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Nephronophthisis 8

MedGen UID:
468521
Concept ID:
CN119610
Disease or Syndrome
Gene (location): RPGRIP1L (16q12.2)

Professional guidelines

PubMed

Gjerstad AC, Skrunes R, Tøndel C, Åsberg A, Leh S, Klingenberg C, Døllner H, Hammarstrøm C, Bjerre AK
Pediatr Nephrol 2023 Apr;38(4):1249-1256. Epub 2022 Aug 22 doi: 10.1007/s00467-022-05706-y. PMID: 35994104Free PMC Article
Iannicelli M, Brancati F, Mougou-Zerelli S, Mazzotta A, Thomas S, Elkhartoufi N, Travaglini L, Gomes C, Ardissino GL, Bertini E, Boltshauser E, Castorina P, D'Arrigo S, Fischetto R, Leroy B, Loget P, Bonnière M, Starck L, Tantau J, Gentilin B, Majore S, Swistun D, Flori E, Lalatta F, Pantaleoni C, Penzien J, Grammatico P; International JSRD Study Group, Dallapiccola B, Gleeson JG, Attie-Bitach T, Valente EM
Hum Mutat 2010 May;31(5):E1319-31. doi: 10.1002/humu.21239. PMID: 20232449Free PMC Article

Recent clinical studies

Etiology

Rao J, Liu X, Mao J, Tang X, Shen Q, Li G, Sun L, Bi Y, Wang X, Qian Y, Wu B, Wang H, Zhou W, Ma D, Zheng B, Shen Y, Chen Z, Luan J, Wang X, Wang M, Dang X, Wang Y, Wu Y, Hou L, Sun S, Li Q, Liu X, Bai H, Yang Y, Shao X, Li Y, Zheng S, Han M, Liu C, Cao G, Zhao L, Qiu S, Dong Y, Zhu Y, Wang F, Zhang D, Li Y, Zhao L, Yang C, Luo X, Chen L, Jiang X, Zhang A, Xu H; for “Internet Plus” Nephrology Alliance of National Center for Children's Care
Clin Genet 2019 Nov;96(5):402-410. Epub 2019 Jul 25 doi: 10.1111/cge.13606. PMID: 31328266
Taskiran EZ, Korkmaz E, Gucer S, Kosukcu C, Kaymaz F, Koyunlar C, Bryda EC, Chaki M, Lu D, Vadnagara K, Candan C, Topaloglu R, Schaefer F, Attanasio M, Bergmann C, Ozaltin F
J Am Soc Nephrol 2014 Aug;25(8):1653-61. Epub 2014 Mar 7 doi: 10.1681/ASN.2013060646. PMID: 24610927Free PMC Article
Halbritter J, Porath JD, Diaz KA, Braun DA, Kohl S, Chaki M, Allen SJ, Soliman NA, Hildebrandt F, Otto EA; GPN Study Group
Hum Genet 2013 Aug;132(8):865-84. Epub 2013 Apr 5 doi: 10.1007/s00439-013-1297-0. PMID: 23559409Free PMC Article
Hirano D, Fujinaga S, Ohtomo Y, Nishizaki N, Hara S, Murakami H, Yamaguchi Y, Hattori M, Ida H
Clin Pediatr (Phila) 2013 Aug;52(8):759-61. Epub 2012 Apr 20 doi: 10.1177/0009922812441390. PMID: 22523277
Zhou W, Otto EA, Cluckey A, Airik R, Hurd TW, Chaki M, Diaz K, Lach FP, Bennett GR, Gee HY, Ghosh AK, Natarajan S, Thongthip S, Veturi U, Allen SJ, Janssen S, Ramaswami G, Dixon J, Burkhalter F, Spoendlin M, Moch H, Mihatsch MJ, Verine J, Reade R, Soliman H, Godin M, Kiss D, Monga G, Mazzucco G, Amann K, Artunc F, Newland RC, Wiech T, Zschiedrich S, Huber TB, Friedl A, Slaats GG, Joles JA, Goldschmeding R, Washburn J, Giles RH, Levy S, Smogorzewska A, Hildebrandt F
Nat Genet 2012 Jul 8;44(8):910-5. doi: 10.1038/ng.2347. PMID: 22772369Free PMC Article

