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IMPAIRED DARK ADAPTATION ASSOCIATED WITH A DISEASED BRUCH MEMBRANE IN PSEUDOXANTHOMA ELASTICUM

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IMPAIRED DARK ADAPTATION ASSOCIATED WITH A DISEASED BRUCH MEMBRANE IN PSEUDOXANTHOMA ELASTICUM. / Hess, Kristina; Gliem, Martin; Birtel, Johannes; Müller, Philipp; Hendig, Doris; Andrews, Colm; Murray, Ian J; Holz, Frank G; Charbel Issa, Peter.

In: RETINA-J RET VIT DIS, Vol. 40, No. 10, 10.2020, p. 1988-1995.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Hess, K, Gliem, M, Birtel, J, Müller, P, Hendig, D, Andrews, C, Murray, IJ, Holz, FG & Charbel Issa, P 2020, 'IMPAIRED DARK ADAPTATION ASSOCIATED WITH A DISEASED BRUCH MEMBRANE IN PSEUDOXANTHOMA ELASTICUM', RETINA-J RET VIT DIS, vol. 40, no. 10, pp. 1988-1995. https://doi.org/10.1097/IAE.0000000000002689

APA

Hess, K., Gliem, M., Birtel, J., Müller, P., Hendig, D., Andrews, C., Murray, I. J., Holz, F. G., & Charbel Issa, P. (2020). IMPAIRED DARK ADAPTATION ASSOCIATED WITH A DISEASED BRUCH MEMBRANE IN PSEUDOXANTHOMA ELASTICUM. RETINA-J RET VIT DIS, 40(10), 1988-1995. https://doi.org/10.1097/IAE.0000000000002689

Vancouver

Hess K, Gliem M, Birtel J, Müller P, Hendig D, Andrews C et al. IMPAIRED DARK ADAPTATION ASSOCIATED WITH A DISEASED BRUCH MEMBRANE IN PSEUDOXANTHOMA ELASTICUM. RETINA-J RET VIT DIS. 2020 Oct;40(10):1988-1995. https://doi.org/10.1097/IAE.0000000000002689

Bibtex

@article{c0cafe7fd01e47249e97bc2c398535dd,
title = "IMPAIRED DARK ADAPTATION ASSOCIATED WITH A DISEASED BRUCH MEMBRANE IN PSEUDOXANTHOMA ELASTICUM",
abstract = "PURPOSE: To characterize dark adaptation in patients with pseudoxanthoma elasticum, a systemic disease leading to calcification of elastic tissue including the Bruch membrane.METHODS: In this prospective case-control study, dark adaptation thresholds were measured using a Goldmann-Weekers dark adaptometer. Additional assessments included best-corrected visual acuity testing, contrast sensitivity, low luminance deficit, and vision-related quality of life.RESULTS: Dark adaptation thresholds were significantly higher, and adaptation periods were prolonged in patients with pseudoxanthoma elasticum (n = 35; 33 with 2 ABCC6 mutations) compared with controls (n = 35). The time to adapt 4 log units (20.6 ± 8.6 vs. 8.0 ± 1.3 minutes) and the mean dark adaptation threshold after 15 minutes (3.5 ± 1.1 vs. 1.8 ± 0.2 log units) were significantly different between patients and controls (both P < 0.001). Low luminance deficits (12.3 ± 6.4 vs. 6.1 ± 4.3 ETDRS letters), contrast sensitivity (1.4 ± 0.3 vs. 1.9 ± 0.1), and low luminance-related quality of life (LLQ score: 1,286 ± 355 vs. 2,167 ± 68) were also significantly worse in patients with pseudoxanthoma elasticum (all, P < 0.001). Two patients were treated with high-dose vitamin A which partially reversed impaired dark adaptation.CONCLUSION: Patients with pseudoxanthoma elasticum often have impaired dark adaptation. Positive effects of vitamin A supplementation may indicate restricted retinal access of vitamin A through the Bruch membrane as one possible underlying pathogenic factor.",
keywords = "Adolescent, Adult, Aged, Bruch Membrane/pathology, Case-Control Studies, Contrast Sensitivity/physiology, Cross-Sectional Studies, Dark Adaptation/physiology, Female, Humans, Male, Middle Aged, Prospective Studies, Pseudoxanthoma Elasticum/drug therapy, Quality of Life, Retinal Diseases/drug therapy, Visual Acuity/physiology, Vitamin A/administration & dosage, Young Adult",
author = "Kristina Hess and Martin Gliem and Johannes Birtel and Philipp M{\"u}ller and Doris Hendig and Colm Andrews and Murray, {Ian J} and Holz, {Frank G} and {Charbel Issa}, Peter",
year = "2020",
month = oct,
doi = "10.1097/IAE.0000000000002689",
language = "English",
volume = "40",
pages = "1988--1995",
journal = "RETINA-J RET VIT DIS",
issn = "0275-004X",
publisher = "Lippincott Williams and Wilkins",
number = "10",

