St Marys Hospital For Children

Bayside, NY, United States

St Marys Hospital For Children

Bayside, NY, United States
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Allen K.,Murdoch Childrens Research Institute | Chong S.,St Marys Hospital for Children | Dias J.A.,Hospital St Joao | Papadopoulou A.,Athens Childrens Hospital
Journal of Pediatric Gastroenterology and Nutrition | Year: 2013

OBJECTIVES:: This review considers the potential for therapeutic advances in the management of eosinophilic oesophagitis (EoE) based on recently increased understanding of the pathophysiology of the disorder. METHODS:: This is a review of publications characterising mucosal changes and leucocyte recruitment patterns in human and experimental EoE. RESULTS:: EoE, although diagnosed by epithelial infiltration of eosinophils, is actually a transmural inflammation in which eosinophil recruitment occurs via the deeper layers. Penetration of eosinophils into the epithelium is variable, explaining the need for multiple biopsies to diagnose what may be a clearly visible disorder. Fibrosis and neuromuscular dysfunction both occur within the subepithelial tissues. Recent murine studies have identified that T-cell recruitment underpins antigen-specific oesophageal eosinophil recruitment. Involvement of innate immunity is also suggested by the role of invariant natural killer T cells in experimental EoE. CONCLUSIONS:: Looking beyond present therapeutic options with a view to future studies, we identify T cells as candidates for "upstream therapy" if antigen specificity or homing markers are determined. Evidence of aeroallergen sensitisation suggests the possibility of lymphocyte priming within nasal-associated lymphoid tissue or Waldeyer ring, with the potential for topical therapy. We consider acquired neuromuscular dysfunction as a therapeutic target in acute symptomatic deterioration or bolus obstruction. We assess possible similarities with therapeutic stratagems for chronic asthma, recognising at the same time the anatomic specificity of the oesophagus and the difficulty in delivering effective topical medication to subepithelial tissues in this location compared with the airway. Copyright © 2013 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.


Nehra D.,Harvard University | Carlson S.J.,Harvard University | Fallon E.M.,Harvard University | Kalish B.,Harvard University | And 5 more authors.
Journal of Parenteral and Enteral Nutrition | Year: 2013

Background: Premature infants are at increased risk for metabolic bone disease, with resulting delayed bone growth, osteopenia, and rickets. Method: A systematic review of the best available evidence to answer a series of questions regarding neonatal patients at risk of metabolic bone disease receiving parenteral or enteral nutrition was undertaken and evaluated using concepts adopted from the Grading of Recommendations, Assessment, Development and Evaluation working group. A consensus process was used to develop the clinical guideline recommendations prior to external and internal review and approval by the American Society for Parenteral and Enteral Nutrition Board of Directors. Questions: (1) What maternal risk factors predispose the neonate to metabolic bone disease? (2) What is the optimal type of feeding to promote neonatal bone health? (3) When and how should vitamin D supplements be administered? (4) Does parenteral nutrition (PN) predispose a neonate to metabolic bone disease, and if so, are there PN formulation recommendations to minimize this risk? © 2013 American Society for Parenteral and Enteral Nutrition.


Fallon E.M.,Harvard University | Nehra D.,Harvard University | Potemkin A.K.,Harvard University | Gura K.M.,Childrens Hospital Boston | And 3 more authors.
Journal of Parenteral and Enteral Nutrition | Year: 2012

Background: Necrotizing enterocolitis (NEC) is one of the most devastating diseases in the neonatal population, with extremely low birth weight and extremely preterm infants at greatest risk. Method: A systematic review of the best available evidence to answer a series of questions regarding nutrition support of neonates at risk of NEC was undertaken and evaluated using concepts adopted from the Grading of Recommendations, Assessment, Development and Evaluation working group. A consensus process was used to develop the clinical guideline recommendations prior to external and internal review and approval by the A.S.P.E.N. Board of Directors. Results/Conclusions: (1) When and how should feeds be started in infants at high risk for NEC? We suggest that minimal enteral nutrition be initiated within the first 2 days of life and advanced by 30 mL/kg/d in infants ≤1000g. (Weak) (2) Does the provision of mother's milk reduce the risk of developing NEC? We suggest the exclusive use of mother's milk rather than bovine-based products or formula in infants at risk for NEC. (Weak) (3) Do probiotics reduce the risk of developing NEC? There are insufficient data to recommend the use of probiotics in infants at risk for NEC. (Further research needed.) (4) Do nutrients either prevent or predispose to the development of NEC? We do not recommend glutamine supplementation for infants at risk for NEC (Strong). There is insufficient evidence to recommend arginine and/or long chain polyunsaturated fatty acid supplementation for infants at risk for NEC. (Further research needed.) (5) When should feeds be reintroduced to infants with NEC? There are insufficient data to make a recommendation regarding time to reintroduce feedings to infants after NEC. (Further research needed.) (JPEN J Parenter Enteral Nutr. 2012;36:506-523). © 2012 American Society for Parenteral and Enteral Nutrition.


