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ABIM Hepatology Board Review

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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 ... – PowerPoint PPT presentation

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Title: ABIM Hepatology Board Review


1
ABIM Hepatology Board Review
  • 6-10-09

2
2009 ABIM exam
3
2008 UNC ITE
  • Commonly missed Hepatology topics
  • Diagnose and Manage Gilberts 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

4
Roadmap
  • Abnormal LFTs
  • NAFLD/NASH
  • ETOH Liver Disease
  • Viral Hepatitis
  • AIH/PBC/PSC
  • Cirrhosis and its complications
  • ALF
  • Transplant
  • Liver disease in Pregnancy
  • Genetic Liver disease

5
Abnormal LFTs
  • 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

6
(No Transcript)
7
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

8
Steatohepatitis
  • Histologic findings include
  • Macrovesicular steatosis
  • Cytologic ballooning
  • Mallory bodies (eosinophilic concretions within
    ballooned hepatocytes)
  • Scattered lobular inflammation
  • Question 87

9
ETOH Liver Disease
  • ASTALT ratio 21 31
  • 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

10
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

11
(No Transcript)
12
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

13
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

14
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

15
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

16
AIH/PBC/PSC
  • PSC
  • Cholestatic LFTs, 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

17
Etiologies of Cirrhosis
  • Viral
  • Hepatitis B
  • Hepatitis C
  • Toxic
  • Alcohol, MTX
  • Metabolic
  • Non Alcoholic Fatty Liver disease (NAFLD)
  • Biliary
  • PSC
  • PBC
  • Genetic/Hereditary
  • Hemachromatosis
  • Wilsons Disease
  • Alpha 1 Antitrypsin
  • Inborn errors of metabolism
  • Others
  • Autoimmune Hepatitis
  • Congestive hepatopathy (aka Cardiac Cirrhosis)
  • Cystic Fibrosis

18
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

19
Assessing the Severity of Cirrhosis
  • 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

20
Complications of Cirrhosis
  • 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

21
Variceal Hemorrhage
  • 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

22
Variceal Hemorrhage
  • 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

23
Variceal Hemorrhage
  • Acute bleeding
  • Endoscopic variceal ligation banding
  • Sclerotherapy
  • Minnesota tube
  • Transjugular Intrahepatic Portosystemic Shunt
    TIPS
  • Question 4

24
Ascites
  • 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

25
SAAG

26
Ascites
  • Low Sodium Diet (lt2g/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

27
Spontaneous Bacterial Peritonitis
  • Peritoneal fluid with gt250 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

28
Hepatic Encephalopathy
  • 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

29
Pathophysiology of Hepatic Encephalopathy
  • 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.

30
Encephalopathy
  • 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)

31
Hepatorenal Syndrome
  • 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

32
Hepatorenal Syndrome
  • 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.

33
Hepatorenal Syndrome
  • 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)

34
Hepatorenal Syndrome
  • 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

35
Hepatopulmonary Syndrome
  • 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

36
Hepatopulmonary Syndrome
  • Etiology
  • Nitric oxide plays a central role in animal
    models
  • Diagnosis
  • Impaired DLCO on PFTs, 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.

37
Portopulmonary Hypertension
  • 1998 WHO symposium defined pulmonary HTN as mean
    PAP gt40mmHG (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.

38
Portopulmonary Hypertension
  • Diagnosis via echocardiography or right heart
    catheterization
  • Treatment
  • Similar to idiopathic pulm HTN anticoagulants,
    vasodilators, prostacyclins (Flolan). No good
    data, many contraindications.

39
Hepatocellular Carcinoma
  • 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

40
Hepatocellular Carcinoma
  • 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

41
Hepatocellular Carcinoma
  • Early Arterial Phase CT
  • heterogeneously enhancing mass 4-5 cm
  • Portal Venous Phase CT
  • Decreased enhancement, iso-enhancing with liver
  • Question 37

42
TIPS
  • 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

43
ALF
  • 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

44
Transplant
  • Status 1 Acute liver failure
  • Status 2 MELD scoring system
  • TB, Cr, INR.
  • Score 6-40. Eval _at_12, transpalnt over 15.
  • Most centers transplant in 22-26 range
  • Status 7 Inactive
  • Question 5
  • Question 43

45
Liver disease in Pregnancy
  • 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

46
Genetic Liver disease
  • Hemachromatosis
  • C282Y homozygote or C282Y / H63D heterozygote
  • Ferritin gt1000, sat gt90
  • Rx phlebotomy or chelation
  • Wilsons 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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