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Gastroenterology > Metabolic liver disease > Flashcards

Metabolic liver disease Flashcards

1
Q

What does MASH stand for and what does it mean?

A

Metabolic dysfunction-associated steatohepatitis (MASH) is the advanced stage of MASLD

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2
Q

Signs of MASH on liver biopsy

A

lobular inflammation
hepatocyte ballooning
progressive liver scarring (fibrosis)

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3
Q

NAFLD pathology definition

A

> 5% hepatocytes expressing fat within them

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4
Q

Causes of liver steatosis

A

NAFLD
Hepatitis C
Alcohol excess
Genetic disorders
Medications
Hypothyroidism
Other rarer causes

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5
Q

Pathophysiology of NAFLD

A

eat too many calories
increase in visceral fat
adipocytes resistant to insulin
fat into portal circulation
fat taken up by liver cells

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6
Q

What is the new name for NAFLD?

A

MASLD
metabolic dysfunction associated steatotic liver disease

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7
Q

What is MetALD?

A

MASLD and alcohol excess

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7
Q

What are patients with NASH/MASH at risk of?

A

hepatocellular carcinoma

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8
Q

Name some liver decompensation events

A

ascites
variceal bleed
encephalopathy
hepatocellular carcinoma
synthetic failure

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9
Q

What score is used to approximate how scarred the liver is?

A

FIB-4
high number = lots of scarring

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10
Q

What are Iron (II) compounds called?

A

ferrous

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11
Q

What are Iron (III) compounds called?

A

ferric

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12
Q

What is organic iron called?

A

haem

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13
Q

How does iron in the body exist?

A

incorporated into Hb
Myoglobin, enzymes, Fe/S clusters
haem-containing cytochromes
stored iron in form of ferritin/haemosiderin
plasma transferrin-bound iron

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14
Q

How does body regulate iron levels?

A

by regulating absorption

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15
Q

Where does most iron absorption occur?

A

duodenum

16
Q

What is transferrin saturation?

A

how saturated transferrin is with iron

17
Q

Iron overload blood results

A

high ferritin
high transferrin saturation (>45%)

18
Q

Iron deficiency blood results

A

low ferritin
low transferrin saturation (<16%)

19
Q

What are the 2 forms iron exists in?

A

inorganic/non-haem = Fe3+ = less soluble

organic/haem = Fe2+ = more soluble

to be soluble, ferric needs to be reduced to ferrous

20
Q

What enzyme reduces ferric to ferrous?

A

duodenal cytochrome b ferrireductase

enzyme is vitamin C dependent

21
Q

What does ferroportin do?

A

allows iron out of duodenum

22
Q

What are transferrins?

A

iron-binding blood plasma glycoproteins that control the level of free iron in biological fluids

23
Q

What can raised ferritin mean?

A

inflammation
if no infection/inflammation = iron overload

24
Q

Clinical sequelae of iron overload

A

pituitary - impaired growth, infertility
thyroid - hypoparathyroidism
heart - dilated cardiomyopathy, cardiac failure
liver - hepatic cirrhosis
pancreas - diabetes mellitus
gonads - hypogonadism

25
Q

How is haemochromatosis inherited?

A

autosomal recessive

26
Q

Describe the inheritance patterns of haemochromatosis

A

Cys282Tyr homozygous = haemochromatosis

Cys282Tyr + H63D compound heterozygous = haemochromatosis

H63D homozygote = does not cause haemochromatosis

27
Q

What is the natural progression of hereditary haemochromatosis?

A

phase of latency

biochemical expression (~20y):
- increased serum iron, transferrin saturation + serum ferritin

Clinical expression (~40-50y):
- fatigue, arthritis, hepatomegaly, skin pigmentation, diabetes
- evolution towards cirrhosis and carcinoma

women generally present later as iron is lost in menstruation

28
Q

At what age is screening for haemochromatosis offered to potentially at risk individuals (FH)?

A

at 18

29
Q

Wilson’s disease inheritance

A

autosomal recessive
not homozygous - normally 2 different mutations

30
Q

How is copper excreted?

A

in bile

31
Q

Wilson’s disease treatment

A

copper-chelating drug:
- penicillamine
- trientine

binds to copper so you pee it out

occasionally transplant needed –> mutation is in liver so transplant is curative

32
Q

Signs of Wilson’s disease

A

Kayser-Fleischer rings
Parkinsonism
Cirrhosis

due to copper building up in areas of body

33
Q

Blood results in Wilson’s

A

low caeruloplasmin
total copper is low (free copper is high but caeruloplasmin with copper bound to it is very low so this makes total copper low)

34
Q

What is present in urine in Wilson’s disease?

A

copper high in urine
measure over 24 hours

35
Q

How is Wilson’s disease diagnosed?

A

DNA sequencing looking for 2 pathogenic mutations
liver biopsy + measure copper –> >250 micrograms/g = Wilson’s

36
Q

Pathogenesis of Wilson’s disease

A

mutations in ATP7B gene on chromosome 13
this gene makes an enzyme that is involved in copper transport
when the enzyme is mutated, copper accumulated in liver and brain and becomes toxic

37
Q

Alpha-1-Antitrypsin deficiency inheritance

A

autosomal codominant

38
Q

When pink globules are seen on liver biopsy, what does this indicate?

A

alpha-1-antitrypsin deficiency

Gastroenterology (43 decks)

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