多囊肾病

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Ravine D, Gibson RN, Walker RG, et al. Evaluation of ultrasonographic diagnostic criteria for autosomal dominant polycystic kidney disease 1. Lancet. 1994 Apr 2;343(8901):824-7.

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Israel GM, Bosniak MA. An update of the Bosniak renal cyst classification system. Urology. 2005 Sep;66(3):484-8.

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Schrier RW, Abebe KZ, Perrone RD, et al; HALT-PKD Trial Investigators. Blood pressure in early autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11;371(24):2255-66.

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Torres VE, Abebe KZ, Chapman AB, et al; HALT-PKD Trial Investigators. Angiotensin blockade in late autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11;371(24):2267-76.

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Eighth Joint National Committee on prevention, detection, evaluation, and treatment of high blood pressure (JNC 8), US. The 2014 JNC 8 and 2017 AHA/ACA guidelines for management of high blood pressure in adults. 2018 [internet publication].

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Torres VE, Chapman AB, Devuyst O, et al; TEMPO 3:4 Trial Investigators. Tolvaptan in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2012 Dec 20;367(25):2407-18.

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Walz G, Budde K, Mannaa M, et al. Everolimus in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2010 Aug 26;363(9):830-40.

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1.  Hateboer N, v Dijk MA, Bogdanova N, et al. Comparison of phenotypes of polycystic kidney disease types 1 and 2. European PKD1-PKD2 Study Group. Lancet. 1999 Jan 9;353(9147):103-7.

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2.  Rossetti S, Burton S, Strmecki L, et al. The position of the polycystic kidney disease 1 (PKD1) gene mutation correlates with the severity of renal disease. J Am Soc Nephrol. 2002 May;13(5):1230-7.

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3.  Rossetti S, Chauveau D, Kubly V, et al. Association of mutation position in polycystic kidney disease 1 (PKD1) gene and development of a vascular phenotype. Lancet. 2003 Jun 28;361(9376):2196-201.

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4.  Magistroni R, He N, Wang K, et al. Genotype-renal function correlation in type 2 autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2003 May;14(5):1164-74.

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5.  Guay-Woodford LM, Desmond RA. Autosomal recessive polycystic kidney disease: the clinical experience in North America. Pediatrics. 2003 May;111(5 Pt 1):1072-80.

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7.  Dalgaard OZ. Bilateral polycystic disease of the kidneys: a follow-up of two hundred and eighty-four patients and their families. Acta Med Scand Suppl. 1957;328:1-255.

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8.  Gabow PA. Autosomal dominant polycystic kidney disease. N Engl J Med. 1993 Jul 29;329(5):332-42.

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9.  Kelleher CL, McFann KK, Johnson AM, et al. Characteristics of hypertension in young adults with autosomal dominant polycystic kidney disease compared with the general U.S. population. Am J Hypertens. 2004 Nov;17(11 Pt 1):1029-34.

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10.  Torres VE, Holley KE, Offord KP. Epidemiology. In: Grantham JJ, Gardner KD, eds. Problems in diagnosis and management of polycystic kidney disease. Kansas City, MO: PKR Foundation; 1985:49-69.

11.  U.S. Renal Data System. USRDS 2007 annual data report. B: Prevalence of reported ESRD. Bethesda, MD: NIH, National Institute of Diabetes and Digestive and Kidney Diseases; 2007:17.

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12.  Simon P, Le Goff JY, Ang KS, et al. Epidemiologic data, clinical and prognostic features of autosomal dominant polycystic kidney disease in a French region [in French]. Nephrologie. 1996;17(2):123-30.

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13.  Davies F, Coles GA, Harper PS, et al. Polycystic kidney disease re-evaluated: a population-based study. Q J Med. 1991 Jun;79(290):477-85.

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14.  Higashihara E, Nutahara K, Kojima M, et al. Prevalence and renal prognosis of diagnosed autosomal dominant polycystic kidney disease in Japan. Nephron. 1998 Dec;80(4):421-7.

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15.  Yersin C, Bovet P, Wauters JP, et al. Frequency and impact of autosomal dominant polycystic kidney disease in the Seychelles (Indian Ocean). Nephrol Dial Transplant. 1997 Oct;12(10):2069-74.

