Institution
Dupont Hospital
Healthcare•Fort Wayne, Indiana, United States•
About: Dupont Hospital is a healthcare organization based out in Fort Wayne, Indiana, United States. It is known for research contribution in the topics: Population & Health care. The organization has 497 authors who have published 474 publications receiving 12495 citations.
Papers published on a yearly basis
Papers
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TL;DR: A model whereby lesion-induced upregulation of axonal importin beta may enable retrograde transport of signals that modulate the regeneration of injured neurons is suggested.
470 citations
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TL;DR: It is concluded that cuffed endotracheal tubes may be used routinely during controlled ventilation in full‐term newborns and children during anesthesia.
Abstract: BackgroundUncuffed endotracheal tubes are routinely used in young children. This study tests a formula for selecting appropriately sized cuffed endotracheal tubes and compares the use of cuffed versus uncuffed endotracheal tubes for patients whose lungs are mechanically ventilated during anesthesia.
437 citations
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TL;DR: Genotype-phenotype studies identified, as carriers of cysteine mutations, 13 of 14 patients with TRAPS and amyloidosis and indicated a lower penetrance of TRAPS symptoms in individuals with noncysteine mutation, suggesting further genetic heterogeneity of the periodic-fever syndromes.
Abstract: Mutations in the extracellular domain of the 55-kD tumor-necrosis factor (TNF) receptor (TNFRSF1A), a key regulator of inflammation, define a periodic-fever syndrome, TRAPS (TNF receptor–associated periodic syndrome [MIM 142680]), which is characterized by attacks of fever, sterile peritonitis, arthralgia, myalgia, skin rash, and/or conjunctivitis; some patients also develop systemic amyloidosis. Elsewhere we have described six disease-associated TNFRSF1A mutations, five of which disrupt extracellular cysteines involved in disulfide bonds; four other mutations have subsequently been reported. Among 150 additional patients with unexplained periodic fevers, we have identified four novel TNFRSF1A mutations (H22Y, C33G, S86P, and c.193−14 G→A), one mutation (C30S) described by another group, and two substitutions (P46L and R92Q) present in ∼1% of control chromosomes. The increased frequency of P46L and R92Q among patients with periodic fever, as well as functional studies of TNFRSF1A, argue that these are low-penetrance mutations rather than benign polymorphisms. The c.193−14 G→A mutation creates a splice-acceptor site upstream of exon 3, resulting in a transcript encoding four additional extracellular amino acids. T50M and c.193−14 G→A occur at CpG hotspots, and haplotype analysis is consistent with recurrent mutations at these sites. In contrast, although R92Q also arises at a CpG motif, we identified a common founder chromosome in unrelated individuals with this substitution. Genotype-phenotype studies identified, as carriers of cysteine mutations, 13 of 14 patients with TRAPS and amyloidosis and indicated a lower penetrance of TRAPS symptoms in individuals with noncysteine mutations. In two families with dominantly inherited disease and in 90 sporadic cases that presented with a compatible clinical history, we have not identified any TNFRSF1A mutation, despite comprehensive genomic sequencing of all of the exons, therefore suggesting further genetic heterogeneity of the periodic-fever syndromes.
326 citations
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TL;DR: Improvements obtained in CCG trials during two successive series of studies (1983–1988 and 1989–1995) are reported, which form the basis for current ALL trials.
Abstract: Since 1968, the Children's Cancer Group (CCG) has treated more than 16,000 children with acute lymphoblastic leukemia (ALL). Herein, we report improvements obtained in CCG trials during two successive series of studies (1983-1988 and 1989-1995). Overall, 10-year EFS was 62% +/- 10% for the 1983-1988 series and 72% +/- 1% for the 1988-1995 series (P< 0.0001). Five-year cumulative rates of isolated CNS relapses were 5.9% and 4.4%. Therapy based on the Berlin-Frankfurt-Munster 76/79 study improved outcomes for intermediate and higher risk patients in the first series. For intermediate risk patients, delayed intensification (DI) was most crucial for improved outcome and cranial irradiation was safely replaced with maintenance intrathecal methotrexate, providing patients received intensified systemic therapy. In the second series, randomized trials showed better outcome with one vs no DI phase for lower risk patients, with two vs one DI phase for intermediate risk patients, and with the CCG 'augmented regimen' for higher risk patients with a slow day 7 marrow response. Cranial irradiation was safely replaced with additional intrathecal methotrexate for higher risk patients with a rapid day 7 marrow response. In a subsequent study, substitution of dexamethasone in place of prednisone in induction and maintenance improved outcome for standard risk patients. All patients received dexamethasone in DI. These successful treatment strategies form the basis for our current ALL trials.
306 citations
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TL;DR: Test results and management data are summarized for 260 patients with diagnoses of Auditory Neuropathy Spectrum Disorder (ANSD), and all showed absent/grossly abnormal auditory brainstem responses (ABR), often ‘ringing’ cochlear microphonics, and the presence or history of otoacoustic emissions.
Abstract: Test results and management data are summarized for 260 patients with diagnoses of Auditory Neuropathy Spectrum Disorder (ANSD). Hearing aids were tried in 85 of these patients, and 49 patients tri...
264 citations
Authors
Showing all 501 results
Name | H-index | Papers | Citations |
---|---|---|---|
Vinay M. Nadkarni | 101 | 646 | 41307 |
Harry T. Chugani | 71 | 374 | 19699 |
Samuel S. Gidding | 68 | 279 | 32888 |
Judith L. Ross | 60 | 195 | 10667 |
Marcella Devoto | 57 | 217 | 14063 |
Kristi L. Kiick | 51 | 180 | 9111 |
Freeman Miller | 51 | 275 | 7335 |
Marc H. Gorelick | 49 | 123 | 7243 |
Robert L. Brent | 49 | 325 | 10672 |
Ricardo Gonzalez | 47 | 248 | 7295 |
Alan R. Spitzer | 46 | 190 | 6709 |
Karen W. Gripp | 44 | 150 | 6255 |
Michael E. Trigg | 44 | 106 | 8719 |
Jeffery L. Twiss | 43 | 108 | 6828 |
Saul Surrey | 43 | 163 | 7304 |