kishore dermatomyocytis

kishore dermatomyocytis (jeey. dii. emm.) hii eka idiopathic dahak myopathy (aay. emm. emm.) aahe jee svayampratikarshaktichya apkriyachi kalpana aahe, jyamule itar guntaguntinmadhye snayu kamkuvat hotat. heey mulanmadhye prakat hote; heey dermatomyocytische balrogatadnya pratirup aahe. jeey. dii. emm. madhye, sharirachi rogpratikarak shakti sampoorn shariratil raktavahinyanvar hallah karate, jyamule vasculitis navachi jaljal hote. sanyukt rajyanmadhye, jeey. dii. emm. ess. chey praman darr varshi prati dashalaksh mulanmage andaje 2-3 prakarane aahe. vanshik gatanmadhye kahee farak asalelya, yuu. keey. madheel ghatana darvarshi prati dashalaksh mulanmadhye 2-3 darmyan asalyache manale jaate. ling gunottar (strih purush) andaje 2:1 aahe. itar idiopathic dahak myopathymadhye samavisht aahe; kishore polymyocytis (pii. emm.), joou durmil aahe aanee praudhanpramane mulanmadhye titka samanya nahee.
chinhe aanee lakshane.
jeey. dii. emm. ess. mule honara vasculitis pramukhyane done prakare prakat hotoh
eka mhanje eka vishisht pural. pural anekada chehara, papanya aanee hatanvar aanee kadhikadhi sandhyanchya varchya tvachevar, jyat taach, gudghe, kopar ii. samavisht asatat, parinam karatat. puralancha rung gulabi jambhala asato aanee tyala heliotrope mhanatat (andaje yach rangachya tyach navachya fulavaroon). haat aanee aanee pural heey ellergy, isab, pachva rog kinwa tvachechya itar samanya sthitisarkhech asatat, parantu heliotrope rung haah jeey. dii. emm. chya dahak prakriyesathi adviteey asato. kahee mulanna calcinosis hoto, jeey tvachekhali calciumche sathe asatat. pural haah rogachya navachya "dermato-" bhagacha srot aahe.
snayu daah heey vasculayatisamule honare dusare lakshan aahe. heey lakshan rogachya navachya '-myocytis' bhagacha srot aahe ('mayo' = snayu, '-etis' = daah). snayunchya jaljalamule snayu kamkuvat hotat, jyamule thakwa, anakalaniyata, samvayaskanshi sharirik sambandh naan thevane aanee akheris akheris chadhane, vastoo uchalane aanee itar hatane keleli kaame karane yasarkhi kaame karnyas asamarthata yeu shakate. itar lakshananmadhye padane, dysphonia kinwa dysphagea yancha samavesh asoo shakato. snayu kamkuvat jhalyamule anekada snayukshay kinwa itar snayu rogache vaidyakeey chukiche nidan hote. kahee rugnanmadhye aakunchan hote, jevha snayu lahan hotat aanee sandhe vakatat; vyayam, vyavsayik upchar aanee sharirik upchar heey talhu shakatat. pratham prabhavit snayu jawalche asatat (i.eae.maan, khande, paath aanee pooat). jeey. dii. emm. asalelya sumare nimmya mulanna tyanchya snayunmadhye dekhil vedna hotat.
itar lakshananmadhye chidchid, vajan qamee hone aanee tondache vran yancha samavesh asoo shakato. jevha mool chidkhor hote, thakale asate, samajikaran karnyas nakhush hote aanee chehara sahajpane swachchh hoto, tevha doctor yaa lakshananchya samoohala "dukkh" mhanoon sambodhatat."
pragati.
jeey. dii. emm. chya pragaticha veg atyant badaloo shakato. jawaljawal serve jeey. dii. emm. rugnanmadhye tvachecha kahee pramanat sahbhag asato. jeey. dii. emm. ess. pural heey sahasa prarambhik lakshan mhanoon udbhavate. kadhikadhi teey itake kirkol asate qee snayunchi lakshane disu lageparyant teey kaay aahe heey olakhale jaau shakat nahee. kahivela snayunchi lakshane kadhich disun yet naheet kinwa mahinyabharat agadi haloohaloo udbhavtat aanee kahivela samanya takdipasoon kahee divasant chalanyas asamarthateparyant jaatat. sahasa, pural suru jhalyanantar aathvade teey mahine snayunchi lakshane disun yetat.
kaaran.
