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101.
Beighley Adam Glynn Ryan Scullen Tyler Mathkour Mansour Werner Cassidy Berry John F. Carr Christopher Abou-Al-Shaar Hussam Aysenne Aimee Nerva John D. Dumont Aaron S. 《Neurosurgical review》2021,44(5):2511-2522
Neurosurgical Review - Aneurysmal subarachnoid hemorrhage (aSAH) is an emergent condition requiring rapid intervention and prolonged monitoring. There are few recommendations regarding the... 相似文献
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Carlos R Ferreira Dillon Kavanagh Ralf Oheim Kristin Zimmerman Julian Stürznickel Xiaofeng Li Paul Stabach R Luke Rettig Logan Calderone Colin MacKichan Aaron Wang Hunter A Hutchinson Tracy Nelson Steven M Tommasini Simon von Kroge Imke AK Fiedler Ethan R Lester Gilbert W Moeckel Björn Busse Thorsten Schinke Thomas O Carpenter Michael A Levine Mark C Horowitz Demetrios T Braddock 《Journal of bone and mineral research》2021,36(5):942-955
Inactivating mutations in human ecto-nucleotide pyrophosphatase/phosphodiesterase-1 (ENPP1) may result in early-onset osteoporosis (EOOP) in haploinsufficiency and autosomal recessive hypophosphatemic rickets (ARHR2) in homozygous deficiency. ARHR2 patients are frequently treated with phosphate supplementation to ameliorate the rachitic phenotype, but elevating plasma phosphorus concentrations in ARHR2 patients may increase the risk of ectopic calcification without increasing bone mass. To assess the risks and efficacy of conventional ARHR2 therapy, we performed comprehensive evaluations of ARHR2 patients at two academic medical centers and compared their skeletal and renal phenotypes with ENPP1-deficient Enpp1asj/asj mice on an acceleration diet containing high phosphate treated with recombinant murine Enpp1-Fc. ARHR2 patients treated with conventional therapy demonstrated improvements in rickets, but all adults and one adolescent analyzed continued to exhibit low bone mineral density (BMD). In addition, conventional therapy was associated with the development of medullary nephrocalcinosis in half of the treated patients. Similar to Enpp1asj/asj mice on normal chow and to patients with mono- and biallelic ENPP1 mutations, 5-week-old Enpp1asj/asj mice on the high-phosphate diet exhibited lower trabecular bone mass, reduced cortical bone mass, and greater bone fragility. Treating the Enpp1asj/asj mice with recombinant Enpp1-Fc protein between weeks 2 and 5 normalized trabecular bone mass, normalized or improved bone biomechanical properties, and prevented the development of nephrocalcinosis and renal failure. The data suggest that conventional ARHR2 therapy does not address low BMD inherent in ENPP1 deficiency, and that ENPP1 enzyme replacement may be effective for correcting low bone mass in ARHR2 patients without increasing the risk of nephrocalcinosis. © 2021 American Society for Bone and Mineral Research (ASBMR). 相似文献
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Albright Christopher A. Kimball Devon Brady John P. Luna Issabella Brown Tiffany Calzo Jerel P. Pennesi Jamie-Lee Blashill Aaron J. 《Archives of sexual behavior》2022,51(5):2523-2533
Archives of Sexual Behavior - Sexual minority men (SMM) remain disproportionately burdened by sexually transmitted infections. Although gay community involvement has been theorized to be protective... 相似文献
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Modified Allen pictures to assess amblyopia in young children 总被引:1,自引:0,他引:1
To produce a test of visual acuity for young children that is more sensitive to amblyopia than current preschool vision tests, the authors surrounded four Allen pictures with "crowding" bars. This modified Allen test was evaluated by measuring acuity of amblyopic children (n = 28) and children (n = 10) and adults (n = 5) with normal eyes. Mean acuities of amblyopic eyes for the modified pictures was 0.8 octaves or nearly three logMar Snellen lines poorer than for the isolated pictures. For nonamblyopic and normal eyes modified picture acuity averaged 0.1 to 0.4 octaves poorer than isolated picture acuity. Average acuities of amblyopic eyes (n = 22) for the modified pictures agreed with line letter acuities. These results suggest that the modified picture test induces contour interaction similar to that of line letter tests, and thus, is a more sensitive test of amblyopia in the preschool child than isolated symbols. 相似文献