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..