jeey. dii. emm. chey mool kaaran adnyat aahe. itar svayampratikar rog rugnanchya kutumbat chalatat mhanoon tyat bahudha anuvanshik ghatak asato. rog samanyatah rogpratikarak pranalichya kriyakalapanna karanibhoot asalelya sthitimule udbhavato joou pahije tassa thambat nahee, parantu bahutek prakarananmadhye trigger heey jawaljawal nishchitpane kaaran nasate. samanya karananmadhye lasikaran, sansarg, dukhapati aanee suryaprakashat jaljal yancha samavesh hoto.
nidan.
jeey. dii. emm. olakhanyasathi nikatvarti snayu kamkuvat hone, tvachevar vishisht pural aanee snayuche uchch utsechak niyamitpane vaparale jaatat. vishisht chumbakeey anunad imaging aanee snayunchya biopsymadhil badal heey pudhil sarvat upayukt nidan nikash manale jaatat, tyanantar electromeogram, calcinosis, dysphonia aanee nelfold capilaroscopivaryl myopathy badal hotat. itar upayukt nikashanmadhye myocytis-vishisht kinwa sambandhit pratipinde, nelfold capilaroscopy, ghatak VIII-sambandhit pratijan, snayu ultrasound, calcinosis aanee neopterin yancha samavesh hoto. manvi-inn-daad-loop jeey. dii. emm. nidanasathi sanganak sahayyit paddhatinni sudharit achookata darshavili aahe. 
upchar.
ekada jeey. dii. emm. chey nidan jhaale qee, upchar haah sahasa antahshira (puls) steroidscha (methylprednisolone, solu-medroll) 3 divasancha abhyaskram asato, tyanantar anek aathvadyansathi tondawate prednisonecha (samanyatah 1-2 mii. gra./kilo sharirachya vajanacha) uchch doses asato. hii krutee sahasa rog niyantranat aanate, bahutek prayogshaletil chachanya samanya mulyanparyant kinwa tyachya jawal aanate. yaa kalat snayunchya lakshananmadhye kahee kirkol sudharana dekhil disun yeu shakate, parantu samanyatah snayunchi purn takad parat milanyas barach vel lagato.
ekada rogachi prakriya niyantranat aalee qee, tyanche dushparinam qamee karnyasathi tondawate steroids haloohaloo qamee kele jaatat. anekada, methotrexet (chemotherapy aushadh) kinwa itar dii. emm. aar. dii. sarkhi steroid-bachav aushadhe, tondawate steroids qamee honyachi bharpai karnyasathi dilee jaatat. ekada tondawate steroids qamee vishari patalivar aale qee, sodanare ghatak dekhil haloohaloo mage ghetale jaau shakatat. rogachi punaravrutti honar nahee yachee khatri karnyasathi tapering prakriyedarmyan prayogshalechya parinamanvar barkaine laksh thewale jaate.
steroids kinwa kinwa kramankachi aushadhe sahan kelee jaat naheet kinwa aprabhavi aahet asha prakarananmadhye, itar upchar aahet jeey vaparoon pahile jaau shakatat. yamadhye siclosporin, infliximab kinwa itar dii. emm. aar. dii. sarkhya itar chemotherapy aushadhancha samavesh aahe. dusare mhanje intravenous immunoglobulin (ivg), jeey. dii. emm. viruddha khoop prabhavi asalyache darshavile gelele eka rakt utpadan aahe.
tvachevaril puralanvar upchar karnyasathi, hydroxychloroquine (plaquinil) sarkhi maleria-virodhi aushadhe sahasa dilee jaatat. sthanik steroid cream (hydrocartisone) kahee rugnanna madat karoo shakatat aanee dahak-virodhi cream (jase qee tachrolimus) khoop prabhavi siddha hott aahet. puralanmule nirman nirman koradya tvachecha samna sunscreencha niyamit vapar kinwa konatihi moisturising cream lavoon keela jaau shakato. bahutek jeey. dii. emm. rugna suryaprakashat janyabaddal atishay sanvedanshil asatat aanee kahee lokanmadhye suryaprakashamule rog houu shakato, mhanoon uchch-ess. pii. eff. sunscreencha darroj vapar karanyachi shifaras kelee jaate.
purvasoochana.
jeey. dii. emm. chey nidan jhalelya aanee tyanchyavar upchar jhalelya mulanpaiki sumare ardhi mule purnapane burry hotil. rog barah jhalyanantar sumare 30 takke lokanna ashaktapana yeil. bahutek mule maaf hotil aanee done varshant tyanchi aushadhe kadhoon taktil, tarr itaranna pratisad denyas jaast vel lagoo shakato kinwa adhik gambhir lakshane asoo shakatat jee saaf honyas jaast vel ghetat.
jeey. dii. emm. chaa eka samanya chirsthayi parinam mhanje balpanicha sandhivat.

