Follow up Study on Visual Prognosis and Complications of Ectopia Lentis Lens

Обзор

This is a single-centre, observational cohort study with the following objectives: Firstly, the investigators aim to evaluate the vision-related quality of life and mental health status in congenital ectopia lentis patients. Secondly, the investigators target to investigate the correlations between genotype and clinical phenotype in patients of congenital ectopia lentis. Thirdly, to explore the metabolic profiles in the aqueous humor (AH) of patients with congenital ectopia lentis. Lastly, this study aims to explore the longitudinal changes of ocular biological parameters and complications in patients with postoperative congenital ectopia lens in the Chinese population.

Подробное описание

Congenital ectopia lens is a rare ophthalmic disease that seriously harms the physical and mental health of patients. Surgery is the only definite treatment at present. However, the changes in vision-related quality of life, mental health status, ocular biological parameters postoperative congenital dislocation of lens have not been systematically studied in the Chinese population.

In this trial, the investigators aim to evaluate the vision-related quality of life and mental health status in congenital ectopia lentis patients, investigate the correlations between genotype and clinical phenotype in patients of congenital ectopia lentis, explore the metabolic profiles in the aqueous humor (AH) of patients with congenital ectopia lentis, and explore the longitudinal changes of ocular biological parameters and complications in patients with postoperative congenital ectopia lens in the Chinese population.

Первичные конечные точки

• Change of best corrected visual acuity [Срок оценки: Non-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation, 3 months, and 1, 2, 3 years postoperation]
• Incidence of complications [Срок оценки: Non-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation, 3 months, and 1, 2, 3 years postoperation.]

Вторичные конечные точки (12)

• Change of axial length [Срок оценки: Non-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation, 3 months, and 1, 2, 3 years postoperation.]
• High order aberrations [Срок оценки: Non-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation, 3 months, and 1, 2, 3 years postoperation]
• Central cornea endothelial cell loss [Срок оценки: Preoperation, and 3 months, and 1, 2, 3 years postoperation]
• The state of zonules [Срок оценки: Non-surgical group is assessed at first visit, and 1, 2, 3 years after enrollment. Surgical group will be assessed preoperation.]
• Anterior chamber angle. [Срок оценки: Non-surgical group is assessed at first visit, and 1, 2, 3 years after enrollment. Surgical group will be assessed preoperation, 3 months, and 1, 2, 3 years postoperation.]
• Tilt and eccentricity of intraocular lens [Срок оценки: 3 months, and 1, 2, 3 years postoperation]
• Intraocular pressure [Срок оценки: Non-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation, 3 months, and 1, 2, 3 years postoperation.]
• Aortic root diameter [Срок оценки: Non-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation and 1, 2, 3 years postoperation]
• Aortic Root (Sinuses of Valsalva) Z-score, adjusted by body-surface-area (Z-score) [Срок оценки: Non-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation and 1, 2, 3 years postoperation]
• Incidence of valvular heart disease [Срок оценки: Non-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation and 1, 2, 3 years postoperation]
• Body mass index (BMI) [Срок оценки: Non-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation and 1, 2, 3 years postoperation.]
• Metacarpophalangeal joint length [Срок оценки: Non-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation and 1, 2, 3 years postoperation.]

Критерии участия

Критерии включения

• Diagnosed with congenital lens dislocation and age ≥ 3 years old.
• Agree to participate in this study with written informed consent from patients or legal guardians.

Критерии исключения

• History of ophthalmic trauma or other ophthalmic surgeries.
• Combined with other ophthalmic diseases such as primary glaucoma, uveitis and corneal disease.
• Patients who could not cooperate in the examinations.

Критерии приведены из реестра в оригинале (на английском). Окончательную оценку соответствия проводит исследовательский центр.

Здоровые добровольцы: Да

Дизайн исследования

Модель наблюдения

Когортное

Центры проведения

Китай · 1 центр

• Guangdong Provincial Clinical Research Center for Ocular Diseases, State Key Laboratory of — Гуанчжоу

Публикации

• Kaur K, Gurnani B. Ectopia Lentis. 2023 Jun 11. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2026 Jan-. Available from http://www.ncbi.nlm.nih.gov/books/NBK578193/ PMID 35201721
• Lin J, Gong N, Cao Q, Zhou Y, Cai Y, Jin G, Young CA, Yang J, Wang Y, Zheng D. What hinders congenital ectopia lentis patients' follow-up visits? A qualitative study. BMJ Open. 2020 Mar 12;10(3):e030434. doi: 10.1136/bmjopen-2019-030434. PMID 32169922
• Guo D, Jin G, Zhou Y, Zhang X, Cao Q, Lian Z, Guo Y, Zheng D. Mutation spectrum and genotype-phenotype correlations in Chinese congenital ectopia lentis patients. Exp Eye Res. 2021 Jun;207:108570. doi: 10.1016/j.exer.2021.108570. Epub 2021 Apr 16. PMID 33844962
• Minsel WR, Bommert H, Pieritz R. [Relationship between speech--formal psychotherapeutic signs and results of client-centered discussion psychotherapy]. Z Klin Psychol Psychother. 1972;20(4):303-10. No abstract available. German. PMID 4662920
• Jin GM, Fan M, Cao QZ, Lin JX, Zhang YC, Lin JQ, Wang YY, Young CA, Zheng DY. Trends and characteristics of congenital ectopia lentis in China. Int J Ophthalmol. 2018 Sep 18;11(9):1545-1549. doi: 10.18240/ijo.2018.09.19. eCollection 2018. PMID 30225232
• Meng X, Cao X, Jia Y, Pan J, Du Y, Li X. Sutured Scleral Fixation of Posterior Chamber Intraocular Lens in Children under the Age of 9 with Congenital Ectopia Lentis. Ophthalmic Res. 2021;64(5):837-843. doi: 10.1159/000516324. Epub 2021 Jul 9. PMID 34247162
• Chen T, Chen J, Jin G, Zhang M, Chen Z, Zheng D, Jiang Y. Clinical Ocular Diagnostic Model of Marfan Syndrome in Patients With Congenital Ectopia Lentis by Pentacam AXL System. Transl Vis Sci Technol. 2021 Jun 1;10(7):3. doi: 10.1167/tvst.10.7.3. PMID 34061947
• Chen Z, Chen T, Zhang M, Chen J, Deng M, Zheng J, Lan LN, Jiang Y. Fibrillin-1 gene mutations in a Chinese cohort with congenital ectopia lentis: spectrum and genotype-phenotype analysis. Br J Ophthalmol. 2022 Dec;106(12):1655-1661. doi: 10.1136/bjophthalmol-2021-319084. Epub 2021 Jul 19. PMID 34281902

Идентификаторы

NCT: NCT05654025 · 2022KYPJ207

Первоисточники (государственные реестры)

• ClinicalTrials.gov (NIH, США)

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