AIM: Our goal was to evaluate the level, degree and direction of deviation in the ontogenesis of patients with mucopolysaccharidosis type II (MPS II) in comparison with the healthy population. MATERIAL AND METHODS: The anthropometric data of a longitudinal study on 28 patients with MPS II, aged from 0.5 to 21 years, were used to analyse the general growth patterns in terms of height, weight and head circumference. The growth trend was assessed with the straight-line regression model. The mathematical structural growth model was used to evaluate the structure of body height growth. RESULTS: A statistically significant negative growth trend for all features was found. Analysis of development structure revealed an earlier onset of the adolescent growth spurt among healthy boys and a lower current velocity of growth than expected values. CONCLUSION: During the first 3 years of life, all observed anthropometric features grew faster than normal. They slowed down by the end of the third year and, in subsequent years, reached lower values when compared with the reference charts. The values obtained from the BTT model showed the structure of body height growth, with particular emphasis on the pubertal spurt, was significantly different from the reference charts.
AIM: Our goal was to evaluate the level, degree and direction of deviation in the ontogenesis of patients with mucopolysaccharidosis type II (MPS II) in comparison with the healthy population. MATERIAL AND METHODS: The anthropometric data of a longitudinal study on 28 patients with MPS II, aged from 0.5 to 21 years, were used to analyse the general growth patterns in terms of height, weight and head circumference. The growth trend was assessed with the straight-line regression model. The mathematical structural growth model was used to evaluate the structure of body height growth. RESULTS: A statistically significant negative growth trend for all features was found. Analysis of development structure revealed an earlier onset of the adolescent growth spurt among healthy boys and a lower current velocity of growth than expected values. CONCLUSION: During the first 3 years of life, all observed anthropometric features grew faster than normal. They slowed down by the end of the third year and, in subsequent years, reached lower values when compared with the reference charts. The values obtained from the BTT model showed the structure of body height growth, with particular emphasis on the pubertal spurt, was significantly different from the reference charts.
Authors: J de Ruijter; L Broere; M F Mulder; A T van der Ploeg; M E Rubio-Gozalbo; S B Wortmann; G Visser; F A Wijburg Journal: J Inherit Metab Dis Date: 2013-10-31 Impact factor: 4.982
Authors: Sung Yoon Cho; Rimm Huh; Mi Sun Chang; Jieun Lee; Younghee Kwun; Se Hyun Maeng; Su Jin Kim; Young Bae Sohn; Sung Won Park; Eun-Kyung Kwon; Sun Ju Han; Jooyoun Jung; Dong-Kyu Jin Journal: J Korean Med Sci Date: 2014-01-28 Impact factor: 2.153