuzvimba.

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.

Kuyerera kwemapuroteni eropa kuumba chikamu chekudzivirira kwekuzvarwa. Zvikamu zvaro C3a uye C5a zvinogona kumisa mast masero zvakananga.

Corticotropin-releasing hormone (CRH)

Complement system

Cromolyn sodium

Iyo mast cell stabilizer inoderedza kusunungurwa kwemurevereri pane kuvharira muyananisi mhedzisiro. Oral formulations inoshanda zvakanyanya pane gastrointestinal turakiti.

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.

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.

Nzira yakarongeka yekubvisa zvinofungidzirwa kuti zvinokonzeresa zvikafu uye kuzviunza zvakare zvakarongeka. Inotakura njodzi yekudya kana ikarebeswa pasina kutarisirwa.

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.

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.

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

Sero remwongo risati rakura rinokwanisa kukura kuva marudzi emasero eropa. Mast cells anosimuka kubva kuCD34-positive progenitors.

Heparin

Hematopoietic progenitor

Hereditary alpha tryptasemia (HaT)

Chimiro chemajini chinosanganisira mamwe makopi eiyo TPSAB1 gene, ichigadzira inoenderera yakasimudzwa yekutanga tryptase. Kugara nhaka mune autosomal dominant pateni.

Heparin

Anticoagulant inowanzoitika yakachengetwa mumast cell granules. Inogona kukonzera maronda asina kujairika kana kubuda ropa mune vamwe varwere.

Histamine kusashivirira

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.

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.

Chikamu che cytokine chinoshandiswa kutaurirana pakati pemasero ekudzivirira. IL-6 uye IL-4 ndedzimwe dzakaburitswa nemaseru maseru.

Ruoko rwekukurumidza, rusina-chaiyo rwemasoja edziviriro aripo kubva pakuzvarwa. Mast cells inhengo.

Interleukin

Mushonga une zvese antihistamine uye mast cell inodzikamisa zvivakwa. Kazhinji zvinoda mavhiki ekushandiswa kwakafanana kusati kwazara.

Ketotifen

KIT D816V

KIT

A receptor pane mast cell surface inosunga stem cell factor. Pakati pemast cell kukura, kurarama, uye kugara kwematishu.

Leukotriene

Iyo chaiyo shanduko muKIT gene inowanikwa mune yakawanda yevakuru systemic mastocytosis. Haisi chikonzero chakasimbiswa cheMCAS.

Leukotriene E4 (LTE4)

Leukotriene

A lipid murevereri akagadzirwa mushure mekuita activation. Yakasimba bronchoconstrictor iyo inowedzerawo vascular permeability uye mucus kugadzirwa.

Muyananisi akavakwa kubva musero membrane lipids mushure mekuita activation pane kuchengetwa pamberi. Prostaglandins uye leukotrienes ndiyo mienzaniso mikuru.

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.

Mast cell

MCAS haisi mastocytosis

Chimiro chinosanganisira kuunganidzwa kweakawandisa mast masero munyama, kazhinji inofambiswa neKIT D816V shanduko mune yevakuru systemic chirwere. Yakasiyana kubva kuMCAS.

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.

Ma membranes akakomberedza uropi uye musana. Mast masero aripo pano, izvo zvinoenderana nekutsvaga nezvezviratidzo zvetsinga.

Chero kemikari inoburitswa nesero remast inoburitsa mhedzisiro pane yakatenderedza tishu. Inosanganisira histamine, tryptase, prostaglandins, uye vamwe vakawanda.

Menings

MRGPRX2

Montelukast

A leukotriene receptor antagonist anoshandiswa kugadzirisa kufema uye egastrointestinal zviratidzo izvo antihistamines hazvisviki.

Iwo matishu akanyorova anonamira mukati memukati senge ura, mhepo, uye mhino. Izere nevanhu vane mast cells.

A mast cell receptor mediating IgE-yakasununguka activation nemimwe mishonga uye peptides. Inobatsira kutsanangura maitiro pasina inotaridza IgE kubatanidzwa.

Mucosa

Histamine metabolite yakayerwa mumaawa makumi maviri nemana ekuunganidza weti sehumbowo hwekuburitswa kwemast cell mediator.

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.

Omalizumab

A biologic therapy yakanangana neIgE, inoshandiswa mune dzimwe nyaya dzinoramba dziri pasi pekutariswa kwenyanzvi.

