Institution
Children's Memorial Hospital
Healthcare•
About: Children's Memorial Hospital is a based out in . It is known for research contribution in the topics: Population & Transplantation. The organization has 5652 authors who have published 8967 publications receiving 283837 citations.
Topics: Population, Transplantation, Medicine, Poison control, Health care
Papers published on a yearly basis
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TL;DR: This study is the first assessment, to date, of the Sepsis-3 definitions in critically ill children and demonstrates use of these definitions in children is feasible and shows promising results.
Abstract: Importance The Third International Consensus Definitions for Sepsis and Septic Shock (Sepsis-3) uses the Sequential Organ Failure Assessment (SOFA) score to grade organ dysfunction in adult patients with suspected infection. However, the SOFA score is not adjusted for age and therefore not suitable for children. Objectives To adapt and validate a pediatric version of the SOFA score (pSOFA) in critically ill children and to evaluate the Sepsis-3 definitions in patients with confirmed or suspected infection. Design, Setting, and Participants This retrospective observational cohort study included all critically ill children 21 years or younger admitted to a 20-bed, multidisciplinary, tertiary pediatric intensive care unit between January 1, 2009 and August 1, 2016. Data on these children were obtained from an electronic health record database. The pSOFA score was developed by adapting the original SOFA score with age-adjusted cutoffs for the cardiovascular and renal systems and by expanding the respiratory criteria to include noninvasive surrogates of lung injury. Daily pSOFA scores were calculated from admission until day 28 of hospitalization, discharge, or death (whichever came first). Three additional pediatric organ dysfunction scores were calculated for comparison. Exposures Organ dysfunction measured by the pSOFA score, and sepsis and septic shock according to the Sepsis-3 definitions. Main Outcomes and Measures The primary outcome was in-hospital mortality. The daily pSOFA scores and additional pediatric organ dysfunction scores were compared. Performance was evaluated using the area under the curve. The pSOFA score was then used to assess the Sepsis-3 definitions in the subgroup of children with confirmed or suspected infection. Results In all, 6303 patients with 8711 encounters met inclusion criteria. Each encounter was treated independently. Of the 8482 survivors of hospital encounters, 4644 (54.7%) were male and the median (interquartile range [IQR]) age was 69 (17-156) months. Among the 229 nonsurvivors, 127 (55.4%) were male with a median (IQR) age of 43 (8-144) months. In-hospital mortality was 2.6%. The maximum pSOFA score had excellent discrimination for in-hospital mortality, with an area under the curve of 0.94 (95% CI, 0.92-0.95). The pSOFA score had a similar or better performance than other pediatric organ dysfunction scores. According to the Sepsis-3 definitions, 1231 patients (14.1%) were classified as having sepsis and had a mortality rate of 12.1%, and 347 (4.0%) had septic shock and a mortality rate of 32.3%. Patients with sepsis were more likely to die than patients with confirmed or suspected infection but no sepsis (odds ratio, 18; 95% CI, 11-28). Of the 229 patients who died during their hospitalization, 149 (65.0%) had sepsis or septic shock during their course. Conclusions and Relevance The pSOFA score was adapted and validated with age-adjusted variables in critically ill children. Using the pSOFA score, the Sepsis-3 definitions were assessed in children with confirmed or suspected infection. This study is the first assessment, to date, of the Sepsis-3 definitions in critically ill children. Use of these definitions in children is feasible and shows promising results.
366 citations
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TL;DR: Clinical and genetic studies suggest odds greater than 10(16):1 that the genetic defect for Holt-Oram syndrome is present on the long arm of chromosome 12 (12q2) and Mutations in a gene on chromosome 12q2 can produce a wide range of disease phenotypes characteristic of the Holt- Oram syndrome.
Abstract: Background The Holt-Oram syndrome is an autosomal dominant condition characterized by skeletal abnormalities that are frequently accompanied by congenital cardiac defects. The cause of these disparate clinical features is unknown. To identify the chromosomal location of the Holt-Oram syndrome gene, we performed clinical and genetic studies. Methods Two large families with the Holt-Oram syndrome were evaluated by radiography of the hands, electrocardiography, and transthoracic echocardiography. Genetic-linkage analyses were performed with polymorphic DNA loci dispersed throughout the genome to identify a locus that was inherited with the Holt-Oram syndrome in family members. Results A total of 19 members of Family A had Holt-Oram syndrome with mild-to-moderate skeletal deformities, including triphalangeal thumbs and carpal-bone dysmorphism. All affected members of Family A had moderate-to-severe congenital cardiac abnormalities, such as ventricular or atrial septal defects or atrioventricular-canal defects...
365 citations
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Florence Demenais1, Florence Demenais2, Patricia Margaritte-Jeannin2, Patricia Margaritte-Jeannin1 +213 more•Institutions (79)
TL;DR: A meta-analysis of GWAS studies for asthma from multiancestral cohorts identifies five new loci and finds that the asthma-associated loci are enriched near enhancer marks in immune cells, suggesting a major role of these loci in the regulation of immunologically related mechanisms.
