40 Days of Thankfulness: Day Twelve

 

Today, I am thankful for Saturdays.  It' the one day of the week I don't have to be anywhere, do anything.  I can do webwork, if I want, but I don't have any deadlines. 

I never have doctor appointments on Saturdays, no medical testing.

No piano students.  I don't even talk to prospective students on Saturdays.

It's a day for maybe brunch, a trip to the farm, maybe a little TV, maybe (most assuredly!)  a nap.

Saturdays are family days, even though our family is smaller than it was.

Saturdays are always full of promise.

Off to see what today's promise is...

40 Days of Thankfulness: Day Eleven

Today, I am thankful for Lisa.  Her photography studio ( http://www.dickensstudio.com/ ) held a Girls Night Out fundraiser which benefitted Cushing's Help.

Last night's raffle raised $600.00 and that will go a long way to keeping the message boards up and running for another year.

You, too, can be thankful for Lisa here.  :)

 

 

 

40 Days of Thankfulness: Day Ten

 

Today I am most thankful for naps!

Pituicytoma in a patient with Cushings disease: case report and review of the literature

K. Schmalisch, J. Schittenhelm, F. H. Ebner, F. Beuschlein, J. Honegger and R. Beschorner

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Abstract

Pituicytoma is an exceptionally rare low-grade glioma (WHO grade I) of the neurohypophysis and infundibulum. We are reporting the case of a 48-year-old man who presented with severe Cushing′s syndrome. Endocrinological evaluation unequivocally confirmed pituitary-dependent Cushing’s syndrome (=Cushing’s disease).

Cranial MR-imaging displayed a conspicuous area in the dorsal and basal pituitary gland and a minimal bulging of the pituitary gland paramedian of the pituitary stalk on the right side. Transsphenoidal inspection revealed a small tumor in the basal and dorsal pituitary gland.

Surprisingly, the definite postoperative histopathological diagnosis of the removed tumor was pituicytoma and not pituitary adenoma. Hence, the microadenoma responsible for Cushing’s disease was not yet removed and persistent hypercortisolism necessitated transsphenoidal re-operation. During re-operation, hemihypophysectomy was performed on the right side. The non-tumorous specimen of the adeno-hypophysis showed signs of Crooke’s hyalinization consistent with Cushing’s disease.

Undetectable postoperative ACTH- and cortisol levels provided clear evidence that the underlying ACTH-source was successfully removed during re-operation.

Coincidence of pituicytoma and pituitary-dependent Cushing’s disease has not previously been reported.

Keywords Pituicytoma - Neurohypophysis - Pituitary tumor - Glioma

J. Honegger and R. Beschorner contributed equally to the work and thus share senior-authorship.

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From http://www.springerlink.com/content/h22022134827275l/

40 Days of Thankfulness: Day Nine

I am so thankful for all my doctors but today I am thankful for Dr. Amir Al-Juburi who saved my life by removing my kidney cancer (renal cell carcinoma).

In 2006 I picked up my husband for a biopsy and took him to an outpatient surgical center. While I was there waiting for the biopsy to be completed, I started noticing blood in my urine and major abdominal cramps. I left messages for several of my doctors on what I should do. I finally decided to see my PCP after I got my husband home.

When Tom was done with his testing, his doctor took one look at me and asked if I wanted an ambulance. I said no, that I thought I could make it to the emergency room ok - Tom couldn't drive because of the anaesthetic they had given him. I barely made it to the ER and left the car with Tom to park. Tom's doctor followed us to the ER and became my new doctor.

When I was diagnosed in the ER with kidney cancer, Tom's doctor said that he could do the surgery but that he would recommend someone even more experienced, Dr. Amir Al-Juburi.

Dr. Amir Al-Juburi has been so kind to me, almost like a kindly grandfather might be, and he got rid of all 10 pounds of my kidney and cancer.

I owe him, the original doctor, and my Cushing's doctors (who will be featured later!), my life.

40 Days of Thankfulness: Day Eight

I called our son today, just to see how he was doing.  I am thankful that he's doing so well for himself, has a good job, good friends, is a fine musician and that he's not afraid to say he loves his parents.  :)

We love you, too!

40 Days of Thankfulness: Day Seven

Weight Watchers.

Yesterday, for the first time that I can ever remember, I bought size 12 pants. Even before Cushing's, I was bigger than this. Maybe it was just the company that makes them, cuts them more generously than others but still.

