Thursday 2 May 2019

Tutorial 2nd. May 2019



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7
RCOG sample questions. There are also sample EMQs & SBAs. Go through all of them as they make for easy marks. TOG has CPD questions in every issue. Make sure you can answer all of them from the last 2 – 3 years.
8
SBA.   Lynch syndrome
9
EMQ. Maternal Mortality definitions
10
EMQ. Phenylketonuria
11
EMQ. Caldicott guardian
12
EMQ. Peutz-Jeghers syndrome

7. RCOG sample questions.

8.  Lynch syndrome.
Abbreviations
CRC:              colorectal cancer.
EC:                endometrial cancer.
HNPCC:        hereditary non-polyposis colo-rectal cancer.
IBD:               inflammatory bowel disease: Crohn’s & ulcerative colitis.
IDDM:          insulin-dependent diabetes mellitus.
Ls:                  Lynch syndrome.
Question 1.           
Lead-in
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.           
Lead-in
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.           
Lead-in
Which, if any, of the following genes can cause Lynch syndrome?
Genes.
A
MLH1
B
MLH2
C
MOH1
D
MSH1
E
MSH6
Option List
A
MLH1 + MLH2 + MOH1
B
MLH1 + MLH2 + MSH1
C
MLH1 + MLH2 + MSH6
D
MLH1 + MSH2 + MSH6
E
None of the above
Question 4.           
Lead-in
Mutations of which 2 of the following genes cause the majority of cases of Lynch syndrome?
Genes.
A
MLH1
B
MLH2
C
MOH1
D
MSH1
E
MSH6
Option List
A
MLH1 + MLH2
B
MLH1 + MSH1
C
MLH1 + MSH2
D
MLH2 + MSH1
E
MLH2 + MSH2
Question 5.           
Lead-in
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.           
Lead-in
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.           
Lead-in
Which, if any, of the following conditions are associated with an risk of Lynch syndrome?
Conditions
acromegaly
Addison’s disease
anosmia
coeliac disease
IBD
IDDM
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
acromegaly + Addison’s disease + anosmia + coeliac disease + IBD + IDDM
H
none
Question 8.           
Lead-in
Which 2 cancers are most likely in women with Lynch syndrome?
Cancers.
A
breast
B
bowel
C
cervix
D
endometrium
E
ovary
F
pancreas
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.           
Lead-in
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.       
Lead-in
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.       
Lead-in
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.       
Lead-in
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.       
Lead-in
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.       
Lead-in
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.       
Lead-in
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.       
Lead-in
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.       
Lead-in
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
Question 18.       
Lead-in
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.       
Lead-in
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.       
Lead-in
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

9.  Maternal Mortality.

Lead-in.
The following scenarios relate to maternal mortality.
Pick the option that best answers the question in each scenario.
Each option can be used once, more than once or not at all.
Option List.
A.   Death of a woman during pregnancy and up to 6 weeks later, including accidental and incidental causes.
B.   Death of a woman during pregnancy and up to 6 weeks later, excluding accidental and incidental causes.
C.   Death of a woman during pregnancy and up to 52 weeks later, including accidental and incidental causes.
D.   Death of a woman during pregnancy and up to 52 weeks later, excluding accidental and incidental causes.
E.    A pregnancy going to 24 weeks or beyond.
F.    A pregnancy going to 24 weeks or beyond + any pregnancy resulting in a live-birth.
G.   Maternal deaths per 100,000 maternities.
H.   Maternal deaths per 100,000 live births.
I.      Direct + indirect deaths per 100,000 maternities.
J.     Direct + indirect deaths per 100,000 live births.
K.   Direct death.
L.    Indirect death.
M. Early death.
N.  Late death.
O.  Extra-late death.
P.   Fortuitous death.
Q.  Coincidental death.
R.   Accidental death.
S.    Maternal murder.
T.    Not a maternal death.
U.  Yes
V.   No.
W.I have no idea.
X.   None of the above.
Abbreviations.
MMR:     Maternal Mortality Rate.
MMRat: Maternal Mortality Ratio.
SUDEP:   Sudden Unexplained Death in Epilepsy.      
Option list.
There is none.
Scenario 1.
What is a Maternal Death?
Scenario 2.
A woman dies from a ruptured ectopic pregnancy at 10 weeks’ gestation. What kind of death is it?
Scenario 3.
A woman dies from a ruptured appendix at 10 weeks’ gestation. What kind of death is it?
Scenario 4.
A woman dies from suicide at 10 weeks’ gestation. What kind of death is it?
Scenario 5.
A woman with a 10-year-history of coronary artery disease dies of a coronary thrombosis at 36 weeks’ gestation. What kind of death is it?
Scenario 6.
A woman has gestational trophoblastic disease, develops choriocarcinomas and dies from it 24 months after the GTD was diagnosed and the uterus evacuated. What kind of death is it?
Scenario 7
A woman develops puerperal psychosis from which she makes a poor recovery. She kills herself when the baby is 18 months old. What kind of death is it?
Scenario 8
A woman develops puerperal psychosis from which she makes a poor recovery. She kills herself when the baby is 6 months old. What kind of death is it?
Scenario 9
What is a “maternity”.
Scenario 10
What is the definition of the Maternal Mortality Rate?
Scenario 11
What is the Maternal Mortality Ratio?
Scenario 12
A woman is diagnosed with breast cancer. She has missed a period and a pregnancy test is +ve. She decides to continue with the pregnancy. The breast cancer does not respond to treatment and she dies from secondary disease at 38 weeks. What kind of death is it?
Scenario 13
A woman who has been the subject of domestic violence is killed at 12 weeks’ gestation by her partner. What kind of death is it?
Scenario 14
A woman is struck by lightning as she runs across a road. As a result she falls under the wheels of a large lorry which runs over abdomen, rupturing her spleen and provoking placental abruption. She dies of haemorrhage, mostly from the abruption. What kind of death is it?
Scenario 15
A woman is abducted by Martians who are keen to study human pregnancy. She dies as a result of the treatment she receives. As this death could only have occurred because she was pregnant, is it a direct death?
Scenario 16
Could a maternal death from malignancy be classified as “Direct”.
Scenario 17
Could a maternal death from malignancy be classified as “Indirect”.
Scenario 18
Could a maternal death from malignancy be classified as “Coincidental”?
Scenario 19.
A pregnant woman is walking on the beach at 10 weeks when she is struck by lightning and dies. What kind of death is this?
Scenario 20.
A woman is sitting on the beach breastfeeding her 2-month old baby when she is struck by lightning and dies. What kind of death is this.

