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Background: Amyotrophic lateral sclerosis (ALS) is a progressive disease characterized by chronic degeneration of upper and lower motor neurons and finally death within 3–5 years usually because of respiratory failure. Riluzole and edaravone are presently available treatments. It may be better to try combination therapy rather than taking individual medications. Objectives: To compare the effectiveness of (edaravone + riluzole) combination therapy versus riluzole therapy alone in slowing down the progression of ALS and to evaluate the role of serum creatinine as a marker of disease progression. Materials and Methods: Observational, randomized, parallel assignment, open label study. Thirty patients with definite and probable ALS were randomly assigned to two treatment groups. The case group received (riluzole + edaravone) for the initial 6 months, followed by riluzole for the next 6 months. The control group received riluzole for 12 months. After 6 and 12 months, changes in ALS functional rating scale (ALSFRS-R), mRS, and Japanese ALS scores were determined. P value <.05 was considered significant. Results: An increase in mRS at 6 months in the case group versus control group was 0.07 versus 0.20, respectively (p =0.02). At 12 months, it was 0.47 versus 0.53, respectively (p =0.17). A decrease in serum creatinine at 6 months in case group versus control group was 0.08 versus 0.09, respectively (p =.82). There was no change in ALS FRS for bulbar symptoms (salivation), 3.46 versus 3.46 in the case group (p =.018) for the first 6 months. Conclusions: Combined with riluzole, edaravone slows disease progression and is safe, but the effect is short-term. Bulbar symptoms respond better to combination therapy. The serum creatinine is helpful in monitoring disease progression. [ABSTRACT FROM AUTHOR]

Abstract: Importance: The incidence of obesity is rising in the United States and has been linked to Obstructive Sleep Apnea (OSA) even in young children. Understanding the role that obesity and OSA play in alterations in metabolic variables that can lead to serious health issues is essential to the care and counseling of affected children. Objectives: To evaluate the association of alterations in metabolic variables, including insulin resistance, to OSA in young, obese children. Design: Retrospective, case-control series. Setting: Tertiary care children's hospital. Participants: Obese children aged 2-12 years who had undergone overnight polysomography and routine laboratory testing for lipid levels, fasting glucose, and insulin from January 1, 2006 to December 31, 2012 were identified from a TransMed Bio-Integration Suite and Epic's clarity database search. Results: A total of 76 patients were included for analysis. Forty-three (56.6%) were male, and the mean age was 8.3±2.5 years (range, 2.4–11.9 years). The mean body mass index (BMI) z score was 2.8±0.75 (range, 1.7–6.3), and all patients were obese (BMI z score>95th percentile). Twenty two patients (28.9%) had an apnea–hypopnea index (AHI) <1/h (no OSA), 27 (35.5%) an AHI≥1<5/h, 12 (15.8%) had an AHI ≥5<9.99/h, and 15 (19.7%) had an AHI≥10/h. There was no significant difference in total cholesterol, triglycerides, high and low density lipoprotein levels, systolic and diastolic blood pressure in those patients with or without OSA. Fasting insulin, blood glucose, and homeostasis model assessment (HOMA) were significantly higher in patients with OSA compared to those with no OSA (p <0.01). AHI correlated to alterations in insulin as well as glucose homeostasis on multivariate analysis. Results from logistic regression analysis showed that fasting insulin (p <0.01), and HOMA (p <0.01) predicted severe OSA independent of age, gender, and BMI z score in these patients. Conclusion: Metabolic alterations in glucose and insulin levels, known to be associated with obesity and increased risk for cardiovascular disease, appear to relate to the severity of OSA in young children. [Copyright &y& Elsevier]

Objectives: To determine if children with sleep disordered breathing who have Medicaid insurance encounter more difficulty accessing an otolaryngologist than those with private insurance., Design: Retrospective study., Setting: Urban tertiary care pediatric hospital., Patients: Children referred for evaluation of sleep disordered breathing (SDB)., Intervention: Survey of patients' parents and guardians., Main Outcome Measure: Timely access to an otolaryngologist in their community., Results: Ninety-seven patients were included. Fifty patients had private insurance, 47 had Medicaid. The mean age was 5 years for those with private insurance and 5.6 years for those with Medicaid (p=0.27). The symptoms of SDB in both groups were similar. It took an average of 1.97 weeks for the children in the private insurance group to get an appointment versus 10.8 weeks for those with Medicaid (p=0.002). The mean distance traveled by the children in the private insurance group was 9.86 miles compared to 18.05 miles for those with Medicaid (p=0.001)., Conclusion: Children who were referred for evaluation of SDB were of similar age and had similar symptoms regardless of insurance type. Children with Medicaid wait longer and travel farther to see an otolaryngologist than children with private insurance., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)