This study suggests that adolescents should be targeted for improved metabolic control. Diabetes team members need to be aware of changing family situations and provide extra support during stressful times. Regular clinic attendance is an important component of intensive diabetes management. Strategies must be developed to improve accessibility to the clinic and to identify patients who frequently miss appointments.
There is a spectrum of incidental thyroid abnormalities in children. These US findings should be interpreted cautiously, avoiding unnecessary referrals and investigations. Intrathyroid ectopic thymus is more common than previously thought, occurs much more frequently in younger boys and should be considered if the lesion has US characteristics of thymus.
Older age and CNS involvement at diagnosis increase the risk of MIDi. In contrast to previous studies, higher BMI was not associated with MIDi in our population.
Many children with ALL gain weight during therapy and fail to return to normal weight after treatment concludes. Subsequent diet, physical activity, and sedentary behavior do not appear to differ between overweight and normal weight survivors. Clinicians should focus on ways to minimize weight gain during therapy rather than waiting for treatment to conclude.
Background
Posterior fossa ependymoma (PFE) comprises 2 groups, PF group A (PFA) and PF group B (PFB), with stark differences in outcome. However, to the authors’ knowledge, the long‐term outcomes of PFA ependymoma have not been described fully. The objective of the current study was to identify predictors of survival and neurocognitive outcome in a large consecutive cohort of subgrouped patients with PFE over 30 years.
Methods
Demographic, survival, and neurocognitive data were collected from consecutive patients diagnosed with PFE from 1985 through 2014 at the Hospital for Sick Children in Toronto, Ontario, Canada. Subgroup was assigned using genome‐wide methylation array and/or immunoreactivity to histone H3 K27 trimethylation (H3K27me3).
Results
A total of 72 PFE cases were identified, 89% of which were PFA. There were no disease recurrences noted among patients with PFB. The 10‐year progression‐free survival rate for all patients with PFA was poor at 37.1% (95% confidence interval, 25.9%‐53.1%). Analysis of consecutive 10‐year epochs revealed significant improvements in progression‐free survival and/or overall survival over time. This pertains to the increase in the rate of gross (macroscopic) total resection from 35% to 77% and the use of upfront radiotherapy increasing from 65% to 96% over the observed period and confirmed in a multivariable model. Using a mixed linear model, analysis of longitudinal neuropsychological outcomes restricted to patients with PFA who were treated with focal irradiation demonstrated significant continuous declines in the full‐scale intelligence quotient over time with upfront conformal radiotherapy, even when correcting for hydrocephalus, number of surgeries, and age at diagnosis (‐1.33 ± 0.42 points/year; P = .0042).
Conclusions
Data from a molecularly informed large cohort of patients with PFE clearly indicate improved survival over time, related to more aggressive surgery and upfront radiotherapy. However, to the best of the authors’ knowledge, the current study is the first, in a subgrouped cohort, to demonstrate that this approach results in reduced neurocognitive outcomes over time.
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