Steven and Alexandra Cohen Childrens Medical Center

New Hyde Park, NY, United States

Steven and Alexandra Cohen Childrens Medical Center

New Hyde Park, NY, United States
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Leibowitz J.,Steven and Alexandra Cohen Childrens Medical Center | Soma V.L.,Steven and Alexandra Cohen Childrens Medical Center | Rosen L.,Feinstein Institute for Medical Research | Rubin L.G.,Steven and Alexandra Cohen Childrens Medical Center
Pediatric Infectious Disease Journal | Year: 2015

Background: Many laboratories use polymerase chain reaction (PCR)-based assays to detect the Clostridium difficile toxin B gene (tcdB) in stool. However, PCR testing experience in pediatric patients is limited. We compared the detection of C. difficile by PCR in hospitalized children with and without diarrhea. Methods: Stool samples from patients aged 1-18 years with diarrhea (symptomatic) and from patients without diarrhea (asymptomatic) were tested for C. difficile tcdB using a commercial PCR assay. Samples were cultured for C. difficile using standard techniques with tcdB PCR and cytotoxicity assays performed on C. difficile isolates. Demographic, clinical and laboratory data were abstracted. Categorical and continuous variables were compared between the 2 groups using Fisher Exact test and the Mann-Whitney test, respectively. Results: Thirty-five of 188 (19%) stool samples from symptomatic patients and 18 of 74 (24%) samples from asymptomatic patients were positive by PCR (P = 0.31). Among PCR-positive patients, symptomatic patients had a significantly higher proportion of subjects who received antimicrobials in the preceding 30 days (P = 0.04) and a greater number of preceding antimicrobial days than did asymptomatic patients (P = 0.02) but were comparable with respect to the other variables analyzed. Conclusions: C. difficile PCR assays are frequently positive in hospitalized children both with and without diarrhea. As we observed a high level of toxigenic C. difficile colonization in children, our findings suggest that a positive C. difficile PCR result in a child with diarrhea should be interpreted with caution. Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved.

Shankar-Hari M.,King's College London | Shankar-Hari M.,Foundation Medicine | Phillips G.S.,Ohio State University | Levy M.L.,Brown University | And 8 more authors.
JAMA - Journal of the American Medical Association | Year: 2016

IMPORTANCE: Septic shock currently refers to a state of acute circulatory failure associated with infection. Emerging biological insights and reported variation in epidemiology challenge the validity of this definition. OBJECTIVE To develop a new definition and clinical criteria for identifying septic shock in adults. DESIGN, SETTING, AND PARTICIPANTS: The Society of Critical Care Medicine and the European Society of Intensive Care Medicine convened a task force (19 participants) to revise current sepsis/septic shock definitions. Three sets of studies were conducted: (1) a systematic review and meta-analysis of observational studies in adults published between January 1, 1992, and December 25, 2015, to determine clinical criteria currently reported to identify septic shock and inform the Delphi process; (2) a Delphi study among the task force comprising 3 surveys and discussions of results from the systematic review, surveys, and cohort studies to achieve consensus on a new septic shock definition and clinical criteria; and (3) cohort studies to test variables identified by the Delphi process using Surviving Sepsis Campaign (SSC) (2005-2010; n = 28 150), University of Pittsburgh Medical Center (UPMC) (2010-2012; n = 1 309 025), and Kaiser Permanente Northern California (KPNC) (2009-2013; n = 1 847 165) electronic health record (EHR) data sets. MAIN OUTCOMES AND MEASURES: Evidence for and agreement on septic shock definitions and criteria. RESULTS The systematic review identified 44 studies reporting septic shock outcomes (total of 166 479 patients) from a total of 92 sepsis epidemiology studies reporting different cutoffs and combinations for blood pressure (BP), fluid resuscitation, vasopressors, serum lactate level, and base deficit to identify septic shock. The septic shock-associated crude mortalitywas 46.5% (95%CI, 42.7%-50.3%), with significant between-study statistical heterogeneity (I2 = 99.5%; χ2 = 182.5; P <.001). The Delphi process identified hypotension, serum lactate level, and vasopressor therapy as variables to test using cohort studies. Based on these 3 variables alone or in combination, 6 patient groupswere generated. Examination of the SSC database demonstrated that the patient group requiring vasopressors to maintain mean BP 65mmHg or greater and having a serum lactate level greater than 2 mmol/L (18mg/dL) after fluid resuscitation had a significantly higher mortality (42.3%[95%CI, 41.2%-43.3%]) in risk-adjusted comparisons with the other 5 groups derived using either serum lactate level greater than 2 mmol/L alone or combinations of hypotension, vasopressors, and serum lactate level 2 mmol/L or lower. These findingswere validated in theUPMCand KPNC data sets. CONCLUSIONS AND RELEVANCE: Based on a consensus process using results from a systematic review, surveys, and cohort studies, septic shock is defined as a subset of sepsis in which underlying circulatory, cellular, andmetabolic abnormalities are associated with a greater risk of mortality than sepsis alone. Adult patients with septic shock can be identified using the clinical criteria of hypotension requiring vasopressor therapy to maintain mean BP 65mmHg or greater and having a serum lactate level greater than 2 mmol/L after adequate fluid resuscitation. Copyright 2016 American Medical Association. All rights reserved.

