Evaluating caregiver-clinician communication for tracheostomy placement in the neonatal intensive care unit: a qualitative inquiry.

Objective: Identify stakeholders' tracheostomy decision-making information priorities in the Neonatal Intensive Care Unit (NICU).

Study Design: English-speaking caregivers and clinicians who participated in NICU tracheostomy discussions between January 2017 and December 2021 were eligible. They reviewed a pediatric tracheostomy communication guide prior to meeting.

Evaluating Caregiver-Clinician Communication for Tracheostomy Placement in the Neonatal Intensive Care Unit: A Qualitative Inquiry.

Objective: Identify stakeholders' tracheostomy decision-making information priorities in the Neonatal Intensive Care Unit (NICU).

Study Design: English-speaking caregivers and clinicians who participated in NICU tracheostomy discussions between January 2017 and December 2021 were eligible. They reviewed a pediatric tracheostomy communication guide prior to meeting.

Speech Disorders in Children With Pompe Disease: Articulation, Resonance, and Voice Measures.

Purpose: Children with Pompe disease, a rare genetic metabolic myopathy, often have speech impairments. In this study, we provide a comprehensive description of articulation, resonance, and voice in children with Pompe disease.

Method: Fifteen children with Pompe disease (11 with infantile-onset Pompe disease [IOPD], four with late-onset Pompe disease [LOPD]) ranging from 6 to 18 years of age participated in standard speech assessments.

Assessment of Dysphonia in Children with Pompe Disease Using Auditory-Perceptual and Acoustic/Physiologic Methods.

Bulbar and respiratory weakness occur commonly in children with Pompe disease and frequently lead to dysarthria. However, changes in vocal quality associated with this motor speech disorder are poorly described. The goal of this study was to characterize the vocal function of children with Pompe disease using auditory-perceptual and physiologic/acoustic methods.

Tongue weakness and atrophy differentiates late-onset Pompe disease from other forms of acquired/hereditary myopathy.

Late-onset Pompe disease (LOPD) is an inherited autosomal recessive progressive metabolic myopathy that presents in the first year of life to adulthood. Clinical presentation is heterogeneous, differential diagnosis is challenging, and diagnostic delay is common. One challenge to differential diagnosis is the overlap of clinical features with those encountered in other forms of acquired/hereditary myopathy.

Respiratory muscle training in late-onset Pompe disease: Results of a sham-controlled clinical trial.

To address progressive respiratory muscle weakness in late-onset Pompe disease (LOPD), we developed a 12-week respiratory muscle training (RMT) program. In this exploratory, double-blind, randomized control trial, 22 adults with LOPD were randomized to RMT or sham-RMT. The primary outcome was maximum inspiratory pressure (MIP).

Novel approaches to quantify CNS involvement in children with Pompe disease.

Objective: To characterize the extent of CNS involvement in children with Pompe disease using brain MRI and developmental assessments.

Methods: The study included 14 children (ages 6-18 years) with infantile Pompe disease (IPD) (n = 12) or late-onset Pompe disease (LOPD) (n = 2) receiving enzyme replacement therapy. White matter (WM) hyperintense foci seen in the brain MRIs were systematically quantified using the Fazekas scale (FS) grading system with a novel approach: the individual FS scores from 10 anatomical areas were summed to yield a total FS score (range absent [0] to severe [30]) for each child.

Training, detraining, and retraining: Two 12-week respiratory muscle training regimens in a child with infantile-onset Pompe disease.

Background: Respiratory muscle weakness is a primary cause of morbidity and mortality in patients with Pompe disease. We previously described the effects of our 12-week respiratory muscle training (RMT) regimen in 8 adults with late-onset Pompe disease [1] and 2 children with infantile-onset Pompe disease [2].

Case Report: Here we describe repeat enrollment by one of the pediatric participants who completed a second 12-week RMT regimen after 7 months of detraining.

3D Printing Neuron Equivalent Circuits: An Undergraduate Laboratory Exercise.

The electrical equivalent circuit for a neuron is composed of common electrical components in a configuration that replicates the passive electrical properties and behaviors of the neural membrane. It is a powerful tool used to derive such fundamental neurophysiological equations as the Hodgkin-Huxley equations, and it is also the basis for well-known exercises that help students to model the passive (Ohmic) properties of the neuronal membrane. Unfortunately, as these exercises require basic knowledge of electronics, they are generally not physically conducted in biomedical courses, but remain merely conceptual exercises in a book or simulations on a computer.

Higher dosing of alglucosidase alfa improves outcomes in children with Pompe disease: a clinical study and review of the literature.

Purpose: Enzyme replacement therapy (ERT) with recombinant human acid-α glucosidase (rhGAA) at standard dose of 20 mg/kg every other week is insufficient to halt the long-term progression of myopathy in Pompe disease.

Methods: We conducted a retrospective study on infantile-onset Pompe disease (IPD) and late-onset Pompe disease (LOPD) patients with onset before age 5 years, ≥12 months of treatment with standard dose ERT, and rhGAA immunogenic tolerance prior to dose escalation. Long-term follow-up of up to 18 years was obtained.

Models for Spiking Neurons: Integrate-and-Fire Units and Relaxation Oscillators.

Relaxation oscillators are nonlinear electronic circuits that produce a repetitive non-sinusoidal waveform when sufficient voltage is applied. In this fashion, they are reminiscent of integrate-and-fire neuron models, except that they also include components with hysteresis, and thus require no threshold rule to determine when an impulse has occurred or to return the voltage to its reset value. Here, I discuss the pros and cons of teaching elementary neurophysiology using first-order linear integrate-and-fire neurons versus relaxation oscillator circuits.

