Isiqulatho
01Yintoni i-MCAS?02Ukuqonda iiSeli zeMast03Kwenzeka ntoni ngeMast Cell Activation04Iimpawu ze-MCAS05Izichukumisi eziqhelekileyo06Kutheni i-MCAS kunzima ukuyifumanisa07Iimeko ezinxulumeneyo08Ukuqondwa Ngoku Kwezizathu09Ukuhlala ne-MCAS10Imibuzo ebuzwa qho11IziBonelelo zokuFunda ezongezelelweyo12Izinto eziphambili zokuThatha13Uluhlu lwamagama
Icandelo le-01
Yintoni i MCAS?
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.
Inkcazo ibalulekile kuba ibeka i-MCAS ngaphandle kweemeko ifana kakhulu. 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
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.
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-MCAS ayikho i-mastocytosis | Lo ngowona mahluko ubalulekileyo kwintsimi, kwaye ukubhidanisa oku kukhokelela ekubhidekeni malunga nayo yonke into esezantsi. | |
|---|---|---|
| I-Mastocytosis xa ithelekiswa ne-MCAS | Mastocytosis | I-MCAS |
| Ukungaqhelekanga okungundoqo | Iiseli zemast ezininzi ziqokelelana kwithishu | Inani eliqhelekileyo leeseli zemast, zisebenza ngokungafanelekanga |
| Ubalo lweeseli | Ukuphakama - ukwanda kwe-clonal | Okuqhelekileyo |
| Isiseko se-tryptase | Ihlala iphakanyisiwe ngokuqhubekayo | Ngokwesiqhelo kwisiseko |
| Ufuzo oluqhelekileyo lokufunyaniswa | IKIT Ukuguqulwa kwe-D816V kwiimeko ezininzi zenkqubo yabantu abadala | Akukho tshintsho lwecausative olusekiweyo olunye |
| Iziphumo zeBiopsy | Iiseli zemast ezingaqhelekanga ziyaboniswa | Akukho ngqokelela yeempawu |
| Uphawu lwesikhumba | Izilonda ze-Maculopapular (urticaria pigmentosa) eziqhelekileyo kwezinye iifom | Akukho silonda esithile sokuxilongwa kwesikhumba |
Indlela eqinisekiswa ngayo
Ubungqina benjongo yobuninzi beeseli
Ipatheni yeempawu + ubungqina bomlamli + impendulo yonyango
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.
I-MCAS ayingokwaliwa kuqhelekileyo
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.
Unyango lwePearl
- Umfanekiso ocetywayo
- 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-MCAS luphazamiseko lwemast cell engafanelekanga ukusebenza, hayi yeemast cell numbers.
- Iiseli zemast ziluncedo ngokusemthethweni kwiiseli zokhuselo lomzimba ezibekwe kwimiqobo yomzimba kwaye zakhelwe ukuphendula kwimizuzwana.
I-Mastocytosis ibandakanya ukugqithisa kweeseli kwaye ingabonakaliswa ngokuthe ngqo; I-MCAS ayinako, yiyo loo nto ukuxilongwa kuyintlanganisela.
Uvavanyo olungalunganga lokwaliwa luxhaphakile kwi-MCAS kwaye ayikhupheli ngaphandle.
Icandelo 02
Ukuqonda Iiseli zeMast
Ukulandela ukuba kutheni i-MCAS iziphatha ngendlela eyenza ngayo, kuyanceda ukwazi ukuba zivela phi ezi seli, zihlala phi, ziphila ixesha elingakanani, kwaye zimamela ntoni.
Imvelaphi kunye nophuhliso
Iiseli zeMast ziqala kumongo wethambo, zivela kwiiseli ze-CD34-positive haematopoietic progenitor cells - kwabemi abafanayo abavelisa 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.
