
sidi55-nte kuravu

daaph deficiency allengil chapple syndrome ennum vilikkunna sidi55 deficiency rogaprathirodha samvidaanathinte apoorva janithaka vaikalyamaan. chapple ennaal "sii. dii. 55 deficiency vithu hyper-aaktiveshan oof compliment, angiopathic thrombosis, seaviour protein-lossing entaroppathi (pii. ell. iii)" ennivaye soochippikkunnu."eee vaikalyam saadhaaranayaayi kuttikkaalathu prakadamaakukayum jeevanu bheeshaniyaakukayum cheyyum. eee avasthayekkurichu özen, thudangiyavar vivarichu. 2017ll.
adayaalangalum lakshanangalum.
hypoprotenemial nayikkunna kadinamaaya protein nashtappedunna entaroppathi chaappilinte savisheshathayaanu. vayaruvedana, ookkaanam, chharddi, vayarilakkam, vishapp kurayal, sareerabhaaram kurayal, edema enniva lakshanangalil ulppedaam. irumbu, ferritin, kaalsyam, magneeshyam, folate, vitaamin dii, vitaamin bii 12 ennivayude kuravinu kaaranamakunna vittumaaraatha malabsarption aalukalkk undu. chila rogikalkku hypogammaglobulineum bandhapetta aavarthichulla shwaasakosha anubadhakal undakam. eee rogikalil kadinamaaya thrombotic vascular oklooshanum kandethaam.
janithakashasthram.
compliment regulator sidi55 jeeninte mutationukal moolamaanu chapple syndrome undaakunnath, ithu protein expression nashtappedunnathilekku nayikkunnu.
anantharaavakaasham.
chapple syndrome praadhamikamaayi autosomal resussive reethiyilaan paarambaryamaayi labhikkunnath. ithinartham saadhaaranayaayi oru kuttikk maathaapithaakkalil ninnu parivartthanam cheytha jeeninte oru pakarpp paarambaryamaayi labhikkunnu, ithu oru homocygus thakaraarin kaaranamaakunnu.
pathophysiology.
chapple syndrominte savisheshathakal complimentated-mediated oottoo immyoon haemolysis, paroxismal noctornal haemoglobinuria ennivayaanu. protein sidi55 (kshaya-thwarithappeduthunna ghadakam ennum ariyappedunnu) amplification ghattam niyanthrikkunnathiloode swathasidhamaaya rogaprathirodha samvidaanathinte bhagamaaya compliment cascade niyantrikkaan sahayikkunnu. sidi55 illaathirikkumbol, pooraka samvidaanam chuvanna rakthaanukkale akramikkukayum avaa nasippikkaan kaaranamaakukayum cheiyunnu (haemolysis).
roganirnayam.
chapple syndrome rogikal saadhaaranayaayi klinikkal avatharanam, histolaji, janithaka parisodhana ennivayude samyojanathiloodeyaanu roganirnayam nadathunnathu. rogalakshanangal vyathyasthamaayirikkaame, rogikalkku saadhaaranayaayi neratheyulla dahananaala lakshanagal, edema, poshakaahaarakkurav, hypoalbuminemia, hypogammaglobulinami enniva kaanapedunnu. kudal biopsy saambilukaludeyo vichedanangaludeyo hystopathological vilayiruthal vipulamaaya lymphangiactasia velippeduthukayum praadhamika kudal lymphangiectasiyude roganirnayam nirddeshikkukayum cheiyunnu. rogikalkku valia-sira thrombosis varanum saadhyathayund.
chikitsa.
roganirnayam nadatthiyaal, chikitsa oru vyakthiyude klinikkal avasthaye adisthaanamaakkiyullathaanu. kurolap sahapravarthakarum oof-label eculisumab, oru manushyavalkkarikkappett aanti-sii5 monoclonal antibody compliment inhibittarum upayogichu rogikale chikilsichu, ithu 18 maasa kaalayalavil prayojanakaramaaya phalangal nalkumennu kaanichu. turkeyile istanbulile marmara sarvakalashaalayileyum merilaandile bethesdayile us naashanal instittyoott oof healthile naashanal instittyoott oof allergy aandu panfective diseasileyum anweshakarkku nilavil eee thakaraarinte roganirnayathinum chikilsaykkumulla puthiya sameepanagal padikkaan klinikkal protocolukal undu.