Diagnosis

Rao J, Liu X, Mao J, Tang X, Shen Q, Li G, Sun L, Bi Y, Wang X, Qian Y, Wu B, Wang H, Zhou W, Ma D, Zheng B, Shen Y, Chen Z, Luan J, Wang X, Wang M, Dang X, Wang Y, Wu Y, Hou L, Sun S, Li Q, Liu X, Bai H, Yang Y, Shao X, Li Y, Zheng S, Han M, Liu C, Cao G, Zhao L, Qiu S, Dong Y, Zhu Y, Wang F, Zhang D, Li Y, Zhao L, Yang C, Luo X, Chen L, Jiang X, Zhang A, Xu H; for “Internet Plus” Nephrology Alliance of National Center for Children's Care
Clin Genet 2019 Nov;96(5):402-410. Epub 2019 Jul 25 doi: 10.1111/cge.13606. PMID: 31328266
Kang HG, Lee HK, Ahn YH, Joung JG, Nam J, Kim NK, Ko JM, Cho MH, Shin JI, Kim J, Park HW, Park YS, Ha IS, Chung WY, Lee DY, Kim SY, Park WY, Cheong HI
Exp Mol Med 2016 Aug 5;48(8):e251. doi: 10.1038/emm.2016.63. PMID: 27491411Free PMC Article
Taskiran EZ, Korkmaz E, Gucer S, Kosukcu C, Kaymaz F, Koyunlar C, Bryda EC, Chaki M, Lu D, Vadnagara K, Candan C, Topaloglu R, Schaefer F, Attanasio M, Bergmann C, Ozaltin F
J Am Soc Nephrol 2014 Aug;25(8):1653-61. Epub 2014 Mar 7 doi: 10.1681/ASN.2013060646. PMID: 24610927Free PMC Article
Zhou W, Otto EA, Cluckey A, Airik R, Hurd TW, Chaki M, Diaz K, Lach FP, Bennett GR, Gee HY, Ghosh AK, Natarajan S, Thongthip S, Veturi U, Allen SJ, Janssen S, Ramaswami G, Dixon J, Burkhalter F, Spoendlin M, Moch H, Mihatsch MJ, Verine J, Reade R, Soliman H, Godin M, Kiss D, Monga G, Mazzucco G, Amann K, Artunc F, Newland RC, Wiech T, Zschiedrich S, Huber TB, Friedl A, Slaats GG, Joles JA, Goldschmeding R, Washburn J, Giles RH, Levy S, Smogorzewska A, Hildebrandt F
Nat Genet 2012 Jul 8;44(8):910-5. doi: 10.1038/ng.2347. PMID: 22772369Free PMC Article
Stone EM, Cideciyan AV, Aleman TS, Scheetz TE, Sumaroka A, Ehlinger MA, Schwartz SB, Fishman GA, Traboulsi EI, Lam BL, Fulton AB, Mullins RF, Sheffield VC, Jacobson SG
Arch Ophthalmol 2011 Jan;129(1):81-7. doi: 10.1001/archophthalmol.2010.330. PMID: 21220633Free PMC Article

