Tuesday, October 27, 2009

(adrenal Cushing's) Patients say thanks to hospital stars

26 October 2009 07:00

The Big C cancer centre and a nurse described as a “guardian angel” have been picked as the champions who go the extra mile from hundreds of staff put forward by patients.

Individuals and departments at the Norfolk and Norwich University Hospital were nominated for the hospital's patient choice awards. And the Big C information and support centre was chosen as the most outstanding department, winning the gold award, while endocrine specialist nurse Sondra Gorick won the gold individual award.

The Big C centre, a collaboration between the hospital and the Big C charity, which was funded with help from Evening News readers, was described by patients as “warm, open and welcoming” and “an oasis of calm in the midst of all the stress”, and staff were praised for being friendly and kind. It was nominated by many people, including Stephanie Brooks, 57, a grandmother-of-four from Hevingham, near Aylsham. The former welfare assistant at Hevingham Primary School was diagnosed with breast cancer last year after a routine mammogram. She said: “I walked through the door scared stiff, but they put you at ease. The care and compassion those people show is just amazing. They arranged for massage for me, and a make-up class with other women who were in my position, and we were laughing and joking together.”

The silver award went to the Davison Day Unit at Cromer Hospital, while Hethel ward won the bronze award. One of the patients who nominated it praised the staff for their care of his dying mother, and wrote: “They went beyond being professional and showed they cared. My mum's last days were spent with wonderful people.”

Mrs Gorick was nominated by nine patients, including Michael and Patricia Stamp, from Drayton, and Judith Snowling, 49, from Cawston, near Aylsham. Mrs Snowling was diagnosed with Cushing's Syndrome, a benign tumour on her adrenal gland, in 2005, and felt Mrs Gorick was “my rock”. The mother-of-two and grandmother-of-two thought she would never recover and would be on steroids for the rest of her life. She said: “There were times I felt so lonely at home but a phone call to Sondra, which sometimes only took seconds, gave me a reason to lift my chin up and carry on. She was just like a guardian angel. She still is really, for other people as well.”

With the help of an operation she is now “fighting fit” and was able to quit her job at Prospect Medical Practice on Aylsham Road, Norwich, to set up her own business, a private meals-on-wheels company.

She said: “Sondra has been amazing. The whole hospital was fantastic. When I first met her she could see that inside I was going to pieces. She held my hand and said: 'You look as though you want to cry.' I said I would do that when I got home, and she said 'Please, I would rather you do it now.' I shall never forget those few words which gave me all the confidence I needed to get through my battle I had ahead.”

NNUH patient choice awards.

NNUH patient choice awards.

Mrs Gorick, who lives in Mulbarton, was has worked at the hospital since 1980, when she started as an auxiliary nurse. She said: “I feel very overwhelmed. I am really pleased and proud and very grateful to all the people who nominated me. But it is not all about me. We are a team. In the background there are so many people working so hard doing things for them, like tests in the lab.

“At the end of the day we are here for the patients. To me it is such an important thing and this job is such a big part of my life.
“If it was me I would want someone to give me a hug, and that is what I do for other people.”

The awards were given out at a ceremony in the Forum on Friday evening. The quality of nominations was so high that the silver award was awarded to two members of staff - consultant plastic and reconstructive surgeon Richard Haywood, and consultant cardiologist Cairistine Grahame-Clarke.
Mr Haywood was nominated by three patients, including Jacquie Lloyd, from Overstrand, near Cromer, and Brenda Egmore, from Mattishall, near Dereham. Mrs Egmore, an accounts assistant and mother-of-four, had her breast rebuilt by Mr Haywood in April following a mastectomy for breast cancer in 2007. She said: “Mr Haywood has given me not only a new breast but the confidence in life again.” She said he makes everyone feel they are important, and took the time to reassure her husband Colin, too. She said: “He just seemed such a genuine person, like he really cared.”

She had her breast rebuilt using fat from her stomach, and said: “I am really pleased with it. Having a reconstruction, the difference it has made, there are no words to describe it.”

She also praised all the nurses in the Weybourne Day Unit, and said: “From being diagnosed with breast cancer right through to having reconstruction I have always felt that somebody cared.”

Dr Grahame-Clarke was nominated by numerous patients and praised for being “such a caring person and a first-class doctor”. One of the most moving nominations was from Hugh Shearer, from Honingham, near Norwich, who died in June aged 83. The former head of Alderman Leach School in Gorleston wrote: “One walks into her consultancy room on a stick and comes out walking on air.”

His family were keen for his nomination to stand, because it was his heartfelt wish for Dr Grahame-Clarke to win an award.

The bronze award went to Mary Gotts, a nurse in the critical care complex. John Thurman, 69, from Eaton, a retired careers advisor at the University of East Anglia, nominated her after spending several days in the hospital's high dependency unit last autumn. He was recovering from three operations for cancer of the jawbone, during which his jaw had to be rebuilt with bone from his leg and his arm. He said: “Mary was outstanding, she was always efficient and well-organised. She really went the extra mile and did good old-fashioned nursing. This makes enormous difference to the well-being of patients.”

See www.eveningnews24.co.uk for a slideshow of pictures of winners and images from the awards ceremony.

Has your life been improved by someone who has gone the extra mile? Contact us on 01603 772485 or sarah.brealey@archant.co.uk.

From http://www.eveningnews24.co.uk/content/news/story.aspx?brand=ENOnline&category=News&tBrand=ENOnline&tCategory=news&itemid=NOED23%20Oct%202009%2022%3A33%3A15%3A677

Wednesday, October 21, 2009

Medical treatment of Cushing's disease: Overview and recent findings

Authors: Stephanie Smooke Praw, Anthony P Heaney

Published Date October 2009
Journal: International Journal of General Medicine

Stephanie Smooke Praw1, Anthony P Heaney1,2

1Department of Medicine, 2Department of Neurosurgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA

Abstract: Cushing's disease, due to pituitary adrenocorticotropic hormone (ACTH) hypersecretion, is the most common etiology of spontaneous excess cortisol production. The majority of pituitary tumors causing Cushing's disease measure <1 cm and the excess morbidity associated with these tumors is mostly due to the effects of elevated, nonsuppressible, ACTH levels leading to adrenal steroid hypersecretion. Elevated circulating cortisol levels lead to abnormal fat deposition, hypertension, diabetes, coronary artery disease, osteoporosis, muscle weakness and psychological disturbances. At experienced centers, initial surgical remission rate via transnasal, transphenoidal resection approaches 80% for tumors less than 1 cm, but may be as low as 30% for larger lesions and long-term recurrence in all groups approaches 25%. Residual disease may be managed with more radical surgery, pituitary-directed radiation, bilateral adrenalectomy, or medical therapy. This paper addresses current and novel therapies in various stages of development for Cushing’s disease.

Keywords: Cushing's disease, treatment, pasireotide, PPAR-γ, 11 β-hydroxysteroid dehydrogenase inhibitors, dopamine agonists

Download article (Free!):  http://dovepress.com/getfile.php?fileID=5388

Tuesday, October 20, 2009

8 new Cushing's bios added. dx include 1 pituitary, 1 diagnosed not sure of type, 6 undiagnosed at http://ow.ly/vt0D or http://ow.ly/vt1n

Misconceptions About Cushing's

Cushing's.  So many people are confused by what it is and what it isn't.  They may have heard of it because a dog they know has it - or, these days, a horse, ferret, rat.  Seems it's way more common in lots of animals but not people.

If people have heard of the "animal version" they might say "Yeah, my dog had that and it was easy to diagnose. We just gave him medication..."

When we first started having bios on the website, sometimes people would say that they had Cushions Disease.  At first I wondered about that but then it started to make more sense.  If you've never heard of the disease,  the doctor mumbles something.  You know you're a little "fluffy" and cushions makes a lot of sense.

Twice in the last week I've seen Cushing's described as Crushings Disease.  That sort of makes sense, too.  Cushing's crushes your plans, relationships, credibility, pretty much everything.

Other misconceptions involve Cushing's symptoms.  Others, especially doctors, will see you gain weight and assume you're eating too much and a good diet will fix everything.  Or see that you're depressed (who wouldn't be!) and offer anti-depressants.

Doctors may say that Cushing's is too rare, that they'll never see a case of it in their practice.  But rare doesn't mean that no one gets it.  Rare doesn't mean that doctors shouldn't test for it.

Then, the anatomy just isn't right.  People say that they have a brain tumor instead of a pituitary tumor.  I just read this on another site: The pituitary gland is on the bottom of the brain...  Umm - not exactly ON the bottom of the brain but maybe close enough for people to get an idea. 

What sorts of things about Cushing's/Cushions/Crushings that just weren't quite right?

Monday, October 19, 2009

Next Cushing's Help Interview with Laura, October 29, 2009

October 29 at 9:00PM eastern, Interview with Laura (LauraNG)

This interview will take place at 9:00 PM eastern - please note later time!

Laura has been struggling since 2007 with various odd things happening to her body.

