Adrenal Disease and pregnancy TOG 2021 Flashcards
What are the 3 main categories of adrenal insufficiency (Addison’s)
Primary: Adrenocoritical Disease
Secondary: ACTH
Tertiaty: CRH
Which hormones are deficiency in prrimary/secondary/tertiaty
Primary: Glucocorticoid, Mineralcorticoid
2nd/3rd: Glucocorticoid alone
Most common cause or primary adrenal insufficiency?
Autoimmune 70-90% (40% will have other autoimmune condition)
Other causes: Haemorrhage 2nd sepsis, major burns, lymphoma, mets and infections e.g. TV
At how many weeks gestation does cortisol peak?
26 weeks
The placenta increases the production of which hormones?
CRH and ACTH
Symptoms of primary adnreal insufficiency?
Weight loss
Vomiting
Hyperpigmentation
Hypoglycaemia
Hyponatraemia
Hyperkalaemia
Adrenal crisis may be triggrtered by stress - infection/delivery/CS
Most cases Dx before pregnancy
How to diagnose primary adrenal insufficiency?
Morning Cortisol levels (levels higher than non pregnant)
Raised ACTH
Loss of cortisol response to synthacthen test
Management of primary addisons in pregnancy?
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
How can acute AI crisis (addisonian crisis present)
Abdo pain
Vomiting
Shock
When can it occur with patients who do not have a known adrenal insufficiency?
Bilateral adrenal necrosis - haemorrhage, sepsis, adrenal vein thrombosis.
Which patients need intrapartum hydrocortisone?
5-20mg+ prednisolone (or equivalent)/day for 3 weeks +
What does of hydrocortisone should be given?
IV 50-100mg TDS for 24 hours
How to treat acute adrenal insufficiency in pregnancy?
- IV access
- Blood - ACTH, cortisol, serum electrolytes, glucose
- 2-3L NaCl 0.9% or 5% dextrose
- IV hydrocortisone 6-8hrly or continous
- Regular obs
- Treat cause
- Fetal assessment
- Taper IV hydrocortisone over 1-3 days
What are the sick day rules?
- Wear medical alert bracelet/neckalce
- Double dose GC in cases of fever/ilnness requiring bedrest
- Provide IM HC self-administer if gastroenteritis/fasting
GC and MC during delivery and postnatally
Delivery Day
Day 1
Day 2
D/C
Follow-up
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
Pregnancy outcome with primary adrenal insufficiency:
Well controlled - risk FGR
Pool controlled - PTL, C/S, poor wound healing, VTE and acute adrenal crisis
What is Cushing syndrome?
Increased cortisol
Can be ACTH dependant/independant
Untreated rarely become pregnanct
Most common cause of Cushing syndrome, in pregnancy and outside pregnancy?
In pregnancy: Adrenal adenoma 60% (do not secrete androgens, so pregnancy more likely)
Outside pregnancy: Pituitary dependent Cushing syndrome 70%
What is pregnancy associated Cushing syndrome?
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
Why is diagnosis of cushings difficult in pregnancy?
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.
What are the differentiating clinical features of cushings vs pregnancy?
- Proximal myopathy
- Easy bruising
- Osteopenia
- Early onset HTN
- Red/purple striae (vs pale in prengancy)
Diagnosis of cushings in pergnancy
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
Effect of cushings on pregnancy
Fetal: Miscarriage, FGR, PTL, stillbirth, neonatal death
Maternal: GDM, HTN, PET, wound infection, heart failure, psychiatric, maternal mortality
What is 1st line treatment for cushings?
1st Surgical treatment
2nd Medical Metyrapone (high BP), cyproheptadine, ketoconazole (terateogenciity) - limited evidence in prennancy
What is Conn’s syndrome, 2 main causes?
Primary hyperaldosteronism
Bilateral idiopathic hyperaldosteronism 60-70%
Unilateral adrenal adenoma 30-40%
Rare: Adrenal carcinoma, adrenal aldosterona secreting adenoma
Incidence of hyperaldosteronism of non pregnant patients with HTN
0.7%
Very few in pregnancy
Clinical features of Conns Disease
HTN
Low K
How much does renin activity increase in pregnancy?
4 fold by 8 weeks
7 fold by 3rd trimester
How much do aldosterone levels increase in pregnancy?
3-8 fold in 1st and 2nd trimester, plateau by 3rd
Who to consider Conn’s syndrome in?
HTN with hypokalaemia
Resistant HTN especially before 20 weeks
How to Dx Conns
Potassium - likely low
Renin - supressed
Aldosterone - high
USS adrenal glands or MRI
Management Conn’s sydrome
- Manage BP - Labetolol, nifedipine, methldopa - K sparing diuretic
- Consider Amiloride
- Consider surgery
Can you give spironolactone in pregnancy?
No anti-androgen effects, feminisation of female offspring
FGR
What type of inheritance is congenital adrenal hyperplasia (CAH)
Autosomal recessive - impair cortisol synthesis due to enzyme deficiency
Most common enzyme deficiency in CAH?
Mutation in CRP21A2 gene, ecoding for 21-hydroxylase deficiency → reduced cortisol and aldosterone - salt losing CAH
Measure 17 Hdyroxyprogesterone
The second most common enzyme deficiency in CAH, how may they present?
11-B hydroxylase (8-9%)
- High doeoxycortisol, mineralcorticoid activity, high BP
Measure androstenedione
How can classical (21HD) present in girls?
Sexual ambiguity: high adrenal androgen, cliterol enlargement, labial fusion.
75% present with salt wasting crisis
How does non classical CAH present?
Simular to PCOS
Hirsutism, primary/secondary amenorrhoea, anovulatory infertility
What is the probability of a child have CAH in mother has
1) Classic CAH
2) non Classical CAH
Classical: 1/200
Non Classical 1/250
Risk of CAH on pregnancy?
Few Cases
- Increased risk miscarriage, PET, FGR, GDM
C/S of android shaped pelvis
What is the most common glucocorticoid used in pregnancy + classical CAH ?
Hydrocortisone, continue pre-pregnancy dose. Doe not stop virilisation
(Dexamathsone should not be used)
How common is pheochromocytoma in pregnancy and outside pregnancy?
0.1% non pregnancy with HTN
Very rare in pregnancy
Clinical features of phaeochromocytoma
- HTN
- Headache
- Palpitations
- Sweating
- Anxiety
- Vomiting
- Glucose intolerance
Phaepchromocytoma is a tumour from which part of the adrenal gland?
Adrenal medulla, secreting excess carecholarmines
10% bilateral
10% extra adrenal
10% malignant
Effect of pheochromocytoma on pregnancy
Fetal FGR, Fetal hypoxia, death (25% undiagnosed, 11% diagnosed), abruptions
Maternal 17% death, arrhythmia, CV event, pulmonary oedema
Diagnosis phaeo
Catecholamine excess - 24 hr urine collection for plasma free metaneprines. Plasma catecholamines
If high → USS, CT, MNIR to find tumour
False positive catecholamine can occur if patient taking which drugs?
TCAs - methyldopa, labetolol
Management of pheochromocytoma in pregnancy?
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
Preferred mode of delivery with phaeochromocytoma?
C/S - risk of acute haemodynamic instability
Hypertensive crisis can be triggered by some medication - Metoclopramide, morphine
Synto - hypotension and tachycardia
Treatment of choice for hypertensive crisis on background pheo?
IV Sodium nitroprusside - rapid acting vasodilator
