Acromegaly is a chronic metabolic disorder caused by abnormal production of growth hormone after the skeleton and other organs finish growing. It most commonly affects adults in middle age, and can result in severe disfigurement, serious complicating conditions, and premature death if unchecked
Acromegaly is a syndrome that results when the anterior pituitary gland produces excess growth hormone (GH) after epiphyseal plate closure at puberty. A number of disorders may increase the pituitary's GH output, although most commonly it involves a GH producing tumor called pituitary adenoma, derived from a distinct type of cell (somatotrophs).The cause of the increased growth hormone release is usually a noncancerous (benign) tumor of the pituitary gland. The pituitary gland, which is located just below the brain, controls the production and release of several different hormones, including growth hormone.
Acromegaly occurs in about 6 of every 100,000 adults. Because of its insidious pathogenesis and slow progression, the disease is hard to diagnose in the early stages and is frequently missed for many years, until changes in external features, especially of the face, become noticeable. Excessive production of growth hormone in children causes gigantism rather than acromegaly.
The effects of acromegaly vary depending on the development stage of the stricken individual. If the condition arises after the end of natural growth, the victim's hands, feet, jaw, forehead, and ribcage will often grow outwards. But when the tumor arises earlier, in someone who hasn't yet reached full height, a phenomenon called gigantism occurs. This state causes continuous growth, ultimately leading to an abnormally tall stature in adulthood. The extremities also grow more than the rest of the body, leading to disproportionately large hands and feet, and a characteristic large-boned face.
Over time acromegaly leads to a host of systemic problems including arthritis, diabetes, and heart disease. The tumor itself, should it grow too large, can press against the optic nerves and cause blindness. Untreated acromegalics rarely live past middle-age.
Surgery to remove the pituitary tumor causing this condition corrects the abnormal growth hormone secretion in most patients. This surgery may not be available to patients in remote locations, so travel to a larger metropolitan area may be necessary for treatment.
Radiation of the pituitary gland is used for people who do not respond to the surgical treatment. However, the reduction in growth hormone levels after radiation is very slow.
The following medications may be used to treat acromegaly:
These medications may be used before surgery, or when surgery is not possible.
After treatment, periodic evaluation is necessary to ensure that the pituitary gland is working normally. Yearly evaluations are recommended.
The extreme height and distinctive features of gigantism have been irresistible to Hollywood. Actors Richard Kiel ("Jaws" in James Bond movies) and Matthew McGrory ("Karl the Giant" in Big Fish) both had acromegaly. Even those acromegalics in other professions frequently seem to end up on the big or little screens. André the Giant and Paul Wright, both professional wrestlers, and Gheorghe Muresan, a basketball player, have all had parts in more than one movie or TV show. Carel Struycken, who played Lurch in the Addams Family movies, was literally pulled off the street when a woman abandoned her car to run after him and offer him a part in Sergeant Pepper's Lonely Hearts Club Band.
Pathology of freaks
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