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Hepatology Board Review

Hepatology Board Review. Hepatitis A. Enterically transmitted-RNA virus Common throughout the world but risk in developing nations Effective vaccine available No chronic state, <1% risk for FHF FHF usually if have underlying liver disease or older age

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Hepatology Board Review

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  1. Hepatology Board Review

  2. Hepatitis A • Enterically transmitted-RNA virus • Common throughout the world but risk in developing nations • Effective vaccine available • No chronic state, <1% risk for FHF • FHF usually if have underlying liver disease or older age • No increase risk of FHF with pregnancy

  3. Hepatitis A IgM detectable at onset of symptoms and disappears by 3 months Hepatitis A IgG persists for years and indicates immunity Management Symptomatic therapy Hydration Rest Treat pruritus Enteric precautions Handwashing Prophylaxis – vaccine and Ig Hepatitis A

  4. Hepatitis E • Enterically-transmitted RNA virus • Endemic along equatorial regions • Central America, Mexico, Africa, Middle East, India, Asia, Southeast Pacific • HEVAB seen in 10-40% of adults > 25

  5. Hepatitis E • Fecal-oral spread (like HAV) • Person to person: Uncommon (unlike HAV) • Large outbreaks accounting for >50% of cases of acute sporadic hepatitis in adults and children in endemic areas • Severity associated with age and pregnancy • Mortality: 0.5-3.0%, pregnant pts:15-25%

  6. Hepatitis B • Outcomes after acute infection: • Infants - 90+% chronicity • Children - ~70% chronicity • Adults - <5% chronicty • Still #1 cause of HCC in world • Sexual and IVDU transmission common • Vaccine may eliminate in this country

  7. Hepatitis B • Must have HB s Ag positive • Then look at HB c Igs and e Ag/Ab • s Ab Positive denotes immune status (either by vaccine or infection) • Core- c Ab (IgG) defines infection w/ HBV but not protective • HBV DNA – detectable early, used to guide therapy

  8. Hepatitis B • To Assess: check serology (HBsAg, HBcAb, HBeAg, HBeAb), LFTs and liver biopsy as indicated • DNA level helpful with therapy • Assess presence of HCC (AFP, U/S vs. CT) • Assess status of liver disease- synthetic fxn (Alb,Bili,INR), presence stigmata CLD and portal HTN

  9. Serological Findings in HBV

  10. Asymptomatic Carriers + HBsAg Normal ALT - HBeAg HBVDNA < 103 Normal liver biopsy Low risk for cirrhosis/complications Active Disease +HBsAg +HBeAg HBV DNA > 104 +/- Abnormal ALT Active inflammation on liver biopsy Active replication/risk of transmission 15 to 40% risk of cirrhosis or complications Chronic HBV

  11. Chronic HBV Therapies available: • PEG interferon for 6-12 months • Nucleos(t)ides (lamivudine, adefovir, entecavir, telbivudine) • IFN may cause decomp in cirrhotics and withdrawal of nuc’s may cause flare HBV

  12. Hepatitis C • 1.8 % in US (about 4-5 million) • 85 % develop chronic infection • 8-20% develop cirrhosis • 5% will die of HCV • #1 reason for OLT today • Blood transfusion before 1992, IVDU, intranasal cocaine, tattoos, prison term, sexual transmission low

  13. Hepatitis C • HCV antibody by ELISA • RIBA- best used to confirm false pos Ab in low pretest probability group • HCV RNA (viral load) • Genotype • Liver biopsy

  14. Hepatitis C • Dual therapy with PEG Interferon and ribavirin is standard of care • Know: EVR, SVR, NR, relapser • Interferon alone yielded about 10-15% SVR • Ribavirin alone has NO role • Genotype 1 (noncirrhotic): 45-55% SVR (48-72 weeks tx) • Genotypes 2-3: 75-85% SVR (24 weeks tx)

  15. Hepatitis C TX CONTRAINDICATED if: • WBC < 3K • Platelets less than 70K (Lower now? 50K) • Severe depression • Renal failure • Untreated autoimmune disease • Decompensated liver disease with ascites/encephalopathy/variceal bleeds. (Tx is “liver transplant”!) • Retinopathy or neuropathy

  16. Hepatitis C Treatment side effects • Cytopenias; note hemolysis with ribavirin • Fatigue, viral symptoms • Depression and suicide • Auto-immune disorders (thyroid, Sjogren’s, SLE) • Teratogenesis with ribavirin (category X)

  17. Hepatitis C • Associated with cryoglobulinemia, PCT, MPGN, thyroid disease • Synergistic with alcohol and fat. • May be #1 cause of HCC in this country (caucasians) • HCC on the rise. Must screen cirrhotic patients with imaging and AFP q 6 months

  18. Reactivation Hep B • Something to consider in sick patients undergoing severe immunosuppression • Chemotx, BMT are the typical settings • PT with hx of HBcAb + (HBsAg negative) • Think about reactivation Hep B in immunosuppressed patients with jaundice, elevated LFTs • Extremely poor prognosis

