Clinical DSA 5: Hepatobiliary - Chronic Liver Disease and Cirrhosis Flashcards

1
Q

Primary complication of chronic hepatitis

A

Cirrhosis => ascites, variceal bleeding, encephalopathy, coagulopathy, hypersplenism (thrombocytopenia)

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2
Q

Complications of chronic HBV infection

A

Cirrhosis
HCC
Polyarteritis nodosa

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3
Q

Who is at the highest risk of developing chronic HBV?

A

Babies who are born to HBsAG+ mothers have 90% risk of chronic HBV

*perinatal transmission

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4
Q

Complications of chronic HCV infection

A

Cirrhosis
HCC
Mixed cryoglobulinemia

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5
Q

HCV prevention

A

If HCV+ do not share razors or toothbrush and practice safe sex

*no HCV vaccine

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6
Q

Diagnosis?

Chronic, progressive liver disease
*Appears healthy with stigmata of cirrhosis
Association with other autoimmune disease
*ASMA+ or ANA+
Hypergammaglobulinemia
AST/ALT > 1000, hyperbili
*Female 30s-50s
Extrahepatic manifestations (rash, arthralgias, keratoconjuctivitis, thyroiditism hemolytic anemia, nephritis, UC)

A

AIH Type 1

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7
Q

Diagnosis?

Chronic, progressive liver disease
*Appears healthy with stigmata of cirrhosis
Association with other autoimmune disease
*Anti-liver/kidney microsomal Abs (anti-LKM)
AST/ALT > 1000, hyperbili
*Children
Extrahepatic manifestations (rash, arthralgias, keratoconjuctivitis, thyroiditism hemolytic anemia, nephritis, UC)

A

AIH Type 2

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8
Q

Treatment for AIH

A

Glucocorticoids
Azathioprine
Monitor LFTs
Possible liver transplant

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9
Q

Continuum of alcohol induced liver disease

A

Steaosis => Steatohepatitis => Cirrhosis

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10
Q

Diagnosis?

Asymptomatic hepatomegaly and mild elevations in ALP and bili
AST>ALT 2:1
CBC shows leukocytosis or leukopenia
Macrocytic anemia
Signs of liver failure (dec albumin, coagulopathy)
History of alcohol abuse

A

Fatty liver (steatosis) phase of alcohol induced liver disease

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11
Q

Diagnosis?

Asymptomatic progresses to signs of liver failure
AST>ALT 2:1
CBC shows leukocytosis or leukopenia
Macrocytic anemia
Signs of liver failure (dec albumin, coagulopathy)
History of alcohol abuse

A

Alcoholic hepatitis (steatohepatitis) phase of alcohol induced liver disease

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12
Q

Diagnostic imaging for alcohol induced liver disease

A

US to rule out gallstones
CT with IV contrast or MRI
US elastography (look for fibrosis)

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13
Q

Liver biopsy finding in alcoholic hepatits and non-alcoholic steatohepatitis (NASH)

A

Mallory-Denk bodies (alcoholic hyaline)

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14
Q

Alcohol induced liver disease treatment and management

A
Abstinance from alcohol
Daily multivitimin
Thiamine 100 mg
Folic acid 1 mg
Zinc
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15
Q

Special consideration of hypoclycemia management in alcohol induced liver disease

A

Administer glucose and thiamine at same time

otherwise may precipitate Wernicke-Korsakoff syndrome

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16
Q

Maddrey’s discriminant function (DF) is a prognostic indicator for _______________

calculated using __ and _______

Values >___ associated with poor prognosis and may benefit from steroids

A

Maddrey’s discriminant function (DF) is a prognostic indicator for alcohol induced liver disease

calculated using PT* and bilirubin*

Values >32* associated with poor prognosis

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17
Q

Glasgow Alcoholic Hepatitis score is a prognostic indicator for _______________

calculated using age, bili, BUN, PT, WBC count

Values >___ associated with poor prognosis and may benefit from glucocorticoids

A

Glasgow Alcoholic Hepatitis score is a prognostic indicator for alcohol induced liver disease

calculated using age, bili, BUN, PT, WBC count

Values >9* associated with poor prognosis

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18
Q

Model for End Stage Liver Disease (MELD/MELD-Na) score >___ is associated with significant mortality in alcoholic hepatitis

A

Model for End Stage Liver Disease (MELD/MELD-Na) score >21 is associated with significant mortality in alcoholic hepatitis

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19
Q

Treatment for severe alcoholic hepatitis (DF ≥ 32 or MELD >21 or GAW ≥ 9)

A

Steroids and pentoxifylline

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20
Q

Liver transplant requirement in alcohol induced liver disease

A

must abstain from alcohol for 6 months to be considered

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21
Q

Diagnosis?

Patient with alcoholic liver disease
Mental status changes
Ataxia
Involuntary eye movements

A

Wernicke encephalopathy

Tx with thiamine

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22
Q

Diagnosis?

Patient with alcoholic liver disease
Severe memory issues
Confabulations/make-up stories

A

Korsakoff syndrome (permanent)

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23
Q

Diagnosis?

Asymptomatic or mild RUQ discomfort
Hepatomegaly
Metabolic syndrome***
Insignificant alcohol history

A

NAFLD

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24
Q

Prevention of NAFLD

A

Regular physical activity and coffee consumption

25
Q

Treatment for NAFLD

A

Lifestyle modification (diet, exercise, vit E, gastric bypass)

Liver transplant in advanced cirrhosis

26
Q

Diagnosis of NAFLD

A

Liver biopsy is diagnostic

Predominantly shows macrovesicular steatosis, no inflammation or fibrosis, Mallory-Denk bodies

*NASH and alcoholic steatohepatitis indistinguishable histologically

27
Q

Diagnosis?

