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Abstract Background Mutations in the genes coding for either dystrophin or dysferlin cause distinct forms of muscular dystrophy. Dystrophin links the cytoskeleton to the sarcolemma through direct interaction with β-dystroglycan. This link extends to the extracellular matrix by β-dystroglycan's interaction with α-dystroglycan, which binds extracellular matrix proteins, including laminin α2, agrin and perlecan, that possess laminin globular domains. The absence of dystrophin disrupts this link, leading to compromised muscle sarcolemmal integrity. Dysferlin, on the other hand, plays an important role in the Ca2+-dependent membrane repair of damaged sarcolemma in skeletal muscle. Because dysferlin and dystrophin play different roles in maintaining muscle cell integrity, we hypothesized that disrupting sarcolemmal integrity with dystrophin deficiency would exacerbate the pathology in dysferlin-null mice and allow further characterization of the role of dysferlin in skeletal muscle. Methods To test our hypothesis, we generated dystrophin/dysferlin double-knockout (DKO) mice by breeding mdx mice with dysferlin-null mice and analyzed the effects of a combined deficiency of dysferlin and dystrophin on muscle pathology and sarcolemmal integrity. Results The DKO mice exhibited more severe muscle pathology than either mdx mice or dysferlin-null mice, and, importantly, the onset of the muscle pathology occurred much earlier than it did in dysferlin-deficient mice. The DKO mice showed muscle pathology of various skeletal muscles, including the mandible muscles, as well as a greater number of regenerating muscle fibers, higher serum creatine kinase levels and elevated Evans blue dye uptake into skeletal muscles. Lengthening contractions caused similar force deficits, regardless of dysferlin expression. However, the rate of force recovery within 45 minutes following lengthening contractions was hampered in DKO muscles compared to mdx muscles or dysferlin-null muscles, suggesting that dysferlin is required for the initial recovery from lengthening contraction-induced muscle injury of the dystrophin-glycoprotein complex-compromised muscles. Conclusions The results of our study suggest that dysferlin-mediated membrane repair helps to limit the dystrophic changes in dystrophin-deficient skeletal muscle. Dystrophin deficiency unmasks the function of dysferlin in membrane repair during lengthening contractions. Dystrophin/dysferlin-deficient mice provide a very useful model with which to evaluate the effectiveness of therapies designed to treat dysferlin deficiency.

Objective: The ability of six curing lights to photocure four resin-based composites (RBCs) in a mold simulating a cavity was compared visually., Materials and Methods: Four RBCs were photocured using the: Woodpecker B for 2x10s, SmartLite Pro 2x10s, Valo Cordless 2x10s, Valo Cordless 2x3s Xtra power, Valo X 2x10s, Valo X 2x5s Xtra power, PowerCure 2x3s mode, Monet 1x1s and Monet 3x1s, in a mold representing a molar Class II restoration. Immediately after photocuring, the RBC specimens were immersed in a solvent to remove the uncured RBC, after which they were photographed and de-identified. Using a REDCap survey, these images were compared visually to compare the ability of the LCUs to photocure the restorations., Results: There were significant differences in how well the LCUs had photocured the RBCs. The SmartLite Pro and Valo X used for two 10s exposures produced restorations rated as the best cured, and the Monet used for 1 s was rated the worst., Conclusions: There were visually apparent differences in how well the LCUs could photocure the RBCs. The Monet used for 1 second produced the worst results for all four RBCs., (Copyright© 2024 Dennis Barber Ltd.)

Objective: Post-traumatic seizure (PTS) is a well-known complication of traumatic brain injury (TBI). The objective of this study was to identify risk factors associated with breakthrough early PTS in TBI patients receiving phenytoin prophylaxis., Methods: This was a single-centered retrospective study including adult patients admitted to the intensive care unit (ICU), had a TBI, and started on phenytoin for seizure prophylaxis within 24 h of admission. The primary outcome was the incidence and factors associated with early PTS, defined as a confirmed seizure on a continuous electroencephalogram within 7 days of TBI. Secondary outcomes included the association between early post-traumatic seizures and ICU length of stay, hospital length of stay, and in-hospital mortality., Results: A total of 105 patients were included in the final analysis. Patients with early PTS were older (65 vs. 48 years old, p  = 0.01), had a higher Marshall score (5 vs. 2, p  = 0.01), were more likely to have a Marshall score > 2 (73 vs. 37%, p  = 0.01), and had more neurosurgeries for hematoma evacuation (57 vs. 19%, p  = 0.01). In patients with early PTS, 57% had a level at the time of seizure, and of those, 87.5% had a therapeutic level (>10 mcg/mL). Patients with early PTS had a longer ICU length of stay (14.7 vs. 5.9 days, p  = 0.04) and a greater proportion of hospital mortality (21 vs. 2%, p  = 0.02)., Conclusion: Patients with higher age, Marshall score, and neurosurgical procedures for hematoma evacuation had higher incidences of breakthrough early PTS despite the use of phenytoin prophylaxis. The majority of patients with early PTS had therapeutic phenytoin levels at the time of seizure when a level was available; however, approximately half (43%) did not have a level., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Generoso, Diep, Hua, Rader, Ran, Lee and Rivera-Lara.)

