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Survival and diagnostic age of 175 Taiwanese patients with mucopolysaccharidoses (1985–2019)

Authors :
Hsiang-Yu Lin
Chung-Lin Lee
Chia-Ying Chang
Pao Chin Chiu
Yin-Hsiu Chien
Dau-Ming Niu
Fuu-Jen Tsai
Wuh-Liang Hwu
Shio Jean Lin
Ju-Li Lin
Mei-Chyn Chao
Tung-Ming Chang
Wen-Hui Tsai
Tzu-Jou Wang
Chih-Kuang Chuang
Shuan-Pei Lin
Source :
Orphanet Journal of Rare Diseases, Vol 15, Iss 1, Pp 1-11 (2020)
Publication Year :
2020
Publisher :
BMC, 2020.

Abstract

Abstract Background Mucopolysaccharidoses (MPSs) are a group of inherited metabolic diseases, which are characterized by the accumulation of glycosaminoglycans, and eventually lead to the progressive damage of various tissues and organs. Methods An epidemiological study of MPS in Taiwan was performed using multiple sources. The survival and diagnostic age for different types of MPS between 1985 and 2019 were evaluated. Results Between 1985 and 2019, there were 175 patients diagnosed with MPS disorders in the Taiwanese population, with a median diagnostic age of 3.9 years. There were 21 (12%), 78 (45%), 33 (19%), 32 (18%) and 11 (6%) patients diagnosed with MPS I, II, III, IV and VI, respectively, with median diagnostic ages of 1.5, 3.8, 4.7, 4.5 and 3.7 years, respectively. Diagnosis of MPS patients was significantly earlier in recent decades (p

Details

Language :
English
ISSN :
17501172
Volume :
15
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Orphanet Journal of Rare Diseases
Publication Type :
Academic Journal
Accession number :
edsdoj.1a7cb82677974c78a14f127068c04131
Document Type :
article
Full Text :
https://doi.org/10.1186/s13023-020-01598-z