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16.  Stengel B, Billon S, Van Dijk PC, et al. Trends in the incidence of renal replacement therapy for end-stage renal disease in Europe, 1990-1999. Nephrol Dial Transplant. 2003 Sep;18(9):1824-33.

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17.  Wakai K, Nakai S, Kikuchi K, et al. Trends in incidence of end-stage renal disease in Japan, 1983-2000: age-adjusted and age-specific rates by gender and cause. Nephrol Dial Transplant. 2004 Aug;19(8):2044-52.

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18.  Cole BR, Conley SB, Stapleton FB. Polycystic kidney disease in the first year of life. J Pediatr. 1987 Nov;111(5):693-9.

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19.  Kääriäinen H. Polycystic kidney disease in children: a genetic and epidemiological study of 82 Finnish patients. J Med Genet. 1987 Aug;24(8):474-81.

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20.  Qian F, Watnick TJ, Onuchic LF, et al. The molecular basis of focal cyst formation in human autosomal dominant polycystic kidney disease type I. Cell. 1996 Dec 13;87(6):979-87.

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21.  Hughes J, Ward CJ, Peral B, et al. The polycystic kidney disease 1 (PKD1) gene encodes a novel protein with multiple cell recognition domains. Nat Genet. 1995 Jun;10(2):151-60.

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22.  International Polycystic Kidney Disease Consortium. Polycystic kidney disease: the complete structure of the PKD1 gene and its protein. Cell. 1995 Apr 21;81(2):289-98.

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23.  Hayashi T, Mochizuki T, Reynolds DM, et al. Characterization of the exon structure of the polycystic kidney disease 2 gene (PKD2). Genomics. 1997 Aug 15;44(1):131-6.

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24.  Qian F, Germino FJ, Cai Y, et al. PKD1 interacts with PKD2 through a probable coiled-coil domain. Nat Genet. 1997 Jun;16(2):179-83.

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25.  Tsiokas L, Kim E, Arnould T, et al. Homo- and heterodimeric interactions between the gene products of PKD1 and PKD2. Proc Natl Acad Sci USA. 1997 Jun 24;94(13):6965-70.

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26.  Qian F, Boletta A, Bhunia AK, et al. Cleavage of polycystin-1 requires the receptor for egg jelly domain and is disrupted by human autosomal-dominant polycystic kidney disease 1-associated mutations. Proc Natl Acad Sci USA. 2002 Dec 24;99(26):16981-6.

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27.  Chauvet V, Tian X, Husson H, et al. Mechanical stimuli induce cleavage and nuclear translocation of the polycystin-1 C terminus. J Clin Invest. 2004 Nov;114(10):1433-43.

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28.  Low SH, Vasanth S, Larson CH, et al. Polycystin-1, STAT6, and P100 function in a pathway that transduces ciliary mechanosensation and is activated in polycystic kidney disease. Dev Cell. 2006 Jan;10(1):57-69.

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29.  Hanaoka K, Qian F, Boletta A, et al. Co-assembly of polycystin-1 and -2 produces unique cation-permeable currents. Nature. 2000 Dec 21-28;408(6815):990-4.

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30.  Gonzalez-Perrett S, Kim K, Ibarra C, et al. Polycystin-2, the protein mutated in autosomal dominant polycystic kidney disease (ADPKD), is a Ca2+-permeable nonselective cation channel. Proc Natl Acad Sci USA. 2001 Jan 30;98(3):1182-7.

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31.  Vassilev PM, Guo L, Chen XZ, et al. Polycystin-2 is a novel cation channel implicated in defective intracellular Ca(2+) homeostasis in polycystic kidney disease. Biochem Biophys Res Commun. 2001 Mar 23;282(1):341-50.

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32.  Nauli SM, Alenghat FJ, Luo Y, et al. Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells. Nat Genet. 2003 Feb;33(2):129-37.

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33.  Hogan MC, Manganelli L, Woollard JR, et al. Characterization of PKD protein-positive exosome-like vesicles. J Am Soc Nephrol. 2009 Feb;20(2):278-88.

