Institution
Boston Children's Hospital
Healthcare•Boston, Massachusetts, United States•
About: Boston Children's Hospital is a healthcare organization based out in Boston, Massachusetts, United States. It is known for research contribution in the topics: Population & Transplantation. The organization has 165409 authors who have published 215589 publications receiving 6885627 citations.
Topics: Population, Transplantation, Poison control, Intensive care, Health care
Papers published on a yearly basis
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University of Michigan1, University of Texas Southwestern Medical Center2, Harvard University3, National Institutes of Health4, University of Pennsylvania5, Children's Hospital of Wisconsin6, Wake Forest University7, Duke University8, East Carolina University9, University of Southern California10, University of Toronto11, Boston Children's Hospital12, Medical University of South Carolina13, University of Utah14, Alfred I. duPont Hospital for Children15, Emory University16, Cardinal Glennon Children's Hospital17
TL;DR: In children undergoing the Norwood procedure, transplantation-free survival at 12 months was better with the RVPA shunt than with the MBT shunt, and the rate of nonfatal serious adverse events at the age of 12 months were similar in the two groups.
Abstract: Background The Norwood procedure with a modified Blalock–Taussig (MBT) shunt, the first palliative stage for single-ventricle lesions with systemic outflow obstruction, is associated with high mortality. The right ventricle–pulmonary artery (RVPA) shunt may improve coronary flow but requires a ventriculotomy. We compared the two shunts in infants with hypoplastic heart syndrome or related anomalies. Methods Infants undergoing the Norwood procedure were randomly assigned to the MBT shunt (275 infants) or the RVPA shunt (274 infants) at 15 North American centers. The primary outcome was death or cardiac transplantation 12 months after randomization. Secondary outcomes included unintended cardiovascular interventions and right ventricular size and function at 14 months and transplantation-free survival until the last subject reached 14 months of age. Results Transplantation-free survival 12 months after randomization was higher with the RVPA shunt than with the MBT shunt (74% vs. 64%, P=0.01). However, the R...
751 citations
TL;DR: Parental report is a better indicator of obesity than teen report of weight status, but parental and teen reports are both poor predictors of adolescent obesity.
Abstract: Objectives. Adolescent obesity is becoming an increasing public health problem. This study determines: 1) differences in teen and parental report of obesity, 2) amount of misclassification using body mass index (BMI) from self-reported versus measured height and weight as an indicator of obesity, and 3) whether misclassification varies by gender and socioeconomic status. Design. Weighted data from 15 483 baseline (T1) youth and parental interviews from the National Longitudinal Study of Adolescent Health were used. Seventy-four percent of teens were reinterviewed 1 year later (T2). Parents reported socioeconomic status indicators and whether their teen was obese. Teens reported height, weight, and weight perception. BMI was calculated from both self-reported height and weight at T1 and T2 and from measured height and weight at T2. Those with a BMI ≥95% corrected for age and gender were considered obese. Results. At T1, nearly one half of teens (47%) reporting they were very overweight were not obese by BMI. For teens obese by BMI, 19.6% were reported to be obese by both parent and teen, 6.4% by teen only, 29.9% by parent only, and 44.2% by neither teen nor parent. For those with persistent obesity, teen and/or parental report failed to identify more than one third (34%) as obese; 23.4% were identified by both teen and parent report, 5.4% by teen report only, and 37.2% by parent only. At T2, the correlation between BMI calculated from self-reported versus measured height and weight for the overall population was very strong (r = .92). Specificity of obesity status based on self-reported BMI, compared with obesity status based on measured BMI was .996; sensitivity, .722; positive predictive value, .860; and negative predictive value, .978. Overall, 3.8% of teens were misclassified using self-report measures. Girls were no more likely than boys to be misclassified as obese using BMI from self-reported height and weight. Conclusions. Parental report is a better indicator of obesity than teen report of weight status, but parental and teen reports are both poor predictors of adolescent obesity. Using BMI based on self-reported height and weight correctly classified 96% as to obesity status. Thus, studies can use self-reported height and weight to understand teen obesity and its correlates/sequelae.
750 citations
TL;DR: This review concludes that there have been substantial advances in the knowledge of the clinical manifestations, pathogenesis, and treatment of herpes simplex virus infections in the 12 years since the last review.
Abstract: IN the 12 years since the last review of herpes simplex virus (HSV) infections in these pages,1 there have been substantial advances in our knowledge of the clinical manifestations, pathogenesis, a...
