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Ocular manifestations of Alport syndrome

http://www.cnophol.com 2010-7-23 11:08:25 中华眼科在线

 

  Ocular anomalies occurred in 13 patients (40.6%) in our group, but only 3 patients showed anterior lenticonus, and 2 patients showed perimacular retinal flecks. It had been reported that ocular characteristic changes always occurred in the late stage of Alport syndrome. Relatively long course of disease (13.2 years vs 9.3 years) and lower positive rate of patients with characteristic changes in our group also confirmed this viewpoint. In this study, we also found that the characteristic ocular changes occurred in patients with Alport syndrome, especially the anterior lenticonus, was mostly accompanied by sensorineural deafness and renal failure. The findings may suggest that occurrence of ocular characteristic changes, especially the anterior lenticonus, implies a worse prognosis of patients with Alport syndrome.

  In summary, ocular anomalies are not requisite for the diagnosis of Alport syndrome. But ocular examination is a precious help for the diagnosis. Ocular anomalies, especially the typical ones can also determine the prognosis of nephropathy. When confronted with such patients, we should get their renal investigations and audiology examination for an early diagnosis of Alport syndrome and appropriate treatment.

  【参考文献】

  1 Alport AC. Hereditary familial congenital haemorrhagic nephritis. Br Med J 1927;1:504506

  2 Srinivasan M, Uzel SG, Gautieri A, Keten S, Buehler MJ. Alport Syndrome mutations in type IV tropocollagen alter molecular structure and nanomechanical properties. J Structural Biology 2009;168(3):503510

  3 Pescucci C, Mari F, Longo I, Vogiatzi P, Caselli R, Scala E, Abaterusso C, Gusmano R, Seri M, Miglietti N, Bresin E, Renieri A. Autosomaldominant Alport syndrome: natural history of a disease due to COL4A3 or COL4A4 gene. Kidney Int 2004;65(5):1598 1603

  4 Kashtan CE. Alport syndrome and thin glomerular basement membrane disease. J Am Soc Nephrol 1998;9(9):17361750

  5 Flinter F, Chantler C. Alports syndrome: inheritance and clinical features. In: Spitzer A, Auner E, eds. Inheritance of Kidney and Renal Tract Disease. Boston: Kluwer Academic 1990:107

  6 Gregory MC, Terreros DA, Barker DF, Fain PN, Denison JC, Atkin CL. Alport syndromeclinical phenotypes, incidence, and pathology. Contrib

  Nephrol 1996;117:128

  7 Colville DJ, Savige J. Alport syndrome: a review of the ocular manifestation. Ophthalmic Genet 1997;18:161173

  8 Kashtam CE, Michael AF. Alport syndrome: from bedside to genome to bedside. Am J Kidney Dis 1993;22:627640

  9 Jacobs M,Jeffrey B,Kriss A,Taylor D,Sa G,Barratt TM. Ophthalmologic assessment of young patients with Alport syndrome. Ophthalmology 1992;99:10391044

  10 Hentati N, Sellami D, Makni K, Kharrat M, Hachicha J, Hammadi A, Feki J. Ocular findings in Alport syndrome: 32 case studies. J Fr

  Ophthalmol 2008;31:597604

  11 Govan JA. Ocular manifestation of Alports syndrome: a hereditary disorder of basement membranes? Br J Ophthalmol 1983;67:493503

  12 Sargon MF, Celik HH, Sener C. Ultrastructure of the lens epithelium in Alports syndrome. Ophthalmogica 1999; 213:3033

  13 Junk AK, Stefani FH, Ludwig K. Bilateral anterior lenticonus. Arch

  Ophthalmol 2000;118:895897

  14 Gehrs KM, Pollock SC, Zilkha G. Clinical features and pathogenesis of Alport retinopathy. Retina 1995;15:305311

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