Adrenal gland disorder explained

Adrenal gland disorders (or diseases) are conditions that interfere with the normal functioning of the adrenal glands.[1] Your body produces too much or too little of one or more hormones when you have an adrenal gland dysfunction. The type of issue you have and the degree to which it affects your body's hormone levels determine the symptoms.[2]

The adrenal gland produces hormones that affects growth, development and stress, and also helps to regulate kidney function. There are two parts of the adrenal glands, the adrenal cortex and the adrenal medulla. The adrenal cortex produces mineralocorticoids, which regulate salt and water balance within the body, glucocorticoids (including cortisol) which have a wide number of roles within the body, and androgens, hormones with testosterone-like function.[3] The adrenal medulla produces epinephrine (adrenaline) and norepinephrine (noradrenaline).

Tumors of the adrenal gland

Adrenal adenoma

See main article: Adrenal adenoma. Adrenal adenomas are benign tumors that start in the cortex of the adrenal gland. They fall into one of two categories: functional or non-secreting. Adenomas that are nonfunctional or have modest levels of hormone secretion may not show any symptoms at all and may remain asymptomatic. But adenomas with high levels of hormones frequently show up with primary hyperaldosteronism, Cushing syndrome, or hyperandrogenism symptoms.[4]

Adrenocortical carcinoma

See main article: Adrenocortical carcinoma. Adrenocortical carcinoma, (ACC), is cancer that develops in the adrenal glands' cortex, or outer layer.[5] Adrenocortical carcinoma usually occurs randomly, independent of a genetic predisposition.[6] The majority of patients' primary complaints at first are indications and manifestations of excess hormones.[7] Due to local tumor growth, some patients have vague symptoms such as flank or abdominal pain, fullness in the abdomen, or early satiety.[8]

Adrenal incidentaloma

See main article: Adrenal incidentaloma. An adrenal incidentaloma is a mass lesion larger than 1 cm in diameter that was unintentionally found through radiologic examination.[9] In patients who do not yet have a confirmed cancer diagnosis, adrenal incidentaloma is infrequently caused by malignancy.[10]

Pheochromocytoma

Pheochromocytoma originates from chromaffin cells and is a kind of neuroendocrine tumor.[11] Pheochromocytomas are generally benign. 10% to 15% of pheochromocytomas have the potential to be cancerous.[12]

Hereditary disorders associated with adrenal tumors

Von Hippel–Lindau disease

See main article: Von Hippel–Lindau disease. Von Hippel–Lindau disease is a rare genetic multi-system disorder where certain body parts develop non-cancerous tumors.[13] Pheochromocytomas are a particular kind of tumor that are linked to von Hippel-Lindau syndrome. VHL gene mutations result in von Hippel-Lindau syndrome.[14]

Multiple endocrine neoplasia

See main article: Multiple endocrine neoplasia. Multiple endocrine neoplasia results in tumors or overgrowth on one or more endocrine glands.[15] Multiple endocrine neoplasia is classified into three main forms: type 1, type 2, and type 4. Multiple endocrine neoplasia can be brought on by mutations in the MEN1, RET, and CDKN1B genes.[16]

Disorders of hormone over/under-production

Addison's disease

See main article: Addison's disease. Addison's disease, or primary adrenal insufficiency, is an uncommon chronic illness characterized by insufficient production of cortisol and aldosterone by the adrenal glands.[17] Chronic primary adrenal insufficiency is typically characterized by an extended period of malaise, fatigue, anorexia, weight loss, joint and back pain, and skin darkening. Mineralocorticoid and glucocorticoid hormone deficiency must be physiologically replaced in order to treat primary adrenal insufficiency.[18]

Adrenal crisis

See main article: Adrenal crisis. Adrenal crisis is a serious, life-threatening complication of adrenal insufficiency. Hypotension, or hypovolemic shock, is the main symptom of adrenal crisis, other indications and symptoms include weakness, anorexia, nausea, vomiting, fever, fatigue, abnormal electrolytes, confusion, and coma.[19] Laboratory testing may detect lymphocytosis, eosinophilia, hyponatremia, hyperkalemia, hypoglycemia, and on occasion, hypercalcemia.[20]

Adrenal insufficiency

See main article: Adrenal insufficiency. Adrenal insufficiency is the clinical sign of insufficient glucocorticoid production or action, with or without concurrent insufficiency in mineralocorticoids and adrenal androgens.[21] Adrenocorticotropic hormone deficiency or exogenous glucocorticoid or opioid medication suppression of adrenocorticotropic hormone can cause adrenal insufficiency, as can primary adrenal disorders. Unintentional weight loss, anorexia, postural hypotension, extreme exhaustion, muscle and abdominal pain, and hyponatraemia are characteristic clinical features.[22]

