Not only was central obesity the most common physical manifestation associated with hypercortisolism among these Cushing's disease patients, but it was also more frequently rated as severe at baseline
Our patientās presentation was unusual as she presented at 40 years old, 10 years earlier than expected for PBMAH; and primarily with complaints of episodic blurry vision. Her symptoms also progressed rapidly as signs and symptoms largely presented over the course of 12 months, however responded well to surgical resection.
Literature regarding endogenous Cushing syndrome (CS) largely focuses on the challenges of diagnosis, subtyping, and treatment. The enigmatic phenomenon of glucocorticoid withdrawal syndrome (GWS), due to rapid reduction in cortisol exposure following treatment of CS, is less commonly discussed but also difficult to manage. We highlight the clinical approach […]
A worldwide, observational study of adults and adolescents with growth hormone deficiency (GHD) found long-term GH replacement was safe. These findings were published in theĀ Journal of Clinical Endocrinology & Metabolism. ā¦ The most common all-cause adverse events included arthralgia (4.6%), peripheral edema (3.9%), headache (3.6%), influenza (2.8%), dep […]
A difference in peak postoperative plasma copeptin as an early marker to predict remission of CD was not consistently present, although the data point to the need for a larger sample size to further evaluate this. However, the utility of this test may be limited to those who develop neither DI nor SIADH postoperatively.
Research opportunity for Human Growth Hormone Deficiency caregivers of adolescent patients. This is a 75 min web-assisted phone interview, and the compensation is $125.
Sadly, we lost another Cushingās patient on Friday, May 9, 2014. Melinda was a member of the Cushingās Help message boards since Jun 24, 2007. She was only 25 and left behind a young son and many loving family members.
Our community will experience a single, brief period of downtime this week. The exact timing is not known as this is a rolling update. There will be no data loss and most communities will see a downtime window of less than 10 minutes.
He died of a presumed heart attack. September 19, 2015 he said ''I was diagnosed hypertensive way back when I was 20. The condition remained for years, and became more acute with my cushings pit tumor. I still have high blood pressure, partially teated with three meds. I'll have to consult my doc and see if this may also be an issue.'
Despite improved outcomes in recent years, increased mortality from CS persists. The causes of death highlight the need to prevent and manage co-morbidities in addition to treating hypercortisolism.
Osilodrostat is associated with rapid normalization of mean urinary free cortisol (mUFC) excretion in patients with Cushing disease and has a favorable safety profile, according to the results of a study published in the Journal of Clinical Endocrinology & Metabolism.
The popular website "How Stuff Work"s is doing a survey of all kinds of diseases and Cushing's is one of them! The idea is simple.āØ Everyone shares experiences with different treatments. StuffThatWorks automatically transforms these experiences into data about which treatments work best, and for whom. Share your information and help get the wo […]
'My name is Caroline and I dont post often but have met a few of you guys and read the board regularly, it has definitely been a godsend to cushies everywhere. The reason I am writing tonight is I have just received devastating information about a dear friend of mine, and a woman some of you may have met during testing. Her name is Kathryn Miller and sh […]
Today is the final day of the Cushingās Awareness Challenge and I wanted to leave you with this word of adviceā¦ To that end, Iām saving some of what I know for future blog posts, maybe even another Cushingās Awareness Challenge next year.
Johns Hopkins’ Dr. Alfredo Quinones-Hinojosa writes:
In 2006, the ABC show “Hopkins” aired. Episodes 1 and 7 featured my patients. I would like to share these videos with you and encourage you to view them to witness what it is like inside the hospital. The emotions are real and many people who follow this page have experienced this first hand. One day we will find a cure for brain cancer.
Jordy is a British man who has been dealing with Cushing’s and many surgeries.
He finds rollercoasters boring, barely broke a sweat zip-wiring off the Tyne bridge and even a parachute jump did not raise his heart rate.
Just a few years ago even the thought of daredevil exploits would have terrified him, but now Jordy Cernik is frightened of nothing.
While that might sound an ideal scenario, the 38-year-oldās new-found bravery is actually the unexpected side-effect of surgery for a rare condition.
Cushingās Syndrome resulted in the dad-of-two having an operation to remove the gland which produces adrenalin, the hormone which makes us feel scared.
He says: āI would never have had the guts to do any of this, but now nothing fazes me. Iām up for anything ā Iām even thinking about doing a wing-walk on a plane too.
āI nearly did a bungee jump a few years ago, but I just couldnāt do it.
“Now I just take whatever is thrown at me and if a challenge helps me raise money for charity, the more daring the better.ā
Over the past four months he has completed the parachute jump and zip-wired from the top of Newcastleās Tyne Bridge and now he is getting ready to complete the last of a trio of challenges ā next monthās Bupa Great North Run.
