This introductory lecture to the epidemiological session in the Workshop on the Exfoliation Syndrome (ES) gathers together figures for the prevalence of ES around the world. Prevalence figures from published reports are shown in the text separately for each country. Four ways of comparing the prevalences are used. 1) Prevalences in people over 60 years of age, 2) Percentages of glaucoma in persons with ES, 3) Percentages of ES in patients with glaucoma or ocular hypertension, with separate statistics for the proportion of capsular glaucoma in patients treated with laser trabeculoplasty (LTP), 4) Prevalence of ES in patients with cataract. The major differences in prevalence can partly be explained by the different techniques used in the investigations. Very few authors have studied people in different countries, which is the best way of' obtaining comparable results. The author has personally studied Finns, Lapps, Eskimos in Greenland, Canada and Alaska, Icelanders, populations in Tunis, India and Peru and four populations in the USSR by the same technique. The prevalences vary from 0 070 in Eskimos to 21 Vo in Finns over 60 years of age, and are at the same high level in Lapps, Finns, Russians in Novosibirsk and Icelanders, but significantly lower in all the others. The results support the opinion that ES is not uniformly distributed in all countries, and this is confirmed by many reports from different countries in this workshop.
ABSTRACT.Purpose: To investigate exfoliation syndrome (ES) in order to elucidate gender distribution and the roles of genetic and climatic factors in its manifestation. Material and Methods:We studied the gender distribution of ES and the association between ES and the appearance of certain climatically induced disorders (pterygium, climatic droplet keratopathy) and the size of pingueculae in populations living in the Arctic region, in temperate regions and in tropical regions. This involved a total of 11 samples taken in eight different countries, comprising 2206 persons of both genders over the age of 50 years. Results: A total of 1051 males were investigated for ES and 147 of them (14.0%) were found to have it. The corresponding figures for females were 1093 and 177 (16.2%). After standardization according to age, no systematic difference between the genders was found in the study. The frequency of ES varied greatly. It was not observed at all in the Inuit (Eskimos) but was found most frequently (about 30%) among the Saami (Lapps), Åland Islanders, Finns, Icelanders and Russians over 70 years of age. After the age of 50, the frequency of ES increases rapidly with age in all populations. However, the curves for ES in people living in the tropics show a delay of about 10 years. In contrast, the frequency of climatically caused changes (pterygium, climatic keratopathy and pronounced pinguecula) mostly peaks at the age of 50 years and is highest in the tropics and in the Arctic. Males in these regions tended to be more affected by climatically caused changes than females. Likewise, in tropical climates, where radiation from the sun is strong, and in Lapland and Novosibirsk, where there is radiation from snow, males showed more evidence of ES than females. However, examination of 506 patients from a private practice in South Finland, who were under observation for glaucoma or for risk of glaucoma, showed females to be in the majority of those with ES. Conclusions: As a rule, climate does not appear to influence the occurrence of ES. However, in tropical countries, where radiation from the sun is strong, ES was more common among males than among females. In the light of its peculiar population distribution, even when climatic factors are considered, there would appear to be an important genetic factor involved in the manifestation of ES.
Mutations in CACNA1F are known to cause the incomplete form of X-linked congenital stationary night blindness (CSNB2). Since the clinical picture of AIED is quite similar to CSNB2, it has long been discussed whether these disorders are allelic or form a single entity. The present study clearly indicates that AIED is also caused by a novel CACNA1F gene mutation.
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