Misc. - 5

Question 1

what is another name for wilson disease?

Answer 1

hepatolenticular degeneration

Question 2

what is wilson’s disease?

Answer 2

abnormal storage of copper

Question 3

why there is abnormal storage of copper in wilson disease?

Answer 3

fail to excrete

Question 4

where do the copper is stored excessively?

Answer 4

Brain and Liver & cornea

Question 5

what is degenerated in brain in wilson’s disease?

Answer 5

lenticular nucleus (putamen and globus pallidus)

Question 6

genetic of wilson’s disease

Answer 6

autosomal recessive

Question 7

pathology of wilson’s disease

Answer 7

pathological extra storage of copper
excessive absorption of copper

Question 8

which gene defect is responsible for wilson?

Answer 8

ATP7B, chromosome 13

Question 9

what does ATP7B do?

Answer 9

transport & incorporates copper with ceruloplasmin

Question 10

onset of wilson’s

Answer 10

10-25

Question 11

presentation of wilson in children?

Answer 11

liver disease

Question 12

presentation of Wilson in adult?

Answer 12

neurological

Question 13

Features of Wilson’s disease: Liver

Answer 13

hepatitis
cirrhosis

Question 14

Features of Wilson’s disease: Brain

Answer 14

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

Question 15

Features of Wilson’s disease: cornea

Answer 15

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

Question 16

Features of Wilson’s disease: systematic

Answer 16

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

Question 17

Dx of Wilson’s disease

Answer 17

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

Question 18

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

Answer 18

90-95%

Question 19

Rx of wilson’s disease

Answer 19

Penicillamine
Trientine hydrocholride
Tetrathiomolhbdate

Question 20

1st line treatment in wilson disease?

Answer 20

Penicillamine (chelates copper)

Question 21

agent under investigation for wilson’s disease?

Answer 21

tetrathiomolybdate

Question 22

alternative to penicillamine in Wilson’s disease

Answer 22

trientine hydrochloride

Question 23

How many countries participate in WMH survey?

Answer 23

approx 30

Question 24

what is WMH survey

Answer 24

world mental health survey

Question 25

what kind of method does WMH survey has

Answer 25

cross sectional

Question 26

what is aim of WMH survey

Answer 26

prevalence and correlates of mental disorders around the globe

Question 27

who perform interveiws in WMH surveys?

Answer 27

Trained lay interviewers

Question 28

which instrument WMH survey use?

Answer 28

WMH-CIDI, a fully structured diagnostic interview