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34.  Zeier M, Fehrenbach P, Geberth S, et al. Renal histology in polycystic kidney disease with incipient and advanced renal failure. Kidney Int. 1992 Nov;42(5):1259-65.

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35.  Hunsicker LG, Adler S, Caggiula A, et al. Predictors of the progression of renal disease in the Modification of Diet in Renal Disease Study. Kidney Int. 1997 Jun;51(6):1908-19.

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36.  Perrone RD, Ruthazer R, Terrin NC. Survival after end-stage renal disease in autosomal dominant polycystic kidney disease: contribution of extrarenal complications to mortality. Am J Kidney Dis. 2001 Oct;38(4):777-84.

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37.  Guay-Woodford LM. Autosomal recessive polycystic kidney disease: clinical and genetic profiles. In: Watson ML, Torres VE, eds. Polycystic kidney disease. New York, NY: Oxford University Press; 1996:237-66.

38.  Gunay-Aygun M, Font-Montgomery E, Lukose L, et al. Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease. Gastroenterology. 2013 Jan;144(1):112-21.e2.

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39.  Pirson Y, Chauveau D, Torres V. Management of cerebral aneurysms in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2002 Jan;13(1):269-76.

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40.  Ravine D, Gibson RN, Walker RG, et al. Evaluation of ultrasonographic diagnostic criteria for autosomal dominant polycystic kidney disease 1. Lancet. 1994 Apr 2;343(8901):824-7.

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41.  Nicolau C, Torra R, Badenas C, et al. Autosomal dominant polycystic kidney disease types 1 and 2: assessment of US sensitivity for diagnosis. Radiology. 1999 Oct;213(1):273-6.

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42.  Kielstein R, Sass HM. Genetics in kidney disease: how much do we want to know? Am J Kidney Dis. 2002 Mar;39(3):637-52.

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43.  Pei Y, Obaji J, Dupuis A, et al. Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 2009 Jan;20(1):205-12.

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44.  Irazabal MV, Rangel LJ, Bergstralh EJ, et al. Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials. J Am Soc Nephrol. 2015 Jan;26(1):160-72.

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45.  Torres VE, Erickson SB, Smith LH, et al. The association of nephrolithiasis and autosomal dominant polycystic kidney disease. Am J Kidney Dis. 1988 Apr;11(4):318-25.

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46.  Torres VE, Wilson DM, Hattery RR, et al. Renal stone disease in autosomal dominant polycystic kidney disease. Am J Kidney Dis. 1993 Oct;22(4):513-9.

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47.  Grampsas SA, Chandhoke PS, Fan J, et al. Anatomic and metabolic risk factors for nephrolithiasis in patients with autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2000 Jul;36(1):53-7.

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48.  Lavi R, Yarnitsky D, Rowe JM, et al. Standard vs atraumatic Whitacre needle for diagnostic lumbar puncture: a randomized trial. Neurology. 2006 Oct 24;67(8):1492-4.

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49.  Arendt K, Demaerschalk BM, Wingerchuk DM, Camann W. Atraumatic lumbar puncture needles: after all these years, are we still missing the point? Neurologist. 2009 Jan;15(1):17-20.

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50.  Nath S, Koziarz A, Badhiwala JH, et al. Atraumatic versus conventional lumbar puncture needles: a systematic review and meta-analysis. Lancet. 2018 Mar 24;391(10126):1197-1204.

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51.  Rochwerg B, Almenawer SA, Siemieniuk RAC, et al. Atraumatic (pencil-point) versus conventional needles for lumbar puncture: a clinical practice guideline. BMJ. 2018 May 22;361:k1920.

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52.  Ahmed SV, Jayawarna C, Jude E. Post lumbar puncture headache: diagnosis and management. Postgrad Med J. 2006 Nov;82(973):713-6.

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53.  Arevalo-Rodriguez I, Ciapponi A, Roqué i Figuls M, et al. Posture and fluids for preventing post-dural puncture headache. Cochrane Database Syst Rev. 2016 Mar 7;(3):CD009199.

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54.  Sujansky E, Kreutzer SB, Johnson AM, et al. Attitudes of at-risk and affected individuals regarding presymptomatic testing for autosomal dominant polycystic kidney disease. Am J Med Genet. 1990 Apr;35(4):510-5.

