Tutorial 6th June 2019

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17	EMQ. Mayer-Rokitansky-Küster-Hauser syndrome
18	SBA. Quinolone antibiotics
19	EMQ. Parvovirus
20	EMQ. Galactosaemia
21	EMQ. Education
22	Viva. Obstructive sleep apnoea

17. EMQ. Mayer-Rokitansky-Küster-Hauser syndrome.

Abbreviations.

AIS:          androgen insensitivity syndrome

AMH:      anti- Müllerian hormone

MRKH:   Mayer-Rokitansky-Küster-Hauser syndrome

MURCS: Müllerian duct aplasia, renal dysplasia and cervical somite anomaly syndrome.

Question 1.           

Lead-in.

What are the main features of MRKH? There is no option list to make life harder.

Question 2.           

Lead-in.

Which, if any, are the main secondary features associated with MRKH?

Option list.

A	anosmia
B	attention-deficit-hyperactivity syndrome
C	auditory anomalies
D	neural tube defects
E	renal anomalies
F	skeletal anomalies

Question 3.           

Lead-in.

How does MRKH syndrome usually present?

Option list.

A	cyclical pain due to haematometra
B	delayed puberty
C	precocious puberty
D	premature menopause
E	primary amenorrhoea
F	recurrent otitis media
G	recurrent urinary tract infection
H	secondary amenorrhoea

Question 4.           

Lead-in.

Which of the following chromosome patterns are typical of MRKH?

Option list.

A	45XO
B	45YO
C	46XX
D	46XY
E	47XXX
F	47XXY

Question 5.           

Lead-in.

What is the approximate incidence of MRKH in newborn girls?

Option list.

A	~ 1 in 1,000
B	~ 1 in 2,000
C	~ 1 in 4,000
D	~ 1 in 6.000
E	~ 1 in 8,000
F	~ 1 in 10,000
G	~ 1 in 100,000
H	the figure is unknown
I	it does not occur

Question 6.           

Lead-in.

What is the approximate incidence of MRKH in newborn boys?

Option list.

A	~ 1 in 1,000
B	~ 1 in 2,000
C	~ 1 in 4,000
D	~ 1 in 6.000
E	~ 1 in 8,000
F	~ 1 in 10,000
G	~ 1 in 100,000
H	the figure is unknown
I	it does not occur

Question 7.           

Lead-in.

Which of the following statements are correct in relation to urinary tract anomalies associated with MRKH?

Option list.

A	absent bladder
B	absent kidney
C	ectopic ureter
D	horseface kidney
E	hypospadias
F	urinary tract anomalies are not part of the syndrome

Question 8.           

Lead-in.

Which of the following statements are correct in relation to skeletal anomalies associated with MRKH?

Option list.

A	absent thumb
B	absent big toe
C	developmental dysplasia of the hip
D	Klippel-Feil anomaly
E	ulnar hypoplasia
F	vertebral fusion
G	skeletal anomalies are not part of the syndrome

Question 9.           

Lead-in.

Which of the following statements are correct in relation to auditory anomalies associated with MRKH?

Option list.

A	absent ear
B	absent stapes
C	acoustic neuroma
D	conductive deafness
E	inductive deafness
F	stapedial ankylosis
G	auditory anomalies are not part of the syndrome

Question 10.       

Lead-in.

What is the recommended first-line management for creation of a neovagina.

Option list.

A	digital dilatation
B	marriage to a virile husband
C	vaginal balloons
D	vaginal dilators
E	vaginoplasty
F	there is no recommended 1st. line management

Question 11.       

Lead-in.

What is what are the key features of Davydov vaginoplasty?

Option list.

A	horseshoe perineal incision with labial flaps used to create a pouch
B	creation of space between bladder and rectum and lining it with amnion
C	creation of space between bladder and rectum and lining it with skin graft
D	creation of space between bladder and rectum and lining it with sigmoid colon
E	creation of space between bladder and rectum and lining it with peritoneum
F	traction via threads running to the abdomen from a vaginal bead

Question 12.       

Lead-in.

What is what are the key features of McIndoe vaginoplasty?

Option list.

A	horseshoe perineal incision with labial flaps used to create a pouch
B	creation of space between bladder and rectum and lining it with amnion
C	creation of space between bladder and rectum and lining it with skin graft
D	creation of space between bladder and rectum and lining it with sigmoid colon
E	creation of space between bladder and rectum and lining it with peritoneum
F	traction via threads running to the abdomen from a vaginal bead

Question 13.       

Lead-in.

What is what are the key features of Vecchietti vaginoplasty?

Option list.

