Tuesday, September 16, 2008

Endo News

September 16, 2008:
DONE! My 37-year-old daughter has Addison's disease.

From http://www.kilgorenewsherald.com/news/2008/0916/advice/009.html

DEAR DR. DONOHUE: My 37-year-old daughter has Addison's disease. Many doctors saw her when she was hospitalized a year ago. She had to go back to the hospital because of stomach upset, back pain and dehydration. Her skin has darkened. She was told she would be fine after she started steroids. This hasn't happened. She is constantly sick. Do you have any good news? -- L.K.

ANSWER: With Addison's disease, the adrenal glands have stopped producing their many hormones. Those hormones include cortisone and aldosterone. Cortisone gives us energy, combats inflammation and figures into many of the body's most important functions. Aldosterone is essential for blood pressure maintenance. Without adrenal gland hormones, the skin darkens, especially the elbow skin and the creases in the hands.

Treatment is straightforward: Replace the missing hormones. Maybe the dosage of her hormone medicines needs revision. If she's hasn't shortly turned the corner, she should get a second opinion from an endocrinologist, a specialist in this kind of illness.



Fetuin-A and its relation to metabolic syndrome and fatty liver disease in obese children before and after weight loss

Thomas Reinehr* and Christian L. Roth

Vestische Hospital for Children and Adolescents Datteln, University of Witten/Herdecke, Germany; Seattle Children's Hospital Research Institute, University of Washington, USA

* To whom correspondence should be addressed. E-mail: T.Reinehr@kinderklinik-datteln.de.

Context: There are very limited data available concerning the relationships between fetuin-A, weight status, non-alcoholic fatty liver disease (NAFLD), and features of the metabolic syndrome (MetS) in obese humans and especially in children.

Objective: To study the longitudinal relationships between fetuin-A, NAFLD, and MetS in obese children.

Design: One-year longitudinal follow-up study.

Setting: Primary care.

Patients: Thirty-six obese and 14 lean children.

Intervention: Outpatient one-year intervention program based on exercise, behavior and nutrition therapy.

Main Outcomes Measures: Changes of weight status (SDS-BMI), waist circumference, fetuin-A, blood pressure, lipids, transaminases, insulin resistance index HOMA, and prevalence of NAFLD (defined by liver ultrasound).

Results: The 12 obese children with NAFLD had significantly higher fetuin-A levels (0.35±0.07g/l) than the 24 obese children without NAFLD (0.29±0.06g/l) and the 14 normal weight children (0.29±0.05g/l). Fetuin-A levels were independent of age, pubertal stage, and gender. Fetuin-A correlated significantly to systolic (r=0.50) and diastolic blood pressure (r=0.41), insulin resistance index HOMA (r=0.28), and HDL-cholesterol (r=-0.31). Changes of fetuin-A correlated significantly to changes of insulin resistance index HOMA (r=0.34), systolic (r=0.31) and diastolic blood pressure (r=0.37), and waist circumferences (r=0.36). Substantial weight loss in 21 children led to a significant decrease of fetuin-A and the prevalence of NAFLD in contrast to the 15 children without substantial weight loss.

Conclusion: Fetuin-A levels were higher in children with NAFLD and were related to insulin resistance and to features of the MetS in both cross-sectional and longitudinal analyses. Therefore, fetuin-A might be a new promising link between obesity and its comorbidities.

Key words: fetuin-A • insulin • triglycerides • obesity • children • weight loss • insulin resistance • metabolic syndrome • blood pressure • HDL- cholesterol • non alcoholic-fatty liver disease



Insulin glargine in the treatment of type 1 and type 2 diabetes 59

Anthony H Barnett

Birmingham Heartlands Hospital, Birmingham, West Midlands, England, UK

Abstract: Insulin glargine is an analogue of human insulin that is modified to provide a consistent level of plasma insulin over a long duration. Pharmacokinetic and pharmacodynamic studies show that a single injection of insulin glargine leads to a smooth 24-hour time–action profile with no undesirable pronounced peaks of activity. In clinical trials, this profile has been associated with at least equivalent, if not better, glycemic control than other traditional basal insulins and a significantly lower rate of overall and nocturnal hypoglycemia. The convenience of a once-daily injection, a lack of need for resuspension (insulin glargine is a clear solution when injected), and lower rates of hypoglycemia should translate into improvements in patient treatment satisfaction. This review appraises the evidence for the view that insulin glargine represents an advance in basal insulin therapy for both type 1 and type 2 diabetes patients.

Keywords: Insulin glargine, basal insulin therapy, diabetes, review



September 15, 2008:
Modern techniques for pituitary radiotherapy
Reviews in Endocrine and Metabolic Disease



Earlier News Items at http://www.cushings-help.com/endo_news.htm

Past endo news

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Saturday, March 29, 2008

Endo News

Analytic Review: Pituitary Tumor Apoplexy: A Review

Pituitary tumor apoplexy is an uncommon syndrome resulting often spontaneously from hemorrhage or infarction of a pre-existing pituitary adenoma. As the primary event involves the adenoma, the syndrome should be referred to as pituitary tumor apoplexy and not as pituitary apoplexy. The sudden increase in sellar contents compresses surrounding structures and portal vessels, resulting in sudden, severe headache, visual disturbances, and impairment in pituitary function..

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Journal of Intensive Care Medicine

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Friday, March 28, 2008

Endo News

Journey to the center of the skull

After leaving the nasal cavity, you emerge at the site of the pituitary gland, deep inside the skull and roughly halfway between the ears. The pituitary gland is a common location for tumors that are usually not cancerous, but can still be dangerous. The reason: These tumors can disrupt the all-important release of hormones by the various glands controlled by the pituitary gland...

