Mccalljohnsen2237

RESULTS More daily summer sun-exposure was reported by both parents reporting for the young survivors (U = 5,522.5, P less then .001; U = 31,412, P less then .001) and by older survivors (U = 5,039.5, P less then .001; U = 29,913, P less then .001). Among younger participants greater sun-exposure was associated with being a CCS, while in older participants, greater sun-exposure was associated with being a CCS, a male, smoker/ex-smoker and was also increased in those with more sensitive skin reaction to sunlight. Focusing only on the CCSs, when treatments were considered, none statistically predicted sun-exposure in young CCSs while older CCSs who received radiotherapy were less likely to spend excess time in the sun. CONCLUSION CCSs have sun-exposure at times of day when sun-related skin damage is highest, despite advice to avoid highest risk times. This data can inform sun-protection programs and lifestyle advice aimed at ameliorating the potential increased risk of skin cancer in CCSs. OBJECTIVES Adult obesity may increase the risks of systemic lupus erythematosus (SLE), and there are genetic links between adult height and SLE. Thus, it is plausible that size earlier in life may be important in the aetiology of SLE as well. We investigated whether birthweight, childhood body mass index (BMI; [kg/m2]), height and growth are associated with risks of adult SLE. METHODS The study included 346,627 children from the Copenhagen School Health Records Register, born 1930-1996 with measured weights and heights from 7-13 years. Birthweight information was available from 1936. Linkages were made to the Danish National Patient Register for information on registrations of SLE. During follow-up, 435 individuals (366 women) were registered with SLE. Cox proportional hazards regressions were performed to estimate hazard ratios (HR) and 95% confidence intervals (CI). RESULTS No differences by sex were detected in any of the associations. Birthweight was not associated with SLE risks. Childhood BMI and height were positively and linearly associated with SLE risks. For BMI at age 7, the HR was 1.11 (95% CI 1.01-1.23) per z-score. For height at age 7, the HR was 1.13 (95% CI 1.02-1.24) per z-score. The estimates were similar in magnitude across all childhood ages for BMI and height. There were limited indications that change in BMI or growth in height during childhood influence the risks of SLE in adulthood. CONCLUSIONS Childhood body size is associated with risks of adult SLE, which supports the hypothesis that early life factors are important in SLE aetiology. OBJECTIVE To determine the frequency of remission defined by the absence of the various disease manifestations of psoriatic arthritis (PsA) and identify predictors for remission. METHODS Patients followed at the PsA clinic between 2000 and 2015 were included. Patients are assessed at 6- to 12-month intervals according to a standard protocol. Remission was defined as a visit that patients had no tender or swollen joints, no inflammatory back pain, no tender entheseal sites, minimal skin involvement with BSA less then 1%, patient pain on visual analog scale (VAS) score of less then 15, patient global disease activity VAS score of less then 20, Health Assessment Questionnaire (HAQ) score less then 0.5. We used imputation approach to determine remission status for visits with incomplete criteria for each patient. RESULTS Data from 985 patients (57% males, average age of 47.4 years) were included in this study. From 2000 to 2015, 175 (18%) patients achieved remission at least once and 92 (9%) experienced sustained remission over at least 2 consecutive visits. In a multivariate Weibull regression analysis for the time to remission, higher BMI was associated with lower chance of remission (HR = 0.96, p = 0.012), while the use of biologics increased the chance of achieving remission (HR = 1.48, p = 0.034). The effect of biologics was also significant on the chance of achieving sustained remission for 2 or more consecutive visits (HR = 1.76, p = 0.020). However, biologics were not significantly associated with sustained remission when it was defined based on 3 or more consecutive visits. CONCLUSION Remission occurred at least once in 18% of the patients with PsA while sustained remission occurred in 9% of the study sample. Having higher BMI would reduce the achievement of remission. The use of biologic agents increased not only the chance of remission, but also the chance of sustained remission for at least 12 months. OBJECTIVES To assess associations between neonatal intensive care unit (NICU)-level patent ductus arteriosus (PDA) treatment rates (pharmacologic or surgical) and neonatal outcomes. STUDY DESIGN This cohort study included infants born at 24-28 weeks of gestation and birth weight less then 1500 g in 2007-2015 in NICUs caring for ≥100 eligible infants in 6 countries. The ratio of observed/expected (O/E) PDA treatment rates was derived for each NICU by estimating the expected rate using a logistic regression model adjusted for potential confounders and network. The primary composite outcome was death or severe neurologic injury (grades III-IV intraventricular hemorrhage or periventricular leukomalacia). The associations between the NICU-level O/E PDA treatment ratio and neonatal outcomes were assessed using linear regression analyses including a quadratic effect (a square term) of the O/E PDA treatment ratio. Blasticidin S inhibitor RESULTS From 139 NICUs, 39 096 infants were included. The overall PDA treatment rate was 45% in the cohort (13%-77% by NICU) and the O/E PDA treatment ratio ranged from 0.30 to 2.14. The relationship between the O/E PDA treatment ratio and primary