| 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 |
|---|---|---|
| Indawo | Indima kwimpilo | Iimpawu xa ukusetyenziswa kungafanelekanga |
| Ulusu kunye nedermis | ukhuselo lomqobo; ukuphendula ekulunyweni, ukuhlatywa, kunye nokwenzakala | Ukugungxula, ukurhawuzelelwa, ukurhawuzelela, idermographism, ukudumba |
| Umgudu wesisu | Ukugcinwa komqobo, ukulawulwa kwe-motility, ukuphendula kwizoyikiso ezityiweyo | Ukuqaqanjelwa, isicaphucaphu, ukurhawuzelela, urhudo, ukuqhina, ukuqunjelwa |
| Iindlela zomoya kunye nezono | Ukukhusela umgca wokuphefumla | Ukuxinana, i-rhinitis, ukuqina komqala, ukuphefumla, ukukhohlela |
| Ejikeleze imithambo yegazi | Ukulawulwa kwethoni yenqanawa kunye nokungena | Ukutshintsha koxinzelelo lwegazi, tachycardia, ukugungxula, presyncope |
| Ubuchopho kunye neemeninges | Zibonelele nge-perivascularly kunye nakwi-meninges; umqondiso we-neuroimmune | Inkungu yengqondo, intloko ebuhlungu, isiyezi (iinkqubo zisaphantsi kophononongo) |
| Ithishu edibanisayo | Ukulungiswa kunye nokulungiswa kwezicubu | Intlungu edibeneyo kunye nemisipha; Ukufaneleka okunokwenzeka kokudibana kwezicubu ezidibeneyo |
Ummongo wethambo
Indawo yemvelaphi yokhokho
Apho isifo se-clonal sivavanywa xa kurhanelwa i-mastocytosis
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.
- Oku kubalulekile kwiikliniki ngeendlela ezimbini. Kuthetha ukuba iimpawu zokuqhuba iiseli zizinzile kunokuba zijike ngokukhawuleza, ke akukho sisombululo sikhawulezileyo sendalo sokulinda. Kwaye kuthetha ukuba unyango oluzinzisa iiseli zemast kufuneka luthathwe ngokuthe rhoqo ngokuhamba kwexesha - utshintsha isimilo sabemi abahlala ixesha elide, ungacimi okwethutyana.
- Izamkeli zomphezulu: yintoni emamela iseli yemast
- Iiseli zeMast zithwala uluhlu olubanzi lwee-receptors ezingaphezulu, kwaye ububanzi bolu luhlu sisizathu sokuba izinto ezininzi ezahlukeneyo zisebenze njengezinto ezibangelayo. Ezinye zezona mpawu zibalaseleyo ziquka:
- 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.
Abamkeli be-CRH - phendula i-corticotropin-releasing hormone, ukubonelela ngendlela ebhaliweyo apho i-physiology yoxinzelelo ifikelela kwiiseli ze-mast.
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.
- 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.
- Iiseli zeMast zisuka kwiCD34+ progenitors kumongo wethambo kodwa ziqole kwithishu, ngoko ke ubalo lwegazi alubonakalisi.
- I-KIT/SCF yokubonisa ingundoqo ekuphuhliseni kwabo - indlela ephazamisekile kwi-mastocytosis yabantu abadala.
Zihlala ixesha elide kwaye ziyakwazi ukubuyisela kwakhona, ngoko ke ulawulo luyazinza kunokuba lufutshane.
Iindidi ezininzi ze-receptor ezingaphaya kwe-IgE zinokuzenza zisebenze, yiyo loo nto iiphaneli ze-allergies zihlala ziphosakele izitshizi ze-MCAS.
Mast Cell Activation
Ukuqalisa lulandelelwano, kwaye inyathelo ngalinye linegama. Ukuyilandela kwenza ukuba iintlobo ezahlukeneyo ezididayo zeempawu ze-MCAS zibe lula kakhulu ukutolika.
I-stimulus ifikelela kwiseli-i-allergen, iziyobisi, ubushushu, uxinzelelo, umgudu, ihomoni yoxinzelelo, usulelo, okanye akukho nto ibonakalayo.
Ii-receptors zomphezulu zibandakanya kunye ne-intracellular signaling cascades iqala. Ukungena ngekhalsiyam linyathelo lokuqala lokuqala.
Iigranule zokugcinwa zixubana nenwebu yeseli kwaye zikhuphe imixholo yazo eyenziwe kwangaphambili kwizicubu ezijikelezileyo - ngaphakathi kwemizuzwana.
Abalamli abenziwe kwangaphambili bakhululwa ngokukhawuleza; abalamli abasandula ukwenziwa njengeprostaglandins kunye neleukotrienes balandela kwimizuzu, kunye neecytokines kwiiyure.
5
Iimpawu
Abalamli basebenza kwimithambo yegazi, izihlunu ezigudileyo, imithambo-luvo, kunye nezinye iiseli zokhuselo lomzimba, zivelisa iziphumo kuzo zonke iinkqubo zamalungu amaninzi.
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 kuqale ukusebenza, yiyo loo nto ezinye iimpawu zifika kakuhle emva kokuqalisa kwaye kutheni ukusabela kunokulibaziseka kweeyure zesibini kamva.
