Curriculum standards
Knowledge guides
LG17: Pituitary, hypothalamus, and electrolyte disorders
Key presentations and conditions
Advanced Trainees will have a comprehensive depth of knowledge of these presentations and conditions.
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.
Epidemiology, pathophysiology, and clinical sciences
Advanced Trainees will have a comprehensive depth of knowledge of the principles of the foundational sciences.
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.
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.
Presentations
- Abdominal pain
- Anosmia
- Dehydration
- Delayed or precocious puberty
- Dizziness
- Galactorrhoea
- Headaches
- Hypernatraemia / Hyponatraemia
- Hyperphagia / Anorexia
- Hypotension
- Nausea
- Neonatal hypoglycaemia
- Polyuria, polydipsia
- Short stature
- Visual loss or vision changes
- Vomiting
- Weight gain or weight loss
Conditions
- Pituitary lesions, such as:
- craniopharyngioma
- pars intermedia cysts
- Rathke cleft cysts
Anterior pituitary hormone deficiencies
- Acquired pituitary hormone deficiencies:
- craniopharyngioma and other space occupying lesions, such as germinoma and pilocytic astrocytoma
- post-surgery
- Congenital hypopituitarism (multiple pituitary hormone deficiencies):
- ectopic posterior pituitary
- genetic causes of hypopituitarism:
- HESX
- PIT1
- PROP1
- interrupted pituitary stalk syndrome
- septo-optic dysplasia
- Pituitary hormone deficiencies:
- ACTH deficiency – central hypoadrenalism
- growth hormone (GH) deficiency
- gonadotrophin deficiency (hypogonadotrophic hypogonadism)
- Kallmann syndrome
- thyroid-stimulating hormone (TSH) deficiency – central hypothyroidism
- Prader–Willi syndrome
Disorders of pituitary hormone excess
- Cushing syndrome
- Pituitary gigantism / acromegaly:
- XLAG
- Prolactinoma:
- AIP
- FIPA
- MEN1
- Thyrotropinomas
Disorders of salt and water balance
- Arginine vasopressin deficiency (AVP-D)
- Arginine vasopressin resistance (AVP-R)
- Cerebral salt wasting (CSW)
- Congenital or acquired:
- germ cell tumour
- Langerhans cell histiocytosis
- Pontine myelinosis (rapid correction of chronic hyponatraemia)
- Syndrome of inappropriate antidiuretic hormone secretion (SIADH)
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
Non-functioning pituitary adenomas
- Functional pituitary adenomas:
- corticotropinomas secreting (Cushing disease)
- somatropinomas secreting (acromegaly)
- thyrotropinomas secreting
- Pituitary apoplexy
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
- Anatomy of hypothalamic-pituitary connections
- Causes and treatments for electrolyte, hypothalamus, and pituitary gland disorders
- Feedback systems for adrenal, gonads, thyroid, and growth hormone
- Functions of the hypothalamus
- Growth hormone’s role in childhood and adult life
- Histology of the normal pituitary gland and of pituitary tumours
- Insulin-like growth factors' (IGFs’) roles and their binding proteins in growth and differentiation
- Natural history of pituitary tumour types
- Normal and abnormal anatomy, embryology, and physiology of the hypothalamus and pituitary gland
Acromegaly
- Causes of elevated IGF-1
- Differentiate between excess growth hormone secretion from functioning pituitary tumour and that of secondary to excess growth hormone-releasing hormone
- Indication for pituitary imaging in acromegaly
- Properties of serum growth hormone and IGF-1 assays
- Somatostatin receptor subclasses, and relevance to therapeutics
- Somatostatin’s role in negative regulation of somatotrope
Diabetes insipidus
- Desmopressin (DDAVP) pharmacology
- Differential diagnoses, including nephrogenic diabetes insipidus and chronic water excess (primary polydipsia)
- Loss of thirst regulation secondary to pituitary / hypothalamic disease and/or surgery
- Management principles of diabetes insipidus unmasked by cortisol and/or thyroid hormone replacement
- Primary causes, including genetics
- Regulation of salt and water balance, including regulation of free water clearance by cortisol and thyroid hormone
- Salt and water balance and limitations of measuring electrolytes, osmolality, and urinary specific gravity, and measurement of strict fluid balance
- Secondary causes and natural history of diabetes insipidus, including:
- infiltrative disorders
- metabolic cause
- surgery
- trauma
- tumours
Prolactinoma
- The mechanism of differing dopamine agonists, particularly with respect to dosing and side effects
- The role of dopamine in negative regulation of lactotrophs
Acromegaly
- Glucose suppression test for diagnosis of acromegaly
- IGF-1 assays
- Interpret glucose suppression test for diagnosis of acromegaly
- Properties of serum prolactin assays
- Serum growth hormone
Investigations
- Ophthalmoscopy of optic nerve
- Principles of and indications for imaging of the hypothalamus and pituitary:
- CT scan
- electrolytes
- MRI
- Properties, principles, and indications for basal and dynamic biochemical investigation of hypothalamo-pituitary disease, including dynamic testing, such as:
- corticotropin-releasing factor
- glucagon / arginine
- gonadotropin-releasing hormone (GnRH)
- OGT for GH excess
- overnight dexamethasone test
- synacthen testing
- thyrotropin-releasing hormone (TRH)
- water deprivation and stimulated copeptin
- Visual field testing and more complex field testing
Procedures
- Clinical assessment of thyroid adequacy and over-replacement
- Pituitary irrradiation and pituitary surgery
- Pre-, peri- and postoperative management of patients with pituitary disease, with emphasis on management of endocrine disturbances
Prolactinoma
- Indication for pituitary imaging in hyperprolactinaemia
- Properties of serum prolactin assays
- Causes and treatments for disorders of the hypothalamus and pituitary
- Differentiate between hyperprolactinaemia from functioning pituitary tumour versus ‘stalk effect’
Central hypoadrenalism
- Adrenal hormone replacement pharmacology
- Monitoring difficulties without feedback hormone
- Over-replacement signs
- Requirements for physical stress and illness
- Stress replacement and precautions
Central hypothyroidism
- Monitoring difficulties
- Over-replacement signs
- Thyroid hormone replacement pharmacology
Functioning pituitary tumours
- Treatments / pharmacology of dopamine agonists, including:
- bromocriptine
- cabergoline
- lanreotide
- octreotide
- somatostatin analogues
GH deficiency
- IGFs’ role and their binding proteins in growth and differentiation