|
20 |
Role-play. PMB |
|
21 |
Role-play.
Developmental
dysplasia of the hip |
|
22 |
EMQ. The
MAGPIE trial |
|
23 |
SBA. Lynch syndrome |
Candidate’s Instructions.
You are an SpR in the “one-stop” PMB clinic. Mary Smith, 55
years old, has been referred by her General Practitioner. She has had some bleeding
since the menopause.
Your task is to take an appropriate history and advise
her about the investigations you feel are appropriate and why.
Referral letter from the
General Practitioner.
Manor Lodge,
High Street,
Bestown.
BE5 S00
Re: Mrs. Mary Smith,
Age 55.
5b High Street,
Bestown.
BE5 SO1
Dear Doctor,
Please see Mrs. Smith who has had bleeding down below. It
is a number of years since she reached the menopause.
Yours sincerely,
James Fewords,
General Practitioner.
21. Developmental
dysplasia of the hip.
Candidate’s instructions.
You are the SpR in the antenatal clinic. Jenny Brown has
been booked in her second pregnancy by the midwife. She has some questions
about congenital dislocation of the hip, which affected her first child, now 6
years old. The midwife who did the booking does not feel able to provide
adequate answers and has asked you to see the patient.
22. The MAGPIE
trial .
Question 1.
Which of the following is true of the Magpie
trial?
Option list.
|
A |
it compared
MgSO4 with placebo in the management of eclampsia / severe PET |
|
B |
it compared
MgSO4 with lytic cocktail in the management of eclampsia / severe
PET |
|
C |
it compared
MgSO4 with phenytoin in the management of eclampsia / severe PET |
|
D |
it compared
MgSO4 with alcohol in the management of threatened premature
labour |
|
E |
it compared
MgSO4 with atosiban in the management of threatened premature
labour |
|
F |
it compared
MgSO4 with ritodrine in the management of threatened premature
labour |
|
G |
it compared
MgSO4 with dexamethasone in the prevention of cerebral palsy due
to extreme prematurity |
|
H |
it compared
MgSO4 with placebo in the prevention of cerebral palsy due to
extreme prematurity |
|
I |
none of the
above |
Question
2. Which
if any of the following are true of the Magpie trial?
Option list.
|
A |
it involved ~ 1,000 women |
|
B |
it involved ~ 10,000 women |
|
C |
it involved ~ 20,000 women |
|
C |
it involved > 20,000 women |
|
D |
it involved ~ 30 hospitals |
|
E |
it involved ~ 50 hospitals |
|
F |
it involved ~ 80 hospitals |
|
G |
it involved > 150 hospitals |
|
H |
it involved 5 countries |
|
I |
it involved 10 countries |
|
J |
it involved 20 countries |
|
K |
it involved 30 countries |
|
L |
it involved 50 countries |
|
M |
it involved >50 countries |
Question
3. Which
if any of the following are true?
Option list.
|
A |
almost 50% of the women were in Africa |
|
B |
almost 50% of the women were in America |
|
C |
almost 50% of the women were in Asia |
|
D |
almost 50% of the women were in Australia / New Zealand |
|
E |
almost 50% of the women were in Europe |
Question
4. Which,
if any, of the following is true of the Magpie trial?
Option list.
|
A |
cerebral palsy rates at 2 years were ↓ by the use of MgSO4
in babies born < 34 weeks |
|
B |
cerebral palsy rates at 2 years were unchanged by the use of MgSO4 in babies
born < 34 week |
|
C |
eclampsia rates were reduced by about half by the use
of MgSO4 |
|
D |
eclampsia rates were reduced by about half by the use
of MgSO4 but only in underdeveloped countries |
|
E |
maternal mortality was significantly ↓ by the use of
MgSO4 |
|
F |
maternal mortality was significantly ↓ by the use of
MgSO4, but only in underdeveloped countries |
|
G |
premature delivery was significantly ↓ by the use of
MgSO4 |
|
H |
perinatal mortality from prematurity was significantly
↓ by the use of MgSO4 |
23. Lynch syndrome.
Abbreviations
CRC: colorectal
cancer.
