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June 21, 2006
 

 

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In This Issue:HappeningsSite NewsMeetingsChat Info
Pictures from the Cushing's Awareness Day Medical Forum
Order the CUSH Cookbook
Adrenal Hyperplasia
CAH screenings in Israel
Cleveland Clinic Reports Experience with Laparoscopic Radical Adrenalectomy
Cortisol the chameleon
Student hopes to rebound from brain surgery, Rare disease caused teen to double weight in a year
Cushing's Syndrome is a Hormonal Condition
Hereditary Cushing's Syndrome Gene
Patients Fighting Cushings Disease
Growth Hormone, Recent advances
Growth Hormone, New drug approval raises hope for cheaper medicine
Neurosurgeon's Memoirs, Another Day in the Frontal Lobe: A Brain Surgeon Exposes Life on the Inside
Pheos, Laparoscopic partial adrenalectomy
Pituitary adenoma predisposition caused by germline mutations in the AIP gene
Pituitary Tumor: Teacher shares fitness lessons
Pituitary Tumor Removal, and video
Pituitary tumors caused by Risperdal?
Post-op pituitary, an actress deals with memory loss after surgery
Testing, NOVA - noninvasive optimal vessel analysis
Newest site features: bios, Helpful Doctors, Videos
US Postage Stamps for Cushing's Awareness
Order Cushing's Awareness Silicone Bands for yourself, a family member or donate to a Cushing's patient at NIH
Upcoming Meetings: ENDO 2006, Boston Convention & Exhibit Center.
Read all about them below.
News!

The Cushing's Awareness Day Medical Forum Display, made by Cheryl Farrar (CherylF), CUSH Vice president Pictures from the Cushing's Awareness Day Medical Forum.

See the first 200 photos in the New Photo Gallery

These pictures were taken by Christy and MaryO April 5-8, 2006




CUSH Cookbooks are here!

The CUSH Cookbooks are only $10.00 each including shipping and handling.

Any profits will go to help bring awareness for Cushings. Thank you!

The cookbooks have about 169 recipes, so it isn't going to be a huge cookbook, but one that includes contributions from many Cushing's message board members..

To purchase a cookbook send a check to:
CUSH
PO Box 1843
Florence, AL.
35631-1843

please indicate on your check "Cookbook" or include a note with payment.

You can also purchase cookbooks through Paypal. Please indicate that the payment is for "cookbook."

Be sure that your correct mailing address is included with payment along with a contact phone number in case we have questions concerning your order.

If anyone has any questions concerning cookbook payments please contact CUSH Treasurer Cathy Gifford at CUSHOrg@aol.com


News:
We welcome your articles, letters to the editor, bios and Cushing's information.
Submit a Story or Article
to either the snailmail CUSH Newsletter or to an upcoming email newsletter at
http://www.cushings-help.com/newsletter_story.htm

Note: These articles are provided in furtherance of the mission of Cushing's Help and Support to help people with Cushing's or other endocrine problems, their friends and families through research, education, support, and advocacy. These news items are intended to serve as background concerning its subject for patient-physician discussions and discussions among Cushing's Help and Support Message Board Members.

These articles contain information by authors and publishers that is subject to the Copyright Act of 1976, and "fair use doctrine" therein, effective on January 1, 1978 (17 U.S.C. § 101 et seq.). Cushing's Help and Support makes no representation that the information and any of the views or comments contained in these articles are completely accurate or current. Cushing's Help and Support takes no responsibility for any of the content.

The Cushing's Awareness Day Medical Forum Display, made by Cheryl Farrar (CherylF), CUSH Vice presidentPictures from the Cushing's Awareness Day Medical Forum in Oklahoma, April 2006.

See the first 200 photos in the new photo gallery at
http://www.cushings-interactive.com/photos/gallery/albums.php

These pictures were taken by Christy and MaryO April 5-8, 2006


CUSH Cookbooks are here!

The CUSH Cookbooks are only $10.00 each including shipping and handling.

Any profits will go to help bring awareness for Cushings. Thank you!

The cookbooks have about 169 recipes, so it isn't going to be a huge cookbook, but one that includes contributions from many Cushing's message board members..

To purchase a cookbook send a check to:
CUSH
PO Box 1843
Florence, AL.
35631-1843
please indicate on your check "Cookbook" or include a note with payment.

You can also purchase cookbooks through Paypal. Please indicate that the payment is for "cookbook."

Be sure that your correct mailing address is included with payment along with a contact phone number in case we have questions concerning your order.

If anyone has any questions concerning cookbook payments please contact CUSH Treasurer Cathy Gifford at CUSHOrg@aol.com



News:
We welcome your articles, letters to the editor, bios and Cushing's information.
Submit a Story or Article
to either the snailmail CUSH Newsletter or to an upcoming email newsletter at
http://www.cushings-help.com/newsletter_story.htm

Note: These articles are provided in furtherance of the mission of Cushing's Help and Support to help people with Cushing's or other endocrine problems, their friends and families through research, education, support, and advocacy. These news items are intended to serve as background concerning its subject for patient-physician discussions and discussions among Cushing's Help and Support Message Board Members.

These articles contain information by authors and publishers that is subject to the Copyright Act of 1976, and "fair use doctrine" therein, effective on January 1, 1978 (17 U.S.C. § 101 et seq.). Cushing's Help and Support makes no representation that the information and any of the views or comments contained in these articles are completely accurate or current. Cushing's Help and Support takes no responsibility for any of the content.

Acromegaly From http://www.news-leader.com/apps/
pbcs.dll/article?AID=/20060613/
LIFE04/606130308/1035

Published June 13, 2006

MEDICAL Q& A »


Acromegaly linked to growth hormone

Condition, which plagued wrestler Andre the Giant, has multiple symptoms and complications.

Jennifer M. Phelps
News-Leader

This week's expert is Dr. JonBen D. Svoboda, an endocrinologist with CoxHealth's Center for Internal Medicine in Springfield.

Q. How is acromegaly defined?

A. Acromegaly is a hormonal disorder caused by an excess of growth hormone, usually secreted by a lesion in the pituitary gland, located in the brain.

During childhood, growth hormone has a crucial role in attaining a person's optimal height. In adulthood, growth hormone plays a subtler role in cholesterol management, blood sugar production, the production of protein, and phosphorus, sodium and water retention.

Q. Where does the word originate?

A. From the Greek words for extremities (acro-) and enlargement (-megaly). The word reflects one of the condition's most common symptoms, the abnormal growth of the hands and feet.

Q: Is it true that tumors are the most common cause of the condition?

A: Yes, the vast majority arises from tumors. Ninety percent of patients with acromegaly have oversecretion of growth hormone from the pituitary gland, but a small percentage can have secretion from a lesion in the pancreas, lung or adrenal gland.

Q. Can you explain the change that takes place in the body when too much growth hormone is secreted?

A. You can get arthritis from the increase in size of bone and cartilage of the arms and legs. The growth of this tissue could also entrap nerves, leading to carpal tunnel syndrome.

A person can have enlargement of their lips, tongue, nose, deepening of their voice or increased hat or shoe size.

A person also can develop diabetes, as growth hormone causes production of blood sugar by the liver, and it can increase the risk of cardiovascular disease and hypertension, due to the metabolic effects of growth hormone. Patients with acromegaly also have an increased risk of colon polyps and cancer.

If a person develops acromegaly during childhood before the long bones have fused, they develop gigantism. If a person develops acromegaly during adulthood, after the long bones have fused, the changes may be subtler, as outlined above. The deceased wrestler Andre the Giant suffered from this disorder, which he developed after his long bones had fused.

Q. What are some common symptoms of acromegaly?

A. Elevated blood sugar; hypertension; thick, oily skin; skin tags; enlarging lips, tongue and nose; worsening snoring; excessive sweating; deepening of the voice; menstrual irregularities in women; impaired vision; and enlargement of the liver, spleen, kidneys and heart.

Q. When should someone seek medical advice?

A. On reading the laundry list of symptoms above, if I looked at each symptom alone, I would begin to think I had acromegaly myself. But when I look at these symptoms as a group, the more of them I have, the more likely I am to have the condition. If many of the above symptoms ring true, it may be advisable to ask your primary-care physician for an opinion.

