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==Medical Therapy==
==Medical Therapy==
*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].  
*Pharmacologic medical therapy is recommended among patients with [[adrenal insufficiency]].
*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [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>
*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
===Disease Name===


* '''1 Stage 1 - Name of stage'''
** 1.1 '''Specific Organ system involved 1'''
*** 1.1.1 '''Adult'''
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)''' 
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 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
*** 1.1.2 '''Pediatric'''
**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose) 
***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 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)
****1.1.2.2 (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'''
===Adrenal insufficiency===
** 2.1 '''Specific Organ system involved 1 '''
 
**: '''Note (1):'''  
* '''Glucocorticoid replacement therapy'''
**: '''Note (2)''':
** 1.1 '''Adult'''
**: '''Note (3):'''
*** 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
*** 2.1.1 '''Adult'''
*** 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
**** Parenteral regimen
*** Alternative regimen (1): [[Cortisone acetate]] 20-25mg PO, once in the morning, lifelong.
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
*** Alternative regimen (2): [[Prednisone]] 3-5mg/day PO, once in the morning lifelong.
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
'''Specific instructions:'''<br>
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
[[Drug interactions]]:<br>
**** Oral regimen
Drugs increasing glucocorticoid dose requirement: Anticonvulsants like Barbiturates, Topiramate, Anti-tubercular drugs, Estrogens, Tamoxifen<br>
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
Drugs decreasing glucocorticoid dose requirement: Licorice, Grapefruit juice, Colestipol<br>
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
Monitoring:<br>
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
Monitoring is done based on clinical improvement.<br>
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
Symptoms that are suggestive of under-dosing: Persistence or the incomplete resolution of fatigue, nausea, postural hypotension, myalgia.<br>
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
Symptoms that are suggestive of overdosing: weight gain, edema, abdominal striae.<br>
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
There is no role of measuring ACTH and serum cortisol levels.<br>
*** 2.1.2 '''Pediatric'''
Side effects of glucocorticoid replacement therapy:<br>
**** Parenteral regimen
Weight gain, edema, increased appetite, weight gain, osteoporosis, dyslipidemia, increased cardiovascular risk.
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
* '''Glucocorticoid replacement therapy'''
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
** 1.2 '''Pediatric'''
***** 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)''''''
*** Preferred regimen: [[Hydrocortisone]] 7-10 mg/m2/day PO in three or four divided doses.
**** Oral regimen
'''Specific instructions: <br>
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
Avoid using prednisolone, hydrocortisone in children <br>
***** 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)
Monitoring: Clinical improvement, tracking growth velocity, body weight, blood pressure, energy levels.
***** 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)
*'''Mineralocorticoid replacement therapy'''
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
**2.1 Adult
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
***Preferred regimen: [[9 α-fludrocortisone]] 0.05-0.2 mg/day PO single dose early morning.
** 2.2  '<nowiki/>'''''Other Organ system involved 2''''''
'''Specific instructions: <br>
**: '''Note (1):'''
Monitoring: <br>
**: '''Note (2)''':
Based on the clinical picture
**: '''Note (3):'''  
Symptoms and signs that are suggestive of under-dosing: [[hypovolemia]], [[orthostatic hypotension]], [[hyperkalemia]], [[hyperuricemia]], increased plasma renin activity.<br>
*** 2.2.1 '''Adult'''
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]].
**** Parenteral regimen
*'''Mineralocorticoid replacement therapy'''
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
**2.2 Pediatric
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
***Preferred regimen: [[9 α-fludrocortisone]] 0.05-0.2 mg/day PO single dose early morning.
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
'''Specific instructions:'''<br>
**** Oral regimen
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>
***** 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==

Revision as of 20:03, 5 November 2020

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

Overview

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

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

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

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]

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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