Adrenal Disease and pregnancy TOG 2021 Flashcards

1
Q

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

A

Primary: Adrenocoritical Disease
Secondary: ACTH
Tertiaty: CRH

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2
Q

Which hormones are deficiency in prrimary/secondary/tertiaty

A

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

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3
Q

Most common cause or primary adrenal insufficiency?

A

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

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

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4
Q

At how many weeks gestation does cortisol peak?

A

26 weeks

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5
Q

The placenta increases the production of which hormones?

A

CRH and ACTH

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6
Q

Symptoms of primary adnreal insufficiency?

A

Weight loss
Vomiting
Hyperpigmentation
Hypoglycaemia
Hyponatraemia
Hyperkalaemia

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

Most cases Dx before pregnancy

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7
Q

How to diagnose primary adrenal insufficiency?

A

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

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8
Q

Management of primary addisons in pregnancy?

A

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

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9
Q

How can acute AI crisis (addisonian crisis present)

A

Abdo pain
Vomiting
Shock

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10
Q

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

A

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

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11
Q

Which patients need intrapartum hydrocortisone?

A

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

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12
Q

What does of hydrocortisone should be given?

A

IV 50-100mg TDS for 24 hours

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13
Q

How to treat acute adrenal insufficiency in pregnancy?

A
  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
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14
Q

What are the sick day rules?

A
  • Wear medical alert bracelet/neckalce
  • Double dose GC in cases of fever/ilnness requiring bedrest
  • Provide IM HC self-administer if gastroenteritis/fasting
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15
Q

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

A

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

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16
Q

Pregnancy outcome with primary adrenal insufficiency:

A

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

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17
Q

What is Cushing syndrome?

A

Increased cortisol
Can be ACTH dependant/independant

Untreated rarely become pregnanct

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18
Q

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

A

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

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19
Q

What is pregnancy associated Cushing syndrome?

A

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

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20
Q

Why is diagnosis of cushings difficult in pregnancy?

A

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.

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21
Q

What are the differentiating clinical features of cushings vs pregnancy?

A
  • Proximal myopathy
  • Easy bruising
  • Osteopenia
  • Early onset HTN
  • Red/purple striae (vs pale in prengancy)
22
Q

Diagnosis of cushings in pergnancy

A

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

23
Q

Effect of cushings on pregnancy

A

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

24
Q

What is 1st line treatment for cushings?

A

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

25
Q

What is Conn’s syndrome, 2 main causes?

A

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

26
Q

Incidence of hyperaldosteronism of non pregnant patients with HTN

A

0.7%
Very few in pregnancy

27
Q

Clinical features of Conns Disease

A

HTN
Low K

28
Q

How much does renin activity increase in pregnancy?

A

4 fold by 8 weeks
7 fold by 3rd trimester

29
Q

How much do aldosterone levels increase in pregnancy?

A

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

30
Q

Who to consider Conn’s syndrome in?

A

HTN with hypokalaemia
Resistant HTN especially before 20 weeks

31
Q

How to Dx Conns

A

Potassium - likely low
Renin - supressed
Aldosterone - high

USS adrenal glands or MRI

32
Q

Management Conn’s sydrome

A
  • Manage BP - Labetolol, nifedipine, methldopa - K sparing diuretic
  • Consider Amiloride
  • Consider surgery
33
Q

Can you give spironolactone in pregnancy?

A

No anti-androgen effects, feminisation of female offspring
FGR

34
Q

What type of inheritance is congenital adrenal hyperplasia (CAH)

A

Autosomal recessive - impair cortisol synthesis due to enzyme deficiency

35
Q

Most common enzyme deficiency in CAH?

A

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

Measure 17 Hdyroxyprogesterone

36
Q

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

A

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

Measure androstenedione

37
Q

How can classical (21HD) present in girls?

A

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

38
Q

How does non classical CAH present?

A

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

39
Q

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

A

Classical: 1/200
Non Classical 1/250

40
Q

Risk of CAH on pregnancy?

A

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

41
Q

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

A

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

42
Q

How common is pheochromocytoma in pregnancy and outside pregnancy?

A

0.1% non pregnancy with HTN
Very rare in pregnancy

43
Q

Clinical features of phaeochromocytoma

A
  • HTN
  • Headache
  • Palpitations
  • Sweating
  • Anxiety
  • Vomiting
  • Glucose intolerance
44
Q

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

A

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

45
Q

Effect of pheochromocytoma on pregnancy

A

Fetal FGR, Fetal hypoxia, death (25% undiagnosed, 11% diagnosed), abruptions

Maternal 17% death, arrhythmia, CV event, pulmonary oedema

46
Q

Diagnosis phaeo

A

Catecholamine excess - 24 hr urine collection for plasma free metaneprines. Plasma catecholamines

If high → USS, CT, MNIR to find tumour

47
Q

False positive catecholamine can occur if patient taking which drugs?

A

TCAs - methyldopa, labetolol

48
Q

Management of pheochromocytoma in pregnancy?

A

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

49
Q

Preferred mode of delivery with phaeochromocytoma?

A

C/S - risk of acute haemodynamic instability

Hypertensive crisis can be triggered by some medication - Metoclopramide, morphine
Synto - hypotension and tachycardia

50
Q

Treatment of choice for hypertensive crisis on background pheo?

A

IV Sodium nitroprusside - rapid acting vasodilator