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Thursday, March 27, 2008

Endo News

Many Doctors Use Placebos on Patients

Placebos are widely used in research trials as a way to control for the influence of the placebo effect. In the case of drug trials, one study group may be given an active drug while another group gets identical treatment with only the active ingredient missing. In theory this lets researchers study only the active ingredient while canceling out the placebo effect...

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Monday, March 24, 2008

Today

Cyclical Cushing's Syndrome Due to Bronchial Carcinoid: Early Diagnosis and Prompt Treatment.

We hereby describe a patient with cyclical Cushing's syndrome due to ectopic production of adrenocorticotropic hormone (ACTH) by a bronchial carcinoid tumor, accurately diagnosed and treated within a short period of time. In addition we describe for the first time careful assessment of cortisol levels in the postoperative period without hormone replacement demonstrating limited need for steroid replacement...

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Saturday, March 22, 2008

Endo News

High prevalence of central adrenal insufficiency in patients with Prader-Willi syndrome

Strikingly, 60% of our PWS patients had central adrenal insufficiency. The high percentage of CAI in PWS patients might explain the high rate of sudden death in these patients, particularly during infection-related stress. Based on our data, one should consider treatment with hydrocortisone during acute illness in PWS patients, unless CAI has recently been ruled out with a metyrapone test...


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Journal of Clinical Endocrinology & Metabolism

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Thursday, March 20, 2008

Latest Endo News

Three years prospective investigation of anterior pituitary function after traumatic brain injury: a pilot study GH deficiency is the most common pituitary deficit 1 and 3 years after TBI (traumatic brain injury). In patients with mild and moderate TBI, pituitary function improves over time in a considerable number of patients, but it may also worsen rarely over the 3-year period. In patients with severe TBI, ACTH and GH deficiencies at 1st year evaluation persist at 3rd year...

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Tuesday, March 18, 2008

Latest Endo News

Advances in the Management of Paediatric Cushing's Disease

Cushing's disease (CD) is rare in the paediatric age range, but may present a difficult therapeutic challenge. Most paediatric endocrinologists have limited experience managing children or adolescents with CD and thus benefit from close consultation with adult colleagues. Prior to definitive treatment, a diagnostic protocol for investigation is required which broadly follows the model for adult patients. Treatment strategies for CD are described and critically appraised. The management of paediatric CD patients after cure also presents challenges for optimizing growth, bone health, reproduction and body composition from childhood into and during adult life...

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Thursday, March 13, 2008

Endo News

Pituitary Disease / Disorder Affects 1 In 5 = 20% Of The U.S. Population

"1 in 5 individuals may have an abnormal growth on their pituitary gland, causing significant health complications. If left undiagnosed and untreated, this can impair normal hormone function resulting in a reduced lifespan," explains Dr. Shereen Ezzat, Professor of Medicine, University of Toronto and member of the Board of Directors of the Pituitary Network Association...

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Tuesday, February 26, 2008

Endo News

Limitations of nocturnal salivary cortisol and urine free cortisol, in the diagnosis of mild Cushing's syndrome Neither a normal UFC nor a normal NSC excludes mild Cushing's Syndrome. Multiple samples (urine/saliva) and DST are needed to make the diagnosis of mild CS...


Update in Pituitary Disease--Cedars Sinaii Publication (UPDATE WITH FULL ARTICLE), new vistas....in unraveling the challenges of pituitary disease A recent paper, for the first time, proposed the aryl hydrocarbon receptor interacting protein (AIP) gene as predisposing to pituitary adenoma...


Stress hormone impacts memory Cortisol production is controlled by the hypothalamic-pituitary axis (HPA), a hormone-producing system involving the hypothalamus and pituitary gland in the brain and the adrenal gland located near the kidney. People with poorly controlled diabetes often have an overactive HPA axis and excessive cortisol produced by the adrenal gland...

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Friday, February 22, 2008

Endo News

Treatment 'could cut out steroids'

Extended use of steroids may result in Cushing's syndrome, a condition marked by rapid weight gain, muscle weakness and skin problems which may lead to high blood pressure, diabetes and heart disease. In some cases, it can shorten life.



Surgical Treatment of Orthopedic Trauma

Derived from Harvey Cushing's remarkable personal collection in the Brain Tumor Registry, “The Legacy of Harvey Cushing: Profiles of Patient Care,” by Aaron A. Cohen-Gadol, MD and Dennis D. Spencer, MD, is a stunning historical account of Cushing's surgical cases and research from 1905 to 1930.

The book showcases the extraordinary contribution that Cushing made to the advancement of neurological surgery in the twentieth century. The perfectly preserved material, that was housed at the Yale University Library for over 60 years, is the oldest catalog of neurological and neuropathological disease. In particular, the book features extraordinary photographs of patients which were painstakingly digitized from glass slides...

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Thursday, February 7, 2008

Life's hard times are an important part of who Linda Miller is today. (pituitary tumor)

Pine Tree instructor receives magazine props for song lyrics

By MAGGIE SOUZA

Thursday, February 07, 2008

Life's hard times are an important part of who Linda Miller is today.

It has helped mold her, but it has also had a lot to do with her recent success in a national lyric-writing contest.

Miller, the TAKS at-risk coordinator for Pine Tree Junior High, won second place in the American Songwriter's recent lyrics competition for her song called "Dear Abby." She was recognized in the magazine's January-February edition for her achievement.

Winning was a complete surprise, said Miller, 51, who found out she'd won on New Year's Day.

"I was thrilled," the Longview resident said animatedly, sitting at her desk in an office behind the junior high's library. "That was the best New Year's ever."