EC: endometrial
cancer.
IBD: inflammatory
bowel disease: Crohn’s & ulcerative colitis.
IDDM: insulin-dependent
diabetes mellitus.
Ls: Lynch
syndrome.
MLH: mutL-homolog
family of DNA, mismatch repair genes.
MMR: mismatch
repair.
MSH: mutS
homolog family of DNA, mismatch repair genes.
Question 1.
What is Lynch syndrome?
Option List
|
A |
auto-immune
condition leading to reduced factor X levels in blood |
|
B |
hereditary condition which increases the risk of many
cancers, particularly breast |
|
C |
hereditary
condition which increases the risk of many cancers, particularly breast &
colorectal |
|
D |
hereditary
condition which increases the risk of many cancers, particularly colorectal
& endometrial |
|
E |
none of
the above |
Question 2.
How is Lynch syndrome inherited?
Option List
|
A |
it is an
autosomal dominant condition |
|
B |
it is an autosomal recessive condition |
|
C |
it is an X-linked dominant condition |
|
D |
it is an X-linked recessive condition |
|
E |
none of the above |
Question 3.
Which, if any, of the following genes can cause Lynch syndrome?
Option List
|
A |
MLH1 +
MLH2 + MOH1 |
|
B |
MLH1 + MLH2 + MSH1 |
|
C |
MLH1 + MLH2 + MSH6 |
|
D |
MLH1 + MSH2 + MSH6 + PMS2 |
|
E |
None of the above |
Question 4.
Mutations of which 2 of the following genes cause most cases of Lynch
syndrome?
Option List
|
A |
MLH1 +
MLH2 |
|
B |
MLH1 + MSH1 |
|
C |
MLH1 + MSH2 |
|
D |
MLH2 + MSH1 |
|
E |
MLH2 + MSH2 |
Question 5.
What is the approximate prevalence of Ls in the UK population?
Option List
|
A |
1 in 50 |
|
B |
1 in 100 |
|
C |
1 in
1,000 |
|
D |
3 in
1,000 |
|
E |
none of the above |
Question 6.
Approximately what % of individuals with Ls have had the diagnosis
established?
Option List
|
A |
< 5% |
|
B |
5 -10% |
|
C |
10-20% |
|
D |
20-30% |
|
E |
>30% |
Question 7.
Which, if any, of the following conditions are associated with an ↑
risk of Ls?
Option List
|
A |
acromegaly
+ Addison’s disease + coeliac disease + IBD + IDDM |
|
B |
acromegaly
+ disease + anosmia + coeliac disease + IBD |
|
C |
acromegaly
+ IBD + IDDM |
|
D |
acromegaly
+ IBD |
|
E |
Addison’s
disease + anosmia + coeliac disease + IBD + IDDM |
|
F |
acromegaly
+ Addison’s disease + anosmia + coeliac disease + IBD + IDDM |
|
G |
none of the above |
Question 8.
Which 2 cancers are most likely in women with Lynch syndrome?
Option List
|
A |
breast +
bowel |
|
B |
breast + pancreas |
|
C |
breast + endometrium |
|
D |
bowel + cervix |
|
E |
bowel + endometrium |
|
F |
bowel + ovary |
|
G |
bowel + pancreas |
|
H |
endometrium + ovary |
Question 9.
What does NICE recommend about screening for Lynch syndrome for the
population
with no
personal history of colorectal cancer?
Option List
|
A |
offer screening to those aged < 50 years with ≥ 1 affected 1st.O
relative |
|
B |
offer screening to those aged < 60 years with ≥ 1
affected 1st.O relative |
|
C |
offer screening to those with ≥ 1 affected 1st.O
relative aged < 50 years at diagnosis |
|
D |
offer screening to those with ≥ 1 affected 1st.O
relative aged < 60 years at diagnosis |
|
E |
none of the above |
Question 10.
