Protsenko M.V., Serpik V.G. 72

Review of the international classification of rare diseases

The article describes the principles of the international classification of rare diseases, proposed by the organization Orphanet. The supranosological nature of rare diseases predetermined the task of developing their new classification, which is more convenient for coding in the framework of their treatment than ICD-11, but coordinated with it. For these purposes, the international organi- zation Orphanet, formed in France in 1997, together with WHO, is developing an additional classification of rare diseases with the assignment of each noso- logical unit its own unique code based on the collection of information and the formation of its own database of their nomenclature. The Orphanet nomencla- ture includes the following elements: a unique Orpha disease code, a common or most common disease name, synonymous disease names, keywords often used to search for a given disease, if any, and the disease definition itself.
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