2008
DOI: 10.1002/ajmg.a.32281
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Growth charts for patients affected with Morquio A disease

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… Show more

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Cited by 131 publications
(169 citation statements)
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“…None of the parents mentioned as an early sign the growth delay, although this is showed in all patients since the 4 years old (18), but as shown on the record, this was the parent or patient perspective, not the medical course, and all patients showed short stature at mature age.…”
Section: Definitive Diagnosismentioning
confidence: 86%
“…None of the parents mentioned as an early sign the growth delay, although this is showed in all patients since the 4 years old (18), but as shown on the record, this was the parent or patient perspective, not the medical course, and all patients showed short stature at mature age.…”
Section: Definitive Diagnosismentioning
confidence: 86%
“…Patients still in childhood were classified according to the Morquio A growth chart for each gender. 4 Two girls, IV_04 and IV_06, were classified as of the attenuated type; the others were classified as of the severe group.…”
Section: Clinical Features and Galns Activitymentioning
confidence: 99%
“…3 The patients of the severe type are also characterized by features such as short stature, genu valgum, odontoid dysplasia, protrusion of the chest, kyphoscoliosis, hypermobility of joints and abnormal gait, 3 and are mostly below the 90th percentile on the Morquio A growth charts. 4 They often have their onsets during infancy, and cannot survive through the second or third decade of life. The patients of the intermediate and mild types, generally called 'attenuated' types, have relatively less skeletal involvement and longer life span.…”
Section: Introductionmentioning
confidence: 99%
“…Patients with a classic (severe) form of MPS IVA have a unique systemic skeletal dysplasia including short-trunk dwarfism, kyphoscoliosis, coxa valga, odontoid hypoplasia, abnormal gait, joint mobility problems, restriction of chest wall movement, and a life span of 20-30 years if an appropriate orthopedic intervention is unavailable. Patients with an attenuated form have a milder skeletal involvement, and most have normal life span (Dũng et al 2013;Yasuda et al 2013;Tomatsu et al 2011Tomatsu et al , 2012aTomatsu et al , 2013aNorthover et al 1996;Montaño et al 2007Montaño et al , 2008Suzuki et al 2001;Hendriksz et al 2013;M€ ollmann et al 2013;Harmatz et al 2013). Patients with MPS IVB show a milder phenotype of skeletal dysplasia than patients with the severe form of MPS IVA.…”
Section: Introductionmentioning
confidence: 99%