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{{Adrenal insufficiency}}
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==Overview==
==Overview==
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
Pharmacologic medical therapy is recommended among patients with [[adrenal insufficiency]]
Pharmacologic medical therapies for [[adrenal insufficiency]] include lifelong [[glucocorticoid]] and [[mineralocorticoid]] replacement therapy. [[Mineralocorticoid]] replacement therapy is only used in patients with [[primary adrenal insufficiency]].


OR
==Medical Therapy==
 
*Pharmacologic medical therapy is recommended among patients with [[adrenal insufficiency]].
Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
*Pharmacologic medical therapies for [[adrenal insufficiency]] include lifelong [[glucocorticoid]] replacement in both primary and secondary [[adrenal insufficiency]]. [[Mineralocorticoids]] replacement is recommended only in [[primary adrenal insufficiency]]. Rarely [[dehydroepiandrosterone]] (DHEA) in considered in patients with [[primary adrenal insufficiency]] in whom there is significant impairment in quality of life, decreased libido, women, depressed mood despite [[glucocorticoid]] and [[mineralocorticoid]] therapy. <ref name="BornsteinAllolio2016">{{cite journal|last1=Bornstein|first1=Stefan R.|last2=Allolio|first2=Bruno|last3=Arlt|first3=Wiebke|last4=Barthel|first4=Andreas|last5=Don-Wauchope|first5=Andrew|last6=Hammer|first6=Gary D.|last7=Husebye|first7=Eystein S.|last8=Merke|first8=Deborah P.|last9=Murad|first9=M. Hassan|last10=Stratakis|first10=Constantine A.|last11=Torpy|first11=David J.|title=Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline|journal=The Journal of Clinical Endocrinology & Metabolism|volume=101|issue=2|year=2016|pages=364–389|issn=0021-972X|doi=10.1210/jc.2015-1710}}</ref> <ref name="OpreaBonnet2019">{{cite journal|last1=Oprea|first1=Alina|last2=Bonnet|first2=Nicolas C. G.|last3=Pollé|first3=Olivier|last4=Lysy|first4=Philippe A.|title=Novel insights into glucocorticoid replacement therapy for pediatric and adult adrenal insufficiency|journal=Therapeutic Advances in Endocrinology and Metabolism|volume=10|year=2019|pages=204201881882129|issn=2042-0188|doi=10.1177/2042018818821294}}</ref><ref>https://www.elsevier.es/index.php?p=revista&pRevista=pdf-simple&pii=S2173509314700698</ref>
 
OR
 
The majority of cases of [disease name] are self-limited and require only supportive care.
 
OR
 
[Disease name] is a medical emergency and requires prompt treatment.
 
OR
 
The mainstay of treatment for [disease name] is [therapy].
 
OR
 
The optimal therapy for [malignancy name] depends on the stage at diagnosis.
 
OR


[Therapy] is recommended among all patients who develop [disease name].


OR
===Adrenal insufficiency===


Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
* '''Glucocorticoid replacement therapy'''
 
** 1.1 '''Adult'''
OR
*** Preferred regimen (1): [[Hydrocortisone]] 15-25mg PO, 2/3 of the total dose in the morning on awakening; 1/3 of the total dose in mid-afternoon lifelong
 
*** Preferred regimen (2): [[Hydrocortisone]] 15-25mg PO, three divided doses (10mg at 7 AM, 5mg at 12 PM, and 2.5-5mg at 4:30 PM. Avoid dosing after 6 PM) lifelong
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
*** Alternative regimen (1): [[Cortisone acetate]] 20-25mg PO, once in the morning, lifelong.  
 
*** Alternative regimen (2): [[Prednisone]] 3-5mg/day PO, once in the morning lifelong.
OR
'''Specific instructions:'''<br>
 
[[Drug interactions]]:<br>
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
Drugs increasing glucocorticoid dose requirement: Anticonvulsants like Barbiturates, Topiramate, Anti-tubercular drugs, Estrogens, Tamoxifen<br>
 
Drugs decreasing glucocorticoid dose requirement: Licorice, Grapefruit juice, Colestipol<br>
OR
Monitoring:<br>
 
Monitoring is done based on clinical improvement.<br>
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
Symptoms that are suggestive of under-dosing: Persistence or the incomplete resolution of fatigue, nausea, postural hypotension, myalgia.<br>
 