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 abaziintloko nento eyenziwa ngumntu ngamnye | Iiseli zeMast ziyakwazi ukukhulula inani elikhulu kakhulu lezinto. Ezi zilandelayo zezona zixoxiwe ngokonyango, kwaye kunye zichaza uninzi lweempawu zeempawu eziqatshelweyo. | Abalamli bemast cell |
|---|---|---|
| Umlamli | Uhlobo | Iziphumo eziphambili |
| Histamine | Uhlobo | Yakhelwe kwangaphambili |
| Oyena 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. | I-Tryptase | Eyona 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. |
| Prostaglandin D2 | I-Tryptase | Isandula ukwenziwa |
| Umlamli we-lipid oveliswe emva kokusebenza. Ibangela i-vasodilation ephawulweyo kunye nokugungxulwa, i-bronchoconstriction, kunye norhudo, kwaye inokuba negalelo ekwehleni okukhulu koxinzelelo lwegazi. Idla ngokubandakanyeka apho ukugungxulwa kuluphawu olubalaseleyo. | I-Tryptase | I-Leukotrienes (LTC4, LTD4, LTE4) |
| I-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. | I-Tryptase | IiCytokines (IL-6, TNF-α, IL-1, IL-4 kunye nabanye) |
| Iimpawu zeeprotheyini eziqhuba ukuvuvukala okubanzi. Iqala kancinci kwaye ithatha ixesha elide kunabalamli abangentla, kwaye kucingelwa ukuba banegalelo ekudinweni, ukugula, kunye nezigulana ezisemgangathweni 'ezifana nomkhuhlane' zichaza ngexesha nasemva kokuvutha. | I-Tryptase | IiChemokines |
| Fumana iiseli ezongezelelweyo zokuzikhusela kwisiza, ukwandisa kunye nokwandisa impendulo yokuvuvukala ngaphaya kwempendulo yokuqala yeseli ye-mast. | Uhlobo | I-Platelet activating factor (PAF) |
| I-lipid mediator enamandla kakhulu eyandisa ukunyanzeliswa kwe-vascular permeability kwaye inokubangela i-hypotension enzima. Ifakwe kubunzima be-anaphylactic reactions kwaye ayivalwanga ngama-antihistamines. | Uhlobo | IHeparin |
I-anticoagulant eyenzeka ngokwemvelo. Inokuba negalelo kwimikrwelo engaqhelekanga okanye ukuthambekela ukopha okuxelwe zezinye izigulana, kwaye ikwathatha inxaxheba kummiselo wendawo yokudumba.
Chymase & carboxypeptidase A3
- I-Granule proteases ebandakanyekayo ekwenziweni ngokutsha kwezicubu, iindlela zokulawula uxinzelelo lwegazi, kunye nokuthotywa kwetyhefu ethile kunye neepeptide.
- Ngenxa yokuba ii-antihistamines zivimba kuphela i-histamine, impendulo engaphelelanga kwii-antihistamines ihambelana ngokupheleleyo ne-MCAS kunobungqina obuchasene nayo. I-Prostaglandins, i-leukotrienes, i-PAF, kunye ne-cytokines azizange zichukunyiswe zezo arhente-nto leyo ingqiqo emva kokongezwa ngenyathelo kwezinye iiklasi zeziyobisi.
- 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.
- Ukusebenza kulandela ukulandelelana: i-trigger, i-activation, i-degranulation, ukukhululwa komlamli, iimpawu.
- Abalamli abenziwe kwangaphambili bakhulula ngemizuzwana; abalamli be-lipid kwimizuzu; iicytokines ngeeyure.
I-Histamine yenye kuphela yabalamli abaninzi, yiyo loo nto i-antihistamines iyodwa ihlala inika isiqabu.
I-PAF kunye ne-prostaglandin inokuqhuba iziphumo ezinzima ze-cardiovascular that antihistamines don't address.