What does NICE recommend in relation to screening for Lynch syndrome in
those with
a new
diagnosis of colorectal cancer?
Option List
|
A |
offer
screening to everyone, regardless of age and family history |
|
B |
offer screening to those aged < 50 years at
diagnosis |
|
C |
offer screening to those aged < 60 years at
diagnosis |
|
D |
offer screening to those aged < 50 years at
diagnosis with + ≥ 1 affected 1st.O relative |
|
E |
offer screening to those aged < 60 years at
diagnosis with + ≥ 1 affected 1st.O relative |
Question 11.
What does NICE recommend about screening for Lynch syndrome for the
population
with no
personal history of thyroid cancer?
Option List
|
A |
offer screening to those aged < 50 years with ≥ 1 affected 1st.O
relative |
|
B |
offer screening to those aged < 60 years with ≥ 1
affected 1st.O relative |
|
C |
offer screening to those with ≥ 1 affected 1st.O
relative aged < 50 years at diagnosis |
|
D |
offer screening to those with ≥ 1 affected 1st.O
relative aged < 60 years at diagnosis |
|
E |
none of the above |
Question 12.
What does NICE recommend in relation to screening for Lynch syndrome in
those
with a new
diagnosis of thyroid cancer?
Option List
|
A |
offer
screening to everyone, regardless of age and family history |
|
B |
offer screening to those aged < 50 years at diagnosis |
|
C |
offer screening to those aged < 60 years at
diagnosis |
|
D |
offer screening to those aged < 50 years at
diagnosis with + ≥ 1 affected 1st.O relative |
|
E |
none of the above |
Question 13.
What does NICE recommend about screening for Lynch syndrome for the
population
with no personal history of endometrial
cancer?
Option List
|
A |
offer screening to those aged < 50 years with ≥ 1 affected 1st.O
relative |
|
B |
offer screening to those aged < 60 years with ≥ 1
affected 1st.O relative |
|
C |
offer screening to those with ≥ 1 affected 1st.O
relative aged < 50 years at diagnosis |
|
D |
offer screening to those with ≥ 1 affected 1st.O
relative aged < 60 years at diagnosis |
|
E |
none of the above |
Question 14.
What does NICE recommend in relation to screening for Lynch syndrome in
those with
a new
diagnosis of endometrial cancer?
Option List
|
A |
offer screening to those aged < 50 years with ≥ 1 affected 1st.O
relative |
|
B |
offer screening to those aged < 60 years with ≥ 1
affected 1st.O relative |
|
C |
offer screening to those with ≥ 1 affected 1st.O
relative aged < 50 years at diagnosis |
|
D |
offer screening to those with ≥ 1 affected 1st.O
relative aged < 60 years at diagnosis |
|
E |
none of the above |
Question 15.
What does NICE recommend about screening for Lynch syndrome for the
population
with no
personal history of colorectal cancer?
Option List
|
A |
offer screening to those aged < 50 years with ≥ 1 affected 1st.O
relative |
|
B |
offer screening to those aged < 60 years with ≥ 1
affected 1st.O relative |
|
C |
offer screening to those with ≥ 1 affected 1st.O
relative aged < 50 years at diagnosis |
|
D |
offer screening to those with ≥ 1 affected 1st.O
relative aged < 60 years at diagnosis |
|
E |
none of the above |
Question 16.
What does NICE recommend in relation to screening for Lynch syndrome in
those with
a new
diagnosis of colorectal cancer?
Option List
|
A |
offer
screening to everyone, regardless of age and family history |
|
B |
offer screening to those aged < 50 years at
diagnosis |
|
C |
offer screening to those aged < 60 years at
diagnosis |
|
D |
offer screening to those aged < 50 years at
diagnosis with + ≥ 1 affected 1st.O relative |
|
E |
offer screening to those aged < 60 years at
diagnosis with + ≥ 1 affected 1st.O relative |
Question 17.
