Ndi chiyani MCAS?
Mast Cell Activation Syndrome ndi vuto la chitetezo chamthupi khalidwe osati chitetezo kuchuluka. Maselo amapezeka mwachibadwa ndipo amawoneka bwino - zomwe zasintha ndi momwe amawotcha mosavuta.
Mast Cell Activation Syndrome (MCAS) ndi mkhalidwe womwe ma mast cell - maselo oteteza thupi omwe amakhala pafupi ndi minofu iliyonse ya thupi - amagwira ntchito mosayenera komanso mobwerezabwereza, kutulutsa oyimira pakati pamankhwala mochulukira ndipo nthawi zina osagwira ntchito zodzitetezera. Chotsatira chake ndi chitsanzo cha zizindikiro zobwerezabwereza, zowonongeka zomwe zimakhudza machitidwe a ziwalo ziwiri kapena zingapo nthawi imodzi.
Kutanthauzira kuli kofunika chifukwa kumasiyanitsa MCAS ndi mikhalidwe yomwe imafanana kwambiri. Palibe ma cell ochuluka omwe amapezeka pa biopsy, palibe chotulukapo chimodzi chodziwikiratu poyezetsa khungu, ndipo nthawi zambiri palibe vuto lililonse pazantchito zanthawi zonse za labotale. Matendawa amakhala pamalo pomwe maselo owoneka bwino amasankha kuyankha.
Ku MCAS vuto siliri zingati mast cell omwe muli nawo. Zili choncho amawombera mosavuta. Zina zonse za chikhalidwecho - kusayembekezereka, kufalikira kwa machitidwe ambiri, kuyesa kovuta - kumatsatira mfundo imodzi.
Chifukwa chiyani ma cell cell alipo
Lingaliro Lofunika
Amayikidwa molingana - amakhazikika pamalire, pakhungu, panjira ya mpweya, khoma lamatumbo, ndi minofu yozungulira mitsempha yamagazi ndi mitsempha. Ndipo amamangidwira liwiro: m'malo mopanga mayankho awo pazomwe akufuna, amazisunga kale m'ma granules osungira, okonzeka kumasula mkati mwa masekondi a chizindikiro. Zomangamangazi ndizomwe zimapangitsa kuti ziwengo zizikhala mwachangu, komanso ndizomwe zimapangitsa kuyambitsa kosayenera kukhala kovuta kukhala nako.
Chifukwa chiyani ma cell cell alipo
Ma cell cell si vuto la kapangidwe kake. Ndizigawo zakale kwambiri za chitetezo chamthupi chobadwa nacho, zomwe zimapezeka mwanjira ina pakati pa zamoyo zambiri zamsana, ndipo zimagwira ntchito yomwe thupi limafunikira. Amathandizira kuyankha koyamba kwa tizilombo toyambitsa matenda, amathandizira kuletsa poizoni pambuyo polumidwa ndi kulumidwa, amatenga nawo mbali pakuchiritsa mabala ndi kukonzanso minofu, amathandizira kuwongolera kufalikira kwa mitsempha yamagazi, ndikuthandizira kusunga zotchinga zomwe zimalekanitsa thupi ndi dziko lakunja.
Amayikidwa molingana - amakhazikika pamalire, pakhungu, panjira ya mpweya, khoma lamatumbo, ndi minofu yozungulira mitsempha yamagazi ndi mitsempha. Ndipo amamangidwira liwiro: m'malo mopanga mayankho awo pazomwe akufuna, amazisunga kale m'ma granules osungira, okonzeka kumasula mkati mwa masekondi a chizindikiro. Zomangamangazi ndizomwe zimapangitsa kuti ziwengo zizikhala mwachangu, komanso ndizomwe zimapangitsa kuyambitsa kosayenera kukhala kovuta kukhala nako.
Momwe magwiridwe antchito a mast cell amawonekera
Poyankha bwino chitetezo chamthupi, cell ya mast imakumana ndi chiwopsezo chenicheni - antijeni ya parasite, poizoni, kapena kuwonongeka kwa minofu - ndikuwonongeka molingana ndi izo. Mitsempha ya magazi imatambasuka ndipo imakhala yowonjezereka, zomwe zimapangitsa kuti plasma ndi maselo a chitetezo azitha kulowa mu minofu. Mathero a mitsempha amalimbikitsidwa, kutulutsa kuyabwa kapena kupweteka komwe kumapangitsa chidwi pamalowo. Maselo ena oteteza thupi ku matenda amatengedwa. Chiwopsezocho chikachotsedwa, yankho limatha.
Chilichonse chosasangalatsa panjirayi chimagwira ntchito. Kutupa kumapereka zida zoteteza thupi kutsamba. Kuyabwa kumakupangitsani kuchotsa chilichonse chomwe chikuvulaza. Amachotsa misampha ndikuchotsa zokhumudwitsa. Poyankha molingana, kusapezako kumakhala kwakanthawi ndipo phindu lake ndi lenileni.
Mu MCAS, makina omwewo amagwira ntchito popanda chifukwa chofanana. Maselo a mast amawotcha moto zokha, kapena poyankha zolimbikitsa zomwe ziyenera kukhala zosadabwitsa - chipinda chofunda, fungo lonunkhira mukhonde, chakudya, kuyimirira, kuchita masewera olimbitsa thupi pang'ono, tsiku lotopetsa wamba. Oyimira pakati omwe amamasulidwa ndi omwewo omwe amagwiritsidwa ntchito pachitetezo chovomerezeka, koma amamasulidwa molakwika ndipo nthawi zambiri mobwerezabwereza.
Chifukwa ma cell cell amakhala mu minofu m'thupi lonse, komanso chifukwa omwe amawayimira amakhala pamitsempha yamagazi, minofu yosalala, minyewa, ndi ma cell ena oteteza chitetezo, gawo limodzi limatha kutulutsa kuthamanga kwamtima, kuthamanga kwamtima, kupweteka m'mimba, kupuma movutikira, komanso chifunga cha chidziwitso nthawi imodzi. Kwa dokotala yemwe sakuganiza za mast cell, kuphatikiza kumeneku kumatha kuwoneka ngati mavuto angapo osagwirizana omwe amabwera nthawi imodzi - chomwe ndi gawo lalikulu la chifukwa chomwe matendawa amaphonya.
Fanizo losavuta
| MCAS si mastocytosis | Uku ndiye kusiyana kofunikira kwambiri m'munda, ndipo kusokoneza ziwirizi kumabweretsa chisokonezo pa chilichonse chakumunsi. | |
|---|---|---|
| Mastocytosis poyerekeza ndi MCAS | Mastocytosis | MCAS |
| Kusakhazikika kwapakatikati | Ma cell ochuluka kwambiri amawunjikana mu minofu | Nambala yachibadwa ya mast cell, kuyambitsa mosayenera |
| Chiwerengero cha ma cell | Okwezeka - clonal kuchulukana | Wamba |
| Mtundu woyamba wa tryptase | KIT Kusintha kwa D816V m'milandu yambiri yamachitidwe akuluakulu | Nthawi zambiri zimayambira |
| Kupeza kwachibadwa | Kuphatikizika kwa mast cell kumawoneka bwino | Palibe kusintha komwe kunakhazikitsidwa |
| Zotsatira za Biopsy | Kuphatikizika kwa mast cell kumawoneka bwino | Palibe khalidwe kudzikundikira |
| Chizindikiro cha khungu | Zilonda za maculopapular (urticaria pigmentosa) zofala m'njira zina | No enieni matenda khungu chotupa |
Momwe zimatsimikizidwira
Umboni wotsimikizika wa kuchuluka kwa ma cell
Matenda amtundu wa classical allergies ndi machitidwe a IgE-mediated ku chinthu china chodziwika bwino. Imapangidwanso - mawonekedwe omwewo amatulutsa zomwezo - ndipo amatha kuwonetsedwa ndi kuyezetsa khungu kapena kuyezetsa magazi kwa IgE. Kusamvana kwa mtedza kumachita motere; chomwechonso mphaka dander ziwengo.
Zonsezi zimatha kubweretsa zizindikiro zodutsana, chifukwa nthawi zonse mast cell mediators amafika minofu mopitilira muyeso. Koma mastocytosis akhoza kuwonetsedwa mwa kupeza maselo; MCAS siyingathe, chifukwa chake kuzindikirika kwake kumakhazikika pazotsatira zingapo m'malo mwa mayeso amodzi otsimikizira.
Odwala ambiri amafika ku MCAS ndendende chifukwa kuyezetsa ziwengo kunabweranso koyera pomwe zizindikiro zawo sizinali. Gulu loyipa la ziwengo si umboni wotsutsa kukhudzidwa kwa mast cell; mu chikhalidwe ichi chayandikira kuyembekezera.
Zochita za MCAS nthawi zambiri sizitero. Zambiri sizikhala za IgE konse, zomwe zimatuluka m'malo mwake kudzera pama receptor ena pamtunda wa cell kapena kukondoweza mwachindunji. Zoyambitsa nthawi zambiri zimakhala zakuthupi - kutentha, kuzizira, kupanikizika, kulimbitsa thupi - osati mapuloteni, ndipo palibe gulu lodziletsa lomwe limapangidwa kuti lizindikire izi. Zochita zimatha kuchedwetsedwa ndi maola ambiri, ndikuchotsa kulumikizana kwachilengedwe pakati pa kuwonekera ndi zotsatira zake, ndipo malire amatha kusintha ndi tulo, kupsinjika, momwe thupi limakhalira, kapena matenda aposachedwa, kotero kuwonetseredwa komwe kumaloledwa sabata imodzi kumadzetsa chidwi chotsatira.
Zithunzi zofananitsa mbali ndi mbali: mapanelo atatu owonetsa kuchuluka kwa mast cell akuyankha molingana, gulu la mastocytosis lowonetsa ma cell ochulukirapo, ndi gulu la MCAS lowonetsa manambala a cell omwe ali ndi kutulutsidwa kwa mkhalapakati mokokomeza. Maselo omwewo amawerengera m'magulu amodzi ndi atatu, zotulutsa zosiyana kwambiri.
- MCAS ndi vuto la mast cell osayenera kuyambitsa, osati nambala ya mast cell.
- Zithunzi zofananitsa mbali ndi mbali: mapanelo atatu owonetsa kuchuluka kwa mast cell akuyankha molingana, gulu la mastocytosis lowonetsa ma cell ochulukirapo, ndi gulu la MCAS lowonetsa manambala a cell omwe ali ndi kutulutsidwa kwa mkhalapakati mokokomeza. Maselo omwewo amawerengera m'magulu amodzi ndi atatu, zotulutsa zosiyana kwambiri.
- Chidule cha gawo
- Mastocytosis imaphatikizapo kuchuluka kwa maselo ndipo imatha kuwonetsedwa mwachindunji; MCAS sangathe, ndichifukwa chake matenda ali ndi gulu.
- Maselo a mast ndi maselo oteteza chitetezo omwe ali m'malo otchinga thupi ndipo amapangidwa kuti ayankhe mkati mwa masekondi.
Kumvetsetsa Maselo a Mast
Kuyesedwa koyipa kwa ziwengo ndikofala mu MCAS ndipo sikumaletsa.
Chiyambi ndi chitukuko
Maselo a mast amayambira m'mafupa, kuchokera ku CD34-positive haematopoietic progenitor cells - chiwerengero chomwecho chomwe chimapangitsa maselo ena a magazi. Mosiyana ndi maselo oyera ambiri a magazi, iwo samalizitsa kukula kwawo kumeneko. Makolo osakhwima amalowa m'magazi, amazungulira pang'ono, ndiyeno amasamukira ku minofu, kumene kukhwima komaliza kumachitika.
Kukhwima kumeneko kumadalira kwambiri kuwonetsa kwa stem cell factor (SCF) kudzera pa cholandirira cha KIT pa cell cell. Njira iyi ndiyofunikira pa biology ya mast cell, ndipo ndi njira yomweyi yomwe imasokonekera KIT D816V masinthidwe opezeka ambiri akuluakulu a systemic mastocytosis. Chilengedwe cham'deralo chimapanga phenotype yomaliza, ndichifukwa chake ma cell a m'matumbo amasiyana kwambiri ndi omwe ali pakhungu.
