28th. January 2016.
44
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SBA. Fragile X syndrome
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45
|
EMQ. Risk management / disciplinary
procedures
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46
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EMQ. Endometrial cancer & FIGO
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47
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Communication skills.
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44. SBA. Fragile
X syndrome
Fragile X syndrome
Abbreviations.
FXS: Fragile
X syndrome
TR: trinucleotide
repeat
Question 1.
Lead-in
Which, if
any, of the following are features of FXS in males?
Option List
A.
|
autism
|
B.
|
epilepsy
|
C.
|
hyper-extensible joints
|
D.
|
learning
difficulty
|
E.
|
post-pubertal macroorchidism
|
Question 2.
Lead-in
Which, if
any, of the following are features of FXS in females?
Option List
A.
|
autism
|
B.
|
epilepsy
|
C.
|
hyper-extensible joints
|
D.
|
learning
difficulty
|
E.
|
post-pubertal ovarian enlargement
|
Question 3.
Lead-in
Why are women
thought to be less affected by FXS than men?
Option List
A.
|
two X
chromosomes dilutes the effect of affected X chromosome
|
B.
|
leonisation
|
C.
|
lionisation
|
D.
|
lyonisation
|
E.
|
none of the above
|
Question 4.
Lead-in
How common
is FXS in males?
Option List
A.
|
1 in
1,000
|
B.
|
1 in 4,000
|
C.
|
1 in 8,000
|
D.
|
1 in 20,000
|
E.
|
1 in 100.000
|
Question 5.
Lead-in
How common
is FXS in females?
Option List
A.
|
1 in
1,000
|
B.
|
1 in 4,000
|
C.
|
1 in 8,000
|
D.
|
1 in 20,000
|
E.
|
1 in 100.000
|
Question 6.
Lead-in
Which gene
is implicated in the causation of FXS?
Option List
A.
|
fragile
X mental retardation 1
|
B.
|
fragile X mitochondrial recognition 1
|
C.
|
fragile X 1
|
D.
|
the gene has not yet been identified
|
E.
|
none of the above
|
Question 7.
Lead-in
Which is
the leading hereditary cause of learning difficulty?
Option List
F.
|
Down’s
syndrome
|
G.
|
fragile X syndrome
|
H.
|
galactosaemia
|
I.
|
homocystinuria
|
J.
|
phenylketonuria
|
Question 8.
Lead-in
Which is
the most common genetic cause of autism?
Option List
A.
|
Down’s
syndrome
|
B.
|
fragile X syndrome
|
C.
|
galactosaemia
|
D.
|
homocystinuria
|
E.
|
phenylketonuria
|
Question 9.
Lead-in
Which mode
of inheritance occurs with FXS?
Option List
A.
|
autosomal
dominant
|
B.
|
autosomal recessive
|
C.
|
X-linked dominant
|
D.
|
X-linked recessive
|
E.
|
none of the above
|
Question 10.
Lead-in
What is
the story about trinucleotide repeats and FXS. What are TRs? Which TRs are
involved with FXS? How are TRs categorised in relation to FXS?
There is no option list – just write your answers.
Question 11.
Lead-in
What is
the FXS premutation?
What are
its key features?
There is no option list – just write your answers.
45. EMQ. Risk
management / disciplinary procedures
Lead-in.
The following scenarios relate to risk management /
disciplinary procedures.
Pick one option from the option list.
Each option can be used once, more than once or not at
all.
Abbreviations.
DOH: Department
of Health.
Option list.
A. allow
the practice to continue
B. stop
the practice until a full investigation has been done
C. stop
the practice permanently
D. arrange
an investigation by a senior consultant from another hospital
E. decide
the practice does not involve added risk
F. declare
the risk to be acceptable
G. cancel
admissions for surgery
H. arrange
adverse incident analysis
I. arrange
audit
J. arrange
research
K. arrange
a formal warning for the doctor
L. arrange
retirement for the doctor
M. arrange
dismissal for the doctor
N. consult
the on-call consultant
O. consult
the Clinical Director
P. consult
the Educational Supervisor / College Tutor
Q. consult
the Medical Director
R. consult
the Chief Executive
S. consult
the Postgraduate Dean.
