Acromegaly is a rare medical condition. Worldwide, it has been estimated that there are only around 2 to 11 people per million people in the general population newly diagnosed with acromegaly each year. There are thought to be between 28 and people per million of the population currently living with the condition long term. Find out how acromegaly is diagnosed and the tests that healthcare professionals may use to assess acromegaly symptoms.
Very rarely, acromegaly can be diagnosed in children and adolescents. Children and adolescents will, however, develop gigantism , whereas adults develop acromegaly. Thus, acromegaly is sporadic, meaning it is not inherited. When acromegaly develops at an early age some cases have an identifiable genetic mutation. Learn about acromegaly including how excess growth hormone released by a pituitary tumor causes this slowly evolving condition and the early symptoms and signs of acromegaly.
Information about the emotional, physical and social challenge of living with acromegaly. Do you have any practical tips that you think other people living with acromegaly might benefit from?
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Symptoms of acromegaly can vary from person to person. Common changes in physical appearance include. Acromegaly develops when the pituitary gland releases too much GH into the body over a long period of time. IGF-I is the hormone that actually causes bones and body tissue to grow. High levels of this hormone also cause changes in how the body processes blood glucose blood sugar and lipids fats , which can lead to type 2 diabetes, high blood pressure, and heart disease.
In more than 9 out of 10 cases, acromegaly is caused by a tumor in the pituitary gland, called a pituitary adenoma. In young adults, acromegaly has been linked to defects in certain genes.
Pituitary tumors are almost always benign, or noncancerous. Some tumors grow slowly, and symptoms of too much GH may not be noticed for many years.
Other tumors may grow rapidly. Depending on its size and location, the tumor may press against other pituitary tissue. Possible effects include. A tumor that grows large in size may also press against nearby parts of the brain. This can lead to other symptoms, such as headaches and vision problems. Some pituitary tumors that create growth hormone can also increase the levels of other hormones in the body. For example, the tumor may produce prolactin, the hormone that prompts the mammary glands to produce milk.
This can lead to breast milk discharge in women. Rarely, acromegaly is caused by tumors located in the hypothalamus—a small area of the brain near the pituitary gland, pancreas , lungs, or other parts of the chest or abdomen. Some of these tumors make growth hormone themselves. But more often, the tumors produce growth hormone-releasing hormone GHRH , a hormone that signals the pituitary gland to make growth hormone.
Doctors most often diagnose acromegaly by ordering two blood tests that help determine if your body is making too much GH. If the blood tests confirm that your body is making too much GH, your doctor will conduct imaging tests to locate and measure the tumor that may be causing the problem.
Two commonly used tests are. Treatment options include surgery, medicines, and radiation therapy. The goals of treatment are to control tumor size, return GH and IGF-I levels back to normal, improve symptoms, and manage related health problems.
No single treatment is right for everyone. Your doctor will recommend a treatment plan that works for you, depending on factors such as your age, tumor size, severity of symptoms, GH and IGF-I levels, and health status. Doctors can remove most pituitary tumors using a method called transsphenoidal surgery. The operation is done through the nose and sphenoid sinus, a hollow space in the skull behind the nasal passages and below the brain.
Two approaches to this surgery are. In both approaches, the surgeon uses advanced MRI imaging to scan the area around the tumor before surgery. He or she then makes a small cut inside your nostril to view the area and remove the tumor using tiny, special tools. In microscopic surgery, the surgeon uses a microscope to magnify the area. In endoscopic surgery, an endoscope camera sends images to a television monitor instead. Risks and results are similar for both approaches. When the tumor that is creating too much GH is not located in the pituitary gland, other types of surgery are used to remove the tumor.
Pituitary gland and hypothalamus The pituitary gland and the hypothalamus are located within the brain and control hormone production. Share on: Facebook Twitter. Show references AskMayoExpert.
Acromegaly and gigantism. Mayo Clinic; Accessed Dec. Kellerman RD, et al. Endocrine and metabolic disorders. In: Conn's Current Therapy Elsevier; Jameson JL, et al. Acromegaly: In: Endocrinology: Adult and Pediatric. Melmed S, et al. Treatment of acromegaly. Information provided in your contribution including your email address will be stocked in. CSV files that will be sent as an email to Orphanet's teams.
These emails might be conserved in the teams' mailboxes, in our backoffice servers but will not be registered in our databases for more information see our section General Data Protection Regulation and data privacy GDPR and Confidentiality. A rare acquired endocrine disease related to excessive production of growth hormone GH and characterized by progressive somatic disfigurement mainly involving the face and extremities and systemic manifestations.
Due to its insidious onset and slow progression, acromegaly is often diagnosed from four to more than ten years after its onset, and is most often diagnosed in middle age average age years. The main clinical features are broadened extremities hands and feet , widened, thickened and stubby fingers, and thickened soft tissue. The facial aspect is characteristic and includes a widened and thickened nose, prominent cheekbones, forehead bulges, thick lips and marked facial lines.
The forehead and overlying skin is thickened, sometimes leading to frontal bossing. There is a tendency towards mandibular overgrowth with prognathism, maxillary widening, tooth separation and jaw malocclusion. The disease also has rheumatologic, cardiovascular, respiratory and metabolic consequences which determine its prognosis.
In very rare cases, acromegaly is due to ectopic secretion of growth hormone-releasing hormone GHRH , responsible for pituitary hyperplasia.
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