Tutorial 13 May 2021

 

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60.	Role-play. Androgen insensitivity syndrome
61.	Structured conversation. Cochrane Collaboration
62.	EMQ. Gestational trophoblastic disease
63.	EMQ. Galactosaemia

 

60.         Role-play. Androgen insensitivity syndrome.

Candidate's Instructions.

The patient is Anastasia Johnstone. She is 17 years old. She attended the gynaecology clinic 1 month ago with primary amenorrhoea. Clinical examination showed an apparently normal young woman with normal breast development but absent pubic and axillary hair. The external genitalia appeared normal. Vaginal examination was not attempted.

She has come today for the results of an ultrasound scan and blood tests. The scan shows absence of the uterus. There are no ovaries in the pelvis. There are bilateral groin masses. The karyotype is 46XY.

Your tasks are to explain the results and their implications and to answer her questions.

 

61.         Structured conversation. Cochrane

Candidate’s instructions.

This is a viva about the Cochrane.

The examiner will ask 10 questions.

The discussion will refer to the chart below.

 

62.         EMQ. Gestational Trophoblastic Disease (GTD)

Abbreviations.

GTD:             gestational trophoblastic disease.

GTN:             Gestational trophoblastic neoplasia.

HM:               hydatidiform mole.

POC:              products of conception.

PSTT:            placental site trophoblastic tumour.

TTC:              trophoblastic tumour centre

Option list.

A.	100%.
B.	20%.
C.	15%.
D.	10%.
E.	5%.
F.	2.5%.
G.	1.5%.
H.	0.5%.
I.	1 in 35.
J.	1 in 55.
K.	1 in 65.
L.	1 in 700.
M.	1 in 1,000.
N.	Ö64.
O.	pr2.
P.	increased.
Q.	reduced.
R.	increased by a factor of 2.
S.	increased by a factor of 5.
T.	increased by a factor of 10.
U.	increased by a factor of 20.
V.	increased by a factor of 30.
W.	increased by a factor of > 100.
X.	hydatidiform mole, both partial and complete.
Y.	hydatidiform mole, both partial and complete and placental site tumour.
Z.	partial mole, complete mole, invasive and metastatic mole, choriocarcinoma, placental site trophoblastic tumour and epithelioid trophoblastic tumour.
AA.	choriocarcinoma invasive and metastatic mole and epithelioid trophoblastic tumour.
BB.	true
CC.	false
DD.	None of the above.

 

Scenario 1.              

List the conditions included in the term GTD. There is no option list, just make a list.

Scenario 2.            

What is the difference between GTD and GTN? Pick one option from the list below.

Option list.

A	GTD comprises the non-malignant conditions, i.e. complete and partial moles. GTN comprises the malignant conditions: invasive mole, choriocarcinoma and PSTT
B	GTD comprises all the trophoblastic conditions; GTN comprises the malignant conditions
C	GTD comprises all the trophoblastic conditions; GTN comprises persistent GTD
D	GTD comprises all the trophoblastic conditions; GTN comprises malignant and potentially malignant conditions, including atypical placental site nodules
E	none of the above

Scenario 3.            

What is the incidence of GTD in the UK?

Scenario 4.            

Which of the following statements, if any, are true of complete hydatidiform molar pregnancy?

A	are usually diploid and with all the chromosomal material of paternal origin
B	are usually triploid, with 2 sets of paternal haploid genes + 1 set of maternal haploid genes
C	are usually triploid, with 1 set of paternal haploid genes + 2 sets of maternal haploid genes
D	are tetraploid or mosaics in up to 10% of cases
E	up to 80% are due to duplication of a single sperm in an egg devoid of maternal chromosomes
F	up to 80% are due to duplication of a single sperm in a normal egg
G	usually result from dispermic fertilisation of a normal egg
H	usually result from dispermic fertilisation of an egg devoid of maternal chromosomes
I	usually has 46XX makeup
J	usually has 46XY makeup
K	the presence of fetal red blood cells defines a mole as partial
L	mitochondrial DNA is maternal
M	mitochondrial DNA is paternal

Scenario 5.            

Which of the following statements, if any, are true of partial hydatidiform molar pregnancy?

Option list.

