Central precocious puberty in spina bifida children: Guidelines for the care of people with spina bifida

Children with spina bifida are at greater risk of developing central precocious puberty (CPP) compared to others. Therefore, early recognition and timely referral for further evaluation by a pediatric endocrinologist allows appropriate management that reduces the impact of CPP. This article discusses the diagnosis and management of CPP in children with spina bifida. This guideline was developed for SB Transition Healthcare Guidelines from the 2018 Spina Bifida Association's Fourth Edition of the Guidelines for the Care of People with Spina Bifida

Mental Health Outcomes during Colorectal Cancer Survivorship: A Review of the Literature

Objective This article reviews literature on adults’ mental health outcomes during acute and long-term colorectal cancer (CRC) survivorship. Methods We identified articles that included at least one measure of psychological symptoms or mental quality of life or well-being through a search of databases (CINAHL, MEDLINE, PsycINFO, and PsycARTICLES). Articles were published between January 2004 and April 2015. Results A significant proportion of CRC survivors experience clinically meaningful levels of anxiety and depressive symptoms or reduced mental well-being across the trajectory of the illness. Demographic, medical, and psychosocial predictors of mental health outcomes were identified. However, few studies were theory-driven, and gaps remain in our understanding of risk and protective factors with respect to mental health outcomes, especially during long-term CRC survivorship. Conclusions Theory-driven longitudinal research with larger samples is required to identify subgroups of CRC survivors with different trajectories of psychological adjustment. Such research would assess adjustment as a function of internal resources (e.g., personality, coping) and external resources (e.g., finances, social support) to inform future interventions for CRC survivors

Predictors of Functional Outcomes following Operative Treatment of Acute Achilles Tendon Ruptures

Introduction: Previous studies involving operative management of Achilles tendon ruptures have attempted to determine if patient factors influence outcomes. No previous study has attempted to identify outcome predictors in patients exclusively undergoing surgical repair. The purpose of this study is to determine if any injury or patient variables were predictive of outcomes following operative management of Achilles ruptures. Methods: Patient demographics including age, sex, body mass index (BMI), comorbidities (diabetes mellitus, depression, anxiety), mechanism of injury (sports, non-sports), and date of injury were collected. Postoperative notes were reviewed to determine compliance. Patients completed the Foot & Ankle Ability Measure (FAAM)-Activities of Daily Living (ADL) and –Sports subscales, and visual analog scale (VAS) for pain. Multivariable regression analysis was performed, and regression coefficients with 95% confidence intervals and p-values were reported. Results: Female sex was associated with lower FAAM-Sports score (-10.11 [-19.73,-0.50]) and a lower Single Assessment Numeric Evaluation score from the FAAM-Sports subscale (-13.79 [-26.28,-1.30]; p=0.0325). History of anxiety was related to a lower FAAM-ADL score (-29.02 [-45.68, -12.36]; p=0.0009), FAAM-Sports score (-33.41 [-64.46, -2.37]; p=0.0368), and a higher VAS pain score (19.83 [4.43, 35.23]; p=0.0128). Age, BMI, a history of depression or diabetes mellitus, mechanism of injury, timing of repair, and patient compliance were not predictive. Discussion: Females and patients with anxiety have significantly poorer outcomes following Achilles tendon repair. Further study is indicated to determine whether these factors are also predictive of outcomes of Achilles ruptures treated non-surgically and how this may affect surgical indications in these patients

Robin sequence: what the multidisciplinary approach can do

Robin sequence (RS) is a commonly encountered triad of micrognathia, glossoptosis, and airway obstruction, with or without a cleft palate. The management of airway obstruction is of paramount importance, and multiple reviews and retrospective series outline the diagnosis and treatment of RS. This article focuses on the multidisciplinary nature of RS and the specialists’ contributions and thought processes regarding the management of the RS child from birth to skeletal maturity. This review demonstrates that the care of these children extends far beyond the acute airway obstruction and that thorough monitoring and appropriate intervention are required to help them achieve optimal outcomes

L-Edge Spectroscopy of Dilute, Radiation-Sensitive Systems Using a Transition-Edge-Sensor Array

We present X-ray absorption spectroscopy and resonant inelastic X-ray scattering (RIXS) measurements on the iron L-edge of 0.5 mM aqueous ferricyanide. These measurements demonstrate the ability of high-throughput transition-edge-sensor (TES) spectrometers to access the rich soft X-ray (100-2000eV) spectroscopy regime for dilute and radiation-sensitive samples. Our low-concentration data are in agreement with high-concentration measurements recorded by conventional grating-based spectrometers. These results show that soft X-ray RIXS spectroscopy acquired by high-throughput TES spectrometers can be used to study the local electronic structure of dilute metal-centered complexes relevant to biology, chemistry and catalysis. In particular, TES spectrometers have a unique ability to characterize frozen solutions of radiation- and temperature-sensitive samples.Comment: 19 pages, 4 figure

