Polycystic kidney disease medical therapy: Difference between revisions

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==Overview==
==Overview==
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
Patients with polycystic kidney disease are treated with lisinopril or telmisartan for control of hypertension, tolvaptan for slowing disease progression, hydration, analgesia and bed rest. The treatment is targeted more towards managing symptoms and disease complications rather than slowing cyst formation.


OR
==Medical Therapy==
 
*Patients with polycystic kidney disease are treated with lisinopril or telmisartan for control of hypertension, tolvaptan for slowing disease progression, hydration, analgesia and bed rest. The treatment is targeted more towards managing symptoms and disease complications rather than slowing cyst formation.<ref name="pmid18832246">{{cite journal| author=Grantham JJ| title=Clinical practice. Autosomal dominant polycystic kidney disease. | journal=N Engl J Med | year= 2008 | volume= 359 | issue= 14 | pages= 1477-85 | pmid=18832246 | doi=10.1056/NEJMcp0804458 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18832246  }} </ref>
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].
===Polycystic kidney disease===
===Polycystic kidney disease===


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** 2.1 '''Adult'''
** 2.1 '''Adult'''
*** Preferred regimen (1): Tolvaptan 60mg PO q24h for 7 days '''(45mg upon awakening and 15mg 8hrs later)'''<ref name="pmid23121377">{{cite journal |vauthors=Torres VE, Chapman AB, Devuyst O, Gansevoort RT, Grantham JJ, Higashihara E, Perrone RD, Krasa HB, Ouyang J, Czerwiec FS |title=Tolvaptan in patients with autosomal dominant polycystic kidney disease |journal=N. Engl. J. Med. |volume=367 |issue=25 |pages=2407–18 |date=December 2012 |pmid=23121377 |pmc=3760207 |doi=10.1056/NEJMoa1205511 |url=}}</ref><ref name="pmid21903984">{{cite journal |vauthors=Higashihara E, Torres VE, Chapman AB, Grantham JJ, Bae K, Watnick TJ, Horie S, Nutahara K, Ouyang J, Krasa HB, Czerwiec FS |title=Tolvaptan in autosomal dominant polycystic kidney disease: three years' experience |journal=Clin J Am Soc Nephrol |volume=6 |issue=10 |pages=2499–507 |date=October 2011 |pmid=21903984 |pmc=3359559 |doi=10.2215/CJN.03530411 |url=}}</ref>   
*** Preferred regimen (1): Tolvaptan 60mg PO q24h for 7 days '''(45mg upon awakening and 15mg 8hrs later)'''<ref name="pmid23121377">{{cite journal |vauthors=Torres VE, Chapman AB, Devuyst O, Gansevoort RT, Grantham JJ, Higashihara E, Perrone RD, Krasa HB, Ouyang J, Czerwiec FS |title=Tolvaptan in patients with autosomal dominant polycystic kidney disease |journal=N. Engl. J. Med. |volume=367 |issue=25 |pages=2407–18 |date=December 2012 |pmid=23121377 |pmc=3760207 |doi=10.1056/NEJMoa1205511 |url=}}</ref><ref name="pmid21903984">{{cite journal |vauthors=Higashihara E, Torres VE, Chapman AB, Grantham JJ, Bae K, Watnick TJ, Horie S, Nutahara K, Ouyang J, Krasa HB, Czerwiec FS |title=Tolvaptan in autosomal dominant polycystic kidney disease: three years' experience |journal=Clin J Am Soc Nephrol |volume=6 |issue=10 |pages=2499–507 |date=October 2011 |pmid=21903984 |pmc=3359559 |doi=10.2215/CJN.03530411 |url=}}</ref>   
***:After 7 days increase to 90mg PO for 7days '''(60mg upon awakening and 30mg 8hrs later)'''
*****:After 7 days increase to 90mg PO for 7days '''(60mg upon awakening and 30mg 8hrs later)'''
***:After 7 days increase to 120mg PO '''(90mg upon awakening and 30mg 8hrs later)'''
*****:After 7 days increase to 120mg PO '''(90mg upon awakening and 30mg 8hrs later)'''
*  
* 3 '''Hematuria'''
* 2 '''Stage 2 - Name of stage'''
*** Pain management<ref name="pmid18832246">{{cite journal| author=Grantham JJ| title=Clinical practice. Autosomal dominant polycystic kidney disease. | journal=N Engl J Med | year= 2008 | volume= 359 | issue= 14 | pages= 1477-85 | pmid=18832246 | doi=10.1056/NEJMcp0804458 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18832246 }} </ref>
** 2.1 '''Specific Organ system involved 1 '''
*** Hydration
**: '''Note (1):'''
*** Bed rest
**: '''Note (2)''':
* 4 '''Dietary sodium restriction'''
**: '''Note (3):'''
** Restrict dietary sodium intake to a maximum of 2 grams per day<ref name="pmid21088290">{{cite journal |vauthors=Torres VE, Grantham JJ, Chapman AB, Mrug M, Bae KT, King BF, Wetzel LH, Martin D, Lockhart ME, Bennett WM, Moxey-Mims M, Abebe KZ, Lin Y, Bost JE |title=Potentially modifiable factors affecting the progression of autosomal dominant polycystic kidney disease |journal=Clin J Am Soc Nephrol |volume=6 |issue=3 |pages=640–7 |date=March 2011 |pmid=21088290 |pmc=3082424 |doi=10.2215/CJN.03250410 |url=}}</ref>
*** 2.1.1 '''Adult'''
** This slows progression of disease and helps with the control of hypertension as well<ref name="pmid27993381">{{cite journal |vauthors=Torres VE, Abebe KZ, Schrier RW, Perrone RD, Chapman AB, Yu AS, Braun WE, Steinman TI, Brosnahan G, Hogan MC, Rahbari FF, Grantham JJ, Bae KT, Moore CG, Flessner MF |title=Dietary salt restriction is beneficial to the management of autosomal dominant polycystic kidney disease |journal=Kidney Int. |volume=91 |issue=2 |pages=493–500 |date=February 2017 |pmid=27993381 |pmc=5237414 |doi=10.1016/j.kint.2016.10.018 |url=}}</ref>
**** 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==

