Uhlu Lokuphakathi
01Yini i-MCAS?02Ukuqonda ama-Mast Cells03Kwenzekani Ngesikhathi Sokuqalisa Kwe-Mast Cell04Izimpawu ze-MCAS05Izimbangela Ezivamile06Kungani i-MCAS Kunzima Ukuyihlonza07Izimo Ezihambisanayo08Ukuqonda Kwamanje Kwezimbangela09Uhlala ne-MCAS10Imibuzo Evame Ukubuzwa11Izinsiza Zokufunda Ezengeziwe12Okuthathwayo Okubalulekile13Uhlu lwamagama
Isigaba 01
Yini I-MCAS?
I-Mast Cell Activation Syndrome (MCAS) yisimo lapho ama-mast cells - amaseli omzimba ahlala cishe kuzo zonke izicubu zomzimba - esebenza ngokungafanele nangokuphindaphindiwe, ekhulula abaxhumanisi bamakhemikhali ngobuningi futhi ngezinye izikhathi abangasebenzisi injongo yokuzivikela ewusizo. Umphumela uba iphethini yezimpawu eziphindaphindayo, zesiqephu ezithinta amasistimu wezitho ezimbili noma ngaphezulu ngesikhathi esisodwa.
Incazelo ibalulekile ngoba ihlukanisa i-MCAS nezimo ezifuze ngayo kancane. Akukho ukunqwabelana okungavamile kwamangqamuzana okutholakala ku-biopsy, akukho ukungezwani komzimba okukodwa ongakubona lapho kuhlolwa ukuhlaba esikhunjeni, futhi ngokuvamile akukho okungavamile nhlobo emsebenzini waselabhorethri ovamile. Lesi sifo sihlala embundwini lapho amangqamuzana abukeka evamile anquma ukuphendula.
Umqondo Oyinhloko
Ku-MCAS inkinga ayikho bangaki mast cells onawo. Kunjalo badubula kalula kanjani. Zonke ezinye izici zesimo - ukungaqiniseki, ukusabalala kwezinhlelo eziningi, ukuhlolwa okunzima - kulandela kulelo qiniso elilodwa.
Kungani ama-mast cells ekhona nhlobo
Ama-mast cell awawona iphutha lokuklama. Ziphakathi kwezingxenye ezindala kakhulu zesimiso somzimba sokuzivikela ezifweni, ezikhona ngandlela thize kuzo zonke izilwane ezinomgogodla, futhi zenza umsebenzi umzimba owudinga ngempela. Bafaka impendulo yokuqala kuma-parasites, basize ukuqeda ubuthi ngemva kokulunywa nokulunywa, babambe iqhaza ekwelapheni amanxeba nasekulungiseni kabusha izicubu, babambe iqhaza ekulawulweni kokungena kwemithambo yegazi, futhi basize ekugcineni izicubu ezivimbelayo ezihlukanisa umzimba nomhlaba wangaphandle.
Zibekwe ngendlela efanele - zigxile emingceleni, esikhunjeni, emzileni womoya, odongeni lwamathumbu, kanye nezicubu ezizungezile imithambo yegazi nezinzwa. Futhi zakhelwe isivinini: kunokuba zikhiqize impendulo yazo ngokufunwa, ziyibamba zenziwe ngaphambili kumagranule esitoreji, zilungele ukukhululwa phakathi nemizuzwana yesiginali. Lezo zakhiwo yikho okwenza ukungezwani komzimba kusheshe, futhi yikho futhi okwenza kusebenze okungalungile kube nzima ukuphila nakho.
Ibukeka kanjani imisebenzi ye-mast cell evamile
Ekuphenduleni okunempilo kokuzivikela komzimba, ingqamuzana le-mast lihlangabezana nosongo lwangempela - i-antigen ye-parasite, ubuthi, noma ukulimala kwezicubu - futhi liwohloke ngokulingana nalo. Imithambo yegazi iyanwebeka futhi ikwazi ukungena kalula, okwenza i-plasma namangqamuzana omzimba omzimba angene esicutshini. Iziphetho zezinzwa ziyashukunyiswa, zikhiqize ukulunywa noma ubuhlungu obudonsela ukunaka kusayithi. Amanye amangqamuzana omzimba omzimba ayabuthwa. Lapho usongo selusuliwe, impendulo iyaxazululeka.
Konke okungajabulisi ngaleyo nqubo kuyasebenza. Ukuvuvukala kuletha amasosha omzimba endaweni. Ukulunywa kukwenza ukuthi ususe noma yini edala umonakalo. Amafinyila abamba futhi axoshe izinto ezicasulayo. Ngempendulo elinganayo, ukungakhululeki kungokwesikhashana futhi inzuzo ingokoqobo.
Ku-MCAS, lowo mshini ofanayo usebenza ngaphandle kwesizathu esilinganayo. Amangqamuzana e-Mast avutha ngokuzenzekelayo, noma ephendula izisusa okufanele zingabonakali - igumbi elifudumele, iphunga emhubheni, ukudla, ukuma, ukuvivinya umzimba okuncane, usuku oluvamile olucindezelayo. Abalamuli abakhululiwe yibo abafanayo abasetshenziswa ekuzivikeleni okusemthethweni, kodwa bakhululwa esimweni esingalungile futhi ngokuvamile ngokuphindaphindiwe.
Ngenxa yokuthi ama-mast cell ahlala ezicutshini kuwo wonke umzimba, futhi ngenxa yokuthi abalamuli bawo basebenza emithanjeni yegazi, imisipha ebushelelezi, imizwa, namanye amangqamuzana omzimba omzimba, isiqephu esisodwa singaveza ukuguquguquka, inhliziyo egijimayo, ukuhlabeka kwesisu, ukuphelelwa umoya, kanye nenkungu yengqondo kanyekanye. Kudokotela ongacabangi ngama-mast cells, leyo nhlanganisela ingase ibukeke njengezinkinga ezimbalwa ezingahlobene ezifika ngesikhathi esisodwa - okuyingxenye enkulu yokuthi kungani isimo sigejwa.
Isifaniso esilula
| I-MCAS ayiyona i-mastocytosis | Lona umehluko owodwa obaluleke kakhulu ensimini, futhi ukudida kokubili kuholela ekudidekeni ngayo yonke into ezansi nomfula. | |
|---|---|---|
| I-Mastocytosis iqhathaniswa ne-MCAS | I-Mastocytosis | I-MCAS |
| Ukungajwayelekile okuyinhloko | Ama-mast cells amaningi kakhulu anqwabelana esicutshini | Inombolo evamile yamaseli e-mast, asebenza ngokungafanele |
| Isibalo samaseli | Ephakeme - ukwanda clonal | Okuvamile |
| I-tryptase eyisisekelo | Ngokuvamile iphakanyiswa ngokuqhubekayo | Ngokuvamile kujwayelekile ekuqaleni |
| Ukutholwa kofuzo okujwayelekile | KIT Ukuguqulwa kwe-D816V ezimweni eziningi zesistimu yabantu abadala | Akukho ukuguqulwa kwe-causative okukodwa okusunguliwe |
| Imiphumela ye-Biopsy | Izibalo ze-mast cell ezingavamile ziyaboniswa | Akukho ukuqoqwa kwesici |
| Uphawu lwesikhumba | Izilonda ze-maculopapular (urticaria pigmentosa) ezivamile kwezinye izinhlobo | Asikho isilonda esithile sesikhumba sokuxilonga |
Kuqinisekiswa kanjani
Ubufakazi obuyinhloso bokweqisa kwamaseli
Iphethini yezimpawu + ubufakazi bomlamuleli + impendulo yokwelashwa
Zombili lezi zimo zingaveza izimpawu ezigqagqene, ngoba kuzo zombili izimo abalamuli be-mast cell bafinyelela izicubu ngokweqile. Kodwa i-mastocytosis ingaboniswa ngokuthola amaseli; I-MCAS ayikwazi, okuyisizathu sokuthi kungani ukuhlonzwa kwayo kuncike kwinhlanganisela yemibandela esikhundleni sokuhlola okukodwa kokuqinisekisa.
I-MCAS ayikona ukungezwani komzimba okujwayelekile
Ukusabela kwe-MCAS akuvamile. Eziningi aziyona i-IgE-mediated nakancane, kunalokho zivela ngamanye ama-receptors endaweni ye-mast cell noma ngokukhuthazwa okuqondile ngokomzimba. Izibangeli zivame ukuba ngokomzimba - ukushisa, amakhaza, ingcindezi, ukuzikhandla - kunokuba amaprotheni, futhi alikho iphaneli yokungezwani komzimba eklanyelwe ukuthola lokho. Ukusabela kungase kubambezeleke ngamahora, kunqamule ukuxhumana okunembile phakathi kokuchayeka kanye nomthelela, futhi umkhawulo ungashintsha ngokulala, ingcindezi, isimo sehomoni, noma ukugula kwakamuva, ngakho ukuchayeka okubekezelelwe isonto elilodwa kuvusa ukusabela ngokulandelayo.
Umtholampilo wePearl
- Okubonakalayo okuphakanyisiwe
- Umfanekiso wokuqhathanisa ohlangothini ngalunye: amaphaneli amathathu abonisa inani le-mast cell elivamile eliphendula ngokulinganayo, iphaneli ye-mastocytosis ebonisa izinombolo zamaseli ezeqile, kanye nephaneli ye-MCAS ebonisa izinombolo zeseli ezivamile ezinokukhululwa komlamuli okuyihaba. Isibalo samaseli afanayo kumaphaneli owodwa namathathu, okukhiphayo okuhluke kakhulu.
- Isifinyezo sesigaba
- I-MCAS ukuphazamiseka kwe-mast cell engafanele ukwenza kusebenze, hhayi izinombolo ze-mast cell.
- Amaseli e-Mast angamaseli omzimba awusizo ngokusemthethweni abekwe emigoqweni yomzimba futhi akhelwe ukuphendula ngemizuzwana.
I-mastocytosis ihilela ukweqisa kwamaseli futhi ingaboniswa ngokuqondile; I-MCAS ayikwazi, yingakho ukuxilongwa kuyinhlanganisela.
Ukuhlolwa kokungezwani kahle komzimba okungalungile kuvamile ku-MCAS futhi akukhiphi.
Isigaba 02
Ukuqonda Amaseli Ensika
Ukulandela ukuthi kungani i-MCAS iziphatha ngendlela eyenza ngayo, kuyasiza ukwazi ukuthi la maseli avelaphi, ahlala kuphi, ahlala isikhathi esingakanani, nokuthi alalela ini.
Umsuka kanye nentuthuko
Ama-mast cell aqala emnkantsheni, avela kumaseli e-CD34-positive haematopoietic progenitor cell - inani elifanayo labantu elidala amanye amaseli egazi. Nokho, ngokungafani namangqamuzana amaningi amhlophe egazi, awaqedi ukukhula kwawo lapho. Okhokho abangavuthiwe bangena emgudwini wegazi, bajikeleze isikhashana, bese bethuthela esicubuni, lapho ukuvuthwa kokugcina kwenzeka khona.
