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$Unique_ID{BRK03423} $Pretitle{} $Title{Acromegaly} $Subject{Acromegaly Marie Disease Gigantism Marfan Syndrome McCune-Albright Syndrome Soto's Syndrome Wermer Syndrome} $Volume{} $Log{} Copyright (C) 1985, 1986, 1987, 1988, 1989, 1990, 1991, 1993 National Organization for Rare Disorders, Inc. 51: Acromegaly ** IMPORTANT ** It is possible that the main title of the article (Acromegaly) is not the name you expected. Please check the SYNONYMS listing to find the alternate name and disorder subdivisions covered by this article. Synonyms Marie Disease Information on the following diseases can be found in the Related Disorders section of this report: Gigantism Marfan Syndrome McCune-Albright Syndrome Soto's Syndrome Wermer Syndrome General Discussion ** REMINDER ** The Information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Acromegaly is a rare, slowly progressive chronic disorder characterized by an excess of growth hormone. Symptoms include abnormal enlargement in bones of the arms, legs, and head. The bones in the jaws and in the front of the skull are typically the most affected. Acromegaly may also cause thickening of the soft tissues of the body, particularly the heart and accelerated growth leading to tall stature. Symptoms The symptoms of Acromegaly generally progress slowly after puberty and become more noticeable during middle age. Facial features gradually get a coarse appearance caused by the growth of soft tissues and cartilage. The facial bones become prominent, the jaw protrudes, and an overbite may cause a wide separation between the teeth. People with Acromegaly eventually get a deep and husky voice. Overgrowth of bone and enlargement of cartilage (hypertrophy) in the joints may result in inflammation and the gradual degeneration of involved joints (osteoarthritis). In some people with Acromegaly the spine may curve from side to side and from front to back (kyphoscoliosis). Acromegaly causes a gradual enlargement of the hands and feet. Compression of the spinal nerves may lead to a variety of functional abnormalities and pain. The skin may become dark and there may be an excessive amount of body hair (hirsutism). In some cases abnormal enlargement of the heart may lead to congestive heart failure. Other symptoms of Acromegaly may include abnormal enlargement of the liver (hepatomegaly), spleen (splenomegaly), and/or kidneys (renal). There may also be enlargement of the thyroid and/or the adrenal glands. Approximately 25 percent of people with Acromegaly have elevated blood pressure (hypertension). Abnormal enlargement of the pituitary gland, located deep within the brain, may cause headaches, visual abnormalities, and/or hormonal imbalances. In approximately 50 percent of people with Acromegaly, excessive levels of growth hormone (GH) secreted by the pituitary gland may influence the production of insulin by the pancreas. This results in elevated levels of blood sugar (glucose). Some people with Acromegaly may have an increased metabolic rate, excessive sweating, and/or an increase in the production of oil (sebum) by the sebaceous glands in the skin. Symptoms that may develop late in the course of Acromegaly include muscle weakness (myopathy) and impaired function of peripheral nerves (nerves that are outside the brain and spinal cord). Vision may become impaired and possibly progress to blindness. If untreated, 25 percent of people with Acromegaly experience an increase in the amount of sugar in their urine (glycosuria), abnormally excessive thirst (polydipsia), and/or abnormally increased appetite (polyphagia). When Acromegaly occurs in young adults it can cause rapid and excessive growth resulting in very tall stature. Causes Acromegaly is a rare disorder that may be caused by the growth of a benign tumor (adenoma) in the pituitary cells that secrete growth hormone (frontal somatotrophic cells). Symptoms develop due to the excessive release (hypersecretion) of growth hormone (GH) by the pituitary gland. Less frequently Acromegaly may be caused by the ineffective control of growth hormone-secreting cells by the hypothalamus (a gland in the brain that regulates hormone secretions). Growth hormone-secreting tumors may sometimes be due to over-stimulation by the hypothalamus. Acromegaly may also be caused by the biologic drug, Human Growth Hormone (hGH). This drug is usually prescribed for children with growth hormone deficiency such as pituitary dwarfism. However, when this drug is used by people with normal levels of growth hormone (such as normal children who want to grow taller, or by weight lifters who want to strengthen muscles), it might cause Acromegaly. Affected Population Acromegaly is a rare disorder that affects males and females in equal numbers. This disorder occurs in approximately 50 to 70 people per million. Related Disorders Symptoms of the following disorders can be similar to those of Acromegaly. Comparisons may be useful for a differential diagnosis: Gigantism is an abnormal condition characterized by excessive height and size. This disorder typically occurs before puberty as a result of the over secretion of growth hormone by the pituitary gland. Gigantism is associated with enlarged soft tissues and late epiphyseal closure (head of the long bones), which results in excessive growth during childhood. Height may reach 7 or 8 feet. Low levels of growth hormone may be secreted by the pituitary gland later in the course of this disorder and result in impaired muscle function and low levels of hormone secretions by the ovaries or testes. Sexual development may be normal or it may be affected by the low levels of circulating sex hormones. In some cases of Gigantism, patients may experience tingling