Adrenal gland disorder

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Medical condition

Adrenal gland disorder

Depiction of location of adrenal glands in human body.
Specialty| Endocrinology

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).[3]

## Tumors of the adrenal gland

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### Adrenal adenoma

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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

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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

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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

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Main article: 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

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### Von Hippel–Lindau disease

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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

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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

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### Addison's disease

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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

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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

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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

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Main article: 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

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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

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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

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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

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* John F. Kennedy, the 35th president of the United States was diagnosed with Addison's disease.[31]
* Some have suggested Jane Austen was an avant la lettre case of Addison's Disease, but others have disputed this.[32]
* Scientist Eugene Merle Shoemaker, co-discoverer of the Comet Shoemaker-Levy 9 had Addison's Disease.[33]

## See also

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* Adrenalism
* Adrenal tumor

## References

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1. ^ Grossman, Ashley B. (May 2, 2022). "Hormonal and Metabolic Disorders". Merck Manuals Consumer Version. Retrieved February 9, 2024.
2. ^ "Adrenal Gland Disorders". National Library of Medicine. January 19, 2024. Retrieved February 9, 2024.
3. ^ a b Adrenal Glands, Johns Hopkins Medicine Health Library.
4. ^ Mahmood, Ejaz; Loughner, Chelsea L.; Anastasopoulou, Catherine (August 17, 2023). "Adrenal Adenoma". StatPearls Publishing. PMID 30969728. Retrieved February 9, 2024.
5. ^ Cleveland Clinic medical professional (October 11, 2017). "Adrenocortical Carcinoma: What Is It, Causes, Symptoms & Outlook". Cleveland Clinic. Retrieved February 9, 2024.
6. ^ "Adrenocortical Carcinoma". Johns Hopkins Medicine. December 7, 2021. Retrieved February 9, 2024.
7. ^ Allolio, Bruno; Fassnacht, Martin (June 1, 2006). "Adrenocortical Carcinoma: Clinical Update". The Journal of Clinical Endocrinology & Metabolism. 91 (6). The Endocrine Society: 2027–2037\. doi:10.1210/jc.2005-2639. ISSN 0021-972X. PMID 16551738.
8. ^ Fassnacht, Martin; Allolio, Bruno (2009). "Clinical management of adrenocortical carcinoma". Best Practice & Research Clinical Endocrinology & Metabolism. 23 (2). Elsevier BV: 273–289\. doi:10.1016/j.beem.2008.10.008. ISSN 1521-690X. PMID 19500769.
9. ^ Young, William F. (February 8, 2007). "The Incidentally Discovered Adrenal Mass". New England Journal of Medicine. 356 (6). Massachusetts Medical Society: 601–610\. doi:10.1056/nejmcp065470. ISSN 0028-4793. PMID 17287480.
10. ^ "UpToDate". UpToDate. Retrieved February 9, 2024.
11. ^ "Pheochromocytoma". National Cancer Institute. February 12, 2020. Retrieved February 9, 2024.
12. ^ Cleveland Clinic medical professional (June 30, 2022). "Pheochromocytoma: Causes, Symptoms & Treatment". Cleveland Clinic. Retrieved February 9, 2024.
13. ^ "Von Hippel-Lindau Disease (VHL)". National Institute of Neurological Disorders and Stroke. November 28, 2023. Retrieved February 9, 2024.
14. ^ "Von Hippel-Lindau syndrome: MedlinePlus Genetics". MedlinePlus. October 1, 2018. Retrieved February 9, 2024.
15. ^ "Multiple Endocrine Neoplasia (MEN)". pennmedicine.org. Retrieved February 9, 2024.
16. ^ "Multiple endocrine neoplasia: MedlinePlus Genetics". MedlinePlus. March 1, 2017. Retrieved February 9, 2024.
17. ^ Cleveland Clinic medical professional (September 20, 2023). "Addison's Disease: What It Is, Causes, Symptoms & Treatment". Cleveland Clinic. Retrieved February 9, 2024.
18. ^ Nieman, Lynnette K.; Chanco Turner, Maria L. (2006). "Addison's disease". Clinics in Dermatology. 24 (4). Elsevier BV: 276–280\. doi:10.1016/j.clindermatol.2006.04.006. ISSN 0738-081X. PMID 16828409.
19. ^ Bouillon R (December 2006). "Acute adrenal insufficiency". Endocrinology and Metabolism Clinics of North America. 35 (4). Elsevier BV: 767–75, ix. doi:10.1016/j.ecl.2006.09.004. PMID 17127145.
20. ^ Rushworth RL, Torpy DJ, Falhammar H (August 2019). "Adrenal Crisis". The New England Journal of Medicine. 381 (9): 852–861\. doi:10.1056/NEJMra1807486. PMID 31461595. S2CID 263427558.
21. ^ Charmandari, Evangelia; Nicolaides, Nicolas C; Chrousos, George P (2014). "Adrenal insufficiency". The Lancet. 383 (9935). Elsevier BV: 2152–2167\. doi:10.1016/s0140-6736(13)61684-0. ISSN 0140-6736. PMID 24503135. S2CID 205970313.
22. ^ Husebye, Eystein S; Pearce, Simon H; Krone, Nils P; Kämpe, Olle (2021). "Adrenal insufficiency". The Lancet. 397 (10274). Elsevier BV: 613–629\. doi:10.1016/s0140-6736(21)00136-7. ISSN 0140-6736. PMID 33484633. S2CID 265816016.
23. ^ El-Maouche D, Arlt W, Merke DP (November 2017). "Congenital adrenal hyperplasia" (PDF). Lancet. 390 (10108): 2194–2210\. doi:10.1016/S0140-6736(17)31431-9. PMID 28576284. S2CID 13737960.
24. ^ a b Speiser PW, Arlt W, Auchus RJ, Baskin LS, Conway GS, Merke DP, Meyer-Bahlburg HFL, Miller WL, Murad MH, Oberfield SE, White PC (2018). "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\. doi:10.1210/jc.2018-01865. PMC 6456929. PMID 30272171.
25. ^ Speiser PW, White PC (August 2003). "Congenital adrenal hyperplasia". The New England Journal of Medicine. 349 (8): 776–88\. doi:10.1056/NEJMra021561. PMID 12930931.
26. ^ La, Betty; Tung, Celestine; Choi, Eugene A.; Nguyen, Ha (1 November 2021). "A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia". AACE Clinical Case Reports. 7 (6): 342–345\. doi:10.1016/j.aace.2021.05.002. PMC 8573279. PMID 34765728.
27. ^ Aubrey Milunsky; Jeff Milunsky (29 January 2010). Genetic Disorders and the Fetus: Diagnosis, Prevention and Treatment. John Wiley and Sons. pp. 600–. ISBN 978-1-4051-9087-9. Retrieved 14 June 2010.
28. ^ "Cushing disease: MedlinePlus Medical Encyclopedia". MedlinePlus. Retrieved February 9, 2024.
29. ^ Dominguez, Alejandro; Muppidi, Vijayadershan; Gupta, Sonu (February 12, 2023). "Hyperaldosteronism". StatPearls Publishing. PMID 29763159. Retrieved February 9, 2024.
30. ^ Rajkumar, Venkatraman; Waseem, Muhammad (August 7, 2023). "Hypoaldosteronism". StatPearls Publishing. PMID 32310452. Retrieved February 9, 2024.
31. ^ Mandel, Lee R. (September 2009). "Endocrine and Autoimmune Aspects of the Health History of John F. Kennedy". Annals of Internal Medicine. 151 (5): 350–354\. doi:10.7326/0003-4819-151-5-200909010-00011. PMID 19721023. S2CID 10969109.
32. ^ Upfal, Annette (2005). "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 (1). BMJ Publishing Group: 3–11\. doi:10.1136/jmh.2004.000193. PMID 23674643.
33. ^ Marsden, Brian (1997-07-18). "Eugene Shoemaker (1928-1997)". Comet Shoemaker-Levy Collision with Jupiter. Jet Propulsion Laboratory. Archived from the original on 11 July 2007. Retrieved 2007-07-25.

