The Epidemiology of Stargardt Disease in the United Kingdom

Kurt Spiteri Cornish, Jason Ho, Susan Downes, Neil W Scott, James Bainbridge, Noemi Lois

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Abstract

Purpose

To establish the incidence of Stargardt disease (STGD) in the United Kingdom and define baseline characteristics of newly diagnosed patients.

Design

Prospective epidemiologic study undertaken under the auspices of the British Ophthalmological Surveillance Unit (BOSU).

Participants

New incident cases of STGD in the United Kingdom reported by ophthalmologists to BOSU during a 12-month period, from June 1, 2012, to June 1, 2013.

Methods

Once a new case of STGD was reported, an incident questionnaire was sent to the reporting ophthalmologist, followed by a follow-up questionnaire (when required) 6 months later.

Main Outcome Measures

Patient demographics, baseline characteristics including visual acuity, and findings on slit-lamp biomicroscopy, as well as diagnostic technologies undertaken at baseline and their findings, including electrophysiology, fundus autofluorescence, fluorescein angiography, and genetic testing.

Results

A total of 81 new cases of STGD were reported during the 12-month period of the study; baseline data were obtained on 70 (86%) of these. These results suggest an annual incidence in the United Kingdom of between 0.110 and 0.128 per 100 000 individuals. The median age of patients at presentation was 27 years, the majority were British (77%), and most (90%) were symptomatic, with a median visual acuity of 0.52 logMAR (Snellen equivalent 20/66).

Conclusions

Even considering possible limitations related to incomplete ascertainment, this is the first prospective epidemiology study that provides indication of the incidence of STGD in the United Kingdom. The incidence of STGD estimated herein appears to be lower than that repeatedly quoted in the literature. Fundus autofluoresence and electrophysiology testing are most commonly used for the evaluation of patients with STGD.
Original languageEnglish
Pages (from-to)508-513
Number of pages6
JournalOphthalmology Retina
Volume1
Issue number6
Early online date21 Apr 2017
DOIs
Publication statusPublished - Nov 2017

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Epidemiology
Electrophysiology
Incidence
Visual Acuity
Fluorescein Angiography
Genetic Testing
Stargardt disease 1
United Kingdom
Epidemiologic Studies
Demography
Prospective Studies
Technology
Ophthalmologists
Surveys and Questionnaires

Cite this

The Epidemiology of Stargardt Disease in the United Kingdom. / Spiteri Cornish, Kurt; Ho, Jason; Downes, Susan; Scott, Neil W; Bainbridge, James; Lois, Noemi.

In: Ophthalmology Retina, Vol. 1, No. 6, 11.2017, p. 508-513.

Research output: Contribution to journalArticle

Spiteri Cornish, Kurt ; Ho, Jason ; Downes, Susan ; Scott, Neil W ; Bainbridge, James ; Lois, Noemi. / The Epidemiology of Stargardt Disease in the United Kingdom. In: Ophthalmology Retina. 2017 ; Vol. 1, No. 6. pp. 508-513.
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abstract = "PurposeTo establish the incidence of Stargardt disease (STGD) in the United Kingdom and define baseline characteristics of newly diagnosed patients.DesignProspective epidemiologic study undertaken under the auspices of the British Ophthalmological Surveillance Unit (BOSU).ParticipantsNew incident cases of STGD in the United Kingdom reported by ophthalmologists to BOSU during a 12-month period, from June 1, 2012, to June 1, 2013.MethodsOnce a new case of STGD was reported, an incident questionnaire was sent to the reporting ophthalmologist, followed by a follow-up questionnaire (when required) 6 months later.Main Outcome MeasuresPatient demographics, baseline characteristics including visual acuity, and findings on slit-lamp biomicroscopy, as well as diagnostic technologies undertaken at baseline and their findings, including electrophysiology, fundus autofluorescence, fluorescein angiography, and genetic testing.ResultsA total of 81 new cases of STGD were reported during the 12-month period of the study; baseline data were obtained on 70 (86{\%}) of these. These results suggest an annual incidence in the United Kingdom of between 0.110 and 0.128 per 100 000 individuals. The median age of patients at presentation was 27 years, the majority were British (77{\%}), and most (90{\%}) were symptomatic, with a median visual acuity of 0.52 logMAR (Snellen equivalent 20/66).ConclusionsEven considering possible limitations related to incomplete ascertainment, this is the first prospective epidemiology study that provides indication of the incidence of STGD in the United Kingdom. The incidence of STGD estimated herein appears to be lower than that repeatedly quoted in the literature. Fundus autofluoresence and electrophysiology testing are most commonly used for the evaluation of patients with STGD.",
author = "{Spiteri Cornish}, Kurt and Jason Ho and Susan Downes and Scott, {Neil W} and James Bainbridge and Noemi Lois",
note = "The authors thank the British Ophthalmological Surveillance Unit (BOSU) for the support received, as well as Mr Barnaby Foot, research coordinator for BOSU, for his help and advice on this project. The authors thank the following ophthalmologists who assisted with data collection for this study: N. Acharya, S. Anwar, V. Bansal, P.N. Bishop, D. Byles, J.S. Chawla, A. Churchill, M. Clarke, B. Dhillon, M. Ekstein, S. George, J. Gillian, J.T. Gillow, D. Gilmour, R. Gray, P.T.S. Gregory, R. Gupta, S.P. Kelly, I.C. Lloyd, A. Lotery, M. McKibbin, R. MacLaren, G. Menon, A.T. Moore, A. Mulvihill, Y. Osoba, R. Pilling, H. Porooshani, A. Raghu Ram, T. Rimmer, I. Russell-Eggitt, M. Sarhan, R. Savides, S. Shafquat, A. Smith, A. Tekriwal, P. Tesha, P. Watts.",
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T1 - The Epidemiology of Stargardt Disease in the United Kingdom