In March of 2009 she was diagnosed with Hashimoto's Disease and Hypothyroid.

Her bio contains a long list of symptoms she has and she has been misdiagnosed with Obesity, Diabetes, Kidney stones, Depression, MS, Manic/Depressive, TIA, Anxiety - stress, Not exercising enough, Not eating properly, Abusive relationship (due to bruising).

In responding to the email confirming this interview Laura wrote:

"When I started reading the bio's of people like myself, I started to cry tears of relief that what I may have, is actually ONE real thing. I cannot tell you how happy I was to find your website and I think it is wonderful that so many people who truly understand how frustrating it is to think we are alone, are willing to put themselves out there as beacons of hope for the rest of us. I felt so strongly, that I wanted to add my bio and offer myself for interview. If I can help one person feel a little calmer while they go through this process, I am more than willing. I don't know if I have Cushing's but if I do, it is important to me to reach out to others because the attacks coming from the physical and mental side of this illness, deserve to be exposed and recognized. Even though I am feeling a bit humiliated, reclusive and embarassed because of what it is doing to me."

Read Laura's entire bio at http://www.cushie.info/index.php?option=com_content&view=article&id=413:laura-undiagnosed-bio&catid=32:bios&Itemid=68

You can listen live at http://www.blogtalkradio.com/CushingsHelp The archives are usually up about 30 minutes after the end of the show and in our podcast area of iTunes by Friday.

The Call-In number for questions or comments is (646) 200-0162.

Cushing's locations page updated, 9 new people added from England, USA. http://ow.ly/vgop

Friday, October 16, 2009

Münchausen By Media

The Internet makes it so easy to develop weird and unusual diseases.  Just plop a symptom into Google and suddenly you find yourself with stomach cancer, Cushing's or other dread diseases.

Even on TV, the ads for lawyers almost convince people they might have mesothelioma and other rare illnesses that might bring you - and them! - bundles of money if you just sue someone.

Magazine ads implore you to "ask your doctor about..." this drug or that you might or might not need.  Your doctor might just give it to you to keep you from asking.  And there's a needless medication that brings profit to the drug company and side effects to you.

TV shows like House and Mystery Diagnosis will show you diseases you never dreamed about.

There's a great topic on the Power Surge message boards, What's the worst "disease or ailment" you've had, where the women discuss the diseases they thought that they had, based on symptoms, what they've seen online, in the news but not based on reality.

I've done it myself.  About the only time I was right was with my Cushing's diagnosis.  That one was a good call. But my thoughts of kidney cancer metastasis haven't come true (yet, anyway!).

There's been information online lately about Münchausen Syndrome.  Wikipedia says:

"...the affected person exaggerates or creates symptoms of illnesses in themselves or their child/children in order to gain investigation, treatment, attention, sympathy, and comfort from medical personnel. In some extremes, people suffering from Münchausen's Syndrome are highly knowledgeable about the practice of medicine, and are able to produce symptoms that result in multiple unnecessary operations. For example, they may inject a vein with infected material, causing widespread infection of unknown origin, and as a result cause lengthy and costly medical analysis and prolonged hospital stay. The role of "patient" is a familiar and comforting one, and it fills a psychological need in people with Münchausen's. It is distinct from hypochondriasis in that patients with Münchausen syndrome are aware that they are exaggerating, whereas sufferers of hypochondriasis believe they actually have a disease."

I think we've all see this, especially online.  It's so easy to sit in the comfort of ones home and add "just a little" to the symptoms, making it more impressive for the readers.

From A Strange Case of Münchausen By Internet:

"...When I first got online, I "met" a young woman who claimed to be a vet, and offered me all kinds of advice about my cat and my tropical fish. She got cancer, slowly declined, then died. We wanted to send flowers, and maybe attend the funeral, and got her ISP to contact her family for us. To our shock, her parents said there was no funeral. She wasn't dead, she wasn't even sick. At least not physically. She'd pulled this kind of "pretend death" several times before, and was in therapy, but every time life got stressful, she'd do it again.

And the Internet is the ideal place for a Munchausen sufferer. With the click of a button, you can find out all kinds of information, to help you pose as anyone you want. People don't expect to see you in person or even talk to you except by e-mail, making deception easier. And often, mailing lists, message boards, etc., will give unqualified support to their members..."

And Media Makes Me Sick:

"...The Internet is hands-down the worst thing to ever happen to the medical world. With Web sites like WebMD, any paranoid hypochondriac like me can jump online, look for symptoms and immediately convince himself he has cancer or Cushing's disease or non-Hodgkin's lymphoma or any other of a million things.

WebMD allows you to find one symptom and then "helps" you by listing 15,000 things it could mean.

Oh my God. I do have a slight ache! That's it. I must have a brain tumor. I'm not kidding, I recently scared myself into thinking I had cancer. It took a specialist, a CT scan and an ultra-sound to convince me otherwise..."

Karen found this older article at http://www.villagevoice.com/2001-06-26/news/cybersickness/1

"...Over nearly three years, from 1998 to 2000, a woman—let's call her Anna—posted to an online support group for people with mental illness. To the larger circle of readers, she acted mostly as friendly counselor. But to a select few, she e-mailed stories of escalating catastrophes. Her husband and two children had perished in a plane crash, she wrote. As a kid, her father had molested her, and she had suffered multiple personality disorder. Finally, she told her trusted—and trusting—confidants that she had just been diagnosed with leukemia.

Gwen Grabb, a psychotherapy intern and mother of three in Los Angeles, says the group believed Anna because she took on the role of helping others, revealing her own difficulties much later, and to an intimate audience. "She was very bright," recalls Grabb. "She was very supportive and kind. One day, she started telling me about `the crash,' what they found in the black box, how you could hear her daughter screaming. I had known her a year. I believed her."

But as the tales became more elaborate and grotesque, Grabb grew suspicious. Along with another group member—Pam Cohen, a bereavement counselor in the Mid-Atlantic region—she did some research and discovered Anna was making it up. It was a shock to all, but worse than that to Cohen. "It is like an emotional rape," she says. People may have been upset over the online life and fatal cancer of the fictional Kaycee, whose creator admitted last month she'd invented the high school character for expressive purposes. But that was geared to a general audience, however easily suckered. Pretenders like Anna hurt a much more vulnerable group—folks who may be seriously ill and are seeking help..."

So - use caution and remember that not everything you read will happen to you!

Thursday, October 15, 2009

Good, Concise Article on Kidney Cancer

MaryO'Note: It was so fast between ending up with bleeding/pain to the ER to diagnosis and surgery that I never had time to learn much about my cancer in pre-op.  This is a great synopsis for anyone who might be having symptoms of kidney cancer or in the testing phases.

Personally, I often worry about recurrences, especially when it's scan time.  A website that I'm on calls that "scanxiety" and I like that term.  The scans don't hurt but you're lying there, imagining what they're finding.

Because of my kidney cancer and scans for that, I was found to have lung nodules.  So, now those are watched through scans.  Because of watching the nodules, I was found to have an enlarged lymph node.  Hopefully that's the end of it.  The node will shrink, the nodules won't grow, the cancer won't come back.

But, I'll always wonder...


Are You Suffering From Kidney Cancer?

by Sunil on October 13, 2009


Kidney Anatomy 300x240 Are You Suffering From Kidney Cancer?

Anyone affected with cancer faces uncertainty and anxiety. He often worries about the illness with a dozen of questions about the situation he or she is experiencing. A person with kidney cancer is no different. A kidney cancer patient will often worry about a lot of things primarily about getting the proper treatment including getting all tumor removed. Additionally, he may worry about whether the cancer will come back or having to undergo an array of medical tests, scheduled treatments and even hospital stays. Medical bills are also prime concern and these can also worry patients with any serious illness. Furthermore, kidney cancer patients often worry about how to hold on with his job despite the illness.

Kidney cancer’s primary choice of treatment is surgery. However, each patient requires different forms of treatment or a combination thereof depending on his age and general health and the stage of cancer. Kidney cancer is resistant to radiation. This is the reason why radiation is only used when there is already lymph node or kidney area involvement. Additionally, radiation treatment is only done when the cancer has already spread to other areas and the cancer cells can no longer be completely removed. It has been observed that drug therapy has been inconsistently effective against kidney cancer. Aside from radical nephrectomy, or the surgical removal of the kidney, some procedures are also being done to kidney cancer patients. These include procedures like arterial embolization, radiation therapy, biological therapy and hormone therapy.

Post-operative care is necessary in order to help kidney cancer patients recover more easily from surgery. He also needs supportive treatment, which includes relief from symptoms and side effects caused by treatments including chemotherapy and radiation. Kidney cancer patients will also benefit from psychological reassurance. This will speed up recovery and even minimize complications.