10.  Topic. Phenylketonuria in pregnancy
Some of these are not true EMQs – some have no option list and you have to decide the correct answer for yourself. This is what you are advised to do in the exam – read the question, decide the answer and then look for it on the option list.
Abbreviations.
BH4:         tetrahydrobiopterin.
BH4D:      tetrahydrobiopterin deficiency.
HPA:        hyperphenylalaninaemia.
PA:           phenylalanine.
PAH:        phenylalanine hydroxylase.
PKU:        phenylketonuria.
Tyr:          tyrosine.
Option list.
A.       
autosomal dominant
B.       
autosomal recessive
C.       
X-linked dominant
D.      
X-linked recessive
E.       
1 in 100,000
F.        
1 in 50,000
G.      
1 in 10,000
H.      
1 in 5,000
I.         
deficiency in phenylalanine hydroxylase
J.         
deficiency in phenylalanine oxidase
K.       
deficiency in phenylalanine transferase
L.        
deficiency in phenylketone hydroxylase
M.    
deficiency in phenylketone oxidase
N.      
raised PA levels
O.      
reduced PA levels
P.       
raised tyrosine levels
Q.      
reduced tyrosine levels
R.       
normal tyrosine levels
S.        
No
T.       
Yes
U.      
unknown
What is PKU? Write your answer – there is no option list.

Question 2.           
What is PKU due to? Use the option list.
Question 3.           
What levels of PA and Tyr are typical in PKU? Use the option list. This is not a real EMQ as there are two answers.
Question 4.           
Is PKU subdivided into different categories? If “yes”, what are the categories? Write your answer – there is no option list.
Question 5.           
How is PKU inherited? Use the option list.
Question 6.           
Which chromosome houses the gene related to PKU transmission?
Question 7.           
How many mutations of the gene related to PKU have so far been identified?
Question 8.           
Is a person with PKU likely to have one or two mutations of the relevant gene?
Question 9.           
What are the main consequences of PKU? Write your answer – there is no option list.
Question 10.       
Which, if any, of the following are characteristic of PKU?
Option list.
A.       
alopecia
B.       
angst
C.       
facial dysmorphism
D.      
facial hair in females and pre-pubertal males
E.       
kyphosis
F.        
macroorchidism in post-pubertal males
Question 11.       
Are fetal PKU levels higher or lower than maternal? Write your answer – there is no option list.
Question 12.       
What is the approximate prevalence of PKU in Caucasians?
Question 13.       
The prevalence of PKU varies between ethnic groups.
Match each of the following ethnic groups to the closest prevalence given in the option list.
Option List
1.       
1 in 1,000
2.       
1 in 2,500
3.       
1 in 5,000
4.       
1 in 10,000
5.       
1 in 100,000
6.       
1 in 150,000
7.       
1 in 200,000
8.       
1 in 1,000,000

Ethnic group
Prevalence
Turkish

Irish

Caucasian

East Asian

Japanese

Finnish

Question 14.       
Is screening for PKU a routine part of the neonatal screen in the UK?
Question 15.       
The test for PKU used to be known by the name of its inventor. Who was he and why did he have a particular interest? There is no option list and no one is going to ask you except me!
Lead-in
What is the main treatment of PKU and what problems are associated with it? There is no option list.
Question 17.       
Lead-in
How long should the main treatment of PKU be continued and why? There is no option list.

