Tutorial 12 July 2021

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Website.

 

1	How to prepare. Part 2.
2	How to prepare. Part 3.
3	Part 3. The 5 domains and creating an agenda.
4	Structured conversation. The Part 3 exam.
5	Basic “blurbs” to write and practise.
6	Role-play. Woman attends for pre-pregnancy counselling as she plans her 1st. pregnancy. Her sister recently had a baby with Down’s syndrome.
7	EMQ. Marfan syndrome
8	SBA. Androgen insensitivity syndrome

 

1.     How to prepare. Part 2.

What to read. StratOG. TOG CPD. RCOG sample questions. Revision system. Study buddies. Intelligent guessing. Statistics. Urogynae. Other specialist tutorial.

 

2.     How to prepare. Part 3.

StratOG. Picking a course. Communication skills. Study partner. Importance of polished introduction to role-plays. Senior doctor thinking: staffing, training, audit, critical incident reporting and analysis.

 

3.     Part 3.

The 5 domains and creating an agenda.

 

4.     Structured conversation. The Part 3 exam.

Candidate’s instructions.

This is a viva station. The examiner will ask you 7 questions about the Part 3 exam.

 

5.     Basic “blurbs” to write and practise.

Setting the scene for breaking bad news, dealing with the information in a GP referral letter, general pre-pregnancy counselling, recessive inheritance, x-linked inheritance, how to ask if the role-player has questions, dealing with information such as a relative with a serious problem,  etc. Make a list

 

6.     Role-play. Pre-pregnancy counselling.

Candidate’s instructions.

You are the SpR in the gynaecology clinic. You have been asked to see Jenny Williams, who has come for pre-pregnancy counselling.

Letter from the General Practitioner.

5 High Street,

Deersworthy, Kent.

DO9 1JY.

Re Mrs. J. Williams,

Manor Place, Deersworthy.

Dear Dr.,

Please see this woman who is planning pregnancy. I understand that her sister has had a baby with Down’s syndrome.

Regards,

Dr. Jolly.

 

7.     EMQ. Marfan syndrome.

Abbreviations.

AR:             aortic root.

ARD:           aortic root dilatation.

cf:               ‘compare with’, derived from the Latin word for ‘to compare’.

Mas:           Marfan’s syndrome.

TGF-β:        transforming growth factor beta.

Scenario 1.              

Which, if any, of the following statements are true in relation to Marfan syndrome?

Option list.

A	Mas is a connective tissue disorder
B	Mas is an autoimmune condition
C	Mas is due to defects in fibrillin-1
D	Mas is due to mutation of the FUN1 gene
E	only one variant of the gene causes Mas
F	the unique variant of the gene that causes Mas explains the uniform phenotype
G	inheritance is autosomal dominant
H	inheritance is autosomal recessive
I	inheritance is X-linked recessive

Scenario 2.              

Which, if any, of the following is the incidence of Mas?

Option list.

A	~ 1 in 500
B	~ 1 in 5,000
C	~ 1 in 50,000
D	~ 1 in 500,000

Scenario 3.              

What % of cases arise from new mutations?

Option list.

A	5%
B	15%
C	20%
D	25%
E	30%
F	>30%

Scenario 4.              

How many mutations of the Marfan gene have been identified?

Option list.

A	< 100
B	101-200
C	201-300
D	301-400
E	401-500
F	>500
G	>1,000
H	>2,000

Scenario 5.              

Which of the following are features of classical Mas?

Option list.

A	arachnodactyly
B	brachydactyly
C	cauda equina syndrome
D	ectopia lentil
E	frequenting of lax joints
F	kyphosis
G	long long bones
H	scoliosis
I	tall stature

Scenario 6.              

Which, if any, of the following are features of the classical Mas?

Option list.

A	aortic coarctation
B	aortic dissection
C	aortic regurgitation
D	aortic root dilatation
E	aortic stenosis
F	mitral regurgitation
G	mitral stenosis
H	pulmonary hypertension
I	tricuspid regurgitation
J	tricuspid stenosis

Scenario 7.              

