Skip to main content

Alport Syndrome

  • Chapter
  • First Online:
Inherited Chorioretinal Dystrophies

Abstract

Alport syndrome (AS) is a progressive haematuric nephritis, associated with high tone sensorineural deafness and inconstant ocular involvement first described as a dominantly inherited disorder in 1927 [1]. The eyes may present microphthalmia, a polymorph posterior corneal dystrophy, a subcapsular cataract, a bilateral and progressive anterior or posterior lenticonus and a dot-like peripheral retinal dystrophy with macular degeneration (Fig. 41.1).

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 139.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 199.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Alport AC. Hereditary familial congenital haemorrhagic nephritis. Br Med J. 1927;I:504–6.

    Article  Google Scholar 

  2. 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.

    Article  PubMed Central  Google Scholar 

  3. Govan JA. Ocular manifestations of Alport’s syndrome: a hereditary disorder of basement membranes? Br J Ophthalmol. 1983;67:493–503.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  4. Hentati N, Sellami D, Makni K, Kharrat M, Hachicha J, Hammadi A, Feki J. Atteinte oculaire au cours du syndrome d’Alport. A propos de 32 cas. J Fr Ophtalmol. 2008;31:597–604.

    Article  CAS  PubMed  Google Scholar 

  5. McCarthy PA, Maino DM. Alport syndrome: a review. Clin Eye Vis Care. 2000;12:139–50.

    Article  PubMed  Google Scholar 

  6. Setâlä K, Ruusuvaara P. Alport syndrome with hereditary macular degeneration. Acta Ophthalmol. 1989;67:409–14.

    Article  Google Scholar 

  7. Ramesh Saxena. Alport syndrome. http://www.emedicine.com/med/topic110.htm. 2008.

  8. Teekhasaenee C, Nimmanit S, Wutthiphan S, Vareesangthip K, Laohapand T, Malasitr P, Ritch R. Posterior polymorphous dystrophy and Alport syndrome. Ophthalmology. 1991;98:1207–15.

    Article  CAS  PubMed  Google Scholar 

  9. Yasuzumi K, Futagami S, Kiyosawa M, Mochizuki M. Bilateral serous retinal detachment associated with Alport’s syndrome. Ophthalmologica. 2000;214:301–4.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Bernard Puech .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Puech, B., De Laey, JJ. (2014). Alport Syndrome. In: Puech, B., De Laey, JJ., Holder, G. (eds) Inherited Chorioretinal Dystrophies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-69466-3_41

Download citation

  • DOI: https://doi.org/10.1007/978-3-540-69466-3_41

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-69464-9

  • Online ISBN: 978-3-540-69466-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics