4.2 Article

Incidence of the Mucopolysaccharidoses in Taiwan, 1984-2004

期刊

AMERICAN JOURNAL OF MEDICAL GENETICS PART A
卷 149A, 期 5, 页码 960-964

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WILEY
DOI: 10.1002/ajmg.a.32781

关键词

incidence; mucopolysaccharidoses; mucopolysaccharidosis II; Taiwan

资金

  1. Mackay Memorial Hospital, Taipei, Taiwan [MMH-E-97004]

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Previous Studies on the incidence of the various types of mucopolysaccharidoses (MPS) in different populations have shown considerable variation. However, information regarding the incidence of NIPS in the Asian population is lacking. An epidemiological study of the NIPS disorders in Taiwan using multiple ascertainment sources was undertaken, and incidences of different types of MPS during the period of 1984-2004 were estimated. We compared our data with previous reports in different populations. The combined birth incidence for all MPS cases was 2.04 per 100,000 live births. MPS II (Hunter syndrome) had the highest calculated birth incidence of 1.07 per 100,000 live births (2.05 per 100,000 male live births), comprising 52% of all NIPS cases diagnosed. The birth incidences of NIPS I (Hurler syndrome), III (Sanfilippo syndrome), IV (Morquio syndrome), and VI (Maroteaux-Lamy syndrome) were 0.11, 0.39, 0.33, and 0.14 per 100,000 live births, respectively, which accounted for 6%, 19%, 16%, and 7% of all NIPS, respectively. No cases of NIPS III D (Sanfilippo syndrome type D), NIPS IV B (Morquio syndrome type 13), NIPS VII (Sly syndrome) or MPS IX were ascertained during the study period. Overall incidence of NIPS in Taiwan was consistent with that reported in Western populations. However, in contrast to the higher incidence of MPS I in most Western populations, this study showed a higher incidence of MPS II in Taiwan. It remains to be investigated whether this discrepancy is attributed to the under-diagnosis of MPS I in Taiwan or to ethnic differences. (c) 2009 Wiley-Liss, Inc.

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