Lokho kukhula kuncike kakhulu ekusayineni kwe-stem cell factor (SCF) ngesamukeli se-KIT endaweni ye-mast cell. Le ndlela imaphakathi ne-mast cell biology, futhi iyindlela efanayo ephazanyiswe yi- KIT Ukuguqulwa kwe-D816V okutholakala ezimweni eziningi zabantu abadala ze-systemic mastocytosis. Indawo yethishu yendawo ibumba i-phenotype yokugcina, yingakho ama-mast cells emathunjini ehluke ngokulinganiswa kulawo asesikhunjeni.
| Ukusabalalisa kuwo wonke umzimba | Amaseli e-Mast asatshalaliswa ngendlela ehlelekile kunokuba afane, agxile lapho umzimba uhlangana khona nomhlaba wangaphandle futhi uzungeze izakhiwo ezithwala amasignali negazi. Lokhu kusatshalaliswa kuchaza uhlamvu lwesistimu eningi ye-MCAS ngokuqondile kunanoma yiliphi elinye iqiniso elilodwa. | Lapho ama-mast cell ahlala khona, nokuthi kukhiqizani lapho eqhuma kabi |
|---|---|---|
| Indawo | Iqhaza empilweni | Izimpawu lapho ukwenza kusebenze kungafanele |
| Isikhumba kanye nesikhumba | Ukuvikela isithiyo; ukuphendula ekulunyweni, ekulunyweni, nasekulimaleni | Ukuqunjelwa, ukulunywa, isifuba, idermographism, ukuvuvukala |
| Umgudu wamathumbu | Ukugcinwa kwezithiyo, ukulawulwa kwe-motility, ukuphendula ezinsongweni ezingenisiwe | Ukuqunjelwa, isicanucanu, i-reflux, isifo sohudo, ukuqunjelwa, ukuqunjelwa |
| Airways kanye sinuses | Ukuzivikela kolwelwesi lokuphefumula | Ukuqunjelwa, i-rhinitis, ukuqina komphimbo, ukukhwehlela, ukukhwehlela |
| Emithanjeni yegazi | Ukulawulwa kwethoni yomkhumbi kanye nokungeneka | Umfutho wegazi uyashintsha, tachycardia, flushing, presyncope |
| Ubuchopho kanye ne-meninges | Kwethulwa nge-perivascularly naku-meninges; ukubonakaliswa kwe-neuroimmune | Inkungu yengqondo, ikhanda elibuhlungu, isiyezi (izindlela zisacutshungulwa) |
| Izicubu ezixhumeneyo | Ukulungiswa kabusha nokulungiswa kwezicubu | Ubuhlungu obuhlangene nemisipha; ukuhlobana okunokwenzeka kokudlulana kwezicubu ezixhumeneyo |
Umnkantsha
Indawo yomsuka wokhokho
Lapho isifo se-clonal sihlolwa lapho kusolwa i-mastocytosis
Qaphela ukungena kobuchopho ngokucophelela. Ama-mast cell akhona ngempela kuma-meninges nasezimeni ze-perivascular ngaphakathi kwesistimu yezinzwa emaphakathi, futhi ukusebenzisana kwe-mast cell-nerve kuyindawo yocwaningo esebenzayo. Kodwa-ke, izindlela ezixhumanisa ukusebenza kwe-mast cell nezimpawu ezithile zemizwa nezingqondo zisalungiswa, futhi izimangalo zemishini eziqinisekayo kule ndawo zidlula ubufakazi bamanje.
Umjikelezo wokuphila
Ama-mast cell aphila isikhathi eside ngokungavamile. Lapho i-neutrophil ingase iphile amahora ambalwa kuya ezinsukwini, amangqamuzana e-tissue mast angaqhubeka amasonto kuya ezinyangeni, futhi ubufakazi bubonisa ukuthi amanye aphila isikhathi eside kakhulu. Bayakwazi futhi ukwenza kabusha i-regranulation - ngemva kokuwohloka, i-mast cell ingakha kabusha izitolo zayo futhi iphendule futhi, kunokuba ife kunqubo.
- Lokhu kubalulekile emtholampilo ngezindlela ezimbili. Kusho ukuthi izimpawu zokushayela kwamangqamuzana zizinzile kunokuphenduka ngokushesha, ngakho-ke akukho ukulungiswa kwemvelo okusheshayo ongakulinda. Futhi kusho ukwelapha okuzinzisa ama-mast cells ngokuvamile kufanele kuthathwe ngokungaguquki ngokuhamba kwesikhathi - ushintsha ukuziphatha kwezakhamuzi ezihlala isikhathi eside, ungasusi okwesikhashana.
- Ama-Surface receptors: lokho i-mast cell elalelayo
- Amaseli e-Mast athwala inqwaba yama-receptors angaphezulu, futhi ububanzi balolo hlu yisizathu sokuthi izinto eziningi ezahlukene zingasebenza njengezibangeli. Ezinye zezimpawu ezinhle kakhulu zihlanganisa:
- I-FceRI - i-high-affinity IgE receptor. Lena indlela ye-allergies yakudala: Amasosha omzimba e-IgE abopha isamukeli, futhi axhumaniswe ngokuphambana ngokwenza kusebenze ama-allergenic triggers.
- I-MRGPRX2 - i-receptor mediating ivula i-IgE-independent ngezidakamizwa ezithile nama-peptide. Ukuhlukaniswa kwezinhlamvu zayo kusize ukuchaza ukusabela okwenzeka ngaphandle kokubandakanyeka kwe-IgE okubonakalayo.
- KIT - i-receptor ye-stem cell factor; okuphakathi kokusinda kwe-mast cell, ukuvuthwa, kanye nokuhlala kwezicubu.
- Ama-receptors ahambisanayo (C3a, C5a) - vumela ukuthi kusebenze ngengalo ehambisanayo yokugonywa okungokwemvelo.
Ama-receptors e-CRH - phendula i-hormone ekhulula i-corticotropin, ihlinzeka ngomzila obhaliwe lapho i-physiology yokucindezeleka ifinyelela kuma-mast cells.
I-Adenosine, i-opioid, i-estrogen namanye ama-receptors - ukufaka isandla kumaphrofayili we-trigger ahlukahlukene kanye nawodwa abonwa ekusebenzeni.
- Ingabe Bewazi?
- Ukuba khona kwemizila eminingi engeyona ye-IgE yingakho ukuhlolwa okujwayelekile kokungezwani komzimba kuvame ukubuya ngokwejwayelekile ku-MCAS. Lezo zivivinyo zakhelwe ukuthola ukuzwela kwe-IgE-mediated — indlela eyodwa phakathi kwezimbalwa ezingamisa i-mast cell.
- Umfanekiso weseli ye-mast enelebula: ulwelwesi lweseli olugcwele ama-receptors aqanjwe igama (i-FcεRI, MRGPRX2, KIT, C3a/C5a, TLRs, CRH-R), ingaphakathi ligcwele ama-granules, elinesikwe elibonisa okuqukethwe yizinhlamvana. Isethi ehambisanayo ebonisa iseli ngaphambi nangemuva kokuwohloka.
- Ama-mast cells asuka ku-CD34+ progenitors emnkantsheni kodwa avuthiwe esicutshini, ngakho izibalo zegazi aziwabonisi.
- Ukusayinda kwe-KIT/SCF kuyinhloko ekuthuthukisweni kwabo - indlela ephazamisekile kumastocytosis amaningi esistimu yabantu abadala.
Ziphila isikhathi eside futhi zingakwazi ukuphindaphinda, ngakho-ke ukuphatha kuyaqina esikhundleni sesifundo esifushane.
Izinhlobo eziningi zama-receptor ngale kwe-IgE zingazivula, yingakho amaphaneli okungezwani komzimba evame ukugeja izibangeli ze-MCAS.
Ukuvuselela Iseli Yezinsimbi
Ukwenza kusebenze ukulandelana, futhi isinyathelo ngasinye sinegama. Ukukulandela kwenza ukuhlukahluka okudidayo kwezimpawu ze-MCAS kube lula kakhulu ukuzihumusha.
Isikhuthazo sifinyelela kungqamuzana - i-allergen, isidakamizwa, ukushisa, ingcindezi, ukuzikhandla, i-hormone yokucindezeleka, ukutheleleka, noma akukho nhlobo isisusa esibonakalayo.
Ama-Surface receptors abamba iqhaza futhi ama-Cascades wokusayina we-intracellular aqala. Ukungena kwe-calcium kuyisinyathelo sokuqala esibalulekile.
Ama-granules esitoreji ahlangana nolwelwesi lweseli futhi akhiphe okuqukethwe kwawo okwenziwe ngaphambilini kuzicubu ezizungezile - ngemizuzwana.
Abalamuli abakhelwe ngaphambilini bakhululwa ngokushesha; abalamuli abasanda kuhlanganiswa njenge-prostaglandin kanye ne-leukotrienes balandela phakathi nemizuzu, nama-cytokines emahoreni amaningi.
5
Izimpawu
Abalamuli basebenza emithanjeni yegazi, imisipha ebushelelezi, imizwa, namanye amangqamuzana omzimba omzimba, akhiqiza imiphumela kuwo wonke amasistimu wezitho eziningi.
Izici ezimbili zalokhu kulandelana zichaza okuningi kwalokho okutholwa yiziguli. Okokuqala, isigaba esisheshayo sidonsa ezitolo ezenziwe ngaphambilini, yingakho ukusabela kungaqala phakathi nemizuzwana. Okwesibili, abalamuli besigaba sakamuva bahlanganiswa ngemva kokuthi kuqale ukusebenza, yingakho ezinye izimpawu zifika kahle ngemva kwe-trigger nokuthi kungani ukusabela kungase kube nokubambezeleka kwamahora wesibili wamagagasi kamuva.
Amaseli amakhulu akhulula abalamuli phakathi amagagasi, hhayi kanye kanye. Abalamuli abakhelwe ngaphambilini bayaphuma ngemizuzwana; abaxhumanisi be-lipid bakhiwa ngamaminithi; ama-cytokines aqoqana phakathi namahora. Ngakho-ke umcimbi owodwa wokwenza kusebenze ungaveza izimpawu usuku lonke.
| Abalamuli abakhulu nokuthi ngamunye wenzani | Ama-mast cell ayakwazi ukukhulula inani elikhulu kakhulu lezinto. Okulandelayo yizona okuxoxwe ngazo kakhulu ngokomtholampilo, futhi ndawonye kubala iningi lesithombe esibonwayo sezimpawu. | Abalamuli be-mast cell abakhulu |
|---|---|---|
| Umlamuleli | Uhlobo | Imiphumela eyinhloko |
| I-Histamine | Uhlobo | Kwakhiwe ngaphambilini |
| Umlamuli owaziwa kakhulu. Ivula imithambo yegazi futhi ikhulise ukungena kwayo, ivuse iziphetho zezinzwa, futhi ikhulise ukukhishwa kwe-asidi yesisu. Ikhiqiza ukuguquguquka, ukulunywa, ukuqubuka, umfutho wegazi ophansi, i-tachycardia, ikhanda elibuhlungu, kanye ne-GI cramping. Isebenza ngama-subtypes ama-receptor amaningi - i-H1 esikhumbeni nasendleleni yomoya, i-H2 emathunjini nasenhliziyweni - yingakho ukuvimba womabili amakilasi kuvame ukusebenza kangcono kunokuvimba noma yikuphi. | I-Tryptase | Iphrotheni eningi kakhulu kuma-mast cell granules kanye nomaka waselabhorethri osungulwe kakhulu wokwenza kusebenze. Inomthelela ekwakhiweni kabusha kwezicubu kanye nokuvuvukala, futhi ingakhulisa ukusabela kwe-mast cell. Inani layo lokuxilonga livela ekubeni licaciswe ngokuqhathaniswa namaseli amakhulu. |
| I-Prostaglandin D2 | I-Tryptase | Okusanda kuhlanganiswa |
| Umxhumanisi we-lipid okhiqizwa ngemuva kokusebenza. Ibangela i-vasodilation ebonakalayo kanye nokushaywa, i-bronchoconstriction, kanye nesifo sohudo, futhi ingaba nomthelela ekwehleni okukhulu komfutho wegazi. Okuvame ukubandakanyeka lapho ukuhlanza kuyisici esivelele. | I-Tryptase | I-Leukotrienes (LTC4, LTD4, LTE4) |
| Ama-bronchoconstrictors anamandla, kakhulu kakhulu kune-histamine ngesisindo. Khulisa ukuvuthwa kwemithambo kanye nokukhiqizwa kwamafinyila, futhi kube nomthelela kukho kokubili izimpawu zendlela yokuphefumula neyomathumbu. I-LTE4 iyalinganiswa emchamweni. | I-Tryptase | Ama-Cytokines (IL-6, TNF-α, IL-1, IL-4 nabanye) |
| Amaprotheni amasignali ashayela ukuvuvukala okubanzi. Iqala kancane futhi isebenza isikhathi eside kunabalamuli abangenhla, futhi kucatshangwa ukuthi ingaba nomthelela ekukhathaleni, ekuguleni komzimba, kanye nekhwalithi ye-systemic 'efana nomkhuhlane' ichaza ngesikhathi nangemuva kokuqubuka. | I-Tryptase | Amakhemikhali |
| Thola amaseli omzimba engeziwe esizeni, akhulise futhi andise impendulo yokuvuvukala ngale kokusabela kokuqala kwe-mast cell. | Uhlobo | I-Platelet activating factor (PAF) |
| Umxhumanisi we-lipid onamandla kakhulu okhuphula ukungeneka kwemithambo futhi ungabangela i-hypotension enzima. Ifakwe ebukhulwini bokusabela kwe-anaphylactic futhi ingavinjelwa ama-antihistamine. | Uhlobo | I-Heparin |
I-anticoagulant eyenzeka ngokwemvelo. Ingase ibe nomthelela ekujuleni okungajwayelekile noma ekuthambekeni ukopha okubikwe ezinye iziguli, futhi ibambe iqhaza ekulawuleni ukuvuvukala kwendawo.