Q: How is a diagnosis made?

A: This is tricky, because if we just measure a growth-hormone level, patients with acromegaly can have a normal level, and normal people can have a growth-hormone level five times above the upper limits of normal. This is because growth-hormone secretion is always changing.

A much more stable screening test is IGF-1, which is a protein made by the liver in response to secretion of growth hormone from the pituitary.

If growth-hormone levels are consistently high over time, the IGF-1 level will likely be elevated.

An even better test is a glucose-suppression test, in which growth hormone is measured after 75 grams of a sugar drink. A normal response to this test is to have the growth hormone drop. In patients with acromegaly, growth hormone is not usually suppressed in this situation.

Q. What does treatment involve, and is surgery required?

A. The most successful treatment is surgical removal of the lesion that produces growth hormone. If the lesion is diagnosed in the later stages, medications and radiation may be needed to suppress production of growth hormone from the remaining tumor.

Q. Once treated, will parts of the body affected by acromegaly return to a normal size?

A. There may be some improvement in the overall symptoms, but usually the body size does not change dramatically after normalization of growth-hormone levels.

Adrenal Hyperplasia From http://www.hindu.com/thehindu/holnus/008200606121340.htm

Lack of key enzyme associated with development of rare tumor

June 12: Researchers at the National Institutes of Health have discovered that a rare tumor of the adrenal glands appears to result from a genetic deficiency of an important enzyme. The enzyme is one of a class of enzymes involved in halting a cell's response to hormones and appears to stop cells from dividing.

The study, published in Nature Genetics and released by EurekAlert was conducted by researchers in NIH's National Institute of Child Health and Human Development. The NIH group collaborated with scientists from the Mayo Clinic, the Cochin Institute in Paris, the University of Paris, Ohio State University in Columbus, and the Universitaire Vaudois in Lausanne, Switzerland, in collecting samples from patients with rare adrenal disorders. Scientists from Sapio Sciences in York, Pennsylvania, assisted in the analysis of the data.

In conducting the study, the researchers used gene arrays to analyze the DNA of patients with a rare tumor of the adrenal glands, known as micronodular adrenocortical hyperplasia, explained the study's senior author, Constantine Stratakis, M.D., D(Med)Sc, Chief of NICHD's Section on Endocrinology and Genetics. The researchers also used the technology to analyze samples of the patients' tumors.

The researchers found four patients who had mutant copies of a gene that contains the information for Phosphodiesterase 11A (PDE11A). Phosphodiesterases are a family of enzymes involved in "switching off" a cell's response to hormones, Dr. Stratakis explained.

For a hormone to affect the cell, it must first bind to a molecule, or receptor, on the cell's surface, analogous to how a key fits into a lock. This action triggers the cell to produce substances known as cyclic nucleotides. These function as "second messengers," often stimulating the cell to begin an activity. In the case of adrenal cells, cyclic nucleotides, such as cyclic AMP and cyclic GMP, may stimulate cell growth or other activities. Once the activity has ended, phosphodiesterases degrade the cyclic nucleotides, thereby halting the cell's response to the hormone.

In the study, the patients' tumors were made up of cells that were deficient in the enzyme PDE11A. This enzyme halts cyclic nucleotide production in adrenal cells as well as in other kinds of cells in the body. Because they lacked PDE11A, the patients' adrenal cells had higher levels of cyclic nucleotides. The researchers believe that these higher cyclic nucleotide levels led to the formation of tumors.

The gene for PDE11A contains the information needed to make 4 slightly different forms of the enzyme. The form of the enzyme that was mutated in the patients who took part in the study was found in large amounts in normal adrenal glands and in even larger amounts in normal prostate glands, Dr. Stratakis added. Other forms of PDE11A are found in several other tissues, including the testes, skeletal muscle, and the heart.

Dr. Stratakis noted that although the evidence associating the mutation in the gene for PDE11A to the development of adrenal tumors was very strong, the study was not capable of proving that the mutation actually caused the tumors. In their article, the researchers wrote that drugs used to treat erectile dysfunction interfere with the functioning of PDE11A. The researchers noted that PDE11A "is partially inhibited" by the drug tadalafil and "weakly" inhibited by sildenafil. They added that there are no reports in the medical literature of malfunctioning adrenal glands or increased adrenal cell growth in users of these drugs.

"However, detailed clinical studies addressing this potential complication are currently lacking," they wrote. Dr. Stratakis and his colleagues are currently planning studies to determine if differences in the gene for PDE11A might influence an individual's cancer risk.

Adrenalectomy Robin (Staticnrg on the boards) found this article:

Cleveland Clinic Reports Experience with Laparoscopic Radical Adrenalectomy

Written by Michael J. Metro, MD
Monday, 31 January 2005
BERKELEY, CA (UroToday Inc.) - Laparoscopic adrenalectomy has become the gold standard approach for benign surgical adrenal disorders such as aldosteronoma, Cushing's disease and pheochromocytoma. BERKELEY, CA (UroToday Inc.) - Laparoscopic adrenalectomy has become the gold standard approach for benign surgical adrenal disorders such as aldosteronoma, Cushing's disease and pheochromocytoma. However, laparoscopic adrenalectomy for solitary metastasis or primary adrenal cancer remains a matter of considerable debate. Adrenal cancer rightfully confers the possibility of carcinomatosis or port site metastasis, as noted in initial published case reports. Given the controversial nature of this topic, the group at Cleveland Clinic headed by Dr. Inderbir Gill reviewed their single center experience with laparoscopic adrenalectomies for malignancy. Their results are reported in the February, 2005 issue of the Journal of Urology.

Their cohort comprised 31 patients (33 adrenalectomies) with preoperative suspicion of a solitary metastasis to the adrenal gland, and those who were incidentally found to have primary adrenal malignancy. Selection criteria for the laparoscopic approach on pre-operative CT were an adrenal mass of 10 cm or less without evidence of peri-adrenal infiltration, caval thrombus or bulky locoregional adenopathy.

A closer look at the study population revealed a mean age of 59.8 years. Mean tumor size was 5 cm. The laparoscopic approach was transperitoneal in 17 cases, retroperitoneal in 15 and transthoracic in 1. Pathology reports indicated a diagnosis of adrenal metastasis in 26 patients; with the primary cancer being renal cell carcinoma (RCC) in 13 patients, colonic metastasis in 5, and lung metastasis in 4 patients. Six patients had primary adrenocortical carcinoma (ACC). Surgical margins of the adrenalectomy specimen were negative for cancer in 19 cases (56%), indeterminate in 2 (6%) and positive in 1 (3%). The pathology report made no mention of margin status in 11 patients (33%).

Analysis revealed a current median follow-up of 26 months. Overall, 15 patients (48%) died and 16 (52%) were alive. Of these 13 (42%) had no evidence of disease. Local recurrence was noted in 7 patients including 3 with metastatic RCC, 2 with metastatic colon cancer and 2 with primary ACC. Surgical margins had been positive in one patient with a local recurrence. One patient with bilateral metastatic adrenal masses from RCC developed carcinomatosis 7 months postoperatively. They noted no port site metastases. Survival was similar in patients with tumors less than 5 cm vs. 5 cm or greater. Survival was not associated with patient age, tumor size, operative time or surgical approach. Survival was compromised in patients who developed local recurrences, with a median survival of 17 months.

In conclusion, their study shows that laparoscopic radical adrenalectomy can be performed with acceptable outcomes in carefully selected patients with a small, organ confined, solitary adrenal metastasis or primary adrenal carcinoma. The results of this group from Cleveland Clinic compare favorably with a contemporary open series from Memorial Sloan Kettering Cancer Center.

J Urol. 2005 Feb; 173(2):519-25

CAH Screening From http://www.htscreening.net/index.aspx?ID=73073
PerkinElmer to Expand Neonatal Screening Program in Israel

Date Posted: Tuesday, June 13, 2006

PerkinElmer, Inc. has announced that its neonatal screening technology has been selected by State of Israel's Chaim Sheba Medical Center, Tel Aviv, to create a comprehensive newborn screening program that is intended to cover every child born in Israel.

Under the terms of this agreement, PerkinElmer will provide the State of Israel with instrumentation, reagents, scientific expertise, and an informatics system that will enhance the nation's neonatal screening and data management capabilities.

