Chronic Liver Disease Flashcards

1
Q

Define Cirrhosis (in pathological terms)

A

An irreversible consequence of chronic hepatic injury w/ 3 key characteristics

  • Destruction of liver cells
  • Chronic inflammation –> fibrosis
  • Hepatocyte regeneration –> nodules
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2
Q

What is the underlying pathology of fibrosis?

A

Due to growth factors released from inflammatory cells/Kupffer cells/hepatocytes
Inflammatory cells due to disease/response to liver cell necrosis

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3
Q

What is the underlying pathology of nodule formation?

A

Due to hepatocyte regeneration
Stellate cell activation –> myofibroblasts formed, secrete collagen –> nodules form
Nodules lack vascular/bile drainage connections

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4
Q

What are the common causes of cirrhosis?

A

Alcoholic liver disease
Non-alcoholic fatty liver disease
Chronic viral hepatitis- Hep B or C

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5
Q

What are the less common causes of cirrhosis?

A
Auto Immune hepatitis
Primary biliary cirrhosis
Primary sclerosing cholangitis
Budd-Chiari syndrome
Cystic fibrosis
Drugs- methotrexate or phenytoin
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6
Q

What are the very rare causes of cirrhosis?

A

Haemochromatosis
Wilson’s
a1-antitrypsin deficiency

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7
Q

What is primary biliary cirrhosis?

A

Auto Immune destruction of intrahepatic bile cannaliculi

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8
Q

What are the risk factors for primary biliary cirrhosis?

A

40-60yr old females (90%)

IBD

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9
Q

How does primary biliary cirrhosis present?

A

Jaundice
Pruritis
Skin xanthomas

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10
Q

How is primary biliary cirrhosis diagnosed?

A

Raised anti-mitochondrial antibodies (AMA)

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11
Q

What are the management options for primary biliary cirrhosis?

A

Replacement of fat soluble vitamins

-progressive condition, 6yr average survival

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12
Q

What is primary sclerosing cholangitis?

A

Autoimmune inflammation & fibrosis around the bile ducts in the liver
-2o cirrhosis due to chronic biliary obstruction

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13
Q

What conditions are associated w/ primary sclerosing cholangitis?

A

IBD (70%)

Cholangiocarcinoma (develops in 10-15%)

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14
Q

What are the management options for primary sclerosing cholangitis?

A

No effective treatment except transplantation

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15
Q

What are the classic sx of chronic liver disease?

A
Fatigue
Wt loss/anorexia
Jaundice
Leg swelling
Bleeding/bruising
Itching
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16
Q

What are the extrahepatic signs of chronic liver disease?

A

Nails - leuconychia, clubbing
Hands - palmar erythema, duyputren’s contracture, liver flap
Skin - pigmentation, spider naevi, striae
Feminization - gynaecomastia, testicular atrophy, loss of body hair
Portal HTN - caput medusae, hepatosplenomegaly, ascites
Hepatocellular failure - bruising, prolonged clotting

17
Q

What are the signs of decompensated liver disease?

A

Encephalopathy
Ascites
Jaundice

18
Q

What factors may precipitate chronic liver disease decompensating?

A

Alcohol binge
Variceal bleed
Hepatotoxic drugs
Portal/hepatic vein thrombosis

19
Q

What is the Child-Pugh score?

A

Gives 1/2 yr mortality scores in chronic liver disease using

  • total bilirubin
  • serum albumin
  • PT/INR
  • ascites
  • hepatic encephalopathy
20
Q

What are the complications of chronic liver disease?

A

Hepatocellular failure
Portal HTN
Malignant change
Renal failure (hepatorenal syndrome)

21
Q

What are the blood investigation of Cirrhosis?

A

Bloods

  • FBC- thrombocytopenia
  • INR- raised
  • Clotting- PT raised
  • LFTs normal unless decompensated
  • U&Es- can have hyponatraemia and in hepatorenal syndrome will be derrangement
  • AFP- hepatocellular carcinoma
  • Albumin- low

Enhanced liver fibrosis

  • <7.7. none to mild
  • 7.7-9.8= moderate
  • > 9.8= severe

Can do viral serology, autoimmune screening, copper levels, Fe studies and a1 antitrypsin levels too

22
Q

What other testing (imaging) can be done for cirrhosis?

A

USS liver

  • nodules on liver surface
  • winding of arteries
  • cancer

CT/MRI- look for ascites, hepatosplenomegaly

Fibroscan
-look at extent of fibrosis and liver elasticity

Endoscopy- for oesophageal varices

Liver biopsy- to confirm diagnosis

Ascitic tap if ascites

23
Q

What is Wilson’s disease?

A
Inborn error of copper metabolism
Deposition of copper in
   -liver
   -basal ganglia
   -cornea (Kayser-Fleischer rings)
24
Q

What are the appropriate investigations in suspected Wilson’s disease?

A
Serum caeruloplasmin (reduced)
Urinary copper (increased)
Liver biopsy (increased copper)
25
Q

What are the management options for Wilson’s disease?

A

Chelating agents (trientene, D-penicillamine)

26
Q

What is Haemachromatosis?

A

Inherited condition of excess iron deposition leading to fibrosis & organ failure

27
Q

What is the classic triad of sx in Haemachromatosis?

A

Bronze skin discolouration
Hepatomegaly
DM
(also hypogonadism - impotence, testicular atrophy)

28
Q

What are the appropriate investigations in suspected Haemachromatosis?

A

Serum Fe/Ferritin (increased)
Total iron binding capacity (decreased)
Genetic testing
Liver biopsy

29
Q

What are the management options for Haemachromatosis?

A

Vensection

-1 unit/wk

30
Q

What are the common complications of cirrhosis?

A
Portal HTN (varices)
Ascites
Encephalopathy
Malnutrition
Hepatorenal Syndrome
Hepatocellular carcinoma
31
Q

How does portal HTN related to cirrhosis present?

A

Often asymptomatic/just hepatosplenomegaly
90% develop gastro-oesophageal varices over 10 yrs
-1/3 bleed

32
Q

How does ascites related to cirrhosis present?

A

Mild abdo pain common

-if severe suspect spontaneous bacterial peritonitis

33
Q

What are the management options for ascites?

A

Initial bed rest & fluid restriction/low Na diet
Spironolactone
Furosemide (if poor response)
Therapeutic paracentesis/albumin infusion (large volume)

34
Q

What are the signs of spontaneous bacterial peritonitis?

A

Fever
Pain
Deterioration in prev well pt

35
Q

How should spontaneous bacterial peritonitis be managed?

A

IV ceftriaxone

Prophylactic ciprofloxacin to start post infection to prevent recurrence
-also start if hepatorenal syndrome, Child Pugh score >9, fluid protein <15

36
Q

What causes encephalopathy?

A

Accumulation of nitrogenous waste in circulation

Leads to cerebral oedema

37
Q

What are the grades of encephalopathy?

A

Grade 1 - altered mood/behaviour, sleep disturbances
Grade 2 - increasing drowsiness/confusion
Grade 3 - stupor, incoherence, restlessness
Grade 4 - coma

38
Q

What are the management options of encephalopathy?

A

ICU admission
20o head tilt
Oral lactulose w/ regular enemas with rifamixin
IV manitol/hyperventilation if cerebral oedema

39
Q

What is hepatorenal syndrome?

A

Where pooling of blood due to portal HTN causes decreased blood flow to kidneys

Leads to RAAS activation and vasoconstriction of kidney arteries causing them to starve and leads to rapid deterioration in renal function

Fatal within 1 wk unless renal transplant