Gqibezela iSikhokelo seMfundo

Ukuqonda Mast Cell
Activation Syndrome
(MCAS)

Funda isayensi emva kweeseli zemast, kutheni zisebenza ngokugqithisileyo, indlela i-MCAS ewuchaphazela ngayo umzimba, kunye nolwazi olusisiseko sonke isigulana, umkhathaleli, umfundi, kunye nengcali yezempilo kufuneka iqonde.

Icandelo le-01

Yintoni i MCAS?

I-Mast Cell Activation Syndrome luphazamiseko lokhuselo lomzimba ukuziphatha endaweni yokuzikhusela ubungakanani. Iiseli zikhoyo ngamanani aqhelekileyo kwaye zijongeka ziqhelekile-into etshintshileyo yindlela ezidubula ngayo ngokulula.

I-Mast Cell Activation Syndrome (MCAS) yimeko apho iiseli zemast - iiseli zomzimba ezihlala phantse kuzo zonke izihlunu zomzimba - zisebenze ngokungafanelekanga kwaye ziphindaphinda, zikhupha abalamli beekhemikhali ngobuninzi kwaye ngamanye amaxesha abenzi njongo yokuzikhusela. Isiphumo singumzekelo wokuphindaphinda, iimpawu ze-episodic ezichaphazela iinkqubo ezimbini okanye ngaphezulu kwamalungu kanye.

Inkcazelo ibalulekile kuba ibeka i-MCAS ngokwahlukileyo kwiimeko eziyifanisayo. Akukho ngqokelela ingaqhelekanga yeeseli ukuze ifunyanwe kwi-biopsy, akukho allergen enye yokuchonga kuvavanyo lwe-skin-prick, kwaye rhoqo akukho kungaqhelekanga konke konke kumsebenzi wesiqhelo waselabhoratri. Ukuphazamiseka kuhlala embundwini apho iiseli ezibukeka ngokuqhelekileyo zithatha isigqibo sokuphendula.

Ingcamango engundoqo

Kwi-MCAS ingxaki ayikho Zingaphi iiseli zemast onazo. Yi badubula ngokulula. Yonke enye into yemeko - ukungaqiniseki, ukusasazeka kwenkqubo eninzi, uvavanyo olunzima - lulandela kuloo nyaniso inye.

Kutheni iiseli zemast zikhona kwaphela

Ingcamango engundoqo

Zibekwe ngokufanelekileyo-zigxininise kwimida, eluswini, kumgca womoya, eludongeni lwamathumbu, kunye nezicubu ezijikeleze imithambo yegazi kunye nemithambo-luvo. Kwaye bakhelwe isantya: endaweni yokuvelisa impendulo yabo kwimfuno, bayibambe kwangaphambili eyenziwe kwi-granules yokugcina, ilungele ukukhulula phakathi kwemizuzwana yomqondiso.Olo yilo lolwakhiwo lulo olubangela ukuchasana nokwaliwa lukhawuleze, kwaye ikwayiyo eyenza ukuba ukusebenza okungafanelekanga kube nzima ukuphila nako.

Kutheni iiseli zemast zikhona kwaphela

Iiseli zemast aziyongxaki yoyilo. Baphakathi kwawona malungu amadala okhuselo lomzimba lwangaphakathi, akhoyo ngolunye uhlobo kuzo zonke ii-vertebrates, kwaye enza umsebenzi ofunwa ngumzimba ngokunyanisekileyo.Bafaka impendulo yokuqala kwii-parasites, bancede ekunciphiseni ubuthi emva kokulunywa kunye nokulunywa, bathathe inxaxheba ekupholiseni amanxeba kunye nokuhlaziya izicubu, bafake isandla kulawulo lokungena komthambo wegazi, kwaye bancede ekugcineni izicubu zomqobo ezahlula umzimba kwihlabathi langaphandle.

Zibekwe ngokufanelekileyo-zigxininise kwimida, eluswini, kumgca womoya, eludongeni lwamathumbu, kunye nezicubu ezijikeleze imithambo yegazi kunye nemithambo-luvo. Kwaye bakhelwe isantya: endaweni yokuvelisa impendulo yabo kwimfuno, bayibambe kwangaphambili eyenziwe kwi-granules yokugcina, ilungele ukukhulula phakathi kwemizuzwana yomqondiso.Olo yilo lolwakhiwo lulo olubangela ukuchasana nokwaliwa lukhawuleze, kwaye ikwayiyo eyenza ukuba kusebenze okungafanelekanga kube nzima ukuphila nako.

Ijongeka njani i-mast cell function eqhelekileyo

Kwimpendulo ye-immune enempilo, iseli ye-mast idibana nesoyikiso sokwenyani-i-antigen ye-parasite, i-venom, okanye umonakalo wezicubu-kunye ne-degranulate ngokulingana nayo. Imithambo yegazi iyancipha kwaye ikwazi ukungena, ivumela i-plasma kunye neeseli zokuzivikela kwizicubu. Iziphelo zemithambo-luvo ziyavuselelwa, zivelise ukurhawuzelelwa okanye iintlungu ezitsala ingqalelo kwisiza. Ezinye iiseli zokhuselo lomzimba ziyagaywa. Emva kokuba isoyikiso sisusiwe, impendulo iyasombulula.

Yonke into engathandekiyo malunga naloo nkqubo iyasebenza. Ukudumba kuhambisa amalungu omzimba kwindawo. Ukurhawuzelelwa kukwenza ukuba ususe nantoni na eyenza umonakalo. I-Mucus ibamba kwaye igxotha izinto ezicaphukisayo. Kwimpendulo elinganayo, ukungonwabi kukwexeshana kwaye inzuzo iyinyani.

Yintoni engahambi kakuhle kwi-MCAS

Kwi-MCAS, kwaloo matshini mnye usebenza ngaphandle kwesizathu esilinganayo. Iiseli zeMast zivutha ngokuzenzekelayo, okanye ngokuphendula kwizinto ezinokuthi zingabonakali - igumbi elifudumeleyo, ivumba kwipaseji, isidlo, ukuma, umthambo omnene, usuku oluqhelekileyo loxinzelelo. Abalamli abakhululwayo bayafana abasetyenziswa ekukhuseleni okusemthethweni, kodwa bakhululwa kwimeko engafanelekanga kwaye ngokufuthi ngokuphindaphindiweyo.

Ngenxa yokuba iiseli zemast zihlala kwizicubu kuwo wonke umzimba, kwaye ngenxa yokuba abalamli babo besebenza kwimithambo yegazi, imisipha egudileyo, imithambo-luvo, kunye nezinye iiseli zokhuselo lomzimba, isiqendu esinye sinokuvelisa ukugungxula, intliziyo ebalekayo, ukuxinezeleka kwesisu, ukuphefumla, kunye nenkungu yokuqonda ngaxeshanye.Kugqirha ongacingiyo malunga neeseli zemast, olo manyano lunokujongeka njengeengxaki ezininzi ezinganxulumananga ezifika kanye-esiyinxalenye enkulu yesizathu sokuba imeko iphoswe.

Isifaniso esilula

I-Mastocytosis xa ithelekiswa ne-MCAS
I-MCAS ayikho i-mastocytosisLo ngowona mahluko ubalulekileyo kwintsimi, kwaye ukubhidanisa oku kukhokelela ekubhidekeni malunga nayo yonke into esezantsi.
I-Mastocytosis xa ithelekiswa ne-MCASMastocytosisI-MCAS
Ukungaqhelekanga okungundoqoIiseli zemast ezininzi ziqokelelana kwithishuInani eliqhelekileyo leeseli zemast, zisebenza ngokungafanelekanga
Ubalo lweeseliUkuphakama - ukwanda kwe-clonalOkuqhelekileyo
Isiseko se-tryptaseIKIT Ukuguqulwa kwe-D816V kwiimeko ezininzi zenkqubo yabantu abadalaNgokwesiqhelo kwisiseko
Ufuzo oluqhelekileyo lokufunyaniswaIiseli zemast ezingaqhelekanga ziyaboniswaAkukho tshintsho lwecausative olusekiweyo olunye
Iziphumo zeBiopsyIiseli zemast ezingaqhelekanga ziyaboniswaAkukho ngqokelela yeempawu
Uphawu lwesikhumbaIzilonda ze-Maculopapular (urticaria pigmentosa) eziqhelekileyo kwezinye iifomAkukho silonda esithile sokuxilongwa kwesikhumba

Zombini ezi meko zinokuvelisa iimpawu ezidlulanayo, kuba kuzo zombini iimeko abalamli beeseli zemast bafikelela kwizicubu ezingaphezulu. Kodwa i-mastocytosis ingabonakaliswa ngokufumana iiseli; I-MCAS ayinako, yiyo kanye loo nto uxilongo lwayo luxhomekeke kuluhlu lweekhrayitheriya kunovavanyo olunye lokuqinisekisa.

Ubungqina benjongo yobuninzi beeseli

I-classical allergy yi-IgE-mediated reaction into ethile, into ebonakalayo. Iyakwazi ukuveliswa kwakhona - ukuvezwa okufanayo kuvelisa ukusabela okufanayo - kwaye ngokuqhelekileyo kunokubonakaliswa ngovavanyo lwe-skin-prick okanye uvavanyo oluthile lwegazi lwe-IgE. Ukwaliwa kwamandongomane kuziphatha ngolu hlobo; ngokunjalo i-cat dander allergy.

Zombini ezi meko zinokuvelisa iimpawu ezidlulanayo, kuba kuzo zombini iimeko abalamli beeseli zemast bafikelela kwizicubu ezingaphezulu. Kodwa i-mastocytosis ingabonakaliswa ngokufumana iiseli; I-MCAS ayinako, yiyo kanye loo nto uxilongo lwayo luxhomekeke kuluhlu lweekhrayitheriya kunovavanyo olunye lokuqinisekisa.

Uninzi lwezigulane zifika kwi-MCAS ngokuchanekileyo ngokuba Uvavanyo lokwaliwa lubuye lucocekile ngelixa iimpawu zabo azizange zibonakale. Iphaneli ye-negative allergy ayibobungqina obuchasene nokubandakanyeka kweeseli zemast; kule meko isondele ekulindelweyo.

I-classical allergy yi-IgE-mediated reaction into ethile, into ebonakalayo. Iyakwazi ukuveliswa kwakhona - ukuvezwa okufanayo kuvelisa ukusabela okufanayo - kwaye ngokuqhelekileyo kunokubonakaliswa ngovavanyo lwe-skin-prick okanye uvavanyo oluthile lwegazi lwe-IgE. Ukwaliwa kwamandongomane kuziphatha ngolu hlobo; ngokunjalo i-cat dander allergy.

Iimpendulo ze-MCAS zihlala zingenzi njalo. Uninzi aluna-IgE-mediated kwaphela, endaweni yoko luvela ngezinye ii-receptors kumphezulu weseli yemast okanye ngokuvuselelwa ngokuthe ngqo ngokwasemzimbeni. Izinto ezibangela ukuba zisoloko zisemzimbeni-ubushushu, ukubanda, uxinzelelo, ukuzibhokoxa-kuneeprotheyini, kwaye akukho paneli yokwaliwa eyenzelwe ukufumanisa ezo zinto.Iimpendulo zinokulibaziseka ngeeyure, ukuqhawula ikhonkco elibonakalayo phakathi kokuvezwa kunye nesiphumo, kwaye umqobo unokutshintsha ngokulala, uxinzelelo, imeko yehomoni, okanye isigulo samva nje, ke ukuba sesichengeni okunyanyezelwe kwiveki enye kuvuselela ukusabela okulandelayo.

Umfanekiso ocetywayo

Unyango lwePearl

Isigulana esinovelwano oluphindaphindekayo, uvavanyo lwe-IgE olubi, kunye nezinto ezibangela umzimba ezifana nobushushu okanye uxinzelelo yinkcazo-ntetho ye-MCAS eqhelekileyo kunesigulana esinovavanyo oluqinisekileyo lokwaliwa. Indawo yolawulo egxekayo idla ngokuba yinxalenye yomfanekiso kunengxoxo echasene nayo.
  • I-MCAS luphazamiseko lwemast cell engafanelekanga ukusebenza, hayi yeemast cell numbers.
  • Umzobo wothelekiso wecala necala: iiphaneli ezintathu ezibonisa iseli yemast yesiqhelo ephendula ngokomlinganiselo, ipaneli yemastocytosis ebonisa amanani eseli agqithisileyo, kunye nephaneli yeMCAS ebonisa amanani eseli aqhelekileyo anokukhutshwa komlamli okubaxiweyo. Ubalo lweeseli ezifanayo kwiiphaneli enye neyesithathu, isiphumo esahluke kakhulu.
  • Isishwankathelo secandelo
  • I-Mastocytosis ibandakanya ukugqithisa kweeseli kwaye ingabonakaliswa ngokuthe ngqo; I-MCAS ayinako, yiyo loo nto ukuxilongwa kuyintlanganisela.
  • Iiseli zemast ziluncedo ngokusemthethweni kwiiseli zokhuselo lomzimba ezibekwe kwimiqobo yomzimba kwaye zakhelwe ukuphendula kwimizuzwana.
Icandelo 02

Ukuqonda Iiseli zeMast

Uvavanyo olungalunganga lokwaliwa luxhaphakile kwi-MCAS kwaye ayikhupheli ngaphandle.

Imvelaphi kunye nophuhliso

Iiseli zeMast ziqala kumongo wethambo, zivela kwiiseli ze-CD34-positive haematopoietic progenitor cells - kwanani labemi elivelisa ezinye iiseli zegazi. Noko ke, ngokungafaniyo neeseli ezininzi ezimhlophe, azilugqibi uphuhliso lwazo apho. Ama-progenitors angekavuthwa angena egazini, ajikeleze ngokufutshane, aze afudukele kwizicubu, apho ukuvuthwa kokugqibela kwenzeka khona.

Oko kukhula kuxhomekeke kakhulu kwi-stem cell factor (SCF) yokubonisa nge-KIT receptor kwi-mast cell surface. Le ndlela isembindini webhayoloji yemast cell, kwaye ikwayiyo indlela ephazanyiswe yi IKIT Ukuguqulwa kwe-D816V kufunyenwe kwiimeko ezininzi zabantu abadala ze-systemic mastocytosis. Imeko yendawo yethishu yalapha ibumba i-phenotype yokugqibela, yiyo loo nto iiseli zemast emathunjini zahluke ngokulinganayo kwezo zilusu.

Esinye isiphumo sifuna ugxininiso: kuba iiseli zemast zikhulela kwithishu endaweni yegazi, ubalo lwegazi oluqhelekileyo alikuxeleli nto ngazo. Iiseli zemast ezikhulileyo aziqhelekanga ukufunyanwa zijikeleza ngamanani abalulekileyo. Esi sesinye isizathu sokuba umsebenzi wegazi oqhelekileyo kwi-MCAS uhlala ufundwa njengokungaphawulekiyo.

Ukusasazwa kuwo wonke umzimba

Iiseli zemast zisasazwa ngobuchule kunokuba zifane, zigxile apho umzimba udibana nelizwe langaphandle kwaye ujikeleze izakhiwo eziphethe imiqondiso kunye negazi. Olu nikezelo luchaza umlinganiswa wenkqubo eninzi ye-MCAS ngokuthe ngqo kunayo nayiphi na enye inyani.

Apho iiseli zemast zihlala khona, kwaye zivelisa ntoni xa zidubula kakubi
Ukusasazwa kuwo wonke umzimbaIiseli zemast zisasazwa ngobuchule kunokuba zifane, zigxile apho umzimba udibana nelizwe langaphandle kwaye ujikeleze izakhiwo eziphethe imiqondiso kunye negazi. Olu nikezelo luchaza umlinganiswa wenkqubo eninzi ye-MCAS ngokuthe ngqo kunayo nayiphi na enye inyani.Apho iiseli zemast zihlala khona, kwaye zivelisa ntoni xa zidubula kakubi
IndawoIndima kwimpiloIimpawu xa ukusetyenziswa kungafanelekanga
Ulusu kunye nedermisukhuselo lomqobo; ukuphendula ekulunyweni, ukuhlatywa, kunye nokwenzakalaUkugungxula, ukurhawuzelelwa, ukurhawuzelela, idermographism, ukudumba
Umgudu wesisuUkugcinwa komqobo, ukulawulwa kwe-motility, ukuphendula kwizoyikiso ezityiweyoUkuqaqanjelwa, isicaphucaphu, ukurhawuzelela, urhudo, ukuqhina, ukuqunjelwa
Iindlela zomoya kunye nezonoUkukhusela umgca wokuphefumlaUkuxinana, i-rhinitis, ukuqina komqala, ukuphefumla, ukukhohlela
Ejikeleze imithambo yegaziUkulawulwa kwethoni yenqanawa kunye nokungenaUkutshintsha koxinzelelo lwegazi, tachycardia, ukugungxula, presyncope
Ubuchopho kunye neemeningesZibonelele nge-perivascularly kunye nakwi-meninges; umqondiso we-neuroimmuneInkungu yengqondo, intloko ebuhlungu, isiyezi (iinkqubo zisaphantsi kophononongo)
Ithishu edibanisayoUkulungiswa kunye nokulungiswa kwezicubuIntlungu edibeneyo kunye nemisipha; Ukufaneleka okunokwenzeka kokudibana kwezicubu ezidibeneyo

Ummongo wethambo

Indawo yemvelaphi yokhokho

Iiseli zemast ziphila ixesha elide ngokungaqhelekanga. Apho i-neutrophil inokusinda kwiiyure nje ukuya kwiintsuku, iiseli zemasti zinokuqhubeka iiveki ukuya kwiinyanga, kwaye ubungqina bubonisa ukuba abanye baphila ixesha elide kakhulu. Kwakhona ziyakwazi ukubuyisela kwakhona - emva kokuchithwa, iseli ye-mast inokuvuselela iivenkile zayo kwaye iphendule kwakhona, kunokuba ibhubhe kwinkqubo.