The contract, to be executed through PerkinElmer's regional distributors HVD Vertriebs Gmbh and Gamidor Limited, will fulfill a recent mandate by the Medical Research Infrastructure Development and Health Services Fund to substantially expand its newborn screening program to cover Israel's entire population and increase the number of tests that will be screened for indications of disease.

"We are honored that the State of Israel has selected PerkinElmer to expand its newborn screening program," said Robert F. Friel, president, PerkinElmer Life and Analytical Sciences.

"We look forward to working with the Chaim Sheba Medical Center to improve the standard of healthcare to newborns," Friel added.

As part of this agreement, Chaim Sheba will use Specimen Gate®, PerkinElmer's screening laboratory information management system.

This software will allow Chaim Sheba to implement a comprehensive solution that will electronically track laboratory workflow from sample receipt and preparation, through the analytical steps, to quality control review, reporting and follow-up.

Israel's enhanced neonatal screening initiative will be built around PerkinElmer's AutoDELFIA® automatic immunoassay system.

To augment Israel's screening capacity in order to accommodate additional tests, PerkinElmer will deliver multiple AutoDELFIA units as well as reagents and equipment.

The three tests that will be sourced from PerkinElmer for Israel's expanded neonatal screening program are: 17(alpha)OH progesterone for screening congenital adrenal hyperplasia (CAH), a hormonal disorder affecting the adrenal glands; and hTSH and T4, both for screening congenital hypothyroidism (CH), which if left untreated can lead to severe mental retardation.

Further Information: http://www.perkinelmer.com

Cortisol From http://www.azcentral.com/arizonarepublic/
arizonaliving/articles/0613cortisol0613.html

Cortisol the chameleon

Hormone fights stress but can lead to dangerous belly fat

Connie Midey
The Arizona Republic
Jun. 13, 2006 12:00 AM

For all the research into cortisol's association with stress, belly fat and poor health, the substance remains a mystery to many people.

"It's not a simple hormone," says Malcolm Low, associate director of the Center for the Study of Weight Regulation at Oregon Health and Science University in Portland. "The issue is trying to sort out the things that are caused directly by too much of it."

Psychologist Marci Gluck, a National Institutes of Health researcher on assignment at Phoenix Indian Center, says studies have established a relationship between high cortisol and potentially dangerous abdominal fat.

"But we haven't really come up with a direct mechanism for how all of this is happening," she says. "If it were just as simple as reducing cortisol, I'm sure the pharmaceutical companies would have picked up on that already."What is cortisol?

Cortisol is a naturally occurring steroid hormone made by the adrenal glands, the triangular glands located on top of each kidney.

"It's an essential hormone for life," Low says. "It acts on virtually every cell in the body."

Levels rise and fall in 24-hour cycles, usually reaching highs from 6 to 8 a.m. and lows about midnight.

But like the adrenaline also secreted by the adrenal glands, cortisol production increases with the body's "fight or flight" response to stressful situations, whether physical or emotional.

How cortisol protects us

Cortisol in healthy amounts helps the body respond to stress, produce energy, regulate heart functions, normalize blood sugar and fight inflammation. Delivered in occasional short bursts, it can increase odds for survival, heighten memory and lower sensitivity to pain.

Synthetic forms, known as hydrocortisone, are used in oral, topical and injected forms to fight allergic reactions and inflammation.

How cortisol can harm us

When chronic stress keeps cortisol levels persistently high, the hormone "switches from being a protector to being a very toxic substance," says Larry Woodruff, senior lecturer in the exercise and wellness department at Arizona State University Polytechnic in Mesa.

It can destroy tissues throughout the body, slow healing and impair memory, and it plays a role in the development of abdominal fat, which has been associated with heart disease and insulin resistance.

What studies are finding

While working in New York, Gluck studied the effects of stress on people with binge-eating disorder. Statistically, they had "nearly significant" higher cortisol levels than the control group after being subjected to stress and were significantly hungrier in the following hour.

(After controlling for insulin levels, the binge eaters' cortisol levels were "significantly" higher.)

A University of California-San Francisco researcher found that healthy, lean women who produced high levels of cortisol in response to stress ate more sweet, high-fat foods when left alone in a room afterward with a basket of snacks, Gluck says.

Low says laboratory mice given a form of cortisol at low levels start eating more.

"It seems to have a permissive, stimulatory effect on their appetite," he says. "But you can't treat that problem by getting rid of the cortisol. You need it for other functions."

Although Low says cortisol research doesn't necessarily prove a cause-and-effect relationship, the hormone's role in other areas of health also is being examined.

In a pilot study, Stanford University researchers measured irregular cortisol levels in Alzheimer's caregivers that put them at risk for heart disease and some cancers.

And an NIH study of monkeys that responded to stress in infancy with high cortisol concentrations found they were more likely to drink alcohol as adults than low-cortisol responders.

'Blocking' supplements

Dietary supplements that claim to aid weight loss and prevent disease by controlling production of cortisol have been criticized as ineffective by the non-profit Center for Science in the Public Interest and the Federal Trade Commission.

"Most of these supplements contain vitamins and natural plant extracts," Low says. "It's pretty much unproven that any of them would have any effect on cortisol."

And even if they did, the supplements wouldn't cause significant weight loss, he says. Most people put on extra weight because they eat too much and exercise too little, not because they have too much cortisol.

Modifying levels naturally

Anything that relieves stress - yoga, reading, music, journaling, a nap - can help lower cortisol levels, Low and Gluck say.

Woodruff says regular exercise will, over time, moderate the adrenaline and cortisol response to stress.

As a result, he says, "the physiological response of an aerobically fit person to stress will be less intense and harmful than that of an unfit person perceiving the same general level of stress."

Gluck suggests preparing for situations that usually lead to stress eating.

"Instead of M&Ms, have healthy foods in your kitchen or workplace," she says. "Most people say, 'I'm not going to do it this time,' but that can be very unrealistic. It's much better to prepare, just in case."

Reach the reporter at connie.midey@arizonarepublic.com or (602) 444-8120.

Cushing's From http://activepaper.olivesoftware.com/Repository
/ml.asp?Ref=Q1RGLzIwMDYvMDYvMTcjQXIwMDEwM w==&
Mode=HTML&Locale=english-skin-custom


Publication: Waukesha Freeman (Conley)
Date:
Jun 17, 2006
Section:
Front Page
Page Number:
1A

Student hopes to rebound from brain surgery
Rare disease caused teen to double weight in a year

by LAWRENCE SILVER Freeman Staff

WAUWATOSA – Sitting in her hospital bed just two days after brain surgery, Claudette Ingold felt positive about her chances of returning to school.

Ingold missed parts of the last school year due to excessive drowsiness and weight gain caused by Cushing’s disease, a rare syndrome that causes the pituitary gland to send out abnormal amounts of certain hormones.

“My goal is to get back to school," Ingold said. “I want to see my friends."

Thanks to a recent successful brain surgery, Ingold has a new shot at a normal life.

The past year has been anything but normal for the 14-year-old, however.

Dr. Glenn Meyer, a Medical College of Wisconsin neurosurgeon, said Cushing’s disease is characterized by a growth near the pituitary gland that causes an abnormal amount of the adrenocorticotropic hormone to be released by the gland into the body.

Meyer, who performed the surgery on Ingold, said the disease causes excessive weight gain, hypertension and diabetes among other problems.

“It affects the body in many complex ways," Meyer said.

Ingold’s mother, Maureen, said her daughter’s weight nearly doubled from 110 pounds in the last year.

The rapid weight gain caused stretch marks to form on the adolescent’s skin from head to toe, Maureen Ingold said.

“She was unrecognizable," Maureen Ingold said.

But initially, Maureen Ingold said, doctors were hesitant to diagnose the disease. They felt she just needed more exercise and a better diet, she said. But the more she dieted, the more weight she gained.

Maureen Ingold said parents should learn about Cushing’s disease if their child has hypertension or is developing diabetes.

“Parents need to realize and push when they are sure their child is not just overweight," Maureen Ingold said. “Children shouldn’t have those problems. Their body should be on the way up, not the way down."

Meyer said he was able to successfully remove the growth during the surgery.

He said Claudette Ingold’s body should be on the way up.

