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Internal Medicine > JH IM Board Review - Acute and Chronic Liver Disease II > Flashcards

JH IM Board Review - Acute and Chronic Liver Disease II Flashcards

1
Q

MC liver disorder causing elevated liver enzymes in the USA?

A

NAFLD.

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2
Q

NAFLD - Prevalence:

A

40%, depending on definition used and population studied.

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3
Q

NAFLD - Epidemiology and race:

A

1/3 of cases of chronic liver disease in the primary care setting.

==> HISPANICS HIGH, AFRICAN AMERICANS LOW.

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4
Q

NAFLD - Leading cause of death:

A

Cardiovascular disease.

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5
Q

How many pts with NASH eventually progress to cirrhosis?

A

15-20%.

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6
Q

MAIN risk factor for NAFLD:

A

Metabolic syndrome ==> Mainly, OBESITY + DM.

==> Insulin resistance is a hallmark.

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7
Q

NAFLD - AST and ALT:

A

Usually AST/ALT <1, UNLESS ADVANCED FIBROSIS OR CIRRHOSIS ==> Then AST/ALT >1.

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8
Q

NAFLD - Hepatomegaly caused by …?

A

Steatosis.

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9
Q

NAFLD - How to Dx:

A

LIVER BIOPSY = GOLD STANDARD.

==> Reveals either steatosis or steatonecrosis +/- fibrosis.

==> In advanced stages, histology loses characteristic fatty infiltration and may be called “cryptogenic” cirrhosis.

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10
Q

NALFD - Tx:

A
  1. Weight loss, strict glucose control, Tx of HTN, and lipid management are recommended.
  2. Vitamin E (800 IU/day) ==> Improves liver histology in biopsy-proven NASH. Unknown effects in pts with DM or cirrhosis.
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11
Q

NAFLD - Insulin sensitizers and lipid-lowering agents?

A

NOT CONCLUSIVELY SHOWN BENEFIT.

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12
Q

NAFLD - Liver transplantation:

A

Prognosis is worse in:

  1. Pts aged 60 or older.
  2. BMI 30 or greater.
  3. DM.

==> Recurrence of NAFLD and NASH after transplantation is well-described.

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13
Q

Hereditary hemochromatosis (HH) - Genetics:

A
  1. C282Y homozygotes ==> 85-90%.
  2. H63D homozygotes.
  3. C282Y/H63D compound heterozygotes.

==> Numerous other mutations eg S65C, G93R.

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14
Q

HH - Pathogenesis:

A

Incr. intestinal iron ABSORPTION:

==> Possibly caused by decr. production of the hepatic hormone HEPCIDIN, resulting in hepatic accumulation of iron.

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15
Q

HH - NON-HFE-related HH include mutations of:

A
  1. Hemojuvelin (HJV) gene.
  2. Hepcidin (HAMP) gene.
  3. Transferrin receptor 2 (TfR2) gene.

==> AFRICAN IRON OVERLOAD (Bantu siderosis) is a non-HFE-related HH that is worsened by dietary iron loading.

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16
Q

HH more common in men or women?

A

10X more common in men.

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17
Q

HH - Hepatomegaly when?

A

EARLY in the disease.

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18
Q

HH - HCC risk?

A

SIGNIFICANT once cirrhosis develops.

==> 5%/y.

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19
Q

HH - Dx - Which clues should prompt HFE mutation analysis?

A
  1. Transferrin saturation >45%.
  2. Elevated FERRITIN in high-risk groups.

==> Gene analysis to detect C282Y or H63D mutations.

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20
Q

HH - Dx - What is the next step if compound heterozygote (C282Y/H63D), C282Y heterozygote, or non-C282Y is found?

A

Need to r/o CONCOMITANT liver or hematologic conditions that may lead to 2o iron overload.

  1. LIVER ==> HCV, HBV, ALD, NAFLD.
  2. HEMATOLOGIC ==> Thal major, frequent blood transfusions, chronic hemodialysis.
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21
Q

HH - Dx - Liver Bx:

A

When:

  1. Ferritin >1000μg/L.
  2. Elevated liver enzymes.

==> To stage degree of fibrosis or cirrhosis, or when not C282Y homozygote to r/o other liver diseases.