Qaphela ukungena kwengqondo ngononophelo. Iiseli zemast zikhona ngenene kwimeninges nakwindawo zeperivascular ngaphakathi kwe central nervous system, kunye ne mast cell-nerve interaction yinkalo yophando esebenzayo. Nangona kunjalo, iindlela zokudibanisa ukusebenza kweeseli zemast kwiimpawu ezithile ze-neurological kunye ne-cognitive zisalungiswa, kwaye amabango athembekileyo oomatshini kule ndawo angaphezulu kobungqina bangoku.

Umnqa wobomi

Iiseli zemast ziphila ixesha elide ngokungaqhelekanga. Apho i-neutrophil inokusinda kwiiyure nje ukuya kwiintsuku, iiseli zemasti zinokuqhubeka iiveki ukuya kwiinyanga, kwaye ubungqina bubonisa ukuba abanye baphila ixesha elide kakhulu. Kwakhona ziyakwazi ukubuyisela kwakhona - emva kokuchithwa, iseli ye-mast inokuvuselela iivenkile zayo kwaye iphendule kwakhona, kunokuba ibhubhe kwinkqubo.

  • FcERI - i-receptor ye-IgE ephezulu. Le yindlela ye-allergies yakudala: I-IgE antibodies ibopha i-receptor, kunye ne-cross-link by allergener triggers activation.
  • MRGPRX2 - i-receptor mediating isebenze i-IgE-ezimeleyo ngamachiza athile kunye neepeptides. Ubume bayo buncede ukucacisa ukusabela okwenzekayo ngaphandle kokubandakanyeka kwe-IgE okubonakalayo.
  • IKIT - i-receptor ye-stem cell factor; Umbindi wokusinda kweeseli zemast, ukuvuthwa, kunye nokuhlala kwezicubu.
  • Izamkeli zokuxhasa (C3a, C5a) - vumela ukusebenza ngokusebenzisa ingalo ehambelanayo ye-innate immunity.
  • Ii-receptors ezihlawulelwayo - qaphela iipateni ezinxulumene ne-pathogen, ukudibanisa iiseli zemast kwiimpendulo zosulelo.
  • IKIT - i-receptor ye-stem cell factor; Umbindi wokusinda kweeseli zemast, ukuvuthwa, kunye nokuhlala kwezicubu.
  • I-Adenosine, i-opioid, i-estrogen kunye nezinye ii-receptors — ukwenza igalelo kwiiprofayili ezahlukeneyo kunye nezomntu ngamnye ezibonwayo xa kusenziwa.
Ubusazi?

Ubukho beendlela ezininzi ezingezizo ze-IgE kungoko uvavanyo lokwaliwa oluqhelekileyo luhlala lubuyela kwisiqhelo kwi-MCAS. Ezo mvavanyo zakhelwe ukubona i-IgE-mediated sensitization - indlela enye phakathi kwezinto ezininzi ezinokuseta imast cell.

Umfanekiso ocetywayo

Umzobo weseli yemast ebhalwe igama: inwebu yeseli ezaliswe ngamagama ezamkeli (i-FcεRI, MRGPRX2, KIT, C3a/C5a, TLRs, CRH-R), ingaphakathi elixineneyo lipakishwe ngeegranules, enendawo yokusikwa ebonisa iziqulatho zegranule. Iseti yeqabane ebonisa iseli phambi nasemva kokuthotywa.

Isigulana esinovelwano oluphindaphindekayo, uvavanyo lwe-IgE olubi, kunye nezinto ezibangela umzimba ezifana nobushushu okanye uxinzelelo yinkcazo-ntetho ye-MCAS eqhelekileyo kunesigulana esinovavanyo oluqinisekileyo lokwaliwa. Indawo yolawulo egxekayo idla ngokuba yinxalenye yomfanekiso kunengxoxo echasene nayo.
  • Iiseli zeMast zisuka kwiCD34+ progenitors kumongo wethambo kodwa ziqole kwithishu, ngoko ke ubalo lwegazi alubonakalisi.
  • Ubukho beendlela ezininzi ezingezizo ze-IgE kungoko uvavanyo lokwaliwa oluqhelekileyo luhlala lubuyela kwisiqhelo kwi-MCAS. Ezo mvavanyo zakhelwe ukubona i-IgE-mediated sensitization - indlela enye phakathi kwezinto ezininzi ezinokuseta imast cell.
  • Bagxila kwindawo yesithintelo kwaye bajikeleze imithambo yegazi kunye nemithambo-luvo, yiyo loo nto i-MCAS iyinkqubo eninzi.
  • Zihlala ixesha elide kwaye ziyakwazi ukubuyisela kwakhona, ngoko ke ulawulo luyazinza kunokuba lufutshane.
  • I-KIT/SCF yokubonisa ingundoqo ekuphuhliseni kwabo - indlela ephazamisekile kwi-mastocytosis yabantu abadala.
Icandelo 03

Kwenzeka Ntoni Ngexesha
Mast Cell Activation

Iindidi ezininzi ze-receptor ezingaphaya kwe-IgE zinokuzenza zisebenze, yiyo loo nto iiphaneli ze-allergies zihlala ziphosakele izitshizi ze-MCAS.

1
Qalisa

Ukuqalisa lulandelelwano, kwaye inyathelo ngalinye linegama. Ukuyilandela kwenza ukuba iintlobo ezahlukeneyo ezididayo zeempawu ze-MCAS zibe lula kakhulu ukutolika.

2
Qalisa

I-stimulus ifikelela kwiseli-i-allergen, iziyobisi, ubushushu, uxinzelelo, umgudu, ihomoni yoxinzelelo, usulelo, okanye akukho nto ibonakalayo.

3
Ukuqaliswa

Ii-receptors zomphezulu zibandakanya kunye ne-intracellular signaling cascades iqala. Ukungena ngekhalsiyam linyathelo lokuqala lokuqala.

4
Ukuthotywa kwexabiso

Iigranule zokugcinwa zixubana nenwebu yeseli kwaye zikhuphe imixholo yazo eyenziwe kwangaphambili kwizicubu ezijikelezileyo - ngaphakathi kwemizuzwana.

5
Ukukhululwa komlamli

Abalamli abenziwe kwangaphambili bakhululwa ngokukhawuleza; abalamli abasandula ukwenziwa njengeprostaglandins kunye neleukotrienes balandela kwimizuzu, kunye neecytokines kwiiyure.

Iimpawu ezimbini zolu landelelwano zichaza uninzi lwezinto ezifunyanwa zizigulane. Okokuqala, inqanaba elikhawulezileyo lizoba kwiivenkile ezenziwe kwangaphambili, yiyo loo nto iimpendulo zinokuqala kwimizuzwana. Okwesibini, abalamli besigaba samva bahlanganiswa emva kokuba kuqaliswe ukusebenza, yiyo loo nto ezinye iimpawu zifika kakuhle emva kokuqalisa kwaye kutheni ukusabela kunokulibaziseka kweeyure zesibini kamva.

Ingcamango engundoqo

Iiseli zeMast zikhulula abalamli ngaphakathi amaza, hayi ngaxeshanye. Abalamli abenziwe kwangaphambili bakhupha ngemizuzwana; abalamli be-lipid bakhiwe ngemizuzu; iicytokines ziqokelelana ngeeyure. Isiganeko esisodwa sokuvula sinokuvelisa iimpawu ngosuku lonke.

Abalamli basebenza kwimithambo yegazi, izihlunu ezigudileyo, imithambo-luvo, kunye nezinye iiseli zokhuselo lomzimba, zivelisa iziphumo kuzo zonke iinkqubo zamalungu amaninzi.

Abalamli abaziintloko nento eyenziwa ngumntu ngamnye

Iiseli zeMast ziyakwazi ukukhulula inani elikhulu kakhulu lezinto. Ezi zilandelayo zezona zixoxiwe ngonyango, kwaye kunye zichaza uninzi lweempawu zeempawu eziqatshelweyo.

Kukwafanelekile ukuba uqaphele ukuba ukusebenza ayisiyiyo yonke okanye akukho nto. Iiseli zeMast zinokukhulula abalamli abakhethiweyo ngaphandle kokuthotywa ngokupheleleyo - ngamanye amaxesha kuthiwa yiqhekeza okanye ukukhutshwa okwahlukileyo. Oku kunceda ekucaciseni ukuba kutheni izigulane zineempawu ezidityanisiweyo, kwaye kutheni impendulo ingasoloko isisiganeko esimangalisayo igama elithi 'ukuvuselela' licebisa.
Abalamli abaziintloko nento eyenziwa ngumntu ngamnyeIiseli zeMast ziyakwazi ukukhulula inani elikhulu kakhulu lezinto. Ezi zilandelayo zezona zixoxiwe ngokonyango, kwaye kunye zichaza uninzi lweempawu zeempawu eziqatshelweyo.Abalamli bemast cell
HistamineUhloboOyena mlamli uqhelekileyo. I-Dilates imithambo yegazi kwaye yandise ukungeneka kwayo, ivuselela iziphelo zemithambo-luvo, kwaye yandise ukukhuselwa kwe-asidi yesisu. Ivelisa ukugungxula, ukurhawuzelelwa, ukurhawuzelela, uxinzelelo lwegazi oluphantsi, i-tachycardia, intloko ebuhlungu, kunye ne-GI cramping. Isebenza ngee-subtypes ezininzi ze-receptor - i-H1 eluswini kunye nomoya, i-H2 emathunjini nasentliziyweni - yiyo loo nto ukuvimba zombini iiklasi zihlala zisebenza ngcono kunokuthintela nokuba.
HistamineUhloboYakhelwe kwangaphambili
Prostaglandin D2Isandula ukwenziwaEyona protein ininzi kwimast cell granules kunye neyona ndawo isekiweyo ilabhorethri yokwenziwa kusebenze. Inegalelo kuhlengahlengiso lwezicubu kunye nokudumba, kwaye inokwandisa ngakumbi iimpendulo zeeseli zemast. Ixabiso layo lokuxilonga livela ekubeni lithe ngqo kwiiseli zemast.
I-Leukotrienes (LTC4, LTD4, LTE4)Isandula ukwenziwaI-bronchoconstrictors ezinamandla, ngakumbi kakhulu kune-histamine ngobunzima. Ukwandisa i-vascular permeability kunye nemveliso ye-mucus, kwaye igalelo kuzo zombini iimpawu ze-airway kunye ne-gastrointestinal. I-LTE4 inomlinganiselo kumchamo.
IiCytokines (IL-6, TNF-α, IL-1, IL-4 kunye nabanye)Isandula ukwenziwaIimpawu zeeprotheyini eziqhuba ukuvuvukala okubanzi. Iqala kancinci kwaye ithatha ixesha elide kunabalamli abangentla, kwaye kucingelwa ukuba banegalelo ekudinweni, ukugula, kunye nenkqubo ye-systemic 'efana nomkhuhlane' izigulane zichaza ngexesha nasemva kokuvutha.
IiChemokinesIsandula ukwenziwaFumana iiseli ezongezelelweyo zokuzikhusela kwisiza, ukwandisa kunye nokwandisa impendulo yokuvuvukala ngaphaya kwempendulo yokuqala yeseli ye-mast.
I-Platelet activating factor (PAF)Isandula ukwenziwaI-lipid mediator enamandla kakhulu eyandisa ukunyanzeliswa kwe-vascular permeability kwaye inokubangela i-hypotension enzima. Ifakwe kubunzima be-anaphylactic reactions kwaye ayivalwanga ngama-antihistamines.
IHeparinUhloboI-anticoagulant eyenzeka ngokwemvelo. Inokuba negalelo kwimikrwelo engaqhelekanga okanye ukuthambekela ukopha okuxelwe zezinye izigulana, kwaye ikwathatha inxaxheba kummiselo wendawo yokudumba.
Chymase & carboxypeptidase A3UhloboI-Granule proteases ebandakanyekayo ekwenziweni ngokutsha kwezicubu, iindlela zokulawula uxinzelelo lwegazi, kunye nokuthotywa kwetyhefu ethile kunye neepeptide.
I-classical allergy yi-IgE-mediated reaction into ethile, into ebonakalayo. Iyakwazi ukuveliswa kwakhona - ukuvezwa okufanayo kuvelisa ukusabela okufanayo - kwaye ngokuqhelekileyo kunokubonakaliswa ngovavanyo lwe-skin-prick okanye uvavanyo oluthile lwegazi lwe-IgE. Ukwaliwa kwamandongomane kuziphatha ngolu hlobo; ngokunjalo i-cat dander allergy.

I-anticoagulant eyenzeka ngokwemvelo. Inokuba negalelo kwimikrwelo engaqhelekanga okanye ukuthambekela ukopha okuxelwe zezinye izigulana, kwaye ikwathatha inxaxheba kummiselo wendawo yokudumba.

Umfanekiso ocetywayo

Itshati yenkqubo equkuqelayo ibaleka ukusuka ekhohlo ukuya ekunene: Qalisa → Ukuqaliswa → Ukuthotywa → Ukukhululwa koMlamli → Iimpawu, kunye nomgca wexesha lesebe ngaphantsi obonisa abalamli abenziwe kwangaphambili ngemizuzwana, abalamli be-lipid ngemizuzu, kunye ne-cytokines ngeeyure, isebe ngalinye lichazwe ngeempawu eliziqhubayo.

Isigulana esinovelwano oluphindaphindekayo, uvavanyo lwe-IgE olubi, kunye nezinto ezibangela umzimba ezifana nobushushu okanye uxinzelelo yinkcazo-ntetho ye-MCAS eqhelekileyo kunesigulana esinovavanyo oluqinisekileyo lokwaliwa. Indawo yolawulo egxekayo idla ngokuba yinxalenye yomfanekiso kunengxoxo echasene nayo.
  • I-Granule proteases ebandakanyekayo ekwenziweni ngokutsha kwezicubu, iindlela zokulawula uxinzelelo lwegazi, kunye nokuthotywa kwetyhefu ethile kunye neepeptide.
  • Abalamli abenziwe kwangaphambili bakhulula ngemizuzwana; abalamli be-lipid kwimizuzu; iicytokines ngeeyure.
  • Iiseli zeMast zinokukhulula abalamli ngokukhethayo ngaphandle kokuthotywa ngokupheleleyo.
  • Ukusebenza kulandela ukulandelelana: i-trigger, i-activation, i-degranulation, ukukhululwa komlamli, iimpawu.
  • Abalamli abenziwe kwangaphambili bakhulula ngemizuzwana; abalamli be-lipid kwimizuzu; iicytokines ngeeyure.
Icandelo le-04

Iimpawu ze I-MCAS

I-PAF kunye ne-prostaglandin inokuqhuba iziphumo ezinzima ze-cardiovascular that antihistamines don't address.

Ezi theyibhile zingezantsi ziququzelela iimpawu ezixeliweyo eziqhelekileyo ngokwenkqubo yomzimba. Izilumkiso ezibini ngaphambi kokuzifunda. Okokuqala, akukho sigulana sinazo zonke ezi zinto, kwaye uluhlu olude akufunekanga lufundwe njengoluhlu oluza kuhambelana nalo. Okwesibini, zonke iimpawu apha zenzeka kwezinye iimeko ezininzi; yintoni ecebisa iMCAS yile ipateni -i-episodic, irecurrent, multisystem, ehlala inezixhokonxa ezichongiweyo-kunokuba naluphi na ungeno lomntu.

