A Narrative Review of Strategies to Optimize Nutrition, Feeding, and Growth among Preterm-Born Infants: Implications for Practice.

Preterm birth is the leading cause of neonatal and under-5 mortality globally, and healthcare-related burden and nutrition-related morbidities are unsustainable, particularly in resource-limited regions. Additionally, preterm infants are susceptible to multiple adverse outcomes including growth faltering, suboptimal neurodevelopment, and multisystemic morbidities. Maturation, healing, repair, and restoration to normalcy in preterm-born infants require optimizing nutrition; only then, prognosis, growth, neurodevelopment, and overall quality of life can improve.

A Systematic Literature Review of the Natural History of Respiratory, Swallowing, Feeding, and Speech Functions in Spinal Muscular Atrophy (SMA).

Background: Respiratory and bulbar dysfunctions (including swallowing, feeding, and speech functions) are key symptoms of spinal muscular atrophy (SMA), especially in its most severe forms. Demonstrating the long-term efficacy of disease-modifying therapies (DMTs) necessitates an understanding of SMA natural history.

Objective: This study summarizes published natural history data on respiratory, swallowing, feeding, and speech functions in patients with SMA not receiving DMTs.

Bulbar function in spinal muscular atrophy (SMA): State of art and new challenges. 21st July 2023, Rome, Italy.

Progressive bulbar involvement is frequent in spinal muscular atrophy, with prevalence and severity of deficits associated with type. The report provides an overview of the presentations made at the workshop grouped into 4 sessions: the first section was dedicated to videofluoroscopy with a revision of the existing protocols and discussion on which one should be used in routine clinical practice and in research settings. The second session was dedicated to interprofessional routine assessments of bulbar function, with a review of the recent clinical tools specifically developed for SMA.

Feeding characteristics of healthy infants without reported feeding impairments throughout the first month of life.

Objective: Elucidate characteristics of feeding performance in healthy infants without reported feeding problems throughout the first month of life.

Study Design: Feeding was monitored in 61 healthy infants by caregiver report for 48 h a week from birth to 4 weeks old. Outcomes included feeding modality, how much they consumed, how long the feed lasted, and how many coughing episodes the infant exhibited.

Establishing Normative Values for Healthy Term Infant Feeding Performance: Neonatal Eating Assessment Tool-Mixed, Oral Feeding Scale, and Early Feeding Skills Assessment.

Purpose: Infants with perceived feeding problems are frequently referred for assessment of their feeding abilities. However, little is known regarding how healthy nondysphagic infants perform on commonly used assessments, making determination of impairment difficult. The aim of this investigation was to elucidate the characteristics of healthy term infant feeding performance using three commonly employed clinical assessments: Neonatal Eating Assessment Tool-Mixed (NeoEat-Mixed), Oral Feeding Scale, and Early Feeding Skills (EFS).

Exploring Alternative Methods to Reduce Milk Flow Rate From Infant Bottle Systems: Bottle Angle, Milk Volume, and Bottle Ventilation.

Purpose: Modifying milk flow rate is a common pediatric dysphagia treatment. Though past investigations have demonstrated how this can be achieved using bottle nipples, little is known about the impact of other bottle modifications. The objective of this investigation is to demonstrate how bottle vent, bottle position, and volume of milk alter bottle pressures and milk flow.

Onasemnogene abeparvovec preserves bulbar function in infants with presymptomatic spinal muscular atrophy: a post-hoc analysis of the SPR1NT trial.

Bulbar function in spinal muscular atrophy has been defined as the ability to meet nutritional needs by mouth while maintaining airway protection and communicate verbally. The effects of disease-modifying treatment on bulbar function are not clear. A multidisciplinary team conducted post-hoc analyses of phase 3 SPR1NT trial data to evaluate bulbar function of infants at risk for spinal muscular atrophy who received one-time gene replacement therapy (onasemnogene abeparvovec) before symptom onset.

