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{{Polycystic kidney disease}}
{{Polycystic kidney disease}}
{{CMG}} {{AE}}
{{CMG}}; {{AE}} {{MKA}}


==Overview==
==Overview==
==Treatment==
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.
Treatment of ADPKD is targeted at managing symptoms and disease complications. There have been no treatments associated with disease regression or slowing of cysts 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>


===Hypertension===
==Medical Therapy==
Up to 75% of patients with ADPKD on imaging without any renal insufficiency are hypertensive.<ref name="pmid2239929">{{cite journal| author=Gabow PA| title=Autosomal dominant polycystic kidney disease--more than a renal disease. | journal=Am J Kidney Dis | year= 1990 | volume= 16 | issue= 5 | pages= 403-13 | pmid=2239929 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2239929 }} </ref> Even in young patients, 50% of those aged 20-34 years are hypertensive despite normal renal function.<ref name="pmid15533729">{{cite journal| author=Kelleher CL, McFann KK, Johnson AM, Schrier RW| title=Characteristics of hypertension in young adults with autosomal dominant polycystic kidney disease compared with the general U.S. population. | journal=Am J Hypertens | year= 2004 | volume= 17 | issue= 11 Pt 1 | pages= 1029-34 | pmid=15533729 | doi=10.1016/j.amjhyper.2004.06.020 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15533729 }} </ref> Treatment is recommended when blood pressure exceeds 130/80 mm Hg for adults, or sex and age adjusted norms for children. ACE inhibitors and ARBs are highly recommended as observational studies have shown better preservation of renal function with these agents. Eventual resistance or blunting of the effects of the these agents with increased volume retention can be encountered. Salt restriction, with addition of thiazide diuretics initially or loop diuretics as a second line, is justified.<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>
*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>


===Hematuria===
===Polycystic kidney disease===
Gross hematuria with significant renal colic secondary to collecting system clots is not uncommon. Treatment usually involves analgesia, bed rest and hydration to increase urinary flow rate to 2-3 liters daily. In most patients, gross hematuria regresses to microscopic hematuria in a few days. Patients should be
advised to avoid sports with risk of abdominal trauma to prevent recurrent episodes.<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>


===Urinary Tract Infections===
* 1 '''Hypertension'''
 
** 1.1 '''Adult'''
===Pain===
*** Preferred regimen (1): [[Lisinopril]] 5-10 mg PO q24h '''(Maximum dose 40mg in 24h)'''<ref name="pmid19696226">{{cite journal |vauthors=Schrier RW |title=Renal volume, renin-angiotensin-aldosterone system, hypertension, and left ventricular hypertrophy in patients with autosomal dominant polycystic kidney disease |journal=J. Am. Soc. Nephrol. |volume=20 |issue=9 |pages=1888–93 |date=September 2009 |pmid=19696226 |doi=10.1681/ASN.2008080882 |url=}}</ref><ref name="pmid253997332">{{cite journal |vauthors=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 |title=Blood pressure in early autosomal dominant polycystic kidney disease |journal=N. Engl. J. Med. |volume=371 |issue=24 |pages=2255–66 |date=December 2014 |pmid=25399733 |pmc=4343258 |doi=10.1056/NEJMoa1402685 |url=}}</ref> 
 
*** Alternative regimen (1): [[Telmisartan]] 20-40 mg PO q24h '''(Maximum dose 80mg in 24h)'''<ref name="pmid25399733">{{cite journal |vauthors=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 |title=Blood pressure in early autosomal dominant polycystic kidney disease |journal=N. Engl. J. Med. |volume=371 |issue=24 |pages=2255–66 |date=December 2014 |pmid=25399733 |pmc=4343258 |doi=10.1056/NEJMoa1402685 |url=}}</ref> 
===Kidney Stones===
* 2 '''Slowing disease progression (Vasopressin receptor antagonist)'''
 
*: '''Note (1):'''  Before initiating therapy fluid intake needs to be restricted for 10-14 hours.
===Renal Failure===
** 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> 
***: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]]<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>
** [[Hydration]]
** [[Bed rest]]
* 4 '''Dietary sodium restriction'''
** 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>
** 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>


==References==
==References==
{{reflist|2}}
{{Reflist|2}}


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Latest revision as of 23:46, 29 July 2020

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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

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
  • 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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