Past News Items: My 37-year-old daughter has Addison’s disease.

old-news

 

Because, sometimes Old News is still valid!

From Tuesday, September 16, 2008

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.

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

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Given Adrenal Symptoms, Blood Test Recommended

adrenal-glands

 

Q: My husband’s recent CT scan of his stomach and digestive system revealed that he has nodules on both adrenal glands. It was suggested that he undergo a blood test to determine whether the nodules are producing hormones.

For 21 months, he has experienced high blood pressure, nausea, diarrhea, anxiety and abdominal pain. Could this be the source of his problems? If so, what course of action would you recommend?

A: The adrenal gland is responsible for the production of several essential hormones.

Tumors, or nodules, of the adrenal glands are common. They can be categorized into those that make hormones and those that don’t, and also by whether the tumors are benign or malignant.

The most common, by far, are benign, nonfunctioning tumors. These are usually discovered on an ultrasound or a CT scan obtained for some other reason.

More than 4 percent of people have an adrenal mass, and 85 percent are nonfunctional.

The symptoms that your husband has, however, raise a concern that he might have a hormone-producing tumor.

Four types of hormones are commonly produced by adrenal tumors: cortisone, aldosterone, sex hormones (estrogen or androgens) and catecholamines (epinephrine and norepinephrine).

A cortisone-producing adrenal tumor causes Cushing’s syndrome. It usually causes weight gain, especially in the abdomen; skin changes, including striae, or “stretch marks”; high blood pressure; and a predisposition to diabetes. Anxiety and abdominal pain are uncommon.

Aldosterone raises blood pressure, so a person with a functioning adrenal tumor making aldosterone usually has high blood pressure, but the other symptoms you mention for your husband aren’t common for this type of tumor.

Adrenal tumors that make epinephrine and the related norepinephrine are called pheochromocytomas. Hypertension is almost universal with this condition, and anxiety is frequently reported.

Tumors that produce sex hormones are rare, and they are present in men with androgen excess or feminization, in the case of estrogen-secreting tumors.

Although your husband’s symptoms aren’t specific for any one condition, the combination of his symptoms and adrenal nodules concerns me.

I agree with the recommendation to look for excess amounts of hormones in the blood. This can often be achieved with a simple blood test; however, a catheter is occasionally placed in the adrenal vein to sample blood coming from the gland (and its nodule) directly.

By comparing one side against the other, doctors can determine which side might be producing excess hormones.

An endocrinologist is the expert most likely to be familiar with these conditions.

Dr. Roach answers letters only in his North America Syndicate column but provides an order form of available health newsletters at http://www.rbmamail.com. Write him at 628 Virginia Dr., Orlando, FL 32853-6475; or ToYour GoodHealth@med. cornell.edu.

From http://www.dispatch.com/content/stories/life_and_entertainment/2015/07/27/given-adrenal-symptoms-blood-test-recommended.html

Multiple aberrant hormone receptors in Cushing’s Syndrome

Eur J Endocrinol. 2015 May 13. pii: EJE-15-0200. [Epub ahead of print]
Multiple Aberrant Hormone Receptors in Cushing’s Syndrome.

Abstract

The mechanisms regulating cortisol production when ACTH of pituitary origin is suppressed in primary adrenal causes of Cushing’s syndrome include diverse genetic and molecular mechanisms. These can lead either to constitutive activation of the cAMP system and steroidogenesis or to its regulation exerted by the aberrant adrenal expression of several hormone receptors, particularly G-protein coupled hormone receptors (GPCR) and their ligands.

Screening for aberrant expression of GPCR in BMAH and unilateral adrenal tumors of patients with overt or subclinical CS demonstrates the frequent co-expression of several receptors. Aberrant hormone receptors can also exert their activity by regulating the paracrine secretion of ACTH or other ligands for those receptors in BMAH or unilateral tumors.

The aberrant expression of hormone receptors is not limited to adrenal Cushing’s syndrome but can be implicated in other endocrine tumors including primary aldosteronism and Cushing’s disease. Targeted therapies to block the aberrant receptors or their ligands could become useful in the future.

PMID:
25971648
[PubMed – as supplied by publisher]

Adrenal insufficiency – how to spot this rare disease and how to treat it

adrenal-glandsAddison’s disease, or adrenal insufficiency, is a rare hormonal disorder of the adrenal glands that affects around 8,400 people in the UK.