Kao C.M.,Steven and Alexandra Cohen Childrens Medical Center | Schneyer R.J.,Steven and Alexandra Cohen Childrens Medical Center | Bocchini Jr. J.A.,Louisiana State University Health Sciences Center
Current Opinion in Pediatrics | Year: 2014

PURPOSE OF REVIEW: To provide a clinically relevant summary of the latest research and recommendations regarding childhood and adolescent immunizations. RECENT FINDINGS: Childhood vaccination has dramatically reduced pediatric morbidity and mortality in the United States. Recent research on childhood and adolescent immunizations has focused on expanding the use of current vaccines for additional subpopulations as well as the development of new vaccines. In particular, data confirming the safety and immunogenicity of vaccines in various groups of children have shaped national guidelines. Furthermore, studies on vaccine uptake, cost-effectiveness, and impact of vaccination have reinforced the importance of adhering to these guidelines. More work needs to be done by providers and parents to increase vaccination coverage rates to better protect children and adolescents from these serious diseases. In this article, selected recent publications and recommendations on the following vaccines are reviewed: influenza, meningococcal conjugate, childhood and adolescent/adult formulations of diphtheria and tetanus toxoids and acellular pertussis, pneumococcal conjugate, and human papillomavirus. SUMMARY: Research on childhood and adolescent vaccinations continues to shape future guidelines. Through this work, we can learn how to optimize the protection of all children and adolescents against vaccine-preventable diseases. © 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins.

Schneyer R.J.,Steven and Alexandra Cohen Childrens Medical Center | Yang C.,Steven and Alexandra Cohen Childrens Medical Center | Bocchini J.A.,Louisiana State University Health Sciences Center
Current Opinion in Pediatrics | Year: 2015

Purpose of review To provide a clinically relevant synopsis of the latest research and recommendations regarding adolescent immunizations. Recent findings Immunization is an important and effective strategy for preventing morbidity and mortality in adolescents. Although there has been progress in recent years, coverage rates in the US remain suboptimal, particularly for the human papillomavirus vaccine. Much work has been done to better understand and address the barriers to adolescent immunization, so that all teens may be protected against serious vaccine-preventable diseases. In addition, several recent studies have focused on the effectiveness of current adolescent vaccines and the development of new vaccines to protect against additional types of human papillomavirus and serotype B Neisseria meningitidis. Decreased pertussis vaccine effectiveness has led to new recommendations for pregnant women, including adolescents, to protect them and their young infants. The present review highlights selected literature on acellular pertussis, meningococcal, and human papillomavirus vaccines. Research findings on various strategies to improve adolescent vaccine uptake are also discussed in this review. Summary Research on adolescent immunizations and their delivery continues to have an impact on clinical practice and will shape future guidelines. Through this work, we can learn how best to protect adolescents against vaccine-preventable diseases. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.

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.