T. consult
the hospital’s lawyer
U. write to
Her Majesty at Buckingham Palace
V. consult
your Medical Defence Body
W. consult
the British Medical Association
X. consult
the RCOG
Y. report
the matter to the GMC
Z. allow
return to work
AA. allow
return to work, but offer support
BB. arrange a
“return to work” package specific to the doctor
CC. none of
the above
Scenario 1
You are the Clinical Director.
1 62-year-old Consultant has been off
work for 8 weeks with a broken arm sustained in a skiing accident. He sends you
a certificate from his specialist to say that he is now fit to return to work.
He indicates that he wishes to return to work immediately. What action will you
take?
Scenario 2
You are the Clinical Director. A
62-year-old Consultant colleague has been off work for 8 weeks with a severe
bereavement reaction to the suicide of a family member. He sends you a
certificate from his GP to say that he is now fit to return to work. He
indicates that he wishes to return to work immediately. What action will you
take?
Scenario 3
You are the Clinical Director. A
62-year-old Consultant colleague has been off work for 6 months after having a
coronary thrombosis. He sends you a certificate from his specialist to say that
he is now fit to return to work. He indicates that he wishes to return to work
immediately. What action will you take?
Scenario 4
You are the Clinical Director.
A 62-year-old Consultant has returned to work after four months’ sick leave
after a coronary thrombosis. He has three cases on his first operating list and
all have complications reported by the Sister on the gynaecology ward. What
action will you take?
Scenario 5.
A Consultant has been in her
first consultant post for two months. Three of the four patients on a single
operating list develop post-operative wound infections. What action will you
take?
Scenario 6.
You have recently been
appointed Clinical Director. A consultant has been in post for ten years and
prefers to operate with the same nurse assistant. No complications have been
reported. What action will you take?
Scenario 7.
You are the Clinical Director. A consultant has an operating list in a peripheral unit 20 miles from the
main hospital. There is no resident doctor with post-operative care being
provided by nurses. The cases dealt with on the list traditionally were minor,
day-cases. You have been told that the
consultant, who was appointed 6 months ago, has recently been doing hysterectomies
and prolapse repairs to get the waiting list down. What action will you take?
Scenario 8.
You are the Clinical Director.
The blood bank informs you that there is a problem with supplies and fully
cross-matched blood cannot be guaranteed for tomorrow’s arranged surgical
cases.
What action will you take?
Scenario 9.
You are the on-call SpR. It is
8 pm. The blood bank informs you that there is a problem with supplies and
fully cross-matched blood cannot be guaranteed for tomorrow’s arranged surgical
cases.
What action will you take?
Scenario 10.
An SpR is half an hour late for
starting his duties on three occasions in one week. His consultant wishes to
have this dealt with as a disciplinary matter to “nip it in the bud” and teach
him a lesson. He reports it to you, the Clinical Director asking you to
discipline the doctor. What action will you take?
Scenario 11
An SpR gets into an argument
with the senior midwife on the labour ward and in the heat of the moment slaps
her across the face. You are the Clinical Director and the matter is reported to
you next day.
Scenario 12
Your consultant is the Clinical
Director and a nasty man. You apply 6 months in advance for study leave for the
week before the written part of the Part Ii MRCOG exam. He tells you that he
plans to go on holiday at that time and you are not going to get any leave. In
addition, he tells you that if you complain about this he will give you a
terrible reference and tell all his consultant friends that you are a waste of
space in order to ruin your career. What action can you take?
Scenario 13
A SpR fails an OSATS, but
falsifies his records to indicate that it has been completed satisfactorily.
