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| | Image = Illu adrenal gland.jpg | | | Image = Illu adrenal gland.jpg |
| | Caption = Adrenal gland | | | Caption = Adrenal gland |
| | DiseasesDB =
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| | ICD10 = {{ICD10|E|27|1|e|20}}-{{ICD10|E|27|4|e|20}}
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| | ICD9 = {{ICD9|255.4}}
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| | ICDO =
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| | OMIM =
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| | MedlinePlus =
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| | MeshID = D000309
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| }} | | }} |
| {{SI}} | | {{Secondary adrenal insufficiency}} |
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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' | | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
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| {{CMG}} | | {{CMG}} {{AE}} {{ADS}} |
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| {{SK}} hypocortisolism; hypocorticism; adrenocortical hypofunction | | {{SK}} hypocortisolism; hypocorticism; adrenocortical hypofunction |
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| ==Overview== | | ==[[Secondary adrenal insufficiency overview|Overview]]== |
| Adrenal insufficiency is a condition in which the [[adrenal gland]]s, located above the [[kidney]]s, do not produce adequate amounts of [[steroid hormone]]s (chemicals produced by the body that regulate organ function), primarily [[cortisol]], but may also include impaired [[aldosterone]] production (a [[mineralocorticoid]]) which regulates [[sodium]], [[potassium]] and water retention.<ref name="addisons_disease.aspx">{{cite journal | author=Eileen K. Corrigan |title=Adrenal Insufficiency (Secondary Addison's or Addison's Disease)|journal=NIH Publication No. 90-3054 |year=2007 |url=http://www.pituitary.org/disorders/addisons_disease.aspx}}</ref><ref>{{MeshName|Adrenal+Insufficiency}}</ref> Craving for salt or salty foods due to the urinary losses of sodium is common.<ref name="PMID 11443143">{{cite journal |author=Ten S, New M, Maclaren N |title=Clinical review 130: Addison's disease 2001 |journal=J. Clin. Endocrinol. Metab. |volume=86 |issue=7 | pages=2909–22 |year=2001 |pmid=11443143 |url=http://jcem.endojournals.org/cgi/content/full/86/7/2909 |doi=10.1210/jc.86.7.2909}}</ref>
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| [[Addison's disease]] and [[congenital adrenal hyperplasia]] can manifest as adrenal insufficiency. If not treated, adrenal insufficiency may result in severe abdominal pains, diarrhea, vomiting, profound muscle weakness and fatigue, [[clinical depression|depression]], extremely low blood pressure ([[hypotension]]), weight loss, kidney failure, changes in mood and personality, and shock (adrenal crisis).<ref name="ch164b.html">{{cite journal | author=Ashley B. Grossman, MD | title=Addison's Disease | journal=Adrenal Gland Disorders | year=2007 | url=http://www.merck.com/mmhe/sec13/ch164/ch164b.html}}</ref> An [[adrenal crisis]] often occurs if the body is subjected to stress, such as an accident, injury, surgery, or severe infection; death may quickly follow.<ref name="ch164b.html" /> | | ==[[Secondary adrenal insufficiency historical perspective|Historical Perspective]]== |
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| Adrenal insufficiency can also occur when the [[hypothalamus]] or the [[pituitary gland]], both located at the base of the [[Human skull|skull]], does not make adequate amounts of the hormones that assist in regulating adrenal function.<ref name="addisons_disease.aspx"/><ref name="urlJAMA -- Adrenal Insufficiency, November 16, 2005, Brender et al. 294 (19): 2528">{{cite journal |author=Brender E, Lynm C, Glass RM |title=JAMA patient page. Adrenal insufficiency |journal=JAMA |volume=294 |issue=19 |page=2528 |year=2005 |pmid=16287965 |doi=10.1001/jama.294.19.2528 |url=http://jama.ama-assn.org/cgi/content/full/294/19/2528}}</ref><ref name="urlDorlands Medical Dictionary:adrenal insufficiency">{{cite web |url=http://www.mercksource.com/pp/us/cns/cns_hl_dorlands_split.jsp?pg=/ppdocs/us/common/dorlands/dorland/four/000053970.htm |title=Dorlands Medical Dictionary:adrenal insufficiency |work= |accessdate=}}</ref> This is called secondary adrenal insufficiency and is caused by lack of production of [[ACTH]] in the pituitary or lack of [[Corticotropin-releasing hormone|CRH]] in the hypothalamus.<ref name="urlSecondary Adrenal Insufficiency: Adrenal Disorders: Merck Manual Professional">{{cite web |url=http://www.merck.com/mmpe/sec12/ch153/ch153c.html |title=Secondary Adrenal Insufficiency: Adrenal Disorders: Merck Manual Professional |work= |accessdate=}}</ref>
| | ==[[Secondary adrenal insufficiency classification|Classification]]== |
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| ==Classification== | | ==[[Secondary adrenal insufficiency pathophysiology|Pathophysiology]]== |
| There are three major types of adrenal insufficiency.