What relationship, if any, exists between Ls and acromegaly?
Option List
|
A |
the risk
of Ls is ↓
in those with acromegaly compared with the general population |
|
B |
the risk
of Ls is ↑
in those with acromegaly compared with the general population |
|
C |
the risk
of Ls is unchanged in those with acromegaly compared with the general
population |
|
D |
the risk
of Ls in unknown in those with acromegaly |
|
E |
|
Question 18.
What is the effect of aspirin consumption on the risk of EC and CRC?
Option List
|
A |
aspirin
reduces the risk of EC and CRC |
|
B |
aspirin
reduces the risk of EC but not CRC |
|
C |
aspirin
reduces the risk of CRC but not EC |
|
D |
aspirin
does not reduce the risk of EC or CRC |
|
E |
aspirin reduces the risk of EC and CRC, but the risks
outweigh the benefits |
Question 19.
A healthy woman of 35 years is diagnosed with Ls? What are the key
elements of the
National Screening
Programme for people with Ls?
There is
no option list – just write down everything you know.
Question 20.
Which, if any, of the following were recommendations made by Monahan et
al, the 30
experts who wrote to the BMJ in 2017.
Option List
|
A |
creation of a national register of
people with Ls |
|
B |
creation of a
post of Consultant in Ls for each NHS Trust |
|
C |
creation of a
post of Clinical Champion for Ls in each NHS Region. |
|
D |
creation of a
post of Clinical Champion for Ls in the DOH. |
|
E |
none of the
above |
With regard to Lynch
syndrome,
1. loss of mismatch repair protein expression
on immunohistochemistry of cancer is diagnostic.
True/False
2. most carriers of the mutation associated
with the syndrome know they have the condition.
True/False
3. the first cancers associated with the
syndrome are predominantly endometrial or ovarian cancers. True/False
4. when cancers occur, they have in them an unusually
high immune infiltrate. True/False
With regard to testing for Lynch syndrome,
5. consent must be sought before definitive germline
testing for Lynch syndrome by a trained professional. True/False
6. immunohistochemical staining of tumours for
the mismatch repair proteins or microsatellite instability analysis are recognised
ways of screening cancers for characteristics suggestive of the syndrome. True/False
7. the National Institute for Health and Care Excellence
endorses universal screening of colorectal cancer patients for Lynch syndrome. True/False
8. most gynaecological cancers found to have aberrant
mismatch repair immunohistochemical staining will be in those with the
syndrome. True/False
9. the addition of MLH1 promotor hypermethylation
testing in a Lynch syndrome diagnostic pathway improves specificity. True/False
Regarding gynaecological surveillance in women with Lynch
syndrome,
10. there is strong evidence to recommend its use.
True/False
11. this should be offered to women around 25 years
of age. True/False
12. counselling should include education on red flag
symptoms of cancer and risk-reducing surgery.
True/False
With regard to risk-reducing strategies for women with Lynch
syndrome,
13. hysterectomy is strongly recommended for all those
with the syndrome. True/False
14. the timing of risk-reducing surgery depends on
the syndrome gene. True/False
15. where possible, a laparoscopic approach is
recommended. True/False
16. aspirin is not recommended as a means of reducing
their overall cancer risk. True/False
Regarding Lynch syndrome-associated gynaecological
cancers,
17. endometrial types that arise as a result of
the syndrome have a poorer prognosis than sporadic types. True/False
18. checkpoint inhibition of the PD-1/PD-L1 pathway
has been shown to be very effective in mismatch repair-deficient cancers. True/False
19. vaccination against these cancers is currently
the focus of research. True/False
20. the Manchester International Consensus guideline
is a useful reference for gynaecologists managing women with these cancers. True/False
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