Revision as of 18:40, 7 June 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [2]

Overview

Patients with polycystic kidney disease are treated with lisinopril or telmisartan for control of hypertension, tolvaptan for slowing disease progression, hydration, analgesia and bed rest. The treatment is targeted more towards managing symptoms and disease complications rather than slowing cyst formation.

Medical Therapy

  • Patients with polycystic kidney disease are treated with lisinopril or telmisartan for control of hypertension, tolvaptan for slowing disease progression, hydration, analgesia and bed rest. The treatment is targeted more towards managing symptoms and disease complications rather than slowing cyst formation.[1]

Polycystic kidney disease

  • 1 Hypertension
    • 1.1 Adult
      • Preferred regimen (1): Lisinopril 5-10 mg PO q24h (Maximum dose 40mg in 24h)[2][3]
      • Alternative regimen (1): Telmisartan 20-40 mg PO q24h (Maximum dose 80mg in 24h)[4]
  • 2 Slowing disease progression (Vasopressin receptor antagonist)
    Note (1): Before initiating therapy fluid intake needs to be restricted for 10-14 hours.
    • 2.1 Adult
      • Preferred regimen (1): Tolvaptan 60mg PO q24h for 7 days (45mg upon awakening and 15mg 8hrs later)[5][6]
          • After 7 days increase to 90mg PO for 7days (60mg upon awakening and 30mg 8hrs later)
            After 7 days increase to 120mg PO (90mg upon awakening and 30mg 8hrs later)
  • 3 Hematuria
      • Pain management[1]
      • Hydration
      • Bed rest
  • 4 Dietary sodium restriction
    • Restrict dietary sodium intake to a maximum of 2 grams per day[7]
    • This slows progression of disease and helps with the control of hypertension as well[8]

