Renal disease in nail-patella syndrome: Clinical and morphologic studies

Renal disease in nail-patella syndrome: Clinical and morphological studies. Clinical and morphological features of seven patients with the nail-patella syndrome are described. Progression to renal failure after a prolonged period of asymptomatic proteinuria is reported. Kidney tissue from these seven patients studied by light, immunofluorescent and electron microscopy demonstrated abnormalities characteristic of this disease. Focal glomerular basement membrane thickening was observed by light microscopy. Immunofluorescent microscopy showed focal glomerular basement membrane and arteriolar staining with serum proteins, predominantly IgM and β1C. Electron microscopy revealed markedly abnormal glomerular basement membranes containing bundles of cross-striated fibrils. These fibrils were more readily demonstrated in phosphotungstic acid-stained sections. The data presented suggest that the inborn error of connective tissue metabolism of the nail-patella syndrome is associated with renal disease as the result of deposition of collagen moieties in glomerular basement membranes with subsequent alterations of glomerular structure and function

Immunologic aspects of the nephrotic syndrome

The nephrotic syndrome is a clinical entity characterized by proteinuria, hypoalbuminemia, edema and hyperlipidemia. All the features of this syndrome are ultimately related to increased permeability of the glomerular capillary to protein. A specific disease entity in its mildest form may result in mild proteinuria insufficient to cause hypoalbuminemia and the other physiological manifestations of the nephrotic syndrome; the same disease in another patient or at another time in the same patient may cause marked proteinuria and the nephrotic state. The principal difference between proteinuria alone and that associated with the nephrotic syndrome in any specific disease would therefore appear to be quantitative, although it is likely that other factors play a role

Anterior cruciate ligament reconstruction with bone-patellar tendon-bone autograft versus allograft in young patients

Objectives: Traditionally, bone-patella tendon-bone (BTB) autograft has been the gold standard graft choice for younger, athletic patients requiring ACL reconstruction. However, donor site morbidity, post-operative patella fracture, and increased operative time have led many surgeons to choose BTB allograft for their reconstructions. Opponents of allografts feel that slower healing time, higher rate of graft failure, and potential for disease transmission makes them undesirable graft choices in athletic patients. The purpose of this study is to evaluate the clinical outcomes, both subjective and objective, of young patients that who have undergone either BTB autograft or allograft reconstructions with a minimum of 2-year follow-up. Methods: One hundred and twenty patients (60 autograft, 60 allograft), age 25 and below at time of surgery, were contacted after being retrospectively identified as patients having an ACL reconstruction with either a BTB allograft or autograft by one senior surgeon. Patients were administered the Lysholm Knee Scoring Scale and IKDC Subjective Knee Evaluation questionnaires. Fifty (25 BTB autograft and 25 BTB allograft) of the 120 returned for physical examination as well as completion of a single leg hop test and laxity evaluation using a KT-1000 arthrometer evaluation. Of the 120 patients contacted, there were a total of 7 failures (5.8%) requiring revision, 6 in the allograft group (86%) and 1 in the autograft group (14%). Results: The average Lysholm scores were 89.0 and 89.56 and the average IKDC scores were 90.8 and 92.1 in the autograft and allograft groups respectively. The differences in the Lysholm scores and the IKDC scores were not significant. The single leg hop and KT-1000 scores were also not significantly different. One autograft patient had a minor motion deficit. Three allograft patients had a grade 1 Lachman and pivot glide. One autograft patient and two allograft patients had mild patellafemoral crepitus. There was no significant difference in anterior knee pain between the two groups Conclusion: There is no significant difference in patient-rated outcome between ACL reconstructions using BTB autografts versus allografts. However, the overall study group did reveal an increased failure rate requiring revision in the allograft group. © The Author(s) 2015

Mechanically induced silyl ester cleavage under acidic conditions investigated by AFM-based single-molecule force spectroscopy in the force-ramp mode

AFM-based dynamic single-molecule force spectroscopy was used to stretch carboxymethylated amylose (CMA) polymers, which have been covalently tethered between a silanized glass substrate and a silanized AFM tip via acid-catalyzed ester condensation at pH 2.0. Rupture forces were measured as a function of temperature and force loading rate in the force-ramp mode. The data exhibit significant statistical scattering, which is fitted with a maximum likelihood estimation (MLE) algorithm. Bond rupture is described with a Morse potential based Arrhenius kinetics model. The fit yields a bond dissociation energy De = 35 kJ mol−1 and an Arrhenius pre-factor A = 6.6 × 104 s−1. The bond dissociation energy is consistent with previous experiments under identical conditions, where the force-clamp mode was employed. However, the bi-exponential decay kinetics, which the force-clamp results unambiguously revealed, are not evident in the force-ramp data. While it is possible to fit the force-ramp data with a bi-exponential model, the fit parameters differ from the force-clamp experiments. Overall, single-molecule force spectroscopy in the force-ramp mode yields data whose information content is more limited than force-clamp data. It may, however, still be necessary and advantageous to perform force-ramp experiments. The number of successful events is often higher in the force-ramp mode, and competing reaction pathways may make force-clamp experiments impossible

Preliminary Results on HAT-P-4, TrES-3, XO-2, and GJ 436 from the NASA EPOXI Mission

EPOXI (EPOCh + DIXI) is a NASA Discovery Program Mission of Opportunity using the Deep Impact flyby spacecraft. The EPOCh (Extrasolar Planet Observation and Characterization) Science Investigation will gather photometric time series of known transiting exoplanet systems from January through August 2008. Here we describe the steps in the photometric extraction of the time series and present preliminary results of the first four EPOCh targets.Comment: 4 pages, 2 figures. To appear in the Proceedings of the 253rd IAU Symposium: "Transiting Planets", May 2008, Cambridge, M

Covariant equations for the three-body bound state

The covariant spectator (or Gross) equations for the bound state of three identical spin 1/2 particles, in which two of the three interacting particles are always on shell, are developed and reduced to a form suitable for numerical solution. The equations are first written in operator form and compared to the Bethe-Salpeter equation, then expanded into plane wave momentum states, and finally expanded into partial waves using the three-body helicity formalism first introduced by Wick. In order to solve the equations, the two-body scattering amplitudes must be boosted from the overall three-body rest frame to their individual two-body rest frames, and all effects which arise from these boosts, including the Wigner rotations and rho-spin decomposition of the off-shell particle, are treated exactly. In their final form, the equations reduce to a coupled set of Faddeev-like double integral equations with additional channels arising from the negative rho-spin states of the off-shell particle.Comment: 57 pages, RevTeX, 6 figures, uses epsf.st

MFI Type Zeolite Aggregates with Nanosized Particles via a Combination of Spray Drying and Steam-Assisted Crystallization (SAC) Techniques

This article belongs to the Special Issue Catalysis on Zeolites and Zeolite-Like Materials II