Zempilo, Diseases and nemibandela
Lupus nephritis: ukuxilongwa, unyango, ukutya
Lupus nephritis yenye iingxaki ezininzi rhoqo systemic lupus zomzimba. Screen - sisifo samathambo okubangelwa kukuvela antibodies emzimbeni, nisamkelela iiproteni "lweenkobe" ngolwasemzini. Ngenxa yoko, ukuphuhlisa ukudumba aseptic kwiindawo ezahlukeneyo zomzimba. Kuquka izintso.
definition
Lupus nephritis, lupus nephritis, okanye - isifo sezintso enzima in systemic lupus zomzimba. Yokwanda kwalo sifo labemi, nge-avareji abantu amane ngamnye amakhulu amawaka abantu. Uninzi abaphethwe abameli kwisiqingatha ezintle phakathi kweminyaka engamashumi amabini ukuya kumashumi amane. Amaninzi, le leengxaki ifumaneka abantu Afro-zeCaribbean.
Isifo ezinokubangelwa ezahlukeneyo nemininzi, ukususela ezi kwezikhumba, ukuya kwizigulo yemfuza, ngoko ke lixesha kubaluleke kakhulu ukuba sinikele ingqalelo iimpawu bonana nogqirha. Ngapha koko, unyango ngokukhawuleza iqale, kokukhona kungcono ALS ubomi yomguli kunye nezempilo.
umyalezo weimeyile
Lupus nephritis nga kuxhokonxwa selangeni ixesha elide rhoqo (uyithandayo solarium okanye ukuhlala kwiindawo enelanga), uxabana amayeza, ntshikilelo ngokusisigxina kunye nokukhulelwa (kwimveku ibonwa nje umzimba angaphandle kunye amajoni omzimba aqale ukuhlasela iiseli unina).
Ukongeza, uphuhliso kwezifo yemfuza ichaphazela likhoboka, ubukho ukungalingani hormone, izifo intsholongwane rhoqo (intsholongwane badityaniswa emzimbeni iseli, esishiya ezisegazini zabo komhlaba kunye bandiqumbise ngaloo impendulo yayo omzimba). Chance yokuphuhlisa sifo emizalwaneni close izihlandlo eziliqela ngaphezulu kwe-avareji ngenxa labemi.
Pathogenesis
Lupus nephritis yinxalenye iliphawu ukuvela kwi systemic lupus zomzimba. Autoantibodies ukuphuhlisa endaweni ukuya DNA lweenkobe kunye uqhagamshelo yayo kunye histones, kwiiproteni kwenkqubo ngumphelelisi kunye cardiolipin kuqala. Isizathu sokuba uhlaselo olunjalo - kuncitshiswa nokunganyamezelwa ezisegazini self, iziphene B kunye lymphocyte T.
enxulumene ngqo kuphuhliso nephritis ekubeni iantigens-unalo izakhiwo utshixelo ukuba nezihlunu ezo tubules sezintso. Xa onjalo zinemoletyhule iproteni encamathele kuyo yeseli ngaphezulu, oko kubangela ukuxubana abasabela kwemichiza ukuba nesiphumo ekuyiyekeni izinto esebenzayo, linyibilike iseli. Oku, ebangela ukusabela kwiintetho ezikralayo, okwenza kukhule kuphela umonakalo.
komzimba bathuthumele
Lupus nephritis e SLE ukuze ibonwa morphological. Ngenxa yokuvulwa izigulane waba utshintsho inwebu le glomeruli kwezintso, icandelo ebonakalayo iiseli, ukwandiswa mesangium, sclerosis semithambo, iziporo nangaphezulu. Ezi mpawu babe zombini omnye kunye glomeruli eziliqela.
Eyona ezithile lupus nephritis yi fibrinoid necrosis capillary noyi ka Henle, kwaye histologically ubonwe karyopyknosis kunye karyorrhexis (ngokwahlukana lysis of nuclei cell). Ukongeza, kukho utshintsho pathognomonic kwi glomerular elingaphantsi inwebu njenge "loops nentambo" kunye nobukho hyaline thrombi kwi mithambo ye emithanjeni ngenxa yokulahlwa kwe nezakhiwo omzimba.
ngezigaba
Ngokwezonyango kunye morphologically amabakala eziliqela elisuke lupus nephritis. Ucalulo of the World Health Organization ngolu hlobo lulandelayo:
- Iklasi lokuqala: glomeruli kuba nesakhiwo eqhelekileyo.
- iklasi yesibini: kukho utshintsho nje kule mesangium.
- iklasi Okwesithathu: glomerulonephritis kunye sisilonda esidumbileyoesingaphakathi of isiqingatha glomeruli.
- grade Okwesine: kwentamo glomerulonephritis.
- grade Okwesihlanu: glomerulonephritis membranous.
- ibanga lesithandathu: sclerosing glomerulonephritis.
