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Keywords:

  • Morquio A disease;
  • growth;
  • MPS IVA;
  • GALNS

Abstract

Children with Morquio A disease grow poorly and become physically handicapped because of systemic bone disease. The purpose of this study was to describe observed growth patterns and their relationship with the physical condition of patients with Morquio A. In a one-center study, questionnaire-based longitudinal and cross sectional data were used to develop growth curves, to assess physical activity and to determine the incidence of surgical procedures in 354 patients with Morquio A. Mean birth lengths of boys and girls were 52.6 and 52.1 cm, respectively. The mean final heights for males and females at 18 years and older were 122.4 ± 21.5 and 113.1 ± 22.6 cm, respectively. These results corresponded to −7.4 SD for males and −7.7 SD for females compared to the normal healthy controls. Mean birth weights for boys and girls were 3.59 ± 0.58 and 3.5 ± 0.7 kg, respectively. The mean body mass index for males and females at over 18 years of age was 24.7 ± 6.1 and 25.6 ± 5.4 kg/m2, respectively. The growth pattern in Morquio A patients was characterized by impaired growth velocity after 1 year of age. This is the first report providing growth charts for patients with Morquio A, which can help with monitoring the disease and assessing the clinical efficacy of treatments. © 2008 Wiley-Liss, Inc.