Monday, 1 October 2018

Tutorial 1st. October 2018




19
EMQ. Surrogacy
20
Viva. The uses of MgSO4 in O&G.
21
Role-play. Neonatal screening.
22
EMQ. Phenylketonuria
23
EMQ. Caldicott guardian
24
EMQ. Peutz-Jeghers syndrome

19. EMQ. Surrogacy.
Surrogacy.
Lead-in.
This question is about surrogacy. For each scenario, pick the best choice from the option list. Each option can be used once, more than once or not at all.
Abbreviations.
CF:              commissioning father
CM:            commissioning mother
CPs:            commissioning parents
PO:             parental order
SM:             surrogate mother
Option List.
a)       CM
b)      CF
c)       CPs
d)      SM
e)      Chairman of the HFEA
f)        Senior judge at the Children and Family Court
g)       traditional surrogacy
h)      gestational surrogacy
i)        HFEA
j)        SSAEW
k)       RCOG Surrogacy Sub-Committee
l)        false
m)    true
n)      none of the above

Scenario 1
List the different types of surrogacy.
Scenario 2.
“Gestational” surrogacy has better “take-home-baby” rates than “traditional” surrogacy.
Scenario 3.
There are approximately 1,000 surrogate pregnancies per annum in the UK.
Scenario 4.
Which national body regulates surrogacy in England?
Scenario 5.
Privately-arranged surrogate pregnancies are illegal and those involved are liable to up to 2 years in prison.
Scenario 6.
List the risks of surrogacy.
Scenario 7.
Obstetricians are legally obliged to take the CPs’ wishes into consideration in managing pregnancy complications or problems.
Scenario 8.
The psychological outcomes of surrogacy are fully understood.
Scenario 9.
The psychological outcomes of surrogacy are more severe after traditional surrogacy.
Scenario 10.
Who has the right to arrange TOP if the fetus is found to have a major congenital abnormality?
Scenario 11.
A SM decides at 10 weeks that she does not wish to be pregnant and arranges to have a TOP. The CPs. hear about this and object strongly. To whom should they apply to have the TOP blocked?
Scenario 12.
A woman has hysterectomy and BSO to deal with extensive endometriosis at the age of 30. She marries two years later and her sister offers to act as surrogate. She undergoes IVF and 4 embryos are created. One is transferred and a successful pregnancy ensues. The baby is adopted by the woman and her husband. The 3 remaining embryos were frozen. Four years later the woman falls out with her sister, but finds another surrogate and wishes to proceed with another pregnancy. The sister says she does not want her eggs to be used and that the frozen embryos should not be transferred. Does the sister have the legal right to block the use of the embryos?
Scenario 13.
A girl born from donor sperm reaches the age of 16 and wishes to know the identity of her genetic father. Does she have the right to this information?
Scenario 14.
A girl born from donor sperm reaches the age of 18 and wins a place at Oxford University to read medicine. Does she have the legal right to get the donor to contribute to her fees?
Scenario 15.
A PO is active from the moment it is completed and signed by the relevant parties.
Scenario 16.
A SM can change her mind at any time and keep the child, even if the egg was not hers.
Scenario 17.
The CPs can change their mind, leaving the SM as the legal mother.
Scenario 18.
A SM’s husband is the legal father until adoption is completed or a PO comes into force.
Scenario 19.
A lesbian couple in a stable, co-habiting relationship can be CPs and become the legal parents of the child of a SM.
Scenario 20.
CPs are likely to get faster legal status as the legal parents through application for a PO rather than applying for adoption.

20. Viva. The uses of MgSO4 in O&G.
Candidate’s instructions.
This is a viva station about the uses of MgSO4 in O&G. To make it harder, the examiner will not ask questions, prompt or otherwise assist. It is up to you to give as full an account of the uses as you can muster.

21. Role-play. Neonatal screening.
Candidate’s instructions.
You are a SpR in year 5.
You are in the antenatal booking clinic and about to see Mary Eccles. She has been booked by a midwife at 10 weeks’ gestation and all is well. She has recently arrived in the UK from the USA and asked about the routine neonatal screening that is done in the UK. She will be having the baby in the UK.

22. EMQ. Phenylketonuria.
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?
Answer.
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
Caucasian

East Asian

Finnish

Irish

Japanese

Turkish

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.

23. EMQ. Caldicott guardian.
Question 1.
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.
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.
The Caldicott Report made numerous recommendations. Which was particularly important for major NHS organisations such as Trusts?
Option List
A.       
the need to appoint a Caldicott Guardian
B.       
the need to create a Caldicott Register
C.        
the need to create a Caldicott Police Department
D.       
the need to create a link between the Caldicott Department and the DOH
E.        
none of the above.
Question 4.
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!

24. EMQ. 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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