Pituitary Dysfunction as a Result of Traumatic Brain Injury

Posted on December 21, 2016 by MaryO

A victim of brain injury can experience many consequences and complications as a result of brain damage. Unfortunately, the problems caused by a traumatic brain injury can extend even beyond what most people think of as the standard symptoms of a brain injury, like mood change and cognitive impairment. One issue which can occur is pituitary dysfunction. If the pituitary gland is damaged due to injury to the brain, the consequences can be dramatic as the pituitary gland works together with the hypothalamus to control every hormonal aspect of a person’s body.

Pituitary dysfunction as a result of a brain injury can be difficult to diagnose, as you may not immediately connect your symptoms to the head injury you experienced. If you did suffer injury to the pituitary gland, you need to know about it so you can get proper treatment. If someone else caused your brain injury to occur, you also want to know about your pituitary dysfunction so you can receive compensation for costs and losses associated with this serious health problem.

The pituitary is a small area of the center of your brain that is about the size of the uvula. The pituitary is surrounded and guarded by bone, but it does hang down.  When it becomes damaged as a result of a brain injury, the damage normally occurs as a result of the fact the pituitary was affected by reduced by reduced blood flow. It can also be harmed directly from the trauma, and only a tiny amount of damage can cause profound consequences.

Many of the important hormones that your body needs are controlled by the pituitary working with the hypothalamus. If the pituitary is damaged, the result can include a deficiency of Human Growth Hormone (HGH). This deficiency can affect your heart and can impact bone development.  Thyroid Stimulating Hormone (TSH) can also be affected, which could result in hypothyroidism. Sex hormones (gonodotropin); Adrenocorticotopic hormone; and many other hormones could be impacted as well, causing fertility problems; muscle loss; sexual dysfunction; kidney problems; fatigue; or even death.

Unfortunately, problems with the pituitary gland may not always be visible on MRIs or other imaging tests because the pituitary is so small. Endocrinologists who handle hormone therapy frequently are not familiar with brain injuries, and may not make the connection that your brain injury was the cause of the problem.

If you begin to experience hormonal issues following an accident, you should be certain to get an accurate diagnosis to determine if your brain injury played a role. If it did, those responsible for causing the accident could be responsible for compensating you for the harm you have experienced to your pituitary and to the body systems which malfunction as a result of your new hormonal issues.

Nelson Blair Langer Engle, PLLC

From http://www.nblelaw.com/posts/pituitary-dysfunction-result-of-traumatic-brain-injury

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Bilateral testicular tumors resulting in recurrent Cushing’s syndrome after bilateral adrenalectomy

Posted on December 21, 2016 by MaryO
Troy Puar1,2, Manon Engels3,4, Antonius E. van Herwaarden4, Fred C. G. J. Sweep4, Christina Hulsbergen-van de Kaa5, Karin Kamphuis-van Ulzen6, Vasileios Chortis7,8, Wiebke Arlt7,8, Nike Stikkelbroeck1, Hedi L Claahsen–van der Grinten3, Ad R.M.M. Hermus1
Corresponding author: Troy Puar, MRCP (UK), Department of Medicine, Div. of Endocrinology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands. Phone: +31 243614599, Fax: +31 243618809, e-mail: Troy_puar@cgh.com.sg
DOI: http://dx.doi.org/10.1210/jc.2016-2702
Received: July 14, 2016
Accepted: November 29, 2016
First Published Online: November 30, 2016
Abstract
Context:

Recurrence of hypercortisolism in patients after bilateral adrenalectomy for Cushing’s disease is extremely rare.

Patient:

We present a rare case of a 27-year-old man who previously underwent bilateral adrenalectomy for Cushing’s disease with complete clinical resolution. Cushingoid features recurred 12 years later, along with bilateral testicular enlargement. Hormonal tests confirmed ACTH-dependent Cushing’s. Surgical resection of the testicular tumors led to clinical and biochemical remission.

Design and Results:
Gene expression analysis of the tumor tissue by qPCR showed high expression of all key steroidogenic enzymes. Adrenocortical-specific genes were 5.1 x 105 (CYP11B1), 1.8 x 102 (CYP11B2) and 6.3 x 104 (MC2R) times higher than non-steroidogenic fibroblast control. This correlated with urine steroid metabolome profiling showing 2-5 fold increases in the excretion of the metabolites of 11-deoxycortisol, 21-deoxycortisol and total glucocorticoids. Leydig-specific genes were 4.3 x 101 (LHCGR) and 9.3 x 100(HSD17B3) times higher than control and urinary steroid profiling showed 2-fold increased excretion of the major androgen metabolites androsterone and etiocholanolone. These distinctly increased steroid metabolites were suppressed by dexamethasone, but unresponsive to hCG stimulation, supporting the role of ACTH, but not LH, in regulating tumor-specific steroid excess.
Conclusion:

We report bilateral testicular tumors occurring in a patient with recurrent Cushing’s disease 12 years after bilateral adrenalectomy. Using mRNA expression analysis and steroid metabolome profiling, the tumors demonstrated both adrenocortical and gonadal steroidogenic properties, similar to testicular adrenal rest tumors found in patients with congenital adrenal hyperplasia. This suggests the presence of pluripotent cells even in patients without CAH.

