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Internal Medicine > Davidson - Liver and Biliary Tract Disease > Flashcards

Davidson - Liver and Biliary Tract Disease Flashcards

1
Q

What do we look for in the hands during examination for liver and biliary disease?

A
  1. Clubbing
  2. Dupuytren’s contracture
  3. Leuconychia
  4. Bruising
  5. Flapping tremor (hepatic encephalopathy)
  6. Palmar erythema
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2
Q

What do we look for in the face during examination for liver and biliary disease?

A
  1. Jaundice
  2. Spider naevi
  3. Parotid swelling
  4. Xanthelasma and jaundiced sclera in a patient with chronic cholestasis.
  5. Kayser-Fleischer rings in Wilson’s disease.
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3
Q

What do we look for in the chest during examination for liver and biliary disease?

A
  1. Loss of body hair
  2. Gynecomastia
  3. Spider naevi
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4
Q

What do we look for in the abdomen during examination for liver and biliary disease?

A
  1. Scars
  2. Distention
  3. Veins
  4. Testicular atrophy
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5
Q

What do we find through palpation/percussion/auscaltation of the abdomen during examination for liver and biliary disease?

A
  1. Hepatomegaly
  2. Splenomegaly
  3. Ascites
  4. Palpable gallbladder
  5. Hepatic bruit (rare)
  6. Tumor
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6
Q

What do we look for in the legs during examination for liver and biliary disease?

A
  1. Bruising

2. Edema

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7
Q

What are the presenting features of liver disease that indicate impairment of liver function?

A
  1. Jaundice –> Failure of bilirubin clearance.
  2. Encephalopathy –> Failure of clearance of by-products of metabolism.
  3. Bleeding
  4. Hypoglycemia
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8
Q

What are the presenting clinical features of liver disease that indicate ongoing presence of aetiological factors (e.g. alcohol)?

A
  1. Effects of aetiological agent, e.g. intoxication, withdrawal, cognitive impairment vs
  2. Effects of liver injury from agent, e.g. encephalopathy.
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9
Q

What are the presenting clinical features of liver disease that indicate effects of chronic liver injury (>6months)?

A
  1. Catabolic status (+/- poor nutrition) –> Skin thinning (paper-money skin), loss of muscle bulk, leuconychia.
  2. Impaired albumin synthesis.
  3. Reduced aldosterone clearance.
  4. Reduced estrogen clearance.
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10
Q

How do we assess encephalopathy?

A

FLAPPING TREMOR:
Jerky forward movements every 5-10sec when arms are outstretched and hands are dorsiflexed suggest hepatic encephalopathy.
COARSER movement than those of tremor.

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11
Q

How do we assess clinically hepatomegaly?

A
  1. Start in the right iliac fossa.
  2. Process up the abdomen 2cm with each breath (through open mouth).
  3. Confirm the lower border of the liver by percussion.
  4. Detect if smooth or irregular, tender or non tender, ascertain shape.
  5. Identify the upper border by percussion.
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12
Q

What are the causes of EXUDATIVE ascites?

A
  1. Carcinoma –> Weight loss + hepatomegaly.

2. TB –> Weight loss + fever.

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13
Q

What are the causes of transudative ascites?

A
  1. Cirrhosis
  2. Renal failure (incl. nephrotic syndrome) –> Generalized and peripheral edema.
  3. Congestive heart failure (elevated jugular venous pressure).
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14
Q

How much does the liver weigh?

A

1.2-1.5 kg.

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15
Q

In how many segments is the liver divided according to subdivisions of the hepatic and portal veins?

A

8

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16
Q

What are the main features of zone 1 hepatocytes (periportal)?

A
  1. Good oxygen supply
  2. Gluconeogenesis
  3. Bile salt formation
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17
Q

What are the main features of zone 3 hepatocytes?

A
  1. Mono-oxygenation
  2. Glycolysis
  3. Lipolysis
  4. Glucuronidation
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18
Q

What is the space of Disse?

A

The space between the hepatocytes and leaky sinusoids.

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19
Q

What does the space of Disse contain?

A

Stellate cells that store vitA and play an important role in regulating liver blood flow.

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20
Q

What is the portal venous contribution to the liver blood supply?

A

50-90%.

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21
Q

What are the dimensions of the common bile duct?

A

Approx:

5cm long + 4-6mm wide.

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22
Q

What is the EXACT location of the gallbladder?

A

Pear-shaped sac typically lying under the right hemiliver, with its fundus located anteriorly behind the tip of the 9th COSTAL CARTILAGE.

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23
Q

What is characteristic of the cystic duct mucosa?

A

Has prominent crescentic folds - Valces of Heister - giving it a beaded appearance on cholangiography.

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24
Q

Mention some general important functions of the liver.

A
  1. Nutrient metabolism
  2. Protein synthesis
  3. Immune functions
  4. Excretion
  5. Storage
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25
Q

What protein does the liver synthesize?

A
  1. Albumin
  2. Coagulation factors
  3. Complement factors
  4. Haptoglobin
  5. Ceruloplasmin
  6. Transferrin
  7. Protease inhibitors (alpha-1 antitrypsin)
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26
Q

What does the liver excrete?

A
  1. Bile salts
  2. Bilirubin
  3. Drugs
  4. Phospholipid
  5. Cholesterol
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27
Q

How much albumin does the liver produce each day?

A

8-14g.

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28
Q

What is now recognized to play a key part in the pathogenesis of hep C?

A

Lipids - facilitating viral entry into hepatocytes.

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29
Q

What is important to keep in mind about deranged PT or INR seen in liver disease?

A

May not directly equate to increased bleeding risk –> these tests do NOT capture the concurrent reduced synthesis of anticoagulant factors including protein C and S.

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30
Q

How much unconjugated bilirubin is produced from the catabolism of heme daily?

A

250-300mg.

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31
Q

How much bile does the liver secrete daily?

A

1-2L.

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32
Q

Which vitamins are storaged in the liver in large amounts?

A

A, D, B12 –> Folate and vitK in smaller amounts.

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33
Q

What percentage of normal liver is composed of immune cells?

A

9%.

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34
Q

What immune cells do we find in the liver?

A
  1. Kupffer cells
  2. B and T cells
  3. Liver macrophages
  4. NK cells
  5. Atypical lymphocytes - phenotype between NK and T cells.
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35
Q

What do Kupffer cells constitute?

A

The largest single mass of tissue-resident macrophages in the body and account for 80% of the phagocytic capacity of this system.

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36
Q

What is the importance of investigation in the management of liver disease?

A
  1. Identification of the presence of liver disease.
  2. Establishing etiology
  3. Understanding disease severity (identification of cirrhosis with its complications).
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37
Q

What are the aims of investigations in patients with suspected liver disease?

A
  1. Detect hepatic abnormality
  2. Measure the severity of liver damage
  3. Detect pattern of liver function test abnormality: hepatitic, or obstructive/cholestatic.
  4. Identify the specific cause
  5. Investigate possible complications
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38
Q

What is the plasma half-life of albumin?

A

About 2 weeks - Albumin levels may be normal in ACUTE liver failure but are almost always reduced in CHRONIC liver failure.

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39
Q

ALT or AST is more specific for hepatocellular damage?

A

ALT.

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40
Q

What is basically the ALP?

A

The collective name given to several different enzymes that hydrolyze phosphate esters at alkaline pH.

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41
Q

What are the main sites of production of ALP in the body?

A
  1. The liver
  2. GI
  3. Bone
  4. Placenta
  5. Kidney
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42
Q

What is GGT?

A

A microsomal enzyme found in many cells and tissues of the body.

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43
Q

Where do we find the highest concentrations of GGT?

A

In the liver where it is produced by hepatocytes and by the epithelium lining small bile ducts.

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44
Q

What is the function of GGT?

A

To transfer glutamyl groups from gamma-glutamyl peptides to other peptides and amino acids.

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45
Q

What does isolated elevation of the serum GGT indicate?

A

Relatively common - May occur during ingestion of microsomal enzyme-inducing drugs, including alcohol, but also in NAFLD.

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46
Q

What other biochemical abnormalities may be found in patients with liver disease?

A
  1. Hyponatremia –> severe liver disease due to production of ADH.
  2. Serum UREA may be REDUCED. (Incr. in GI hemorrhage).
  3. High urea + raised bilirubin + high Cr + low urinary sodium –> Hepatorenal syndrome.
  4. Very high ferritin –> hemochromatosis.
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47
Q

Which drugs may increase levels of GGT?

A
  1. Barbiturates
  2. Carbamazepine
  3. Ethanol
  4. Griseofulvin
  5. Isoniazid
  6. Rifampicin
  7. Phenytoin
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48
Q

What abnormalities may be found in the peripheral blood count?

A
  1. Normocytic normochromic anemia –> Recent GI hemorrhage.
  2. Macrocytosis –> Alcohol misuse + target cells in any jaundice patient also result in macrocytosis.
  3. Leukopenia
  4. Leukocytosis with cholangitis, alcoholic hep, hepatic abscesses.
  5. Thrombocytopenia –> Both from hypersplenism + reduced prod. of thrombopoietin.
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49
Q

Can thrombocytosis occur in patients with liver disease?

A

It is unusual but may occur in those with active GI hemorrhage and, rarely, in hepatocellular carcinoma.

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50
Q

What is the normal half-life of vitK-dependent coagulation factors?

A

5-72h.

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51
Q

What are the main screening tests for chronic liver disease?

A
  1. Hep B surface antigen
  2. Hep C antibody
  3. Liver autoantibodies
  4. Immunoglobulins
  5. Ferritin
  6. Alpha-1 antitrypsin
  7. Ceruloplasmin
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52
Q

What is the clinical clue of alcoholic liver disease?

