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Confirmation of mapping to ICD-10 Q87.4 for ORPHA:60030 Loeys-Dietz syndrome #260

Open OrphanetSweden opened 4 weeks ago

OrphanetSweden commented 4 weeks ago

Dear all,

We were hoping you could confirm that the mapping to ICD-10 is correct for ORPHA:60030 Loeys-Dietz syndrome. The mapping listed in Orphanet resources is to ICD-10 Q87.4 (Marfan syndrome) NTBT. The Swedish board of Helath and wellfare are using ICD-10 SE "Q87.8W Other specified congenital malformation syndromes, not elsewhere classified" for the disorder, which should correspond to "Q87.8 Other specified congenital malformation syndromes, not elsewhere classified" in the international classification.

The question arose while handeling a coding request from the Information centre for rare diseases.

Best regards, Orphanet Sweden

MCecile-US14 commented 5 days ago

Dear @Seed-Orphanet,

Could you please check this request for ICD-10 alignment and let @OrphanetSweden know the final decision ? (General reminder: the database is currently not accessible for any modifications, please note we cannot guarantee when the changes can be implemented) Thank you, Marie-Cécile