A	horseshoe perineal incision with labial flaps used to create a pouch
B	creation of space between bladder and rectum and lining it with amnion
C	creation of space between bladder and rectum and lining it with skin graft
D	creation of space between bladder and rectum and lining it with sigmoid colon
E	creation of space between bladder and rectum and lining it with peritoneum
F	traction via threads running to the abdomen from a vaginal bead

Question 14.       

Lead-in.

What is what are the key features of Williams vaginoplasty?

Option list.

A	horseshoe perineal incision with labial flaps used to create a pouch
B	creation of space between bladder and rectum and lining it with amnion
C	creation of space between bladder and rectum and lining it with skin graft
D	creation of space between bladder and rectum and lining it with sigmoid colon
E	creation of space between bladder and rectum and lining it with peritoneum
F	traction via threads running to the abdomen from a vaginal bead

TOG CPD questions.  Answer as true or false

With regard to the MRKH syndrome.

1.     there is failure of development of the mesonephric ducts.

2.     the phenotype and genotype are female.

3.     studies have established a link between the syndrome and the use of diethylstilboestrol in pregnancy.

4.     symmetrical uterovaginal aplasia is found in type I disorders.

5.     renal abnormalities are seen in more than half of cases.

6.     skeletal abnormalities are reported in up to one-fifth of cases.

7.     up to one-quarter of women have a malformed ear or auditory canal.

8.     the close proximity of the Müllerian and Wolffian duct derivatives to the duct in the developing embryo explains the higher association of malformations of the kidneys with this condition.

9.     vaginal agenesis is caused by failure of the caudal part of the Müllerian duct system to develop.

10. magnetic resonance imaging is the gold standard tool.

11. two-dimensional ultrasound scanning is not useful for associated renal tract abnormalities.

12. complete androgen insensitivity syndrome is an important differential diagnosis.

13. the presence of cyclical abdominal pain will rule out the diagnosis, as it indicates the presence of functioning endometrium.

With regard to the creation of a neovagina,

14. it is recommended that treatment is initiated as soon as the diagnosis is made.

15. psychological support to women undergoing this procedure is of the utmost importance.

16. vaginal dilators are acceptable as an option for first-line therapy.

17. Ingram’s modified Frank’s technique involves the use of vaginal dilators.

With regard to the surgical creation of a neovagina,

18. in the Davydov procedure the neovagina is lined with peritoneum.

With regard to fertility in women with the MRKH syndrome,

19. transvaginal egg retrieval is recognised to be difficult during in vitro fertilisation.

20. the condition has been shown to be transmissible to the offspring.

18. SBA. Quinolone antibiotics.

Not all of the questions are true SBAs as some have more than one answer – this reduces the amount of typing I have to do and the size of the document.

Abbreviations.

FQ:             fluoroquinolone.

QUI:           quinolone.

Question  1.         

Lead-in

Which, if any, of the following drugs are QUIs or FQs? 

Drugs

A.	cimetidine
B.	ciprofloxacin
C.	nalidixic acid
D.	neomycin
E.	nitrofurantoin

Question  2.         

Lead-in

Which, if any, of the following statements are true in relation to QUIs & FQs? This is not a true SBA as there may be more than one answer.

Statements

A.	nalidixic acid is an older quinolone and is mainly excreted in the urine
B.	ciprofloxacin is effective against most Gram +ve and –ve bacteria and 1st- line treatment for pneumococcal pneumonia.
C.	ciprofloxacin is contraindicated in pregnancy due to the ↑ risk of neonatal haemolysis
D.	many staphylococci are resistant to quinolones
E.	quinolones are particularly useful in the treatment of MRSA

Question  3.         

Lead-in

Which was the first QUI antibiotic?

Option List

A	acetylsalicylic acid
B	nalidixic acid
C	oxalic acid
D	pipemidic acid
E	none of the above

Question  4.         

Lead-in

How do QUI and FQ antibiotics work? There is only one correct answer.

Option List

A	impair bacterial DNA coiling
B	impair bacterial DNA binding
C	impair bacterial RNA action
D	impair bacterial mitochondrial action
E	none of the above.

Question  5.         

Lead-in

Which, if any, of the following QUIs & FQs is not available for prescription in the UK. There is only one correct answer.

Option List

A	ciprofloxacin
B	levofloxacin
C	nalidixic acid
D	moxifloxacin
E	ofloxacin

Question  6.         

Lead-in

Which, if any, of the following statements are true in relation to the quinolones and fluoroquinolones and pregnancy? This is not a true SBA as there may be more than one answer.

Option list.

A.	FQs are newer than QUIs with better systemic spread and efficacy
B.	QUIs concentrate in urine but have a special affinity for cartilage
C.	consumption of a FQ in the 1st. trimester is grounds for TOP
D.	if an FQ is used, norfloxacin and ciprofloxacin should be considered 1st.
E.	FQs are linked to a risk of discolouration of the teeth of offspring

Question  7.         

