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Bulletin scientifique en langues trangres appliques

L'anglais sur objectifs spcifiques

Floriana Olivia SANDU

Gigantism and Acromegaly - living under the skin of an extreme body
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Article

Abstract

Gigantism is a growth defect, similar to acromegaly, the difference between the two diseases being the period in which they occur: gigantism (or giantism) occurs during childhood and acromegaly appears in adulthood. Excessive growth hormone is almost always caused by a noncancerous (benign) pituitary tumor. Children develop great stature; adults develop deformed bones but do not grow taller. Heart failure, weakness, and vision problems are common. In children, rapid growth may not seem abnormal at first. Eventually, however, the abnormality of the extreme growth becomes clear. In adults, because the changes induced by high levels of growth hormone occur slowly, acromegaly is diagnosed long after the first symptoms appear. A person with the condition will take on a certain appearance: large hands and feet, larger facial features, jaw, and cheekbones, and of course excessive height, if affected at or before puberty. The stature and weight attained due to gigantism can cause a reduction in mobility, and make movement awkward and slow. Stopping or reducing the overproduction of growth hormone is not easy; thus, doctors may need to use a combination of surgery, radiation therapy, and drug therapy.

Texte intgral

1Living with gigantism : a new day,

2a new attempt to a normal life.

3Gigantism is the name of a particular growth defect that occurs during childhood, from over-exposure to growth hormone. This is similar to another disease, acromegaly, which arises from similar types of tumors but occur in adulthood, when bones have fused and cannot lengthen.

4Gigantism is not a currently used medical term and there is no precise definition of the degree of tallness that qualifies a person to be termed a "giant". The term has been typically applied to those whose height is not just in the upper 1% of the population but several standard deviations above average for persons of the same sex, age, and ethnic ancestry. Typical adult heights of Americans and Europeans to whom the term might be applied are 215 - 260 cm (7.5 - 8.5 feet), although the term is rarely applied to basketball players and those whose heights appear to be the healthy result of normal genetics and nutrition.

5The condition is not hereditary, is not passed on from parent to child via the genetic material, however, some people believe that a set of conditions, or family tendencies, can be passed on, which makes the child more likely than normal to develop the condition. In one case, a man reported that several members of his family have had several pituitary related problems: diabetes and cryptorchidism, for instance.

6Excessive growth hormone is almost always caused by a noncancerous (benign) pituitary tumor. Children develop great stature, adults have deformed bones but do not grow taller. Heart failure, weakness, and vision problems are common. The diagnosis is based on blood tests and x-rays of the skull and hands. Other imaging tests are done to look for the cause. A combination of surgery, radiation therapy, and drug therapy is used to treat the overproduction of growth hormone.

7Growth hormone stimulates the growth of bones, muscles, and many internal organs. Excessive growth hormone, therefore, leads to abnormally robust growth of all these tissues. Overproduction of growth hormone is almost always caused by a noncancerous (benign) pituitary tumor (adenoma). Certain rare tumors of the pancreas and lungs can also produce hormones that stimulate the pituitary to produce excessive amounts of growth hormone, with similar consequences.

8If excessive growth hormone production starts before the growth plates closed (that is, in children), the condition produces gigantism. The long bones grow enormously. A person grows to unusually great stature, and the arms and legs lengthen. Puberty may be delayed, and genitals may not fully develop.

9In most cases, excessive production of growth hormone begins between the age of 30 and 50, long after the growth plates of the bones have closed. Increased growth hormone in adults produces acromegaly, in which the bones become deformed rather than elongated. Because changes occur slowly, they are usually not recognized for years.

10The person's facial features become coarse, while hands and feet swell. Larger rings, gloves, shoes, and hats are needed. Overgrowth of the jawbone (mandible) can cause the jaw to protrude (prognathism). Cartilage in the voice box (larynx) may thicken, making the voice deep and husky. The ribs may thicken, creating a barrel chest. Joint pain is common; after many years, crippling degenerative arthritis may occur.