āThe doctors didnāt tell me this could be one of the side-effects of the operation,ā says Jordy. āBut then the condition is so rare I donāt think they know everything about Cushingās yet.
āDoing the skydive was the ultimate test. I thought that if I was ever going to get scared again then that would be the moment.
“But as we took off in the plane I felt nothing, and when I edged towards the door to jump I felt nothing, and even when I leapt out and pulled my parachute, I didnāt feel scared at all.
āIt can be quite frustrating as well though.
“The first time I realised I had changed was when I went on the rides at a theme park with my kids and I just didnāt feel a thing. I just sat there, bored.ā
However, the last of his hat-trick of challenges, the Run, will require him to push through the ever-present pain which he has endured for years as a result of Cushingās.
Britainās biggest mass participation event, for which The Daily Mirror is a media partner, takes place over a 13.1 mile course from Newcastle to South Shields.
But the syndrome has left Jordy, from Jarrow, near Newcastle, with arthritis, back problems and brittle bones. Worse still, the absence of adrenalin means he now lacks one of the bodyās natural painkillers.
āIām always in pain,ā he says. āIāve just had to learn to zone it out day-to-day and Iām going to have to do that even more when Iām on the run.ā
Cushingās affects around one in 50,000 people in Britain.
It causes a malfunction of the adrenal and pituitary glands which means increased amounts of corticosteroids are produced ā often leading to massive, irregular weight gain.
In just three years 5ft 8in Jordy ballooned from 11st 5lb to almost 17st.
While his limbs remained slim, the former Territorial Army recruit saw the pounds pile around the major organs in his torso and head.
āI went through years of hell and I can only describe it as living in someone elseās body,ā says the part-time radio presenter and events host.
āI developed this big round moon face and really quite large man boobs, which was so embarrassing.
āBut there was absolutely nothing I could do about it. I could go to the gym six days a week and still couldnāt lose any of the weight.
“One of the worst things was that people would stare.
“Sometimes theyād take the mickey ā often to try and make me feel better, by making light of things ā but it would almost always hurt my feelings.
āAnd my career as a presenter suffered. I tried to play up to the character of being a big, jolly chap but I always felt I was too fat for TV, which is what I would have liked to do a lot more of.ā
But it was the effect on his home life with wife Tracy, 43, and daughters Aimee, seven, and four-year-old Eive that for him was far worse.
āI had other really difficult symptoms which included profuse sweating which meant I couldnāt even hold my kids without wrapping them in towels first,ā he says.
āAnyone who has children knows how hard that is, not to be able to do normal things. I often used to be in tears.
“Another symptom was extreme grumpiness, so I would find myself suddenly getting really angry and just exploding at them, plus I was always too exhausted to play with them. It was terrible.ā
Jordy believes he can trace his symptoms back 15 years although his Cushingās was only diagnosed in 2005.
He had visited his local surgery with a string of complaints, but by chance saw a different doctor one day and the syndrome was diagnosed.
āI donāt have any ill-feeling about that,ā he says, ābecause the syndrome can be tricky to spot, partly because it is so rare.ā
He went on to have both his pituitary and adrenal glands removed but needed a total of seven operations between 2005 and 2010 and not all went smoothly.
During one to remove his pituitary gland, which is inside the skull, the lining of his brain burst due to the stress of repeated surgery.
And while removing a rib to access the adrenal gland in his torso, his lung was punctured.
That wasnāt the end of the complications. He later developed severe meningitis and ended up on a life-support machine.
āBut I still consider myself lucky,ā he says. āThe doctors told me, āYou died twice really, you shouldnāt even be hereā.ā
Things have begun to look up in the past few years, however. The Cushingās is in remission and Jordy has lost four stone.
His life hasnāt returned to normal entirely ā he still has to take 30 pills a day, a cocktail of painkillers and hormones, plus drugs to slow the corrosion of his bones.
He has also been diagnosed with another rare condition, sarcoidosis, which creates nodules of irregular cells in the body and can cause serious complications. Heās convinced he has always had it but it has lain dormant until his body was at its most vulnerable.
At present the nodules can only be found on his skin and heās being monitored to ensure that it doesnāt spread to his internal organs.
Thanks to the surgery, his life has improved enormously since 2010.
In July he had a breast reduction op which not only improved his appearance but also removed the dangerous accumulation of fat around his heart.
Part of this new chapter involves taking part in the Great North Run and raising money for the Cash for Kids appeal run by his local radio station Metro Radio.
The appeal aims to help children and young people in the North East who are disabled or have special needs, or those who suffer from abuse or neglect.
Jordyās fundraising goal is a relatively modest Ā£1,000, but for him joining the half marathonās 56,000 participants on September 15 will be as rewarding as hitting his target.
āI really donāt know if Iāll be able to complete the course.ā he says. āBut Iām looking forward to it and Iām going to give it my best shot.
