Abstract：
UNASSIGNED: To explore the clinical and molecular characteristics, diagnosis, and treatment of early-onset autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV) in Chinese patients.
UNASSIGNED: A retrospective, interventional case series was assembled from three ADNIV patients.
UNASSIGNED: The three ADNIV cases harbored de novo CAPN5 mutations (p.Arg289Trp and p.Leu73Val). The ages of onset ranged from 11 months to 2 years. All the cases presented with vitreous opacity and subretinal inflammatory exudations. During the postoperative follow-up, all the patients manifested with exaggerated postoperative inflammatory responses. An intravitreal Ozurdex injection could not effectively control ocular inflammation in ADNIV. Laser spots after panretinal photocoagulation were partly visible.
UNASSIGNED: Two de novo CAPN5 mutations (p.Leu73Val and p.Arg289Trp) could cause early-onset ADNIV. Panretinal photocoagulation during vitrectomy and an intravitreal Ozurdex injection could not significantly stop the progression of subretinal exudations and ocular inflammation in early-onset ADNIV patients.
UNASSIGNED: A retrospective, interventional case series was assembled from three ADNIV patients.
UNASSIGNED: The three ADNIV cases harbored de novo CAPN5 mutations (p.Arg289Trp and p.Leu73Val). The ages of onset ranged from 11 months to 2 years. All the cases presented with vitreous opacity and subretinal inflammatory exudations. During the postoperative follow-up, all the patients manifested with exaggerated postoperative inflammatory responses. An intravitreal Ozurdex injection could not effectively control ocular inflammation in ADNIV. Laser spots after panretinal photocoagulation were partly visible.
UNASSIGNED: Two de novo CAPN5 mutations (p.Leu73Val and p.Arg289Trp) could cause early-onset ADNIV. Panretinal photocoagulation during vitrectomy and an intravitreal Ozurdex injection could not significantly stop the progression of subretinal exudations and ocular inflammation in early-onset ADNIV patients.
摘要：
■探索临床和分子特征，诊断,中国患者早发性常染色体显性遗传新生血管性炎性玻璃体视网膜病变（ADNIV）的治疗。
■回顾，介入病例系列由3例ADNIV患者组成。
■三例ADNIV病例均具有从头CAPN5突变（p。Arg289Trp和p.Leu73Val）。发病年龄为11个月至2岁。所有病例均表现为玻璃体混浊和视网膜下炎症渗出。术后随访期间,所有患者均表现为术后炎症反应过度。玻璃体内注射Ozurdex不能有效控制ADNIV中的眼部炎症。全视网膜光凝后的激光斑点部分可见。
■两个从头CAPN5突变（p。Leu73Val和p.Arg289Trp）可引起早发性ADNIV。玻璃体切除术期间的全视网膜光凝和玻璃体内注射Ozurdex不能显着阻止早发性ADNIV患者视网膜下渗出和眼部炎症的进展。
■回顾，介入病例系列由3例ADNIV患者组成。
■三例ADNIV病例均具有从头CAPN5突变（p。Arg289Trp和p.Leu73Val）。发病年龄为11个月至2岁。所有病例均表现为玻璃体混浊和视网膜下炎症渗出。术后随访期间,所有患者均表现为术后炎症反应过度。玻璃体内注射Ozurdex不能有效控制ADNIV中的眼部炎症。全视网膜光凝后的激光斑点部分可见。
■两个从头CAPN5突变（p。Leu73Val和p.Arg289Trp）可引起早发性ADNIV。玻璃体切除术期间的全视网膜光凝和玻璃体内注射Ozurdex不能显着阻止早发性ADNIV患者视网膜下渗出和眼部炎症的进展。