Icandelo 04
| 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. | 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 | Uphawu |
| Amanqaku | Ukugungxula |
| Idla ngokuchaphazela ubuso, intamo nesifuba ngesiquphe; uphawu oluxelwa rhoqo | Ukurhawuzelelwa (pruritus) |
| Ingenzeka kunye okanye ngaphandle kwerhashalala ebonakalayo | Izilonda (urticaria) |
| Unyuswe, ukurhawuzelela ukumanzisa; inokuba yedlula kwaye ifuduke | Idermographism |
| Iiwelts eziphakanyiswe kukuphulula ngokuqinileyo ulusu-uphawu olubonakalayo olubonakalayo | I-Angioedema |
| 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. | 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. |
|---|---|
| Ukulibaziseka ukuphiliswa kwenxeba | Ingxelo yezinye izigulane; iindlela ezingamiselwanga ngokupheleleyo |
| Ukuphefumla | Ukuxinana ngeempumlo kunye ne-rhinitis |
| Amaxesha amaninzi ezingapheliyo kunye non-onyaka | Ukuqina komqala |
| Ifuna uvavanyo olungxamisekileyo ukuba luqhubela phambili - lunokuthi lubonise i-anaphylaxis | Ukuphefumla kunye nokuphefumla kancinci |
| I-bronchoconstriction eqhutywa yi-leukotriene yindlela eyaziwayo | Ukukhohlela okungapheliyo |
| 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. | 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. |
|---|---|
| Uvakalelo kumavumba kunye neekhemikhali ezisemoyeni | Ixhaphake kakhulu ingxelo yokubhanqa uphawu |
| Intliziyo | Tachycardia |
| Intliziyo ebalekayo, rhoqo ngaphandle kokuzibhokoxa | Ukungazinzi koxinzelelo lwegazi |
| Ingajinga kulo naliphi na icala ngexesha leeqephu | Presyncope kunye nesyncope |
| Intloko ekhanyayo okanye isiqaqa, rhoqo xa umile | Ukungakhululeki kwesifuba |
| 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. | 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. |
|---|---|
| I-Anaphylaxis | Okubukhali, ngokukhawuleza, okunokubeka ubomi esichengeni - jonga icandelo likaxakeka |
| Amathumbu | Ukukrala kwesisu kunye neentlungu |
| Phakathi kweempawu eziqhelekileyo ezixelwe ngokubanzi | Isicaphucaphu kunye nokugabha |
| Inokulandela ukutya okanye kwenzeke ngokuzimeleyo | Urhudo kunye/okanye ukuqhina |
| I-Motility inokwahluka phakathi kweziqephu kunye nexesha | I-Reflux |
| 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. | 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. |
|---|---|
| Ukuqunjelwa kunye nokusebenza kwakhona kokutya | Uqhuba rhoqo uthintelo lokutya- bona izilumkiso kwicandelo 9 |
| I-neurological and cognitive | Inkungu yengqondo |
| Ubunzima bokugxila, ukukhumbula, kunye nokufumana amagama | Intloko kunye ne-migraine |
| I-Migraine ixelwe kumazinga aphezulu kule ndawo | Isiyezi |
| Ihlala igqithana ngeempawu zentliziyo kunye ne-autonomic | Ukudinwa |
| Zihlala zibukhali kwaye ziphakathi kwezona mpawu zixeliweyo zikhubaza | Uvakalelo lweemvakalelo |
| 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. | 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. |
|---|---|
| Iimvakalelo ze-neuropathic | Ukurhawuzelelwa, ukutshisa, okanye ukuba ndindisholo okuxelwe zezinye izigulana |
| I-Musculoskeletal, i-genitourinary, kunye nengqondo | Intlungu edibeneyo kunye nemisipha |
| Ngamana ukudibana neemeko zethishu edibeneyo ekuxoxwe ngayo kwicandelo lesi-7 | Iintlungu zamathambo |
| Uvavanyo lweziqinisekiso, ngakumbi apho i-mastocytosis iqwalaselwa | Ukungxamiseka kwesinyi kunye nokuphindaphinda |
| Iimpawu ezinjenge-Interstitial-cystitis zixelwa | Iimpawu ziyahluka kunye nomjikelo wokuya exesheni |
| Impembelelo yeHormonal kwiiseli zemast ibhaliwe | Iziqendu ezifana nexhala |
Ukukhutshwa komlamli kunokuvelisa ngokunyanisekileyo ukubetha kwentliziyo, ukuphefumla, kunye nemvakalelo yokugwetywa
Ukutshintsha kweemvakalelo kunye nokucaphuka
Zombini kwiziphumo zomlamli nasekuphileni nesifo esinganyangekiyo, esingaqondwa kakuhle
- 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.
- Kutheni iimpawu zihluke kakhulu phakathi kwezigulane
- Abantu ababini abanoxilongo olufanayo banokubonisa phantse ngokungafaniyo ngendlela eyahlukileyo. Zininzi izinto ezinegalelo, kwaye uninzi lusaqondwa ngokungaphelelanga:
- 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.
Iimeko ezikhoyo. Uxilongo oludlulayo olufana ne-POTS okanye ukunyanzeliswa kwe-hypermobility kuguqula umfanekiso wekliniki ngokubanzi.