I've been a *bit* off-program since the summer but this will inspire me to get back with it.  I want to buy more 12s...and maybe 10s sometime.

 

 

40 Days of Thankfulness: Day Six

Simple but it says it...

Treatment of Cushing disease: overview and recent findings

Authors: Tatiana Mancini, Teresa Porcelli, Andrea Giustina

Published Date October 2010 , Volume 2010:6 Pages 505 - 516 DOI 10.2147/TCRM.S12952

Tatiana Mancini¹, Teresa Porcelli², Andrea Giustina^2
1Department of Internal Medicine and Medical Specialties, San Marino Hospital, San Marino, Republic of San Marino, ²Department of Medical and Surgical Sciences, University of Brescia, Brescia, Italy

Abstract:

Endogenous Cushing syndrome is an endocrine disease caused by excessive secretion of adrenocorticotropin hormone in approximately 80% of cases, usually by a pituitary corticotroph adenoma (Cushing disease [CD]). It is a heterogeneous disorder requiring a multidisciplinary and individualized approach to patient management.

The goals of treatment of CD include the reversal of clinical features, the normalization of biochemical changes with minimal morbidity, and long-term control without recurrence. Generally, the treatment of choice is the surgical removal of the pituitary tumor by transsphenoidal approach, performed by an experienced surgeon. Considering the high recurrence rate, other treatments should be considered.

Second-line treatments include more radical surgery, radiation therapy, medical therapy, and bilateral adrenalectomy. Drug treatment has been targeted at the hypothalamic or pituitary level, at the adrenal gland, and also at the glucocorticoid receptor level. Frequently, medical therapy is performed before surgery to reduce the complications of the procedure, reducing the effects of severe hypercortisolism.

Commonly, in patients in whom surgery has failed, medical management is often essential to reduce or normalize the hypercortisolemia, and should be attempted before bilateral adrenalectomy is considered. Medical therapy can be also useful in patients with CD while waiting for pituitary radiotherapy to take effect, which can take up to 10 years or more.

So far, results of medical treatment of CD have not been particularly relevant; however, newer tools promise to change this scenario. The aim of this review is to analyze the results and experiences with old and new medical treatments of CD and to reevaluate medical therapies for complications of CD and hypopituitarism in patients with cured CD.

Keywords: ketoconazole, somatostatin analogs, dopamine agonists, rosiglitazone, Cushing disease, glucocorticoids, hypopituitarism

From http://www.dovepress.com/treatment-of-cushing-disease-overview-and-recent-findings-peer-reviewed-article-TCRM

40 Days of Thankfulness: Day Five

Today I am thankful that we finished our taxes a day ahead of the extended deadline!

40 Days of Thankfulness: Day Four

Today, I'm thankful for my "Cushie Car".

Since I started attending Cushing's events, I've always tried to rent a PT Cruiser. There's just been something about them that I liked.

A couple years ago, after my son was out of school, I was getting tired of driving the Mom-Carpool-Van everywhere. I think my mom was getting tired of stepping up into it, too. She doesn't drive anymore, so she relies on whatever vehicle I'm driving to get her to appointments. So, I was delighted when she offered to pay for part of it. She didn't realize it was going to be bright blue, though. I think she was thinking of navy or something more dignified.

Anyway, I had the car picked out, and the exact color I wanted and set off to the car dealership with my specs. They told me that the Cruiser didn't come in this shade of blue. I'm sure that they wanted to sell one of the colors they had on the lot.

I got back to them and told them that I found this car online and I could go get it myself. Suddenly the dealership was able to get it for me, too, so I won out. WooHoo.

I'm just loving this car. :)

From a Cushing's get-together in Columbus, OH, 2007, the yellow version :)

40 Days of Thankfulness: Day Three

I am thankful today for our CSA farm, Great Country Farms.  We've been members there since our son was young, since 1995.  We just love being out in the country, picking whatever is available seasonally, having fresh fruits and vegges delivered.  The Zurschmeides are the nicest people you could ever want to meet, too.

From their website:

The goal of Great Country Farms is to offer a way for everyone to experience farm life and enjoy the benefits of its bounty.

Great Country Farms is a 200 acre working farm situated at the base of the Blue Ridge Mountains outside the village of Bluemont, VA. Great Country Farms offers produce as well as the farm experience to its customers. 

