Adrenal Disease and pregnancy TOG 2021

Question 1

What are the 3 main categories of adrenal insufficiency (Addison’s)

Answer 1

Primary: Adrenocoritical Disease
Secondary: ACTH
Tertiaty: CRH

Question 2

Which hormones are deficiency in prrimary/secondary/tertiaty

Answer 2

Primary: Glucocorticoid, Mineralcorticoid
2nd/3rd: Glucocorticoid alone

Question 3

Most common cause or primary adrenal insufficiency?

Answer 3

Autoimmune 70-90% (40% will have other autoimmune condition)

Other causes: Haemorrhage 2nd sepsis, major burns, lymphoma, mets and infections e.g. TV

Question 4

At how many weeks gestation does cortisol peak?

Answer 4

26 weeks

Question 5

The placenta increases the production of which hormones?

Answer 5

CRH and ACTH

Question 6

Symptoms of primary adnreal insufficiency?

Answer 6

Weight loss
Vomiting
Hyperpigmentation
Hypoglycaemia
Hyponatraemia
Hyperkalaemia

Adrenal crisis may be triggrtered by stress - infection/delivery/CS

Most cases Dx before pregnancy

Question 7

How to diagnose primary adrenal insufficiency?

Answer 7

Morning Cortisol levels (levels higher than non pregnant)
Raised ACTH
Loss of cortisol response to synthacthen test

Question 8

Management of primary addisons in pregnancy?

Answer 8

Joint obs and endocrinology
- Hydrocortisone 15-25mg in 2-3 doses
- Fludrocortisone 0.1mg/day

Increase dose in stress/labour - IM/IV hydrocortisone 100-200mg/day and weaned over a number of days to prevent profound hypotension

Question 9

How can acute AI crisis (addisonian crisis present)

Answer 9

Abdo pain
Vomiting
Shock

Question 10

When can it occur with patients who do not have a known adrenal insufficiency?

Answer 10

Bilateral adrenal necrosis - haemorrhage, sepsis, adrenal vein thrombosis.

Question 11

Which patients need intrapartum hydrocortisone?

Answer 11

5-20mg+ prednisolone (or equivalent)/day for 3 weeks +

Question 12

What does of hydrocortisone should be given?

Answer 12

IV 50-100mg TDS for 24 hours

Question 13

How to treat acute adrenal insufficiency in pregnancy?

Answer 13

1. IV access
2. Blood - ACTH, cortisol, serum electrolytes, glucose
3. 2-3L NaCl 0.9% or 5% dextrose
4. IV hydrocortisone 6-8hrly or continous
5. Regular obs
6. Treat cause
7. Fetal assessment
8. Taper IV hydrocortisone over 1-3 days

Question 14

What are the sick day rules?

Answer 14

• Wear medical alert bracelet/neckalce
• Double dose GC in cases of fever/ilnness requiring bedrest
• Provide IM HC self-administer if gastroenteritis/fasting

Question 15

GC and MC during delivery and postnatally
Delivery Day
Day 1
Day 2
D/C
Follow-up

Answer 15

Delivery Day: 200mg/day - divided doses IV or PO, hold fludrocortisone
Day 1: 100mg/day, hold fludrocortisone
Day 2 50mg/day, hold fludrocortisone
D/C 30-35mg/day, restart fludrocortisone
Follow-up: FU in Endometritis clinic, reduce dose to pre-pregnancy level, adjust dose fludro, encourage BF

Question 16

Pregnancy outcome with primary adrenal insufficiency:

Answer 16

Well controlled - risk FGR
Pool controlled - PTL, C/S, poor wound healing, VTE and acute adrenal crisis

Question 17

What is Cushing syndrome?

Answer 17

Increased cortisol
Can be ACTH dependant/independant

Untreated rarely become pregnanct

Question 18

Most common cause of Cushing syndrome, in pregnancy and outside pregnancy?

Answer 18

In pregnancy: Adrenal adenoma 60% (do not secrete androgens, so pregnancy more likely)
Outside pregnancy: Pituitary dependent Cushing syndrome 70%

Question 19

What is pregnancy associated Cushing syndrome?

Answer 19

Onset during gestation or within 12 months of delivery or miscarriage

e.g. nodular hyperplasia of adrenals stimulated by placentally produced ACTH, or pre-exciting adrenal adenoma stimulated by ACTH from the placenta

Question 20

Why is diagnosis of cushings difficult in pregnancy?

Answer 20

Pregnancy already has raised cortisol levels, low dose dexamthetonse suppression test not accurate

Some symptoms cross over with pregnancy - weight gain, striae, HTN, fatquyre, GTT.

Question 21

What are the differentiating clinical features of cushings vs pregnancy?

Answer 21

• Proximal myopathy
• Easy bruising
• Osteopenia
• Early onset HTN
• Red/purple striae (vs pale in prengancy)

Question 22

Diagnosis of cushings in pergnancy

Answer 22

Pregnancy specific ranges for plasma and urinary cortisol
Low ACTGH, increased cortisol which does not response to high dose dexamethasone test
US, CT NRU of the adrenals +/- CT/MRI head

Question 23

Effect of cushings on pregnancy

Answer 23

Fetal: Miscarriage, FGR, PTL, stillbirth, neonatal death
Maternal: GDM, HTN, PET, wound infection, heart failure, psychiatric, maternal mortality

Question 24

What is 1st line treatment for cushings?