The adrenal glands are about the size of a pea and perched on top of the kidneys, and affect the body’s production of the hormones cortisol and sometimes aldosterone.

When someone suffers from adrenal insufficiency, those glands aren’t producing a sufficient amount of these hormones. This can have a detrimental effect on someone’s health and well-being. But because the symptoms are similar to a host of other conditions, Addison’s disease can prove tough to isolate.

What to look out for

According to advice provided by the NHS, the symptoms in the early stages of Addison’s disease, which affects both men and women, are gradual and easy to misread as they’re similar to many other conditions.

People can experience severe fatigue, muscle weakness, low moods, loss of appetite, unintentional weight loss, low blood pressure, nausea, vomiting and salt craving.

“Symptoms are often misread or ignored until a relatively minor infection leads to an abnormally long convalescence, which prompts an investigation,” says Professor Wiebke Arlt from the Centre for Endocrinology, Diabetes & Metabolism at the University of Birmingham.

Life-threatening condition

If Addison’s disease is left untreated, the level of hormones produced by the adrenal gland will gradually decrease in the body. This will cause symptoms to get progressively worse and eventually lead to a potentially life-threatening situation called an adrenal, or Addisonian, crisis. Signs include severe dehydration; pale, cold, clammy skin; rapid, shallow breathing; extreme sleepiness; severe vomiting and diarrhoea. If left untreated, it can prove fatal, so the patient should be admitted to hospital as an emergency.

Back to basics

To understand the disorder, it’s important to get to grips with the basics and that means understanding what the adrenal glands are – and so to the science.

“Adrenal glands have an inner core (known as the medulla) surrounded by an outer shell (known as the cortex) ,” explains Arlt.
The inner medulla produces adrenaline, the ‘fight or flight’ stress hormone. While the absence of this does not cause the disease, the cortex is more critical.

“It produces the steroid hormones that are essential for life: cortisol and aldosterone,” he adds.

“Cortisol mobilises nutrients, enables the body to fight inflammation, stimulates the liver to produce blood sugar and also helps control the amount of water in the body. Aldosterone, meanwhile, regulates the salt and water levels, which can affect blood volume and pressure.”

Why does it happen?

The disorder occurs if the adrenal glands are destroyed, absent or unable to function and failure of the glands themselves is known as primary adrenal insufficiency.

“It’s most often caused by autoimmune disease where the body’s immune system mounts an attack against its own adrenal glands,” explains Arlt.

“However it can also be caused by infection, most importantly by tuberculosis and sometimes by both adrenal glands being surgically removed.”

The pituitary effect

Another important cause is any disease affecting the pituitary gland, which is located behind the nose at the bottom of the brain.
“The pituitary is the master gland that tells the other glands in the body what to do,” continues Arlt.

“The pituitary gland produces a hormone called ACTH (adrenocorticotropic hormone to give it its full name), which travels in the blood stream to the adrenal glands.

“Here it acts as a signal, causing the adrenal glands to produce more cortisol. If the pituitary gland stops making ACTH, [then] cortisol production by the adrenals is no longer controlled properly and a condition called secondary adrenal insufficiency arises.”

But in most cases, aldosterone is still produced, which means that people suffering from secondary adrenal insufficiency have fewer problems than those with primary adrenal insufficiency.

Determining a diagnosis

Due to the ambiguous nature of the symptoms, a Short Synacthen Test (SST) needs to be performed in order to diagnose adrenal insufficiency.

“This measures the ability of the adrenal glands to produce cortisol in response to (the pituitary hormone) ACTH,” says Arlt. “When carrying out this test, a baseline blood sample is drawn before injecting a dose of ACTH, followed by drawing a second blood sample 30 to 60 minutes later. Failing adrenal glands will not be able to produce a certain level of cortisol.”

Getting treatment

If someone has been conclusively diagnosed with adrenal insufficiency, they should receive adrenal hormone replacement therapy as advised by an endocrinologist, a doctor specialising in hormone-related diseases.

“A normal adrenal gland does not need supplements to function properly and there is no recognised medical condition called ‘adrenal fatigue’,” warns Arlt.

