Neurofibromatosis type 1 medical therapy: Difference between revisions

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__NOTOC__
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{{Neurofibromatosis type 1}}
{{Neurofibromatosis type 1}}
{{CMG}}; {{AE}}
{{CMG}}; {{AE}}[[User:MoisesRomo|Moises Romo M.D.]]


==Overview==
==Overview==
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
There is no treatment for [[neurofibromatosis type 1]]; the mainstay of therapy is supportive care. [[Therapy]] for a patient with [[neurofibromatosis type 1]] is aimed at decreasing [[symptoms]] and improving [[quality of life]]. At every [[age]], different problems may develop, so a specific approach is necessary for the age and presentation of the individual. Supportive therapy for [[neurofibromatosis type 1]] includes pain relief, [[psychotherapy]], and [[antidepressants]].


OR
<br />
 
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==
==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 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'''
*At every age, different problems may appear, so a specific approach is necessary for the age and presentation of the individual.<ref name=":0">{{cite journal|doi=10.1016/B978-0-444-62702-5.00004-4.}}</ref>
** 2.1 '''Specific Organ system involved 1 '''
*Supportive therapy for [[neurofibromatosis type 1]] includes [[pain]] relief, [[psychotherapy]], and [[antidepressants]].<ref name="ListernickCharrow1989">{{cite journal|last1=Listernick|first1=Robert|last2=Charrow|first2=Joel|last3=Greenwald|first3=Mark J.|last4=Esterly|first4=Nancy B.|title=Optic gliomas in children with neurofibromatosis type 1|journal=The Journal of Pediatrics|volume=114|issue=5|year=1989|pages=788–792|issn=00223476|doi=10.1016/S0022-3476(89)80137-4}}</ref>
**: '''Note (1):'''
*[[Stimulant]] [[medications]] should be considered for patients with [[Attention deficit hyperactivity disorder|attention deficitis]] (ADHD).<ref name="MautnerKluwe2010">{{cite journal|last1=Mautner|first1=V.-F.|last2=Kluwe|first2=L.|last3=Friedrich|first3=R. E.|last4=Roehl|first4=A. C.|last5=Bammert|first5=S.|last6=Hogel|first6=J.|last7=Spori|first7=H.|last8=Cooper|first8=D. N.|last9=Kehrer-Sawatzki|first9=H.|title=Clinical characterisation of 29 neurofibromatosis type-1 patients with molecularly ascertained 1.4 Mb type-1 NF1 deletions|journal=Journal of Medical Genetics|volume=47|issue=9|year=2010|pages=623–630|issn=0022-2593|doi=10.1136/jmg.2009.075937}}</ref>
**: '''Note (2)''':
*[[Ketotifen]] ([[mast cell]] blocker that produces its [[depigmentation]]) has been proposed to treat some of the [[symptoms]] of [[pruritus]], [[pain]], and [[muscle weakness]] associated with [[neurofibromatosis type 1]]. The dose of [[ketotifen]] is 2 to 4 milligrams daily.<ref name="pmid8481017">{{cite journal |vauthors=Riccardi VM |title=A controlled multiphase trial of ketotifen to minimize neurofibroma-associated pain and itching |journal=Arch Dermatol |volume=129 |issue=5 |pages=577–81 |date=May 1993 |pmid=8481017 |doi= |url=}}</ref><ref name="urlscielo.isciii.es">{{cite web |url=http://scielo.isciii.es/pdf/odonto/v21n5/original1.pdf |title=scielo.isciii.es |format= |work= |accessdate=}}</ref>
**: '''Note (3):'''
*[[Chemotherapy]] may be necessary for certain [[tumors]].<ref name=":0" /> Carboplatin-based [[chemotherapy]] combined with [[vincristine]], is the preferred therapy for [[neurofibromatosis type 1]] associated low-grade [[gliomas]].<ref name="pmid26235348">{{cite journal |vauthors=Dodgshun AJ, Maixner WJ, Heath JA, Sullivan MJ, Hansford JR |title=Single agent carboplatin for pediatric low-grade glioma: A retrospective analysis shows equivalent efficacy to multiagent chemotherapy |journal=Int. J. Cancer |volume=138 |issue=2 |pages=481–8 |date=January 2016 |pmid=26235348 |doi=10.1002/ijc.29711 |url=}}</ref>
*** 2.1.1 '''Adult'''
*[[Bevacizumab]] has demonstrated good response on vision testing in cases of refractory [[optic pathway glioma]].<ref name="pmid19165892">{{cite journal |vauthors=Packer RJ, Jakacki R, Horn M, Rood B, Vezina G, MacDonald T, Fisher MJ, Cohen B |title=Objective response of multiply recurrent low-grade gliomas to bevacizumab and irinotecan |journal=Pediatr Blood Cancer |volume=52 |issue=7 |pages=791–5 |date=July 2009 |pmid=19165892 |doi=10.1002/pbc.21935 |url=}}</ref>
**** Parenteral regimen
*[[Interferon gamma]] has been proposed for cases of patients with [[Von Recklinghausen's Disease|Von Recklinghausen]] which have intractable or inoperable neurofibromas.<ref name="pmid12692353">{{cite journal |vauthors=Nakayama J, Tanaka T, Arakawa F, Terao H, Shimura H, Ikeda S, Kuroki M |title=Gamma interferon gene transfection efficiently inhibits proliferation of neurofibroma cell lines in vitro |journal=J. Dermatol. |volume=30 |issue=3 |pages=181–8 |date=March 2003 |pmid=12692353 |doi=10.1111/j.1346-8138.2003.tb00369.x |url=}}</ref><ref name="urlscielo.isciii.es" />
***** 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)


