
snitzler syndrome

snitzler syndrome yaa snitzler syndrome ack durlabh bimari haye jisaki visheshta purani pitti (articaria) aur aavadhik bukhaar, huddy dard aur jodon mein dard (kabhi-kabhi jodon kii soojan key saath), vajan mein kamee, aswasthata, thakan, soojan lymph granthiyon aur badhee hui pliha aur yakrit kii madhya ayu key aspas shuru hoti haye.
schnitzler syndrome koo ack autoinflammatory vikaar maanaa jaataa haye aur aamtaur parr anakinra key saath ilaaj kiya jaataa haye, joe interleukin 1 koo rokta haye. yeh upchaar sthiti koo niyantrit kartaa haye lekin iska ilaaj naheen kartaa haye. lagbhag 15 pratishat logon mein jatilataaen vikasit hoti hain, lekin sthiti aam taur parr jeevan koo chhota naheen karati haye.
vargeekaran.
snitzler syndrome ack dair seey shuru honey vaalaa autoinflammatory vikaar haye.
sanket aur lakshan.
samanya shuruat lagbhag 55 varsh kii ayu mein hoti haye, aur lakshan baar-baar honey vaale pitti hotey hain, jyaadaatar dhad aur angon parr, aksar baar-baar bukhaar, jodon mein dard, huddy mein dard, maanspeshiyon mein dard, sirdard, thakan aur vajan mein kamee key saath.
kaaran.
2017 taka bimari kaa kaaran naheen samajha gayaa thaa.
nidan.
rakt pareekshan vishisht gaama-globulin (monoclonal gamopathy) kii uchch sandrata koo darshaate hain. isamen lagbhag hamesha κ-prakaar kii halki shrinkhalaaen hoti hain. ack prakaar jisamen igg uthaaya gayaa haye, kaa varnan kiya gayaa haye, joe ack-dasvaan samanya prateet hotaa haye. immunoglobulin mootra mein bains johns protein key roop mein dikhaai dey sakate hain. soojan key sanket aksar maujood hotey hainh inmein shwet rakt koshika kii sankhya mein vriddhi (leukocytosis) aur erythrocyte avasaadan darr aur see-pratikriyaasheel protein shamil hain. purani bimari kaa anaemia hoe sakata haye. huddy kii asaamaanyataon koo radiological imaging (aksar ghanatva mein vriddhi yaa austiosclerosis) yaa biopsy parr dekhaa jaa sakata haye.
naidaanik maandandon key dou sett hain, 2001 mein prakaashit lipaskar mandand aur 2012 mein usa shahar mein ack baithak mein utpaadit kiye gaye strasberg mandand.
honthon key mandand key liye pitti, monoclonal iggam kii upasthiti aur nimnalikhit mein seey cumm seey cumm 2 kii aavashyakta hoti haih bukhaar, jodon kaa dard yaa gathiya, huddy kaa dard, sooje hue lymph nodes, badhee hui pliha yaa yakrit, badhee hui erythrocyte avasaadan darr, shwet rakt koshikaon kaa uchch star, aur huddy kii imaging mein samasyaon key nishkarsh.
strasberg mandand mein, vyakti key paas pitti aur monoclonal iggam yaa igg kii upasthiti honey chaahiye. snitzler kaa nidan taba kiya jaataa haye jaba vyakti koo nimn mein seey dou, yaa nimnalikhit mein seey teen, yaa dou, yaa teen, baar-baar bukhaar, huddy kii imaging mein asamanyata, huddy key dard key saath yaa binaa, twacha biopsy mein neutrophil ghuspaith key nishkarsh, shwet rakt koshikaon key uchch star yaa see-pratikriyaasheel protein.
anya sthitiyaan joe aavadhik bukhaar, paraprotein yaa puraane pitti kaa kaaran bunn sakati hain jinhen khariz curr diyaa jaanaa chaahiye, unamen shamil hain (aur inhin taka seemit naheen hain) autoimmune yaa autoinflammatory vikaar jaise kii vayask-shuruat sthir roga, angioadema, lymphoma yaa anirdhaarit mahatva (aim. jee. aiss.) kii monoclonal gamopathy jaise hematological vikaar, pitti key anya kaaran, cryoglobulinemia, mastocytosis, chronic neonatal shuruat bahu-pranali soojan roga yaa koyal-wells syndrome.
upchaar.
2017 taka, snitzler key ilaaj key liye koee dawa sweekrit naheen kii gaee thee. 2005 mein ubhare jaane key baad seey interleukin 1 gatividhi koo rokane vaali davaaen pasandida upchaar rahee hain; 2012 seey ack sarvasammati dishanirdesh ney anakinra key saath upchaar kii sifarish kii haye. corticosteroids, cyclooxygenase inhibitors, interferon alfa jaisi immunospressant davaaen prabhaavi hoe sakati hain. 2020 kii ack sameeksha mein bataaya gayaa kii canakinumb "snitzler syndrome key rogiyon mein anukool suraksha profile key saath ack prabhaavi deerghakaalik upchaar thaa.
juuna 2018 mein aine. aich. aiss. ingland ney ack "naidaanik commissioning neetih aavadhik bukhaar aur autoinflammatory vikaaron (sabhi umr) key ilaaj key liye anakinra" prakaashit kii, jisamen kahaa gayaa kii "anakinra
snitzler syndrome key pralekhit nidan vaale rogiyon mein pahali pankti key upchaar key roop mein upayog kiya jaa sakata haye.
kyonki anakinra schnitzler syndrome kii poorna maafi koo prerit karane key liye bahut adhik aur teji seey prabhaavi haye, yeh sujhaav diyaa gayaa haye kii joe rogi anakinra kaa jawab naheen dete hain, unamen nidan parr punarvichaar kiya jaanaa chaahiye. anakinra upchaaraatmak naheen haye, haalaanki; upchaar bund honey key turant baad lakshan phir seey dikhaai dete hain.
parinam.
aam taur parr anakinra key saath upchaar prakop koo rokta haye lekin yadi upchaar bund curr diyaa jaataa haye tou way phir seey shuru hoe jaate hain. lagbhag 15-20% logon mein, ack jatilata key roop mein ack lymphoproliferative vikaar, aamtaur parr waldenstrom kaa macroglobulinamia, vikasit hotaa haye. snitzler syndrome vaale logon mein bhee aeye. aeye. amyeloidosis kii soochana millie haye.
snitzler syndrome vaale rogiyon mein jeevan avadhi samanya aabaadi seey bahut alag naheen dikhaai gaee haye.
mahamari vigyaan.
yeh ack durlabh sthiti haye; sitambar 2014 taka, 281 mamle darj kiye gaye they aur 2017 taka lagbhag 300 mamle darj kiye gaye they.
itihas.
iss bimari kaa naam francisi twacha visheshagya lillion schnitzler key naam parr rakhaa gayaa haye, jinhone pahali baar 1972 mein iss syndrome kaa varnan kiya thaa. auto-inflammatory rogon key vargeekaran aur paribhasha parr ack delphi adhyayan, 2018 mein prakaashit, jise vaikalpik naam maanaa jaataa haye "dair seey shuru honey vaali gamopathy key saath baar-baar eurticaria aur bukhaar" lekin ise bahut cumm samarthan milaa.