A

History

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53
Q

What is the initial test for alcoholic liver disease?

A
  1. LFTs –> AST>ALT

2. High MCV.

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54
Q

What additional test for alcoholic liver disease may be ordered?

A

Random blood alcohol.

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55
Q

What is the clinical clue for NAFLD?

A

Metabolic syndrome (central obesity, diabetes, HTN).

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56
Q

What is the initial test in NAFLD?

A

LFTs.

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57
Q

What additional tests may be ordered in NAFLD?

A

Liver biopsy.

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58
Q

What is the initial test for chronic hep B/C?

A

HBsAg and HCV antibody.

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59
Q

What additional tests may be ordered in chronic hep B/C?

A

HBeAg
HBeAb
HBV-DNA
HCV-RNA

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60
Q

What is the clinical clue for PBC?

A

Itching - raised ALP.

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61
Q

What is the initial test for PBC?

A

Antimitochondrial antibody (AMA).

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62
Q

What additional tests may be ordered in PBC?

A

Liver biopsy.

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63
Q

What is the clinical clue in PSC?

A

IBD

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64
Q

What is the initial test for PSC?

A

MRCP

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65
Q

What additional tests may be ordered for PSC?

A

ANCA

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66
Q

What is the clinical clue for autoimmune hepatitis?

A

Other autoimmune diseases.

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67
Q

What is the initial test for autoimmune hepatitis?

A

ASMA, ANA, LKM, immunoglobulin.

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68
Q

What additional tests may be ordered in autoimmun hepatitis?

A

Liver biopsy.

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69
Q

What is the clinical clue for hemochromatosis?

A

Diabetes/ joint pain.

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70
Q

What is the initial test for hemochromatosis?

A

Transferrin sat and ferritin.

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71
Q

What additional test may be ordered for hemochromatosis?

A

HFE gene test.

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72
Q

What is the clinical clue of Wilson disease?

A

Neurological signs and hemolysis.

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73
Q

What is the initial test for Wilson disease?

A

Ceruloplasmin.

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74
Q

What additional tests may be ordered for Wilson disease?

A

24h urinary copper.

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75
Q

What is the clinical clue for alpha-1 antitrypsin deficiency?

A

Lung disease.

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76
Q

How is US-guided liver biopsy performed?

A

Percutaneously with Trucut or Menghini needle, usually through an intercostal space under local anesthesia, or radiologically using a transjugular approach.

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77
Q

What are the conditions required for a safe percutaneous liver biopsy?

A
  1. Cooperative patient
  2. PT 80x10^9/L
  3. Exclusion of bile duct obstruction, localised skin infection, advanced COPD, marked ascites + severe anemia.
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78
Q

What are the main complications of percutaneous liver biopsy?

A
  1. Abdominal and/or shoulder pain.
  2. Bleeding
  3. Biliary peritonitis
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79
Q

When can liver biopsies be carried out in patients with defective hemostasis?

A

IF:

  1. The defect is corrected with fresh frozen plasma and platelet transfusion.
  2. Biopsy is obtained by the transjugular route.
  3. The procedure is conducted percutaneously under US control and the needle track is then plugged with procoagulant material.
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80
Q

In what patients should we ALSO avoid liver biopsies?

A

In patients with potentially resectable malignancy –> potential risk of tumor dissemination.
Operative or laparoscopic liver biopsy may sometimes be valuable.

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81
Q

Non-invasive markers of liver fibrosis have been developed and can reduce the need for liver biopsy to assess the extent of fibrosis in some settings. Mention some of them.

A
  1. Alpha-2 macroglobulin
  2. Haptoglobin
  3. Routine clinical biochemistry tests
    Used in the Fibrotest.
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82
Q

More recently newer classifications have been developed to reflect differences in presentation and outcome of acute liver failure. Mention one of them.

A

Divides acute liver failure into hyperacute, acute, and subacute:
Hyperacute –> Jaundice to encephalopathy in Jaundice to encephalopathy in 8-28d.
Subacute –> Jaundice to encephalopathy in 29d-12wks.

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83
Q

In hyperacute, acute, or subacute ALF is cerebral edema common?

A

In hyperacute + acute.

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84
Q

What percentage of ALF remains the cause unknown?

A

10% –> Cryptogenic or Non A-E viral hepatitis.

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85
Q

What is the cardinal manifestation of ALF?

A

Cerebral disturbance –> Hepatic encephalopathy and/or cerebral edema).
But in the early stages this can be mild and episodic and so its absence does not exclude a significant acute liver injury.

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86
Q

What are the initial features of ALF?

A

Often subtle:

  1. Reduced alertness
  2. Poor concentration
  3. Progressing through behavioral abnormalities –> restlessness and aggressive outbursts, to drowsiness and coma.
  4. Cerebral edema may occur –> with its complications.
  5. General features –> Weakness, nausea, vomiting.
  6. OCCASIONALLY –> right hypochondrial discomfort.
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87
Q

In ALF can death occur before jaundice?

A

Yes –> Jauncide may not be present at the outset and sometimes it is rare (Reye’s).

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88
Q

How do we assess clinical grade of hepatic encephalopathy?

A

Through 4 grades.

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89
Q

What are the clinical signs of grade 1 hepatic encephalopathy?

A
  1. Poor concentration
  2. Slurred speech
  3. Slow mentation
  4. Disordered sleep rhythm
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90
Q

What are the clinical signs in grade 2 hepatic encephalopathy?

A
  1. Drowsy but easily rousable.
  2. Occasional aggressive behavior.
  3. Lethargic
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91
Q

What are the clinical signs of grade 3 hepatic encephalopathy?

A
  1. Marked confusion
  2. Drowsy
  3. Sleepy but responds to pain and voice
  4. Gross disorientation
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92
Q

What are the clinical signs of grade 4 hepatic encephalopathy?

A
  1. Unresponsive to voice
  2. May or may not respond to painful stimuli
  3. Unconscious
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93
Q

What are the causes of ALF?

A

Drugs –> 70-80%
Cryptogenic –> 5-10%
Viral infections –> 5%
Poisons –> <5%

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94
Q

Which drugs may cause ALF?

A
  1. Paracetamol
  2. Halothane
  3. Antituberculous drugs
  4. Ecstasy
  5. Herbal remedies
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95
Q

Mention some miscellaneous causes of ALF?

A
  1. Wilson disease
  2. Acute fatty liver of pregnancy
  3. Shock and cardiac failure
  4. Budd-Chiari syndrome
  5. Leptospirosis
  6. Liver metastases
  7. Lymphoma
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96
Q

What is the best screening test for acute hep B infection?

A

Hep B core IgM antibody –> Liver damage is due to the immunological response to the virus, which has often been eliminated, and the test for HBsAg may be negative.

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97
Q

What is the test of greatest prognostic value in ALF?

A

PT - should be carried out at least twice daily.

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98
Q

Mention some major investigations to determine the cause of ALF?

A
  1. Toxicology screen of blood and urine.
  2. HBsAg, IgM anti-HBc.
  3. IgM anti-HAV
  4. Anti-HEV, HCV, CMV, HSV, EBV
  5. Ceruloplasmin, serum copper, urinary copper, slit-lamp eye examination.
  6. Autoantibodies: ANA, ASMA, LKM, SLA (soluble liver antigen).
  7. Immunoglobulins
  8. US of liver and Doppler of hepatic veins.
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99
Q

Mention some adverse prognostic criteria in ALF due to paracetamol toxicity? (mortality >90% –> referral for possible liver transplantation).

A

H>50nmol/L (pH3.38mg/dL +

  1. PT >100 secs +
  2. Grade 3 or 4 encephalopathy.
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100
Q

Mention some adverse prognostic criteria in ALF in non-paracetamol cases? (mortality >90% –> referral for possible liver transplantation).

A 
PT>100secs OR
ANY three of the following:
1. Jaundice to encephalopathy in >7d.
2. Age 40.
3. Indeterminate or drug-induced causes.
4. Bilirubin >17.6mg/dL
5. PT>50secs. OR
Factor V level <15% and encephalopathy grade 3 or 4.
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101
Q

What does the cardiorespiratory monitoring in ALF involve?

A
  1. Pulse
  2. BP
  3. Central venous pressure
  4. Respiratory rate
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102
Q

What does the neurological monitoring in ALF involve?

A
  1. Intracranial pressure monitoring

2. Conscious level

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103
Q

What does the fluid balance monitoring in ALF involve?

A
  1. Hourly output (urine, vomiting, diarrhea).

2. Input: oral, IV.

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104
Q

What does the blood analyses monitoring in ALF involve?

A
  1. Arterial blood gases
  2. Peripheral blood count (incl. platelets
  3. Na, K, HCO3, Ca, Mg
  4. Creatinine, urea
  5. Glucose (2hourly in acute phase)
  6. PT
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105
Q

What does the infection surveillance monitoring in acute liver failure involve?

A
  1. Cultures: Blood, urine, throat, sputum, cannula sites.
  2. CXR
  3. Temperature
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106
Q

What are the possible complications of ALF?

A
  1. Encephalopathy + cerebral edema.
  2. Hypoglycemia
  3. Metabolic acidosis
  4. Infection (bacterial, fungal)
  5. Renal failure
  6. Multi-organ failure (hypotension and respiratory failure)
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107
Q

What is the 1-year survival following liver transplantation for ALF?

A

About 60% and improving.

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108
Q

What must be done when abnormal LFTs are detected?

A
  1. Thorough history should be compiled.
  2. Determine patients alcohol consumption.
  3. Drug use (prescribed drugs or otherwise).
  4. Risk factors for viral hepatitis (blood transfusions, injecting drug use, tattoos).
  5. Presence of autoimmune diseases
  6. Family history
  7. Neurological symptoms
  8. Features of metabolic syndrome
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109
Q

What must be done when we find incr. bilirubin ONLY in an asymptomatic patient?