Question 18.       
Lead-in
A woman with PKU is planning her first pregnancy at the age of 22. She has been off the PKU-restricted diet since the age of 10 and can barely remember being on it. Should she be advised to re-start the diet? If ‘yes’, when should she start and what explanation would you give for the advice?
Question 19.       
Lead-in
Is breast-feeding advisable for women with PKU?
Question 20.       
Lead-in
Are any other therapeutic approaches available? If ‘yes’, what are they and how do they work? If ‘yes’ use the option list for the mode of action.

There is a recent TOG article by Munyame et al.
The linked CPD questions are here:
Send your answers and I’ll send mine.

11. Caldicott Guardian.
Question 1.
Lead-in
Which of the following statements is true of the Caldicott Guardian?
Option List
A
it is a large lizard, unique to the Galapagos Islands
B
it is the Trust Board member responsible for child safeguarding procedures
C
it is the Trust Board member responsible for complaint procedures
D
it is the person within a Trust responsible for patient confidentiality in relation to information
E
it is the person within a Trust responsible for dealing with bullying
Question 2.
Lead-in
The Caldicott Report identified 6 basic principles. What are they?
Option list.
There is none. Imagine that there is information about you stored on the computers of the local NHS Trust. What conditions would you want to lay down about sharing of that information within the Trust, with other NHS organisations and with non-NHS organisations?
Question 3.
Lead-in
The Caldicott Report made numerous recommendations. Which was particularly important for major NHS organisations such as Trusts?
Option List

  1.  
the need to appoint a Caldicott Guardian

  1.  
the need to create a Caldicott Register

  1.  
the need to create a Caldicott Police Department

  1.  
the need to create a link between the Caldicott Department and the DOH

  1.  
none of the above.
Question 4.
Lead-in
What is the definition of the key role deriving from the answer to question 3?
Option List
There is none lest it give you the answer to question 3!

 12. Peutz-Jeghers syndrome.
Scenario 1.             
Which, if any, of the following are characteristics of PJS?
Option list.
A.       
buccal pigmentation
B.       
gastro-intestinal hamartomas
C.       
perianal pigmentation
D.      
increased risk of breast cancer
E.       
increased risk of cervical adenoma malignum
F.        
increased risk of colo-rectal cancer
G.      
increased risk of endometrial cancer
H.      
increased risk of ovarian cancer
I.         
increased risk of pancreatic cancer
J.         
increased risk of prostate cancer
K.       
increased risk of stomach cancer
Scenario 2.             
What is the approximate prevalence of PJS?
Option list.
A.       
< 1 in 1,000
B.       
1 in 1,000 to 1 in 10,000
C.       
1 in 10,000 to 1 in 100,000
D.      
1 in 25,000 to 1 in 100,000
E.       
1 in 25,000 to 1 in 200,000
F.        
1 in 25,000 to 1 in 300,000
G.      
1 in 300,000 to 1 in 500,000
H.      
< 1 in 500,000
Scenario 3.             
What is the mode of inheritance in PJS?
Option list.
A
autosomal dominant
B
autosomal recessive
C
X-linked dominant
D
X-linked recessive
E
Y-linked dominant
F
Y-linked recessive
G
triplet repeat
Scenario 4.             
Which, if any, of the following statements are true of PJS?
Option list.
A
PJS only occurs in families with other affected members
B
PJS mainly occurs in families with other affected members
C
PJS may arise de-novo in families with no other affected members
D
PJS may arise de-novo in families with other affected members
E
PJS does not arise de-novo in families with no other affected members
Scenario 5.             
What is the approximate lifetime risk of developing cancer in PJS?
Option list.
A.       
10%
B.       
20%
C.       
30%
D.      
40%
E.       
50%
F.        
60%
G.      
70%
H.      
80%
I.         
90%
J.         
>90%
Scenario 6.             
What is the relevance of SK11 to PJS?
Option list.
A.       
It is part of the postcode of the Peutz-Jeghers Society
B.       
It is the name of the gene most commonly associated with PJS
C.       
It is the Ornithological Society’s code for the Orkney Skua
D.      
Somatic mutations have been found in cervical cancer
E.       
None of the above


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