• 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 fusion and growth is still occurring. Excess GH can cause a similar condition called gigantism.

    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, malignant tumors of other organs (pancreas, adrenal, lung) may be the source of excess GH.
    Pituitary Gland
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    Risk Factors

    Risk factors that increase your chance of developing acromegaly include:
    • Family history (rare)
    • Age: 40-45 years old

    Symptoms

    Symptoms usually develop slowly over time.
    In children, the bones are elongated 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 (protrusion of brow and lower jaw)
      • Jaw (teeth do not line up correctly when the mouth is closed)
      • Lips
      • Tongue
    • Carpal tunnel syndrome
    • 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 legs and arms
    • Sleep apnea
    • Arthritis and other joint problems, especially in the jaw
    • Hypothyroidism
    • Abnormally large liver, kidneys, spleen, heart, and/or other internal organs, which can lead to: In women:
      • Irregular menstrual cycles
      • Galactorrhea (abnormal production of breast milk) in about 50% of cases
      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.
    Blood tests will be done to measure the level of:
    • Insulin-like growth factor (IGF-I)
    • Growth hormone releasing hormone (GHRH)
    • Other pituitary hormones
    A glucose tolerance test may also be given to see if the GH level drops. It will not drop in cases of acromegaly.
    If these tests confirm acromegaly, the following may be done to locate the tumor that is causing the disorder:

    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 (thyroid, adrenal, 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 becoming more popular as the primary treatment approach.

    Radiotherapy

    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 (Dostinex)—given orally
    • Pergolide (Permax)—given orally
    • Bromocriptine (Parlodel)—may be given before surgery to shrink tumor
    • Octreotide (Sandostatin)—given by injections (may be the most effective medication for this condition)
    • Pegvisomant —given by injections if not responding to other forms of treatment

    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/

    The Neuroendocrine Clinical Center and Pituitary Tumor Center http://pituitary.mgh.harvard.edu

    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 May 23, 2012. Accessed October 30, 2012.

    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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