{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T04:10:04Z","timestamp":1790223004591,"version":"4.1.0"},"reference-count":45,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,5]]},"DOI":"10.2215\/cjn.02650313","type":"journal-article","created":{"date-parts":[[2014,4,11]],"date-time":"2014-04-11T00:24:21Z","timestamp":1397175861000},"page":"881-888","source":"Crossref","is-referenced-by-count":52,"title":["Low-Dose Rapamycin (Sirolimus) Effects in Autosomal Dominant Polycystic Kidney Disease"],"prefix":"10.2215","volume":"9","author":[{"given":"William E.","family":"Braun","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jesse D.","family":"Schold","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian R.","family":"Stephany","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rita A.","family":"Spirko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian R.","family":"Herts","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-10-20230601","doi-asserted-by":"crossref","first-page":"1287","DOI":"10.1016\/S0140-6736(07)60601-1","article-title":"Autosomal dominant polycystic kidney disease.","volume":"369","author":"Torres","year":"2007","journal-title":"Lancet"},{"key":"R2-10-20230601","doi-asserted-by":"crossref","first-page":"1505","DOI":"10.1681\/ASN.V1181505","article-title":"Quantification and longitudinal trends of kidney, renal cyst, and renal parenchyma volumes in autosomal dominant polycystic kidney disease.","volume":"11","author":"King","year":"2000","journal-title":"J Am Soc Nephrol"},{"key":"R3-10-20230601","doi-asserted-by":"crossref","first-page":"2122","DOI":"10.1056\/NEJMoa054341","article-title":"Volume progression in polycystic kidney disease.","volume":"354","author":"Grantham","year":"2006","journal-title":"N Engl J Med"},{"key":"R5-10-20230601","doi-asserted-by":"crossref","first-page":"479","DOI":"10.2215\/CJN.09500911","article-title":"Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease.","volume":"7","author":"Chapman","year":"2012","journal-title":"Clin J Am Soc Nephrol"},{"key":"R6-10-20230601","doi-asserted-by":"crossref","first-page":"1833","DOI":"10.1681\/ASN.2009020162","article-title":"Family history of renal disease severity predicts the mutated gene in ADPKD.","volume":"20","author":"Barua","year":"2009","journal-title":"J Am Soc Nephrol"},{"key":"R7-10-20230601","doi-asserted-by":"crossref","first-page":"3013","DOI":"10.1681\/ASN.2006080835","article-title":"Cyst number but not the rate of cystic growth is associated with the mutated gene in autosomal dominant polycystic kidney disease.","volume":"17","author":"Harris","year":"2006","journal-title":"J Am Soc Nephrol"},{"key":"R8-10-20230601","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1016\/S0092-8674(00)81793-6","article-title":"The molecular basis of focal cyst formation in human autosomal dominant polycystic kidney disease type I.","volume":"87","author":"Qian","year":"1996","journal-title":"Cell"},{"key":"R9-10-20230601","doi-asserted-by":"crossref","first-page":"2523","DOI":"10.1093\/hmg\/ddp147","article-title":"Renal injury is a third hit promoting rapid development of adult polycystic kidney disease.","volume":"18","author":"Takakura","year":"2009","journal-title":"Hum Mol Genet"},{"key":"R10-10-20230601","doi-asserted-by":"crossref","first-page":"1087","DOI":"10.2215\/CJN.00900112","article-title":"Detected renal cysts are tips of the iceberg in adults with ADPKD.","volume":"7","author":"Grantham","year":"2012","journal-title":"Clin J Am Soc Nephrol"},{"key":"R11-10-20230601","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1517\/13543781003588491","article-title":"Novel targets for the treatment of autosomal dominant polycystic kidney disease.","volume":"19","author":"Belibi","year":"2010","journal-title":"Expert Opin Investig Drugs"},{"key":"R12-10-20230601","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1038\/ki.2009.128","article-title":"Autosomal dominant polycystic kidney disease: The last 3 years.","volume":"76","author":"Torres","year":"2009","journal-title":"Kidney Int"},{"key":"R13-10-20230601","doi-asserted-by":"crossref","first-page":"F1","DOI":"10.1152\/ajprenal.00014.2012","article-title":"Mammalian target of rapamycin and the kidney. I. The signaling pathway.","volume":"303","author":"Lieberthal","year":"2012","journal-title":"Am J Physiol Renal Physiol"},{"key":"R14-10-20230601","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1016\/j.molcel.2006.03.029","article-title":"Prolonged rapamycin treatment inhibits mTORC2 assembly and Akt\/PKB.","volume":"22","author":"Sarbassov","year":"2006","journal-title":"Mol Cell"},{"key":"R15-10-20230601","doi-asserted-by":"crossref","first-page":"F180","DOI":"10.1152\/ajprenal.00015.2012","article-title":"Mammalian target of rapamycin and the kidney. II. Pathophysiology and therapeutic implications.","volume":"303","author":"Lieberthal","year":"2012","journal-title":"Am J Physiol Renal