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Related Concept Videos

Adrenal Gland Disorders01:27

Adrenal Gland Disorders

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Adrenal gland disorders manifest when the production of adrenal hormones deviates from the norm, resulting in either excessive or insufficient concentrations.
Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...
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Hypothalamic-Pituitary Axis01:37

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The response to stress—be it physical or psychological, acute or chronic—involves activation of the Hypothalamic-Pituitary-Adrenal (HPA) axis. The HPA axis is part of the neuroendocrine system because it involves both neuronal and hormonal communication. Its function is to regulate homeostatic systems—metabolic, cardiovascular, and immune—providing the necessary means to respond to a stressor.
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Hormones of the Adrenal Glands01:31

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Adrenal hormones play a pivotal role in maintaining the body's electrolyte balance and orchestrating responses to stress, showcasing the intricate functions of the adrenal cortex and medulla.
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The sympathetic pathways of the collateral ganglia and adrenal medulla serve unique but interconnected roles in the sympathetic response.
Collateral Ganglia
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Anatomy of the Adrenal Glands01:17

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The adrenal or supra-renal glands, situated above the kidneys and aligned with the twelfth rib, are paired pyramid-shaped structures crucial for the body's stress response. During stress, these glands secrete hormones vital for adaptive physiological reactions.
These glands possess a distinctive yellow tinge due to the stored cholesterol and fatty acids required for hormone synthesis. They are encased in a fibrous capsule and cushioned by fat.
The adrenal gland comprises two distinct...
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GPCRs Regulate Adenylyl Cylase Activity01:09

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Some GPCRs transmit signals through adenylyl cyclase (AC), a transmembrane enzyme. AC helps synthesize second messenger cyclic adenosine monophosphate (cAMP). AC catalyzes cyclization reaction and converts ATP to cAMP by releasing a pyrophosphate. The pyrophosphate is further hydrolyzed to phosphate by the enzyme pyrophosphatase, which drives cAMP synthesis to completion. However, cAMP is rapidly degraded to 5′ AMP by the enzymes phosphodiesterase (PDE), preventing overstimulation of...
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Ectopic ACTH- and/or CRH-Producing Pheochromocytomas.

Patrick F Elliott1, Thomas Berhane1, Oskar Ragnarsson2,3

  • 1Department of Medicine, Royal Darwin Hospital, Darwin, Australia.

The Journal of Clinical Endocrinology and Metabolism
|July 26, 2020
PubMed
Summary

Ectopic pheochromocytomas secreting adrenocorticotropic hormone (ACTH) and/or corticotrophin-releasing hormone (CRH) are rare but manageable. Diagnosis can be challenging, but outcomes are generally favorable following treatment.

Keywords:
Cushing syndromeadrenal massdiabeteshypertensionhypokalemiamortality

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Area of Science:

  • Endocrinology and Metabolism
  • Oncology
  • Nephrology

Background:

  • Pheochromocytomas secreting catecholamines are well-characterized.
  • Limited data exists on ectopic adrenocorticotropic hormone (ACTH) and/or corticotrophin-releasing hormone (CRH)-secreting pheochromocytomas.
  • Understanding the characteristics, management, and outcomes of these rare tumors is crucial.

Purpose of the Study:

  • To review the clinical characteristics and outcomes of patients with ACTH- and/or CRH-secreting pheochromocytomas.
  • To consolidate existing knowledge on this rare endocrine tumor subtype.

Main Methods:

  • Systematic literature search of PubMed/MEDLINE and Web of Science up to February 10, 2020.
  • Inclusion of original articles, case reports, and case series reporting individual patient data.
  • Extraction of data on demographics, clinical presentation, comorbidities, biochemistry, imaging, histopathology, and outcomes.

Main Results:

  • Ninety-nine cases of ACTH- and/or CRH-secreting pheochromocytomas were identified from 91 articles.
  • Median age at diagnosis was 49 years, with a 2:1 female to male ratio.
  • Common presentations included Cushing syndrome (81%), hypertension (93%), and diabetes (54%); postoperative improvements in blood pressure, glucose control, and biochemistry were observed in most patients, with infections being the most frequent complication.

Conclusions:

  • Ectopic ACTH- and/or CRH-secreting pheochromocytoma should be considered in patients with ACTH-dependent Cushing syndrome and an adrenal mass.
  • Despite diagnostic challenges, patient outcomes are generally favorable.
  • Further research may elucidate optimal diagnostic and management strategies.