749 citations
University of Toronto1, German Cancer Research Center2, Harvard University3, Broad Institute4, University of British Columbia5, Heidelberg University6, Memorial Sloan Kettering Cancer Center7, Vanderbilt University8, Sanford-Burnham Institute for Medical Research9, Medical University of Vienna10, Curie Institute11, University of Lausanne12, Seoul National University13, Johns Hopkins University14, University of Lyon15, University of Pittsburgh16, University of Michigan17, University of Alabama at Birmingham18, Catholic University of the Sacred Heart19, Erasmus University Rotterdam20, Boston Children's Hospital21, University of Washington22, Masaryk University23, McMaster University24, Hamilton Health Sciences25, Duke University26, McGill University27, McGill University Health Centre28, Newcastle University29, University of California, San Francisco30, University of Debrecen31, Tohoku University32, Saint Louis University33, Washington University in St. Louis34, University of California, Los Angeles35, Emory University36, University of Cincinnati37, Kumamoto University38, Semmelweis University39, University of Arkansas for Medical Sciences40, University of Naples Federico II41, Chonnam National University42, University of São Paulo43, University of Colorado Denver44, University of Ulsan45, University of Calgary46, Stanford University47
TL;DR: Somatic copy number aberrations (SCNAs) in 1,087 unique medulloblastomas are reported, including recurrent events targeting TGF-β signalling in Group 3, and NF-κB signalling in Groups 4, which suggest future avenues for rational, targeted therapy.
Abstract: Medulloblastoma, the most common malignant paediatric brain tumour, is currently treated with nonspecific cytotoxic therapies including surgery, whole-brain radiation, and aggressive chemotherapy. As medulloblastoma exhibits marked intertumoural heterogeneity, with at least four distinct molecular variants, previous attempts to identify targets for therapy have been underpowered because of small samples sizes. Here we report somatic copy number aberrations (SCNAs) in 1,087 unique medulloblastomas. SCNAs are common in medulloblastoma, and are predominantly subgroup-enriched. The most common region of focal copy number gain is a tandem duplication of SNCAIP, a gene associated with Parkinson's disease, which is exquisitely restricted to Group 4α. Recurrent translocations of PVT1, including PVT1-MYC and PVT1-NDRG1, that arise through chromothripsis are restricted to Group 3. Numerous targetable SCNAs, including recurrent events targeting TGF-β signalling in Group 3, and NF-κB signalling in Group 4, suggest future avenues for rational, targeted therapy.
749 citations
TL;DR: It is reported for the first time that impaired but not abolished NF-κB signaling in humans results in two related syndromes that associate specific developmental and immunological defects.
Abstract: The molecular basis of X-linked recessive anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID) has remained elusive. Here we report hypomorphic mutations in the gene IKBKG in 12 males with EDA-ID from 8 kindreds, and 2 patients with a related and hitherto unrecognized syndrome of EDA-ID with osteopetrosis and lymphoedema (OL-EDA-ID). Mutations in the coding region of IKBKG are associated with EDA-ID, and stop codon mutations, with OL-EDA-ID. IKBKG encodes NEMO, the regulatory subunit of the IKK (IκB kinase) complex, which is essential for NF-κB signaling. Germline loss-of-function mutations in IKBKG are lethal in male fetuses. We show that IKBKG mutations causing OL-EDA-ID and EDA-ID impair but do not abolish NF-κB signaling. We also show that the ectodysplasin receptor, DL, triggers NF-κB through the NEMO protein, indicating that EDA results from impaired NF-κB signaling. Finally, we show that abnormal immunity in OL-EDA-ID patients results from impaired cell responses to lipopolysaccharide, interleukin (IL)-1β, IL-18, TNFα and CD154. We thus report for the first time that impaired but not abolished NF-κB signaling in humans results in two related syndromes that associate specific developmental and immunological defects.
748 citations
Authors
Showing all 165661 results
| Name | H-index | Papers | Citations |
|---|---|---|---|
| Walter C. Willett | 334 | 2399 | 413322 |
| Frederick E. Shelton | 327 | 1485 | 295883 |
| Robert Langer | 281 | 2324 | 326306 |
| Graham A. Colditz | 261 | 1542 | 256034 |
| Frank B. Hu | 250 | 1675 | 253464 |
| George M. Whitesides | 240 | 1739 | 269833 |
| Eugene Braunwald | 230 | 1711 | 264576 |
| Ralph B. D'Agostino | 226 | 1287 | 229636 |
| Mark J. Daly | 204 | 763 | 304452 |
| Eric B. Rimm | 196 | 988 | 147119 |
| Virginia M.-Y. Lee | 194 | 993 | 148820 |
| Bernard Rosner | 190 | 1162 | 147661 |
| Stuart H. Orkin | 186 | 715 | 112182 |
| Mark Hallett | 186 | 1170 | 123741 |
| Ralph Weissleder | 184 | 1160 | 142508 |