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ABIM Hepatology Board Review. 6-10-09. 2009 ABIM exam. 2008 UNC ITE. Commonly missed Hepatology topics Diagnose and Manage Gilbert’s Syndrome Elevated indirect bilirubin, esp. during stress or other illness Genetic test now avail, no treatment needed Diagnose and treat AIP

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2008 unc ite
2008 UNC ITE

  • Commonly missed Hepatology topics

    • Diagnose and Manage Gilbert’s Syndrome

      • Elevated indirect bilirubin, esp. during stress or other illness

      • Genetic test now avail, no treatment needed

    • Diagnose and treat AIP

      • Acute intermittent visceral pain and neurologic sx

      • Urine porphyrins (urine PBG – spot, followed by 24hr ALA PBG and porphyrin)

      • Rx: avoid stressors that exacerbate dz, IV Hemin products

      • Watch out for vampires

        • Red urine, phosphorescent teeth, photosensitive skin


Roadmap
Roadmap

  • Abnormal LFT’s

  • NAFLD/NASH

  • ETOH Liver Disease

  • Viral Hepatitis

  • AIH/PBC/PSC

  • Cirrhosis and its complications

  • ALF

  • Transplant

  • Liver disease in Pregnancy

  • Genetic Liver disease


Abnormal lft s
Abnormal LFT’s

  • Asymptomatic patient

  • History!

  • Conceptualize if the pattern

    • Hepatocellular injury (elevated AST, ALT)

    • Cholestatic injury (elevated Tbili, AP, GGT)

    • Assess synthetic function (Tbili, INR +/- Cr)

  • Most common causes are NAFLD, chronic hepatitis and ETOH


Nafld nash
NAFLD/NASH

  • NAFLD encompasses simple steatosis to NASH to cirrhosis

    • Highly prevalent ~30%

    • Not all steatosis progresses

    • NASH is high risk to progress

  • Risk Factors

    • DM, HTN, Hyperlipidemia,Central obesity

  • Rx is to control risk factors


Steatohepatitis
Steatohepatitis

  • Histologic findings include

    • Macrovesicular steatosis

    • Cytologic ballooning

    • Mallory bodies (eosinophilic concretions within ballooned hepatocytes)

    • Scattered lobular inflammation

      • Question #87


Etoh liver disease
ETOH Liver Disease

  • AST:ALT ratio 2:1 – 3:1

  • AST rarely exceeds 300-400 unless another process is present

  • Elevated GGT, AP may be normal

  • AFLD

  • ETOH hepatitis carries 50% mortality rate

    • Rx Pentoxiphylline 400 TID or prednisilone 40 QD x 1 month

      • Question #78

      • Question #88


Viral hepatitis
Viral Hepatitis

  • Acute Viral Hepatitis

    • Most commonly A & B, but all hepatotropic viruses can cause an acute illness

  • Tests

    • in general IgM for acute disease and IgG or total for chronic disease

  • A : HAV IgM

  • B : HBsAg, HBc IgM

  • C : HCV Ab*, HCV RNA

  • D : HBsAg*, HDV IgM

  • E : HEV IgM – RNA test now avail

    • Question #13

    • Question # 33


Viral hepatitis1
Viral Hepatitis

  • Chronic Viral Heptitis

    • A & E do not have a chronic form

    • D can only occur in setting of Chronic B

  • HBV

    • Only ~5% of adults get chronic HBV

    • 90% of neonates get chronic HBV

    • 20-30% progress to cirrhosis

    • Rx: INF vs. oral nucleoside analogues

      • Only treat those with active replication

      • Usually long term – few seroconvert


Viral hepatitis2
Viral Hepatitis

  • HCV

    • 70-80% exposed develop chronic infection

    • Current #1 indication for OLT in U.S.

