Acromegaly

  • Definition

    Growth hormone (GH) controls the growth of soft tissue and bone. Elevated GH causes an excess of bone and soft tissue growth. In adults, this can cause a rare disorder called acromegaly. It can cause serious complications and early death if not treated.
    In young children, bone growth is still occurring. Excess GH can cause a similar condition called gigantism. Gigantism causes dramatic growth in children.
  • Causes

    The pituitary gland is a small gland located at the base of the brain. It produces many hormones, including GH.
    In most cases, the elevation of GH is caused by a benign tumor of this gland. In a small number of cases, cancerous tumors of other organs, such as the pancreas, adrenal, or lung, may be the source of excess GH.
    Pituitary Gland
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  • Risk Factors

    Factors that increase your chance of developing acromegaly include:
    • Age: 40-45 years old
    • Family history—rare
  • Symptoms

    Symptoms usually develop slowly over time.
    In children, the bones are longer and cause soft tissue swelling. If not treated, children can grow to a height of 7-8 feet.
    Symptoms and complications in adults may include:
    • Abnormally large growth and deformity of the:
      • Hands—rings no longer fit
      • Feet—need a bigger size shoe
      • Face—bulging of brow and lower jaw
      • Jaw—teeth do not line up correctly when the mouth is closed
      • Lips
      • Tongue
    • Skin changes, such as:
      • Thickened, oily, and sometimes darkened skin
      • Severe acne
      • Excessive sweating and unpleasant body order due to enlargement of the sweat glands
    • Deepening voice due to enlarged sinuses, vocal cords, and soft tissues of the throat
    • Fatigue and weakness in the legs and arms
    • Joint pain, especially in the jaw
    • In women:
      • Irregular menstrual cycles
      • Abnormal production of breast milk
    • In men:
  • Diagnosis

    The doctor will ask about your symptoms and medical history. A physical exam will be done. Acromegaly is often not diagnosed until years after its onset.
    Your bodily fluids may be tested. This can be done with blood tests.
    Images may be taken of your bodily structures. This can be done with:
  • Treatment

    The goals of treatment are to:
    • Reduce production of GH to normal levels
    • Stop and reverse the symptoms caused by excess GH
    • Correct other endocrine abnormalities, such as thyroid, adrenal, and sex organs
    • Reduce the tumor size
    Treatment may include:
    Surgery
    The tumor that is believed to be causing acromegaly may be removed. In most cases, this is the preferred treatment. However, drug treatment is increasing in popularity.
    Radiotherapy
    In adults, external beams of radiation are used to shrink the tumor. It is most often used when surgery cannot be used or when medications have failed.
    Medication
    Drugs may be given to reduce the level of GH. These include:
    • Cabergoline
    • Pergolide
    • Bromocriptine—may be given before surgery to shrink tumor
    • Octreotide—may be the most effective medication for this condition
    • Pegvisomant—used if other treatments have failed
  • Prevention

    There are no known steps to prevent acromegaly. Early treatment will help to prevent serious complications.
  • RESOURCES

    National Institute of Diabetes & Digestive & Kidney Diseases http://www.niddk.nih.gov

    Pituitary Network Association http://www.pituitary.org

    CANADIAN RESOURCES

    Canadian Society of Endocrinology & Metabolism http://www.endo-metab.ca

    Health Canada http://www.hc-sc.gc.ca

    References

    Abrams P, Alexopoulou O, Abs R, et al. Optimalization and cost management of lanreotide-Autogel therapy in acromegaly. Eur J Endocrinol . 2007;571-577.

    Acromegaly. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed . Updated November 7, 2012. Accessed July 26, 2013.

    Acromegaly treatment consensus workshop participants: guidelines for acromegaly management. J Clin Endocrinol Metab . 2002; 87:4054-4058.

    Cook DM. AACE Acromegaly Guidelines Taskforce. American Association of Clinical Endocrinologists medical guidelines for clinical practice for the diagnosis and treatment of acromegaly. Endoc Pract . 2004;10:213-225.

    Glustina A, Barkan A, Casanueva FF, et al. Criteria for cure of acromegaly: a consensus statement. J Clin Endocrinol Metab . 2000;85:526-529.

    Katznelson L, Atkinson JL, Cook DM, Ezzat SZ, Hamrahian AH, Miller KK. American association of clinical endocrinologists medical guidelines for clinical practice for the diagnosis and treatment of acromegaly - 2011 update: executive summary. Endocr Pract . 2011;17(4):636-646.

    Melmed S. Medical progress: acromegaly. N Engl J Med . 2006;355:2558-2573.

    Paisley AN, Trainer PJ. Medical treatment in acromegaly. Curr Opin Pharmacol . 2003;3:672-677.

    Sherlock M, Woods C, Sheppard MC. Medical therapy in acromegaly. Nat Rev Endocrinol . 2011;7(5):291-300.

    Trainer PJ, Drake WM, Katzneison L, et al. Treatment of acromegaly with the growth hormone-receptor antagonist pegvisomant. N Engl J Med . 2000; 342:1171-1177.

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