Kukho kwakhona kudidi Serova, apho yabela focal, ehlasela, membranous, mesangioproliferative, glomerulonephritis mesangiocapillary kunye fibroplastic.
iimpawu
Membranous lupus nephritis kengoko neempawu uyawabophelela kunye facultative. Enye obubonakala na proteinuria inyanzelekile, oko kukuthi, ubukho iiprothini yokuchama. Kwakhona, ungenza rhoqo ukufumana hematuria, leuco - kunye limfotsituriyu. Ezi iimpawu zibonisa ubukho ukudumba kwi izintso kunokuba nje kuphela SLE.
Kunye ukuqhubela phambili inkqubo samathambo liyenyuka iimpawu ukusilela kwezintso kuboniswa kumanqanaba creatinine egazini kunye nomchamo, ubuthathaka, silale nomonde States soporous.
Ngaba kancinane inkqubela yaye ngokukhawuleza nephritis okuqhubekayo. Ukuba isifo iphuhlisa ngokucotha, kubakho umchamo kunye nephrotic syndrome. Ngaphezu koko, lupus nephritis nga kuqhutywa ngohlobo opholileyo okanye efukamileyo, xa zonke ezo mpawu ekhoyo proteinuria nje ezingephi.
Ngokukhawuleza phambili lupus nephritis sifana kakhulu ukuya glomerulonephritis zakudala. ukusilela kwezintso ikhula ngokukhawuleza, kukho hematuria gross, ukwanda koxinzelelo lwegazi, kwaye nephrotic syndrome. Kwiimeko kakhulu ngayo ukuphuhlisa coagulation zahanjiswa intravascular (DIC).
Lupus nephritis kubantwana
Ngo 1/5 ezigulane systemic lupus zomzimba iimpawu yokuqala ukuvela ebuntwaneni. Xa abantwana abancinane ngaphezu kweminyaka eli-10, oko phantse lungenzeki, kodwa kukho inkcazo yokuba kumatyala SLE emntwaneni kunye iinyanga enesiqingatha ubudala.
Uphuhliso esi sifo kubantwana ayikho yahlukile abadala. Lo mfanekiso kwezonyango zinokohluka: ukusuka nazimpawu ukuya ngokukhawuleza eqhubela phambili. ukusilela Acute kwezintso kunqabile.
Uninzi iimpawu ezahlukahlukeneyo SLE kwi umntwana:
- erythema phezu kobuso;
- irhashalala discoid emzimbeni;
- uvakalelo ukuba kwelanga;
- zizilonda mucosal;
- ukudumba namalungu;
- isifo sezintso;
- yaphula inkqubo luvo;
- ukwanda ukopha;
- izifo ngumzimba;
- ubukho omzimba antinuclear.
Ukuba ekliniki inemisebenzi ubuncinane ezine kule kuluhlu, sitsho ngengqiniseko ukuba umntwana systemic lupus zomzimba. nomonakalo wezintso kubantwana kunqabile kuza kucaca. Ngokuqhelekileyo, iimpawu ekungabonini ukusuka glomerulonephritis ukuya ekubonakalisweni of antiphospholipid syndrome.
Kuqikelelo lwemozulu yophuhliso sifo abantwana abangenabani ngakumbi. Yiminyaka elishumi emva kokuba kuqulunqo dialysis kwesifo kufuna ekhulwini 10 kuphela kwizigulane.
ekuxilongweni
Yintoni enika ugqirha isizathu sokuba ukrokrela lupus nephritis? Uxilongo kudla isekelwe kwidatha lwezonyango kunye elebhu-kuqinisekiswa ezikhoyo SLE:
- Iintlungu kunye nokudumba kwamalunga;
- irhashalala phezu ulusu lobuso ngohlobo ibhabhathane;
- phambi kwimbali effusion em- (unepleurisy, pericarditis);
- umzimba fast, nomkhuhlane.
Uhlalutyo jikelele igazi waphawula anaemia, sinciphise iiplatelets, ukunyuka kweqondo erythrocyte neentlenge, zehla ukuqinisa iiprothini. Kuba uphethwe Kubalulekile ukuchonga antibodies DNA lweenkobe.
Ngokuqhelekileyo, oku ngokwaneleyo ukuze benze une systemic lupus zomzimba kunye, ngenxa yelupus nephritis. Nangona kunjalo, imbonakalo iiprothini umchamo kungalindiswa iminyaka omnye okanye emibini ukususela ekuqaleni kwesi sifo. Kwimeko enjalo, ugqirha ithembele enzyme immunoassay kunye ubhaqo antibodies. Ukuba akukho kungqinwe data elebhu, kuyimfuneko ukuqhubeka uphendlo diagnostic, ingakumbi izigulane kwamadoda, ngenxa nosology zabo, oku kunqabile.