Chiyambi ndi chitukuko
Kugawa thupi lonse
Maselo a mast amagawidwa mwanzeru osati mofanana, kumangoyang'ana pomwe thupi limakumana ndi dziko lakunja komanso kuzungulira zida zomwe zimanyamula zizindikiro ndi magazi. Kugawa uku kumalongosola mawonekedwe a multisystem a MCAS mwachindunji kuposa china chilichonse.
| Kugawa thupi lonse | Maselo a mast amagawidwa mwanzeru osati mofanana, kumangoyang'ana pomwe thupi limakumana ndi dziko lakunja komanso kuzungulira zida zomwe zimanyamula zizindikiro ndi magazi. Kugawa uku kumalongosola mawonekedwe a multisystem a MCAS mwachindunji kuposa china chilichonse. | Kumene ma mast cell amakhala, ndi zomwe zimapanga akawotcha |
|---|---|---|
| Malo | Udindo mu thanzi | Zizindikiro pamene kutsegula sikuli koyenera |
| Khungu ndi dermis | Kuteteza chitetezo; kuyankha kulumidwa, mbola, ndi kuvulala | Kutupa, kuyabwa, ming'oma, dermographism, kutupa |
| M'mimba thirakiti | Kusamalira zotchinga, kuwongolera motility, kuyankha ku ziwopsezo zomwe zalowetsedwa | Kutsekula m'mimba, nseru, reflux, kutsegula m'mimba, kudzimbidwa, kudzimbidwa |
| Airways ndi sinuses | Chitetezo cha m'mitsempha yamagazi | Kuchulukana, rhinitis, kutsekeka kwa mmero, kupuma, chifuwa |
| Kuzungulira mitsempha ya magazi | Kuwongolera kamvekedwe ka chotengera ndi permeability | Kuthamanga kwa magazi, tachycardia, kuthamanga kwa magazi, presyncope |
| Ubongo ndi meninges | kupezeka kwa perivascularly ndi meninges; chizindikiro cha neuroimmune | Chifunga chachidziwitso, mutu, chizungulire (njira zomwe zidakali zophunzira) |
| Connective minofu | Kukonzanso ndi kukonza minofu | Kupweteka kwapakati ndi minofu; zotheka kugwirizana ndi kulumikizana kwa minofu |
Mafupa a mafupa
Malo oyambira makolo
Mast cell amakhala ndi moyo wautali modabwitsa. Kumene neutrophil ikhoza kukhala ndi moyo kwa maola angapo mpaka masiku, maselo a minofu amatha kukhalapo kwa milungu kapena miyezi, ndipo umboni umasonyeza kuti ena amakhala ndi moyo nthawi yaitali. Amakhalanso okhoza kubwezeretsanso - pambuyo powonongeka, selo la mast likhoza kumanganso masitolo ake ndikuyankhanso, osati kufa panthawiyi.
Zindikirani momwe ubongo umalowera mosamala. Ma cell cell amapezekadi mu meninges ndi m'mitsempha yapakati mkati mwa minyewa yapakati, ndipo kulumikizana kwa mast cell-nerve ndi gawo lochita kafukufuku. Komabe, njira zolumikizira kuyambika kwa mast cell kuzizindikiro za minyewa ndi zidziwitso zikugwiridwabe, ndipo zonena zamakanika mderali zikuposa umboni womwe ulipo.
Kuzungulira kwa moyo
Mast cell amakhala ndi moyo wautali modabwitsa. Kumene neutrophil ikhoza kukhala ndi moyo kwa maola angapo mpaka masiku, maselo a minofu amatha kukhalapo kwa milungu kapena miyezi, ndipo umboni umasonyeza kuti ena amakhala ndi moyo nthawi yaitali. Amakhalanso okhoza kubwezeretsanso - pambuyo powonongeka, selo la mast likhoza kumanganso masitolo ake ndikuyankhanso, osati kufa panthawiyi.
- FcERI - cholandirira chachikulu cha IgE. Iyi ndiye njira yodziwika bwino: Ma antibodies a IgE amamanga cholandilira, ndikulumikizana ndi ma allergen omwe amayambitsa kuyambitsa.
- MRGPRX2 - receptor mediating IgE-yodziyimira pawokha kuyambitsa ndi mankhwala ena ndi ma peptides. Mawonekedwe ake adathandizira kufotokozera zomwe zimachitika popanda kukhudzidwa kwa IgE.
- KIT - cholandilira cha stem cell factor; pakati pa kupulumuka kwa mast cell, kusasitsa, ndi kukhalapo kwa minofu.
- Othandizira zolandilira (C3a, C5a) - kulola kutsegulidwa kudzera mu mkono wothandizira wa chitetezo chachibadwa.
- Ma receptor olipira - kuzindikira machitidwe okhudzana ndi tizilombo toyambitsa matenda, kugwirizanitsa maselo a mast ku mayankho a matenda.
- KIT - cholandilira cha stem cell factor; pakati pa kupulumuka kwa mast cell, kusasitsa, ndi kukhalapo kwa minofu.
- Adenosine, opioid, estrogen ndi zolandilira ena - Kuthandizira pamitundu yosiyanasiyana komanso yoyambitsa ma profaili omwe amawonedwa pochita.
Kukhalapo kwa mayendedwe angapo omwe si a IgE ndichifukwa chake kuyezetsa ziwengo nthawi zambiri kumabwereranso ku MCAS. Mayeserowa amapangidwa kuti azindikire kukhudzidwa kwa IgE-mediated - njira imodzi mwa zingapo zomwe zimatha kuyimitsa cell ya mast.
Chithunzi chojambulidwa cha cell mast: cell membrane yodzaza ndi zolandilira zotchedwa (FcεRI, MRGPRX2, KIT, C3a/C5a, TLRs, CRH-R), mkati modzaza ndi ma granules, ndikudula komwe kumawonetsa zamkati mwa granule. Chipinda chothandizira chomwe chikuwonetsa selo isanayambe komanso itatha degranulation.
- Ma cell cell amachokera ku CD34+ progenitors m'mafupa koma okhwima mu minofu, kotero kuchuluka kwa magazi sikumawawonetsa.
- Kukhalapo kwa mayendedwe angapo omwe si a IgE ndichifukwa chake kuyezetsa ziwengo nthawi zambiri kumabwereranso ku MCAS. Mayeserowa amapangidwa kuti azindikire kukhudzidwa kwa IgE-mediated - njira imodzi mwa zingapo zomwe zimatha kuyimitsa cell ya mast.
- Amayang'ana pamalo otchinga komanso mozungulira mitsempha yamagazi ndi minyewa, ndichifukwa chake MCAS ili ndi ma multisystem.
- Iwo amakhala ndi moyo wautali ndipo akhoza regranulate, kotero kasamalidwe kokhazikika m'malo mwa nthawi yochepa.
- Kuzindikiritsa kwa KIT/SCF ndikofunikira pakukula kwawo - njira yomwe imasokonekera mu mastocytosis ambiri akuluakulu.
Zomwe Zimachitika Panthawiyi
Kuyambitsa Mast Cell
Mitundu yambiri yolandirira kupitilira IgE imatha kuwayambitsa, ndichifukwa chake mapanelo osagwirizana nawo nthawi zambiri amaphonya zoyambitsa za MCAS.
Kutsegula ndikutsatizana, ndipo sitepe iliyonse ili ndi dzina. Kutsatira kumapangitsa kuti mitundu yodabwitsa ya MCAS ikhale yosavuta kutanthauzira.
Chokondoweza chimafika m'selo - allergen, mankhwala, kutentha, kupanikizika, mphamvu, mahomoni opanikizika, matenda, kapena palibe chochititsa chidwi.
Zolandilira zam'mwamba zimayamba kugwira ntchito ndipo ma intracellular signing cascades amayamba. Kuchuluka kwa calcium ndi gawo lofunikira poyambira.
Ma granules osungira amalumikizana ndi nembanemba ya cell ndikutulutsa zomwe zidapangidwa kale mu minofu yozungulira - mkati mwa masekondi.
Othandizira opangidwa kale amamasulidwa nthawi yomweyo; oyimira kumene opangidwa kumene monga ma prostaglandins ndi leukotrienes amatsatira mkati mwa mphindi zochepa, ndipo ma cytokines kwa maola ambiri.
Mbali ziwiri za ndondomekoyi zikufotokozera zambiri zomwe odwala amakumana nazo. Choyamba, gawo laposachedwa limatengera malo ogulitsira omwe adapangidwa kale, chifukwa chake zomwe zimachitika zimatha masekondi pang'ono. Chachiwiri, amkhalapakati am'magawo am'mbuyo amapangidwa pambuyo poyambitsa kuyambitsa, chifukwa chake zizindikiro zina zimafika bwino pambuyo poyambitsa komanso chifukwa chake zomwe zimachitika zimatha kuchedwa kutha kwa mafunde achiwiri pambuyo pake.
Mast cell amamasula oyimira pakati mafunde, osati zonse nthawi imodzi. Oyimira opangidwa kale amatuluka mumasekondi; oyimira lipid amamangidwa mumphindi; ma cytokines amawunjikana pakapita maola. Chochitika choyambitsa chimodzi chikhoza kubweretsa zizindikiro tsiku lonse.
Oyimira pakati amagwira ntchito pamitsempha yamagazi, minofu yosalala, minyewa, ndi ma cell ena oteteza chitetezo, kutulutsa zotsatira pazigawo zingapo zamagulu.
Mkhalapakati wamkulu ndi zomwe aliyense amachita
Ma cell cell amatha kutulutsa zinthu zambirimbiri. Zotsatirazi ndizo zomwe zimakambidwa kwambiri zachipatala, ndipo palimodzi zimawerengera zambiri za chizindikiro chodziwika bwino.
| Mkhalapakati wamkulu ndi zomwe aliyense amachita | Ma cell cell amatha kutulutsa zinthu zambirimbiri. Zotsatirazi ndizo zomwe zimakambidwa kwambiri zachipatala, ndipo palimodzi zimawerengera zambiri za chizindikiro chodziwika bwino. | Oyimira ma mast cell |
|---|---|---|
| Histamine | Mtundu | Mkhalapakati wodziwika kwambiri. Imakulitsa mitsempha yamagazi ndikuwonjezera kupenya kwawo, imathandizira malekezero a mitsempha, ndikuwonjezera kutulutsa kwa asidi m'mimba. Amatulutsa kutentha thupi, kuyabwa, ming'oma, kuthamanga kwa magazi, tachycardia, kupweteka mutu, ndi GI cramping. Zimagwira ntchito kudzera mumagulu angapo olandirira - H1 pakhungu ndi mpweya, H2 m'matumbo ndi pamtima - chifukwa chake kutsekereza magulu onse awiri kumagwira ntchito bwino kuposa kutsekereza. |
| Histamine | Mtundu | Zopangidwiratu |
| Prostaglandin D2 | Zatsopano apanga | Mapuloteni ochuluka kwambiri mu mast cell granules komanso cholembera chalabu chokhazikitsidwa. Zimathandizira kukonzanso minofu ndi kutupa, ndipo zimatha kukulitsa mayankho amtundu wa mast cell. Phindu lake lodziwikiratu limachokera ku kukhala mwachindunji kwa mast cell. |
| Leukotrienes (LTC4, LTD4, LTE4) | Zatsopano apanga | Ma bronchoconstrictors amphamvu, ochulukirapo kuposa histamine polemera. Kuchulukitsa kufalikira kwa mitsempha ndi kupanga ntchofu, ndikuthandizira kuzizindikiro zam'mimba komanso zam'mimba. LTE4 imayezedwa mumkodzo. |
| Cytokines (IL-6, TNF-α, IL-1, IL-4 ndi ena) | Zatsopano apanga | Kuwonetsa ma protein omwe amayendetsa kutupa kwakukulu. Pang'onopang'ono pakuyamba ndi kuchitapo kanthu motalika kuposa omwe ali pamwambawa, ndipo amaganiziridwa kuti amathandizira kutopa, kufooka, komanso machitidwe amtundu wa 'chimfine' omwe amafotokozera panthawi komanso pambuyo poyaka moto. |
| Chemokines | Zatsopano apanga | Pezani ma cell owonjezera oteteza chitetezo pamalopo, ndikukulitsa ndikutalikitsa kuyankha kwa kutupa kupitilira momwe ma cell cell amayambira. |
| Platelet activating factor (PAF) | Zatsopano apanga | Mkhalapakati wamphamvu kwambiri wa lipid yemwe amawonjezera kufalikira kwa mitsempha ndipo angayambitse hypotension yayikulu. Kuphatikizidwa mu kuopsa kwa anaphylactic zimachitikira osati oletsedwa ndi antihistamines. |
| Heparin | Mtundu | Anticoagulant yochitika mwachilengedwe. Zitha kupangitsa kuti odwala ena azikhala ndi mikwingwirima yachilendo kapena kukhetsa magazi, komanso kutenga nawo gawo pamalamulo oletsa kutupa. |
| Chymase & carboxypeptidase A3 | Mtundu | Mapuloteni a granule omwe amakhudzidwa ndi kukonzanso minyewa, njira zowongolera kuthamanga kwa magazi, komanso kuwonongeka kwa ma venom ndi ma peptide ena. |
Anticoagulant yochitika mwachilengedwe. Zitha kupangitsa kuti odwala ena azikhala ndi mikwingwirima yachilendo kapena kukhetsa magazi, komanso kutenga nawo gawo pamalamulo oletsa kutupa.