A

Recheck with conjugated bilirubin –> exclude hemolysis –> Reassure, as likely Gilbert’s syndrome.

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110
Q

What must be done if increased GGT ONLY is found in asymptomatic patients?

A

Determine whether:

  1. NAFLD/incr. BMI –> Stage disease –> Lifestyle modifications.
  2. Enzyme induction from drugs –> Review current medication.
  3. Alcohol –> Alcohol abstinence.
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111
Q

What must be done when abnormal ALP or serum transaminases <2x upper limit are found in an asymptomatic patient?

A
  1. Check GGT if raised ALP –> Alcohol abstinence, stop hepatotoxic drugs, advise weight loss if BMI>25.
  2. Recheck LFT in 3-6months.
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112
Q

What must be done if LFTs are persistently abnormal or we have an abnormal ALP and serum transaminases >2x upper limit in an asymptomatic patient?

A

Liver screen, i.e.

  1. Full history
  2. Chronic liver disease screen
  3. US abdomen
  4. HBsAg
  5. HCV Ab
  6. Alpha-1 AT
  7. Autoimmune profile
  8. Ferritin
  9. Ceruloplasmin
  10. Immunoglobulins
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113
Q

Mention some causes of mild elevation of serum transaminases (<100U/L)?

A
  1. Chronic hep C
  2. Chronic hep B
  3. Hemochromatosis
  4. Fatty liver disease
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114
Q

Mention some causes of moderate elevation of serum transaminases (100-300 U/L).

A

As with mild elevation plus:

  1. Alcoholic hepatitis
  2. Non-alcoholic hepatitis
  3. Autoimmune hepatitis
  4. Wilson’s disease
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115
Q

Mention some causes of major elevation of serum transaminases (>300U/L).

A
  1. Drugs
  2. Acute viral hep
  3. Autoimmune liver disease
  4. Ischemic liver
  5. Toxins (e.g. Amanita phalloides poisoing)
  6. Flare of chronic hep B.
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116
Q

Mention some causes of INTRAHEPATIC cholestatic jaundice.

A
  1. PBC
  2. PSC
  3. Alcohol
  4. Drugs
  5. Hepatic infiltrations (lymphoma, granuloma, amyloid, metastases)
  6. Cystic fibrosis
  7. Severe bacterial infections
  8. Pregnancy
  9. Inherited cholestatic liver disease, e.g. benign recurrent intrahepatic cholestasis
  10. Chronic right heart failure
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117
Q

Mention causes of EXTRAHEPATIC cholestatic jaundice.

A
  1. Carcinoma –> Ampullary, pancreatic, bile duct (cholangiocarcinoma), liver metastases.
  2. Choledocholithiasis
  3. Parasitic infection
  4. Traumatic biliary strictures
  5. Chronic pancreatitis
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118
Q

What symptoms may be key history points in patients with jaundice?

A
  1. Itching PRECEDING jaundice
  2. Abdominal pain (suggests stones)
  3. Weight loss (chronic liver disease or malignancy)
  4. Dark urine and pale stools
  5. Fever+/- rigors
  6. Dry eyes, dry mouth
  7. Fatigue
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119
Q

When is jaundice usually clinically detectable?

A

When the plasma bilirubin exceeds 2.5mg/dL.

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120
Q

What happens in pre-hepatic jaundice?

A

Cause by either hemolysis or by congenital hyperbilirubinemia and is characterized by an isolated raised bilirubin level.

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121
Q

What is the most common form of non-hemolytic hyperbilirubinemia?

A

Gilbert syndrome.

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122
Q

What happens in hepatocellular jaundice?

A

The concentrations of BOTH unconjugated and conjugated bilirubin increase –> Due to acute or chronic liver disease.

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123
Q

Besides viral infection and drugs, what else can elevate the ALT >1000?

A

Hepatic ischemia.

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124
Q

What can cause cholestatic (obstructive) jaundice?

A
  1. Failure of hepatocytes to initiate bile flow.
  2. Obstruction of the bile ducts or portal tracts.
  3. Obstruction of bile flow in the extrahepatic bile ducts between the porta hepatis and the papilla of Vater.
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125
Q

What are the early features of cholestasis?

A
  1. Jaundice
  2. Dark urine
  3. Pale stools
  4. Pruritus
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126
Q

What are the late features of cholestasis?

A
  1. Malabsorption (ADEK)
  2. Weight loss
  3. Steatorrhea
  4. Osteomalacia
  5. Bleeding tendency
  6. Xanthelasma and xanthomas
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127
Q

What are the clinical features of cholangitis?

A
  1. Fever
  2. Rigors
  3. Pain (if gallstones present)
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128
Q

What is likely the cause of static or increasing jaundice?

A
  1. Carcinoma
  2. PBC
  3. PSC
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129
Q

What is the most likely cause of fluctuating jaundice?

A
  1. Choledocholithiasis
  2. Stricture
  3. Pancreatitis
  4. Choledochal cyst
  5. PSC
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130
Q

What is the most likely cause if we have cholestatic jaundice with abdominal pain?

A
  1. Choledocholithiasis
  2. Pancreatitis
  3. Choledochal cyst
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131
Q

What is the most likely cause if we have cholestatic jaundice with cholangitis?

A
  1. Stone
  2. Stricture
  3. Choledochal cyst
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132
Q

What is the most likely cause if we have cholestatic jaundice with abdominal scar?

A
  1. Stone

2. Stricture

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133
Q

What is the most likely cause if we have cholestatic jaundice with irregular hepatomegaly?

A

HCC

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134
Q

What is the most likely cause if we have cholestatic jaundice with palpable gallbladder?

A

Carcinoma below the cystic duct (usually pancreas).

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135
Q

What is the most likely cause if we have cholestatic jaundice with abdominal mass?

A
  1. Carcinoma
  2. Pancreatitis (cyst)
  3. Choledochal cyst
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136
Q

What is the most likely cause if we have cholestatic jaundice with occult blood in stools?

A

Ampullary tumor.

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137
Q

What does the Courvoisier’s law suggest?

A

Jaundice due to a malignant biliary obstruction (e.g., pancreatic cancer).

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138
Q

What is the Charcot triad?

A

Characterizes cholangitis:

  1. Jaundice
  2. RUQ pain
  3. Fever
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139
Q

Mention some causes of hepatomegaly.

A
  1. Liver metastases
  2. Multiple or large hepatic cysts
  3. Cirrhosis (early): NAFLD, alcohol, hemochromatosis.
  4. Hepatic vein outflow obstruction.
  5. Infiltration: amyloid.
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140
Q

Mention some common causes of ascites.

A
  1. Malignant disease (hepatic, peritoneal).
  2. Cardiac failure
  3. Hepatic cirrhosis
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141
Q

Mention some other causes of ascites.

A
  1. Hypoproteinemia –> Nephrotic syndrome, protein-losing enteropathy, malnutrition.
  2. Pancreatitis.
  3. Lymphatic obstruction
  4. Hepatic venous occlusion (Budd-Chiari, veno-occlusive disease)
  5. Infection (TB)
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142
Q

Mention some rare causes of ascites.

A
  1. Meigs syndrome

2. Hypothyroidism

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143
Q

What happens in Meigs syndrome?

A

Right pleural effusion with or without ascites and a benign ovarian tumor –> Ascites resolves on removal of the tumor.

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144
Q

What is though to be the main factor leading to ascites in cirrhosis?

A

Splanchnic vasodilation.
Mediated by vasodilators (mainly NO) that are released when portal hypertension causing shunting of blood into the systemic circulation.

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145
Q

What is the appearance of the ascitic fluid in cirrhosis?

A

Clear, straw-coloured or light green.

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146
Q

What is the appearance of the ascitic fluid in malignant disease?

A

Bloody.

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147
Q

What is the appearance of the ascitic fluid in infection?

A

Cloudy.

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148
Q

What is the appearance of the ascitic fluid in biliary communication?

A

Heavy bile staining.

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149
Q

What is the appearance of the ascitic fluid in lymphatic obstruction?

A

Milky-white (chylous).

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150
Q

Mention some useful investigations regarding the ascitic fluid.

A
  1. Total albumin (plus serum albumin) and protein –> to calculate the serum-ascites albumin gradient (SAAG).
  2. Amylase
  3. Neutrophil count
  4. Cytology
  5. Microscopy and culture
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151
Q

What is the hepatic hydrothorax?

A

In 10% of patients with ascites, a RIGHT pleural effusion is found –> Most are small and only identified on CXR.

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152
Q

What is 96% predictive that ascites is due to portal HTN?

A

A gradient of more than 11g/L.

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153
Q

What is the main difference between transudate ascites caused by venous outflow obstruction due to cardiac failure and the one cause by cirrhosis?

A

Unlike cirrhosis, the TOTAL PROTEIN CONTENT is usually above 25g/L.

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154
Q

What identifies pancreatic ascites?

A

Ascites amylase activity above 1000U/L.

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155
Q

What does the management of ascites involve?

A
  1. Restricting Na and H2O intake.
  2. Diuretics –> No more than 1L/day removal.
  3. Paracentesis
  4. Patient should be weighed regularly.
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156
Q

Mention some drugs with high Na content that should be avoided in ascites.

A
  1. Alginates
  2. Antacids
  3. Antibiotics
  4. Phenytoin
  5. Sodium valproate
  6. Effervescent preparations (e.g. aspirin, Ca, paracetamol)
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157
Q

Mention some drugs that promote sodium retention and should be avoided in ascites.

A
  1. Carbenoxolone
  2. Corticosteroids
  3. Metoclopramide
  4. NSAIDs
  5. Estrogens
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158
Q

What is the 1st line diuretic for ascites?

