Tutorial 23rd. February 2017

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23 February 2017.

81	SBA. Hyperandrogenism, PCOS
82	EMQ. DSDs. AIS, Kallman’s & Swyer’s syndromes
83	SBA. Pertussis & pregnancy

Question 81       SBA. Hyperandrogenism, PCOS.

Hyperandrogenism, ovarian hyperthecosis and PCOS.

Abbreviations.

ACTH:      adreno-corticotrophic hormone released by the anterior pituitary to stimulate release of glucocorticoids from the adrenal cortex

CRH:        corticotrophin-releasing hormone released by the hypothalamus to stimulate ACTH release from the anterior pituitary

DHEA:     dehydroepiandrosterone

DHEAS:   dehydroepiandrosterone sulphate

DHT:        dihydrotestosterone

FT:            free testosterone

PCO:        polycystic ovaries

PCOS:      polycystic ovary syndrome

SHBG:      sex-hormone binding globulin

T:              testosterone

Question 1.

Lead-in

The following statements relate to androgen production by the adrenal gland. Which, if any, are true?

Statements

A.	adrenal androgens are mainly produced in the adrenal medulla
B.	adrenal androgens are produced from pregnenolone derived from cholesterol
C.	testosterone is the main adrenal androgen
D.	DHEA is the most potent activator of the androgen receptor
E.	DHEAS is a useful measure of adrenal androgen production as it is almost entirely produced in the adrenal
F.	DHEA is the main ovarian androgen
G.	androstenedione is the main ovarian androgen

Option List

1.	A + B
2.	A + C
3.	A + B + D + E
4.	B + C
5.	B + E
6.	B + E + G
7.	C + D + E

Question 2.

Lead-in

Which, if any, of the following statements are true in relation to blood testosterone in healthy women?

Statements

A.	50% is bound to SHBG
B.	80% is bound to SHBG
C.	49% is bound to albumin
D.	19% is bound to albumin
E.	1% is free

Option List

1	A + C
2	A + C + E
3	A + D
4	A + D + E
5	B + D
6	B + D + E
7	D + E

Question 3.

Lead-in

The following statements relate to androgen receptors. Which, if any, are true?

Statements

A	androgen receptors are located on cell membranes
B	androgens diffuse across cell membranes and attach to mitochondrial androgen receptors
C	androgens diffuse across cell membranes and attach to nuclear androgen receptors
D	androgen receptors in the brain are located in the pre-optic area of the hypothalamus
E	androgen receptors are not present in breast tissue
F	androgen receptors in bone are important for bone mineralisation

Option List

i	A + D + E + F
ii	B + D + E + F
iii	C + D + E + F
iv	A + E + F
v	C + D + F

Question 4.

Lead-in

Which, if any, of the following substances are significant activators of the androgen receptor?

Option List

A.	androstenedione
B.	DHEA
C.	DHEAS
D.	DHT
E.	T

Option List

1	A + B + C
2	A + B + C + D + E
3	B + C
4	B + C + D + E
5	D
6	D + E

Question 5.

Lead-in

Approximately what proportion of circulating testosterone in healthy women is ovarian in origin?

Option List

A.	< 5%
B.	5 - ≤10%
C.	10 - ≤15%
D.	15 - ≤20%
E.	25%

Question 6.

Lead-in

What is the major pathway for metabolism / excretion of testosterone in healthy women

Option List

A.	aromatisation in peripheral tissues
B.	hepatic metabolism
C.	hepatic metabolism and conjugation with urinary excretion as 17-ketosteroids
D.	hepatic metabolism and conjugation with urinary excretion as 17-OH progesterone
E.	urinary excretion as esters of testosterone

Question 7.

Lead-in

Which of the following statements is true about testosterone assay in most hospitals?

Option List

A.	assays are accurate in both male and female ranges
B.	assays are accurate in the male range, but not the female
C.	assays consistently give results that are greater than they should be for women
D.	assays consistently give results that are less than they should be for women
E.	assays may give results that are half of what they should be

Question 8.

Lead-in

What testosterone level is usually taken as indicating a need to exclude serious pathology in women?

Option List

A.	≥ 1 nmol/l
B.	≥ 2 nmol/l
C.	≥ 3 nmol/l
D.	≥  5nmol/l
E.	≥  10nmol/l

Lead-in.

What criteria are now used to define PCOS? What are they called? Where do they come from?

Question 10.

Lead-in

Which, if any, of the following statements are true in relation to PCOS and Stein-Leventhal syndrome (SLs).

Option List

A.	PCOS used to be known as SLs
B.	PCOS and SLs are synonyms
C.	the definition of PCOS includes less severe cases than those included in SLs
D.	Stein-Leventhal was one person
E.	the original paper was presented at a meeting in New Orleans – nice work, if you can get it!