Miller entered the contest in the fall after learning about it through an online networking site for songwriters and musicians.

Though writing Christian contemporary music is her first passion, she submitted two of her country songs, including "Dear Abby," which she wrote more than a decade ago.

Winning a lyric-writing contest has added fuel to the fire, Miller said, adding the accolade will give her more clout as a musician.

"It kind of lets you know how you stack up to others."

Miller's love for music runs deep and is shared by her parents and siblings.

"I grew up in a musical family," said Miller, the daughter of John and Grace Grimes. Her father was a pastor, and the family lived outside the city for a while. They finally settled in Longview in time for Miller to start her sophomore year of high school as a Lobo.

"When I was young, (music) was one of our forms of recreation," said Miller, who wrote and sang a song with her two sisters in the early '90s that gained national airplay.

It wasn't until the late '80s, while battling with a devastating illness, that Miller became serious about her songwriting.

"At that time, it was a healing thing for me," said Miller, who suffered through a year of painful symptoms before doctors discovered that she had a tumor on her pituitary gland, and Cushing's disease as a result of that.

The tumor was removed via brain surgery — over a Christmas holiday so that she could miss as little school as possible.

"After that time, the songs started pouring," Miller said, adding that her spirituality grew stronger as well. "When you go through something like that, it just changes you."

Since then, writing lyrics has been one of the most enjoyable things about life.

Having won an award, Miller plans to move full-speed ahead with her musical aspirations. She's already been working with a local producer, in addition to collaborating with a team in Dallas.

What she wants most, Miller said, is to be able to share her words with the rest of the world.

"We all have these purposes here in life," she said. "Some of us, it takes longer to figure out. ... I know that I've been blessed, and I don't take it lightly."



Find this article at:
http://www.news-journal.com/news/content/news/stories/2008/02/07/02072008_PT_teacher.html

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Wednesday, February 6, 2008

Endo News

The girl, 10, who could die from shock just by watching a scary film ~ Polyglandular Addison's disease/Jenny's Pennies

Jennifer is one of just six known sufferers of polyglandular Addison's disease, which causes her to become ill whenever she is surprised or shocked.

The condition means she is unable to produce adrenaline in response to alarm or any sudden form of emotional or physical stress...

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Simplified Evaluation Tool for Physicians to Personalize Diabetes Treatment Programs

Patients wear the CGMS iPro Recorder for three days, after which physicians can review the data and use the results to uncover glucose patterns and optimize patient therapy. Based on the detailed glycemic profiles collected from the CGMS iPro Recorder, physicians can better tailor diabetes treatment programs for each patient. This may be particularly helpful for patients who experience inconsistent high and low glucose levels, who experience hypoglycemia unawareness and who generally desire better control, as well as for women with gestational diabetes and pregnant women with diabetes...

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Tuesday, January 22, 2008

Endo News

Evaluation of the Pituitary Function with Insulin Tolerance (Hypoglycaemia) Testing: Are There Any Differences Using Insulin Lispro Compared to Regular Insulin?

Background/Aim: The insulin tolerance test (ITT) remains the gold standard for evaluating the pituitary function, but has potential risks when hypoglycaemia is induced. There are scarce data using short-acting insulin analogs for ITTs. This pilot study compares the effects of insulin lispro (LPI) with regular insulin (RGI) during an ITT...




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Classical Forms of Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency in Adults

During childhood, the main aims of the medical treatment of congenital adrenal hyperplasia (CAH) secondary to 21-hydroxylase are to prevent salt loss and virilization and to attain normal stature and normal puberty.

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Both from Hormonal Research

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Wednesday, January 16, 2008

Endo News

RxTrials Institute Drug Pipeline Alert

Ipsen will provide funding for research programs in the field of proliferative diseases with a particular emphasis on novel therapeutic concepts for the treatment of pituitary adenomas, the company said...

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FDA News


The Diagnosis of Polycystic Ovary Syndrome: The Criteria Are Insufficiently Robust for Clinical Research

Polycystic ovary syndrome (PCOS) is a common disorder but has considerable phenotypic variability and this has led to controversy over its exact definition and diagnosis. The objective of this study was to review the recently proposed diagnostic criteria to determine whether they were sufficiently robust for clinical and research practise...

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MedScape

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Monday, January 14, 2008

Endo News

Hypothalamic–pituitary insufficiency following infectious diseases of the central nervous system

Hypothalamic–pituitary insufficiency may have diverse causes. The aim of this study was to determine the incidence of hypothalamic–pituitary insufficiency in patients with previous infectious diseases of the central nervous system (CNS) of different etiologies and mild-to-moderate clinical course...

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European Journal of Endocrinology

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Thursday, January 10, 2008

Pediatric Pituitary Adenomas

Pediatric Pituitary Adenomas

A total of 20 patients, including 12 females and 8 males, comprise the study group. Mean age at onset of symptoms was 14.0 years (range, 5–18 years). Four patients had onset of symptoms before the age of 12 years. The majority of patients presented with headaches (n = 12), visual disturbances (n = 12) or, in females, menstrual dysfunction (n = 9/12). Tumor size based on radiographic data was known for 19 tumors; 12 adenomas were greater than 1 cm in greatest dimension, and 7 were less than 1 cm. On follow-up, 2 patients with total gross tumor resections had recurrent adenomas; time to recurrence was 5 months and 17 months, respectively. Nine adenomas stained solely for prolactin, 5 for adrenocorticotropic hormone, and 3 for growth hormone. Two stained for growth hormone and prolactin. One did not stain with hormone antibodies...

