Document Detail


Epidemiology of retinitis pigmentosa in Denmark.
MedLine Citation:
PMID:  11921605     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A nation-wide registration of Danish cases of retinitis pigmentosa (RP) provided 1890 persons diagnosed during the period 1850-1989. Prevalent at 1 January 1988 were 1301 persons (1:3943) comprising a multitude of different RP-types. Age specific prevalence rates demonstrated increasing rates of RP during the first four decades of life and a rather stable prevalence over the next 20-30 years. Corrected for incompleteness, a late decrease was found, reflecting an incomplete ascertainment of the oldest patients. A moving average method indicated an even later steady state value for the age-specific prevalence. The Danish prevalence figures were standardized according to the WHO World Standardized Prevalence Rates and compared with large studies from the USA and UK. No statistically significant difference was found. Usher syndrome was present in 12% of all RP-cases and Bardet-Biedl syndrome comprised 5%. Mental retardation was found in 144 cases (11%), mostly characterized by atypical RP. Nineteen per cent of patients affected by nonsystemic RP had an onset later than 30 years of age, whereas only a few per cent of persons with systemic RP had an RP onset after age 30 years. The Mendelian inheritance type of all cases was evaluated according to an unambiguous genetic classification, finding a larger amount of X-linked RP compared with other studies. Among nonsystemic RP-cases, 14.3% were found to be inherited as an X-linked trait whereas only 8.4% were autosomal dominantly inherited. The largest fraction was, as in previous materials, the simplex group (isolated cases) comprising 42.9% of the nonsystemic RP patients. Some factors influencing the results are discussed, with special emphasis on the problems associated with precise definitions of the Mendelian inheritance groups. A diagram according to the author's definition was constructed as a guideline ready for clinical application.
Authors:
Marianne Haim
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Acta ophthalmologica Scandinavica. Supplement     Volume:  -     ISSN:  1395-3931     ISO Abbreviation:  Acta Ophthalmol Scand Suppl     Publication Date:  2002  
Date Detail:
Created Date:  2002-03-29     Completed Date:  2002-09-04     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9516974     Medline TA:  Acta Ophthalmol Scand Suppl     Country:  Denmark    
Other Details:
Languages:  eng     Pagination:  1-34     Citation Subset:  IM    
Affiliation:
National Eye Clinic for the Visually Impaired, Rymarksvej I, DK-2900 Hellerup, Denmark.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Age Distribution
Age of Onset
Aged
Aged, 80 and over
Child
Child, Preschool
Denmark / epidemiology
Female
Humans
Incidence
Infant
Infant, Newborn
Male
Middle Aged
Pedigree
Prevalence
Registries
Retinitis Pigmentosa / classification,  epidemiology*,  genetics,  pathology
Sex Distribution

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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