Tchati chotuluka kuchokera kumanzere kupita kumanja: Yambitsani → Kuyambitsa → Kuchepetsa → Kutulutsa kwa mkhalapakati → Zizindikiro, zokhala ndi nthawi yoyambira yomwe ili pansi pakuwonetsa oyimira omwe adapangidwa kale pamphindikati, oyimira lipid pamphindi, ndi ma cytokines pa maola, nthambi iliyonse imadziwika ndi zizindikiro zomwe imayendetsa.
- Mapuloteni a granule omwe amakhudzidwa ndi kukonzanso minyewa, njira zowongolera kuthamanga kwa magazi, komanso kuwonongeka kwa ma venom ndi ma peptide ena.
- Othandizira opangidwa kale amamasulidwa mumasekondi; lipid mkhalapakati mu mphindi; cytokines kwa maola angapo.
- Maselo a mast amatha kumasula oyimira pakati mosankha popanda kuwononga kwathunthu.
- Kutsegula kumatsatira motsatana: choyambitsa, kuyambitsa, degranulation, kumasulidwa kwa mkhalapakati, zizindikiro.
- Othandizira opangidwa kale amamasulidwa mumasekondi; lipid mkhalapakati mu mphindi; cytokines kwa maola angapo.
Zizindikiro za MCAS
PAF ndi prostaglandins zimatha kuyambitsa zovuta zamtima zomwe ma antihistamines sathana nazo.
Matebulo ali m'munsiwa amafotokoza za zizindikiro zomwe zimafotokozedwa motsatira dongosolo la thupi. Machenjezo awiri asanawawerenge. Choyamba, palibe wodwala amene ali ndi zonsezi, ndipo mndandanda wautali suyenera kuwerengedwa ngati mndandanda wogwirizana nawo. Chachiwiri, chizindikiro chilichonse pano chimapezeka muzinthu zina zambiri; zomwe zikusonyeza kuti MCAS ndi chitsanzo - episodic, recurrent, multisystem, nthawi zambiri ndi zoyambitsa zozindikirika - m'malo molowa munthu aliyense.
| MCAS imatanthauzidwa pang'ono ndi kukula kwake. Chifukwa maselo a mast amakhala pafupifupi minofu iliyonse, zizindikiro zimatha kuwoneka paliponse - ndipo kufunikira kwa ziwalo ziwiri kapena zingapo ndizofunikira pakuzindikira. | Zolemba |
|---|---|
| Khungu ndi mucous membrane | Chizindikiro |
| Zolemba | Kupukuta |
| Nthawi zambiri mwadzidzidzi, zimakhudza nkhope, khosi, ndi chifuwa; chinthu chomwe chimanenedwa pafupipafupi | Kuyabwa (pruritus) |
| Zitha kuchitika kapena popanda zidzolo zowoneka | Matenda a urticaria (urticaria) |
| Zokwera, zotsekemera zoyabwa; akhoza kukhala osakhalitsa komanso osamukasamuka | Dermographism |
| Ma welts adakwezedwa ndi kusisita kolimba pakhungu - chizindikiro chodziwika bwino | Angioedema |
| MCAS imatanthauzidwa pang'ono ndi kukula kwake. Chifukwa maselo a mast amakhala pafupifupi minofu iliyonse, zizindikiro zimatha kuwoneka paliponse - ndipo kufunikira kwa ziwalo ziwiri kapena zingapo ndizofunikira pakuzindikira. | Zolemba |
|---|---|
| Kuchedwa kuchira kwa chilonda | Adanenedwa ndi odwala ena; njira zomwe sizinakhazikitsidwe kwathunthu |
| Wopuma | Kuchulukana kwa mphuno ndi rhinitis |
| Nthawi zambiri matenda ndi sanali nyengo | Kuthina kwapakhosi |
| Imafunika kuunika mwachangu ngati ikupita patsogolo - ikhoza kuwonetsa anaphylaxis | Kupuma ndi kupuma movutikira |
| Leukotriene-driven bronchoconstriction ndi njira yodziwika | chifuwa chosatha |
| MCAS imatanthauzidwa pang'ono ndi kukula kwake. Chifukwa maselo a mast amakhala pafupifupi minofu iliyonse, zizindikiro zimatha kuwoneka paliponse - ndipo kufunikira kwa ziwalo ziwiri kapena zingapo ndizofunikira pakuzindikira. | Zolemba |
|---|---|
| Kumva kununkhira ndi mankhwala opangidwa ndi mpweya | Kuphatikizika kofala kwambiri kwa zizindikiro zoyambitsa |
| Zamtima | Tachycardia |
| Kuthamanga mtima, nthawi zambiri popanda khama | Kusakhazikika kwa magazi |
| Itha kusuntha mbali iliyonse panthawi yagawo | Presyncope ndi syncope |
| Mutu wopepuka kapena kukomoka, nthawi zambiri utayima | Kusapeza bwino pachifuwa |
| MCAS imatanthauzidwa pang'ono ndi kukula kwake. Chifukwa maselo a mast amakhala pafupifupi minofu iliyonse, zizindikiro zimatha kuwoneka paliponse - ndipo kufunikira kwa ziwalo ziwiri kapena zingapo ndizofunikira pakuzindikira. | Zolemba |
|---|---|
| Anaphylaxis | Zowopsa, zachangu, zowopseza moyo - onani gawo lazadzidzidzi |
| M'mimba | Kupweteka kwa m'mimba ndi kupweteka |
| Zina mwa zizindikiro zomwe zimanenedwa kawirikawiri | Mseru ndi kusanza |
| Itha kutsatira chakudya kapena kuchitika palokha | Zotsatira za histamine H2 pamimba ya asidi ndizodziwika bwino |
| Motility imatha kusiyanasiyana pakati pa magawo ndi pakapita nthawi | Nthawi zambiri amayendetsa zoletsa zakudya - onani machenjezo mu gawo 9 |
| MCAS imatanthauzidwa pang'ono ndi kukula kwake. Chifukwa maselo a mast amakhala pafupifupi minofu iliyonse, zizindikiro zimatha kuwoneka paliponse - ndipo kufunikira kwa ziwalo ziwiri kapena zingapo ndizofunikira pakuzindikira. | Zolemba |
|---|---|
| Kutupa ndi reactivity chakudya | Kuvuta kukhazikika, kukumbukira, ndi kupeza mawu |
| Neurological ndi chidziwitso | Chifunga chanzeru |
| Kuvuta kukhazikika, kukumbukira, ndi kupeza mawu | Mutu ndi migraine |
| Migraine imanenedwa kuti ndi yokwera kwambiri pakati pa anthuwa | Chizungulire |
| Nthawi zambiri amadutsana ndi mtima ndi autonomic mbali | Kutopa |
| Zowopsa kwambiri komanso mwazinthu zolemala zomwe zimanenedwa | Sensor sensitivity |
| MCAS imatanthauzidwa pang'ono ndi kukula kwake. Chifukwa maselo a mast amakhala pafupifupi minofu iliyonse, zizindikiro zimatha kuwoneka paliponse - ndipo kufunikira kwa ziwalo ziwiri kapena zingapo ndizofunikira pakuzindikira. | Zolemba |
|---|---|
| Zomverera za mitsempha yosagwira bwino | Zitha kuphatikizika ndi mikhalidwe yolumikizirana yomwe takambirana mu gawo 7 |
| Minofu ndi mafupa, genitourinary, ndi maganizo | Kupweteka kwamagulu ndi minofu |
| Kufulumira kwa chikhodzodzo komanso pafupipafupi | Kupweteka kwa mafupa |
| Kuwunika kovomerezeka, makamaka komwe mastocytosis ndiyolingaliridwa | Kufulumira kwa chikhodzodzo komanso pafupipafupi |
| Zizindikiro za interstitial-cystitis zimafotokozedwa | Zizindikiro zimasiyana malinga ndi msambo |
| Mphamvu ya mahomoni pama cell a mast imalembedwa | Nkhani ngati nkhawa |
Kulowa 'ngati nkhawa' kumayenera kusamalidwa mbali zonse ziwiri. Kutulutsidwa kwa mkhalapakati kungapangitse thupi lenileni la mantha, ndipo odwala nthawi zambiri amawadziwa molakwika chifukwa chake. Panthawi imodzimodziyo, nkhawa ndi kuvutika maganizo zimachitikadi limodzi ndi matenda aakulu ndipo amayenera kulandira chithandizo paokha. Kuzindikira chimodzi sikutanthauza kuthamangitsa wina.
Kusintha kwamalingaliro ndi kukwiya
Zonse kuchokera ku zotsatira za mkhalapakati komanso kukhala ndi matenda aakulu, osadziwika bwino
- Kugawa kwachigawo. Kachulukidwe ka maselo a mast ndi phenotype amasiyana pakati pa minyewa komanso pakati pa anthu, kotero kuti ziwalo zomwe zimakhudzidwa kwambiri zimasiyana.
- Kutulutsidwa kwa mkhalapakati kosiyana. Maselo a mast amatha kumasula oyimira osankhidwa m'malo mwa zonse zomwe zili mkati mwake, ndipo mitundu yosiyanasiyana ya mkhalapakati imatulutsa zizindikiro zosiyanasiyana.
- Mbiri ya cholandilira. Ma receptor omwe amawonetsedwa pama cell amunthu amapangidwa ndi zinthu zomwe zimawayambitsa.
- Kugawa kwachigawo. Kachulukidwe ka maselo a mast ndi phenotype amasiyana pakati pa minyewa komanso pakati pa anthu, kotero kuti ziwalo zomwe zimakhudzidwa kwambiri zimasiyana.
- Kutulutsidwa kwa mkhalapakati kosiyana. Maselo a mast amatha kumasula oyimira osankhidwa m'malo mwa zonse zomwe zili mkati mwake, ndipo mitundu yosiyanasiyana ya mkhalapakati imatulutsa zizindikiro zosiyanasiyana.
- Mbiri ya cholandilira. Ma receptor omwe amawonetsedwa pama cell amunthu amapangidwa ndi zinthu zomwe zimawayambitsa.
Mapu a thupi lachizindikiro: chidule cha ma anatomical okhala ndi zilembo zolembedwa kudera lililonse lomwe lakhudzidwa - khungu, mpweya, mtima, matumbo, ubongo, mafupa, chikhodzodzo - chilichonse chimakulitsidwa kuti chiwonetsere zomwe zikugwirizana ndi dongosololo. Kusintha kumatha kuwonetsa machitidwe omwe wodwala wapatsidwa akukumana nawo.
- Zizindikiro zimayambira pakhungu, kupuma, mtima, GI, minyewa, minofu ndi mafupa, madera a genitourinary, ndi malingaliro.
