Matenda osokoneza bongo amachititsa kuti magazi aziwotchera
Granulomatosis ndi polyangiitis (GPA), yomwe imadziwika kwambiri monga granulomatosis ya Wegener, ndi matenda osadziwika bwino omwe amachititsa kutupa kwa mitsempha m'magazi osiyanasiyana.
Zimayambitsa
Monga momwe zilili ndi matenda onse, GPA imadziwika ndi chitetezo cha m'thupi chitatha awry. Pazifukwa zosadziwika, thupi likhoza kulakwitsa kuzindikira minofu yeniyeni m'mitsempha ya magazi.
Pofuna kukhala ndi vuto lodziwika, maselo a chitetezo amatha kuyendetsa maselo ndikupanga nodule wolimba wotchedwa granuloma .
Mapangidwe a granulomas angapangitse kukula kwa kutupa kosaneneka mitsempha ya magazi (yomwe imadziwika kuti vasculitis ). Pakapita nthawi, izi zingathe kufooketsa ziwiyazo ndikuzipangitsa kuti ziphuphuke, kawirikawiri pa malo a kukula kwa granulomatous. Zikhozanso kuyambitsa mitsempha ya magazi kuti ikhale yolimba komanso yopapatiza, kudula magazi ku ziwalo zazikulu za thupi.
GPA imakhudza kwambiri mitsempha yaing'ono yazing'ono. Ngakhale kuti mapepala, mapapu, ndi impso ndizo zikuluzikulu zowonongeka, GPA ingasokonezenso khungu, ziwalo, ndi manjenje. Mtima, ubongo, ndi m'mimba zimakhudzidwa kawirikawiri.
GPA imakhudza amuna ndi akazi mofanana, makamaka pakati pa zaka makumi anayi ndi makumi asanu ndi limodzi. Zili ngati matenda osadziwika bwino omwe amakhala ndi chiwerengero cha anthu 10 miliyoni okha.
Zizindikiro Zoyambirira ndi Zizindikiro
Zizindikiro za GPA zimasiyanasiyana chifukwa cha kutupa kwakukulu. Kumayambiriro kwa matenda, zizindikirozi zimakhala zosavuta komanso zosadziwika bwino monga mphuno yamphongo, ululu wamkati, kupopera, ndi kupuma kwa msana.
Komabe, pamene matendawa akufalikira, zizindikiro zina zikuluzikulu zikhoza kukula, monga:
- Kutaya thupi
- Kutopa
- Kutaya njala
- Fever
- Magazi a m'mphuno
- Mazunzo pachifuwa (kapena popanda kupuma pang'ono)
- Kupweteka kwakumutu kwa pakati
Chikhalidwe chonse cha zizindikirozi nthawi zambiri zimakhala zovuta kuti munthu adziwe matenda. Si zachilendo, mwachitsanzo, kuti GPA isadziŵe bwino ndi kuchitidwa ngati matenda opuma. Ndizopokha ngati madokotala sangapeze umboni wa tizilombo kapena tizilombo toyambitsa matenda omwe angayambe kufufuza, makamaka ngati pali umboni wa misala.
Zizindikiro Zooneka
Monga matenda owonongeka, GPA ikhoza kuvulaza imodzi kapena ziwalo zingapo nthawi yomweyo. Ngakhale kuti zizindikiro zimakhala zosiyana, chomwe chimayambitsa matenda (vasculitis) chimatha kupita kuchipatala kuti chidziwitse ngati pali ziwalo zingapo.
Zizindikiro zodabwitsa za GPA zingaphatikizepo:
- Kugwa kwa mlatho wamphuno chifukwa cha septum perforated (yomwe imatchedwanso "mphuno yachisiti" yotayika yofanana ndi yomwe imawonedwa ndi ntchito yayitali ya cocaine)
- Kuwonongeka kwa dzino chifukwa cha kuwonongeka kwa mafupa
- Kupweteka kwakumva kwapadera kumayambitsa kuwonongeka kwa khutu lamkati
- Kukula kwa kukula kwa granulomatous mu mbali za diso
- Liwu limasintha chifukwa cha kuchepa kwa trachea
- Magazini mumtsuko ( hematuria )
- Kuthamanga kwa impso mwamsanga kumatsogolera ku impso kulephera
- Chifuwa ndi mitsempha yamagazi chifukwa cha mapangidwe a zilonda zam'mimba m'mapapu
- Matenda a nyamakazi (omwe poyamba amapezeka ngati nyamakazi ya nyamakazi )
- Kukula kwa zikopa zofiira kapena zofiirira pa khungu ( purpura )
- Kuwongolera, kumangirira, kapena kutentha komwe kunayambitsa kuwonongeka kwa mitsempha ( ubongo )
Njira Zogwiritsira Ntchito
Kuzindikira kwa GPA kumangopangidwa pambuyo pa zizindikiro zingapo, zosagwirizanitsa zimakhala zosadziwika kwa nthawi yaitali. Ngakhale pali mayeso a magazi omwe angapezeke kuti adziwe matenda omwe amagwiritsidwa ntchito ndi matenda, kukhalapo (kapena kusowa) kwa ma antibodies sikukwanira kutsimikizira (kapena kukana) matenda.
