The 6-minute walk test and other endpoints in Duchenne muscular dystrophy: longitudinal natural history observations over 48 weeks from a multicenter study.

IntroductionDuchenne muscular dystrophy (DMD) subjects ≥5 years with nonsense mutations were followed for 48 weeks in a multicenter, randomized, double-blind, placebo-controlled trial of ataluren. Placebo arm data (N = 57) provided insight into the natural history of the 6-minute walk test (6MWT) and other endpoints.MethodsEvaluations performed every 6 weeks included the 6-minute walk distance (6MWD), timed function tests (TFTs), and quantitative strength using hand-held myometry.ResultsBaseline age (≥7 years), 6MWD, and selected TFT performance are strong predictors of decline in ambulation (Δ6MWD) and time to 10% worsening in 6MWD. A baseline 6MWD of <350 meters was associated with greater functional decline, and loss of ambulation was only seen in those with baseline 6MWD <325 meters. Only 1 of 42 (2.3%) subjects able to stand from supine lost ambulation.ConclusionFindings confirm the clinical meaningfulness of the 6MWD as the most accepted primary clinical endpoint in ambulatory DMD trials

ОЦІНКА ЯКОСТІ НАДАННЯ МЕДИЧНИХ ПОСЛУГ СЕРЕДНІМ МЕДИЧНИМ ПЕРСОНАЛОМ В УМОВАХ ПРИВАТНОГО МЕДИЧНОГО ЦЕНТРУ

The article analyzes the literature and classifies the quality of the nursing staff, according to which the analysis of the quality of the nurses of Private Medical Ophthalmologic Center «Professor Zahurskyi Eye Surgery Center» for 2015 and suggests the ways to improve the optimization of the medical staff to achieve better treatment outcomes and satisfaction of patients.У статті проведено аналіз літератури з даної проблеми та класифіковано показники якості роботи середнього медичного персоналу. Проведено аналіз якості роботи медичних сестер приватного медичного офтальмологічного центру ТОВ «Центр хірургії ока професора Загурського» за 2015 р. та запропоновано шляхи вдосконалення та оптимізації роботи медичного персоналу для досягнення кращих результатів лікування та задоволеності пацієнтів.

Data Collection for Current U.S. Wind Energy Projects: Component Costs, Financing, Operations, and Maintenance; January 2011 - September 2011

DNV Renewables (USA) Inc. (DNV) used an Operations and Maintenance (O&M) Cost Model to evaluate ten distinct cost scenarios encountered under variations in wind turbine component failure rates. The analysis considers: (1) a Reference Scenario using the default part failure rates within the O&M Cost Model, (2) High Failure Rate Scenarios that increase the failure rates of three major components (blades, gearboxes, and generators) individually, (3) 100% Replacement Scenarios that model full replacement of these components over a 20 year operating life, and (4) Serial Failure Scenarios that model full replacement of blades, gearboxes, and generators in years 4 to 6 of the wind project. DNV selected these scenarios to represent a broad range of possible operational experiences. Also in this report, DNV summarizes the predominant financing arrangements used to develop wind energy projects over the past several years and provides summary data on various financial metrics describing those arrangements

Compact Broadband Antenna with Vicsek Fractal Slots for WLAN and WiMAX Applications

This paper aims to design a compact broadband antenna for wireless local area network (WLAN) and worldwide interoperability for microwave access (WIMAX) applications. The suggested antenna consists of an octagonal radiator with Vicsek fractal slots and a partial ground plane, it is printed on FR-4 dielectric substrate, and its global dimension is 50 x 50 x 1.6 mm(3). The antenna is designed and constructed using both CST MICROWAVE STUDIO(R) and CADFEKO electromagnetic solver, and in order to validate the acquired simulation results, the antenna is manufactured and tested using vector network analyzer E5071C. The measurement results show that the designed antenna attains a broadband bandwidth (S-11 < -10 dB) from 2.48 to 6.7 GHz resonating at 3.6 and 5.3 GHz, respectively. The broadband bandwidth covers the two required bands: WiMAX at the frequencies 2.3/2.5/3.3/3.5/5/5.5 GHz and WLAN at the frequencies 3.6/2.4-2.5/4.9-5.9 GHz. In addition, the suggested antenna provides good gains of 2.78 dBi and 5.32 dBi, omnidirectional measured radiation patterns in the E-plane and the H-plane and high efficiencies of 88.5% and 84.6% at the resonant frequencies. A close agreement of about 90% between simulation and measurement results is noticed

Kinetics of error generation in homologous B-family DNA polymerases

The kinetics of forming a proper Watson–Crick base pair as well incorporating bases opposite furan, an abasic site analog, have been well characterized for the B Family replicative DNA polymerase from bacteriophage T4. Structural studies of these reactions, however, have only been performed with the homologous enzyme from bacteriophage RB69. In this work, the homologous enzymes from RB69 and T4 were compared in parallel reactions to determine the relative abilities of the two polymerases to incorporate correct nucleotides as well as to form improper pairings. The kinetic rates for three different exonuclease mutants for each enzyme were measured for incorporation of an A opposite T and an A opposite furan as well as for the formation of A:C and T:T mismatches. The T4 exonuclease mutants were all ∼2- to 7-fold more efficient than the corresponding RB69 exonuclease mutants depending on whether a T or furan was in the templating position and which exonuclease mutant was used. The rates for mismatch formation by T4 were significantly reduced compared with incorporation opposite furan, much more so than the corresponding RB69 mutant. These results show that there are kinetic differences between the two enzymes but they are not large enough to preclude structural assumptions for T4 DNA polymerase based on the known structure of the RB69 DNA polymerase