She’ll mature naturally, he said, but she will have to take hormone supplements for the rest of her life.

“She will need careful followup," Meyer said. “Time well tell."

Maureen Ingold said she hopes her daughter will take something away from the adversity of the past year.

“I hope maybe there is some direction of life she can take from this," Maureen Ingold said. “I hope she will want to go help somebody after this."

Claudette Ingold said the first thing she plans to do when she gets healthy is spend time with her friends.

“I believe I am going to get better," Claudette Ingold said. “I just have to hope."






Submitted photos Due to Cushing’s disease, 14-year-old Claudette Ingold of Waukesha gained nearly double her approximate 110-pound weight from March 2005 to April 2006. Ingold had surgery Tuesday to remove the benign tumor in her brain that was causing the problem.

Cushing's From http://www.forbes.com/forbeslife/health/
feeds/hscout/2006/05/26/hscout532783.html

Health Tip: Cushing's Syndrome is a Hormonal Condition
05.26.06, 12:00 AM ET

(HealthDay News) -- Cushing's syndrome affects as many as 15 million people each year, typically between the ages of 20 and 50. It occurs when the body's tissues have been exposed for an extended period to too much of a hormone called cortisol.

According to the U.S. Endocrine and Metabolic Diseases Information Service, people with Cushing's often gain weight in the upper body, and have an abundance of fat around the neck, a round face, and thin arms and legs. Cushing's also leads to weakened bones and thin skin, which may bruise easily.

Treatment options include surgery, radiation, chemotherapy or a medication regimen that prevents production of cortisol, the NIDDK says. If medications taken for other conditions are causing Cushing's, your doctor may adjust the dosage to control both problems.

Cushing's From http://www.medicalnewstoday.com/
medicalnews.php?newsid=45052

Hereditary Cushing's Syndrome Gene
13 Jun 2006 - 0:00am (PDT)

Two Seville-based scientists of the Molecular Genetics Laboratory of Virgen Macarena Hospital have discovered the gene responsible for the hereditary Cushing's syndrome, a disease that is the result of an increase of the blood cortisol level, a hormone produced by the adrenal glands; patients suffer a serious of symptoms such as obesity, marks in their face, chest and shoulders, sometimes with an infection and an increased quantity of urine and excessive thirst (which may indicate the excess of glucose in the blood), among others.

According to the work carried out by doctors of the University Hospital, the fault is in the gene of the protein kinase A of chromosome 17. The mutation increases the quantity of cortisol in the blood anomalously, which shows the first symptoms when boys and girls reach puberty. ‘Generally, they begin to put on weight without a justified cause, their blood pressure increases, they have menstruation disorders and violet stretch marks can appear in their breasts, hips and legs', said Alfonso Gentil, assistant lecturer of the Endocrinology Department of Virgen Macarena Hospital.

This research work describes the mutation found in 12 families in France, the USA and Spain, and connects for first time the Cushing's syndrome with a specific genetic anomaly. That's why this disease can be eventually diagnosed in molecular genetics laboratories on a prenatal basis or before it becomes clinically apparent, as it will be possible to identify what relatives of a patient are disease carriers.

This research was carried out at the Molecular Genetics Laboratory of Virgen Macarena Hospital, in Seville, and led by Dr. Miguel Lucas, from a study made in the Endocrinology and Nutrition Department by Dr. Gentil. It consisted on genetically assessing nine members of a family, where two of them -an 18-year-old girl and her aunt, in the 40's- were already diagnosed and surgically treated to eliminate the syndrome. The clinical trials consisted of extracting the DNA from the blood in order to check the segregation and link to gene of the protein kinase A and after that, determinate the sequence.

When the results were ready, the researchers of Virgen Macarena Hospital contacted Constantine Stratakis, a prestigious pathologist of Bethesda University, in Maryland, USA, who after learning of the Seville family case, put the Seville doctors in contact with the Cochin Institute of Paris in order to publish the work in the May edition of the international journal ‘Journal Clinical Endocrinology and Metabolism' .

About the ANDALUCÍA INVESTIGA

Andalusia's I+D+I public system includes over 18,000 researchers and more than 1,700 research groups. Together with Madrid and Cataluña, Andalusia has a great potential, with 14% of the nation's total scientific output. This privileged position has been possible thanks both to the regional government's financial support to the I+D+I and the aid of the private sector. Last January 9, the Andalusian Programme for the Spreading of Scientific Knowledge created the news agency InnovaPress in Spanish, with the aim of providing prompt information about scientific developments in the region. The project follows the European Commission's guidelines for the creation of a major scientific news agency that will enhance the spread of such information, and will serve as a new tool for the EU's economic reforms set out in Lisbon, as debated in Brussels last December 2004. Now, from April 3rd, InnovaPress will also be available in English, in an attempt to spread scientific developments and results to mass media, researchers, and universities. The Andalusian Programme for the Spreading of Scientific Knowledge is sponsored by the Andalusian Ministry of Innovation, Science and Enterprise. This is a pioneering project in Europe, which has managed to gather research, higher education and knowledge-based industry, I.T.s, and entreprenurial, industrial and energy policies. With the interaction of all these agents in mind, the Andalusian Ministry of Innovation, Science and Enterprise intends to create a major social alliance that will enable an “innovation explosion" in Andalusia as exclusive guarantee of progress and welfare development. In this vein, the Andalusian Regional Government has increased its investment in I+D+I by 37% with regard to the previous year, when investment was also increased by 32%. This shows the government's commitment to double investment in this area in the course of the present term of office. Some of the actions carried out by the regional government include a brand new Multi-annual Plan for University Infrastructure Investment for the period 2006-2010, with a total budget of 480 million Euros for the ten Andalusian public universities. With this new Plan, investment in university infrastructure and equipmentmte agents in mind,ry policiesm groups. is increased by 32% with regard to the previous five-year period. Also, within five years, the Plan will match all investment made in the last 12 years (1994-2005), which amounted to 504 million Euros. Also, a new system of incentives has been established for Research Groups and Excellence Projects, reaching a total of 87 million Euros, which means an increase of 79 million with regard to the previous official announcement made by the regional government before the new system was in force. In the new system, 12 million Euros have been allocated as incentives to the work of 1731 research groups, and 30 million more have made it possible to start 219 excellence projects dealing with Life Sciences, Food and Agriculture, Information Technologies, etc. Through InnovaPress, the Andalusian Programme for the Spreading of Scientific Knowledge will channel this new sustainable economic and social model, based on the creation of social welfare, respect for the environment and equal opportunities, within the major framework of the second modernisation of Andalusia.

ANDALUCÍA INVESTIGA
Consejería de Innovación, Ciencia y Empresa
c/ Albert Einstein s/n

info@andaluciainvestiga.com
Cushing's from http://www.medfordnews.com/articles/
index.cfm?artOID=330628&cp=10996

Monday, May 15, 2006
Oregon Health & Science Univ Help
Patients Fighting Cushings Disease

Portland, Oregon - A doctor didn't advise Vermont's Karen Nolan*** (Rooon on the boards) that she might be one of the scant 3.5 per million people diagnosed annually with Cushing's disease - another Cushing's patient did. After reading Nolan's post on an Internet message board, another patient suggested Nolan's lab results and symptoms could indicate pituitary disease and that help might be found more that 3,000 miles away at Oregon Health & Science University.

"She sent me a link to the Cushing's Help and Support Web site (www.cushings-help.com) a patient-created and maintained site, and suggested an Oregon doctor known for solving difficult cases," said Nolan. That doctor is William Ludlam, M.D., Ph.D., director of the OHSU Pituitary Unit, one of the largest neuroendocrine centers in the country.

Cushing's disease is a form of Cushing's syndrome and is caused by a tumor in the brain's pituitary gland that secretes excess levels of a hormone called ACTH. Elevated ACTH in turn stimulates the adrenal glands to produce excess cortisol, wreaking havoc on the body. Symptoms of the disease include: uncontrolled obesity, rounded face, increased fat around the neck, and fragile skin that bruises easily and heals poorly. The bones are weakened, and routine activities such as bending, lifting or rising from a chair may lead to backaches, rib and spinal column fractures.