Which, if any, of the following are features of the classical Mas?

Option list.

A	cataract
B	chronic obstructive airways disease
C	glaucoma
D	myopia
E	pulmonary bullous changes
F	pulmonary fibrosis
G	recurrent pneumothorax
H	stretch marks
I	striae distensae
J	melanoma

Scenario 8.              

Which, if any, of the following are features of the classical Mas?

Option list.

A	cauda equina syndrome
B	dural ecstasy
C	dural ectasia
D	dural ectoplasm
E	hydrocephalus

Scenario 9.              

Approximately what percentage of those with Mas have cardiac involvement?

Option list.

A	< 30
B	40
C	50
D	60
E	70
F	80
G	≥ 90

Scenario 10.           

What is the generally accepted cut-off for aortic replacement in the non-pregnant?

Option list.

A	AR > 3 cm.
B	AR > 4 cm.
C	AR > 5 cm.
D	AR > 7.5 cm.
E	AR > 10 cm.
F	none of the above

Scenario 11.           

What is the generally accepted cut-off for high risk associated with pregnancy?

Option list.

A	AR > 3 cm.
B	AR > 4 cm.
C	AR > 5 cm.
D	AR > 7.5 cm.
E	AR > 10 cm.
F	none of the above

Scenario 12.           

Which, if any, of the following statements are true in relation to the maternal risks associated with an AR greater than the high-risk cut-off?

Option list.

A	worsening aortic dilatation
B	aortic dissection more likely
C	aortic stenosis
D	cervical incompetence more likely
E	ectropion lentis more common
F	gestational hypertension more common
G	HELLP syndrome more common
H	hydramnios more common
I	pulmonary embolism more common

Scenario 13.           

Which, if any, of the following statements are true in relation to the fetal risks associated with maternal Mas?

Option list.

A	↑ risk of breech presentation at term
B	↑ risk of neural tube defect
C	↑ risk of perinatal mortality
D	↑ risk of preterm birth
E	25% risk of Mas
F	none of the above

 

TOG CPD. 2007. 19. 1. These are open access, so are reproduced here.

Pregnancy is associated with

1.     increased pulmonary blood flow.                                                                 True / False

2.     decreased stroke volume.                                                                              True / False

 3.    increased heart rate.                                                                                      True / False

Indication for elective caesarean section includes

4.     aortopathy with aortic root > 4 cm.                                                              True / False

5.     aortic dissection or aneurysm.                                                                      True / False

In women with congenital heart disease predictors for adverse neonatal events include

6.     prior maternal cardiac event.                                                                        True / False

7.     presence of left-to-right shunts.                                                                   True / False

Regarding Marfan syndrome and pregnancy,

8.     the risk of aortic dissection or rupture is reduced.                                     True / False

9.     overall maternal mortality is about 1 in 4.                                                   True / False

10.   aortic root diameter should be monitored throughout pregnancy.                            True / False

The offspring of a mother with congenital heart disease

11.   are at increased risk of inheriting congenital heart disease.                                  True / False

12.   are at increased risk of complications during the neonatal period.                     True / False

Tetralogy of Fallot is

13.   the most common form of cyanotic heart disease.                                   True / False

14.   often associated with pulmonary regurgitation.                                        True / False

In women with congenital heart disease

15.   ventouse delivery under epidural anaesthesia is generally recommended.       True / False

16.   cardiac arrhythmia is one of the main primary cardiac events in pregnancy. True / False

In pregnant women with coarctation of the aorta,

17.   blood pressure must be carefully controlled to avoid low birthweight.            True / False

In women with transposition of the great arteries

18.   even if this has been repaired, arrhythmia can occur in pregnancy.                            True / False

In women with cyanotic heart disease without pulmonary hypertension

19    up to 1 in 2 pregnancies end in premature delivery.                                   True / False

In women with pulmonary vascular disease

20    about a third of pregnancies will result in intrauterine growth restriction. True / False

 

8.     SBA. Androgen insensitivity syndrome.

Abbreviations.