composite outcome was U-shaped, with the nadir at a ratio of 1.13 and a significant quadratic effect (P less then .001). U-shaped relationships were also identified with death, severe neurologic injury, and necrotizing enterocolitis. CONCLUSIONS Both low and high PDA treatment rates were associated with death or severe neurologic injury, whereas a moderate approach was associated with optimal outcomes. OBJECTIVE To evaluate whether teaching mothers about neonatal jaundice will decrease the incidence of acute bilirubin encephalopathy among infants admitted for jaundice. STUDY DESIGN This was a multicenter, before-after and cross-sectional study. Baseline incidences of encephalopathy were obtained at 4 collaborating medical centers between January 2014 and May 2015 (Phase 1). Structured jaundice instruction was then offered (May to November 2015; Phase 2) in antenatal clinics and postpartum. Descriptive statistics and logistic regression models compared 3 groups 843 Phase 1 controls, 338 Phase 2 infants whose mothers received both antenatal and postnatal instruction (group A), and 215 Phase 2 infants whose mothers received no instruction (group B) either because the program was not offered to them or by choice. RESULTS Acute bilirubin encephalopathy occurred in 147 of 843 (17%) Phase 1 and 85 of 659 (13%) Phase 2 admissions, which included 63 of 215 (29%) group B and 5 of 338 (1.5%) group A infants. OR for having acute bilirubin encephalopathy, comparing group A and group B infants adjusted for confounding risk factors, was 0.12 (95% CI 0.03-0.60). Delayed care-seeking (defined as an admission total bilirubin ≥18 mg/dL at age ≥48 hours) was the strongest single predictor of acute bilirubin encephalopathy (OR 11.4; 6.6-19.5). Instruction decreased delay from 49% to 17%. Other major risk factors were home births (OR 2.67; 1.69-4.22) and hemolytic disease (hematocrit ≤35% plus bilirubin ≥20 mg/dL) (OR 3.03; 1.77-5.18). The greater rate of acute bilirubin encephalopathy with home vs hospital birth disappeared if mothers received jaundice instruction. CONCLUSIONS Providing information about jaundice to mothers was associated with a reduction in the incidence of bilirubin encephalopathy per hospital admission. We conducted a secondary analysis of a prospective study of infants ≤60 days of age who were febrile to assess the diagnostic accuracy of automated vs manual immature neutrophils for invasive bacterial infections. Although manual counts were superior compared with automated counts, bands had suboptimal accuracy overall and had significant variability in test characteristics based on methodology. OBJECTIVE To describe the health status of young patients affected by inborn errors of metabolism that require adherence to a restricted diet (IEMRDs) and to describe and compare their self- and proxy (parent)-reported quality of life (QoL) with reference values. STUDY DESIGN A cross-sectional study was conducted in 2015-2017 in patients affected by IEMRDs (except phenylketonuria) younger than 18 years. Data collection was based on medical records, clinical examinations, parents' and children's interviews, and self-reported questionnaires. Measurements included clinical and healthcare data, child and family environment data, and self- and proxy (parent)-reported QoL. RESULTS Of the 633 eligible participants, 578 were recruited (50.3% boys; mean age 8.7 years); their anthropometric status did not differ from the general population. Approximately one-half of them had at least 1 complication of the disease. Their self-reported global QoL did not differ from that of the general population. However, relations with friends and leisure activities QoL domains were negatively impacted, whereas relations with medical staff, relations with parents, and self-esteem QoL domains were positively impacted. Their proxy (parent)-reported QoL was negatively impacted. CONCLUSIONS Young patients affected by IEMRDs present a high rate of clinical complications. Although their proxy (parent)-reported QoL was negatively impacted, their self-reported QoL was variably impacted (both positively and negatively). These results may inform counseling for those who care for affected patients and their families. Tumor profiling has been shown to benefit patients with rare or refractory metastatic cancer, but several limitations hamper its use in daily clinical practice. We aim to assess the added benefit of a comprehensive tumor profiling, including factors predictive of response to targeted and cytotoxic therapy, in the treatment of refractory or rare solid tumors outside of a formal clinical trial. Patients were included between 2013 and 2017. Multiplatform comprehensive tumor profiling (CTP) was performed on FFPE specimens. Tumor response was evaluated by imaging using the RECIST criteria version 1.1. The PFS ratio was defined as PFS under CTP-guided therapy (PFS2)/PFS under previous standard therapy (PFS1). A clinical benefit was identified if the PFS ratio exceeded the 1.3 threshold value. In total, 184 patients were enrolled among whom 104 were evaluable for the PFS ratio. Objective response rates (ORR) were equal to 25% (CI95 16.6-33.4%) and 36.5% (CI95 27.2-45.8%) on the last therapy before CTP and on the CTP-guided therapy respectively (P-value=0.058 on paired proportion comparison test). The proportion of patients achieving a PFS2/PFS1 ratio≥1.3 was equal to 50%. The median PFS1 was statistically lower than PFS2 (120 days compared to 184 days respectively, P-value log rank 0.01). These results confirm the feasibility and the added benefit of a CTP in patients with refractory tumors in daily clinical practice especially in patients not able to enter a clinical trial.