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| * '''Primary adrenal insufficiency''' is due to impairment of the adrenal glands.
| | ==[[Secondary adrenal insufficiency causes|Causes]]== |
| ** One subtype is called [[idiopathic]] or unknown cause of adrenal insufficiency.
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| ** 80% are due to an [[autoimmune disease]] called [[Addison's disease]] or [[autoimmune adrenalitis]].
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| ** Other cases are due to [[congenital adrenal hyperplasia]] or an [[adenoma]] (tumor) of the adrenal gland.
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| * '''Secondary adrenal insufficiency''' is caused by impairment of the [[pituitary gland]] or [[hypothalamus]].<ref name="Hypopituitary">{{cite web |url=http://www.webmd.com/a-to-z-guides/hypopituitary |title=hypopituitary |year=2006}}</ref> The most common cause in the United States is exogenous steroid use. Other causes are a pituitary adenoma or microadenoma, hypothalamic tumor (surgical removal of a pituitary tumor can also suppress production of ACTH and lead to adrenal deficiency); [[Sheehan's syndrome]], which is associated with impairment of only the pituitary gland.
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| * '''Tertiary adrenal insufficiency''' is due to hypothalamic disease and decrease in corticotropin releasing factor (CRF).<ref>http://www.endotext.org/adrenal/adrenal13/adrenal13.htm</ref>
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| == Causes == | | ==[[Differentiating Secondary adrenal insufficiency from other diseases|Differentiating Adrenal Insufficiency from other Diseases]]== |
| ===Common Causes===
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| * '''Acute adrenal insufficiency'''
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| ** [[Addison's disease]] ([[autoimmune adrenalitis]])
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| ** [[Septic shock]]
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| ** [[Waterhouse-Friderichsen syndrome]]
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| ** [[Addison's disease#Addisonian crisis|Addisonian crisis]] in case of:
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| *** Discontinuing [[corticosteroid]] therapy without tapering the dosage
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| *** Surgery, illness or any other form of stress in patients with long-term [[corticosteroid]] therapy
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| * '''Chronic adrenal insufficiency'''
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| ** [[Addison's disease]]
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| ** [[Congenital adrenal hyperplasia|Congenital Adrenal Hyperplasia]]
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| ** [[Antiphospholipid syndrome]]
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| ** [[Haemochromatosis]]
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| ===Causes by Organ System=== | | ==[[Secondary adrenal insufficiency epidemiology and demographics|Epidemiology and Demographics]]== |
| {|style="width:80%; height:100px" border="1"
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| |style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
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| |style="height:100px"; style="width:75%" border="1" bgcolor="Beige" | [[Arteritis]], [[Hypotension]], [[Hemorrhage]], [[Infarction]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Chemical / poisoning'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Dermatologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Drug Side Effect'''
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| |bgcolor="Beige"| [[Corticosteroid withdrawal]], [[Prednisone]], [[Prednisolone]], [[Dexamethasone]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Ear Nose Throat'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Endocrine'''
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| |bgcolor="Beige"| [[Adrenal aplasia]] / hypoplasia, [[Adrenal metastases]], After surgery of [[cortisol-secreting tumor]], [[Bilateral adrenalectomy ]], [[Congenital adrenal hyperplasia]], [[Glucocorticoid deficiency 1]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Environmental'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Gastroenterologic'''
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| |bgcolor="Beige"| [[Hemochromatosis ]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Genetic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Hematologic'''