  19. Alcoholic Liver Disease • Classic presentation of ETOH Hepatitis: RUQ pain/fever/jaundice (without biliary/gallbladder dz), leukocytosis • AST/ALT ratio >2, generally under 300-500 • Outcome assessment: Maddrey’s Discriminant Function = 4.6 x (PT - control PT) + bilirubin • DF > 32 high risk and merits steroids/Pentoxifylline • Caveat to steroids - not used with any signs of active infection or bleeding

  20. NAFLD/NASH • Common cause of LFTs < 4X nl, (if ETOH/Viral/other chronic hepatitis excluded). ALT>AST • Can be associated with progressive liver disease (#1 cause of cryptogenic cirrhosis) • Classic scenario: metabolic syndrome (overweight, impaired fasting glucose/DM, low HDL/high TG’s, SBP >130) • Liver biopsy can distinguish bland fatty liver from NASH • Treatment: weight loss, data on thiazolidinediones promising

  21. Autoimmune Hepatitis • Classic scenario-young female (there is a second peak in 60’s), constitutional sx’s, increased transaminases, globulin fraction, positive ANA and/or anti-smooth muscle antibodies • Treatment: steroids, azathioprine • Type 1 is “classic” w/ ANA/ASMA+ • Type 2 is anti-LKM1 Ab + (children)

  22. Primary Biliary Cirrhosis • Chronic cholestatic disease of middle aged mujeres • Sx: pruritus, fatigue • Most are asymptomatic at diagnosis (classically e alk phos and + anti-mitochondrial antibody [AMA]) • Other immune disease assoc: Sjogrens, CREST, scleroderma, celiac disease • UDCA works to delay/prevent cirrhosis and transplant

  23. Primary Sclerosing Cholangitis • Middle aged males with ulcerative colitis • 70% of pts with PSC have UC; 7% of pts with UC have PSC • Diagnose by finding beaded intra- and extrahepatic bile ducts on imaging (MRCP or ERCP) • Colitis and PSC - very high risk of colonic cancer • Treatment: none work, best is liver transplant (when severe) • 10% develop cholangiocarcinoma

  24. Fulminant Hepatic Failure • FHF = encephalopathy within 8 weeks of onset of sx • Elevated liver tests, TBili, or INR do not equal FHF in absence of encephalopathy • Major complications - cerebral edema (#1 cause death), infection, coagulopathy, renal failure • Treatment: support in ICU, then transplant

  25. Fulminant Hepatic Failure

  26. Liver Transplantation • Indicated in chronic liver pt with < 80-90% survival at 1 yr. Listing criteria now based on MELD (Bili, INR, Creat) - refer when MELD > 15 • “Status 1” = Fulminant hepatic failure • Contraindications: AIDS (HIV relative contraindication), extrahepatic malignancy, advanced cardiopulmonary disease, active drug/ETOH, MOSF, active infection, large liver tumor (>5 cm) or >3 tumors (>3cm), age>70

  27. Drug-Induced Liver Disease • Idiosyncratic (most) vs. dose related (e.g. Tylenol) • Hepatitis-like: INH, NSAIDs, sulfa • Cholestatic: estrogens, anabolic steroids, Augmentin, • Peliosis hepatis: anabolic/androgenic steroids, contraceptives • Fulminant: INH, acetaminophen, antbx’s • Steatosis: valproate, MTX, amiodarone, HAART • Granulomas: allopurinol, hydralazine, quinidine, procainamide

  28. Acetaminophen • #1 cause of FHF in US/UK • Usually more than 10g single dose (can be less in ETOH abuse), • Treatment: NG lavage/charcoal if < 4 hours, NAC 140 mg/kg load then 70 mg/kg q4h for 17 doses • Criteria for predicting HIGH mortality and need to be in transplant center: pH<7.3 OR, INR>6.5 + creatinine>3.4 + coma, OR factor V less than 10 % (i.e. King’s College criteria) • “Therapeutic misadventures”

  29. Hemochromatosis • Prevalence 1/250-300 Northern European descent, carrier rate 1/10-12 people • Autosomal recessive dz; HFE gene on Chromosome 6 • Accumulate iron through life • Cirrhosis starting in 40s • Iron also in heart, joints (fingers/wrist), pancreas, skin, pituitary, testes

  30. Hemochromatosis

  31. Hemochromatosis • Iron sat > 45% • Ferritin >1000 • HFE gene mutation is BEST TEST (homozygous for C282Y mutation or compound hetero for C282Y/H63D) • Liver biopsy – not needed for dx, useful to determine extent of liver disease

  32. Hemochromatosis

  33. Hemochromatosis • Phlebotomy for life (keep ferritin < 50) • Screen for cirrhosis and HCC • Refer for OLT if indicated. • If iron removed before cirrhosis; normal life expectancy • Complications: cirrhosis, HCC, cardiomyopathy, DM, hypogonadism • Need to recommend family screening: 25% chance in siblings, 5% in kids

  34. Wilson’s Disease • Rare disease (1 in 30,000 incidence) • Defect on chromosome 13 • Inability to transport copper within the hepatocyte • Deficiency of biliary copper excretion • Poor incorporation of copper into ceruloplasmin • Excess total body copper • Liver, brain, kidney, bone, cornea/lens, RBC

  35. Wilson’s Disease • Clinical presentation (usually before age 40) • Hepatic – acute/chronic hepatitis, ALF, cirrhosis • Neurologic – motor disorders • Psych – depression, personality changes, phobias • Lab diagnosis • Low serum ceruloplasmin • 24 hour urine copper • Liver copper • Tx: penicillamine, trientine, zinc • OLT for ALF or decompensated cirrhosis • Family screening!

