Adrenal Insufficiency: Primary and Secondary

Posted on November 20, 2016 by MaryO

By Dr Tomislav Meštrović, MD, PhD

Adrenal insufficiency is a condition that develops when most of the adrenal gland is not functioning normally. Primary adrenal insufficiency arises due to the damage of the glands or because of using drugs that halt synthesis of cortisol. On the other hand, secondary adrenal insufficiency stems from processes that inhibit the secretion of the adrenocorticotropic hormone (ACTH) by the hypophysis as a result of a hypothalamic or pituitary pathology. The former is sometimes also referred to as tertiary adrenal insufficiency.

Adrenal insufficiency is still a significant challenge for both patients and their physicians, but also scientists and researchers. In the past decade, long-term studies with adequate follow-up have shown a surge in mortality and morbidity, as well as impaired quality of life in individuals with this condition.

Primary Adrenal Insufficiency

In developed countries, the most common cause of primary adrenal insufficiency is autoimmune adrenalitis, whereas in the developing world tuberculosis is still considered a primary causative factor. Moreover, in young males, an X-linked adrenoleukodystrophy (also known as the less severe form of adrenomyeloneuropathy) must also be considered.

Histopathologically, in autoimmune primary adrenal insufficiency, there is a diffuse mononuclear cell infiltrate that can gradually progress to atrophy. Primary adrenal insufficiency is linked to both cortisol and mineralocorticoid deficiency.

Recent research drew attention to drug-related and infectious causes of adrenal insufficiency. Antifungal agents are known to substantially reduce cortisol synthesis, while imunosuppression associated with human immunodeficiency virus (HIV) has resulted in a resurgence of infectious causes, most notably tuberculous and CMV adrenalitis.

Secondary Adrenal Insufficiency

Secondary adrenal insufficiency has three principal causes: adrenal suppression after exogenous glucocorticoid or ACTH administration, abnormalities of the hypothalamus or pituitary gland that lead to ACTH deficiency, as well as adrenal suppression upon the correction of endogenous glucocorticoid hypersecretion.

Any lesion of the hypophysis or hypothalamus can result in secondary adrenal insufficiency; some of the examples are space-occupying lesions such as adenomas, craniopharyngiomas, sarcoidosis, fungal infections, trauma, and also metastases from distant malignant processes.

The histologic appearance of the adrenal glands in secondary adrenal insufficiency can range from normal to complete atrophy of the cortex (with preserved medulla). In contrast to primary adrenal insufficiency, secondary types are associated with the lack of cortisol, but not mineralocorticoid deficiency.

Clinical Features of Adrenal Insufficiency

The clinical presentation of adrenal insufficiency is related to the rate of onset and severity of adrenal deficiency. In a large number of cases, the disease has a gradual onset, thus the diagnosis can be made only when the affected individual presents with an acute crisis due to an inadequate rise in cortisol secretion during a physiologic stress. Such acute adrenal insufficiency (also known as the Addisonian crisis) is a medical emergency.

On the other hand, the course of chronic adrenal insufficiency is more subtle and insidious, with the predomination of symptoms such as fatigue, weakness, weight loss, diarrhea or constipation, muscle cramps, pain in joints and postural hypotension (low blood pressure). Salt craving and low-grade fever may also be present.

The classic physical finding that can help in differentiating primary from secondary adrenal failure is hyperpigmentation of the skin or the “suntan that does not fade”. Furthermore, patients with secondary adrenal insufficiency may present with additional symptoms related to pituitary disease (e.g., menstrual disturbances, loss of libido, galactorrhea, or hypothyroidism).

Laboratory Findings and Management

In cases of adrenal insufficiency, the complete blood count usually reveals anemia, neutropenia, eosinophilia, and relative lymphocytosis. Common chemical abnormalities include metabolic acidosis and prerenal azotemia, while hyponatremia, hypoglycemia, and hyperkalemia may also be present.

A cosyntropin stimulation test (also known as ACTH or Synacthen test) is required to establish the diagnosis of adrenal insufficiency. Magnetic resonance imaging (MRI) of the hypophysis in secondary adrenal insufficiency and computed tomography (CT) of the adrenal glands in primary adrenal insufficiency can aid in establishing a diagnosis. The adrenal glands appear normal in cases of autoimmune disorder.

Glucocorticoid replacement in patients with adrenal insufficiency can be lifesaving. Nevertheless, renal crisis is still a threat to patients’ lives, which is why awareness and adequate preventative measures receive increasing attention in the recent years.

