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Endocrinology

Acromegaly is a very rare endocrine disorder with a prevalence of about 1 in 200,000. Typically diagnosed between the ages of 40 and 60, it affects both genders equally. It results from excessive secretion of growth hormone, mostly due to a pituitary adenoma, leading to increased levels of IGF-1. Symptoms include distinct facial changes, deep voice, carpal tunnel syndrome, and organomegaly. Diagnosis involves glucose tolerance testing and MRI. Management may include surgery, radiotherapy, and pharmacotherapy with somatostatin analogs or GH receptor antagonists.

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Endocrinology

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  1. Endocrinology Acromegaly

  2. Acromegaly • Very rare • Prevalence in the order of 1 in 200,000 • Usually diagnosed between age 40 and 60 • No difference in gender susceptibility • Insidious onset

  3. Pathogenesis • Most commonly caused by pituitary adenoma • Increased secretion of growth hormone • Acts in liver to release IGF-1 (insulin-like growth factor)

  4. So what are the symptoms? • Tumour • Growth hormone • IGF-1

  5. On examination • Characteristic facial appearance: Coarse, Frontal bossing, ↑sinuses, ↑ tongue, Prognathism (jaw protrusion), separation of teeth • Deep voice • Carpal tunnel syndrome • Hand & foot enlargement • Visual fields (bitemporalhemianopia) • Organomegaly: Goitre, Hepatosplenomegaly

  6. Investigations • Glucose tolerance test with measurement of growth hormone level. • (Should be inhibited by glucose) • (Growth hormone secretion is episodic and so a random GH alone is unlikely to be useful) • Evidence of other pituitary involvement • MRI scan to identify adenoma

  7. Management • Surgery: trans-sphenoidaladenomectomy or craniotomy for very large tumours. • Pituitary radiotherapy: useful if tumour is not fully removed and reduces GH progressively over years. • Drugs: • Somatostatin analogues (octreotide, lanreotide) suppress GH in 60% • Dopamine agonists (bromocriptine, cabergoline) lower but rarely normalize GH • GH receptor antagonist (pegvisomant) normalizes IGF-I in >90% of pts.

  8. Bonus marks management Management of: • ↑ Cardiovascular morbidity & mortality – from HTN, impaired GTT (25%), Diabetes Mellitus (10%) • ↑ Cardiac failure (heart muscle disease), ↑IHD, ↑CVD • Obstructive sleep apnoea • Arthropathy (50%) • Osteoporosis • Colorectal cancer Complication of treatment: hypopituitarism

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