The Zurschmeide Family has been farming in Loudoun County for over 35 years and Great Country Farms was started by the second generation of  Zurschmeides in Loudoun in 1994. The farm started with a Community Supported Agriculture Program (CSA) which has grown over the years and delivers produce to homes as far as Alexandria and Arlington.   In 1996 and again in 2007, the Loudoun County Chamber of Commerce voted Great Country Farms, "Agribusiness of the Year" for its unique efforts to farm in a difficult climate through innovation, rather than selling out and growing houses.

We owe it all to Farmer Bob for instilling a love of farming in us. He still visits the farm regularly on weekends to continue that tradition for the new generation of farm visitors.  Be sure to look for him on your next visit. As our farm grows, we are thrilled to welcome the next generation of Zurschmeides in the fields, behind the counter, making Kettle Corn and as the Pumpkin Princess of course!

We are thrilled to still be a family farm in our growing county and welcome you to visit our farm, pick some produce, create some memories and begin your own farm adventure as part of our farm family.

 

Other pictures from one of my blogs.

Children with Cushing’s syndrome: primary pigmented nodular adrenocortical disease should always be suspected

Renata Marques Gonçalves da Silva, Emília Pinto, Suzan M. Goldman, Cássio Andreoni, Teresa C. Vieira and Julio Abucham

 

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Abstract

Primary Pigmented Nodular Adrenocortical Disease (PPNAD) is a rare form of bilateral adrenocortical hyperplasia that is inherited in an autosomal dominant manner and leads to ACTH-independent Cushing’s syndrome (CS). PPNAD may be isolated or associated with Carney Complex (CNC). For the diagnosis of PPNAD and CNC, in addition to the hormonal and imaging tests, searching for PRKAR1A mutations may be recommended.

The aims of the present study are to discuss the clinical and molecular findings of two Brazilian patients with ACTH-independent CS due to PPNAD and to show the diagnostic challenge CS represents in childhood. Description of two patients with CS and the many sequential steps for the diagnosis of PPNAD is provided. Sequencing analysis of all coding exons of PRKAR1A in the blood, frozen adrenal nodules (patients 1 and 2) and testicular tumor (patient 1) is performed. After several clinical and laboratory drawbacks that misled the diagnostic investigation in both patients, the diagnosis of PPNAD was finally established and confirmed through pathology and molecular studies. In patient 1, sequencing of PRKAR1A gene revealed a novel heterozygous 10-bp deletion in exon 3, present in his blood, adrenal gland and testicular tumor.

The etiologic diagnosis of endogenous CS in children is a challenge that requires expertise and a multidisciplinary collaboration for its prompt and correct management.

Although rare, PPNAD should always be considered among the possible etiologies of CS, due to the high prevalence of this disease in this age group.

Keywords  Cushing’s syndrome - PPNAD - Carney complex - PRKAR1A mutation - ACTH-independent CS

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From http://www.springerlink.com/content/3095564q1tlv5508/

Back Ache And Hypercortisolism

Hypercortisolism is an extended medical time period that defines Cushing’s syndrome. Cushing’s syndrome is a hyperactive disorder that affects the adrenal cortex and leads to extreme secretion of cortisol, which is handed from Glucocorticoids. Cushing’s syndrome can increase sex hormones and mineralocorticoids.

The pituitary glands are stimulated by hypothalamic. The pituitary glands are additionally affected by carcinoma and/or adenoma. As nicely, the adrenal glands are affected by hyperplasia when Cushing’s syndrome is present. When Cushing’s syndrome is current, exogenous secretes into the ACTH through the neoplasm, which is malignant. It continues onto the gallbladder and lungs. You will need to read the anatomy of the skeleton system to see the way it affects the spinal column, which in turn causes back pain.

The dysfunction prolongs or submits excessive administration of ACTH and/or Glucocorticoids into the system, which transmits to the cortex. Since ACTH is secreted excessively into the system, it causes joint ache, edema, fragile pores and skin, weight achieve, hypertension, ecchymosis, fatigue, weak spot, hirsutism, temper swings, and so on. The signs carry onto create acne, stomach striae, slow healing, moon face, muscle waste, recurrent infections, buffalo humps, gynecomastia, truncal weight problems, and so on. We see that weight problems, joint pain, weight acquire, edema, and other components of the dysfunction causes back ache as well.

