Use of fiber-containing enteral formula in pediatric clinical practice: an expert opinion review.

INTRODUCTION: Children who require enteral nutrition often report gastrointestinal symptoms. There is a growing interest in nutrition formulas that meet nutritional requirements and also maintain gut ecology and function. Fiber-containing enteral formulas can improve bowel function, promote the growth of healthy gut microbiota, and improve immune homeostasis. Nonetheless, guidance in clinical practice is lacking. AREAS COVERED: This expert opinion article summarizes the available literature and collects the opinion of eight experts on the importance and use of fiber-containing enteral formulas in pediatrics. The present review was supported by a bibliographical literature search on Medline via PubMed to collect the most relevant articles. EXPERT OPINION: The current evidence supports using fibers in enteral formulas as first-line nutrition therapy. Dietary fibers should be considered for all patients receiving enteral nutrition and can be slowly introduced from six months of age. Fiber properties that define the functional/physiological properties of the fiber must be considered. Clinicians should balance the dose of fiber with tolerability and feasibility. Introducing fiber-containing enteral formulas should be considered when initiating tube feeding. Dietary fiber should be introduced gradually, especially in fiber-naïve children, with an individualized symptom-based approach. Patients should continue with the fiber-containing enteral formulas they tolerate best.

Tolerability and safety of a semi-elemental enteral formula with partially hydrolyzed guar gum (PHGG) in tube-fed children aged 1-4 years: An open-label, single-arm study.

BACKGROUND AND AIMS: Partially hydrolyzed guar gum (PHGG) is a water-soluble fiber supporting digestive health with well-established safety and efficacy. This open-label, single-arm, multicenter trial aimed to assess the tolerability and safety of a semi-elemental enteral formula containing PHGG at 12 g/L in tube-fed young children. METHODS: Children aged 1-4 years with stable conditions requiring tube feeding to provide ≥80% of their nutritional needs received the study formula for seven days. Tolerability, safety, adequacy of energy/protein intake, and weight change were assessed. RESULTS: Of 24 children (mean age 33.5 months; 10 [41.7%] female), 23 (95.8%) commenced treatment and 18 (75%) completed the study. All children had underlying neuro-developmental disabilities, often in association with gastrointestinal comorbidities requiring treatment for constipation (70.8%) or gastroesophageal reflux (66.7%). The formula was well-tolerated by 19 (82.6%) subjects, while 4 (17.4%; 95% CI: 5%, 39%) subjects withdrew early from the study due to gastrointestinal intolerance. The mean (SD) percentage energy and protein intake across the 7-day period were 103.5% (24.7) and 139.5% [50], respectively. Weight remained stable over the 7-day period (p = 0.43). The study formula was associated with a shift towards softer and more frequent stools. Pre-existing constipation was generally well controlled, and 3/16 (18.7%) subjects ceased laxatives during the study. Adverse events were reported in 12 (52%) subjects and were deemed 'probably related' or 'related' to the formula in 3 (13%) subjects. Gastrointestinal adverse events appeared more common in fiber-naïve patients (p = 0.09). CONCLUSION: The present study indicates that the study formula was safe and generally well tolerated in young tube-fed children. GOV IDENTIFIER: NCT04516213.

Variation of plasma citrulline as a predictive factor for weaning off long-term parenteral nutrition in children with neonatal short bowel syndrome.