Cancer risk in childhood-onset systemic lupus

INTRODUCTION: The aim of this study was to assess cancer incidence in childhood-onset systemic lupus erythematosus (SLE). METHODS: We ascertained cancers within SLE registries at 10 pediatric centers. Subjects were linked to cancer registries for the observational interval, spanning 1974 to 2009. The ratio of observed to expected cancers represents the standardized incidence ratio (SIR) or relative cancer risk in childhood-onset SLE, versus the general population. RESULTS: There were 1020 patients aged <18 at cohort entry. Most (82%) were female and Caucasian; mean age at cohort entry was 12.6 years (standard deviation (SD) = 3.6). Subjects were observed for a total of 7,986 (average 7.8) patient-years. Within this interval, only three invasive cancers were expected. However, 14 invasive cancers occurred with an SIR of 4.7, 95% confidence interval (CI) 2.6 to 7.8. Three hematologic cancers were found (two non-Hodgkin’s lymphoma, one leukemia), for an SIR of 5.2 (95% CI 1.1 to 15.2). The SIRs stratified by age group and sex, were similar across these strata. There was a trend for highest cancer occurrence 10 to 19 years after SLE diagnosis. CONCLUSIONS: These results suggest an increased cancer risk in pediatric onset SLE versus the general population. In absolute terms, this represents relatively few events. Of note, risk may be highest only after patients have transferred to adult care

A point-of-care clinical trial comparing insulin administered using a sliding scale versus a weight-based regimen

Background Clinical trials are widely considered the gold standard in comparative effectiveness research (CER) but the high cost and complexity of traditional trials and concerns about generalizability to broad patient populations and general clinical practice limit their appeal. Unsuccessful implementation of CER results limits the value of even the highest quality trials. Planning for a trial comparing two standard strategies of insulin administration for hospitalized patients led us to develop a new method for a clinical trial designed to be embedded directly into the clinical care setting thereby lowering the cost, increasing the pragmatic nature of the overall trial, strengthening implementation, and creating an integrated environment of research-based care

The effectiveness and experience of self-management following acute coronary syndrome : a review of the literature

Evidence of the effectiveness of self-management interventions among people with acute coronary syndrome remains inconclusive. Findings from the patients' experiences in relation to self-management following acute coronary syndrome provided important insights into what problems patients might have encountered during self-managing recovery and what support they might need, which can be used to inform the development of self-management interventions. Theoretical or conceptual frameworks have been minimally employed in these studies and should be incorporated in future development and evaluation of self-management interventions as a way of ensuring clarity and consistency related to how interventions are conceptualised, operationalised and empirically studied. Further research is needed to evaluate self-management interventions among people following acute coronary syndrome for sustained effect and within different health care contexts

Single hadron response measurement and calorimeter jet energy scale uncertainty with the ATLAS detector at the LHC

The uncertainty on the calorimeter energy response to jets of particles is derived for the ATLAS experiment at the Large Hadron Collider (LHC). First, the calorimeter response to single isolated charged hadrons is measured and compared to the Monte Carlo simulation using proton-proton collisions at centre-of-mass energies of sqrt(s) = 900 GeV and 7 TeV collected during 2009 and 2010. Then, using the decay of K_s and Lambda particles, the calorimeter response to specific types of particles (positively and negatively charged pions, protons, and anti-protons) is measured and compared to the Monte Carlo predictions. Finally, the jet energy scale uncertainty is determined by propagating the response uncertainty for single charged and neutral particles to jets. The response uncertainty is 2-5% for central isolated hadrons and 1-3% for the final calorimeter jet energy scale.Comment: 24 pages plus author list (36 pages total), 23 figures, 1 table, submitted to European Physical Journal

Amplification of thymidylate synthetase in metastatic colorectal cancer patients pretreated with 5-fluorouracil-based chemotherapy

Resistance to 5-fluorouracil (5-FU) represents a major contributor to cancer-related mortality in advanced colorectal cancer patients. Genetic variations and expression alterations in genes involved in 5-FU metabolism and effect have been shown to modulate 5-FU sensitivity in vitro, however these alterations do not fully explain clinical resistance to 5-FU-based chemotherapy. To determine if alterations of DNA copy number in genes involved in 5-FU metabolism impacted clinical resistance to 5-FU-based chemotherapy, we assessed thymidylate synthetase (TYMS) and thymidine phosphorylase (TYMP) copy number in colorectal liver metastases. DNA copy number of TYMS and TYMP was evaluated using real time quantitative PCR in frozen colorectal liver metastases procured from 62 patients who were pretreated with 5-FU-based chemotherapy prior to surgical resection (5-FU exposed) and from 51 patients who received no pretreatment (unexposed). Gain of TYMS DNA copy number was observed in 18% of the 5-FU exposed metastases, while only 4% of the unexposed metastases exhibited TYMS copy gain (p=0.036). No significant differences were noted in TYMP copy number alterations between 5-FU exposed and unexposed metastases. Median survival time was similar in 5-FU exposed patients with metastases containing TYMS amplification and those with no amplification. However, TYMS amplification was associated with shorter median survival in patients receiving post-resection chemotherapy (hazard ratio = 2.7, 95% confidence interval = 1.1 to 6.6; p=0.027). These results suggest amplification of TYMS amplification as a putative mechanism for clinical resistance to 5-FU-based chemotherapy and may have important ramifications for the post-resection chemotherapy choices for metastatic colorectal cancer