- Umda kunye nomthwalo owongezelekayo. Ukulala, uxinzelelo, imeko yehomoni, kunye nosulelo lwamva nje lonke utshintsho olusisiseko sokusebenza kwakhona, ke umntu omnye uyahluka ngokuhamba kwexesha.
- Imiba yemfuza. I-Hereditary alpha tryptasemia kunye nolunye uhlobo lofuzo lunokuguqula umboniso-indawo yophando olusebenzayo kunokuba ulwazi oluzinzile.
- 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.
- 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.
Ukunikezelwa kuyahluka ngokubanzi phakathi kwezigulane kunye nexesha phakathi kwesigulane esifanayo.
Iziqendu ezinjengexhala zinokuqhutywa ngumlamli; oku akufuneki kusetyenziswe ukugxotha imeko, okanye ukugxotha iimfuno zokwenyani zempilo yengqondo.
Icandelo 05
Eqhelekileyo Izichukumisi
Izichukumisi azifane zisebenze zodwa. Iiseli zeMast ziphendula kumthwalo oqokelelweyo, yiyo loo nto isidlo esinye, igumbi, okanye umsebenzi omnye ungabi nabungozi ngenye imini kwaye unganyamezeleki kolandelayo.
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
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
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.
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.
Ukuvezwa kokusingqongileyo
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.
Ukungalali
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.
- Kutheni izinto ezibangela ukuba zihluke kakhulu
- 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.
- I-infographic ye-Threshold: ibha ethe tye emele umqobo wokusabela, eneebhloko ezipakishwe ngaphantsi kobushushu, ukulala kakubi, uxinzelelo, ukutya okudala, kunye nokuzibhokoxa. Bonisa imo yokuqala apho iibhloko ezintathu zihlala ngaphantsi komgca, kwaye okwesibini apho ibhloko yesine ityhala isitaki ngaphezulu kwayo - amalungu afanayo, iziphumo ezahlukeneyo.
- 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.
Izinto ezibangela amayeza kufuneka zixoxwe kunye nomyalelo, ungalokothi uzilawule ngokwakho kuluhlu.
Iiseti ze-trigger ziyahluka phakathi kwabantu kunye nokutshintsha ngokuhamba kwexesha, ke ukugcinwa kweerekhodi zobuqu kubalulekile.
Icandelo 06
Kutheni i-MCAS i
Kunzima uku Uxilongo
Ukulibaziseka koxilongo kwi-MCAS ayikokusilela koogqirha ngabanye. Ilandela kwiimpawu ezithile, ezichongiweyo zemeko kunye novavanyo olusetyenziselwa ukuyiphanda.
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.
Uvavanyo lwebhubhoratri oluqhelekileyo
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.
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.
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.
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
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.
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.
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.
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.
- Ulibaziseko kuxilongo
- Indibaniselwano engentla ivelisa ukulibaziseka okude. Izigulana ngokuqhelekileyo zihanjiswa ngeendlela ezininzi - i-gastroenterology, i-dermatology, i-cardiology, i-allergies, i-neurology, i-psychiatry - nganye ihlola ummandla omnye wengxaki yenkqubo.Amanani apapashiweyo kunye novavanyo lwesigulana ngokuqhelekileyo luchaza ukulibaziseka okulinganiswa kwiminyaka, kwaye uqikelelo malunga neshumi leminyaka lucatshulwa rhoqo, nangona amanani anjalo eza kunye nemida yokwenyani yendlela kwaye kufuneka afundwe njengezalathiso kunokuba zichaneke.
- Umzobo wexesha lohambo lokuxilonga olumele: iimpawu zokuqala ngonyaka onguziro; utyelelo lokuqala lukagqirha; ukudluliselwa kwi-gastroenterology nge-endoscopy eqhelekileyo; idermatology enesiphumo sonyango lweempawu; i-cardiology ngokubeka iliso kweHolter eqhelekileyo; ukuthunyelwa kwengqondo; emva koko i-allergies/immunology kunye novandlakanyo lwemast cell ekugqibeleni.Indawo nganye echazwe ngexesha elidlulileyo kunye namanqaku 'amanqaku esiphumo esiqhelekileyo', okwenza ukuba ukuqokelelwa kweemvavanyo ezingenayo ibonakale njengepatheni kunoluhlu lweziganeko ezizimeleyo.
- 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.
Ukuqhelana nogqirha kuyahluka; imeko ingene kwikharityhulam kutsha nje.
Iikhrayitheriya zokuxilonga zihlala ziphikiswa ngokunyanisekileyo, ngoko ke izimvo zeengcali zinokwahluka ngokusemthethweni.