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Cleveland Clinic

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Wednesday, January 9, 2008

“Subclinical Cushing's syndrome” is not subclinical: improvement after adrenalectomy in 9 patients

“Subclinical Cushing's syndrome” is not subclinical: improvement after adrenalectomy in 9 patients

Overall, 24 patients underwent adrenalectomy for adrenal cortisol hypersecretion, of which 9 were found to have subclinical Cushing's Syndrome. Median serum cortisol was 2.0 ?g/dL (range, 1.1–6.1) after 1-mg overnight dexamethasone suppression testing. Suspicious clinical findings on preoperative examination included skin bruising, unexplained weight gain, proximal muscle weakness, abnormal fat pads, skin thinning, fatigue, and facial plethora. During a median follow-up period of 5 months (range, 1–30 months), all 8 patients with easy bruising noted resolution postoperatively. Fatigue improved in 4 of 5 patients, muscle weakness in 6 of 8 patients, and weight in 7 of 9 patients, with a median body mass index change of –2.0 kg/m2 (range, –7.1 to +0.5 kg/m2)...

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American Association of Endocrine Surgeons

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Expression of vasopressin receptors in ACTH-independent macronodular bilateral adrenal hyperplasia causing Cushing's syndrome

Expression of vasopressin receptors in ACTH-independent macronodular bilateral adrenal hyperplasia causing Cushing's syndrome: molecular, immunohistochemical and pharmacological correlates

Cortisol secretion in ACTH-independent macronodular adrenal hyperplasia (AIMAH) causing Cushing's syndrome can be controlled by illegitimate receptors. The aim of the present study was to characterize the molecular, immunohistochemical, and pharmacological profiles of vasopressin receptors in cells derived from three patients with AIMAH (H1–H3), in order to evaluate the role of ectopic vasopressin receptors in the physiopathology of hypercortisolism.

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Journal of Endocrinology

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Tuesday, January 8, 2008

C-Reactive Protein and Cushing's

C-Reactive Protein and Cushing's

Alicia writes: "I came across this article about c-reactive protein. I was interested in it because I have elevated c-reactive protein, and that has thrown the doctors off because they think that that means I have some underlying autoimmune thing going on. Well this article is really interesting. They actually looked at the molecular shape of c-reactive protein in six disease states, Cushing's being one of them, and found that c-reactive protein actually changes "shape" (and if you know a little bit about molecular chemistry and proteins then you know that shape is very important to how proteins are able to function) in certain disease states. So, in Cushing's disease they found that two carbohydrate groups are actually missing off the protein. Now c-reactive protein is active in our bodeis in the acute inflammation process and is a necessary part of the immune system. They didn't go into this specifically, but they talked about the protein's decreased ability to bind to its targets due to these missing pieces, and I was thinking this may be a huge piece of why people with Cushing's have "weakened immune systems." They didn't really delve into those aspects specifically though as their research was really cutting edge, the first time anyone had ever looked at the molecular shape and hypothesized that it might be different in diseased states. This article was in 2003, and not in the US, so I am really hoping that there has been more research since then. And ultimately, wouldn't it be great if a "test" for Cushing's could come out of this research, just by isolating someone's CRP and looking for these very specific changes. I will put the link to the article in the post, but I am warning you it is a very dense article and even I skimmed over most of their methodology, but I would recommend reading the introduction and the conclusion, they are both moderately easy to follow along."

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Sunday, January 6, 2008

Pituitary Adenoma/Tumor

ACTH cell adenomas : clinical manifestations of the physical concentric obesity moon face, buffalo back, more blood quality, abdominal skin with large purple leg stripes, like the increase in vellus hair. Severe amenorrhea, the loss of libido, malaise, or even bedridden. Some patients with hypertension, diabetes and so on...

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Doctors120

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Friday, January 4, 2008

FDA Accepts Samaritan's Cushing's SP-6300 IND and Clears Phase II Study

FDA Accepts Samaritan's Cushing's SP-6300 IND and Clears Phase II Study

The U.S. Food and Drug Administration (FDA) has completed its regulatory review of Samaritan's IND (Investigational New Drug) application for Cushing's syndrome SP-6300 and declared it has not identified any deficiencies in its IND filing. Accordingly, Samaritan can proceed with its proposed Phase II clinical study of Cushing's syndrome SP-6300's efficacy in patients experiencing Hypercortisolism...

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Wednesday, January 2, 2008

Laparoscopic surgery is safe for large adrenal lesions

Laparoscopic surgery is safe for large adrenal lesions

The purpose of this paper was to assess the safety of laparoscopic adrenalectomy in large adrenal tumours. Because of its many advantages over conventional open surgery, laparoscopic adrenalectomy has become the preferred approach for removal of benign adrenal tumours...

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European Journal of Surgical Oncology (EJSO)

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Tuesday, January 1, 2008

Health Alert: Adrenal Crisis Causes Death in Some People Who Were Treated With hGH

Health Alert: Adrenal Crisis Causes Death in Some People Who Were Treated With hGH

Treatment with hGH does not cause adrenal crisis, but because a number of people lacking growth hormone also lack ACTH, adrenal crisis has occurred in some people who were treated with hGH. In earlier updates we have talked about how adrenal crisis can be prevented, but people continue to die from adrenal crisis, which is brought on by lack of cortisol. These deaths can be prevented. Please talk to your doctor about whether you are at risk for adrenal crisis...

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From: National Endocrine and Metabolic Diseases Information Service

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Tuesday, December 18, 2007

Samaritan Pharmaceuticals Begins Trading On OTC Bulletin Board Under Ticker Symbol SPHC

Samaritan Pharmaceuticals Begins Trading On OTC Bulletin Board Under Ticker Symbol SPHC

Samaritan has partnered its Phase II/III oral HIV viral-entry inhibitor drug. Its promising memory-restoring Alzheimer's drug Caprospinol expects to enter a Phase I clinical trial after acute toxicity study and its High Stress Cortisol drug to counteract Cushing's disease expects to enter Phase II clinical trials...

