Cone ERG responses in patients with Smith-Lemli-Opitz Syndrome (SLOS).
Doc Ophthalmol
; 121(2): 85-91, 2010 Oct.
Article
em En
| MEDLINE
| ID: mdl-20440536
ABSTRACT
PURPOSE:
To evaluate cone and cone-driven retinal function in patients with Smith-Lemli-Opitz syndrome (SLOS), a condition characterized by low cholesterol. Rod and rod-driven function in patients with SLOS are known to be abnormal.METHODS:
Electroretinographic (ERG) responses to full-field stimuli presented on a steady, rod suppressing background were recorded in 13 patients who had received long-term cholesterol supplementation. Cone photoresponse sensitivity (S(CONE)) and saturated amplitude (R(CONE)) parameters were estimated using a model of the activation of phototransduction, and post-receptor b-wave and 30 Hz flicker responses were analyzed. The responses of the patients were compared to those of control subjects (N = 13).RESULTS:
Although average values of both S(CONE) and R(CONE) were lower than in controls, the differences were not statistically significant. Post-receptor b-wave amplitude and implicit time and flicker responses were normal.CONCLUSIONS:
The normal cone function contrasts with the significant abnormalities in rod function that were found previously in these same patients. Possibly, cholesterol supplementation has a greater protective effect on cones than on rods as has been demonstrated in the rat model of SLOS.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Células Fotorreceptoras Retinianas Cones
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Síndrome de Smith-Lemli-Opitz
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Eletrorretinografia
Limite:
Adolescent
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Adult
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Child
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Child, preschool
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Female
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Humans
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Male
Idioma:
En
Ano de publicação:
2010
Tipo de documento:
Article