Primary biliary cirrhosis medical therapy: Difference between revisions

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Revision as of 21:25, 11 August 2017

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

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
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 name10 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. '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)
- 2.1 Specific Organ system involved 2
  - 2.1.1 Adult
    - Preferred regimen (1): drug name 500 mg PO q8h
  - 2.1.2 Pediatric
    - Preferred regimen (1): drug name 50 mg/kg/day PO q8h (maximum, 500 mg per dose)

2 Stage 2 - Name of stage
- 2.1 Specific Organ system involved 1
  Note (1):
  
  Note (2):
  
  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) '(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 '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

Template:WH Template:WS

@@ Line 5: / Line 5: @@
 ==Overview==
+There is no treatment for [disease name]; the mainstay of therapy is supportive care.
-==Medical Therapy==
+OR
-There is no known cure, but medication may slow the progression so that a normal lifespan and quality of life may be attainable for many patients. Specific treatment for fatigue, which may be debilitating in some patients, is limited and currently undergoing trials.
+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
-* Ursodeoxycholic acid ([[Ursodiol]]) is the most frequently used treatment. This helps reduce the cholestasis and improves blood test results ([[liver function tests]]). It has a minimal effect on symptoms and whether it improves prognosis is controversial.
+Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
-* To relieve itching caused by bile acids in circulation, which would normally be removed by the liver, [[cholestyramine]] (a [[bile acid sequestrant]]) may be prescribed to absorb bile acids in the gut and be eliminated, rather than re-enter the blood stream. Alternative agents include [[naltrexone]] and [[rifampicin]].
+OR
-* To relieve fatigue associated with primary biliary cirrhosis, current studies indicate that Provigil (modafinil) may be effective without damaging the liver.<ref>[[Modafinil#Primary_biliary_cirrhosis]]<br/>{{cite journal |author=Ian Gan S, de Jongh M, Kaplan MM |title=Modafinil in the treatment of debilitating fatigue in primary biliary cirrhosis: a clinical experience |journal=Dig. Dis. Sci. |volume=54 |issue=10 |pages=2242–6 |year=2009 |month=October |pmid=19082890 |doi=10.1007/s10620-008-0613-3 |url=http://www.springerlink.com/content/f0207x6110847113/}}<br/>{{cite journal |author=Kumagi T, Heathcote EJ |title=Primary biliary cirrhosis |journal=Orphanet J Rare Dis |volume=3 |pages=1 |year=2008 |pmid=18215315 |pmc=2266722 |doi=10.1186/1750-1172-3-1 |url=http://www.ojrd.com/content/3//1 |quote=[http://www.ojrd.com/content/3/1/1#B157 Ref 157 viz:]}}<br/>{{cite journal |author=Jones DE, Newton JL |title=An open study of modafinil for the treatment of daytime somnolence and fatigue in primary biliary cirrhosis |journal=Aliment. Pharmacol. Ther. |volume=25 |issue=4 |pages=471–6 |year=2007 |month=February |pmid=17270003 |doi=10.1111/j.1365-2036.2006.03223.x |url=http://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0269-2813&date=2007&volume=25&issue=4&spage=471}}</ref>  Though off-patent, the limiting factor in the use of modafinil in the U.S. is cost.   The manufacturer, Cephalon, has made agreements with manufacturers of generic modafinil to provide payments in exchange for delaying their sale of modafinil.<ref>[[Modafinil#Patent_protection_and_antitrust_litigation]]<br/>{{cite journal |author=Carrier MA |title=Provigil: A Case Study of Anticompetitive Behavior |journal=Hastings Science & Technology Law Journal |volume=3 |issue=2 |pages=441–452 |year=2011 |url=http://hstlj.org/content/vol3/iss2/v3i2carrier.pdf |format=PDF}}</ref>  The FTC has filed suit against Cephalon alleging anti-competitive behavior.<ref>http://www.ftc.gov/os/caselist/0610182/080213complaint.pdf</ref>
+Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
-* Patients with PBC have poor lipid-dependent absorption of vitamins A, D, E, K.<ref name="BaconO'Grady2006">{{cite book|author1=Bruce R. Bacon|author2=John G. O'Grady|title=Comprehensive clinical hepatology|url=http://books.google.com/?id=ec0G9HGiR8MC&pg=PA283|accessdate=29 June 2010|year=2006|publisher=Elsevier Health Sciences|isbn=978-0-323-03675-7|pages=283–}}</ref> Appropriate supplementation is recommended when bilirubin is elevated.<ref>{{cite journal|last=Lindor|first=KD|coauthors=Gershwin, ME; Poupon, R; Kaplan, M; Bergasa, NV; Heathcote, EJ; American Association for Study of Liver, Diseases|title=Primary biliary cirrhosis.|journal=Hepatology (Baltimore, Md.)|date=2009 Jul|volume=50|issue=1|pages=291-308|pmid=19554543|doi=10.1002/hep.22906}}</ref> Multivitamins (esp. vitamin D) and calcium are also recommended.
+OR
-* Patients with PBC are at elevated risk of developing [[osteoporosis]]<ref>{{cite journal|last=Collier|first=Jane|title=Guidelines on the management of osteoporosis associated with chronic liver disease|journal=Gut|year=2002|volume=50|pages=i1-i9|pmid=11788576|accessdate=14 June 2012|pmc=1867644}}</ref> and [[esophageal varices]]<ref>{{cite journal|last=Ali|first=AH|coauthors=Sinakos, E; Silveira, MG; Jorgensen, RA; Angulo, P; Lindor, KD|title=Varices in early histological stage primary biliary cirrhosis.|journal=Journal of Clinical Gastroenterology|date=2011 Aug|volume=45|issue=7|pages=e66-71|pmid=20856137|doi=10.1097/MCG.0b013e3181f18c4e}}</ref>  as compared to the general population and others with liver disease. Screening and treatment of these complications is an important part of the management of PBC.
+Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
-*As in all liver diseases, [[alcoholic beverage]]s are contraindicated.
+OR
-As in all liver diseases, excessive consumption of [[alcohol]] is contraindicated.
+Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
-===Medications to avoide===
+==Medical Therapy==
+*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
+*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
+*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===
-{{MedCondContrPI
+* '''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. ''''''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)
+** 2.1 '''Specific Organ system involved 2'''
+*** 2.1.1 '''Adult'''
+**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
+*** 2.1.2  '''Pediatric'''
+**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)
-|MedCond =Primary biliary cirrhosis|Fenofibrate|Gemfibrozil}}
+* 2 '''Stage 2 - Name of stage'''
+** 2.1 '''Specific Organ system involved 1 '''
+**: '''Note (1):'''
+**: '''Note (2)''':
+**: '''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) ''''''(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==
-{{reflist|2}}
+{{Reflist|2}}
-[[Category:Gastroenterology]]
-[[Category:Hepatology]]
-[[Category:Disease]]
 {{WH}}
 {{WS}}