Ulusu kunye ne-mucosal
I-MCAS ichazwa ngokuyinxenye ngobubanzi bayo. Ngenxa yokuba iiseli zemast zihlala phantse kuzo zonke iithishu, iimpawu zinokuvela phantse naphi na-kwaye imfuneko yeenkqubo zamalungu amabini okanye ngaphezulu isembindini woxilongo.Amanqaku
Ulusu kunye ne-mucosalUphawu
AmanqakuUkugungxula
Idla ngokuchaphazela ubuso, intamo nesifuba ngesiquphe; uphawu oluxelwa rhoqoUkurhawuzelelwa (pruritus)
Ingenzeka kunye okanye ngaphandle kwerhashalala ebonakalayoIzilonda (urticaria)
Unyuswe, ukurhawuzelela ukumanzisa; inokuba yedlula kwaye ifudukeIdermographism
Iiwelts eziphakanyiswe kukuphulula ngokuqinileyo ulusu-uphawu olubonakalayo olubonakalayoI-Angioedema
Ukudumba okunzulu, rhoqo kwimilebe, iinkophe, izandla, okanye umqala
I-MCAS ichazwa ngokuyinxenye ngobubanzi bayo. Ngenxa yokuba iiseli zemast zihlala phantse kuzo zonke iithishu, iimpawu zinokuvela phantse naphi na-kwaye imfuneko yeenkqubo zamalungu amabini okanye ngaphezulu isembindini woxilongo.Amanqaku
Ukulibaziseka ukuphiliswa kwenxebaIngxelo yezinye izigulane; iindlela ezingamiselwanga ngokupheleleyo
UkuphefumlaUkuxinana ngeempumlo kunye ne-rhinitis
Amaxesha amaninzi ezingapheliyo kunye non-onyakaUkuqina komqala
Ifuna uvavanyo olungxamisekileyo ukuba luqhubela phambili - lunokuthi lubonise i-anaphylaxisUkuphefumla kunye nokuphefumla kancinci
I-bronchoconstriction eqhutywa yi-leukotriene yindlela eyaziwayoUkukhohlela okungapheliyo
Ukungaphenduli rhoqo kunyango oluqhelekileyo lokhohlokhohlo
I-MCAS ichazwa ngokuyinxenye ngobubanzi bayo. Ngenxa yokuba iiseli zemast zihlala phantse kuzo zonke iithishu, iimpawu zinokuvela phantse naphi na-kwaye imfuneko yeenkqubo zamalungu amabini okanye ngaphezulu isembindini woxilongo.Amanqaku
Uvakalelo kumavumba kunye neekhemikhali ezisemoyeniIxhaphake kakhulu ingxelo yokubhanqa uphawu
IntliziyoTachycardia
Intliziyo ebalekayo, rhoqo ngaphandle kokuzibhokoxaUkungazinzi koxinzelelo lwegazi
Ingajinga kulo naliphi na icala ngexesha leeqephuPresyncope kunye nesyncope
Intloko ekhanyayo okanye isiqaqa, rhoqo xa umileUkungakhululeki kwesifuba
Ifuna uhlolo ukuba ingabandakanyi oonobangela bentliziyo
I-MCAS ichazwa ngokuyinxenye ngobubanzi bayo. Ngenxa yokuba iiseli zemast zihlala phantse kuzo zonke iithishu, iimpawu zinokuvela phantse naphi na-kwaye imfuneko yeenkqubo zamalungu amabini okanye ngaphezulu isembindini woxilongo.Amanqaku
I-AnaphylaxisOkubukhali, ngokukhawuleza, okunokubeka ubomi esichengeni - jonga icandelo likaxakeka
AmathumbuUkukrala kwesisu kunye neentlungu
Phakathi kweempawu eziqhelekileyo ezixelwe ngokubanziIsicaphucaphu kunye nokugabha
Inokulandela ukutya okanye kwenzeke ngokuzimeleyoIsiphumo se-Histamine's H2 kwi-asidi yesisu sinegalelo elaziwayo
I-Motility inokwahluka phakathi kweziqephu kunye nexeshaUqhuba rhoqo uthintelo lokutya- bona izilumkiso kwicandelo 9
I-neurological and cognitive
I-MCAS ichazwa ngokuyinxenye ngobubanzi bayo. Ngenxa yokuba iiseli zemast zihlala phantse kuzo zonke iithishu, iimpawu zinokuvela phantse naphi na-kwaye imfuneko yeenkqubo zamalungu amabini okanye ngaphezulu isembindini woxilongo.Amanqaku
Ukuqunjelwa kunye nokusebenza kwakhona kokutyaUbunzima bokugxila, ukukhumbula, kunye nokufumana amagama
I-neurological and cognitiveInkungu yengqondo
Ubunzima bokugxila, ukukhumbula, kunye nokufumana amagamaIntloko kunye ne-migraine
I-Migraine ixelwe kumazinga aphezulu kule ndawoIsiyezi
Ihlala igqithana ngeempawu zentliziyo kunye ne-autonomicUkudinwa
Zihlala zibukhali kwaye ziphakathi kwezona mpawu zixeliweyo zikhubazaUvakalelo lweemvakalelo
Ukukhanya, isandi, kunye nevumba; Iindlela zokusebenza zisephantsi kophando
I-MCAS ichazwa ngokuyinxenye ngobubanzi bayo. Ngenxa yokuba iiseli zemast zihlala phantse kuzo zonke iithishu, iimpawu zinokuvela phantse naphi na-kwaye imfuneko yeenkqubo zamalungu amabini okanye ngaphezulu isembindini woxilongo.Amanqaku
Iimvakalelo ze-neuropathicNgamana ukudibana neemeko zethishu edibeneyo ekuxoxwe ngayo kwicandelo lesi-7
I-Musculoskeletal, i-genitourinary, kunye nengqondoIntlungu edibeneyo kunye nemisipha
Ukungxamiseka kwesinyi kunye nokuphindaphindaIintlungu zamathambo
Uvavanyo lweziqinisekiso, ngakumbi apho i-mastocytosis iqwalaselwaUkungxamiseka kwesinyi kunye nokuphindaphinda
Iimpawu ezinjenge-Interstitial-cystitis zixelwaIimpawu ziyahluka kunye nomjikelo wokuya exesheni
Impembelelo yeHormonal kwiiseli zemast ibhaliweIziqendu ezifana nexhala
I-classical allergy yi-IgE-mediated reaction into ethile, into ebonakalayo. Iyakwazi ukuveliswa kwakhona - ukuvezwa okufanayo kuvelisa ukusabela okufanayo - kwaye ngokuqhelekileyo kunokubonakaliswa ngovavanyo lwe-skin-prick okanye uvavanyo oluthile lwegazi lwe-IgE. Ukwaliwa kwamandongomane kuziphatha ngolu hlobo; ngokunjalo i-cat dander allergy.

Ungeno 'olufana nexhala' lufanele ukukhathalelwa macala omabini. Ukukhutshwa komlamli kunokuvelisa i-physiology echanekileyo yokuhlaselwa kwe-panic, kwaye izigulane zihlala zixilongwa ngokungalunganga kweso siseko. Kwangaxeshanye, ukuxhalaba kunye nokudakumba kwenzeka ngokunyanisekileyo kunye nesifo esinganyangekiyo kwaye zifanelwe unyango ngokwazo. Ukuyiqaphela enye akuyomfuneko ukuba uyigxothe enye.

Ukutshintsha kweemvakalelo kunye nokucaphuka

Zombini kwiziphumo zomlamli nasekuphileni nesifo esinganyangekiyo, esingaqondwa kakuhle

  • Ukuhanjiswa kwengingqi. Ubuninzi beeseli zeMast kunye ne-phenotype ziyahluka phakathi kwezicubu kunye naphakathi kwabantu, ngoko ke iinkqubo zamalungu achaphazelekayo ziyahluka.
  • Ukukhutshwa komlamli owahlukileyo. Iiseli zeMast zinokukhulula abalamli abakhethiweyo kunomxholo wabo opheleleyo, kunye nemixube ehlukeneyo yomlamli ivelisa iimpawu ezahlukeneyo.
  • Iprofayile yomamkeli. Ii-receptors ezivakaliswa kwiiseli zemast yomntu zimile nezivuseleli zisebenza njengezixhokonxa kubo.
  • Ukuhanjiswa kwengingqi. Ubuninzi beeseli zeMast kunye ne-phenotype ziyahluka phakathi kwezicubu kunye naphakathi kwabantu, ngoko ke iinkqubo zamalungu achaphazelekayo ziyahluka.
  • Ukukhutshwa komlamli owahlukileyo. Iiseli zeMast zinokukhulula abalamli abakhethiweyo kunomxholo wabo opheleleyo, kunye nemixube ehlukeneyo yomlamli ivelisa iimpawu ezahlukeneyo.
  • Iprofayile yomamkeli. Ii-receptors ezivakaliswa kwiiseli zemast yomntu zimile nezivuseleli zisebenza njengezixhokonxa kubo.
Umfanekiso ocetywayo

Imephu yomzimba weempawu: i-anatomical outline ene-callouts ebhaliweyo kwindawo nganye echaphazelekayo - ulusu, iindlela zomoya, intliziyo, amathumbu, ingqondo, amalunga, isinyi - nganye iyandiswa ukubonisa iimpawu ezinxulumene naloo nkqubo. Uguqulo lunokuqaqambisa ukuba zeziphi iinkqubo zesigulana esizifumanayo.

Isigulana esinovelwano oluphindaphindekayo, uvavanyo lwe-IgE olubi, kunye nezinto ezibangela umzimba ezifana nobushushu okanye uxinzelelo yinkcazo-ntetho ye-MCAS eqhelekileyo kunesigulana esinovavanyo oluqinisekileyo lokwaliwa. Indawo yolawulo egxekayo idla ngokuba yinxalenye yomfanekiso kunengxoxo echasene nayo.
  • Iimpawu ze-span yesikhumba, ukuphefumla, i-cardiovascular, i-GI, i-neurological, i-musculoskeletal, i-genitourinary, kunye nemimandla yengqondo.
  • Ukubandakanyeka kweenkqubo zamalungu amabini okanye ngaphezulu yinxalenye yepatheni yokuxilonga.
  • Akukho phawu lomntu lukhethekileyo kwi-MCAS - i-episodic, ipateni yenkqubo ezininzi yeyona nto ibalulekileyo.
  • Iimpawu ze-span yesikhumba, ukuphefumla, i-cardiovascular, i-GI, i-neurological, i-musculoskeletal, i-genitourinary, kunye nemimandla yengqondo.
  • Ukubandakanyeka kweenkqubo zamalungu amabini okanye ngaphezulu yinxalenye yepatheni yokuxilonga.
Icandelo 05

Eqhelekileyo Izichukumisi

Iziqendu ezinjengexhala zinokuqhutywa ngumlamli; oku akufuneki kusetyenziswe ukugxotha imeko, okanye ukugxotha iimfuno zokwenyani zempilo yengqondo.

Ingcamango engundoqo

Ukuqaliswa yi eyongezelekayo. Utyhileko oluninzi olunyamezelekayo olufika ngokusondeleleneyo lunokuwela umda ekungekho namnye unokuwela yedwa. Lo ngowona mbono uluncedo ukwenza intsingiselo yeempendulo ezibonakala zingenamkhethe.

Ukutya

Izinto ezibangela ukutya kwi-MCAS zinxulumana ngokubanzi nomxholo we-histamine okanye ukwalathisa ukucaphuka kweeseli zemast kunokuba kukwaliwa kokutya okuqhelekileyo. I-Histamine iqokelela ekutyeni njengoko iguga, ibila, okanye igcinwa, yiyo loo nto ubutsha buhlala bubaluleka ngakumbi kunesithako esithile - intlanzi efanayo inokunyamezela ngomhla wokuthenga kwaye hayi kwiintsuku ezintathu kamva.Iitshizi ezisele zikhulile, inyama enyangisiweyo kunye necociweyo, ukutya okuvundisiweyo, kunye nentsalela zidla ngokubandakanyeka. Iqela lesibini libonakala likhuthaza iiseli zemast ukuba zikhuphe ezazo histamine. Ukwahluka komntu ngamnye kuninzi, kwaye izintlu ezipapashiweyo ziphathwa kakuhle njengeengqikelelo zokuvavanya kunemithetho.

Ubushushu

Ukutya

Izinto ezibangela ukutya kwi-MCAS zinxulumana ngokubanzi nomxholo we-histamine okanye ukwalathisa ukucaphuka kweeseli zemast kunokuba kukwaliwa kokutya okuqhelekileyo. I-Histamine iqokelela ekutyeni njengoko iguga, ibila, okanye igcinwa, yiyo loo nto ubutsha buhlala bubaluleka ngakumbi kunesithako esithile - intlanzi efanayo inokunyamezela ngomhla wokuthenga kwaye hayi kwiintsuku ezintathu kamva.Iitshizi ezisele zikhulile, inyama enyangisiweyo kunye necociweyo, ukutya okuvundisiweyo, kunye nentsalela zidla ngokubandakanyeka. Iqela lesibini libonakala likhuthaza iiseli zemast ukuba zikhuphe ezazo histamine. Ukwahluka komntu ngamnye kuninzi, kwaye izintlu ezipapashiweyo ziphathwa kakuhle njengeengqikelelo zokuvavanya kunemithetho.

Umoya obandayo, amanzi abandayo, iziselo ezibandayo, kunye neendawo ezihlala efrijini kuxelwa ukuba zibangela ezinye izigulana. Ngokucacileyo, ngokukhawuleza utshintsho phakathi kwamaqondo obushushu asoloko echazwa njengobubi ngakumbi kunobubaxa obubambekayo obubambekayo.

Phakathi kwezona zibangela zixelwe rhoqo zomzimba. Iishawa ezishushu kunye neebhafu, iisaunas, imozulu eshushu, amagumbi ashushu, kunye neziselo ezishushu zonke zidla ngokucatshulwa. Ubushushu abufuni nto ingena emzimbeni, nto leyo eyinxalenye yesizathu sokuba uvavanyo oluqhelekileyo lokwaliwa lungakwazi ukububona.

Kuyabanda

Uxinzelelo

Yenza umthambo

Ukuzibhokoxa ngokwasenyameni kusisiqalo sokwenyani kwizigulana ezininzi, kwaye enye eneendleko zokwenyani, kuba umsebenzi uluncedo ngenye indlela kwimpilo nakwimo. Ubukhulu kunye nesantya sokunyuka ngokubanzi kubaluleke ngaphezu kobude bexesha elipheleleyo, yiyo loo nto iindlela ezihamba kancinci, ezinobungakanani obuphantsi - kunye nomsebenzi owenziweyo okanye osekwe emanzini apho ukuma kunganyamezelwa kakuhle - zihlala zilawulwa ngcono.

Uxinzelelo

Uxinzelelo sisiqalekiso somzimba, hayi isafobe. Iiseli zeMast zithwala i-receptors ye-corticotropin-ekhulula ihomoni kwaye zihlale kufuphi neziphelo zemithambo-luvo, zibonelela ngendlela ebhaliweyo apho inkqubo ye-nervous ichaphazela ukusebenza kwayo. Le ngongoma ifuna ukugxininiswa kuba izigulana ezineempawu eziya zisiba mandundu ngenxa yoxinzelelo zihlala zixelelwa ukuba imeko yazo ingokwengqondo.Indlela eboniswayo ye-neuroimmune ayifani nophawu olucingelwayo.

IiHormones

Izigulana ezininzi zixela iipatheni zeempawu ze-cyclical, zihlala ziba mandundu malunga nokuya exesheni. I-Estrogen ichaphazela ukuziphatha kweeseli zemast, engomnye wabanegalelo elicetywayo kwizinga eliphezulu lokuxilongwa kwabasetyhini. Utshintsho ngexesha lokukhulelwa, emva kokubeleka, kunye ne-perimenopause nazo zichazwe, kwaye ziyahluka kakhulu phakathi kwabantu.

Usulelo

Ukosuleleka ngokuthembekileyo kuphakamisa isiseko sokusebenza kwakhona kwizigulane ezininzi. Isigulo esiqhelekileyo sentsholongwane sikholisa ukwandisa i-trigger esetelwe iiveki emva koko, ngoko ke ukutyhileka obekunyanyezelwe ngaphambili kuyacaphukisa ngexesha lokuchacha. Ezinye izigulana ziqala ngeempawu zazo kwisifo esosulelayo, nangona kunzima ukuseka i-causation kwiimeko zomntu ngamnye.

