Quantifying coding integrity and reliability of ICD-11 MMS for rare disease
registration: a case study of the Chinese rare disease catalogue
#MMPMID41340132
Bai X
; Guo J
; Zhang M
; Wang Y
; Li N
BMC Med Inform Decis Mak
2025[Dec]; 25
(1
): 440
PMID41340132
show ga
INTRODUCTION: Epidemiological data on rare diseases (RDs) affect the accurate
scientific assessment of these diseases and lead to many issues in policy-making,
healthcare systems, and legislation. The coding system is crucial for accurately
identifying and calculating the incidence rates of each RD. This study focuses on
the effectiveness of collecting RD data via the ICD-11 and examines whether the
ICD-11 can fully support RD statistics. The findings of this study should provide
a foundation for replacing the ICD-10 with the ICD-11. METHODS: This study
included 121 RDs from the first "Rare Disease Catalogue"in China. The diseases
were recoded independently by two experts in the ICD-11 MMS. A comparative
analysis was conducted on the distributions of chapters, code types, and index
terms in the ICD-10 and ICD-11 MMS. RESULTS: This study analysed 121 rare
diseases (RDs) from China's first Rare Disease Catalogue. These RDs mapped to 204
ICD-10 codes (1.4% of all codes), including 76 (37.3%) non-index terms, and to
171 ICD-11 MMS codes (0.96% of all codes). The proportion of RD codes was
significantly lower in ICD-11 than in ICD-10 (0.96% vs. 1.4%, P?
|*Clinical Coding/standards
[MESH]
|*International Classification of Diseases/standards
[MESH]
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