Cirrhosis & Encephalopathy

DEFINITION:

EPIDEMIOLOGY:

Cirrhosis and chronic liver disease accounted for more than 25,000 deaths and 375,000 hospitalizations in 1998

ETIOLOGIES:

(See also corresponding chapters on each etiology for more detail)

Alcohol
Viral Hepatitis: (chronic HBV, HCV, HDV infection)
Autoimmune hepatitis: (female, ↑ IgG, +ANA, +ASMA)
Congenital: Hemochromatosis, Wilson’s disease, α1-antitrypsin deficiency, Congenital hepatic fibrosis
Metabolic diseases: NASH/NAFLD
Biliary tract disease: PBC or PSC, Secondary biliary cirrhosis (calculus, neoplasm, post-op stricture, biliary atresia)
Vascular diseases: Budd-Chiari syndrome, R-sided heart failure, Constrictive pericarditis
Cryptogenic: may reflect terminal progression of NAFLD or some may be non/missed diagnosed AIH

PATHOPHYSIOLOGY:

A late stage of progressive hepatic fibrosis characterized by distortion of the hepatic architecture and formation of regenerative nodules

CLINICAL MANIFESTATIONS/PHYSICAL EXAM:

Subclinical or may present as progressive liver dysfunction (jaundice, coagulopathy, encephalopathy) and/or portal hypertension (ascites, varices)
Liver:
- Enlarged, palpable, firm, nodular » shrunken and nodular
Signs of Liver Failure:
- Jaundice, spider angiomata (marker of chronicity), palmar erythema, Dupuytren’s contractures, white nail lines (Muehrcke’s lines) & proximal nail beds (Terry’s nails), ↑ parotid & lacrimal glands, gynecomastia, testicular atrophy, asterixis, encephalopathy, fetor hepaticus
- Asterixis, seen with: Cirrhosis (PSE), Renal (Uremia), Lung (CO₂ retention)
Signs of Portal HTN:
- Splenomegaly, ascites, dilated superficial abdominal veins (caput medusae), epigastric ‘Cruveilhier-Baumgarten’ venous hum

LABORATORY STUDIES:

↑ bilirubin, ↑ PT, ↓ Albumin, ± ↑ aminotransferases and ↑ Aφ (variable)
↓ ↓ Na
Anemia (marrow suppression, hypersplenism, iron and/or folate deficiencies)

Neutropenia (hypersplenism)

Thrombocytopenia (hypersplenism, ↓ Thrombopoietin production by liver)

DIAGNOSTIC STUDIES:

± Liver biopsy (percutaneous or transjugular); For indications and contraindications, See also Liver-LFTs (Chapter 4.20)
Abdominal U/S doppler: liver size; rule out Hepatocellular cancer (HCC), ascites, assess patency of portal, splenic and hepatic veins
Hepatic serologies (HBsAg, anti-HBs, anti-HCV)
Autoimmune hepatitis studies (IgG, ANA, ASMA)
Fe & Cu studies
α1-AT phenotype
AMA (PBC), p-anca (PSC)
Echocardiogram (if concerned about right-sided heart failure)
AFP to screen for HCC (with U/S)