A

Spironolactone (100-400mg/d).

It can cause painful gynecomastia and hyperK –> substitute with amiloride (5-10mg/d).

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159
Q

What may improve diuresis?

A

Bed rest –> More renal blood flow in the horizontal position.

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160
Q

Mention some complications of ascites.

A
  1. Renal failure
  2. Hepatorenal syndrome
  3. Spontaneous bacterial peritonitis
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161
Q

In what percentage of patients with advanced cirrhosis and ascites do we see hepatorenal syndrome?

A

10%.

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162
Q

How many types of hepatorenal syndrome do we have?

A

2 types.

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163
Q

What are the clinical features of type I hepatorenal syndrome?

A
  1. Progressive oliguria
  2. Rapid rise in serum Cr
  3. Very poor prognosis (death in less than 1 month without treatment).
  4. Usually NO proteinuria
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164
Q

What are the clinical features of type II hepatorenal syndrome?

A
  1. In patients with refractory ascites.
  2. Moderate and stable increase in serum Cr.
  3. Better prognosis than type 1.
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165
Q

Recurrence of spontaneous bacterial peritonitis is common. What may reduce this?

A

Prophylactic quinolones such as norfloxacin or ciprofloxacin.

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166
Q

What is the prognosis of ascites?

A

Only 10-20% of patients survive 5 years from the 1st appearance of ascites due to cirrhosis.
Mortality at 1 year is 50% following first episode of bacterial peritonitis.

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167
Q

What is basically hepatic encephalopathy?

A

A neuropsychiatric syndrome caused by liver disease.

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168
Q

What is the differential diagnosis of hepatic encephalopathy?

A
  1. Intracranial bleed (subdural, or extradural hematoma).
  2. Drug or alcohol intoxication.
  3. Delirium tremens/alcohol withdrawal.
  4. Wernicke’s encephalopathy.
  5. Primary psychiatric disorders
  6. Hypoglycemia
  7. Neurological Wilson disease
  8. Post-ictal state
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169
Q

Mention some factor that precipitate hepatic encephalopathy.

A
  1. Drugs (especially sedatives, antidepressants)
  2. Dehydration (including diuretics, paracentesis)
  3. Portosystemic shunt
  4. Infection
  5. Hypokalemia
  6. Constipation
  7. Increased protein load (including gi bleeding)
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170
Q

Are focal neurologic signs features of hepatic encephalopathy?

A

No - if present other causes must be sought.

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171
Q

What is the hepatocerebral degeneration?

A

CHRONIC hepatic encephalopathy –> Combination of symptoms: Cerebellar dysfunction, Parkinsonian syndromes, spastic paraplegia, and dementia.

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172
Q

What is the basis of the Pathophysiology of hepatic encephalopathy?

A

Disturbance of brain function provoked by circulating neurotoxins that are normally metabolized by the liver.

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173
Q

What do most patients with hepatic encephalopathy have?

A

Some degree of hepatic failure and portosystemic shunt.

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174
Q

Mention some substances that are considered important factors in hepatic encephalopathy.

A
  1. Ammonia
  2. Other nitrogenous substances produced in the gut by bacteria.
  3. GABA
  4. Octopamine
  5. Amino acids
  6. Mercaptans
  7. Fatty acids that act as neurotransmitters
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175
Q

How is the diagnosis of hepatic encephalopathy being made?

A

Clinically, although when doubt:

EEG shows diffuse slowing of the normal alpha waves with eventual development of delta waves.

176
Q

Can increased concentrations of ammonia be seen in the absence of hepatic encephalopathy?

A

Yes - so little diagnostic value.

177
Q

What are the principles to treat hepatic encephalopathy?

A

To treat or remove precipitating causes and to suppress the production of neurotoxins by bacteria in the bowel.

178
Q

What medication do we give to patients with hepatic encephalopathy?

A
  1. Lactulose 15-30mL 3x daily –> osmotic laxative.

2. Rifaximin 400mg 3 times daily –> reduces bacterial content in the bowel.

179
Q

What are the MCCs of cirrhosis worldwide?

A
  1. Chronic viral hep
  2. Prolonged excessive alcohol consumption
  3. NAFLD
180
Q

Mention some causes of cirrhosis.

A
  1. Alcohol
  2. Chronic viral hep B,C
  3. NAFLD
  4. Immune –> PSC and autoimmune liver disease.
  5. Biliary –> PBC, secondary biliary cirrhosis, cystic fibrosis.
  6. Genetic –> Wilson, alpha-1 AT, hemochromatosis.
  7. Cryptogenic (15%)
  8. Chronic venous outflow obstruction
  9. Any chronic liver disease
181
Q

Mention some clinical features of cirrhosis when it is not asymptomatic.

A
  1. Hepatomegaly (although liver may be small)
  2. Jaundice
  3. Ascites
  4. Circulatory changes –> Palmar erythema, spider telangiectasia, cyanosis.
  5. Endocrine changes –> Loss of libido, hair loss, gynecomastia, testicular atrophy, impotence, Breast atrophy, irregular menses, amenorrhea.
  6. Hemorrhagic tendencies
  7. Portal HTN
  8. Hepatic (portosystemic) encephalopathy
  9. Other –> Pigmentation, clubbing, Dupuytren’s contracture.
182
Q

When is hepatomegaly due to cirrhosis common?

A

In ALD and hemochromatosis.

183
Q

When do we see a reduction in cirrhotic liver size?

A

In viral hep and autoimmune liver disease.

184
Q

Is palmar erythema of great diagnostic value?

A

Can be seen early in cirrhosis but it is of limited diagnostic value, as it occurs in many conditions associated with a hyperdynamic circulation, including normal pregnancy, as well as being found in some healthy people.

185
Q

What etiology is suggested by pigmentation in cirrhosis?

A

Hemochromatosis and in any cirrhosis associated with prolonged cholestasis.

186
Q

Central cyanosis is a late or early finding in cirrhosis?

A

Late –> Pulmonary AV shunts develop –> hypoxemia.

187
Q

Mention some features of chronic liver failure.

A
  1. Worsening synthetic liver function –> Prolonged PT and albumin.
  2. Jaundice
  3. Portal HTN
  4. Variceal bleeding
  5. Hepatic encephalopathy
  6. Ascites –> SBP, hepatorenal failure.
188
Q

What must be done once the diagnosis of cirrhosis is made?

A

Endoscopy for esophageal varices and repeated every 2 years.

189
Q

What is the prognosis in patients with cirrhosis?

A

Only 25% survive 5-year from diagnosis.
25% for up to 10 years.
More favorable prognosis for those with corrected underlying cause: alcohol misuse, hemochromatosis, Wilson.

190
Q

What is the normal hepatic venous pressure gradient?

A

5-6mmHg.

191
Q

What is the gradient in clinically significant portal HTN?

A

Above 10mmHg and risk of variceal bleeding increases beyond a gradient of 12mmHg.

192
Q

What are the 5 general categories of causes of portal HTN?

A
  1. Prehepatic presinusoidal
  2. Intrahepatic presinusoidal
  3. Sinusoidal
  4. Intrahepatic post Sinusoidal
  5. Post hepatic post sinusoidal
193
Q

What are the causes of pre hepatic pre Sinusoidal portal HTN?

A
  1. Portal vein thrombosis due to sepsis (umbilical, portal pyaemia) or procoagulopathy or secondary to cirrhosis.
  2. Abdominal trauma including surgery.
194
Q

What are the intrahepatic pre Sinusoidal causes of cirrhosis?

A
  1. Schistosomiasis
  2. Congenital hepatic fibrosis
  3. Drugs
  4. Vinyl chloride
  5. Sarcoidosis
195
Q

What are the Sinusoidal causes of portal HTN?

A
  1. Cirrhosis
  2. Polycystic liver disease
  3. Nodular reactive hyperplasia
  4. Metastatic malignant disease
196
Q

Mention an intrahepatic post Sinusoidal cause of portal HTN?

A

Veno-occlusive disease

197
Q

Mention a post hepatic post Sinusoidal cause of portal HTN?

A

Budd-Chiari syndrome.

198
Q

Mention some complications of portal HTN?

A
  1. Variceal bleeding –> esophageal, gastric, rectal (rare).
  2. Congestive gastropathy
  3. Hypersplenism
  4. Ascites
  5. Iron deficiency anemia
  6. Renal failure
  7. Hepatic encephalopathy
199
Q

What is the cardinal finding of portal HTN?

A

Splenomegaly

200
Q

What happens in Cruveilhier-Baumgarten syndrome?

A

A large umbilical collateral vessel has a blood flow sufficient to give a venous hum on auscaltation.

201
Q

What are the sites of portosystemic shunt in portal HTN?

A
  1. Distal esophagus
  2. Stomach
  3. Rectum
  4. Anterior abdominal wall
  5. Renal
  6. Lumbar
  7. Ovarian
  8. Testicular vasculature
202
Q

What is more common in hypersplenism seen in portal HTN? Thrombocytopenia, anemia, or leukopenia?

A

Thrombocytopenia.
Leukopenia occasionally.
If anemia is found, a source of bleeding sought be sought.

203
Q

What is the risk for variceal bleed in presence of portal hypertension?

A

7% for small Varices

30% for large ones.

204
Q

What does primary prevention of variceal bleeding involve?

A

Propranolol (80-160mg/day) or nadolol –> reduce portal venous pressure + HR.
Also selective carvedilol.
Caution in patients unable to tolerate beta blockers.

205
Q

What does the emergency management of bleeding involve?

A
  1. IV fluids (colloid)
  2. Vasopressor (terlipressin)
  3. Prophylactic antibiotics (cephalosporin IV)
  4. Emergency endoscopy
  5. Variceal band ligation
  6. PPI
  7. Phosphate enema and/or lactulose.
206
Q

What is the reason for the IV fluids and vasopressor (terlipressin) in emergency management of bleeding?

