Long-term follow-up and treatment in nine boys with X-linked creatine transporter defect

The creatine transporter (CRTR) defect is a recently discovered cause of X-linked intellectual disability for which treatment options have been explored. Creatine monotherapy has not proved effective, and the effect of treatment with L-arginine is still controversial. Nine boys between 8 months and 10 years old with molecularly confirmed CRTR defect were followed with repeated 1H-MRS and neuropsychological assessments during 4–6 years of combination treatment with creatine monohydrate, L-arginine, and glycine. Treatment did not lead to a significant increase in cerebral creatine content as observed with H1-MRS. After an initial improvement in locomotor and personal-social IQ subscales, no lasting clinical improvement was recorded. Additionally, we noticed an age-related decline in IQ subscales in boys affected with the CRTR defect

Iodine-125 brachytherapy for brain tumours - a review

Iodine-125 brachytherapy has been applied to brain tumours since 1979. Even though the physical and biological characteristics make these implants particularly attractive for minimal invasive treatment, the place for stereotactic brachytherapy is still poorly defined

Risk factors for recurrent emergency department visits for hyperglycemia in patients with diabetes mellitus

Abstract Background Patients with poorly controlled diabetes mellitus may present repeatedly to the emergency department (ED) for management and treatment of hyperglycemic episodes, including diabetic ketoacidosis and hyperosmolar hyperglycemic state. The objective of this study was to identify risk factors that predict unplanned recurrent ED visits for hyperglycemia in patients with diabetes within 30 days of initial presentation. Methods We conducted a 1-year health records review of patients ≥18 years presenting to one of four tertiary care EDs with a discharge diagnosis of hyperglycemia, diabetic ketoacidosis, or hyperosmolar hyperglycemic state. Trained research personnel collected data on patient characteristics and determined if patients had an unplanned recurrent ED visit for hyperglycemia within 30 days of their initial presentation. Multivariate logistic regression models using generalized estimating equations to account for patients with multiple visits determined predictor variables independently associated with recurrent ED visits for hyperglycemia within 30 days. Results There were 833 ED visits for hyperglycemia in the 1-year period. 54.6% were male and mean (SD) age was 48.8 (19.5). Of all visitors, 156 (18.7%) had a recurrent ED visit for hyperglycemia within 30 days. Factors independently associated with recurrent hyperglycemia visits included a previous hyperglycemia visit in the past month (odds ratio [OR] 3.5, 95% confidence interval [CI] 2.1–5.8), age 20 mmol/L (OR 2.2, 95% CI 1.3–3.7), having a family physician (OR 2.2, 95% CI 1.0–4.6), and being on insulin (OR 1.9, 95% CI 1.1–3.1). Having a systolic blood pressure between 90–150 mmHg (OR 0.53, 95% CI 0.30–0.93) and heart rate >110 bpm (OR 0.41, 95% CI 0.23–0.72) were protective factors independently associated with not having a recurrent hyperglycemia visit. Conclusions This unique ED-based study reports five risk factors and two protective factors associated with recurrent ED visits for hyperglycemia within 30 days in patients with diabetes. These risk factors should be considered by clinicians when making management, prognostic, and disposition decisions for diabetic patients who present with hyperglycemia

Decentral solar energy database

Geomatics Synthesis Research project is commissioned by external partners. The research was carried out under the flag of the CERISE Topsector Switch2SmartGrids research program (www.cerise-project.nl).UrbanismArchitecture and The Built EnvironmentGeomatic

The course of health-related quality of life after the diagnosis of childhood cancer: a national cohort study

Abstract Background Comprehensive insight in the longitudinal development of health-related quality of life (HRQOL) after childhood cancer diagnosis could improve quality of care. Thus, we aimed to study the course and biopsychosocial determinants of HRQOL in a unique national cohort of children with cancer. Methods HRQOL of 2154 children with cancer was longitudinally reported (median: 3 reports) between diagnosis and 5 years after, using the pediatric quality of life inventory generic core scales (PedsQL). HRQOL was modelled over time since diagnosis using mixed model analysis for children 2–7 years (caregiver-reports) and ≥ 8 years (self-reports). Differences in the course between hematological, solid and central nervous system malignancies were studied. Additional associations of demographics, disease characteristics (age at diagnosis, relapse, diagnosis after the national centralization of childhood cancer care and treatment components) and caregiver distress (Distress thermometer) were studied. Results Overall, HRQOL improved with time since diagnosis, mostly in the first years. The course of HRQOL differed between diagnostic groups. In children aged 2–7 years, children with a solid tumor had most favorable HRQOL. In children aged ≥ 8 years, those with a hematological malignancy had lower HRQOL around diagnosis, but stronger improvement over time than the other diagnostic groups. In both age-groups, the course of HRQOL of children with a CNS tumor showed little or no improvement. Small to moderate associations (β: 0.18 to 0.67, p < 0.05) with disease characteristics were found. Centralized care related to better HRQOL (β: 0.25 to 0.44, p < 0.05). Caregiver distress was most consistently associated with worse HRQOL (β: − 0.13 to − 0.48, p < 0.01). Conclusions The HRQOL course presented can aid in identifying children who have not fully recovered their HRQOL following cancer diagnosis, enabling early recognition of the issue. Future research should focus on ways to support children, especially those with a CNS tumor, for example by decreasing distress in their caregivers