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Curriculum standards

Knowledge guides

LG19: Adrenal disorders

Key presentations and conditions

Advanced Trainees will have a comprehensive depth of knowledge of these presentations and conditions.

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Less common or more complex presentations and conditions

Advanced Trainees will understand these presentations and conditions.

Advanced Trainees will understand the resources that should be used to help manage patients with these presentations and conditions.

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Epidemiology, pathophysiology, and clinical sciences

Advanced Trainees will have a comprehensive depth of knowledge of the principles of the foundational sciences.

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Investigations, procedures, and clinical assessment tools

Advanced Trainees will know the scientific foundation of each investigation and procedure, including relevant anatomy and physiology. They will be able to interpret the reported results of each investigation or procedure.

Advanced Trainees will know how to explain the investigation or procedure to patients, families, and carers, and be able to explain procedural risk and obtain informed consent where applicable.

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Important specific issues

Advanced Trainees will identify important specialty-specific issues and the impact of these on diagnosis and management and integrate these into care.

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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:
      • glucocorticoid use
    • 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:
      • ACTH dependent:
    • 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