The signs are thought of earlier than diagnostics is conducted. Medical doctors will use a wide range of assessments to find Hypercortisolism or Cushing’s syndrome. In brief, Cushing’s syndrome is a condition set up by weak muscles and obesity, or irregular situations of the physique’s functions. The exams performed to show Cushing’s syndrome include blood chemistry, dexamethasone suppression, X-rays, GTT, CT scans, angiography, ultrasonography, and so on. Throughout testing docs will search for decreases in “17-OHCS,” osteoporosis, tumors, particularly in the pituitary glands and adrenal glands, decreases in potassium, will increase in cortisol, sodium, Aldosterone, ACTH, etc. Doctors will also search for decreases in eosinophilis, pink blood cells, and white blood cells.

When the situation is noted, docs suggest management. Diets are instructed, which include low-calorie, sodium, carbohydrates, etc. The patient is ordered to take excessive-protein and potassium regimens as well. Activity is ordered, but solely as tolerated by the patient.

Once management begins, the doctor will monitor the patient. During monitoring your physician will perform extra checks, which include UO, I/O, VS, glucose, ketones, and so on. Radiation remedy is prescribed within the worst conditions.

Cushing’s syndrome can lead to additional problems, together with nephrosclerosis, inadequate adrenal, fractures, arteriosclerosis, infections, diabetes mellitus, hypertension, CHF, arrhythmias, psychosis, and so on.

If you are diagnosed with Cushing’s syndrome, it is important to maintain your weight loss plan, steadiness fluids, relaxation, and restrict intake of water. Your physician will arrange a routine and/or management scheme, which you need to comply with accordingly to avoid additional complications. Since this dysfunction affects the whole body and puts you liable to fractures, peptic ulcers, and many others, you will need to observe precise orders.

Fractures can result in critical back pain. Fractures are outlined in medical terms as permanence breaks of the bones. Cushing’s syndrome places you susceptible to fractures, which may embody greenstick, avulsions, pathologic, melancholy, indirect, spiral, compound, compressed, etc. In addition to fractures, weight problems will cause back pain. If attainable, attempt to scale back your weight. You may ask your medical doctors about exercises suited for your condition, which you can act on to reduce weight. Your physician might counsel some steps you may take to scale back weight as well.

Cushing’s syndrome can cause back pain, but numerous other illnesses could cause pain to the again as well, together with cholecystitis. Learn extra concerning the inflammatory illness to see the way it causes again pain.

 

From http://www.relievechronicbackpains.com/474

New drug targets cortisol production in Cushing’s disease

Pasireotide was effective at reducing the cortisol levels and improving associated signs and symptoms in patients with Cushing’s disease, according to data from a phase 3 trial that were presented at the 14^th Congress of the European Neuroendocrine Association.

Cushing’s disease is caused when the adrenal glands produce excess cortisol. Pasireotide (SOM230, Novartis) controls the secretion of excess cortisol, which is triggered by adrenocorticotropic hormones, which are secreted by a benign pituitary tumor. The drug currently has orphan drug status in the United States and Europe. There are no medical treatments approved for the disease, and other options, including surgery and radiotherapy, have a limited benefit.

The PASPORT-CUSHINGS trial included 162 patients who had moderate to severe persistent/recurrent or de novo Cushing’s disease and were ineligible for surgery. The patients were randomly assigned to pasireotide 600 mcg twice daily or 900 mcg twice daily. The primary endpoint was the normalization of urinary free cortisol levels, which are used to diagnose and monitor Cushing’s disease.

At 3 months, patients whose urinary free cortisol levels were higher than their baseline levels were unblinded, and they received an increased dose of pasireotide. The remaining patients continued their assigned double blind dose. After 6 months, the trial was open label.

At 6 months, the urinary free cortisol levels were normalized in 26.3% of the patients in the 900-mcg group, meeting the primary endpoint. The urinary free cortisol levels were normalized in 14.6% of the patients in the 600-mcg group, which did not meet the primary endpoint. Also at 6 months, the median urinary free cortisol in both groups declined by 47.9%. At 12 months, the median reduction in urinary free cortisol levels was 67.6% in the 600-mcg group and 62.4% in the 900-mcg group.

The reduced urinary free cortisol levels also led to an improvement in the clinical symptoms of Cushing’s disease and reduced blood pressure, cholesterol and BMI. The most common adverse effects were diarrhea, nausea and hyperglycemia. There currently are studies focusing on the optimal management of the hyperglycemia.

 

From http://www.endocrinetoday.com/view.aspx?rid=76113

40 Days of Thankfulness: Day Two

I am thankful that today is Columbus Day and that it is a holiday where I live.  So, today is a "bonus day"!

