Acromegaly is a rare but very serious disease frequently referred to as “Gigantism.” It is the direct result of an overproduction and secretion of the adenohypophyseal growth hormone. True to its name, the disease is characterized by an enlargement of the organs, bones, and soft tissues, as well as a thickening of the skin and bodily membranes; and is frequently difficult to diagnose, as symptoms often do not appear until middle age. Typically the disease results in heart failure caused by an enlargement of the heart, organ failure due to pressure or thickening of the outer membrane of the organ, or brain hemorrhaging caused by a benign or malignant pituitary adenoma.
To date, there are three known forms of the disease: Cushing’s disease, Nelson Syndrome, and Prolactinoma. Each is characterized by different phenotypic attributes commonly linked by an enlargement of all or some particular body parts. Prolactinoma is the most frequently recognized form of gigantism. It is characterized by an overall enlargement of the body, and was brought to public attention nearly twenty years ago by pro-wrestler and actor Andre the Giant, who later died of heart failure in the early 1990s. Nelson’s disease and Cushing Syndrome are similar to each other; however there is now controversy as to whether these two diseases are even linked to Prolactinoma since they are not commonly characterized by an overall enlargement of the body, but rather an extreme overproduction of soft and fatty tissue. These diseases cause thickening of the bones, skin, and soft tissue, but often appear after the person has finished growing, making the patient appear overweight and unable to speak and move with fluidity since the tongue and hands are hindered. All three diseases tend to have the same outcome: organ failure or brain hemorrhaging.
In the past twenty years, controversy has arisen as to whether there are in fact three forms of the disease, what characterizes differentiation between each one, and how each should be treated accordingly. The problem is until recently, scientists have based their diagnoses almost entirely upon phenotypic characteristics and what is known about pituitary adenomata; only now are scientists beginning to analyze the genes and hormones involved on the micro level. The following articles examine methods in which testing for the disease takes place. Recent studies tend to revolve mainly around the IGF (insulin like growth factor) -binding protein, the proposed cause of pituitary adenoma. However, it is not entirely known how to test for this protein; whether to measure free IGF or total IGF-binding proteins.
In the case of acromegaly, a massive influx of GH (growth hormone) is produced by the pituitary gland leading to expansion of tissues and bone mass. Because it controls most of the other major glands in the body, the pituitary is often described as “the master gland,” according to Dr. Joseph Verbalis, the chief of endocrinology at Georgetown University Medical Center.
An example of acromegaly can be seen in Galapagos Giant Tortoises. The original ancestors of the tortoises were most likely of normal size; the species evolved over generations and became the present day giants of the Galapagos. This is due to a phenomenon seen in many island ecosystems where gigantism exists because there is no longer any need to hide from predators and because there are no other similar animals to compete with for food. Other extreme cases of this phenomenon include the Flores Giant Rat, and the legendary Komodo dragon.
Furthermore, this disease exists not only in animals but amongst humans as well. One famous instance is of Andre the Giant. Andre was a French professional wrestler and actor believed to be anywhere between 6’10” and 7’4” tall. His great size was a result of acromegaly, and led to him being dubbed “The Eighth Wonder of the World.” At an earlier age, Andre was drafted into France’s peacetime army, but was rejected during his physical because there were no shoes large enough to fit him. Another example of such a behemoth man comes from the story of Gheorghe Muresan. At 7’7”, Gheorghe is to date the largest man to ever play in the National Basketball Association. Muresan enjoyed a successful but brief career; his body was only able to withstand 7 years of service in the league. Muresan has such a benign tumor, a large part of which was removed in an operation just before he came to the United States. He continues to receive injections twice a day to control the condition.
Thanks to earlier recognition of the disorder along with the development of new medicines and medical techniques to control it, Muresan was saved. However, one man by the name of Robert Wadlow lived in an era before such treatment was available. Born in 1918, Wadlow was the tallest man ever on record. Wadlow grew so rapidly that by age 8, he was already 6’2. Five years later, Robert had already jumped to a height of 7 foot 4, and a size 25 shoe. Unfortunately, Wadlow’s size began to take its toll: he required leg braces to walk, and had little feeling in his legs and feet. On July 15, 1940, Robert Wadlow died, but not before he had grown to a full 8’11”. He was buried in a half-ton coffin that required 12 pallbearers to carry; he was only 22. It is very unlikely that mankind will see anyone like him again.
Since acromegaly is often difficult to diagnose until later in life, recent studies are focusing on the best and most efficient way to identify a problem before major irreversible damage occurs. Unfortunately, because the disease is so rare, major symptoms generally have to occur before the afflicted is even tested for the disease. However, it is also hereditary and with heredity comes early testing in patients where the disease is prevalent among the family. The problem arises then as to how to test early, since growth hormone therapy performed on a patient who does not definitely have the disease may be more damaging than leaving the actual disease untreated. Due to a lack of sufficient funding and support as well as the rarity of the disease, research regarding treatment as well as screening and testing procedures is extremely limited.