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55.  De Rycke M, Georgiou I, Sermon K, et al. PGD for autosomal dominant polycystic kidney disease type 1. Mol Hum Reprod. 2005 Jan;11(1):65-71.

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56.  Tuffs A. Court allows preimplantation genetic diagnosis in Germany. BMJ. 2010 Jul 12;341:c3741.

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57.  Gabow PA, Johnson AM, Kaehny WD, et al. Factors affecting the progression of renal disease in autosomal-dominant polycystic kidney disease. Kidney Int. 1992 May;41(5):1311-9.

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58.  Rossetti S, Consugar MB, Chapman AB, et al. Comprehensive molecular diagnostics in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2007 Jul;18(7):2143-60.

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59.  Hannig VL, Erickson SM, Phillips JA 3rd. Utilization and evaluation of living-related donors for patients with adult polycystic kidney disease. Am J Med Genet. 1992 Nov 1;44(4):409-12.

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60.  Connor A, Lunt PW, Dolling C, et al. Mosaicism in autosomal dominant polycystic kidney disease revealed by genetic testing to enable living related renal transplantation. Am J Transplant. 2008 Jan;8(1):232-7.

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61.  Fick GM, Johnson AM, Hammond WS, et al. Causes of death in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1995 Jun;5(12):2048-56.

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62.  Schwab SJ, Bander SJ, Klahr S. Renal infection in autosomal dominant polycystic kidney disease. Am J Med. 1987 Apr;82(4):714-8.

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63.  Parfrey PS, Bear JC, Morgan J, et al. The diagnosis and prognosis of autosomal dominant polycystic kidney disease. N Engl J Med. 1990 Oct 18;323(16):1085-90.

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64.  Churchill DN, Bear JC, Morgan J, et al. Prognosis of adult onset polycystic kidney disease re-evaluated. Kidney Int. 1984 Aug;26(2):190-3.

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65.  Jouret F, Lhommel R, Beguin C, et al. Positron-emission computed tomography in cyst infection diagnosis in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2011 Jul;6(7):1644-50.

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66.  Israel GM, Bosniak MA. An update of the Bosniak renal cyst classification system. Urology. 2005 Sep;66(3):484-8.

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67.  Graumann O, Osther SS, Osther PJ. Characterization of complex renal cysts: a critical evaluation of the Bosniak classification. Scand J Urol Nephrol. 2011 Mar;45(2):84-90.

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68.  Chen YZ, Gao Q, Zhao XZ, et al. Systematic review of TCF2 anomalies in renal cysts and diabetes syndrome/maturity onset diabetes of the young type 5. Chin Med J (Engl). 2010 Nov;123(22):3326-33.

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69.  Irazabal MV, Huston J 3rd, Kubly V, et al. Extended follow-up of unruptured intracranial aneurysms detected by presymptomatic screening in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2011 Jun;6(6):1274-85.

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70.  Belz MM, Fick-Brosnahan GM, Hughes RL, et al. Recurrence of intracranial aneurysms in autosomal-dominant polycystic kidney disease. Kidney Int. 2003 May;63(5):1824-30.

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71.  Gibbs GF, Huston J 3rd, Qian Q, et al. Follow-up of intracranial aneurysms in autosomal-dominant polycystic kidney disease. Kidney Int. 2004 May;65(5):1621-7.

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72.  Schrier RW, Belz MM, Johnson AM, et al. Repeat imaging for intracranial aneurysms in patients with autosomal dominant polycystic kidney disease with initially negative studies: a prospective ten-year follow-up. J Am Soc Nephrol. 2004 Apr;15(4):1023-8.

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73.  Schrier RW, Abebe KZ, Perrone RD, et al; HALT-PKD Trial Investigators. Blood pressure in early autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11;371(24):2255-66.

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74.  Torres VE, Abebe KZ, Chapman AB, et al; HALT-PKD Trial Investigators. Angiotensin blockade in late autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11;371(24):2267-76.