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Introduction:: Morbidity and mortality in adults with late-onset Pompe disease (LOPD) results primarily from persistent progressive respiratory muscle weakness despite treatment with enzyme replacement therapy (ERT). To address this need, we have developed a 12-week respiratory muscle training (RMT) program that provides calibrated, individualized, and progressive pressure-threshold resistance against inspiration and expiration. Our previous results suggest that our RMT regimen is safe, well-tolerated, and results in large increases in respiratory muscle strength.

Auditory-Perceptual Speech Features in Children With Down Syndrome.

Speech disorders occur commonly in individuals with Down syndrome (DS), although data regarding the auditory-perceptual speech features are limited. This descriptive study assessed 47 perceptual speech features during connected speech samples in 26 children with DS. The most severely affected speech features were: naturalness, imprecise consonants, hyponasality, speech rate, inappropriate silences, irregular vowels, prolonged intervals, overall loudness level, pitch level, aberrant oropharyngeal resonance, hoarse voice, reduced stress, and prolonged phonemes.

Recording EMG Signals on a Computer Sound Card.

For a few dollars and in a few minutes, a simple circuit can be built to permit a cheap external sound card and a laptop computer to be used as a portable data acquisition system for recording EMG. The circuit uses a common audio amplifier integrated circuit to increase the gain of the EMG signals recorded from EMG surface electrodes and to match the impedance of the electrode-skin interface with the input impedance of the sound card. Data can be recorded using open source sound editing software and analyzed offline using simple Python code.

Staged treatment and acceptability guidelines in early psychosis study (STAGES): A randomized placebo controlled trial of intensive psychosocial treatment plus or minus antipsychotic medication for first-episode psychosis with low-risk of self-harm or aggression. Study protocol and baseline characteristics of participants.

Aim: It is now necessary to investigate whether recovery in psychosis is possible without the use of antipsychotic medication. This study will determine (1) whether a first-episode psychosis (FEP) group receiving intensive psychosocial interventions alone can achieve symptomatic remission and functional recovery; (2) whether prolonging the duration of untreated psychosis (DUP) in a sub-group according to randomisation will be associated with a poorer outcome and thereby establish whether the relationship between DUP and outcome is causative; and (3) whether neurobiological changes observed in FEP are associated with the psychotic disorder or antipsychotic medication. Baseline characteristics of participants will be presented.

Moderated Online Social Therapy: A Model for Reducing Stress in Carers of Young People Diagnosed with Mental Health Disorders.

Family members caring for a young person diagnosed with the onset of mental health problems face heightened stress, depression, and social isolation. Despite evidence for the effectiveness of family based interventions, sustaining access to specialist family interventions is a major challenge. The availability of the Internet provides possibilities to expand and sustain access to evidence-based psychoeducation and personal support for family members.

Breadboard Amplifier: Building and Using Simple Electrophysiology Equipment.

Electrophysiology is a valuable skill for the neuroscientist, but the learning curve for students can be steep. Here we describe a very simple electromyography (EMG) amplifier that can be built from scratch by students with no electronics experience in about 30 minutes, making it ideal for incorporating into a laboratory activity. With few parts and no adjustments except the gain, students can begin physiology experiments quickly while having the satisfaction of having built the equipment themselves.

Pencil-and-Paper Neural Networks: An Undergraduate Laboratory Exercise in Computational Neuroscience.

Although it has been more than 70 years since McCulloch and Pitts published their seminal work on artificial neural networks, such models remain primarily in the domain of computer science departments in undergraduate education. This is unfortunate, as simple network models offer undergraduate students a much-needed bridge between cellular neurobiology and processes governing thought and behavior. Here, we present a very simple laboratory exercise in which students constructed, trained and tested artificial neural networks by hand on paper.

The Impact of a Mindfulness Based Program on Perceived Stress, Anxiety, Depression and Sleep of Incarcerated Women.

Incarcerated women enter the prison setting with remarkable histories of trauma, mental health and substance abuse issues. Given the stress of incarceration and separation from their children, families, and significant others, it is not surprising that many women experience increased anxiety, depression, and problems with sleep. Due to these negative outcomes, it is imperative to find efficient non-pharmacological interventions.

Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining.

Background: Determine the effects of a 12-week respiratory muscle training (RMT) program in late-onset Pompe disease (LOPD).

Methods: We investigated the effects of 12-weeks of RMT followed by 3-months detraining using a single-subject A-B-A experimental design replicated across 8 adults with LOPD. To assess maximal volitional respiratory strength, our primary outcomes were maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP).

Temperature Measurement Inside Protective Headgear: Comparison With Core Temperatures and Indicators of Physiological Strain During Exercise in a Hot Environment.

Non-invasive temperature monitoring with a sensor inside protective headgear may be effective in detecting temperatures that are associated with heat illness. The purpose was to establish the relationship between in-hardhat temperatures (Tih) and core temperature (Tc) as measured by rectal (Tre) and esophageal (Tes) probes. Thirty males (age 24.

Compensatory plasticity restores locomotion after chronic removal of descending projections.

Homeostatic plasticity is an important attribute of neurons and their networks, enabling functional recovery after perturbation. Furthermore, the directed nature of this plasticity may hold a key to the restoration of locomotion after spinal cord injury. Here we studied the recovery of crawling in the leech Hirudo verbana after descending cephalic fibers were surgically separated from crawl central pattern generators shown previously to be regulated by dopamine.

Quantitative assessment of lingual strength in late-onset Pompe disease.

Introduction: Skeletal muscle is common in late-onset Pompe disease (LOPD). Recent data implicate common bulbar muscle involvement (i.e.