Estimation of prognosis and prevalence of @entity6391 and XXXX in Norway.

@entity1 @entity15439 @entity6391
@entity6391 was diagnosed in 101 @entity1 from 53 families. The prognosis for visual function was most favourable for the autosomal dominant group (38 @entity1 from 8 families). The autosomal recessive group (40 @entity1 from 25 families) and the 19 solitary cases were very heterogeneous, with prognosis ranging from favourable to very bad. There was a higher intrafamiliar correlation in the autosomal recessive than in the autosomal dominant group. In 28 @entity1 from 18 families with @entity15439 , almost all had good visual function until 30 years of age, and few had useful visual function after the age of 50. The age when the @entity1 were registered varied between the different genetic types of @entity6391 , reflecting differences in prognosis. Therefore, ascertainment probability and prevalence were calculated for each genetic group separately. The prevalence of @entity6391 in Norway, all genetic groups included, was calculated to be 1/4440, the autosomal dominant type of the disease being the most frequent. The prevalence of @entity15439 was calculated to be 3.6/100,000. Both @entity6391 and @entity15439 were more prevalent in Laps.