11In both gigantism and acromegaly, the tongue may enlarge and become more furrowed. Coarse body hair, which typically darkens, increases as the skin thickens. The sebaceous and sweat glands in the skin enlarge, producing excessive perspiration and often an offensive body odour. The heart usually enlarges, and its function may be so severely impaired that heart failure occurs. Sometimes a person feels disturbing sensations and weakness in the arms and legs as enlarging tissues compress the nerves. Nerves that carry messages from the eyes to the brain may also be compressed, causing loss of vision, particularly in the outer visual fields. The pressure on the brain may also cause severe headaches.

12Nearly all women with acromegaly have irregular menstrual cycles. Some women produce breast milk even though they are not breastfeeding (galactorrhea) because of either too much growth hormone or a related increase in prolactin. About one third of men who have acromegaly develop erectile dysfunction. There is also an increased likelihood of developing diabetes mellitus, high blood pressure (hypertension), heart failure, sleep apnea, and certain tumors, particularly affecting the large intestine, which may become cancerous. Life expectancy is reduced in people with untreated acromegaly.

13In children, rapid growth may not seem abnormal at first. Eventually, however, the abnormality of the extreme growth becomes clear.

14In adults, because the changes induced by high levels of growth hormone occur slowly, acromegaly is not often diagnosed unless the first symptoms appear. Serial photographs may help a doctor establish the diagnosis. An x-ray examination of the skull may show thickening of the bones and enlargement of the nasal sinuses. X-rays of the hands show thickening of the bones under the fingertips and swelling of the tissue around the bones. Blood sugar levels and blood pressure may be high.

15A person with the condition will take on a certain appearance, with large hands and feet, larger facial features, jaw, and cheekbones, and of course excessive height, if affected at or before puberty.

16The stature and weight attained due to gigantism can cause a reduction in mobility, and make movement awkward and slow. It can be important to maintain some form of exercise programme, to counteract this.

17The diagnosis is confirmed by blood tests, which usually show high levels of both growth hormone and insulin-like growth factor 1 (IGF-1). Because growth hormone is released in short bursts and the levels of growth hormone often fluctuate dramatically even in people without acromegaly, a single high level of growth hormone in the blood is insufficient to make the diagnosis. Doctors must give something that would normally suppress growth hormone levels, most commonly a glucose drink (the oral glucose tolerance test), and show that normal suppression does not occur. This test is not necessary when the clinical features of acromegaly are obvious, the IGF-1 level is high, or a tumor is seen in the pituitary on scanning.

18A computed tomography (CT) or magnetic resonance imaging (MRI) scan is usually done to look for abnormal growths in the pituitary gland. Because acromegaly is usually present for some years before being diagnosed, a tumor is seen on these scans in most people.

19Stopping or reducing the overproduction of growth hormone is not easy; thus, doctors may need to use a combination of surgery, radiation therapy, and drug therapy.

20Surgery by an experienced surgeon is currently regarded as the best first treatment for most people with acromegaly caused by a tumor. It results in an immediate reduction in tumor size and growth hormone production, most often without causing deficiency of other pituitary hormones. Unfortunately, tumors are often large by the time they are found, and surgery alone does not usually produce a cure. Radiation therapy is often used as a follow-up treatment, particularly if a substantial amount of the tumor remains after surgery and acromegaly persists.

21Radiation therapy involves the use of supervoltage irradiation, which is less traumatic than surgery. This treatment may take several years to have its full effect, however, and often results in later deficiencies of other pituitary hormones, as normal tissue is often also affected. More directed radiation therapy, such as stereotactic radiosurgery, is being tried to speed results and spare the normal pituitary tissue.

22Drug therapy can also be used to lower growth hormone levels. Occasionally, bromocriptine and other drugs that act like dopamine are of some benefit. The most effective drugs, however, are those that are forms of somatostatin, the hormone that normally blocks growth hormone production and secretion. These drugs include octreotide and its newer long-acting analogs, which only have to be given about once a month. These drugs are effective in controlling acromegaly in many people as long as they continue to be taken (they do not provide a cure). Their use has been limited by the need to inject them and by their high cost. This may cause such drugs to become longer acting and more readily available. Several new growth hormone blocker drugs, such as pegvisomant are now available and may be useful for people who do not respond to somatostatin-type drugs.

