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Acromegaly Drug Today Medical Times

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Acromegaly is a rare disease whose prevalence is about 7 cases per 100,000, with an annual incidence of 0.3 cases per 100,000. The characteristics of Acromegaly include exaggerated somatic growth arising from increased growth hormone (GH) secretion in an adult after the epiphyses (rounded end of long bones) have fused. Excess GH in children leads to Gigantism.

About 98% of the cases of Acromegaly happen due to GH secreting adenoma of the anterior pituitary (a pea-sized endocrine gland at the base of the brain), that is usually benign. The tumour may secrete GH alone or GH and prolactin.

Excessive GH stimulates the production of insulin-like growth factor I (IGF-I) mainly in the liver, which in turn stimulates general tissue growth. This is referred to as GH/IGF-1 Axis. The other rare causes of Acromegaly are hypothalamic tumours, ectopic secretion from carcinoid tumours or non small-cell lung cancers, that release growth hormone-releasing hormone (GHRH) and cause secondary increase of GH.

The symptoms of Acromegaly include skeletal and soft tissue growth and deformities, cardiac, respiratory, neuromuscular, endocrine and metabolic complications due to the effect of the GH and IGF-1 on various tissues.

Acromegaly patients may have facial changes such as large lips, nose and tongue, frontal bossing of the skull, prognathism (protrusion of the lower jaw), teeth separation. As a result of excessive bone and soft tissue growth in extremities, requirement for increased shoe and ring size may become necessary. This may be accompanied by increased sweating, heat intolerance, oiliness of the skin, fatigue and weight gain.

Arthritis due to bone growth, skin thickening in the face and hands, sleep apnea due to large tongue and soft tissue growth in the airway which may sometimes complicate endotracheal intubation during medical procedures when the patient cannot breathe on their own.

The diagnosis of Acromegaly may be delayed by at least ten years due to insidious onset and the slow progression of the signs of Acromegaly. Mean age at diagnosis is 40-45 years. At the time of diagnosis, about 75% of the patients have macro-adenoma (tumour size over 1 cm) of pituitary.

The best single test is the measurement of IGF-1, because of its long half life and stable serum levels as compared to GH. GH levels may also be measured.

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