References

  1. ↑ 1.0 1.1 Grantham JJ (2008). "Clinical practice. Autosomal dominant polycystic kidney disease". N Engl J Med. 359 (14): 1477–85. doi:10.1056/NEJMcp0804458. PMID 18832246.
  2. ↑ Schrier RW (September 2009). "Renal volume, renin-angiotensin-aldosterone system, hypertension, and left ventricular hypertrophy in patients with autosomal dominant polycystic kidney disease". J. Am. Soc. Nephrol. 20 (9): 1888–93. doi:10.1681/ASN.2008080882. PMID 19696226.
  3. ↑ Schrier RW, Abebe KZ, Perrone RD, Torres VE, Braun WE, Steinman TI, Winklhofer FT, Brosnahan G, Czarnecki PG, Hogan MC, Miskulin DC, Rahbari-Oskoui FF, Grantham JJ, Harris PC, Flessner MF, Bae KT, Moore CG, Chapman AB (December 2014). "Blood pressure in early autosomal dominant polycystic kidney disease". N. Engl. J. Med. 371 (24): 2255–66. doi:10.1056/NEJMoa1402685. PMC 4343258. PMID 25399733.
  4. ↑ Schrier RW, Abebe KZ, Perrone RD, Torres VE, Braun WE, Steinman TI, Winklhofer FT, Brosnahan G, Czarnecki PG, Hogan MC, Miskulin DC, Rahbari-Oskoui FF, Grantham JJ, Harris PC, Flessner MF, Bae KT, Moore CG, Chapman AB (December 2014). "Blood pressure in early autosomal dominant polycystic kidney disease". N. Engl. J. Med. 371 (24): 2255–66. doi:10.1056/NEJMoa1402685. PMC 4343258. PMID 25399733.
  5. ↑ Torres VE, Chapman AB, Devuyst O, Gansevoort RT, Grantham JJ, Higashihara E, Perrone RD, Krasa HB, Ouyang J, Czerwiec FS (December 2012). "Tolvaptan in patients with autosomal dominant polycystic kidney disease". N. Engl. J. Med. 367 (25): 2407–18. doi:10.1056/NEJMoa1205511. PMC 3760207. PMID 23121377.
  6. ↑ Higashihara E, Torres VE, Chapman AB, Grantham JJ, Bae K, Watnick TJ, Horie S, Nutahara K, Ouyang J, Krasa HB, Czerwiec FS (October 2011). "Tolvaptan in autosomal dominant polycystic kidney disease: three years' experience". Clin J Am Soc Nephrol. 6 (10): 2499–507. doi:10.2215/CJN.03530411. PMC 3359559. PMID 21903984.
  7. ↑ Torres VE, Grantham JJ, Chapman AB, Mrug M, Bae KT, King BF, Wetzel LH, Martin D, Lockhart ME, Bennett WM, Moxey-Mims M, Abebe KZ, Lin Y, Bost JE (March 2011). "Potentially modifiable factors affecting the progression of autosomal dominant polycystic kidney disease". Clin J Am Soc Nephrol. 6 (3): 640–7. doi:10.2215/CJN.03250410. PMC 3082424. PMID 21088290.
  8. ↑ Torres VE, Abebe KZ, Schrier RW, Perrone RD, Chapman AB, Yu AS, Braun WE, Steinman TI, Brosnahan G, Hogan MC, Rahbari FF, Grantham JJ, Bae KT, Moore CG, Flessner MF (February 2017). "Dietary salt restriction is beneficial to the management of autosomal dominant polycystic kidney disease". Kidney Int. 91 (2): 493–500. doi:10.1016/j.kint.2016.10.018. PMC 5237414. PMID 27993381.

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