I-Chymase ne-carboxypeptidase A3
- Ama-Granule protease abandakanyeka ekwenziweni kabusha kwezicubu, izindlela zokulawula umfutho wegazi, kanye nokuwohloka kobuthi obuthile nama-peptide.
- Ngoba ama-antihistamine avimba i-histamine kuphela, impendulo engaphelele kuma-antihistamine ihambisana ngokuphelele ne-MCAS kunobufakazi obuphikisana nayo. I-Prostaglandin, i-leukotrienes, i-PAF, nama-cytokines awathintwa yilawo ma-ejenti - okuyisizathu sokwengezwa kwezidakamizwa kancane kancane.
- Inqubo ye-flowchart egijima kwesokunxele iye kwesokudla: Qalisa → Ukuqalisa → Ukwehliswa kwegranulation → Ukukhishwa komlamuli → Izimpawu, ezinomugqa wesikhathi ohlanganisa igatsha ngaphansi obonisa abalamuli abakheke ngaphambilini ngemizuzwana, abaxhumanisi be-lipid ngamaminithi, nama-cytokines ngamahora, igatsha ngalinye lichazwe ngezimpawu eliziqhubayo.
- Ukwenza kusebenze kulandela ukulandelana: i-trigger, activation, degranulation, ukukhululwa komlamuli, izimpawu.
- Abalamuli abakhelwe ngaphambilini bakhululwa ngemizuzwana; abaxhumanisi be-lipid ngamaminithi; ama-cytokines amahora amaningi.
I-Histamine ingomunye wabalamuli abaningi, yingakho ama-antihistamine ewodwa evame ukunikeza ukukhululeka okuyingxenye.
I-PAF kanye ne-prostaglandin ingashayela imiphumela enzima yenhliziyo nemithambo yegazi i-antihistamines engakhulumi ngayo.
Isigaba 04
| I-MCAS ichazwa ngokwengxenye ngobubanzi bayo. Ngenxa yokuthi ama-mast cell ahlala cishe kuzo zonke izicubu, izimpawu zingavela cishe noma kuphi - futhi imfuneko yezinhlelo zezitho ezimbili noma ngaphezulu ibalulekile ekuxilongweni. | Amathebula angezansi ahlela izimpawu ezivame ukubikwa ngohlelo lomzimba. Izixwayiso ezimbili ngaphambi kokuzifunda. Okokuqala, asikho isiguli esinakho konke lokhu, futhi uhlu olude akufanele lufundwe njengohlu lokuhlola oluzohambisana nalo. Okwesibili, zonke izimpawu lapha zenzeka kwezinye izimo eziningi; yini ephakamisa ukuthi i-MCAS iyona iphethini - i-episodic, i-recurrent, multisystem, ngokuvamile enezibangeli ezibonakalayo - kunanoma yikuphi okufakiwe ngakunye. |
|---|---|
| Isikhumba kanye ne-mucosal | Uphawu |
| Amanothi | Ukushaywa umoya |
| Ngokuvamile kungazelelwe, kuthinta ubuso, intamo, nesifuba; isici esibikwa njalo | Ukuluma (pruritus) |
| Kungenzeka noma ngaphandle kokuqhuma okubonakalayo | I-Hives (i-urticaria) |
| Ama-welts aphakanyisiwe, alumayo; ingase ibe yesikhashana futhi ifuduke | I-Dermographism |
| Ama-welts aphakanyiswe ukuphulula okuqinile kwesikhumba - uphawu olubonakalayo olubonakalayo | I-Angioedema |
| I-MCAS ichazwa ngokwengxenye ngobubanzi bayo. Ngenxa yokuthi ama-mast cell ahlala cishe kuzo zonke izicubu, izimpawu zingavela cishe noma kuphi - futhi imfuneko yezinhlelo zezitho ezimbili noma ngaphezulu ibalulekile ekuxilongweni. | Amathebula angezansi ahlela izimpawu ezivame ukubikwa ngohlelo lomzimba. Izixwayiso ezimbili ngaphambi kokuzifunda. Okokuqala, asikho isiguli esinakho konke lokhu, futhi uhlu olude akufanele lufundwe njengohlu lokuhlola oluzohambisana nalo. Okwesibili, zonke izimpawu lapha zenzeka kwezinye izimo eziningi; yini ephakamisa ukuthi i-MCAS iyona iphethini - i-episodic, i-recurrent, multisystem, ngokuvamile enezibangeli ezibonakalayo - kunanoma yikuphi okufakiwe ngakunye. |
|---|---|
| Ukulibaziseka ukuphola kwesilonda | Kubikwe ezinye iziguli; izindlela ezingakasungulwa ngokugcwele |
| Okokuphefumula | Ukucinana kwamakhala kanye ne-rhinitis |
| Ngokuvamile okungapheli futhi okungeyona isizini | Ukuqina komphimbo |
| Idinga ukuhlolwa okuphuthumayo uma kuqhubeka - kungase kubonise i-anaphylaxis | Ukuhefuzela kanye nokuphelelwa umoya |
| I-bronchoconstriction eqhutshwa yi-leukotriene iyindlela eyaziwayo | Ukukhwehlela okungapheli |
| I-MCAS ichazwa ngokwengxenye ngobubanzi bayo. Ngenxa yokuthi ama-mast cell ahlala cishe kuzo zonke izicubu, izimpawu zingavela cishe noma kuphi - futhi imfuneko yezinhlelo zezitho ezimbili noma ngaphezulu ibalulekile ekuxilongweni. | Amathebula angezansi ahlela izimpawu ezivame ukubikwa ngohlelo lomzimba. Izixwayiso ezimbili ngaphambi kokuzifunda. Okokuqala, asikho isiguli esinakho konke lokhu, futhi uhlu olude akufanele lufundwe njengohlu lokuhlola oluzohambisana nalo. Okwesibili, zonke izimpawu lapha zenzeka kwezinye izimo eziningi; yini ephakamisa ukuthi i-MCAS iyona iphethini - i-episodic, i-recurrent, multisystem, ngokuvamile enezibangeli ezibonakalayo - kunanoma yikuphi okufakiwe ngakunye. |
|---|---|
| Ukuzwela amakha namakhemikhali asemoyeni | Ukumataniswa kwezimpawu zokuqalisa okubikwe kakhulu |
| Inhliziyo nemithambo yegazi | I-Tachycardia |
| Inhliziyo egijimayo, ngokuvamile ngaphandle kokuzikhandla | Ukungaqini komfutho wegazi |
| Ingashwibeka kunoma iyiphi indlela phakathi neziqephu | I-presyncope ne-syncope |
| Ikhanda elikhanyayo noma ukuquleka, ngokuvamile lapho umile | Ukungakhululeki kwesifuba |
| I-MCAS ichazwa ngokwengxenye ngobubanzi bayo. Ngenxa yokuthi ama-mast cell ahlala cishe kuzo zonke izicubu, izimpawu zingavela cishe noma kuphi - futhi imfuneko yezinhlelo zezitho ezimbili noma ngaphezulu ibalulekile ekuxilongweni. | Amathebula angezansi ahlela izimpawu ezivame ukubikwa ngohlelo lomzimba. Izixwayiso ezimbili ngaphambi kokuzifunda. Okokuqala, asikho isiguli esinakho konke lokhu, futhi uhlu olude akufanele lufundwe njengohlu lokuhlola oluzohambisana nalo. Okwesibili, zonke izimpawu lapha zenzeka kwezinye izimo eziningi; yini ephakamisa ukuthi i-MCAS iyona iphethini - i-episodic, i-recurrent, multisystem, ngokuvamile enezibangeli ezibonakalayo - kunanoma yikuphi okufakiwe ngakunye. |
|---|---|
| I-Anaphylaxis | Kunzima, kuyashesha, okungaba yingozi empilweni - bona isigaba sezimo eziphuthumayo |
| Amathumbu | Ukuqina kwesisu nobuhlungu |
| Phakathi kwezimpawu ezivame ukubikwa sezizonke | Isicanucanu nokuhlanza |
| Kungase kulandele ukudla noma kwenzeke ngokuzimela | Uhudo kanye/noma ukuqunjelwa |
| I-Motility ingase ihluke phakathi kweziqephu kanye nesikhathi | I-Reflux |
| I-MCAS ichazwa ngokwengxenye ngobubanzi bayo. Ngenxa yokuthi ama-mast cell ahlala cishe kuzo zonke izicubu, izimpawu zingavela cishe noma kuphi - futhi imfuneko yezinhlelo zezitho ezimbili noma ngaphezulu ibalulekile ekuxilongweni. | Amathebula angezansi ahlela izimpawu ezivame ukubikwa ngohlelo lomzimba. Izixwayiso ezimbili ngaphambi kokuzifunda. Okokuqala, asikho isiguli esinakho konke lokhu, futhi uhlu olude akufanele lufundwe njengohlu lokuhlola oluzohambisana nalo. Okwesibili, zonke izimpawu lapha zenzeka kwezinye izimo eziningi; yini ephakamisa ukuthi i-MCAS iyona iphethini - i-episodic, i-recurrent, multisystem, ngokuvamile enezibangeli ezibonakalayo - kunanoma yikuphi okufakiwe ngakunye. |
|---|---|
| Ukuqunjelwa kanye nokusebenza kabusha kokudla | Ivamise ukushayela imikhawulo yokudla - bona izexwayiso esigabeni 9 |
| I-Neurological and cognitive | Inkungu yengqondo |
| Ubunzima bokugxilisa ingqondo, ukukhumbula, kanye nokuthola amagama | Ikhanda elibuhlungu kanye ne-migraine |
| I-Migraine ibikwa ngamanani aphezulu kulesi sibalo sabantu | Isiyezi |
| Ivamise ukugqagqana nezici zenhliziyo nemithambo yegazi | Ukukhathala |
| Okuvame ukuba nzima futhi phakathi kwezimpawu ezibikiwe ezikhubaza kakhulu | Ukuzwela kwezinzwa |
| I-MCAS ichazwa ngokwengxenye ngobubanzi bayo. Ngenxa yokuthi ama-mast cell ahlala cishe kuzo zonke izicubu, izimpawu zingavela cishe noma kuphi - futhi imfuneko yezinhlelo zezitho ezimbili noma ngaphezulu ibalulekile ekuxilongweni. | Amathebula angezansi ahlela izimpawu ezivame ukubikwa ngohlelo lomzimba. Izixwayiso ezimbili ngaphambi kokuzifunda. Okokuqala, asikho isiguli esinakho konke lokhu, futhi uhlu olude akufanele lufundwe njengohlu lokuhlola oluzohambisana nalo. Okwesibili, zonke izimpawu lapha zenzeka kwezinye izimo eziningi; yini ephakamisa ukuthi i-MCAS iyona iphethini - i-episodic, i-recurrent, multisystem, ngokuvamile enezibangeli ezibonakalayo - kunanoma yikuphi okufakiwe ngakunye. |
|---|---|
| Imizwa ye-neuropathic | Ukuluma, ukushisa, noma ukuba ndikindiki okubikwe ngezinye iziguli |
| I-musculoskeletal, i-genitourinary, neyomqondo | Ubuhlungu obuhlangene nemisipha |
| Ingase idlulelane nezimo zezicubu ezixhumeneyo okuxoxwe ngazo esigabeni 7 | Ubuhlungu bethambo |
| Iqinisekisa ukuhlolwa, ikakhulukazi lapho i-mastocytosis icatshangwa khona | Ukuphuthuma kwesinye kanye nobuningi |
| Izimpawu ezifana ne-Interstitial-cystitis ziyabikwa | Izimpawu ziyahlukahluka ngomjikelezo wokuya esikhathini |
| Ithonya lamahomoni kuma-mast cells libhaliwe | Iziqephu ezifana nokukhathazeka |
Ukukhululwa komlamuli kungaveza ngempela ukushayeka kwenhliziyo, ukuphelelwa umoya, nomuzwa wokulahlwa
Ukushintsha kwemizwa nokucasuka
Kokubili kusuka emiphumeleni yomlamuleli nasekuphileni nesifo esingamahlalakhona, esingaziwa kahle
- Ukungena 'okufana nokukhathazeka' kufanele ukunakekelwa kuzo zombili izinkomba. Ukukhululwa komlamuli kungaveza i-physiology eqondile yokuhlasela kwe-panic, futhi iziguli zivame ukutholakala ngokungalungile ngaleyo sisekelo. Ngesikhathi esifanayo, ukukhathazeka nokucindezeleka kuhambisana ngempela nesifo esingelapheki futhi kufanele ukwelashwa ngokwako. Ukuqaphela eyodwa akudingi ukuxosha omunye.
