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MRCPSYCH_Paper B_MENTOR & More_Dr. ART > Misc. - 5 > Flashcards

Misc. - 5 Flashcards

1
Q

what is another name for wilson disease?

A

hepatolenticular degeneration

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2
Q

what is wilson’s disease?

A

abnormal storage of copper

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3
Q

what there is abnormal storage of copper in wilson disease?

A

fail to excrete

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4
Q

where do the copper is stored excessively?

A

Brain and Liver & cornea

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5
Q

what is degenerated in brain in wilson’s disease?

A

lenticular nucleus (putamen and globus pallidus)

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6
Q

genetic of wilson’s disease

A

autosomal recessive

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7
Q

pathology of wilson’s disease

A

pathological extra storage of copper
excessive absorption of copper

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8
Q

which gene defect is responsible for wilson?

A

ATP7B, chromosome 13

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9
Q

what does ATP7B do?

A

transport & incorporates copper with ceruloplasmin

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10
Q

onset of wilson’s

A

10-25

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11
Q

presentation of wilson in children?

A

liver disease

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12
Q

presentation of Wilson in adult?

A

neurological

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13
Q

Features of Wilson’s disease: Liver

A

hepatitis
cirrhosis

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14
Q

Features of Wilson’s disease: Brain

A

Basal ganglia degeneration
Speech and behavioural problems
Asterixis (flapping tremor)
Chorea
Dementia

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15
Q

Features of Wilson’s disease: cornea

A

Kayser-Fleischer ring (brown ring on the edge of cornea)

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16
Q

Features of Wilson’s disease: systematic

A

-Renal Tubular acidosis (Fanconi syndrome)
-Haemolysis
-Blue nails

17
Q

Dx of Wilson’s disease

A

Reduced serum caeruloplasmin
Reduced serum copper
Increased 24 hours copper in urine

18
Q

% of patients who will show reduced caeroluprlamin and copper serum level

A

90-95%

19
Q

Rx of wilson’s disease

A

Penicillamine
Trientine hydrocholride
Tetrathiomolhbdate

20
Q

1st line treatment in wilson disease?

A

Penicillamine (chelates copper)

21
Q

agent under investigation for wilson’s disease?

A

tetrathiomolybdate

22
Q

alternative to penicillamine in Wilson’s disease

A

trientine hydrochloride

23
Q

How many countries participate in WMH survey?

A

approx 30

24
Q

what is WMH survey

A

world mental health survey

25
Q

what kind of method does WMH survey has

A

cross sectional

26
Q

what is aim of WMH survey

A

prevalence and correlates of mental disorders around the globe

27
Q

who perform interveiws in WMH surveys?

A

Trained lay interviewers

28
Q

which instrument WMH survey use?

A

WMH-CIDI, a fully structured diagnostic interview

MRCPSYCH_Paper B_MENTOR & More_Dr. ART (62 decks)

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