AIS:             androgen insensitivity syndrome.

Question 1.

Lead-in

What is the estimated prevalence of AIS?

Option List

A	2-5 per 100,000 boys at birth
B	5-10 per 100,000 girls at birth
C	2-5 per 100,000 genetic males at birth
D	5-10 per 100,000 genetic females at birth
E	none of the above.

Question 2.

Lead-in

Which of the following sub-types of AIS do not exist?

Sub-types

A	complete AIS
B	incomplete AIS
C	mild AIS
D	partial AIS
E	total AIS

Option List

A	1
B	2
C	3
D	4
E	5
F	1 + 3
G	2 + 3
H	2 + 5
I	3 + 5
J	4 + 5

Question 3.

Lead-in

How common is partial AIS?

Option List

A	at least as common as complete AIS
B	at least as common as total AIS
C	less common than mild AIS
D	as common as incomplete AIS
E	none of the above.

Question 4.

Lead-in

How common is incomplete AIS?

Option List

A	at least as common as complete AIS
B	at least as common as total AIS
C	less common than mild AIS
D	as common as partial AIS
E	none of the above.

Question 5.

Lead-in

How common is mild AIS?

Option List

A	at least as common as complete AIS
B	at least as common as total AIS
C	less common than complete AIS
D	as common as partial AIS
E	none of the above.

Question 6.

Lead-in

No more prevalence!!

What is the mode of inheritance of AIS?

Option List

A	autosomal dominant
B	autosomal recessive
C	X-linked dominant
D	X-linked recessive
E	mitochondrial

Question 7.

Lead-in

What proportion of AIS is due to new mutations?

Option List

A	0%
B	1 – 20%
C	21 – 40%
D	41-60%
E	61-80%

Question 8.

Lead-in

Which gene is involved in AIS?

Option List

A	androgen receptor gene
B	aromatase receptor gene
C	androstenedione gene
D	oestrogen receptor gene
E	none of the above

Question 9.

Lead-in

How many mutations have been described of the gene which is involved in AIS?

Option List

A	0-10
B	11-100
C	101-200
D	201-300
E	>300

Question 10.

Lead-in

Which is the most common clinical presentation in AIS?

Option List

A	ambiguous genitalia
B	precocious puberty
C	premature menopause
D	primary amenorrhoea
E	secondary amenorrhoea

Question 11.

Lead-in

Which of the following are more common in AIS?

Option List

A	anlagen
B	coarctation of the aorta
C	“coast of Maine” pigmentation pattern
D	renal tract anomalies
E	none of the above.

Question 12.

Lead-in

A woman of 20 is found to have AIS. She has a pre-pubertal sister. What is the chance that the sister also has AIS, assuming that the condition is not due to a new mutation in the elder sister?

Option List

A	1 in 1
B	1 in 2
C	1 in 3
D	1 in 4
E	1 in 16

Question 13.

Lead-in

What is the risk of the gonads becoming malignant in AIS?

Option List

A	10%
B	20%
C	30%
D	> 30%
E	accurate risk not known

Question 14.

Lead-in

Which, if any, of the following are true in relation to the androgen receptor gene (ARG)?

Option List

A	achondroplasia is linked to mutations of the ARG
B	alopecia areata is linked to mutations of the ARG
C	benign prostatic hyperplasia is linked to mutations of the ARG
D	diabetes is linked to mutations of the ARG
E	spinal bulbar muscular atrophy is linked to mutations of the ARG
F	none of the above is linked to mutations of the ARG

Question 15.

Lead-in

Which, if any, of the following are androgen receptor antagonists?

Option List

A	aspirin
B	cannabis
C	cimetidine
D	cyproterone acetate
E	flutamide
F	spironolactone