~~~~i~iifi" i GYNAECOLOGY

Self-assessment questions: Gynaecological endocrinology

Question Al

Al)

Fig. Al -This female infant has congenital adrenal hyperplasia

a) What is the commonest enzyme defect? b) What is the mode of inheritance? c) If untreated and the child survives into adulthood,

would the final height be tall or short?

A21 a) What is the likely diagnosis? b) What other abnormalities are associated with this

condition? c) What is the likely eventual fertility?

Question B: answer the following true or false.

Bl) The following statements concern thyroid func- tion in pregnancy: a) The serum TSH may normally be suppressed to

< 0.1 mU/L in the first trimester. b) Free T3 and T4 rise in the second half of

pregnancy. c) Hydatidiform mole may be associated with hyper-

thyroidism. d) Hyperemesis gravidarum may respond to small

doses of carbimazole. e) Postpartum thyroid dysfunction occurs in lo-20%

of women.

B2) Regarding pituitary prolactinomas: a) They are found in approximately 10% of women

dying of non-endocrine disease at post mortem. b) Patients with microprolactinomas should be main-

Fig. A2 - This child also had geographical pigmentation

4

4

4

tained on dopamine agonist therapy throughout pregnancy. Women with untreated microprolactinomas should be discouraged from breast feeding. Microprolactinomas may regress spontaneously in 25% of cases or more. The effects of oestrogen deficiency are the main indication for treatment of young women with microprolactinomas and amenorrhoea.

B3) Impotence in the male: a) Should be investigated by arteriography if no obvi-

ous cause is found. b) In diabetes mellitus is always the result of an auto-

nomic neuropathy. c) Can be caused by thiazide diuretics. d) In paraplegics responds well to treatment with

intracorporeal papaveretum.

Current Obsrerrics and Gynnecology (1991) 1, 244-246 Q 1991 Longman Group UK Ltd 244

SELF ASSESSMENT: GYNAECOLOGICAL ENDOCRINOLOGY 245

e) Ejaculation usually occurs normally provided an erection can be achieved.

Cl) A patient is found to have a very low serum oestriol and low prolactin concentration at 36 weeks of pregnancy. In an earlier pregnancy she delivered a male infant later diagnosed as having ichthyosis. a) What is the likely diagnosis? b) Why is the maternal serum oestriol concentration

low’! c) Is vaginal delivery contraindicated?

C2) An obese 18-year-old girl presents with a 6 month history of increasing weight, oligomenorrhoea and acne. The ovaries are shown to be polycystic on ultra- sound scan. a) Classically what happens to the SHBG concen-

tration and what role does SHBG play? b) What is the insulin concentration likely to be and

how is insulin important in this condition? c) Where does the abnormality lie in this condition?

C3) A 30-year-old lady presents with an 18 month history of secondary amenorrhoea associated with hot flushes. The serum FSH concentration is 45 IU/L and the LH concentration 30 TU/L. a) What further investigations would you perform? b) What treatment would you offer? c) If she elects not to have treatment and returns 12

months later with regular menstruation, and nor- mal FSH and LH concentrations, what diagnosis would you consider?

C4) An 18-year-old patient presents with a history of increasing hair growth on her face, lower abdomen and upper legs. a) What is the single most important biochemical

investigation in this patient? b) What is the value of measuring serum 17-hydroxy-

progesterone? c) Once serious disease has been excluded, what treat-

ment can be considered for excessive hair growth?

C5) A 35year-old lady gives a history of losing a milk-like fluid from both nipples. She is not pregnant or lactating and her periods are regular. a) What is the likelihood of her having a significant

endocrine abnormality? b) What investigations would you perform? c) If investigations are normal, how could you treat

the galactorrhoea?

A 1 a) 2 1 hydroxylase deficiency. b) Autosomal recessive. c) Short: the increased androgens of CAH lead

to a growth spurt in early childhood. However, epiphyses fuse early and final height is short.

A2 a) McCune-Albright syndrome. b) Polyostotic fibrous dysplasia and fractures. c) Normal.

Answer B

Bla) T b) F ~ They rise in first 6 weeks of pregnancy but

are normal in the latter half of pregnancy.

c) T d) T e) T

B2 a) T b) F - rarely necessary. Microprolactinomas do

not seem to become macroprolactinomas and rapid expansion in pregnancy has only been described in macroadenomas. Bromocriptine therapy prevents breast-feeding.

c) F - off dopamine agonist therapy, women with microprolactinomas can breast-feed normally.

d) T e) T

B3 a) F b) F -- often, but can be psychological.

c) T d) F ~ NB papaverine.

e) T

Answer C

Cl a)

b)

c)

C2 a)

b)

c)

Placental sulphatase deficiency. Patients are unable to hydrolyse DHAS or 16cl-hydroxy-DHAS and the placenta cannot produce normal amounts of oestrogen. No ~- but often fail to go into spontaneous labour and go post-term, and cervical dystocia can occur which is resistant to oxytocics.

SHBG concentrations are usually low, and therefore the free testosterone concentration is elevated. Hyperinsulinaemia is likely. High insulin levels and high insulin-like-growth factor 1 levels may increase ovarian androgen production as well as decreasing SHBG production. Unknown. There are abnormalities at all levels. which leads to a vicious circle:

i)

ii) iii)

iv)

v) vi) vii)

Hypothalamus ~ altered GnRH pro- duction. Pituitary - altered LH pulsatility. Ovary - increased androgen production. altered oestrogen production, anovu- lation. Adrenal ~ increased androgen production. Liver - decreased SHBG production. Pancreas - increased insulin production. Adipose - peripheral conversion of andro- gens to oestrogens.

246 CURRENT OBSTETRICS AND GYNAECOLOGY

C3 a)

b)

c)

C4 a)

b)

c)

oestradiol, karyotype, ovarian antibodies, ovarian biopsy if pregnancy is being con- sidered. HRT or ovum donation if premature meno- pause confirmed. The ‘resistant ovary syndrome.’

Serum testosterone concentration. If this is greater than 5 nmol/L there is a 20% chance of an androgen secreting tumour. A serum tes- tosterone concentration of less than 5 nmol/L almost excludes this condition, although there have been occasional reports of tumours hav- ing their activity suppressed by the OCP. To exclude late-onset, non-classical congenital adrenal hyperplasia. i) Local - shaving, bleaching, depilatory

creams, waxing and electrolysis.

C5 a)

b)

c)

ii) Hormonal - Combined oral contraceptive pill. A desogestrel containing preparation e.g. Marvelon or Mercilon may be better as desogestrel is less androgenic than other progestogens. - Cyproterone acetate (So-100 mg) in combination with cyclical oestrogens. - Prednisolone may induce ovulation and menstruation in polycystic ovarian syn- drome but rarely improves hirsutism.

Less than 10% with regular periods, over 50% if oligomenorrhoea. Prolactin, thyroid function tests. There is no indication for CT scan if prolactin is within reference range. Bromocriptine - may work, but may not.