Reviewed by Susha Cheriyedath, MSc

From http://www.news-medical.net/health/Adrenal-Insufficiency-Primary-and-Secondary.aspx

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Severe Trauma May Damage The Brain as Well as the Psyche

Posted on October 24, 2016 by MaryO

NOTE: This is only a portion of the article.  Read the entire post at http://www.nytimes.com/1995/08/01/science/severe-trauma-may-damage-the-brain-as-well-as-the-psyche.html?pagewanted=all

Cortisol is a major means the body uses, with adrenaline, to arouse itself so quickly; its action, for example, triggers an increase in blood pressure and mobilizes energy from fat tissue and the liver.

“The dark side of this picture is the neurological effects,” said Dr. Sapolsky. “It’s necessary for survival, but it can be disastrous if you secrete cortisol for months or years on end. We’ve known it could lead to stress-exacerbated diseases like hypertension or adult onset diabetes. But now we’re finding the hippocampus is also damaged by these secretions.”

Studies in animals show that when glucocorticoids are secreted at high levels for several hours or days, there is a detectable effect on memory, though no neuronal death. But with sustained release from repeated stress, “it eventually kills neurons in the hippocampus,” said Dr. Sapolsky. “This has been shown solidly in rats, with the cell biology well understood.”

A parallel effect has long been known among patients with Cushing’s disease, a hormonal condition in which tumors in the adrenal or pituitary glands or corticosteroid drugs used for a prolonged time cause the adrenal glands to secrete high levels of a hormone called ACTHm and of cortisol. Such patients are prone to a range of diseases “in any organ with stress sensitivity,” including diabetes, hypertension and suppression of the immune system, said Dr. Sapolsky.

Cushing’s patients also have pronounced memory problems, especially for facts like where a car was parked. “The hippocampus is essential for transferring such facts from short-term to long-term memory,” said Dr. Sapolsky.

In 1993, researchers at the University of Michigan reported that magnetic resonance imaging had shown an atrophy and shrinkage of the hippocampus in patients with Cushing’s disease; the higher their levels of cortisol, the more shrinkage.

In an apparent paradox, low levels of cortisol in post-trauma victims were found in a separate research report, also in the July issue of The American Journal of Psychiatry. Dr. Rachel Yehuda, a psychologist at Mount Sinai Medical School in New York City, found the lower levels of cortisol in Holocaust survivors who had been in concentration camps 50 years ago and who still had post-traumatic symptoms.

“There are mixed findings on cortisol levels in trauma victims, with some researchers finding very high levels and others finding very low levels,” said Dr. Sapolsky. “Biologically speaking, there may be different kinds of post-traumatic stress.”

In a series of studies, Dr. Yehuda has found that those post-trauma patients who have low cortisol levels also seem to have “a hypersensitivity in cell receptors for cortisol,” she said. To protect itself, the body seems to reset its cortisol levels at a lower point.

The low cortisol levels “seem paradoxical, but both too much and too little can be bad,” said Dr. Yehuda. “There are different kinds of cells in various regions of the hippocampus that react to cortisol. Some atrophy or die if there is too little cortisol, some if there is too much.”

Dr. Yehuda added, “In a brain scan, there’s no way to know exactly which cells have died.”

To be sure that the shrinkage found in the hippocampus of trauma victims is indeed because of the events they suffered through, researchers are now turning to prospective studies, where before-and-after brain images can be made of people who have not yet undergone trauma, but are at high risk, or who have undergone it so recently that cell death has not had time to occur.

Dr. Charney, for example, is planning to take M.R.I. scans of the brains of emergency workers like police officers and firefighters and hopes to do the same with young inner-city children, who are at very high risk of being traumatized over the course of childhood and adolescence. Dr. Pitman, with Dr. Yehuda, plans a similar study of trauma victims in Israel as they are being treated in emergency rooms.

Dr. Yehuda held out some hope for people who have suffered through traumatic events. “It’s not necessarily the case that if you’ve been traumatized your hippocampus is smaller,” she said. She cited research with rats by Dr. Bruce McEwen, a neuroscientist at Rockefeller University, showing that atrophied dendritic extensions to other cells in the hippocampus grew back when the rats were given drugs that blocked stress hormones.

Dr. Sapolsky cited similar results in patients with Cushing’s disease whose cortisol levels returned to normal after tumors were removed. “If the loss of hippocampal volume in trauma victims is due to the atrophy of dendrites rather than to cell death, then it is potentially reversible, or may be so one day,” he said.

NOTE: This is only a portion of the article.  Read the entire post at http://www.nytimes.com/1995/08/01/science/severe-trauma-may-damage-the-brain-as-well-as-the-psyche.html?pagewanted=all

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Causes of Cushing’s Syndrome

Posted on October 2, 2016 by MaryO

Cushing’s syndrome—also referred to as hypercortisolism—is fairly rare. However, researchers have boiled down a few key causes of Cushing’s syndrome, which you’ll read about below.