A

IV fluids –> Extracellular volume replacement.

Terlipressin –> reduces portal pressure, acute bleeding and risk of early rebleeding.

207
Q

What is the reason for prophylactic antibiotics, emergency endoscopy in emergency management of bleeding?

A

Prophylactic antibiotics –> reduces incidence of SBP.

Emergency endoscopy –> Confirms variceal rather than ulcer bleed.

208
Q

Why do we give phosphate enema and/or lactulose in emergency management of bleeding?

A

To prevent hepatic encephalopathy.

209
Q

What is the dose for terlipressin in esophageal variceal bleeding?

A

2mg IV 4x daily until bleeding stops, and then 1mg 4x daily up to 72 hours.

210
Q

What target group should be treated with caution when given terlipressin?

A

Ischemic heart disease or peripheral vascular disease patients.

211
Q

What happens in congestive gastropathy?

A

Seen in long standing portal HTN –> chronic congestion recognizable at endoscopy as multiple areas of punctuate erythema (“snake skin gastropathy).

212
Q

What is the best initial treatment for congestive gastropathy?

A

Propranolol (80-160mg/day) to reduce portal HTN.

213
Q

Mention some causes of viral hepatitis.

A
  1. Hep A
  2. Hep B +/- D
  3. Hep C
  4. Hep E
  5. CMV
  6. EBV
  7. HSV
  8. Yellow fever virus
214
Q

Mention the main complications of acute viral hepatitis.

A
  1. Acute liver failure
  2. Cholestatic hepatitis (hep A)
  3. Aplastic anemia
  4. Chronic liver disease and cirrhosis (hep B and C)
  5. Relapsing hepatitis
215
Q

What are the incubation periods for the 5 hep viruses?

A 
A--> 2-4weeks.
B--> 4-20weeks.
C--> 2-26weeks.
D--> 6-9weeks.
E--> 3-8weeks.
216
Q

For which hep viruses do we have a vaccine?

A

A and B.

217
Q

In occasional outbreaks, what is the vehicle of hep A transmission?

A

Water and shellfish.

218
Q

What is the Dane particle?

A

Hep B virus without HBsAg.

219
Q

How many people have serological evidence of past or current infection with hep B?

A

1/3 of the population and approx. 350-400million are chronic carriers.

220
Q

What is the MCC of hep B infection worldwide?

A

Vertical transmission from the mother to child in the perinatal period and carries the highest risk of ongoing chronic infection.

221
Q

Mention the main sources of hep B infection and risk of chronic infection.

A 
Horizontal (10%):
1. Injection drug use
2. Infected unscreened blood products
3. Tattoos/acupuncture needles
4. Sexual (homosexual, heterosexual)
5. Close living quarters/playground.
Vertical transmission (90%): HBsAg positive mother.
222
Q

What mechanisms contribute to an initially absent adaptive immune response to HBV?

A
  1. Introduction of antigen in the neonatal period is tolerogenic.
  2. Presentation of such antigen within the liver, promotes tolerance.
  3. Very high loads of antigens may lead to “exhaustion” of cellular immune responses.
    However the state of tolerance is not permanent.
223
Q

In how many phases can we divide chronic hep B infection?

A

5 phases.

224
Q

What does HBsAg indicate?

A

Active infection.

225
Q

What are the 5 phases of chronic hep B infection?

A
  1. Immune tolerant phase
  2. Immune reactive HBsAg positive chronic hep phase
  3. Inactive carrier phase
  4. HBeAg-negative chronic hep phase
  5. HBsAg-negative phase
226
Q

What is the serology of immune tolerant phase?

A 
HBsAg +
HBeAg +
Anti HBe Ab -
Viral load +++
ALT --> Normal/low.
227
Q

What are the main features of immune tolerant phase?

A
  1. Prolonged in perinatally infected individuals.
  2. May be short or absent if infected as an adult.
  3. High viral load and so very infectious.
228
Q

What is the serology of immune reactive HBsAg positive chronic hep B phase?

A 
HBsAg +
HBeAg + 
Anti HBe Ab -
Viral load ++
ALT raised
229
Q

What are the features of “immune reactive” HBeAg positive chronic hep phase?

A
  1. May last weeks or years.
  2. High risk of cirrhosis or HCC if prolonged.
  3. Increased chance of spontaneous loss of HBeAg with seroconversion to anti-HBe Ab-positive state.
230
Q

What is the serology of inactive carrier phase?

A 
HBsAg +
HBeAg -
Anti-HBe Ab + 
Viral load +/-
ALT normal
231
Q

What is the main feature of inactive carrier phase?

A

Low risk of cirrhosis or HCC in majority.

232
Q

What is the serology of HBeAg-negative chronic hep phase?

A

HBsAg +
HBeAb -
Anti-HBe Ab +
Viral load fluctuating/raised

233
Q

What are the main features of HBeAg-negative chronic hep phase?

A
  1. May represent late immune Reactivation or presence of pre-core mutant HBV.
  2. High risk of cirrhosis or HCC.
234
Q

What is the serology of HBsAg negative phase?

A 
HBsAg -
HBeAg -
Anti-HBe Ab +
Viral load -/+-
ALT normal
235
Q

What is the main feature of HBsAg negative phase?

A

Ultrasensitive techniques may detect low levels of HBV even after HBsAg loss.

236
Q

What does HBeAg indicate?

A

Viral replication - reflects active replication of the virus in the liver.

237
Q

What is the expected serology for the incubation period of HepB?

A

HBsAg +
Anti HBc IgM +
Anti-HBC IgG -
Anti-HBs -

238
Q

What is the expected serology of early acute hep B?

A

HBsAg +
Anti HBc IgM +
Anti-HBC IgG -
Anti-HBs -

239
Q

What is the expected serology of established hep B?

A

HBsAg +
Anti HBc IgM +
Anti-HBC IgG +
Anti-HBs -

240
Q

What is the occasional serology of established hep B?

A

HBsAg -
Anti HBc IgM +
Anti-HBC IgG -
Anti-HBs -

241
Q

What is the serology of convalescence (3-6months) after hep B?

A

HBsAg -
Anti HBc IgM +/-
Anti-HBC IgG +
Anti-HBs +/-

242
Q

What is the serology of Convalescence (6-9months) after hep B?

A

HBsAg -
Anti HBc IgM -
Anti-HBC IgG +
Anti-HBs +

243
Q

What is the serology of post infection hep B?

A

HBsAg -
Anti HBc IgM -
Anti-HBC IgG +
Anti-HBs +/-

244
Q

What is the serology of immunization without infection of hep B?

A

HBsAg -
Anti HBc IgM -
Anti-HBC IgG -
Anti-HBs +

245
Q

What does the absence of HBeAg usually imply?

A

Low viral replication - EXCEPTION HBeAg-negative chronic hep B - pre-core mutant.

246
Q

What is the magnitude of viral load during active viral replication of HBV?

A

10^5 copies/mL, as indicated by the presence of e antigen.

247
Q

How many HBV genotypes have been identified using PCR?

A

A-H.

A responds better to pegylated IFN-alpha than C and D.

248
Q

What percentage of hep B progress to acute liver failure?

A

Less than 1%.

249
Q

What percentage of hep B infections recovers fully?

A

90-95%.

250
Q

How much time must pass for recovery from acute hep B to occur?

A

6 months characterized by the appearance of antibodies to viral antigens.

251
Q

What is the goal of treatment in hep B?

A
  1. HBeAg seroconversion
  2. HBV-DNA reduction
  3. Normalization of the LFTs
252
Q

What are the two different types of drugs used to treat hep B?

A
  1. Direct acting nucleoside/nucleotide analogues

2. Pegylated IFN-alpha

253
Q

What are the at-risk groups meriting hep B vaccination in low-endemic areas?

A
  1. Parenteral drug users
  2. Men who have sex with men
  3. Close contacts of infected individuals
  4. Patients on chronic hemodialysis
  5. Patients with chronic liver disease
  6. Medical, nursing and lab personnel
254
Q

Which virus is more infectious between HBV, HCV and HIV?

A

HBV 10x more infectious than HCV which is 10x more infectious than HIV.

255
Q

How much time may it take for anti HCV antibodies to appear in the blood following acute infection such as a needlestick injury?

A

6-12 weeks –> HCV RNA can be found as early as 2-4 weeks.

256
Q

What are the risk factors for acquisition chronic hep C infection?

A
  1. IV drug misuse (95% of cases in UK).
  2. Unscreened blood products
  3. Vertical transmission 3% risk
  4. Needlestick injury 3% risk
  5. Iatrogenic parenteral transmission (I.e. Contaminated vaccination needles).
  6. Sharing toothbrushers/razors
257
Q

How many HCV genotypes exist?

A

6 common viral genotypes.
No effect on progression of disease but does affect response to treatment.
1 is more common in Northern Europe and less easy to eradicate with traditional IFN-alpha and rivabirin-based treatment than 2 and 3.

258
Q

What is the most common scoring system used in hep C?

A

The Metavir system, which scores fibrosis from 1-4, the latter equating cirrhosis.

259
Q

What is the current treatment for hep C?

A

Triple therapy –> Rivabirin + Pegylated IFN-alpha + PI such as telaprevir and boceprevir.

260
Q

What are the risk factors for progression of hep C to cirrhosis?

A
  1. Male gender
  2. Immunosuppression (such as Coinfection with HIV)
  3. Prothrombotic states
  4. Heavy alcohol
261
Q

Mention some causes of abnormal liver blood tests in HIV infection?