  • Current therapy is Peg-INF and ribavirin

    • Genotype 1 most common in U.S. and most difficult to treat

    • 48 weeks of weekly sq injections and BID ribavirin

      • Monitor mental health (depression/suicide)

      • Cytopenias

      • Thyroid dysfunction

      • Flu-like symptoms

      • Fatigue

        • Question #73


Aih pbc psc
AIH/PBC/PSC

  • AIH

    • May present in asymptomatic patient or a life-threatening flare

    • 30-50 y.o. WF, AST/ALT 400-500

      • +ANA, elevated IG levels. Biopsy required for Dx

        • Plasma cell infiltrate, “interface hepatitis”

    • Treat with prednisone and long term AZA

    • Recurs after OLT, but good survival


Aih pbc psc1
AIH/PBC/PSC

  • PBC

    • 90% cases are women

    • Puritus and fatigue most common symptoms

    • Disease of mostly intrahepatic bile ducts

    • Elevated AP, GGT, +AMA, +ANA

      • Some patients have clinically identical dz but AMA(-)

        • “autoimmune cholangiopathy” or “seronegative PBC”

    • Rx Urso 12-15mg/Kg/day


Aih pbc psc2
AIH/PBC/PSC

  • PSC

    • Cholestatic LFT’s, +pANCA, +ASMA, ERCP required for dx (MRCP?)

    • Large duct disease

      • Usually widespread but patients may have a “dominant stricture”

      • “Beads on a string” found at ERCP

      • 70-80% also have IBD (UC)

      • 10-15% develop cholangiocarcinoma

    • Rx Urso, treat dominant strictures, treat episodes of cholangitis, OLT

      • Question #25


Etiologies of cirrhosis

Viral

Hepatitis B

Hepatitis C

Toxic

Alcohol, MTX

Metabolic

Non Alcoholic Fatty Liver disease (NAFLD)

Biliary

PSC

PBC

Genetic/Hereditary

Hemachromatosis

Wilson’s Disease

Alpha 1 Antitrypsin

Inborn errors of metabolism

Others

Autoimmune Hepatitis

Congestive hepatopathy (aka Cardiac Cirrhosis)

Cystic Fibrosis

Etiologies of Cirrhosis


Pathophysiologic changes in cirrhosis

The liver is unable to process bile resulting in jaundice and scleral icterus

Bile pigments deposit in the skin causing pruritus

The liver cannot produce clotting factors resulting in coagulopathy

The liver cannot make albumin contributing to ascites and edema

Capacity to metabolize drugs is reduced leading to longer half lives of active metabolites and accumulation of toxic byproducts

Hypoglycemia may occur in end stage disease

Scarring leads to increased resistance of blood flow through the liver and results in portal hypertension

Pathophysiologic Changes in Cirrhosis


Assessing the severity of cirrhosis
Assessing the Severity of Cirrhosis and scleral icterus

  • Liver biopsy can give grade of inflammation and stage of fibrosis

  • Model for End-Stage Liver Disease (MELD) uses objective data to predict 3 month survival

    • Used in transplant selection

    • Bilirubin, INR, Creatinine

  • Child-Pugh score

    • Divides cirrhotics into class A,B,C to predict 1-2 year mortality

    • Bilirubin, INR, Albumin, Ascites, Encephalopathy


Complications of cirrhosis
Complications of Cirrhosis and scleral icterus

  • Once a patient develops complications of cirrhosis they have decompensated disease.

    • Variceal hemorrhage

    • Ascites

    • Spontaneous bacterial peritonitis

    • Hepatorenal syndrome

    • Hepatopulmonary syndrome

    • Portopulmonary hypertension

    • Hepatocellular carcinoma

    • Hepatic encephalopathy


Variceal hemorrhage
Variceal Hemorrhage and scleral icterus

  • Most devastating complication of cirrhosis and portal hypertension

    • Occurs in 25-40% of cirrhotics

    • Prior to current therapy, mortality 30% for a single episode and only 1/3 survived for one year afterwards

    • Mortality remains high


Variceal hemorrhage1
Variceal Hemorrhage and scleral icterus

  • Therapy

    • Prevention!

      • All patients with cirrhosis need a screening EGD

      • Non selective beta-blockers reduce portal hypertension

      • Endoscopic variceal ligation now accepted as primary prophylaxis as well in new AASLD guidelines


Variceal hemorrhage2
Variceal Hemorrhage and scleral icterus

  • Acute bleeding

    • Endoscopic variceal ligation “banding”

    • Sclerotherapy

    • Minnesota tube

    • Transjugular Intrahepatic Portosystemic Shunt “TIPS”

      • Question #4


Ascites
Ascites and scleral icterus

  • Cirrhosis (75%)

    • Most common cause of ascites

    • Most common complication of cirrhosis

    • Other causes occur more frequently in cirrhotics

  • Malignancy (10%)

  • Cardiac (3%)

  • TB (2%)

  • Pancreatic Ascites(1%)

  • SAAG calculation


SAAG and scleral icterus


Ascites1
Ascites and scleral icterus

  • Low Sodium Diet (<2g/d)