Congenital adrenal hyperplasia

Congenital adrenal hyperplasia is a group of autosomal recessive disorders characterized by impaired cortisol synthesis.[23] [24] It results from the deficiency of one of the five enzymes required for the synthesis of cortisol in the adrenal cortex.[25] Most of these disorders involve excessive or deficient production of hormones such as glucocorticoids, mineralocorticoids, or sex steroids,[26] [24] and can alter development of primary or secondary sex characteristics in some affected infants, children, or adults.[27]

Cushing's disease

See main article: Cushing's disease. Cushing's disease is an illness where an excess of adrenocorticotropic hormone (ACTH) is released by the pituitary gland. Cushing syndrome can be brought on by Cushing disease.[28]

Hyperaldosteronism

See main article: Hyperaldosteronism. Hyperaldosteronism is caused by the adrenal gland's overproduction of the hormone aldosterone. The excess production of the adrenal gland, specifically the zona glomerulosa, is the cause of primary hyperaldosteronism. Excessive renin-angiotensin-aldosterone system activation results in secondary hyperaldosteronism.[29]

Hypoaldosteronism

See main article: Hypoaldosteronism. Hypoaldosteronism is a clinical condition marked by either an aldosterone deficiency or impaired tissue-level action of the hormone. Angiotensin I to Angiotensin II conversion, adrenal aldosterone synthesis and secretion, abnormal target tissue response to aldosterone, and renal renin production and secretion are all potential causes of the disorder.[30]

