
deerghakaalik baar-baar honey vaalaa multifocal austiomyelitis

deerghakaalik aavarti multifocal austiomyelitis (see. aar. aim. oh.) ack durlabh sthiti haye (1:1,000,000), jisamen haddiyon mein ghaav, soojan aur dard hotaa haye. ise "multifocal" kahaa jaataa haye kyonki yeh shareer key vibhinn hisson mein, mukhya roop seey haddiyon mein, aur "austiomyelitis" mein dikhaai dey sakata haye kyonki yeh usa bimari key samaan haye, halanki kramo binaa kissy sankraman key dikhaai detaa haye.
kramo kii paribhasha vikasit hoe rahee haye. kaee doctors aur lekhon ney kramo koo ack autoimmune bimari key roop mein varnit kiya haye jisamen austiomyelitis key samaan lakshan hain, lekin sankraman key binaa. kuchh doctors ney socha kii kramo saifo syndrome seey sambandhit thaa. shodh abb kramo koo ack viraasat mein millie autoinflammatory bimari key roop mein vargeekrit kartaa haye, lekin abhee taka isake liye jimmedaar sateek jeane yaa anya karanon koo alag naheen kiya gayaa haye.
lakshan aur sanket.
lakshnon mein huddy aur jodon kaa dard, twacha kii laali yaa soojan, soojan aantr roga, psoriasis aur hatheliyon aur/yaa pairon key talavon parr fafole jaise ghaav shamil hoe sakate hain.
kaaran.
kuchh visheshagyon kaa maanana haye kii unhone marker dee18aiss60 key ack durlabh elil key saath kramo key beach ack kadee kii khoj kii haye, jisake parinamswarup 18 kaa ack haplotype saapeksh jokhim (hrr) haye. anya visheshagyon ney paaya kii elpin2 mein utparivartan puraane aavarti multifocal austiomyelitis key syndrome roop kaa kaaran banata haye jise maizeed syndrome key roop mein jaanaa jaataa haye, jabaki pstp2 mein utparivartan vikaar key murine roop kaa kaaran banata haye. deerghakaalik aavarti multifocal austiomyelitis key kaaran mein elpin2 aur pstp2, pstp2 key maanav homolog dwara nibhai gaee bhoomikaaen anishchit hain. peshevar siddhant viraasat mein mille jeane kii disha mein aage badh rahey hain.
nidan.
see. aar. aim. oh./see. aine. oh. bahishkaran kaa nidan haye. iska matlab haye kii nidan karane seey pahale anya beemariyon koo door kiya jaanaa chaahiye. aam taur parr, kaee parikshanon kii aavashyakta hoti haye, jaise kii rakt pareekshan, aiks-ray, huddy scan, aim. aar. aaee. aur aksar huddy biopsy.
vargeekaran.
isaki soojan prakriti, isaki baar-baar honey vaali bhadak aur kissy bhee gyaat rogjanak kii kamee key kaaran, kramo koo ack autoinflammatory roga key roop mein punah vargeekrit kiya gayaa haye. iss vishesh vargeekaran mein vanshaanugat prakaar (parivarik bhoomadhyasagariya bukhaar, mavelonet kinnez kii kamee, tii. aine. aff. receptor associated periodic syndrome, cryopyrin-associated periodic syndrome, blau syndrome, pyogenic sterile arthuritis, pioderma gangrenosum aur acney syndrome, kramo) aur multifactorial vikaar (krohan aur behet roga) donon shamil hain. kramo koo abb ack autoimmune naheen maanaa jaataa haye, balki ack viraasat mein millie, autoinflammatory bimari maanaa jaataa haye.
upchaar.
see. aar. aim. oh./see. aine. oh. kaa ilaaj aam taur parr ack visheshagya doctor (baal roga sandhi roga visheshagya) dwara kiya jaataa haye, jise see. aar. aim. oh./see. aine. oh. key rogiyon key saath anubhav hotaa haye.