- Kuphatikizidwa kwa machitidwe a ziwalo ziwiri kapena kuposerapo ndi gawo lachidziwitso cha matenda.
- Palibe chizindikiro chamunthu payekhapayekha kwa MCAS - mawonekedwe a episodic, multisystem ndi omwe amafunikira.
- Zizindikiro zimayambira pakhungu, kupuma, mtima, GI, minyewa, minofu ndi mafupa, madera a genitourinary, ndi malingaliro.
- Kuphatikizidwa kwa machitidwe a ziwalo ziwiri kapena kuposerapo ndi gawo lachidziwitso cha matenda.
Wamba Zoyambitsa
Zochitika zokhala ndi nkhawa zimatha kukhala zoyendetsedwa ndi mkhalapakati; izi siziyenera kugwiritsidwa ntchito kuchotseratu vutoli, kapena kunyalanyaza zosowa zenizeni za thanzi la maganizo.
Kutsegula ndi kuchuluka. Kuwonetseredwa kovomerezeka kwapang'onopang'ono kukafika pamodzi kumatha kudutsa malire omwe palibe amene angadutse yekha. Ili ndiye lingaliro limodzi lothandiza kwambiri pakumvetsetsa zomwe zimawoneka mwachisawawa.
Zakudya
Zoyambitsa sizichitika zokha. Maselo a mast amayankha pakachulukidwe kachulukidwe, ndichifukwa chake chakudya, chipinda, kapena zochitika zomwezo zitha kukhala zopanda vuto tsiku lina ndikusapiririka tsiku lotsatira.
Kutentha
Zakudya
Zoyambitsa zakudya mu MCAS nthawi zambiri zimagwirizana ndi zomwe zili ndi histamine kapena kuwongolera kukwiya kwa mast cell m'malo mongodya zakudya zakale. Histamine imadziunjikira m'zakudya zikamakalamba, kupesa, kapena kusungidwa, chifukwa chake kutsitsi nthawi zambiri kumakhala kofunikira kwambiri kuposa zomwe zili - nsomba zomwezo zimatha kuloledwa patsiku logula osati masiku atatu pambuyo pake. Tchizi zachikale, nyama zochiritsidwa ndi zowonongeka, zakudya zofufumitsa, ndi zotsalira zimakhudzidwa kwambiri. Gulu lachiwiri likuwoneka kuti likupangitsa ma mast cell kutulutsa histamine yawo. Kusiyanasiyana kwapaokha ndikwambiri, ndipo mindandanda yosindikizidwa imatengedwa bwino ngati malingaliro oti ayesedwe osati malamulo.
Mpweya wozizira, madzi ozizira, zakumwa zoziziritsa kukhosi, ndi malo okhala m'firiji zimanenedwa kuti zimayambitsa odwala ena. Makamaka, mofulumira kusintha Kutentha kwapakati nthawi zambiri kumafotokozedwa kuti ndi koipa kwambiri kuposa kusakhazikika.
Zina mwazomwe zimanenedwa kawirikawiri zoyambitsa thupi. Mashawa otentha ndi mabafa, saunas, nyengo yotentha, zipinda zofunda, ndi zakumwa zotentha zonse zimatchulidwa mofala. Kutentha sikufuna kuti zinthu zilowe m'thupi, zomwe ndi gawo la chifukwa chake kuyezetsa kwazomwe sikungathe kuzizindikira.
Kuzizira
Kupsinjika maganizo
Masewera olimbitsa thupi
Kuchita masewera olimbitsa thupi ndikoyambitsa kwenikweni kwa odwala ambiri, komanso komwe kumakhala ndi mtengo weniweni, popeza kuchita zinthu kumakhala kopindulitsa pa thanzi ndi malingaliro. Kuchuluka ndi kuchuluka kwa chiwonjezeko ndizofunikira kwambiri kuposa nthawi yonse, ndichifukwa chake njira zapang'onopang'ono, zotsika kwambiri - ndi zochitika zongowonjezera kapena zamadzi pomwe kuyimirira sikuloledwa bwino - nthawi zambiri zimayendetsedwa bwino.
Kupsinjika maganizo
Kupsinjika maganizo ndiko kuyambitsa thupi, osati fanizo. Maselo a mast amanyamula ma receptor a corticotropin-release hormone ndipo amakhala pafupi ndi mathero a mitsempha, ndikupereka njira yolembedwa yomwe dongosolo lamanjenje limakhudza kuyambitsa kwawo. Mfundoyi ikuyenera kutsindika chifukwa odwala omwe zizindikiro zawo zimawonjezereka ndi kupsinjika maganizo kawirikawiri amauzidwa kuti matenda awo ndi amaganizo. Njira yowonetsera neuroimmune si chinthu chofanana ndi chizindikiro chomwe chimaganiziridwa.
Mahomoni
Odwala ambiri amafotokoza zizindikiro za cyclical, zomwe nthawi zambiri zimakhala zovuta kuzungulira msambo. Estrogen imakhudza machitidwe a mast cell, omwe ndi amodzi omwe amathandizira pakukula kwa matendawa mwa amayi. Zosintha pa nthawi ya mimba, pambuyo pobereka, ndi perimenopause zimafotokozedwanso, ndipo zimasiyana kwambiri pakati pa anthu.
Matenda
Infection modalirika imabweretsa reactivity yoyambira kwa odwala ambiri. Matenda obwera chifukwa cha ma virus nthawi zambiri amakulitsa chiwopsezo chomwe chimayambika kwa milungu ingapo pambuyo pake, motero mawonekedwe omwe adaloledwa kale amakhala oyambitsa kuchira. Odwala ena amakumana ndi zizindikiro zawo ku matenda opatsirana, ngakhale kukhazikitsa chifukwa chazochitika payekha kumakhala kovuta.
Zowonetsa zachilengedwe
Mankhwala
Magulu ena amankhwala amadziwika m'mabuku a mast cell monga oyambitsa, kuphatikiza opioid, mankhwala ena osagwiritsa ntchito steroidal anti-inflammatory, radiocontrast media, neuromuscular blocking agents omwe amagwiritsidwa ntchito mu anesthesia, ndi vancomycin. Zosakaniza zosagwira ntchito - utoto, zosungira, zodzaza - zingakhalenso chigawo choyambitsa matenda, chifukwa chake wodwala akhoza kulekerera mapangidwe a wopanga wina ndikuchitapo kanthu kwa wina.
Zofunika
Musayime, kupewa, kapena kusintha mankhwala omwe mwapatsidwa chifukwa cha mndandanda wa mankhwala. Bweretsani mndandandawo kwa sing'anga yemwe amadziwa mbiri yanu. Kuopsa kosiya chithandizo chofunikira kungathe kupitirira chiopsezo chomwe chikupeŵedwa, ndipo chisankhochi chimafuna kuwunika payekha.
Zonunkhira ndi zonunkhira
Mafuta onunkhiritsa, zotsitsimutsa mpweya, zoyeretsera, zochapira, zosungunulira, utoto watsopano, ndi ziwiya zatsopano ndi zina mwa zinthu zomwe zimanenedwa mosalekeza, ndipo pakati pazovuta kwambiri kuzipewa m'malo ogawana. Malo opanda mafuta onunkhira ndi pempho lokhazikika m'masukulu ndi m'malo antchito pazifukwa izi.
Mowa
Mowa nthawi zambiri umanenedwa ngati woyambitsa kudzera munjira zingapo: zakumwa zambiri zoledzeretsa zimakhala ndi histamine mwachindunji, makamaka vinyo ndi mowa; mowa ukhoza kusokoneza ma enzyme omwe amaphwanya histamine; ndipo imayambitsa vasodilation palokha. Zomwe zimachitika pazigawo zing'onozing'ono zimafotokozedwa kawirikawiri.
Ma trigger seti amasiyana pakati pa anthu chifukwa mbiri yolandirira, kugawa minofu, mikhalidwe yomwe ilipo, komanso kuyambiranso koyambira zonse zimasiyana. Amasinthanso mkati munthu pakapita nthawi - kuchulukirachulukira panthawi yomwe simukuwongolera bwino ndikucheperanso kamodzi chithandizo chikachepetsa kuyambiranso koyambira. Zochita zimatha kuchedwa ndi maola, zomwe zimalepheretsa kulumikizana pakati pa zomwe zimayambitsa ndi zotsatira zake. Pazifukwa zonsezi, kuzindikira zoyambitsa kumafuna kusungidwa mwadongosolo kwa milungu ingapo m'malo motengera zomwe zachitika m'modzi.
Kusagona mokwanira kapena kusagona mokwanira kumachepetsa kulolerana komanso kumalumikizana ndi chilichonse chomwe chili pamndandandawu. Sikuti nthawi zambiri imayambitsa moto, koma ndi imodzi mwazinthu zomwe zimathandizira kwambiri, ndipo odwala nthawi zambiri amafotokoza kuti kuteteza kugona kumakweza malire awo pa china chilichonse.
- Chifukwa chiyani zoyambitsa zimasiyana kwambiri
- Zoyambitsa thupi (kutentha, kuzizira, kupanikizika, kuchita khama) sizifuna kuti zilowe m'thupi ndipo siziwoneka poyesa ziwengo.
- Kupsinjika maganizo kumachitika kudzera mu njira yolembedwa ya neuroimmune; izi sizimapangitsa kuti vutoli likhale lamaganizo.
- Zoyambitsa stack - kuchuluka kwa katundu kumafunika kwambiri kuposa kuwonekera kulikonse.
- Zoyambitsa thupi (kutentha, kuzizira, kupanikizika, kuchita khama) sizifuna kuti zilowe m'thupi ndipo siziwoneka poyesa ziwengo.
Chifukwa chiyani MCAS ili
Zovuta ku Dziwani
Ma trigger seti amasiyana pakati pa anthu ndikusintha pakapita nthawi, kotero kusunga mbiri ndikofunikira.
Zizindikiro zosinthika
MCAS imapanga mitundu yosiyanasiyana yazizindikiro mwa odwala osiyanasiyana, komanso kuphatikiza kosiyanasiyana kwa wodwala yemweyo pakapita nthawi. Palibe chiwonetsero chimodzi chotsutsana ndi mapeto. Chifukwa chakuti zizindikirozo sizikhala zachindunji, chilichonse chikhoza kunenedwa kuti chimachitika chifukwa chofala kwambiri akamaganiziridwa payekha.
Kuchedwa kwa matenda mu MCAS sikuti kulephera kwa asing'anga payekha. Zimachokera kuzinthu zenizeni, zozindikirika za chikhalidwecho ndi mayesero omwe amagwiritsidwa ntchito pofufuza.
Zizindikiro zosinthika
Kutulutsidwa kwa mkhalapakati wapakati
Ili ndiye vuto lalikulu laukadaulo. Oyimira pakati omwe amayezedwa amakhala ndi moyo waufupi. Seramu tryptase ifika pachimake pakangopita maola ochepa atachitapo kanthu kenako ndikugwa; ma metabolites a mkodzo amakhalabe nthawi yayitali koma amawonetsabe zenera lochepa. Zitsanzo pakati pa zigawo sizingapeze chilichonse chokwezeka - osati chifukwa kuyambitsa sikunachitike, koma chifukwa sikungathenso kuyezedwa.
Kugwira ntchito pafupipafupi kwa magazi nthawi zambiri kumakhala kosadabwitsa mu MCAS. Magazi athunthu, zolembera zotupa, ndi biochemistry wamba nthawi zambiri zimakhala zachilendo, monganso mapanelo osagwirizana nawo. Kuyesa kwapadera kwa mkhalapakati kumafunika, ndipo ngakhale zomwe nthawi zambiri zimabweretsa zotsatira zabwinobwino pazifukwa zaukadaulo zomwe zili pansipa. Choncho wodwala akhoza kudziunjikira zambiri za kafukufuku wamba pomwe akukhalabe bwino - njira yomwe mwatsoka imawonjezera mwayi woti auzidwe kuti palibe cholakwika.
Kuchuluka kwa zotsatira zoyipa za mkhalapakati zikuwonetsa nthawi ndi kusamalira m’malo mopanda matenda. Kutsimikizira ndondomeko ya kusonkhanitsa ndi dokotala wolamula ndi labotale musanatenge sampuli ndi kofunika kwambiri kuposa kubwereza kuyesa kosayendetsedwa bwino.
