Pediatric tertiary care institutions are well positioned to provide multidisciplinary, intensive interventions for pediatric obesity known as stage 3 treatment. One contributor to the difficulty in administering this treatment is the high rate of patient attrition. Little is known about the practices used by pediatric weight-management clinics and groupbased programs to minimize attrition. Hospital members and nonmembers of FOCUS on a Fitter Future were surveyed on the methods used to engage and retain obese children in their clinics and programs. Shortly thereafter, a benchmarking activity that centered on rates of patient nonattendance at initial and follow-up clinic visits was initiated among FOCUS-group-participating hospitals. Clinic-and group-based program results were contrasted. Staff from groupbased programs reported that the majority of patients did not complete even 50% of program follow-up visits. Multiple patient/family-and clinic/program-level barriers to retention were identified. Attention to successful techniques should be paid during planning for new programs and improvement of established ones. Pediatrics 2011;128:S59-S64

Developmental abnormalities of the brain such as cobblestone lissencephaly, cerebellar cysts, pontine hypoplasia, and brainstem bowing are hallmarks of DG and should prompt consideration of these diagnoses. Brain imaging in individuals with DG helps to predict outcomes, especially language development, aiding clinicians in prognostic counseling.

Limb girdle muscular dystrophy 2I is a slowly progressive muscular dystrophy due to mutations in the Fukutin-related protein (FKRP) gene. Clinicians are frequently asked if physical activity is harmful for pediatric patients with limb girdle muscular dystrophy 2I. The primary objective of this study was to determine if there is a relationship between self-reported childhood activity level and motor function and respiratory function in older children and adults with limb girdle muscular dystrophy 2I. We compared retrospective self-reported middle school activity level and sport participation with age at onset of weakness, 10-meter walk test and forced vital capacity later in life in 41 participants with FKRP mutations. We found no relationship between activity level in childhood and disease course later in life, suggesting that self-directed physical activity in children with limb girdle muscular dystrophy 2I does not negatively affect disease progression and outcome.

Introduction: Reported frequencies of cardiomyopathy in limb girdle muscular dystrophy R9 (LGMDR9) vary. We describe the frequency and age at onset of cardiomyopathy in an LDMDR9 cohort. Methods: Echocardiograms from 56 subjects (157 echocardiograms) with LGMDR9 were retrospectively reviewed. The cumulative probability of having an abnormal echocardiogram as a function of age was assessed by survival analysis for interval-censored data by genotype. Correlations between cardiac and clinical function were evaluated. Results: Twenty-five (45%) participants had cardiomyopathy. The median age at first abnormal echocardiogram for subjects homozygous for the c.826C>A variant was 54.2 y compared to 18.1 y for all other fukutin-related protein (FKRP) genotypes (P < .0001). There was a weak correlation between ejection fraction and 10-Meter Walk Test speed (r = 0.25), but no correlation with forced vital capacity (r = 0.08). Discussion: Cardiomyopathy is prevalent among those with LGMDR9 and occurs later in subjects homozygous for the c.826C>A mutation. These data will help to guide surveillance and management.

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