Symptoms that are suggestive of overdosing: weight gain, edema, abdominal striae.<br>
==Medical Therapy==
There is no role of measuring ACTH and serum cortisol levels.<br>
*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].  
Side effects of glucocorticoid replacement therapy:<br>
*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
Weight gain, edema, increased appetite, weight gain, osteoporosis, dyslipidemia, increased cardiovascular risk.
*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
* '''Glucocorticoid replacement therapy'''
*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
** 1.2 '''Pediatric'''
===Disease Name===
*** Preferred regimen: [[Hydrocortisone]] 7-10 mg/m2/day PO in three or four divided doses.
'''Specific instructions: <br>
Avoid using prednisolone, hydrocortisone in children <br>
Monitoring: Clinical improvement, tracking growth velocity, body weight, blood pressure, energy levels.


* '''1 Stage 1 - Name of stage'''
*'''Mineralocorticoid replacement therapy'''
** 1.1 '''Specific Organ system involved 1'''
**2.1 Adult
*** 1.1.1 '''Adult'''
***Preferred regimen: [[9 α-fludrocortisone]] 0.05-0.2 mg/day PO single dose early morning.
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)''' 
'''Specific instructions: <br>
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
Monitoring: <br>
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
Based on the clinical picture
**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
Symptoms and signs that are suggestive of under-dosing: [[hypovolemia]], [[orthostatic hypotension]], [[hyperkalemia]], [[hyperuricemia]], increased plasma renin activity.<br>
**** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
Symptoms and signs of overdosing: [[hypertension]], [[hypokalemia]], [[edema]]. If a patient on [[fludrocortisone]] develops hypertension first step is to reduce the dose. If the blood pressure remains elevated, start an [[antihypertensive]] medication, and continue [[fludrocortisone]].
**** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
*'''Mineralocorticoid replacement therapy'''
*** 1.1.2 '''Pediatric'''
**2.2 Pediatric
**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
***Preferred regimen: [[9 α-fludrocortisone]] 0.05-0.2 mg/day PO single dose early morning.
***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose) 
'''Specific instructions:'''<br>
***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
In newborns and children, higher dose upto 0.5mg of [[fludrocortisone]] maybe required because of lower mineralocorticoid sensitivity. <ref name="EspositoPasquali2018">{{cite journal|last1=Esposito|first1=Daniela|last2=Pasquali|first2=Daniela|last3=Johannsson|first3=Gudmundur|title=Primary Adrenal Insufficiency: Managing Mineralocorticoid Replacement Therapy|journal=The Journal of Clinical Endocrinology & Metabolism|volume=103|issue=2|year=2018|pages=376–387|issn=0021-972X|doi=10.1210/jc.2017-01928}}</ref>
***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
****1.1.2.2 (Specific population e.g. '<nowiki/>'''''children < 8 years of age'''''')
***** Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose) 
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
** 1.2 '''Specific Organ system involved 2'''
*** 1.2.1 '''Adult'''
**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
*** 1.2.2  '''Pediatric'''
**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)


* 2 '''Stage 2 - Name of stage'''
*'''Dehydroepiandrosterone (DHEA):''' <br>
** 2.1 '''Specific Organ system involved 1 '''
Its use is not routinely recommended.<br>
**: '''Note (1):'''
A six-month trial of DHEA can be considered in patients with significant impairment in quality of life, decreased libido, women, depressed mood despite glucocorticoid and mineralocorticoid therapy. If there is no improvement at the end of six months, its use has to be discontinued.  
**: '''Note (2)''':
Contraindications: People with breast and prostate cancer.
**: '''Note (3):'''
*** 2.1.1 '''Adult'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
*** 2.1.2 '''Pediatric'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '<nowiki/>'''''(Contraindications/specific instructions)''''''
**** Oral regimen
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
** 2.2  '<nowiki/>'''''Other Organ system involved 2''''''
**: '''Note (1):'''
**: '''Note (2)''':
**: '''Note (3):'''
*** 2.2.1 '''Adult'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
*** 2.2.2 '''Pediatric'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
**** Oral regimen
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)


==References==
==References==

Latest revision as of 20:19, 5 November 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ayeesha Kattubadi, M.B.B.S[2]

Overview

Pharmacologic medical therapy is recommended among patients with adrenal insufficiency Pharmacologic medical therapies for adrenal insufficiency include lifelong glucocorticoid and mineralocorticoid replacement therapy. Mineralocorticoid replacement therapy is only used in patients with primary adrenal insufficiency.