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| |bgcolor="Beige"| [[Anticoagulation]], [[Coagulopathy ]], [[Embolus]], [[Leukemia]], [[Lymphoma ]], [[Thrombosis]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Iatrogenic'''
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| |bgcolor="Beige"| [[Iatrogenic]], [[Radiation therapy]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Infectious Disease'''
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| |bgcolor="Beige"| [[AIDS]], [[Blastomycosis]], [[CMV]], [[Coccidiomycosis]], [[Cryptococcosis]], [[Histoplasmosis ]], [[Mycobacterium avium intracellulaire]] ([[MAI]]), [[Sepsis]], [[Syphilis]], [[Toxoplasmosis]], [[Tuberculosis]], [[Waterhouse-Friderichson syndrome ]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Musculoskeletal / Ortho'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Neurologic'''
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| |bgcolor="Beige"| [[Adrenoleukodystrophy]], [[Coma]], [[Craniopharyngioma]], [[Panhypopituitarism]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Nutritional / Metabolic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Obstetric/Gynecologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Oncologic'''
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| |bgcolor="Beige"| [[Craniopharyngioma]], [[Kaposi's sarcoma]], [[Leukemia]], [[Lymphoma ]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Opthalmologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Overdose / Toxicity'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Psychiatric'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Pulmonary'''
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| |bgcolor="Beige"| [[Sarcoidosis]],
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Renal / Electrolyte'''
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| |bgcolor="Beige"| [[Uremia]], [[Waterhouse-Friderichson syndrome ]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Rheum / Immune / Allergy'''
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| |bgcolor="Beige"| [[Autoimmune]], [[Sarcoidosis]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Sexual'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Trauma'''
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| |bgcolor="Beige"| [[Trauma]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Urologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Dental'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Miscellaneous'''
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| |bgcolor="Beige"| [[Amyloidosis]], [[Idiopathic]], [[Iatrogenic]], [[Radiation therapy]], [[Surgery]], Surgical removal of [[pituitary]], Surgical removal of [[hypothalamus]]
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| |-
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| |}
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| ===Causes in Alphabetical Order=== | | ==[[Secondary adrenal insufficiency risk factors|Risk Factors]]== |
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| | ==[[Secondary adrenal insufficiency natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ==Diagnosis== | | ==Diagnosis== |
| ===Symptoms===
| | [[Secondary adrenal insufficiency history and symptoms| History and Symptoms]] | [[Secondary adrenal insufficiency physical examination | Physical Examination]] | [[Secondary adrenal insufficiency laboratory findings|Laboratory Findings]] | [[Secondary adrenal insufficiency electrocardiogram|Electrocardiogram]] | [[Secondary adrenal insufficiency chest x ray|Chest X Ray]] | [[Secondary adrenal insufficiency CT|CT]] | [[Secondary adrenal insufficiency MRI|MRI]] | [[Secondary adrenal insufficiency ultrasound|Ultrasound]] | [[Secondary adrenal insufficiency other imaging findings|Other Imaging Findings]] | [[Secondary adrenal insufficiency other diagnostic studies|Other Diagnostic Studies]] |
| There is often the slow insidious onset of the following symptoms (in alphabetical order):
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| *[[Dehydration]]
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| *[[Diarrhea]]
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| *[[Disorientation]]
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| *[[Dizziness]]
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| *[[Hypoglycemia]]
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| *[[Muscle aches]]
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| *[[Nausea]]
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| *[[Tanning]] of the skin that may be patchy or even all over the body. Characteristic sites of tanning are skin creases (e.g. of the hands) and the inside of the cheek ([[buccal mucosa]]).