see. aar. aim. oh./see. aine. oh. key upchaar key lakshyon mein shamil hainh
pratyek rogi key liye see. aar. aim. oh./see. aine. oh. alag-alag hotaa haye. harr bachcha harr upchaar kaa jawab naheen detaa haye. aapke doctor koo aapke bachche key liye kaam karane vaali dawa khojane seey pahale kaee davaaon koo aazmaane kii aavashyakta hoe sakati haye. gambhir mamalon mein, doctor bimari key ilaaj key liye davaaon koo jodd sakate hain. aapka doctor aapke aur aapke bachche key saath kaam karega taaki sabse achchha ilaaj khojane mein madad mill sakey. kuchh see. aar. aim. oh./see. aine. oh. rogiyon key liye, roga koo gair-steroidal anty-inflammatory davaaon (aine. aiss. aeye. dee. aiss.) key saath prabandhit kiya jaa sakata haye. nsaids pahali pankti kaa upchaar haye. haalaanki, yadi nsaid prabhaavi naheen hain, yaa yadi aapka bachcha nsaid koo achchi tarah seey bardasht naheen kartaa haye, tou doosari pankti key upchaar upalabdh hain.
pahali pankti key upchaaron mein naproxon (alev), selecoxib (celebreaks) meloxicum (mobic), pyroxicum (felden), indomethacin (indocin), diclophenac (voltaren) shamil hain.
doosari pankti key upchaaron mein corticosteroids (prednisone/prednisolone), methotrexet (ottrexup, rasuvo, trexal), salfaasaalaazin (azalfideen), pamidronet (aradia), zoladronic acid (zometa), adalymimab (humira), etaner (enbrail), infliximab (remicade) shamil hain.
inn davaaon kaa upayog anya soojan aur/yaa huddy kii sthiti vaale bachchon mein bhee kiya jaataa haye. inn davaaon koo lete samay dushprabhaav hoe sakate hain.
purvanuman.
roga kaa purvanuman aapke bachche kii vyaktigat bimari aur upchaar key prati pratikriya parr nirbhar karega. apane bachche key baal roga sandhi roga visheshagya key saath roga kaa nidan karane key baare mein charcha karna sabse achchha haye.
mahamari vigyaan.
ack baar see. aar. aim. oh. koo bachpan kii bimari maanaa jaataa thaa, lekin vayaskon koo iska pataa chalaa haye. prabhaavit kii ayu 4 seey 14 varsh taka hoti haye, jisamen 10 ausat ayu hoti haye. jaisa kii oopar kahaa gayaa haye, kramo 1:1,000,000 hotaa haye aur mukhya roop seey 5:1 anupaat vaali ladkiyon mein hotaa haye. iska matlab haye kii 60 laakh mein seey shayed 5 ladakiyan aur 1 ladka iss sthiti mein hoga.
majeed syndrome.
maizeed syndrome ack autoinflammatory vikaar haye jisamen kramo, janmajaat pechish, anaemia aur neutrophilic dermatosis shamil hain. aaj taka, majeed syndrome vaale dou asambandhit parivaaron kii soochana millie haye. donon parivaaron mein ell. pee. aine. 2 mein utparivartan paae gaye hain. yahaan humm ack nae utparivartan key saath majeed syndrome key saath ack teesare raktavaahika parivaar kii report karate hain. rogi, ack 3 varsheeya arabi ladki, koo hepatosplanomagely aur anaemia navjat key roop mein thaa. 15 maheene kii umr mein, unhein bukhaar aur multifocal austiomyelitis key baar-baar honey vaale episode vikasit hue. isake alawa, asthi majja aspiration ney mahatvapurna pechish (doshpurna laal koshika gathan) kaa pradarshan kiya, joe maizeed syndrome kaa sujhaav detaa haye. rogi aur usaki maan mein coding anukram aur ell. pee. aine. 2 key splies saiton koo anukramit kiya gayaa thaa. rogi mein exon 17 (c.2327 + 1g> c) key daataa splies site mein ack samroopata ekal-aadhar parivartan kaa pataa chalaa thaa; usaki maan iss site parr vishamta thee. yeye detta maizeed syndrome key kaaran mein elpin2 utparivartan kii bhoomika kii pushti karate hain.
janmajaat pechish, joe kii ack baar phir seey honey vaalaa raktalpata aur deerghakaalik multifocal austiomyelitis, agyaat kaaran kii asamanya bachpan kii beemaariyaan, teen bachchon (dou bhaaiyon aur ack mahila chachere bhaai) mein hui. unake maataa-pitaa raktavaahika vaale hain, aur unaki bimari kaa naidaanik pathyakram samaan thaa. donon bhaaiyon koo bhee sweet syndrome thaa. iss parivaar mein deerghakaalik aavarti multifocal austiomyelitis aur janmajaat pechishjanit anaemia key saath mithaai syndrome kaa sambandh bataata haye kii yeye durlabh sthitiyaan paraspar sambandhit hoe sakati hain.