Nxulunyaniswe Iimeko
I-MCAS ixelwa rhoqo kunye nezinye iimeko ezininzi. Amandla obungqina ahluke kakhulu phakathi kolu nxulumano, kunye nemiba yomahluko.
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-Postural Orthostatic Tachycardia Syndrome (POTS)
I-POTS luhlobo lwe-dysautonomia oluphawulwa ngokunyuka kwesantya sentliziyo ngokugqithisileyo ekumeni, ngokufuthi ngentloko ekhanyayo kunye nokudinwa. Ukwenzeka ngokubambisana ne-MCAS kuchazwe kakuhle kuncwadi. Iinkcazo ezicetywayo ziquka abalamli beeseli ze-mast ezichaphazela ithoni ye-vascular kunye nokulawulwa kwe-autonomic, kodwa ulwalathiso lwalo naluphi na ubudlelwane be-causal alusungulwa.Zombini ezi meko zinokuvelisa i-tachycardia, isiyezi, kunye nokudinwa, nto leyo eyenza kube nzima ukunika iimpawu kwenye okanye kwenye.
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.
Irritable bowel syndrome (IBS)
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
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
| Iimeko ze-Autoimmune | Iimeko ezahlukeneyo ze-autoimmune zixelwa ecaleni kwe-MCAS, kubandakanya i-thyroid autoimmunity. Iiseli zeMast zithatha inxaxheba kulawulo lokhuselo lomzimba kwaye zinxibelelane neempendulo eziguqukayo zokhuselo lomzimba, zibonelela ngesiseko esivakalayo sokwabelana ngedysregulation, kodwa iindlela ezithile eziqhagamshela i-MCAS kwizifo ezithile zokuzikhusela komzimba azikasekwa. | Amandla obungqina ngokujonga nje krwaqu |
|---|---|---|
| Imeko | Ubume bobudlelwane | Ubume bobungqina |
| Urticaria engapheliyo | Ukusebenza kweeseli zemast kungundoqo kwisifo ngokwaso | Ubume bobungqina |
| Indlela esekiweyo | Umbefu | Abalamli beeseli zeMast baqhuba i-bronchoconstriction |
| I-Hereditary alpha tryptasemia | Iimpawu zemfuzo eziphakamisa isiseko se-tryptase; kuchaphazela uvavanyo lokutolika | Uphawu olumiselweyo; Ulwalamano lweempawu lusacaciswa |
| IIPOTS | Ukwenzeka rhoqo; iziphumo zomlamli kwithoni yemithambo ecetywayo | Umbutho unike ingxelo; indlela yokusebenza ayisonjululwanga |
| 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. | hEDS / hypermobility | Ukwenzeka rhoqo; ukubandakanyeka kwezicubu ezidibeneyo zicetywayo |
| Umbutho unike ingxelo; indlela yokucinga | Amazinga aphezulu axeliweyo; abalamli be-vasoactive babonakala | Umbutho unike ingxelo; indlela ephantsi kophando |
| IBS | Iimpawu ziyadibana; ukufunyaniswa kweeseli zemast kuguquguquka | Ukugqithelana 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-Autoimmune | Ingxelo yokwenzeka ngokubambisana; ukwabelwana kwe-immune dysregulation ecetywayo |
- 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.
Uqhagamshelo olude lwe-COVID lolokuqala kwaye akufuneki lunikezelwe njengoko lusekiwe.
Kuyo yonke le ndawo, umbutho uye waphoswa ngokuphindaphindiweyo nge-causation - funda amabango ngononophelo.
Icandelo le-08
Ukuqonda Ngoku
ye Oonobangela
Isishwankathelo esinyanisekileyo kukuba unobangela we-MCAS awaziwa. Okulandelayo kwahlula oko kusekiweyo koko kusasele kuyingqikelelo.
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.
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.
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.
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.
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.
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
Ukuguqulwa kwe-Epigenetic - utshintsho kwi-gene expression ngaphandle kotshintsho kulandelelwano olungaphantsi-lucetywayo njengendlela yokubonakaliswa kwemvelo kunokuvelisa utshintsho oluhlala luhlala kwi-mast cell behaviour. Olu luqikelelo lwenqanaba lokuqala. Kusengqiqweni ngokwebhayoloji kwaye kufanelekile ukuphandwa, kwaye okwangoku kukho ubungqina obuthe ngqo obulinganiselweyo obuthe ngqo kwi-MCAS.