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| *[[Tiredness]]
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| *[[Vomiting]]
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| *[[Weakness]]
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| *[[Weight loss]]
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| ===Physical Examination===
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| ====Vital Signs====
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| *[[Hypotension|Low blood pressure]] that falls further when standing ([[orthostatic hypotension]])
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| *[[Cardiovascular collapse]] may be present
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| ====Skin====
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| *There can be tanning of the skin. [[Tanning]] of the skin that may be patchy or even all over the body. Characteristic sites of tanning are skin creases (e.g. of the hands) and the inside of the cheek ([[buccal mucosa]]).
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| *[[Vitiligo]] may also be present.<ref name="ch164b.html"/>
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| ====Neck====
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| [[Goitre]] may be present
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| ===Laboratory Findings===
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| ====ACTH Stimulation Test====
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| If the person is in [[adrenal crisis]], the [[ACTH stimulation test]]<ref>{{cite journal|pmid=21656493 | doi=10.1024/0040-5930/a000174 | volume=68 | issue=6 | title=[Adrenal insufficiency--diagnosis and treatment in clinical practice] | year=2011 | month=June | author=Henzen C | journal=Ther Umsch | pages=337–43}}</ref> may be given. If not in crisis, cortisol, ACTH, aldosterone, renin, potassium and sodium are tested from a blood sample before the decision is made if the ACTH stimulation test needs to be performed. X-rays or CT of the adrenals may also be done.<ref name="addisons_disease.aspx"/> The best test for adrenal insufficiency of autoimmune origin, representing more than ninety percent of all cases in a Western population, is measurement of 21-hydroxylase autoantibodies.
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| {| border="4" cellspacing="1" cellpadding="4" style="text-align:center; margin:10px;"
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| | style="background:#ddcef2" | '''Source of pathology|| style="background:#cef2e0" |'''CRH|| style="background:#cef2e0" |'''ACTH|| style="background:#cef2e0" |'''DHEA|| style="background:#cef2e0" |'''DHEA-S|| style="background:#cef2e0" |'''cortisol||style="background:#cef2e0;" |'''aldosterone|| style="background:#cef2e0" |'''renin|| style="background:#cef2e0" |'''Na|| style="background:#cef2e0" |'''K|| style="background:#f2e0ce" |'''Causes'''<sup>5</sup>
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| | style="background:#cedff2" | '''hypothalamus'''<br>(tertiary)<sup>1</sup>|| style="background:#F8F8F8" |low|| style="background:#F8F8F8"|low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low<sup>3</sup>||style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low||style="background:#F8F8F8" |tumor of the hypothalamus (adenoma), [[antibodies]], [[environment (biophysical)|environment]] (i.e. toxins), [[head injury]]
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| | style="background:#cedff2" | '''pituitary'''<br>(secondary)|| style="background:#F8F8F8" |high<sup>2</sup>|| style="background:#F8F8F8"|low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low<sup>3</sup>||style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |low||style="background:#F8F8F8" |tumor of the pituitary ([[adenoma]]), antibodies, environment, head injury,<br>'''surgical removal'''<sup>6</sup>, Sheehan's syndrome
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| | style="background:#cedff2" | '''adrenal glands'''<br>(primary)<sup>7</sup>|| style="background:#F8F8F8" |high|| style="background:#F8F8F8"|high|| style="background:#F8F8F8" |high|| style="background:#F8F8F8" |high|| style="background:#F8F8F8" |low<sup>4</sup>||style="background:#F8F8F8" |low|| style="background:#F8F8F8" |high|| style="background:#F8F8F8" |low|| style="background:#F8F8F8" |high||style="background:#F8F8F8" |tumor of the adrenal (adenoma), [[Stress (biology)|stress]], antibodies, environment, [[Addison's Disease]], [[Physical trauma|trauma]], surgical removal ([[Segmental resection|resection]]), [[wikt:miliary|miliary]] [[tuberculosis]] of the adrenal