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Monday, December 17, 2007

A ‘smart’ type of Cushing's syndrome

A ‘smart’ type of Cushing's syndrome

Cushing's syndrome results from lengthy and inappropriate exposure to excessive concentrations of either endogenous or exogenous glucocorticoids. This case report describes a patient with a novel type of Cushing's syndrome due to the use of party drugs. A 35-year-old woman had gained 8 kg body weight in 5 months and complained of anxiety. She showed a Cushing-like appearance and mild hypertension (blood pressure, BP 150/95 mmHg). She reported daily use of increasing doses of {gamma}-hydroxybutyric acid (GHB), a popular party drug. ACTH plasma levels were in the upper normal range (41 ng/l), with normal plasma cortisol (0.36 µmol/l). She showed an abnormal overnight 1 mg dexamethasone suppression test (cortisol 0.38 µmol/l). The urinary excretion of free cortisol in 24 h was also increased (0.47 µmol/24 h). CT scanning of the abdomen showed normal adrenals. After stopping GHB intake she lost 7 kg body weight and her BP normalized (BP 135/80 mmHg). GHB is a popular party drug in the Netherlands, but it is also used as a narcotic and for the treatment of narcolepsy. We hypothesize that GHB may bind to the pituitary gland {gamma}-aminobutyric acid-B receptors leading to ACTH overproduction.

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European Journal of Endocrinology

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False-positive inferior petrosal sinus sampling in the diagnosis of Cushing's disease

False-positive inferior petrosal sinus sampling in the diagnosis of Cushing's disease

Inferior petrosal sinus sampling (IPSS) for adrenocorticotropic hormone (ACTH) levels in patients with Cushing's syndrome has become a useful method to distinguish ACTH-secreting pituitary tumors (Cushing's disease) from other causes of the syndrome, principally ectopic adrenocorticotropin secretion by an occult tumor.

Although the test is generally regarded as highly specific, the authors recently encountered two patients whose IPSS measurements were falsepositive for Cushing's disease. The results of IPSS suggested a pituitary origin of ACTH secretion in both patients, but transsphenoidal surgery failed to disclose a pituitary adenoma or to improve postoperative plasma cortisol levels.

Both patients subsequently were found to have an ACTH-secreting carcinoid tumor of the lung. The false-positive IPSS studies were due to periodic hormonogenesis. The patients must be hypercortisolemic at the time IPSS is performed for the study to be valid.

KEY WORDS • Cushing's syndrome • Cushing's disease • inferior petrosal sinus sampling • carcinoid tumor

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Tuesday, December 11, 2007

Food-dependent Cushing's syndrome: from molecular characterization to therapeutical results

Food-dependent Cushing's syndrome: from molecular characterization to therapeutical results N M Albiger, G Occhi, B Mariniello, M Iacobone1, G Favia1, A Fassina2, D Faggian3, F Mantero and C Scaroni

Department of Medical and Surgical Sciences Endocrinology Unit, University of Padua, via Ospedale 105, 35128 Padua, Italy, Departments of 1 Endocrine Surgery, 2 Pathology and 3 Laboratory Medicine,, University of Padua, 1-3: Giustiniani, 35128. Padua, Italy

(Correspondence should be addressed to N M Albiger; Email: nalbiger@yahoo.com)

Abstract


Objective: Cortisol secretion in ACTH-independent macronodular adrenal hyperplasia (AIMAH) may be regulated by the aberrant expression of several G-protein-coupled receptors. Bilateral adrenalectomy is the treatment of choice in most cases. We searched for aberrant receptor expression in a patient with AIMAH and evaluated the response to medical and surgical treatment.

Patient: A 35-year-old woman with amenorrhea, hirsutism, and hypertension presented ACTH-independent cortisol secretion with high androgen levels. Abdominal computed tomography showed bilateral adrenal macronodules (4.5 cm right and 1.0 cm left). Scintigraphy with I131-norcholesterol showed bilateral uptake, prevalent on the right side. Several in vivo stimulation tests were assessed before and after treatment and in vitro studies were performed after unilateral adrenalectomy.

Results: Plasma cortisol increased after a standard meal test (60%) and oral glucose loading (147%), and the response was blunted by pretreatment with 100 µg s.c. octreotide. The therapy with long-acting release octreotide (octreotide-LAR) showed an improvement in urinary free cortisol (UFC) levels. Unilateral adrenalectomy was performed and histopathology revealed macronodular AIMAH. Cortisol and androgens increased after perifusion of tumoral tissue with glucose-dependent insulinotropic polypeptide (GIP), and GIP and LH-receptor overexpression was found in both the adrenal nodules and the adjacent cortex. After surgery, UFC and androgen levels normalized followed by clinical improvement.

Conclusions: GIP and LH-receptor expression may coexist in AIMAH, influencing the functional and morphological phenotype. Aberrant hormone receptor expression enables specific pharmacological treatment, but long-term studies are needed to evaluate its real efficacy. Unilateral adrenalectomy may be a safe initial option, particularly for asymmetric bilateral adrenal enlargements.

From: European Journal of Endocrinology

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Monday, December 10, 2007

Patients With Mild Cushing Syndrome May Benefit From Adrenalectomy

Patients With Mild Cushing Syndrome May Benefit From Adrenalectomy

Researchers at UT Southwestern Medical Center have found that patients with a mild form of Cushing syndrome, a metabolic disorder caused by adrenal tumors, demonstrate substantial clinical improvement after adrenalectomy.

