acromegalia


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ac·ro·meg·a·ly

(ak'rō-meg'ă-lē), [MIM*102200]
A disorder marked by progressive enlargement of peripheral parts of the body, especially the head, face, hands, and feet, resulting from excessive secretion of somatotropin; organomegaly and metabolic disorders occur; diabetes mellitus may develop.
Synonym(s): acromegalia
[acro- + G. megas, large]

acromegalia

acromegaly

A disease caused by excess growth hormone (GH) by anterior pituitary or extrapituitary origin that occurs after the closure of epiphyseal plates after puberty. Excess GH production that precedes the closure of the epiphyseal growth plates results in gigantism in afflicted children and adolescents

Aetiology
Secretion of GH by anterior pituitary; GH-releasing hormone by hypothalamic tumours; ectopic GH production by small-cell carcinoma of lung, carcinoids, islet cell tumours, adrenal adenomas or other endocrine tumours.
 
Clinical findings
Coarsened, enlarged facies, lips, tongue, nose, jaw, hands, feet, supraorbital ridge and frontal bones, widely spaced teeth, bone proliferation in extremities, soft tissue thickening, hyperhidrosis, macroglossia, headache, amenorrhoea, impotence, somnolence, moodiness, glucose intolerance, cardiomegaly with heart failure (acromegalic heart disease), hypertension, carpal tunnel syndrome, sleep apnoea.

Management
Surgery—endonasal transphenoidal excision of the pituitary tumour; medical—somatostatin analogues (e.g., octreotide, lanreotide, which inhibit growth hormone production); in unresponsive cases dopamine agonists may work.
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