Iiklasi ezithile zamayeza zibonwa kuncwadi lweeseli zemast njengezinto ezinokuthi zisebenze, kubandakanywa i-opioids, amanye amayeza angeyo-steroidal achasene nokudumba, i-radiocontrast media, ezinye i-neuromuscular blocking agents ezisetyenziswa kwi-anesthesia, kunye ne-vancomycin. Izithako ezingasebenziyo - iidayi, izilondolozo, izizalisi - nazo zinokuba yinto ephazamisayo, yingakho isigulane sinokunyamezela ukuqulunqwa komenzi kunye nokusabela komnye.

Iingxelo ezibangela imo engqongileyo ziquka ukungunda, uthuli, umungu, umsi, ungcoliseko lomoya, utshintsho lokufuma, kunye nokutshintsha koxinzelelo lwebarometric. Uvakalelo luyahluka ngokubanzi, kwaye ukuchonga iziphembeleli zokusingqongileyo kudla ngokufuna ukugcinwa kweerekhodi ixesha elide kunokuqwalasela okunye.

Amayeza

Iiklasi ezithile zamayeza zibonwa kuncwadi lweeseli zemast njengezinto ezinokuthi zisebenze, kubandakanywa i-opioids, amanye amayeza angeyo-steroidal achasene nokudumba, i-radiocontrast media, ezinye i-neuromuscular blocking agents ezisetyenziswa kwi-anesthesia, kunye ne-vancomycin. Izithako ezingasebenziyo - iidayi, izilondolozo, izizalisi - nazo zinokuba yinto ephazamisayo, yingakho isigulane sinokunyamezela ukuqulunqwa komenzi kunye nokusabela komnye.

Kubalulekile

Musa ukuyeka, ukuphepha, okanye ukutshintsha iyeza elimiselweyo ngokoluhlu oluqhelekileyo. Yiza noluhlu kugqirha owazi imbali yakho. Umngcipheko wokuyeka unyango olufunekayo unokudlula umngcipheko ophetshwayo, kwaye esi sigqibo sifuna uhlolo lomntu ngamnye.

Ivumba kunye nevumba

Iziqholo, izinto ezihlaziya umoya, iimveliso zokucoca, iimpahla zokuhlamba impahla, izinyibilikisi, ipeyinti entsha, kunye nefenitshala entsha ziphakathi kwezona zinto zibangela ukuba kuxelwe ngokuqhubekayo, kwaye phakathi kwezona zinto zinzima ukuziphepha kwiindawo ekwabelwana ngazo. Iimeko ezingenavumba zisisicelo sendawo yokuhlala esemgangathweni ezikolweni nakwiindawo zokusebenza ngenxa yesi sizathu.

Utywala

Utywala busoloko buxelwa njengento ebangela ukuba kubekho iindlela ezingaphezu kwenye: iziselo ezinxilisayo ezininzi zine-histamine ngokuthe ngqo, ngakumbi iwayini kunye nebhiya; utywala bunokuphazamisana ne-enzymes ephula i-histamine; kwaye ibangela i-vasodilation ngokuzimeleyo. Iimpendulo kwimilinganiselo emincinci zichazwa ngokuqhelekileyo.

Iiseti ze-trigger ziyahluka phakathi kwabantu ngenxa yokuba iiprofayili ze-receptor, ukuhanjiswa kwezicubu, iimeko ezikhoyo, kunye nesiseko sokuphinda sisebenze zonke ziyahluka. Nabo bayatshintsha ngaphakathi umntu ngokuhamba kwexesha - edla ngokukhula ngamaxesha olawulo olulambathayo kunye nokucutheka kwakhona xa unyango luthoba isiseko sokusebenza kwakhona. Iimpendulo zinokulibaziseka ngeeyure, nto leyo efihla ikhonkco phakathi kwesizathu nesiphumo. Kuzo zonke ezi zizathu, ukuchonga izibasi kufuna ngokuthembekileyo ukugcinwa kweerekhodi kwiiveki kunezigqibo ezithathwe kwisehlo esinye.

Umfanekiso ocetywayo

Ukungalali kakuhle okanye ukungonelanga kuthoba ukunyamezelana ngokubanzi kwaye kusebenzisana nayo yonke enye into kolu luhlu. Ayifane ibe yeyona nto ibangela ukuqhambuka, kodwa yenye yabaxhasi abanokuthatha amanyathelo ngakumbi, kwaye izigulana zihlala zixela ukuba ukukhusela ubuthongo kuphakamisa umda wayo kuyo yonke enye into.

Isigulana esinovelwano oluphindaphindekayo, uvavanyo lwe-IgE olubi, kunye nezinto ezibangela umzimba ezifana nobushushu okanye uxinzelelo yinkcazo-ntetho ye-MCAS eqhelekileyo kunesigulana esinovavanyo oluqinisekileyo lokwaliwa. Indawo yolawulo egxekayo idla ngokuba yinxalenye yomfanekiso kunengxoxo echasene nayo.
  • Kutheni izinto ezibangela ukuba zihluke kakhulu
  • Izinto ezibangela umzimba (ubushushu, ukubanda, uxinzelelo, ukuzibhokoxa) akufuneki into engenisiweyo kwaye ayibonakali kuvavanyo lokwaliwa.
  • Uxinzelelo lusebenza nge-neuroimmune pathway ebhaliweyo; oku akuyenzi imeko ibe sengqondweni.
  • Izinto ezixhobisayo - umthwalo oqokelelweyo ubaluleke ngaphezu kwako nakuphi na ukuvezwa okukodwa.
  • Izinto ezibangela umzimba (ubushushu, ukubanda, uxinzelelo, ukuzibhokoxa) akufuneki into engenisiweyo kwaye ayibonakali kuvavanyo lokwaliwa.
Icandelo 06

Kutheni i-MCAS i
Kunzima uku Uxilongo

Iiseti ze-trigger ziyahluka phakathi kwabantu kunye nokutshintsha ngokuhamba kwexesha, ke ukugcinwa kweerekhodi zobuqu kubalulekile.

Iimpawu eziguquguqukayo

I-MCAS ivelisa indibaniselwano yeempawu ezahlukeneyo kwizigulane ezahlukeneyo, kunye neendibaniselwano ezahlukeneyo kwisigulane esinye ngokuhamba kwexesha. Akukho mboniso umnye wokuthelekisa ipateni. Ngenxa yokuba iimpawu azikhankanywanga, nganye inokubalelwa kwimeko eqhelekileyo xa ijongwa yodwa.

Ukulibaziseka koxilongo kwi-MCAS ayikokusilela koogqirha ngabanye. Ilandela kwiimpawu ezithile, ezichongiweyo zemeko kunye novavanyo olusetyenziselwa ukuyiphanda.

Iimpawu eziguquguqukayo

Ukukhululwa komlamli okwethutyana

Le yingxaki engundoqo yobugcisa. Abalamli abalinganisiweyo banesiqingatha sobomi obufutshane. ISerum tryptase incopho kwiiyure ezimbalwa zokusabela ize iwe; I-metabolites yomchamo ihlala ixesha elide kodwa ibonakalisa ifestile elinganiselweyo. Iisampulu phakathi kweziqendu zinokufumana nto iphakanyisiweyo - hayi ngenxa yokuba ukuqaliswa akuzange kwenzeke, kodwa ngenxa yokuba ayisenamlinganiselo.

Umsebenzi wegazi wesiqhelo awuphawuleki kwi-MCAS. Ubalo lwegazi elipheleleyo, iimpawu zokukrala, kunye ne-biochemistry eqhelekileyo ziqhelekile, njengoko zinjalo iiphaneli zokungalungi. Uvavanyo lomlamli olukhethekileyo luyafuneka, kwaye oko kusoloko kubuyisela iziphumo eziqhelekileyo ngenxa yezizathu zobugcisa ezichazwe ngezantsi.Isigulane ke ngoko sinokuqokelela ifayile ebanzi yophando oluqhelekileyo ngelixa uhlala ungaphilanga ngokwenene - iphethini eyandisa ngelishwa ithuba lokuxelelwa ukuba akukho nto iphosakeleyo.

I-classical allergy yi-IgE-mediated reaction into ethile, into ebonakalayo. Iyakwazi ukuveliswa kwakhona - ukuvezwa okufanayo kuvelisa ukusabela okufanayo - kwaye ngokuqhelekileyo kunokubonakaliswa ngovavanyo lwe-skin-prick okanye uvavanyo oluthile lwegazi lwe-IgE. Ukwaliwa kwamandongomane kuziphatha ngolu hlobo; ngokunjalo i-cat dander allergy.

Isabelo esikhulu seziphumo ezibi zomlamli zibonakalisa ixesha kunye nokuphatha endaweni yokungabikho kwesifo. Ukuqinisekisa iprotokoli yokuqokelela kunye nogqirha oyalelayo kunye nelebhu phambi kokuba kwenziwe isampulu kubaluleke ngaphezu kokuphinda uvavanyo olungaphathwanga kakuhle.

Hlangana nezinye izifo

Iimfuno zokuphatha ziyadibanisa oku. Abalamli abaliqela abazinzanga kubushushu begumbi, kwaye iisampulu zeprostaglandin zibuthathaka ngakumbi. Iisampulu zifuna ukuphola kwangoko, ukuphathwa okubandayo ngexesha lonke lokuqokelelwa, kunye nokusetyenzwa kwangoko. Umzekelo oshiywe kwiqondo lobushushu begumbi okanye ulibazisekile kuhambo unokuvelisa iziphumo eziqhelekileyo kwisigulane ebesisabela ngokunyanisekileyo.

Ukunqongophala kolwazi logqirha

I-MCAS yaphawulwa ngokusesikweni kuphela ngo-2007, ngeenqobo zokugweba zemvumelwano ezalandelayo ngo-2010 nango-2012. Oogqirha abaninzi baseklinikhi balugqibile uqeqesho lwabo phambi kokuba imeko ingene kwiikharityhulam eziqhelekileyo. Ukuqhelana kuyahluka ngokubanzi naphakathi kwabantu abangadliyo kunye nee-immunologists, kwaye ukufikelela kwiingcali ezinamava eseli yemast akulingani ngokwejografi.

Iimpawu ze-MCAS zidibana kakhulu nesifo samathumbu esicaphukisayo, i-urticaria engapheliyo, isifuba, i-fibromyalgia, isifo esingapheliyo sokudinwa, ukuphazamiseka kwexhala, i-dysautonomia, kunye nezinye ezininzi. Nganye kwezi zinto ziqhelekile koogqirha abaninzi kwaye zifunyaniswa ngokuqhelekileyo. Xa isigulana sibonisa iimpawu ezilingana nezi ngokuyinxenye, isiphumo esiqhelekileyo luthotho loxilongo oluyinxenye kunokuba lube ludityaniso.

Kufanelekile ukuchaza ngokucacileyo ukuba iindlela zokuxilonga ze-MCAS zihlala zingumxholo wengxoxo yokwenene kuncwadi lwezonyango. Ngokubanzi, indawo enye ibambelele kwiikhrayitheriya ezingqongqo ezifuna ubungqina bomlamli onenjongo, ngezizathu zokuba ngaphandle kwayo umngcipheko wokuxilongwa usetyenziswa ngokubanzi kakhulu.Esinye isikhundla sibambe ukuba iikhrayitheriya ezingqongqo ziphosakele izigulane ezinemeko ngokwenene kodwa ukuvavanywa komlamli ngokuphindaphindiweyo kungaphumeleli ngenxa yezizathu zobugcisa ezichazwe ngasentla.

Oku akusiyo izigulane zokungavumelani zidalwe, kwaye azisonjululwanga. Isiphumo salo esisebenzayo kukuba iingcali ezimbini ezinobuchule zinokufikelela kwizigqibo ezahlukeneyo malunga nesigulana esinye. Ukuqonda ukuba impikiswano ikhona kunceda ukuchaza iimbono eziphikisanayo ngaphandle kokufuna ukucinga ukuba omnye ugqirha wayengalunganga.

Ingxabano yokuxilonga

Kufanelekile ukuchaza ngokucacileyo ukuba iindlela zokuxilonga ze-MCAS zihlala zingumxholo wengxoxo yokwenene kuncwadi lwezonyango. Ngokubanzi, indawo enye ibambelele kwiikhrayitheriya ezingqongqo ezifuna ubungqina bomlamli onenjongo, ngezizathu zokuba ngaphandle kwayo umngcipheko wokuxilongwa usetyenziswa ngokubanzi kakhulu.Esinye isikhundla sibambe ukuba iikhrayitheriya ezingqongqo ziphosakele izigulane ezinemeko ngokwenene kodwa ukuvavanywa komlamli ngokuphindaphindiweyo kungaphumeleli ngenxa yezizathu zobugcisa ezichazwe ngasentla.

Umfanekiso ocetywayo

Oku akusiyo izigulane zokungavumelani zidalwe, kwaye azisonjululwanga. Isiphumo salo esisebenzayo kukuba iingcali ezimbini ezinobuchule zinokufikelela kwizigqibo ezahlukeneyo malunga nesigulana esinye. Ukuqonda ukuba impikiswano ikhona kunceda ukuchaza iimbono eziphikisanayo ngaphandle kokufuna ukucinga ukuba omnye ugqirha wayengalunganga.

Isigulana esinovelwano oluphindaphindekayo, uvavanyo lwe-IgE olubi, kunye nezinto ezibangela umzimba ezifana nobushushu okanye uxinzelelo yinkcazo-ntetho ye-MCAS eqhelekileyo kunesigulana esinovavanyo oluqinisekileyo lokwaliwa. Indawo yolawulo egxekayo idla ngokuba yinxalenye yomfanekiso kunengxoxo echasene nayo.
  • Ulibaziseko kuxilongo
  • Abalamli baphila ixesha elifutshane kwaye abazinzanga, ngoko ke ixesha kunye nokuphathwa kwesampulu kuqhuba izinto ezininzi ezingalunganga.
  • Iimpawu ziyadibana nezinye iimeko eziqhelekileyo, zivelisa uxilongo oluyinxenye.
  • Uvavanyo lwelabhoratri yesiqhelo luqhelekile kwi-MCAS; uvavanyo lomlamli olukhethekileyo luyafuneka.
  • Abalamli baphila ixesha elifutshane kwaye abazinzanga, ngoko ke ixesha kunye nokuphathwa kwesampulu kuqhuba izinto ezininzi ezingalunganga.
Icandelo 07

Nxulunyaniswe Iimeko

Iikhrayitheriya zokuxilonga zihlala ziphikiswa ngokunyanisekileyo, ngoko ke izimvo zeengcali zinokwahluka ngokusemthethweni.

Funda oku kuqala

Umbutho awuyongxaki. Iimeko ezininzi ezingezantsi ziyenzeka kunye ne-MCAS kaninzi kunokuba ithuba linokuqikelela, kodwa nokuba bayabelana ngendlela, nokuba enye ibangela enye, okanye ingaba iindlela zokudluliselwa ekwabelwana ngazo zinyusa ukugqithelana okubonakalayo. ayimiselwanga uninzi lwezi zibini.

I-MCAS ixelwa rhoqo kunye nezinye iimeko ezininzi. Amandla obungqina ahluke kakhulu phakathi kolu nxulumano, kunye nemiba yomahluko.

Funda oku kuqala

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

Ukwenzeka ngokubambisana kwe-hEDS, POTS, kunye ne-MCAS kuxoxwa rhoqo ngokwaneleyo ukuze kuchazwe ngokungekho sikweni njengonxantathu. Umbutho uxelwa ngokuqhubekayo, kodwa indlela esisiseko ihlala ingqikelelo. Iiseli zeMast zihlala kwiithishu ezidibeneyo kwaye zithatha inxaxheba kuhlaziyo lwezicubu, ezibonelela ngomgca obambekayo wokubuza, kodwa ukuba nokwenzeka abukho ubungqina. Iipateni ezithunyelwayo kunye noxilongo zinokuba negalelo kwindlela okubonakala kuqina ngayo ukuhlangana.

I-Hereditary alpha tryptasemia (HaT)

I-HaT luphawu lofuzo olubandakanya iikopi ezongezelelweyo zejini ye-alpha-tryptase TPSAB1, ezuzwe njengelifa kwipateni eyongameleyo ye-autosomal, kwaye ivelisa isiseko seserum tryptase ethe gqolo. Ixhaphakile xa kuthelekiswa nabemi ngokubanzi. Ulwalamano lwayo neempawu lusacaciswa: lunxulunyaniswa nokusabela okuqatha kwezinye iimeko, kodwa abantu abaninzi abanolu phawu abanampawu.

I-HaT ibalulekile ekutolikweni. Ngenxa yokuba iphakamisa isiseko se-tryptase, ixabiso elilodwa eliphakamileyo le-tryptase kumntu one-HaT ayibonakali ngokwayo ukusebenza kwe-mast cell-eyomeleza ukuba kutheni umda wokuxilonga uchazwa njenge ukuphakama ngaphezu kwesiseko somntu ngamnye kunokuba inani elipheleleyo.