Glick R.D.,Steven and Alexandra Cohen Childrens Medical Center | Pashankar F.D.,Yale University | Pappo A.,St Jude Childrens Research Hospital | Laquaglia M.P.,Sloan Kettering Cancer Center
Journal of Pediatric Hematology/Oncology | Year: 2012

Pancreatoblastoma is a very rare childhood tumor originating from the epithelial exocrine cells of the pancreas. It is the most common malignant pancreatic tumor in young children and has a mean age of diagnosis of 5 years. It is slow growing and its presentation is varied and often non-specific. Tumors tend to be quite large and appropriate cross sectional imaging is very important to assess for extent, metastatic disease, and resectability. Biopsy for tissue diagnosis is essential. Complete surgical resection is the goal of therapy although many patients are unresectable at initial diagnosis and require neoadjuvant chemotherapy. Adjuvant chemotherapy is also recommended and chemotherapeutic regimens involve cisplatin and doxorubicin. Even with curative resections, these lesions have a high recurrence rate and patients must be followed closely. Knowledge of this rare tumor is important for the clinician confronted with a large retroperitoneal mass in a young child. © 2012 by Lippincott Williams & Wilkins.

Pilapil M.,Steven and Alexandra Cohen Childrens Medical Center | 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.

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.

Fox J.E.,Steven and Alexandra Cohen Childrens Medical Center | Volpe L.,St Marys Hospital For Children | Bullaro J.,St Marys Hospital For Children | Kakkis E.D.,Ultragenyx Pharmaceutical | Sly W.S.,Saint Louis University
Molecular Genetics and Metabolism | Year: 2015

Mucopolysaccharidosis type VII (MPS VII, Sly syndrome) is a very rare lysosomal storage disease caused by a deficiency of the enzyme β-glucuronidase (GUS), which is required for the degradation of three glycosaminoglycans (GAGs): dermatan sulfate, heparan sulfate, and chondroitin sulfate. Progressive accumulation of these GAGs in lysosomes leads to increasing dysfunction in numerous tissues and organs. Enzyme replacement therapy (ERT) has been used successfully for other MPS disorders, but there is no approved treatment for MPS VII. Here we describe the first human treatment with recombinant human GUS (rhGUS), an investigational therapy for MPS VII, in a 12-year old boy with advanced stage MPS VII. Despite a tracheostomy, nocturnal continuous positive airway pressure, and oxygen therapy, significant pulmonary restriction and obstruction led to oxygen dependence and end-tidal carbon dioxide (ETCO2) levels in the 60-80mmHg range, eventually approaching respiratory failure (ETCO2 of 100mmHg) and the need for full-time ventilation. Since no additional medical measures could improve his function, we implemented experimental ERT by infusing rhGUS at 2mg/kg over 4h every 2weeks for 24weeks. Safety was evaluated by standard assessments and observance for any infusion associated reactions (IARs). Urinary GAG (uGAG) levels, pulmonary function, oxygen dependence, CO2 levels, cardiac valve function, liver and spleen size, and growth velocity were assessed to evaluate response to therapy. rhGUS infusions were well tolerated. No serious adverse events (SAEs) or IARs were observed. After initiation of rhGUS infusions, the patient's uGAG excretion decreased by more than 50%. Liver and spleen size were reduced within 2weeks of the first infusion and reached normal size by 24weeks. Pulmonary function appeared to improve during the course of treatment based on reduced changes in ETCO2 after off-ventilator challenges and a reduced oxygen requirement. The patient regained the ability to eat orally, gained weight, and his energy and activity levels increased. Over 24weeks, treatment with every-other-week infusions of rhGUS was well tolerated with no SAEs, IARs, or hypersensitivity reactions and was associated with measurable improvement in objective clinical measures and quality of life. © 2014 Elsevier Inc.

Vlachos A.,Feinstein Institute for Medical Research | Vlachos A.,Steven and Alexandra Cohen Childrens Medical Center | Blanc L.,Feinstein Institute for Medical Research | Blanc L.,Steven and Alexandra Cohen Childrens Medical Center | And 2 more authors.
Expert Review of Hematology | Year: 2014

Diamond Blackfan anemia (DBA) is an inherited bone marrow failure syndrome. As with the other rare inherited bone marrow failure syndromes, the study of these disorders provides important insights into basic biology and, in the case of DBA, ribosome biology; the disruption of which characterizes the disorder. Thus DBA serves as a paradigm for translational medicine in which the efforts of clinicians to manage DBA have informed laboratory scientists who, in turn, have stimulated clinical researchers to utilize scientific discovery to provide improved care. In this review we describe the clinical syndrome Diamond Blackfan anemia and, in particular, we demonstrate how the study of DBA has allowed scientific inquiry to create opportunities for progress in its understanding and treatment. © 2014 Informa UK, Ltd.

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