āNot feeling fear may feel like the power of a superhero, but what I really need for the Great North Run is superhero strength.ā
The Bupa Great North Run is Britainās biggest mass participation event and is organised by Nova International.
It will include world class athletes Mo Farah, Haile Gebrselassie and Kenenisa Bekele ā plus 56,000 other runners.
The event is live on BBC One on Sunday 15th September between 9.30am to 13.30
Faculty of Medicine, University of Belgrade, Department of Neuroendocrinology, Clinical Center Serbia, Dr Subotic 13, 11000 Belgrade, Serbia. popver@eunet.rs
Abstract
Identification of adults with GH deficiency (GHD) is challenging because clinical features of adult GHD are not distinctive and because clinical suspicion must be confirmed by biochemical tests.
Adults are selected for testing for adult GHD if they have a high pretest probability of GHD, ie, if they have hypothalamic-pituitary disease, if they have received cranial irradiation or central nervous system tumor treatment, or if they survived traumatic brain injury or subarachnoid hemorrhage.
Testing should only be carried out if a decision has already been made that if deficiency is found it will be treated. There are many pharmacological GH stimulation tests for the diagnosis of GHD; however, none fulfill the requirements for an ideal test having high discriminatory power; being reproducible, safe, convenient, and economical; and not being dependent on confounding factors such as age, gender, nutritional status, and in particular obesity.
In obesity, GH secretion is reduced, GH clearance is enhanced, and stimulated GH secretion is reduced, causing a false-positive result. This functional hyposomatotropism in obesity is fully reversed by weight loss. In conclusion, GH stimulation tests should be avoided in obese subjects with very low pretest probability.
New research shows that patients who are “biochemically cured” of Cushing’s syndrome have levels of brain metabolites which are associated with neural damage. This will have implications for treatment of Cushing’s patients, but might also suggest that patients using high levels of glucocorticoid drugs may suffer similar long-term problems. The work was presented yesterday at the European Congress of Endocrinology in Copenhagen.
Cushing’s syndrome is an endocrine disease causing an overproduction of theĀ stressĀ hormone cortisol. Surgery and medical treatment can normalise cortisol levels, however recently it has been shown that “biochemically cured” patients continue to have memory problems. Now for the first time a group of researchers from the Sant Pau Hospital in Barcelona has scanned the brains of patients who had suffered from Cushing’s syndrome and found that they exhibit changed levels of brain metabolites, which are associated with memory and cognitive impairments. This finding may also have clinical implications for otherwise healthy patients who take high levels of glucocorticoid drugs for inflammatory, rheumatoid diseases, allergies and probably everyday chronic stress.
Cortisol (a glucocorticoid hormone), is naturally produced by the adrenal glands in response to stress. Long term exposure to high levels of cortisol is known to be associated with a range of cognitive impairments – this is true for Cushing’s syndrome patients, and probably would be also for those who take glucocorticoid drugs.
Eugenia Resmini and colleagues, working at the Centre for Biomedical Research on Rare Diseases (CIBERER), Sant Pau hospital in Barcelona, used proton magnetic resonance spectroscopy to measure a series of metabolites in the hippocampus of the brains of 18 patients who had been treated for Cushing’s syndrome, and compared these results to 18 healthy control subjects. They found that levels of the metabolite NAA (NAcetyl-aspartate) were significantly lower in the Cushing’s patients, indicating neural dysfunction, whereas Glx (Glutamate +Glutamine) levels were higher, suggesting that glial cells were proliferating as a repair mechanism.
According to Dr Resmini MD, PhD, Endocrinologist at the Centre for Biomedical Research on Rare Diseases (CIBERER), Hospital de Sant Pau, Barcelona, Spain:
“Patients with Cushing’s syndrome are exposed to abnormally high levels of glucocorticoids, which is associated with a wide range of cognitive impairments, as well as loss of brain volume. We studied the hippocampus, which is a critical area for learning and memory and, as it is rich in glucocorticoid receptors, is especially vulnerable to glucocorticoid overexposure. Cushing’s syndrome patients with severe memory impairment are known to have a smaller hippocampus. We have now found abnormal levels of metabolites in the hippocampi of Cushing’s patients with normal hippocampal volumes, indicating that these are early markers of glucocorticoid neurotoxicity, which would precede hippocampal volume reduction.
“Identifying these metabolites as a marker would be a way of allowing earlier diagnosis and treatment of cognitive impairments. This may also allow us to monitor patients taking glucocorticoid drugs, which have potentially damaging side effects. On the other hand, the fact that these markers are still present in Cushing’s patients after being “biochemically cured”, may show that once cognition has been damaged in Cushing’s syndrome, it may not be fully reversible. For this reason an earlier diagnosis of the disease and a rapid normalization of hypercortisolism would avoid the progression of hippocampal damage and of memory problems”.
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