Taking care of a loved one who is suffering from kidney cancer is not easy. If the patient has to undergo surgery, you need to encourage him to express his fears and anxieties. You can talk to him and assure him that his body will be able to adapt soon to the kidney loss. It will also be beneficial if the patient is aware of any possible side effects after radiation or chemotherapy. His doctor or nurse may be able to talk with him about these. Medical professionals most often teach the patient some post-operative procedures to make the recovery easier. These include diaphragmatic breathing, proper coughing and how to support the incision while coughing. After surgery, you can aid the patient by regularly reminding him to comply with the doctor’s prescribed outpatient treatment including taking all the necessary medications. The doctor almost always will prescribe an annual follow-up chest x-ray. This procedure will rule out any spread of cancer to the lungs. Additionally, a procedure called intravenous pyelography maybe prescribed every 6 to 12 months in order to check for any budding tumors.

Finally, the length of recovery time from treatment including surgery, radiation or chemotherapy varies from individual to individual. Patients who undergo surgery will often complain of post-operative pain and discomfort accompanied with weakness and exhaustion. Family support and understanding are important during a patient’s recovery period.

From http://www.healthcarendiet.com/2009/10/13/are-you-suffering-from-kidney-cancer/

Predicting Recurrence of Nonfunctioning Pituitary Adenomas

Endocrinology (T.-W.N., E.J.L.), Pituitary Tumor Clinic and Institute of Endocrinology (T.-W.N., S.H.K., E.J.L.), and Pathology, Yong-Dong Severance Hospital (H.J.J.), Pathology Ilsan Hospital (M.-K.L.), Pathology (T.S.K.), and Neurosurgery (S.H.K.) and Yonsei Brain Research Institute, Yonsei University College of Medicine, 120-752 Seoul, Korea

* To whom correspondence should be addressed. E-mail: SUNKIM@YUHS.AC or ejlee423@yuhs.ac.

Context: Nonfunctioning pituitary adenomas are commonly diagnosed as large tumors. Most are detected incidentally during imaging studies or as a result of neurological manifestations. Depending on severity, most patients with large tumors require surgery and adjunctive therapies because of the high rate of tumor recurrence. The ability to predict the recurrence of a tumor at the time of the initial surgery would be helpful in deciding whether adjunctive therapy is necessary and decreasing morbidity. We investigated the use of several cellular markers for predicting the recurrence of nonfunctioning pituitary adenomas.

Objective: A tissue array block was made using tissue from 35 cases of nonfunctioning pituitary adenomas (16 cases with early recurrence ?4 yr after surgery, 10 cases with late recurrence >4 yr after surgery, and nine cases without recurrence). Levels of tumor tissue cellular markers associated with cell proliferation or apoptosis were analyzed, and immunohistochemical study of cellular markers was conducted using sectioned slides from the tissue array block.

Results: High Ki-67 and TUNEL labeling indexes were associated with recurrent nonfunctioning pituitary adenomas. Tumors with a high level of expression of phospho-Akt, phospho-p44/42 MAPK, and PTTG1 were associated with early recurrence. However, high levels of expression of phospho-CREB and ZAC1 were inversely associated with recurrence.

Conclusions: Tumors with high levels of expression of phospho-Akt and phospho-p44/42 MAPK and low levels of expression of phospho-CREB and ZAC1 should be followed closely and may require adjunctive therapy to prevent tumor recurrence.

From http://jcem.endojournals.org/cgi/content/abstract/jc.2009-0471v1

Wednesday, October 14, 2009

Adrenal crisis in treated Addison’s disease: a predictable but under-managed event

K White, Clinical Advisory Panel, Addison's Disease Self-Help Group, Hertford, SG13 8AZ, United Kingdom
W Arlt, Division of Medical Sciences, University of Birmingham College of Medical and Dental Sciences, Birmingham, United Kingdom

Katherine White, Email: kgwhite@addisons.org.uk


Adrenal crisis is a life-threatening event that occurs regularly in Addison’s patients receiving standard replacement therapy. Patient reports suggest that it is an under-estimated and under-managed event.


To assess the frequency of adrenal crisis is diagnosed patients and to understand the factors contributing to the risks of adrenal crisis.


We conducted a postal survey of Addison’s patients in four countries (UK N=485, Canada (N=148), Australia (N= 1237) and New Zealand (N=85) in 2003, asking about patients’ experiences of adrenal crisis and their demographic characteristics. In 2006 a shorter, follow-up survey was conducted in the UK (N=261).


The frequency and causes of adrenal crisis were compared across both surveys. Demographic data from the 2003 survey was analysed to establish the main variables associated with an elevated risk of crisis.


Around 8% of diagnosed cases can be expected to need hospital treatment for adrenal crisis annually, with exposure to gastric infection the single most important factor predicting the likelihood of adrenal crisis. Concomitant diabetes and/or asthma increase the frequency of adrenal crises reported by patients.


The endocrinologist has a responsibility to ensure that Addison’s patients have adequate access to life-saving emergency injection materials and repeated, practical training sessions in how to use them, while the GP plays a vital role as in arranging prompt emergency admissions.

From http://www.eje.org/cgi/content/abstract/EJE-09-0559v1

Tuesday, October 13, 2009

Persistent increase of osteoprotegerin levels after cortisol normalization in patients with Cushing’s syndrome

Valentina Camozzi, Francesca Sanguin, Nora Albiger, Carla Scaroni, Franco Mantero, Martina Zaninotto, Annachiara Frigo, Michele Piccolo and Giovanni Luisetto

V Camozzi, Departement of Medical and Surgical Sciences, Padua University, Endocrinology Unit, Padova, Italy
F Sanguin, Departement of Medical and Surgical Sciences, Padua University, Endocrinology Unit, Padova, Italy
N Albiger, Padova, Italy
C Scaroni, Padova, Italy
F Mantero, Departement of Medical and Surgical Sciences, Padua University, Endocrinology Unit, Padova, Italy
M Zaninotto, Department of Laboratory Medicine,Padua University, Padova, Italy
A Frigo, Department of Environmental Medicine and Public Health, Padova, Italy
M Piccolo, Departement of Medical and Surgical Sciences, Padua University, Endocrinology Unit, Padova, Italy
G Luisetto, Departement of Medical and Surgical Sciences, Padua University, Endocrinology Unit, Padova, Italy

Valentina Camozzi, Email: camozzina@libero.it


Objective: Osteoprotegerin (OPG) has been identified as a decoy receptor that inhibits osteoclast differentiation and recently as a paracrine regulator of vascular calcification. OPG is suppressed by glucocorticoids (GCs). Design: aim of this study was to evaluate OPG and bone metabolism in Cushing’s syndrome (CS) before and after cure.

Methods: 29 patients with CS (26 women and 3 men, mean age: 40.7-2.8 ys) and 27 age-, sex- and gonadal status- matched controls were studied for bone mineral density (BMD), bone metabolism, OPG, and receptor activator of nuclear factor-kB ligand (RANKL). Eighteen patients were studied from 6 to18 months after surgery, with normalization of cortisol levels in 12 of them.

Results: BMD was significantly lower in CS than in controls (lumbar spine:0.6±0.02 and 1.01±0.02, p<0.0001; femoral neck: 0.73±0.22 and 0.81±0.2, p=0,02). OPG was significantly higher and osteocalcin (OC) was significantly lower in CS than in controls (4.5±0.4 and 3.2±0.3 pmol/L, p= 0.02; 16.2+/-9.3 ng/ml and 21.2+/8.6ng/ml, p=0.03 respectively). Ca, P and PTH were similar. A significant positive correlation was found between serum cross laps (CLs) and OC levels (r2=0.43, p<0.01). After cure we found no difference in OPG levels but a significant increase of OC levels (16,4 ± 11 to 37,2 ±15; p=0,03 ng/ml, p=0.03).

Conclusion: In CS we found increased OPG that remained unchanged after recovery. High OPG was also observed in microvascular damage and was associated to an increased cardiovascular mortality. Therefore, high levels of OPG could reflects the persistent damage of the GCs on cardiovascular system.

From http://www.eje.org/cgi/content/abstract/EJE-09-0800v1

Robin (staticnrg) is Spreading the Word!

Robin, author of Survive the Journey, is this week's host of Grand Rounds. Her theme is “Participatory Medicine”. You can read this week’s edition here.

When I chose the theme for this week's Grand Rounds, I chose it because it is near and dear to my heart. Before I knew what it was called, I was looking for a "participatory medicine" model. Long before I heard the term "ePatient", I was one.

Also, in conjunction with the Grand Rounds, she was interviewed by a Doctor from Medscape.  The finished article can be read here: Rare Disease Sufferers Connect With Internet

Patients with rare diseases often feel like outsiders. They suffer not only the effects of their illness but also from a lack of access to specialist care and patient education. Luckily, the Internet has helped patients with rare diseases learn more about their conditions and connect with others who have the same symptoms and/or diagnosis.

Robin S, who publishes the blog Survive the Journey, has faced a dilemma such as this with Cushing's disease...

Way to go, Robin!

Dr. Edward Laws, neurosurgeon. "Simply the Best"

Although this is an older article and Dr Laws has moved from UVa, he is still a great doctor!

From http://www.virginia.edu/insideuva/2004/07/pituitary_center.html

Simply the Best
Pituitary Center brings life-changing treatment to thousands

Dr. Edward Laws prepares to remove a pituitary tumor from a patient.

