Publications by authors named "Liberman L"

Unlabelled: Dystrophinopathies - a group of hereditary X-linked neuromuscular diseases characterized by worsening fibrofatty degeneration of skeletal muscles, muscular weakness, low exercise tolerance, as well as orthopedic, cardiovascular and respiratory complications. Study of the effectiveness of physical medical rehabilitation in patients with neuromuscular diseases, evaluation of the influence of external and internal factors on functional capabilities and effectiveness of the conducted rehabilitation are highly relevant.

Objective: To evaluate the effectiveness of physical medical rehabilitation of patients with dystrophinopathies at the outpatient stages of the disease's course and the influence of anthropometric characteristics and functional status of patients' motor capabilities.

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Auditory-nerve fibers (ANFs) from a given cochlear region can vary in threshold sensitivity by up to 60 dB, corresponding to a 1000-fold difference in stimulus level, although each fiber innervates a single inner hair cell (IHC) via a single synapse. ANFs with high-thresholds also have low spontaneous rates (SRs) and synapse on the side of the IHC closer to the modiolus, whereas the low-threshold, high-SR fibers synapse on the side closer to the pillar cells. Prior biophysical work has identified modiolar-pillar differences in both pre- and post-synaptic properties, but a comprehensive explanation for the wide range of sensitivities remains elusive.

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Auditory-nerve fibers (ANFs) from a given cochlear region can vary in threshold sensitivity by up to 60 dB, corresponding to a 1000-fold difference in stimulus level, although each fiber innervates a single inner hair cell (IHC) via a single synapse. ANFs with high-thresholds also have low spontaneous rates (SRs) and synapse on the side of the IHC closer to the modiolus, whereas the low-threshold, high-SR fibers synapse on the side closer to the pillar cells. Prior biophysical work has identified modiolar-pillar differences in both pre- and post-synaptic properties, but a comprehensive explanation for the wide range of sensitivities remains elusive.

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Background: cardiomyopathy is a distinct subset of arrhythmogenic cardiomyopathy, reported primarily in adults, that has predominantly left ventricular involvement and features of myocarditis. Clinical characteristics, risk stratification, and management of pediatric patients with variants are not well known. We sought to identify phenotypic features and prognosis of pediatric patients with pathogenic or likely pathogenic variants.

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The sensors used in the Internet of Medical Things (IoMT) network run on batteries and need to be replaced, replenished or should use energy harvesting for continuous power needs. Additionally, there are mechanisms for better utilization of battery power for network longevity. IoMT networks pose a unique challenge with respect to sensor power replenishment as the sensors could be embedded inside the subject.

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Background: Mentoring is vital to career development in academic medicine, and communication underlies all aspects of the mentoring relationship. Although training research mentors has been shown to be effective, few academic medicine faculties have received training in how to mentor. The investigators developed a novel intervention, the Mentor Communication Skills Training for Oncology Faculty ("Comskil Mentor Training") and examined feasibility and preliminary efficacy.

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Our sense of hearing is mediated by cochlear hair cells, of which there are two types organized in one row of inner hair cells and three rows of outer hair cells. Each cochlea contains 5-15 thousand terminally differentiated hair cells, and their survival is essential for hearing as they do not regenerate after insult. It is often desirable in hearing research to quantify the number of hair cells within cochlear samples, in both pathological conditions, and in response to treatment.

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Background: Premature ventricular contraction (PVC) burden is a risk factor for heart failure and cardiovascular death in patients with structural heart disease. Long-term electrocardiographic monitoring can have a significant impact on PVC burden evaluation by further defining PVC distribution patterns.

Objective: This study aimed to ascertain the optimal duration of electrocardiographic monitoring to characterize PVC burden and to understand clinical characteristics associated with frequent PVCs and nonsustained ventricular tachycardia in a large US cohort.