23Yao Defen of China, (born 15 July 1972) claims to be the tallest woman in the world. The Guinness Book of World Records said American Sandy Allen was the world's tallest woman until Allen's death on August 13, 2008. She weighs 200 kg (440 lbs) and sizes 57 (EU) (around 20 US) feet. Her gigantism is due to a tumor in her pituitary gland.

24Yao Defen was born to poor farmers in the town of Liuan in the Anhui province of Shucheng County. At birth she weighed 6.16 pounds. At the age of 3 she was eating more than three times the amount of food that other three-year-olds were eating. When she was 11 years old she was about 6 foot 2 inches tall. She was 6 foot 8 inches tall by the age of 15. The story of this giant woman began to spread rapidly after she went to see a doctor at the age of15 for an illness. After that, many companies attempted to train her to be a sports star. The plans were abandoned, however, because Defen was too weak. Because she is illiterate, since 1992 Yao Defen has been forced to earn a living by travelling with her father and performing.

25Yao Defen's giant stature was caused by a large tumor in the pituitary gland of her brain, which was releasing too much growth hormone and caused excessive growth in her bones. Six years ago, a hospital in Guangzhou Province removed the tumor, and she stopped growing.

26The tumor recurred and she was treated in Shanghai in 2007, but was sent home for 6 months hoping that medication would reduce her tumor enough to allow surgery. It remained unknown whether the second surgery was ever undergone. A British television programme presented a documentary on her and helped raise money so that she could get proper medical care. Two doctors specialized in acromegaly agreed to help Yao. She was taken to a nearby city hospital, where imaging procedures revealed that a small portion of her tumor that had been removed many years before, still remained, causing problems, including weakening vision as it pressed against her optic nerve. She returned home, then was admitted for a month under observation in the larger Shanghai Ruijin Hospital, and given dietary supplements. In that hospital, her growth hormone was greatly slowed down, although it is still a problem. On her return home to her mother and brother, she was able to use crutches, unassisted by others, and was given a six-month supply of medicines and supplements hoping to improve her condition enough to undergo surgery.

27Yao Defen currently suffers from hypertension, heart disease, poor nutrition, and osteoporosis. Acromegaly often results from a tumor within the pituitary gland that causes excess growth hormone secretion. As a result, the body's features become enlarged. It can also delay the onset of puberty as is Yao Defens case. She has no secondary sex characteristics. Potential complications without necessary surgery include blindness and eventually premature death. She lives near her mother (who is only 4 ft 8 inches tall) in a small village in rural China.

28The 2010 edition of Guinness Book of World Records lists Sultan Kosen (born 10 December 1982) as the tallest living man, having the largest hands (10.8 inches) (27.5 centimeters) and feet (14.4 inches) (36.5 centimeters). According to Guinness, Sultan Kosen is the tallest living human reaching an average height of 8'1" (2,46.5 m) when measured by GWR in Ankara, Turkey, in February 2009. He takes over the title from China's Bao Xishun, who stands 'just' 2.36 metres (seven feet 8.95 inches). However, Sultan Kosen was still growing and at a more recent meeting with then tallest man of the U.S. George Bell, Sultan Kosen was 8'2" tall.

29Sultan Kosenwas unable to complete his studies because of his extreme height, but works occasionally as a farmer to support his family. He has three brothers and a sister, who are all normal-sized, but his rate of growth occurred at the age of 10 because of a tumour which caused too much growth hormone to be released from his pituitary gland. The tumour was successfully removed through surgery and he was thought to have stopped growing last year (2008). He uses walking sticks and grows tired quickly if he stands. Another pretender to the tallest title, Ukrainian Leonid Stadnyk, who claims to be 10.5 centimetres taller than Sultan Kosen, fails to qualify for the record because he refused to be measured by Guinness World Records officials. Guinness editor-in-chief Craig Glenday travelled to Turkey to personally validate Sultan Kosen's height under strict guidelines, measuring him three times in one day because bodies expand and shrink throughout the day.