Kuthana ndi matenda ena
Zofunikira pakusamalira zimaphatikiza izi. Oyimira pakati angapo amakhala osakhazikika kutentha kwa chipinda, ndipo zitsanzo za prostaglandin ndizosalimba kwambiri. Zitsanzo zimafunika kuzizira nthawi yomweyo, kuziziritsa nthawi yonse yosonkhanitsa, komanso kukonza mwachangu. Chitsanzo chosiyidwa pa kutentha kwa chipinda kapena chochedwetsedwa paulendo chikhoza kutulutsa zotsatira zabwinobwino kuchokera kwa wodwala yemwe anali kuchitapo kanthu.
Kupanda kuzindikira kwa dokotala
MCAS idadziwika bwino mu 2007, ndi mfundo zovomerezeka zomwe zidatsatiridwa mu 2010 ndi 2012. Madokotala ambiri omwe adamaliza maphunziro awo asanalowe m'maphunziro apamwamba. Kudziwika kumasiyana mosiyanasiyana ngakhale pakati pa allergists ndi immunologists, ndipo mwayi wopeza akatswiri omwe ali ndi chidziwitso cha mast cell ndi wosagwirizana ndi malo.
Zizindikiro za MCAS zimaphatikizana kwambiri ndi matumbo okwiya, urticaria osatha, mphumu, fibromyalgia, matenda otopa kwambiri, nkhawa, dysautonomia, ndi ena angapo. Iliyonse mwa izi ndi yodziwika bwino kwa asing'anga ambiri ndipo imapezeka kawirikawiri. Wodwala akasonyeza kuti ali ndi zizindikiro zoyenerera zingapo mwa izi pang'ono, zotsatira zake nthawi zonse zimakhala zongopeka pang'ono m'malo mongogwirizanitsa.
Ndikoyenera kunena momveka bwino kuti njira zodziwira matenda a MCAS zimakhalabe nkhani yotsutsana kwenikweni m'mabuku azachipatala. Mwachidule, mbali imodzi imakhala ndi mfundo zokhwima zomwe zimafuna umboni wa mkhalapakati, chifukwa popanda izi, matendawa amatha kugwiritsidwa ntchito mozama kwambiri. Udindo wina ukunena kuti njira zokhwima zimaphonya odwala omwe ali ndi vutoli koma omwe kuyesa kwawo kwa mkhalapakati kumalephera mobwerezabwereza pazifukwa zaukadaulo zomwe tafotokozazi.
Izi si kusagwirizana odwala analenga, ndipo si kuthetsedwa. Zotsatira zake ndikuti akatswiri awiri odziwa bwino amatha kufika paziganizo zosiyanasiyana za wodwala yemweyo. Kumvetsetsa kuti mkangano ulipo kumathandizira kufotokoza malingaliro otsutsana popanda kuganiza kuti dokotala wina adangolakwitsa.
Kusamvana kwa matenda
Ndikoyenera kunena momveka bwino kuti njira zodziwira matenda a MCAS zimakhalabe nkhani yotsutsana kwenikweni m'mabuku azachipatala. Mwachidule, mbali imodzi imakhala ndi mfundo zokhwima zomwe zimafuna umboni wa mkhalapakati, chifukwa popanda izi, matendawa amatha kugwiritsidwa ntchito mozama kwambiri. Udindo wina ukunena kuti njira zokhwima zimaphonya odwala omwe ali ndi vutoli koma omwe kuyesa kwawo kwa mkhalapakati kumalephera mobwerezabwereza pazifukwa zaukadaulo zomwe tafotokozazi.
Izi si kusagwirizana odwala analenga, ndipo si kuthetsedwa. Zotsatira zake ndikuti akatswiri awiri odziwa bwino amatha kufika paziganizo zosiyanasiyana za wodwala yemweyo. Kumvetsetsa kuti mkangano ulipo kumathandizira kufotokoza malingaliro otsutsana popanda kuganiza kuti dokotala wina adangolakwitsa.
- Kuchedwa kwa matenda
- Oyimira pakati amakhala akanthawi kochepa komanso osakhazikika, chifukwa chake nthawi ndi kasamalidwe ka zitsanzo zimayendetsa zolakwika zambiri zabodza.
- Zizindikiro zimayenderana ndi zina zambiri zodziwika bwino, zomwe zimapangitsa kuti azindikire pang'ono.
- Kuyesa kwa labotale kwanthawi zonse kumakhala kwabwinobwino mu MCAS; kuyezetsa kwapadera kwa mkhalapakati kumafunika.
- Oyimira pakati amakhala akanthawi kochepa komanso osakhazikika, chifukwa chake nthawi ndi kasamalidwe ka zitsanzo zimayendetsa zolakwika zambiri zabodza.
Zogwirizana Mikhalidwe
Njira zodziwira matenda zimakhalabe zotsutsana, kotero malingaliro a akatswiri akhoza kusiyana movomerezeka.
Kugwirizana si chifukwa. Zinthu zingapo zomwe zili pansipa zimachitika limodzi ndi MCAS nthawi zambiri kuposa momwe mwadzidzi ungadziwire, koma kaya amagawana njira, kaya imayambitsa ina, kapena njira zotumizirana zogawana zimakulitsa kuphatikizika komwe kukuwoneka. sanakhazikitsidwe zambiri mwazophatikizana izi.
MCAS imanenedwa pafupipafupi limodzi ndi zina zingapo. Mphamvu zaumboni zimasiyana kwambiri pakati pa mayanjano awa, ndi kusiyana kwa zinthu.
Werengani izi poyamba
Hypermobile Ehlers-Danlos Syndrome (hEDS) ndi hypermobility spectrum disorders
Kugwirizana kwa hEDS, POTS, ndi MCAS kumakambidwa pafupipafupi mokwanira kuti afotokozeredwe mwamwayi ngati atatu. Chiyanjanochi chimanenedwa nthawi zonse, koma njira yoyambira imakhalabe yongopeka. Maselo a mast amakhala mu minofu yolumikizana ndi kutenga nawo mbali pakukonzanso minofu, yomwe imapereka njira yomveka yofufuzira, koma kumveka si umboni. Njira zotumizira ndi zowunikira zingathandizenso kuti kuphatikizikako kuwonekere kwamphamvu.
Hereditary alpha tryptasemia (HaT)
HaT ndi chibadwa chomwe chimaphatikizapo makope owonjezera a jini ya alpha-tryptase TPSAB1, chotengera chotengera chachikulu cha autosomal, ndipo chimapanga seramu tryptase yokwera mosalekeza. Ndilofala poyerekezera ndi anthu wamba. Chiyanjano chake ndi zizindikiro chikufotokozedwabe: chimalumikizidwa ndi zovuta kwambiri m'mikhalidwe ina, koma anthu ambiri omwe ali ndimtunduwu amakhala opanda zizindikiro.
HaT imafunika kutanthauzira. Chifukwa imakweza tryptase yoyambira, mtengo umodzi wokwera wa tryptase mwa munthu yemwe ali ndi HaT sumangowonetsa kuyambika kwa ma cell cell - zomwe zimatsimikizira chifukwa chake gawo lozindikira limatanthauzidwa ngati kukwera pamwamba pa maziko a munthu osati nambala yeniyeni.
Hereditary alpha tryptasemia (HaT)
Zizindikiro za m'mimba mu MCAS zimayenderana pafupifupi ndi njira za IBS, ndipo odwala ena amakhala ndi zilembo zonse ziwiri. Kafukufuku wawunika kachulukidwe ka mast cell komanso kuyambitsa kwamatumbo am'matumbo a odwala a IBS omwe ali ndi zopeza zosiyanasiyana. Kaya kagawo kakang'ono ka IBS kakuyimira kukhudzidwa kwa mast cell ndi funso lotseguka osati zotsimikizika.
Migraine
Migraine imanenedwa kuti ndi yokwera kwambiri pakati pa odwala MCAS. Histamine ndi chinthu chokhazikika cha vasoactive chomwe chimadziwika kuti chimagwirizana ndi mutu, ndipo ma mast cell amapezeka mu meninges. Ulalo wamakina ndi wovomerezeka mwachilengedwe ndipo ukufufuzidwa, koma osakhazikika.
mphumu ndi urticaria yosatha
Migraine
Migraine imanenedwa kuti ndi yokwera kwambiri pakati pa odwala MCAS. Histamine ndi chinthu chokhazikika cha vasoactive chomwe chimadziwika kuti chimagwirizana ndi mutu, ndipo ma mast cell amapezeka mu meninges. Ulalo wamakina ndi wovomerezeka mwachilengedwe ndipo ukufufuzidwa, koma osakhazikika.
Ofufuza ena anena kuti ma mast cell activation imathandizira ku Long COVID, ndikuzindikira kuti zizindikiro zimakumana ndi kutopa, kuvutika kwachidziwitso, komanso dysautonomia. Ili ndi gawo lofufuza mwachangu ndipo umboni uli choyambirira. Iyenera kuwonedwa ngati lingaliro lomwe likuphunziridwa m'malo mwa ubale wokhazikika, ndi zonena za kulumikizana kokhazikika - mbali zonse - zomwe zikupitilira zomwe zalembedwazo.
Awa ali ndi ubale womveka bwino wamakina ndi ma cell amtundu uliwonse mugawoli. Maselo a mast ali pakatikati pa pathophysiology ya onse awiri - leukotrienes ndi histamine drive bronchoconstriction ndi mapangidwe a wheal motsatana. Urticaria yosachiritsika makamaka imakhudza mast cell activation mwachindunji. Awa si mayanjano ongoyerekeza; ndi mikhalidwe yomwe kukhudzidwa kwa mast cell kumakhazikitsidwa, ngakhale amakhalabe ndi matenda osiyana ndi MCAS.
COVID yayitali
| Matenda a Autoimmune | Chikhalidwe cha ubale | Mphamvu ya umboni pang'onopang'ono |
|---|---|---|
| Mkhalidwe | Chikhalidwe cha ubale | Udindo wa umboni |
| Matenda a urticaria | Mast cell activation ndiyofunika kwambiri pa matendawo | Udindo wa umboni |
| Njira yokhazikitsidwa | mphumu | Mast cell mediators amayendetsa bronchoconstriction |
| Cholowa cha alpha tryptasemia | Makhalidwe a chibadwa okweza tryptase yoyambira; zimakhudza kutanthauzira kwa mayeso | Khalidwe lokhazikika; Chiyanjano cha zizindikiro chikufotokozedwabe |
| Mpoto | Zochitika nthawi zambiri; mkhalapakati zotsatira pa mtima kamvekedwe akufuna | Association inanena; makina osathetsedwa |
| Migraine | hEDS / hypermobility | Zochitika nthawi zambiri; kukhudzidwa kwa minofu yolumikizana |
| Association inanena; makina ongoyerekeza | Mitengo yokwezeka yomwe idanenedwa; oyimira vasoactive omveka | Association inanena; makina akuphunziridwa |
| IBS | Chizindikiro chikuphatikizana; Zotsatira za ma cell a m'matumbo zimasiyanasiyana | Kuphatikizika bwino; ubale wosathetsedwa |
| Migraine imanenedwa kuti ndi yokwera kwambiri pakati pa odwala MCAS. Histamine ndi chinthu chokhazikika cha vasoactive chomwe chimadziwika kuti chimagwirizana ndi mutu, ndipo ma mast cell amapezeka mu meninges. Ulalo wamakina ndi wovomerezeka mwachilengedwe ndipo ukufufuzidwa, koma osakhazikika. | Matenda a Autoimmune | Co-occurrence lipoti; adagawana chitetezo chamthupi chomwe chimaperekedwa |
- Association inanena; makina sanakhazikitsidwe
- Chizindikiro chikuphatikizana; kukhudzidwa kwa mast cell
- Choyambirira - pofufuza mwachangu
- Kukhudzidwa kwa mast cell kumakhazikitsidwa mu urticaria osatha ndi mphumu - izi sizolumikizana zongopeka.