Lead-in

Which of the following is true about the warning issued by the FDA in 2008 in relation to QUIs & FQs?

Option List

A	they may cause congenital cartilage defects
B	they may cause congenital deafness
C	they may cause tendonitis and tendon rupture
D	they may cause prolongation of the Q-T interval
E	none of the above

Question  8.         

Lead-in

Which of the following is true about the warning issued by the FDA in 2011 in relation to QUIs & FQs?

Option List

A	they may cause exacerbation of eczema
B	they may cause exacerbation of hypertension
C	they may cause exacerbation of multiple sclerosis
D	they may cause exacerbation of myasthenia gravis
E	they may cause exacerbation of SLE

Question  9.         

Lead-in

Which of the following is true about the warning emphasised by the FDA in 2013 in relation to QUIs & FQs?

Option List

A	they may cause aortic dissection
B	they may cause mitral stenosis
C	they may cause pancreatitis
D	they may cause peripheral neuropathy
E	they may cause flare of SLE

Question  10.     

Lead-in

FDA issued a warning in July 2016. Which, if any, of the following were included? This is not a true SBA as there may be more than one answer.

Option List

A	the risks generally outweigh the benefits
B	QUIs & FQs should not be used for acute sinusitis,
C	QUIs & FQs should not be used for exacerbation of chronic bronchitis
D	QUIs & FQs should not be used for uncomplicated UTI
E	QUIs & FQs may be useful for anthrax and plague

Question  11.     

Lead-in

FDA issued a warning in July 2018 about the use of FQs in pregnancy. Which, if any, of the following were included in the reasons for its publication?

Option List

A	to strengthen previous warnings about hyperglycaemia and mental health risks
B	to strengthen previous warnings about hypoglycaemia and mental health risks
C	to strengthen previous warnings about the risk of ASD in the offspring
D	to strengthen previous warnings about the risk of acute pancreatitis
E	to strengthen previous warnings about the risk of PET

Question  12.     

Lead-in

The FDA issued a warning in December 2018 about the use of FQs in pregnancy. Which, if any, of the following was included? This is an SBA with only one correct answer.

Option List

A	↑ risk of atrial fibrillation
B	↑ risk of aortic aneurysm and rupture
C	↑ risk of mitral stenosis
D	↑ risk of pulmonary hypertension
E	↑ risk of ulcerative colitis

19. EMQ. Parvovirus.

Option list.

There is none: make up your own answers!

Scenario 1.             

What type of virus is parvovirus?

Scenario 2.             

Is the title B19 something to do with the American B19 bomber, its potentially devastating bomb load and the comparably devastating consequences of the parvovirus on human erythroid cell precursors?

Scenario 3.             

PVB19 in the UK occurs in mini-epidemics at 3 – 4 year intervals, usually during the summer months.

Scenario 4.             

Which animal acts as the main reservoir for infection?

Scenario 5.             

What is the approximate incidence of maternal parvovirus infection in the UK?

Scenario 6.             

What percentage of UK adults are immune to parvovirus infection?

Scenario 7.             

What names are given to acute infection in the human?

Scenario 8.             

What is the incubation period for parvovirus infection?

Answer: 14-21 days according to GOVRIP.

Scenario 9.             

What is the duration of infectivity for parvovirus infection?

Scenario 10.         

What are the usual symptoms of parvovirus infection in the adult?

Scenario 11.         

What is the incidence of parvovirus infection in pregnancy?

Scenario 12.         

How is recent infection diagnosed?

Scenario 13.         

How long does PvIgM persist and why is this important?

Scenario 14.         

What is the rate of vertical transmission of parvovirus infection?

Scenario 15.         

Are women with parvovirus infection who are asymptomatic less likely to pass the virus to their fetuses?

Scenario 16.         

To what degree is parvovirus infection teratogenic?

Scenario 17.         

What proportion of pregnancies infected with parvovirus are lost?

Scenario 18.         

What is the timescale for the onset of hydrops?

Scenario 19.         

Laboratories are advised to retain bloods obtained at booking for at least 2 years for possible future reference. True or false?

Scenario 20.         

What ultrasound features would trigger consideration of cordocentesis?

Scenario 21.         

Must suspected parvovirus infection be notified to the authorities?

Scenario 22.         

Possible parvovirus infection does not need to be investigated after 20 week’s gestation.

Scenario 23.         

If serum is sent to the laboratory from a woman with a rash in pregnancy for screening for rubella, the laboratory should automatically test for parvovirus infection too.

20. EMQ. Galactosaemia.

Abbreviations.