Physiol"},{"key":"R16-10-20230601","doi-asserted-by":"crossref","first-page":"830","DOI":"10.1056\/NEJMoa1003491","article-title":"Everolimus in patients with autosomal dominant polycystic kidney disease.","volume":"363","author":"Walz","year":"2010","journal-title":"N Engl J Med"},{"key":"R17-10-20230601","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1056\/NEJMoa0907419","article-title":"Sirolimus and kidney growth in autosomal dominant polycystic kidney disease.","volume":"363","author":"Serra","year":"2010","journal-title":"N Engl J Med"},{"key":"R18-10-20230601","doi-asserted-by":"crossref","first-page":"1031","DOI":"10.1681\/ASN.2009121302","article-title":"Sirolimus therapy to halt the progression of ADPKD.","volume":"21","author":"Perico","year":"2010","journal-title":"J Am Soc Nephrol"},{"key":"R19-10-20230601","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1681\/ASN.2004080660","article-title":"Rapamycin markedly slows disease progression in a rat model of polycystic kidney disease.","volume":"16","author":"Tao","year":"2005","journal-title":"J Am Soc Nephrol"},{"key":"R20-10-20230601","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1093\/ndt\/gfi181","article-title":"Inhibition of mTOR with sirolimus slows disease progression in Han:SPRD rats with autosomal dominant polycystic kidney disease (ADPKD).","volume":"21","author":"Wahl","year":"2006","journal-title":"Nephrol Dial Transplant"},{"key":"R21-10-20230601","doi-asserted-by":"crossref","first-page":"437","DOI":"10.4067\/S0716-97602009000400005","article-title":"Mycophenolate mofetil versus rapamycin in Han:SPRD rats with polycystic kidney disease.","volume":"42","author":"Zhang","year":"2009","journal-title":"Biol Res"},{"key":"R22-10-20230601","doi-asserted-by":"crossref","first-page":"2349","DOI":"10.1093\/ndt\/gfp129","article-title":"Long-term rapamycin therapy in the Han:SPRD rat model of polycystic kidney disease (PKD).","volume":"24","author":"Zafar","year":"2009","journal-title":"Nephrol Dial Transplant"},{"key":"R23-10-20230601","doi-asserted-by":"crossref","first-page":"5466","DOI":"10.1073\/pnas.0509694103","article-title":"The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease.","volume":"103","author":"Shillingford","year":"2006","journal-title":"Proc Natl Acad Sci U S A"},{"key":"R24-10-20230601","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1681\/ASN.2009040421","article-title":"Rapamycin ameliorates PKD resulting from conditional inactivation of Pkd1.","volume":"21","author":"Shillingford","year":"2010","journal-title":"J Am Soc Nephrol"},{"key":"R25-10-20230601","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1681\/ASN.2011040340","article-title":"Dose-dependent effects of sirolimus on mTOR signaling and polycystic kidney disease.","volume":"23","author":"Novalic","year":"2012","journal-title":"J Am Soc Nephrol"},{"key":"R26-10-20230601","doi-asserted-by":"crossref","first-page":"604","DOI":"10.7326\/0003-4819-150-9-200905050-00006","article-title":"A new equation to estimate glomerular filtration rate.","volume":"150","author":"Levey","year":"2009","journal-title":"Ann Intern Med"},{"key":"R27-10-20230601","doi-asserted-by":"crossref","first-page":"102","DOI":"10.2215\/CJN.04310709","article-title":"The HALT polycystic kidney disease trials: Design and implementation.","volume":"5","author":"Chapman","year":"2010","journal-title":"Clin J Am Soc Nephrol"},{"key":"R29-10-20230601","doi-asserted-by":"crossref","first-page":"1631","DOI":"10.1515\/CCLM.2008.303","article-title":"A rapid liquid chromatography-tandem mass spectrometry analysis of whole blood sirolimus using turbulent flow technology for online extraction.","volume":"46","author":"Wang","year":"2008","journal-title":"Clin Chem Lab Med"},{"key":"R30-10-20230601","doi-asserted-by":"crossref","first-page":"483","DOI":"10.2214\/ajr.176.2.1760483","article-title":"Accuracy of volumetric measurements after virtual right hepatectomy in potential donors undergoing living adult liver transplantation.","volume":"176","author":"Kamel","year":"2001","journal-title":"AJR Am J Roentgenol"},{"key":"R31-10-20230601","doi-asserted-by":"crossref","first-page":"1560","DOI":"10.1681\/ASN.V8101560","article-title":"Identification of patients with autosomal dominant polycystic kidney disease at highest risk for end-stage renal disease.","volume":"8","author":"Johnson","year":"1997","journal-title":"J Am Soc Nephrol"},{"key":"R32-10-20230601","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1681\/ASN.2011010084","article-title":"Macrophages promote cyst growth in polycystic kidney disease.","volume":"22","author":"Karihaloo","year":"2011","journal-title":"J Am Soc Nephrol"},{"key":"R33-10-20230601","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1002\/path.2920","article-title":"Cystogenic potential of CD133+ progenitor cells of human polycystic kidneys.","volume":"225","author":"Carvalhosa","year":"2011","journal-title":"J