The cause of Cushing’s syndrome boils down to: Your body is exposed to too much cortisol. There are a few ways that this over-exposure can happen, including taking certain medications and having a tumor on your pituitary gland or adrenal gland.

Can Taking Corticosteroids Cause Cushing’s Disease?
One particular type of medication can cause Cushing’s syndrome: corticosteroids. But rest assured: Not all steroid medications cause Cushing’s syndrome. It’s more common to develop Cushing’s syndrome from steroids you take in pill form or steroids you inject. Steroid creams and steroids you inhale are not common causes of Cushing’s syndrome.

Some steroid medications have the same effect as the hormone cortisol does when produced in your body. But as with an excessive production of cortisol in your body, taking too much corticosteroid medications can, over time, lead to Cushing’s syndrome.

It’s common for people with asthma, rheumatoid arthritis, and lupus to take corticosteroids. Prednisone (eg, Deltasone) is an example of a corticosteroid medication.

Other Cushing’s Disease Causes
Your body can over-produce cortisol or adrenocorticotropic hormone (ACTH). The pituitary gland secretes ACTH, which is in charge of stimulating the adrenal glands to produce cortisol, and the adrenal glands are responsible for releasing cortisol into the bloodstream.

Cortisol performs important tasks in your body, such as helping to maintain blood pressure and regulate how your body metabolizes proteins, fats, and carbohydrates, so it’s necessary for your body to maintain normal levels of it.

The following can cause excessive production of cortisol or ACTH, leading to Cushing’s syndrome.

  • Pituitary gland tumors: A benign (non-cancerous) tumor of the pituitary gland can secrete an excess amount of ACTH, which can cause Cushing’s syndrome. Also known as pituitary adenomas, benign tumors of the pituitary gland affect women 5 times more often than men.
  • Adrenal gland tumors: A tumor in one of your adrenal glands can lead to Cushing’s syndrome by causing too much cortisol to enter your bloodstream. Most of these tumors are non-cancerous (called adrenal adenomas).

    Cancerous adrenal tumors—called adrenocortical carcinomas—are relatively rare. These types of tumors typically cause extremely high levels of cortisol and very rapid development of symptoms.

  • Other tumors in the body: Certain tumors that develop outside the pituitary gland can also produce ACTH. When this happens, it’s known as ectopic ACTH syndrome. Ectopic means that something is in an abnormal place or position. In this case, only the pituitary gland should produce ACTH, so if there is a tumor producing ACTH and it isn’t located on the pituitary, it’s ectopic.

    It’s unusual to have a tumor that secretes ACTH outside the pituitary. These tumors are usually found in the pancreas, lungs, or thyroid, and they can be benign or malignant (cancerous).

    The most common forms of ACTH-producing tumors are small cell lung cancer, which accounts for about 13% of all lung cancer cases, and carcinoid tumors—small, slow-growing tumors that arise from hormone-producing cells in various parts of the body.

  • Familial Cushing’s syndrome: Although it’s rare, Cushing’s syndrome can develop from an inherited tendency to have tumors on one or more of your endocrine glands. Some inherited conditions, such as multiple endocrine neoplasia (MEN 1), can involve tumors that over-produce cortisol or ACTH, leading to Cushing’s syndrome.

If you think you could have Cushing’s syndrome or you have questions about the causes of Cushing’s syndrome, talk to your doctor immediately.

Written by | Reviewed by Daniel J. Toft MD, PhD, adapted from  http://www.endocrineweb.com/conditions/cushings-syndrome/cushings-syndrome-causes

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Pituitary Patient Support Group Meeting in Santa Monica, CA

Posted on September 19, 2016 by MaryO

patient-support-meeting

Pituitary Patient Support Group Meeting!
Saturday–November 19th, 2016
“We will be Live Streaming on Facebook!!!”

Speaker: Garni Barkhoudarian, MD
Topic: “Advancements in Pituitary Surgery-Better Treatments, Better Quality of Life”
Meeting: 10:00am-11:00am
Breakfast Snack will be served 10:00am-11:00am
Lunch will be served 11:30am following the meeting
Family and Friends Welcome!
Please RSVP: Sharmyn McGraw at pituitarybuddy@hotmail.com or message on FB

Filed under: Cushing's, Meetings and Conferences, pituitary | Tagged: , , , , | Leave a comment »

Interview with a Doctor on Trans-Sphenoidal surgery

Posted on August 3, 2016 by MaryO

Dr. Julius July: Neurosurgeon at the Neuroscience Center of Siloam Hospitals Lippo Village Karawaci 

A SIMPLE AND QUICK WAY TO REMOVE TUMORS VIA SURGERY THROUGH THE NOSTRIL

The mention of the word “surgery” evokes images of lengthy and elaborate procedures that involve delicate acts of cutting, abrading or suturing different parts of the body to treat an injury or disease.