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22
Q

HH - Dx - Screen 1st-degree members:

A

Either via serum transferrin saturation and ferritin.

==> Or by genetic testing.

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23
Q

HH - Tx - Phlebotomy:

A

1 unit WEEKLY or BIWEEKLY (as tolerated) until goal serum ferritin of 50 to 100μg/L, followed by maintenance phlebotomy at intervals.

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24
Q

HH - Phlebotomy can improve:

A
  1. Cardiac function.
  2. Diabetes.
  3. Skin hyperpigmentation.
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25
Q

HH - Phlebotomy CANNOT improve:

A
  1. Arthropathy.
  2. Established cirrhosis.
  3. Testicular atrophy.
  4. Cardiomyopathy.
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26
Q

HH - Tx - Iron chelation (deferoxamine):

A

Reasonable alternative when phlebotomy IS RESTRICTED BY ANEMIA.

==> It is also the 1st line therapy for iron overload because of ineffective erythropoiesis.

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27
Q

HH - Tx - What to avoid?

A

VITAMIN C.

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28
Q

AAT Def. - Lung and liver disease?

A
  1. ABSOLUTE AAT deficiency leads to LUNG disease ALONE.

2. RELATIVE AAT deficiency leads to LIVER +/- LUNG disease.

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29
Q

AAT def. - Lung disease results from …?

A

An imbalance between destructive neutrophil elastase + protective AAT in the lung.

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30
Q

AAT def. - Liver injury results from …?

A

Intrahepatocytic AAT accumulation within the RER.

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31
Q

MC GENETIC cause of PEDIATRIC LIVER DISEASE:

A

AAT deficiency.

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32
Q

MC genetic cause of COPD:

A

AAT def.

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33
Q

AAT def. - Clinical presentation as early as?

A

4-8 weeks.

  1. Persistent jaundice.
  2. Elevated aminotransferases.
  3. Hepatomegaly.
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34
Q

AAT def. - Portal HTN may develop in …?

A

Late childhood or early adolescence.

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35
Q

AAT def. may rarely be associated with:

A
  1. Necrotizing panniculitis.

2. WEGENER.

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36
Q

AAT def. - Dx - When to consider screening?

A

In adults with chronic idiopathic hepatitis OR cryptogenic cirrhosis.

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37
Q

AAT def. - Dx - How to screen first?

A

Using SERUM AAT levels.

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38
Q

AAT def. - Dx - When to obtain the phenotype?

A

Phenotype analysis if AAT level is below normal.

==> ZZ phenotype associated with advanced liver disease.

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39
Q

AAT def. - Histopathology:

A

PAS(+) + Diastase-resistant globules in hepatocytic ER.

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40
Q

AAT def. - Tx - Under investigation?

A

IV infusions of AAT derived from pooled plasma or obtained by recombinant DNA methods.

==> Under investigation for LUNG disease.

==> NOT successful for LIVER disease.

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41
Q

AAT def. - Tx - Liver transplantation?

A

The ONLY VIABLE THERAPY for the liver disease component ==> CURATIVE.

==> 5y post-transplant survival of 80-85% in adults.

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42
Q

Wilson - ATP7B mutation:

A

==> Decr. biliary EXCRETION of hepatocellular copper.

==> Cu accumulation in the liver + brain + cornea + kidneys with subsequent injury thought to caused by oxidative stress.

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43
Q

ATP7B mutation also prevents integration …?

A

Of copper into ceruloplasmin (ie apoceruloplasmin).

==> Shortens ceruloplasmin’s half-life ==> Causing lower blood concentration.

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44
Q

Wilson - Prevalence:

A

30/ million.

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45
Q

Wilson disease - Acute liver failure can present with …?

A
  1. COOMBS-NEGATIVE hemolytic anemia.

2. Acute renal failure.

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46
Q

Wilson - AP?

A

Inappropriately SUBNORMAL.

47
Q

Wilson - In 1 study, AP/Bilirubin ratio …?

A

AP/Bil <4 + AST/ALT >2.2 ==> 100% sens and spec for identifying acute liver failure because of Wilson disease.