You are the Educational Advisor and this is brought to your attention. What
action will you take ?>
Scenario 14
You are the Clinical Director. A
SpR2 uploaded reflective practice putting himself in a good light after a case
which had been handled sub-optimally by him. What action will you take?
Scenario 15
You are an FY2 and assist
the senior consultant at a hysterectomy. The operation goes well initially, but
then there is a lot of bleeding and a ureter is cut. The consultant urologist
attends and repairs the ureter. The woman bleeds vaginally that evening and is
taken back to theatre by another consultant and ends up in the ICU. You became
convinced during the operation that you could smell alcohol on the consultant
gynaecologist’s breath. What are your responsibilities?
Scenario 16
When do you need to inform the Consultant on-call?
Scenario 17
When do you need to inform the Clinical Director?
Scenario 18
When do you need to inform the Medical Director?
Scenario 19
When do you need to inform the GMC?
Scenario 20
What are the roles of the BMA and MDU?
Scenario 21
What are the differences between verbal and written
warnings?
Scenario 22.
Stolen case-notes.
A question along these lines has come in the exam. Deema
sent me most of the details and others have added bits.
Option
list.
|
A.
|
Report events to the Caldicott Guardian
|
|
B.
|
Report events to the Chief Executive
|
|
C.
|
Report events to the General Medical Council
|
|
D.
|
Report events to the NHSLA as a “never event”
|
|
E.
|
Report events to the NHSLA as a “serious incident”
|
|
F.
|
Report events to the Root Cause Analysis Team
|
|
G.
|
Report events as a serious adverse incident to the Risk
Management Team
|
|
H.
|
Report events to the Trust Information Management
Committee
|
|
I.
|
Suspend the doctor until a full investigation has been
done
|
A SpR1 has been asked to carry out an audit and 50 sets
of case-notes are to be used.
He is given 49 sets of notes and a day in which to go
through them and extract the necessary data.
This he does in the hospital.
The final set of notes cannot be found initially, but are
found two weeks later.
The doctor is given the notes on a Friday afternoon as he
is leaving for home.
He decides to take the notes home to extract the data.
On the way home he stops at his favourite supermarket.
When he emerges, his car has been stolen with the notes
inside.
He reports the theft to the police.
He informs you, the Clinical Director, on the Monday when
he returns to work.
What action will you take?
Pick one option from the option list.
Scenario 23.
Lead-in.
You are the SpR for the delivery unit. During a quiet moment
you head for the staff room adjacent to the operating theatre for a coffee. As
you pass the anaesthetic room you hear loud snoring. You look in and find the
on-call anaesthetic registrar unconscious on his back on the floor with an anaesthetic
mask by his face attached to a cylinder of nitrous oxide.
What action will you take?
Pick one option from the option list.
Option list.
|
|
call for help
|
|
|
go back to the labour ward and pretend that nothing has
happened
|
|
|
go back to the labour ward and inform the senior midwife
|
|
|
phone the GMC
|
|
|
phone the on-call consultant anaesthetist
|
|
|
phone the on-call consultant obstetrician
|
|
|
phone the police
|
|
|
put the anaesthetist in the recovery position and remove
the mask
|
|
|
none of the above
|
Scenario 24.
Lead-in.
You are the SpR for the delivery unit. During a quiet moment
you head for the staff room adjacent to the operating theatre for a coffee. As
you pass the anaesthetic room you hear loud snoring. You look in and find the
on-call anaesthetic registrar unconscious on his back on the floor with an
anaesthetic mask by his face attached to a cylinder of nitrous oxide.
What action will you take next?
Pick one option from the option list.
Option list.
|
|
call for help
|
|
|
go back to the labour ward and pretend that nothing has
happened
|
|
|
go back to the labour ward and inform the senior midwife
|
|
|
phone the GMC
|
|
|
phone the on-call consultant anaesthetist
|
|
|
phone the on-call consultant obstetrician
|
|
|
phone the police
|
|
|
put the anaesthetist in the recovery position and remove
the mask
|
|
|
none of the above
|
Scenario 25.