A 
Hepatitic blood tests:
1. Chronic hep C and B
2. Antiretroviral drugs
3. CMV 
Cholestatic blood tests:
1. TB
2. Atypical mycobacterium
3. Sclerosing Cholangitis due to microsporidia
262
Q

How are liver abscesses classified?

A
  1. Pyogenic
  2. Hydatid
  3. Amoebic
263
Q

What is the mortality of liver abscesses?

A

20-40%.

264
Q

What is the MCC of death in patients with liver abscess?

A

Failure to make the diagnosis.

265
Q

Mention some causes of pyogenic liver abscesses.

A
  1. Biliary obstruction (Cholangitis)
  2. Hematogenous –> portal vein (mesenteric infections) or hepatic artery (bacteremia).
  3. Direct extensiom
  4. Trauma –> penetrating or non penetrating.
  5. Infection of liver or cyst.
266
Q

What are the usual suspects in Pyogenic liver abscesses?

A
  1. E.coli
  2. Strep milleri
  3. Anaerobes (bacteroides)
267
Q

Why is it often difficult to diagnose a liver abscess?

A

Atypical presentations are common.

Also necrotic colorectal metastases can be misdiagnosed as hepatic abscesses.

268
Q

What antibiotics should be given for a liver abscess?

A
  1. Metronidazole
  2. Ampicillin
  3. Gentamicin
269
Q

What is the etiologic agent of hydatid cysts?

A

Echinococcus granulosus.

270
Q

What is the structure of hydatid cysts?

A
  1. Outer layer derived from the host.
  2. Intermediate laminated layer
  3. Inner germinal layer
    Can be single or multiple.
271
Q

What happens to chronic hydatid cysts?

A

They are calcified.

272
Q

What does the treatment of hydatid cysts involve?

A
  1. Albendazole or mebendazole prior to definitive therapy.
  2. In the ABSENCE OF COMMUNICATIONS WITH THE BILIARY TREE –> percutaneous aspiration of the cyst followed by injection of 100% ETHANOL and then re-aspiration of the cysts contents (PAIR).
  3. When there is a communication –> surgery.
273
Q

What is the etiologic agent of amoebic liver abscesses?

A

Entamoeba histolytica.

274
Q

What is the average alcohol consumption of a man with cirrhosis?

A

160g/day for 8 years.

275
Q

Mention some risk factors for alcoholic liver disease.

A
  1. Drinking pattern
  2. Gender
  3. Genetics
  4. Nutrition
276
Q

Which gene has been recently implicated in the pathogenesis of ALD and NAFLD?

A

PNPLA3 - also known as adiponutrin.

277
Q

How much time does alcohol take to reach peak blood concentrations?

A

After 20 minutes.

278
Q

Mention some pathogenic features of alcoholic liver disease.

A
  1. Alcoholic hepatitis:
    a. Lipogranuloma
    b. Neutrophil infiltration
    c. Mallory’s hyaline
    d. Pericellular fibrosis
  2. Macrovesicular steatosis
  3. Fibrosis and cirrhosis
  4. Central hyaline sclerosis
279
Q

Describe the multiple pathways that lead to alcoholic liver disease.

A
  1. Alcohol –> Gut permeability –> Endotoxin –> Kupffer cells –> TNF-a + IL-6 –> Inflammation –> ALD.
  2. Alcohol –> Acetaldehyde –> Adducts –> ALD.
  3. Alcohol –> CYP2E1 –> Ox. stress, lipid peroxidation, low glutathione.
  4. Coexistent disorders, e.g. viral hep, hemochromatosis.
  5. Genetic susceptibility.
280
Q

What percentage of patients with severe alcoholic hepatitis, will also have cirrhosis at presentation?

A

80%.

281
Q

What are the 3 clinical syndromes of alcoholic liver disease?

A
  1. Fatty liver
  2. Alcoholic hepatitis
  3. Cirrhosis
282
Q

What are the features of fatty liver?

A
  1. Asymptomatic abnormal liver biochem.

2. Normal/large liver.

283
Q

What are the main features of alcoholic hepatitis?

A
  1. Jaundice
  2. Malnutrition
  3. Hepatomegaly
  4. Features of portal HTN (ascites, encephalopathy)
284
Q

Which biological marker may suggest and support a history of alcohol misuse?

A

Macrocytosis, particularly in the absence of anemia.

285
Q

What is the Maddrey score?

A

In alcoholic hepatitis –> Discriminant function (DF)
DF=[4.6x increase in PT (sec)] + Bilirubin (mg/dL).
Enables the clinician to assess prognosis.

286
Q

When are corticosteroids useful in alcoholic hepatitis?

A

In severe alcoholic hepatitis - Maddery score >32.

–> Improve survival at 28 days from 65% to 85%.

287
Q

What is the role of oral pentoxifylline in alcoholic hepatitis?

A

Reduces inpatient mortality, particularly from HEPATORENAL FAILURE, from 46% to 25%.

288
Q

What does NAFLD represent?

A

A spectrum of liver disease encompassing simple fatty infiltration (steatosis), fat and inflammation (NASH), and cirrhosis IN THE ABSENCE OF EXCESSIVE ALCOHOL CONSUMPTION.

289
Q

With what is NASH associated?

A
  1. Progressive liver fibrosis
  2. Cirrhosis
  3. Liver cancer
  4. Incr. CV risk
290
Q

With what other conditions is NAFLD associated?

A 
Strongly with:
1. Obesity
2. Diabetes type II
3. Dyslipidemia
May considered to be the hepatic manifestation of metabolic syndrome.
291
Q

What are the progressive changes from steatosis to NASH to Cirrhosis?

A 
Steatosis:
a. UP FA influx
b. DOWN FA oxidation
c. UP FA synthesis
d. DOWN VLDL assembly
e. Insulin resistance 
NASH
a. TNF-a
b. Oxidant stress
c. Endotoxin
d. Immune factors
Cirrhosis
a. TGF-beta
b. Stellate cell activation
292
Q

What is the 2-hit hypothesis for NAFLD?

A

“First hit” –> Steatosis

“Second hit” –> Progress to steatohepatitis

293
Q

What are the possible “hits” in NAFLD?

A
  1. Oxidative stress –> free radicals produced during FA ox.
  2. Direct lipotoxicity
  3. Gut-derived endotoxin
  4. Cytokine release (TNF-a)
  5. Endoplasmic reticulum stress
294
Q

What is the average age of NASH patients?

A

40-50

295
Q

What is the average age for NASH-cirrhosis?

A

50-60

296
Q

On what is the histological definition of NASH based?

A 
On a combination of 3 lesions:
1. Steatosis
2. Hepatocellular injury
3. Inflammation
with a mainly zone 3 distribution.
297
Q

What is important to keep in mind about the diagnostic process of NASH?

A

The hepatic fat content tends to diminish as cirrhosis develops and so NASH is likely to be under-diagnosed in the setting of advanced liver disease, where it is thought to be the underlying cause of 30-75% of cases in which no specific etiology is identified –> so-called “cryptogenic cirrhosis”.

298
Q

What are the two autoimmune disease patterns for the liver?

A
  1. Primary hepatocellular injury (autoimmune hep)

2. Biliary epithelial cell injury (primary biliary cirrhosis, and PSC)

299
Q

Which Ig is elevated in autoimmune hep?

A

IgG particularly.

300
Q

What is the suggested mechanism of autoimmune hep?

A
  1. Cross reactivity with viruses such as HAV and EBV in immunogenetically susceptible individuals.
  2. Typically, those with HLA-DR3 and 4.
301
Q

What is the most frequently seen autoantibody pattern in autoimmune hep?

A

High titre of ANA and ASMA + IgG hyperglobulinaemia. (type I autoimmune hep in old classification).

302
Q

What autoantibody pattern of autoimmune hep is associated with anti-LKM antibodies?

A

Pediatric autoimmune hep –> They recognize CYP450-IID6 expressed on the hepatocyte membrane.
More resistant to treatment than ANA-positive disease.

303
Q

Which infection can result in adult onset of anti-LKM?

A

Chronic hep C.

304
Q

What is the most recent autoantibody pattern of autoimmune hep that is recognized?

A

Anti-soluble liver antigen.

305
Q

What are the features of autoimmune hep?

A
  1. Insidious onset
  2. Fatigue, anorexia
  3. Jaundice
  4. Associated autoimmune diseases
  5. 25% have ACUTE onset –> Like viral hep, which does not resolve.
306
Q

Can we see ASMA antibodies in other conditions besides autoimmune hep?

A

Yes - Reported in infectious mononucleosis and a variety of malignant diseases.

307
Q

What does the liver biopsy in autoimmune hep show?

A

Interface hepatitis, with/without cirrhosis.

308
Q

Can autoimmune hep result in end-stage disease, despite treatment?

A

Yes - despite prednisolone and azathioprine.

309
Q

Mention some conditions associated with autoimmune hep.

A
  1. Migrating polyarthritis
  2. Urticarial rashes
  3. Lymphadenopathy
  4. Hashimoto
  5. Thyrotoxicosis
  6. Myxedema
  7. Pleurisy
  8. Coombs(+) hemolytic anemia
  9. Transient pulmonary infiltrates
  10. Ulcerative colitis
  11. Glomerulonephritis
  12. Nephrotic syndrome
310
Q

What is the typical presentation of PBC?

A
  1. Itching
  2. Tiredness
    May be found incidentally as the result of routine blood tests.
311
Q

Is smoking related to PBC?

A

Yes - more common among smokers.

312
Q

PBC is associated with which genetic loci?

A

HLA-DR8, IL-12, IL-12R.

313
Q

Where are the AMA of PBC directed?

A

At pyruvate dehydrogenase complex, a mitochondrial enzyme complex that plays a key role in cellular energy generation.

314
Q

Mention an PBC specific ANA.