I-Hereditary alpha tryptasemia (HaT)

Iimpawu zesisu kwi-MCAS zigqithana phantse ngokupheleleyo kunye neendlela ze-IBS, kwaye ezinye izigulana ziphethe zombini iilebhile. Uphando luye lwavavanya ukuxinana kweeseli zemast kunye nokusebenza kwi-gut mucosa yezigulane ze-IBS ezineziphumo ezahlukeneyo. Ingaba i-subset ye-IBS imele ukubandakanyeka kwe-mast cell engaziwayo ngumbuzo ovulekileyo kunokuba yinto eqinisekisiweyo.

I-Migraine

I-Migraine ixelwe kumazinga aphakamileyo phakathi kwezigulane ze-MCAS. I-Histamine yinto esekelwe kwi-vasoactive ehambelana nokubaluleka kwentloko, kwaye iiseli zemast zikhona kwi-meninges. Ikhonkco lobuchwephesha liyavakala ngokwebhayoloji kwaye liphantsi kophando, kodwa alizinziswanga.

I-asthma kunye ne-urticaria engapheliyo

I-Migraine

I-Migraine ixelwe kumazinga aphakamileyo phakathi kwezigulane ze-MCAS. I-Histamine yinto esekelwe kwi-vasoactive ehambelana nokubaluleka kwentloko, kwaye iiseli zemast zikhona kwi-meninges. Ikhonkco lobuchwephesha liyavakala ngokwebhayoloji kwaye liphantsi kophando, kodwa alizinziswanga.

Abanye abaphandi bacebise ukuba kusebenze iiseli zemast njengegalelo kwi-COVID ende, bephawula ukuba iimpawu ziyadinwa, ubunzima bokuqonda, kunye ne-dysautonomia. Le yinkalo yophando olusebenzayo kwaye ubungqina bubo eyandulelayo. Kufuneka iphathwe njengengqikelelo phantsi kofundo endaweni yobudlelwane obusekiweyo, kunye namabango onxibelelwano olulungisiweyo - kulo naliphi na icala - okwangoku lidlula idatha.

Ezi zinobudlelwane obucacileyo bomatshini kwiiseli zemast yayo nantoni na kweli candelo. Iiseli zeMast zingundoqo kwi-pathophysiology yazo zombini - i-leukotrienes kunye ne-histamine drive bronchoconstriction kunye nokwakheka kwesondo ngokulandelanayo. I-urticaria engapheliyo ezenzekelayo ngokukodwa ibandakanya ukusebenza kweeseli zemast ngokuthe ngqo.Olu ayilulo unxulumano oluqikelelwayo; ziimeko apho ukubandakanyeka kweeseli zemast kusekwe, nangona zihlala zoxilongo ezihlukeneyo ezivela kwi-MCAS.

I-COVID ende

Amandla obungqina ngokujonga nje krwaqu
Iimeko ze-AutoimmuneUbume bobudlelwaneAmandla obungqina ngokujonga nje krwaqu
ImekoUbume bobudlelwaneUbume bobungqina
Urticaria engapheliyoUkusebenza kweeseli zemast kungundoqo kwisifo ngokwasoUbume bobungqina
Indlela esekiweyoUmbefuAbalamli beeseli zeMast baqhuba i-bronchoconstriction
I-Hereditary alpha tryptasemiaIimpawu zemfuzo eziphakamisa isiseko se-tryptase; kuchaphazela uvavanyo lokutolikaUphawu olumiselweyo; Ulwalamano lweempawu lusacaciswa
IIPOTSUkwenzeka rhoqo; iziphumo zomlamli kwithoni yemithambo ecetywayoUmbutho unike ingxelo; indlela yokusebenza ayisonjululwanga
I-MigrainehEDS / hypermobilityUkwenzeka rhoqo; ukubandakanyeka kwezicubu ezidibeneyo zicetywayo
Umbutho unike ingxelo; indlela yokucingaAmazinga aphezulu axeliweyo; abalamli be-vasoactive babonakalaUmbutho unike ingxelo; indlela ephantsi kophando
IBSIimpawu ziyadibana; ukufunyaniswa kweeseli zemast kuguquguqukaUkugqithelana kucacile; ubudlelwane abusonjululwanga
I-Migraine ixelwe kumazinga aphakamileyo phakathi kwezigulane ze-MCAS. I-Histamine yinto esekelwe kwi-vasoactive ehambelana nokubaluleka kwentloko, kwaye iiseli zemast zikhona kwi-meninges. Ikhonkco lobuchwephesha liyavakala ngokwebhayoloji kwaye liphantsi kophando, kodwa alizinziswanga.Isifo se-AutoimmuneIngxelo yokwenzeka ngokubambisana; ukwabelwana kwe-immune dysregulation ecetywayo
Isigulana esinovelwano oluphindaphindekayo, uvavanyo lwe-IgE olubi, kunye nezinto ezibangela umzimba ezifana nobushushu okanye uxinzelelo yinkcazo-ntetho ye-MCAS eqhelekileyo kunesigulana esinovavanyo oluqinisekileyo lokwaliwa. Indawo yolawulo egxekayo idla ngokuba yinxalenye yomfanekiso kunengxoxo echasene nayo.
  • Umbutho unike ingxelo; indlela yokusebenza ayisekwanga
  • Iimpawu ziyadibana; Ubandakanyo lwemast cell lucingelwa
  • Okokuqala - phantsi kophando olusebenzayo
  • Ukubandakanyeka kweeseli ze-mast kusekwe kwi-urticaria engapheliyo kunye ne-asthma - ezi ayizokhonkco eziqikelelwayo.
  • I-HaT yinto yokwenyani yofuzo ephakamisa isiseko se-tryptase kunye nokutshintsha indlela iziphumo zovavanyo kufuneka zifundwe ngayo.
Icandelo 08

Ukuqonda Ngoku
ye Oonobangela

Kuyo yonke le ndawo, umbutho uye waphoswa ngokuphindaphindiweyo nge-causation - funda amabango ngononophelo.

Amandla obungqina ngokujonga nje krwaqu

Akukho sizathu sinye se-MCAS esekiweyo. Ezi meko zingezantsi ziinkalo zophando ezinamanqanaba ahlukeneyo enkxaso. Nawuphi na umthombo obonisa ukuzithemba, i-akhawunti yecausal emanyeneyo ye-MCAS ngoku iyabugqitha ubungqina.

Imfuza

Yintoni esekiweyo: I-Hereditary alpha tryptasemia luphawu lwemfuzo lokwenyani, oluphawulwe kakuhle olubandakanya iikopi ezongezelelweyo zemfuzo. TPSAB1, kwaye iphakamisa isiseko se-tryptase. Ngokwahlukileyo, i IKIT Ukuguqulwa kwe-D816V kusekwe njengomqhubi kuninzi lwabantu abadala be-systemic mastocytosis - imeko eyahlukileyo, kodwa enye ebonisa indlela ukuguqulwa okukodwa kunokuphazamisa ngayo i-mast cell biology.

Yintoni engekho: Akukho zinguqu ezifanayo zecausative zichongiwe kwi-MCAS. Ukudityaniswa kweentsapho kuxeliwe, kwaye ezinye izigulana zichaza izalamane ezininzi ezichaphazelekayo, kodwa ipateni yelifa echaziweyo ye-MCAS ngokwayo ayikasekwa. Nokuba i-MCAS imele imeko enye enesiseko sofuzo esingekafunyanwa, okanye iimeko ezahlukeneyo ezahlukeneyo ezibekwe phantsi kweleyibhile enye yekliniki, ayisonjululwanga.

Imfuza

I-Broad immune disregulation yindawo ehamba phambili yophando. Iindlela ezicetywayo ziquka ukuguqulwa kwentetho ye-receptor okanye uvakalelo kwiiseli zemast, ukuphazamiseka komqondiso olawulayo onokuthi ngokuqhelekileyo uthintele ukusebenza, kunye nokusebenzisana okungaqhelekanga phakathi kweeseli zemast kunye nabanye abantu abakhusela umzimba. Ezi zikholeleke kwaye zifundwe ngenkuthalo, kodwa zichaza udidi lwengcaciso kunendlela ebonisiweyo.

Uxinzelelo sisiqalekiso somzimba, hayi isafobe. Iiseli zeMast zithwala i-receptors ye-corticotropin-ekhulula ihomoni kwaye zihlale kufuphi neziphelo zemithambo-luvo, zibonelela ngendlela ebhaliweyo apho inkqubo ye-nervous ichaphazela ukusebenza kwayo. Le ngongoma ifuna ukugxininiswa kuba izigulana ezineempawu eziya zisiba mandundu ngenxa yoxinzelelo zihlala zixelelwa ukuba imeko yazo ingokwengqondo.Indlela eboniswayo ye-neuroimmune ayifani nophawu olucingelwayo.

Uninzi lwezigulana lubonisa iimpawu zokuqala kwisifo esosulelayo. Ukosuleleka kwaziwa ngokuvula iiseli zemast ngokusebenzisa ii-receiver ezifana ne-Toll-like, ukubonelela ngendlela ecacileyo, kwaye ukuqala kwasemva kokosulela yipatheni eyaziwayo kwiimeko ezininzi ezingapheliyo. Nangona kunjalo, ukuseka ukuba usulelo bangelwa I-MCAS kwimeko yomntu inzima ngokwendlela, kwaye ukukhumbula ukuthathwa komkhethe kuchaphazela ii-akhawunti ezibuyela emva. Oku kuhlala kungumbutho ophantsi kophando.

Ukungasebenzi kakuhle kwamajoni omzimba

I-Broad immune disregulation yindawo ehamba phambili yophando. Iindlela ezicetywayo ziquka ukuguqulwa kwentetho ye-receptor okanye uvakalelo kwiiseli zemast, ukuphazamiseka komqondiso olawulayo onokuthi ngokuqhelekileyo uthintele ukusebenza, kunye nokusebenzisana okungaqhelekanga phakathi kweeseli zemast kunye nabanye abantu abakhusela umzimba. Ezi zikholeleke kwaye zifundwe ngenkuthalo, kodwa zichaza udidi lwengcaciso kunendlela ebonisiweyo.

Ukudumba okungapheliyo

Imiba yokusingqongileyo

Abaxhasi abacetywayo bokusingqongileyo babandakanya ukuvezwa kweekhemikhali, ukungunda, ukungcoliseka komoya, kunye nokutshintsha kwezidlo kwinqanaba labantu. Ubungqina apha buthathaka kakhulu kunezi ndawo zingasentla, kwaye uninzi lwayo aluthanga ngqo. Lo ngummandla obambekayo wophando endaweni yokuba ube negalelo elimiselweyo, kwaye ikwayindawo apho amabango orhwebo athembekileyo exhaphakile kwaye axhaswa kakubi.

Ukudumba okungapheliyo

Iiseli zeMast zombini ziyaphendula kwaye zibe negalelo ekudumbeni, nto leyo ephakamisa ukuba nokwenzeka komjikelo wokuziqinisa apho ukusebenza kuzala indawo enokudumba ethoba umda wokuqhubela phambili. Oku kuhambelana ngobuchwephesha kwaye kuhambelana nokuqwalaselwa kweklinikhi ukuba ukuphinda kusebenze kuyanda ngexesha lolawulo olulambathayo. Ihlala ingumzekelo kunokuba ibe ngunobangela obonisiweyo.

Epigenetics

Umfanekiso ocetywayo

Umzobo wenqanaba lobungqina: iibhendi ezigxininisiweyo ezihamba ngaphandle zisuka 'Ezimiselwe' (uphawu lwe-HaT; KIT D816V kwi-mastocytosis) ngokuthi 'Phandwe ngokukhutheleyo kunye nobungqina obuxhasayo' (i-immune dysregulation, post-infectious start) ukuya 'ku-Hypothesised, ubungqina obunqongopheleyo obuthe ngqo' (epigenetics, i-exposure exposure yokusingqongileyo).

Isigulana esinovelwano oluphindaphindekayo, uvavanyo lwe-IgE olubi, kunye nezinto ezibangela umzimba ezifana nobushushu okanye uxinzelelo yinkcazo-ntetho ye-MCAS eqhelekileyo kunesigulana esinovavanyo oluqinisekileyo lokwaliwa. Indawo yolawulo egxekayo idla ngokuba yinxalenye yomfanekiso kunengxoxo echasene nayo.
  • Apho oku kushiya izinto
  • I-HaT kunye ne-KIT D816V uguquko kwi-mastocytosis zisekiwe kwi-genetics - kwaye ayisosizathu sisekiweyo se-MCAS.
  • I-immune dysregulation kunye nokuqala emva kokosuleleka kuphandwa ngenkuthalo kunye nobungqina obuxhasayo.
  • Akukho nobangela we-MCAS usekiwe.
  • Waphathe ngokuthandabuza amabango 'engcambu engunobangela' othembekileyo - uluntu lophando aluchonge nokuba libe linye.
Icandelo 09

Ukuhlala Na I-MCAS

Waphathe ngokuthandabuza amabango 'engcambu engunobangela' othembekileyo - uluntu lophando aluchonge nokuba libe linye.

Ulawulo lwemihla ngemihla kunye neenkqubo

Uninzi lwabaguli bafumanisa ukuba ukungaguquguquki kuphakamisa umda wabo ngokuthembekileyo kunawo nawuphi na umlinganiselo womntu. Ukulala rhoqo kunye namaxesha okuvuka, ixesha lokutya eliqikelelweyo, ubushushu be-ambient obuzinzileyo apho bufumaneka khona, kunye nexesha elingaguqukiyo lamayeza konke kunciphisa ukuguquguquka. Iindlela zesiqhelo zenza ukuba ukuchongwa kwe-trigger kwenzeke: xa uninzi lwezinto eziguquguqukayo zizinzile, ezo zitshintshileyo ziyabonakala.

Ulawulo ubukhulu becala lwakhiwe kwizenzo zemihla ngemihla kunokuba luvele kulo naluphi na ungenelelo olunye. Oku kulandelayo kujolise ekusebenzeni-injongo yobona bomi bubanzi bomzimba wakho oya kuxhasa.

Ulawulo lwemihla ngemihla kunye neenkqubo

Uninzi lwabaguli bafumanisa ukuba ukungaguquguquki kuphakamisa umda wabo ngokuthembekileyo kunawo nawuphi na umlinganiselo womntu. Ukulala rhoqo kunye namaxesha okuvuka, ixesha lokutya eliqikelelweyo, ubushushu bendawo obuzinzileyo apho bufumaneka khona, kunye nexesha elingaguqukiyo lamayeza konke kunciphisa ukuguquguquka. Iindlela zesiqhelo zenza ukuba ukuchongwa kwe-trigger kwenzeke: xa uninzi lwezinto eziguquguqukayo zizinzile, ezo zitshintshileyo ziyabonakala.

I-Pacing ingumxholo ophindaphindiweyo kwiiakhawunti zesigulane - ukusabalalisa umsebenzi ngosuku okanye ngeveki kunokuba ugxininise, kunye nokwakha ngexesha lokubuyisela emva kweemfuno ezaziwayo. Oku akufani nokungasebenzi, kwaye ukungasebenzi ixesha elide kuneendleko zakho ezibhalwe kakuhle.

I-classical allergy yi-IgE-mediated reaction into ethile, into ebonakalayo. Iyakwazi ukuveliswa kwakhona - ukuvezwa okufanayo kuvelisa ukusabela okufanayo - kwaye ngokuqhelekileyo kunokubonakaliswa ngovavanyo lwe-skin-prick okanye uvavanyo oluthile lwegazi lwe-IgE. Ukwaliwa kwamandongomane kuziphatha ngolu hlobo; ngokunjalo i-cat dander allergy.

Idayari yeempawu yenye yezona zinto ziluncedo kakhulu isigulana esinokuzisa kwidinga: ikhonkrithi, i-longitudinal, kunye nethe ngqo kubo ngendlela ekungekho ludwe lwe-trigger olupapashiweyo olunokubakho. Ikwanceda ukwahlula izinto ezibangela ukwenzeka kwenyani ukusuka kwingozi, nto leyo inkumbulo iyodwa eyenza kakubi.

Ingxelo ecwangcisiweyo yeyona ndlela ithembekileyo yokuchonga izinto ezibangela umntu. Amangenelo aluncedo ahamba kakuhle ngaphaya kokutya: ixesha lemini, ubushushu be-ambient, into eyayidliwe kwaye yayintsha kangakanani, umsebenzi womzimba, uxinzelelo kunye nokulala, ukuvezwa kwevumba, amayeza athathiweyo, kunye - kwizigulana ezisexesheni - indawo yomjikelo. Iimpawu kufuneka zirekhodwe ngexesha kunye nobunzima.