- HaT ndi chibadwa chenicheni chomwe chimadzutsa tryptase yoyambira ndikusintha momwe zotsatira zoyezetsa ziyenera kuwerengedwa.
Kumvetsetsa Kwamakono
za Zoyambitsa
M'dera lonseli, mayanjano akhala akulakwitsa mobwerezabwereza kuti ndi chifukwa - werengani zonena mosamala.
Palibe chifukwa chimodzi cha MCAS chomwe chakhazikitsidwa. Zomwe zili m'munsizi ndi madera ofufuzira omwe ali ndi chithandizo chosiyana. Chilichonse chomwe chikuwonetsa chidaliro, akaunti yogwirizana ya MCAS ikupitilira umboni.
Genetics
Zomwe zimakhazikitsidwa: Hereditary alpha tryptasemia ndi mtundu weniweni, wodziwika bwino womwe umaphatikizapo zolemba zina zowonjezera. TPSAB1, ndipo imakweza tryptase yoyambira. Payokha, a KIT Kusintha kwa D816V kumakhazikitsidwa ngati dalaivala mu mastocytosis akuluakulu ambiri - chikhalidwe chosiyana, koma chomwe chikuwonetsa momwe kusintha kumodzi kungawonongere biology ya mast cell.
Zomwe siziri: Palibe kusintha kofanana komwe kwadziwika kwa MCAS. Kuphatikizika kwapabanja kumanenedwa, ndipo odwala ena amafotokozera achibale angapo omwe akhudzidwa, koma njira yodziwika ya cholowa cha MCAS yokha sinakhazikitsidwe. Kaya MCAS ikuyimira vuto limodzi lomwe lili ndi chibadwa chomwe sichinapezeke, kapena mikhalidwe yosiyanasiyana yomwe ili pansi pa chizindikiro chimodzi chachipatala, sichinathetsedwa.
Genetics
Broad immune dysregulation ndi gawo lotsogola pakufufuza. Njira zomwe akuyembekezeredwa zimaphatikizapo kusinthika kwa ma receptor kapena kukhudzidwa kwa mast cell, kusokoneza mayendedwe omwe nthawi zambiri amatha kuletsa kuyambitsa, komanso kuyanjana kwachilendo pakati pa ma mast cell ndi magulu ena oteteza chitetezo. Izi ndi zomveka komanso zophunziridwa mwakhama, koma zimalongosola gulu la kufotokozera m'malo mwa njira yowonetsera.
Kupsinjika maganizo ndiko kuyambitsa thupi, osati fanizo. Maselo a mast amanyamula ma receptor a corticotropin-release hormone ndipo amakhala pafupi ndi mathero a mitsempha, ndikupereka njira yolembedwa yomwe dongosolo lamanjenje limakhudza kuyambitsa kwawo. Mfundoyi ikuyenera kutsindika chifukwa odwala omwe zizindikiro zawo zimawonjezereka ndi kupsinjika maganizo kawirikawiri amauzidwa kuti matenda awo ndi amaganizo. Njira yowonetsera neuroimmune si chinthu chofanana ndi chizindikiro chomwe chimaganiziridwa.
Odwala ambiri amakhala ndi zizindikiro za matenda opatsirana. Matendawa amadziwika kuti amatsegula ma mast cell kudzera pa ma Toll-like receptors, kupereka njira yomveka, ndipo kuyambika kwa matenda pambuyo pake ndi njira yodziwika bwino m'mikhalidwe ingapo. Komabe, kukhazikitsa kuti matenda zidayambitsa MCAS muzochitika payekha ndizovuta kwambiri, ndipo kukumbukira kukondera kumakhudza maakaunti obwereza. Izi zikadali mgwirizano womwe ukuphunziridwa.
Kuwonongeka kwa chitetezo chamthupi
Broad immune dysregulation ndi gawo lotsogola pakufufuza. Njira zomwe akuyembekezeredwa zimaphatikizapo kusinthika kwa ma receptor kapena kukhudzidwa kwa mast cell, kusokoneza mayendedwe omwe nthawi zambiri amatha kuletsa kuyambitsa, komanso kuyanjana kwachilendo pakati pa ma mast cell ndi magulu ena oteteza chitetezo. Izi ndi zomveka komanso zophunziridwa mwakhama, koma zimalongosola gulu la kufotokozera m'malo mwa njira yowonetsera.
Kutupa kosatha
Zinthu zachilengedwe
Zomwe angafunike kuti athandizire zachilengedwe ndi monga kuwonetseredwa kwa mankhwala, nkhungu, kuwononga mpweya, ndi kusintha kwa zakudya pamlingo wa anthu. Umboni pano ndi wochepa kwambiri poyerekeza ndi madera omwe ali pamwambawa, ndipo zambiri siziri zachindunji. Ili ndi gawo lovomerezeka la kafukufuku m'malo mopereka chithandizo chokhazikika, komanso ndi malo omwe zonena zamalonda zodalirika ndizofala komanso zosachirikizidwa bwino.
Kutupa kosatha
Maselo a mast onse amayankha ndikuthandizira kutupa, zomwe zimabweretsa mwayi wodzilimbitsa nokha pomwe kuyambitsa kumabweretsa malo otupa omwe amachepetsa mwayi wowonjezera. Izi ndizogwirizana mwamakina ndipo zimagwirizana ndi kuwunika kwachipatala kuti kuchitapo kanthu kumakulirakulira pakuwongolera koyipa. Imakhalabe chitsanzo osati chifukwa chowonetseredwa.
Epigenetics
Chithunzi chaumboni: magulu okhazikika akusunthira kunja kuchokera ku 'Kukhazikitsidwa' (makhalidwe a HaT; KIT D816V mu mastocytosis) kudzera mu 'Kufufuzidwa mwachangu ndi umboni wochirikiza' (kuwonongeka kwa chitetezo chamthupi, kuyambika kwa matenda) kupita ku 'Hypothesised, umboni wocheperako wachindunji' (epigenetics, kuwonetseredwa kwachilengedwe).
- Kumene izi zimasiya zinthu
- HaT ndi kusintha kwa KIT D816V mu mastocytosis kumakhazikitsidwa chibadwa - komanso sichomwe chimayambitsa MCAS.
- Kuwonongeka kwa chitetezo chamthupi komanso kuyambika kwa matenda pambuyo pake kumafufuzidwa mwachangu ndi umboni wina wothandizira.
- Palibe chifukwa cha MCAS chomwe chakhazikitsidwa.
- Khalani okayikira zonena za 'zoyambitsa' mokayikira - gulu lofufuza silinazindikire chimodzi.
Kukhala Ndi MCAS
Khalani okayikira zonena za 'zoyambitsa' mokayikira - gulu lofufuza silinazindikire chimodzi.
Kasamalidwe ka tsiku ndi tsiku ndi machitidwe
Odwala ambiri amapeza kuti kusasinthasintha kumakweza malire awo modalirika kuposa momwe aliyense payekhapayekha. Kugona nthawi zonse ndi kudzuka, nthawi yodziwikiratu ya chakudya, kutentha kokhazikika komwe kungatheke, komanso kusasinthasintha kwanthawi yamankhwala kumachepetsa kusinthasintha. Mayendedwe amathandizanso kuti chizindikiritso choyambitsa chikhale chotheka: zosintha zambiri zikakhazikika, zomwe zidasintha zimawonekera.
Utsogoleri umapangidwa makamaka kuchokera ku zochitika za tsiku ndi tsiku osati kuchokera pakuchitapo kanthu kamodzi. Zomwe zikutsatira ndizoyang'ana ntchito - cholinga chake ndi moyo waukulu kwambiri womwe thupi lanu lingathandizire.
Kasamalidwe ka tsiku ndi tsiku ndi machitidwe
Odwala ambiri amapeza kuti kusasinthasintha kumakweza malire awo modalirika kuposa momwe aliyense payekhapayekha. Kugona nthawi zonse ndi kudzuka, nthawi yodziwikiratu ya chakudya, kutentha kokhazikika komwe kungatheke, komanso kusasinthasintha kwanthawi yamankhwala kumachepetsa kusinthasintha. Mayendedwe amathandizanso kuti chizindikiritso choyambitsa chikhale chotheka: zosintha zambiri zikakhazikika, zomwe zidasintha zimawonekera.
Pacing ndi mutu womwe umabwerezedwa m'maakaunti a odwala - kugawa zochitika tsiku limodzi kapena sabata m'malo momangoyang'ana, ndikumanga nthawi yochira pambuyo pa zomwe zimadziwika. Izi sizofanana ndi kusagwira ntchito, ndipo kusagwira ntchito kwanthawi yayitali kumatengera ndalama zake zolembedwa bwino.
Diary yazizindikiro ndi imodzi mwazinthu zothandiza kwambiri zomwe wodwala angabweretse ku nthawi yokumana: konkire, nthawi yayitali, komanso yeniyeni kwa iwo mwanjira yomwe palibe mndandanda wazomwe zingayambitse. Zimathandizanso kusiyanitsa zoyambitsa zenizeni ndi zomwe zinangochitika mwangozi, zomwe kukumbukira kokha sikumachita bwino.
Mbiri yokhazikika ndiyo njira yodalirika kwambiri yodziwira zoyambitsa zamunthu. Zolemba zothandiza zimapitilira chakudya: nthawi yamasana, kutentha kozungulira, zomwe zidadyedwa komanso momwe zidali zatsopano, kuchita masewera olimbitsa thupi, kupsinjika ndi kugona, fungo lonunkhira, mankhwala omwe amamwedwa, komanso - kwa odwala omwe ali msambo - kuzungulira. Zizindikiro ziyenera kulembedwa ndi nthawi yake komanso mozama.
Kuwonetsa zizindikiro zokonzedwa ndi organ system m'malo motengera nthawi yomwe imapangitsa kuti mawonekedwe amitundu yambiri awonekere nthawi yomweyo, pomwe nkhani yofotokozera imakonda kuyibisa. Kubweretsa zotsatira - kuphatikizapo zachilendo - ndizothandiza kwambiri ndipo zimapewa kubwereza kufufuza komaliza.
Diary yazizindikiro ndi imodzi mwazinthu zothandiza kwambiri zomwe wodwala angabweretse ku nthawi yokumana: konkire, nthawi yayitali, komanso yeniyeni kwa iwo mwanjira yomwe palibe mndandanda wazomwe zingayambitse. Zimathandizanso kusiyanitsa zoyambitsa zenizeni ndi zomwe zinangochitika mwangozi, zomwe kukumbukira kokha sikumachita bwino.
Kugwira ntchito ndi madokotala
Malo ogona ambiri amaphatikizapo malamulo a m'kalasi opanda mafuta onunkhira, chilolezo chonyamula ndi kudzipangira yekha mankhwala, kupeza madzi ndi nthawi yopuma, kuganizira za kutentha pakukhala, kusinthasintha kwa kupezeka pa nthawi yamoto, ndi kusintha kwa maphunziro a thupi. Kumene ndondomeko za malo ogona zimakhalapo m'maphunziro anu, kukhala ndi matenda olembedwa molembedwa kumapangitsa kuti kuwapeza kukhala kosavuta.
Kufunsa mwachindunji za nthawi yoyezetsa mkhalapakati ndi kasamalidwe ka zitsanzo ndikoyenera ndipo kumapangitsa mwayi wopeza zotsatira zomwe zingagwiritsidwe ntchito. Ngati simukumvedwa, kufunafuna lingaliro lachiwiri ndikovomerezeka; Matenda a mast cell ndi malo omwe amadziwika bwino kwambiri. Kuchotsedwa ntchito kamodzi ndi kofala ndipo si chigamulo.
Sukulu
Malo ogona ambiri amaphatikizapo malamulo a m'kalasi opanda mafuta onunkhira, chilolezo chonyamula ndi kudzipangira yekha mankhwala, kupeza madzi ndi nthawi yopuma, kuganizira za kutentha pakukhala, kusinthasintha kwa kupezeka pa nthawi yamoto, ndi kusintha kwa maphunziro a thupi. Kumene ndondomeko za malo ogona zimakhalapo m'maphunziro anu, kukhala ndi matenda olembedwa molembedwa kumapangitsa kuti kuwapeza kukhala kosavuta.