- Kungani izimpawu zehluke kakhulu phakathi kweziguli
- Abantu ababili abanokuxilongwa okufanayo bangaveza ngendlela ehluke ngendlela ecishe ingabonakali. Kunezici ezimbalwa ezinomthelela, futhi eziningi zisaqondwa ngokungaphelele:
- Ukusatshalaliswa kwesifunda. Ukuminyana kwe-mast cell kanye ne-phenotype kuyahlukahluka phakathi kwezicubu naphakathi kwabantu ngabanye, ngakho-ke izinhlelo zezitho ezithinteke kakhulu ziyehluka.
- Ukukhishwa komlamuli ohlukile. Amaseli e-Mast angakhulula abalamuli abakhethiwe kunokuqukethwe kwabo okugcwele, futhi izingxube ezihlukene zokuxhumanisa ziveza izimpawu ezihlukene.
- Iphrofayili yesamukeli. Ama-receptors avezwa kuma-mast cell omuntu abumba okuyizisusa ezisebenza njengezibangeli kuwo.
Izimo ezikhona. Ukuxilongwa okugqagqene okufana ne-POTS noma i-hypermobility kushintsha sonke isithombe somtholampilo kakhulu.
- I-Threshold kanye nomthwalo okhulayo. Ukulala, ingcindezi, isimo sehomoni, kanye nokutheleleka kwakamuva konke ukusebenza kabusha kwesisekelo sokushintsha, ngakho-ke umuntu ofanayo uyahlukahluka ngokuhamba kwesikhathi.
- Izici zofuzo. I-alpha tryptasemia yofuzo nokunye ukuhlukahluka kofuzo kungase kuguqule isethulo - indawo yocwaningo olusebenzayo kunolwazi oluzinzile.
- Ibalazwe lomzimba wezimpawu: uhlaka lwe-anatomical olunama-callout anelebula endaweni ngayinye ethintekile - isikhumba, imigudu yomoya, inhliziyo, amathumbu, ubuchopho, amalunga, esinyeni - ngasinye siyanwebeka ukuze sibonise izimpawu ezihlobene nalolo hlelo. Ukuguqula kungagqamisa ukuthi yiziphi izinhlelo isiguli esithile esihlangabezana nazo.
- Izimpawu zesikhumba, ukuphefumula, inhliziyo, i-GI, i-neurological, musculoskeletal, genitourinary, kanye nezizinda zengqondo.
- Ukufakwa kwezinhlelo zezitho ezimbili noma ngaphezulu kuyingxenye yephethini yokuxilonga.
Isethulo siyahluka kakhulu phakathi kweziguli futhi ngokuhamba kwesikhathi phakathi kwesiguli esifanayo.
Iziqephu ezinjengokukhathazeka zingaqhutshwa ngumlamuleli; lokhu akufanele kusetshenziselwe ukususa lesi simo, noma ukulahla izidingo zangempela zempilo yengqondo.
Isahluko 05
Okuvamile Izicupha
Izibangeli azivamile ukusebenza zodwa. Amaseli e-Mast asabela ekuthwaleni okwandayo, yingakho ukudla okufanayo, igumbi, noma umsebenzi ungaba yingozi ngolunye usuku futhi ungabekezeleleki ngolunye usuku.
Ukwenza kusebenze okunqwabelanayo. Ukuchayeka okubekezelelwa ngakunye okuningana okufika ndawonye kungawela umkhawulo okungekho okungawela yedwa. Lona umbono owodwa osebenziseka kakhulu wokwenza umqondo wokusabela ngokusobala okungahleliwe.
Ukudla
Izimbangela zokudla ku-MCAS ngokuvamile zihlobene nokuqukethwe kwe-histamine noma ukuqondisa ukucasuka kwe-mast cell esikhundleni sokungavumelani nokudla kwakudala. I-histamine inqwabelana ekudleni njengoba iguga, ivubela, noma igcinwa, yingakho ubusha buvame ukuba nendaba ngaphezu kwesithako esithile - inhlanzi efanayo ingase ibekezelelwe ngosuku lokuthenga hhayi ezinsukwini ezintathu kamuva. Ushizi osekhulile, inyama eselashiwe negayiwe, ukudla okuvutshelwe, kanye nezinsalela kuvame ukuthinteka. Iqembu lesibili libonakala likhuthaza amaseli e-mast ukuthi akhiphe awawo i-histamine. Ukwehluka ngakunye kukhulu, futhi izinhlu ezishicilelwe ziphathwa kangcono njengezinto ezicatshangelwayo okufanele zihlolwe kunemithetho.
Ukushisa
Phakathi kwezibangeli zomzimba ezibikwa kakhulu. Amashawa ashisayo namabhavu, ama-sauna, isimo sezulu esishisayo, amagumbi afudumele, neziphuzo ezishisayo konke kuvame ukucashunwa. Ukushisa akudingi lutho ukuze kungene emzimbeni, okuyingxenye yokuthi kungani ukuhlolwa okujwayelekile kwe-allergy kungakwazi ukukubona.
Kuyabanda
Umoya obandayo, amanzi abandayo, iziphuzo ezibandayo, nendawo efrijini kubikwa ukuthi kuyimbangela kwezinye iziguli. Ngokuphawulekayo, ngokushesha ushintsho phakathi kwamazinga okushisa kuvame ukuchazwa njengokubi kakhulu kunanoma yiliphi izinga elibanjwayo eliqinile.
Ukuzivocavoca
Ukuzikhandla ngokomzimba kuyisisusa sangempela seziguli eziningi, futhi kunezindleko zangempela, njengoba umsebenzi uzuzisa impilo nesimo sengqondo. Ukuqina nezinga lokukhuphuka ngokuvamile kubaluleke ngaphezu kobude besikhathi esiphelele, yingakho izindlela ezihamba kancane, eziphansi kakhulu - kanye nomsebenzi osuselwe emanzini lapho ukuma kungabekezelelwa kahle - kuvame ukulawulwa kangcono.
Ukucindezeleka
Ukucindezeleka kuyisiqalo sokuphila, hhayi isifenqo. Amaseli e-Mast athwala ama-receptors e-hormone ekhulula i-corticotropin futhi ahlala eduze neziphetho zemizwa, anikeze umzila obhaliwe lapho isimiso sezinzwa sithonya ukusebenza kwawo. Leli phuzu lidinga ukugcizelelwa ngoba iziguli izimpawu zazo eziya ziba zimbi kakhulu ngenxa yokucindezeleka zivame ukutshelwa ukuthi isimo sazo singokwengqondo. Indlela ekhombisayo ye-neuroimmune ayiyona into efanayo nophawu olucatshangelwayo.
Amahomoni
Iziguli eziningi zibika amaphethini ezimpawu ze-cyclical, ngokuvamile ziba zimbi eduze nokuya esikhathini. I-Estrogen ithonya ukuziphatha kwe-mast cell, okungenye yesandla esihlongozwayo esilinganisweni esiphezulu sokuxilongwa kwabesifazane. Izinguquko ngesikhathi sokukhulelwa, emva kokubeletha, kanye ne-perimenopause nazo ziyachazwa, futhi ziyahlukahluka kakhulu phakathi kwabantu.
Amagciwane
Ukutheleleka kuphakamisa ngokuthembekile ukusebenza kabusha kwesisekelo ezigulini eziningi. Isifo esivamile esibangelwa amagciwane ngokuvamile sinweba i-trigger emisiwe amasonto ngemva kwalokho, ngakho ukuchayeka okwakubekezelelwe ngaphambili kuyavusa inkanuko phakathi nokululama. Ezinye iziguli ziqala ukuqala kwezimpawu zazo ekuguleni okuthelelanayo, nakuba ukusungula imbangela ezimweni ngazinye kunzima.
Ukuchayeka kwemvelo
Imithi
Amakilasi athile emithi aqashelwa ezincwadini zamangqamuzana amakhulu njengezici ezingase zibe izishoshovu, okuhlanganisa ama-opioid, ezinye izidakamizwa ezingezona ukuvuvukala, imidiya ye-radiocontrast, ezinye izinto ezivimbela i-neuromuscular ezisetshenziswa ekubulaleni izinzwa, kanye ne-vancomycin. Izithako ezingasebenzi - odayi, izivimbelakuvunda, izigcwalisi - nakho kungaba yisici esicasulayo, yingakho isiguli singase sibekezelele ukwakheka komkhiqizi oyedwa futhi sisabele komunye.
Okubalulekile
Ungayeki, ugweme, noma uguqule umuthi owunikeziwe ngesisekelo sohlu olujwayelekile. Letha uhlu kumtholampilo owazi umlando wakho. Ingozi yokuyeka ukwelashwa okudingekayo ingadlula ingozi egwenywayo, futhi lesi sinqumo sidinga ukuhlolwa komuntu ngamunye.
Amakha nephunga
Amakha, izinto zokuhlanza umoya, imikhiqizo yokuhlanza, imikhiqizo yokuwasha, izincibilikisi, upende omusha, nefenisha entsha kuphakathi kwezimbangela ezibikwa njalo, futhi phakathi kwezinto okunzima kakhulu ukuzigwema ezindaweni ezihlanganyelwe. Izindawo ezingenawo amakha ziyisicelo sokuhlala esijwayelekile ezikoleni nasezindaweni zokusebenza ngenxa yalesi sizathu.