"Cushing's tumors are almost always benign. However, they can be devastating to a patient's health and quality of life. They can even be fatal without proper treatment," said Ludlam, a professor of medicine (endocrinology, diabetes and clinical nutrition) in the OHSU School of Medicine. "The Internet is changing the face of rare diseases like Cushing's. Patients tell me they simply "Googled" their symptoms and there it was."

Cushing's can be difficult to diagnose. Many patients who contact Ludlam first test positive for the disease but subsequent test results are negative. In addition, because the disease is relatively rare, all physicians do not necessarily have the expertise to effectively identify the disease. In fact, many patients are repeatedly told they are not even sick and that the problem can be solved simply through diet and exercise.

"Many times with Cushing's, only the most textbook cases are readily diagnosed," explained Ludlam. "However, even these patients, who display the physical signs of the disease and have consistently positive tests often go undiagnosed for years. But there is also a large pool of people with Cushing's completely below the radar of most clinicians. They might not have the physical symptoms or possibly their biochemistry isn't consistently positive, but they still have it."

These two photos taken a few years apart of Cushing's patient Lisa Eldridge demonstrate the tremendous impact the disease can have on a person's body weight.

"When my primary care physician suspected Cushing's, he sent me for evaluation," said Lisa Eldridge ***, who is now Nolan's support group friend. "After having both positive and negative tests for Cushing's and an unusual thyroid finding, I was told I had Graves Hyperthyroidism, a disease known for making a person rail thin. However, I was gaining weight uncontrollably. After consulting the Internet, I was even less convinced my problem was thyroid in nature. If I had Cushing's, I knew it was intermittent. At this point I'd lost eight years of my life."

Historically, according to Dr. Ludlam, Cushing's patients are sick on average five to 10 years before diagnosis.

Eldridge's continued research led her to realize that she needed a specialist who recognized the disease could be intermittent while utilizing a conservative, yet aggressive testing protocol. Having an experienced pituitary neurosurgeon on the team who handled 50 or more cases a year was also a must.

"The one thing I was sure about after all my research was my choice in Dr. Ludlam and the OHSU team. Whether he told me I did or didn't have Cushing's, I knew his was an opinion I could trust."

Both Nolan and Eldridge met Jaimie Augustine*** on Cushing's Help and Support. The Three Musketeers, as they call themselves, instantly bonded last year after finally meeting in person at OHSU. Augustine, a California native, even refers to Karen as her Cushie Mama. At 22, Augustine already has had two pituitary surgeries aimed at combating the disease.

"According to an old Irish proverb: 'It's better to laugh than to cry,'" says Augustine, "We laugh over everything, especially our wildly fluctuating weight gain. Nothing's sacred. It's the only way to get through this."

Nolan is the group's entertainment director during trips to OHSU.

"Whenever we fly out to Portland, there's always a Cushie party in the works," said Nolan, "We bring the "Testers," the "Pre-ops" and the "Alumni" returning for post-op testing together. And of course we always enjoy seeing our local Cushies from Oregon and Washington State". Eldridge, who grew up in Maine muses, "Oregonians are like Mainers-very warm and friendly. Traveling to Portland is like going home."

For more information on OHSU, go to http://www.ohsu.edu/


*** Message Board Members

Growth Hormone From http://www.therapeuticsdaily.com/
news/article.cfm?contentValue=940964
&contentType=sentryarticle&channelID=26

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Reports from University of Padua describe recent advances


Health & Medicine Week - Jun. 08, 2006

2006 JUN 8 - (NewsRx.com) -- Reports from University of Padua describe recent advances.

This trend article about University of Padua is an immediate alert from NewsRx to identify developing directions of research.

Study 1: According to recent research from Italy, recombinant human growth hormone (GH) affects metabolic parameters and preclinical atherosclerotic markers in hypopituitary patients with growth hormone deficiency.

"This study examines the effects of growth hormone replacement on body composition, insulin sensitivity, lipid profile, endothelial dysfunction and carotid intima media thickness in patients with adult-onset growth-hormone (GH) deficiency," wrote S. Benedini and colleagues, University of Padua.

They ...

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The complete article is 1002 words long.

Growth Hormone From http://www.mercurynews.com/mld/
mercurynews/news/local/14755407.htm

Tue, Jun. 06, 2006
New drug approval raises hope for cheaper medicine
BIOTECH COMPANIES QUESTION EFFECTIVENESS

By Steve Johnson
Mercury News

When federal regulators approved a new version of a popular growth-hormone drug last week, some people saw a big crack develop in the wall that has protected biotechnology products from cheaper imitations.

Until now, there have been few if any low-cost copycat versions of biotech drugs, leaving many patients who take such medicine saddled with staggering pharmacy bills.

Biotech companies have largely blocked the sale of imitations, arguing that the drugs are unproven and could be unsafe.

``This is a very serious potential issue,'' said Matthew Gardner, president of BayBio, which represents biotech firms in the Bay Area.

But patient advocates claim consumers are being hurt by not having more access to biotech copies. That's why they're hailing the approval of Omnitrope, a new version of growth hormone, as great news for people like nine-year-old Oliver Young of Larkspur. He takes growth hormone to counteract a genetic disorder called Prader-Willi syndrome, which can cause short stature, learning problems and speech defects, among other problems.

No price has been set for Omnitrope. But it is widely expected to be at least 25 percent cheaper than current growth-hormone drugs. They can cost $20,000 a year, with some insurers requiring patients to pay about half that themselves.

``Coverage varies dramatically, from families who have small to minimal co-payments to others who are paying $800 a month,'' said Oliver's mom, Wendy Young, who runs a Prader-Willi support group. ``It's better for everyone if ultimately this medicine becomes less expensive.''

Generic versions of traditional, chemically synthesized drugs are common. The U.S. Food and Drug Administration routinely permits generic copies to be sold once the patent on the original expires. And because generics don't have to undergo the same rigorous testing as the original, they cost less to make and can be priced lower.

The savings to consumers can be substantial. Once a second generic version of a drug hits the market, it usually sells for about half the original's price and multiple copies often cost 80 percent less, according to the FDA.

But biotech executives have largely objected over the years to generic versions of their medicines. Because biotech drugs are made through biological processes and tend to be complicated to manufacture, the industry has long claimed it's unwise to assume generic versions are as safe and effective as the original. Consequently, Congress and the FDA have resisted allowing biotech copies.

Nonetheless, with patents for many biotech drugs expiring soon, generic manufacturers are pressuring the government to ease those restrictions.

In July 2003, Sandoz, a generic drug maker owned by Novartis of Switzerland, asked the FDA to let it sell Omnitrope. The drug, modeled after Pfizer's market-leading Genotropin, generally is regarded as a relatively easy biotech product to make. And because much already is known about how growth hormones work, Sandoz argued that the FDA should approve it quickly.

But the idea was opposed by the Biotechnology Industry Organization and some of its members, notably Genentech of South San Francisco, which sells a different kind of growth-hormone drug. The objections prompted the FDA to take its time in reviewing Omnitrope. Sandoz had to get a federal court order in April this year to force the agency to make a decision.

In approving Omnitrope on May 30, the FDA insisted it wasn't inviting a flood of biotech generics.

For one thing, it said Omnitrope technically wasn't a generic, which would have required it to be biologically equivalent to Genotropin. Instead, the FDA said, Omnitrope was a ``follow-on'' medicine, meaning it only needed to be similar.

Moreover, the FDA said the law generally barred it from permitting biotech imitations. It noted that growth-hormone and insulin products are governed by the Federal Food, Drug and Cosmetic Act, which permits copies. But vaccines and most other biotech drugs are under the Public Health Service Act, which would have to be amended to allow for generics, the FDA said. Even so, the FDA's action is viewed by some as opening the door for biotech imitators.

Omnitrope is the first FDA-approved biotech copy not produced by the original drug's manufacturer and Sandoz intends to market it as a generic. Moreover, Rep. Henry Waxman, D-Los Angeles, is considering introducing an amendment to the Public Health Services Act this summer, according to his staff.

Waxman had been waiting for the FDA to act on Omnitrope before introducing the measure. By approving Sandoz's drug, his staff said, Waxman believes the agency now has provided evidence that generic manufacturers can make biotech copies as good as the original.

Passing such an amendment could prove difficult, given the opposition of politically influential drug firms.