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75.  Torres VE, Wilson DM, Burnett JC Jr, et al. Effect of inhibition of converting enzyme on renal hemodynamics and sodium management in polycystic kidney disease. Mayo Clin Proc. 1991 Oct;66(10):1010-7.

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76.  Watson ML, Macnicol AM, Allan PL, et al. Effects of angiotensin converting enzyme inhibition in adult polycystic kidney disease. Kidney Int. 1992 Jan;41(1):206-10.

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77.  Sallée M, Rafat C, Zahar JR, et al. Cyst infections in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2009 Jul;4(7):1183-9.

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78.  Millar M, Tanagho YS, Haseebuddin M, et al. Surgical cyst decortication in autosomal dominant polycystic kidney disease. J Endourol. 2013 May;27(5):528-34.

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79.  Lifson BJ, Teichman JM, Hulbert JC. Role and long-term results of laparoscopic decortication in solitary cystic and autosomal dominant polycystic kidney disease. J Urol. 1998 Mar;159(3):702-5.

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80.  Casale P, Meyers K, Kaplan B. Follow-up for laparoscopic renal denervation and nephropexy for autosomal dominant polycystic kidney disease-related pain in pediatrics. J Endourol. 2008 May;22(5):991-3.

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81.  Resnick M, Chang AY, Casale P. Laparoscopic renal denervation and nephropexy for autosomal dominant polycystic kidney disease related pain in adolescents. J Urol. 2006 Jun;175(6):2274-6.

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82.  Chapuis O, Sockeel P, Pallas G, et al. Thoracoscopic renal denervation for intractable autosomal dominant polycystic kidney disease-related pain. Am J Kidney Dis. 2004 Jan;43(1):161-3.

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83.  Valente JF, Dreyer DR, Breda MA, et al. Laparoscopic renal denervation for intractable ADPKD-related pain. Nephrol Dial Transplant. 2001 Jan;16(1):160.

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84.  Mufti UB, Nalagatla SK. Nephrolithiasis in autosomal dominant polycystic kidney disease. J Endourol. 2010 Oct;24(10):1557-61.

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85.  Wiebers DO, Whisnant JP, Huston J 3rd, et al; International Study of Unruptured Intracranial Aneurysms Investigators. Unruptured intracranial aneurysms: natural history, clinical outcome, and risks of surgical and endovascular treatment. Lancet. 2003 Jul 12;362(9378):103-10.

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86.  Diringer MN. Management of aneurysmal subarachnoid hemorrhage. Crit Care Med. 2009 Feb;37(2):432-40.

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87.  Bederson JB, Connolly ES Jr, Batjer HH, et al. Guidelines for the management of aneurysmal subarachnoid hemorrhage: a statement for healthcare professionals from a special writing group of the Stroke Council, American Heart Association. Stroke. 2009 Mar;40(3):994-1025.

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88.  Dorhout Mees SM, Rinkel GJ, Feigin VL, et al. Calcium antagonists for aneurysmal subarachnoid haemorrhage. Cochrane Database Syst Rev. 2007 Jul 18;(3):CD000277.

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89.  Salehipour M, Jalaeian H, Salahi H, et al. Are large nonfunctional kidneys risk factors for posttransplantation urinary tract infection in patients with end-stage renal disease due to autosomal dominant polycystic kidney disease? Transplant Proc. 2007 May;39(4):887-8.

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90.  Rozanski J, Kozlowska I, Myslak M, et al. Pretransplant nephrectomy in patients with autosomal dominant polycystic kidney disease. Transplant Proc. 2005 Mar;37(2):666-8.

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91.  Dunn MD, Portis AJ, Elbahnasy AM, et al. Laparoscopic nephrectomy in patients with end-stage renal disease and autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2000 Apr;35(4):720-5.

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92.  Abbott KC, Agodoa LY. Polycystic kidney disease in patients on the renal transplant waiting list: trends in hematocrit and survival. BMC Nephrol. 2002 Aug 23;3:7.

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93.  Eighth Joint National Committee on prevention, detection, evaluation, and treatment of high blood pressure (JNC 8), US. The 2014 JNC 8 and 2017 AHA/ACA guidelines for management of high blood pressure in adults. 2018 [internet publication].