}

RIS

TY - JOUR

T1 - IMPAIRED DARK ADAPTATION ASSOCIATED WITH A DISEASED BRUCH MEMBRANE IN PSEUDOXANTHOMA ELASTICUM

AU - Hess, Kristina

AU - Gliem, Martin

AU - Birtel, Johannes

AU - Müller, Philipp

AU - Hendig, Doris

AU - Andrews, Colm

AU - Murray, Ian J

AU - Holz, Frank G

AU - Charbel Issa, Peter

PY - 2020/10

Y1 - 2020/10

N2 - PURPOSE: To characterize dark adaptation in patients with pseudoxanthoma elasticum, a systemic disease leading to calcification of elastic tissue including the Bruch membrane.METHODS: In this prospective case-control study, dark adaptation thresholds were measured using a Goldmann-Weekers dark adaptometer. Additional assessments included best-corrected visual acuity testing, contrast sensitivity, low luminance deficit, and vision-related quality of life.RESULTS: Dark adaptation thresholds were significantly higher, and adaptation periods were prolonged in patients with pseudoxanthoma elasticum (n = 35; 33 with 2 ABCC6 mutations) compared with controls (n = 35). The time to adapt 4 log units (20.6 ± 8.6 vs. 8.0 ± 1.3 minutes) and the mean dark adaptation threshold after 15 minutes (3.5 ± 1.1 vs. 1.8 ± 0.2 log units) were significantly different between patients and controls (both P < 0.001). Low luminance deficits (12.3 ± 6.4 vs. 6.1 ± 4.3 ETDRS letters), contrast sensitivity (1.4 ± 0.3 vs. 1.9 ± 0.1), and low luminance-related quality of life (LLQ score: 1,286 ± 355 vs. 2,167 ± 68) were also significantly worse in patients with pseudoxanthoma elasticum (all, P < 0.001). Two patients were treated with high-dose vitamin A which partially reversed impaired dark adaptation.CONCLUSION: Patients with pseudoxanthoma elasticum often have impaired dark adaptation. Positive effects of vitamin A supplementation may indicate restricted retinal access of vitamin A through the Bruch membrane as one possible underlying pathogenic factor.

AB - PURPOSE: To characterize dark adaptation in patients with pseudoxanthoma elasticum, a systemic disease leading to calcification of elastic tissue including the Bruch membrane.METHODS: In this prospective case-control study, dark adaptation thresholds were measured using a Goldmann-Weekers dark adaptometer. Additional assessments included best-corrected visual acuity testing, contrast sensitivity, low luminance deficit, and vision-related quality of life.RESULTS: Dark adaptation thresholds were significantly higher, and adaptation periods were prolonged in patients with pseudoxanthoma elasticum (n = 35; 33 with 2 ABCC6 mutations) compared with controls (n = 35). The time to adapt 4 log units (20.6 ± 8.6 vs. 8.0 ± 1.3 minutes) and the mean dark adaptation threshold after 15 minutes (3.5 ± 1.1 vs. 1.8 ± 0.2 log units) were significantly different between patients and controls (both P < 0.001). Low luminance deficits (12.3 ± 6.4 vs. 6.1 ± 4.3 ETDRS letters), contrast sensitivity (1.4 ± 0.3 vs. 1.9 ± 0.1), and low luminance-related quality of life (LLQ score: 1,286 ± 355 vs. 2,167 ± 68) were also significantly worse in patients with pseudoxanthoma elasticum (all, P < 0.001). Two patients were treated with high-dose vitamin A which partially reversed impaired dark adaptation.CONCLUSION: Patients with pseudoxanthoma elasticum often have impaired dark adaptation. Positive effects of vitamin A supplementation may indicate restricted retinal access of vitamin A through the Bruch membrane as one possible underlying pathogenic factor.

KW - Adolescent

KW - Adult

KW - Aged

KW - Bruch Membrane/pathology

KW - Case-Control Studies

KW - Contrast Sensitivity/physiology

KW - Cross-Sectional Studies

KW - Dark Adaptation/physiology

KW - Female

KW - Humans

KW - Male

KW - Middle Aged

KW - Prospective Studies

KW - Pseudoxanthoma Elasticum/drug therapy

KW - Quality of Life

KW - Retinal Diseases/drug therapy

KW - Visual Acuity/physiology

KW - Vitamin A/administration & dosage

KW - Young Adult

U2 - 10.1097/IAE.0000000000002689

DO - 10.1097/IAE.0000000000002689

M3 - SCORING: Journal article

C2 - 31834130

VL - 40

SP - 1988

EP - 1995

JO - RETINA-J RET VIT DIS

JF - RETINA-J RET VIT DIS

SN - 0275-004X

IS - 10

ER -