``When and if this comes to Congress, our voices will be heard,'' said Walter Moore, Genentech's vice president of government affairs.

But with consumers clamoring for cheaper drugs, other experts say, many lawmakers may be inclined to make it easier for biotech generics to be sold.

``There is a lot of pressure in Congress to provide a pathway for eventually these kinds of products to be approved,' said Alan Mendelson, a Menlo Park lawyer with Latham & Watkins, who counsels many biotech companies.

``Hopefully Congress hears the call,'' said Kathleen Jaeger, chief executive officer of the Generic Pharmaceutical Association. ``There is so much potential savings to be garnered by having generics in the marketplace.''

Contact Steve Johnson at sjohnson@mercurynews.com or (408) 920-5043.

Neurosurgeon's book From http://www.cleveland.com/entertainment/
plaindealer/index.ssf?/base/entertainment/
1148113834243910.xml&coll=2

Doc expertly dissects the business of brains
Sunday, May 21, 2006
John A. Vaughn

Most memoirists face the challenge of showing why their story is worth reading; after all, who doesn't have crazy families, inspiring teachers or midlife crises? Authors of medical memoirs, however, face the converse. Since their experiences are inherently dramatic and exotic to most readers, plot can overwhelm content. Such books can devolve into a shooting script for "ER."

Thankfully, Katrina Firlik's new memoir, "Another Day in the Frontal Lobe," walks that fine line between navel-gazing and melodrama to provide a thoroughly entertaining and candid look at the rarefied world of modern neurosurgery.

"The brain is soft," she begins. "Some of my colleagues compare it to toothpaste, but that's not quite right. It doesn't spread like toothpaste."

Firlik, 35, who grew up in Shaker Heights, declares tofu closest to the texture of a healthy brain. She then swings into a brisk consideration of her early influences, including visits to Harvey Cushing's grave in Lake View Cemetery and the stories of her surgeon father.

The daughter updates those stories with her own, capturing the humorous and casually dismissive culture of modern medical training; after finishing the book, you could hold your own on morning rounds. You'd know that if the chief resident tells you he's in the Ortho library, he's really working out in the hospital gym; you'd laugh as hysterically as the rest of the team at the "stat" dermatology page placed by your jealous intern, and you'd realize that any physician wearing a bow tie is almost surely a neurologist.

Firlik includes the set pieces of the genre - the bizarre cases, the life-and-death O.R. scenes, the hushed withdrawal of life support - but what makes them so powerful is that rather than focusing on the spectacle, she shows you that they are often just routine, inevitable results of decisions made long before she was involved. Yes, she is responsible for that teenager surviving his car accident because she drilled a hole in his skull to decompress the blood clot squeezing his brain, but no more so than the passer-by who called 9-1-1 or the kid himself had he just worn his seat belt.

The only readers who might be disappointed in "Frontal Lobe" are those expecting an exploration of what it's like to be a woman in the alpha-male realm of surgery. Firlik was the first woman admitted to the neurosurgery residency at the University of Pittsburgh Medical Center, but other than saying that she chooses not to be easily offended by "minor things that could be construed as sexist," she doesn't delve into the challenges unique to female physicians.

I'd love to be in the surgeons' lounge when Firlik's colleagues discuss her book - demythologizing the profession is not a favorite procedure. But this thoughtful and forthright memoir is actually more comforting for its lack of white-coated superheroes.

It's nice to know that ordinary people can do such extraordinary things.

Vaughn is a physician and critic in Columbus.

To reach John A. Vaughn:

books@plaind.com

Pheos From http://www.urotoday.com/264/
conference_reports/selected_abstracts/adrenals.html

April 30, 2004



LAPAROSCOPIC ADRENAL SURGERY FOR RECURRENT, HEREDITARY PHAEOCHROMOCYTOMA

Nambirajan T., Jeschke S., Leeb K., Janetschek G.

Elisabethinen Hospital, Department of Urology, Linz, Austria

INTRODUCTION & OBJECTIVES: Patients with hereditary form of phaeochromocytoma develop multiple tumours at young age and are prone for recurrences. Laparoscopic partial adrenalectomy removes the tumour, preserving the adrenal cortical function and avoiding hormonal replacement therapy. It is feasible to perform second adrenal sparing surgery laparoscopically for recurrences, which is illustrated with these 5 cases.

MATERIAL & METHODS:

RESULTS: Laparoscopic partial adrenalectomy was feasible in all but one patient despite previous surgery. There was no need for conversion to open surgery. There were no intra or postoperative complications. Histology confirmed phaeochromocytoma in all patients and there were no malignancies. At follow-up, blood pressure and urine catecholamines were within normal levels in all patients except one (no 5), who has asymptomatic elevation with a recurrent tumour on the contra lateral side. He is under surveillance. The patient 3 was pregnant at 20 weeks of gestation and she completed pregnancy with a full term baby. None of the patients required steroid replacement.

CONCLUSIONS: Laparoscopic partial adrenalectomy is feasible after previous explorations and is technically easier if the previous approach had been laparoscopic as well. Laparoscopy is the ideal approach in patients prone for recurrent tumours.

TRANSPERITONEAL LAPAROSCOPIC ADRENALECTOMY: LESSON LEARNED FROM MORE THAN 200 CONSECUTIVE CASES

Cestari A., Guazzoni G., Centemero A., Riva M., Zanoni M., Naspro R., Bellinzoni P., Rigatti P.

Vita-Salute, San Raffaele Hospital - Ville Turro, Urology, Milan, Italy

INTRODUCTION & OBJECTIVES: Laparoscopic adrenalectomy is considered the treatment of choice for the surgical ablation of most benign adrenal lesions. Several approaches and several surgical techniques have been described. We present our 12-year experience in laparoscopic transperitoneal adrenal surgery.

MATERIAL & METHODS: Between October 1992 and September 2003, 231 laparoscopic approaches to the adrenal gland have been performed, namely 185 unilateral adrenalectomy (79 right, 106 left, 65 Conn's disease, 42 Cushing's disease, 37 Phaeochromocytoma, 32 non functioning adenomas and 9 malignancy), 19 bilateral adrenalectomy and 8 cases of conservative surgery. The patients were placed in a 60- degree flank position with the bed flexed to increase the surgical field; the first step of the intervention was the early ligation of the adrenal vein, as a land mark to correctly dissect the adrenal gland.

RESULTS: The laparoscopic procedure was successfully completed in all but 5 cases which were converted into open surgery (1 for duodenal injury during pneumoperitoneum induction with open access and 2 during procedures for malignancy). Mean operative time was 152 min. in the unilateral group, 235 min in the bilateral group and 84 min in the conservative group. Delayed complications included 3 cases of hem peritoneum which were drained surgically, 3 cases of severe blood loss which were treated with blood transfusions, 2 cases of wound infection. Patients were able to ambulate on the morning of the first postoperative day and were discharged respectively 2.7, 5 and 1.5 days after surgery in the unilateral, bilateral and conservative group.

CONCLUSIONS: Laparoscopic transperitoneal adrenalectomy is a safe and effective, minimally invasive approach for patients with benign functioning or non functioning adrenal masses. This technique has low morbidity, minimal postoperative analgesic requirements, short hospital stay.

LAPAROSCOPIC ADRENALECTOMY FOR LARGE TUMOURS (5-10CM)

Droupy S.1, Hammoudi Y.1, Eschwège P.1, Young J.2, Giuliano F.2, Benoit G.1

1CHU de Bicêtre, Department of Urology, Le Kremlin Bicêtre, France, 2CHU de Bicêtre, Department of Endocrinology, Le Kremlin Bicêtre, France

INTRODUCTION & OBJECTIVES: Laparoscopic adrenalectomy has become the standard technique for the surgical removal of the adrenal gland. The limits of laparoscopy have not been defined and evolve with surgeons experience. We reviewed our experience with laparoscopic treatment of adrenal tumours from 5 to 10 cm diameter in order to determine feasibility and carcinological safety.

MATERIAL & METHODS: From 1997 to 2002, 56 laparoscopic adrenalectomy were performed by one surgeon. 6 bilateral, 34 for adrenal tumour less than 5 cm and 10 for tumours from 5 to 10 cm. The first 21 were performed using the retroperitoneal laparoscopic approach and the 35 last using a transperitoneal laparoscopic approach. Causes of discover were ultrasonography for abdominal pain (4), Cushing disease (2), a CT scan for breast cancer (2), flank pains (1) and hypertension (1). Patients were 7 women and 3 men, from 28 to 82 years of age.

