Fanconi syndrome medical therapy: Difference between revisions

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__NOTOC__
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{{Fanconi syndrome}}
{{Fanconi syndrome}}
{{CMG}}; {{AE}}
Tyrosinemia{{CMG}}; {{AE}}


==Overview==
==Overview==
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
Definitive treatment of Fanconi syndrome in most of the cases is the treatment of the underlying cause( which sometimes is not practical)  and resolving the exposure to the contributor compounds(In exogenous causes/Tyrosinemia/ Galactosemia/ Hereditary fructose intolerance).


OR
Temporary treatment involves replacement therapy which is the current mainstay of therapy. Replacement therapy regimen is depended on the severity of disease and the extent of urinary loss of each ingredient and therefore varies substantially among individuals.


Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
Some of the most important concepts of cause-specific medical therapies is described below.  


OR
==Medical Therapy==
===Fanconi syndrome due to Cystinosis===


The majority of cases of [disease name] are self-limited and require only supportive care.
* 1.1.1 '''Adult and Pediatric'''


OR
* '''Cystine-lowering Agents'''
 
** Preferred regimen (1): Cysteamine 60-90 mg/kg/day q.i.d. every 6 h '''(specific instructions: <1 years: Safety and efficacy not established)''' 
[Disease name] is a medical emergency and requires prompt treatment.
*'''Chelating agents'''
 
**Preferred regimen (2): Penicillamine 30 mg/kg/day PO divided BID/QID
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'''
=== Fanconi syndrome due to Wilson disease ===
** 1.1 '''Specific Organ system involved 1'''
* 2.1.1 '''Adult and pediatrics'''
*** 1.1.1 '''Adult'''
* '''Removal of the copper:'''
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)''' 
** Preferred regimen (1): D-penicillamine 20 mg/kg PO q12h
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
** Alterantive regimen (1):  Trientine hydrochloride 500 to 750 mg PO q12h/q6h
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
* '''Preventing reaccumulation:'''
**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
** Preferred regimen (1): Zinc acetate PO 50 mg q8h
**** 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)
** 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'''
=== Fanconi syndrome due to Tyrosinemia ===
** 2.1 '''Specific Organ system involved 1 '''
* 2.1.1 '''Adult and pediatrics'''
**: '''Note (1):'''
*  Preferred regimen (1): NTBC 1 mg/kg/day
**: '''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==
==References==

Revision as of 10:13, 13 June 2018

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

Overview

Definitive treatment of Fanconi syndrome in most of the cases is the treatment of the underlying cause( which sometimes is not practical) and resolving the exposure to the contributor compounds(In exogenous causes/Tyrosinemia/ Galactosemia/ Hereditary fructose intolerance).

Temporary treatment involves replacement therapy which is the current mainstay of therapy. Replacement therapy regimen is depended on the severity of disease and the extent of urinary loss of each ingredient and therefore varies substantially among individuals.

Some of the most important concepts of cause-specific medical therapies is described below.

Medical Therapy

Fanconi syndrome due to Cystinosis

  • 1.1.1 Adult and Pediatric
  • Cystine-lowering Agents
    • Preferred regimen (1): Cysteamine 60-90 mg/kg/day q.i.d. every 6 h (specific instructions: <1 years: Safety and efficacy not established)
  • Chelating agents
    • Preferred regimen (2): Penicillamine 30 mg/kg/day PO divided BID/QID

Fanconi syndrome due to Wilson disease

  • 2.1.1 Adult and pediatrics
  • Removal of the copper:
    • Preferred regimen (1): D-penicillamine 20 mg/kg PO q12h
    • Alterantive regimen (1):  Trientine hydrochloride 500 to 750 mg PO q12h/q6h
  • Preventing reaccumulation:
    • Preferred regimen (1): Zinc acetate PO 50 mg q8h

Fanconi syndrome due to Tyrosinemia

  • 2.1.1 Adult and pediatrics
  •  Preferred regimen (1): NTBC 1 mg/kg/day

References

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