Abbruzzese L.D.,Columbia University | Rao A.K.,Columbia University | Bellows R.,Spear Physical Therapy | Figueroa K.,YAI NYL Gramercy School | And 3 more authors.
Gait and Posture | Year: 2014

This study examined the dual-task interference effects of complexity (simple vs. complex), type of task (carrying a pitcher vs. tray), and age (young adults vs. 7-10 year old children) on temporal-spatial and variability measures of gait. All participants first walked on the GAITRite® walkway without any concurrent task, followed by four dual-task gait conditions. The group of children had a more variable step length and step time than adults across all walking conditions. They also slowed down, took fewer, smaller steps and spent more time in double limb support than adults in the complex dual task conditions. Gait in healthy young adults and school aged children was relatively unaffected by concurrent performance of simple versions of the manual tasks. Our overall analysis suggests that dual-task gait in school aged children is still developing and has not yet reached adult capacity. This study also highlights the critical role of task demand and complexity in dual-task interference. © 2014 Elsevier B.V. All rights reserved.


PubMed | St Marys Hospital For Children, Sloan Kettering Cancer Center, Swedish Medical Center, Spear Physical Therapy and 2 more.
Type: Journal Article | Journal: Gait & posture | Year: 2014

This study examined the dual-task interference effects of complexity (simple vs. complex), type of task (carrying a pitcher vs. tray), and age (young adults vs. 7-10 year old children) on temporal-spatial and variability measures of gait. All participants first walked on the GAITRite walkway without any concurrent task, followed by four dual-task gait conditions. The group of children had a more variable step length and step time than adults across all walking conditions. They also slowed down, took fewer, smaller steps and spent more time in double limb support than adults in the complex dual task conditions. Gait in healthy young adults and school aged children was relatively unaffected by concurrent performance of simple versions of the manual tasks. Our overall analysis suggests that dual-task gait in school aged children is still developing and has not yet reached adult capacity. This study also highlights the critical role of task demand and complexity in dual-task interference.


Hendy H.M.,Psychology Program | Seiverling L.,St Marys Hospital For Children | Lukens C.T.,Children's Hospital of Philadelphia | Williams K.E.,Penn State Hershey Medical Center
Children's Health Care | Year: 2013

The present study examined psychometric properties of the Brief Autism Mealtime Behavior Inventory (BAMBI) when applied to a population of children with feeding problems. The new scale was renamed the Brief Assessment of Mealtime Behavior in Children (BAMBIC) for wider clinical usefulness. Parents completed questionnaires with the original BAMBI, the Child Eating Behavior Questionnaire, and the Parent Mealtime Action Scale. The revised BAMBIC developed with factor analysis had stronger psychometrics than the original scale and produced three subscales of feeding problems: Limited Variety, Food Refusal, and Disruptive Behavior. More Limited Variety was reported for boys than girls, and more Food Refusal was reported for younger children and children with special needs. © 2013 Copyright Taylor & Francis Group, LLC.


Murray M.T.,Columbia University | Pavia M.,St Marys Hospital For Children | Jackson O.,Elizabeth Seton Pediatric Center | Keenan M.,Sunshine Childrens Home and Rehabilitation Center | And 6 more authors.
American Journal of Infection Control | Year: 2015

Children in pediatric long-term care facilities (pLTCFs) have complex medical conditions and increased risk for health care-associated infections (HAIs). We performed a retrospective study from January 2010-December 2013 at 3 pLTCFs to describe HAI outbreaks and associated infection control interventions. There were 62 outbreaks involving 700 cases in residents and 250 cases in staff. The most common interventions were isolation precautions and education and in-services. Further research should examine interventions to limit transmission of infections in pLTCFs. Copyright © 2015 by the Association for Professionals in Infection Control and Epidemiology, Inc. Published by Elsevier Inc. All rights reserved.


Murray M.T.,Columbia University | Cohen B.,Columbia University | Neu N.,Columbia University | Neu N.,Elizabeth Seton Pediatric Center | And 5 more authors.
American Journal of Infection Control | Year: 2014

Pediatric long-term care facilities (pLTCFs) provide for children with chronic, complex medical needs and therefore face unique challenges for infection prevention and control (IP&C). At a conference in 2012, pLTCF providers reported IP&C issues of greatest concern in a survey. Major concerns included the lack of IP&C best practice guidelines, multidrug-resistant bacteria, and viral respiratory infections. Best practice guidelines for IP& C specific to pLTCF populations should be developed and evaluated. Copyright © 2014 by the Association for Professionals in Infection Control and Epidemiology, Inc. Published by 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.


Krishnaswamy S.,St Marys Hospital for Children | Shriber L.,Nazareth College | Srimathveeravalli G.,Sloan Kettering Cancer Center
Journal of Computer Assisted Learning | Year: 2014

This study investigated the effects of a robot-mediated visual motor program on improving the visual motor skills of children ages 5 to 11 years with learning disabilities and visual motor delays. It also compared the effects of a robotic program with traditional occupational therapy and tested the reliability of the computer-generated scores. A randomized experimental design was used with 25 students assigned to either a robotic program group or traditional occupational therapy. The primary outcome measure was the Developmental Test of Visual Motor Integration (VMI). Non-parametric statistics evaluated the effects of intervention and compared the two methods. The correlation between the computer-based scoring methods and the VMI was tested. The children who received the robotic program demonstrated significant gains in visual motor performance. The correlation between the computer-based scoring and the VMI was moderate, but not significant. © 2013 John Wiley & Sons Ltd.

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