Abstract: We examined common variation in asthma risk by conducting a meta-analysis of worldwide asthma genome-wide association studies (23,948 asthma cases, 118,538 controls) of individuals from ethnically diverse populations. We identified five new asthma loci, found two new associations at two known asthma loci, established asthma associations at two loci previously implicated in the comorbidity of asthma plus hay fever, and confirmed nine known loci. Investigation of pleiotropy showed large overlaps in genetic variants with autoimmune and inflammatory diseases. The enrichment in enhancer marks at asthma risk loci, especially in immune cells, suggested a major role of these loci in the regulation of immunologically related mechanisms.
363 citations
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Cincinnati Children's Hospital Medical Center1, University of Texas at Austin2, Emory University3, Wayne State University4, University of Toronto5, East Carolina University6, Baylor College of Medicine7, Eastern Virginia Medical School8, Children's National Medical Center9, University of Texas Southwestern Medical Center10, University of Alabama at Birmingham11, Nemours Foundation12, Case Western Reserve University13, Columbia University14, University of Pennsylvania15, Medical University of South Carolina16, State University of New York System17, University of South Carolina18, Harvard University19, University of South Alabama20, University of Miami21, University of Mississippi22, Children's Memorial Hospital23, Duke University24, Georgia Regents University25, University of Southern California26
TL;DR: High-risk children with sickle cell anaemia and abnormal TCD velocities who have received at least 1 year of transfusions, and have no MRA-defined severe vasculopathy, hydroxycarbamide treatment can substitute for chronic transfusions to maintain TCD velocity and help to prevent primary stroke.
363 citations
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National and Kapodistrian University of Athens1, Gunma University2, University of Manchester3, University of Wisconsin-Madison4, University of Southampton5, The Chinese University of Hong Kong6, University of Cape Town7, Swiss Institute of Allergy and Asthma Research8, Washington University in St. Louis9, University of Padua10, National University of Singapore11, Paris Descartes University12, University of Verona13, Duke University14, Creighton University15, Pontifical Catholic University of Chile16, Ain Shams University17, National Health Service18, Medical University of Vienna19, Karolinska Institutet20, University of Virginia21, University of Ulsan22, Chang Gung University23, Stellenbosch University24, Medical University of Łódź25, Charité26, University of South Florida27, Sungkyunkwan University28, University of Colorado Denver29, Nippon Medical School30, Charles University in Prague31, Children's Memorial Hospital32, Royal Children's Hospital33, Federal University of Paraná34, Children's Mercy Hospital35, University of Queensland36, Pontifícia Universidade Católica do Rio Grande do Sul37, Princess Margaret Hospital for Children38, University of Denver39, University of Turku40, Mahidol University41, Nova Southeastern University42, University of Gothenburg43, University of Zurich44, Kaiser Permanente45
TL;DR: The purpose of this document is to highlight the key messages that are common to many of the existing guidelines, while critically reviewing and commenting on any differences, thus providing a concise reference.
Abstract: Asthma is the most common chronic lower respiratory disease in childhood throughout the world. Several guidelines and/or consensus documents are available to support medical decisions on pediatric asthma. Although there is no doubt that the use of common systematic approaches for management can considerably improve outcomes, dissemination and implementation of these are still major challenges. Consequently, the International Collaboration in Asthma, Allergy and Immunology (iCAALL), recently formed by the EAACI, AAAAI, ACAAI, and WAO, has decided to propose an International Consensus on (ICON) Pediatric Asthma. The purpose of this document is to highlight the key messages that are common to many of the existing guidelines, while critically reviewing and commenting on any differences, thus providing a concise reference. The principles of pediatric asthma management are generally accepted. Overall, the treatment goal is disease control. To achieve this, patients and their parents should be educated to optimally manage the disease, in collaboration with healthcare professionals. Identification and avoidance of triggers is also of significant importance. Assessment and monitoring should be performed regularly to re-evaluate and fine-tune treatment. Pharmacotherapy is the cornerstone of treatment. The optimal use of medication can, in most cases, help patients control symptoms and reduce the risk for future morbidity. The management of exacerbations is a major consideration, independent of chronic treatment. There is a trend toward considering phenotype-specific treatment choices; however, this goal has not yet been achieved.
360 citations
Authors
Showing all 5672 results
Name | H-index | Papers | Citations |
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Jorge E. Cortes | 163 | 2784 | 124154 |
Marc C. Hochberg | 127 | 691 | 87268 |
Michael Andreeff | 117 | 959 | 54734 |
Bharat Bhushan | 116 | 1276 | 62506 |
Donald M. Lloyd-Jones | 115 | 706 | 112655 |
David N. Herndon | 108 | 1227 | 54888 |
Frederick J. Schoen | 102 | 434 | 42611 |
Kathryn M. Edwards | 102 | 628 | 39467 |
Alan R. Dyer | 95 | 283 | 44252 |
Mark C. Willingham | 94 | 394 | 36167 |
Nicholas Katsanis | 93 | 348 | 34133 |
Peter D. Gluckman | 92 | 525 | 33375 |
Helga Refsum | 90 | 316 | 37463 |
Dale A. Schoeller | 90 | 391 | 30776 |
Shlomo Shinnar | 90 | 288 | 25621 |