M'malo mwake, matendawa amapangidwa kuchokera ku zizindikiro, ma test lab, X-rays, ndi zotsatira za kufufuza.
Zida zina zingagwiritsidwe pofuna kuthandizira kupeza matenda, kuphatikizapo chiwopsezo cha minofu yokhudzidwa. Chiwopsezo cha m'mapapo nthawi zambiri ndi malo abwino kwambiri kuyamba ngakhale palibe zizindikiro za kupuma. Mitundu yambiri ya kupuma, mosiyana, imakhala yopindulitsa chifukwa 50 peresenti sichisonyeza zizindikiro za granulomas kapena kuwonongeka kwa minofu.
Chimodzimodzinso, chifuwa cha X-ray kapena CT chingawonetsere kuchepa kwa m'mapapo mwa anthu omwe ali ndi mapapu ena osiyana.
Pamodzi, kuphatikiza mayeso ndi zizindikiro zingakhale zokwanira kuthandizira kugonana kwa GPA.
Chithandizo Chamakono
Zaka za m'ma 1970 zisanafike, tizilombo ta Wegener tinkaona kuti pafupifupi kufala kwapadziko lonse, kawirikawiri chifukwa cholephera kupuma kapena uremia (chikhalidwe chomwe chimakhudzana ndi mankhwala osokoneza bongo m'magazi).
Zaka zaposachedwapa, kuphatikizapo mankhwala a dose corticosteroid ndi mankhwala osokoneza chitetezo cha chitetezo cha mthupi zatsimikiziranso kuti zowonjezereka zimapindula mu 75 peresenti ya milandu.
Pochita khama kuchepetsa kutupa ndi corticosteroids ndikuwongolera njira yodzimvera yokhayokha ndi mankhwala osokoneza bongo monga cyclophosphamide, anthu ambiri omwe ali ndi GPA akhoza kukhala moyo wathanzi, wathanzi ndikukhalabe okhululukidwa kwa zaka 20 kapena kuposerapo.
Pambuyo pa mankhwala oyamba, mankhwala a corticosteroid amachepetsedwa ngati matendawa akuyendetsedwa. Nthawi zina, mankhwalawa amaletsedwa.
Cyclophosphamide, mosiyana, kawirikawiri imayikidwa kwa miyezi itatu kapena sikisi ndikusintha kupita ku china, chochepa kwambiri cha mankhwala osokoneza bongo. Nthawi ya mankhwala ochiritsira ikhoza kusiyana koma kawirikawiri imatha chaka chimodzi kapena ziwiri musanayambe kusintha kusintha kwa mlingo.
Kwa anthu omwe ali ndi matenda oopsa, ena, machitidwe okhwima angapangidwe, kuphatikizapo:
- Mankhwala apamwamba opanga mankhwala
- Kusinthanitsa kwa plasma (kumene magazi amagawanika kuti achotse autoantibodies)
- Kupatsirana kwa impso
Kuthamangitsani
Ngakhale kuti anthu ambiri amatha kubwezeretsedwa, pafupifupi 50 peresenti ya mankhwalawa amatha kubwerera m'mbuyo. Komanso, anthu omwe ali ndi GPA ali pachiopsezo cha mavuto aakulu a nthawi yaitali, kuphatikizapo kuperewera kwa impso, kumva kumva, ndi kumva. Njira yabwino yopewera izi ndikuti muzifufuza nthawi zonse ndi dokotala komanso nthawi zonse zoyezetsa magazi ndi zojambula.
Ndi kasamalidwe kabwino ka matendawa, 80 peresenti ya odwala odwala bwino adzakhala ndi moyo zaka zisanu ndi zitatu. Matenda atsopano opatsirana ndi anti-antibody komanso deriicillin -derivative monga CellCept (mycophenolate mofetil) angapangitse patsogolo zotsatira zake m'zaka zikubwerazi.
> Zotsatira:
> Almouhawis, H ;; Leao, J .; Fedele, S. ndi Porter, S. "Wegener's granulomatosis: ndondomeko ya zochitika zachipatala komanso ndondomeko yowunika ndikuchiritsidwa." Lembani Mlomo Njira Zamankhwala. 2013; 42: 507-516.
> Fortin, P .; Tejani, A .; Bassett, K ;; ndi Musini, V. "Kutenga immunoglobulin mophatikizapo mankhwala ochiritsira a wegener's granulomatosis." Cochrane Data Syst Rev. 2013; 1: DOI: 10.1002 / 14651858.CD007057.pub3
> Silva, S .; Ma specks, U .; Kaira, S. et al. "" Mycophenolate Mofetil ya Induction ndi Kusungirako Kukhululukidwa mu Microscopic Polyangiitis ndi Wowongoka Kwambiri Kuphatikizika Kwa Mphindi-Mlandu Woyenera, Wowonongeka Woyendetsera Galimoto. " Chipatala J Am Soc Nephrol. 2010; 5 (3): 445-453.