Metabolic response to exercise in childhood brain tumor survivors: A pilot controlled study.

BACKGROUND/OBJECTIVES: Survival rates in children diagnosed with malignant brain tumors exceed 70%. A higher risk of dyslipidemia, central obesity, and insulin resistance has been reported among these children. We investigated substrate utilization during submaximal exercise. DESIGN/METHODS: Ten brain tumor survivors and 10 healthy children were matched by sex, age, and Tanner stage. Participants completed a submaximal incremental exercise test to determine their fat and carbohydrate oxidation rates. RESULTS: The relative oxygen volume (VO2 ) peak was significantly higher in the control group than in the survivors of childhood brain tumors (43.3 ± 11.9 and 32.4 ± 10.2 mL/kg /min, P = .04). At the same relative exercise intensity, there was no difference in the carbohydrate or lipid oxidation rate between the two groups, or in the maximal fat oxidation (MFO) rate, or in the heart rate or percentage of VO2 peak to reach MFO. Healthy children achieved MFO at significantly higher muscular power than did brain tumor survivors (47.9 ± 20.8 and 21.8 ± 9.6 W, P = .003). CONCLUSION: Because child brain tumor survivors are less physically fit than healthy children, and substrate utilization during submaximal exercise is not different, physical activity should be promoted for child brain tumor survivors.

Low Vitamin D Levels at Birth and Early Respiratory Outcome in Infants With Gestational Age Less Than 29 Weeks.

BACKGROUND: Vitamin D (VitD) is involved in lung development but its influence on respiratory distress syndrome of extremely preterm (EPT) infants have been little investigated. In this study, we examined the influence of low vitamin D status at birth on early respiratory outcomes of this vulnerable infant population. METHODS: Cord blood 25(OH)D levels ≤ 75 nmol/L were considered as Low vitamin D levels. Stepwise logistic regression and classification regression-tree analyses were used and the primary outcome was the combined outcome of death or mechanical ventilation need by the end of the first week (death or MV DoL7) as a marker od RDS severity. RESULTS: The mean (SD) GA and birth weight were 26 (1.4) weeks and 801 (212) gr, respectively; 81/109 (74%) infants had low 25(OH)D levels. Infants with low VitD levels had 25% higher initial FiO2 levels (p < 0.05) and were more likely to be mechanically ventilated on DoL7 (36 vs. 7%, p < 0.05). Adjusted for gestational age, they had 10-fold higher odds of death or MV DoL7 (p < 0.01). By regression tree analysis, the rate of death or MV DoL7 increased from 18 to 71% in infants with GA < 26 weeks and with cord blood 25(OH)D levels higher and lower than 74 nmol/L, respectively (p < 0.05). CONCLUSION: Low vitamin D levels at birth are associated with early adverse respiratory outcomes in infants with GA less 29 weeks. Further largest studies are needed to confirm this association.

Quick and sustained clinical response to MEK inhibitor I in a NF1 patient with neurofibromas.

Neurofibromatosis 1 (NF1) is an autosomal dominant tumour predisposition disorder with a birth incidence of about 1 in 2,700 and prevalence of 1 in 4,560. The NF1 gene codes for an ubiquitous protein: neurofibromin. Neurofibromin interacts with the proto-oncogene RAS to suppress tumour formation. Individuals with germline inactivation of the NF1 gene have a propensity to develop both benign and malignant tumours. We report the case of a 12-year-old child with NF1, diagnosed at the age of 15 months, for whom the clinical course has been marked by the appearance of multiple cutaneous and paraspinal neurofibromas responsible for impaired walking, motor deficiency and pain. A treatment with an MEK inhibitor, trametinib, was initiated and led to a quick and sustained clinical response.