I am thankful also that Columbus discovered this land making it possible for my Dad and grandmother to come here after my grandfather was killed in Peshawa, India during World War I.

Thanks, Christopher!

 

40 Days of Thankfulness: Day One

 

I am thankful, believe it or not, that I had Cushing's.  Mind you, I wouldn't want to have it now, although diagnoses and surgeries seem "easier" now. Having Cushing's taught me a lot, including how to stick up for myself, how to read medical books to learn more about my disease, how to do web design, how to navigate NIH.  It taught me patience, how to make phone calls.  It brought me a lot of new friends.

I am also thankful that people are becoming more empowered and participating in their own diagnoses, testing and treatment.  This have changed a lot since my diagnosis in 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 thankful 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.

40 Days of Thankfulness: Day Zero

I have decided to try Facebook's 40 Days of Thankfulness and I'm going to post all 40 days here since I know that it will auto-post to Facebook and my blogs.

More info about this can be found at http://www.facebook.com/home.php?sk=group_131938326857520&ap=1#!/event.php?eid=151092964927873&index=1

Although I have had several problems in my life (and who hasn't?), I'm sure I can come up with at least 40 things I'm thankful for. So starting tomorrow, I'll be posting them here.

Pituitary Tumors Discussion with Dr Roberto Salvatori

Johns Hopkins endocrinologist Roberto Salvatori, MD, talks about pituitary tumors and treatment options.

Program Notes:

0:20    Pituitary called master gland
1:13    Hormones produced by pituitary
2:12    Referred by internist, dentist
3:04    Clinically significant tumor more common than thought
4:06    Incidentalomas
5:09    Medication and hormone use
6:13    Way of replacing most hormones
6:45    Surgical indications
7:31    Most cases access through small incisions
8:40    Sometimes surgery required later
9:20    A few genetic syndromes
10:15  Very important to see expert
11:00  Ongoing studies
11:37  End

 

Play this podcast at http://www.hopkinsmedicine.org/neurology_neurosurgery/video_gallery/podcasts/salvatori-pituitary-tumors.html

Hrayr Shahinian Discusses the Endoscopic Procedure That Revolutionizes Pituitary Surgery

Hrayr Shahinian, the Founder of the Skull Base Institute Explains the Many Benefits of the Endoscopic Procedure that Revolutionizes Pituitary Surgery.

FOR IMMEDIATE RELEASE
PRLog (Press Release) – Oct 08, 2010 – Hrayr Shahinian, Founder of the Skull Base Institute - One of the most extraordinary advances pioneered at the Skull Base Institute is the minimally invasive, fully endoscopic approach to treating pituitary tumors (pituitary adenomas) and other skull base disorders. This innovative procedure utilizes a tiny endoscope - 2.7 mm wide and 20 cm long - with angled tips that is inserted through the nostril and into the skull base. This approach offers numerous advantages in terms of the surgery and recovery period.

First, since the camera is "placed" at the tip of the endoscope, surgeons have a vivid panoramic view of the brain. They can look around corners and make a full visual assessment. This panoramic view also provides surgeons with the ability to remove the entire tumor in most cases. This process is in sharp contrast to the traditional approach, which requires viewing the tumor site through a microscope outside the skull at a focal distance that limits visibility.

Since the point of entry is through a nostril, no incision is required. Consequently, there is no scarring, no nasal packing, and the brain is undisturbed. The time required for the actual surgical procedure, the length of hospital stay and overall recovery time are dramatically reduced. Patients return home within 24-48 hours of surgery and enjoy a rapid overall recovery, and return to work and normal activities.

The Fully Endoscopic technique was simultaneously and independently pioneered at the Skull Base Institute and another major university medical center in Pittsburgh in 1996. Since then more than one thousand patients with pituitary tumors from all fifty states of our nation and international patients from countries such as Australia, Belgium, Canada, Egypt, France, Germany, Israel, Italy, Japan, Mexico, Poland, South Korea, Switzerland have sought surgical care at the Skull Base Institute.

Today, our research and development is focused on yet further innovating the field of Minimally Invasive Brain and Skull Base surgery in general and pituitary surgery in particular. High definition intraoperative digital imaging, 3D endoscopy, custom designed microinstruments, headsup displays and even nanotechnology are all being tapped in order to provide our patients with the most cutting edge and yet least invasive approaches to rid them of their tumors.