A	are usually diploid and with paternal chromosomal material
B	are usually triploid, with 2 sets of paternal haploid genes + 1 set of maternal haploid genes
C	are usually triploid, with 1 set of paternal haploid genes + 2 sets of maternal haploid genes
D	are tetraploid or mosaics in up to 10% of cases
E	up to 80% are due to duplication of a single sperm in an egg devoid of maternal chromosomes
F	up to 80% are due to duplication of a single sperm in a normal egg
G	usually result from dispermic fertilisation of a normal egg
H	usually result from dispermic fertilisation of an egg devoid of maternal chromosomes
I	usually has 46XX makeup
J	usually has 46XY makeup
K	the presence of fetal red blood cells defines a mole as partial
L	mitochondrial DNA is maternal
M	mitochondrial DNA is paternal

Scenario 6.            

What is the ratio of complete: partial moles?

Scenario 7.            

What is the risk of molar pregnancy at age < 15 compared to age 30?

Scenario 8.            

What is the risk of molar pregnancy at age > 45 compared to age 30?

Scenario 9.            

What is the risk of molar pregnancy in a subsequent pregnancy after a complete mole?

Option list.

A	< 1%
B	1-2%
C	3-5%
D	6-10%
E	11-20%
F	> 20%

Scenario 10.        

What is the risk of molar pregnancy in a subsequent pregnancy after a partial mole?

Use the option list from the previous question.

Scenario 11.        

Which, if any, of the following are more common in pregnancy after a molar pregnancy? This is not a true EMQ as there may be > 1 correct answer.

Option list.

A	anaemia
B	eclampsia / severe PET
C	intrauterine growth retardation
D	miscarriage
E	pulmonary embolism
F	PPH
G	none of the above

Scenario 12.        

What is the risk of molar pregnancy in a subsequent pregnancy for the woman who has had two molar pregnancies?

Scenario 13.        

Which, if any, of the following statements about hCG are true?

A	is a glycoprotein
B	shares its α sub-unit with FSH, LH & TSH
C	shares its α sub-unit with FSH & LH but not TSH
D	shares its β sub-unit with FSH, LH & TSH
E	shares its β sub-unit with FSH & LH but not TSH
F	β-core exists as a sub-type of β-hCG
G	nicked free-β exists as a sub-type of β-hCG
H	c-terminal peptide exists as a sub-type of β-hCG
I	hCG β core fragment may lead to false –ve results with urine pregnancy tests
J	heterophile antibodies may give false +ve hCG results
K	heterophile antibodies are not found in urine

Scenario 14.        

Which, if any, of the following statements are true in relation to the diagnosis of molar pregnancy?

A	definite diagnosis is usually made by ultrasound
B	definitive diagnosis requires a +ve test for P57KIP2
C	definitive diagnosis requires an hCG level > twice the median value for gestation
D	definite diagnosis requires histological examination
E	none of the above

Scenario 15.           

Cystic placental spaces in the placenta and a ratio of transverse to anterioposterior

measurements of the gestation sac <1.5 are strongly suggestive of a partial mole. True / False.

Scenario 16.        

Which, if any, of the following statements are true about preparation of the cervix before evacuation of molar pregnancy?

A	medical preparation is of proven efficacy in making suction evacuation easier
B	medical preparation with prostaglandins ↑ trophoblastic embolisation
C	medical preparation with prostaglandins ↑ the risk of needing chemotherapy
D	GTG 38 recommends the use of laminaria tents
E	none of the above

Scenario 17.        

Which, if any, of the following statements are true about evacuation of molar pregnancies?

A	medical management is recommended for both CMs and PMs to ↓ the risk of bleeding
B	medical management is recommended for both CMs and PMs to ↓ the risk of dissemination of trophoblastic tissue
C	medical management is recommended for both CMs and PMs to ↓ the risk of uterine perforation
D	suction evacuation is recommended for both CMs and PMs
E	suction evacuation is recommended for CMs
F	suction evacuation is recommended for PMs so long as fetal parts are not too big
G	mifepristone + misoprostol treatment is an acceptable alternative to suction evacuation.
H	oxytocin administration after suction evacuation is recommended to ↓ bleeding
I	none of the above

Scenario 18.           

Which, if any, of the following statements are true about urinary hCG testing in relation to molar pregnancy?

