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DEFINIIE
FIBROZA
difuz
EPIDEMIOLOGIE
POVARA ("disease burden"): 300.000 de decese pe an n lume 2% din necropsii 10% din alcoolici, dup 25 de ani CGH Fundeni, anul 2001
1681 / 8768 internri (20%)
ETIOLOGIE
VIRAL - VHB+-VHD, VHC ALCOOLIC (nutriional) COLESTAZ intra & extrahepatic METABOLIC (Wilson, hemocromatoza, glicogenoze) IMUN (LES) TOXICE (amanita phalloides, P, CCl4) staz venoas (boal veno-oclusiv, Budd Chiari, ICC, CPC, valvulopatii) BY BASS MALNUTRIIE KWASHIORKOR GENETIC (grup A, HLA B8, B40, DW3) CRIPTOGENIC
PATOGENIE
NECROZA
COLAPS LOBULAR
REGENERARE NODULAR
SEPTURI FIBROASE
PATOGENIE
NODULI DE REGENERARE
mping sinusoidele la periferie insuficien circulatorie n centrul nodulilor
PATOGENIE
FIBROZ
CELULE
fibroblast - reversibil, fibrocit - ireversibil sintez FIBRE (colagen)
FIBRE
colagen stric arhitectura lobular
FIBRONECTINA, LAMININA
n membrana bazal rol chemotactic pentru fibroblast - limfokine, monokine
PATOGENIE
ALTE MODIFICRI
modificri vasculare
unturi arteriovenoase intrahepatice i sistemice
HISTOPATOLOGIE
HISTOPATOLOGIE
FICAT MACROSCOPIC
ATROFIC HIPERTROFIC AMBELE
CLINICA
ASIMPTOMATIC = descoperire intamplatoare ( operatii, analize, necropsii) SIMPTOMATIC SIMPTOME
astenie, adinamie, fatigabilitate, anorexie, disconfort abdomen superior
SEMNE
febra cutanat - icter, paloare, edeme, echimoze, stelue vasculare, leuconichie, Chwostek, Dupuytren, buye carminate, ginecomastie, ... locale - ficat, neregulat, dur, ascuit, - splina, - ascit.. alte - TA, AV, epistaxis, infecii, malabsorbie, ...
CLINICA
COMPLICAII Ascita Encefalopatie HDS 20% oligosimptomatic
PARACLINIC
1. 2. 3. 4. 5. 6. 7. COLESTATIC INSUFICIEN HEPATOCELULAR INFLAMATOR CITOLIZ IMUNOLOGIC HEMATOLOGIC MORFOLOGIC
PARACLINIC
1. COLESTATIC
bilirubin, stercobilinogen, urobilinogen fosfataza alcalin, GGT, 5 nucleotidaza colesterol total acizi biliari
PARACLINIC
2. INSUFICIEN HEPATOCELULAR
proteine - albumina, fibrinogen, II, V, VII, IX, X, T Quick, transferina, ceruloplasmina, transcortina, tiroxin beta globulina aa aromatici - liniari colseterol aldosteron, sex-hormoni amoniogeneza, urereogeneza detoxifiere teste de traversare - BSP, roz bengal
PARACLINIC
3. INFLAMATOR
labilitate seric globuline raport albumine / globuline VSH fibrinogen
PARACLINIC
4. CITOLIZ
aminotransferaze ALT, AST, raportul de Rittis LDH, GDH, OCT, GGT sideremie derivai de acid colic
PARACLINIC
5. IMUNOLOGIC
markeri virali VHB, VHD, VHC hepatita autoimun si CBP - ANA, ASMA, anti LKM1, AMA imunograma IgA, IgM, IgG AFP celule LE
PARACLINIC
6. HEMATOLOGIC
citopenie anemie hemolitic, feripriv, macrocitar leucocitoz
PARACLINIC
7. MORFOLOGIC
ECO - marg ascuit, noduli, desen vascular srcit n periferie, manon hiperecogen vascular, HTP - vase, splenomegalie PBH EDS - varice esogastrice, gastropatie presiune portal laparoscopie, CT (scintigrama, splenoportografie,)
DIAGNOSTIC
"Ciroz hepatic 1. atrofic / hipertrofic, 2. de etiologie ..., 3. activ / inactiv 4. compensat / decompensat portal i/sau parenchimatos 5. complicat cu ... (6. tratament cu )"
DIAGNOSTIC DIFERENIAL
Alte cauze ale simptomelor si semnelor
hepatomegalie splenomegalie HTP HDS encefalopatie ...
PROGNOSTIC
Ascita Encefalopatie Bilirubina Albumina AP
1
2 3
absent
uoar moderat - sever
0
I - II III - IV
<2
2-3 >3
> 3.5
2.8 - 3.5 < 2.8
60 - 70%
40 - 60% < 40%
5 - 6 Child A
7 - 9 Child B
10 - 15 Child C
= Child PUGH
PROGNOSTIC
FAVORABIL
alcool, cauz cunoscut, hipertrofic
NEFAVORABIL
Scor Child crescut, Na+, TA
+/- FAVORABIL
rspuns la tratament
TRATAMENT
MSURI GENERALE ETIOLOGIC PATOGENIC SIMPTOMATIC AL COMPLICAIILOR TRANSPLANT HEPATIC
TRATAMENT
MSURI GENERALE
repaus diet - nu alcool, nu condimente, nu... evitare transfuzii vaccinare VHA, VHB caz nu cauz
TRATAMENT
ETIOLOGIC
Stop alcool Lamivudina n ciroza compensat VHB Interferon plus Ribavirina numai pentru ciroza compensat VHC (un fel de hepatit cronic care la PBH are scor Knodell de fibroz 4) - fr nici o alt modificare Desferoxamina (chelator Fe) n hemocromatoz D penicilamina (chelator Cu) n boala Wilson
TRATAMENT
PATOGENIC
cortizon n ciroza autoimun acid ursodeoxicolic (UDCA) n ciroza biliar primitiv
SIMPTOMATIC
hepatoprotector, lipotrop, stabilizant membranar
UDCA n orice ciroz 10 mg/kgc silimarin antioxidante - vitamina E fosfolipide
TRATAMENT
TRANSPLANT HEPATIC
pentru END STAGE LIVER DISEASE (pacientul are un prognostic infaust pe termen lung, mediu si uneori chiar scurt) list de ateptare - criterii difereniate indicaii individualizate n funcie de etiologie comorbiditi asociate severitate afectare hepatic centru de transplant sistem de asigurri de sntate Institutul Clinic Fundeni ~ 50 LTx
CONCLUZII
MAI BINE E S PREVII DECT S TRATEZI costuri enorme, eficacitate redus a tratamentului, gref hepatic cu via limitat PREVENIRE CAUZE alcoolism, vaccinare VHB, profilaxie infecie VHB, VHC (sexual, acte medicale, briciul de la frizerie, manichiur, pedichiur, stomatolog) - nu se desinfecteaz cu alcool! DEPISTARE CAUZE SI TRATARE N STADII REVERSIBILE (hepatit, steatoz)
PERSPECTIVE
AUTOTRANSPLANT DE HEPATOCITE PROBLEMA = ARHITECTURA LOBULARA