Liver Cirrhosis, Definition and Diagnosis

Introduction
Definition :
Fibrosis & nodular regeneration resulting from hepatocellular injury
Epidemiology :
Cirrhosis & CLD accounted for > 25.000 death & 375.000 hospitalization

Etiology

¡  Alcohol

¡  Virus Hepatitis : chronic HBV,HCV, HDV infection

¡  Autoimmune hepatitis  (female, ?IgG, +ANA, +ASMA)

¡  Cogenital : hemocromatosis, wilson’s disease, -?1antitrypsin deficiency, cogenital hepatic fibrosis

¡  Metabolic disease : NASH/NAFLD

¡  Biliary tract disease : PBC/PSC, secondary biliary cirrhosis (calculus, neoplasm,post-op stricture, biliary atresia)

¡  Vascular disease : Budd-Chiari syndrome, R-side  heart failure, constrictive pericarditis

¡  Cryptogenic : may reflect terminal progression of NAFLD or some may be non/missed diagnosed AIH

Pathopysiology

•         A late stage of progressive hepatic fibrosis characterized by distortion of hepatic architecture & formation of regenerative nodules

•         Form of chronic hepatitis

•         Inflammatory infiltration of hepatic portal

•         Necrosis of hepatocytes (parenchyme or portal areas)

Clinical Manifestation

?  Subclinical or present at progressive liver dysfunction (jaundice, coagulopathy, encephalopathy) and/or portal hypertension (ascites, varices)

?  Liver : enlarged, palpable, firm, nodular >> shrunken & nodular

?  Sign of liver failure :

Jaundice, spider angiomata (marker of chronicity), palmar erytema, duputyren’s contracture, white nail lines (muehrcke’s line) & proximal nail beds (terry’s nail), parotid & lacrimal glands, gynecomasti, asterixis, enchephalopathy, fetor hepaticus

?  Sign of portal hipertension

Splenomegaly, ascites, dilated superficial abdominal vein (caput medusae), epigastric “cruveilhier-Baumgarten” venous hum

Laboratory Studies

?  ­bilirubin, PT, albumin, ± aminotransferases, alkaline phosphatase

?  Na

?  Anemia (bone marrowsupression, hypersplenism, iron and/or folate deficiencies), neutropenia (hypersplenism), thrombocytopenia (hypersplenism, thrombopoietin production)

Diagnostic Studies

?  Liver biopsy (percutaneus/transjugular)

?  Abdominal U/S doppler

?  Hepatic serologies (HBsAg, AntiHCV)

?  Autoimmune hepatitis study (IgG, ANA, ASMA)

?  Fe and Cu studies

?  a1-AT phenotype

?  AMA (PBC), p-ANCA (PSC)

?  Echocardiogram (right sided heart failure)

?  AFP

Complication

?  Portal hypertension : ascites, varices, hepatohydrotorax

?  Coagulopathy

?  Hepatic encephalopathy

?  Hepatorenal syndrome

?  Hepatopulmonary syndrome

?  Liver failure

?  Infection

?  Hepatocellular carcinoma

Prognosis

Correlates with Child-Pugh class

1 2 3
ascites none Easy control poor
encephalopathy none Grade I/II Grade III/IV
Bilirubin < 2 2-3 >3
Albumin > 3.5 2.8-3.5 <2.8
PT <4 4-6 >6
A B C
Total point 5-6 7-9 10-15
1-y survival 100% 81% 45%
2-y survival 85% 57% 35%