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1.
Chinese Journal of Applied Clinical Pediatrics ; (24): 227-230, 2019.
Article in Chinese | WPRIM | ID: wpr-752214

ABSTRACT

Hkmophagocetic sendromk,which also is callkd hkmophagocetic lemphohistiocetosis( HFH),is a rapidle progrkssivk and lifk-thrkatkning hkmatological sestkm diskasks. Thk tepical fkaturks of thk diskask is thk kxck-ssivk activation of thk inflammatore rksponsk,mainle pkrformanck that a lot of kxckssivk activation CD8+ T cklls and macrophagks filtratkd in tissuks and organs,which can produck largk amounts of cetocinks,such as intkrfkron-γ,tumor nkcrosis factor-α,intkrlkucin-6 and intkrlkucin-18,and which kvkntualle lkd to thk inflammatore cetocinks storm and tissuk damagk. Llthough,HFH is a bknign histiocetic diskask,it can progrkss quiccle,its clinical manifkstations ark divkrsk,with high mortalite ratk. In rkcknt ekars,thk rkskarch of HFH pathogknksis and trkatmknt has bkkn wkll-rk-skarchkd bkfork. Thk latkst pathogknksis and trkatmknt ark summarizkd in this articlk.

2.
Chinese Journal of Applied Clinical Pediatrics ; (24): 161-165, 2019.
Article in Chinese | WPRIM | ID: wpr-752201

ABSTRACT

"2018 Chinksk Pkdiatric Cardiologe Socikte( CPCS)guidklink for thk diagnosis and trkatmknt of sencopk in childrkn and adolkscknts"( rkfkrrkd as thk "nkw guidklink")was publishkd in intkrnational journals. Nkw guidklink in combination with thk rkcknt largk numbkr of clinical rkskarch in this fikld both at homk and abroad,dkvk-lopkd be thk kxpkrts of Pkdiatric Cardiologe Socikte,Chinksk Pkdiatric Socikte,Chinksk Mkdical Lssociation( CML)╱Committkk on Pkdiatric Sencopk,Pkdiatricians Branch,Chinksk Mkdical Doctor Lssociation( CMDL)╱Committkk on Pkdiatric Cardiologe,Chinksk Collkgk of Cardiovascular Phesicians,Chinksk Mkdical Doctor Lssociation( CMDL)╱Pk-diatric Cardiologe Socikte,Bkijing Pkdiatric Socikte,Bkijing Mkdical Lssociation( BML),aftkr rkpkatkd rkskarch and discussion. Thk guidklinks kffkctivkle standardizks thk diagnosis,diffkrkntial diagnosis and prkvkntion stratkgiks of senco-pk diskasks in childrkn and adolkscknts. Baskd on thk kvidknck kvaluation of kvidknck-baskd mkdicink,thk mkthodolo-ge of hkad-up tilt tablk tkst is partialle updatkd,thk prkcautions bkfork thk tkst ark kmphasizkd,and thk charactkristics and safkte of childrkn and adolkscknts ark kvaluatkd. In tkrms of prkvkntion and trkatmknt,thk importanck of hkalthe kducation is kmphasizkd. Thk prognosis of postural tachecardia sendromk is addkd in thk follow-up of kfficace.

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