  36. Alpha-1 Antitrypsin Deficiency • Most common metabolic disease of the liver • AAT degrades proteases (neutrophil elastase) • Increased risk of lung and/or liver disease • can have without the other • PiMM - normal phenotype • PiZZ phenotype - leads to disease • Dx: AAT phenotyping, liver bx to confirm • Tx: no tobacco, liver transplant for severe liver disease (lung disease consider AAT replacement therapy)

  37. Alpha-1 Antitrypsin Deficiency

  38. Budd-Chiari Syndrome • Hallmark is hepatic venous outflow obstruction • can occur from hepatic venules to right atrium • “classic” form = hepatic vein obstruction • Etiology: • Western countries: thrombosis from myeloproliferative (PCV), hypercoag states, OCP use • Asia/Africa: IVC webs • Hepatomegaly, RUQ pain, ascites

  39. Budd-Chiari Syndrome • Dx: Doppler US to visualize clot/lack of flow in hepatic veins • Tx: • anticoagulation, diuretics • decompression -TIPS or surgical portosystemic shunt. OLT for decompensated cirrhosis or failed shunts • NB: Sinusoidal obstruction syndrome - complication of bone marrow transplant. Hint is immediate weight gain (ascites) and jaundice 10-20 days after transplant

  40. Ischemic Hepatopathy • MARKED increase in ALT/AST - up to 100 x ULN - followed by rapid decline over 7 to 10 days. • Clue: marked bump in LDH and rise in creatinine • Can occur post resuscitation or low BP event • short latency (24 hours) after event • No liver specific therapy usually needed • Rarely leads to FHF and never causes cirrhosis

  41. Cirrhosis • Assess etiology: Rx specific • #1 cause in US – alcohol • NASH rising fast • Assess severity: Child’s class, MELD score • Assess OLT candidacy • Eval for decompensation: ascites, varices, PSE; prophylaxis as indicated • Screen for HCC : US and AFP every 6 months • Immunize against HAV/HBV

  42. Operative Risk • Acute Hepatitis - high mortality 9.5% • Bottom line: avoid surgery unless life-saving • Cirrhosis - Child’s Classification (TBili, Alb, ascites, PSE, and PT/INR) • A: 10% • B: 31% • C: 76% • MELD is being increasingly used for pre-op risk stratification

  43. Postoperative Jaundice • Intrahepatic Cholestasis: • Parenchymal disease: anesthesia, drugs, TPN, sepsis, ischemia • Increased bili load: transfusions, hemolysis, hematoma reabsorption, Gilbert’s • Extrahepatic Cholestasis: CBD stones, stricture, pancreatitis, acalculous cholecystitis • Undiagnosed cirrhosis pre-op up to 50% will develop jaundice post-op

  44. Varices Esophageal Gastric

  45. Variceal Hemorrhage • Most serious complication of portal HTN • 1/3 of cirrhosis-related deaths • 40-60% of cirrhosis pts develop varices • 30% of these pts will bleed within 2 yrs of dx • 30% mortality with bleeding episode • Rebleed risk is 30-55% • Best predictor of bleeding is variceal size • All patients with large varices should receive primary prophylaxis • Non-selective beta-blockers – current standard of care • Banding – reserved for BB failure/contraindications

  46. Variceal Hemorrhage • Treatment Modalities • Medical: • Octreotide, somatostatin, terlipressin, vasopressin • Antibiotic prophylaxis • Endoscopic: band ligation (sclerotherapy is second line treatment) • Balloon tamponade – temporary fix only • TIPS – reserved for failure of above • Tissue adhesive (fibrin glue) – for gastric varices • Avoid overresuscitation (increases splanchnic pressures) • Initial treatment successful in 90% • Continued banding and beta-blockers for secondary prophylaxis as outpatient

  47. Ascites • Most common form of decompensation in cirrhosis • 85% of cases of ascites are due to cirrhosis • Physical exam • Checking for flank dullness followed by shifting dullness is best test • Checking for a fluid wave or puddle sign (cruel) is poorly sensitive/specific

  48. Paracentesis - Indications • New onset ascites • ANY hospital admission for ANY patient with ascites (unexpected SBP in 10 - 27%) • Patients with ascites who demonstrate clinical deterioration • Evaluation of response to treatment for SBP • Therapeutic paracentesis for tense ascites

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