Notable people with adrenal gland disorders

See also

Further reading

External links

Notes and References

  1. Web site: Grossman . Ashley B. . Hormonal and Metabolic Disorders . Merck Manuals Consumer Version . May 2, 2022 . February 9, 2024.
  2. Web site: Adrenal Gland Disorders . National Library of Medicine . January 19, 2024 . February 9, 2024.
  3. http://www.hopkinsmedicine.org/healthlibrary/conditions/endocrinology/adrenal_glands_85,P00399/ Adrenal Glands
  4. Web site: Mahmood . Ejaz . Loughner . Chelsea L. . Anastasopoulou . Catherine . Adrenal Adenoma . StatPearls Publishing . August 17, 2023 . 30969728 . February 9, 2024.
  5. Web site: Cleveland Clinic medical professional . Adrenocortical Carcinoma: What Is It, Causes, Symptoms & Outlook . Cleveland Clinic . October 11, 2017 . February 9, 2024.
  6. Web site: Adrenocortical Carcinoma . Johns Hopkins Medicine . December 7, 2021 . February 9, 2024.
  7. Allolio . Bruno . Fassnacht . Martin . Adrenocortical Carcinoma: Clinical Update . The Journal of Clinical Endocrinology & Metabolism . The Endocrine Society . 91 . 6 . June 1, 2006 . 0021-972X . 10.1210/jc.2005-2639 . 2027–2037. 16551738 .
  8. Fassnacht . Martin . Allolio . Bruno . Clinical management of adrenocortical carcinoma . Best Practice & Research Clinical Endocrinology & Metabolism . Elsevier BV . 23 . 2 . 2009 . 1521-690X . 10.1016/j.beem.2008.10.008 . 273–289. 19500769 .
  9. Young . William F. . The Incidentally Discovered Adrenal Mass . New England Journal of Medicine . Massachusetts Medical Society . 356 . 6 . February 8, 2007 . 0028-4793 . 10.1056/nejmcp065470 . 601–610. 17287480 .
  10. Web site: UpToDate . UpToDate . February 9, 2024.
  11. Web site: Pheochromocytoma . National Cancer Institute . February 12, 2020 . February 9, 2024.
  12. Web site: Cleveland Clinic medical professional . Pheochromocytoma: Causes, Symptoms & Treatment . Cleveland Clinic . June 30, 2022 . February 9, 2024.
  13. Web site: Von Hippel-Lindau Disease (VHL) . National Institute of Neurological Disorders and Stroke . November 28, 2023 . February 9, 2024.
  14. Web site: Von Hippel-Lindau syndrome: MedlinePlus Genetics . MedlinePlus . October 1, 2018 . February 9, 2024.
  15. Web site: Multiple Endocrine Neoplasia (MEN) . pennmedicine.org . February 9, 2024.
  16. Web site: Multiple endocrine neoplasia: MedlinePlus Genetics . MedlinePlus . March 1, 2017 . February 9, 2024.
  17. Web site: Cleveland Clinic medical professional . Addison's Disease: What It Is, Causes, Symptoms & Treatment . Cleveland Clinic . September 20, 2023 . February 9, 2024.
  18. Nieman . Lynnette K. . Chanco Turner . Maria L. . Addison's disease . Clinics in Dermatology . Elsevier BV . 24 . 4 . 2006 . 0738-081X . 10.1016/j.clindermatol.2006.04.006 . 276–280. 16828409 .
  19. Bouillon R . Acute adrenal insufficiency . Endocrinology and Metabolism Clinics of North America . 35 . 4 . 767–75, ix . December 2006 . 17127145 . 10.1016/j.ecl.2006.09.004 . Elsevier BV .
  20. Rushworth RL, Torpy DJ, Falhammar H . Adrenal Crisis . The New England Journal of Medicine . 381 . 9 . 852–861 . August 2019 . 31461595 . 10.1056/NEJMra1807486 . 263427558 .
  21. Charmandari . Evangelia . Nicolaides . Nicolas C . Chrousos . George P . Adrenal insufficiency . The Lancet . Elsevier BV . 383 . 9935 . 2014 . 0140-6736 . 10.1016/s0140-6736(13)61684-0 . 2152–2167. 24503135 . 205970313 .
  22. Husebye . Eystein S . Pearce . Simon H . Krone . Nils P . Kämpe . Olle . Adrenal insufficiency . The Lancet . Elsevier BV . 397 . 10274 . 2021 . 0140-6736 . 10.1016/s0140-6736(21)00136-7 . 613–629. 33484633 . 265816016 .
  23. El-Maouche D, Arlt W, Merke DP . Congenital adrenal hyperplasia . Lancet . 390 . 10108 . 2194–2210 . November 2017 . 28576284 . 10.1016/S0140-6736(17)31431-9 . 13737960 .
  24. vanc . 30272171. 2018. Speiser. P. W.. Arlt. W.. Auchus. R. J.. Baskin. L. S.. Conway. G. S.. Merke. D. P.. Meyer-Bahlburg HFL. Miller. W. L.. Murad. M. H.. Oberfield. S. E.. White. P. C.. Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society Clinical Practice Guideline. The Journal of Clinical Endocrinology and Metabolism. 103. 11. 4043–4088. 10.1210/jc.2018-01865. 6456929.
  25. Speiser PW, White PC . Congenital adrenal hyperplasia . . 349 . 8 . 776–88 . August 2003 . 12930931 . 10.1056/NEJMra021561.
  26. La . Betty . Tung . Celestine . Choi . Eugene A. . Nguyen . Ha . A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia . AACE Clinical Case Reports . 1 November 2021 . 7 . 6 . 342–345 . 10.1016/j.aace.2021.05.002 . 34765728 . 8573279 .
  27. Book: Aubrey Milunsky. Jeff Milunsky. Genetic Disorders and the Fetus: Diagnosis, Prevention and Treatment. 14 June 2010. 29 January 2010. John Wiley and Sons. 978-1-4051-9087-9. 600–.
  28. Web site: Cushing disease: MedlinePlus Medical Encyclopedia . MedlinePlus . February 9, 2024.
  29. Web site: Dominguez . Alejandro . Muppidi . Vijayadershan . Gupta . Sonu . Hyperaldosteronism . StatPearls Publishing . February 12, 2023 . 29763159 . February 9, 2024.
  30. Web site: Rajkumar . Venkatraman . Waseem . Muhammad . Hypoaldosteronism . StatPearls Publishing . August 7, 2023 . 32310452 . February 9, 2024.
  31. Mandel . Lee R. . Endocrine and Autoimmune Aspects of the Health History of John F. Kennedy . Annals of Internal Medicine . 151 . 5 . 350–354 . September 2009 . 10.7326/0003-4819-151-5-200909010-00011 . 19721023 . 10969109 .
  32. Upfal. Annette. Jane Austen's lifelong health problems and final illness: New evidence points to a fatal Hodgkin's disease and excludes the widely accepted Addison's. Medical Humanities. 31. 2005. 3–11. 10.1136/jmh.2004.000193. 23674643. BMJ Publishing Group. 1. free.
  33. Web site: Brian . Marsden . Eugene Shoemaker (1928-1997) . 2007-07-25 . Comet Shoemaker-Levy Collision with Jupiter . 1997-07-18 . . https://web.archive.org/web/20070711025314/http://www2.jpl.nasa.gov/sl9/news81.html. 11 July 2007 . live .

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