CIROZE
-
Upload
lleticia87 -
Category
Documents
-
view
110 -
download
3
Transcript of CIROZE
CIROZE
CIROZATERMEN GENERIC PENTRU TOATE FORMELE DE BOALA CRONICA DIFUZA CARACTERIZATA PRIN : 1. 2. 3. 4. PIERDEREA CELULARA COLAPS SI FIBROZA RETELEI RETICULINICE DISTORSIONAREA PATULUI VASCULAR REGENERARE NODULARA
CIROZA - este un proces difuz cu fibroza si formare de noduli ce succed necrozei hepato celulare. CLASIFICARE ANATOMICA MACRONODULARA MICRONODULARA 1. 2. 3. ETIOLOGICA VIRALA ( B, C, D) ALCOOLICA METABOLICA Fe Cu 1AT glicogenoza IV galactozemie COLESTATICA BLOC VENOS S. BUDD CHIARI I. CARDIACA TOXICE MEDICAMENTOASE IMUNOLOGICE CRIPTOGENETICE
4. 5. 6. 7. 8.
DIAGNOSTIC1. 2. ANAMNEZA EXAMEN FIZIC FOETOR, ICTER, PURPURA, DEGETE HIPOCRATICE, STELUTE VASCULARE ERITROZA PALMARA, GINECOMASTIE ATROFIE TESTICULARA, EDEME
3. 4. 5.
HEPATOMEGALIE / FICAT MIC SPLENOMEGALIE CIRCULATIE COLATERALA
ANATOMIA PATOLOGICA
1. 2. 3. 4. 5.
INFLAMATIA PORTALA / INTRALOBULARA STAZA BILIARA PROLIFERARE DUCTULARA / DUCTOPENIE GRANULOAME ANOMALII SPECIFICE - CORPII MALLORY - INCARCARE CU - Fe - Cu - PAS +
PIERDEREA DE HEPATOCITE
1. ICTER 2. ASCITA 3. EDEME 4. TULBURARI NEUROLOGICE 5. CASEXIE 6. DECES
FIBROZA1. DISTORSIONAREA CAPILARELOR
2.
HIPERTENSIUNE PORTALA
VARICE ESO - GASTRICE
SPLENOMEGALIE
REGENERAREA1. MODIFICARI ALE FORMEI 2. TULBURARI LIMFATICE
ASCITA
DIAGNOSTICUL CLINIC1. SEMNE GENERALE a) HABITUS CHVOSTEK b) BUZE CARMINATE c) PALME HEPATICE d) GINECOMASTIE e) PILOZITATE GINOIDA f) ATROFIA TESTICULARA
2. SEMNE LOCALEa) ANOMALII ALE DIMENSIUNILOR b) MODIFICARE DE CONSISTENTA c) SPLENOMEGALIE d) ASCITA OMFALOCEL e) CIRCULATIE COLATERALA CAPUT MEDUSAE
Clinical Features of Chronic Liver Disease
Fatigue Vascular spiders Liver - may be enlarged or shrunken Enlarged spleen Finger clubbing Advanced disease
Ascites Bruising Oesophageal varices
A 47-year-old m
DIAGNOSTIC ANATOMIC1. MACROSCOPIC - LAPAROSCOPIE - SCINTIGRAFIE - ECHOGRAFIE - TOMOGRAFIE 2. MICROSCOPIC - COLORATII RETICULINA - COLORATII COLAGEN
DIAGNOSTIC BIOLOGIC1. 2. EXPLORARI ETIOLOGICE ( VIRUSOLOGICE, IMUNOLOGICE ) EXPLORARI FUNCTIONALE a ) SINDROM INFLAMATOR b ) SINDROM CITOCITIC c ) SINDROM COLESTATIC d ) SINDROM INSUFICIENTA CELULARA 3. 4. HEMATOLOGIE IMPUSE DE COMPLICATII ( Electroliti, amoniac, uree )
SCORUL CHILD - PUGH PUNCTEENCEFALOPATIE ASCITA BILIRUBINA ALBUMINEMIE T.P ( % ) CLASA A 3.5 g % >54 CLASA B 7 9
2MINIMA MINIMA 3.5 5 2.8 3.5 45 54
3COMA ABUNDENTA >5 9
TESTE CANTITATIVE EVALUAREA GRAVITATII BOLII / PROGNOSTIC ALEGEREA TERAPIEI SORTAREA GRUPELOR OMOGENE EVALUAREA TERAPIEI ADAPTAREA POSOLOGIEI pentru droguri metabolizate
NORMAL
CIROZA
PROGNOSTIC PROST
VERDE INDOCIANIN ( ml / min ) GALACTOZA CAFEINA AMINOPIRINA ( CO2 )
1200
400
< 900
400 140 >0.04
200 50 > 0.018
< 100 < 17 anormale
2. AfecteazaFicatul Pancreasul Inima Rinichii Articulatiile
Istoria Sheldon 1935 descrie 311 cazuri Simon 1974 asociaza boala cu HLAA3 si A7 Feder 1996 izoleaza gena HFE
Boala se transmite autosomal recesiv Gena HFE mutanta C 282 Y Dar ! 25% homozigoti 50% heterozigoti 25% sanatosi
PatogenieGena mutanta (cisteina-tirozina) creste absorbtia intestinala a fierului prin incapacitatea acestuia de a patrunde in enterocit.
Istoria naturala1. 2. Acumulare asimptomatica Anomalii biologice Cresterea sideremiei Cresterea coeficientului de saturare a transferinei - Cresterea feritinei 3. Manifesta clinic - Astenie fizica - Dureri articulare
Criterii de diagnostic1. 2. 3. Fe hepatic crescut Fe hepatic> 80 mmoli/g Cresterea indexului Fe hepatic (concentratie/varsta>1.9) 4. Spolierea a 5 g Fe fara anemie 5. Genetic - Homozigotie C 282 Y - Heterozigotie C 282 Y + H 63 D
Dg Diferential in HC Suprasarcina Fe din mutatia genei SLC11A3 - receptorului transferinei Aceruloplasminemia Atransferinemia congenitala Hemocromatoza neonatala Hemocromatoza juvenila
Secundare Aport alimentar Aport parenteral Anemii hemolitice Hemodializa Boli hepatice (hepatita C,ciroza alcoolica,SHNA) Porfiria cutanata Sunturi porto-cave
Sindromul cataracta -hiperferitinemie- Descris 1995 in Italia si Franta - Boala transmisa autosomal dominant - Caracterizata prinFe seric normal Saturare normala a transferinei Feritinemie crescuta
BAZA GENETIC~ HFE - HHC gen` MHC I TfR - C7922
DIAGNOSTIC ASTENIE ARTRALGII HEPATOMEGALIE 95% DIABET 60% ARTROPATII 25% HIPERPIGMENTARE
HISTOPATOLOGIE FIER COLORABIL 3,4 FIER HEPATIC > 80 M/g INDEX HEPATIC > 2 SCOATEREA A 5g DE FIER F~R~ ANEMIE CY282M
TRATAMENT FLEBOTOMIE TRANSFERIN~ 50% SATURAT~
DESFERIOXAMIN~ 2g/zi
Boala PolichisticaVariante 1. Hepato-renala 2. Exclusiv hepatica
A 44-year-old wom