Haim M, Holm N V, Rosenberg T
National Eye Clinic for the Visually Impaired, Copenhagen, Denmark.
Acta Ophthalmol (Copenh). 1992 Apr;70(2):165-77. doi: 10.1111/j.1755-3768.1992.tb04120.x.
The aim of this study was a complete survey of all patients in Denmark with a diagnosis of retinitis pigmentosa. The study was performed during the period 1986-1989 and included all persons living in Denmark from 1850 to 1989. We describe the methods of registration from medical and non-medical sources, based on defined criteria. The inclusion criteria were fulfilled by 1890 persons, 1056 males and 834 females. Evaluation of this 'Danish Retinitis Pigmentosa Register' demonstrates an overall completeness of about 80%, increasing from 40% for birth cohorts before 1911 to 90% for patients born since 1981. Concerning diagnostic confidence, 73% of the probands were characterized as certain, 16% as probable, and 11% as possible. Non-systemic cases comprised 61% of all probands and 31% had systemic disease, the rest being unclassified with respect to systemic involvement. The material is considered very suitable for epidemiological treatment and continuous clinical and genetic investigations.
本研究的目的是对丹麦所有诊断为色素性视网膜炎的患者进行全面调查。该研究在1986年至1989年期间进行，涵盖了1850年至1989年居住在丹麦的所有人。我们基于既定标准描述了从医学和非医学来源进行登记的方法。共有1890人符合纳入标准，其中男性1056人，女性834人。对这个“丹麦色素性视网膜炎登记册”的评估表明，总体完整性约为80%，从1911年以前出生队列的40%增至1981年以后出生患者的90%。关于诊断可信度，73%的先证者被判定为确定，16%为可能，11%为疑似。非系统性病例占所有先证者的61%，31%患有系统性疾病，其余在系统性受累方面未分类。该资料被认为非常适合进行流行病学研究以及持续的临床和遗传学调查。