Photo by Andrew Shurtleff

Dr. Edward Laws prepares to remove a pituitary tumor from a patient. These tumors account for 20 percent of all primary brain tumors, and Laws is internationally recognized as the top pituitary surgeon.

By Fariss Samarrai

Dr. Edward LawsDr. Edward Laws stands on a wooden pedestal as he extracts a brain tumor through a woman’s right nostril. His resident, Dr. Adam Kanter, and a team of operating room specialists, have prepared the patient for the procedure by first anesthetizing her, and then, by sending long and slim instruments up the woman’s nostril, they have removed a thin wafer of bone, allowing direct access through the air sinuses to the pituitary gland at the base of the brain.

Laws’ job is to remove a tumor that has grown on the pituitary by carefully scraping loose and periodically removing fragments of the mass of bad cells while being careful to leave the healthy tissue of the pituitary and the brain intact. He does this while looking at the inside of the patient’s head through a binocular endoscope with a micro-camera attached, allowing everyone in the OR to observe the procedure on a video monitor.

He pulls out another fragment of the tumor.

“These tumors have the texture of tofu,” he says, though the tissue he is extracting is bloody. He mentions in passing that he used to tell people that pituitary tumors have the texture of tapioca, but that many people don’t seem to know what that is anymore.

Laws removes a final fragment of the tumor, and his work is done for now. The procedure is called trans-sphenoidal surgery — basically access-through-the-nose surgery. By day’s end Laws will have removed pituitary tumors from six patients, likely making the lives of these people immeasurably better.

Laws is director of the Pituitary Center at U.Va. He has removed pituitary tumors from more than 4,300 patients, more than any other doctor in the world. Most of these surgeries have been performed at the U.Va. center he helped found in 1972. Laws is internationally recognized as the top pituitary tumor surgeon. Medical residents from around the world come to U.Va. to train under him.

Illustration by Craig Luce

Pituitary Tumors
Where they are and how they are removed

The pituitary gland is located at the base of the brain near the spinal cord, in the lower center of the head. This gland, normally the size of a pea, is the master gland that controls the production of all hormones. The pituitary controls growth, sexual maturation, fertility, metabolism, stress response and fluid balances. Pituitary tumors are reached by passing long and slim surgical instruments through the air sinuses to the diseased gland.

The procedure, like all planned operations, is methodical, calm, controlled, predictable, with precautions in place for possible problems or emergencies. The operation is so un-invasive, it more resembles routine dental work than major surgery. Yet, Laws and his colleagues are working at the very edge of the brain. There is little room here for error.

In past years, pituitary surgery was more dramatic, and intensive, for both the surgeon and the patient. It involved removing a portion of the skull and pushing the brain aside to reach the pituitary. The recovery period was long, and there was much greater risk for damage and complications. Laws and others invented and refined the current technique — passage through the nose — which rarely involves direct contact with the brain, even as they operate within its midst.

The pituitary gland is located at the base of the brain near the spinal cord, in the lower center of the head. This gland, normally the size of a pea, is the master gland that controls the production of all hormones. The pituitary controls growth, sexual maturation, fertility, metabolism, stress response and fluid balances.

For reasons not well understood, the pituitary is subject to the slow growth of tumors. These tumors are the third-most common type of primary brain tumor, accounting for 20 percent of all brain tumors. Though these tumors are almost always benign, they aren’t friendly. They can cause the pituitary gland to produce excessive or insufficient amounts of hormones, leading to a vast array of unpleasant symptoms, including severe and chronic headaches, infertility, sexual dysfunction, fatigue, weight gain, depression, apathy, sleep disorders, memory loss, and in some cases, loss of vision. Pituitary disorders also are the cause of Cushing’s syndrome, which results in obesity, “moonlike” features of the face, excess body hair and thin brittle bones.

Pituitary surgeries are discussed prior to and after each operation.

Photographs by Andrew Shurtleff

Pituitary surgeries are discussed prior to and after each operation.

Because the symptoms of pituitary disorders are so varied, it can take years to arrive at a correct diagnosis,” Laws said. “Patients often think that their health problems are attributed to just getting older.”

It takes a brain scan in the form of magnetic resonance imaging to confirm the presence of a pituitary tumor. Sometimes the tumor is three times the size of the gland itself, putting pressure on the brain and optic nerve, resulting in headaches and vision loss. Small tumors often can be treated with hormones and drugs. Radiation may shrink the tumor or inhibit its growth. If the tumor continues to grow, and symptoms worsen, surgery may be necessary.

Laws discovered early, at the beginning of his medical training at Johns Hopkins University, that he was interested in neurosurgery. His fascination with the brain has continued throughout his 40-year career as a surgeon and brain tumor researcher.

“How beautiful the brain is,” he said recently. “I’m amazed with what happens with the human brain, and I love dealing with it in an intimate fashion.” He describes the brain as “one of the greatest of God’s creations.”

But he’s happy to fix that creation when something goes wrong.

“We can restore vision,” he said of pituitary surgery. “We can restore fertility and reverse body changes. This is enormously satisfying.”

These tumors are identified using magnetic resonance imaging.

These tumors are identified using magnetic resonance imaging.

One of the first patients Laws treated with the through-the-nose surgical method was a 21-year-old man named Harvey Gartner. The surgery was performed in 1969 at The Johns Hopkins Hospital where Laws was a neurosurgery resident. Gartner had been growing rapidly since he was 6 months old. His bones were elongated, and he suffered from arthritis and other ailments.

“Those were tough years,” Gartner said of his youth.

The operation took 12 hours to complete, and all of the pituitary gland was removed, as was standard at the time. Gartner, now 56, is on lifetime hormone replacement therapy, but his health is generally good.

“I would have died,” if not for the surgery, he said.

Gartner is now a computer programmer and businessman in Jacksonville, Fla. He has remained in contact with Laws, and the pediatrician who cared for him throughout his life, Dr. Robert Blizzard, a U.Va. professor emeritus of pediatric endocrinology. At the time of Gartner’s surgery, Hopkins was the top pituitary center in the United States. That title has long since shifted to U.Va. as a result of Laws, Blizzard and others coming here to establish their interdisciplinary center.

Today, surgeons rarely need to remove the entire pituitary gland. Instead, when surgery is necessary, they remove only the tumor attached to the gland. The entire procedure, including prepping the patient for surgery and the post-operative period, can be accomplished in a couple of hours. The patient is able to return home in two or three days and can resume normal activities in two to four weeks.

Within a few days after surgery, the patient’s severe headaches will have diminished. Vision will be restored within a day in cases where the tumor had pressed against the optic nerve. Body changes, such as weight loss and normalization of facial features, will occur in about six months. Throughout the patient’s life, teams of endocrinologists and other specialists will measure the patient’s hormone levels and develop and refine treatment plans. Laws said some patients might not even need hormone replacement therapy.

“I don’t think anybody does this work better than we do at U.Va.,” Laws said recently between surgeries. “We’re improving our techniques and reducing the recurrence of tumors after surgery.”

At this moment he is called back into the operating room. Another patient is prepped and waiting.

Cutting-edge center
For treatment and research

The interdisciplinary Pituitary Center at U.Va. pools the talents of leading specialists in endocrinology, neurosurgery, neuro-ophthalmology, neuropathology and other areas. The specialists work as a team to diagnose patients and develop treatment plans.

Health care professionals at the center evaluate about 500 new patients each year, most of them referrals from around the Commonwealth, and the rest from other states and around the world. More than 260 new patients are operated on each year at the hospital and more than a thousand patients per year are treated at the center’s clinic at Fontaine Research Park.

Every 18 months the center brings together patients and referring physicians. The center provides training and education for fellows, medical students and residents.

Center specialists are developing a national and international referral base, providing pituitary information to physicians and patients worldwide.

Researchers with the center are constantly looking for ways to control and perhaps someday stop the development of pituitary growths.

“We want to understand why these tumors develop in the first place,” said Dr. Edward Laws, the center director.

Clinical trials are conducted at the center to evaluate the effectiveness of new treatments, and Laws and other surgeons are always refining surgical techniques and developing new ones. They have operated on more than 350 patients with a “gamma knife” — a precise beam of gamma radiation that can evaporate abnormal pituitary tissue. This, and other techniques, keep the center on the cutting edge.

Corcept Therapeutics will sell stock and warrants to fund study and submission for Corlux

Associated Press
10/13/09 8:10 AM PDT

MENLO PARK, CALIF. — Corcept Therapeutics Inc. said Tuesday it expects to raise about $18 million by selling stock and options to institutional investors.

Corcept said the money will be used to complete enrollment of patients in a late stage clinical trial of its drug candidate Corlux as a treatment for Cushing's syndrome, and to cover the cost of filing for Food and Drug Administration marketing clearance.

The $18 million is a gross estimate and does not include expenses connected to the offering.