Utshwala
Utshwala buvame ukubikwa njengesiqalo emzileni ongaphezu kowodwa: iziphuzo eziningi ezidakayo zine-histamine ngokuqondile, ikakhulukazi iwayini nobhiya; utshwala bungaphazamisa ama-enzyme aphula i-histamine; futhi kubangela i-vasodilation ngokuzimela. Ukusabela kumanani amancane kuvame ukuchazwa.
Ukuswela ubuthongo
Ukungalali kahle noma okunganele kwehlisa ukubekezelelana ngokubanzi futhi kuhlanganyele nazo zonke ezinye izinto kulolu hlu. Akuvamile ukuba kube ukuphela kwembangela yokuqubuka, kodwa kungenye yabanikelayo abathintekayo, futhi iziguli zivame ukubika ukuthi ukuvikela ukulala kuphakamisa umkhawulo wazo kukho konke okunye.
- Kungani ama-trigger ahluka kakhulu
- Amasethi e-trigger ayahluka phakathi kwabantu ngabanye ngenxa yokuthi amaphrofayili ama-receptor, ukusatshalaliswa kwezicubu, izimo ezikhona kanye, kanye nokusebenza kabusha kwesisekelo konke kuyahluka. Nabo bayashintsha ngaphakathi umuntu ngokuhamba kwesikhathi - kuvame ukwanda ngezikhathi zokulawula okungekuhle kanye nokuncipha futhi uma ukwelashwa kwehlisa ukusebenza kabusha kwesisekelo. Ukusabela kungase kubambezeleke ngamahora, okusitha isixhumanisi phakathi kwembangela nomphumela. Kuzo zonke lezi zizathu, ukukhomba izibangeli kudinga ukugcinwa kwerekhodi okuhlelekile emavikini amaningi kuneziphetho ezithathwe esehlakalweni esisodwa.
- I-Threshold infographic: ibha evundlile emele i-threshold yokusabela, enamabhulokhi anqwabelene ngaphansi kokushisa okulebulwe, ukungalali kahle, ingcindezi, ukudla okudala, nokuzikhandla. Bonisa isimo sokuqala lapho amabhulokhi amathathu ehlala ngezansi komugqa, kanye nesesibili lapho ibhulokhi yesine iphusha isitaki ngaphezu kwaso - izingxenye ezifanayo ezifanayo, umphumela ohlukile.
- Izitaki zokucupha — umthwalo oqongelelekayo ubaluleke ngaphezu kwanoma yikuphi ukuchayeka okukodwa.
- Izinto ezibangela umzimba (ukushisa, ukubanda, ingcindezi, ukuzikhandla) azidingi into engenisiwe futhi azibonakali ekuhlolweni kokungezwani komzimba.
Izibangeli zemithi kufanele kuxoxwe ngazo nodokotela, ungalokothi uzilawule yena ohlwini.
Amasethi e-trigger ayahluka phakathi kwabantu futhi agudluke ngokuhamba kwesikhathi, ngakho ukugcinwa kwerekhodi kubalulekile.
Isahluko 06
Kungani i-MCAS injalo
Kunzima uku Ukuxilonga
Ukubambezeleka kokuxilonga ku-MCAS akukhona ngokuyinhloko ukwehluleka kodokotela abangabodwana. Kulandela ezicini ezithile, ezikhonjwayo zesimo kanye nokuhlolwa okusetshenziselwa ukusiphenya.
Izimpawu eziguquguqukayo
I-MCAS ikhiqiza inhlanganisela yezimpawu ezihlukene ezigulini ezihlukene, nezinhlanganisela ezahlukene esigulini esifanayo ngokuhamba kwesikhathi. Ayikho iphrezentheshini eyodwa yokufanisa iphethini emelene nayo. Ngenxa yokuthi izimpawu aziqondile ngazinye, ngasinye singabalelwa esimeni esivame kakhulu uma sicatshangelwa sodwa.
Ukuhlolwa kwaselabhorethri okujwayelekile
Umsebenzi wegazi ojwayelekile ngokuvamile awuphawuleki ku-MCAS. Isibalo segazi eligcwele, izimpawu zokuvuvukala, kanye ne-biochemistry evamile kuvame ukujwayelekile, njengamaphaneli okungezwani komzimba nezinto ezithile. Ukuhlolwa komlamuli okukhethekile kuyadingeka, futhi nalokho kuvame ukubuyisela imiphumela evamile ngezizathu zobuchwepheshe ezivezwe ngezansi. Ngakho-ke isiguli singaqongelela ifayela elibanzi lophenyo oluvamile kuyilapho sihlala singaphilile ngempela - iphethini ngeshwa eyandisa amathuba okuba sitshelwe ukuthi akukho okungalungile.
Ukukhululwa komlamuli ngezikhathi ezithile
Lena inkinga yezobuchwepheshe emaphakathi. Abalamuli abalinganisiwe banesigamu sokuphila esifushane. I-serum tryptase ifinyelela isiqongo phakathi namahora ambalwa okusabela bese iyawa; ama-metabolites omchamo aphikelela isikhathi eside kodwa asabonisa iwindi elilinganiselwe. Ukusampula phakathi kweziqephu kungase kuthole lutho oluphakeme - hhayi ngoba ukuqalisa akuzange kwenzeke, kodwa ngoba akusalinganiseki.
Izimfuneko zokuphatha zihlanganisa lokhu. Abalamuli abaningana abazinzile ekamelweni lokushisa, futhi amasampula e-prostaglandin antekenteke kakhulu. Amasampuli ngokuvamile adinga ukugodola ngokushesha, ukuphatha okubandayo kulo lonke iqoqo, nokucutshungulwa ngokushesha. Isifanekiso esishiywe ekamelweni lokushisa noma esibambezelekile ekuhambeni singaveza umphumela ojwayelekile ovela esigulini ebesisabela ngokweqiniso.
Isabelo esikhulu semiphumela yomlamuli ongemuhle siyabonisa isikhathi nokuphatha ngaphandle kokungabikho kwezifo. Ukuqinisekisa iphrothokholi yokuqoqwa nodokotela o-odayo kanye nelabhorethri ngaphambi kokuthatha amasampula kubaluleke kakhulu kunokuphinda ukuhlolwa okungaphathwanga kahle.
Hlangana nezinye izifo
Izimpawu ze-MCAS zigqagqana kakhulu nesifo samathumbu esicasulayo, i-urticaria engapheli, isifuba somoya, i-fibromyalgia, isifo sokukhathala okungamahlalakhona, ukuphazamiseka kokukhathazeka, i-dysautonomia, nezinye ezimbalwa. Ngayinye yalezi zijwayele kakhulu odokotela abaningi futhi kuvame ukuxilonga. Lapho isiguli sibonisa izimpawu ezilingana ezimbalwa zalezi ngokwengxenye, umphumela ovamile uwuchungechunge lokuxilonga ingxenye kunokuba kube okuhlanganisayo.
Ukuntuleka kokuqaphela udokotela
I-MCAS yabonakala ngokusemthethweni kuphela ngo-2007, ngemibandela yokuvumelana eyalandela ngo-2010 nango-2012. Odokotela abaningi abasebenzayo baphothula ukuqeqeshwa kwabo ngaphambi kokuba isimo singene ezinhlelweni ezijwayelekile. Ukujwayelana kuyahluka kakhulu ngisho nakuma-allergist kanye nodokotela bamasosha omzimba, futhi ukufinyelela kochwepheshe abanolwazi lwe-mast cell akulingani ngokwendawo.
Ingxabano yokuxilonga
Kufanelekile ukusho ngokusobala ukuthi indlela yokuxilonga ye-MCAS ihlala iyisihloko senkulumompikiswano yangempela ngaphakathi kwezincwadi zezokwelapha. Ngokubanzi, isikhundla esisodwa sibambelele kumbandela oqinile odinga ubufakazi bomlamuli obunenhloso, ngezizathu zokuthi ngaphandle kwakho ubungozi bokuxilonga busetshenziswa kabanzi. Esinye isikhundla sithi imibandela eqinile igeja iziguli ezinesimo sangempela kodwa ukuhlolwa komlamuli wazo okuphindelelayo kwehluleka ngenxa yezizathu zobuchwepheshe ezichazwe ngenhla.
Lokhu akukona ukungaboni ngaso linye okwenziwe yiziguli, futhi akuxazululeki. Umphumela wayo ongokoqobo ukuthi ochwepheshe ababili abanekhono bangase bafinyelele iziphetho ezihlukene ngesiguli esifanayo. Ukuqonda ukuthi impikiswano ikhona kusiza ukuchaza imibono engqubuzanayo ngaphandle kokudinga ukucabanga ukuthi udokotela oyedwa wayenephutha.
- Ukubambezeleka kokuxilonga
- Inhlanganisela engenhla ikhiqiza ukubambezeleka okude. Iziguli ngokuvamile zihanjiswa ngochwepheshe abaningi - i-gastroenterology, i-dermatology, i-cardiology, i-allergies, i-neurology, i-psychiatry - ngayinye ihlola isifunda esisodwa senkinga yesistimu. Izibalo ezishicilelwe nezinhlolovo zesiguli ngokuvamile zichaza ukubambezeleka okukalwa ngeminyaka, futhi izilinganiso cishe phakathi neminyaka eyishumi zivame ukucashunwa, nakuba izibalo ezinjalo ziza nemikhawulo yangempela yendlela yokusebenza futhi kufanele zifundwe njengenkomba esikhundleni sokunemba.
- Umfanekiso womugqa wesikhathi wohambo lokuxilonga olumele: izimpawu zokuqala ngonyaka onguziro; ukuvakasha kwe-GP okokuqala; ukudluliselwa ku-gastroenterology nge-endoscopy evamile; i-dermatology enomphumela wokwelashwa kwezimpawu; i-cardiology ngokuqapha okujwayelekile kwe-Holter; ukudluliselwa kwengqondo; bese kuba yi-allergies/immunology kanye nokuhlolwa kwe-mast cell. I-nodi ngayinye echazwe ngesikhathi esidlulile kanye nomaka 'bemiphumela evamile', okwenza ukunqwabelana kokuhlola okunegethivu kubonakale njengephethini kunochungechunge lwezehlakalo ezingazodwa.
- Ukuhlolwa kwaselabhorethri okujwayelekile kuvame ukujwayelekile ku-MCAS; ukuhlolwa komlamuli okukhethekile kuyadingeka.
- Abalamuli baphila isikhashana futhi abazinzile, ngakho-ke isikhathi nokuphatha amasampula kushayela amagethivu amaningi angamanga.
Ukujwayela kodokotela kuyahlukahluka; isimo singene kwikharikhulamu muva nje.
Indlela yokuxilonga isalokhu kuphikiswana ngayo, ngakho imibono yochwepheshe ingahluka ngokusemthethweni.
Okuhlobene Izimo
I-MCAS ivame ukubikwa ngokuhambisana nezinye izimo ezimbalwa. Amandla obufakazi ahluka kakhulu phakathi kwalezi zinhlangano, kanye nezindaba zomehluko.
Funda lokhu kuqala
Ukuhlangana akuyona imbangela. Izimo ezimbalwa ezingezansi ziyenzeka kanye ne-MCAS kaningi kunalokho okungabikezelwa ithuba, kodwa noma ngabe zabelana ngendlela ethile, noma ngabe enye idala enye, noma izindlela zokudlulisela ezabiwe zikhulisa ukugqagqana okusobala akusungulwa kokuningi kwalokhu kumataniswa.
I-Postural Orthostatic Tachycardia Syndrome (POTS)
I-POTS iwuhlobo lwe-dysautonomia ebonakala ngokwenyuka kwezinga lokushaya kwenhliziyo lapho umile, ngokuvamile ngokuba nekhanda elilula nokukhathala. Ukwenzeka ngokubambisana ne-MCAS kuchazwe kahle ezincwadini. Izincazelo ezihlongozwayo zihlanganisa abalamuli be-mast cell abathinta ithoni ye-vascular kanye nokulawulwa kokuzimela, kodwa isiqondiso sanoma ibuphi ubuhlobo be-causal abusungulwa. Zombili lezi zimo zingaveza i-tachycardia, isiyezi, nokukhathala, okwenza kube nzima ukukhomba izimpawu kwesinye noma kwesinye.