A	testing should be done 3 weeks after medical evacuation of complete moles
B	testing should be done 3 weeks after surgical evacuation of complete moles
C	testing should be done 3 weeks after medical evacuation of partial moles
D	testing should be done 3 weeks after surgical evacuation of complete moles
E	testing should be done 3 weeks after medical evacuation of ‘failed’ pregnancy
F	testing should be done 3 weeks after surgical evacuation of ‘failed’ pregnancy
G	testing should be done 3 weeks after medical evacuation of ‘failed’ pregnancy, but only if POC have not been sent for histological examination
H	testing should be done 3 weeks after surgical evacuation of ‘failed’ pregnancy, but only if POC have not been sent for histological examination
I	testing should be done 3 weeks after medical evacuation of incomplete miscarriage
J	testing should be done 3 weeks after surgical evacuation of incomplete miscarriage
K	testing should be done 3 weeks after medical evacuation of incomplete miscarriage, but only if POC have not been sent for histological examination
L	testing should be done 3 weeks after surgical evacuation of incomplete miscarriage, but only if POC have not been sent for histological examination
M	none of the above

Scenario 19.           

Which, if any, of the following statements are true in relation to histological examination of POC after TOP?

A	it should be done in all cases to exclude GTD
B	it should be done in all cases that have not had pre-op ultrasound examination in case the pregnancy was an unsuspected ectopic. Absence of trophoblastic tissue on histology will raise suspicion of the diagnosis
C	it should be done in all cases where ultrasound has not shown a viable pregnancy
D	it should be done in all cases where ultrasound has not shown fetal parts.
E	none of the above

Scenario 20.           

Which, if any, of the following statements are true in relation to RhD and TOP?

A	CMs have no RhD
B	PMs have no RhD
C	Anti-D should be withheld until histological results are available
D	‘C’ is true, but only in relation to CMs
E	‘C’ is true, but only in relation to PMs
F	none of the above

Scenario 21.        

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

A	always arises from molar pregnancy
B	may occur after normal pregnancy and livebirth
C	may arise as primary ovarian neoplasia
D	the incidence after complete molar pregnancy is greater than after partial molar pregnancy
E	the incidence after livebirth is estimated at 1 in 50,000

Scenario 22.        

Which, if any, of the following statements are true in relation to p57^KIP2?

A	it is a tumour suppressor gene, found in complete and partial moles but not choriocarcinoma
B	takes us to the world of genomic imprinting
C	is an example of uniparental disomy
D	is a gene found in chromosomes of maternal origin, but not paternal
E	is a gene found in chromosomes of paternal origin, but not maternal
F.	can help to distinguish complete and partial moles
G.	none of the above

Scenario 23.        

What is the risk of persistent GTD after a complete mole?

Scenario 24.           

What is the risk of requiring chemotherapy after a complete mole?

Scenario 25.           

What is the risk of persistent GTD after a partial mole?

Scenario 26.        

What is the risk of requiring chemotherapy after a partial mole?

Scenario 27.        

What is the risk of requiring chemotherapy with hCG level > 20,000 i.u. 4⁺¹ weeks after evacuation?

Scenario 28.        

What is the overall risk of requiring chemotherapy after molar pregnancy in the UK?

Scenario 29.        

What is the risk of requiring chemotherapy in the USA compared with the UK?

Scenario 30.        

Which, if any, of the following are grounds for offering chemotherapy after hydatidiform mole?

Scenario 31.        

What are the risk factors included in the FIGO scoring system?

Scenario 32.        

Which, if any, of the following statements is true about the recommended treatment of low-risk GTN?

A	risk should be assessed using the FIGO scoring system
B	risk should be assessed using the RCOG scoring system
C	methotrexate alone is recommended
D	methotrexate combined with folinic acid is recommended
E	methotrexate alternating with folinic acid is recommended
F.	methotrexate combined with etoposide acid is recommended
G	methotrexate combined with dactinomycin is recommended

Scenario 33.        

Which, if any, of the following is the most common side-effect of methotrexate?

A	alopecia
B	anaemia
C	aphasia
D	nausea
E	myelosuppression
F.	none of the above.

Scenario 34.        

Which, if any, of the following statements are true about the recommended duration of follow-up after GTD? This is not a true EMQ as there may be > 1 correct answer.

A	6 months from the time the hCG falls to normal
B	6 months from the date of evacuation of the GTD if the hCG falls to normal within 56 days
C	6 months from the date of the hCG falling to normal if it does so within 56 days
D	6 months from the date of evacuation of the GTD if the hCG falls to normal after 56 days
E	6 months from the date of the hCG falling to normal if it does so after 56 days
F.	56 days after the first full moon after the evacuation of the GTD

Scenario 35.        

