AcromegalyAcromegaly 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.
How Is Acromegaly Diagnosed?
If acromegaly is suspected, a doctor must measure the GH level in a person’s blood to determine if it is elevated. However, a single measurement of an elevated blood GH level is not enough to diagnose acromegaly: Because GH is secreted by the pituitary in impulses, or spurts, its concentration in the blood can vary widely from minute to minute. At a given moment, a person with acromegaly may have a normal GH level, whereas a GH level in a healthy person may even be five times higher.
More accurate information is obtained when GH is measured under conditions that normally suppress GH secretion. Health care professionals often use the oral glucose tolerance test to diagnose acromegaly because drinking 75 to 100 grams of glucose solution lowers blood GH levels to less than 1 nanogram per milliliter (ng/ml) in healthy people. In people with GH overproduction, this suppression does not occur. The oral glucose tolerance test is a highly reliable method for confirming a diagnosis of acromegaly.
Physicians also can measure IGF-I levels, which increase as GH levels go up, in people with suspected acromegaly. Because IGF-I levels are much more stable than GH levels over the course of the day, they are often a more practical and reliable screening measure. Elevated IGF-I levels almost always indicate acromegaly. However, a pregnant woman’s IGF-I levels are two to three times higher than normal. In addition, physicians must be aware that IGF-I levels decline with age and may also be abnormally low in people with poorly controlled diabetes or liver or kidney disease.
After acromegaly has been diagnosed by measuring GH or IGF-I levels, a magnetic resonance imaging (MRI) scan of the pituitary is used to locate and detect the size of the tumor causing GH overproduction. MRI is the most sensitive imaging technique, but computerized tomography (CT) scans can be used if the patient should not have MRI. For example, people who have pacemakers or other types of implants containing metal should not have an MRI scan because MRI machines contain powerful magnets.
If a head scan fails to detect a pituitary tumor, the physician should look for non-pituitary “ectopic” tumors in the chest, abdomen, or pelvis as the cause of excess GH. The presence of such tumors usually can be diagnosed by measuring GHRH in the blood and by a CT scan of possible tumor sites.
What Are The Symptoms Of Acromegaly?
The name acromegaly comes from the Greek words for “extremities” and “enlargement,” reflecting one of its most common symptoms—the abnormal growth of the hands and feet. Swelling of the hands and feet is often an early feature, with patients noticing a change in ring or shoe size, particularly shoe width. Gradually, bone changes alter the patient’s facial features: The brow and lower jaw protrude, the nasal bone enlarges, and the teeth space out.
Other symptoms of acromegaly include :-
- joint aches
- thick, coarse, oily skin
- skin tags
- enlarged lips, nose, and tongue
- deepening of the voice due to enlarged sinuses and vocal cords
- sleep apnea—breaks in breathing during sleep due to obstruction of the airway
- excessive sweating and skin odor
- fatigue and weakness
- impaired vision
- abnormalities of the menstrual cycle and sometimes breast discharge in women
- erectile dysfunction in men
- decreased libido
What Causes Acromegaly?
Acromegaly is caused by excess production of certain growth hormones. These include growth hormone (GH) and insulin-like growth factor-1 (IGF-1). In most cases, this results from a small, noncancerous tumor on the pituitary gland.
Acromegaly is caused by prolonged overproduction of GH by the pituitary gland. The pituitary produces several important hormones that control body functions such as growth and development, reproduction, and metabolism. But hormones never seem to act simply and directly. They usually “cascade” or flow in a series, affecting each other’s production or release into the bloodstream.
GH is part of a cascade of hormones that, as the name implies, regulates the physical growth of the body. This cascade begins in a part of the brain called the hypothalamus. The hypothalamus makes hormones that regulate the pituitary. One of the hormones in the GH series, or “axis,” is growth hormone-releasing hormone (GHRH), which stimulates the pituitary gland to produce GH.
Secretion of GH by the pituitary into the bloodstream stimulates the liver to produce another hormone called insulin-like growth factor I (IGF-I). IGF-I is what actually causes tissue growth in the body. High levels of IGF-I, in turn, signal the pituitary to reduce GH production.
How Common Is Acromegaly?
Small pituitary adenomas are common, affecting about 17 percent of the population. 1 However, research suggests most of these tumors do not cause symptoms and rarely produce excess GH. 2 Scientists estimate that three to four out of every million people develop acromegaly each year and about 60 out of every million people suffer from the disease at any time.3 Because the clinical diagnosis of acromegaly is often missed, these numbers probably underestimate the frequency of the disease.
What Is The Pituitary Gland?
The pituitary is a small gland (about the size of a pea) located at the base of the brain, behind the eyes. It is sometimes referred to as a "master gland" because it secretes hormones that control the function of many other parts of the endocrine system.
Surgical Management Of Acromegaly
According to the American Association of Clinical Endocrinologists (AACE) Guidelines, surgery should be considered first-line therapy for all acromegaly patients.
Surgical intervention aims to provide the following: -
- Immediate reduction in GH levels
- Elimination of the tumor mass and reversal of associated symptoms, including headache and visual impairment
- Alleviation of comorbidities such as hypertension, sleep apnea, arthritis, etc.
- Preservation of pituitary function and restoration of endocrine deficits caused by the tumor
- Prevention of tumor recurrence
- Ability to retrieve and test tissue
How Is Acromegaly Treated?
Currently, treatment options include surgical removal of the tumor, medical therapy, and radiation therapy of the pituitary.
Goals of treatment are to
- reduce excess hormone production to normal levels
- relieve the pressure that the growing pituitary tumor may be exerting on the surrounding brain areas
- preserve normal pituitary function or treat hormone deficiencies
- improve the symptoms of acromegaly
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