Hypothalamic-Pituitary Axis Disorder

Acromegaly

•    Excess growth hormone (GH) IGF-1

•    Features: large hands/feet, coarse facial features, prognathism (large jaw), sweating, visual field defects 

                                                                                (bitemporal hemianopia), glucose intolerance/diabetes

•    Diagnosis:

o    Screening: IGF-1

o    Confirmatory: oral glucose tolerance test – GH fails to suppress

o    Pituitary MRI to localise adenoma

•    Treatment:

o    First-line: trans-sphenoidal surgery

o    Alternatives: somatostatin analogues (octreotide), GH receptor antagonists (pegvisomant), radiotherapy


Prolactinoma

•    Most common functioning pituitary tumour

•    Features: galactorrhoea, amenorrhoea, infertility, low libido, visual field defects (if large)

•    Diagnosis:

o    serum prolactin (rule out pregnancy, hypothyroidism, drugs)

o    Pituitary MRI

•    Treatment:

o    First-line: dopamine agonistscabergoline > bromocriptine

o    Surgery if medical therapy fails or compressive symptoms


Hypopituitarism

•    Deficiency of one or more pituitary hormones

•    Features: fatigue, loss of libido, amenorrhoea, cold intolerance, hypotension

•    Labs: cortisol, TSH/FT4, LH/FSH, GH/IGF-1

•    Causes: pituitary tumours, apoplexy, Sheehan’s, trauma, irradiation

•    Treatment:

o    Replace hormones in correct order: hydrocortisone first, then thyroxine ± sex steroids/GH


Diabetes Insipidus (Cranial)

•    ADH deficiency inability to concentrate urine

•    Features: polyuria, polydipsia, nocturia

•    Diagnosis:

o    Water deprivation test: no urine concentration

o    Responds to desmopressin (confirms cranial DI)

•    Causes: idiopathic, neurosurgery, trauma, tumours

•    Treatment: desmopressin (DDAVP)


Pituitary Apoplexy

•    Sudden haemorrhage/infarction of pituitary (often into adenoma)

•    Features: sudden headache, visual loss, ophthalmoplegia, shock

•    Emergency: MRI brain, immediate IV hydrocortisone, supportive care ± surgery


Non-Functioning Pituitary Adenoma

•    Most common type of pituitary adenoma

•    No hormone excess

•    Features: mass effect – headache, bitemporal hemianopia, hypopituitarism

•    Diagnosis: MRI + full pituitary hormone panel

•    Treatment: trans-sphenoidal resection ± radiotherapy


Cushing’s Disease (Pituitary ACTH Excess)

•    ACTH-secreting pituitary adenoma cortisol

•    Features: central obesity, moon face, purple striae, proximal weakness, hypertension

•    Diagnosis:

o    1 mg dexamethasone suppression test: no suppression

o    Confirm with high-dose dexamethasone or CRH test

o    MRI pituitary

•    Treatment: trans-sphenoidal resection ± ketoconazole/metyrapone


Empty Sella Syndrome

•    Herniation of subarachnoid space into sella compressed/flattened pituitary

•    Primary: idiopathic (often incidental)

•    Secondary: post-surgery/radiation

•    May cause hypopituitarism or be asymptomatic


Sheehan’s Syndrome

•    Postpartum pituitary infarction due to massive blood loss/hypotension

•    Features: failure to lactate, amenorrhoea, fatigue

•    May progress to full hypopituitarism

•    Requires lifelong hormone replacement


Kallmann Syndrome

•    Congenital GnRH deficiency + anosmia

•    Features: delayed puberty, infertility, hypogonadotropic hypogonadism

•    Often associated with midline defects (cleft lip/palate)

•    More common in males


Investigation Principles

•    Visual field testing – essential in macroadenomas

•    Hormone panels – test all axes: cortisol, TSH/FT4, LH/FSH, prolactin, IGF-1

•    MRI pituitary – gold standard for structural lesions

•    Dynamic testing – suppression/stimulation tests where needed (e.g. OGTT for GH, dexamethasone for ACTH)


Treatment Principles

•    Trans-sphenoidal surgery – first-line for macroadenomas

•    Medical therapy – somatostatin analogues (acromegaly), dopamine agonists (prolactinoma), 

                                                                                                steroidogenesis blockers (Cushing’s)

•    Hormone replacement – replace cortisol before thyroxine in hypopituitarism

•    Long-term follow-up: regular TFTs, cortisol, IGF-1, visual fields, MRI

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Author & Educational Disclaimer


Author:

Dr Phillip Cockrell BM FRCP DipClinEd


Dr Phillip Cockrell is a UK Consultant Physician in Internal Medicine, currently working at Queen Alexandra Hospital, Portsmouth University Hospitals NHS Trust. He has previously worked as a registrar across Intensive Care Medicine, Gastroenterology, Cardiology, Stroke Medicine, Acute Medicine, and Respiratory Medicine.


He has held senior leadership roles including Associate Clinical Director of the Acute Medical Unit, Clinical Director of Internal Medicine, and Chief of Medicine. Dr Cockrell has over 15 years’ experience in postgraduate medical education, having lectured extensively across the MRCP syllabus and contributed to MRCP revision teaching and course development.


Dr Cockrell holds a Bachelor of Medicine (BM), Fellowship of the Royal College of Physicians (FRCP), and a Diploma in Clinical Education (DipClinEd). His teaching approach is based on structured consolidation of complex medical topics to support efficient and effective revision for postgraduate examinations.


Purpose of this content:

The material on this page is intended solely for educational purposes to support revision for the MRCP (UK) Part 1 examination. It reflects examination-relevant principles of internal medicine and is designed to aid learning and pattern recognition.


Medical disclaimer:

This content is designed for postgraduate medical examination revision and does not constitute medical advice, diagnosis, or treatment guidance and must not be used as a substitute for professional clinical judgement, local guidelines, or specialist consultation. Clinical decisions should always be made in the context of individual patient circumstances and current national guidance.