Question 11.

Lead-in

Which, if any, of the following statements are true in relations to hyperandrogenism in pregnancy?

Statements

A.	maternal hyperandrogenism has been postulated as a cause of PCOS in the offspring
B.	maternal hyperandrogenism is usually due to conditions that pre-dated the pregnancy
C.	total T levels are higher and SHBG levels are higher in pregnancy
D.	total T levels are lower and SHBG levels are higher in pregnancy
E.	unilateral, solid ovarian masses + hyperandrogenism carry an ↑ risk of malignancy

Option List

1	A + B + C + E
2	A + B + D + E
3	A + C + E
4	B + C + E
5	C + E

Question 12.

Lead-in

Which, if any, of the following statements are true in relations to hyperandrogenism in pregnancy?

Option List

A.	is most often due to persisting corpus luteum
B.	is most often due to adrenal adenoma
C.	is most often due to consumption of androgenic drugs
D.	is most often due to ovarian luteomas & theca lutein cysts
E.	is most often due to ovarian hyperthecosis

Question 13.

Lead-in

Which, if any, of the following statements are true in relation to ovarian hyperthecosis (OH).

Statements

A	OH is the most common cause of hyperandrogenism in postmenopausal women
B	approximately 10% of premenopausal women with hyperandrogenism have OH
C	is associated with the presence of luteinised theca cell nests in the adrenal stroma
D	is associated with higher testosterone levels than are typical of PCOS
E	is associated with more severe clinical features than occur in women with PCOS

Option List

1	A + B + C + E
2	A + B + D + E
3	A + C + E
4	A + D + E
5	B + C + D +E

 Question 14.

Lead-in

Lead-in

Which, if any, of the following statements are true in relation to ovarian hyperthecosis.

Statements

A	acanthosis nigricans may be a consequence
B	clinical features reduce with a trial of dexamethasone
C	endometrial hyperplasia and cancer are more common
D	onset of clinical features is usually sudden and progression is rapid
E	significant insulin resistance is common
F	testosterone levels exceed those in PCOS and may be > 5nmol/l.

Option List

1	A + B + C + E
2	A + B + D + E
3	A + C + E
4	B + C + E
5	C + E
6	F

Question 15.

Lead-in

Lead-in

Which, if any, of the following statements are true in relation to acanthosis nigricans.

Statements

A	acanthosis nigricans only occurs in those of Afro-Caribbean descent
B	obesity is a common cause
C	acanthosis nigricans is a good marker for insulin resistance
D	acanthosis progresses to malignant melanoma in 5% of cases
E	acanthosis nigricans of sudden onset may indicate malignancy
F	acanthosis nigricans responds well to local steroid ointments

Option List

1	A + B + C + E
2	A + B + D + E
3	A + C + E
4	B + C + E
5	C + E
6	F

Question 16.

Lead-in

List all the causes of hyperandrogenism that you can think of.

There is no option list – the list will come with the answers.

Question 17.

Lead-in

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

Statements

A	ovarian androgen-secreting tumours are mostly Brenner tumours
B	ovarian androgen-secreting tumours produce significant ↑ of testosterone levels
C	ovarian androgen-secreting tumours produce significant ↑ of serum DHEAS & urinary 17-ketosteroids
D	ovarian androgen-secreting tumours usually result in early virilisation
E	ovarian androgen-secreting tumours are less common than adrenal androgen-secreting tumours

Option List

i	A + B + C + D + E
ii	A + B + D
iii	B + C + D
iv	B + D
v	B + E

Question 18.

Lead-in

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

Statements

A	adrenal androgen-secreting tumours are mostly Brenner tumours
B	adrenal adenomas produce significant ↑ of cortisol and aldosterone levels
C	adrenal carcinomas significant ↑ of androgens and cortisol
D	adrenal androgen-secreting tumours usually result in early virilisation
E	adrenal androgen-secreting tumours are associated with ↑↑ in levels of testosterone, DHEAS and urinary 17-ketosteroids that do not ↓ with dexamethasone

Option List

i	A + B + C + D + E
ii	A + C + D + E
iii	B + C + D + E
iv	B + C + D
v	C + D + E

CPD questions from TOG 15.3

Polycystic ovary syndrome and the differential diagnosis of hyperandrogenism

Androgen excess in women is associated with,

1.     menstrual irregularity.

With regard to normal androgen physiology in women,

2.     the adrenal medulla makes dehydroepiandrosterone sulfate.

3.     less than 10% of testosterone is bound to sex hormone binding globulin.

With regard to androgen action and metabolism,

4.     androgens are excreted unchanged in the urine.

