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Liver > Metabolic Disease > Flashcards

Metabolic Disease Flashcards

1
Q

What is non-alcoholic steatohepatitis?

A

High liver fat content can lead to inflammation and fibrosis

Rule out vother causes of liver disease

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2
Q

What are risk factors for NASH?

A

Female
Obesity
Older age
DM

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3
Q

What are clinical features of NASH?

A

Asymptomatic

May be hepatomegaly

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4
Q

What Ix in NASH?

A 
LFT all raised
Lipids
HbA1c
USS
Liver biopsy
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5
Q

What Mx in NASH?

A

Control risk factors
Assess CVS risk
Avoid alcohol
Monitor for cirrhosis DM HCC

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6
Q

Wilson’s disease P/A - inheritance pattern?

A

Autosomal recessive copper transport disorder

Abnormal hepatobiliary copper excretion

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7
Q

What are clinical features of Wilson’s syndrome?

A 
Hepatits
Cirrhosis
Fulminant liver failure
Tremor
Dysarthria
Dysphagia
Dystonia
Dementia
Parkinsonism
Kayser-fleischer rings in iris
Haemolysis
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8
Q

What are Ix for Wilson’s disease

A 
Copper studies
Urine copper 
Raised LFT
Serum copper
Liver biopsy - 
MRI - basal ganglia degeneration
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9
Q

Mx for Wilson’s disease

A

Avoid food with high copper - liver, chocolate, nuts
Check water sources

Lifelong penicillinamine
Monitor FBC, urinary copper and protein excretion

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10
Q

P and inheritance pattern of hereditary haematochromatosis?

A

Autosomal recessive - increased absorption of iron leading to iron deposition in the joints, liver, heart, pancreas, pituitary, adrenals and skin

Middle aged men

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11
Q

Clinical features of hereditary haematochromatosis

A 
Tiredness, arthralgia
Slate-grey skin pigmentation
Signs of chronic liver disease
Hepatomegaly
Cirrhosis
Dialted cardiomyopathy

DM
Hypogonadism
Pituitary dysfunction

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12
Q

Ix in hereditary haematochromatosis

A

LFTs raised
Ferritin raised
Raised transferrin sarutation

Liver and cardiac MRI

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13
Q

Mx in hereditary haematochromatosis

A

Venesection - needed for life
Desferroxamine
Monitor LFT, glucose, iron

Well balanced diet
Alcohol abstinence
Liver transplant
Screen for HCC

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14
Q

What is A1AT deficiency

A

Inherited disorder affecting lung (emphysema) and liver (cirrhosis and HCC)

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15
Q

Clinical features of A1AT deficiency?

A

Dyspnoea form emphysema
Cirrhosis
Cholestatic jaundice

Liver disease in children
Lung disease in adults

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16
Q

What Ix in A1AT deficiency?

A

Serum A1AT levels low
Lung function testing - FEV1 - obstructive pattern

Liver biosy - PAS+ve
Phenutyping

17
Q

Mx of A1AT deficiency?

A

Smoking cessation
Vaccination to prevent lung infections
A1AT IV from human plasma

Lung transplant

Liver (8 decks)

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