Relationships among Height, Weight, Body Mass Index, and Age in Taiwanese Children with Different Types of Mucopolysaccharidoses
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 reg...
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doaj-abf895fd3cf74b00b0ad5e1d5af9bba52020-11-25T02:16:16ZengMDPI AGDiagnostics2075-44182019-10-019414810.3390/diagnostics9040148diagnostics9040148Relationships among Height, Weight, Body Mass Index, and Age in Taiwanese Children with Different Types of MucopolysaccharidosesHsiang-Yu Lin0Chung-Lin Lee1Pao Chin Chiu2Dau-Ming Niu3Fuu-Jen Tsai4Wuh-Liang Hwu5Shio Jean Lin6Ju-Li Lin7Tung-Ming Chang8Chih-Kuang Chuang9Shuan-Pei Lin10Department of Medicine, MacKay Medical College, New Taipei City 252, TaiwanDepartment of Pediatrics, MacKay Memorial Hospital, Hsinchu 300, TaiwanDepartment of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung 800, TaiwanInstitute of Clinical Medicine, National Yang-Ming University, Taipei 100, TaiwanDepartment of Pediatrics, China Medical University Hospital, Taichung 400, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, Taipei 100, TaiwanDepartment of Pediatrics, Chi Mei Medical Center, Tainan 700, TaiwanDepartment of Pediatrics, Chang Gung Memorial Hospital at Linkou, Taoyuan 33305, TaiwanDepartment of Pediatric Neurology, Changhua Christian Children’s Hospital, Changhua 500, TaiwanDepartment of Medical Research, MacKay Memorial Hospital, Taipei 100, TaiwanDepartment of Medicine, MacKay Medical College, New Taipei City 252, TaiwanBackground: 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 <i>z</i> 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 (<i>n</i> = 9), −2.31, 0.19, and 0.84 for MPS II (<i>n</i> = 49), −0.42, 0.08, and −0.12 for MPS III (<i>n</i> = 27), −6.02, −2.04, and 0.12 for MPS IVA (<i>n</i> = 30), and −4.46, −1.52, and 0.19 for MPS VI (<i>n</i> = 14), respectively. MPS IVA had the lowest mean <i>z</i> 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 <i>z</i> scores for height and weight were negatively correlated with increasing age for all types of MPS (<i>p</i> < 0.01). Of 32 patients younger than 5 years of age, 16 (50%), and 23 (72%) had positive <i>z</i> scores of height and weight, respectively. A substantial number of younger patients with MPS I, II, III, and IVA had a positive height <i>z</i> score. The median age at diagnosis was 3.9 years (<i>n</i> = 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.https://www.mdpi.com/2075-4418/9/4/148body mass indexgrowthheightmucopolysaccharidosisweight |
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DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Hsiang-Yu Lin Chung-Lin Lee Pao Chin Chiu Dau-Ming Niu Fuu-Jen Tsai Wuh-Liang Hwu Shio Jean Lin Ju-Li Lin Tung-Ming Chang Chih-Kuang Chuang Shuan-Pei Lin |
spellingShingle |
Hsiang-Yu Lin Chung-Lin Lee Pao Chin Chiu Dau-Ming Niu Fuu-Jen Tsai Wuh-Liang Hwu Shio Jean Lin Ju-Li Lin Tung-Ming Chang Chih-Kuang Chuang Shuan-Pei Lin Relationships among Height, Weight, Body Mass Index, and Age in Taiwanese Children with Different Types of Mucopolysaccharidoses Diagnostics body mass index growth height mucopolysaccharidosis weight |
author_facet |
Hsiang-Yu Lin Chung-Lin Lee Pao Chin Chiu Dau-Ming Niu Fuu-Jen Tsai Wuh-Liang Hwu Shio Jean Lin Ju-Li Lin Tung-Ming Chang Chih-Kuang Chuang Shuan-Pei Lin |
author_sort |
Hsiang-Yu Lin |
title |
Relationships among Height, Weight, Body Mass Index, and Age in Taiwanese Children with Different Types of Mucopolysaccharidoses |
title_short |
Relationships among Height, Weight, Body Mass Index, and Age in Taiwanese Children with Different Types of Mucopolysaccharidoses |
title_full |
Relationships among Height, Weight, Body Mass Index, and Age in Taiwanese Children with Different Types of Mucopolysaccharidoses |
title_fullStr |
Relationships among Height, Weight, Body Mass Index, and Age in Taiwanese Children with Different Types of Mucopolysaccharidoses |
title_full_unstemmed |
Relationships among Height, Weight, Body Mass Index, and Age in Taiwanese Children with Different Types of Mucopolysaccharidoses |
title_sort |
relationships among height, weight, body mass index, and age in taiwanese children with different types of mucopolysaccharidoses |
publisher |
MDPI AG |
series |
Diagnostics |
issn |
2075-4418 |
publishDate |
2019-10-01 |
description |
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 <i>z</i> 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 (<i>n</i> = 9), −2.31, 0.19, and 0.84 for MPS II (<i>n</i> = 49), −0.42, 0.08, and −0.12 for MPS III (<i>n</i> = 27), −6.02, −2.04, and 0.12 for MPS IVA (<i>n</i> = 30), and −4.46, −1.52, and 0.19 for MPS VI (<i>n</i> = 14), respectively. MPS IVA had the lowest mean <i>z</i> 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 <i>z</i> scores for height and weight were negatively correlated with increasing age for all types of MPS (<i>p</i> < 0.01). Of 32 patients younger than 5 years of age, 16 (50%), and 23 (72%) had positive <i>z</i> scores of height and weight, respectively. A substantial number of younger patients with MPS I, II, III, and IVA had a positive height <i>z</i> score. The median age at diagnosis was 3.9 years (<i>n</i> = 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. |
topic |
body mass index growth height mucopolysaccharidosis weight |
url |
https://www.mdpi.com/2075-4418/9/4/148 |
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