Presentations
- Atypical genitalia
- Cardiac arrythmia
- Electrolyte disturbance
- Fatigue
- Fractures
- Hirsutism
- Hypertension
- Hypotension
- Incidental adenoma
- Menstrual disturbance
- Metabolic syndrome
- Palpitations
- Sweating / Flushing
- Weight gain / Obesity
- Weight loss
Conditions
- Adrenal nodular disease:
- functional:
- aldosterone-secreting tumours
- catecholamine-producing tumours:
- paraganglioma
- phaeochromocytoma
- Conn syndrome / hyperaldosteronism
- Cushing syndrome / hypercortisolism
- feminising tumours
- virilising tumours
- non-functional:
- macronodular adrenal hyperplasia
- Hypercortisolism:
- ACTH dependent
- ACTH independent
- Isolated glucocorticoid deficiency
- Primary adrenal insufficiency:
- adrenal hypoplasia congenita
- autoimmune:
- Addison’s disease
- polyglandular autoimmune syndromes (APS 1, APS 2)
- congenital adrenal hyperplasia
- drug-related:
- idiopathic
- infarction:
- adrenal haemorrhage of the newborn
- infection
- Secondary adrenal insufficiency:
- ACTH / CRH deficiency
- ceased glucocorticoid therapy
- Virilising or sex steroid excess syndromes
For each presentation and condition, Advanced Trainees will know how to:
Synthesise
- recognise the clinical presentation
- identify relevant epidemiology, prevalence, pathophysiology, and clinical science
- take a comprehensive clinical history
- conduct an appropriate examination
- establish a differential diagnosis
- plan and arrange appropriate investigations
- consider the impact of illness and disease on patients and their quality of life when developing a management plan
Manage
- provide evidence-based management
- prescribe therapies tailored to patients' needs and conditions
- recognise potential complications of disease and its management, and initiate preventative strategies
- involve multidisciplinary teams
Consider other factors
- identify individual and social factors and the impact of these on diagnosis and management
Conditions
- Adrenal cancer:
- adrenal metastasis
- primary
- Inherited adrenal disorders:
- isolated glucocorticoid deficiency:
- familial glucocorticoid resistance
- isolated mineralocorticoid deficiency
- X-linked adrenoleukodystrophy
For each presentation and condition, Advanced Trainees will know how to:
Synthesise
- recognise the clinical presentation
- identify relevant epidemiology, prevalence, pathophysiology, and clinical science
- take a comprehensive clinical history
- conduct an appropriate examination
- establish a differential diagnosis
- plan and arrange appropriate investigations
- consider the impact of illness and disease on patients and their quality of life when developing a management plan
Manage
- provide evidence-based management
- prescribe therapies tailored to patients' needs and conditions
- recognise potential complications of disease and its management, and initiate preventative strategies
- involve multidisciplinary teams
Consider other factors
- identify individual and social factors and the impact of these on diagnosis and management
Clinical science
- Anatomy and function of the sympathetic / parasympathetic ganglia
- Anatomy, histopathology, and physiology of the normal and the abnormal adrenal gland (cortex and medulla)
- Epidemiology and natural history of adrenal hormone excess disorders
- Epidemiology of genetic predisposition to adrenal disease
- Fetal adrenal gland development
- Function and regulation of the adrenal gland
- Hypothalamic-pituitary-adrenal axis
- Prenatal treatment of CYP21 deficiency
- Presentations of adrenal disease at different ages
- Steroidogenesis pathway
Diagnostic workup
- Aetiology, clinical manifestations, and pathology of adrenal hormone deficiency, particularly hypercortisolism
- Aetiology, clinical mandifestations, and pathology of adrenal hormone excess:
- hyperaldosteronism
- hypercortisolism:
- Cushing syndrome
- ectopic ACTH production
- iatrogenic
- nodular adrenal hyperplasia
- virilising tumours
- Genetic disorders affecting the adrenal gland
- History, physical examination, and diagnostic workup (laboratory and imaging studies) of people suspected of presenting with adrenal disease, particularly focusing on growth data
- Limitations, indications, and interpretation of biochemical assays in the diagnostic workup of adrenal disorders:
- complex diagnostic workup and pitfalls in Cushing syndrome
- diagnostic workup and pitfalls in hyperaldosteronism
Surgical and medical management considerations
- Emergency and long-term management of hypoadrenalism:
- prescribing and monitoring of glucocorticoids and/or mineralocorticoids
- screening for associated disorders
- Indications and need for surgical management in:
- Cushing disease (transsphenoidal resection)
- Cushing syndrome (adrenal tumour resection, adrenalectomy)
- Management of congenital adrenal hyperplasia and consideration of:
- biochemistry to guide long-term management using androgen and renin levels, including capillary profiles over 24 hours of 17-hydroxyprogesterone
- interpreting growth and development in the follow-up of congenital adrenal hyperplasia
- other measures to guide management or treatment of congenital adrenal hyperplasia, such as:
- ambulatory blood pressure monitoring
- bone age
- gonadotropin-releasing hormone (GnRH) analogue therapy
- stress replacement of glucocorticoids and precautions
- Medical management of adrenal disorders of excess hormone production:
- monitoring of treatment efficacy
- monitoring of underlying condition
- Pharmacological principles of medications used in adrenal disorders and their requirements at different ages:
- alpha receptor antagonists
- glucocorticoid treatment (e.g., for chronic inflammatory diseases or malignancies)
- mineralocorticoid receptor antagonists
- mineralocorticoids and salt replacement
- steroid biosynthesis inhibitors, such as ketoconazole, metyrapone, and mitotane
Investigations
- Dynamic endocrine testing:
- specific understanding of indications (and contraindications):
- short synacthen test
- workup for suspected Cushing syndrome, such as:
- 24-hour urinary cortisol
- dexamethasone suppression tests
- midnight salivary cortisol testing
- Imaging:
- adrenal ultrasound
- CT adrenal protocol
- functional PET imaging
- MRI (with contrast)
- other, such as inferior petrosal sinus sampling
- Laboratory biochemistry:
- genotyping for CAH and interpretation for phenotype-genotype correlation
- interpretation of biochemical testing in the clinical context
- properties, principles, and indications for biochemical investigation of adrenal disease, including the hypothalamic-pituitary-adrenal axis:
- baseline testing
- dynamic testing
- specific understanding of the timing, patient preparation, and assay platforms suited for adrenal hormone testing
- Less commonly performed tests, such as:
- fludrocortisone suppression test
- saline suppression test
Procedures
- Indications for adrenalectomy
- Indications for adrenal vein sampling
- Pre-, peri- and postoperative management of patients with adrenal disease, with particular emphasis on adrenal crisis and hypertensive crisis mitigation
Evidence-based practice
- Evidence for best practice, and applying this using clinical judgement and individual circumstances, in partnership with patients
- Multidisciplinary care / review from centres of expertise wherever necessary
General management considerations
- Clinical risk with intercurrent illness management planning when applicable, such as:
- medical alert bracelet
- sick day steroid plan
- Education of families regarding the time-course of polyglandular autoimmune disorders, including risk of Addisonian crisis
- Environmentally sustainable practices in clinical care
- Impact of adrenal disease and/or genetic diagnoses on the patient and their family or carers
- Long-term management of patients with adrenal disorders, including optimisation of growth, puberty, and quality of life measures
- Longitudinal and multidisciplinary care needs of people with adrenal disease
- Options for improving equitable access to comprehensive care for individuals, such as:
- multidisciplinary involvement
- resources appropriate to patients’ language and cultural needs
- use of telehealth and other digital health tools
- Pre-, peri- and postoperative management of patients with adrenal disease:
- adequate glucocorticoid replacement in adrenal insufficiency and appropriate blockade in phaeochromocytoma
- Screening and genetic counselling of patients with inherited adrenal disease and referral of their family members, if appropriate, and with informed consent