Development of an International SMA Bulbar Assessment for Inter-professional Administration.

Background: Progressive weakness can affect bulbar muscles in individuals with moderate to severe forms of spinal muscular atrophy (SMA). The paucity of standardized, valid bulbar assessments capturing clinically significant deficits in SMA impedes the ability to monitor function, facilitate intervention, or detect treatment response.

Objective: To fill this void, an international multidisciplinary team gathered to develop an agreed upon consensus-derived assessment of bulbar function in SMA for inter-professional administration to enhance our ability to monitor disease progression, support clinical management, and evaluate treatment effects.

Patients with Spinal Muscular Atrophy Type 1 Achieve and Maintain Bulbar Function Following Onasemnogene Abeparvovec Treatment.

Background: Improvement and maintenance of bulbar function are goals of disease-modifying treatments for spinal muscular atrophy (SMA). Lack of standardized measures and a widely accepted definition of bulbar function represents a gap in SMA care.

Objective: A multidisciplinary team conducted post-hoc analyses of pooled data from one phase 1 (START) and two phase 3 (STR1VE-US, STR1VE-EU) studies to define and evaluate bulbar function of infants with SMA type 1 after receiving one-time gene replacement therapy, onasemnogene abeparvovec.

Assessing Bulbar Function in Spinal Muscular Atrophy Using Patient-Reported Outcomes.

Background: Novel Spinal Muscular Atrophy (SMA) treatments have demonstrated improvements on motor measures that are clearly distinct from the natural history of progressive decline. Comparable measures are needed to monitor bulbar function, which is affected in severe SMA.

Objective: To assess bulbar function with patient-reported outcome measures (PROs) and determine their relationships with clinical characteristics.

Dysphagia Phenotypes in Spinal Muscular Atrophy: The Past, Present, and Promise for the Future.

Purpose The aim of this study was to provide clinicians with an overview of literature relating to dysphagia in spinal muscular atrophy (SMA) to guide assessment and treatment. Method In this clinical focus article, we review literature published in Scopus and PubMed between 1990 and 2020 pertaining to dysphagia in SMA across the life span. Original research articles that were published in English were included.

Effect of a standardized fluoroscopic procedural approach on fluoroscopy time during infant modified barium swallow studies.

Background: Pediatric dysphagia is a term used to describe dysfunctional feeding and swallowing in infants and children. It is estimated that about 1% of children in the United States are affected by these swallowing problems annually. The modified barium swallow study (MBSS) is considered by many as the gold standard in oropharyngeal swallowing assessment.

Respiratory-Swallow Training Methods: Accuracy of Automated Detection of Swallow Onset, Respiratory Phase, Lung Volume at Swallow Onset, and Real-Time Performance Feedback Tested in Healthy Adults.

Background Preliminary studies have shown that respiratory-swallow training (RST) is a successful treatment for oropharyngeal head and neck cancer patients with refractory dysphagia. Refining the RST protocol with automated analysis software to provide real-time performance feedback has the potential to improve accessibility, reproducibility, and translation to diverse clinical settings. Method An automated software program for data acquisition and analysis developed to detect swallows, determine respiratory phase, calculate lung volume at the onset of the swallow, and provide real-time performance feedback was tested for feasibility in a small cohort of healthy adults.

The effect of preterm birth, recurrent laryngeal nerve lesion, and postnatal maturation on hyoid and thyroid movements, and their coordination in infant feeding.

Movements of the hyoid and thyroid are critical for feeding. These structures are often assumed to move in synchrony, despite evidence that neurologically compromised populations exhibit altered kinematics. Preterm infants are widely considered to be a neurologically compromised population and often experience feeding difficulties, yet measuring performance, and how performance matures in pediatric populations is challenging.

Capturing infant swallow impairment on videofluoroscopy: timing matters.