Corcept said it will sell 12.6 million units at $1.43 per share. The units will consist of one share of stock, and a warrant to buy 0.35 shares at an exercise price of $1.66 per share. Buyers include current Corcept investors such as Longitude Capital Management, Sutter Hill Ventures, Alta Partners and new investors including Federated Kaufmann Funds.

The sale is expected to close on Friday.

The company is testing Corlux as a treatment for Cushing's syndrome and psychotic depression. Cushing's syndrome occurs when the body produces to much of the hormone cortisol, or by overexposure to cortisol. Symptoms can include high blood sugar and blood pressure, fatigue, and weak muscles.


From http://www.sfexaminer.com/economy/ap/64084462.html

Another Cushing's Patient has Died :(

I did not know Bonny personally but she was an Australian member of the Cushing's Help message boards who rarely posted. 

She was only 45 at the time of her death October 12, 2009.  I've known far too many Cushies who have died far too young from this disease.


Bonnie's Avatar

Bonny wrote July 1, 2009

I was sick with ALL the symptoms (about 30-40) for 5 years. Finally got correctly diagonosed and had my left Adrenal Gland and its tumour removed in June 2007. The recovery was long and hellish. The worst symptom after the operation was 3 months of constant itching literally from my scalp to my heels and every inch of skin in between. I also had pain in every single joint of my body, along with all the pre op symptoms that took a long long time to improve.

Now two and a half years on, I have a second tumour... on the same side! No idea how that can be seeing as the gland is gone. My Endo is overseas so until he comes back I don't know much, but they are running more tests and I am waiting for a surgery date to go through it all over again!

All the symptoms are horrible, but last time I particularly hated the fractures (still have a few of those),as they made life so difficutlt and painful, but also relly hated losing half my hair, and the weight gain and moon face. Feeling awful is terrible, but when you add the things that make you look horrible too, its pretty hard to take.

As a single parent, (divorced), life is very hard with Cushings as you don't have anyone else to do the things for you that you cant do yourself, or help you with your own personal stuff.


Before and after Cushing's pictures.

Rest in peace, Bonny!


Beth said it best on Facebook

(I) lost a very strong, courageous friend to the very disease she suffers from.. your pain is gone now, Bonny.. Rest well and thank you for touching my life. ♥

Monday, October 12, 2009

Swine Flu And Asthma: NIH Prepares To launch 2009 H1N1 Influenza Vaccine Trial In People With Asthma

The National Institutes of Health is preparing to launch the first government-sponsored clinical trial to determine what dose of the 2009 H1N1 influenza vaccine is needed to induce a protective immune response in people with asthma, especially those with severe disease. The study is cosponsored by the National Institute of Allergy and Infectious Diseases (NIAID) and the National Heart, Lung, and Blood Institute (NHLBI), both part of NIH.

"People with severe asthma often take high doses of glucocorticoids that can suppress their immune system, placing them at greater risk for infection and possibly serious disease caused by 2009 H1N1 influenza virus," says NIAID Director Anthony S. Fauci, M.D. "We need to determine the optimal dose of 2009 H1N1 influenza vaccine that can be safely administered to this at-risk population and whether one or two doses are needed to produce an immune response that is predictive of protection."

The study plan has been submitted to the Food and Drug Administration for review. With FDA allowing it to proceed, the clinical trial will be conducted at seven sites across the United States that participate in NHLBI's Severe Asthma Research Program.

This program already has a well-characterized group of participants with mild, moderate or severe asthma who may be eligible for this new study. These groups are largely distinguished by the amount and frequency of glucocorticoids needed to control asthma symptoms. People with mild disease may not need glucocorticoids, or may require low doses of inhaled glucocorticoids; those with moderate asthma need low to moderate doses of inhaled glucocorticoids; and those with severe asthma need high doses of inhaled glucocorticoids and frequently use oral glucocorticoids as well.

Individuals who already have been infected with 2009 H1N1 influenza or have received a 2009 H1N1 influenza vaccination will not be eligible for the study.
"The results of this study will have immediate implications for individuals with severe asthma as well as those who have milder asthma," says NHLBI Director Elizabeth G. Nabel, M.D.

Early results from other clinical trials of 2009 H1N1 influenza vaccines in healthy adults have shown that a single 15-microgram dose of 2009 H1N1 influenza vaccine without adjuvant is well tolerated and induces a strong immune response in most participants. The same vaccine also generates an immune response that is expected to be protective in healthy children ages 10 to 17 years. Ongoing trials are comparing the immune response to one and two doses of 15- or 30-micrograms of vaccine given three weeks apart in various populations.

The Centers for Disease Control and Prevention has recommended that certain at-risk populations receive the new H1N1 vaccine as a priority before the general population. These target populations include pregnant women, health care providers and individuals with underlying chronic medical conditions, including asthma.

People who have severe asthma may be particularly at risk for infection with the 2009 H1N1 influenza virus. A report published in 2004 suggested that some people who took high doses of glucocorticoids to treat their asthma may receive less protection from influenza vaccines against some strains of influenza. Early in the 2009 H1N1 flu outbreak a CDC review of hospital records found that people with asthma have a four-fold increased risk of being hospitalized with infection compared to the general population.

The study will enroll approximately 350 people with mild, moderate and severe asthma. Participants will be organized into two groups: those with mild or moderate asthma and those with severe asthma. Half of the participants in each group will receive a 15-microgram dose of vaccine, and the other half a 30-microgram dose. Three weeks later, each participant will receive a second dose of the same amount. The strength of the immune response induced by the vaccine will be determined in blood samples by measuring the level of antibodies against 2009 H1N1 flu virus.

Safety data will be collected and examined throughout the course of the study by trial investigators and by an independent safety monitoring committee. Participants will be monitored for any side effects they may experience because of the vaccine, as well as asthma attacks that occur during the study period.
The vaccine to be used in the trial, manufactured by Novartis, contains inactivated 2009 H1N1 influenza virus and therefore cannot cause anyone to become infected with the virus.

The trial will be conducted at the following locations:
Cleveland Clinic, Ohio
Emory University, Atlanta
University of Pittsburgh Asthma Institute
University of Virginia, Charlottesville
University of Wisconsin, Madison
Wake Forest University, Winston-Salem, N.C.
Washington University School of Medicine, St. Louis

Detailed information about this study can be found on the ClinicalTrials.gov Web site at http://clinicaltrials.gov/ct2/results?term=H1N1+AND+asthma.
NIAID Office of Communications
NIH/National Institute of Allergy and Infectious Diseases

New! Discussion/Debate Topics

I've started yet another blog to go with the Cushie Info website.

From time to time, I'll be posting topics for discussion/debate there and your comments are welcome.  On the right side of this blog is a little comment area so you can see the last 5 comments and/or questions.  If by some magic there are a lot of comments, I'll make that more than 5.

Just click on the Jump To link to participate in the debate!  Thanks.


Oh, and by the way - the newest blog is here: http://www.cushie.info/blog/

A Reappraisal of Diagnosing GH Deficiency in Adults

Journal of Clinical Endocrinology & Metabolism , doi:10.1210/jc.2009-1134

A Reappraisal of Diagnosing GH Deficiency in Adults: Role of Gender, Age, Waist Circumference, and Body Mass Index

Annamaria Colao*, Carolina Di Somma, Silvia Savastano, Francesca Rota, Maria Cristina Savanelli, Gianluca Aimaretti, and Gaetano Lombardi

Department of Molecular and Clinical Endocrinology and Oncology (A.C., C.D.S., S.S., F.R., M.C.S., G.L.), Section of Endocrinology, University of Naples "Federico II," Italy, 80131 Naples, Italy; and Section of Endocrinology (G.A.), Department of Clinical and Experimental Medicine, University "A. Avogadro" del Piemonte Orientale, 28100 Novara, Italy

* To whom correspondence should be addressed. E-mail: colao@unina.it.

Objective: The objective of the study was to reevaluate the diagnostic accuracy of GH peak after GHRH plus arginine test (GHRH+ARG) according to patients' age, body mass index (BMI), and waist circumference to diagnose GH deficiency (GHD).

Outcome Measures: GH peak after GHRH+ARG and IGF-I levels reported as SD score.

Subjects: Subjects included 408 controls (218 women, 190 men, aged 15–80 yr) and 374 patients with hypopituitarism (167 women, 207 men, aged 16–83 yr).

Results: In the (elderly) healthy subjects 15–25 yr old (young), 26–65 yr old (adults) and older than 65 yr, GH cutoffs were 15.6, 11.7, and 8.5 µg/liter, 11.8, 8.1, and 5.5 µg/liter, and 9.2, 6.1, and 4.0 µg/liter, respectively, in the lean, overweight, and obese subjects. Waist circumference was the best predictor of GH peak (t = -7.6, P < 0.0001) followed by BMI (t = -6.7, P < 0.0001) and age (t = -5.7, P < 0.0001). Based on the old (<9.1 µg/liter) and new GH cutoff, 286 (76.5%) and 276 (73.8%) of 374 hypopituitary patients had severe GHD. The receiving-operator characteristic analysis showed GH cutoffs in line with the third percentile or slightly higher results so that the prevalence of GHD increased to 90.1%.