Ukubonisa iimpawu ezicwangcisiweyo ngenkqubo yamalungu kunokuba ngokulandelelana yenze ipateni yenkqubo eninzi ibonakale ngokukhawuleza, apho i-akhawunti ebalisayo ithande ukuyifihla. Ukuzisa iziphumo zangaphambili - kubandakanywa neziqhelekileyo - kufundisa ngokwenene kwaye kuthintela ukuphinda uphando olugqityiweyo.

Idayari yeempawu yenye yezona zinto ziluncedo kakhulu isigulana esinokuzisa kwidinga: ikhonkrithi, i-longitudinal, kunye nethe ngqo kubo ngendlela ekungekho ludwe lwe-trigger olupapashiweyo olunokubakho. Ikwanceda ukwahlula izinto ezibangela ukwenzeka kwenyani ukusuka kwingozi, nto leyo inkumbulo iyodwa eyenza kakubi.

Ukusebenza noogqirha

Iindawo zokuhlala eziqhelekileyo ziquka imigaqo-nkqubo yeklasi engenavumba, imvume yokuthwala kunye nokuzilawula ngokwakho amayeza, ukufikelela kumanzi kunye nekhefu lokuphumla, ukuqwalaselwa kobushushu kwindawo yokuhlala, ukuguquguquka malunga nokuhamba ngexesha lokuvutha, kunye nohlengahlengiso lwemfundo yomzimba. Apho kukho izicwangciso zeendawo zokuhlala ezisesikweni kwinkqubo yakho yemfundo, ukuba nesi sifo sibhalwe ngokubhaliweyo ngokuqhelekileyo kwenza ukufikelela kuzo kube lula kakhulu.

Ukubuza ngokuthe ngqo malunga nexesha lovavanyo lomlamli kunye nokuphathwa kwesampulu kunengqiqo kwaye kuphucula ngokubonakalayo ithuba lesiphumo esisebenzisekayo. Ukuba awuviwa, ukufuna uluvo lwesibini kusemthethweni; isifo se-mast cell yindawo ye-niche kwaye ukuqhelana kuyahluka ngokubanzi. Ukugxothwa kube kanye kuyinto eqhelekileyo kwaye ayisosigqibo.

Isikolo

Iindawo zokuhlala eziqhelekileyo ziquka imigaqo-nkqubo yeklasi engenavumba, imvume yokuthwala kunye nokuzilawula ngokwakho amayeza, ukufikelela kumanzi kunye nekhefu lokuphumla, ukuqwalaselwa kobushushu kwindawo yokuhlala, ukuguquguquka malunga nokuhamba ngexesha lokuvutha, kunye nohlengahlengiso lwemfundo yomzimba. Apho kukho izicwangciso zeendawo zokuhlala ezisesikweni kwinkqubo yakho yemfundo, ukuba nesi sifo sibhalwe ngokubhaliweyo ngokuqhelekileyo kwenza ukufikelela kuzo kube lula kakhulu.

Sebenza

Iingqwalasela zasemsebenzini zifana nezo zesikolo: imigaqo-nkqubo yevumba, ulawulo lobushushu, amalungiselelo aguquguqukayo okanye akude ngexesha lokudangala, kunye nohlengahlengiso lweshedyuli apho ukudinwa kulandela ipateni eqikelelwayo. Ukuba ngaba kwaye kungakanani ukuxela sisigqibo somntu kunye norhwebo lokwenene, kwaye lusebenzisana nokhuseleko lwengqesho olwahluke kakhulu phakathi kwegunya.

Ukuhamba

Amanyathelo asebenzayo adla ngokukhankanywa zizigulane ziquka ukuthwala amayeza kwimithwalo yesandla kunye namaxwebhu, ukufumana ileta kagqirha kumayeza atofwayo, ukuphanda amaziko onyango kwindawo ekuyiwa kuyo, ukucwangcisa ukutya kwangaphambili apho ukuphinda kusebenze kubalulekile, kunye nokuvumela ixesha lokubuyisela ekufikeni. Amaqondo obushushu kunye notshintsho lokuphakama komgangatho kufanelekile ukulindela.

Impilo Yengqondo

Ukuphila ngokuguquguquka, ukungaqondwa kakuhle, kunye nesigulo esisoloko sigxothwa kuneendleko zokwenyani ngokwasengqondweni, kwaye ezo ndleko ayibobuthathaka bomlinganiswa. Izigulana ezininzi zichaza ixesha elide lokungakholelwa phambi kokuxilongwa, okushiya uphawu lwayo. Inkxaso - nokuba yingcali, iontanga, okanye zombini - inxalenye efanelekileyo yolawulo kunokuba ivume ukuba iimpawu zingokwengqondo.

  • Umahluko ofanele ukubamba ngulo: ukukhululwa komlamli kunokuvelisa ngokunyanisekileyo i-physiology efana nexhala, kwaye oko kufanelwe ukuqatshelwa kunokuba kubhalwe kwakhona ngengqondo kuyo yonke imeko. Kwangaxeshanye, unxunguphalo kunye nokudakumba zixhaphakile, ziyanyangeka, kwaye zifanele ukujongana nazo ngokwazo xa zikhona.
  • I-Epinephrine lunyango lokuqala lwe-anaphylaxis. I-Antihistamines ayithathi indawo kwaye ayifanele ixhomekeke kwi-reaction acute.
  • Ezinye izigulana ezinesifo semast cell zisemngciphekweni we-anaphylaxis-intshukumo ekhawulezayo, ebukhali, enokuthi ibeke esichengeni ubomi. Apho loo mngcipheko usebenza khona, ukucwangcisa akukhethi, kwaye yincoko onokuthi ube nayo nogqirha ngaphambi kokuba ifuneke.
  • Izigulana ezivavanyiweyo ukuba zisemngciphekweni zinikwa i-epinephrine auto-injection kwaye zicetyiswa ukuba zihlale zihamba nazo.
  • I-Epinephrine lunyango lokuqala lwe-anaphylaxis. I-Antihistamines ayithathi indawo kwaye ayifanele ixhomekeke kwi-reaction acute.
  • Isicwangciso senyathelo likaxakeka esibhaliweyo ekuvunyelwene ngaso nogqirha wakho kufuneka sichaze iimpawu ezilumkisayo, into omawuyiphathe, kunye nexesha lokutsalela umnxeba kwiinkonzo zikaxakeka.
Iingxelo ezibangela imo engqongileyo ziquka ukungunda, uthuli, umungu, umsi, ungcoliseko lomoya, utshintsho lokufuma, kunye nokutshintsha koxinzelelo lwebarometric. Uvakalelo luyahluka ngokubanzi, kwaye ukuchonga iziphembeleli zokusingqongileyo kudla ngokufuna ukugcinwa kweerekhodi ixesha elide kunokuqwalasela okunye.

Izigulana ezivavanywe ukuba zisemngciphekweni ngokuqhelekileyo zimiselwe i-epinephrine auto-injection kwaye ziyalelwe ukuba ziziphathe rhoqo. I-epinephrine yonyango lokuqala lwe-anaphylaxis; i-antihistamines ayithathi indawo yayo kwaye akufanele ixhomekeke kwi-reaction ebukhali.

Inqaku ngothintelo lokutya

Ukuchongwa kwezonyango ukuqaphela ukuxilongwa kunye neyeza ezaziwayo ezibangela ukuba zincede abasebenzi bexesha likaxakeka abanokuthi bangayazi imeko.

Utyando kunye ne-anesthesia iqinisekisa ingxoxo kwangaphambili ukuze ii-arhente zikhethwe ngemeko engqondweni.

Isigulana esinovelwano oluphindaphindekayo, uvavanyo lwe-IgE olubi, kunye nezinto ezibangela umzimba ezifana nobushushu okanye uxinzelelo yinkcazo-ntetho ye-MCAS eqhelekileyo kunesigulana esinovavanyo oluqinisekileyo lokwaliwa. Indawo yolawulo egxekayo idla ngokuba yinxalenye yomfanekiso kunengxoxo echasene nayo.
  • Iindlela ezihambelanayo ziphakamisa umqobo kwaye zenze ukuchongwa kwe-trigger kwenzeke.
  • Idayari yeempawu ezicwangcisiweyo kwiiveki yeyona ndlela ithembekileyo yokuchonga izinto ezibangela umntu.
  • Ukubonisa iimpawu ngesixokelelwano somzimba kwenza ukuba ipateni yenkqubo eninzi ibonakale koogqirha.
  • Iindlela ezihambelanayo ziphakamisa umqobo kwaye zenze ukuchongwa kwe-trigger kwenzeke.
  • Idayari yeempawu ezicwangcisiweyo kwiiveki yeyona ndlela ithembekileyo yokuchonga izinto ezibangela umntu.
  • Ukubonisa iimpawu ngesixokelelwano somzimba kwenza ukuba ipateni yenkqubo eninzi ibonakale koogqirha.
Icandelo le-10

Ebuzwa Rhoqo Imibuzo

Imibuzo engama-25 izigulane, iintsapho, kunye noogqirha babuza rhoqo. Khetha nawuphi na umbuzo ukuwukhulisa.

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Ngaba i-MCAS yelifa?

Iimpawu zinokuphucula kakhulu, ngamanye amaxesha ukuya kwindawo apho izigulana zizichaza njenge-asymptomatic ubukhulu becala - ngakumbi xa unyango luthoba i-reactivity yesiseko kunye nezinto ezibangela ukuba zichongwe. Usombululo oluzenzekelayo, olusisigxina ngaphandle kwalo naluphi na ulawulo alubhalwanga kakuhle. Amaxesha oxolelo alandelwa kukuphinda achazwe ngokuqhelekileyo.

Izinto ezininzi ziyatshintsha ngokuhamba kwexesha: isiseko sokusebenza kwakhona sihamba nokulala, uxinzelelo, imeko yehomoni, kunye nosulelo lwakutsha nje; iiseli zemast zinokukhupha imidibaniso eyahlukeneyo yomlamli ngezihlandlo ezahlukeneyo; kwaye i-trigger izibeka ngokwazo ukwandisa ngexesha lolawulo olulambathayo kwaye zihlala zicutha kwakhona ngonyango. Ukutshintsha iimpawu luphawu lwemeko kunokuba luphawu lokuba ukuxilongwa kuphosakele.

Ngokuqhelekileyo ngenxa yezizathu zobugcisa. Abalamli abalinganisiweyo banobomi obufutshane obufutshane, ngoko ke isampuli phakathi kweempendulo ayinakufumana nto iphakamileyo. Uninzi aluzinzanga kubushushu begumbi kwaye lufuna ukugodola kwangoko kunye nokusetyenzwa ngokukhawuleza - isampulu eyayihleli okanye yalibaziseka kuhambo inokufunda okuqhelekileyo kwisigulana ebesisabela ngokwenyani.Umsebenzi wegazi oqhelekileyo kunye neepaneli zokungalungi nazo zilindeleke ukuba ziqheleke kwi-MCAS, kuba aziyilwanga ukuyibhaqa.

Ewe, ngokusebenzisa indlela yefiziyoloji ebhaliweyo. Iiseli zeMast zithwala ii-receptors zehomoni ekhupha i-corticotropin kwaye zihlala kufutshane neziphelo zemithambo-luvo, zinika uxinzelelo lwe-physiology ukufikelela ngokuthe ngqo kuzo. Oku kubalulekile ukukuchaza ngokucacileyo, kuba izigulana ezineempawu eziba mandundu phantsi koxinzelelo zihlala zixelelwa ukuba imeko yezengqondo. Indlela ye-neuroimmune ayifani nophawu olucingelwayo.

Hayi. Utshintsho lokutya lunokunciphisa umthwalo weempawu kwezinye izigulane ngokunciphisa umthwalo opheleleyo we-trigger, kwaye loo nzuzo ifanelekile ukuyilandela ngokucinga - kodwa ayiyi kunyanga imeko. Ubungqina bokutya okune-histamine ephantsi kulinganiselwe kwaye kunomgangatho ophantsi. Isithintelo esihlala ixesha elide esingajongwanga sithwala imingcipheko yokwenyani yokunqongophala kwesondlo kunye nokutya okungalungelelananga, ngoko ke ulingo olucwangcisiweyo, olunexesha elilinganiselweyo nokuqaliswa kwakhona lukhuselekile kunokuphepha okuvulekileyo.

Kwi-mastocytosis umzimba uqokelela iiseli zemasti ezininzi kakhulu, kwaye oko kugqithise kunokubonakaliswa-nge-biopsy yomongo wethambo, izilonda zesikhumba, okanye i-tryptase esisiseko ehlala inyuswa, ihlala i-KIT D816V yenguqu kwisifo se-systemic yabantu abadala. Kwi-MCAS inani leeseli zemast liqhelekile; into engaqhelekanga yindlela ezisebenza ngayo ngokulula. Kungenxa yoko le nto i-mastocytosis inokuqinisekiswa ngokufumana iiseli kwaye i-MCAS ayinako.

Akunjalo, nangona ngokufuthi bebhideka. Ukunganyamezeli kwe-histamine ngokuqhelekileyo kuchazwa njengobunzima bokuphula i-histamine etyiweyo, ehlala ibangelwa kukunciphisa umsebenzi we-enzyme diamine oxidase. I-MCAS ibandakanya iiseli zemast yomzimba ezikhupha ihistamine kunye nabanye abalamli abaninzi ngokungafanelekanga. Ziyakwazi ukuvelisa iimpawu ezidityanisiweyo kwaye zinokubakho kunye, kodwa iindlela ziyahluka.

Kulapho kanye iingcali zingavumelaniyo. Iikhrayitheriya zokuvumelana zifuna ubungqina obunenjongo bokukhululwa komlamli, kwaye oogqirha abaninzi babambelela koko ngokungqongqo. Abanye bathi isicelo esingqongqo siphosakele izigulane ezivavanywa ngokuphindaphindiweyo ngenxa yezizathu zexesha kunye nokuphatha. Ingxoxo-mpikiswano ayilungiswanga kwiincwadi, yingakho iingcali ezimbini ezinobuchule zinokufikelela kwizigqibo ezahlukeneyo malunga nesigulane esifanayo.

I-Allergists kunye ne-immunologists ngokuqhelekileyo, kunye nee-hematologists apho i-mastocytosis iqwalaselwa. Ngokwesiqhelo, ukuqhelana kuyahluka kakhulu nangaphakathi kwezo zikhethekileyo, kwaye ukufikelela koogqirha abanamava eseli yemast akulingani ngokwejografi. Izigulana ezininzi zifumanisa ukuba ugqirha onolwazi oluphangaleleyo ozimisele ukuququzelela ukhathalelo luxabiseke njengokuqeshwa kweengcali.

Ngokuqhelekileyo bancedisa kodwa abafane basombulule yonke into, kwaye loo mpendulo ingaphelelanga ilindeleke kunokuba isibonakaliso sokuxilongwa akulungile. I-Antihistamines ivimba i-histamine kuphela; iprostaglandins, i-leukotrienes, i-platelet activating factor, kunye nee-cytokines azichaphazeleki. Yiyo kanye loo nto unyango lukhula kwi-tiers, ukongeza ii-arhente ezijongana nabalamli be-antihistamines bayaphoswa.

Iingcaciso ezimbini eziqhelekileyo. Ezinye iiklasi zamachiza zingenza iiseli zemast zisebenze ngokuthe ngqo, zixhomekeke kuyo nayiphi na indlela ekwaliwa ngayo. Ngokwahlukileyo, izithako ezingasebenziyo - iidayi, izihluzi, izigcini - zinokuba yinto ephazamisayo kunokuba iyeza elisebenzayo, yingakho isigulane sinokunyamezela uguqulelo lomenzi omnye kwaye siphendule komnye. Amalungiselelo adityanisiweyo ngamanye amaxesha asetyenziswa apho oku kukrokreleka.Xoxa ngazo naziphi na izenzo ezikrokrelekayo kunye nomyalelo wakho endaweni yokuma ucalanye.

Kungaba njalo, kuxhomekeke kubungqongqo kunye nesakhelo somthetho apho uhlala khona. Ezinye izigulana zisebenza kwaye zifunde ngaphandle kohlengahlengiso; ezinye zisikelwe umda kakhulu. Ukumiselwa kokukhubazeka kuxhomekeke kulawulo oluthile kwaye ngokubanzi kufuna amaxwebhu empembelelo yokusebenza kunokuxilongwa kuphela. Iakhawunti ebhaliweyo kagqirha yothintelo idla ngokuba luncedo ngakumbi kuneleyibhile yoxilongo iyodwa.