Liver disease and panacinar emphysema
Most commonly presents in children

A

alpha 1 antitrypsin deficiency

28
Q

Alpha 1 antitrypsin deficiency pathogenesis

A

Defective a1-AT gene => misfolded protein

  • Misfolded protein accumulates in liver leading to liver damage
  • Decreased functional a1-AT leads to disinhibition of proteases leading to pulmonary emphysema

a1-AT is a protein that inactivates proteases

29
Q

Adult smoker presentation of a1-AT deficiency

A

Liver disease
COPD/emphysema of upper lobes

*tx is smoking cessation

30
Q

Child presentation of a1-AT deficiency

A

Liver disease
Panacinar emphysema of lower lobes

*tx is liver transplant in severe cases (most common cause of child liver transplant)

31
Q

a1-AT phenotypes

A

PiZ or PiZZ have severe deficiencies

PiS have mild deficiency

Pim is phenotypically normal

Pi gene on c14

32
Q

Complications of a1-AT deficiency

A

Emphysema at young age

Micronodular cirrhosis => HCC risk

33
Q

Diagnosis?

Cirrhosis with hepatomegaly
Bronze skin
Diabetes mellitus
Cardiac dysfunction
>45% transferrin saturation

HFE gene mutation
Increased iron absorption in duodenum
Hemosiderin (iron) deposits in organs

A

Hemochromatosis

34
Q

Progression of hemochromatosis

A

Early: nonspecific symptoms (fatigue, arthralgia)

Later: Cirrhosis with hepatomegaly, Bronze skin, Diabetes mellitus, Cardiac dysfunction, arthropathy

35
Q

Hemochromatosis treatments

A

Phlebotomy

In hemochromatosis due to transfusions use Deferoxamine (chelating agent)

36
Q

Hemochromotosis complications

A

Cirrhosis, diabetes mellitus, heart failure

Infection risk for siderophilic organisms (Y. enterocolitica, V. vulnificus, L. monocytogenes)

37
Q

Hemochromotosis screening

A

Test for HFE mutation in any patient with iron overload

Iron studies and HFE testing in all first degree family members

38
Q

Diagnosis?

Child or young adult (<40) presents with neurologic and psychiatric problems
Kayser-Fleischer rings
Low serum ceruloplasmin
Coombs - hemolytic anemia
Copper accumulation in liver, brain, eyes
ATP7B mutation

A

Wilson disease

AR disorder, impaired copper excretion into bile and failure to incorporate copper into ceruloplasmin

39
Q

Wilson disease treatment

A

Oral penicillamine (increases urinary excretion of chelated copper)

Liver transplant in severe case (cirrhosis)

40
Q

Diagnosis?

Elevated AST/ALT
Elevated BNP
Hepatojugular reflux is present
Nutmeg liver

A

Right heart failure

41
Q

Diagnosis?

Rapid and striking elevation in ALT/AST over 5000
Rapid rise in LDH

A

Ischemic hepatitis (shock liver)

*caused by acute MI, arrhythmia, septic/hemorrhagic shock

42
Q

Initial imaging study for cirrhosis

A

RUQ US + Doppler of hepatic/portal veins

43
Q

Complications of cirrhosis

A

Risk of HCC

Portal HTN

44
Q

Survival prognostic scores for cirrhosis

A

Child-Turcotte-Pugh score (A,B,C with C being most severe)

MELD/MELD-Na score (>10 is worsening, >14 for transplant list)

45
Q

Tests to order for MELD/MELD-Na score

A
Bili
Creatinine
Na
INR
Dialysis recently?
46
Q

Tests to order for Child-Turcotte-Pugh score

A

Bili
Albumin
PT/INR
PE for encephalopathy and ascites

47
Q

Screening for HCC in cirrhosis pts

A

alpha fetoprotein and US every 6 months

48
Q

Diagnosis?

Abdominal distension in chronic liver disease, portal HTN
Abdominal US shows fluid in peritoneum
Shifting dullness on percussion (requires 1500 mL of fluid)

A

Ascites

49
Q

SAAG score

A

serum albumin - ascites fluid albumin

> 1.1 indicates ascites due to portal HTN

50
Q

Who gets paracentesis?

A

Pt with new onset ascites

Pt admitted to hospital with known cirrhosis and ascites

Pt with known cirrhosis and ascites deteriorates quickly

51
Q

How to diagnose spontaneous bacterial peritonitis

A

Paracentesis of ascites fluid
WBC with diff of fluid
PMN (total neutrophils) >250 = neutrocytic ascites
highly sus

52
Q

Treatment and prevention of spontaneous bacterial peritonitis (SBP)

A

Abx (prophylactic or treatment)

53
Q

Prevention of esophageal varicieal bleeding/rebleeding

A

beta blockers

band ligation (30% chance of rebleed)

54
Q

Treatment for bleeding esophageal varicies

A

Resuscitation in ICU
Emergent EGD with banding
IVF +/- blood products
Correct coagulopathy

Abx, octreotide, TIPS, liver transplant

55
Q

Grades of hepatic encephalopathy

A
  1. mild confusion
  2. drosiness
  3. stupor
  4. coma
56
Q

Diagnosis?

Alteration of mental status in the presence of liver failure
Elevated ammonia (no correlation of amount to severity of mental status change)
GI bleeding
Constipation

A

Hepatic encephalopathy

57
Q

Treatment of hepatic encephalopathy

A

Lactulose (produce 2-3 soft stools per day)

acidifies colon and traps ammonium for excretion

58
Q

Diagnosis?

Cirrhosis
Cachexia, abdominal pain, fever, jaundice
Abnormal LFTs
Abnormal US or rising alpha fetoprotein

A

HCC