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The characterization of functional disturbances in Chinese patients with Bietti’s crystalline dystrophy at different fundus stages

  • Retinal Disorders
  • Published:
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Abstract

Purpose

The aim of this study was to characterize the functional and clinical disturbances and screen the optimal functional tests in assessing Bietti’s crystalline dystrophy (BCD) patients by a cross-sectional method.

Methods

The clinical characteristics of BCD were studied in 15 Chinese patients using fundoscopy, fundus fluorescein angiography (FFA), and autofluorescence (AF). The functional features were evaluated by full-field electroretinography (fERG), 85º and 30º perimetry, multifocal ERG (mERG), and chromatic pupillometry.

Results

The 15 patients were separated into three clinical stages according to their fundus features. fERG- and mERG- showed reduced reponses in the early stages. Substages could be further defined according to the fERG results in the intermediate stages. Reduced pupillary light reflex (PLR) activities with blue-and white-light stumili existed in all patients. The most reduced PLR activities were elicited in the advanced stage of patients who had other nonresponsive functional tests.

Conclusions

This study identified the most sensitive functional methods for assessing BCD patients, and the significance of PLR in the advanced stages. In addition, the defined-substages can help us understand the disease more clearly.

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Acknowledgments

This work was supported by the National Basic Research Program of China (973 Program, No. 2007CB512203) and the Innovation and Clinical Technology Fund of Southwest Hospital, Third Military Medical University, Chongqing, China (SWH2007007). The authors thank all of the patients who participated in the study and their family members. They also thank Zhenglin Yang at the Molecular Biology Lab, People's Hospital of Sichuan Province, China, for supporting the genetic study of BCD. The authors thank Dr. T. FitzGibbon for comments and suggestions on earlier drafts of the paper.

Financial disclosures

The authors have no proprietary or commercial interest in any of the materials discussed in this article.

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Author notes

  1. Dan Ning Liu

    Present address: Department of Ophthalmology, The Second Affiliated Hospital, Chongqing Medical University, Chongqing, 400010, China

Authors and Affiliations

  1. Southwest Hospital, Southwest Eye Hospital, Third Military Medical University, Chongqing, 400038, China

    Dan Ning Liu, Yong Liu, Xiao Hong Meng & Zheng Qin Yin

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  1. Dan Ning Liu
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  2. Yong Liu
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Corresponding author

Correspondence to Zheng Qin Yin.

Additional information

All of the authors have full control of all primary data, and they agree to allow Graefe's Archive for Clinical and Experimental Ophthalmology to review the data upon request. The clinical trials had been registered in Chinese Clinical Trial Registry (ChiCTR-TRC-00000411).

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Liu, D.N., Liu, Y., Meng, X.H. et al. The characterization of functional disturbances in Chinese patients with Bietti’s crystalline dystrophy at different fundus stages. Graefes Arch Clin Exp Ophthalmol 250, 191–200 (2012). https://doi.org/10.1007/s00417-011-1809-3

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  • DOI: https://doi.org/10.1007/s00417-011-1809-3

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