ORPHAcodes use for the coding of rare diseases: comparison of the accuracy and cross country comparability

Table 3 Distribution of ORPHAcodes collected during the study period per country/region and per disease prevalence class^a

Disease prevalence class	Czech Republic		Malta		Romania		Spain		Veneto region-IT		All countries/regions
Disease prevalence class	N	%	N	%	N	%	N	%	N	%	N	%
1–9/10,000	29	16.1	77	22.6	23	27.1	135	10.1	66	10.0	160	9.7
1–9/100,000	69	38.3	117	34.3	31	36.5	299	22.3	203	30.9	357	21.8
1–9/1,000,000	39	21.7	51	15.0	15	17.6	212	15.8	143	21.8	244	14.9
< 1/1,000,000	43	23.9	96	28.1	16	18.8	695	51.8	245	37.3	879	53.6
Total	180	100	341	100	85	100	1341	100	657	100	1640	100

^aThe value of the disease prevalence class has been attributed to ORPHAcodes for which a reported prevalence was available in the Orphanet nomenclature (version July 2022) and according to the method described in [2]

ISSN: 1750-1172