GA:             galactose

GAA:          galactosaemia

Scenario 1.             

What is galactosemia? There is no option list.

Scenario 2.             

What is the mode of inheritance? There is no option list.

Scenario 3.             

Which of the following is the most common cause of galactosemia in Caucasians?

Option list.

A	mutation of the GALE gene
B	mutation of the GALF gene
C	mutation of the GALK gene
D	mutation of the GALk1 gene
E	mutation of the GALT gene

Scenario 4.             

What is the mutation which causes Classical Galactosaemia?

Option list.

A	Q188L
B	Q188M
C	Q188R
D	R188L
E	R188M
F	R188R
G	None of the above

Scenario 5.             

What is the Duarte mutation? There is no option list.

Scenario 6.             

What are the main sources of galactose? There is no option list.

Scenario 7.             

What is the approximate prevalence of galactosemia? There is no option list.

Scenario 8.             

Which of the following groups has the highest prevalence of galactosaemia?

Option list.

A	Armenians
B	Ashkenazi Jews
C	French absinthe drinkers
D	Irish campers
E	Irish travellers
F	Masai
G	Scottish campers
H	None of the above

Scenario 9.             

Which is the most common mutation in the group with the highest incidence of galactosemia? There is no option list.

Scenario 10.         

Which, if any, of the following are linked to untreated GAA in the newborn?

Option list.

A	­ risk of coagulation problems
B	­ risk of congenital hypothyroidism
C	­ risk of diabetes
D	­ risk of diarrhoea
E	­ risk of failure to thrive
F	­ risk of liver failure
G	­ risk of renal failure
H	­ risk of staphylococcal infection

Scenario 11.         

What are the main problems associated with non-treatment of galactosaemia in adults? There is no option list.

Scenario 12.         

Which, if any, of the following statements are true in relation to the effects of a galactose-reduced diet (GRD) on long-term complications (LTCs)?

Option list.

A	a GRD has a major protective effect on LTCs, but only if started within 2 weeks of birth
B	a GRD has a major protective effect on LTCs, but only if started within 12 weeks of birth
C	a GRD has a major protective effect on LTCs, but only if followed meticulously
D	a GRD has a major protective effect on LTCs, but only if started within 2 weeks of birth and continued for life
E	a GRD has a major protective effect on LTCs, but only if started within 2 weeks of birth and continued for life
F	none of the above

Scenario 13.         

Is screening for galactosaemia included in the UK neonatal screening programme? If not, why not?

21. EMQ. Education.

Option list.

1. brainstorming.
2. brainwashing
3. cream cake circle.
4. Delphi technique.
5. demonstration & practice using clinical model.
6. doughnut round.
7. interactive lecture with EMQs.
8. lecture.
9. 1 minute preceptor method.
10. teaching peers / junior colleagues
11. schema activation.
12. schema refinement.
13. small group discussion.
14. snowballing.
15. snowboarding.
16. true
17. false

Scenario 1.

A woman is admitted with an eclamptic seizure. The acute episode is dealt with and she is put on an appropriate protocol. You wish to use the case to outline key aspects of PET and eclampsia to the two medical students who are on the labour ward with you. Which would be the most appropriate approach?

Scenario 2.

You have been asked to provide a summary of the key aspects of the recent Maternal Mortality Meeting to the annual GP refresher course. There are likely to be 100 attendees. Which would be the most appropriate approach?

Scenario 3.

You have been asked to teach a new trainee the use of the ventouse. Which would be the most appropriate approach?

Scenario 4.

You have been asked to teach a group of medical students about PPH. To your surprise you find that they have good basic knowledge. Which technique will you apply to get the most from the teaching session?

Scenario 5.

Your consultant has asked you to get the unit’s medical students to prepare some questions about breech delivery which they can ask of their peers when they next meet. Which technique will you use?

Scenario 6.

You have been asked to discuss 2ry. amenorrhoea with your unit’s medical students. You are uncertain about the amount of basic physiology and endocrinology they remember from basic science teaching. Which technique will you use?

Scenario 7

The RCOG has asked you to chair a Green-top Guideline development committee. You find that there is very little by way of research evidence to help with the process. The College has assembled a team of consultants with expertise and interest in the subject. Which technique would be best to reach consensus on the various elements of the GTG?

Scenario 8

Which of the listed teaching techniques is least likely to lead to deep learning?

Scenario 9

An interactive lecture with EMQs is the best method of teaching. True or false.

Scenario 10

Only 20% of what is taught in a lecture is retained. True or false.

Scenario 11.

The main role of the teacher is information provision. True or false.

Scenario 12.

The main role of the teacher is to be a role model.  True or false.

22. Viva. Obstructive sleep apnoea.

The examiner will ask you a series of questions.