  • Managed with Diuretics

    • Furosemide for volume

    • Spironolactone for renin-angitensin system and some diuresis

  • Must watch renal function and sodium closely

    • Diuretic dosing often limited by hyponatremia

    • Diuretic induced acute or chronic renal failure is common

  • Diuretic refractory/resistant ascites requires another intervention

    • TIPS, Denver shunt, routine LVP


Spontaneous bacterial peritonitis
Spontaneous Bacterial Peritonitis and scleral icterus

  • Peritoneal fluid with >250 PMN

  • Typically due to bacterial translocation from gut

    • E. coli is the most common pathogen

  • Treat with 3rd gen. cephalosporin or quinolone

  • Secondary Prophalaxis

    • Norfloxacin 400mg qd

    • Ciprofloxacin 750mg q week

    • SMZ-TMP never has been tested in a trial with mortality but used frequently


Hepatic encephalopathy
Hepatic Encephalopathy and scleral icterus

  • Reversible decrease in neurologic function

    • May be subtle from sleep disturbance to outright coma with focal neurologic signs

    • Often a precipitating factor

      • Infection

      • Volume depletion/dehydration

      • Hyponatremia

      • Acidosis

      • drugs


Pathophysiology of hepatic encephalopathy
Pathophysiology of Hepatic Encephalopathy and scleral icterus

  • Nitrogenous substances derived from the gut adversely affect brain function

  • Ammonia is the best known metabolite associated with HE

  • Compounds gain access to the systemic circulation via decreased metabolism in liver and/or portosystemic shunts

  • Experimental models describe derangements in glutamine, serotonin, GABA, and catecholamine metabolism.


Encephalopathy
Encephalopathy and scleral icterus

  • First line therapy is lactulose/bowel catharsis

    • Theoretically lactulose acidifies bowel and prevents NH3 absorption

  • Non absorbable ABX

    • Rifaximin

    • Metronidazole

    • Neomycin

  • Misc

    • Zinc

    • Special diets (medium/branched chain FA)


Hepatorenal syndrome
Hepatorenal Syndrome and scleral icterus

  • Development of acute renal failure in a patient with cirrhosis or fulminant hepatic failure

    • End stage of a sequence of events that reduces perfusion of kidneys

  • Clinical presentation

    • Oliguiria

    • Low urine sodium (often undetectable)

    • Bland urine sediment

    • Systemic hypotension

    • Absence of another cause of renal failure


Hepatorenal syndrome1
Hepatorenal Syndrome and scleral icterus

  • Type I

    • 50% reduction of plasma creatinine clearance to a level below 20ml/min or doubling of serum creatinine in less than 2 weeks. Rapidly fatal.

  • Type II

    • Less severe than type I, more indolent and primarily characterized by diuretic refractory ascities.


Hepatorenal syndrome2
Hepatorenal Syndrome and scleral icterus

  • Treatment options

    • Liver transplant. Renal dysfunction improves after transplant.

    • Midodrine (alpha-1 agonist) and Octreotide (somatostatin analog)

      • Midodrine promotes systemic vasoconstriction, octreotide inhibits vasodilitaion of splanchnic vasculature. End result is improved perfusion of kidneys.

    • Norepinephrine

    • Vasopressin analogs

    • TIPS (controversial)

    • Dialysis (only as a bridge to transplant)


Hepatorenal syndrome3
Hepatorenal Syndrome and scleral icterus

  • Prevention is key in certain clinical situations

    • SBP – administration of albumin

    • Primary prophylaxis for SBP in patients with low albumin also reduces HRS

    • Alcoholic Hepatitis – use of steroids or pentoxiphylline


Hepatopulmonary syndrome
Hepatopulmonary Syndrome and scleral icterus

  • HPS

    • Liver disease

    • Increased alveolar-arterial gradient on room air

    • Evidence of intrapulmonary vascular dilitations (intrapulmonary shunting)

  • Platypnea and orthodeoxia are the major symptom and sign

  • No consistent correlation with severity of liver disease

    • Presence of HPS independently worsens prognosis of liver disease

    • Spider angiomata and hyperdynamic circulation associated with HPS


Hepatopulmonary syndrome1
Hepatopulmonary Syndrome and scleral icterus

  • Etiology

    • Nitric oxide plays a central role in animal models

  • Diagnosis

    • Impaired DLCO on PFT’s, evidence of intrapulmonary shunt on contrast enhanced echocardiography, technetium labeled macro-aggregated albumin scan, and pulmonary angiography

  • Treatment

    • Minimal role for medical therapy

    • Supplemental oxygen

    • Liver transplant best treatment, reversal of shunting in most patients post transplant.