Lead-in.
You are the Clinical Director. It is the morning after the
events in scenarios 22 and 23.
The on-call consultant obstetrician comes to see you are
reports what has happened.
What action will you take?
Pick one option from the option list.
Option list.
|
|
discuss the case with the Chief Executive
|
|
|
discuss the case with the Medical Defence Union
|
|
|
discuss the case with the Medical Director
|
|
|
discuss the case with the Medical Director
|
|
|
discuss the case with the most senior person in the
personnel department
|
|
|
discuss the case with the Postgraduate Dean
|
|
|
report the anaesthetic registrar to the GMC
|
|
|
resign from being Clinical Director to avoid stress
|
|
|
summon the anaesthetic registrar to give him a severe
telling-off
|
46. EMQ. Endometrial
cancer & FIGO
Lead-in.
The following scenarios relate to endometrial cancer.
Pick one option from the option list.
Each option can be used once, more than once or not at
all.
Option list.
Scenario 1.
A histology report on
endometrial curetting is of 95% adenocarcinoma with 5% solid, non-squamous
areas. What is the FIGO grade?
Scenario 2.
A histology report on
endometrial curetting is of 90% adenocarcinoma with 10% solid, non-squamous
areas. What is the FIGO grade?
Scenario 3.
A histology report on
endometrial curetting is of 50% adenocarcinoma with 50% solid, non-squamous
areas. What is the FIGO grade?
Scenario 4.
A woman undergoes surgery for carcinoma of the
endometrium. Histology shows the tumour is confined to the body of the uterus
with 5% myometrial invasion. What is the FIGO staging?
Answer.
Scenario 5.
A woman undergoes surgery for carcinoma of the
endometrium. Histology shows the tumour is confined to the body of the uterus
with 25% myometrial invasion. What is the FIGO staging?
Scenario 6.
A woman undergoes surgery for carcinoma of the
endometrium. Histology shows the tumour is confined to the body of the uterus
with 60% myometrial invasion. What is the FIGO staging?
Scenario 7
A woman undergoes surgery for carcinoma of the
endometrium. Histology shows the tumour is confined to the uterus. There is no
myometrial invasion, but there is extension to the endocervical endothelium.
What is the FIGO staging?
Scenario 8
A woman undergoes surgery for carcinoma of the
endometrium. Histology shows the tumour is confined to the uterus. There is no
myometrial invasion, but there is extension to the stroma of the cervix. What
is the FIGO staging?
Scenario 9
A woman undergoes surgery for carcinoma of the endometrium.
Peritoneal washings are +ve but there is no other evidence of spread outside
the uterus. There is no myometrial invasion. There is extension to the stroma
of the cervix. What is the FIGO staging?
Scenario 10
A woman undergoes surgery for carcinoma of the
endometrium. There is no evidence of extension outside the uterus. There is
myometrial invasion through to and including the serosa. What is the FIGO
staging?
Scenario 11
A woman undergoes surgery for carcinoma of the
endometrium. There is myometrial invasion. Tumour is noted in the vagina. There
is no evidence of disease elsewhere. What is the FIGO staging?
Scenario 12
A woman undergoes surgery for carcinoma of the
endometrium. There is myometrial invasion. Tumour is noted in the vagina.
Positive pelvic nodes are found, but no other lymphatic involvement. There is
no distant spread. What is the FIGO staging?
Scenario 13
A woman undergoes surgery for carcinoma of the
endometrium. There is myometrial invasion. Tumour is noted in the vagina. The
tumour involves the mucosa of the bladder. There is no lymphatic or distant
spread. What is the FIGO staging?
47. Communication
skills.
Introduce
yourself and explain recessive inheritance to a woman who has been found to be
a carrier of cystic fibrosis.
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