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94.  Wiebers DO, Whisnant JP, Huston J 3rd, et al. Unruptured intracranial aneurysms: natural history, clinical outcome, and risks of surgical and endovascular treatment. Lancet. 2003 Jul 12;362(9378):103-10.

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95.  Shillingford JM, Murcia NS, Larson CH, et al. The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease. Proc Natl Acad Sci USA. 2006 Apr 4;103(14):5466-71.

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96.  Torres VE, Meijer E, Bae KT, et al. Rationale and design of the TEMPO (tolvaptan efficacy and safety in management of autosomal dominant polycystic kidney disease and its outcomes) 3-4 study. Am J Kidney Dis. 2011 May;57(5):692-9.

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97.  Torres VE, Chapman AB, Devuyst O, et al; TEMPO 3:4 Trial Investigators. Tolvaptan in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2012 Dec 20;367(25):2407-18.

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98.  Torres VE, Chapman AB, Devuyst O, et al; REPRISE Trial Investigators. Tolvaptan in later-stage autosomal dominant polycystic kidney disease. N Engl J Med. 2017 Nov 16;377(20):1930-42.

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99.  van Keimpema L, Nevens F, Vanslembrouck R, et al. Lanreotide reduces the volume of polycystic liver: a randomized, double-blind, placebo-controlled trial. Gastroenterology. 2009 Nov;137(5):1661-8.e1-2.

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100.  Hogan MC, Masyuk TV, Page LJ, et al. Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J Am Soc Nephrol. 2010 Jun;21(6):1052-61.

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101.  Caroli A, Antiga L, Cafaro M, et al. Reducing polycystic liver volume in ADPKD: effects of somatostatin analogue octreotide. Clin J Am Soc Nephrol. 2010 May;5(5):783-9.

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102.  Caroli A, Perico N, Perna A, et al. Effect of longacting somatostatin analogue on kidney and cyst growth in autosomal dominant polycystic kidney disease (ALADIN): a randomised, placebo-controlled, multicentre trial. Lancet. 2013 Nov 2;382(9903):1485-95.

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103.  Serra AL, Poster D, Kistler AD, et al. Sirolimus and kidney growth in autosomal dominant polycystic kidney disease. N Engl J Med. 2010 Aug 26;363(9):820-9.

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104.  Walz G, Budde K, Mannaa M, et al. Everolimus in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2010 Aug 26;363(9):830-40.

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105.  Perico N, Antiga L, Caroli A, et al. Sirolimus therapy to halt the progression of ADPKD. J Am Soc Nephrol. 2010 Jun;21(6):1031-40.

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106.  Liu YM, Shao YQ, He Q. Sirolimus for treatment of autosomal-dominant polycystic kidney disease: a meta-analysis of randomized controlled trials. Transplant Proc. 2014 Jan-Feb;46(1):66-74.

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107.  Chrispijn M, Gevers TJ, Hol JC, et al. Everolimus does not further reduce polycystic liver volume when added to long acting octreotide: results from a randomized controlled trial. J Hepatol. 2013 Jul;59(1):153-9.

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108.  Franz DN, Belousova E, Sparagana S, et al. Efficacy and safety of everolimus for subependymal giant cell astrocytomas associated with tuberous sclerosis complex (EXIST-1): a multicentre, randomised, placebo-controlled phase 3 trial. Lancet. 2013 Jan 12;381(9861):125-32.

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109.  Bissler JJ, Kingswood JC, Radzikowska E, et al. Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet. 2013 Mar 9;381(9869):817-24.

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110.  Xue C, Dai B, Mei C. Long-term treatment with mammalian target of rapamycin inhibitor does not benefit patients with autosomal dominant polycystic kidney disease: a meta-analysis. Nephron Clin Pract. 2013;124(1-2):10-6.

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111.  Herrera GA. C-erb B-2 amplification in cystic renal disease. Kidney Int. 1991 Sep;40(3):509-13.

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112.  Wilson SJ, Amsler K, Hyink DP, et al. Inhibition of HER-2(neu/ErbB2) restores normal function and structure to polycystic kidney disease (PKD) epithelia. Biochim Biophys Acta. 2006 Jul;1762(7):647-55.

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