Ocular changes in some progressive hereditary nephropathies View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

1987-09

AUTHORS

J. L. Dufier, D. Orssaud, P. Dhermy, M. C. Gubler, M. F. Gagnadoux, M. Broyer

ABSTRACT

Ocular involvement is a common feature of three herediatary nephropathies: cystinosis, nephronophthisis and Alport's syndrome. The follow-up of 25 cases of infantile cystinosis over a period of 26 years demonstrated that the corneal and retinal epithelium were affected in the same way as the kidney epithelium. Corneal involvement induced photophobia and discomfort, but actual blindness was mainly due to retinal involvement, and therefore no corneal graft was performed. The use of topical cysteamine appears to be promising, but its production raises many questions so no definitive conclusions may be made. Since 1965, 51 patients with nephronophthisis have had ocular examinations; all patients had characteristic clinical symptoms and histological findings. The first group, which consisted of 18 children, all under 10 years of age, was found to have obvious chorioretinal degeneration. The second group, which consisted of 11 children, had a normal ocular examination and normal electroretinogram (ERG). The third group, consisting of 22 children, had a normal clinical examination but ERG tracings with variable alterations. In 28 instances of Alport's syndrome, two types of ocular abnormalities have been observed. In six cases, an anterior lenticonus was noted, which caused a major decline in visual acuity. Retinopathy, which did not affect vision, was observed in 13 cases. More... »

PAGES

525-530

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/bf00849264

DOI

http://dx.doi.org/10.1007/bf00849264

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1034432993

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/3153327


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209 rdf:type schema:Organization
210 grid-institutes:grid.412134.1 schema:alternateName Inserm U 192, Hôpital Necker Enfants Malades, Paris, France
211 Service de Néphrologie Pédiatrique, Hôpital Necker-Enfants Malades, 149 Rue de Sèvres, 75015, Paris, France
212 schema:name Inserm U 192, Hôpital Necker Enfants Malades, Paris, France
213 Service de Néphrologie Pédiatrique, Hôpital Necker-Enfants Malades, 149 Rue de Sèvres, 75015, Paris, France
214 rdf:type schema:Organization
215 grid-institutes:grid.414200.3 schema:alternateName Consultation d'Ophtalmologie, Hôpital Laennec, 42 Rue de Sèvres, F-75007, Paris, France
216 schema:name Consultation d'Ophtalmologie, Hôpital Laennec, 42 Rue de Sèvres, F-75007, Paris, France
217 rdf:type schema:Organization
 




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