Hrayr Shahinian, M.D. is regarded as a pioneer in the field of medicine for his revolutionary use of minimally invasive endoscopy in performing skull base surgery.

# # #

Hrayr Shahinian, M.D. is regarded as a pioneer in the field of medicine for his revolutionary use of minimally invasive endoscopy in performing skull base surgery.

From http://www.prlog.org/10986580-hrayr-shahinian-discusses-the-endoscopic-procedure-that-revolutionizes-pituitary-surgery.html

Pituitary apoplexy: re-evaluation of risk factors for bleeding into pituitary adenomas and impact on outcome

Diane Laurence Möller-Goede, Michael Brändle, Klara Landau, Rene L Bernays and Christoph Schmid

D Möller-Goede, Division of Endocrinology and Diabetes, Department of Internal Medicine, Cantonal Hospital St. Gallen, St. Gallen, Switzerland
M Brändle, Division of Endocrinology and Diabetes, Department of Internal Medicine, Cantonal Hospital St. Gallen, St. Gallen, Switzerland
K Landau, Department of Ophthalmology, University Hospital Zurich, Zurich, Switzerland
R Bernays, Department of Neurosurgery, University Hospital of Zurich, Zurich, Switzerland
C Schmid, Division of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital Zurich, Zurich, Switzerland

Correspondence: Diane Möller-Goede, Email: diane.moeller@planconsulte.ch

Objective: To assess frequency, symptoms and outcome of pituitary apoplexy (PA) among pituitary adenoma patients, to gain better insight into risk factors for bleeding into pituitary adenoma, and to estimate the sequelae of PA by means of a matched control group.

Method: By reviewing charts of 574 patients with pituitary adenoma we analysed incidence, symptoms and outcome of PA, and potential risk factors for developing PA by means of a control group (patients with pituitary adenoma without PA).

Results: 42 suffered from PA; all had macroadenomas. 30/217 male (14%) and 12/179 female (7%) macroadenoma patients, 32/194 patients with clinically non-functioning (16.5%) and 10/202 with clinically active (5.0%) macroadenoma were affected. Antithrombotic therapy predisposed patients to PA (p = 0.026), diabetes mellitus and hypertension did not (p = 1.00). Patients with PA and pituitary adenoma patients without PA had similar frequencies of hypopituitarism (45 vs. 48%, p > 0.05) and visual field defects (38 vs. 55%, p > 0.05), but ophthalmoplegia was significantly more common (76 vs. 5%, p < 0.001) in patients with PA. Nearly all patients were treated by surgery; most recovered from ophthalmoplegia whereas visual function improved only moderately. Endocrine outcome was worse in patients with PA compared to patients without PA.

Conclusions: Male gender and characteristics of the adenoma itself (especially tumour size and tumour type) rather than patient's cardiovascular risk factors such as diabetes and hypertension seem to predispose to PA; antithrombotic therapy may also be important.

From http://www.eje.org/cgi/content/abstract/EJE-10-0651v1

Is metabolic syndrome a mild form of Cushing’s syndrome?

Armand Krikorian and Mehreen Khan

From the issue entitled "Special Issue on Cushing's Syndrome, Guest Editor: David C. Aron"

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Abstract

The Metabolic Syndrome is a diagnosis of increasing prevalence that is noted to share multiple clinical features with Cushing’s syndrome. Several studies suggest abnormalities in the Hypothalamic-Pituitary-Adrenal axis to be associated with this disease and tissue-specific hypercortisolemia is being investigated as a possible contributing factor. More research is needed to explore the relation between cortisol and the metabolic syndrome which, if confirmed, will have major therapeutic and public health implications.

Keywords  Metabolic syndrome - 11-beta hydroxysteroid dehydrogenase - Cushing’s

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From http://www.springerlink.com/content/m84l2376633194v5/

Addison's Disease – Will Your Insurance Cover the Price of Therapy?

Addison's disease was first described by Dr. Thomas Addison, whom the disease is called after, in 1849. Also known as adrenal insufficiency, Addison's is a malfunction of the adrenal glands which causes the glands for fail to supply sufficient of the hormones cortisol, aldosterone, or both. It's an uncommon condition that is typically brought on by harm to the gland by an autoimmune dysfunction or an infection. It is a considerably silent disease in that the signs progress so slowly that the particular person does not realize they've the disease till they expertise a very stressful scenario and the adrenal gland fails to supply sufficient hormones to help the physique deal with it.