5.     testosterone binds to a nuclear receptor.

With regard to the clinical presentation of hyperandrogenism,

6.     the Ferriman-Gallwey score is useful in objectively assessing the severity of hirsutism.

7.     deepening voice and breast atrophy are features suggestive of an adrenal tumour.

Regarding the biochemical assessment of hyperandrogenic patients,

8.     serum testosterone >5 nmol/l should prompt further investigation.

Regarding the pathophysiology of polycystic ovary syndrome,

9.     a combination of genetic and lifestyle factors are likely to be causative.

10.   arrest of follicular development is characteristic

Regarding the differential diagnoses of hyperandrogenism,

11.   ovarian hyperthecosis is a disease of childhood.

12.   congenital adrenal hyperplasia is often diagnosed in infancy.

13.   the most common virilising adrenal tumours are the Sertoli-Leydig cell type.

With regard to the pathophysiology of hyperandrogenism,

14.   approximately 50% of circulating androgens are conjugated with either glucuronic or sulfuric acid.

15. In hyperandrogenaemic women with PCOS, it has been shown that there is an increased risk of breast cancer.

With regard to the quantification of androgens in secondary care institutions in the UK,

16.   automated immunoassays on whole serum are known to consistently overestimate serum testosterone concentrations.

In cases of hyperandrogenism,

17.   ovarian hyperthecosis accounts for less than 50% of cases in postmenopausal women.

18.   the non-classic 21-hydroxylase deficiency tends to typically present in childhood.

19.   luteomas of the ovary are one of the most common causes of gestational hyperandrogenism.

20.   unilateral solid ovarian lesions as a cause have an increased risk of malignancy when presenting in pregnancy.

Question 82.     EMQ. DSDs. AIS, Kallmann’s & Swyer’s syndromes.

AIS, MRKH and Swyer’s syndrome

Lead-in.

The following scenarios relate to disorders of sexual development.

Pick the option from the option list that best fits each scenario.

Each option can be used once, more than once or not at all.

Abbreviations.

AIS:       androgen insensitivity syndrome.

AMH:    anti-Müllerian hormone.

CAH:     congenital adrenal hyperplasia.

CAI:       complete androgen insensitivity syndrome.

DSD:     disorder of sexual differentiation.

KS:         Kallmann’s syndrome.

LMB:     Laurence-Moon-Biedl syndrome.

MRKH:  Mayer-Rokitansky- Küster-Hauser syndrome.

PAI:       partial androgen insensitivity syndrome.

PW:       Prader-Willi syndrome.

SW:       Swyer’s syndrome.

TU:        Turner’s syndrome.

UPD:     uni-parental disomy.

Option list.

A.        has a uterus of normal size for her age.

B.         has a uterus that is hypoplastic for her age.

C.         has a vestigial uterus (anlagen).

D.        has no uterus.

E.         commonly has esthiomene

F.         I don’t know and I don’t care.

G.        the question makes no sense.

H.        none of the above.

Scenarios.

1.     a girl with congenital adrenal hyperplasia at the start of puberty.

2.     a girl with complete androgen insensitivity syndrome at the start of puberty.

3.     a girl with a disorder of sexual differentiation at the start of puberty.

4.     a girl with Kallmann’s syndrome at the start of puberty.

5.     a girl with Laurence-Moon-Biedl syndrome at the start of puberty.

6.     a girl with Mayer-Rokitansky-Kuster-Hauser syndrome at the start of puberty.

7.     a girl with partial androgen insensitivity syndrome at the start of puberty.

8.     a girl with Prader-Willi syndrome at the start of puberty.

9.     a girl with Swyer’s syndrome at the start of puberty.

10.   a girl with Turner’s syndrome at the start of puberty.

Question 83.     SBA. Pertussis & pregnancy.

Lead-in. Why is pertussis of current concern in obstetrics?

Option List

A	Recent research has linked pertussis in the 1st. trimester with an ↑risk of congenital heart disease
B	There has been a mini-epidemic of pertussis since 2011 with an increase in maternal deaths and deaths of babies < 3 months
C	There has been a mini-epidemic of pertussis since 2011 with an increase in deaths of babies < 3 months
D	The infecting organism causing pertussis has become increasingly drug-resistant
E	Pertussis in the 2nd. trimester doubles the risk of premature delivery < 32 weeks

Question 2.

Lead-in

Which of the following statements is true?

Option List

A	Pertussis is not a notifiable disease
B	Pertussis is a notifiable disease
C	Pertussis is not a notifiable disease, but cases should be reported to the local bacteriologist
D	Pertussis is not a notifiable disease, but cases should be subject to audit

Question 3.

Lead-in

Which organism causes whooping cough?