Background: Necrotizing enterocolitis (NEC) is a devastating disease of intestinal inflammation that primarily affects premature infants. A potential risk factor for necrotizing enterocolitis is exposure of the premature neonatal intestine to environmental bacteria and their proinflammatory products such as lipopolysaccharide. The metalloenzyme alkaline phosphatase (ALP) has been shown to reduce lipopolysaccharide-mediated inflammation. Additionally, premature rat pups have reduced alkaline phosphatase activity and expression as compared to full term pups. To explore the possibility that the human premature neonatal intestine has a paucity of alkaline phosphatase activity, we measured endogenously produced intestinal alkaline phosphatase activity in meconium as a function of gestational age. To test whether breast milk could serve as a source of exogenous alkaline phosphatase to the neonatal intestine through ingestion, we measured alkaline phosphatase activity in breast milk across a range of time points post-birth., Methods: Alkaline phosphatase activity was quantified in 122 meconium samples from infants of gestational ages ranging from 24 to 40 weeks and in 289 breast milk samples collected from 78 individual mothers between days 2-49 post-birth., Results: We observed a strong positive correlation between the meconium alkaline phosphatase activity and gestational age, with preterm infants having lower meconium alkaline phosphatase activities than early term or term infants. Breast milk alkaline phosphatase activity was highest in the first week post-birth, with peak alkaline phosphatase activity at day 2 post-birth, followed by relatively low alkaline phosphatase activity in weeks 2-7., Conclusions: Our results are consistent with the two major risk factors for necrotizing enterocolitis development, preterm birth and lack of breast milk feeding, both contributing to a paucity of alkaline phosphatase activity and impaired capacity to detoxify proinflammatory bacterial products such as lipopolysaccharide.

The incidence of neurocognitive disorders, which may impair the ability of older adults to perform activities of daily living (ADLs), rises with age. Depressive symptoms are also common in older adults and may affect ADLs. Safe storage and utilization of firearms are complex ADLs, which require intact judgment, executive function, and visuospatial ability, and may be affected by cognitive impairment. Depression or cognitive impairment may cause paranoia, delusions, disinhibition, apathy, or aggression and thereby limit the ability to safely utilize firearms. These problems may be superimposed upon impaired mobility, arthritis, visual impairment, or poor balance. Inadequate attention to personal protection may also cause hearing impairment and accidents. In this article, we review the data on prevalence of firearms access among older adults; safety concerns due to age-related conditions; barriers to addressing this problem; indications prompting screening for firearms access; and resources available to patients, caregivers, and health care providers., (© The Author(s) 2014.)

Adults with intellectual and other developmental disabilities (IDD) are now living to late life. Whether geriatricians are being trained to provide care for this clinically complex subpopulation of elders has not been examined. Two thirds of all geriatric fellowship directors in the United States responded to a Web-based survey of curriculum and training in this area. Forty-four percent rated training in this area as "important" or "very important," but only 26% of directors rated their graduates as moderately or highly competent in caring for elders with IDD. We report our survey findings and implications for future curricular development.

Background: Traumatic injuries in older patients are complex and have significant impact on the individual, their family, and trauma centers. We hypothesized that greater attention to the timely identification and comprehensive management of comorbid conditions would improve outcomes in the care of the older injured patient., Methods: This was a prospective, descriptive study of all patients 65 years of age and older admitted to the trauma service. Each elder was seen by a specialist in geriatrics ideally within 24 hours of admission to the trauma service. A standardized consult was developed for the electronic medical record and utilized in every case. Data were obtained from the trauma registry to characterize the nature of injury, injury severity, and outcome. Data from the geriatric consult were compiled to determine the impact of age- associated conditions on the outcome., Results: There were 285 injured patients, aged 65 years and older. Of these, 114 were seen in consultation. Age range was 65 to 96 years, with an average of 77.7 years. Injury Severity Score range was 3 to 75, with an average of 9.3. There were an equal number of male and female patients. Falls and motor vehicle collisions predominated. Geriatricians identified the following: 14% of patients presented with alcohol issues; 36% of patients exhibited signs of delirium; and 46% of patients presented with new medical conditions. Geriatricians assisted with advanced care planning in 15% of cases; disposition decisions to promote function in 49%; made medication changes in 65%; decreased inappropriate medications in 20%; and assisted with pain management in 42%. Trauma surgeons followed one or more recommendations in 91%., Conclusion: Outcomes of older patients can be improved through geriatricians' expertise by addressing new and existing medical issues and reducing hospital-acquired complications such as functional decline, falls, delirium, and death.