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| |}
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| {| border="2" cellspacing="1" cellpadding="2" style="margin:10px;"
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| | '''1'''|| Automatically includes diagnosis of secondary (hypopituitarism)
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| | '''2'''|| Only if CRH production in the hypothalamus is intact
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| | '''3'''|| Value doubles or more in stimulation
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| | '''4'''|| Value less than doubles in stimulation
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| | '''5'''|| Most common, does not include all possible causes
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| | '''6'''|| Usually because of very large tumor (macroadenoma)
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| | '''7'''|| Includes Addison's disease
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| |}
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| ==Treatment== | | ==Treatment== |
| *;Adrenal crisis
| | [[Secondary adrenal insufficiency medical therapy|Medical Therapy]] | [[Secondary adrenal insufficiency surgery|Surgery]] | [[Secondary adrenal insufficiency primary prevention|Primary Prevention]] | [[Secondary adrenal insufficiency secondary prevention|Secondary Prevention]] | [[Secondary adrenal insufficiency cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Secondary adrenal insufficiency future or investigational therapies|Future or Investigational Therapies]] |
| **Intravenous fluids<ref name="ch164b.html"/>
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| **Intravenous steroid ([[Solu-Cortef]]/injectable hydrocortisone) later hydrocortisone, prednisone or methylpredisolone tablets<ref name="ch164b.html"/>
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| **Rest
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| *;Cortisol deficiency (primary and secondary)
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| **[[Hydrocortisone]] (Cortef)
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| **[[Prednisone]] (Deltasone)
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| **[[Prednisolone]] (Delta-Cortef)
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| **[[Methylprednisolone]] (Medrol)
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| **[[Dexamethasone]] (Decadron)
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| *;Mineralocorticoid deficiency (low aldosterone)
| | ==Case Studies== |
| **Fludrocortisone Acetate
| | [[Secondary adrenal insufficiency case study one|Case#1]] |
| (To balance sodium, potassium and increase water retention)<ref name="ch164b.html"/>
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| ==See also== | | ==Related Chapters== |
| * [[Addison disease]], primary adrenocortical insufficiency | | * [[Addison disease]], primary adrenocortical insufficiency |
| * [[ACTH stimulation test]] | | * [[ACTH stimulation test]] |
| * [[Cushing's syndrome]], overproduction of cortisol | | * [[Cushing's syndrome]], overproduction of [[cortisol]] |
| * [[Insulin tolerance test]], another test used to identify sub-types of adrenal insufficiency | | * [[Insulin tolerance test]], another test used to identify sub-types of [[adrenal insufficiency]]. |
| * [[Adrenal fatigue]] (hypoadrenia), a term used in [[alternative medicine]] to describe a believed exhaustion of the adrenal glands | | * [[Adrenal fatigue]] (hypoadrenia), a term used in [[alternative medicine]] to describe a believed exhaustion of the [[adrenal glands]]. |
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| ==References==
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| {{reflist|2}}
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| {{Endocrine pathology}} | | {{Endocrine pathology}} |
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| [[Category:Endocrinology]] | | [[Category:Endocrinology]] |
| [[Category:Disease]] | | [[Category:Disease]] |
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| [[ca:Insuficiència suprarenal]]
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| [[de:Nebennierenrindeninsuffizienz]]
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| [[es:Insuficiencia adrenal]]
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| [[fr:Insuffisance surrénalienne]]
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| [[he:תת פעילות בלוטת יותרת הכליה]]
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