RESULTS: All the tumours were removed by laparoscopy: the first one using the retroperitoneal approach and 9 using the transperitoneal approach as handling of the tumour was difficult in the confined retroperitoneal space.

Mean operative time was 155 min. (90-215). No preoperative complication occurred. Postoperative complications were one pleural effusion and one haematoma on a trocar site needing two days more of hospital stay. Mean hospital stay was 4.1 days (3-6). Pathological examination showed 3 adrenocortical carcinomas, 2 adenomas (Weiss 1), 2 cavernous lymphangiomas, one leiomyosarcoma, one myolipoma and one adenomatoide tumour. Mean follow-up was 29 months (7 to 60). One patient with a metastatic adrenocortical carcinoma at the time of surgery progressed under OP’DDD. Two patients with adrenocortical carcinomas are free of disease after 60 and 48 months.

CONCLUSIONS: Laparoscopic removal of adrenal tumours more than 5 cm is feasible and carcinologically safe. Tumours are carcinomas or rare tumours for which radical adrenalectomy is required. The transperitoneal laparoscopic approach seems to be more appropriate for large tumours.

ADRENAL METASTASES OF UNKNOWN PRIMARY MALIGNANCY - WHAT IS THE DIAGNOSTIC WAY?

Wunderlich H.1, Reichelt O.1, Hindermann W.2, Kosmehl H.3, Schubert J.1

1Friedrich-Schiller-University, Department of Urology, Jena, Germany, 2Friedrich- Schiller-University, Department of Pathology, Jena, Germany, 3Helios-Clinic, Department of Pathology, Erfurt, Germany

INTRODUCTION & OBJECTIVES: The approach to incidental adrenal masses depends on whether the patient is known to have a primary malignancy. Adrenal glands are frequently involved in metastatic disease. The extent of diagnostic imaging in metastatic findings has been reconsidered, in view of the detection of the primary tumour.

MATERIAL & METHODS: In a series of 14.793 autopsies from 1985 till 1995 323 adrenal metastases were revealed. More than half (68,2%) of all adrenal tumours were metastases, of which more than 40% were bilateral.

RESULTS: Most of the metastatic tumours were carcinoma (306/323 metastases), sarcoma (8/323 metastases), malignant melanoma (5/323 metastases) and malignant lymphomas (4/323 metastases). Metastatic disease from lung carcinoma was seen in 195 out of 323 adrenal metastases. Adenocarcinoma and small cell carcinoma were the most common subtype. The second and third most common sites were breast cancer and colorectal cancer in female patients and renal cell carcinoma and colorectal as well as pancreas carcinoma in male patients. Urological carcinomas have shown a low frequency of adrenal metastases, except of renal cell carcinoma. Adrenal metastases in prostate cancer were seen in 5 out of 229 cases, in transitional cell carcinoma of the urinary bladder in 3 out of 125 patients and in transitional cell carcinoma of the renal pelvic in 2 out of 17 cases. In malignant tumours of the testes (n=36) and the penis (n=4) no adrenal metastases were found.

CONCLUSIONS: As soon as adrenal metastasis is suspected without knowing the primary tumour diagnostic work-up needs to be focused on kidney, liver, spleen, GIsystem, breast and especially on lung. Adrenal exploration has to be considered as long as no primary tumour is diagnosed.

Pituitary Adenoma From http://www.news-medical.net/?id=18220
Pituitary adenoma predisposition caused by germline mutations in the AIP gene

Medical Studies/Trials Published: Thursday, 1-Jun-2006

A recent Finnish study identifies a low-penetrance gene defect which predisposes carriers to intracranial tumors called pituitary adenomas. In particular individuals carrying the gene defect are susceptible to such tumors which secrete growth hormone.

Excess of growth hormone results in conditions called acromegaly and gigantism. Identification of this gene defect using DNA-chip technologies is an example how genetic research can tackle more and more demanding tasks, such as identification of predisposition genes conferring a low absolute but high relative risk. The results are published in the journal Science.

The research group, lead by professor Lauri Aaltonen (University of Helsinki, Finland) and Dr Outi Vierimaa (Oulu University Hospital, Finland) providing the initial observations leading to the investigations, aimed at unravelling the genetic basis of susceptibility to pituitary adenomas. Pituitary adenomas are common benign neoplasms, accounting for approximately 15 % of intracranial tumors.

Most common hormone-secreting pituitary tumor types oversecrete prolactin or growth hormone (GH), which together with local compressive effects account for their substantial morbidity. Oversecretion of GH causes acromegaly or gigantism. Acromegaly is characterized by coarse facial features, protruding jaw, and enlarged extremities. The potentially severe symptoms of untreated acromegaly, develop slowly and the condition is difficult to diagnose early. Gigantism refers to excessive linear growth occurring due to GH oversecretion when epiphyseal growth plates are still open, in childhood and adolescence. Genetic predisposition to pituitary tumors has been believed to be rare.

The researchers detected three clusters of familial pituitary adenoma in Northern Finland. Genealogy data reaching back to 1700's was available. Two first clusters could be linked by genealogy. The researchers hypothesized that a previously uncharacterized form of low-penetrance pituitary adenoma predisposition (PAP) would contribute to the disease burden in the region. The researchers had previously characterized a population based cohort diagnosed with GH secreting pituitary adenoma (somatotropinoma) in Oulu University Hospital (OUH). These data were linked to the pedigree information, to identify additional affected distant relatives. The PAP phenotype - very low penetrance susceptibility to somatotropinoma and prolactinoma - did not fit well to any of the known familial pituitary adenoma syndromes. These syndromes are defined by familial occurrence of the disease, and the low penetrance of PAP appeared unique. Low penetrance means hereditary predisposition which relatively rarely leads to actual disease - but which may cause much more effect on population level than high-penetrance disease susceptibility which typically is very uncommon.

Utilizing modern chip-based technologies the research group identified mutations in the AIP gene as the underlying cause. Further work on the functional role of this gene should prove informative in revealing key cellular processes involved in genesis of pituitary adenomas, including potential drug targets.

It has not been previously realized that genetic predisposition to pituitary adenoma, in particular GH oversecreting type, can account for a significant proportion of cases. The study not only reveals this aspect of the disease, but also provides molecular tools for efficient identification of predisposed individuals. Without pre-existing risk awareness, the patients are typically diagnosed after years of delay, leading to significant morbidity. Simple tools for efficient clinical follow-up of predisposed individuals are available, such as monitoring GH in blood samples.

In a general sense, the results suggest that inherited tumor susceptibility may be more common than previously thought. The identification of the PAP gene indicates that with the new DNA-chip based technologies it is possible to identify the causative genetic defects in the low-penetrance conditions even in the absence of a strong family history.

http://www.helsinki.fi

Pituitary Tumor From: http://www.tucsoncitizen.com/ss/body/15649

How I Did It
Health 101: Teacher shares fitness lessons
SANDRA VALDEZ GERDES
Published: 06.19.2006

If she could do it, anyone can.

So says Donita Montgomery, 29, a local teacher who lost 87 pounds and feels unstoppable - especially when she's kickboxing.

At 5 feet 7 inches, she now weighs 145. It wasn't that long ago, however, that she was 202 pounds and wearing a size 22.

