PCOS and DD of hyperandrogegism TOG 2013 Flashcards

1
Q

What can androgen excess cause?

A

Hirsuitism
Androgenic alopecia
Acne
Ovarian Dysfunction
Virilisation
Masculinsation

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2
Q

Main androgen produced by adrenal gland

A

DHEA and DHEA-S

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3
Q

What androgens does the ovary produce

A

20% of DHEA
50% of androstenedione
25% of testosterone

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4
Q

How does testosterone circulate in blood stream

A

80% bound SHBG
19% bound albumin
1% free testosterone or its active dihydrotestosterone

Bound androgens (DHEA-S, DHEA, androstenedione is converted to testosterone by peripheral tissues)

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5
Q

Clinical assessment for androgen excess

A

Height, weight and body mass index.
Distribution and extent of adiposity.
Skin thinning or bruising (seen in Cushing’s syndrome).
Acne, especially over the face, neck, back and chest.
Degree, pattern and severity of hirsutism.
Acanthosis nigricans (velvety skin hyperpigmentation), associated with insulin resistance.
Deepened voice.
Male pattern balding.
Breast atrophy.
Clitoromegaly.
Loss of normal feminine body shape.

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6
Q

What scoring system is used to scale hirsutism?

A

Ferriman-Gallwey
9 Sites 0 = no terminal hair 4= complete and heavy cover

8-15 mild hirsutism
> moderate/severe

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7
Q

How to use testosterone levels in assessment of hyperandrogegism

A

2-5 mol/L - likely PCOS
>5 - other causes ?andoren secreting tumour

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8
Q

Gold standard for testosterone quantitation

A

Mass spectrometry

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9
Q

What are the differentials for hyperadrogenism?

A

PCOS
Ovarian hyperthecosis
Congenital adrenal hyperplasia
Cushing’s syndrome
Androgen-secreting tumour
- Adrenal origin
- Ovarian origin
Exogenous androgen administration
Gestational hyperandrogenism

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10
Q

Presentation of CAH with classical 21 hydroxylase deficiency

A

Female infants virlised
Adrenal crisis

Low cortisol, low aldosterone, high androgens
Low Na, High K

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10
Q

Biochemical findings in PCOS

A

Mild to moderate elevation in free and total testosterone
Elevated DHEA-S
Low SHBG
LH/FSH ratio elevated

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11
Q

Explained hyperthecosis.
- In whom does it typically present
- How high are testosterone levels

A

Describes lutenised theca cells in ovarian storm

Typically postmenopausal
Severe hyperandrogegism, testosterone >7 - virilisation

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11
Q

Most common enzyme deficiency in CAH, which metabolite is raised

A

21 hydroxylase deficiency
17-OHP raised

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12
Q

When to patients present with non classical 21 hydroxylase deficiency

A

Cortisol/aldosterone pathway maintained 20-60% normal function.

Present early adults with menstrual disturbance or hirsutism. Precocious puberty.

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13
Q

How does 11B hydroxylase deficiency present. What is its metabolite which is raised?

A

Metabolite 11 deoxycortisol

Low cortisol
High aldosterone
High androgens

Amibigious genitalia in female
No adrenal crisis
High Na, Low K

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14
Q

What proportion of women with cushings syndrome have hirsutism?

A

80%

15
Q

What hormone is raised in cushings? How does it present?

A

High cortisol
Central weight gain
Facial plethora
Supraclaviclular fat pads
Abdominal striae
High androgen - hirsutism, acne, male pattern baldness

16
Q

What are the causes of Cushing syndrome?

A

ACTH secreting pituitary tunmours (Cushings disease)
Autonomous cortisol secretion by adrenal gland - neoplasm or hyperplasia
Exogenous glucocorticoids
Ectopic ACTH from neoplasia - small cell lung cancer

17
Q

How do androgen secreting tumours typically present? Most common virilising ovarian tumour?

A

Mimic PCOS but sudden onset and rapid progression

Sertoli leydig tumours

18
Q

Hormone profile of ovarian androgen securing tumours vs adrenal androgen secreting tumour

A

High testosterone
Normal DHEA-S and 17 ketosteroids

Adrenal tumours - high DHE-A and urinary 17 ketosteroids levels, not suppressed by dexamethasone