Therapy

Gjerstad AC, Skrunes R, Tøndel C, Åsberg A, Leh S, Klingenberg C, Døllner H, Hammarstrøm C, Bjerre AK
Pediatr Nephrol 2023 Apr;38(4):1249-1256. Epub 2022 Aug 22 doi: 10.1007/s00467-022-05706-y. PMID: 35994104Free PMC Article
Avcı B, Baskın E, Gülleroğlu K, Çaltık Yılmaz A, Kantar A, Akdur A, Moray G, Haberal M
Exp Clin Transplant 2022 May;20(Suppl 3):122-125. doi: 10.6002/ect.PediatricSymp2022.O39. PMID: 35570616
Torres VE
Semin Nephrol 2008 May;28(3):306-17. doi: 10.1016/j.semnephrol.2008.03.003. PMID: 18519091Free PMC Article
Torres VE
Clin J Am Soc Nephrol 2008 Jul;3(4):1212-8. Epub 2008 Apr 23 doi: 10.2215/CJN.05281107. PMID: 18434616
Henriksson C, Andersen HJ, Gustafsson A, Gelin LE
Acta Paediatr Scand 1975 Nov;64(6):833-8. doi: 10.1111/j.1651-2227.1975.tb03933.x. PMID: 171908

Prognosis

Avcı B, Baskın E, Gülleroğlu K, Çaltık Yılmaz A, Kantar A, Akdur A, Moray G, Haberal M
Exp Clin Transplant 2022 May;20(Suppl 3):122-125. doi: 10.6002/ect.PediatricSymp2022.O39. PMID: 35570616
Tsukamoto T, Tanaka M, Komiya T, Ueda S, Takasu K, Takahara S, Koizumi A, Muso E
Clin Exp Nephrol 2008 Feb;12(1):82-8. Epub 2008 Jan 5 doi: 10.1007/s10157-007-0004-7. PMID: 18175055
Scolari F, Ghiggeri GM, Casari G, Amoroso A, Puzzer D, Caridi GL, Valzorio B, Tardanico R, Vizzardi V, Savoldi S, Viola BF, Bossini N, Prati E, Gusmano R, Maiorca R
Nephrol Dial Transplant 1998 Oct;13(10):2536-46. doi: 10.1093/ndt/13.10.2536. PMID: 9794556
Hildebrandt F, Strahm B, Nothwang HG, Gretz N, Schnieders B, Singh-Sawhney I, Kutt R, Vollmer M, Brandis M
Kidney Int 1997 Jan;51(1):261-9. doi: 10.1038/ki.1997.31. PMID: 8995741
Witzleben CL, Sharp AR
Hum Pathol 1982 Aug;13(8):728-33. doi: 10.1016/s0046-8177(82)80295-5. PMID: 7106736

Clinical prediction guides

Stone EM, Cideciyan AV, Aleman TS, Scheetz TE, Sumaroka A, Ehlinger MA, Schwartz SB, Fishman GA, Traboulsi EI, Lam BL, Fulton AB, Mullins RF, Sheffield VC, Jacobson SG
Arch Ophthalmol 2011 Jan;129(1):81-7. doi: 10.1001/archophthalmol.2010.330. PMID: 21220633Free PMC Article
Salomon R, Saunier S, Niaudet P
Pediatr Nephrol 2009 Dec;24(12):2333-44. Epub 2008 Jul 8 doi: 10.1007/s00467-008-0840-z. PMID: 18607645Free PMC Article
Schuermann MJ, Otto E, Becker A, Saar K, Rüschendorf F, Polak BC, Ala-Mello S, Hoefele J, Wiedensohler A, Haller M, Omran H, Nürnberg P, Hildebrandt F
Am J Hum Genet 2002 May;70(5):1240-6. Epub 2002 Mar 27 doi: 10.1086/340317. PMID: 11920287Free PMC Article
Hildebrandt F, Strahm B, Nothwang HG, Gretz N, Schnieders B, Singh-Sawhney I, Kutt R, Vollmer M, Brandis M
Kidney Int 1997 Jan;51(1):261-9. doi: 10.1038/ki.1997.31. PMID: 8995741
Giedion A
Pediatr Radiol 1979 Feb 26;8(1):32-8. doi: 10.1007/BF00973675. PMID: 431989

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