Palpitations

Kuziva kurova kwemwoyo, kunowanzotsanangurwa sekumhanya, kurova, kana kusajairika.

Perivascular

Platelet activating factor (PAF)

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.

Prostaglandin D2

Presyncope

Kunzwa kwekuda kufenda, pasina kurasikirwa nekuziva.

Izwi rekurapa rekukwenya.

A lipid murevereri anokonzera kuoneka vasodilation, flushing, bronchoconstriction, uye manyoka. Kakawanda kwakakurumbira uko kutsvaira kunotonga.

Pruritus

Izwi rekurapa rekukwenya.

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.

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.

Chimiro che mastocytosis umo masero akawandisa anoungana munhengo dzemukati, kazhinji neKIT D816V shanduko.

Kukanganisa muviri wose pane imwe nzvimbo yenzvimbo.

Kunzwa kunhuhwirira uye makemikari emhepo

Systemic mastocytosis

Chikumbaridzo

Kurova kwemoyo kusingaite, kunowanzo kutaurwa panguva yemast cell reaction.

Chikumbaridzo

TPSAB1

Toll-like receptor

Trigger

A receptor inoziva mapatani ane hukama nehutachiona, inobatanidza mast cell activation kune hutachiona.

Tryptase

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.

Urticaria

Mikoko - yakasimudzwa, yakafunuka welts inokonzerwa nemurevereri kusunungurwa muganda. Chronic spontaneous urticaria inosanganisira mast cell activation zvakananga.

Urticaria pigmentosa

Kuva nemhedzisiro pamudziyo weropa dhayamita kana kupera. Histamine uye prostaglandin D2 zvine vasoactive zvakasimba.

Vasoactive

Vasodilation

Kuwedzera kwetsinga, kuwedzera kuyerera kweropa uye kuderedza BP. Inogadzira flushing uye inogona kukonzera light-headedness.

Kunzwisisa Mast CellActivation Syndrome(MCAS)

Chii MCAS?

Sei mast cells aripo zvachose

Why mast cells aripo zvachose

Izvo zvakajairika mast cell function inotaridzika

Uchapupu hwechinangwa chekuwedzera kwesero

Kunzwisisa Mast Cells

Origin uye kukura

Kugovera muviri wose

Nzvimbo panobva masero ekutanga

Hupenyu kutenderera

Chii Chinoitika PanguvaMast Cell Activation

Varevereri vakuru uye zvinoitwa nemumwe nemumwe

Zviratidzo zve MCAS

Kuchinja kwemaitiro uye kushatirwa

Common Zvikonzero

Zvokudya

Kupisa

Stress

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.

Nei MCAS iriZvakaoma ku Kuongorora

Variable zviratidzo

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

Intermittent mediator release

Kubatana nezvimwe zvirwere

Kushaikwa kwekuziva kwachiremba

Diagnostic gakava

Associated Conditions

Postural Orthostatic Tachycardia Syndrome (POTS)

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

Hereditary alpha tryptasemia (HaT)

Hereditary alpha tryptasemia (HaT)

Migraine

Asthma uye chisingaperi urticaria

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.

Kunzwisisa Kwazvinoye Zvinokonzera

Genetics

Genetics

Kusashanda zvakanaka kweimmune system

Kuzvimba kwenguva refu

Kugara Na MCAS

Kutarisira kwezuva nezuva uye maitiro

Kutarisira kwezuva nezuva uye maitiro

Kushanda nevarapi

Utano hwepfungwa

Chirevo pamusoro pekurambidzwa kwekudya

Inowanzo bvunzwa Mibvunzo

Kuwedzera Kudzidza Zviwanikwa

Patient Guides

Medical Guidelines

Mapepa Okutsvakurudza

Professional Masangano

Mabhuku

Kuverengera kwenguva refu kwevarwere nevarapi.

Masangano Anotsigira

Clinical Miedzo

Parizvino kunyorera mast cell zvidzidzo - links kuti zviwedzerwe

Tsanangudzo yemashoko

Iwe Hausi Ndega

Kunzwisisa Mast Cell
Activation Syndrome
(MCAS)

Chii Chinoitika Panguva
Mast Cell Activation

Nei MCAS iri
Zvakaoma ku Kuongorora

Kunzwisisa Kwazvino
ye Zvinokonzera