30However, others have claimed to be even taller. People of extreme height have always been exaggerating their true height. In history many giants have claimed to be the tallest men alive, or even the tallest men ever. Some of the circus, sideshow and freakshow giants did not allow themselves to be measured.

31Tall persons are often discriminated. In fact, all unknown or rare diseases are considered to be weird. People only see the surface and do not try to find out the essence.

32These are the feelings of a tall, abnormal person, surrounded by normal people:

33Yes, I'm considered tall. And you know what bugs me most? When people say, <<Oh, you're so tall!>>.

34Oh sure, there are advantages to being tall. You can see over the cubicles in the office "cube farm." You get a better view in a crowd without having to stand on your toes. I even recall a study that was completed that showed that taller people were usually paid better and were promoted more often than their shorter counterparts (although not studying the report in detail, there could be other factors that influenced this). But did you ever consider how often tall people hit their heads? Or how about squeezing into a small car? I would certainly like to have the option of owning a small car, but alas, it is the domain of the "height challenged" elite.

35Just because we're tall, doesn't mean we always have things handed to us on a plate. It doesn't mean that we should have to suffer because we are well beyond the "average" height. There is handicapped seating on buses, handicapped parking, even maternity parking. Why can't there be tall person reserved seating on airplanes? Is this such a terrible thing to accommodate? I don't mind giving my height when ordering an airline ticket, if it puts me on a list to get a seat with extra legroom.

36Yes, the sky IS blue in my world, and yes, I believe in a free and just society. Maybe I'm wrong on both counts because of all the bumps on my head.......or could it be that the bumps are just making me taller?

37Another person with acromegaly says: When I was born, I was a long baby. I am now 6 ft. 6 in. tall. Most of my clothing has to be bought from a mens large and tall shop. Most of these clothes cost more than regular clothes, considerably more. Just because my parents were tall and I am tall, is this discrimination that my clothes cost more?

38I've been discriminated against because of my stature at some of my jobs. My first job I was being trained as a cashier at a food joint, then abruptly I was put in the kitchen. My sister was friend's with one of the managers, and he told her that the reason that I was put into the kitchen was because I 'scared' the customers.

39I think height can be a disability. People have made extremely rude, inaccurate judgements about me because of my height, and some people, especially short men, are extremely intimated by tall people and do lash out.

40They should be evaluated on the basis of their qualifications, not their height. Inside the giant body is a human being exactly the same as you, as your neighbours, friends or family. The persons with acromegaly have the same feelings as all people do, have dreams, moments of joy or sadness. It is probably very hard to live under the skin of a huge body, but life is a miracle, although sometimes is difficult to adapt to its challenges. As Julia Child said: "Being tall is an advantage, especially in business. People will always remember you. And if you're in a crowd, you'll always have some clean air to breathe."

Bibliographie

References :

TORTORA G, GRABOWSKI S, 2003, Principles of anatomy and physiology, 10th ed. John Wiley and Sons.

R.SEELY, 2005, Esentials of anatomy and physiology, 5th ed. McGraw Hill.

COLLINS Douglas, 2007, Differential Diagnosis in Primary Care, , Lippincott Williams

& Wilkins.

SPRINGHOUSE, 2005 , Professional Guide to Diseases (Eighth Edition), Lippincott Williams & Wilkins

www.emedicine.medscape.com

http://www.thetallestman.com/

www.wings-over-the-world.blogspot.com

Pour citer ce document

Floriana Olivia SANDU, Gigantism and Acromegaly - living under the skin of an extreme body, Bulletin scientifique en langues trangres appliques [En ligne], L'anglais sur objectifs spcifiques, Numro 1/2013, Bulletin scientifique en langues trangres appliques, mis jour le : 19/04/2018, URL : http://revues-eco.refer.org/BSLEA/index.php?id=278.