Affiliations
  • 1Department of Medicine, Division of Endocrinology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 2Department of Endocrinology, Changi General Hospital, Singapore 529889, Singapore.
  • 3Department of Paediatrics, Division of Endocrinology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 4Department of Laboratory Medicine, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 5Department of Pathology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 6Department of Radiology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 7Institute of Metabolism and Systems Research, University of Birmingham, Birmingham N15 2TT, United Kingdom.
  • 8Centre for Endocrinology, Diabetes, and Metabolism, Birmingham Health Partners, Birmingham B15 2TH, United Kingdom.

– See more at: http://press.endocrine.org/doi/abs/10.1210/jc.2016-2702#sthash.F4lfWg9j.dpuf

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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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Hypopituitarism – Deficiency in Pituitary Hormone Production

Posted on June 30, 2016 by MaryO

By Yolanda Smith, BPharm

Hypopituitarism is a health condition in which there is a reduction in the production of hormones by the pituitary gland.

The pituitary gland is located at the base of the brain and is responsible for the production of several hormones, including:

  • Adrenocorticotropic hormone (ACTH), which controls the production of the vital stress hormones cortisol and dehydroepiandrosterone (DHEA) in the adrenal gland
  • Thyroid stimulating hormone (TSH), which controls the production of hormones by the thyroid gland
  • Luteinizing hormone (LH) and follicle-stimulating hormone (FSH), which control the secretion of the primary sex hormones and affect fertility
  • Growth hormone (GH), which regulates the growth processes in childhood and other metabolic processes throughout life
  • Prolactin (PRL), which facilitates the production of breast milk
  • Oxytocin, which is crucial during labor, childbirth and lactation
  • Antidiuretic hormone (ADH), also known as vasopressin, which regulates the retention of water and the blood pressure

An individual with hypopituitarism shows a deficiency in one or more of these hormones. This inevitably leads to abnormal body function, as an effect of the low levels of the hormone in the body, and may result in symptoms.

Causes

Hypopituitarism is most commonly due to the destruction, compression or inflammation of pituitary tissue by a brain tumor in that region. Other causes include:

  • Head injury
  • Infections such as tuberculosis
  • Ischemic or infarct injury
  • Radiation injury
  • Congenital and genetic causes
  • Infiltrative diseases such as sarcoidosis

Symptoms

General symptoms that are associated with pituitary hormone deficiency include:

  • Weakness and fatigue
  • Decreased appetite
  • Weight loss
  • Sensitivity to cold
  • Swollen facial features or body

There are also likely to be more specific symptoms according to the type of pituitary hormone deficiency, such as:

  • ACTH deficiency:
    • abdominal pain
    • low blood pressure
    • low serum sodium levels
    • skin pallor
  • TSH deficiency:
    • generalized body puffiness
    • sensitivity to cold
    • constipation
    • impaired memory and concentration
    • dry skin
    • anemia
  • LH and FSH deficiency:
    • reduction in libido
    • erectile dysfunction in men
    • abnormal menstrual periods
    • vaginal dryness in women
    • difficulty in conceiving
    • infertility.
  • GH deficiency:
    • slow growth
    • short height
    • an increase in body fat

Treatment

The first step in the treatment of hypopituitarism is to identify the cause of the condition.

Secondly, the hormones that are deficient must be identified. From this point, the appropriate treatment decisions can be made to promote optimal patient outcomes.

Hormone replacement therapy is the most common type of treatment for a patient with hypopituitarism.

This may involve supplementation of one or more hormones that are deficient, to reduce or correct the impact of the deficiency.

Follow Up

As hormone replacement therapy is expected to continue on a lifelong basis, it is important that patients have a good understanding of the therapy.

It is especially important to educate patients on what to do in case of particular circumstances that may change their hormone requirements.

For example, during periods of high stress, the demand for many hormones is increased, and the dose of hormone replacement may need to be adjusted accordingly.

It is recommended that patients have regular blood tests to monitor their hormone levels and ensure that they are in the normal range.

Patients should also carry medical identification, such as a medical bracelet or necklace, to show that they are affected by hypopituitarism and inform others about their hormone replacement needs and current treatment. This can help to meet their medical needs in case of any emergency.

Epidemiology

Hypopituitarism is a rare disorder that affects less than 200,000 individuals in the United States, with an incidence of 4.2 cases per 100,000 people per year.

The incidence is expected to be higher in certain subsets of the population, such as those that have suffered from a brain injury. Statistics in reference to these population groups have not yet been determined.