and/or burning sensations in the arms and/or legs (peripheral neuropathy). Marfan Syndrome is a rare inherited disorder that affects the connective tissues of the heart and blood vessels (cardiovascular system); the musculoskeletal system (ligaments and muscles) is also affected. The major features of this disorder include unusually tall stature, and large hands and feet. People with Marfan Syndrome may have significant cardiovascular problems including mitral valve prolapse, degeneration and enlargement of the aorta, and/or a bulge in the wall of the aorta (aortic aneurysm). Other symptoms may include excessive joint mobility, flat feet, extreme muscle weakness (hypotonia), a protruding or indented breast bone (sternum), and curvature of the spine (scoliosis). (For more information on this disorder, choose "Marfan" as your search term in the Rare Disease Database.) McCune-Albright Syndrome (Acromegaly and Hyperprolactemia) is a rare multi-system disorder characterized by the abnormal growth of fibrous bone tissue (polyostotic fibrous dysplasia). Bones most frequently affected include those of the arms and legs, pelvis, ribs, and/or the base of the skull. Symptoms of McCune-Albright Syndrome may include pain, shortening of the limbs, and/or the appearance of patchy brown spots on the skin (cafe-au- lait macules). During childhood people with McCune-Albright Syndrome grow rapidly and become taller than children of equal age. However, growth stops prematurely so that adults with McCune-Albright Syndrome are shorter than normal. Early puberty (precocious puberty), as early as three months of age can occur, and is more common in females than males. (For more information on this disorder, choose "McCune-Albright" as your search term on the Rare Disease Database.) Soto's Syndrome is a rare inherited disorder characterized by abnormally large birth weight and excessive growth (over the 90th percentile) during the first 2 to 3 years of life. Physical characteristics may include an abnormally large and long head, a slight bulge of the forehead, large hands and feet, wide spread eyes (hypertelorism), and/or downslanting eyes. Other symptoms of Soto's Syndrome may include clumsiness, an awkward gait, and/or mild developmental retardation. (For more information on this disorder, choose "Soto" as your search term in the Rare Disease Database.) Wermer syndrome (Type I Multiple Endocrine Neoplasia or Polyendocrine Adenomatosis) is a rare inherited disorder characterized by excessive growth, multiple tumors, and/or excessive hormone secretion. The symptoms of Wermer Syndrome may include diarrhea and abdominal pain. Children with this disorder may have low blood sugar (hypoglycemia); adults may have peptic ulcers. Occasionally epileptic seizures may also occur. Therapies: Standard Acromegaly is usually treated by the partial or total surgical removal of the pituitary gland. This surgical procedure may also be supplemented by radiation treatment (proton beam, heavy particle, and supravoltage irradiation). If the entire pituitary gland is removed, lifelong hormonal replacement therapy is essential. Growth hormone suppressors (drugs that depress the production of growth hormone) have been used including the female sex hormones estrogen or medroxyprogesterone, the phenothiazine derivative chlorpromazine. Somatostatin (Sandostatin), the natural body substance that inhibits the secretion of growth hormone, is also used as a treatment for Acromegaly. Sandostatin has been found to be effective in 50 to 60 percent of cases of Acromegaly. In mild cases of Acromegaly or in elderly people with this disorder, dopamine agonists such as bromocriptine have been found to reduce growth hormone levels when used as an adjunctive therapy (along with other drugs). Therapies: Investigational Treatment of Acromegaly with a somatostatin analog (SMS 201-995), known as "selective mini-somatostatin" can significantly lower the mean plasma growth hormone levels when given preoperatively. This drug is able to shrink invasive pituitary macroadenomas and improve surgical remission rates. Treatment with this somatostatin analog is available in many research- oriented medical centers within the United States. More research is needed before this drug can be approved by the FDA. This disease entry is based upon medical information through February 1993. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Acromegaly, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 National Arthritis and Musculoskeletal and Skin Diseases (NIAMS) Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 Human Growth Foundation (HGF) 7777 Leesburg Pike P.O. Box 3090 Falls Church, VA 22043 (703) 883-1773 (800) 451-6434 References CECIL TEXTBOOK OF MEDICINE, 19th Ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1990. Pp. 1234-1236. TEXTBOOK OF ENDOCRINOLOGY, 8TH ED.: Jean D. Wilson and Daniel W. Foster, Editors; W.B. Saunders Co., 1992. Pp. 268-290. ACROMEGALIC HEART DISEASE: INFLUENCE OF TREATMENT OF THE ACROMEGALY ON THE HEART: R. P. Hayward, et al.; Quarterly Journal Med (January 1987: issue 62(237)). Pp. 41-58. THE PATHOGENESIS OF ACROMEGALY: CLINICAL AND IMMUNOCYTOCHEMICAL ANALYSIS IN 75 PATIENTS: E.R. Laws, Jr., et al.; Journal Neurosurg (July 1985: issue 63(1)). Pp. 35-38. SOMATOSTATIN PROVES EFFECTIVE IN TREATING RESISTANT ACROMEGALY: Andrea J. Clark; Research Resources Reporter (April 1988). Pp. 5-7. SOMATOMEDIN-C LEVELS IN TREATED AND UNTREATED PATIENTS WITH ACROMEGALY: F. Roelfsema, et al.; Clin Endocrinol (Oxford) (February 1987: issue 26(2)). Pp. 137-144. ACROMEGALY: J.D. Nabarro; Clin Endocrinol (Oxford) (April 1987: issue 26(4)). Pp. 481-512. ACROMEGALY, Shlomo Melmed, M.B., CH.B., N Eng J Med, (April 5, 1990, issue 322 (14)). Pp. 966-977.