48
Q

Wilson - Can also affect with other organs (besides liver and neuro/psychiatric abnormalities):

A
  1. Kidneys (nephrolithiasis).
  2. Heart (cardiomyopathy).
  3. Pancreas (pancreatitis).
  4. Endocrine system (hypoparathyroidism, infertility).
  5. Skeletal system (osteoporosis).
49
Q

Wilson - Dx - When to suspect?

A

Consider in ANY individual between ages 3 and 55 with acute hepatitis or jaundice of unclear causes.

50
Q

Wilson - Dx - Kayser-Fleischer rings are present in …?

A

95% of pts with NEUROLOGIC manifestations.

==> ONLY 40-60% of pts with mainly HEPATIC manifestations.

51
Q

Kayser-Fleischer rings specific for Wilson?

A

NOT specific ==> Can manifest in CHRONIC CHOLESTATIC DISEASES.

52
Q

Wilson - Dx - Serum ceruloplasmin?

A

Usually markedly LOW.

==> May be NORMAL or even MILDLY ELEVATED in the setting of acute inflammation.

53
Q

Wilson - Dx - Urine copper excretion?

A

Typically >100μg, but even levels greater than 40μg warrant further work-up

==> NOT specific; levels greater than 100μg can be seen in AIH + other chronic liver diseases.

54
Q

Wilson - Dx - Penicillamine challenge during 24h urine collection?

A

Has only been standardized for children.

55
Q

Wilson - When to liver Bx?

A

When biochem is indeterminate.

==> Liver Bx for copper quantification and histology.

56
Q

Wilson - Liver Bx results?

A
  1. Copper >250μg/g of dry liver weight confirms diagnosis.

2. Copper <50μg/g in UNTREATED pts EXCLUDES Wilson.

57
Q

Wilson - Histopathology:

A

Early histology shows:

  1. Micro/macrovesicular steatosis.
  2. Glycogenated hepatocytic nuclei.
  3. Focal necrosis.
58
Q

Wilson - Tx - Primary Tx:

A

CHELATION with oral D-penicillamine or trientine.

==> TRIENTINE is better tolerated and more commonly used in the USA.

==> Neurologic exacerbation after starting Tx is more common with D-penicillamine.

==> Monitor Tx using 24h urinary copper excretion.

59
Q

Wilson - Tx - Role of oral zinc?

A

Induces enterocyte METALLOTHIONEIN ==> Binds dietary copper and prevents its GI absorption.

==> Use in PREsymptomatic pts or those on maintenance Tx.

60
Q

Wilson - Which foods to avoid?

A

Shellfish, mushrooms, chocolate, organ meats ==> High Cu concentrations.

61
Q

Wilson - Liver transplantation?

A

Corrects the fundamental defect in hepatic copper excretion and should be offered to pts with advanced or acute fulminant disease.

62
Q

Pregnancy-specific liver diseases - Abnormal liver tests occur in …?

A

3-5% of all pregnancies.

63
Q

Pregnancy-specific liver diseases - Etiology:

A

Several different disease processes but NO clearly defined causes.

64
Q

Pregnancy-specific liver diseases - Pathogenesis?

A

Abnormal INTRAMITOCHONDRIAL fatty acid oxidation has been linked to acute fatty liver of pregnancy (AFLP).

==> This is LESS established in HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome.

65
Q

Pregnancy-specific liver diseases - Mutations?

A
  1. Several possible mutations, particularly of long-chain 3-hydroxyachyl-CoA dehydrogenase (LCHAD).
  2. Babies of mothers with AFLP should undergo genetic screening for LCHAD mutations.
66
Q

Pregnancy-specific liver diseases - All pregnancy-specific liver diseases tend to be recurrent in subsequent pregnancies, except for …?

A

AFLP.

==> Notable exception is women who carry LCHAD mutation ==> Recurrence in 20-70% of subsequent pregnancies.

67
Q

Viral hep - MC CHRONIC VIRAL HEP WORLDWIDE?

A

HBV.

68
Q

Viral hep - MC CHRONIC VIRAL HEP IN THE USA?

A

HCV.

69
Q

HAV - How many are positive for Anti-HAV in the USA?

A

37%.

70
Q

HAV - Incubation period:

A

2 to 6 weeks.

71
Q

HAV - Transmission:

A

F-O transmission.