<br />
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}

Latest revision as of 16:42, 1 September 2020

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

Overview

There is no treatment for neurofibromatosis type 1; the mainstay of therapy is supportive care. Therapy for a patient with neurofibromatosis type 1 is aimed at decreasing symptoms and improving quality of life. At every age, different problems may develop, so a specific approach is necessary for the age and presentation of the individual. Supportive therapy for neurofibromatosis type 1 includes pain relief, psychotherapy, and antidepressants.


Medical Therapy


References

  1. ↑ 1.0 1.1 . doi:10.1016/B978-0-444-62702-5.00004-4. Check |doi= value (help). Missing or empty |title= (help)
  2. ↑ Listernick, Robert; Charrow, Joel; Greenwald, Mark J.; Esterly, Nancy B. (1989). "Optic gliomas in children with neurofibromatosis type 1". The Journal of Pediatrics. 114 (5): 788–792. doi:10.1016/S0022-3476(89)80137-4. ISSN 0022-3476.
  3. ↑ Mautner, V.-F.; Kluwe, L.; Friedrich, R. E.; Roehl, A. C.; Bammert, S.; Hogel, J.; Spori, H.; Cooper, D. N.; Kehrer-Sawatzki, H. (2010). "Clinical characterisation of 29 neurofibromatosis type-1 patients with molecularly ascertained 1.4 Mb type-1 NF1 deletions". Journal of Medical Genetics. 47 (9): 623–630. doi:10.1136/jmg.2009.075937. ISSN 0022-2593.
  4. ↑ Riccardi VM (May 1993). "A controlled multiphase trial of ketotifen to minimize neurofibroma-associated pain and itching". Arch Dermatol. 129 (5): 577–81. PMID 8481017.
  5. ↑ 5.0 5.1 "scielo.isciii.es" (PDF).
  6. ↑ Dodgshun AJ, Maixner WJ, Heath JA, Sullivan MJ, Hansford JR (January 2016). "Single agent carboplatin for pediatric low-grade glioma: A retrospective analysis shows equivalent efficacy to multiagent chemotherapy". Int. J. Cancer. 138 (2): 481–8. doi:10.1002/ijc.29711. PMID 26235348.
  7. ↑ Packer RJ, Jakacki R, Horn M, Rood B, Vezina G, MacDonald T, Fisher MJ, Cohen B (July 2009). "Objective response of multiply recurrent low-grade gliomas to bevacizumab and irinotecan". Pediatr Blood Cancer. 52 (7): 791–5. doi:10.1002/pbc.21935. PMID 19165892.
  8. ↑ Nakayama J, Tanaka T, Arakawa F, Terao H, Shimura H, Ikeda S, Kuroki M (March 2003). "Gamma interferon gene transfection efficiently inhibits proliferation of neurofibroma cell lines in vitro". J. Dermatol. 30 (3): 181–8. doi:10.1111/j.1346-8138.2003.tb00369.x. PMID 12692353.

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