The primary remedy of Addison's disease is through medication. Patients are prescribed hormone replacements resembling hydrocortisone or prednisone for cortisol insufficiency and a mineralocorticoid for aldosterone insufficiency. Depending on the underlying explanation for the disease, secondary remedy may be necessary. For example, autoimmune issues are the principle explanation for Addison's. Therefore, your doctor could prescribe additional medications and treatments to handle that downside as well. Cancer of the adrenal gland is also a explanation for this disease and you may need surgery and chemotherapy to do away with the cancer.

Insurance firms will probably cowl the cost of your medical care since remedy typically entails doctor's visits and medication. You will most likely be scheduling common appointments along with your doctor, so when you find yourself searching for health care insurance coverage be sure to evaluate the cost of copays for office visits. Additionally, you will want to find a plan that may pay some or the entire cost of your prescription drugs since there is no cure for Addison's disease and you will be on treatment for the rest of your life. Having your insurance coverage firm decide up the tab will save you money over the lengthy term.

Addison's disease is classed as a preexisting condition. Therefore, if you change insurance policy you may be subject to a ready period or end up paying increased premiums to your plan. To help you discover one of the best deal within the shortest amount of time, use a medical health insurance quote web site to get insurance coverage quotes from a number of providers. You will be able to do a facet by facet comparability of the completely different plans supplied which is able to make it simple to see how a lot you will be paying out of pocket to deal with your illness.

Untreated Addison's is potentially fatal but, with the correct medical care, you may reside a protracted and healthy life.

From http://www.pillowtalkmedia.com/uncategorized/addisons-disease-will-your-insurance-cover-the-price-of-therapy/

Endoscopic Transsphenoidal Pituitary Surgery: Evidence of an Operative Learning Curve

In Publish Ahead of Print on October 5, 2010 at 9:00 AM

Full article access for Neurosurgery subscribers.

Paul Leach, FRCS, Ahmed A. Abou-Zeid, MD, Tara Kearney, MD, Julian Davis, PhD, Peter Trainer, PhD, Kanna K. Gnanalingham, PhD

BACKGROUND: The use of the fiberoptic endoscope is a recent innovation in pituitary surgery.

OBJECTIVE: To investigate the evidence of an operative learning curve after the introduction of endoscopic transsphenoidal surgery in our unit.

METHODS: The first 125 patients who underwent endoscopic transnasal transsphenoidal surgery for pituitary fossa lesions between 2005 and 2007 performed by 1 surgeon were studied. Changes in a number of parameters were assessed between 2 equal 15-month time periods: period 1 (53 patients) and period 2 (72 patients).

RESULTS: There were 67 patients (54%) with nonfunctioning adenomas, 22 (18%) with acromegaly, and 10 (8%) with Cushing’s disease. Between study periods 1 and 2, there was a decrease in the mean duration of surgery for nonfunctioning adenomas (from 120 minutes to 91 minutes; P < .01). This learning effect was not apparent for functioning adenomas, the surgery for which also took longer to perform. The proportion of patients with an improvement in their preoperative visual field deficits increased over the study period (from 80% to 93%; P < .05). There were nonsignificant trends toward improved endocrine remission rates for patients with Cushing’s disease (from 50% to 83%), but operative complications, notably the rates of hypopituitarism, did not change. Overall length of hospital stay decreased between time periods 1 and 2 (from 7 to 4 days median; P < .01).

CONCLUSION: The improvements in the duration of surgery and visual outcome noted after about 50 endoscopic procedures would favor the existence of an operative learning curve for these parameters. This further highlights the benefits of subspecialization in pituitary surgery.

Full article access for Neurosurgery subscribers.

From http://neurosurgerycns.wordpress.com/2010/10/05/ahead-of-print-endoscopic-transsphenoidal-pituitary-surgery-evidence-of-an-operative-learning-curve/

Pasireotide Shows Promise for Cushing's Disease

by Megan Brooks

October 1, 2010 (Liege, Belgium) — In a phase 3 study of patients with Cushing's disease, the investigational somatostatin analogue SOM230 (pasireotide; Novartis) significantly reduced urinary free cortisol (UFC) levels in a majority of subjects and normalized UFC levels in about one fourth.

As mean UFC decreased, there was improvement in associated clinical signs and symptoms, including reductions in blood pressure, total cholesterol, and body weight, the investigators say.