- Apho oku kushiya izinto
- Kusenokwenzeka ukuba i-MCAS njengoko ichazwa ngoku ibandakanya ngaphezulu kwenkqubo enye esisiseko, kunye nokuba amaqela alebhile ekliniki adibanisa izigulane eziya kuthi ekugqibeleni zohlulwe ngendlela. Uphando luyaqhubeka kuzo zonke iinkalo ezingentla.Kwizigulane, intsingiselo esebenzayo kukuba ulawulo lujolise kubalamlamli kunye nezinto ezibangela ukuba kube ngunobangela osisiseko - kwaye ukukrokra kuyaqinisekiswa kuyo nayiphi na imveliso okanye iprotocol ebanga ukujongana nesizathu esibangela ukuba uluntu lophando lungabonakali.
- 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).
- Akukho nobangela we-MCAS usekiwe.
- I-HaT kunye ne-KIT D816V uguquko kwi-mastocytosis zisekiwe kwi-genetics - kwaye ayisosizathu sisekiweyo se-MCAS.
Igalelo lokusingqongileyo kunye ne-epigenetic zingqikelelo ezinobungqina obuthe ngqo obulinganiselweyo.
Waphathe ngokuthandabuza amabango 'engcambu engunobangela' othembekileyo - uluntu lophando aluchonge nokuba libe linye.
Icandelo 09
Ukuhlala Na I-MCAS
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.
Ukulandelela iimpawu kunye nokufumana izinto ezibangela
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.
Iiveki ezimbini bubuncinane phambi kokuba iipateni zifundeke, kwaye zide zibengcono. Ngenxa yokuba ukusabela kunokulibaziseka ngeeyure, ukutyhileka kwezinto kunokuhlala kakuhle ngaphambi kokuba iimpawu zibonakale. Jonga ukuphindaphinda kwiziganeko kuneenkcazo zesiganeko esisodwa - injongo yi-hypothesis yokuvavanya, kungekhona isigwebo.
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
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.
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.
- Ukulungela ngokungxamisekileyo
- 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.
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.
Iikopi ezinikwe usapho, oogxa, okanye abasebenzi besikolo zithetha ukuba abanye banokuthatha inyathelo ukuba awukwazi.
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.
- Inqaku ngothintelo lokutya
- Ukutya okuphantsi kwe-histamine kunye nokuphelisa kuzanywa ngokubanzi, kwaye ubungqina obuxhasayo bulinganiselwe kwaye bunomgangatho ophantsi. Uthintelo olwandisiweyo olungajongwanga luthwala umngcipheko wokwenyani wokunqongophala kwesondlo kunye nokutya okungalungelelananga, kwaye loo mngcipheko uphezulu kwabakwishumi elivisayo. Ulingo olucwangcisiweyo, olunexesha elilinganiselweyo kunye nokuqaliswa kwakhona okucwangcisiweyo - ngokufanelekileyo kunye nokubandakanyeka kwe-dietitian - yindlela ekhuselekileyo.
- Kuyafaneleka ukubiza igama lomgibe ngokuthe ngqo: kunokwenzeka ukunciphisa izinto ezibangela ukuba kube nzima ukuba ubomi buvumelane phantse nantoni na. Irejimeni ephelisa ukusabela ngokuphelisa umsebenzi, isikolo, ukutya okwahlukahlukeneyo, nokunxibelelana nabanye iye yatshintsha enye indlela yokwenzakala kwenye.
- 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.
Apho umngcipheko we-anaphylaxis usebenza, i-epinephrine kunye nesicwangciso sesenzo esibhaliweyo siyimfuneko - i-antihistamines ayithathi indawo.
Uthintelo olunobundlavini lokutya luthwala imingcipheko yokwenyani kwaye kufuneka lucwangciswe, lubekelwe ixesha, kwaye lube phantsi kweliso.
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Ngaba i-MCAS yelifa?
Ngaba abantwana bangayenza i-MCAS?
Ngaba i-MCAS inganyamalala ngokwayo?
Kutheni iimpawu zam zihlala zitshintsha?
Kutheni iziphumo zam zovavanyo zihlala ziqhelekile?
Ngaba uxinzelelo lunokubangela amadangatye?
Ngaba ukutya kuyayinyanga i-MCAS?
Yintoni umahluko phakathi kwe-MCAS kunye ne-mastocytosis?
Ngaba i-MCAS iyafana nokunganyamezeli kwe-histamine?
Ngaba ndiyaludinga uvavanyo lwe-positive ukuze ndifunyanwe?
Loluphi uhlobo logqirha olunyanga i-MCAS?
