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New Message on Pituitary Chat

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From: PTResearcher2
Message 1 in Discussion

  What causes gigantism? 
http://www.sciam.com/article.cfm?id=what-causes-gigantism-sandy-allen 
Yesterday, Sandy Allen, the world's tallest woman, passed away at the age of 
53. Endocrinologist Daniel Kelley explains how she grew so big 
By Nikhil Swaminathan  
Sandy Allen—the world's tallest woman, according to Guinness World Records—died 
on Wednesday (August 13) in the Indiana nursing home where she lived, the 
Associated Press reports. Allen was 53 years old and stood seven feet, seven 
inches (2.3 meters) tall—a full inch taller than Chinese basketball star Yao 
Ming. 
As a child, Allen developed a tumor in her pituitary gland—a pea-size structure 
at the base of the brain that secretes several hormones that are key to the 
body's function, including human growth hormone. As a result of the tumor, she 
developed gigantism—her bones grew excessively, causing, among other things, 
her prodigious height. 
With the large proportions came a host of health issues. Although the cause of 
Allen's death is unknown, she suffered from diabetes, frequent infections, 
breathing difficulties and kidney failure. She spent much of her later years 
confined to a wheelchair. 
After a career largely devoted to educating children about accepting others' 
differences, she returned to Indiana and eventually lived in Heritage House 
Convalescent Center in her hometown of Shelbyville. It's the very same nursing 
home where Guinness's oldest living woman, 115-year-old Edna Parker, resides. 
ScientificAmerican.com rang up Daniel Kelley, medical director of the 
Neuro-Endocrine Tumor Center at Saint John's Health Center in Santa Monica, 
Calif., to find out more about Allen's condition. 
Is gigantism a normal consequence of pituitary gland tumors?
I don't know her story at all, but it certainly sounds to me like she was an 
acromegalic giant. That's the only way it happens--if they had a pituitary 
tumor. The tumor is making excess growth hormone--it's a growth 
hormone-secreting adenoma that developed prior to her bone growth stopping, 
when she was still growing. If someone has excess growth hormone, they become 
excessively tall and become an acromegalic giant. 
How common are these pituitary tumors?
About one in 20 people will have an abnormality in their pituitary gland…. And 
based on some recent demographic studies, about one in 1,000 people probably 
have a symptomatic pituitary adenoma [the tumor Allen had]. So, a subset of 
those--maybe 10 percent would have acromegaly, but it's hard to know. 
According to the AP story on her death, Allen said she had her growth 
surgically stopped in her early 20s. How would doctors do that?
She probably had the tumor removed. The standard treatment for someone who has 
a pituitary tumor is to remove it through endonasal surgery [in which surgeons 
go through the nose to cut out the tumor]. The goal is remove all the adenoma 
to protect the normal pituitary gland. 
The other treatment options for someone with acromegaly or gigantism, as she 
had, is radiotherapy--radiosurgery or stereotactic radiotherapy—that is, giving 
focused radiation to the pituitary tumor to avoid damage to surrounding 
structures. 
Then the third are medications that block the effects of growth hormone or 
block the actions of insulin growth factor 1. That's what's called the "second 
messenger of growth hormone." So, when the growth hormone is released by the 
pituitary, the liver makes IGF1, and that's how you get the effects of growth 
hormone--through IGF1. 
For people with acromegaly, aside from the gigantism, are there other frequent 
health defects?
The problem with acromegaly is really quite severe. It's a benign adenoma 99.9 
percent of the time, but it can cause health problems including hypertension 
[high blood pressure], diabetes, obstructive sleep apnea [when breathing stops 
during sleep], carpal tunnel syndrome [pain, numbness or a tingling sensation 
in the hand or fingers]. So people with untreated acromegaly gigantism, in 
addition to the growth problems, they can have premature death because of the 
other associated problems. They get organomegaly--so all their organs get big, 
too--so they have a higher incidence of heart disease. Then you couple that 
with hypertension and diabetes and you can imagine they don't live very long. 
How old was she when she died?
Fifty-three. 
Is that on the high end?
No. Most people who get a growth hormone secreting adenoma are adults. Children 
or adolescents that get a growth hormone-secreting tumor develop gigantism, in 
addition to the other problems associated with the excess growth hormone, 
because their bones have not stopped growing. 
So, if you take your average adult who gets acromegaly--and say he has sleep 
apnea and hypertension and diabetes, if it's caught early enough, between three 
and five years, usually the hypertension and diabetes become easier to control. 
While a lot of the metabolic changes go away, the bony changes don't. What 
you'll see in someone with acromegaly is they get what's called frontal bossing 
[an enlarged forehead], prognathism--their jaw juts out--they get spreading of 
the teeth, they get enlarged hands and feet. Your height doesn't increase, but 
your shoe size and ring size goes up. Your tongues gets big; that's why you 
snore a lot and get sleep apnea. And then your organs get big. 
How does the pituitary adenoma arise?
Most people with pituitary adenoma have a spontaneous [genetic] mutation. We 
don't really quite know what triggers them. There are some instances--there's 
something called multiple endocrine neoplasia, which is associated with 
familial pituitary tumors. But, that's pretty uncommon.

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