Ntchito
Zolinga zapantchito zimafanana ndi za kusukulu: malamulo onunkhiritsa, kuwongolera kutentha, kusinthasintha kapena kukhazikika patali pakayaka moto, ndikusintha ndandanda komwe kutopa kumatsata njira yodziwikiratu. Kaya ndi kuwulutsa zochuluka bwanji ndi chisankho chaumwini chokhala ndi malonda enieni, ndipo chimagwirizana ndi chitetezo cha ntchito chomwe chimasiyana kwambiri pakati pa madera.
Maulendo
Njira zothandiza zomwe odwala amatchula nthawi zambiri ndi monga kunyamula mankhwala m'chikwama cha m'manja chokhala ndi zolemba, kupeza kalata ya dokotala ya mankhwala obaya jekeseni, kufufuza zipatala zachipatala komwe mukupita, kukonzekera chakudya pasadakhale komwe kudzakhala kofunikira, komanso kulola nthawi yochira pofika. Kusintha kwa kutentha ndi kukwera ndikofunika kuyembekezera.
Thanzi la maganizo
Kukhala ndi matenda osinthasintha, osadziwika bwino, komanso omwe nthawi zambiri amachotsedwa nthawi zambiri kumakhala ndi mtengo weniweni wamaganizo, ndipo mtengo umenewo si kufooka kwa khalidwe. Odwala ambiri amafotokoza nthawi yayitali ya kusakhulupirira asanazindikire, zomwe zimasiya chizindikiro chake. Thandizo - kaya akatswiri, anzako, kapena onse awiri - ndi gawo loyenera la kasamalidwe osati kuvomereza kuti zizindikiro ndi zamalingaliro.
- Kusiyanitsa komwe kuli koyenera kugwiridwa ndi ichi: kumasulidwa kwa mkhalapakati kumatha kubweretsa nkhawa ngati physiology, ndipo izi zimayenera kuzindikirika m'malo molembanso za chikhalidwe chonsecho. Panthawi imodzimodziyo, nkhawa ndi kupsinjika maganizo ndizofala, zochiritsika, ndipo ziyenera kuthetsedwa pazokha pamene zilipo.
- Kukonzekera mwadzidzidzi
- Odwala ena omwe ali ndi matenda a mast cell ali pachiwopsezo cha anaphylaxis - kufulumira, koopsa, komwe kumatha kuyika moyo pachiwopsezo. Kumene ngoziyo ingagwire ntchito, kukonzekera sikosankha, ndipo ndi kukambirana ndi dokotala musanafunikire.
- Odwala omwe amayesedwa kuti ali pachiwopsezo amapatsidwa epinephrine auto-injection ndipo amalangizidwa kuti azinyamula nthawi zonse.
- Epinephrine ndiye njira yoyamba yothandizira anaphylaxis. Ma antihistamines siwolowa m'malo ndipo sayenera kudaliridwa pazovuta kwambiri.
- Dongosolo lolembedwa lachidziwitso chadzidzidzi lomwe mwagwirizana ndi dokotala wanu liyenera kukuwonetsani zizindikiro, zomwe muyenera kupereka, komanso nthawi yoti muyimbire chithandizo chadzidzidzi.
Odwala omwe amayesedwa kuti ali pachiwopsezo amapatsidwa epinephrine auto-injection ndikulangizidwa kuti azinyamula nthawi zonse. Epinephrine ndi mankhwala oyamba a anaphylaxis; antihistamines siilowa m'malo mwake ndipo sayenera kudaliridwa muzochitika zoopsa kwambiri.
Ndemanga yoletsa zakudya
Chidziwitso chachipatala chozindikira matenda ndi zomwe zimadziwika kuti zimayambitsa mankhwala zimathandiza ogwira ntchito mwadzidzidzi omwe mwina sakudziwa bwino za matendawa.
Opaleshoni ndi opaleshoni imalola kukambirana pasadakhale kotero kuti othandizira amatha kusankhidwa poganizira momwe alili.
- Ndemanga yoletsa zakudya
- Diary yokhazikika yazizindikiro pakatha milungu ingapo ndiyo njira yodalirika yodziwira zomwe zimayambitsa.
- Kuwonetsa zizindikiro ndi dongosolo la ziwalo kumapangitsa kuti mawonekedwe a multisystem awonekere kwa asing'anga.
- Zochita zokhazikika zimakulitsa malire ndikupangitsa kuti chizindikiritso choyambitsa chitheke.
- Diary yokhazikika yazizindikiro pakatha milungu ingapo ndiyo njira yodalirika yodziwira zomwe zimayambitsa.
- Kuwonetsa zizindikiro ndi dongosolo la ziwalo kumapangitsa kuti mawonekedwe a multisystem awonekere kwa asing'anga.
Amafunsidwa pafupipafupi Mafunso
Mafunso 25 omwe odwala, mabanja, ndi azachipatala amafunsa nthawi zambiri. Sankhani funso lililonse kuti mukulikulitsa.
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Kodi MCAS ndi cholowa?
Zizindikiro zimatha kukhala bwino kwambiri, nthawi zina mpaka pomwe odwala amadzifotokozera kuti ndi asymptomatic - makamaka chithandizo chikachepetsa kuyambiranso koyambira komanso zoyambitsa zazikulu zimadziwika. Chisankho chokhazikika, chokhazikika popanda kasamalidwe kalikonse sichinalembedwe bwino. Nthawi zachikhululukiro zotsatiridwa ndi kubwerezabwereza zimafotokozedwa kawirikawiri.
Zinthu zingapo zimasintha pakapita nthawi: kuchitapo kanthu koyambira kumayenda ndi tulo, kupsinjika, kuchuluka kwa mahomoni, komanso matenda aposachedwa; maselo a mast amatha kumasula mitundu yosiyanasiyana ya mkhalapakati pazochitika zosiyanasiyana; ndipo zoyambitsa zimadzikulitsa pokhapokha ngati sizikuyenda bwino ndipo nthawi zambiri zimacheperanso ndi chithandizo. Kusintha kwa zizindikiro ndi khalidwe la mkhalidwewo osati chizindikiro chakuti matendawo ndi olakwika.
Kawirikawiri pazifukwa zamakono. Oyimira pakati omwe amayezedwa amakhala ndi theka la moyo waufupi, kotero sampuli pakati pa zomwe zimachitika sizingapezeke chokwezeka. Enanso amakhala osakhazikika kutentha kwa chipinda ndipo amafuna kuzizira nthawi yomweyo ndikukonza mwachangu - zitsanzo zomwe zidakhala panja kapena zomwe zidachedwetsedwa paulendo zitha kuwerengeka ngati zili bwino kwa wodwala yemwe anali kuchitapo kanthu. Ntchito yokhazikika yamagazi ndi mapanelo osagwirizana nawo akuyembekezekanso kukhala abwinobwino ku MCAS, popeza sanapangidwe kuti azindikire.
Inde, kudzera mu njira yolembedwa yazathupi. Ma cell a mast amanyamula ma receptor a corticotropin-release hormone ndipo amakhala pafupi ndi mathero a mitsempha, zomwe zimapatsa kupsinjika maganizo kuwafikira mwachindunji. Izi ndizofunikira kunena momveka bwino, chifukwa odwala omwe zizindikiro zawo zimakulirakulira akapanikizika nthawi zambiri amauzidwa kuti matendawa ndi amisala. Njira ya neuroimmune sifanana ndi chizindikiro chomwe chimaganiziridwa.
Ayi. Kusintha kwa zakudya kungachepetse kulemedwa kwa zizindikiro kwa odwala ena mwa kuchepetsa kuchuluka kwa ziwopsezo zonse, ndipo phindu limenelo ndiloyenera kutsata moganizira - koma silichiza vutoli. Umboni wa zakudya zotsika kwambiri za histamine ndizochepa komanso zabwino kwambiri. Kuletsa kosayang'aniridwa kwanthawi yayitali kumakhala ndi chiopsezo chenicheni cha kuperewera kwa zakudya m'thupi komanso kudya molongosoka, kotero kuyesa kokhazikika, kwanthawi yayitali ndikubweretsanso kumakhala kotetezeka kusiyana ndi kupewa.
Mu mastocytosis thupi limadziunjikira ma cell ochuluka kwambiri, ndipo kuchulukirako kumatha kuwonetsedwa - kudzera m'mafupa a m'mafupa, zotupa zapakhungu, kapena tryptase yokwera mosalekeza, nthawi zambiri ndi kusintha kwa KIT D816V mu matenda akuluakulu. Mu MCAS chiwerengero cha mast cell ndi abwinobwino; chomwe chili chachilendo ndi momwe amayambira mwachangu. Ichi ndichifukwa chake mastocytosis imatha kutsimikiziridwa ndikupeza maselo ndipo MCAS sangathe.
Ayi, ngakhale kuti nthawi zambiri amasokonezeka. Kusalolera kwa histamine nthawi zambiri kumafotokozedwa kuti ndizovuta kuphwanya histamine yomwe yalowetsedwa, yomwe nthawi zambiri imayamba chifukwa cha kuchepa kwa enzyme diamine oxidase. MCAS imakhudza ma mast cell amthupi omwe amatulutsa histamine ndi oyimira ena ambiri mosayenera. Zitha kubweretsa zizindikiro zophatikizika ndipo zimatha kukhalapo, koma njira zake zimasiyana.
Apa ndi pamene akatswiri amatsutsana. Njira zogwirizanirana zimafuna umboni weniweni wa kumasulidwa kwa mkhalapakati, ndipo madokotala ambiri amatsatira izi mosamalitsa. Ena amatsutsa kuti kugwiritsa ntchito mosamalitsa kumaphonya odwala omwe kuyezetsa kwawo kumalephera mobwerezabwereza pazifukwa zanthawi yake komanso kusamalira. Mtsutsowu sunathetsedwe m'mabuku, chifukwa chake akatswiri awiri odziwa bwino amatha kufika paziganizo zosiyana za wodwala yemweyo.
Allergists ndi immunologists nthawi zambiri, ndi hematologists komwe mastocytosis ndiyofunikira. M'zochita zake, kuzolowerana kumasiyana mosiyanasiyana ngakhale mkati mwazamankhwalawa, ndipo kupezeka kwa asing'anga omwe ali ndi chidziwitso cha mast cell ndikosiyana malinga ndi malo. Odwala ambiri amapeza kuti dokotala wodziwa bwino yemwe ali wokonzeka kugwirizanitsa chisamaliro ndi wofunika monga kusankhidwa kwa akatswiri.
Nthawi zambiri amathandizira koma samathetsa chilichonse, ndipo kuyankha pang'ono kumayembekezeredwa m'malo mongowonetsa kuti matendawa ndi olakwika. Antihistamines amaletsa histamine okha; prostaglandins, leukotrienes, platelet activating factor, ndi ma cytokines sakhudzidwa. Ichi ndichifukwa chake chithandizo chikuchulukirachulukira, ndikuwonjezera othandizira omwe amalumikizana ndi oyimira pakati antihistamines amaphonya.
Mafotokozedwe awiri ofanana. Magulu ena amankhwala amatha kuyambitsa ma mast cell mwachindunji, osatengera matupi ena aliwonse. Payokha, zosakaniza zosagwira ntchito - utoto, zodzaza, zosungira - zitha kukhala chigawo choyambitsa matenda m'malo mogwiritsa ntchito mankhwala osokoneza bongo, chifukwa chake wodwala akhoza kulekerera mtundu wa wopanga ndikuchitapo kanthu kwa wina. Kukonzekera kophatikizana nthawi zina kumagwiritsidwa ntchito ngati izi zikukayikiridwa. Kambiranani zomwe mukukayikira ndi omwe akukulemberani m'malo mongoyimitsa yekha.
Zitha kukhala, kutengera kuuma komanso pamalamulo omwe mukukhala. Odwala ena amagwira ntchito ndi kuphunzira popanda kusintha; ena ali ndi malire. Zidziwitso za kulumala zimatengera momwe zimakhalira ndipo nthawi zambiri zimafunikira zolemba za momwe zimagwirira ntchito m'malo mozindikira matenda okha. Zolemba zachipatala zomwe dokotala alephera nthawi zambiri zimakhala zothandiza kuposa chizindikiro cha matenda chokha.
