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TL;DR: It is hypothesized that osteopenia develops early in life, during the natural course of type 1 Gaucher disease (GD1), and that its response to treatment is maximal during this period, with Imiglucerase results in amelioration of oste Openia in all age groups, with the greatest improvements in younger patients.
Abstract: Background
In Gaucher disease (GD), acid-β-glucosidase (GBA1) gene mutations result in defective glucocerebrosidase and variable combinations of hematological, visceral, and diverse bone disease. Osteopenia is highly prevalent, but its age of onset during the natural course of GD is not known. It is also unclear if the degree of improvement in osteopenia, secondary to imiglucerase enzyme therapy, differs by the age of the patient.
102 citations
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TL;DR: In this paper, a cross-sectional, web-based survey of children and adolescents was conducted to estimate the real-world global prevalence of atopic dermatitis in the pediatric population and by disease severity.
Abstract: Background Little is known on the current global prevalence of atopic dermatitis (AD) in the pediatric population. Objective To estimate the real-world global prevalence of AD in the pediatric population and by disease severity. Methods This international, cross-sectional, web-based survey of children and adolescents (6 months to Results Among 65,661 responders, the 12-month diagnosed AD prevalence (ISAAC plus self-reported diagnosis) ranged from 2.7% to 20.1% across countries; reported AD (ISAAC only) was 13.5% to 41.9%. Severe AD evaluated with both PtGA and POEM was generally less than 15%; more subjects rated AD as mild on PtGA than suggested by POEM. No trends in prevalence were observed based on age or sex; prevalence was generally lower in rural residential settings than urban or suburban. Conclusion This global survey in 18 countries revealed that AD affects a substantial proportion of the pediatric population. Although prevalence and severity varied across age groups and countries, less than 15% had severe AD.
102 citations
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TL;DR: Anti-TGF-β Ab therapy reduces blood pressure, proteinuria, and the renal injury associated with hypertension and renal medullary fibrosis in this group of rats.
Abstract: This study examined the role of transforming growth factor-β (TGF-β) in the development of hypertension and renal disease in 9-wk-old male Dahl salt-sensitive (Dahl S) rats fed an 8% NaCl diet for 3 wk. The rats received an intraperitoneal injection of a control or an anti-TGF-β antibody (anti-TGF-β Ab) every other day for 2 wk. Mean arterial pressure was significantly lower in Dahl S rats treated with anti-TGF-β Ab (177 ± 3 mmHg, n = 12) than in control rats (190 ± 4 mmHg, n = 17). Anti-TGF-β Ab therapy also reduced proteinuria from 226 ± 20 to 154 ± 16 mg/day. Renal blood flow, cortical blood flow, and creatinine clearance were not significantly different in control and treated rats; however, medullary blood flow was threefold higher in the treated rats than in the controls. Despite the reduction in proteinuria, the degree of glomerulosclerosis and renal hypertrophy was similar in control and anti-TGF-β Ab-treated rats. Renal levels of TGF-β1 and -β2, α-actin, type III collagen, and fibronectin mRNA decreased in rats treated with anti-TGF-β Ab. To examine whether an earlier intervention with anti-TGF-β Ab would confer additional renoprotection, these studies were repeated in a group of 6-wk-old Dahl S rats. Anti-TGF-β Ab therapy significantly reduced blood pressure, proteinuria, and the degree of glomerulosclerosis and renal medullary fibrosis in this group of rats. The results indicate that anti-TGF-β Ab therapy reduces blood pressure, proteinuria, and the renal injury associated with hypertension.
102 citations
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TL;DR: In a mouse model of Pompe disease, glycogen was more efficiently removed from the heart than from skeletal muscle for all enzymes, and overall, the CHO cell-derived rhGAA reduced glycogen to a greater extent than that observed with the other enzymes.
102 citations
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TL;DR: An analysis of spt23Δand mga2Δ strains shows that Spt23p and Mga2p differentially activate and regulate OLE1 transcription, suggesting that the two proteins may interact to modulate OLE 1 gene expression.
102 citations
Authors
Showing all 3085 results
Name | H-index | Papers | Citations |
---|---|---|---|
George M. Whitesides | 240 | 1739 | 269833 |
Stephen J. O'Brien | 153 | 1062 | 93025 |
Robert B. Jackson | 132 | 458 | 91332 |
Glenn M. Chertow | 128 | 764 | 82401 |
Jon Clardy | 116 | 983 | 56617 |
John J. Fung | 115 | 1011 | 52924 |
Robert B. Colvin | 111 | 556 | 52034 |
Sergio Giralt | 109 | 1024 | 48513 |
Paul Saftig | 107 | 356 | 49929 |
Robert J. Desnick | 102 | 694 | 39698 |
Robert A. Soslow | 87 | 427 | 29014 |
Richard J. Roman | 84 | 461 | 23760 |
Diana W. Bianchi | 81 | 405 | 24554 |
Paolo Raggi | 80 | 439 | 33332 |
Helmut G. Rennke | 77 | 256 | 33959 |