Answer 24

1st Surgical treatment
2nd Medical Metyrapone (high BP), cyproheptadine, ketoconazole (terateogenciity) - limited evidence in prennancy

Question 25

What is Conn's syndrome, 2 main causes?

Answer 25

Primary hyperaldosteronism Bilateral idiopathic hyperaldosteronism 60-70% Unilateral adrenal adenoma 30-40% Rare: Adrenal carcinoma, adrenal aldosterona secreting adenoma

Question 26

Incidence of hyperaldosteronism of non pregnant patients with HTN

Answer 26

0.7% Very few in pregnancy

Question 27

Clinical features of Conns Disease

Answer 27

HTN Low K

Question 28

How much does renin activity increase in pregnancy?

Answer 28

4 fold by 8 weeks 7 fold by 3rd trimester

Question 29

How much do aldosterone levels increase in pregnancy?

Answer 29

3-8 fold in 1st and 2nd trimester, plateau by 3rd

Question 30

Who to consider Conn's syndrome in?

Answer 30

HTN with hypokalaemia Resistant HTN especially before 20 weeks

Question 31

How to Dx Conns

Answer 31

Potassium - likely low Renin - supressed Aldosterone - high USS adrenal glands or MRI

Question 32

Management Conn's sydrome

Answer 32

- Manage BP - Labetolol, nifedipine, methldopa - K sparing diuretic - Consider Amiloride - Consider surgery

Question 33

Can you give spironolactone in pregnancy?

Answer 33

No anti-androgen effects, feminisation of female offspring FGR

Question 34

What type of inheritance is congenital adrenal hyperplasia (CAH)

Answer 34

Autosomal recessive - impair cortisol synthesis due to enzyme deficiency

Question 35

Most common enzyme deficiency in CAH?

Answer 35

Mutation in CRP21A2 gene, ecoding for 21-hydroxylase deficiency → reduced cortisol and aldosterone - salt losing CAH Measure 17 Hdyroxyprogesterone

Question 36

The second most common enzyme deficiency in CAH, how may they present?

Answer 36

11-B hydroxylase (8-9%) - High doeoxycortisol, mineralcorticoid activity, high BP Measure androstenedione

Question 37

How can classical (21HD) present in girls?

Answer 37

Sexual ambiguity: high adrenal androgen, cliterol enlargement, labial fusion. 75% present with salt wasting crisis

Question 38

How does non classical CAH present?

Answer 38

Simular to PCOS Hirsutism, primary/secondary amenorrhoea, anovulatory infertility

Question 39

What is the probability of a child have CAH in mother has 1) Classic CAH 2) non Classical CAH

Answer 39

Classical: 1/200 Non Classical 1/250

Question 40

Risk of CAH on pregnancy?

Answer 40

Few Cases - Increased risk miscarriage, PET, FGR, GDM C/S of android shaped pelvis

Question 41

What is the most common glucocorticoid used in pregnancy + classical CAH ?

Answer 41

Hydrocortisone, continue pre-pregnancy dose. Doe not stop virilisation (Dexamathsone should not be used)

Question 42

How common is pheochromocytoma in pregnancy and outside pregnancy?

Answer 42

0.1% non pregnancy with HTN Very rare in pregnancy

Question 43

Clinical features of phaeochromocytoma

Answer 43

- HTN - Headache - Palpitations - Sweating - Anxiety - Vomiting - Glucose intolerance

Question 44

Phaepchromocytoma is a tumour from which part of the adrenal gland?

Answer 44

Adrenal medulla, secreting excess carecholarmines 10% bilateral 10% extra adrenal 10% malignant

Question 45

Effect of pheochromocytoma on pregnancy

Answer 45

Fetal FGR, Fetal hypoxia, death (25% undiagnosed, 11% diagnosed), abruptions Maternal 17% death, arrhythmia, CV event, pulmonary oedema

Question 46

Diagnosis phaeo

Answer 46

Catecholamine excess - 24 hr urine collection for plasma free metaneprines. Plasma catecholamines If high → USS, CT, MNIR to find tumour

Question 47

False positive catecholamine can occur if patient taking which drugs?

Answer 47

TCAs - methyldopa, labetolol

Question 48

Management of pheochromocytoma in pregnancy?

Answer 48

1) Surgical resection, optimally before 24 weeks 2) a blockade (pehnoxybenzamine, prazosin, doxazoin) to control HTN, then B blockade to control tachycardia - adequate alpha blockade for at least 3 days before surgery

Question 49

Preferred mode of delivery with phaeochromocytoma?

Answer 49

C/S - risk of acute haemodynamic instability Hypertensive crisis can be triggered by some medication - Metoclopramide, morphine Synto - hypotension and tachycardia

Question 50

Treatment of choice for hypertensive crisis on background pheo?

Answer 50

IV Sodium nitroprusside - rapid acting vasodilator