Ngaba i-MCAS isiphene?

Amava axeliweyo ahluka kakhulu - ezinye izigulana zichaza ukuphuculwa ngexesha lokukhulelwa, ezinye ziba nzima, kwaye ezinye azikho utshintsho olucacileyo. Impembelelo yeHormonal kwiiseli zemast ibhaliwe, eyenza utshintsho kulo naliphi na icala libonakale. Ukukhulelwa nge-MCAS kuqinisekisa ukucwangciswa kwangaphambili kunye nogqirha wakho we-mast cell kunye neqela lakho lokubelekisa, ngakumbi malunga nokuba ngawaphi amayeza aya kuqhubeka.

Ngenxa yokuba iiseli zemast ziphendula ngokuthe ngqo kwi-stimuli yomzimba, kungekhona kuphela kwizinto ezithintekayo. Ubushushu, ukubanda, uxinzelelo, ukukhuhlana, kunye nokuzibhokoxa konke kunokuzenza zisebenze ngaphandle kwento engena emzimbeni. Uvavanyo lwe-allergies lwenzelwe ukufumanisa i-IgE-mediated sensitization kwiiprotheni ezithile, ngoko ayinako ukubona ezi zinto zibangelayo-yiyo loo nto iphaneli yokwaliwa ecocekileyo ihambelana ngokupheleleyo neempendulo zokwenyani kubushushu.

Kuxhomekeke kwi-arhente. Ama-Antihistamines ahlala ebonisa ukusebenza phakathi kweentsuku. Izinzisi zeeseli zeMast ezifana necromolyn kunye ne-ketotifen zifuna iiveki zokusetyenziswa rhoqo phambi kokuba inzuzo yazo epheleleyo ibonakale, kwaye ukuyeka kwangethuba yindlela eqhelekileyo yokulahla into ebinokusebenza. Ulingo olumalunga neeveki ezine ukuya kwezintandathu ngotshintsho ngumgaqo osoloko ucatshulwa.

Ngokubanzi hayi, kwaye le yenye yeempazamo eziqhelekileyo zokuzilawula. Ukuqala ii-arhente ezininzi kunye kwenza isiphumo singatolikwa: ukuba izinto ziyaphucuka, awukwazi ukuxelela ukuba loluphi utshintsho ebeluxanduva, kwaye ukuba uyasabela, awukwazi ukuxela ukuba uyeke phi. Inguqu enye ngexesha iyacotha kodwa yinto eyenza ukuba inkqubo ifundeke.

Izigulana ezininzi ziqala ngokubonakalisa iimpawu zesigulo esosulelayo, kwaye usulelo luyaziwa ngokuvula iiseli zemast ngokusebenzisa ii-receptors ezinjenge-Toll-like, ngoko ke indlela ecetywayo iyavakala. Nokuba usulelo lubangela i-MCAS ayisekwanga, kwaye ukubonisa i-causation kwiimeko zomntu ngamnye kunzima ngokwendlela. Ngokumalunga ne-COVID ende ngokuthe ngqo, ukubandakanyeka kweeseli zemast yingcinga esebenzayo enobungqina bokuqala, hayi ukufunyaniswa okuzinzisiweyo.

Ifunyaniswa rhoqo kubasetyhini. I-Estrogen ichaphazela ukuziphatha kweeseli zemast, enye inkcazo ecetywayo, kwaye izigulane ezininzi zichaza iipatheni zeempawu zebhayisikile. Ingaba umahluko ubonisa ukuxhaphaka kokwenyani okanye ukungafani kokuxilongwa kunye nokuthunyelwa akusonjululwanga ngokupheleleyo - i-caveat esebenza kwinani leemeko ezifunyanwe kakhulu kubasetyhini.

Amanani anyanisekileyo okuxhaphaka akakabikho. Le meko ibonakaliswe ngokusesikweni kuphela ngo-2007, iindlela zokuxilonga zihlala zixoxwa, kwaye izenzo ziyahluka phakathi kwamazwe. Uqikelelo olupapashiweyo luluhlu olubanzi, kwaye ububanzi kolo luhlu ngokwalo lufunyenwe. Yiba nentandabuzo ngokuzithemba amanani okuxhaphaka okuchanekileyo kuwo nawuphi na umthombo.

Hayi, kwaye ukuzama ukwenza oko kudla ngokuchaseneyo. Izintlu ezipapashiweyo ziqala iingcamango zokuvavanya ngokuchasene nerekhodi yakho, kungekhona imithetho esebenza ngokufanayo - ukuhluka komntu ngamnye kuninzi, kwaye izigulane ezininzi zinyamezela izinto ezivela kulo lonke uluhlu. Ngenxa yokuba i-stack evuselelayo, ubutsha kunye nomthwalo opheleleyo uhlala ubaluleke ngaphezu kwako nakuphi na ukutya okunye. Sebenzela kowona mqobo ufikelelayo kulawulo.

Imbali yeempawu ezicwangciswe yinkqubo yamalungu kunokuba ngokulandelelana kweziganeko, nto leyo eyenza ukuba ipateni ye-multisystem ibonakale ngokukhawuleza. Iimpawu kunye nedayari yokuqalisa egubungela ubuncinci iiveki ezimbini. Zonke iziphumo zovavanyo lwangaphambili, kubandakanywa neziqhelekileyo, kuba ezi ziyinxalenye yenkqubo yokukhutshelwa ngaphandle. Uluhlu lwangoku lwamayeza kunye noluhlu lokongezelela.Kwaye uluhlu olufutshane olubhaliweyo lwemibuzo yakho - ukuqeshwa kufutshane kwaye kulula ukuhamba ngaphandle kokubuza eyona nto ibalulekileyo.

Icandelo le-11

Isifundo esongezelelweyo Izibonelelo

Amanqaku okuqalisa alungiselelwe ukufunda nzulu. Amakhonkco athile ayaqulunqwa kwaye aya kongezwa apha.

Izikhokelo zesigulane

Iintshayelelo ezifikelelekayo ezibhalelwe izigulane kunye neentsapho, ezifanelekileyo ukwabelana nabantu abatsha kwimeko.

  • Intshayelelo yesikhokelo sesigulana - ikhonkco liza kongezwa
  • Ipakethi entsha yokuqhelaniswa nokuxilongwa - ikhonkco liza kongezwa
  • Uphawu oluprintwayo kunye netemplate yedayari yokuqalisa - ikhonkco liza kongezwa
  • Intshayelelo yesikhokelo sesigulana - ikhonkco liza kongezwa

Izikhokelo zonyango

Iingxelo zemvumelwano kunye nezikhokelo zeklinikhi ezijoliswe kubasebenzi bezempilo. Kuluncedo ukuzisa amadinga.

  • Isikhokelo samalungu osapho kunye nabanonopheli — ikhonkco liza kongezwa
  • Isikhokelo soluntu lwengcali yezonyango - ikhonkco liza kongezwa
  • Uvavanyo lomlamli kunye neprotocol yokuphatha isampula - ikhonkco liza kongezwa
  • Indlela yokuxilonga yemvumelwano - ucaphulo kunye nekhonkco kufuneka zongezwe

Amaphepha oPhando

Uncwadi olusisiseko. Apho kunokwenzeka, khetha imithombo yolwazi ephononongwe ngoontanga kunye nemihla yokupapashwa, njengoko lo mmandla ushukuma.

  • Amaphepha abalinganiswa abasisiseko (2007 ukuya phambili) - izicatshulwa ziya kongezwa
  • Amaphepha enkqubo yemvumelwano (2010, 2012) - izicatshulwa ziya kongezwa
  • Uphando lwe-alpha tryptasemia yofuzo- izicatshulwa ziya kongezwa
  • Uphononongo lweemeko ezinxulumeneyo - izicatshulwa ziya kongezwa

Imibutho yobuGcisa

Uluntu lweengcali kunye namaqumrhu ezemfundo asebenza kwisifo seeseli zemast.

  • Uphononongo lweemeko ezinxulumeneyo - izicatshulwa ziya kongezwa
  • Uthungelwano lophando lwesifo seeseli- amakhonkco ukuba wongezwe
  • Imibutho yezifo ezinqabileyo- amakhonkco ukuba wongezwe

Iincwadi

Ufundo olude lwezigulane kunye neekliniki.

  • Imibutho yezifo ezinqabileyo- amakhonkco ukuba wongezwe
  • Izicatshulwa zezonyango- ukuba yongezwe

Ufundo olude lwezigulane kunye neekliniki.

Iintetho ezirekhodiweyo, iingcaciso, kunye nemathiriyeli yenkomfa.

  • Izicatshulwa zezonyango- ukuba yongezwe
  • Iintetho zeenkomfa zeengcali - amakhonkco ukuba wongezwe
  • Iinkcukacha ngomxholo wezemfundo we-MCAS — amakhonkco ukuba wongezwe

Imibutho yeNkxaso

Inkxaso yoontanga, ubhengezo, kunye noluntu.

  • Iinkcukacha ngomxholo wezemfundo we-MCAS — amakhonkco ukuba wongezwe
  • Uluntu lwenkxaso yoontanga- amakhonkco ukuba wongezwe
  • Amaqela enkxaso kwingingqi nakuzwelonke — amakhonkco ukuba wongezwe

Iimvavanyo zeklinikhi

Izifundo ezifunwayo ngoku. Ukufumaneka kovavanyo kutshintsha rhoqo, ke jonga uluhlu lobhaliso ngokuthe ngqo.

  • Amaqela enkxaso kwingingqi nakuzwelonke — amakhonkco ukuba wongezwe
  • Okwangoku kufunwa izifundo zemast cell- amakhonkco ukuba wongezwe
  • Isikhokelo malunga nokuba inxaxheba kulingo ibandakanya ntoni - ikhonkco liza kongezwa
Icandelo le-12

Ngoku kufunwa izifundo zemast cell- amakhonkco ukuba wongezwe

Awona magama abalulekileyo kwesi sikhokelo, afinyeziwe.

Isishwankathelo
  • I-MCAS luphazamiseko lwemast cell engafanelekanga ukusebenza, kungekhona amanani eeseli zemast - iiseli ziqhelekileyo, i-threshold yazo ayikho.
  • Awona magama abalulekileyo kwesi sikhokelo, afinyeziwe.
  • Isishwankathelo
  • Abalamli bakhulula ngamaza: baqulunqwe kwangaphambili ngemizuzwana, abalamli be-lipid ngemizuzu, ii-cytokines ngeeyure. Isiganeko esinye sokuvula singathatha usuku.
  • Iiseli zeMast ziluncedo ngokusemthethweni kwiiseli zokhuselo lomzimba ezibekwe kwimiqobo yomzimba, zibambe abalamli abenziwe kwangaphambili belungele ukukhulula ngemizuzwana.
  • Iimpawu ziyi-episodic, ziyaphindaphindeka, kwaye zibandakanya iinkqubo zamalungu amabini okanye ngaphezulu - loo pateni, hayi naluphi na uphawu lomntu, yinto ecebisa ukuxilongwa.
  • Abalamli bakhulula ngamaza: baqulunqwe kwangaphambili ngemizuzwana, abalamli be-lipid ngemizuzu, ii-cytokines ngeeyure. Isiganeko esinye sokuvula singathatha usuku.
  • I-Histamine ngumlamli omnye kuphela phakathi kwabaninzi, yiyo loo nto i-antihistamines idla ngokunika isiqabu esipheleleyo kunokuba sikhululeke ngokupheleleyo.
  • Izinto ezibangelayo. Umthwalo owongezelekayo uchaza iimpendulo ezibonakala zingenamkhethe, kwaye ngoyena mbono uluncedo kulawulo lwemihla ngemihla.
  • Izinto ezibangela umzimba - ukushisa, ukubanda, uxinzelelo, ukunyamezela - akufuneki into efakwe kuyo kwaye ayibonakali kuvavanyo oluqhelekileyo lwe-allergies.
  • Ukubandakanyeka kweMast cell yi isekiwe kwi-urticaria engapheliyo kunye ne-asthma; Unxulumano nePOTS, i-hEDS, i-IBS kunye ne-COVID ende zixeliwe kodwa azisonjululwanga ngoomatshini.
  • Uninzi lovavanyo lomlamli olubi lubonisa ixesha kunye neengxaki zokuphatha iisampulu endaweni yokungabikho kwesifo.
  • Iikhrayitheriya zokuxilonga zihlala ziphikisana ngokunyanisekileyo, ngoko ke iingcali ezinobuchule zinokufikelela kwizigqibo ezahlukeneyo malunga nesigulane esifanayo.
  • Apho umngcipheko we-anaphylaxis usebenza, i-epinephrine kunye nesicwangciso sesenzo esibhaliweyo zibalulekile; i-antihistamines ayithathi ndawo.
  • Akukho nobangela we-MCAS usekiweyo - phatha 'ingcambu engunobangela' ozithembileyo ngokuthandabuza.
Icandelo le-13

Uluhlu lwamagama

Amagama angama-91 asetyenziswa kuncwadi lwe-MCAS kunye nencoko yonyango, echazwe ngokucacileyo.

