Bilateral anterior lenticonus in a young patient with  Alport’s syndrome: Alport sendromuna bağlı bilateral anterior lentikonüslü genç bir hasta

Adnan  Aksoy; İbrahim Yaşar; Murat Aslankurt; Lokman Aslan; Oğuz Oğuzhan

doi:10.5455/GMJ-30-163945

Authors

Adnan Aksoy Departments of Ophtalmology Sütçü İmam University Medical School
İbrahim Yaşar Departments of Ophtalmology Sütçü İmam University Medical School
Murat Aslankurt Departments of Ophtalmology Sütçü İmam University Medical School
Lokman Aslan Departments of Ophtalmology Sütçü İmam University Medical School
Oğuz Oğuzhan Departments of ENT Diseases, Sütçü İmam University Medical School

DOI:

https://doi.org/10.5455/GMJ-30-163945

Keywords:

Alport’s syndrome, anterior lenticonus, renal dysfunction, hearing dysfunction

Abstract

Alport's syndrome is a familial disease affecting basement membrane collagen and characterized by progressive kidney disfunction, hearing disorder and ocular abnormalities. Twenty four years old man presented with visual impairment in our outpatients clinic. Bilateral anterior lenticonus were seen in ocular slit lamp examination. After clinical examinations, audiological tests and nephrological evaluation, the case was diagnosed as Alport's syndrome. Although males and females are affected equally in this disease, males have poorer prognosis than females owing to renal failure. It is intended to remind this uncommon disease and create awareness for ophthalmologists in the present article.

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References

Kashtan CE. Alport Syndrome: Phenotypic heterogenity of progressive hereditary nephritis. Pediatr Nephrol 2000; 14: 502-512.

McCarty PA, Maino DM. Alport syndrome: A review. Clinical Eye and Vision Care 2000; 12: 139-150.

Flinter FA, Cameron JS, Chantler C, Houston I, Bobrow M. Genetics of classic Alport’s syndrome. The Lancet 1988; Oct 29: 1232-41.

Yıldırım C, Yakut E, Altınsoy Hİ. Alport Sendromunda Göz Bulguları. Türkiye Klinikleri Oftalmoloji Dergisi 1998; 7(2): 134-36.

Colville DJ, Savige J. Alport syndrome. A review of the ocular manifestations. Ophthalmic Genet 1997; 18(4): 161-73.

Seymenoğlu G, Baser EF. Ocular manifestations and surgical results in patients with Alport syndrome. J Cataract Refract Surg 2009; 35(7): 1302-6.

Aslanzadeh GA, Gharabaghi D, Naderi N. Clear lens phacoemulsification in the anterior lenticonus due to Alport Syndrome: two case reports. J Med Case Reports. 2008; 2: 178.

Gupta A, Ramesh Babu K, Srinivasan R, Mohanty D. Clear lens extraction in Alport syndrome with combined anterior and posterior lenticonus or ruptured anterior lens capsule. J Cataract Refract Surg. 2011; 37(11): 2075-8.

Liu YB, Tan SJ, Sun ZY, Li X, Huang BY, Hu QM. Clear lens phacoemulsification with continuous curvilinear capsulorhexis and foldable intraocular lens implantation for the treatment of a patient with bilateral anterior lenticonus due to Alport syndrome. J Int Med Res. 2008 Nov Dec;36(6):1440-4.

Grondalski SJ, Bennet GR. Alport's syndrome. Review and case report. Optometry &Vision Science 1989; 66: 396-8.

Pajari H, Setälä K, Heiskari N, Kääriäinen H, Rosenlöf K, Koskimies O. Ocular findings in 34 patients with alport syndrome: Correlation of the findings to mutations in COL4A5 gene. Acta Ophthalmologica Scandinavica 1999; 77: 214-7.

Yin-Yin C, You-Ming P, Yu-Mei L. Delayed diagnosis of Alport syndrome without hematuria. Iran J Kidney Dis 2014; 8(3): 250-1.

Orhan D. Herediter nefropatiler. 22. Ulusal Patoloji Kongresi, Nefropatoloji kursu, Antalya 2012.

Nadasdy T, Sişlva FG. Adult renal disease, In diagnostic surgical pathology. Eds. Sternberg SS, Antonioli DA, Carter D, Mills SE, Oberman HA. Vol 23rd ed. Lippincott Williams & Wilkins Phildalphia. 1999; 1733-6.

Kashtan CE, Michael AF. Alport syndrome. Kidney Int 1996; 50: 1445-63.