Option List

A	Bordella pertussis
B	Bacteroides pertussis
C	Rotavirus whoopoe
D	Respiratory syncytial virus pertussis
E	None of the above

Question 4.

Lead-in

What is the origin of the name of the infecting organism?

Option List

A	It is named after one of doctors who first isolated it
B	It is named after the town where the first recorded outbreak occurred
C	The organism was first isolated from the staff of a bordello in Madrid
D	None of the above
E	I refuse to answer this stupid question

Question 5.

Lead-in

What is the main reservoir of the organism that causes pertussis?

Option List

A	pigs
B	pigeons
C	budgerigars
D	humans
E	none of the above

Question 6.

Lead-in

What is the epidemiology of pertussis?

Option List

A	the condition is endemic
B	the condition is endemic with mini-epidemics every 3-5 years
C	the condition is endemic with mini-epidemics most years in the winter months
D	the condition is epidemic, with outbreaks at roughly three-year intervals
E	the condition is epidemic, with outbreaks at unpredictable intervals

Question 7.

Lead-in

Which, if any, of the following statements are true in relation to pertussis infection in unvaccinated but otherwise healthy pregnant women?

Statements

A	< 10% will need to be admitted to hospital
B	20-30% will need to be admitted to hospital
C	> 50% will need to be admitted to hospital
D	20% will get pneumonia
E	1% will die of the infection

Option List

1	A + C + D + E
2	A + C + E
3	B + C + D
4	B + D + E
5	B + E

Question 8.

Lead-in

Which, if any, of the following statements are true in relation to pertussis infection in unvaccinated but otherwise healthy babies < 2 months old?

Statements

A	< 10% will need to be admitted to hospital
B	20-30% will need to be admitted to hospital
C	> 50% will need to be admitted to hospital
D	20% will get pneumonia
E	1% will die of the infection

Option List

1	A + D
2	B + E
3	A + D + E
4	B + D + E
5	C + D + E

Question 9.

Lead-in

What is the incubation period for pertussis?

 Option list

A	<6 days
B	6-10 days
C	6-20 days
D	10-20 days
E	none of the above

Question 10.

Lead-in

The following statements relate to practical issues that are current for obstetricians in relation to pertussis?

Statements

A	The DOH has advised that all pregnant women be immunised to reduce maternal death rates.
B	The DOH has advised that all pregnant women be immunised to reduce deaths in babies < 3 months.
C	The DOH has advised that all babies be immunised at birth.
D	The DOH advised that “Boostrix- IPV” would replace “Repevax” for use in pregnancy from July 2014.
E	The DOH has advised that immunisation of pregnant women be continued until 2019

Option List

1	A + C + D + E
2	A + C + E
3	B + C + D
4	B + D + E
5	B + E

Question 11.

Lead-in

Which, if any, of the following statements are true in relation to pertussis vaccine.

Option List

A	“Boostrix- IPV” is a vaccine for pertussis only
B	“Repevax” is a vaccine for pertussis only
C	“Boostrix- IPV”& “Repevax” are live, attenuated vaccines
D	“Boostrix- IPV” & “Repevax” are vaccines against diphtheria, tetanus and polio as well as pertussis
E	“Boostrix- IPV”  & “Repevax” are acellular

Question 12.

Lead-in

Which, if any, of the following statements are true in relation to pertussis vaccine.

Statements

A	The currently recommended vaccine is a live vaccine using a strain that does not produce pertussis toxin but generates a strong immune response
B	The currently recommended vaccine is an activated vaccine
C	The currently recommended vaccine is an inactivated vaccine
D	The currently recommended vaccine is acellular
E	The currently recommended vaccine is made using recombinant technology

Option List

1	A + B + C + D +E
2	A + B + C + D +E
3	A + B + C + D +E
4	A + B + C + D +E
5	A + B + C + D +E

Question 13.

Lead-in

Which, if any, of the following statements are true in relation to pertussis vaccine.

Statements

A	adult antibody response to a pertussis booster peaks after two weeks
B	adult antibody response to a pertussis booster declines significantly in the months after it peaks
C	adult antibody response to a pertussis booster declines gradually from about 1 year after it peaks
D	mother-baby antibody transfer occurs at the same rate at all gestations after 16 weeks
E	mother-baby antibody transfer occurs maximally from about 28 weeks

Option List

1	A + B
2	A + B + D
3	A + C + D
4	B + D
5	C + E

Question 14.

Lead-in

Which, if any, of the following statements are true in relation to the JCVI’s advice of the best time to administer pertussis vaccine in pregnancy?

Option List

A	20 - 24 weeks
B	25- 28 weeks
C	28 - 32 weeks
D	28 - 34 weeks
E	30 - 36 weeks

Question 84.     EMQ..