The study, appearing in the December issue of the journal Surgery, is the largest series of surgical outcomes reported in patients with subclinical Cushing syndrome to date, said Dr. Richard Auchus, associate professor of internal medicine at UT Southwestern and co-author of the study.

"We don't have enough data to come out with a definitive statement that everyone with an adrenal tumor and mild cortisol excess should have the adrenal tumor removed," Dr. Auchus said. "We can say, however, that there are many people with large adrenal tumors who, while not meeting classical criteria for Cushing syndrome, nonetheless suffer from the hormonal disorder and will benefit from surgery."

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Friday, November 30, 2007

Adrenal Insufficiency in Critical Illness

Adrenal Insufficiency in Critical Illness

One of the more controversial areas in critical care in recent decades relates to the issue of adrenal insufficiency and its treatment in critically ill patients. There is no consensus on which patients to test for adrenal insufficiency, which tests to use and how to interpret them, whether to use corticosteroids, and, if so, who to treat and with what dose...

From Journal of Intensive Care Medicine

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Thursday, November 29, 2007

Patient's Symptoms Of Cushing's Disease Beginning To Abate After Endoscopic Pituitary Cure

Patient's Symptoms Of Cushing's Disease Beginning To Abate After Endoscopic Pituitary Cure

BrendaW wrotes: "This is an excellent article that not only describes this type of surgery (endoscopic) and its seemingly effortless recovery, but offers fairly clear explanations of several areas of concern for Cushing's patients. For example, the difference between Cushing's Disease and Cushing's Syndrome, why the levels of Cortisol in our bodies can mean a rise or fall in aches and pains, and a brief description/recommendation of LA's prestigious Cedars-Sinai Medical Center..."

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from medicalnewstoday.com

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Wednesday, November 28, 2007

Cushing's syndrome in women with polycystic ovaries and hyperandrogenism

Cushing's syndrome in women with polycystic ovaries and hyperandrogenism

A 41-year-old woman presented at an endocrinology–gynecology clinic. Seven years previously she had tbeen investigated at another hospital for primary infertility of 5 years standing, associated with secondary amenorrhea for the preceding 6 months, oily skin, acne and facial hirsutism. At the time, laparoscopy and dye infusion had demonstrated multiple, small ovarian cysts with patent oviducts. A diagnosis of polycystic ovarian syndrome (PCOS) was made. Attempts at ovulation induction were then undertaken with 50 mg clomifene citrate on days 2–6 of the menstrual cycle, following which she conceived aged 35 years and carried a successful pregnancy to term, giving birth to a healthy baby girl....


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Nature Clinical Practice Endocrinology & Metabolism (2007)

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No Scalpel In Hand, (UVa) Surgeons Treat Gamma Knife Patient

No Scalpel In Hand, (UVa) Surgeons Treat Gamma Knife Patient

Gingras, who resides in Johnson City, TN, suffers from a pituitary tumor that has caused him to develop Cushing's disease, a rare disorder that over-stimulates hormone production in the adrenal gland. When surgery and conventional radiation therapy failed to eradicate his tumor, doctors recommended Gamma Knife treatment....


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University of Virginia Health System

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Friday, November 23, 2007

Endo News

Samuel L. Leonard, Hormone Researcher Dr. Leonard was still a graduate student when he began his studies of sex hormones, produced at the base of the brain in the pituitary gland...

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Wednesday, October 31, 2007

Articles from The Journal of Neurosurgery, September 2007 Volume 23, Number 3

Articles from The Journal of Neurosurgery, September 2007 Volume 23, Number 3

  • Brain atrophy and cognitive deficits in Cushing's disease by Chirag G. Patil, M.D., Shivanand P. Lad, M.D., Ph.D., Laurence Katznelson, M.D., and Edward R. Laws Jr., M.D.

  • Current state of the art in the diagnosis and surgical treatment of Cushing disease: early experience with a purely endoscopic endonasal technique by Amir R. Dehdashti, M.D. and Fred Gentili, M.D., F.R.C.S.C.

  • Cushing disease: use of perioperative serum cortisol measurements in early determination of success following pituitary surgery by Armand Krikorian, M.D., Dima Abdelmannan, M.D., Warren R Selman, M.D., and Baha M Arafah, M.D.

  • Cyclic Cushing syndrome: definitions and treatment implications by Dennis A. Velez, M.D., Marc R. Mayberg, M.D., and William H. Ludlam, M.D., Ph.D.

  • Diagnosis and Management of Cushing Disease by William T. Couldwell, M.D., Ph.D.

  • Diagnostic approach to Cushing disease by Bradley A. Gross, B.S., Stefan A. Mindea, M.D., Anthony J. Pick, M.D., James P. Chandler, M.D., and H. Hunt Batjer, M.D.

  • Evaluation and management of Cushing syndrome in cases of negative sellar magnetic resonance imaging by Jay Jagannathan, M.D., Jason P. Sheehan, M.D., Ph.D., and John A. Jane Jr., M.D.

  • Medical management of Cushing disease by Bradley A. Gross, B.S., Stefan A. Mindea, M.D., Anthony J. Pick, M.D., James P. Chandler, M.D., and H. Hunt Batjer, M.D.

  • National trends, complications, and outcomes following transsphenoidal surgery for Cushing's disease from 1993 to 2002 by Chirag G. Patil, M.D., Shivanand P. Lad, M.D., Ph.D., Griffith R. Harsh, M.D., Edward R. Laws Jr., M.D., and Maxwell Boakye, M.D.

  • Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management by Magdalena J. Banasiak, M.D. and Ali R. Malek, M.D.