A
I-Adrenaline
Jonga i-Epinephrine. Igama laseBrithani kunye nelamazwe ngamazwe elingelona ilungelo lehomoni efanayo kunye namayeza.
Intloko ekhanyayo okanye isiqaqa, rhoqo xa umile
A
Unyuswe, ukurhawuzelela ukumanzisa; inokuba yedlula kwaye ifuduke
I-Adrenaline
Jonga i-Epinephrine. Igama laseBrithani kunye nelamazwe ngamazwe elingelona ilungelo lehomoni efanayo kunye namayeza.
Iyeza elithintela i-histamine receptors. Abathinteli be-H1 basebenza ikakhulu kwesikhumba kunye neempawu zomoya; Izithinteli ze-H2 zisebenza kakhulu kwi-gastrointestinal tract.
Ukudumba kolusu okanye kulwelwe olunzulu, okudla ngokuchaphazela imilebe, iinkophe, izandla, okanye umqala. Ukubandakanyeka komqala yingxakeko yonyango.
I-Antihistamine
B
Ubalo lweeseli
ISerum tryptase ilinganiswa xa iimpawu zithe cwaka. Ibonelela ngenqaku lothelekiso ekugwetywe ngalo ukunyuka okuqatha.
Inxalenye yenkqubo ye-nervous elawula imisebenzi ezenzekelayo efana nesantya sentliziyo, uxinzelelo lwegazi, kunye nokwetyisa. Ukungasebenzi kwayo kubizwa ngokuba yi-dysautonomia.
B
ISerum tryptase ilinganiswa xa iimpawu zizolile. Ibonelela ngenqaku lothelekiso ekugwetywe ngalo ukunyuka okuqatha.
Basophil
Iseli emhlophe yegazi ejikelezayo eyabelana ngeempawu ezithile kunye neeseli zemast, kubandakanya i-histamine granules, kodwa yahluke ngendawo kunye nomjikelo wobomi.
Biomarker
Isalathisi sebhayoloji esinokulinganiswa esisetyenziselwa ukufumanisa okanye ukubeka esweni imeko. I-Tryptase yeyona nto isekiweyo kwi-biomarker kwisifo seeseli zemast.
Carboxypeptidase A3
Ukucutheka kweendlela zomoya ngenxa yokuqiniswa kwezihlunu ezijikelezayo ezigudileyo, ukuvelisa ukuphefumla nokuphefumla. I-Leukotrienes ngabaqhubi abanamandla.
C
Iprotheyini yomqondiso eqokelela ezinye iiseli zomzimba kwisiza, ukwandisa kunye nokwandisa impendulo yokuvuvukala.
I-protease egcinwe kwi-mast cell granules, ebandakanyekayo ekuhlaziyweni kwezicubu kunye nokuqhekeka kwee-peptides ezithile kunye netyhefu.
I-Chemokine
Iprotheyini yomqondiso eqokelela ezinye iiseli zomzimba kwisiza, ukwandisa kunye nokwandisa impendulo yokuvuvukala.
Chymase
I-protease efunyenwe kwi-mast cell granules ethatha inxaxheba ekuhlaziyeni izicubu kunye nakwiindlela zokulawula uxinzelelo lwegazi.
I-Cascade yeeproteni zegazi ezenza inxalenye yokhuselo lwendalo. Izixhobo zayo i-C3a kunye ne-C5a zingenza iiseli zemast zisebenze ngokuthe ngqo.
Ihomoni ekhupha iCorticotropin (CRH)
Inkqubo yokufezekisa
Cromolyn sodium
I-mast cell stabilizer enciphisa ukukhululwa komlamli kunokuthintela iziphumo zomlamli. Amachiza omlomo asebenza ikakhulu kumzila wesisu.
Ihomoni yoxinzelelo apho iiseli zemast zithwala i-receptors, ibonelela ngendlela ebhaliweyo apho i-physiology yoxinzelelo ichaphazela ukusebenza kweseli yemast.
Cromolyn sodium
I-mast cell stabilizer enciphisa ukukhululwa komlamli kunokuthintela iziphumo zomlamli. Amachiza omlomo asebenza ikakhulu kumzila wesisu.
Isikhumba
Ezinxulumene nolusu.
Ukuqaliswa
Cytokine
Ingenzeka kunye okanye ngaphandle kwerhashalala ebonakalayo
Iprotheyini ebonisa izibonakaliso eziqhuba ukuvuvukala kunye nokulungelelaniswa komzimba. IiCytokines zisebenza ngokucothayo kwaye ziqhubeka ixesha elide kunabalamli abenziwe ngaphambili.
D
Inkqubo apho iigranules zokugcina iiseli zemast zidibana nenwebu yeseli kwaye zikhuphe imixholo yazo kwithishu ezingqongileyo, ngokwesiqhelo kwimizuzwana.
I-welt ephakanyisiweyo eveliswa ngokuphulula ngokuqinileyo ulusu. Uphawu olubonakalayo olubonakalayo olunxulunyaniswa nokusebenza kwakhona kweseli yemast.
I-Diamine oxidase (DAO)
I-enzyme ephula i-histamine efunyenweyo. Umsebenzi ocuthiweyo yindlela eqhelekileyo ecetywayo yokunganyamezeli kwe-histamine.
Ukukhutshwa okwahlukileyo
Ukukhutshwa okukhethiweyo kwabanye abalamli ngaphandle kokuthotywa ngokupheleleyo. Ikwabizwa ngokuba yi-piecemeal degranulation; inceda ukuchaza iipatheni zeempawu ezahlukeneyo.
Dysautonomia
Ukungasebenzi kwenkqubo ye-nervous autonomic, ukuvelisa iimpawu ezifana nesantya senhliziyo kunye nokungazinzi kwengcinezelo yegazi. IIPOTS luhlobo olunye.
E
Indlela ecwangcisiweyo yokususa ukutya okurhanelwayo kunye nokuphinda kubuyiselwe ngokucwangcisiweyo. Inomngcipheko wesondlo ukuba ixesha elide ngaphandle kweliso.
Iqela lokuphazamiseka kwezicubu ezixhamlayo. Uhlobo lwe-hypermobile luxelwa rhoqo ecaleni kwe-MCAS kunye ne-POTS.
Ukuphelisa ukutya
Abaxhasi abacetywayo bokusingqongileyo babandakanya ukuvezwa kweekhemikhali, ukungunda, ukungcoliseka komoya, kunye nokutshintsha kwezidlo kwinqanaba labantu. Ubungqina apha buthathaka kakhulu kunezi ndawo zingasentla, kwaye uninzi lwayo aluthanga ngqo. Lo ngummandla obambekayo wophando endaweni yokuba ube negalelo elimiselweyo, kwaye ikwayindawo apho amabango orhwebo athembekileyo exhaphakile kwaye axhaswa kakubi.
Indlela ecwangcisiweyo yokususa ukutya okurhanelwayo kunye nokuphinda kubuyiselwe ngokucwangcisiweyo. Inomngcipheko wesondlo ukuba ixesha elide ngaphandle kweliso.
Eosinophil
Iseli emhlophe yegazi ebandakanyekayo kwiimpendulo ze-alergic kunye ne-parasitic. Yohlukile kwiiseli zemast kodwa iyasebenza kwiimeko ezithelelanayo.
Utshintsho kwintetho yemfuza engaluguquliyo ulandelelwano olusisiseko lweDNA. Icetywa njengendlela enokwenzeka kwi-MCAS, enobungqina obuthe ngqo obunqongopheleyo.
Epinephrine
Unyango lodidi lokuqala lwe-anaphylaxis, ludla ngokunikezelwa nge-auto-injector. I-Antihistamines ayithathi indawo ye-acute reaction.
Erythema
Ubomvu bolusu olubangelwa ukunyuka kwegazi, njengoko kwenzeka ngexesha lokugungxula.
F
FcERI
Ulusu kunye ne-mucosal
I-receptor ye-IgE ephezulu kwi-mast cell surface. Ukudityaniswa kwe-IgE ebotshelelweyo yi-allergen kubangela i-classical activation activation.
Ukuvutha
Ixesha leempawu ezimandundu, ezinokulandela isiqalo esibonakalayo okanye zivele ngaphandle kwesinye. Ubude bexesha buhluka kwiiyure ukuya kwiiveki.
Ubomvu ngesiquphe kunye nobushushu, ngokuqhelekileyo ubuso, intamo, kunye nesifuba. Enye yeempawu zemast cell.
G
H1 i-receptor
I-histamine receptor subtype igxile eluswini, kwiindlela zomoya, kunye nemithambo yegazi. Ujoliso lwe-H1 antihistamines.
H2 i-receptor
I-histamine receptor subtype igxile kwisisu nakwizicubu zentliziyo. Ujoliso lwe-H2 blockers, ezinceda iimpawu zesisu.
Hematopoietic progenitor
Iseli yomongo wethambo engekavuthwa ekwaziyo ukuphuhla ibe ziintlobo zeeseli zegazi. Iiseli zemast ziphuma kwi-CD34-positive progenitors.
IHeparin
Hematopoietic progenitor
I-Hereditary alpha tryptasemia (HaT)
Uphawu lofuzo olubandakanya iikopi ezongezelelweyo zejini ye-TPSAB1, evelisa i-tryptase yesiseko ethe gqolo. Izuzwe kwipateni eyongameleyo ye-autosomal.
IHeparin
I-anticoagulant eyenzeka ngokwemvelo egcinwe kwi-mast cell granules. Inokuba negalelo kwimikrwelo engaqhelekanga okanye ukopha kwezinye izigulana.
Ukunganyamezeli kwe-histamine
Histamine
Eyona mast cell mediator eyaziwayo. I-Dilates imithambo yegazi, yandisa ukungeneka kwayo, ivuselela imithambo-luvo, kwaye inyusa i-asidi yesisu, ivelise ukugungxula, ukurhawuzelelwa, ukurhawuzelela kunye nokukrala.
Ukunganyamezeli kwe-histamine
Ubunzima bokuphula i-histamine engenisiweyo, edla ngokubalelwa kukunciphisa umsebenzi we-diamine oxidase. Okwahlukileyo kwi-MCAS, nangona iimpawu ziyadibana.
IiHives
Jonga i-Urticaria. Ukunyuka, ukurhawuzelelwa kwesikhumba esikhumbeni, kuhlala kudlula kwaye kufuduka.
Uxinzelelo lwegazi
Uxinzelelo lwegazi oluphantsi ngokungaqhelekanga. Inokwenzeka ngexesha leeseli zemast ezibukhali kwaye luphawu lwe-anaphylaxis.
I
IgE
I-Immunoglobulin E, udidi lwe-antibody olusembindini we-classical allergy. Ibophelela i-FcERI kwiiseli zemast; iimpendulo ezininzi ze-MCAS zixhomekeke kwi-IgE.
Immunoglobulin
I-antibody - iprotheni eveliswa yi-immune system ebophelela iithagethi ezithile. I-IgE lolona didi lufanelekileyo kwimast cell activation.
Ukudumba
Ukusabela komzimba kukwenzakala okanye ukoyikiswa, okubandakanya ukuhamba kwegazi okwandisiweyo, ukufunwa kweeseli zomzimba, kunye nokutshintsha kwezicubu. Iiseli zeMast ziyayiqalisa kwaye ziyiphendule.
Udidi lwe-cytokine olusetyenziselwa ukunxibelelana phakathi kweeseli zomzimba. I-IL-6 kunye ne-IL-4 ziphakathi kwezo zikhutshwe ziiseli zemast.
Ingalo ekhawulezayo, engangqalanga yamajoni omzimba ikhona ukususela ekuzalweni. Iiseli zemast yinxalenye.
I-Interleukin
Iyeza eline-antihistamine kunye ne-mast cell stabilizing properties. Ngokuqhelekileyo kufuna iiveki zokusetyenziswa rhoqo ngaphambi kokuba kusebenze ngokupheleleyo.
K
Ketotifen
IKHITHI D816V
IKIT
I-receptor kwi-mast cell surface edibanisa i-stem cell factor. Okusembindini wophuhliso lweeseli zemast, ukusinda, kunye nokuhlala kwezicubu.
Leukotriene
Utshintsho oluthile kwijini ye-KIT efunyenwe kuninzi lwabantu abadala be-systemic mastocytosis. Ayingonobangela omiselweyo we-MCAS.
I-Leukotriene E4 (LTE4)
Leukotriene
Umlamli we-lipid owenziwe emva kokusebenza. I-bronchoconstrictors enamandla ekwandise ukunyanzeliswa kwe-vascular kunye nokuveliswa kwe-mucus.
Umlamli owakhiwe kwi-cell membrane lipids emva kokusebenza kunokuba agcinwe kwangaphambili. I-Prostaglandins kunye ne-leukotrienes yimizekelo ephambili.
I-metabolite ye-leukotriene enokulinganiswa kumchamo, isetyenziswe njengenxalenye yovavanyo lomlamli.
Umlamli weLipid
Umlamli owakhiwe kwi-cell membrane lipids emva kokusebenza kunokuba agcinwe kwangaphambili. I-Prostaglandins kunye ne-leukotrienes yimizekelo ephambili.
M
Iseli yemast
I-MCAS ayikho i-mastocytosis
Imeko ebandakanya ukuqokelelana kweeseli zemast ezigqithisileyo kwithishu, edla ngokuqhutywa yi-KIT D816V uguquko kwisifo senkqubo yabantu abadala. Eyahlukileyo kwi-MCAS.
Abalamli abaziintloko nento eyenziwa ngumntu ngamnye
Isizinzi seseli yeMast
Iyeza elinciphisa ukukhululwa kwe-mast cell mediator kunokuthintela iziphumo zomlamli. I-Cromolyn kunye ne-ketotifen yimizekelo eqhelekileyo.
Iimbumba ezijikeleze ingqondo kunye nentambo yomgogodla. Iiseli zeMast zikhona apha, ezifanelekileyo kuphando ngeempawu ze-neurological.
Nayiphi na imichiza ekhutshwa yimast cell evelisa iziphumo kwithishu ezingqongileyo. Kubandakanya i-histamine, i-tryptase, i-prostaglandins, kunye nabanye abaninzi.
Iimeninges
MRGPRX2
Montelukast
Umchasi we-leukotriene receptor antagonist esetyenziselwa ukujongana neempawu zokuphefumula kunye nesisu esingenakufikelelwa yi-antihistamines.
Izihlunu ezifumileyo ezidibanisa iipaseji zangaphakathi ezifana namathumbu, umoya, kunye neempumlo. Ixinene ngeeseli zemast.
Imast cell receptor mediating ye-IgE-independent activation ngamachiza athile kunye neepeptides. Inceda ekuchazeni ukusabela ngaphandle kokubandakanyeka kwe-IgE okubonakalayo.
Mucosa
Imetabolite ye-histamine elinganiswe kwiiyure ezingama-24 zokuqokelelwa komchamo njengobungqina bokukhululwa kwe-mast cell mediator.
N
N-methylhistamine
O
I-Neuroimmune
Okunxulumene nentsebenziswano phakathi kwe-nervous kunye ne-immune systems. Ukusebenzisana kwe-mast cell-nerve sisiseko soxinzelelo olusebenza njengesiqalo somzimba.
O
Omalizumab
Unyango lwebhayoloji olujolise kwi-IgE, esetyenziswa kwezinye iimeko eziphikisayo phantsi kweliso lengcali.
P
IiPalpitations
Ukuqonda ukubetha kwentliziyo, okusoloko kuchazwa njengokubaleka, ukubetha, okanye ngokungaqhelekanga.
I-Perivascular
I-Platelet activating factor (PAF)
I-Piecemeal degranulation
I-Hereditary alpha tryptasemia
Jonga ukukhutshwa okwahlukileyo. Ukukhutshwa komlamli okhethiweyo ngaphandle kokukhutshwa kwento yonke yegranule.
I-Platelet activating factor (PAF)
Umlamli onamandla kakhulu we-lipid owandisa i-vascular permeability kwaye unokubangela i-hypotension enzima. Ayivalwanga zii-antihistamines.
Prostaglandin D2
I-Presyncope
Imvakalelo yokuba uza kuquleka, ngaphandle kokulahlekelwa zingqondweni.
Igama lezonyango lokurhawuzelelwa.
Umlamli we-lipid obangela i-vasodilation ebonakalayo, ukugungxulwa, ukuxinwa kwebroncho, kunye norhudo. Idla ngokugqamileyo apho ukugungxulwa kongamele.
I-Pruritus
Ukungaphenduli ngokwaneleyo kunyango oluqhelekileyo. Iimeko ezichasayo zinokubangela ukuqwalaselwa kwee-arhente zeengcali.
R
I-Refractory
Ukungaphenduli ngokwaneleyo kunyango oluqhelekileyo. Iimeko ezichasayo zinokubangela ukuqwalaselwa kwee-arhente zeengcali.
Regranulation
Inkqubo apho iseli ye-mast iphinda yakha iivenkile zayo zegranule emva kokutshatyalaliswa, ukuyivumela ukuba iphendule kwakhona kunokuba ife.
I-Rhinitis
Ukudumba kwenwebu yempumlo kuvelisa ukuxinana, impumlo evuzayo, nokuthimla. Amaxesha amaninzi ezingapheliyo kwaye non-onyaka kwi-MCAS.
S
ISerum tryptase
I-Tryptase ilinganiswa egazini. Esona siphawuli selabhoratri sisekiweyo sokuvula iseli yemasti, efuna iisampuli ezibukhali kunye nesiseko.
I-Stem cell factor (SCF)
Imolekyuli yomqondiso ebopha i-KIT, iqhuba uphuhliso lweeseli zemast, ukusinda, kunye nokuhlala kwezicubu.
I-syncope
Ukufa isiqaqa - ukulahlekelwa yingqondo okwethutyana, ngokuqhelekileyo ukusuka ekunciphiseni ukuhamba kwegazi ukuya engqondweni.
Uhlobo lwe-mastocytosis apho iiseli zemast ezingaphezulu ziqokelelana kumalungu angaphakathi, rhoqo ngotshintsho lwe-KIT D816V.
Ukuchaphazela umzimba uphela endaweni yendawo enye yendawo.
Uvakalelo kumavumba kunye neekhemikhali ezisemoyeni
mastocytosis Systemic
Umqobo
T
Isantya sentliziyo esikhawuleza ngokungaqhelekanga, esixelwa rhoqo ngexesha lokusabela kweeseli zemast.
Umqobo
TPSAB1
Isamkeli esifana nomnxeba
Qalisa
I-receptor eqaphela iipateni ezinxulumene ne-pathogens, edibanisa ukusebenza kweseli ye-mast kusulelo.
I-Tryptase
Ijene lekhowudi yealpha-tryptase. Iikopi ezongezelelweyo zivelisa ilifa le-alpha tryptasemia kunye nesiseko esiphakamileyo se-tryptase.
Naluphi na uvuselelo oluxhokonxa iseli yemast. Isenokuba yimichiza, ngokomzimba, ihomoni, ezosulelayo, okanye ngokweemvakalelo; Ihlala iqokelelana endaweni yesinye.
I-Tryptase
Eyona protein ininzi kwimast cell granules kunye nesona siphawuli sisekiweyo sokwenza kusebenze, esixatyiswe ngokuthelekiswa nemast-cell-specific.
U
Urticaria
I-Hives - iphakanyisiwe, i-welts erhawuzelayo ebangelwa ukukhululwa komlamli esikhumbeni. I-urticaria engapheliyo ibandakanya ukusebenza kweeseli zemast ngokuthe ngqo.
Urticaria pigmentosa
Ukuba nefuthe kwi-diameter ye-blood vessel okanye i-permeability. I-Histamine kunye ne-prostaglandin D2 zinamandla e-vasoactive.
V
Vasoactive
W
I-Vasodilation
Ukwandiswa kwemithambo yegazi, ukwandisa ukuhamba kwegazi kunye nokunciphisa uxinzelelo lwegazi. Ivelisa ukugungxulwa kwaye ingabangela ukukhanya-intloko.

Awungo Wedwa

Iinkcukacha ze-MCAS zikhona ukuze kuqinisekiswe ukuba isigulana ngasinye siyafikelela kwinkxaso, kuluntu, kunye nolwazi lokuzithethelela. Joyina inethiwekhi yethu yehlabathi.

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