Background: Infant videofluoroscopic swallow studies (VFSSs) require clinicians to make determinations about swallowing deficits based on a limited number of fluoroscopically observed swallows. Although airway protection is known to decline throughout a bottle-feed, the paucity of data regarding the timing of this degradation has limited the development of procedural protocols that maximize diagnostic validity.

Objective: We tested the stability of key components of swallow physiology and airway protection at four standardized timepoints throughout the VFSS.

First Steps Towards Development of an Instrument for the Reproducible Quantification of Oropharyngeal Swallow Physiology in Bottle-Fed Children.

The incidence of feeding/swallowing impairments (deglutition disorders) in young children is rising and poses serious acute and long-term health consequences. Accurate detection and prompt intervention can lessen the impact of dysphagia-induced sequelae. Videofluoroscopic Swallow Studies (VFSSs) are used to make critical decisions for medically fragile children despite procedural variability and the lack of agreed upon measures for interpreting and reporting results.

Effect of Single-Use, Laser-Cut, Slow-Flow Nipples on Respiration and Milk Ingestion in Preterm Infants.

Purpose: Single-use, laser-cut, slow-flow nipples were evaluated for their effect on respiration and milk ingestion in 13 healthy preterm infants (32.7-37.1 weeks postmenstrual age) under nonlaboratory, clinical conditions.

Dysphagia in infants with single ventricle anatomy following stage 1 palliation: Physiologic correlates and response to treatment.

Background: Deficits in swallowing physiology are a leading morbidity for infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliation. Despite the high prevalence of this condition, the underlying deficits that cause this post-operative impairment remain poorly understood.

Objective: Identify the physiologic correlates of dysphagia in infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliative surgery.

Efficacy of Six Tasks to Clear Laryngeal Mucus Aggregation.

Purpose: Clinicians commonly teach patients alternative clearing behaviors to reduce coughing and hard throat clearing with the assumption that these behaviors clear mucus from the vocal folds. Yet there is limited evidence of the effectiveness of these alternative behaviors at clearing mucus. This study's purpose was to evaluate the efficacy of reducing laryngeal mucus aggregation using alternative approaches in comparison with hard coughing and hard throat clearing in people with and without voice disorders.

The physiologic coupling of sucking and swallowing coordination provides a unique process for neonatal survival.

Aim: Although the coordination of sucking and swallowing is critical for successful oral intake in neonates, the mechanisms that facilitate this coordination are not well understood. This investigation sought to clarify the mechanisms that facilitate this coordination, by comparing sucks that were coordinated with swallows and sucks that were completed in isolation.

Methods: Ten neonates with a median gestational age of 28.

Respiratory-swallow training in patients with head and neck cancer.

Objective: To test a novel intervention to train swallowing to occur in the midexpiratory to low expiratory phase of quiet breathing to improve swallowing safety and efficiency.

Design: Safety and efficacy nonrandomized controlled trial with 1-month follow-up.

Setting: Ambulatory clinics.

Costs of pediatric stroke care in the United States: a systematic and contemporary review.

A substantial literature exists regarding cost-of-care outcomes in adult stroke, however less is known about pediatric stroke. The objective of this review of the literature was to examine studies of costs associated with pediatric stroke care. Six studies reporting data from individuals who experienced a pediatric stroke were included in the review.

Preliminary investigation of the effect of pulse rate on judgments of swallowing impairment and treatment recommendations.

Reducing fluoroscopic pulse rate, a method used to reduce radiation exposure from modified barium swallow studies (MBSSs), decreases the number of images available from which to judge swallowing impairment. It is necessary to understand the impact of pulse rate reduction on judgments of swallowing impairment and, consequentially, treatment recommendations. This preliminary study explored differences in standardized MBSS measurements [Modified Barium Swallow Impairment Profile (MBSImP™©) and Penetration Aspiration Scale (PAS) Scores] between two pulse rates: 30 and simulated 15 pulses per second (pps).