Download - Colangite Sclerosante
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CASO CLINICO
• RAGAZZO DI 17 ANNI CON ITTERO
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ANAMNESI (1)
• 17 Mesi prima del ricovero.
–Febbre
–Diarrea ematica
–SGPT 75 U. ( V.N. < 20)
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DIARREA ACUTA
• Inflammatory Bowel Disease (IBD)
• Cause Infettive
• Cause Neoplastiche
• Cause Vascolari
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IMPORTANTI RILIEVI ANAMNESTICI
• IBD• Immunosoppressione (AIDS!!)
• Antibiotici• Radiochemioterapia antineoplastica• Viaggi
• Origine geografica
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DIARREA INFETTIVA(1)
• VIRUS– Norwalk
– Rotavirus
– Astrovirus
– Calicivirus
– Adenovirus
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DIARREA INFETTIVA (2)
• PARASSITI
– Giardia
– Entameba
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DIARREA INFETTIVA (3)
• BATTERI– Campilobacter
– Salmonella
– Shigella
– Escherichia Coli
– Clostridium difficile
– Yersinia
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ANAMNESI (2)
• Positività per clostridium difficile– N.B. non antibiotici prima dell’episodio
• Terapia con metronidazolo per 1 mese• Scomparsa della diarrea
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ANAMNESI (3)
4 settimane prima del ricovero:– Dolori addominali alti, crampiformi
– Nausea– Feci molli– Febbre 38°C
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ANAMNESI(4)
• 5 giorni prima del ricovero:– Dolore emitorace sinistro
– Tosse secca– Leucocitosi– Neutrofilia
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AL RICOVERO
• Temperatura 38,3 °C, polso 85/ min.
• Ittero sclerale e cutaneo
• Torace:
– Rantoli basali emitorace sinistro
• Radiografia: polmonite basale sinistra
– Dolore retrosternale alla digitopressione
• Addome: dolenzia diffusa, non epatomegalia
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IPERBILIRUBINEMIA INDIRETTA (1)
• EMOLISI– Ereditaria
• Sferocitosi,Elissocitosi,Carenze Enzimatiche
• Sckle cell anaemia
– Acquisita• Microangiopatia
• Emoglobinuria parossistica notturna
• Disordini Immuni
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IPERBILIRUBINEMIA INDIRETTA (2)
• ERITROPOIESI INEFFICACE
– Carenza di Vit. B12, acido folico o ferro
• DA FARMACI
– Rifampicina, Probenecid, Ribavirina, penicillina
• EREDITARIE
– S. di Crigler-Najjar I e II
– S. di Gilbert
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IPERBIIRUBINEMIA MISTA(EPATOCELLULARE) (1)
• VIRALE– Virus dell’epatite A, B, C, D, E.– Virus di Ebstein Barr (EBV)– Citomegalovirus– Herpes simplex
• ALCOOL• FARMACI
– Dose dipendenti– Idiosincrasici
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IPERBILIRUBINEMIA MISTA(EPATOCELLULARE) (2)
• TOSSINE AMBIENTALI– Cloruro di vinile
• MORBO DI WILSON• EPATITE AUTOIMMUNE
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IPERBILIRUBINEMIA DIRETTA (1)
• COLESTASI INTRAEPATICA– Epatiti virali ( forma fibrosante colestatica)
– Epatite alcoolica colestatica
– Da Farmaci• Steroidi anabolizzanti e contraccettivi
• Numerosissimi altri farmaci
– Cirrosi biliare primitiva
– Colangite sclerosante primitiva
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IPERBILIRUBINEMIA DIRETTA (2)
• COLESTASI INTRAEPATICA (2)• Sindrome dei dotti biliari evanescenti
– Rigetto, Sarcoidosi, Alcuni Farmaci
• Colestasi benigna ricorrente (ereditaria)• Gravidanza• Nutrizione parenterale totale• Sepsi• Sindrome paraneoplastica• Malattia veno-occlusiva
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IPERBILIRUBINEMIA DIRETTA (3)
• COLESTASI EXTRAEPATICA– Maligna
• Colangiocarcinoma• Carcinoma della testa del pancreas• Ampulloma• Aumento dei linfonodi portali
– Benigna• Litiasi coledocica• Colangite sclerosante primitiva• Pancreatite Cronica• Colangiopatia da AIDS
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ESAME FECI
• Tossina del Clostridium difficile: negativa
• Sangue occulto: +++
• Fibre muscolari non digerite
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ESAME URINE
–Bilirubina : +++-
–Urobilinogeno ++--
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ESAME EMOCROMOCITOMETRICO
• Ematocrito: 37 %• Leucociti. 16.000
• Piastrine 504.000• Formula leucocitaria: N. 86, L. 7 , M. 7 %
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ESAMI DI LABORATORIO(1)
• Calcemia 8,0 mg %• Colesterolemia 104 mg %
• Protidemia.– Totale 7,8 g %
– Globuline 5,5 g %
– Albumine 2,3 g %
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ESAMI DI LABORATORIO(2)
• SGOT: x 1,5• SGPT: x 1,5
• Fosfatasi alcalina : x 3• Gamma G.T.: x 4• Dosaggio di farmaci e droghe: neg.