The aim of this paper is to present the Lithuanian experience in creating a geriatric care system with the hope that it can be of some use to other countries undergoing similar transitions and changes. The Lithuanian population is old with 20% of the population being 60 years and older, and 15% being 65 years and older, with the trend toward a rapidly aging society. In 1990, Soviet rule in Lithuania ceased and the country regained its independence. With its recent acceptance into the European Union, the nation has set goals to meet the recommended requirements for the care of the elderly. The changes began with specialist training in gerontology and geriatrics, and were an essential first step to bridge the gap between the real and the future needs. Later, the Geriatric Care System Development Program was approved by the Ministry of Health. Despite its difficult past, Lithuania is confronting the challenges of health and social care of the aging population. It is embracing the opportunities to initiate policies and related measures to meet these challenges, and is potentially setting an example of good practice as a new European community., (Copyright 2006 S. Karger AG, Basel.)

Among older adults who drive, the rate of those involved in fatal crashes rises after age 70. Problem driving in older adults involves visual, cognitive, and motor skills, which may decline with aging and chronic disease. Physicians and other health care providers may not be prepared to evaluate and advise the older patient on the emotion-loaded topic of driving ability. Although it is difficult to identify prospectively a high-risk driver, a targeted history and physical exam are useful clinical tools. Two models are proposed to guide the health care provider and patient through the process of limiting or ending the impaired older driver's time behind the wheel.

A scheme-technical, computational and experimental analysis has been made of different schemes of the coordinate scale of modern computer-aided x-ray tomographs. The authors provide evidence for the concept with the following subdivision: (a) the coordinate scale is divided by a closed ring rule with an insignificant number of bench mark holes; (b) an electronic device which divides the distance between the holes of the rule according to the required number of small-sized samples. The schemes of the indicated concept implementation permit choosing an adequate decision for the scanning systems with different arrangements and parameters.

One hundred sixty-five patients with operative prostatic disease were investigated for hematologic abnormalities. The tests were useful in the clinical management of 6 (3.7 per cent) patients. Preoperative hematologic screening tests are recommended in patients with prostatic cancer, potential sepsis, and in patients with a known bleeding tendency.

Evaluation of impairment of the upper extremity is the product of a team effort by the physician, occupational therapist, physical therapist, and rehabilitation counselor. A careful recording of the anatomic impairment should be made because this is critical in determining the subsequent functional activities of the extremity. The measurement criteria for clinical and functional evaluation includes condition assessment instruments. Some assess the neurovascular system, others assess movements including the monitoring of articular motion and musculotendinous function. Sensibility assessment instruments measure sympathetic response and detect single joint stimulus, discrimination, quantification, and recognition abilities. A detailed description of each assessment is recorded and physical capacity evaluation is only one component of the entire vocational evaluation. This evaluation answers questions regarding the injured worker's ability to return to his previous job. The work simulator is a useful instrument that allows rehabilitation and testing of the injured upper extremity. Job site evaluation includes assessment criteria for work performance, work behavior, and work environment.

Gallbladder visualization using the newer IVP contrast materials is rare, and occurs in cases of depressed renal function or acute ureteral obstruction. Mechanisms are discussed.

The results of the technological support for the creation of the software for the first whole body USSR CPT-5000 Tomograph are being discussed. The questions for the consideration: creation of the means totality for the construction of operation pseudoenvironment and usage of the structural programming principles.

The use of computers in our daily home and work environment is increasing. Occupational therapy computer users and clinicians need to be aware of the psychological and physical adaptations which are required by individuals using video display terminals (VDTs). Ergonomics, the study of the interaction between the worker and the work environment, is dedicated to the promotion of worker health, safety, and productivity. The rehabilitationist's goals have always been to bring the patient to his or her optimal level of functioning, often involving the adaptation of the individual workstation in a retrospective manner. However, it is the role of the ergonomist to design work environments which anticipate and adapt to the physical and psychological needs of its workers. This paper will review basic ergonomic design principles for VDT workstations, which can assist the occupational therapist.

The proposed method is aimed at estimation of instability of the intensity of X-ray radiation and its relevance to noise parameters of the estimated data in Computer Tomograph CPT-5000. During the investigation two types of X-ray tubes were used. The experimental results have been obtained as well as special requirements to X-ray intensity instability for the IVth generation X-ray Computer Tomograph unit.

The article may serve as a handbook for the user of the second generation computed X-ray tomographs for human brain examination.

Urologists have been reticent to approach vesicovaginal fistulas via the vaginal route. The Latzko partial colpocleisis seems to be an easier, faster and less traumatic procedure than advocated in the literature. The success rate is high and the procedure is highly recommended.

Baxic clotting mechanisms are reviewed. The clinical and laboratory aspects of disseminated intravascular coagulation and fibrinolysis are discussed, and an approach is suggested to those problems encountered by the urologist.