Umahluko lupus nephritis kubaguli SLE kunye endocarditis ziibhakteriya, exacerbations nokoyika isifo samathambo, ezigula ezininzi, kohlobo B, kunye amyloidosis syndrome, uEnoki-Schonlein purpura.
Inqanaba lokuqala zonyango
Unyango yelupus nephritis - yinkqubo ende kwaye usoloko udiniwe ukuba isoloko ihlala ixesha elide. Kuthatha kuqhubeka ngokwezigaba ezibini. Inxaxheba kwinqanaba lokuqala ibekwe exacerbation. Injongo unyango - ukufezekisa uxolelo ezinzileyo, okanye ubuncinane ukunciphisa kubonakaliswa lwezonyango.
Imithi kufuneka ziqale ngokukhawuleza kangangoko kunokwenzeka ukususela ekudalweni loxilongo. Yonke into yenzeka ngokukhawuleza kangangokuba iintsuku ezintlanu ukuya ezisixhenxe ukulibazisa inokuba impazamo obulalayo. Ukuba umsebenzi nkqubo iphantsi (kuya kubonisa titers omzimba), ugqirha kufuneka kuphela ukusetyenziswa corticosteroids ngeedosi eziphezulu isithuba seenyanga ezimbini, kulandele ukuhla okucothayo idosi (yasuka ukurhoxisa eli chiza ayikwazi, angaba bemkhanyela adrenal).
Ukuba kwikhondo ngokukhawuleza kakhulu kwesi sifo, ukongeza steroids, butofelwe elawulwa ngeedosi eziphezulu kakhulu cytostatics. Olu nyango ukubetha kwayo yenziwa phakathi kweenyanga ezintandathu. Kuphela emva kweli thuba angaqala ukunciphisa ithamo iziyobisi, kwaye sidlulisele isigulane ukuba amayeza ngomlomo.
Musa ukulibala ukuba izigulane SLE badla nokuphuhlisa ziyithumele coagulation intravascular, ngoko kucetyiswa ukuba athathe amanyathelo othintelo, ezizezi:
- ukutofelwa igazi kwanangamacandelo alo;
- emthanjeni "Trental";
- Ulawulo ongama iiyunithi 2.5 amawaka "lweeHedfowuni".
isigaba sesibili unyango
Lupus nephritis kwi SLE sesibini isigaba athatha kunye steroids kunye neziyobisi cytotoxic. Kuphela ithamo ukuba zincinane kakhulu. ngokucothayo kakhulu, ngaphezu kweenyanga ezine ukuya kwezintandathu, ithamo "Prednisolone" titrated to milligrams 10 kilogram sobunzima omzimba ngalinye. arhente Cytotoxic yabalula yamisela amathamo bolus kanye qho emva kweenyanga ezintathu, kwaye ukuba nemiba ezintle sifo, koko ugqithele inaliti enye qho emva kweenyanga ezintandathu.
therapy kulondolozo olunjalo kangangeminyaka. Ekuhambeni kwexesha, idityaniswa (ukuba kuyimfuneko) ukuthintelwa yeziphumo ebezingalindelekanga iziyobisi kunye nonyango nomqondiso.
Kodwa esifike ungaluqalanga unyango kwi pesenti ezilishumi elinesihlanu ezigulane basengaba ukusilela kwezintso. Kulo mzekelo, kunokunceda kuphela olugqitha esikhumbeni okanye kidney abohlukeneyo. Ngelishwa, le unyango azifumaneki kuluntu jikelele.
Yokudla lupus nephritis
Ukuze kugcinwe umsebenzi sezintso nezigulane lupus nephritis kumele ukuba zichithe imithetho ethile;
- Sela ezininzi iziselo ukuya ukuhluza kakuhle igazi kunye nokugcina inqanaba emzimbeni.
- Ukutya kufuneka ibe izixa amancinci potassium, iphosphate kunye protein, ekubeni ezi zinto zibe nefuthe elibi nezintso ezonakeleyo.
- Nika imikhuba emibi.
- Yenzani umthambo ekukhanyeni.
- Rhoqo khangela uxinzelelo lwakho lwegazi.
- Nciphisa ukutya okunamafutha.
- Musa ukuthatha NSAIDs kuba nefuthe elibi nezintso.
Ukuba isigulane ilandela ezi zincomo, umgangatho wobomi kakuhle kakhulu, kwaye abantu abane iphile athembisayo.
Outlook
Liqikelele ukuphatha lupus nephritis membranous kuxhomekeke kwindlela uxhelekile izintso kunye nokuqala unyango. I ngaphambili isigulane aye kugqirha, kokukhona ukuba nokwenzeka ukuba ayaphumelela.
Kwiminyaka engamashumi amane eyadlulayo, yaba ligcuntswana kuphela izigulane lupus nephritis abaye baphila ngaphezu konyaka ukusuka isifo. Ngenxa iindlela zale mihla unyango kunye isifo, izigulane unokulindela sempilo seminyaka emihlanu-ngaphezu.
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