I-Hypermobile Ehlers-Danlos Syndrome (hEDS) kanye nokuphazamiseka kwe-hypermobility spectrum
Ukwenzeka ngokubambisana kwe-hEDS, POTS, ne-MCAS kuxoxwa kaningi ngokwanele ukuze kuchazwe ngokungakahleleki njengokuthathu. Inhlangano ibikwa ngokungaguquki, kodwa indlela eyisisekelo isalokhu iyinkoleloze. Amaseli e-Mast ahlala kuzicubu ezixhumeneyo futhi abambe iqhaza ekwenziweni kabusha kwezicubu, okunikeza umugqa ozwakalayo wophenyo, kodwa ukuba nokwenzeka akubona ubufakazi. Amaphethini okudlulisela kanye nokuxilonga nawo angase abe nomthelela ekutheni ukugqagqana kubonakala kuqine kangakanani.
I-Hereditary alpha tryptasemia (HaT)
I-HaT iyisici sofuzo esibandakanya amakhophi engeziwe ofuzo lwe-alpha-tryptase I-TPSAB1, itholwa njengefa kuphethini ebusayo ye-autosomal, futhi ikhiqiza i-serum tryptase ye-serum eqhubekayo eqhubekayo. Kuvamile uma kuqhathaniswa nomphakathi. Ukuhlobana kwayo nezimpawu kusacaciswa: kuhlotshaniswa nokusabela okubucayi kwezinye izimo, kodwa abantu abaningi abanalesi sici abanazimpawu.
I-HaT ibalulekile ekutolikeni. Ngenxa yokuthi iphakamisa i-tryptase eyisisekelo, inani eliphakeme elilodwa le-tryptase eliphakanyisiwe kumuntu one-HaT alibonisi ngokwalo ukusebenza kwe-mast cell - okuqinisa ukuthi kungani umkhawulo wokuxilonga uchazwa ngokuthi ukuphakama ngaphezu kwesisekelo somuntu kunenombolo ephelele.
I-Irritable bowel syndrome (IBS)
Izimpawu zamathumbu ku-MCAS zidlulana cishe ngokuphelele nemibandela ye-IBS, futhi ezinye iziguli ziphethe womabili amalebula. Ucwaningo luhlole ukuminyana kwe-mast cell kanye nokusebenza ku-gut mucosa yeziguli ze-IBS ngokutholwa okuhlukile. Ukuthi isethi engaphansi ye-IBS imele ukubandakanyeka kwe-mast cell okungaziwa kuwumbuzo ovulekile esikhundleni seqiniso eliqinisekisiwe.
I-Migraine
I-Migraine ibikwa ngamanani aphakeme phakathi kweziguli ze-MCAS. I-Histamine iyisithako se-vasoactive esisunguliwe esinokubaluleka okubonakalayo ekhanda, futhi ama-mast cell akhona kuma-meninges. Isixhumanisi semishini siyabonakala ngokwebhayoloji futhi singaphansi kophenyo, kodwa asixazululiwe.
Isifuba somoya kanye ne-urticaria engapheli
Lawa anobudlelwane obucace kakhulu bemishini kumaseli amakhulu anoma yini kulesi sigaba. Amaseli e-Mast ayingxenye ye-pathophysiology yakho kokubili - i-leukotrienes ne-histamine drive bronchoconstriction kanye nokwakheka kwamasondo ngokulandelana. I-urticaria ezenzakalelayo ehlala njalo ikakhulukazi ihilela ukusebenza kwe-mast cell ngqo. Lezi akuzona izinhlangano eziqagelayo; ziyizimo lapho ukubandakanyeka kwe-mast cell kumiswa khona, nakuba zihlala ziyizifo ezihlukile ezivela ku-MCAS.
I-COVID ende
| Izimo ze-Autoimmune | Izimo ezahlukahlukene ze-autoimmune zibikwa eceleni kwe-MCAS, kufaka phakathi i-thyroid autoimmunity. Amangqamuzana e-Mast abamba iqhaza ekulawuleni amasosha omzimba futhi ahlanganyele nezimpendulo zokuzivikela eziguquguqukayo, okuhlinzeka ngesisekelo esizwakalayo sokuphazamiseka okwabiwe, kodwa izindlela ezithile ezixhumanisa i-MCAS nezifo ezithile zokuzivikela komzimba azikasungulwa. | Amandla obufakazi ngokubuka nje |
|---|---|---|
| Isimo | Ubunjalo bobudlelwano | Isimo sobufakazi |
| I-urticaria engapheli | Ukusebenza kwe-Mast cell kuwumgogodla wesifo ngokwaso | Isimo sobufakazi |
| Indlela esunguliwe | Isifuba somoya | Ama-mast cell mediators ashayela i-bronchoconstriction |
| I-Hereditary alpha tryptasemia | Isici sofuzo esikhulisa i-tryptase yokuqala; kuthinta incazelo yokuhlolwa | Isici esimisiwe; ubudlelwano bezimpawu busacaciswa |
| AMAPHOSTHI | Okuvame ukwenzeka ngokubambisana; imiphumela yomlamuli kuthoni yemithambo ehlongozwayo | Inhlangano ibikiwe; indlela engaxazululiwe |
| I-HaT ibalulekile ekutolikeni. Ngenxa yokuthi iphakamisa i-tryptase eyisisekelo, inani eliphakeme elilodwa le-tryptase eliphakanyisiwe kumuntu one-HaT alibonisi ngokwalo ukusebenza kwe-mast cell - okuqinisa ukuthi kungani umkhawulo wokuxilonga uchazwa ngokuthi ukuphakama ngaphezu kwesisekelo somuntu kunenombolo ephelele. | I-hEDS / hypermobility | Okuvame ukwenzeka ngokubambisana; ukubandakanyeka kwezicubu ezixhumeneyo kuhlongozwayo |
| Inhlangano ibikiwe; indlela yokucabanga | Amazinga aphezulu abikiwe; abaxhumanisi be-vasoactive abazwakalayo | Inhlangano ibikiwe; indlela esacwaningwayo |
| I-IBS | Ukugqagqana kwezimpawu; ukutholakala kwe-gut mast cell okuguquguqukayo | Ukugqagqana kucacile; ubudlelwano abuzange buxazululwe |
| I-Migraine ibikwa ngamanani aphakeme phakathi kweziguli ze-MCAS. I-Histamine iyisithako se-vasoactive esisunguliwe esinokubaluleka okubonakalayo ekhanda, futhi ama-mast cell akhona kuma-meninges. Isixhumanisi semishini siyabonakala ngokwebhayoloji futhi singaphansi kophenyo, kodwa asixazululiwe. | Isifo se-Autoimmune | Kubikiwe ukuhlangana; ukwabiwa kwe-immune dysregulation okuhlongozwayo |
- Inhlangano ibikiwe; indlela ayikasungulwa
- Ukugqagqana kwezimpawu; ukubandakanyeka kwe-mast cell kucatshangelwe
- Okwandulelayo - ngaphansi kophenyo olusebenzayo
- Ukubandakanyeka kwe-mast cell kusungulwa ku-urticaria engapheli kanye nesifuba somoya - lezi akuzona izixhumanisi zokuqagela.
- I-HaT iwuphawu lwangempela lofuzo oluphakamisa i-tryptase eyisisekelo futhi lushintshe indlela imiphumela yokuhlolwa okufanele ifundwe ngayo.
Uxhumo olude lwe-COVID lungokokuqala futhi akufanele lwethulwe njengoba lumisiwe.
Kuyo yonke le ndawo, ukuhlobana kuye kwenziwa iphutha ngokuphindaphindiwe njengembangela - funda izimangalo ngokucophelela.
Isahluko 08
Ukuqonda Kwamanje
kwe Izimbangela
Isifinyezo esiqotho ukuthi imbangela ye-MCAS ayaziwa. Okulandelayo kwehlukanisa lokho okusunguliwe kulokho okusasele kuyi-hypothesis.
Ayikho imbangela eyodwa ye-MCAS esunguliwe. Amafektha angezansi yizindawo zophenyo ezinamazinga ahlukene okusekela. Noma yimuphi umthombo owethula i-akhawunti ethembekile, ehlanganisiwe ye-MCAS okwamanje uyabudlula ubufakazi.
I-Genetics
Yini esunguliwe: I-alpha tryptasemia yofuzo iyisici sofuzo sangempela, esinezici ezinhle ezibandakanya amakhophi engeziwe I-TPSAB1, futhi iphakamisa i-tryptase eyisisekelo. Ngokwehlukana, i KIT Ukuguqulwa kwe-D816V kusungulwa njengomshayeli kuningi labantu abadala be-systemic mastocytosis - isimo esihlukile, kodwa esibonisa ukuthi ukuguqulwa okukodwa kungaphazamisa kanjani i-mast cell biology.
Ukucindezeleka kuyisiqalo sokuphila, hhayi isifenqo. Amaseli e-Mast athwala ama-receptors e-hormone ekhulula i-corticotropin futhi ahlala eduze neziphetho zemizwa, anikeze umzila obhaliwe lapho isimiso sezinzwa sithonya ukusebenza kwawo. Leli phuzu lidinga ukugcizelelwa ngoba iziguli izimpawu zazo eziya ziba zimbi kakhulu ngenxa yokucindezeleka zivame ukutshelwa ukuthi isimo sazo singokwengqondo. Indlela ekhombisayo ye-neuroimmune ayiyona into efanayo nophawu olucatshangelwayo.
Yini engekho: Akukho ukuguqulwa kwe-causative okufanayo okuhlonziwe ku-MCAS. Ukuhlangana komndeni kuyabikwa, futhi ezinye iziguli zichaza izihlobo eziningi ezithintekile, kodwa iphethini yefa echaziwe ye-MCAS ngokwayo ayikasungulwa. Ukuthi i-MCAS imele isimo esisodwa esinesisekelo sofuzo esingakatholakali, noma izimo ezimbalwa ezihlukene eziqoqwe ngaphansi kwelebula yomtholampilo eyodwa, akuxazululwa.
Ukungasebenzi kahle kwamasosha omzimba
I-Broad immune dysregulation iyindawo ehamba phambili yophenyo. Izindlela ezihlongozwayo zifaka phakathi ukuguquguquka kokuvezwa kwesamukeli noma ukuzwela kuma-mast cell, ukusayinda okuphazamisayo okulawula okungavamise ukuvimba ukuqalisa, nokusebenzisana okungavamile phakathi kwamaseli e-mast nezinye izakhamuzi zokuzivikela ezifweni. Lokhu kunengqondo futhi kufundwe ngenkuthalo, kodwa kuchaza isigaba sencazelo kunendlela ebonisiwe.
Iziguli eziningi zibonisa izimpawu zokuqala zesifo esithathelwanayo. Ukutheleleka kwaziwa ngokwenza amaseli e-mast asebenze ngama-receptors afana ne-Toll, ahlinzeka ngomzila ozwakalayo, futhi ukuqala kwangemva kokutheleleka kuyiphethini eyaziwayo ezimeni ezimbalwa ezingelapheki. Nokho, ukusungula ukuthi ukutheleleka kubangelwa I-MCAS esimweni ngasinye inzima kakhulu ngokwendlela, futhi ukukhumbula ukuchema kuthinta ama-akhawunti abheka emuva. Lokhu kuseyinhlangano esacwaningwayo.
