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Five children with open lung biopsy diagnosed interstitial lung disease were followed (mean of 27.2 years) and evaluated for surfactant protein gene mutations. Four of the children were originally diagnosed as desquamative interstitial pneumonitis and one as chronic interstitial pneumonitis. All had good response to chloroquine or hydroxychloroquine treatment for periods of 7�C38 months. Lung function tests, incremental exercise tests, and rentgenological studies were performed in the children. Surfactant protein gene mutations were searched in all the patients and in part of their families. Three of the patients, aged now 32, 29, and 37 years, feel well and have normal lung function, while two of the patients, both females, aged 28 and 37 years, conduct normal activities of daily Selleckchem INCB024360 living, have healthy children but have clinical, physiological and rentgenological evidence of restrictive lung disease. All five patients were found to have surfactant protein C gene (SFTPC) mutations, three of them with the most common mutation (p.I73T) and the other two RhoC with new mutations of surfactant protein C gene (p.I38F and p.V39L). We conclude that detection of surfactant protein mutations should be attempted in all children presenting with interstitial lung disease. Furthermore, treatment with hydroxychloroquine should be considered in children with SFTPC mutations. Prospective evaluation of hydroxychloroquine therapy in a greater number of www.selleckchem.com/products/i-bet151-gsk1210151a.html patients is needed. Pediatr Pulmonol. 2014; 49:1097�C1105. ? 2013 Wiley Periodicals, Inc. ""Sport participation is especially important for patients with asthma in that it decreases psychosocial and physiological problems associated with inactivity. However, adolescents with asthma seem to participate less in sports compared to their non-asthmatic peers. The current study tested the direct associations between maternal sport-specific factors and sport club participation of early adolescents with asthma and the indirect effect through adolescent's sport-specific cognitions. During home visits, 261 adolescents (aged 10�C15) completed questionnaires about self-efficacy, beliefs regarding sport participation, and their actual sport club participation. Their mothers reported their sport-specific support, beliefs about offspring's and own sport participation, their own levels of physical activity, and their self-efficacy to stimulate offspring to participate in sports. Path analyses were used to examine the direct and indirect associations of maternal sport-specific factors with adolescents sport club participation via adolescent sport-specific cognitions. Analyses showed that maternal sport-specific support (��?=?0.20, P?=?0.007) and self-efficacy to stimulate offspring to participate in sports (��?=?0.20, P?=?0.027) related positively to adolescents' sport club participation. Adolescents' self-efficacy (indirect effect?=?0.09, SE?=?3.01, P?