Portopulmonary hypertension
Portopulmonary Hypertension and scleral icterus

  • 1998 WHO symposium defined pulmonary HTN as mean PAP >40mmHG (TR jet 3.0-3.5 m/s) and portopulmonary HTN as pulm HTN in association with portal HTN without other risk factors for secondary pulmonary HTN.

  • Etiology unknown

    • Presumed vasoactive substances like seratonin, IL-1, endothelin-1, glucagon, secretin, thromboxane B2 and VIP that are produced by splanchnic circulation and not metabolized by liver plus a genetic predisposition.


Portopulmonary hypertension1
Portopulmonary Hypertension and scleral icterus

  • Diagnosis via echocardiography or right heart catheterization

  • Treatment

    • Similar to idiopathic pulm HTN: anticoagulants, vasodilators, prostacyclins (Flolan). No good data, many contraindications.


Hepatocellular carcinoma
Hepatocellular Carcinoma and scleral icterus

  • 5th most common cancer worldwide

    • nearly 1,000,000 new cases annually as of 2007

    • increasing incidence

  • HBV single most important etiologic factor worldwide

    • Risk increased without cirrhosis

  • HCV and ETOH main risk factors in West


Hepatocellular carcinoma1
Hepatocellular Carcinoma and scleral icterus

  • Diagnosis possible without biopsy

    • AFP not sensitive or specific, levels over 200ng/dL highly suspicious for HCC

    • characteristic appearance with contrasted imaging

      • HCC has arterial blood supply that demonstrates uptake during early arterial phase and contrast washout in the delayed venous phase

      • biopsy is warranted in a liver mass larger than 2 cm without typical radiographic findings and no elevation in AFP


Hepatocellular carcinoma2
Hepatocellular Carcinoma and scleral icterus

  • Early Arterial Phase CT

    • heterogeneously enhancing mass 4-5 cm

  • Portal Venous Phase CT

    • Decreased enhancement, iso-enhancing with liver

      • Question #37


TIPS and scleral icterus

  • Transjugular Intrahepatic Portosystemic Shunt

    • Shunt placed between hepatic vein and portal vein with goal of reducing portal hypertension

    • Utilized for varices and refractory ascites

    • Controversial for HRS and HPS

    • Lethal for PP-HTN

    • Complications include hepatic encephalopathy and hepatic failure


ALF and scleral icterus

  • Encephalopathy that develops within 8 weeks of acute severe liver injury

    • Definition has transplant implications

    • Acetaminophen most common in U.S.

    • Know the difference between labs showing liver injury and synthetic dysfunction

      • e.g. decrease in transaminases after acute HBV infection, but INR drifting upwards…

    • Rapid referral to transplant center

      • Question #43


Transplant
Transplant and scleral icterus

  • Status 1 : Acute liver failure

  • Status 2 : MELD scoring system

    • TB, Cr, INR.

    • Score 6-40. Eval @12, transpalnt over 15.

      • Most centers transplant in 22-26 range

  • Status 7 : Inactive

    • Question #5

    • Question #43


Liver disease in pregnancy
Liver disease in Pregnancy and scleral icterus

  • HEV, VZV, HSV hepatitis may be more severe, but risk of acquisition unchanged

  • Cholestatsis of pregnancy

    • Puritus can be severe

    • Check fasting bile acids

      • Close monitoring (mom is ok, risk to fetus), urso, deliver baby

  • HELLP

    • Hemolysis, elevated LFTs, low platelets

      • Deliver baby

  • Acute fatty liver of pregnancy

    • Large, acutely swollen liver on U/S

      • Deliver baby

        • Question #29


Genetic liver disease
Genetic Liver disease and scleral icterus

  • Hemachromatosis

    • C282Y homozygote or C282Y / H63D heterozygote

      • Ferritin >1000, % sat >90%

    • Rx phlebotomy or chelation

  • Wilson’s Disease

    • Ceruloplasm is LOW!

      • KF rings, 24 hr urine copper confirm dx

  • A1AT

    • Homozygous ZZ most common genetic liver disease in kids

    • Often emphysema develops later

      • Question #19


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