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ESAMI DI LABORATORIO(3)
• ANA: 1: 180 pattern omogeneo• A. Antimuscolo liscio: 1: 80
• A. Antimitocondrio : neg.• Test per Epatite A : neg• Test per Epatite B : antigene : neg
• anticorpo : pos.• Test per Epatite C : neg.
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ALTRI ESAMI
• Ecografia Addominale: negativa
• Tomografia computerizzata : negativa, anche con mezzo di contrasto
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INFEZIONE DA CLOSTRIDIUM DIFFICILE
• Rara nei giovani non predisposti.• Fattori predisponenti.
– Ospedalizzazione– Uso di antibiotici– Età avanzata– Procedure gastrointestinali non chirurgiche– Immunocompromissione– Malattia sottostante
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DIARREA E CLOSTRIDIUM DIFFICILE
• Non fattori di rischio
• Terapia con metronidazolo lunga
• Transaminasi elevate ( non usuali)
• Ittero: non spiegabile.
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DIAGNOSI DIFFERENZIALE
• Individuare disordine clinico capace di causare:– Malattia intestinale
– Protidodispersione
– Malattia epatica
– Polmonite ( intercorrente ? )
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MALATTIA INTESTINALE
• Infezione ( improbabile)• Sprue
• Inflammatory Bowel Disease (IBD)– Colite ulceratica
– Morbo di Crohn
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DIAGNOSI DI SPRUEARGOMENTI A FAVORE
• Causa dispersione proteica• Si associa con malattie epatiche autoimmuni
– Epatite
– Cirrosi biliare primitiva
– Colangite sclerosante primitiva
• Presenta elevazione aspecifica delle transaminasi
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DIAGNOSI DI SPRUEARGOMENTI CONTRARI
• ETA’– Bambini piccoli ed adulti
– Non sono presenti dolori nei quadranti alti dell’addome ( Epatopatia associata ?)
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DIAGNOSI DI IBDARGOMENTI A FAVORE
• Frequente associazione con:– Perdita di proteine
– Disturbi autoimmuni epatobiliari
– Diarrea ematica
– Febbre ( specie colite ulcerosa)
– Leucocitosi
– Dolore retrosternale• Interessamento esofageo del m. di Crohn
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DIAGNOSI DI IBDARGOMENTI CONTRARI
• Diarrea modesta
• Ematochezia non intensa
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EAPATOPATIA
• Segni di colestasi presenti:• Bilirubina coniugata
• Fosfatasi alcalina
• Gamma Glutamil transferasi
• Tuttavia:– Non calcoli
– Non dilatazione delle via biliari extraepatiche
– Fegato di dimensioni normali
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EPATOPATIA-EPATITIVIRALI
• Diagnosi improbabile:– Transaminasi troppo basse
– Marcatori virali negativi
– HBSAg positivo• vaccinazione
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EPATOPATIA
• Frequente associazione fra:
– IBD e
• Epatite autoimmune (AH)
• Colangite primitiva sclerosante (PSC)
– 10 % dei casi
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ESAMI DIAGNOSTICI (1)
• A.
• Transaminasi
• Fosfatasi alcalina
• Gamma G.T.
• B
• Colangiografia (con esami dei dotti intraepatici)
• Biopsia epatica
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ESAMI DIAGNOSTICI
Marcatori Positivi Negativi Caso
--------------------------------------------------------------
ANA AH, PSC +--
ASMA AH, PSC +++
AMA PBC AH, ASC ----
pANCA PSC AH +++
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DIAGNOSI CLINICO-LABORATORISTICA
• COLANGITE SCLEROSANTE PRIMITIVA, ASSOCIATA A MORBO DI CROHN
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COLANGIOGRAFIA RETROGRADA
• Nessuna evidenza di ostruzione delle vie biliari extraepatiche
• Evidenza di stenosi dei dotti biliari extraepatici
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PROGNOSI
• MORBO DI CROHN:– Controllabile con la terapia
• COLANGITE SCLEROSANTE PRIMITIVA.– progressiva
– aumentato rischio di cancro
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TERAPIA
• Acido ursodesossicolico• Prednisone
– Sintomatici, ma incapaci di arrestare il decorso della PSC
• TRAPIANTO DI FEGATO
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Alcune figure di questa presentazione sono state tratte da:
Harrison on LineScientific American Medicine
NEJM 346,271,2002