Arnold Palmer Hospital for Children

About: Arnold Palmer Hospital for Children is a healthcare organization based out in Orlando, Florida, United States. It is known for research contribution in the topics: Medicine & Ventricle. The organization has 347 authors who have published 416 publications receiving 6925 citations. The organization is also known as: Arnold Palmer Hospital for Children & Women.

Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis—State of the Art: Cystic Fibrosis Foundation Consensus Conference

Abstract: Because of the difficulties of recognizing allergic bronchopulmonary aspergillosis (ABPA) in the context of cystic fibrosis (because of overlapping clinical, radiographic, microbiologic, and immunologic features), advances in our understanding of the pathogenesis of allergic aspergillosis, new possibilities in therapy, and the need for agreed-upon definitions, an international consensus conference was convened. Areas addressed included fungal biology, immunopathogenesis, insights from animal models, diagnostic criteria, epidemiology, the use of new immunologic and genetic techniques in diagnosis, imaging modalities, pharmacology, and treatment approaches. Evidence from the existing literature was graded, and the consensus views were synthesized into this document and recirculated for affirmation. Virulence factors in Aspergillus that could aggravate these diseases, and particularly immunogenetic factors that could predispose persons to ABPA, were identified. New information has come from transgenic animals and recombinant fungal and host molecules. Diagnostic criteria that could provide a framework for monitoring were adopted, and helpful imaging features were identified. New possibilities in therapy produced plans for managing diverse clinical presentations.

Genomic analysis of diffuse intrinsic pontine gliomas identifies three molecular subgroups and recurrent activating ACVR1 mutations

Abstract: Diffuse intrinsic pontine glioma (DIPG) is a fatal brain cancer that arises in the brainstem of children, with no effective treatment and near 100% fatality. The failure of most therapies can be attributed to the delicate location of these tumors and to the selection of therapies on the basis of assumptions that DIPGs are molecularly similar to adult disease. Recent studies have unraveled the unique genetic makeup of this brain cancer, with nearly 80% found to harbor a p.Lys27Met histone H3.3 or p.Lys27Met histone H3.1 alteration. However, DIPGs are still thought of as one disease, with limited understanding of the genetic drivers of these tumors. To understand what drives DIPGs, we integrated whole-genome sequencing with methylation, expression and copy number profiling, discovering that DIPGs comprise three molecularly distinct subgroups (H3-K27M, silent and MYCN) and uncovering a new recurrent activating mutation affecting the activin receptor gene ACVR1 in 20% of DIPGs. Mutations in ACVR1 were constitutively activating, leading to SMAD phosphorylation and increased expression of the downstream activin signaling targets ID1 and ID2. Our results highlight distinct molecular subgroups and novel therapeutic targets for this incurable pediatric cancer.

Defining Pediatric Malnutrition A Paradigm Shift Toward Etiology-Related Definitions

Abstract: Lack of a uniform definition is responsible for underrecognition of the prevalence of malnutrition and its impact on outcomes in children. A pediatric malnutrition definitions workgroup reviewed existing pediatric age group English-language literature from 1955 to 2011, for relevant references related to 5 domains of the definition of malnutrition that were a priori identified: anthropometric parameters, growth, chronicity of malnutrition, etiology and pathogenesis, and developmental/ functional outcomes. Based on available evidence and an iterative process to arrive at multidisciplinary consensus in the group, these domains were included in the overall construct of a new definition. Pediatric malnutrition (undernutrition) is defined as an imbalance between nutrient requirements and intake that results in cumulative deficits of energy, protein, or micronutrients that may negatively affect growth, development, and other relevant outcomes. A summary of the literature is presented and a new classification scheme is proposed that incorporates chronicity, etiology, mechanisms of nutrient imbalance, severity of malnutrition, and its impact on outcomes. Based on its etiology, malnutrition is either illness related (secondary to 1 or more diseases/injury) or non-illness related, (caused by environmental/behavioral factors), or both. Future research must focus on the relationship between inflammation and illness-related malnutrition. We anticipate that the definition of malnutrition will continue to evolve with improved understanding of the processes that lead to and complicate the treatment of this condition. A uniform definition should permit future research to focus on the impact of pediatric malnutrition on functional outcomes and help solidify the scientific basis for evidence-based nutrition practices.

Consensus Statement of the Academy of Nutrition and Dietetics/American Society for Parenteral and Enteral Nutrition Indicators Recommended for the Identification and Documentation of Pediatric Malnutrition (Undernutrition)

Abstract: The Academy of Nutrition and Dietetics (the Academy) and the American Society for Parenteral and Enteral Nutrition (A.S.P.E.N.), utilizing an evidence-informed, consensus-derived process, recommend that a standardized set of diagnostic indicators be used to identify and document pediatric malnutrition (undernutrition) in routine clinical practice. The recommended indicators include z scores for weight-for-height/length, body mass index-for-age, or length/height-for-age or mid-upper arm circumference when a single data point is available. When 2 or more data points are available, indicators may also include weight gain velocity (<2 years of age), weight loss (2-20 years of age), deceleration in weight for length/height z score, and inadequate nutrient intake. The purpose of this consensus statement is to identify a basic set of indicators that can be used to diagnose and document undernutrition in the pediatric population ages 1 month to 18 years. The indicators are intended for use in multiple settings (eg, acute, ambulatory care/outpatient, residential care). Several screening tools have been developed for use in hospitalized children. However, identifying criteria for use in screening for nutritional risk is not the purpose of this paper. Clinicians should use as many data points as available to identify and document the presence of malnutrition. The universal use of a single set of diagnostic parameters will expedite the recognition of pediatric undernutrition, lead to the development of more accurate estimates of its prevalence and incidence, direct interventions, and promote improved outcomes. A standardized diagnostic approach will also inform the prediction of the human and financial responsibilities and costs associated with the prevention and treatment of undernutrition in this vulnerable population and help to further ensure the provision of high-quality, cost-effective nutritional care.

Respiratory Syncytial Virus Genomic Load and Disease Severity Among Children Hospitalized With Bronchiolitis: Multicenter Cohort Studies in the United States and Finland

Abstract: Background. We investigated whether children with a higher respiratory syncytial virus (RSV) genomic load are at a higher risk of more-severe bronchiolitis. Methods. Two multicenter prospective cohort studies in the United States and Finland used the same protocol to enroll children aged <2 years hospitalized for bronchiolitis and collect nasopharyngeal aspirates. By using realtime polymerase chain reaction analysis, patients were classified into 3 genomic load status groups: low, intermediate, and high. Outcome measures were a length of hospital stay (LOS) of ≥3 days and intensive care use, defined as admission to the intensive care unit or use of mechanical ventilation. Results. Of 2615 enrolled children, 1764 (67%) had RSV bronchiolitis. Children with a low genomic load had a higher unadjusted risk of having a length of stay of ≥3 days (52%), compared with children with intermediate and those with high genomic loads (42% and 51%, respectively). In a multivariable model, the risk of having a length of stay of ≥3 days remained significantly higher in the groups with intermediate (odds ratio [OR], 1.43; 95% confidence interval [CI], 1.20–1.69) and high (OR, 1.58; 95% CI, 1.29–1.94) genomic loads. Similarly, children with a high genomic load had a higher risk of intensive care use (20%, compared with 15% and 16% in the groups with low and intermediate genomic loads, respectively). In a multivariable model, the risk remained significantly higher in the group with a high genomic load (OR, 1.43; 95% CI, 1.03–1.99). Conclusion. Children with a higher RSV genomic load had a higher risk for more-severe bronchiolitis.