This widely-held perception has led some to develop an irrational fear of surgery–especially if an operation involves a critical organ, such as the heart, or in the case of trans-sphenoidal surgery, a procedure used to remove tumors from the hormone-regulating pituitary gland located at the base of the brain.

Though the procedure has been around in different forms for the past three decades, individuals who may be in dire need of it might fear or avoid it.

To demystify this specific method of surgery, J+ spoke with Julius July, a neurosurgeon at the Neuroscience Center of Siloam Hospitals Lippo Village Karawaci. He has performed hundreds of trans-sphenoidal operations on patients throughout the country since 2008. Below is our interview, edited for length and clarity.

Tell us more about trans-sphenoidal surgery.

The goal is to extract benign tumors of the pituitary gland that are called pituitary adenoma. The pituitary gland controls different secretions of hormones. If there is a tumor and it grows large, one of the consequences could be that a patient goes blind. It can also lead to symptoms manifesting in other parts of the body due to excess hormone production, depending on the type of hormone affected by the tumor.

What does a neurosurgeon do during the procedure?

As neurosurgeons we use an endoscope with a camera attached to it and insert the instrument through the nostril. We go through the right nostril and through the sinus to reach the tumor and remove it. Once that is done, we add a coagulant to prevent bleeding. The operation takes only an hour to 90 minutes to perform and is minimally invasive. People come in and expect the surgery to last five or six hours. They hear “surgery” and fearfully assume that. But modern trans-sphenoidal surgery is simple, only lasting one to two hours.

What’s the prognosis after surgery?

In 80 percent of cases, all it takes is one surgery to remove a tumor. However, some need repeated intervention, while others require radiation. Some tumors want to invade their surroundings. In these cases, the surrounding area is a blood vessel. We can’t totally remove that type of tumor. But such cases are rare. If a patient needs more than two operations, we usually recommend radiation, because who wants to have a lot of operations?

What are the symptoms of pituitary adenoma?

Symptoms depend on whether a tumor affects hormone production or the optic nerve. The principal complaints are related to a patient’s field of vision becoming narrower. If there is a tumor in the pituitary gland area, the eye can’t see too widely. The tumors would press on the optic nerve, which leads to the periphery of your vision getting blurry.

If the tumor affects hormone production, the symptoms depend on the specific type of hormone that the tumor has affected. Different hormones have different roles. Excess prolactin hormones can lead to women–or even men–producing breast milk. If a woman who isn’t pregnant is producing breast milk, they need to be checked. The basic ingredient of milk is calcium. Without treatment, the woman will have porous bone problems. It also leads to reduced libido. If men have an excess of these prolactin hormones, they cannot get erections and will become impotent.

How does these problem develop in the first place?

Mutations lead to the creation of these benign tumors. Some things make mutations easier, such as smoking or exposure to radiation or specific chemicals. It could be anything. You could have eaten tofu and it had formalin or some meatballs with borax. Preventing it obviously requires a healthy lifestyle, but that’s easier said than done.

It’s not just one thing that causes these tumors.

Who does this pituitary tumor affect?

It affects both genders equally, more or less. The risk of pituitary adenoma compared to all other types of brain tumors is 15 percent. Children are also affected, though the condition is statistically much more likely to afflict adults. Of my patients, two in 70 would be children.

How is it diagnosed?

The doctor will check your hormones after a blood test and identify the problem. For example, if the condition affects growth hormones, a person can grow to two meters or more in height, which leads to gigantism. Alternatively, a condition could lead to horizontal growth–a bigger tongue, bigger fingers and changing shoes each month. The tongue can become so big that it causes breathing problems. Growth hormone overproduction is like a factory with the machine working overtime. As a result, a person’s life span can get cut in half. The heart works overtime, they keep growing and they die prematurely.

How many operations do you perform a year?

I’ve been doing these operations since 2008. I handle 60 to 70 such surgeries a year.

Any notable success stories to share?

One patient from Central Java came in blind. I examined him and said that there was no way we could save his vision by removing his tumor. He was crying. He had been blind for a week. But if no action was taken, the tumor would keep growing and would lead him to becoming crippled. At the end, he decided that he still wanted the operation. Surprisingly though, after the operation, he was able to see. Three months later, he was driving and reading newspapers. It was a fascinating case.

From http://www.thejakartapost.com/news/2016/07/30/well-being-trans-sphenoidal-surgery.html

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