Hepatology Flashcards

0
Q

What causes ascites?

A

Low albumin and increased hepatic portal pressure.

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1
Q

Other than decompensated liver disease, what other causes of encephalopathy are there?

A

Infection, dietary protein indiscretion, GI bleeding, drugs and electrolyte disturbance.

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2
Q

What are the surface anatomy markings for the liver?

A

Upper margin of the liver can be traced by a line drawn along the left and right 6th intercostal space with the anterior axillary lines on both sides providing the lateral extent. The right lobe is tucked under the dome of the diaphragm and at its maximum span can be measured if you drop a vertical line from the junction of the right upper liver margin and the anterior axillary line down to the tip of the 10th rib.

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3
Q

Describe the inheritance and notable features of Peutz-Jeghers Syndrome.

A

Rare autosomal dominant disorder characterised by hamartomatous polyps affecting the intestine and hyper pigmented freckles, or macules, visible on the lips and the buccal mucosa.

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4
Q

Name the skin signs looked for in the abdominal examination.

A

Xanthelasma, Peutz-Jeghers Syndrome, spider naevi, anaemia, jaundice and haemachromatosis.

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5
Q

What causes spider naevi to form?

A

It is thought that impaired oestrogen detoxification plays a role in their development and additionally they are only present in areas drained by the superior vena cava. As such, they are found in the face, neck, chest and upper arms, but not below the level of the atria.

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6
Q

What is xanthelasma?

A

These are sharply demarcated yellowish papules caused by subcutaneous accumulation of cholesterol laden macrophages that develop on or around the eyelids. They are usually symmetrical and are commonly found on the upper eyelid near the medial canthus.

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7
Q

What is kolynychia?

A

Concave spooning of the nails.

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8
Q

What does kolynychia indicate?

A

Long standing iron deficiency.

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9
Q

Define leukonychia and what it indicates.

A

The nail loses transparency and assumes the same white colour shading as the lunule. This is associated with chronic low albumin. In chronic decompensated liver disease where albumin is almost always subnormal is often associated with this sign. Other causes of low albumin include nephrotic syndrome might also reveal leukonychia.

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10
Q

What is clubbing?

A

It is loss of the bail bed angle, known as Lovibond’s angle. This can also be assessed visually by Schamroth’s test. The loss of Lovibond’s angle is due to changes in the periungal connective tissue. First there is local interstitial oedema, and if the condition progresses there is abnormal accumulation of vascular connective tissue added to by local vasodilation. The nail bed is infiltrated by oedema and hypertrophic vascular tissue lifts the proximal nail bed to obliterate Lovibond’s angle and gives the proximal ball a spongy sensation when palpated.

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11
Q

What is dupuytren’s contracture?

A

A thickening and puckering of the fascia underlying the skin covering the palm of the hand. This can progress to cause contractures of the fingers starting with the little finger and progressing sometimes to the middle finger. Noted to be more common in alcohol dependency but the association with liver disease is really indirect. Also occurs in non-alcoholics and therefore is not a sensitive marker of alcohol misuse.

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12
Q

What does palmar erythema indicate and what is the physiology behind it?

A

Particularly noticeable in the thenar and hypothenar eminences, it is associated with vasodilation, and thus with spider naevi. Vasodilatory products are associated with derangement of liver function and, thus, these signs may be seen in a patient with decompensated liver disease.

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13
Q

What is asterixis and why does it occur?

A

Asterixis is the liver flap and is a sign of hepatic encephalopathy caused by decompensated liver disease and portal hypertension.

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14
Q

What is the Kayser-Fleischer Ring sign?

A

It is a pigmented ring at the outer edge of the cornea where it abuts on the sclera. It is due to copper deposition and occurs in Wilson’s disease which is an inherited metabolic disorder characterised by liver damage and cirrhosis progressing to neurological disease with Parkinsonian features. This disease is caused by excess of copper deposition in the liver and the basal ganglia of the brain.

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15
Q

What is Budd-Chiari syndrome?

A

Condition caused by occlusion of the hepatic veins that drain the liver. It presents with the classical triad of abdo pain, ascites and hepatomegaly. 75% of cases are primary (idiopathic).

16
Q

What factors are considered in the Child-Pugh classification of liver cirrhosis?

A
  • Bilirubin
  • Albumin
  • Prothrombin Time
  • Encephalopathy
  • Ascites
17
Q

How would you manage Wilson’s Disease?

A
  • Penicillamine (chelates copper)
  • Trientine hydrochloride is an alternative chelating agent that may become first line in the future
  • Tetrathiomolybdate is a newer agent that hasn’t been approved yet.
18
Q

What is the gene defect responsible for Wilson’s Disease?

A

ATP7B gene located on chromosome 13

19
Q

What are the signs of Wilson’s Disease?

A
  • Liver: hepatitis, cirrhosis
  • Neurological: basal ganglia degeneration, speech and behavioral problems are often the first manifestations. Also asterixis, chorea and dementia
  • Kayser-Fleischer rings
  • Renal tubular acidosis (esp. Fanconi Syndrome)
  • Haemolysis
  • Blue nails
20
Q

How do you diagnose Wilson’s Disease?

A
  • Reduced serum caeruloplasmin
  • increased 24 hour urinary copper excretion
  • History and examination
21
Q

When does Wilson’s Disease usually present?

A

Between the ages of 10 and 25