Izici zemvelo
Abahlanganyeli bezemvelo abahlongozwayo bahlanganisa ukuchayeka kwamakhemikhali, isikhunta, ukungcoliswa komoya, kanye nezinguquko zokudla ezingeni labantu. Ubufakazi lapha bubuthakathaka kakhulu kunobezindawo ezingenhla, futhi obuningi babo abuqondile. Lena indawo ezwakalayo yophenyo esikhundleni sokuba negalelo elisunguliwe, futhi kuyindawo lapho izimangalo zezohwebo ezizethembayo zivamile futhi zisekelwa kabi.
Ukuvuvukala okungapheli
Amaseli e-Mast womabili asabela futhi anomthelela ekuvuvukeni, okuphakamisa amathuba okuba khona kwemijikelezo yokuziqinisa lapho ukwenza kusebenze kuzala indawo evuvukalayo eyehlisa umkhawulo wokuqhubeka kusebenze. Lokhu kuhambisana ngokomshini futhi kuhambisana nokubonwa komtholampilo ukuthi ukuphinda kusebenze kuyakhula phakathi nokulawula okungeyikho. Ihlala iyimodeli esikhundleni sesizathu esibonisiwe.
I-Epigenetics
Ukuguqulwa kwe-epigenetic - izinguquko ekukhulumeni kofuzo ngaphandle koshintsho ekulandelaneni okungaphansi - kuhlongozwe njengendlela lapho ukuchayeka kwemvelo kungaveza izinguquko ezihlala njalo ekuziphatheni kwe-mast cell. Lena i-hypothesis yesigaba sokuqala. Kunengqondo ngokwebhayoloji futhi kufanele kuphenywe, futhi okwamanje kunobufakazi obuqondile obulinganiselwe obuqondile be-MCAS.
- Lapho lokhu kushiya izinto
- Kungenzeka ukuthi i-MCAS njengoba ichazwa njengamanje ihlanganisa inqubo engaphezu kweyodwa eyisisekelo, nokuthi amalebula omtholampilo ahlanganisa ndawonye iziguli ezizogcina zihlukaniswe ngendlela. Ucwaningo luyaqhubeka kuzo zonke izindawo ezingenhla. Ezigulini, okushiwo okungokoqobo ukuthi abaphathi baqondise kubaxazululi kanye nezibangeli esikhundleni sembangela ewumsuka - nokuthi ukungabaza kuyafaneleka kunoma yimuphi umkhiqizo noma umthetho olandelwayo othi ulungisa umsuka umphakathi wocwaningo ongakawuphawuli.
- Umdwebo wesigaba sobufakazi: amabhande agxilile ahambela ngaphandle asuka 'Kumisiwe' (isici se-HaT; i-KIT D816V ku-mastocytosis) ngokusebenzisa 'Ukuphenywa okusebenzayo ngobufakazi obusekelayo' (ukungasebenzi kahle kwamasosha omzimba, ukuqala kwangemva kokutheleleka) ukuya 'ku-Hypothesised, ubufakazi obuqondile obulinganiselwe' (i-epigenetics, ukuchayeka okubonakalayo kwezemvelo.
- Ayikho imbangela ye-MCAS esunguliwe.
- I-HaT kanye nokuguqulwa kwe-KIT D816V ku-mastocytosis kuwufuzo olumisiwe - futhi akuyona imbangela eqinisekisiwe ye-MCAS.
Iminikelo yemvelo kanye ne-epigenetic iyimibono enobufakazi obuqondile obulinganiselwe.
Phatha izimangalo 'eziwumsuka' ngokuzethemba ngokungabaza - umphakathi wocwaningo awukazihlonzi esisodwa.
Isahluko 09
Ukuhlala Na I-MCAS
Ukuphatha kwakhiwe kakhulu ngokusebenza kwansuku zonke kunanoma yikuphi ukungenelela okukodwa. Okulandelayo kuqondiswe ekusebenzeni - inhloso impilo ebanzi kunazo zonke ezosekelwa yi-physiology yakho.
Ukuphathwa kwansuku zonke kanye nezinqubo
Iziguli eziningi zithola ukuthi ukungaguquguquki kuphakamisa umkhawulo wazo ngokuthembekile kunanoma yisiphi isilinganiso somuntu ngamunye. Ukulala okuvamile nezikhathi zokuvuka, isikhathi sokudla esibikezelwayo, izinga lokushisa lendawo elizinzile lapho kufinyeleleka khona, kanye nesikhathi semithi esingaguquki konke kunciphisa ukuhlukahluka. Imijikelezo iphinde yenze ukuhlonza i-trigger kwenzeke: uma okuhlukile okuningi kuzinzile, lezo ezishintshile ziyabonakala.
I-Pacing itimu ephindaphindayo kuma-akhawunti esiguli — ukusabalalisa umsebenzi usuku lonke noma iviki kunokugxilisa kuwo, nokwakha ngesikhathi sokuthola kabusha ngemva kwezidingo ezaziwayo. Lokhu akufani nokungasebenzi, futhi ukungasebenzi isikhathi eside kunezindleko zakho ezibhalwe kahle.
Ukulandelela izimpawu nokuthola izimbangela
Irekhodi elihlelekile liwumzila othembeke kakhulu wokuhlonza izibangeli zomuntu siqu. Okufakiwe okuwusizo kudlula kakhulu ukudla: isikhathi sosuku, izinga lokushisa lendawo, okudliwe nokuthi bekukusha kangakanani, ukuvivinya umzimba, ingcindezi nokulala, ukuchayeka kwephunga, imithi ephuziwe, kanye - neziguli ezisesikhathini - ukuma komjikelezo. Izimpawu kufanele zirekhodwe ngesikhathi kanye nokuqina.
Amasonto amabili cishe abuncane ngaphambi kokuba amaphethini afundeke, futhi amade angcono. Ngenxa yokuthi ukusabela kungase kubambezeleke ngamahora, ukuchayeka okubalulekile kungase kuhlale kahle ngaphambi kwalesi sibonakaliso. Bheka impinda kuzo zonke izehlakalo kunezincazelo zemicimbi eyodwa - umgomo uwumbono ongawuhlola, hhayi isinqumo.
Idayari yezimpawu ingenye yezinto eziwusizo kakhulu isiguli esingaziletha ku-aphoyintimenti: ukhonkolo, i-longitudinal, futhi ethize kubo ngendlela okungekho hlu lwezicupho ezishicilelwe olungaba khona. Kuyasiza futhi ukuhlukanisa izibangeli zangempela kokuzenzekela, okuyinto inkumbulo iyodwa eyenza kabi.
Ukusebenza nodokotela
Ukwethula izimpawu ezihleliwe ngohlelo lwesitho kunokuba ngokulandelana kwezikhathi kwenza iphethini yesistimu eningi ibonakale ngokushesha, lapho i-akhawunti elandisayo ivame ukuyifihla. Ukuletha imiphumela yangaphambili - okuhlanganisa nejwayelekile - kufundisa ngempela futhi kugwema ukuphinda uphenyo oluqediwe.
Ukubuza ngokuqondile mayelana nesikhathi sokuhlola umlamuli kanye nokuphathwa kwesampula kunengqondo futhi kuthuthukisa ngokubonakalayo ithuba lomphumela osebenzisekayo. Uma ungezwakali, ukufuna umbono wesibili kusemthethweni; isifo se-mast cell siyindawo ye-niche futhi ukujwayela kuyahluka kakhulu. Ukuxoshwa kanye nje kuvamile futhi akusona isinqumo.
Isikole
Izindawo zokuhlala ezivamile zihlanganisa izinqubomgomo zekilasi ezingenawo amakha, imvume yokuthwala nokuzinikeza imithi, ukufinyelela emanzini nekhefu lokuphumula, ukucabangela izinga lokushisa lapho uhlezi, ukuguquguquka lapho ufika ngesikhathi sokuvutha, kanye nokulungiswa kwemfundo yokuzivocavoca. Lapho kunezinhlelo zokulala ezisemthethweni ohlelweni lwakho lwezemfundo, ukubhala ukuthi ukutholakala kwezifo kubhalwe phansi ngokuvamile kwenza ukufinyelela kuzo kube lula kakhulu.
Sebenza
Ukucatshangelwa kwendawo yokusebenza kufana nalezo zesikole: izinqubomgomo zephunga, ukulawula izinga lokushisa, ukuhlela okuguquguqukayo noma okukude ngesikhathi sokuvutha, nokulungiswa kweshejuli lapho ukukhathala kulandela iphethini ebikezelwayo. Ukuthi noma kungakanani okumele kudalulwe kuyisinqumo somuntu siqu esinokuhwebelana kwangempela, futhi sisebenzisana nokuvikelwa kokuqashwa okuhluke kakhulu phakathi kwezindawo ezithile.
Ukuhamba
Izinyathelo ezisebenzayo ezivame ukushiwo iziguli zihlanganisa ukuphatha imithi emithwalweni yesandla enemibhalo, ukuthola incwadi kadokotela yemithi ejovwayo, ukucwaninga izikhungo zezokwelapha lapho uya khona, ukuhlela ukudla kusenesikhathi lapho ukusabela kubalulekile, kanye nokuvumela isikhathi sokululama lapho ufika. Ukushintsha kwezinga lokushisa nokuphakama kufanele kulindelwe.
Impilo yengqondo
Ukuphila nesifo esishintshashintshayo, esingaqashelwanga kahle, futhi esivame ukuxoshwa kunezindleko zangempela zengqondo, futhi lokho kubiza akukona ubuthakathaka bomlingisi. Iziguli eziningi zichaza isikhathi eside sokungakholwa ngaphambi kokuxilongwa, okushiya uphawu lwazo. Ukusekela - kungakhathaliseki ukuthi uchwepheshe, ontanga, noma kokubili - kuyingxenye enengqondo yokuphatha esikhundleni sokuvuma ukuthi izimpawu zingokwengqondo.
- Umehluko okufanele ubanjwe yilokhu: ukukhululwa komlamuli kungaveza isayensi yengqondo efana nokukhathazeka, futhi lokho kufanele ukuqashelwa esikhundleni sokuphinda kubhalwe kabusha ngokwengqondo kuso sonke isimo. Ngesikhathi esifanayo, ukukhathazeka nokucindezeleka kujwayelekile, kuyalapheka, futhi kufanelekile ukubhekana nakho ngokwako uma kukhona.
- Ukulungela izimo eziphuthumayo
- Ezinye iziguli ezinesifo se-mast cell zisengozini ye-anaphylaxis - ukusabela okusheshayo, okunzima, okungase kube yingozi empilweni. Lapho lokho kuba sengozini, ukuhlela akuyona into yokuzikhethela, futhi kuyingxoxo ongaba nayo nodokotela ngaphambi kokuba kudingeke.
- Iziguli ezibhekwa njengezisengozini ngokuvamile zinikezwa ama-epinephrine auto-injection futhi zelulekwa ukuthi ziwathwale njalo.
- I-epinephrine iwumugqa wokuqala wokwelashwa kwe-anaphylaxis. Ama-Antihistamine awathathi indawo futhi akufanele kuthenjelwe kuwo ekuphenduleni okunzima kakhulu.
- Uhlelo olubhaliwe lwesenzo sezimo eziphuthumayo okuvunyelenwe ngalo nodokotela wakho kufanele luveze izimpawu eziyisixwayiso, lokho okumelwe ukwenze, nokuthi kufanele ushayele nini abezimo eziphuthumayo.
Iziguli ezibhekwa njengezisengozini ngokuvamile zinikezwa ama-epinephrine auto-injection futhi ziyalelwa ukuthi ziwathwale njalo. I-epinephrine iwumugqa wokuqala wokwelapha we-anaphylaxis; ama-antihistamine awathathi indawo yawo futhi akufanele kuthenjelwe kuwo ekuphenduleni okunzima kakhulu.
Amakhophi anikezwe umndeni, ozakwabo, noma abasebenzi besikole asho ukuthi abanye bangakwazi ukwenza okuthile uma ungakwazi.