A

Antibody against the nuclear pore antigen gp210.

315
Q

Unlike autoimmune hep, which Ig is frequently elevated in PBC?

A

IgM.

316
Q

What are the clinical features of PBC?

A
  1. Fatigue, which may precede diagnosis for years.
  2. Pruritus (may precede jaundice by months or years!)
  3. Itching usually worse at the limbs.
  4. May be right upper abdominal discomfort.
  5. Rarely, bone pain or fractures (from osteoporosis, osteomalacia).
317
Q

What conditions are associated with PBC?

A
  1. Autoimmune and connective tissue diseases occur with incr. frequency in PBC.
  2. Sicca syndrome
  3. Systemic sclerosis
  4. Celiac disease
  5. Thyroid diseaase (hypothyroidism should always be considered in patients with fatigue).
318
Q

Is hypercholesterolemia common in PBC?

A

Yes - not diagnostic, and worsens as disease progresses.

319
Q

What percentage of patients with PBC have AMA?

A

Over 95%.

320
Q

In PBC does US show any signs of biliary obstruction?

A

No.

321
Q

Is liver biopsy useful in PBC?

A

Only if there is diagnostic uncertainty - The histology correlates poorly with the clinical features.

322
Q

What is the management of PBC?

A

Hydrophilic ursodeoxycholic acid (UDCA) - 13-15mg/kg/day –>

  1. Improves bile flow.
  2. Replaces toxic hydrophobic bile acids in the bile acid pool.
  3. Reduces apoptosis of the biliary epithelium.
323
Q

What happens in patients who fail to respond to UDCA?

A

Have an incr. risk of developing end-stage liver disease compared to patients showing full response.

324
Q

In PBC, what is the most reliable marker of declining liver function?

A

Serum bilirubin.

325
Q

What is the 5-year survival after liver transplantation in PBC?

A

Over 80% –> Disease will RECUR in over 1/3 at 10 years.

326
Q

What is the cause of pruritus seen in PBC?

A

Main symptom requiring treatment.

Cause is unknown –> Up-regulation of opioid receptors + incr. levels of endogenous opioids may play a role.

327
Q

What do we give for the pruritus in PBC?

A

Colestyramine. (also rifampicin and naltrexone).

328
Q

What is the cause of fatigue in PBC?

A

Unknown –> It may reflect intracerebral changes due to cholestasis. (1/3 of patients with PBC).

329
Q

What happens in AMA-negative PBC?

A
  1. Features of PBC, but no AMA in the serum.
  2. LFTs, Ig levels, ANA titres –> Higher than in AMA-positive PBC.
  3. Clinical course mirrors classical PBC.
330
Q

What is another name for AMA-negative PBC?

A

Autoimmune cholangitis.

331
Q

Evidence for an autoimmune pathophysiology is weaker in PBC or in PSC?

A

PSC.

332
Q

What is the incidence of PSC?

A

6.3/100.000

333
Q

What percentage of patients with PSC will develop cholangiocarcinoma?

A

10-30%.

334
Q

What is useful to remember about PSC?

A

Although it targets 25-40 men, it can occur at ANY age –> Important cause of chronic liver disease in children.

335
Q

What are the generally accepted diagnostic criteria of PSC?

A
  1. Generalized beading and stenosis of the biliary system on cholangiography.
  2. Absence of choledocholithiasis (or history of bile duct surgery).
  3. Exclusion of bile duct cancer, by prolonged follow-up.
336
Q

For what is the term “secondary sclerosing cholangitis” used?

A

To describe the typical bile duct changes of PSC when a clear predisposing factor for duct fibrosis can be identified.

337
Q

Mention some causes of secondary sclerosing cholangitis.

A
  1. Previous bile duct surgery with stricturing and cholangitis.
  2. Bile duct stones causing cholangitis.
  3. Intrahepatic infusion of 5-fluorodeoxyuridine.
  4. Insertion of formalin into hepatic hydatid cysts.
  5. Insertion of alcohol into hepatic tumors.
  6. Parasitic infections (Clonorchis)
  7. Autoimmune pancreatitis/ IgG4-associated cholangitis
  8. Acquired immunodeficiency syndrome (result from CMV or Cryptosporidium infection).
338
Q

Mention some diseases associated with PSC?

A
  1. UC
  2. Crohn
  3. Chronic pancreatitis
  4. Retroperitoneal fibrosis
  5. Riedel’s thyroiditis
  6. Retro-orbital tumors
  7. Immune deficiency states
  8. Sjogren syndrome
  9. Angio-immunoplastic lymphadenopathy
  10. Histiocytosis X
  11. Autoimmune hemolytic anemia
  12. Autoimmune pancreatitis/IgG4-associated cholangitis
339
Q

What percentage of patients with PSC have ALSO UC?

A

About 2/3.

340
Q

What percentage of patients with PSC develop UC?

A

3-10%.

341
Q

What is the prevalence of PSC in patients with Crohn?

A

1%.

342
Q

PSC+UC results in higher risk for colorectal neoplasia?

A

Yes.

343
Q

What HLA connection has been found for PSC?

A

HLA A1, B8, DR3, DRW52.

344
Q

How is the diagnosis of PSC usually being made?

A

Incidentally - Persistent ALP in a patient with UC.

345
Q

What are the main clinical features of PSC?

A
  1. Fatigue
  2. Intermittent jaundice
  3. Weight loss
  4. RUQ pain
  5. Pruritus
  6. Hepatosplenomegaly
346
Q

What is the key investigation now for PSC?

A

MRCP - Usually diagnostic.

347
Q

How can PSC lead to “vanishing bile duct syndrome”?

A

Fibrosis spreads, progressing inevitably to biliary cirrhosis - obliterative cholangitis leads to the so-called “vanishing bile duct syndrome”.

348
Q

Is there any cure for PSC?

A

No.

349
Q

What do we use for PSC?

A

UDCA –> May have benefit in terms of reducing colon carcinoma risk.

350
Q

What is the 5-year survival in PSC patients who have undergone orthotopic transplantation?

A

80-90% –> Condition may recur.

351
Q

With what condition is IgG4-associated cholangitis closely associated?

A

With autoimmune PANCREATITIS.

352
Q

What is the presentation of IgG4-associated cholangitis?

A
  1. Obstructive jaundice
  2. Cholangiographic appearance of PSC
  3. With/without hilar cholangiocarcinoma
353
Q

Which Ig is raised in IgG4-associated cholangitis?

A

IgG4. Lymphoplasmacytic infiltrate in liver biopsy –> IgG4-positive plasma cells.

354
Q

What is an important difference between PSC and IgG4-associated cholangitis?

A

The latter responds to corticosteroids.

355
Q

What are the critical steps to be taken in diagnosing hepatic mass lesions?

A
  1. Determining the presence, nature and severity of any underlying chronic liver disease, as the differential diagnosis is very different in patients with or without chronic liver disease.
  2. Use of optimal (usually multiple) imaging modalities.
356
Q

What is the clinical presentation of HCC?

A
  1. Underlying cirrhosis
  2. Deterioration of their liver function –> Jaundice, ascites, variceal bleeding.
  3. Weight loss, anorexia, abdominal pain.
  4. Hepatomegaly or right hypochondrial mass.
  5. Vascularity in tumor.
357
Q

What percentage of HCCs produce AFP?

A

60%.

358
Q

Can we see an AFP elevation in the presence of active hep B and C viral replication?

A

Yes - Also very HIGH in paracetamol toxicity (acute hepatic necrosis).

359
Q

What is the normal value of AFP?

A

over 400ng/mL in AGGRESSIVE HCC.

360
Q

In which patients is histological confirmation advisable to exclude metastatic tumor?

A

In patients with large tumors, who do not have cirrhosis or hep B, in order to confirm the diagnosis of HCC.

361
Q

How often should high-risk patients be screened for HCC?

A

Every 6 months - AFP and US.

362
Q

What is the management of HCC?

A

Curative therapies include:

  1. Liver transplantation
  2. Hepatic resection
  3. Ablative therapy
    - -> Depends on the presence/absence of cirrhosis, underlying liver function, tumor size, and tumor multicentricity.
363
Q

What is the treatment of choice for non-cirrhotic patients with HCC?

A

Hepatic resection - 5year survival about 50%.

364
Q

What are the possible treatment options for HCC?

A
  1. Hepatic resection
  2. Liver transplantation
  3. Percutaneous therapy (ethanol, radiofrequency ablation)
  4. Trans-arterial chemo-embolization
  5. Chemotherapy (sorafenib)
365
Q

What is the action of sorafenib?

A

Multikinase inhibitor.

366
Q

What is the target group of fibrolamellar HCC?

A

Rare variant different from HCC in that it occurs in YOUNG ADULTS (M=F) in the absence of hep B infection and cirrhosis.

367
Q

What happens in fibrolamellar HCC?

A
  1. Often large at presentation and AFP is usually normal.

2. Histology –> Malignant hepatocytes surrounded by dense fibrous stroma.

368
Q

What is the treatment of choice for fibrolamellar HCC?

A

Surgical resection.

369
Q

What are the sites of origin of secondary malignant tumors of the liver?

A
  1. Lung
  2. Breast
  3. Abdomen
  4. Pelvis
    Single or multiple –> Peritoneal dissemination frequently results in ascites.
370
Q

In secondary malignant tumors of the liver, is the primary neoplasm symptomatic?

A

Asymptomatic in 50%.

Liver enlargement + weight loss - jaundice may be present.

371
Q

What is the most common biochemical abnormality in secondary malignant liver tumors?

A

A raised ALP, but LFTs may be normal.

372
Q

What are the MC benign liver tumors?

A

Hemangiomas - 1-20% of the population. –> Most smaller than 5cm –> Rarely cause symptoms.