Conclusions: The results of the current study show that waist circumference and BMI are the strongest predictors of GH peak after GHRH+ARG followed by age. However, the old cutoff value of 9.0 µg/liter was in line with the new cutoffs in 95% of patients.

From http://jcem.endojournals.org/cgi/content/abstract/jc.2009-1134v1

Hydrocortisone Dosing during Puberty in Patients with Classical Congenital Adrenal Hyperplasia: An Evidence-Based Recommendation

Journal of Clinical Endocrinology & Metabolism, doi:10.1210/jc.2009-0942
The Journal of Clinical Endocrinology & Metabolism Vol. 94, No. 10 3882-3888
Copyright © 2009 by The Endocrine Society

Hydrocortisone Dosing during Puberty in Patients with Classical Congenital Adrenal Hyperplasia: An Evidence-Based Recommendation

Walter Bonfig, Susanne Bechtold Dalla Pozza, Heinrich Schmidt, Philipp Pagel, Dietrich Knorr and Hans Peter Schwarz

University Children’s Hospital (W.B., S.B.D.P., H.S., D.K., H.P.S.), Ludwig Maximilians University, Division of Pediatric Endocrinology, D-80337 Munich, Germany; and Division of Bioinformatics (P.P.), Technical University, D-80337 Munich, Germany

Address all correspondence and requests for reprints to: Walter Bonfig, M.D., University Children’s Hospital, Division of Endocrinology, Ludwig Maximilians University, Lindwurmstr. 4, D-80337 Munich, Germany. E-mail: walter.bonfig@med.uni-muenchen.de.

Context: Patients with congenital adrenal hyperplasia (CAH) are at risk for early pubertal development and diminished pubertal growth. Liberal treatment with glucocorticoids will prevent early puberty but may inhibit growth outright.

Objective: The aim of the study was to determine an optimal range for hydrocortisone dosing during puberty in children with classical CAH who were exclusively treated with hydrocortisone.

Methods: The effects of glucocorticoid treatment for classical CAH were retrospectively analyzed in 92 patients (57 females). Growth pattern, final height (FH), and mean daily hydrocortisone dose were recorded.

Results: Pubertal growth was significantly reduced in all patients: salt-wasting (SW) females, 13.8 ± 7.4 cm; simple virilizing (SV) females, 13.1 ± 6.2 cm; vs. reference, 20.3 ± 6.8 cm (P < 0.05); and SW males, 17.7 ± 6.7 cm; SV males, 16.2 ± 5.7 cm; vs. reference, 28.2 ± 8.2 cm (P < 0.05). Decreased pubertal growth resulted in FH at the lower limit of genetic potential (corrected FH in SW females, –0.6 ± 0.9; SV females, –0.3 ± 0.9; SW males, –0.8 ± 0.8; and SV males, –1.0 ± 1.0). During puberty, mean daily hydrocortisone dose was 17.2 ± 3.4 mg/m2 in females (SW, 17.0 ± 3.3; SV, 17.4 ± 3.5) and 17.9 ± 2.5 mg/m2 in males (SW, 17.4 ± 2.0; SV, 18.7 ± 3.1). In a logistic regression model, a significant correlation between hydrocortisone dose and FH was found (P < 0.01), and the positive predictive value for short stature rose from below 30% to above 60% when hydrocortisone dose exceeded 17 mg/m2.

Conclusion: With conventional hydrocortisone treatment, pubertal growth is significantly reduced in both sexes, resulting in a FH at the lower limit of genetic potential. These deleterious effects on pubertal growth can be reduced if hydrocortisone does not exceed 17 mg/m2.

From http://jcem.endojournals.org/cgi/content/abstract/94/10/3882

Cushing’s: the worst case scenario

Ami found this older abstract from last year.  Please, if you think you have Cushing's - or any serious disease - advocate for yourself before it's too late.  Over the years, too many Cushies have died. It's time to take charge of your life, the only one you will ever have...

Endocrine Abstracts (2008) 15 S58

Cushing’s: the worst case scenario

Viv Thornton-Jones

Churchill Hospital, Oxford, UK.

We present the case of a 40-year-old female who was referred to our Department in 1993, for further management following the diagnosis of Cushing’s disease. She proceeded to a transsphenoidal adenenomatectomy (TSA) which resulted in a biochemical cure.

In 1998 she presented with recurrence of Cushing’s Disease, which was managed by a 2nd TSA followed by external beam irradiation.

Bilateral adrenalectomy followed a year later, due to the inability to control her disease.

In 2001 she presented with Nelson’s Syndrome managed by a 3rd TSA followed this time with Gamma Knife surgery.

In 2004 she presented with manifestations consistent with recurrence of Nelson’s Syndrome and proceeded to a 4th TSA. Despite the risk of blindness, the patient agreed to a second course of Gamma Knife treatment for the possibility of tumour control.

Over the next 2 years her clinical picture deteriorated, resulting in a right partial ptosis and a sixth nerve palsy.

She was referred to an Oncologist who offered her Chemotherapy, but she refused treatment.

The patient was then in the care of the Palliative Care Team and she died peacefully at home in 2006.

Endocrine Abstracts (2008) 15 S58

From http://www.endocrine-abstracts.org/ea/0015/ea0015S58.htm

Sunday, October 11, 2009

ACTH-producing pheochromocytoma: Clinical considerations and concise review of the literature

M.F. Nijhoffab, O.M. Dekkersac, L.J. Vlemingb, J.W.A. Smita, J.A. Romijna, A.M. PereiraaCorresponding Author Informationemail address

Received 5 May 2009; received in revised form 9 July 2009; accepted 10 August 2009. published online 01 September 2009.


We present a patient with a rare cause of ectopic ACTH-dependent Cushing's syndrome, caused by a pheochromocytoma. The case provides clues for a detailed discussion on the pitfalls and diagnostic difficulties in establishing the correct underlying cause of ACTH-dependent Cushing's syndrome. It clearly demonstrates that the relative contributions of clinical, biochemical, and radiological clues in establishing the correct underlying cause of Cushing's syndrome may differ considerably between Cushing's disease and Cushing's syndrome due to ectopic overproduction of ACTH. In addition, the literature concerning ACTH-producing pheochromocytomas is reviewed.


Keywords: Pheochromocytoma, Adrenocorticotropic hormone, Cushing's syndrome, ACTH syndrome, Ectopic

a Department of Endocrinology & Metabolism, C4-R, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands

b Department of Internal Medicine, HAGA, location Leyenburg, Leyweg 2752545 CH the Hague, The Netherlands

c Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands

Corresponding Author InformationCorresponding author. Tel.: +31 71 6263082; fax: +31 71 524 8146.

PII: S0953-6205(09)00159-9


© 2009 European Federation of Internal Medicine. Published by Elsevier Inc All rights reserved.


From http://www.ejinme.com/article/PIIS0953620509001599/abstract?rss=yes

Saturday, October 10, 2009

Sam and Jackie Cushing's episode on Mystery Diagnosis

Jackie (samsmom) first started dealing with Cushing's in her family when her youngest child was born in 1999.

Jackie has appeared on the Discovery Health tv show pilot, Mystery Diagnosis, discussing her fight for a cure for her younger daughter, as well.

Download from amazon.com: Mystery Diagnosis

Later at the NIH, the gene (PDE11A) responsible for Sam's illness was found. Her father carried the gene as did her two sisters.

Sam's doctor at NIH, Dr Stratakis has written several papers on Sam's case including one that was published in April, 2008. For more on PDE11A or iMad, please see A cAMP-specific phosphodiesterase (PDE8B) that is mutated in adrenal hyperplasia is expressed widely in human and mouse tissues: a novel PDE8B isoform in human adrenal cortex.

Jordan, Jackie's oldest daughter, has recently had her adrenals removed. She had one removed laparoscopically and the other was an open adrenalectomy. She participated in this Interview.

Read Jackie's bio
Listen to Archived Interview from May 15, 2008

NEW! Download from amazon.com: Mystery Diagnosis

Friday, October 9, 2009

"Mild" is probably not diagnosable esp with typo! @helpareporter XX is seeking women (non-experts) between 30 & 55 who have Mild Crushing's.

Reading a Great Book


I'm reading this book on my Kindle right now...

Read more about this in this blog post:


Well: Searching for the Right Diagnosis

By By Tara Parker-Pope

Published: October 8, 2009

In today's Doctor and Patient column, Dr. Pauline Chen explores the art and science of medical diagnosis.

Read this article at http://well.blogs.nytimes.com/2009/10/08/searching-for-the-right-diagnosis/

Participatory Medicine

This is kind of a "cheat" post since it's a compilation of other posts, web pages, message board posts and some original thoughts.  I'm writing it to submit to Robin's Grand Rounds, being hosted next week on her blog.

For all of my early life, I was the good, compliant, patient.  I took whatever pills the doctor prescribed, did whatever tests h/she (most always a he) wrote for.  Believed that whatever he said was the absolute truth.  He had been to med school.  He knew what was wrong with me even though he didn't live in my body 24/7 and experience what I did. 

