Cushing’s Syndrome Revealing Carney Complex: A Case Report

 

Carney complex (CNC) is a rare multisystem disorder, inherited in an autosomal dominant manner and characterized by distinctive spotty skin pigmentation, myxomas and endocrine abnormalities.

We report a case of a 35-year-old patient diagnosed with Cushing’s syndrome complicated with an impaired glucose tolerance (IGT) and a severe psychiatric disturbance. The diagnosis of CNC was made by having two major criteria, namely a primary pigmented nodular adrenal disease (PPNAD) and thyroid carcinoma.

Read the entire report here: Cushing’s Syndrome Revealing Carney Complex: A Case
Report

The Pituitary Gland: Small But Mighty

The pituitary gland works hard to keep you healthy, doing everything from ensuring proper bone and muscle growth to helping nursing mothers produce milk for their babies. Its functionality is even more remarkable when you consider the gland is the size of a pea.

“The pituitary is commonly referred to as the ‘master’ gland because it does so many important jobs in the body,” says Karen Frankwich, MD, a board-certified endocrinologist at Mission Hospital. “Not only does the pituitary make its own hormones, but it also triggers hormone production in other glands. The pituitary is aided in its job by the hypothalamus. This part of the brain is situated above the pituitary, and sends messages to the gland on when to release or stimulate production of necessary hormones.”

These hormones include:

  • Growth hormone, for healthy bone and muscle mass
  • Thyroid-stimulating hormone, which signals the thyroid to produce its hormones that govern metabolism and the body’s nervous system, among others
  • Follicle-stimulating and luteinizing hormones for healthy reproductive systems (including ovarian egg development in women and sperm formation in men, as well as estrogen and testosterone production)
  • Prolactin, for breast milk production in nursing mothers
  • Adrenocorticotropin (ACTH), which prompts the adrenal glands to produce the stress hormone cortisol. The proper amount of cortisol helps the body adapt to stressful situations by affecting the immune and nervous systems, blood sugar levels, blood pressure and metabolism.
  • Antidiuretic (ADH), which helps the kidneys control urine levels
  • Oxytocin, which can stimulate labor in pregnant women

The work of the pituitary gland can be affected by non-cancerous tumors called adenomas. “These tumors can affect hormone production, so you have too little or too much of a certain hormone,” Dr. Frankwich says. “Larger tumors that are more than 1 centimeter, called macroadenomas, can also put pressure on the area surrounding the gland, which can lead to vision problems and headaches. Because symptoms can vary depending on the hormone that is affected by a tumor, or sometimes there are no symptoms, adenomas can be difficult to pinpoint. General symptoms can include nausea, weight loss or gain, sluggishness or weakness, and changes in menstruation for women and sex drive for men.”

If there’s a suspected tumor, a doctor will usually run tests on a patient’s blood and urine, and possibly order a brain-imaging scan. An endocrinologist can help guide a patient on the best course of treatment, which could consist of surgery, medication, radiation therapy or careful monitoring of the tumor if it hasn’t caused major disruption.

“The pituitary gland is integral to a healthy, well-functioning body in so many ways,” Dr. Frankwich says. “It may not be a major organ you think about much, but it’s important to know how it works, and how it touches on so many aspects of your health.”

Learn more about Mission Hospital. Learn more about Dr. Frankwich.

From http://www.stjhs.org/HealthCalling/2016/December/The-Pituitary-Gland-Small-but-Mighty.aspx

Pituitary Gland: Normal Function and Assessment

Abstract

This computer-based, interactive module introduces preclinical medical students to normal pituitary function and outlines its assessment. Solid understanding of these topics is requisite to learning clinical disorders of the pituitary.

Existing resources largely target learners at earlier or later stages of training; thus, we created this resource to address needs of medical students during a first- or second-year endocrine course. A module format was selected to promote interactive, independent learning.

Two cohorts of medical students completed the 40-minute module: 172 second-year students who had completed a year of basic sciences in the traditional curriculum and 180 foundation-phase students in a three-semester combined basic and clinical sciences curriculum (due to a change in the medical school curriculum at our institution). In both instances, the module was completed before start of clinical pituitary content. A static set of PowerPoint slides accompanied the module to facilitate note taking.

Test Your Knowledge slides were inserted to ensure grasp of key terms/concepts before moving to subsequent slides. A short question-and-answer session was held following module completion to clarify points of confusion. Students rated effectiveness of the module as 4.6 out of 5, commenting on its clarity, organization, high-yield nature, and utility in preparing for clinical material.

Faculty noted greater understanding of foundational pituitary principles and more engaging discussions. The percentage of pituitary-related questions answered correctly on the midterm exam increased.

Finally, success of the pituitary module prompted development of adrenal, thyroid, and parathyroid modules that now comprise the Endocrine Organs Introduction Series in our curriculum.