373
Q

What is the target group of focal nodular hyperplasia?

A

Women under the age of 40.

374
Q

Cystic liver disease may be associated with what?

A

Polycystic renal disease.

375
Q

Mention some drugs that should be avoided in cirrhosis.

A
  1. NSAIDs
  2. ACE inhibitors
  3. Codeine
  4. Narcotics
  5. Anxiolytics
376
Q

Why are NSAIDs contraindicated in cirrhosis?

A
  1. Reduced renal blood flow

2. Mucosal ulceration

377
Q

What is the toxicity of NSAIDs in cirrhosis?

A
  1. Hepatorenal failure

2. Bleeding varices

378
Q

What is the problem and what is the toxicity of ACE inhibitors in cirrhosis?

A

Problem –> Reduced renal blood flow.

Toxicity –> Hepatorenal failure.

379
Q

What is the problem and the toxicity of codeine in cirrhosis?

A

Problem –> Constipation

Toxicity –> Hepatic encephalopathy

380
Q

What is the problem and what is the toxicity of narcotics in cirrhosis?

A

Problem –> Constipation, drug accumulation.

Toxicity –> Hep. encephalopathy.

381
Q

What is the problem and what is the toxicity of anxiolytics in cirrhosis?

A

Problem –> Drugs accumulation.

Toxicity –> Hep. encephalopathy.

382
Q

Which drugs may cause cholestasis?

A
  1. Chlorpromazine
  2. High-dose estrogens
  3. Flucloxacillin
383
Q

Which drugs may cause cholestatic hepatitis?

A
  1. NSAIDs
  2. Co-amoxiclav
  3. Statins
384
Q

Which drugs may cause acute hep?

A
  1. Rifampicin

2. Isoniazid

385
Q

Which drug may cause non-alcoholic steatohep?

A

Amiodarone

386
Q

Which drugs may cause venous outflow obstruction?

A
  1. Busulfan

2. Azathioprine

387
Q

Which drug may cause fibrosis of the liver?

A

Methotrexate

388
Q

What is the most common picture of drug-induced liver injury?

A

The picture of mixed cholestatic hep.

389
Q

What is the key to diagnosing acute drug-induced liver disease?

A

Take a detailed drug history.

390
Q

What are the steps for the diagnosis of acute drug-induced liver disease?

A
  1. Tabulate the drugs taken - Prescribed, self-administered.
  2. Establish whether hepatotoxicity is reported in the literature.
  3. Relate the time at which the drugs were taken to the onset of illness –> 4-8wks (usual).
  4. Establish the effect of stopping the drugs on normalization of liver biochemistry –> Hepatic LFTs (2months)/Cholestatic/mixed LFTs (6months).
  5. Exclude other causes - viral hep, biliary disease.
  6. Consider liver biopsy.
391
Q

What is the MC antibiotic to cause abnormal LFTs?

A

Co-amoxiclav –> It may not produce symptoms until 10-42d after it is stopped.

392
Q

What is the rule about drug-induced liver disease?

A

Most drugs cause reversible liver injury and hepatic fibrosis is very uncommon. (except methotrexate)

393
Q

Mention 3 causes of primary hemochromatosis.

A
  1. Hereditary hemochromatosis
  2. Congenital aceruloplasminemia
  3. Congenital atransferrinemia
394
Q

Mention some causes of secondary iron overload?

A
  1. Parenteral iron-loading (e.g. repeated blood transfusion)
  2. Iron-loading anemia (thalassemia, sideroblastic, pyruvate kinase def.)
  3. Liver disease
395
Q

Mention some causes of complex iron overload?

A
  1. Juvenile hemochromatosis
  2. Neonatal hemochromatosis
  3. Alcoholic liver disease
  4. Porphyria cutanea tarda
  5. African iron overload (Bantu siderosis)
396
Q

What is the normal iron content of the body?

A

4g - In hemochromatosis –> 20-60g.

397
Q

Where does iron deposition in the liver occur first?

A

In the periportal hepatocytes extending later to all hepatocytes –> MACROnodular cirrhosis.

398
Q

Where else can excess of liver iron occur?

A

In alcoholic cirrhosis, but this is mild by comparison with hemochromatosis.

399
Q

Which are the 2 main mutations in the HFE gene of hemochromatosis?

A

C282Y (Cysteine to tyrosine)

H63D (Histidine to aspartate) –> Less severe form.

400
Q

What is the clinical presentation of hemochromatosis?

A
  1. Men over 40 –> Features of liver disease, DM, or heart failure.
  2. Fatigue or arthropathy.
  3. Leaden-grey skin pigmentation due to excess melanin occurs.
  4. Arthritis with chondrocalcinosis secondary to Ca pyrophosphate deposition is common.
  5. Cardiac failure or cardiac dysrhythmia may occur due to iron deposition in the heart.
401
Q

What transferrin sat is suggestive of iron overload?

A

Of more than 45%.

402
Q

What is the upper limit for ferritin in hemochromatosis?

A

No significant liver disease with ferritin lower than 1000μg/L.

403
Q

What other condition may cause significant ferritin elevation?

A

Adult Still’s disease.

404
Q

Which index provides quantification of liver iron?

A

The Hepatic Iron Index (HII).

405
Q

What is the treatment for HHC?

A

Weekly venesection of 500mL blood (250mg iron) until the serum iron is normal –> This may take 2 years or more.

406
Q

What is the aim in the treatment of HCC?

A

To reduce ferritin to under 50μg/L.

407
Q

Will joint pain improve after treatment of HHC?

A

Unlike the liver and cardiac problems, it is unpredictable.

408
Q

Do we biopsy the liver in HHC?

A

It is only indicated in asymptomatic relatives if the LFTs are abnormal and/or the serum ferritin is greater than 1000μg/L because these features are associated with fibrosis or cirrhosis.

409
Q

What is the main cause of death in HHC?

A

HCC –> Screening is mandatory.

410
Q

What is another name for Wilson’s disease?

A

Hepatolenticular degeneration.

411
Q

What is the inheritance pattern of Wilson’s?

A

AR.

412
Q

What is the most important route of copper excretion?

A

Via the bile.

413
Q

What is the main problem in Wilson?

A

Almost always there is a failure of synthesis of ceruloplasmin.

414
Q

What are the organs that are primarily affected in Wilson?

A
  1. Eyes
  2. Liver
  3. Basal ganglia
  4. Kidneys
  5. Skeleton
415
Q

What does the ATP7B gene encode?

A

A member of the copper-transporting P-type adenosine triphosphate family, which functions to export copper from various cell types.

416
Q

How many ATP7B mutations have been described?

A

At least 200 –> Most cases are compound heterozygotes with 2 different mutations in ATP7B.

417
Q

When do the symptoms of Wilson usually arise?

A

Between 5-45yrs.

418
Q

When does hepatic disease occur in Wilson?

A

Predominantly in childhood and early adolescence, although it can present in adults in their fifties.

419
Q

When does the neurological damage tend to present in Wilson?

A

Basal ganglia syndromes and dementia –> LATE adolescence.

420
Q

Mention 2 rarely presenting features of Wilson?

A
  1. Osteoporosis

2. Renal tubular damage

421
Q

What is a big problem in Wilson’s liver disease?

A

Episodes of acute hep, sometimes recurrent –> Fulminant hepatic failure in children –> Liberation of free copper into blood –> Massive hemolysis + renal tubulopathy.

422
Q

When should we seriously consider the possibility of Wilson in a patient?

A

In any patient under 40 presenting with recurrent acute hep or chronic liver disease of unknown cause –> especially when accompanied by HEMOLYSIS.

423
Q

What may be an early symptom of Wilson disease?

A

Unusual clumsiness for age.

424
Q

What percentage of adults with Wilson have Kayser-Fleischer rings?

A

60% (less often in children but almost always in neurological Wilson’s disease).

425
Q

What is the best single lab clue to Wilson’s diagnosis?

A

Low serum ceruloplasmin.

426
Q

What can reduce serum ceruloplasmin besides Wilson?

A

Advanced liver failure from ANY cause. Occasionally, the ceruloplasmin is normal in Wilson.

427
Q

What is the drug of choice for Wilson?

A

D-penicillamine –> 1.5g/day –> sufficient dose to produce cupriuresis.
Must continue for life, even through pregnancy.

428
Q

Can we abruptly stop penicillamine?

A

NO –> Risk of acute liver failure.

429
Q

What are the side effects of penicillamine?

A
  1. Rashes
  2. Protein-losing nephropathy
  3. Lupus-like syndrome
  4. Bone marrow depression
430
Q

What is α1-AT?

A

A serine protease inhibitor (Pi) produced by the liver.

431
Q

What is the main problem with the mutated form of α1-ΑΤ (PiZ)?

A

It cannot be secreted into the blood by liver cells because it is retained within the endoplasmic reticulum of the hepatocyte.

432
Q

What are the main liver manifestations of AAT def.?

A
  1. Cholestatic jaundice in the neonatal period (neonatal hep).
  2. Chronic hep.
  3. Cirrhosis
  4. HCC
433
Q

Can AAT def be an exacerbating factor for liver disease of other etiologies?

A

Yes - The possibility of DUAL pathology should be considered in patients in whom severity of disease, such as ALD, appears disproportionate to the level of underlying insult.

434
Q

Is there a specific treatment for AAT def.?

A

No treatment - the risk of severe and early-onset emphysema means that ALL patients should stop smoking.

435
Q

What is the 1st step during the examination of the abdomen for liver and biliary disease?

A

OBSERVATION:

  1. Unkempt
  2. Smell of alcohol or fetor hepaticus
  3. Encephalopathy
  4. Weight loss
  5. Scratch marks from itching

Internal Medicine (106 decks)

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