72
Q

HAV - Several clinical patterns:

A
  1. Self-limited hep +/- jaundice (typical).
  2. Prolonged cholestasis (uncommon).
  3. Protracted relapsing course (uncommon).
  4. Fulminant liver failure (rare).
73
Q

HAV - What percentage of pts develops a prolonged and relapsing course and with what is it associated?

A

2-15%.

  1. Arthritis.
  2. Vasculitis.
  3. Cryoglobulinemia.

==> Relapses resemble the original episode, including serum anti-HAV IgM positivity.

74
Q

HAV - Who is at risk for fulminant liver failure?

A
  1. Elderly.

2. Those with concomitant chronic liver disease.

75
Q

HAV - Chronic infection?

A

NEVER results in chronic infection.

76
Q

HAV vaccines for:

A
  1. Travelers to endemic areas.
  2. Military workers.
  3. IV drug users.
  4. Chronic liver disease.
  5. Family members of infected pts.
77
Q

HAV - Post exposure proph?

A

Ig prophylaxis is 80-90% effective if given WITHIN 2 WEEKS following exposure.

78
Q

HAV - Role of prednisolone?

A

In the PROLONGED CHOLESTATIC variant.

==> 30mg daily tapered over 21 days ==> MAY shorten the duration of JAUNDICE/PRURITUS.

79
Q

HBV - Epidemiology:

A

350 million individuals chronically infected worldwide.

==> Carrier rates vary greatly depending on geographic region.

80
Q

HBV - 3 categories of prevalence:

A
  1. LOW (0.1-2%) ==> USA, Australia, Western Europe.
  2. INTERMEDIATE (3-7%) ==> Japan, Mediterranean basin, Latin America, Middle East.
  3. HIGH (8-20%) ==> China, Southeast Asia, sub-Saharan Africa.
81
Q

What percentage of chronic HBV carriers eventually develops serious complications?

A

15-40%.

82
Q

High risk for vertical transmission?

A

85-90% in mothers with HIGH SERUM HBV DNA levels + Presence of HBeAg.

83
Q

HBV - High-risk adults:

A
  1. Health care workers.
  2. IVDA.
  3. Hemodialysis pts.
  4. MSM.
  5. Sexually promiscuous individuals.
  6. Institutionalized pts.
  7. Immigrants from HBV-endemic regions.
  8. Household members of chronic carriers.
84
Q

HBV - Chronic hep and fulminant liver failure?

A

<5% ==> Chronic hep.

0.1-0.5% ==> Fulminant liver failure.

85
Q

HBV - Chronicity for neonates and 1-5yo?

A

Neonates ==> 90% chronic hep B AT BIRTH.

1-5yo ==> 25-50%.

86
Q

HBV - Mortality from fulminant course?

A

80%.

87
Q

HBV - What is associated with an increased risk of HCC, even without cirrhosis?

A

Persistent presence of HBeAg + Elevated serum HBV DNA.

88
Q

HBV - Incubation?

A

1-4 months.

89
Q

HBV - Symptomatic acute hep (eg abdominal pain, low-grade fever, nausea, jaundice) occurs in …?

A

30% OF ADULTS.

10% OF CHILDREN younger than 4 years of age.

==> Clinical symptoms usually normalize after 1 to 3 months.

90
Q

HBV - Chronic inactive carriers with normal liver chemistries?

A
  1. Presence of anti-HBe.
  2. Minimal serum HBV DNA.
  3. At risk for HCC.

==> This phase of the disease is also known as IMMUNE TOLERANCE.

91
Q

HBV reactivation:

A
  1. Presence of anti-HBe.
  2. High serum HBV DNA.
  3. Intermittently elevated ALT.

==> Can occur in immunosuppression.

92
Q

HBV - Extrahepatic manifestations:

A

10-20% of chronic HBV.

  1. PAN.
  2. Membranous or MPGN.
  3. Mixed cryoglobulinemia.
93
Q

HBV - Dx:

A
  1. Serology.