Which, if any, of the following statements are true about the recommended duration of follow-up after GTD? This is not a true EMQ as there may be > 1 correct answer.

A	6 months from the time the hCG falls to normal
B	6 months from the date of evacuation of the GTD if the hCG falls to normal within 56 days
C	6 months from the date of the hCG falling to normal if it does so within 56 days
D	6 months from the date of evacuation of the GTD if the hCG falls to normal after 56 days
E	6 months from the date of the hCG falling to normal if it does so after 56 days
F.	56 days after the first full moon after the evacuation of the GTD

Scenario 36.        

What is the approximate cure rate for GTN with a FIGO risk score <6?

A	70%
B	80%
C	90%
D	95%
E	98%
F.	100%

Scenario 37.        

What is the approximate cure rate for GTN with a FIGO risk score >7?

A	70%
B	80%
C	90%
D	95%
E	98%
F.	100%

Scenario 38.        

When should the possibility of persistent GTD be investigated after non-molar pregnancy?

A	if there is abnormal bleeding
B	if there is persistent abnormal bleeding
C	if there is cough
D	if there is new-onset dyspnoea
E	if there is pleurodynia

Scenario 39.        

A woman wishes to become pregnant after a pregnancy with GTD. Which, if any, of the following statements are true about the advice she should be given about an appropriate inter-pregnancy interval?

A	not before follow-up is complete
B	not for at least 3/12 after completion of follow-up
C	not for at least 6/12 after completion of follow-up
D	not for at least 12/12 after completion of follow-up
E	she should be advised not to become pregnant if chemotherapy was needed
F	not for at least 6 months after completion of follow-up if chemotherapy was needed
G	none of the above

Scenario 40.        

Which of the following statements are true about combined hormonal contraception use after GTD?

A	it may increase the risk of GTN if used before hCG levels have returned to normal
B	is not associated with additional risk
C	intra-uterine contraceptives are preferable

Scenario 41.        

Which, if any, of the following statements are true about the long-term issues for women who have needed chemotherapy for GTN?

A	the menopause is likely to be earlier
B	the risk of other cancers is not increased
C	there is evidence of ↑ risk of breast cancer
D	there is evidence of ↑ risk of colon cancer
E	there is evidence of ↑ risk of myeloid leukaemia
F	there is evidence of ↑ risk of melanoma
G	there is evidence of ↑ risk of breast cancer
H	there is no evidence of addition risk with HRT

Scenario 42.        

A woman had a complete mole in her first pregnancy. She is pregnant for the second time. What is the risk that it is another molar pregnancy?

Scenario 43.        

A woman has had two molar pregnancies. What is the risk of molar pregnancy if she becomes pregnant again?

Scenario 44.        

A woman has had three molar pregnancies. What is the risk of molar pregnancy if she becomes pregnant again?

Scenario 45.        

Which, if any, of the following statements are correct in relation to recurrence of molar pregnancy?

A	the histological type is likely to be the same
B	the histological type in recurrent mole after a complete mole is likely to be partial mole
C	the histological type in recurrent mole after a partial mole is likely to be complete mole
D	the histological type after PSTT is likely to be choriocarcinoma
E	none of the above

Scenario 46.        

A woman has a normal pregnancy after treatment for hydatidiform mole. Which, if any, of the following statements are true about the need for hCG testing 6 weeks after the pregnancy?

A	testing is optional
B	testing is only needed for women with persisting GTD
C	testing is only needed for women with persisting GTN
D	testing is only needed for women who have needed chemotherapy
E	testing should be offered to all women who use hair dye

Scenario 47.        

A woman has asked about support after a diagnosis of hydatidiform mole. What support is available from regional trophoblastic tumour centres?

A	continuing support is not available from TTCs and she should be put in touch with the local gynae cancer support nurse
B	support is available from TTCs but only in leaflet form
C	support is available from TTCs in the form of support groups
D	telephone support is available from TTCs
E	support is only available from the National GTD Support Society

 

63.         EMQ. Galactosaemia.

Some of the questions have no option list. The technique for the exam is to decide your answer before you read the option list. The absence of an option list forces this behaviour!

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 galactosaemia 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?