Reviewed by Dr Liji Thomas, MD.

References

From http://www.news-medical.net/health/Hypopituitarism-Deficiency-in-Pituitary-Hormone-Production.aspx

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Surgery Preferred Option in Cushing’s Disease for Best Survival

Posted on June 21, 2016 by MaryO

Patients with Cushing’s disease who have been in remission for more than 10 years still have an increased mortality risk compared with the general population, says an international team of researchers, who found the risk of early death was particularly increased in those with Cushing’s and accompanying circulatory disease.

Richard N Clayton, MD, department of medicine, Keele University, Stoke-on-Trent, United Kingdom, showed that Cushing’s disease, which is characterized by increased secretion of adrenocorticotropic hormone by the anterior pituitary gland, is associated with an increased mortality risk of more than 60% and a median survival of around 40 years.

In patients who also had circulatory disease, the mortality risk was even higher, say Dr Clayton and colleagues.

However, patients who had undergone curative pituitary surgery had a long-term risk of death no different from that of the general population. US Endocrine Society guidelines published last August recommend that optimal treatment of Cushing’s syndrome involves direct surgical removal of the causal tumor.

But Dr Clayton and colleagues point out that even patients who undergo pituitary surgery will nevertheless “require lifelong follow-up at a center experienced in dealing with this condition, having regular checks for diabetes, hypertension, and other cardiovascular risk factors.”

The study was published online June 2 in Lancet Diabetes & Endocrinology.

In an accompanying editorial, Rosario Pivonello, MD, PhD, department of clinical medicine and surgery, section of endocrinology, University of Naples Federico II, Italy, and colleagues write that, although surgery is not suitable for all patients, “Prompt pituitary surgery might be the preferred treatment for Cushing’s disease to guarantee the best mortality outcome.”

Calling for further research to better understand why one treatment “has a better effect on mortality than another,” they state: “The results from this study might also motivate rapid interventions, cure, and long-term follow-up in patients with Cushing’s disease — even for a long time after hypercortisolism resolution.”

Studying Those Who Have Survived More Than 10 Years

Dr Clayton and colleagues explain that previous studies have explored mortality in patients with Cushing’s disease during either active disease or remission. But the outcome of patients in remission, especially long-term remission, is still a matter of debate, and assessing long-term survival has been limited by various methodological differences. To overcome some of these issues, they performed a retrospective analysis of case records from specialist referral centers in the United Kingdom, Denmark, the Netherlands, and New Zealand.

They identified 320 patients diagnosed with Cushing’s disease and cured for a minimum of 10 years at enrollment and had no relapses during the study period. The ratio of women to men was 3:1.

Median patient follow-up was 11.8 years, yielding a total of 3790 person-years of follow-up 10 years after cure. There was no difference in follow-up between countries. And as there were no significant demographic and clinical differences between men and women, the data were pooled.

During the study 16% of patients died. Median survival was 31 years for women and 28 years for men, at approximately 40 years following remission. The overall standardized mortality ratio (SMR) for all-cause mortality compared with the general population was 1.61 (P = .0001).

Patients with Cushing’s and circulatory disease had an SMR vs the general population of 2.72 (P < .0001), but deaths from cancer among those who had survived Cushing’s disease were not higher than the general population, at an SMR of 0.79 (P = .41).

Patients with Cushing’s and diabetes also had an increased mortality risk, at a hazard ratio (HR) of 2.82 (P < .0096) compared with the general population, while hypertension was not significantly associated with increased mortality, at an HR of 1.59 (P = .08).

There was also an association between mortality and number of treatments, at an HR of 1.77 for two vs one treatment (P = .08) and an HR of 2.6 for three vs one treatment (P = .02).

Pituitary Surgery Alone Associated With No Increased Risk of Death

Pituitary surgery performed as the first and only treatment was associated with an SMR vs the general population of 0.94 compared with an SMR of 2.58 for other patients (P < .0005).

Patients who had pituitary surgery only had a median survival of 31 years compared with 24 years if surgery had been required at any time (P = 0.03).

The research team states: “For patients who have been cured of Cushing’s disease for 10 years or more, treatment complexity and an increased number of treatments, reflecting disease that is more difficult to control, appears to negatively affect survival.”

“Pituitary surgery alone achieves a mortality outcome that is not different from the normal population and should be performed in a center of excellence,” they conclude.

However, in the editorial, Dr Pivonello and colleagues point out that the surgical approach “is not a treatment option for some patients, either because of contraindications (eg, severe clinical complications) or because of an absence of clear indication for surgery (eg, tumor is not completely removable by surgery).”

The authors and editorialist have reported no relevant financial relationships.

Lancet Diabetes Endocrinol. Published online June 2, 2016. Abstract, Editorial

From http://www.medscape.com/viewarticle/865073#vp_2

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