Ngaba i-antihistamines iya kulungisa yonke into?
Kutheni ndisabela kumayeza ekufanele ukuba ayanceda?
Ngaba i-MCAS isiphene?
Ngaba i-MCAS inokubangela i-anaphylaxis?
Ngaba ukukhulelwa kuyayichaphazela i-MCAS?
Kutheni ubushushu bundenza ndisabele xa ndingadinwa nantoni na?
Kuthatha ixesha elingakanani ukuba unyango lusebenze?
Ngaba kufuneka nditshintshe amayeza amaninzi ngexesha elinye ukuze ndibengcono ngokukhawuleza?
Ngaba i-COVID-19 okanye olunye usulelo lunokubangela i-MCAS?
Ngaba i-MCAS ixhaphake kakhulu kwabasetyhini?
Ixhaphake kangakanani i-MCAS?
Ngaba kufuneka ndikuphephe konke ukutya kuluhlu oluphezulu lwe-histamine?
Kufuneka ndize ngantoni kwidinga lam lokuqala lengcali?
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
- Isikhokelo samalungu osapho kunye nabanonopheli — ikhonkco liza kongezwa
- Izikhokelo zonyango
- Iingxelo zemvumelwano kunye nezikhokelo zeklinikhi ezijoliswe kubasebenzi bezempilo. Kuluncedo ukuzisa amadinga.
- Indlela yokuxilonga yemvumelwano - ucaphulo kunye nekhonkco kufuneka zongezwe
Isikhokelo soluntu lwengcali yezonyango - ikhonkco liza kongezwa
Uvavanyo lomlamli kunye neprotocol yokuphatha isampula - ikhonkco liza kongezwa
- Iingqwalaselo ze-perioperative kunye ne-anesthesia - ikhonkco liza kongezwa
- Amaphepha oPhando
- Uncwadi olusisiseko. Apho kunokwenzeka, khetha imithombo yolwazi ephononongwe ngoontanga kunye nemihla yokupapasha amanqaku, njengoko lo mmandla uhamba.
- 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
- Iingcali zoluntu kunye nemibutho yezemfundo esebenza kwisifo semast cell.
Imibutho yesizwe kunye neyezizwe ngezizwe / i-immunology - amakhonkco ukuba wongezwe
Uthungelwano lophando lwesifo seeseli- amakhonkco ukuba wongezwe
- Imibutho yezifo ezinqabileyo- amakhonkco ukuba wongezwe
- Iincwadi
Ufundo olude lwezigulane kunye neekliniki.
Izihloko ezijoliswe kwisigulana - ukuba yongezwe
- Izicatshulwa zezonyango- ukuba yongezwe
- Iividiyo ezifundisayo
- Iintetho ezirekhodiweyo, iingcaciso, kunye nemathiriyeli yenkomfa.
Iividiyo zokuchaza intshayelelo- amakhonkco ukuba wongezwe
Iintetho zeenkomfa zeengcali - amakhonkco ukuba wongezwe
- Iinkcukacha ngomxholo wezemfundo we-MCAS — amakhonkco ukuba wongezwe
- Imibutho yeNkxaso
- Inkxaso yoontanga, ubhengezo, kunye noluntu.
Imibutho exhasa izigulane - 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.
Ngoku kufunwa izifundo zemast cell- amakhonkco ukuba wongezwe
Isikhokelo malunga nokuba yintoni na inxaxheba kulingo— ikhonkco liza kongezwa
- Isitshixo Izinto ezithathwayo
- Awona magama abalulekileyo kwesi sikhokelo, afinyeziwe.
- Isishwankathelo
- I-MCAS luphazamiseko lwemast cell engafanelekanga ukusebenza, kungekhona yeenombolo zeeseli ze-mast - iiseli ziqhelekileyo, i-threshold yazo ayikho.
- 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.
- Iiphaneli zokwaliwa ezingalunganga kunye nomsebenzi wesiqhelo wegazi wesiqhelo ulindeleke kwi-MCAS kwaye awuyikhupheli ngaphandle.
- 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.
- 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.
- Akukho nobangela we-MCAS usekiweyo - phatha 'ingcambu engunobangela' ozithembileyo ngokuthandabuza.
Apho umngcipheko we-anaphylaxis usebenza, i-epinephrine kunye nesicwangciso sesenzo esibhaliweyo zibalulekile; i-antihistamines ayithathi ndawo.
Injongo yobona bomi buphangaleleyo kwiphysiology yakho iya kuxhasa - hayi obona bomi buncinci bunqanda zonke iimpawu.
Mast Cell Activation