I know a lot of people are still like this.  Their doctor is like a god to them.  He can do no wrong - even if they don't feel any better after treatment, even if they feel worse.  "But the doctor said..."

Anyway, I digress.

All this changed for me in 1983.

At first I noticed I'd stopped having my periods and, of course, I thought I was pregnant. I went to my Gynecologist who had no explanation. Lots of women lose their periods for a variety of reasons so no one thought that this was really significant.

Then I got really tired, overly tired. I would take my son to a half hour Choir rehearsal and could not stay awake for the whole time. I would lie down in the back of the van, set an alarm and sleep for the 30 minutes.

A whole raft of other symptoms started appearing - I grew a beard (Hirsuitism), gained weight even though I was on Weight Watchers and working out at the gym nearly every day, lost my period, everything hurt, got what is called a "moon face" and a "buffalo hump" on the back of my neck. I also got stretch marks. I was very depressed but it's hard to say if that was because of the hormone imbalance or because I felt so bad and no one would listen to me.

I came across a little article in the Ladies Home Journal magazine which said "If you have these symptoms...ask your doctor about Cushing's". After that, I started reading everything I could on Cushing's and asking my doctors. Due to all my reading at the library and medical books I bought, I was sure I had Cushing's but no one would believe me. Doctors would say that Cushing's Disease is too rare, that I was making this up and that I couldn't have it.

I asked doctors for three years - PCP, gynecologist, neurologist, podiatrist - all said the now-famous refrain.  It's too rare.  You couldn't have Cushing's.  I kept persisting in my reading, making copies of library texts even when I didn't understand them, keeping notes.  I just knew that someone, somewhere would "discover" that I had Cushing's.

My husband was on the doctors' sides.  He was sure it was all in my mind (as opposed to all in my head!) and he told me to just think "happy thoughts" and it would all go away.

A Neurologist gave me Xanax. Since he couldn't see my tumor with his Magnetic Resonance Imaging (MRI) machine there was "no possibility" that it existed. Boy was he wrong!

Later in 1986 I started bruising incredibly easily. I could touch my skin and get a bruise. On New Year's Day of 1987 I started bleeding under the skin. My husband made circles around the outside perimeter each hour with a marker, like the rings of a tree. When I went to my Internist the next day he was shocked at the size. He now thought I had a blood disorder so he sent me to a Hematologist/Oncologist.

Fortunately, the Hematologist/Oncologist ran a twenty-four hour urine test and really looked at me. Both he and his partner recognized that I had Cushing's. Of course, he was sure that he did the diagnosis.  No matter that I had been pursuing this with other doctors for 3 years.

It was not yet determined if it was Cushing's Disease (Pituitary) or Syndrome (Adrenal). However, he couldn't help me any further so the Hematologist referred me to an Endocrinologist.

The Endocrinologist, of course, didn't trust the other tests I had had done so I was back to square one. He ran his own multitude of tests. He had to draw blood at certain times like 9 AM. and 5 PM. There was a dexamethasone suppression test where I took a pill at 10 p.m. and gave blood at 9 am the next day. I collected gallons of urine in BIG boxes (Fun in the fridge!). Those were from 6 a.m. to 6 a.m. to be delivered to his office by 9 a.m. same day. I was always worried that I'd be stopped in rush hour and the police would ask about what was in that big container. I think I did those for a week. He also did standard neurological tests and asked lots of questions.

When the endo confirmed that I had Cushing's in 1987 he sent me to a local hospital where they repeated all those same tests for another week and decided that it was not my adrenal gland (Cushing's Syndrome) creating the problem. The doctors and nurses had no idea what to do with me, so they put me on the brain cancer ward.

When I left this hospital after a week, we didn't know any more than we had before.

As luck would have it, NIH (National Institutes of Health, Bethesda, Maryland) was doing a clinical trial of Cushing's. I live in the same area as NIH so it was not too inconvenient but very scary at first to think of being tested there. At that time I only had a choice of NIH, Mayo Clinic and a place in Quebec to do this then-rare pituitary surgery called a Transsphenoidal Resection. I chose NIH - closest and free. After I was interviewed by the Doctors there, I got a letter that I had been accepted into the clinical trial. The first time I was there was for 6 weeks as an inpatient. More of the same tests.

There were about 12 of us there and it was nice not to be alone with this mystery disease. Many of these Cushies (mostly women) were getting bald, couldn't walk, having strokes, had diabetes. One was blind, one had a heart attack while I was there. Towards the end of my testing period, I was looking forward to the surgery just to get this whole mess over with. While I was at NIH, I was gaining about a pound a day!

The MRI still showed nothing, so they did a Petrosal Sinus Sampling Test. That scared me more than the prospect of surgery. (This test carries the risk of stroke and uncontrollable bleeding from the incision points.) Catheters were fed from my groin area to my pituitary gland and dye was injected. I could watch the whole procedure on monitors. I could not move during this test or for several hours afterwards to prevent uncontrolable bleeding from a major artery. The test did show where the tumor probably was located. Also done were more sophisticated dexamethasone suppression tests where drugs were administered by IV and blood was drawn every hour (they put a heplock in my arm so they don't have to keep sticking me). I got to go home for a weekend and then went back for the surgery - the Transsphenoidal Resection. I fully expected to die during surgery (and didn't care if I did) so I signed my will and wrote last letters to those I wanted to say goodbye to. During the time I was home just before surgery, a college classmate of mine (I didn't know her) did die at NIH of a Cushing's-related problem. I'm so glad I didn't find out until a couple months later!

November 3, 1987, the surgeon, Dr. Ed Oldfield, cut the gum above my front teeth under my upper lip so there is no scar. He used tiny tools and microscopes. My tumor was removed successfully. In some cases (not mine) the surgeon uses a plug of fat from the abdomen to help seal the cut. Afterwards, I was in intensive care overnight and went to a neurology ward for a few days until I could walk without being dizzy. I had some major headaches for a day or two but they gave me drugs (morphine) for those. Also, I had cotton plugs in my nostrils. It was a big day when they came out. I had diabetes insipidus (DI) for a little while, but that went away by itself - thank goodness!

I had to use a foam product called "Toothies" to brush my teeth without hitting the incision. Before they let me go home, I had to learn to give myself an injection in my thigh. They sent me home with a supply of injectible cortisone in case my level ever fell too low (it didn't). I was weaned gradually off cortisone pills (scary). I now take no medications. I had to get a Medic Alert bracelet. I will always need to tell medical staff when I have any kind of procedure - the effects of my excess cortisone will remain forever.

I went back to the NIH for several follow-up visits of a week each where they did all the blood and urine testing again. After a few years NIH set me free. Now I go to my "outside" endocrinologist every year for the dexamethasone suppression test, 24-hour urine and regular blood testing.

As I get further away from my surgery, I have less and less chance that my tumor will grow back. I have never lost all the weight I gained and I still have the hair on my chin but most of my other symptoms are gone. I am still and always tired and need a nap most days. I do not, however, still need to take whole days off just to sleep.

I consider myself very lucky that I was treated before I got as bad as some of the others on my floor at NIH but think it is crazy that these symptoms are not taken seriously by doctors.

My story goes on and if you're interested some is on this blog and some is here:

Forbes Magazine | MaryO's bio | Cushing's and Cancer Blog | Guest Speakers | Interview Archive  1/3/08 | Cushing's Awareness Day Testimonial Archive  4/8/08 |

Because of this experience in getting a Cushing's diagnosis - and later, a prescription for growth hormone - I was concerned that there were probably other people not being diagnosed with Cushing's. When I searched online for Cushing's, all the sites that came up were for dogs and horses with Cushing's.  Not what I was looking for!

In July of 2000, I was talking with my dear friend Alice, who runs a wonderful menopause site, Power Surge, wondering why there weren't many support groups online (OR off!) for Cushing's.  This thought percolated through my mind for a few hours and I realized that maybe this was my calling.  Maybe I should be the one to start a network of support for other "Cushies" to help them empower themselves.

I wanted to educate others about the awful disease that took doctors years of my life to diagnose and treat - even after I gave them the information to diagnose me.  I didn't want anyone else to suffer for years like I did.  I wanted doctors to pay more attention to Cushing's disease.

The first website (http://www.cushings-help.com) went "live" July 21, 2000.  It was just a single page of information. The message boards began September 30, 2000 with a simple message board which then led to a larger one, and a larger.  Today, in 2009, we have over 6 thousand members.  Some "rare disease"!

The message boards are now very active and we have weekly online text chats, weekly live interviews, local meetings, conferences, email newsletters, a clothing exchange, a Cushing's Awareness Day Forum, podcasts, phone support and much more. Because I wanted to spread the word to others not on "the boards" we have extended out to social networking sites - twitter groups, facebook groups, twines, friendfeeds, newsletters, websites, chat groups, multiply.com, and much, much more.

People are becoming more empowered and participating in their own diagnoses, testing and treatment.  This have changed a lot since 1983!