AU - Spiteri Cornish, Kurt

AU - Ho, Jason

AU - Downes, Susan

AU - Scott, Neil W

AU - Bainbridge, James

AU - Lois, Noemi

N1 - The authors thank the British Ophthalmological Surveillance Unit (BOSU) for the support received, as well as Mr Barnaby Foot, research coordinator for BOSU, for his help and advice on this project. The authors thank the following ophthalmologists who assisted with data collection for this study: N. Acharya, S. Anwar, V. Bansal, P.N. Bishop, D. Byles, J.S. Chawla, A. Churchill, M. Clarke, B. Dhillon, M. Ekstein, S. George, J. Gillian, J.T. Gillow, D. Gilmour, R. Gray, P.T.S. Gregory, R. Gupta, S.P. Kelly, I.C. Lloyd, A. Lotery, M. McKibbin, R. MacLaren, G. Menon, A.T. Moore, A. Mulvihill, Y. Osoba, R. Pilling, H. Porooshani, A. Raghu Ram, T. Rimmer, I. Russell-Eggitt, M. Sarhan, R. Savides, S. Shafquat, A. Smith, A. Tekriwal, P. Tesha, P. Watts.

PY - 2017/11

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N2 - PurposeTo establish the incidence of Stargardt disease (STGD) in the United Kingdom and define baseline characteristics of newly diagnosed patients.DesignProspective epidemiologic study undertaken under the auspices of the British Ophthalmological Surveillance Unit (BOSU).ParticipantsNew incident cases of STGD in the United Kingdom reported by ophthalmologists to BOSU during a 12-month period, from June 1, 2012, to June 1, 2013.MethodsOnce a new case of STGD was reported, an incident questionnaire was sent to the reporting ophthalmologist, followed by a follow-up questionnaire (when required) 6 months later.Main Outcome MeasuresPatient demographics, baseline characteristics including visual acuity, and findings on slit-lamp biomicroscopy, as well as diagnostic technologies undertaken at baseline and their findings, including electrophysiology, fundus autofluorescence, fluorescein angiography, and genetic testing.ResultsA total of 81 new cases of STGD were reported during the 12-month period of the study; baseline data were obtained on 70 (86%) of these. These results suggest an annual incidence in the United Kingdom of between 0.110 and 0.128 per 100 000 individuals. The median age of patients at presentation was 27 years, the majority were British (77%), and most (90%) were symptomatic, with a median visual acuity of 0.52 logMAR (Snellen equivalent 20/66).ConclusionsEven considering possible limitations related to incomplete ascertainment, this is the first prospective epidemiology study that provides indication of the incidence of STGD in the United Kingdom. The incidence of STGD estimated herein appears to be lower than that repeatedly quoted in the literature. Fundus autofluoresence and electrophysiology testing are most commonly used for the evaluation of patients with STGD.

AB - PurposeTo establish the incidence of Stargardt disease (STGD) in the United Kingdom and define baseline characteristics of newly diagnosed patients.DesignProspective epidemiologic study undertaken under the auspices of the British Ophthalmological Surveillance Unit (BOSU).ParticipantsNew incident cases of STGD in the United Kingdom reported by ophthalmologists to BOSU during a 12-month period, from June 1, 2012, to June 1, 2013.MethodsOnce a new case of STGD was reported, an incident questionnaire was sent to the reporting ophthalmologist, followed by a follow-up questionnaire (when required) 6 months later.Main Outcome MeasuresPatient demographics, baseline characteristics including visual acuity, and findings on slit-lamp biomicroscopy, as well as diagnostic technologies undertaken at baseline and their findings, including electrophysiology, fundus autofluorescence, fluorescein angiography, and genetic testing.ResultsA total of 81 new cases of STGD were reported during the 12-month period of the study; baseline data were obtained on 70 (86%) of these. These results suggest an annual incidence in the United Kingdom of between 0.110 and 0.128 per 100 000 individuals. The median age of patients at presentation was 27 years, the majority were British (77%), and most (90%) were symptomatic, with a median visual acuity of 0.52 logMAR (Snellen equivalent 20/66).ConclusionsEven considering possible limitations related to incomplete ascertainment, this is the first prospective epidemiology study that provides indication of the incidence of STGD in the United Kingdom. The incidence of STGD estimated herein appears to be lower than that repeatedly quoted in the literature. Fundus autofluoresence and electrophysiology testing are most commonly used for the evaluation of patients with STGD.

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DO - 10.1016/j.oret.2017.03.001

M3 - Article

VL - 1

SP - 508

EP - 513

JO - Ophthalmology Retina

JF - Ophthalmology Retina

SN - 2468-6530

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ER -