Endocrine Society issues new guidelines on hypopituitarism

The Endocrine Society today issued a Clinical Practice Guideline that recommends treating insufficient hormone levels in individuals with hypopituitarism by replacing hormones at levels as close to the body’s natural patterns as possible.

The guideline, titled “Hormonal Replacement in Hypopituitarism in Adults: An Endocrine Society Clinical Practice Guideline,” was published online and will appear in the November 2016 print issue of The Journal of Clinical Endocrinology & Metabolism (JCEM), a publication of the Endocrine Society.

Hypopituitarism, or pituitary insufficiency, occurs when the pituitary gland does not produce sufficient amounts of hormones–the chemical signals that regulate respiration, reproduction, growth, metabolism, sexual function and other important biological functions. The pituitary gland is often called the master gland because the hormones it produces impact many bodily functions. As a result, hypopituitarism can cause a range of symptoms, according to the Hormone Health Network.

The rare disorder can occur due to abnormal development or later in life as a result of a tumor, traumatic brain injury, hemorrhage or autoimmune condition, according to the Society’s

“Hypopituitarism can manifest as low levels of a variety of hormones, including cortisol, thyroid hormone, estrogen, testosterone and growth hormone,” said Maria Fleseriu, MD, FACE, of Oregon Health & Science University in Portland, OR. Fleseriu chaired the task force that developed the guideline. “The goal of treatment should be to restore hormone levels as close to healthy levels as possible The interactions between these hormones also are very important, and patients might require dose changes of one or more of the replacement hormones after starting or discontinuing another one.”

In recommending treatment options, the guideline task force followed the overriding principle of using hormone replacement therapy dose size and timing to mimic the body’s natural functioning as closely as possible.

Accurate and reliable measurements of hormones play a central role in diagnosing hypopituitarism and monitoring the effectiveness of treatments, Fleseriu said. Healthcare providers need to keep in mind technical considerations to ensure the testing procedure is as accurate as possible.

The guideline addresses special circumstances that may affect the treatment of patients with hypopituitarism, including pregnancy care, post-surgical care following pituitary or other operations, treatment in combination with anti-epilepsy medication, and care following pituitary apoplexy–a serious condition that occurs when there is bleeding into the gland or blood flow to it is blocked.

Recommendations from the guideline include:

  • Measurements of both free thyroxine and thyroid-stimulating hormone are needed to evaluate central hypothyroidism, a condition where the thyroid gland does not produce enough hormones because it isn’t stimulated by the pituitary gland.
  • People who have central hypothyroidism should be treated with levothyroxine in doses sufficient to raise levels of the thyroid hormone free thyroxine to the upper half of the reference range.
  • Growth hormone stimulation testing should be used to diagnose patients with suspected growth hormone deficiency.
  • People who have proven cases of growth hormone deficiency and no contraindications should be offered growth hormone replacement as a treatment option.
  • Premenopausal women who have central hypogonadism, a condition where the sex glands produce minimal amounts or no hormones, can undergo hormone treatment, provided there are no contraindications.
  • People producing abnormally large volumes of dilute urine should be tested for central diabetes insipidus–a rare condition that leads to frequent urination–by analyzing the concentration of their blood and urine.
  • For patients who have low levels of glucocorticoid hormones, hydrocortisone can be given in a daily single or divided dose.
  • All hypopituitarism patients should be instructed to obtain an emergency card, bracelet or necklace warning about the possibility of adrenal insufficiency.
  • Patients who are suspected of having an adrenal crisis due to secondary adrenal insufficiency should receive an immediate injection of 50 to 100 milligrams of hydrocortisone.
  • People who have central adrenal insufficiency should receive the lowest tolerable dose of hydrocortisone replacement on a long-term basis to reduce the risk of metabolic and cardiovascular disease.
Source:

The Endocrine Society

From http://www.news-medical.net/news/20161013/Endocrine-Society-issues-new-guidelines-on-hypopituitarism.aspx

A Team Effort to Treat a Pea-Sized Gland

HYANNIS – Endoscopic surgery for pituitary tumors involves the use of small instruments, but neurosurgeon Nicholas Coppa, MD, FAANS, is quick to say it takes a big team to make the surgeries a success.

“It’s very much a collaborative effort among endocrinology, neurosurgery and otolaryngology specialties,” he said.

