ContextThe transition of patients with Prader–Willi syndrome (PWS) to adult life for medical care is challenging because of multiple comorbidities, including hormone deficiencies, obesity and cognitive and behavioral disabilities.ObjectiveTo assess endocrine management, and metabolic and anthropometric parameters of PWS adults who received (n = 31) or not (n = 64) transitional care, defined as specialized pediatric care followed by a structured care pathway to a multidisciplinary adult team.Patients and study designHormonal and metabolic parameters were retrospectively recorded in 95 adults with PWS (mean ± s.d. age 24.7 ± 8.2 years, BMI: 39.8 ± 12.1 kg/m²) referred to our Reference Center and compared according to transition.ResultsAmong the entire cohort, 35.8% received growth hormone (GH) during childhood and 16.8% had a GH stimulation test after completion of growth. In adulthood, 14.7% were treated with GH, 56.8% received sex-hormone therapy, whereas 91.1% were hypogonadic and 37.9% had undergone valid screening of the corticotropic axis. The main reason for suboptimal endocrine management was marked behavioral disorders. Patients receiving transitional care were more likely to have had a GH stimulation test and hormonal substitutions in childhood. They also had a lower BMI, percentage of fat mass, improved metabolic parameters and fewer antidepressant treatments. Transitional care remained significantly associated with these parameters in multivariate analysis when adjusted on GH treatment.ConclusionA coordinated care pathway with specialized pediatric care and transition to a multidisciplinary adult team accustomed to managing complex disability including psychiatric troubles are associated with a better health status in adults with PWS.
<b><i>Objective:</i></b> Estimating glucose variability (GV) through within-day coefficient of variation (%CV<sub>w</sub>) is recommended for patients with type-1 Diabetes (T1D). High-GV (hGV) is defined as %CV<sub>w</sub>>36%. However, continuous glucose monitoring (CGM) devices provide exclusively total-CV (%CV<sub>T</sub>). We aimed to assess consequences of this disparity. <p><b><i>Research Design and Methods:</i></b> We retrospectively calculated both %CV<sub>T</sub> and %CV<sub>W </sub>of consecutive T1D patients from their CGM raw data during 14 days. Patients with hGV with %CV<sub>T</sub>>36% and %CV<sub>w</sub>≤36% were called the “inconsistent-GV group”.</p> <p><b><i>Results:</i></b> 104 patients were included. Mean %CV<sub>T</sub> and %CV<sub>w</sub> were 42.4+/-8% and 37.0+/-7.4% respectively (p<0.0001). Using %CV<sub>T</sub>, 81 patients (73.6%) were classified as hGV whereas 59 (53.6%) using %CV<sub>W </sub>(p<0.0001) corresponding to 22 patients (21%) in the “<i>inconsistent-GV</i> population”.</p> <p><b><i>Conclusions:</i></b> Evaluation of GV through %CV in patients with T1D is highly dependent on the calculation method and then must be standardized.</p>
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