Citation

Kirk D, Smith KW. Pituitary gland: normal function and assessment. MedEdPORTAL Publications. 2016;12:10430. http://dx.doi.org/10.15766/mep_2374-8265.10430

Educational Objectives

After completing this module, the learner will be able to:

  1. Describe the normal function and regulation of the pituitary gland, including names and actions of the anterior and posterior pituitary hormones.
  2. Understand the basic approach to laboratory assessment of the pituitary.
  3. Differentiate between anterior and posterior pituitary origin, function, and regulation.
  4. List the hormones produced by the pituitary gland.
  5. Discuss for each pituitary hormone: hypothalamic stimulating/inhibiting factors and their clinical uses, basic physiologic function, and regulation (feedback loop).
  6. Describe factors that affect growth hormone levels.
  7. Understand the tests for growth hormone excess and deficiency.
  8. Define a primary versus secondary endocrine disorder.

Keywords

  • Endocrine, Endocrinology, Pituitary, Module, Preclinical Medical Education

More information at https://www.mededportal.org/publication/10430

‘Adrenal Fatigue’ Not Always Used Accurately

Dear Dr. Roach: I had apoplexy, a ruptured pituitary tumor, developed panhypopituitarism, then adrenal insufficiency. I am doing fairly well with cortisol replacement, thyroid supplement and oral diabetic medicine.

My problem is exhaustion that comes on very easily. I have other ailments to blame, too — chronic pain from fibromyalgia and tendinitis. I am 67. I am still able to work. Is adrenal fatigue a real issue, and if so, what can be done about it? — S.M.

Answer: The term “adrenal fatigue” is increasingly used, and not always correctly — or, at least, it is used in cases where it’s not clear if that is actually the case. But let me start by discussing what has happened to you. Pituitary apoplexy is bleeding into the pituitary gland, usually into a pituitary tumor, as in your case. This may cause severe headaches and vision changes, and often it prevents the pituitary from making the many important hormones that control the endocrine glands and regulate the body.

For example, without TSH from the pituitary gland, the thyroid won’t release thyroid hormone, and importantly, the adrenal gland can’t make cortisol without the influence of ACTH from the pituitary.

Rather than trying to replace TSH, ACTH and the other pituitary hormones, it is easier to directly replace the hormones made by the adrenal, thyroid and gonads. That’s why you are taking cortisol and thyroid hormone, and why younger women take estrogen and men testosterone. Although there is nothing wrong with your thyroid and adrenal glands, they simply won’t work unless stimulated.

Inadequate adrenal function from any cause leads to profound fatigue, and in the presence of severe stress, such as surgery or major infection, the body’s need for cortisol increases dramatically. Unless enough adrenal hormone is given in response, the result can be an immediate life-threatening condition called an Addisonian crisis.

Readers may email questions to ToYourGoodHealth@med.cornell.edu.

From http://www.vnews.com/To-Your-Good-Health–Adrenal-Fatigue–not-Always-Used-Accurately-1802516

“My feet are killing me!” An unusual presentation of Cushing’s syndrome

Adverse effects of steroid excess on bone metabolism are well established but presentation of Cushing’s syndrome with metabolic bone disease is reported to be uncommon. We describe a case of Cushing’s syndrome presenting with pathological fractures probably present for 8 years before diagnosis.

A 33 year old nurse first sustained spontaneous stress fractures of her metatarsals in 1994, with repeated fractures occurring up to 2002. In 2001 she developed hypertension, acute lumbar back pain and gained weight.

In 2002 she was admitted to hospital with chest/back pain. Lumbar spine X-ray showed new fracture of L3,old fractures of L4/5,with fractured ribs on CXR. Isotope bone scan revealed multiple hot spots. MRI showed collapse of T8 with features consistent with malignant disease. The primary malignancy was sought and a left-sided 1.5 centimetre thyroid nodule detected.

Suspicious cytology prompted thyroid lobectomy revealing follicular variant of papillary carcinoma. T8 biopsy revealed chronic infection with Propionobacteria rather than metastatic carcinoma. Despite antibiotic therapy further spontaneous vertebral fractures developed. Bone densitometry revealed Z scores of minus 2.4 at L2-4, minus 2.5 and 2.9 at the hips.

Referral to our centre prompted investigations for Cushing’s syndrome. Serum potassium was 4.1 millimols per litre, androgens, calcitonin and urinary catecholamines all normal. TSH was suppressed by T4 therapy. Urinary free cortisol values were raised,(563-959 nanomols per 24hours) with loss of diurnal rhythm in cortisol secretion (9am 429-586,midnight 397-431 nanomols per litre)and no suppression on low or high dose dexamethasone. Abdominal CT showed a 3.5 centimetre adrenal mass. These findings were consistent with adrenal dependent Cushing’s syndrome. Risedronate and metyrapone were commenced before adrenalectomy, completion thyroidectomy and ablative radioiodine.
Comment: Cushing’s syndrome may present with spontaneous fractures in both axial and appendicular skeleton in the absence of marked clinical features. This case demonstrates the importance of thorough investigation of unexplained fractures.

LM Albon, JD Rippin & JA Franklyn

From http://www.endocrine-abstracts.org/ea/0005/ea0005p26.htm

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