Relationships among Height, Weight, Body Mass Index, and Age in Taiwanese Children with Different Types of Mucopolysaccharidoses
by
Hsiang-Yu Lin 1,2,3,4,5,6
, Chung-Lin Lee 7,8, Pao Chin Chiu 9, Dau-Ming Niu 8,10, Fuu-Jen Tsai 11, Wuh-Liang Hwu 12, Shio Jean Lin 13, Ju-Li Lin 14, Tung-Ming Chang 15,16, Chih-Kuang Chuang 3,17,* and Shuan-Pei Lin 1,2,3,18,*
Hsiang-Yu Lin 1,2,3,4,5,6, Chung-Lin Lee 7,8, Pao Chin Chiu 9, Dau-Ming Niu 8,10, Fuu-Jen Tsai 11, Wuh-Liang Hwu 12, Shio Jean Lin 13, Ju-Li Lin 14, Tung-Ming Chang 15,16, Chih-Kuang Chuang 3,17,* and Shuan-Pei Lin 1,2,3,18,*
1
Department of Medicine, MacKay Medical College, New Taipei City 252, Taiwan
2
Department of Pediatrics, MacKay Memorial Hospital, Taipei 100, Taiwan
3
Department of Medical Research, MacKay Memorial Hospital, Taipei 100, Taiwan
4
MacKay Junior College of Medicine, Nursing and Management, Taipei 100, Taiwan
5
Department of Medical Research, China Medical University Hospital, China Medical University, Taichung 400, Taiwan
6
Institute of Biomedical Sciences, MacKay Medical College, New Taipei City 252, Taiwan
7
Department of Pediatrics, MacKay Memorial Hospital, Hsinchu 300, Taiwan
8
Institute of Clinical Medicine, National Yang-Ming University, Taipei 100, Taiwan
9
Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung 800, Taiwan
10
Department of Pediatrics, Taipei Veterans General Hospital, Taipei 100, Taiwan
11
Department of Pediatrics, China Medical University Hospital, Taichung 400, Taiwan
12
Department of Pediatrics, National Taiwan University Hospital, Taipei 100, Taiwan
13
Department of Pediatrics, Chi Mei Medical Center, Tainan 700, Taiwan
14
Department of Pediatrics, Chang Gung Memorial Hospital at Linkou, Taoyuan 33305, Taiwan
15
Department of Pediatric Neurology, Changhua Christian Children’s Hospital, Changhua 500, Taiwan
16
Department of Biological Science and Technology, College of Biological Science and Technology, National Chiao Tung University, Hsinchu 300, Taiwan
17
College of Medicine, Fu-Jen Catholic University, Taipei 100, Taiwan
18
Department of Infant and Child Care, National Taipei University of Nursing and Health Sciences, Taipei 100, Taiwan
*
Authors to whom correspondence should be addressed.
Diagnostics 2019, 9(4), 148; https://doi.org/10.3390/diagnostics9040148
Received: 23 August 2019 / Revised: 4 October 2019 / Accepted: 12 October 2019 / Published: 14 October 2019
(This article belongs to the Special Issue Mucopolysaccharidoses: Diagnosis, Treatment, and Management)
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.
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Keywords:
body mass index; growth; height; mucopolysaccharidosis; weight
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MDPI and ACS Style
Lin, H.-Y.; Lee, C.-L.; Chiu, P.C.; Niu, D.-M.; Tsai, F.-J.; Hwu, W.-L.; Lin, S.J.; Lin, J.-L.; Chang, T.-M.; Chuang, C.-K.; Lin, S.-P. Relationships among Height, Weight, Body Mass Index, and Age in Taiwanese Children with Different Types of Mucopolysaccharidoses. Diagnostics 2019, 9, 148.
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