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Bonagura V.R.,Steven and Alexandra Cohen Childrens Medical Center | Bonagura V.R.,Feinstein Institute for Medical Research
Annals of Allergy, Asthma and Immunology | Year: 2013

Objective To illustrate the need for individualized immunoglobulin therapy in patients with primary immunodeficiency diseases (PIDDs) and review current evidence on how best to identify the biological serum IgG level in patients with antibody-deficient PIDD. Data Sources Two case studies from the author's clinical practice are discussed. PubMed and Ovid MEDLINE databases were searched for articles pertaining to serum immunoglobulin levels in patients with PIDD and the relation of trough IgG levels to infection incidence and outcomes. Study Selections Articles and case studies were selected for their relevance to the individualization of IgG therapy for patients with PIDD. The case studies support the position that each patient has a specific "biological" serum IgG level associated with decreasing or preventing recurrent infection. Results Patients with antibody-deficient PIDD are routinely treated with lifelong immunoglobulin replacement. Although a starting dose has been suggested, the dose of IgG that maintains serum IgG levels that protect against severe or recurrent infection has not been determined. It is likely the serum IgG level required to prevent infection in these patients varies as it does in normal individuals. This biological serum IgG level must be identified for each patient by plotting documented infections vs serum IgG levels over time. Conclusion Clinical experience and recent evidence suggest that optimal treatment of patients with PIDD involves individualizing IgG treatment to identify the optimal IgG serum levels that are required for each patient to become free of recurrent infection or pneumonia instead of trying to achieve a single optimal serum IgG level for all patients. © 2013 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved. Source


Stewart D.L.,University of Louisville | Ryan K.J.,Health Outcomes and Pharmacoeconomics | Seare J.G.,Optum Inc | Pinsky B.,Optum Inc | And 2 more authors.
BMC Infectious Diseases | Year: 2013

Background: Palivizumab has been shown to decrease the incidence of hospitalization due to respiratory syncytial virus (RSV) in infants at risk of severe RSV disease. We examined the association between compliance with palivizumab dosing throughout the RSV season and risk of RSV-related hospitalization in clinical practice.Methods: Subjects who were born and discharged from the hospital before the RSV season and received ≥1 palivizumab dose during their first RSV season were identified from a large US commercial health insurance database between 01/01/03 and 12/31/09. Subjects were deemed compliant if they received ≥5 palivizumab doses without gaps (>35 days) and their first dose was received by November 30. RSV-related hospitalizations were identified using ICD-9-CM diagnosis codes and examined over 2 observation periods: post-index dose and RSV season. A Cox proportional hazard model was used to evaluate the association between non-compliance and RSV-related hospitalization.Results: Of the 5,003 subjects who received palivizumab, 62% were deemed non-compliant. Non-compliant subjects had significantly higher unadjusted rates of RSV-related hospitalizations compared to compliant subjects during both observation periods (post-index: 6.1 vs. 2.8 per 100 infant seasons, p < 0.001; RSV season: 5.9% vs. 2.3%; p < 0.001). In multivariate analyses, non-compliance was significantly associated with higher risk of RSV-related hospitalization (HR = 2.01; p < 0.001). Of the 225 RSV-related hospitalizations observed during the RSV season, 61 (27%) occurred before the first dose of palivizumab.Conclusions: Subjects who did not receive monthly dosing of palivizumab throughout the RSV season had significantly higher rates of RSV-related hospitalizations. The RSV-related hospitalizations prior to the first dose of palivizumab suggest some dosing was started too late. © 2013 Stewart et al.; licensee BioMed Central Ltd. Source


Pilapil M.,Steven and Alexandra Cohen Childrens Medical Center | Pilapil M.,The New School | Delaet D.,Mount Sinai School of Medicine
Current Opinion in Pediatrics | Year: 2015

PURPOSE OF REVIEW: Adolescents and young adults with special health care needs (SHCN) are uniquely vulnerable to health risk behaviors including smoking, alcohol and illicit drug use, and sexual risk-taking. Their likelihood of experiencing adverse health outcomes because of these behaviors may be beyond that experienced by their healthier peer group. Pediatric providers are responsible for appropriately counseling these patients about healthy lifestyles. This review provides some background regarding these health risks among adolescents and young adults with SHCN with particular focus on three populations: childhood cancer survivors, congenital heart disease patients, and those with intellectual disability. RECENT FINDINGS: Young adults and adolescents with chronic medical conditions are as likely - and perhaps more likely - to engage in health risk behaviors. However, these behaviors are not fully addressed by primary care providers. SUMMARY: Pediatric providers are encouraged to ask adolescents and young adults with SHCN about their understanding of, and engagement in, health risk behaviors. A multidisciplinary approach to encourage a healthy lifestyle within this population may have significant health benefits. © 2015 Wolters Kluwer Health, Inc. Source


Gump W.C.,Norton Neuroscience Institute | Skjei K.L.,University of Louisville | Karkare S.N.,Steven and Alexandra Cohen Childrens Medical Center
Neurosurgical Focus | Year: 2013

Reports on seizure outcomes following surgery for lesional epilepsy consistently cite extent of resection as a significant predictor of outcome. Unfortunately, gross-total resection is not technically feasible in all cases of medically refractory tumor-associated epilepsy. Here, the authors present the case of a 4-year-old girl whose epilepsy was medically controlled after 1-stage electrocorticography-guided subtotal resection (STR) of a large diffuse protoplasmic astrocytoma. They also review the modern literature on epilepsy associated with brain tumors. Outcomes are compared with those following surgical treatment of focal cortical dysplasia and vascular lesions. Gross-total lesional resection shows significant superiority across pathologies and anatomical regions. Despite a considerable number of STRs yielding seizure freedom, other favorable treatment factors have not been defined. Although gross-total lesional resection, if possible, is clearly superior, tailored surgery may still offer patients a significant opportunity for a good outcome. Treatment factors yielding successful seizure control following STR remain to be fully elucidated. © AANS, 2013. Source


Roy S.,University of Houston | Smith L.P.,Steven and Alexandra Cohen Childrens Medical Center
American Journal of Otolaryngology - Head and Neck Medicine and Surgery | Year: 2015

Objectives This study was designed to assess the ability of carbon dioxide (CO2) lasers and radiofrequency ablation devices (Coblator) (ArthoCare Corporation, Sunnyvale, CA) to ignite either a non-reinforced (polyvinylchloride) endotracheal tube (ETT) or an aluminum and fluoroplastic wrapped silicon (laser safe) ETT at varying titrations of oxygen in a mechanical model of airway surgery. Methods Non-reinforced and laser safe ETTs were suspended in a mechanical model imitating endoscopic airway surgery. A CO2 laser set at 5-30 watts was fired at the ETT at oxygen concentrations ranging from 21% to 88%. The process was repeated using a radiofrequency ablation (RFA) device. All trials were repeated to ensure accuracy. Results The CO2 laser ignited a fire when contacting a non-reinforced ETT in under 2 seconds at oxygen concentrations as low as 44%. The CO2 laser could not ignite a laser safe ETT under any conditions, unless it struck the non-reinforced distal tip of the ETT. With the RFA, a fire could not be ignited with either reinforced or non-reinforced ETTs. Conclusions RFA presents no risk of ignition in simulated airway surgery. CO2 lasers should be utilized with a reinforced ETT or no ETT, as fires can easily ignite when lasers strike a non-reinforced ETT. Decreasing the fraction of inspired oxygen reduces the risk of fire. © 2015 Elsevier Inc. All rights reserved. Source

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