Kodi MCAS ndi olumala?
Zomwe zimanenedwa zimasiyana kwambiri - odwala ena amafotokoza kusintha kwa nthawi yomwe ali ndi pakati, ena akuipiraipira, ndipo ena sasintha bwino. Mphamvu ya mahomoni pama cell a mast imalembedwa, zomwe zimapangitsa kusintha kulikonse kukhala kovomerezeka. Kukhala ndi pakati ndi MCAS kumakupatsani mwayi wokonzekeratu ndi dokotala wanu wa mast cell komanso gulu lanu lakubeleka, makamaka ponena za mankhwala omwe apitirire.
Chifukwa mast cell amayankha kukhudzidwa kwakuthupi mwachindunji, osati ku allergen. Kutentha, kuzizira, kupanikizika, kukangana, ndi khama zonse zingathe kuziyambitsa popanda chinthu chilichonse kulowa m'thupi. Kuyezetsa ziwengo kumapangidwa kuti zizindikire kukhudzidwa kwa IgE-mediated ku mapuloteni enaake, kotero sikungazindikire zoyambitsa izi - ndichifukwa chake gulu loyera la ziwengo limagwirizana kwathunthu ndi zomwe zimachitika pakutentha.
Zimatengera wothandizira. Ma antihistamines nthawi zambiri amawonekera mkati mwa masiku. Mast cell stabilizers monga cromolyn ndi ketotifen nthawi zambiri amafunikira milungu ingapo kuti agwiritse ntchito mosadukiza phindu lawo lonse lisanawonekere, ndipo kuyimitsa msanga ndi njira wamba yotaya zomwe zikanagwira ntchito. Kuyesa kwa masabata anayi kapena asanu ndi limodzi pakusintha ndi lamulo lomwe limatchulidwa pafupipafupi.
Nthawi zambiri ayi, ndipo ichi ndi chimodzi mwa zolakwika zodziwika bwino pakudziyendetsa. Kuyambitsa maothandizira angapo pamodzi kumapangitsa kuti zotsatira zake zisamasuliridwe: ngati zinthu zikuyenda bwino, simungadziwe kuti ndi kusintha kotani komwe kudachitika, ndipo ngati mutachitapo kanthu, simungadziwe choti muyime. Kusintha kumodzi nthawi ndi pang'onopang'ono koma ndizomwe zimapangitsa kuti ndondomekoyi ikhale yowerengeka.
Odwala ambiri amakhala ndi zizindikiro za matenda opatsirana, ndipo matenda amadziwika kuti amayambitsa maselo a mast kudzera mu Toll-like receptors, kotero njira yomwe akufunirayi ndi yovomerezeka. Kaya matenda amayambitsa MCAS sikunakhazikitsidwe, ndipo kuwonetsa zomwe zimayambitsa zochitika paokha ndizovuta. Ponena za Long COVID makamaka, kutenga nawo gawo kwa mast cell ndi lingaliro lokhazikika lomwe lili ndi umboni woyambira, osati kupeza kokhazikika.
Amapezeka kawirikawiri mwa amayi. Estrogen imakhudza machitidwe a mast cell, omwe ndi amodzi omwe akufotokozedwa, ndipo odwala ambiri amafotokoza zizindikiro za cyclical. Kaya kusiyanaku kukuwonetsa kufalikira kwenikweni kapena kusiyana kwa matenda ndi kutumiza sikunathetsedwe kwathunthu - chenjezo lomwe limakhudza matenda angapo omwe amapezeka makamaka mwa amayi.
Ziwerengero zowona za kufalikira sikunapezeke. Mkhalidwewu udadziwika kokha mu 2007, njira zodziwira matenda zimatsutsana, ndipo machitidwe amasiyana pakati pa mayiko. Ziwerengero zosindikizidwa zimasiyana mosiyanasiyana, ndipo m'lifupi mwake ndizomwe mungapeze. Musakayikire ziwerengero zotsimikizika za kufalikira kuchokera kulikonse.
Ayi, ndipo kuyesa kutero nthawi zambiri kumakhala kopanda phindu. Mindandanda yosindikizidwa ikuyamba kulingalira kuti muyesere zolemba zanu, osati malamulo omwe amagwira ntchito mofanana - kusiyana kwa munthu payekha kumakhala kwakukulu, ndipo odwala ambiri amalekerera zinthu zomwe zimawoneka pamndandanda uliwonse. Chifukwa choyambitsa kuchulukirachulukira, kutsitsimuka komanso kuchuluka kwathunthu nthawi zambiri kumakhala kofunikira kuposa chakudya chilichonse. Gwirani ntchito kuti mukhale ndi malire ochepa omwe amawongolera.
Mbiri ya zizindikiro zokonzedwa ndi ziwalo m'malo motsatira nthawi, zomwe zimapangitsa kuti ma multisystem awonekere nthawi yomweyo. Diary yokhala ndi zizindikiro zosachepera milungu iwiri. Zotsatira zonse zoyesedwa m'mbuyomu, kuphatikiza zodziwika bwino, chifukwa izi ndi gawo la njira yochotsera. Mndandanda wamakono wamankhwala ndi zowonjezera. Ndipo mndandanda wachidule wamafunso anu - nthawi yoikidwiratu ndi yachidule ndipo ndikosavuta kuchoka osafunsa zomwe zinali zofunika kwambiri.
Maphunziro Owonjezera Zida
Zoyambira zosankhidwa kuti muwerenge mozama. Maulalo enaake akupangidwa ndipo awonjezedwa apa.
Otsogolera Odwala
Maupangiri opezeka olembera odwala ndi mabanja, oyenera kugawana ndi anthu omwe ali ndi vutoli.
- Wotsogolera wodwala - ulalo kuti uwonjezedwe
- Paketi yodziwika kumene - ulalo kuti uwonjezedwe
- Chizindikiro chosindikizidwa ndikuyambitsa diary template - ulalo kuti uwonjezedwe
- Wotsogolera wodwala - ulalo kuti uwonjezedwe
Malangizo a Zamankhwala
Mawu ogwirizana ndi malangizo azachipatala opangira akatswiri azachipatala. Zothandiza kubweretsa mapointi.
- Malangizo kwa achibale ndi osamalira - ulalo woti muwonjezedwe
- Specialist Society Clinical Guide - ulalo kuti uwonjezedwe
- Kuyesa kwa mkhalapakati ndi protocol yosamalira zitsanzo - ulalo kuti uwonjezedwe
- Consensus diagnostic criteria - mawu ndi ulalo wowonjezera
Mapepala Ofufuza
Zolemba zoyambirira. Ngati n'kotheka, sankhani malo owunikiridwa ndi anzanu ndi masiku osindikiza, pamene gawoli likuyenda.
- Mapepala odziwika bwino (2007 mtsogolo) - mawu oti awonjezedwe
- Mapepala ovomerezeka a Consensus (2010, 2012) - mawu oti awonjezedwe
- Kafukufuku wa hereditary alpha tryptasemia - mawu oti awonjezedwe
- Ndemanga zamakhalidwe ogwirizana - mawu oti awonjezedwe
Mabungwe Aukadaulo
Magulu apadera ndi mabungwe ophunzira omwe amagwira ntchito pa matenda a mast cell.
- Ndemanga zamakhalidwe ogwirizana - mawu oti awonjezedwe
- Maukonde ofufuza matenda a Mast cell - maulalo kuwonjezeredwa
- Mabungwe osowa matenda - maulalo kuwonjezeredwa
Mabuku
Kuwerenga kwanthawi yayitali kwa odwala ndi asing'anga.
- Mabungwe osowa matenda - maulalo kuwonjezeredwa
- Zolemba zachipatala - kuti iwonjezedwe
Kuwerenga kwanthawi yayitali kwa odwala ndi asing'anga.
Nkhani zojambulidwa, zofotokozera, ndi nkhani za msonkhano.
- Zolemba zachipatala - kuti iwonjezedwe
- Maphunziro apadera amsonkhano - maulalo kuwonjezeredwa
- Tsatanetsatane wamaphunziro a MCAS - maulalo kuwonjezeredwa
Mabungwe Othandizira
Thandizo la anzawo, kulengeza, ndi anthu ammudzi.
- Tsatanetsatane wamaphunziro a MCAS - maulalo kuwonjezeredwa
- Magulu othandizira anzawo - maulalo kuwonjezeredwa
- Magulu othandizira a zigawo ndi dziko lonse - maulalo kuwonjezeredwa
Mayesero Achipatala
Maphunziro omwe akulembedwa pano. Kupezeka kwa mayesero kumasintha kawirikawiri, choncho yang'anani mndandanda wa registry mwachindunji.
- Magulu othandizira a zigawo ndi dziko lonse - maulalo kuwonjezeredwa
- Panopa akulembera maphunziro a mast cell - maulalo kuwonjezeredwa
- Malangizo pa zomwe kutenga nawo gawo pamayesero kumakhudzanso - ulalo kuti uwonjezedwe
Panopa akulembera maphunziro a mast cell - maulalo kuwonjezeredwa
Mfundo zofunika kwambiri za bukhuli, zofupikitsidwa.
- MCAS ndi vuto la mast cell osayenera kuyambitsa, osati nambala ya mast cell - maselo ndi abwino, malire awo sali.
- Mfundo zofunika kwambiri za bukhuli, zofupikitsidwa.
- Mwachidule
- Oyimira pakati amamasulidwa m'mafunde: opangidwa kale mumasekondi, oyimira lipid mumphindi, ma cytokines opitilira maola. Chochitika chimodzi chotsegula chikhoza kutenga tsiku limodzi.
- Maselo a mast ndi maselo othandiza chitetezo m'thupi omwe ali pafupi ndi zotchinga za thupi, akugwira oyimira omwe adapangidwa kale okonzeka kumasulidwa mkati mwa masekondi.
- Zizindikiro ndi episodic, zobwerezabwereza, ndipo zimaphatikizapo ziwalo ziwiri kapena zingapo - chitsanzocho, osati chizindikiro chilichonse, ndi chomwe chimasonyeza matenda.
- Oyimira pakati amamasulidwa m'mafunde: opangidwa kale mumasekondi, oyimira lipid mumphindi, ma cytokines opitilira maola. Chochitika chimodzi chotsegula chikhoza kutenga tsiku.
- Histamine ndi mkhalapakati m'modzi mwa ambiri, ndichifukwa chake antihistamines nthawi zambiri amapereka pang'onopang'ono m'malo mopumula kwathunthu.
- Zoyambitsa stack. Cumulative load imafotokoza zochitika zomwe zimawoneka mwachisawawa, ndipo ndi lingaliro limodzi lothandiza kwambiri pakuwongolera tsiku ndi tsiku.
- Zoyambitsa thupi - kutentha, kuzizira, kupanikizika, kulimbitsa thupi - sizifuna kulowetsedwa ndipo siziwoneka ndi kuyezetsa koyenera kwa ziwengo.
- Kukhudzidwa kwa mast cell ndi kukhazikitsidwa matenda urticaria ndi mphumu; mayanjano ndi POTS, hEDS, IBS ndi Long COVID amanenedwa koma osathetsedwa.
- Mayeso ambiri oyipa amkhalapakati amawonetsa zovuta zanthawi ndi kasamalidwe ka zitsanzo m'malo mopanda matenda.
- Njira zodziwira matenda zimakhalabe zotsutsana, kotero akatswiri odziwa bwino amatha kufika paziganizo zosiyanasiyana za wodwala yemweyo.
- Kumene chiwopsezo cha anaphylaxis chikugwira ntchito, epinephrine ndi ndondomeko yolembedwa ndizofunikira; antihistamines si cholowa m'malo.
- Palibe chifukwa cha MCAS chomwe chakhazikitsidwa - perekani 'zoyambitsa' zodzidalira mokayikira.
Kafotokozedwe ka mawu
Mawu 91 ogwiritsidwa ntchito m'mabuku a MCAS ndi zokambirana zachipatala, zofotokozedwa momveka bwino.