When I had my Cushing's over 20 years ago, I never thought that I would meet another Cushing's patient in real life or online. Back then, I'd never even been aware that there was anything like an "online". I'm so glad that people struggling with Cushing's today don't have to suffer anymore thinking that they're the only one who deals with this.

Because of my work on the websites - and, believe me it is a ton of work! - I have had the honor of meeting over a hundred other Cushies personally at local meetings, conferences, at NIH (the National Institutes of Health in Bethesda, MD where I had my final diagnosis and surgery). It occurred to me once that this is probably more than most endocrinologists will ever see in their entire career. I've also talked to countless others on the phone. Amazing for a "rare" disease!

I don't know what pushed me in 1983, how I got the confidence and self-empowerment to challenge these doctors and their non-diagnoses over the years.  I'm glad that I didn't suffer any longer than I did and I'm glad that I have a role in helping others to find the medical help that they need.

Thursday, October 8, 2009

4 new and updated Cushing's bios added. dx include 1 pituitary, 3 undiagnosed http://ow.ly/tnWb OR http://ping.fm/mv5E0
Cushing's locations page updated, 4 new people added from England, US. http://ow.ly/tnVr

Wednesday, October 7, 2009

Utility of Salivary Cortisol Measurements in Cushing’s Syndrome and Adrenal Insufficiency

by Hershel Raff

Endocrine Research Laboratory, Aurora St. Luke’s Medical Center, Milwaukee, Wisconsin 53215; and Division of Endocrinology, Metabolism, and Clinical Nutrition, Department of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin 53226

Address all correspondence and requests for reprints to: Hershel Raff, Ph.D., St. Luke’s Physician’s Office Building, 2801 West KK River Parkway, Suite 245, Milwaukee Wisconsin 53215. E-mail: hraff@mcw.edu.

Context: The measurement of cortisol in saliva is a simple, reproducible, and reliable test to evaluate the normal and disordered control of the hypothalamic-pituitary-adrenal (HPA) axis. There are a variety of simple methods to obtain saliva samples without stress, making this a robust test applicable to many different experimental and clinical situations.

Evidence Acquisition: Ovid Medline and PubMed from 1950 to present were searched using the following strategies: [<saliva or salivary>and<cortisol or hydrocortisone>and<Cushing or Cushing’s>] and [<saliva or salivary>and<cortisol or hydrocortisone>and<adrenal insufficiency or hypoadrenalism or hypopituitarism or Addison’s disease>]. The bibliographies of all relevant citations were evaluated for any additional appropriate citations.

Evidence Synthesis: Measurement of an elevated late-night (2300 to 2400 h) salivary cortisol has a greater than 90% sensitivity and specificity for the diagnosis of endogenous Cushing’s syndrome. Late-night salivary cortisol measurements are also useful to monitor patients for remission and/or recurrence after pituitary surgery for Cushing’s disease. Because it is a surrogate for plasma free cortisol, the measurement of salivary cortisol may be useful during an ACTH stimulation test in patients with increased plasma binding protein concentrations due to increased estrogen, or decreased plasma binding protein concentrations during critical illness. Most reference laboratories now offer salivary cortisol testing.

Conclusions: It is expected that the use of the measurement of salivary cortisol will become routine in the evaluation of patients with disorders of the HPA axis.

From http://jcem.endojournals.org/cgi/content/abstract/94/10/3647

Tuesday, October 6, 2009

Gamma knife radiosurgery: a safe and effective salvage treatment for pituitary tumors not controlled despite conventional radiotherapy

Francesca Swords, , John Monson, , G.m. Besser, , Shern Chew, , Wm Drake, , Ashley Grossman,  and Pn Plowman,

F Swords, Endocrinology, Norfolk and Norwich University Hospitals NHS Foundation Trust , Norfolk, NR4 7UY, United Kingdom
J Monson, Department of Endocrinology, St Bartholomew's Hospital, London, United Kingdom
G Besser, Department of Endocrinology, St Bartholomew's Hospital, London, United Kingdom
S Chew, Department of Endocrinology, St Bartholomew's Hospital, London, United Kingdom
W Drake, Department of Endocrinology, St Bartholomew's Hospital, London, United Kingdom
A Grossman, London, United Kingdom
P Plowman, Department of Oncology, St Bartholomew's Hospital, London, United Kingdom

Francesca Swords, Email: francesca.swords@nnuh.nhs.uk

Objective: We report the use of "Gamma knife" radiosurgery (GK) in 25 patients with pituitary adenomas not cured despite conventional therapy, including external beam radiotherapy.

Patients and Methods: All patients had previously received conventional radiotherapy (CRT) a mean of 11.8 years prior to receiving GK; 23/25 had also undergone pituitary surgery on at least one occasion. Seventeen had hyper-functioning adenomas which were still requiring medical therapy without adequate biochemical control - 10 somatotroph adenomas, 6 corticotroph adenomas and one prolactinoma, while 8 patients had non-functioning pituitary adenomas (NFPAs).

Results: Following GK, mean growth hormone (GH) fell by 49% at 1 year in patients with somatotroph tumours. Serum insulin-like growth factor 1(IGF-1) fell by 32% at 1 year and 38% at 2 years. To date, 80% of patients with acromegaly have achieved normalisation of IGF-1, and 30% have also achieved a mean GH level <1.8ng/ml correlating with normalised mortality. 75% NFPAs showed disease stabilisation or shrinkage post-GK. The patient with a prolactinoma showed a dramatic response: 75% reduction in prolactin at 2 years, with marked shrinkage on magnetic resonance imaging. The results in corticotoph adenomas were variable.

Prior to GK 72% of patients were panhypopituitary, and 42% of the remainder have developed new anterior pituitary hormone deficiencies to date. No other adverse events have been detected at a mean follow up 36.4 months.

Conclusions: These data indicate that GK is a safe and effective adjunctive treatment for patients with NFPAs and acromegaly not satisfactorily controlled with surgery and radiotherapy.

From http://www.eje.org/cgi/content/abstract/EJE-09-0493v1

Monday, October 5, 2009

3 new and updated Cushing's bios added. dx include 1 adrenal, 2 undiagnosed
http://ping.fm/LeAXS OR http://ow.ly/sLc4
Cushing's locations page updated, 3 new people added from Canada, Japan, US. http://ow.ly/sKRO

Pituitary Microadenoma

Pituitary microadenomas are benign tumors affecting the pituitary gland that smaller than 10mm. They can be functional or non-functional. Functional microadenomas produce one of several different types of hormones, while non-functional do not produce any hormones.

Some examples of hormones produce by functional microademonas are thyroid, prolactin, growth, and ACTH. Symptoms vary depending on if the tumor is functional or not and also the location of the tumor. Symptoms can include tiredness, restlessness, headaches, vomiting, and dizziness.

These tumors can be diagnosed in a variety of ways. MRI, CT, blood tests, petrosal sinus testing, and eye exams are all used in determining a diagnosis. Treatment again is dependent upon the type and location of the microadenoma. It ranges from drug therapy to surgery to radiation treatment.

I have included 2 MRI images showing pituitary microadenomas.

From http://radpathology.blogspot.com/2009/10/pituitary-microadenoma.html

Friday, October 2, 2009

NIH Research Festival http://ping.fm/yXPTU

(pituitary) New Technique for Brain Tumor Removal Promises Less Pain

Neurosurgeons are constantly looking for new ways to remove delicate brain tumors. The latest technology allows some of these growths to be removed through the nose.


Louis Leal of Poteet is getting ready for brain surgery. A non-cancerous pituitary tumor is threatening his vision and ruining his overall health. Leal said, "I mean, I'm just so fatigued. It's very, it's moody. You get very moody."

At St. Luke's Baptist Hospital in San Antonio, neurosurgeon Dr. J.D. Day of the UT Health Science Center is performing Leal's surgery in a new way. He starts with a special tool that marries the patient's brain scans to the instruments he'll work with. This image guidance system provides a precise "map" of sorts to help the doctor avoid critical structures while removing the tumor.

When the lights go down, a scope is inserted through one nostril, elongated instruments through the other, allowing the surgeon to work efficiently without an incision.

Day said, "They don't have nearly as much pain. They don't have as much swelling. You know, the good old fashioned way, people would have a cut under their lip, they'd be swollen here through the face, bruised."

The angled scopes and better imaging give the surgeon a much broader view of where he's working. He's able to suck out part of the tumor and use tools to remove the rest through the nostril. This patient's hope is to return to his job soon.

"I come from a family where we're used to working, so I want to get back to work," Leal said.

Dr. Day says patients are able to recover in their own homes much sooner. "Most patients are out of the hospital in two days after having brain surgery," Day said.

Pituitary tumors are some of the most common benign brain tumors. They show up as often as one in 2,500 people. Not all of those require surgery, but for those who need the procedure, this approach through the nose should provide an easier recovery and in theory, a better outcome.

Courtesy of Wendy Rigby with CBS News

From http://www.kauz.com/news/national/63163282.html

Thursday, October 1, 2009