Le malattie autoinfiammatorie in pediatria-Brachi.pdf

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Antibiotics response - Adeno, HSV, EBV, CMV Ab ± EZIOLOGIA ? Infective ? Genetic ? Autoinflammatory ? No ethnic background Positive familial history “During pFAPA attacks complemet, IL-1 related and IFN-induced genes were significantly over expressed…our findings of marked induction of genes encoding innate immune molecules during pFAPA flares, taken together with the cardinal findings of oral and pharyngeal inflammation and cervical adenopathy, strongly suggest a microbial trigger, perhaps amplified in the susceptible host at a particulare stage of development” Stojanov et al “Periodic fever, aphtous stomatitis, pharyngitis and adenitis (PFAPA) is a disorder of innate immunity on Th1 activation responsive to IL-1 blockade” PNAS 2011; 108:7148-53
o Diagnosi principalmente clinica o Descritta per la prima volta nel 1987 da Marshall DIAGNOSI
Page 1 and 2: LE MALATTIE AUTOINFIAMMATORIE IN PE
Page 3 and 4: 1. Febbri periodiche: periodiche FM
Page 5 and 6: L’approccio al paziente con sindr
Page 7 and 8: FEBBRE RICORRENTE NON INFETTIVA Si
Page 9: P eriodic F ever A phtous stomatiti
Page 13 and 14: SINDROME PFAPA Febbre >38°, durat
Page 15 and 16: FANS: scarsa risposta TERAPIA STER
Page 17 and 18: RUOLO DELLA TONSILLECTOMIA RUOLO DE
Page 19 and 20: FEBBRE MEDITERRANEA FAMILIARE (FMF)
Page 21 and 22: GENE MEFV Il gene MEFV è espresso
Page 23 and 24: Dolore addominale (95%) Dolore art
Page 25 and 26: ESAMI DI LABORATORIO Nessun test di
Page 27 and 28: COLCHICINA Caratteristiche alcaloi
Page 29 and 30: COLCHICINA E GRAVIDANZA Both T, va
Page 31 and 32: E E SE SE LA LA COLCHICINA COLCHICI
Page 33 and 34: Fig. 3. Tumor necrosis factor recep
Page 35 and 36: TRAPS: SINTOMI ASSOCIATI Mialgie Do
Page 37 and 38: ESAMI DI LABORATORIO NESSUN TEST DI
Page 39 and 40: TERAPIA TERAPIA TRAPS TRAPS STEROI
Page 41 and 42: SINDROME DA IPER-IGD (HIDS) Nota an
Page 43 and 44: BIOSINTESI DEL COLESTEROLO ↑↑ c
Page 45 and 46: SDR DA IPERIGD: CARATTERISTICHE CLI
Page 47 and 48: ESAMI DI LABORATORIO Durante l’at
Page 49 and 50: TERAPIA HIDS STEROIDI e FANS duran
Page 51 and 52: … RIASSUMENDO RIASSUMENDO… RIAS
Page 53 and 54: CRIOPIRINO-PATIE: BASI GENETICHE Tc
Page 55 and 56: Il gene NLPR3 (o CIAS-1) codifica p
Page 57 and 58: CINCA, MWS, FCAS Essendo secondari
Page 59 and 60: CLINICA CINCA: Cute: rash, tipicame
Page 61 and 62:
FAMILIAL COLD AUTOINFLAMMATORY SYND
Page 63 and 64:
DIAGNOSI - Clinica (andamento ricor
Page 65 and 66:
CRIOPIRINO-PATIE Cute Orticaria neo
Page 67 and 68:
Prospettive in Pediatria, Luglio-Se
Page 69 and 70:
SINDROME DI BLAU (GRANULOMATOSI SIS
Page 71 and 72:
DISORDINI PIOGENICI Sindrome PAPA
Page 73 and 74:
Sindrome DIRA -Autosomica recessiva
Page 75 and 76:
FMF FMF Etnia (Mediterraneo) febbre
Page 77 and 78:
FARMACI BIOLOGICI Hanno un potente
Page 79 and 80:
TRE AGENTI ANTI TNF(TUMOR TNF NECRO
Page 81 and 82:
AGENTI BIOLOGICI ANTI IL-1 IL 1 A p
Page 84:
Grazie per l’attenzione!
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Le malattie autoinfiammatorie in pediatria-Brachi.pdf

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