Reproduction 17

Question 1

Describe precocious puberty

Answer 1

Onset of secondary sexual characteristics before 8 yrs (girls) or 9yrs (boys)

Linked to short stature

Central- sex hormones produced too early by the HPG axis, idiopathic, environmental endocrine disruptors, obsesity

Peripheral- sex hormones produced by atypical means- adrenal hyperplasia or tumour

Question 2

Describe delayed puberty

Answer 2

Absence of secondary sexual characteristics by 14 years (girls) or 16yrs (boys)

95% constitutional, chemo/radiotherapy, pituitary tumours, Turner syndrome, Kallman syndrome, androgen insentivity syndrome and 5alpha reductase syndrome

Question 3

Describe problems with the mentrual cycle

Answer 3

Amenorrhea- absence of menstrual cycles for >=6 months, primary- no menarche by 16, secondary- ceased Oligomenorhe- irregular cycles

Question 4

What are the presenting symptoms for endocrine disorders effecting female reproduction?

Answer 4

Oligo/amenorrhea

Infertility

Oestrogen deficiency- hot flushes, poor libido, painful intercourse

Hyperandrogenism- hirsuitism, acne, androgenic alopecia

Galactorrhoea (producing milk when you havent had a baby)

Question 5

How would you diagnose HPG axis dysfunction in females?

Answer 5

Within the HPG axis this can go wrong:

Central patholgy, Gonal damage/failure, POlycystic ovart syndrome

Pregnancy test for amenorrhoea

FSH/LH on day 2/3- ovarian reserve

Progesterone on day 21 for ovulation

Progesterone challenge test for amenorrheic women. GIve progesterone for 5 days which induces a period. (Medoxyprogesterone acetate for 5 days)

Bleed 2-7 days after course

Question 6

what are the possible primary causes for endocrine dysfunction in women?

Answer 6

Ovarian insensitivity- normal GnRH, high FSH/LH due to lack of negative feedback

Premature ovarian failure - Ammenorrhea, low oestrogen, high LH/FSH. Caused by turner syndrome, autoimmunity

Turner syndrome- X0 leads to oocyte death whichvh leads to ovarian dysgenesis Treat with GH, androgen and oestrogen

Chemotherapy/radiotherapy- preserve fertility: freezing embryos- 25%, freezing eggs- 10%

Question 7

What is premature ovarian failure?

Answer 7

Amenorrhea, low oestrogen, high FSH/LH for 40yrs- 1% of women Caused- often unknown, Turner syndrome, autoimmune, iatrogenic- chemotherapy and radiotherapy Surgery

Question 8

Central causes of endocrine reproductive dysfunction

Answer 8

Gonadotrophin secretion is low/absent (low FSH/LH) due to problems with the hypothalamus, pituitary. Low oestrogen

Question 9

What is hyperprolactinaemia ?

Answer 9

Increase prolactin release from the lactotroph cells in the anterior pituitary

Suppresses FSH/LH

Leads to oligo/amenorrhoea and galactorrhoea

Can be physiological- lactational amenorrhoea, a prolactin secreting tumour, tumours affecting the pituitary stalk suppressing dopamine release or dopamine antagonists

Treat with surgery or with dopamine agonists

Question 10

Describe Kallman syndrome

Answer 10

More common in men than women

The GnRH neurones fail to migrate to the hypothalamus (Anosmia in 75%) Anorexia, over exercise and stress–> CRH–> Suppression of GnRH And Obesity- adipose is oestrogenic and suppresses FSH and decreases fertility

Question 11

Descirbe polycystic ovarian syndrome

Answer 11

commonest endocrine condiiton effecting 10% of all menopausal women

INcreased risk of ovarian hyperstimulaition syndrome

Increased GnRH pulsative release causes an inbalance in ration of LH/FSH. Also has increase insulin reistance, causing hyperinsulinemia. Both of these factors contribute to excess androgens causing arrest in antral follicle developoment (follicle dont develop propley and are not ovulated)

Question 12

What is dysmenorrhoea?

Answer 12

painful periods about 50% of women and 10% severely Primary- excessive endometrial prostglandins, uterine hypercontractility, decreased blood flow, nerve hypersensitivity Secondary- endometriosis, pelvic inflammatory disease, fibroids, ovarian cysts

Question 13

Describe congenital adrenal hyperplasia

Answer 13

21-hydroxylase deficiency Neonatal/infancy presentation- adrenal androgen excess (virilised female- ambiguous genitalia) Aldosterone deficiency- salt wasting

Question 14

List endocrine disorders affecting females that affect reproduction

Answer 14

Central pathology with HPG axis- Hypothalamic/pituitary disease Gonadal damage/failure Polycystic ovary disease Turner syndrome Chemo/radiotherapy Premature ovarian failure Hyperprolactinaemia Kallman syndrome Congenital adrenal hyperplasia

Question 15

List endocrine disorders affecting males and affect reproduction

Answer 15

Klinefelter syndrome

Acquired damage- chemo/radiotherapy (testes are external, so less likely to be affected by chemo/radio therapy outside the body)

Central causes- low or absent gonadotrophin secretion due to problems with the hypothalamus or pituitary or low testosterone

Kallman syndrome

Androgen insensitivity syndrome

5alpha reductase deficiency

Question 16

What are the presenting symptoms in males with endocrine disorders affecting reproduction?

Answer 16

Loss of libido, reduced sexual behaviour, impotence Infertility Reduced testicular volume Gynaecomastia Loss of body hair Decreased muscle mass, female fat distribution

Diagnosed with testosterone levels (testicular function) and FSH/LH (HPG axis)

Primary cause- Testicular insensitivity High FSH/LH due to absence of negative feedback from testosterone

Question 17

Descriebe Klinefelter syndrome

Answer 17

47XXY Azoospermia, gynaecomastia

Firm pea-sized testes, low testosterone, high FSH/LH

Question 18

Describe androgen insensitivity syndrome

Answer 18

Testicular feminisation (testes get smaller wth people that take steriods)

Mutations in the androgen receptor (AR)- spectrum partial–>complete 46XY

Testis develop and produce testosterone but the foetus is insensitive to androgen and develops female genitalia and is assigned female at birth

Presentation- inguinal hernia and primary amenorrhoea.

AIS Males unresponsive to DHR- external genitals are feminised, no female reproductive syndrome

Question 19

Describe 5alpha reductase deficiency

Answer 19

46XY

Unable to convert testosterone to dihydrotestosterone

Female or ambiguous genitalia

Primary amenorrhoea

Virilisation at puberty- male secondary sex characteristics

Question 20

What are the risks of testosterone replacement and androgen abuse?

Answer 20

Psychological changes Prostate cancer Atrophy of testes Azoospermia- infertility Polycythaemia Cardiovascular- cardiac muscle hypertrophy, hypertension, arthymias