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Acute noise-induced loss of synapses between inner hair cells (IHCs) and auditory nerve fibers (ANFs) has been documented in several strains of mice, but the extent of post-exposure recovery reportedly varies dramatically. If such inter-strain heterogeneity is real, it could be exploited to probe molecular pathways mediating neural remodeling in the adult cochlea. Here, we compared synaptopathy repair in CBA/CaJ vs.

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Nanodroplet formation is important to achieve supersaturation of active pharmaceutical ingredients (APIs) in an amorphous solid dispersion. The aim of the current study was to explore how polymer composition, architecture, molar mass, and surfactant concentration affect polymer-drug nanodroplet morphology with the breast cancer API, GDC-0810. The impact of nanodroplet size and morphology on dissolution efficacy and drug loading capacity was explored using polarized light microscopy, dynamic light scattering, and cryogenic transmission electron microscopy.

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Article Synopsis
  • Clinically significant bradycardia in children is rare but can lead to serious health issues, sometimes requiring a pacemaker.
  • The primary causes include complete heart block (CHB) and sinus node dysfunction, with pacemakers typically needed for children with CHB.
  • The report discusses three pediatric cases where oral albuterol effectively treated bradycardia, avoiding the immediate need for surgical pacemaker implantation.
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Acoustic overexposure can eliminate synapses between inner hair cells (IHCs) and auditory nerve fibers (ANFs), even if hair-cell function recovers. This synaptopathy has been extensively studied by confocal microscopy, however, understanding the nature and sequence of damage requires ultrastructural analysis. Here, we used focused ion-beam scanning electron microscopy to mill, image, segment and reconstruct ANF terminals in mice, 1 day and 1 week after synaptopathic exposure (8-16 kHz, 98 dB SPL).

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Cardiopulmonary exercise testing (CPET) is an important tool in assessing the functional status of patients with pulmonary arterial hypertension (PAH). During CPET, continuous electrocardiography (ECG) is used as a marker of exercise-induced ischemia or arrhythmia. We hypothesize that ECG changes with exercise may be an early indicator of clinical worsening in PAH and could predict adverse outcomes.

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Our sense of hearing is mediated by cochlear hair cells, localized within the sensory epithelium called the organ of Corti. There are two types of hair cells in the cochlea, which are organized in one row of inner hair cells and three rows of outer hair cells. Each cochlea contains a few thousands of hair cells, and their survival is essential for our perception of sound because they are terminally differentiated and do not regenerate after insult.

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Hypertrophic cardiomyopathy (HCM), a common cardiomyopathy in children, is an important cause of morbidity and mortality. Early recognition and appropriate management are important. An electrocardiogram (ECG) is often used as a screening tool in children to detect heart disease.

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Article Synopsis
  • The study investigates outcomes for children with second-degree heart block to see if they are at risk for developing complete heart block (CHB) or needing a pacemaker (PM) in the long term.
  • Researchers analyzed data from 20 children with certain types of second-degree block observed between 2009 and 2021 and found that 30% needed a PM or developed CHB.
  • Key risk factors linked to disease progression included having second-degree block at a maximum sinus rate, a lower than normal average heart rate, and the presence of 2:1 block on an initial ECG.
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Unlabelled: Becker muscular dystrophy (BMD) is inherited X-linked neuromuscular disease characterized by progressive fatigue, atrophy, hypotonia and muscle weakness, that is predominantly located in muscles of pelvic girdle, femurs and lower leg. There are only singular studies at present showing the efficacy of different training programs for patients with muscular dystrophy, and there are no recommendations allowing to detect the optimal motor regimen, that is effective and safe for such patients.

Objective: To evaluate the efficacy of regular dynamic aerobic exercises in children with BMD, who are able to self-sustained movement.

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Background: Wolff-Parkinson-White syndrome is associated with sudden cardiac death from rapid conduction through the accessory pathway in atrial fibrillation. Adult patients are at higher risk for sudden cardiac death if the shortest-pre-excited-RR-interval in atrial fibrillation (SPERRI) is ≤250 milliseconds (msec) during electrophysiologic study. Exclusive conduction through the atrioventricular node in atrial fibrillation is presumed to convey lower risk.