Pathol"},{"key":"R34-10-20230601","doi-asserted-by":"crossref","first-page":"3091","DOI":"10.1002\/eji.200324074","article-title":"Macrophage colony-stimulating factor-dependent macrophage proliferation is mediated through a calcineurin-independent but immunophilin-dependent mechanism that mediates the activation of external regulated kinases.","volume":"33","author":"Comalada","year":"2003","journal-title":"Eur J Immunol"},{"key":"R35-10-20230601","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1038\/sj.emboj.7601522","article-title":"PSGL-1 and mTOR regulate translation of ROCK-1 and physiological functions of macrophages.","volume":"26","author":"Fox","year":"2007","journal-title":"EMBO J"},{"key":"R36-10-20230601","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1016\/j.ajpath.2011.04.036","article-title":"Intermediate volume on computed tomography imaging defines a fibrotic compartment that predicts glomerular filtration rate decline in autosomal dominant polycystic kidney disease patients.","volume":"179","author":"Caroli","year":"2011","journal-title":"Am J Pathol"},{"key":"R37-10-20230601","doi-asserted-by":"crossref","first-page":"854","DOI":"10.1681\/ASN.2005070697","article-title":"Comparison of methods for determining renal function decline in early autosomal dominant polycystic kidney disease: The consortium of radiologic imaging studies of polycystic kidney disease cohort.","volume":"17","author":"Rule","year":"2006","journal-title":"J Am Soc Nephrol"},{"key":"R38-10-20230601","doi-asserted-by":"crossref","first-page":"1701","DOI":"10.1111\/j.1600-6143.2010.03152.x","article-title":"Therapeutic mTOR inhibition in autosomal dominant polycystic kidney disease: What is the appropriate serum level?","volume":"10","author":"Canaud","year":"2010","journal-title":"Am J Transplant"},{"key":"R39-10-20230601","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1093\/hmg\/ddn325","article-title":"The tuberous sclerosis proteins regulate formation of the primary cilium via a rapamycin-insensitive and polycystin 1-independent pathway.","volume":"18","author":"Hartman","year":"2009","journal-title":"Hum Mol Genet"},{"key":"R40-10-20230601","doi-asserted-by":"crossref","first-page":"879","DOI":"10.1056\/NEJMe1006925","article-title":"mTOR inhibitors in polycystic kidney disease.","volume":"363","author":"Watnick","year":"2010","journal-title":"N Engl J Med"},{"key":"R41-10-20230601","doi-asserted-by":"crossref","first-page":"2037","DOI":"10.1681\/ASN.V5122037","article-title":"Dietary protein restriction, blood pressure control, and the progression of polycystic kidney disease.","volume":"5","author":"Klahr","year":"1995","journal-title":"J Am Soc Nephrol"},{"key":"R42-10-20230601","first-page":"287","article-title":"mTOR inhibitor and autosomal dominant polycystic kidney disease.","volume":"364","author":"Walz","year":"2011","journal-title":"N Engl J Med"},{"key":"R44-10-20230601","first-page":"361","article-title":"Risk factors for hospitalizations resulting from pulmonary embolism after renal transplantation in the United States.","volume":"14","author":"Tveit","year":"2001","journal-title":"J Nephrol"},{"key":"R46-10-20230601","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1097\/01.TP.0000071203.62964.DA","article-title":"Sirolimus does not increase the risk for postoperative thromboembolic events among renal transplant recipients.","volume":"76","author":"Langer","year":"2003","journal-title":"Transplantation"},{"key":"R47-10-20230601","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1007\/s10157-005-0403-6","article-title":"Nephrotic syndrome and IgA nephropathy in polycystic kidney disease.","volume":"10","author":"Hiura","year":"2006","journal-title":"Clin Exp Nephrol"},{"key":"R48-10-20230601","doi-asserted-by":"crossref","first-page":"109","DOI":"10.2215\/CJN.00120605","article-title":"A prospective, open-label trial of sirolimus in the treatment of focal segmental glomerulosclerosis.","volume":"1","author":"Tumlin","year":"2006","journal-title":"Clin J Am Soc Nephrol"},{"key":"R49-10-20230601","doi-asserted-by":"crossref","first-page":"326","DOI":"10.2215\/CJN.03751106","article-title":"High sirolimus levels may induce focal segmental glomerulosclerosis de novo.","volume":"2","author":"Letavernier","year":"2007","journal-title":"Clin J Am Soc Nephrol"}],"container-title":["Clinical Journal of the American Society of Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/01277230-201405000-00010","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T13:45:22Z","timestamp":1786369522000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/01277230-201405000-00010"}},"subtitle":["An Open-Label Randomized Controlled Pilot Study"],"short-title":[],"issued":{"date-parts":[[2014,5]]},"references-count":45,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2014]]}},"URL":"https:\/\/doi.org\/10.2215\/cjn.02650313","relation":{},"ISSN":["1555-9041"],"issn-type":[{"value":"1555-9041","type":"print"}],"subject":[],"published":{"date-parts":[[2014,5]]}}}