Dr. Coppa frequently works with endocrinologist Catalina Norman, MD, PhD, and ear, nose and throat surgeon Ross Johnston, MD.

The pituitary gland sits at the base of the brain. It makes important hormones that control several different systems in the body and help maintain normal body function.

“The overwhelming majority of patients with big tumors present with visual problems,” said Dr. Coppa. “They get tunnel vision from a tumor putting pressure on the vision nerves.

Many patients’ pituitary problems are detected incidentally while the physician is trying to diagnose a set of symptoms, most commonly headaches, he added. A variety of asymptomatic tumors are detected this way.

A subset of pituitary tumors secrete excess hormones, which create syndromes characterized by whatever hormone is being secreted in excess, Dr. Coppa added. Oftentimes these problems are diagnosed by an endocrinologist.

Before coming to Neurosurgeons of Cape Cod – now known as Cape Cod Healthcare Neurosurgery – in 2013, Dr. Coppa was professor of skull base surgery at Oregon Health and Science University’s Northwest Pituitary Center. He has performed more than 200 endoscopic surgeries for pituitary tumors, sinonasal malignancies and anterior skull base encephaloceles. The procedure is fairly new on Cape Cod, he said.

The pituitary gland is about the size of a pea, so operating on it is a tricky and delicate procedure.

The surgeon commonly works with an endoscope inserted through one nostril, and microsurgical instruments through the other nostril. This allows him to maneuver to the surgical area.

According to the Northwest Pituitary Center’s web site, “The tube is connected to a TV monitor that helps your doctor see the surgical area even more clearly than with a microscope. Your doctor can also use intraoperative neuro-navigation to perform image-guided surgery, based on a pre-operative CT scan or MRI. This helps the doctor see exactly where the tumor is and avoid damaging healthy brain tissue that is nearby.”

Nasal endoscopy for the neurosurgeon has really taken off in the last 10 years, according to Dr. Coppa. The main reason for the increase is because the technique allows better visualization of the anatomy, he said.

“We find that it allows, at least in my experience, more maneuverability of your micro-surgical instruments. That’s been very satisfying for patients. The nasal morbidity [adverse effects] is lower compared to historic ways of doing it.”

Ear, nose and throat doctors use trans-nasal surgery to treat many sinus conditions, said Dr. Coppa. But the procedure is predominantly used by neurosurgeons for pituitary tumors, other tumors of the skull base and malignancies of the sinus cavity that often invade the brain.

After endoscopic pituitary surgery, patients are typically in the hospital for several days and resume day-to-day activities within that first week.

By BILL O’NEILL, OneCape Health News

 

From http://www.capecod.com/newscenter/a-team-effort-to-treat-a-pea-sized-gland/

Polycystic ovarian syndrome and Cushing’s syndrome: A persistent diagnostic quandary

European Journal of Obstetrics & Gynecology and Reproductive Biology, 02/10/2014  Clinical Article

Brzana J, et al. – This study aims to retrospectively review institutional records of female patients of reproductive age with Cushing’s disease (CD) and determine if and how many had been previously diagnosed as having solely PCOS. To determine whether clinical patterns might be useful in identifying appropriate candidates for hypercortisolism screening in women suspected of PCOS. Prolonged exposure to hypercortisolism has been linked with increased mortality and morbidity. Tests for hypercortisolism in all the PCOS cases authors report led to an appropriate CD diagnosis. Future research should focus on when and which (if not all) women with suspected PCOS should be tested for hypercortisolism.

Methods

  • The study included 50 patients with pathologically proven CD at Oregon Health & Science University, Northwest Pituitary Center between 2006 and 2011.
  • Physical, clinical, and biochemical features for hypercortisolism were compared.

Results

  • Of 50 patients with pathologically proven CD, 26 were women of reproductive age.
  • Of these, half had previously been diagnosed with and treated initially solely for PCOS.
  • Hirsutism and menstrual abnormalities were more common in the group with an initial PCOS diagnosis than in the group with an initial CD diagnosis.

From http://www.mdlinx.com/endocrinology/newsl-article.cfm/5055779/ZZ4747461521296427210947/?news_id=2364&newsdt=021014&subspec_id=1509&utm_source=Focus-On&utm_medium=newsletter&utm_content=Top-New-Article&utm_campaign=article-section