Medical Therapy


Adrenal insufficiency

  • Glucocorticoid replacement therapy
    • 1.1 Adult
      • Preferred regimen (1): Hydrocortisone 15-25mg PO, 2/3 of the total dose in the morning on awakening; 1/3 of the total dose in mid-afternoon lifelong
      • Preferred regimen (2): Hydrocortisone 15-25mg PO, three divided doses (10mg at 7 AM, 5mg at 12 PM, and 2.5-5mg at 4:30 PM. Avoid dosing after 6 PM) lifelong
      • Alternative regimen (1): Cortisone acetate 20-25mg PO, once in the morning, lifelong.
      • Alternative regimen (2): Prednisone 3-5mg/day PO, once in the morning lifelong.

Specific instructions:
Drug interactions:
Drugs increasing glucocorticoid dose requirement: Anticonvulsants like Barbiturates, Topiramate, Anti-tubercular drugs, Estrogens, Tamoxifen
Drugs decreasing glucocorticoid dose requirement: Licorice, Grapefruit juice, Colestipol
Monitoring:
Monitoring is done based on clinical improvement.
Symptoms that are suggestive of under-dosing: Persistence or the incomplete resolution of fatigue, nausea, postural hypotension, myalgia.
Symptoms that are suggestive of overdosing: weight gain, edema, abdominal striae.
There is no role of measuring ACTH and serum cortisol levels.
Side effects of glucocorticoid replacement therapy:
Weight gain, edema, increased appetite, weight gain, osteoporosis, dyslipidemia, increased cardiovascular risk.

  • Glucocorticoid replacement therapy
    • 1.2 Pediatric
      • Preferred regimen: Hydrocortisone 7-10 mg/m2/day PO in three or four divided doses.

Specific instructions:
Avoid using prednisolone, hydrocortisone in children
Monitoring: Clinical improvement, tracking growth velocity, body weight, blood pressure, energy levels.

  • Mineralocorticoid replacement therapy

Specific instructions:
Monitoring:
Based on the clinical picture Symptoms and signs that are suggestive of under-dosing: hypovolemia, orthostatic hypotension, hyperkalemia, hyperuricemia, increased plasma renin activity.
Symptoms and signs of overdosing: hypertension, hypokalemia, edema. If a patient on fludrocortisone develops hypertension first step is to reduce the dose. If the blood pressure remains elevated, start an antihypertensive medication, and continue fludrocortisone.

  • Mineralocorticoid replacement therapy

Specific instructions:
In newborns and children, higher dose upto 0.5mg of fludrocortisone maybe required because of lower mineralocorticoid sensitivity. [4]

  • Dehydroepiandrosterone (DHEA):

Its use is not routinely recommended.
A six-month trial of DHEA can be considered in patients with significant impairment in quality of life, decreased libido, women, depressed mood despite glucocorticoid and mineralocorticoid therapy. If there is no improvement at the end of six months, its use has to be discontinued. Contraindications: People with breast and prostate cancer.

References

  1. ↑ Bornstein, Stefan R.; Allolio, Bruno; Arlt, Wiebke; Barthel, Andreas; Don-Wauchope, Andrew; Hammer, Gary D.; Husebye, Eystein S.; Merke, Deborah P.; Murad, M. Hassan; Stratakis, Constantine A.; Torpy, David J. (2016). "Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline". The Journal of Clinical Endocrinology & Metabolism. 101 (2): 364–389. doi:10.1210/jc.2015-1710. ISSN 0021-972X.
  2. ↑ Oprea, Alina; Bonnet, Nicolas C. G.; Pollé, Olivier; Lysy, Philippe A. (2019). "Novel insights into glucocorticoid replacement therapy for pediatric and adult adrenal insufficiency". Therapeutic Advances in Endocrinology and Metabolism. 10: 204201881882129. doi:10.1177/2042018818821294. ISSN 2042-0188.
  3. ↑ https://www.elsevier.es/index.php?p=revista&pRevista=pdf-simple&pii=S2173509314700698
  4. ↑ Esposito, Daniela; Pasquali, Daniela; Johannsson, Gudmundur (2018). "Primary Adrenal Insufficiency: Managing Mineralocorticoid Replacement Therapy". The Journal of Clinical Endocrinology & Metabolism. 103 (2): 376–387. doi:10.1210/jc.2017-01928. ISSN 0021-972X.

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