Umazisi wezokwelapha oqaphela ukuxilongwa kanye nezimbangela zemithi ezaziwayo zisiza abasebenzi bezimo eziphuthumayo okungenzeka abasazi lesi simo.
Ukuhlinzwa nokubulala izinzwa kuqinisekisa ukuxoxisana kusengaphambili ukuze ama-agent akwazi ukukhethwa kucatshangwa ngesimo.
- Inothi lokuvinjelwa kokudla
- Ukudla okune-histamine ephansi nokuqeda kuzanywa kabanzi, futhi ubufakazi obusekelayo bunomkhawulo futhi busezingeni eliphansi. Ukuvinjelwa okunwetshiwe okungagadiwe kunobungozi bangempela bokuntuleka kokudla okunomsoco kanye nokudla okungenamsoco, futhi leyo ngozi iphezulu entsheni. Uhlolo oluhlelekile, olunomkhawulo wesikhathi nokwethulwa kabusha okuhlelekile - ngokufanelekile ngokubandakanyeka kwabadlayo - kuyindlela ephephile.
- Kuyafaneleka ukuqamba igama lesicupho ngokuqondile: kungenzeka ukunciphisa izimbangela ngobudlova kangangokuthi ukuphila kuvumelana cishe lutho. Uhlelo oluqeda ukusabela ngokuqeda umsebenzi, isikole, izinhlobonhlobo zokudla, nokuxhumana nabantu luye lwahweba uhlobo oluthile lokulimaza lwaba olunye.
- Izinqubo ezingaguquguquki ziphakamisa umkhawulo futhi zenze ukuhlonzwa kwembangeli kwenzeke.
- Idayari yezimpawu ehleliwe emavikini amaningi iyindlela ethembeke kakhulu yokuhlonza izibangeli zomuntu siqu.
- Ukwethula izimpawu ngohlelo lwesitho kwenza iphethini ye-multisystem ibonakale kodokotela.
Lapho ubungozi be-anaphylaxis busebenza, i-epinephrine kanye nohlelo lwesenzo olubhaliwe kubalulekile - ama-antihistamine awayona into ebambele.
Ukuvinjelwa kokudla okunolaka kunezingozi zangempela futhi kufanele kuhlelwe, kunqunyelwe isikhathi, futhi kugadwe.
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Ingabe i-MCAS iwufuzo?
Ingabe izingane zingathuthukisa i-MCAS?
Ingabe i-MCAS inganyamalala iyodwa?
Kungani izimpawu zami zilokhu zishintsha?
Kungani imiphumela yami yokuhlolwa ihlale ijwayelekile?
Ingabe i-stress ingabangela ama-flares?
Ingabe ukudla kungayelapha i-MCAS?
Uyini umehluko phakathi kwe-MCAS ne-mastocytosis?
Ingabe i-MCAS iyafana nokungabekezelelani kwe-histamine?
Ngabe ngidinga ukuhlolwa ukuthi ngi-positive ukuze ngitholwe?
Hlobo luni lukadokotela oluphatha i-MCAS?
Ingabe ama-antihistamine azokulungisa konke?
Kungani ngisabela emithini okufanele isize?
Ingabe i-MCAS iwukukhubazeka?
Ingabe i-MCAS ingabanga i-anaphylaxis?
Ingabe ukukhulelwa kuyayithinta i-MCAS?
Kungani ukushisa kungenza ngisabele lapho ngingezwani nalutho?
Kuthatha isikhathi esingakanani ukuthi ukwelashwa kusebenze?
Ingabe kufanele ngishintshe imithi eminingana ngesikhathi esisodwa ukuze ngibe ngcono ngokushesha?
Ingabe i-COVID-19 noma ezinye izifo zingavusa i-MCAS?
Ingabe i-MCAS ivame kakhulu kwabesifazane?
Ivamise kangakanani i-MCAS?
Ingabe kufanele ngigweme konke ukudla okusohlwini lwe-histamine ephezulu?
Yini okufanele ngiyilethe ekuqokweni kwami kokuqala kochwepheshe?
Umlando wezimpawu ohlelwa yisistimu yesitho kunokulandelana kwezikhathi, okwenza iphethini yesistimu eningi ibonakale ngokushesha. Idayari yezimpawu kanye ne-trigger ehlanganisa okungenani amasonto amabili. Yonke imiphumela yokuhlolwa yangaphambilini, kuhlanganise nevamile, njengoba lena iyingxenye yenqubo yokukhishwa. Uhlu lwamanje lwemithi kanye nesengezo. Futhi uhlu olufushane olubhaliwe lwemibuzo yakho - ama-aphoyintimenti mafushane futhi kulula ukuhamba ngaphandle kokubuza ukuthi yini ebaluleke kakhulu.
Isigaba 11
Ukufunda Okwengeziwe Izinsiza
- Amaphuzu okuqala akhethiwe ukuze afundwe ngokujulile. Izixhumanisi ezithile ziyahlanganiswa futhi zizokwengezwa lapha.
- Abaqondisi Besiguli
- Izingeniso ezifinyelelekayo ezibhalelwe iziguli nemindeni, ezilungele ukwabelana nabantu abasha kulesi simo.
- Umhlahlandlela wesiguli oyisingeniso - isixhumanisi esizongezwa
Iphakethe le-orientation elisanda kutholwa - isixhumanisi esizongezwa
Uphawu oluphrintekayo kanye nesifanekiso sedayari - isixhumanisi esizongezwa
- Umhlahlandlela wamalungu omndeni nabanakekeli — isixhumanisi esizongezwa
- Iziqondiso Zokwelashwa
- Izitatimende zokuvumelana neziqondiso zomtholampilo ezihloselwe ochwepheshe bezokunakekelwa kwempilo. Kuyasiza ukuletha kuma-aphoyintimenti.
- I-Consensus diagnostic criteria - ingcaphuno nesixhumanisi okufanele kwengezwe
Isiqondiso somtholampilo somphakathi ochwepheshe - isixhumanisi esizongezwa
Ukuhlolwa komlamuli kanye nesampula yokuphatha iphrothokholi - isixhumanisi esizongezwa
- Ukucatshangelwa kwe-perioperative kanye ne-anesthesia - isixhumanisi esizongezwa
- Amaphepha Ocwaningo
- Izincwadi eziyisisekelo. Lapho kungenzeka khona, ncamela imithombo ebuyekezwe ngontanga kanye nezinsuku zokushicilelwa kwenothi, njengoba le nkambu ihamba.
- Amaphepha okulinganisa okuyisisekelo (2007 kuya phambili) - izingcaphuno ezizokwengezwa
Amaphepha wemibandela yokuvumelana (2010, 2012) - izingcaphuno ezizokwengezwa
Ucwaningo lwe-alpha tryptasemia - izingcaphuno ezizokwengezwa
- Ukubuyekezwa kwezimo ezihambisanayo - izingcaphuno ezizokwengezwa
- Izinhlangano Zochwepheshe
- Imiphakathi yochwepheshe kanye nezinhlangano zezemfundo ezisebenza ngesifo se-mast cell.
Imiphakathi kazwelonke neyamazwe ngamazwe yokungezwani komzimba nezifo/i-immunology — izixhumanisi ezizongezwa
Amanethiwekhi ocwaningo lwesifo se-Mast cell - izixhumanisi ezizongezwa
- Izinhlangano zezambulela zezifo ezingavamile - izixhumanisi ezizongezwa
- Izincwadi
Ukufundwa kwefomu elide kweziguli nabelaphi.
Izihloko ezigxile esigulini - ezongezwa
- Imibhalo eyinkomba yomtholampilo — ezongezwa
- Amavidiyo Okufundisa
- Izinkulumo ezirekhodiwe, izichazi, nezinto zenkomfa.
Amavidiyo esichazamazwi esingeniso - izixhumanisi ezizongezwa
Izinkulumo zenkomfa ezikhethekile - izixhumanisi ezizongezwa
- Imininingwane yokuqukethwe kwezemfundo kwe-MCAS - izixhumanisi ezizongezwa
- Izinhlangano Ezisekelayo
- Ukwesekwa kontanga, ukumela, nomphakathi.
Izinhlangano ezimela iziguli - izixhumanisi ezizongezwa
Imiphakathi yokusekela ontanga - izixhumanisi ezizongezwa
- Amaqembu asekelayo ezifunda kanye nakazwelonke - izixhumanisi ezizongezwa
- Izilingo Zomtholampilo
- Izifundo eziqashwayo njengamanje. Ukutholakala kwesilingo kushintsha njalo, ngakho hlola uhlu lokubhalisa ngokuqondile.
Njengamanje kuqashwa ama-mast cell studies - izixhumanisi ezizongezwa
Isiqondiso mayelana nokuthi ukubamba iqhaza kuhilelani - isixhumanisi esizongezwa
- Ukhiye Okuthathwayo
- Imiqondo ebaluleke kakhulu evela kulo mhlahlandlela, ifingqiwe.
- Ngamafuphi
- I-MCAS ukuphazamiseka kwe-mast cell engafanele ukwenza kusebenze, hhayi izinombolo ze-mast cell - amaseli ajwayelekile, umkhawulo wabo awukho.
- Amaseli e-Mast angamaseli omzimba awusizo ngokusemthethweni abekwe emigoqweni yomzimba, abambe abalamuli enziwe ngaphambilini balungele ukukhululwa phakathi nemizuzwana.
- Izimpawu ziyi-episodic, ziyaphindaphindeka, futhi zibandakanya amasistimu wezitho ezimbili noma ngaphezulu - lelo phethini, hhayi noma yiluphi uphawu olulodwa, yilo eliphakamisa ukuxilongwa.
- Abalamuli bakhululwa ngamagagasi: bakheke ngaphambili ngemizuzwana, abalamuli be-lipid ngamaminithi, ama-cytokines ngaphezu kwamahora. Umcimbi owodwa wokwenza kusebenze ungadonsa usuku.
- I-Histamine ingumlamuleli oyedwa kuphela phakathi kwabaningi, yingakho ama-antihistamine ngokuvamile enikeza impumuzo ngokwengxenye esikhundleni sokukhululeka okuphelele.
- Icupha isitaki. Umthwalo oqoqiwe uchaza ukusabela okubukeka kungahleliwe, futhi kuwumbono owodwa owusizo kakhulu wokuphatha kwansuku zonke.
- Izinto ezibangela umzimba - ukushisa, amakhaza, ingcindezi, ukuzikhandla - azidingi into engenisiwe futhi azibonakali ekuhlolweni okujwayelekile kokungezwani komzimba.
- Amaphaneli okungezwani kahle komzimba nomsebenzi ojwayelekile ojwayelekile wegazi kulindeleke ku-MCAS futhi awakukhiphi.
- Ukuhlolwa okuningi komlamuli okungalungile kubonisa izinkinga zesikhathi kanye nesampula yokuphatha esikhundleni sokungabikho kwesifo.
- Indlela yokuxilonga isalokhu kuphikiswana ngayo, ngakho ochwepheshe abanekhono bangafinyelela iziphetho ezihlukene ngesiguli esifanayo.
- Ukubandakanyeka kwe-Mast cell emisiwe ku-urticaria engapheli kanye ne-asthma; izinhlangano ne-POTS, i-hEDS, i-IBS kanye ne-Long COVID zibikwa kodwa azixazululwanga.
- Ayikho imbangela ye-MCAS esunguliwe - phatha 'izimbangela eziyinhloko' ngokuzethemba ngokungabaza.
Lapho ubungozi be-anaphylaxis busebenza, i-epinephrine kanye nohlelo lwesenzo olubhaliwe kubalulekile; ama-antihistamine awangeni esikhundleni.
Umgomo impilo ebanzi kunazo zonke ezosekelwa yi-physiology yakho - hhayi impilo encane egwema zonke izimpawu.
Ukuvuselela Iseli Yezinsimbi