  • Nelson syndrome: historical perspectives and current concepts by Mark Hornyak, M.D., Martin H. Weiss, M.D., Don H. Nelson, M.D., and William T. Couldwell, M.D., Ph.D.

  • The role of inferior petrosal sinus sampling in the diagnostic localization of Cushing's disease

  • Transsphenoidal surgery for Cushing's disease: a review of success rates, remission predictors, management of failed surgery, and Nelson's Syndrome by Daniel F. Kelly, M.D.

  • Treatment options for Cushing disease after unsuccessful transsphenoidal surgery by James K. Liu, M.D., Maria Fleseriu, M.D., Johnny B. Delashaw Jr., M.D., Ivan S. Ciric, M.D., William T. Couldwell, M.D., and Ph.D.



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Friday, October 26, 2007

Genetics of Adrenal Tumors Associated With Cushing's Syndrome: A New Classification for Bilateral Adrenocortical Hyperplasias

Genetics of Adrenal Tumors Associated With Cushing's Syndrome: A New Classification for Bilateral Adrenocortical Hyperplasias


Adrenocortical causes of Cushing's syndrome include isolated and sporadic adenoma causes as well as rare carcinoma causes and bilateral hyperplasias. The classification of various forms of adrenal hyperplasias can be based on histologic, biochemical, and genetic features...

Medscape

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Thursday, October 11, 2007

Pulling Out Tumors Through Your Nose

Pulling Out Tumors Through Your Nose

Brain surgery involves actually cracking open skulls, which often leads to post-op complications and a lengthy recovery. But Amin Kassam, chairman of the department of neurological surgery at the University of Pittsburgh Medical Center and director of its Minimally Invasive Endoneurosurgery Center, has found a way to leave your melon intact: He performs brain surgery through the nose...

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Medi-Mation / Popular Science


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Does Stress Cause Disease? It Doesn't Help, Reviewers Say

Does Stress Cause Disease? It Doesn't Help, Reviewers Say

Cortisol, the primary effector of HPA activation in humans, regulates a broad range of physiological processes, including anti-inflammatory responses; metabolism of carbohydrates, fats, and proteins; and gluconeogenesis...


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MedPage Today

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Wednesday, October 10, 2007

PDE11A as a Novel Therapeutic Target for Inherited Form of Cushing Syndrome and Endocrine Tumors

PDE11A as a Novel Therapeutic Target for Inherited Form of Cushing Syndrome and Endocrine Tumors

Cushing Syndrome, a disorder associated with excess production of a steroid hormone, cortisol, affects up to 10 per 15 million people every year. Cushing Syndrome may be caused by several reasons such as cortisol-producing endocrine tumors and can be inherited in some instances. Surgery of the adrenal tumor is the most common method of treatment. New diagnostic and therapeutic approaches need to be developed for successful management of the disease...

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From NIH

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Tuesday, October 9, 2007

Cyclical Cushing's syndrome: an update

Cyclical Cushing's syndrome: an update

Summary: Cyclical Cushing's syndrome is a pattern of hypercortisolism in which the biochemistry of cortisol production fluctuates rhythmically. This syndrome is often associated with fluctuating symptoms and signs. This type of case was initially thought to be rare. It has, however, recently been recognized as occurring much more frequently. The phenomenon is important because it can, if not recognized, lead to errors in diagnosis and differential diagnosis of the syndrome and in assessment of therapeutic outcomes. All of these can have very serious clinical consequences. Clinical researchers, including ourselves, have developed criteria, protocols and dynamic biochemical tools to detect cycling in patients with hypercortisolism. Unfortunately, the mechanisms causing the abnormal pathophysiology have not been well elucidated but some recent insights have been gained. The review discusses strateges for diagnosing and managing this important subgroup of patients with hypercortisolism...


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Current Opinion in Endocrinology, Diabetes and Obesity

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Wednesday, October 3, 2007

Alopecia or Why People Go Bald

Alopecia or Why People Go Bald

Among women the cause of hair loss can be an hyper-sensitivity to male hormones or their increased amount in the body because of the following diseases: polycystic ovaries, congenital dysfunction of cortex of adrenal glands, Cushing syndrome, tumours of adrenal glands and ovaries...

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EditorsChoice

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Pheochromocytoma and Extraadrenal Abdominal Paraganglioma

Pheochromocytoma and Extraadrenal Abdominal Paraganglioma

Conclusion: Carbidopa enhances the sensitivity of 18F-DOPA PET for adrenal pheochromocytomas and extraadrenal abdominal paragangliomas by increasing the tumor-to-background ratio of tracer uptake. The sensitivity of 18F-DOPA PET for metastases of paraganglioma appears to be limited....

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Journal of Nuclear Medicine

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Monday, October 1, 2007

News Items

Growth hormone replacement throughout life: Insights into age-related responses to treatment

The adult growth hormone deficiency (GHD) syndrome is a well-defined clinical entity. Although the symptoms of GHD are not age specific, their relative importance differs depending on the patient’s age, and the impact of GHD varies throughout adult life...

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Growth Hormone & IGF Research




Woman without pituitary gland, gives birth to healthy baby

A 25-year-old woman whose pituitary gland had been removed through surgery following detection of a tumour, has given birth to a girl child after conceiving through intra-uterine insemination at a city hospital.

Describing it as a "rarest of the rare case", Dr V Sunitha of Vikram Hospital said today that a patient who had undergone pituitary tumour surgery, conceiving and having a normal gestation and delivering a healthy baby was a rarity...