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Growth- and health-promoting bacteria can boost crop productivity in a sustainable way. Pseudomonas simiae WCS417 is such a bacterium that efficiently colonizes roots, modifies the architecture of the root system to increase its size, and induces systemic resistance to make plants more resistant to pests and pathogens. Our previous work suggested that WCS417-induced phenotypes are controlled by root cell-type-specific mechanisms.

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Objectives: In patients with single ventricle physiology and a systemic right ventricle, tricuspid valve regurgitation increases the risk of adverse outcomes, and tricuspid valve intervention at the time of staged palliation further increases that risk in the postoperative period. However, long-term outcomes of valve intervention in patients with significant regurgitation during stage 2 palliation have not been established. The purpose of this study is to evaluate the long-term outcomes after tricuspid valve intervention during stage 2 palliation in patients with right ventricular dominant circulation in a multicenter study.

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Stimuli-responsive polymers functionalized with reactive inorganic groups enable creation of macromolecular structures such as hydrogels, micelles, and coatings that demonstrate smart behavior. Prior studies using poly(N-isopropyl acrylamide-co-3-(trimethoxysilyl)propyl methacrylate) (P(NIPAM-co-TMA)) have stabilized micelles and produced functional nanoscale coatings; however, such systems show limited responsiveness over multiple thermal cycles. Here, polymer architecture and TMA content are connected to the aqueous self-assembly, optical response, and thermo-reversibility of two distinct types of PNIPAM/TMA copolymers: random P(NIPAM-co-TMA), and a 'blocky-functionalized' copolymer where TMA is localized to one portion of the chain, P(NIPAM-b-NIPAM-co-TMA).

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This research aims to characterize the adsorption morphology of block copolymer dispersants of the styrene-block-4-vinylpyridine family (S4VP) on the surface of multi-walled carbon nanotubes (MWCNT) in a polar organic solvent, ,-dimethyl formamide (DMF). Good, unagglomerated dispersion is important in several applications such as fabricating CNT nanocomposites in a polymer film for electronic or optical devices. Small-angle neutron scattering (SANS) measurements, using the contrast variation (CV) method, are used to evaluate the density and extension of the polymer chains adsorbed on the nanotube surface, which can yield insight into the means of successful dispersion.

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Diversifying the future cancer research workforce requires that students engage in cancer research, persist in paths toward science, technology, engineering, mathematics, and medicine (STEMM) fields, and choose cancer research careers. The Summer Clinical Oncology Research Experience (SCORE) Program at Memorial Sloan Kettering, designed in 2010 to engage undergraduate (U) and post-baccalaureate (PB) students from diverse backgrounds in cancer research, is an 8-week summer program pairing an U or PB student with a faculty mentor to conduct cancer research. We report demographics and career paths for 2010-2019 SCORE students.

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Poloxamers─triblock copolymers consisting of poly(ethylene oxide) (PEO) and poly(propylene oxide) (PPO)─have demonstrated cell membrane stabilization efficacy against numerous types of stress. However, the mechanism responsible for this stabilizing effect remains elusive, hindering engineering of more effective therapeutics. Bottlebrush polymers have a wide parameter space and known relationships between architectural parameters and polymer properties, enabling their use as a tool for mechanistic investigations of polymer-lipid bilayer interactions.

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Article Synopsis
  • - TANGO2 deficiency disorder (TDD) is a rare genetic condition that leads to developmental delays, seizures, and serious metabolic and cardiac issues, and this study aimed to outline its natural history.
  • - The research gathered data from 73 patients across 16 countries, revealing that most children show normal early development but experience a decline in milestones and various symptoms like ataxia and dystonia starting from ages 1 to 3.
  • - Findings suggest that B-complex or multivitamin supplements can significantly reduce the occurrence of metabolic crises in these patients, offering a potential preventive measure.
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