УЧАСТЬ МЕДИЧНОЇ СЕСТРИ У ПРОВЕДЕННІ ДОСЛІДЖЕННЯ ФУНКЦІЇ ЗОВНІШНЬОГО ДИХАННЯ

The article describes the role of the nurse in the investigation of respiratory function, the methodology of spirography performing, and main spirohraphy indexes are presented. Basic requirements for nurse from functional diagnostics unit are highlighted.У статті висвітлено участь медичної сестри в дослідженні функції зовнішнього дихання, методику виконання спірографії, наведено основні спірографічні показники. Окремо висвітлено основні вимоги до роботи медичної сестри функціональної діагностики

УЧАСТЬ МЕДИЧНОЇ СЕСТРИ У ПРОВЕДЕННІ ДОСЛІДЖЕННЯ ФУНКЦІЇ ЗОВНІШНЬОГО ДИХАННЯ

The article describes the role of the nurse in the investigation of respiratory function, the methodology of spirography performing, and main spirohraphy indexes are presented. Basic requirements for nurse from functional diagnostics unit are highlighted.У статті висвітлено участь медичної сестри в дослідженні функції зовнішнього дихання, методику виконання спірографії, наведено основні спірографічні показники. Окремо висвітлено основні вимоги до роботи медичної сестри функціональної діагностики

Sheldon-Hall syndrome

Sheldon-Hall syndrome (SHS) is a rare multiple congenital contracture syndrome characterized by contractures of the distal joints of the limbs, triangular face, downslanting palpebral fissures, small mouth, and high arched palate. Epidemiological data for the prevalence of SHS are not available, but less than 100 cases have been reported in the literature. Other common clinical features of SHS include prominent nasolabial folds, high arched palate, attached earlobes, mild cervical webbing, short stature, severe camptodactyly, ulnar deviation, and vertical talus and/or talipes equinovarus. Typically, the contractures are most severe at birth and non-progressive. SHS is inherited in an autosomal dominant pattern but about half the cases are sporadic. Mutations in either MYH3, TNNI2, or TNNT3 have been found in about 50% of cases. These genes encode proteins of the contractile apparatus of fast twitch skeletal muscle fibers. The diagnosis of SHS is based on clinical criteria. Mutation analysis is useful to distinguish SHS from arthrogryposis syndromes with similar features (e.g. distal arthrogryposis 1 and Freeman-Sheldon syndrome). Prenatal diagnosis by ultrasonography is feasible at 18–24 weeks of gestation. If the family history is positive and the mutation is known in the family, prenatal molecular genetic diagnosis is possible. There is no specific therapy for SHS. However, patients benefit from early intervention with occupational and physical therapy, serial casting, and/or surgery. Life expectancy and cognitive abilities are normal

ЧЕМПІОНАТИ БРИГАД ШВИДКОЇ МЕДИЧНОЇ ДОПОМОГИ ЯК СКЛАДОВА ЄДИНОЇ СИСТЕМИ НАДАННЯ ЕКСТРЕНОЇ МЕДИЧНОЇ ДОПОМОГИ В УКРАЇНІ: ДОСЯГНЕННЯ І ПЕРСПЕКТИВИ

The national ambulance championships play an important role in the improving of the level of theoretical knowledge and practical skills, problems' analysis and recognizing the directions of their solving, initiation of international cooperation.Національні чемпіонати швидкої медичної допомоги мають вагоме значення для вдосконалення рівня теоретичних знань і практичних навичок, аналізу проблем служби, визначення напрямків їх вирішення, започаткування міжнародного співробітництва

Categorizing natural history trajectories of ambulatory function measured by the 6-minute walk distance in patients with Duchenne muscular dystrophy

High variability in patients' changes in 6 minute walk distance (6MWD) over time has complicated clinical trials of treatment efficacy in Duchenne muscular dystrophy (DMD). We assessed whether boys with DMD could be grouped into classes that shared similar ambulatory function trajectories as measured by 6MWD. Ambulatory boys aged 5 years or older with genetically confirmed DMD who were enrolled in a natural history study at 11 care centers throughout Italy were included. For each boy, standardized assessments of 6MWD were available at annual intervals spanning 3 years. Trajectories of 6MWD vs. age and trajectories of 6MWD vs. time from enrollment were examined using latent class analysis. A total of 96 boys were included. At enrollment, the mean age was 8.3 years (mean 6MWD: 374 meters). After accounting for age, baseline 6MWD, and steroid use, four latent trajectory classes were identified as explaining 3-year 6MWD outcomes significantly better than a single average trajectory. Patient trajectories of 6MWD change from enrollment were categorized as having fast decline (n\ue2\u80\u89=\ue2\u80\u8925), moderate decline (n\ue2\u80\u89=\ue2\u80\u8919), stable function (n\ue2\u80\u89=\ue2\u80\u8937), and improving function (n\ue2\u80\u89=\ue2\u80\u8915) during the 3-year follow-up. After accounting for trajectory classes, the standard deviation of variation in 6MWD was reduced by approximately 40%. The natural history of ambulatory function in DMD may be composed of distinct trajectory classes. The extent to which trajectories are associated with novel and established prognostic factors warrants further study. Reducing unexplained variation in patient outcomes could help to further improve DMD clinical trial design and analysis