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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Vanderbilt University1, Royal Prince Alfred Hospital2, Medical University of Vienna3, Loyola University Chicago4, Mayo Clinic5, Pennsylvania State University6, University of Gothenburg7, University of California, San Francisco8, NewYork–Presbyterian Hospital9, University of Florida10, Indiana University – Purdue University Indianapolis11, Hannover Medical School12, Boston Children's Hospital13, Cleveland Clinic14, University of Kentucky15, University of Oregon16, University of California, Los Angeles17, Duke University18
TL;DR: Patients with CCTGA are increasingly subject to CHF with advancing age; this complication is extremely common by the fourth and fifth decades.
567 citations
University of Tartu1, Haukeland University Hospital2, Boston Children's Hospital3, University of Helsinki4, University of Cagliari5, University of Pisa6, University of Medicine and Dentistry of New Jersey7, University of Regensburg8, Heidelberg University9, University of Oxford10, National Institute for Biological Standards and Control11
TL;DR: It is concluded that IL-22 and IL-17F are key natural defenders against CMC and that the immunodeficiency underlying CMC in both patient groups has an autoimmune basis.
Abstract: Chronic mucocutaneous candidiasis (CMC) is frequently associated with T cell immunodeficiencies. Specifically, the proinflammatory IL-17A–producing Th17 subset is implicated in protection against fungi at epithelial surfaces. In autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED, or autoimmune polyendocrine syndrome 1), CMC is often the first sign, but the underlying immunodeficiency is a long-standing puzzle. In contrast, the subsequent endocrine features are clearly autoimmune, resulting from defects in thymic self-tolerance induction caused by mutations in the autoimmune regulator (AIRE). We report severely reduced IL-17F and IL-22 responses to both Candida albicans antigens and polyclonal stimulation in APECED patients with CMC. Surprisingly, these reductions are strongly associated with neutralizing autoantibodies to IL-17F and IL-22, whereas responses were normal and autoantibodies infrequent in APECED patients without CMC. Our multicenter survey revealed neutralizing autoantibodies against IL-17A (41%), IL-17F (75%), and/ or IL-22 (91%) in >150 APECED patients, especially those with CMC. We independently found autoantibodies against these Th17-produced cytokines in rare thymoma patients with CMC. The autoantibodies preceded the CMC in all informative cases. We conclude that IL-22 and IL-17F are key natural defenders against CMC and that the immunodeficiency underlying CMC in both patient groups has an autoimmune basis.
567 citations
TL;DR: A review of triggerable materials that range in scale from nano to macro, and are activated by a range of stimuli, that may enhance therapeutic effectiveness and reduce systemic toxicity.
Abstract: Triggerable drug delivery systems enable on-demand controlled release profiles that may enhance therapeutic effectiveness and reduce systemic toxicity. Recently, a number of new materials have been developed that exhibit sensitivity to visible light, near-infrared (NIR) light, ultrasound, or magnetic fields. This responsiveness can be triggered remotely to provide flexible control of dose magnitude and timing. Here we review triggerable materials that range in scale from nano to macro, and are activated by a range of stimuli.
567 citations
University of Toronto1, Tulane University2, Ohio State University3, Hannover Medical School4, University of Texas Southwestern Medical Center5, Washington University in St. Louis6, University of California, San Francisco7, Laval University8, Boston Children's Hospital9, University of Hamburg10, University of Pennsylvania11, Sheba Medical Center12, University of Padua13, University of Pittsburgh14, Oregon Health & Science University15, Medical College of Wisconsin16, University of New South Wales17, Medical University of South Carolina18, University of Manitoba19, Tel Aviv University20, Tel Aviv Sourasky Medical Center21, Saint George Hospital22, University of Wisconsin-Madison23
TL;DR: An extensive assessment of mutation burden through sequencing analysis of >81,000 tumors from pediatric and adult patients, including tumors with hypermutation caused by chemotherapy, carcinogens, or germline alterations, uncovered new driver mutations in the replication-repair-associated DNA polymerases and a distinct impact of microsatellite instability and replication repair deficiency on the scale of mutation load.
567 citations
University of California, San Francisco1, Washington University in St. Louis2, Lund University3, University Health Network4, Princess Alexandra Hospital5, Royal Melbourne Hospital6, University of Chicago7, Deutsche Sammlung von Mikroorganismen und Zellkulturen8, University of Florida9, University of Alabama at Birmingham10, Ohio State University11, Boston Children's Hospital12, Johns Hopkins University13, Nationwide Children's Hospital14, University of Colorado Denver15
TL;DR: Both near-haploid and low-hypodiploid leukemic cells show activation of Ras-signaling and phosphoinositide 3-kinase (PI3K)-signaling pathways and are sensitive to PI3K inhibitors, indicating that these drugs should be explored as a new therapeutic strategy for this aggressive form of leukemia.
Abstract: The genetic basis of hypodiploid acute lymphoblastic leukemia (ALL), a subtype of ALL characterized by aneuploidy and poor outcome, is unknown. Genomic profiling of 124 hypodiploid ALL cases, including whole-genome and exome sequencing of 40 cases, identified two subtypes that differ in the severity of aneuploidy, transcriptional profiles and submicroscopic genetic alterations. Near-haploid ALL with 24-31 chromosomes harbor alterations targeting receptor tyrosine kinase signaling and Ras signaling (71%) and the lymphoid transcription factor gene IKZF3 (encoding AIOLOS; 13%). In contrast, low-hypodiploid ALL with 32-39 chromosomes are characterized by alterations in TP53 (91.2%) that are commonly present in nontumor cells, IKZF2 (encoding HELIOS; 53%) and RB1 (41%). Both near-haploid and low-hypodiploid leukemic cells show activation of Ras-signaling and phosphoinositide 3-kinase (PI3K)-signaling pathways and are sensitive to PI3K inhibitors, indicating that these drugs should be explored as a new therapeutic strategy for this aggressive form of leukemia.
567 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 |