## Further reading

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* Vaughan, E.Darracott (2004). "Diseases of the adrenal gland". Medical Clinics of North America. 88 (2). Elsevier BV: 443–466\. doi:10.1016/s0025-7125(03)00149-4. ISSN 0025-7125. PMID 15049587.
* Takayanagi, R.; Miura, K.; Nakagawa, H.; Nawata, H. (2000). "Epidemiologic study of adrenal gland disorders in Japan". Biomedicine & Pharmacotherapy. 54. Elsevier BV: 164s – 168s. doi:10.1016/s0753-3322(00)80036-0. ISSN 0753-3322. PMID 10915016.

## External links

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* Cleveland Clinic
* Mount Sinai

Classification|

* ICD-11: 5A7Z
* ICD-10: E27.9
* ICD-9-CM: 255.9
* MeSH: D000307
* SNOMED CT: 30171000

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External resources|

* Scholia: Q4684717

Authority control databases

National|

* United States
* Czech Republic
* Latvia
* Israel

Other|

* Yale LUX

* v
* t
* e

Adrenal gland disorder

Hyperfunction|

| Aldosterone|

* Hyperaldosteronism
* Primary aldosteronism
* Conn syndrome
* Bartter syndrome
* Glucocorticoid remediable aldosteronism
* AME
* Liddle's syndrome
* 17α CAH
* Pseudohypoaldosteronism

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Cortisol|

* Cushing's syndrome
* Pseudo-Cushing's syndrome
* Steroid-induced osteoporosis

Sex hormones|

* 21α CAH
* 11β CAH

Hypofunction|

| Aldosterone|

* Hypoaldosteronism
* 21α CAH
* 11β CAH

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Cortisol|

* CAH
* Lipoid
* 3β
* 11β
* 17α
* 21α

Sex hormones|

* 17α CAH
* Inborn errors of steroid metabolism

Adrenal insufficiency|

* Adrenal crisis
* Adrenalitis
* Xanthogranulomatous
* Addison's disease
* Waterhouse–Friderichsen syndrome

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