Montgomery, now a size 9, recalls that her most recent weight gain came in 2002. Her sedentary lifestyle and job as a fifth-grade teacher made it easy to pile on the pounds because she spent most of the day sitting.
"You pretty much just stay in one place and teach. There's not a lot of movement," she said. In addition, irresistible yet fattening treats were always available at staff meetings and training sessions. Two years ago, her life changed. Her sister joined Naturally Women on Tucson's East Side (now Darla's Fitness for Women) and nagged her to join. In January 2004, she did, and has gone six days a week ever since. "It was sort of a New Year's Resolution. I didn't like the way I looked anymore, and my daughter at that time was a year and a half, and I couldn't play with her," Montgomery said. With determination, a caring health club staff and a personal trainer, she lost the weight at a rate of 1 to 2 pounds a week. "I think the fact that I took it slow helped," Montgomery said. She didn't have unrealistic expectations. She wasn't looking for a quick fix. She wanted a lifestyle change. She cut her diet to 1,200 calories per day, but allowed herself one "bad day" each week, on Saturdays, to indulge in chocolate or junk food. As time went on, the treat days became less important. Montgomery won't lie. "It was definitely hard to stick to it," she said. At first, she could only do 15 minutes on the treadmill. "I just didn't have enough energy to go longer." But each week she did a little more. She plugged along, and kept a food journal to hold herself accountable. Today, she sticks to the same 1,200-calorie diet and exercises daily. "I go straight from picking up my daughter (at day care) to the gym." Her biggest challenge then was finding time to exercise. "I couldn't go before work because they didn't have the day care open, and after work it was hard because I'd get home after 7 p.m., but I needed to do it. I needed to make a difference for myself. I need to put myself first." Another challenge for Montgomery was exercising despite a brain tumor: pituitary adenoma. The tumor caused her body to simulate pregnancy and menopause at the same time, she said. "So the harder I worked out, the more my body thought I was starving a baby and it would store fat," she said. The tumor was removed in 1997 and two more grew, so she's on medication for those, she said. "It's harder to work out," she said, but worth the payoff. Exercising "gave me more energy for everything" and "I was a much nicer person." It relieved her job stress so she could arrive home to her family happier and more energetic. "I don't think it's selfish to put yourself first, because if not, you start to resent everybody. To be happy you need to be first sometimes." Her best moments are when former students fail to recognize her. "One kid said, 'You don't even look like the same person,' " Montgomery said. Then with a hint of pride in her voice, she added, "And I don't."

MONTGOMERY'S SAMPLE WORKOUT Sunday, Monday, Friday: Cardio for 70 minutes: 35 minutes on the elliptical followed by 35 minutes (interval training) on the treadmill. Wednesday: 35 minutes on the treadmill, then a one-hour kickboxing or spinning class. Tuesday, Thursday: Weightlifting for 30 minutes. One week, she does upper body on Tuesday with a trainer and lower body on Thursday by herself. The next week, she alternates.
 
MONTGOMERY'S SAMPLE DIET Breakfast (7:30 a.m.) -Two slices of buttered toast, and water. Morning snack (10:30) - Wheat thins, one serving or about 14 crackers, and string cheese. Water. Lunch (12:30 p.m.) - Turkey sandwich on regular white bread with mustard, and Yoplait yogurt. Water. Afternoon snack (3) An apple and another string cheese. Water. Dinner (7) - Chicken patties, chicken breast or tacos. She eats what she wants as long as it's within calorie range. Her meat serving is about the size of a fist.
 
MONTGOMERY'S SUCCESS TIPS

1. Don't be discouraged. Everyone has to start somewhere. Join a health club that offers personalized service. Make sure it's a place where they care about you and your success, and know their members by name.


2. Reduce your calories. Consult your physician to learn how many calories you need each day and stick to it. Montgomery started on a 1,200-calorie-a-day diet. She kept track, but didn't fret about fat or carbs as long as she stayed in her range. If she didn't know the calorie content of a food, she skipped it. She allowed herself one "bad day" each week for junk food or dining out. She took healthful snacks with her to staff meetings. She ate several times a day to avoid getting too hungry and binge eating later.


3. Take it slow. Don't push yourself too hard or expect fast results. Success will come if you commit to a program and take it slow.


4. Drink a lot of water. Montgomery replaced soda and other beverages with water at each meal. She now drinks 200 ounces of water per day - a 1.5 liter (50 oz.) bottle four times a day. Water will help flush out toxins and salt, keep you hydrated and help you to feel full between meals.


5. Don't give up (on yourself). If you fall off the horse, get back in the saddle. If you mess up, start again. Eventually there will be bigger gaps between the slip-ups. For teachers: Keep your routine during summer vacation and work out earlier in the day, if possible.
Pituitary Tumor Removal From http://www.nbc10.com/health/9263541/detail.html

NBC10.com
Doctors Perform Brain Surgery Through Nose

Pituitary Tumor Removed Without Opening Skull

POSTED: 6:47 pm EDT May 23, 2006

Physicians at Thomas Jefferson University Hospital performed brain surgery Tuesday using some of the newest technology around.

The doctors removed a tumor from a patient without making an external incision in his head or face. They removed a pituitary tumor through the patient's nose.

Instead of the traditional, more invasive surgery, which requires opening up the brain and skull, Dr. Marc Rosen and Dr. James Evans performed minimally invasive surgery.

The doctors used an endoscope to visualize and then remove a pituitary microadenoma. With the new low-risk procedure, surgeons can operate on benign and cancerous tumors in patients who previously might not have been considered candidates for surgery.

Patients can also begin radiation and chemotherapy sooner than if they had open surgery.

Usually, these pituitary tumors have no symptoms, but when they do, people can have headaches, decreased energy, lowered sex drive or weight gain -- symptoms sometimes thought to be linked to aging.

The operation was broadcast live on the Internet. If you would like to see a video of the operation, Click here.

Copyright 2006 by NBC10.com. All rights reserved.

Pituitary Tumors From http://www.allheadlinenews.com/articles/7003760576

Risperdal May Cause 70 Percent Of Pituitary Tumors.

May 31, 2006 3:00 a.m. EST

Yvonne Lee - All Headline News Staff Reporter

Washington, D.C. (AHN) - A new study suggests Risperdal may be linked to 70 percent of pituitary tumors.

The Food and Drug Administration worked in conjunction with Duke University on the study, which found the antipsychotic and similar medications may cause pituitary tumors.

The researchers caution the study's findings suggest, rather than prove, a link.

Healthday News reports Risperdal is the most commonly-prescribed medication among atypical antipsychotics. These are used to treat schizophrenia, paranoia, and manic-depressive disorders.

Study co-author Dr. P. Murali Doraiswamy, a psychiatrist at Duke University Medical Center, says, "Atypical antipsychotics are lifesaving medications for a lot of people. By no means are we advocating that people stop using them, especially risperidone."

The findings appear in the June 2 issue of Pharmacotherapy.

Post-op brain tumor From http://www.gazette.net/stories/
061406/bethnew194239_31965.shtml

Memorizing lines to remember her life
An actress deals with memory loss after surgery to remove a brain tumor
Wednesday, June 14, 2006

by Olivia Doherty

Staff Writer



Click here to enlarge this photo

David S. Spence⁄The Gazette

Thirteen years ago, Caris Corfman, 51, lost her ability to make new memories when she had a brain tumor removed. Yet the actress, who had built a career over 15 years with roles on Broadway, off-Broadway and in local theater, has continued to perform in a role more personal than ever – a story of her life.

~~~~~
For a woman who cannot remember anything new, Caris Corfman has an impeccable memory.

She remembers growing up in Bethesda, attending Walter Johnson High School and wanting to be a dancer and an actor.

She remembers her first Broadway experience — "a dream" — performing alongside Jane Seymour and Ian McKellen straight out of graduate school.

She remembers the pain of unbearable headaches that later turned out to be a brain tumor.

Corfman easily recalls the productions she has acted in, the names of fellow actors and even the lines from monologues she has performed. Yet she cannot remember meeting new people five minutes after introducing herself and she cannot remember the beginning of a conversation when the topic changes.

Thirteen years ago, Corfman, 51, lost her short-term memory as a result of having her tumor removed. The actress who had played roles in films, on television, on Broadway, off Broadway and in local theater could no longer memorize her lines.

"I didn’t know what to do with my life," Corfman said, startlingly aware of her disability. "I couldn’t remember anything so my career was gone."

Still, she felt the itch of the stage.

With help from childhood friend Brad Watkins, who is now producing director at the Olney Theatre Center, Corfman has continued to perform in a play about her life. Last weekend, she presented her show in Philadelphia at the First Person Festival.

When Corfman began to emerge from the cloudiness of her operations, Watkins said, she bugged "everyone who would listen" about getting back on a stage.

"Because she lacks short-term memory and because she had been so altered by this trauma her body had gone through, we all sort of placated and pooh-poohed her for some time and would change the subject rather quickly," he said.

Eventually, Watkins encouraged her to write, telling Corfman,