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ZeeNews

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Sunday, September 30, 2007

Pituitary Tumors in Children

Pituitary Tumors in Children

In rare cases, children may develop tumors on the pituitary gland. Such tumors are usually benign (non-cancerous) and do not spread to other parts of the body.

But, because the pituitary gland is very important in helping to regulate the hormones of the body, even a small disruption can have serious effects on mood, on the ability to focus and concentrate, as well as on growth and overall maturation...

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Services of Health

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Saturday, September 29, 2007

he efficacy of fractionated radiotherapy and stereotactic radiosurgery for pituitary adenomas

The efficacy of fractionated radiotherapy and stereotactic radiosurgery for pituitary adenomas: long-term results of 125 consecutive patients treated in a single institution

BACKGROUND: The objective of this retrospective cohort study was to define the efficacy and safety of fractionated radiotherapy (FRT) and stereotactic radiosurgery (SRS) for the treatment of patients with pituitary adenoma..


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Scottsdale Healthcare, Scottsdale, AZ

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The Adrenal Gland

The Adrenal Glands

In some cases, an adrenal tumor can cause excess production of hormones. Patients may experience severe headaches, anxiety, palpitations, sweating, and a rapid heart rate. Overproduction of cortisol can lead to the development of Cushing's syndrome, a condition characterized by obesity, high blood pressure, high glucose levels, menstrual problems, fragile skin and stretch marks. Excess production of aldosterone can cause high blood pressure and low potassium levels...

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From WSOCTV
September 28, 2007

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Friday, September 28, 2007

Bilateral Laparoscopic Adrenalectomy For Corticotropin-Dependent Cushing's Syndrome: A Review of the Mayo Clinic Experience

Bilateral Laparoscopic Adrenalectomy For Corticotropin-Dependent Cushing's Syndrome: A Review of the Mayo Clinic Experience

There has been a rapid shift from open to laparoscopic approaches in adrenal surgery, but the safety and efficacy of bilateral laparoscopic adrenalectomy (BLA) in patients with corticotropin (ACTH)-dependent Cushing's syndrome continues to be defined...


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From the Mayo Clinic
September 27, 2007

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Thursday, September 27, 2007

Travel to India For Adrenal Surgery

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Thursday, September 20, 2007

Steroid withdrawal syndrome after successful treatment of Cushing's syndrome: a reminder

Steroid withdrawal syndrome after successful treatment of Cushing's syndrome: a reminder

Steroid withdrawal syndrome (SWS) usually refers to relapse of the disease being treated after withdrawal of glucocorticoid therapy, or the symptoms of adrenal insufficiency which occur when glucocorticoids are rapidly reduced or stopped. A less well-recognised form of SWS is that which develops when patients experience a symptom complex similar to that of adrenal insufficiency despite acceptable cortisol levels. We describe three patients who presented with this form of SWS following surgical treatment for endogenous Cushing's syndrome...

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From Department of Endocrinology, Manchester Royal Infirmary

September 20, 2007

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Tuesday, September 18, 2007

Pituitary macroadenomas: are combination antiplatelet and anticoagulant therapy contraindicated? A case report

From Pituitary macroadenomas: are combination antiplatelet and anticoagulant therapy contraindicated? A case report

A 57-year-old female with a known pituitary macroadenoma was treated for suspected acute coronary syndrome with aspirin, clopidogrel and full dose enoxaparin. She developed a severe and sudden headache, nausea and vomiting and visual deterioration. A CT scan showed haemorrhage into the pituitary macroadenoma. She underwent neurosurgical decompression. Post-operatively her visual fields and acuity returned to baseline. She was continued on hydrocortisone and thyroxine replacement on discharge.....

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Journal of Medical Case Reports
September 18, 2007

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Friday, September 14, 2007

Pituitary Tumors/Cushing's Disease

Pituitiary [sic] Tumor

Cushings Disease: This syndrome is caused by tumors on the ACTH (Adrenal Corticotrophin Hormone) secreting cells of the pituitary gland. Patients with this problem develop fat deposits in strange places (Moon face, Buffalo hump on the back of the neck), spontaneous scarring of the skin along the belly that look striated, pimples in adults, high blood pressure and elevated body temperature. These tumors are usually so small that the surgeon might have a difficult time finding the little "bad pearl" in the gland during surgery. This is the one time when small can be bad, especially if the surgeon is unable to locate and remove the tumor! ACTH secreting tumors, although small and troublesome, are readily cured by surgery alone....


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From Brain Surgery Journal, September 9, 2007

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Tuesday, September 4, 2007

Cushing's Disease, Cushing's Syndrome, Endocrine Disorders, Pituitary Tumor

As Robin posted on digg. Please vote for this site!

Cushing's Disease/Syndrome is one of several endocrine disorders hard to diagnose but devastating to the persons who have them.

This site leads to helpful and informative news, research, and doctors for anyone dealing with endocrine disorders or who suspects an endocrine disorder.

There is also a wonderful support board filled with similar people.

digg story

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Monday, September 3, 2007

Upcoming Media

Upcoming Media

Kate (Fairley on the boards) and her doctor Dr. Ted Friedman, will be featured on the National Geographic channel,

Tuesday, September 18, 2007, at 9PM;
Wednesday, September 19, 12AM and
Sunday, September 23, 4PM.

The show, called the Science of Obesity, will focus on Cushing's syndrome.

Today, 127 million adult Americans are considered overweight, but few reach the extreme proportions of the exceptionally obese. What are the physical stresses of weighing more than 500 pounds and what steps can reverse it? NGC provides an understanding of what happens inside the bodies of these massive people & why a person can pack on hundreds more pounds than those with typical weight challenges. The show explores the genetics behind weight gain & medical advances available to help prevent it.

More info here

Discuss this TV show.

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