“Either the adrenal gland is fine and needs no treatment or there is adrenal insufficiency due to adrenal or pituitary failure.”

So if in doubt, don’t self-diagnose but book an appointment with your GP.

For more information, visit Addison’s Disease Self-Help Group (www.addisons.org.uk) or Pituitary Foundation.

From https://home.bt.com/lifestyle/wellbeing/adrenal-insufficiency-how-to-spot-this-rare-disease-and-how-to-treat-it-11363985141306

Common Asthma Steroids Linked to Side Effects in Adrenal Glands

(Reuters Health) – After stopping steroids commonly prescribed for asthma and allergies, a significant number of people may experience signs of malfunctioning in the adrenal glands, a European study finds.

So-called adrenal insufficiency can be dangerous, especially if the person’s body has to cope with a stress like surgery, injury or a serious illness, the study authors say.

“The takeaway message of the study is that in corticosteroid use there is a substantial risk of adrenal insufficiency,” senior author Dr. Olaf Dekkers, an endocrinologist at Aarhus University in Denmark, said by email. “Patients should be aware of this risk and be informed about potential symptoms.”

Those symptoms can include fatigue, dizziness, weight loss and salt cravings, the authors write in the Journal of Clinical Endocrinology and Metabolism.

Corticosteroids are man-made drugs designed to mimic the hormone cortisol, which the adrenal glands produce naturally. The drugs are usually used to counter inflammation in a wide range of conditions, including asthma, psoriasis, rheumatoid arthritis, lupus, blood cancers and organ transplants.

People with adrenal insufficiency do not make enough of two hormones, cortisol and aldosterone. Cortisol helps the body respond to stress, recover from infections and regulate blood pressure and metabolism. Aldosterone helps maintain the right amounts of salt, potassium and water in the body.

While on steroids, the body often produces less of these hormones naturally, and after coming off the drugs it can take a while for natural production to ramp back up. The result is adrenal insufficiency, which can be treated with medication to replace cortisol or aldosterone.

Dekkers and colleagues analyzed 74 research articles published from 1975 to 2014, covering a total of 3753 study participants, to see how different doses and types of corticosteroid treatment might impact the likelihood of developing adrenal insufficiency after treatment.

Researchers found the risk of adrenal insufficiency was highest when corticosteroids were taken orally or injected, and lower with inhaled, nasal or topical treatment.

When they looked just at patients using steroids for asthma, the researchers found that the risk of adrenal insufficiency was about 7 percent with inhaled corticosteroids, but about 44 percent with other formulations including oral medication.

Only about 2 percent of asthma patients on the lowest dose of steroids experienced adrenal insufficiency, compared with about 22 percent on the highest doses.

Similarly, slightly more than 1 percent of asthma patients on short-term steroids developed adrenal insufficiency, compared with about 27 percent on long-term treatment.

There is no way to safely halt treatment with corticosteroids that can rule out the potential for adrenal insufficiency, Dekkers said.

The side effect is more likely when patients take higher doses of steroids or remain on treatment for longer than three weeks, said Dr. Roberto Salvatori, medical director of the pituitary center at Johns Hopkins Hospital in Baltimore.

“It’s likely, and it’s often overlooked because most often the people who prescribe corticosteroids aren’t endocrinologists; they are in other specialities and they don’t recognize the symptoms of adrenal insufficiency,” said Salvatori, who wasn’t involved in the study.

He gives his patients on corticosteroids medical identification bracelets or necklaces to wear so they can be identified as at risk for adrenal insufficiency in an emergency. “This is a very important issue that’s not on the radar screen,” he said.

To be sure, more physicians are aware of the risk now than in the 1970s, and the standard doses and durations of corticosteroid treatment have been reduced in part because of this risk, said Dr. Douglas Coursin, a professor at the University of Wisconsin School of Medicine and Public Health in Madison. He, too, advises medical alert bracelets for patients on long-term or high-dose treatment.

“In the past, patients with asthma, certain immune diseases, those receiving some cancer therapies and those who had a solid organ transplant received higher doses for longer periods of time,” Coursin, who wasn’t involved in the study, said by email. “Overall, I think the risk may be lower than outlined in the study because of practice changes.”

SOURCE: bit.ly/1PjRHYw Journal of Clinical Endocrinology and Metabolism, online April 6, 2015.

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