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2022
It is built from checkable properties of the record itself: whether the article has a DOI, how many independent sources confirmed its data, whether the required fields are filled in, and how recent the latest confirmation is. Disagreement between sources and a missing identifier lower it. A value of 80 or above means the record was published without further checking; 60 to 79 means it is published at reduced confidence, with a manual check advisable. A record verified by an editor by hand is never pushed below 90 by a recalculation. This scores our data about the article, not the article itself.
The prevalence of the disease in different countries of the world varies widelyfrom 1 in 117,000 to 1 in 476,000 population. Annual incidence is reported to be 1 in 80,000 livebirths but this figure may underestimate disease prevalence. When late-onset variants of thedisease are considered, a prevalence of approximately 1 in 3,000 has been suggested.