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Acromegaly is a hormonal disorder that results from too much growth
hormone (GH) in the body. The pituitary, a small gland in the brain,
makes GH. In acromegaly, the pituitary produces excessive amounts of GH.
Usually the excess GH comes from benign, or noncancerous, tumors on the
pituitary. These benign tumors are called adenomas.
Acromegaly is most often diagnosed in middle-aged adults, although
symptoms can appear at any age. If not treated, acromegaly can result in
serious illness and premature death. Acromegaly is treatable in most
patients, but because of its slow and often “sneaky” onset, it often is
not diagnosed early or correctly. The most serious health consequences
of acromegaly are type 2 diabetes, high blood pressure, increased risk
of cardiovascular disease, and arthritis. Patients with acromegaly are
also at increased risk for colon polyps, which may develop into colon
cancer if not removed.
When GH-producing tumors occur in childhood, the disease that results is
called gigantism rather than acromegaly. A child’s height is determined
by the length of the so-called long bones in the legs. In response to GH,
these bones grow in length at the growth plates—areas near either end of
the bone. Growth plates fuse after puberty, so the excessive GH
production in adults does not result in increased height. However,
prolonged exposure to excess GH before the growth plates fuse causes
increased growth of the long bones and thus increased height.
Pediatricians may become concerned about this possibility if a child’s
growth rate suddenly and markedly increases beyond what would be
predicted by previous growth and how tall the child’s parents are.
What are the symptoms of acromegaly ?
Symptoms of acromegaly vary depending on how long the patient has had
the disease. The following are the most common symptoms. However, each
individual may experience symptoms differently:
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swelling of the hands and feet
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facial features become coarse as bones grow
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body hair becomes coarse as the skin thickens and/or darkens
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increased perspiration accompanied with body odor
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protruding jaw
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voice deepening
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enlarged lip, nose, and tongue
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thickened ribs (creating a barrel chest)
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joint pain
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degenerative arthritis
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enlarged heart
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enlargement of other organs
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strange sensations and weakness in arms and legs
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snoring
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fatigue and weakness
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headaches
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loss of vision
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irregular menstrual cycles in women
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breast milk production in women
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impotence in men
The symptoms of acromegaly may resemble other conditions or medical
problems. Consult a physician for diagnosis.
How is acromegaly diagnosed ?
Due to the subtlety of the symptoms, acromegaly is often not diagnosed
until years later. In addition to a complete medical history and medical
examination, diagnostic procedures for acromegaly may include:
serial photos taken over the years to observe physical changes in the
patient
x-rays to detect bone thickening
blood tests to check the growth hormone level
Treatment Options
Effective treatment of acromegaly aims to reduce or control tumor mass,
regulate hypersecretion of growth hormone (GH) and insulin-like growth
factor-1 (IGF-1), and alleviate the signs and symptoms of the disease.
To achieve these goals, your healthcare professional may utilize various
treatment options, including surgery, medical therapy, and/or radiation.
According the to the American Association of Clinical Endocrinologists (AACE)
Guidelines, surgery is the "first management option" for nearly everyone
with acromegaly. However, in those cases where surgery is not possible,
medical therapy may be used as first-line treatment.
Surgery – The goal of surgical treatment for acromegaly is to remove the
pituitary tumor, reducing the associated symptoms such as headache, and
normalize GH and IGF-1 levels. This type of surgery is called
transsphenoidal. With this type of procedure, the surgeon reaches the
pituitary through the nose (transnasal) or through an incision in the
upper lip (translabial) and is able to remove the tumor, relieving
pressure and immediately reducing GH levels.
Medical Therapy – Three different medical treatments are used to treat acromegaly.
* Somatostatin analogues (SSAs) work directly at the site of the
pituitary tumor and shut off GH production. As a result, these drugs are
proven to lower GH and IGF-1 levels in 50 to 70 percent of patients.
* GH receptor agonists (GHRAs) are designed to interfere with the action
of GH and normalize IGF-1 levels, however they do not lower GH levels.
* Dopamine agonists have not been proven as effective as somatostatin
analogues and GH receptor agonists at lowering GH or IGF-1 levels, and
they only normalize IGF-1 in some patients.
Radiation Therapy – Radiation is most commonly used as adjuvant therapy
(following surgery) in those cases where some tumor is still present and
medications are not working. The full effect of this therapy may not
occur for many years.
Everyone responds to treatment differently, so you and your healthcare
professional should work together to determine which treatment option or
combination of approaches is right for you.
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