2. Positive HBV DNA PCR.

94
Q

Serologic Dx of HBV - HBsAg seen in:

A
  1. Acute infection - Early period.
  2. Chronic infection.
  3. Reactivation/Exacerbation.
95
Q

Serologic Dx of HBV - Anti-HBs:

A
  1. Acute infection, recovery period.
  2. Resolved previous infection.
  3. HBV immunization.
96
Q

Serologic Dx of HBV - HBc-IgM:

A
  1. Acute infection, early period.
  2. Acute infection, window period.
  3. Reactivation/Exacerbation (+/-).
97
Q

Serologic Dx of HBV - HBc-IgG:

A
  1. Acute infection, window period.
  2. Acute infection, recovery period.
  3. Chronic infection.
  4. Reactivation/exacerbation.
  5. Resolved prenious infection.
98
Q

Serologic Dx of HBV - HBeAg:

A
  1. Acute infection, early period.
  2. Chronic infection (+/-).
  3. Reactivation/exacerbation (+/-).
99
Q

Serologic Dx of HBV - Anti-HBe:

A
  1. Acute infection, window period.
  2. Acute infection, recovery period.
  3. Chronic infection (+/-).
  4. Reactivation/Exacerbation.
  5. Resolved previous infection.
100
Q

Serologic Dx of HBV - HBV DNA:

A
  1. Acute infection, early period.
  2. Acute infection, window period.
  3. Acute infection, recovery period.
  4. Chronic infection (variable).
  5. Reactivation/exacerbation.
101
Q

HBV - Indications for treatment - HBeAg (+):

A

HBV DNA >20.000 IU/mL +

ALT >2X normal OR moderate inflammation/significant fibrosis on liver Bx.

102
Q

HBV - Indiciations for Tx - HBeAg (-):

A

HBV DNA >2.000 IU/mL +

ALT greater than 2x normal OR moderate inflammation/significant fibrosis on liver Bx.

103
Q

HBV - Indications for treatment - Presence of cirrhosis:

A

Presence of cirrhosis +

HBV DNA >2.000 IU/mL OR persistent ALT elevation.

104
Q

HBV - Tx - Drugs:

A
  1. Standard IFN-a.
  2. Peg IFN-a.
  3. Nucleos(t)ide analogues ==> Lamivudine, adefovir, entecavir, tenofovir, telbivudine.

==> Are all USA FDA-approved for the Tx of chronic HBV.

105
Q

HBV - Tx - Role of IFN-a:

A

Administered for a PREDEFINED duration and has the HIGHEST rate of HBeAg seroconversion (up to 50%).

==> Significant side effects (eg flu-like symptoms, bone marrow suppression, hair loss, and mood changes).

106
Q

HBV - Tx - Role of IFN-a - Predictors of HBeAg seroconversion include:

A
  1. Elevated pretreatment ALT.
  2. High histologic activity index.
  3. Low serum HBV DNA.
  4. HBV genotypes A and B.
107
Q

HBV - Tx - Role of IFN-a - Contraindications?

A

In decompensated liver disease.

108
Q

HBV - Tx - Role of entecavir and tenofovir:

A

Most impressive reductions in HBV DNA + The LOWEST resistance rates among the nucleos(t)ide analogues.

==> Variable durations of Tx depend on pretreatment HBeAg status and viral response.

109
Q

HBV - Tx - Role of entecavir and tenofovir - Durations of therapy:

A
  1. HBeAg (+) ==> Continue for AT LEAST 6 MONTHS AFTER HBeAg seroconversion + Undetectable HBV DNA.
  2. HBeAg (-) ==> Continue INDEFINITELY until HBsAg clearance.
  3. Decompensated cirrhosis ==> LIFELONG Tx.
110
Q

Acute HBV - Antiviral Tx?

A

NOT NEEDED.

> 95% spontaneous recovery among immunocompetent individuals.

111
Q

Acute HBV - When to use antiviral Tx?

A

Fulminant liver disease and protracted severe course.

112
Q

Acute HBV - WHAT antivirals to use?

A
  1. May use lamivudine or telbivudine when anticipating short Tx duration.
  2. Less preferable ==> Adefovir (weak antiviral activity, nephrotoxic potential) + Tenofovir (nephrotoxic potential).
113
Q

Acute HBV - IFN-a?

A

CONTRAINDICATED.

114
Q

HBV and HCC screening?

A

Routine HCC screening in HIGH RISK HBV carriers:

  1. Asian men >40.
  2. Asian women >50.
  3. Africans >20.
  4. Cirrhotic pts.

Internal Medicine (106 decks)

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