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Acromegaly facts for kids

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Acromegaly
Acromegaly facial features.JPEG
Facial features of a person with acromegaly. The cheekbones are pronounced, the forehead bulges, the jaw is enlarged, and facial lines are prominent.
Symptoms Enlargement of the hands, feet, forehead, jaw, and nose, thicker skin, deepening of the voice
Complications Type 2 diabetes, sleep apnea, high blood pressure
Usual onset Middle age
Causes Too much growth hormone
Diagnostic method Blood tests, medical imaging
Treatment Surgery, medications, radiation therapy
Medication Somatostatin analogue, GH receptor antagonist
Prognosis Usually normal (with treatment), 10 year shorter life expectancy (no treatment)
Frequency 6 per 100,000 people

Acromegaly is a disorder that results from excess growth hormone (GH) after the growth plates have closed. The initial symptom is typically enlargement of the hands and feet. There may also be enlargement of the forehead, jaw, and nose. Other symptoms may include joint pain, thicker skin, deepening of the voice, headaches, and problems with vision. Complications of the disease may include type 2 diabetes, sleep apnea, and high blood pressure.

Acromegaly is usually caused by the pituitary gland producing excess growth hormone. In more than 95% of cases the excess production is due to a benign tumor, known as a pituitary adenoma. The condition is not inherited from a person's parents.

Rarely acromegaly is due to a tumor in another part of the body. Diagnosis is by measuring growth hormone after a person has drunk a glucose solution, or by measuring insulin-like growth factor I in the blood. After diagnosis, medical imaging of the pituitary is carried out to determine if an adenoma is present. If excess growth hormone is produced during childhood, the result is the condition gigantism rather than acromegaly.

Treatment options include surgery to remove the tumor, medications, and radiation therapy. Surgery is usually the preferred treatment; the smaller the tumor, the more likely surgery will be successful. If surgery is not used or does not work, somatostatin analogues or GH receptor antagonists may be used. Radiation therapy may be used if neither surgery nor medications are completely effective. Without treatment, life expectancy is reduced by 10 years; with treatment, life expectancy is not reduced.

Upon successful treatment, symptoms and complications generally improve substantially or disappear, including headaches, visual disturbances, excess sweating, and diabetes. Soft-tissue swellings generally decrease and acromegaly-associated facial features gradually return towards normal, although this may take some time. Life expectancy after the successful treatment of early acromegaly is equal to that of the normal population.

Acromegaly affects about 6 per 100,000 people. It is most commonly diagnosed in middle age. Males and females are affected with equal frequency. It was first described in the medical literature by Nicolas Saucerotte in 1772.

The term is Greek; akron' - meaning "extremity" and mega meaning "large".

Signs and symptoms

Features that result from high level of GH or expanding tumor include:

  • Headaches – often severe and prolonged
  • Soft tissue swelling visibly resulting in enlargement of the hands, feet, nose, lips and ears, and a general thickening of the skin
  • Soft tissue swelling of internal organs, notably the heart and the kidneys, also the vocal cords resulting in a thick, deep voice and slowing of speech
  • Generalized expansion of the skull at the fontanelle
  • Pronounced brow protrusion, often with ocular distension (frontal bossing)
  • Pronounced lower jaw protrusion, enlargement of the tongue and teeth spacing
  • Acrochordon (skin tags)
  • Carpal tunnel syndrome

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