The study results were presented here at the European Neuroendocrine Association 14th Congress by Beverly M. Biller, MD, an endocrinologist at Massachusetts General Hospital in Boston.

"Cushing's disease represents a high unmet medical need," Dr. Biller and colleagues write in a meeting abstract.

"A sizable number of patients with Cushing's disease are not adequately treated with surgery, and there is currently no effective medical treatment for Cushing's," William H. Ludlam, MD, PhD, who has been involved in studies of pasireotide, added in an interview with Medscape Medical News. Dr. Ludlam is director of the Seattle Pituitary Center at the Swedish Neuroscience Institute in Washington.

"While there are other somatostatin analogues available, they do not have as broad an effect as pasireotide," noted Dr. Ludlam. "There are 5 subtype receptors of somatostatin," he explained, "and most available drugs only target 1 subtype. Pasireotide targets 4 of them, and, importantly, it targets subtype 5, which is frequently expressed by the pituitary tumors common in Cushing's disease."

"Several studies on this drug have shown an effect in Cushing's, with approximately half of the patients having clinically relevant improvement," Dr. Ludlam said.

The PASPORT-CUSHINGS Study

PASPORT-CUSHINGS (Pasireotide Clinical Trial Portfolio–Cushing's Disease) is the largest randomized study to evaluate a medical therapy in patients with Cushing's disease. In the 12-month trial, 135 patients with moderate to severe persistent/recurrent Cushing's disease and 27 with de novo Cushing's disease who were not eligible for surgery were randomized in a double-blind fashion to receive either 600 or 900 μg subcutaneous injections of pasireotide. In the group as a whole, baseline UFC levels were higher in the 600- than in the 900-µg group (1156 vs 782 nmol/24 h). The primary end point was the proportion of patients who achieved normalization of UFC after 6 months without dose up-titration, relative to randomized dose.

At 6 months, 12 patients in the 600-µg group (14.6%; 95% confidence interval [CI], 7.0 - 22.3) and 21 in the 900-µg group (26.3%; 95% CI, 16.6 - 35.9) met the primary end point. After 12 months, the proportion of responders, regardless of dose up-titration, was 13.4% and 25.0%, respectively, for the 600 and 900-μg groups.

The median reduction in UFC at 6 months was 47.9% for both groups. At 12 months, median UFC was reduced by 67.6% in the 600-μg group and by 62.4% in the 900-μg group. Serum and salivary cortisol and plasma ACTH decreased with both doses, the study team notes.

They also report that patients who failed to achieve a reduction of 50% from baseline in UFC levels by month 2 were unlikely to show improvement at 6 or 12 months.

Adverse Effects Were Common But Mostly Mild

The most frequently reported adverse effects with pasireotide were diarrhea (58.0%), nausea (46.9%), hyperglycemia (38.9%), cholelithiasis (29.6%), abdominal pain (20.4%), diabetes mellitus (17.9%), fatigue (11.7%), and increased glycosylated hemoglobin (10.5%). Most adverse effects were grade 1 or 2.

"Most of the drugs used to treat Cushing's have significant side effects," Dr. Ludlam said. "Cholelithiasis is not ideal, but is not a life-threatening side effect, and hyperglycemia can easily be managed; besides, cortisol itself causes hyperglycemia. I have had a number of patients on the drug and have seen first hand that the drug is pretty well tolerated, and I have seen the benefits," Dr. Ludlam added.

Linda S. Werner, MD, a diabetes, metabolism, and endocrinology specialist with Endocrine Associates, of Bridgeport, Connecticut, who was not involved in the study, said: "If this new drug can normalize the free cortisol in one fourth and reduce the UFC in 48% [of patients], it should have a real role in treating Cushing's."

Dr. Werner made the point that "drugs are used to treat Cushing's when surgery is either not possible or doesn't fully work. Cushing's is so rare," she added, "that even tertiary care centers only have a handful of patients. I do have one patient, but he had successful surgery and so doesn't need anticortisol drugs."

According to a statement from Novartis, the data from this trial will form the basis for regulatory filing for pasireotide later this year. The drug currently has orphan drug status for Cushing's disease in the United States and Europe.

The study was funded by Novartis AG. Several of the investigators work for Novartis or have financial relationships with the company. Dr. Werner has disclosed no relevant financial relationships.

European Neuroendocrine Association (ENEA) 14th Congress: Abstract OC-1.7. Presented September 22, 2010.

From http://www.medscape.com/viewarticle/729756?src=rss