https://scholars.lib.ntu.edu.tw/handle/123456789/525786
標題: | Relationships among height, weight, body mass index, and age in taiwanese children with different types of mucopolysaccharidoses | 作者: | Lin H.-Y. Lee C.-L. Chiu P.C. Niu D.-M. Tsai F.-J. WUH-LIANG HWU Lin S.J. Lin J.-L. Chang T.-M. Chuang C.-K. Lin S.-P. |
關鍵字: | Body mass index; Growth; Height; Mucopolysaccharidosis; Weight | 公開日期: | 2019 | 出版社: | MDPI AG | 卷: | 9 | 期: | 4 | 起(迄)頁: | 48 | 來源出版物: | Diagnostics | 摘要: | Background: Children with mucopolysaccharidosis (MPS) generally appear unaffected at birth but may develop multiple clinical manifestations including profound growth impairment as they grow older. Each type of MPS has a variable age at onset and variable rate of progression, however, information regarding growth in Asian children is limited. Methods: This retrospective analysis included 129 Taiwanese patients with MPS (age range, 0.7 to 19.5 years, median age, 7.9 years) from eight medical centers in Taiwan from January 1996 through December 2018. Results: The mean z scores for the first recorded values of height, weight, and body mass index in the patients' medical records were -4.25, -1.04, and 0.41 for MPS I (n = 9), -2.31, 0.19, and 0.84 for MPS II (n = 49), -0.42, 0.08, and -0.12 for MPS III (n = 27), -6.02, -2.04, and 0.12 for MPS IVA (n = 30), and -4.46, -1.52, and 0.19 for MPS VI (n = 14), respectively. MPS IVA had the lowest mean z scores for both height and weight among all types of MPS, followed by MPS VI, MPS I, MPS II, and MPS III, which showed the mildest growth retardation. Both z scores for height and weight were negatively correlated with increasing age for all types of MPS (p < 0.01). Of 32 patients younger than 5 years of age, 16 (50%), and 23 (72%) had positive z scores of height and weight, respectively. A substantial number of younger patients with MPS I, II, III, and IVA had a positive height z score. The median age at diagnosis was 3.9 years (n = 115). Conclusions: The patients with MPS IVA had the most significant growth retardation among all types of MPS, followed by MPS VI, MPS I, MPS II, and MPS III. The height and weight of the MPS patients younger than 2-5 years of age were higher than those of healthy individuals, however, their growth significantly decelerated in subsequent years. Understanding the growth curve and potential involved in each type of MPS may allow for early diagnosis and timely management of the disease, which may improve the quality of life. ? 2019 by the authors. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-85076808410&doi=10.3390%2fdiagnostics9040148&partnerID=40&md5=fd5d03c56959583e62bf2d4b8878e1b2 https://scholars.lib.ntu.edu.tw/handle/123456789/525786 |
ISSN: | 2075-4418 | DOI: | 10.3390/diagnostics9040148 | SDG/關鍵字: | adolescent; adult; Article; body height; body mass; body weight; child; cohort analysis; enzyme activity; growth curve; growth retardation; height; human; Hunter syndrome; Hurler syndrome; infant; major clinical study; medical record review; Morquio syndrome; mucopolysaccharidosis; onset age; retrospective study; Sanfilippo syndrome; Taiwan; Taiwanese |
顯示於: | 醫學系 |
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