BACKGROUND & AIMS: Long-term parenteral nutrition (PN) is the mainstay of the therapeutic strategy in intestinal failure (IF) due to neonatal short bowel syndrome (SBS). Our aim was to identify prognostic factors for PN weaning and to assess if measuring plasma citrulline concentrations over time could account for the intestinal adaptation in progress. METHODS: This retrospective study included children with neonatal SBS with surgical measurement of the residual bowel length and repeated plasma citrulline assessments during a 4-year follow-up. The degree of IF was assessed by the PN dependency index (PN caloric intake/Resting energy expenditure). The analysis was carried out according to SBS anatomical groups: end-jejunostomy (type 1), jejuno-colic (type 2) and jejuno-ileal anastomosis (type 3). RESULTS: Fifty-five patients (8 type 1, 27 type 2, 20 type 3) were included. None of the patients with SBS type 1, 11 (41%) with type 2 and 11 (55%) with type 3 were weaned off during the follow-up period. Plasma citrulline levels significantly increased with time in patients who were finally weaned off PN; conversely, the levels did not consistently increase in patients who were still on PN at the end of the study period. There was an inverse relationship between plasma citrulline levels and the PN dependency index. The increasing citrulline levels had a positive effect on the probability of weaning, 2.7 times higher for each point increase in citrulline. No significant effect of age and residual bowel length at baseline was found. CONCLUSION: The increased plasma citrulline level over time in addition to the SBS anatomical type is a reliable marker for subsequent PN weaning. The prediction of PN weaning assessed solely by the residual bowel length or a single measurement of citrulline is insufficient and should also take into account the anatomical type of SBS and repeated measurements of plasma citrulline levels.

A hidden side of the COVID-19 pandemic in children: the double burden of undernutrition and overnutrition.

The COVID-19 pandemic has deteriorated key determinants of health and caused major upheavals around the world. Children, although less directly affected by the virus, are paying a heavy price through the indirect effects of the crisis, including poor diet, mental health impact, social isolation, addiction to screens and lack of schooling and health care, particularly among vulnerable groups. This paper is aimed at discussing the potential impact of this pandemic on children's nutrition and lifestyle. Preliminary data from the literature and from our survey show significant disruptions in nutrition and lifestyle habits of children. While undernutrition is expected to worsen in poor countries, obesity rates could increase in middle- and high-income countries especially among precarious groups widening the gap in health and social inequalities.The real impact of the COVID-19 pandemic on children extends well beyond that of a viral infection. This crisis has public health implications that could have life-long consequences on children. It requires effective and targeted measures mainly for vulnerable children and households to guarantee children's basic rights for optimal nutrition, health and development.

Cannabinoids Improve Gastrointestinal Symptoms in a Parenteral Nutrition-Dependent Patient With Chronic Intestinal Pseudo-Obstruction.

Chronic intestinal pseudo-obstruction (CIPO) is a rare and challenging cause of pediatric intestinal failure, requiring long-term parenteral nutrition in most cases. Despite optimal management, some patients experience chronic abdominal pain and recurrent obstructive episodes with a major impact on their quality of life. Cannabinoids have been successfully used in some conditions. However, their use in CIPO has never been reported in the literature. We report a case of successful use of medicinal cannabinoids in a patient with CIPO, resulting in a significant reduction of abdominal pain, vomiting, and subocclusive episodes and increased appetite and weight, without major adverse events. Although further observations are required to consolidate these findings, this case may be helpful for other patients suffering from the same condition.

Recent insights into trace element deficiencies: causes, recognition and correction.

PURPOSE OF REVIEW: Trace elements are vital components involved in major body functions. Cases of trace elements deficiencies are increasingly encountered in clinical practice, although often underrecognized. This review gives a thorough insight into the newest findings on clinical situations associated with trace elements deficiencies in children and adults, their recognition and management. RECENT FINDINGS: Trace elements deficiencies are frequently found in various conditions, most commonly in burns, bariatric surgery, intestinal failure, renal replacement therapy, oncology, critical illness and cardiac surgery. The main trace elements involved are selenium, zinc, copper and iron. Trace elements deficiencies are associated with increased risk of morbidity and mortality. Recognition of clinical signs of trace elements deficiencies can be challenging. Although trace elements supplementation is indisputable in many circumstances, it is still debatable in other situations such as sepsis and cardiac surgery. SUMMARY: Recent findings on trace elements deficiencies could have important implications on health outcomes. Trace elements delivery is a core component of nutritional care. Front-line clinicians should be aware of at-risk clinical situations to provide correct and timely intervention. Future research should be directed towards investigating the potential benefits of antioxidant trace elements supplementation in children in whom studies are scarce, especially in critical conditions such as burns, sepsis and cardiac surgery.

Nutritional challenges in children with primary immunodeficiencies undergoing hematopoietic stem cell transplant.

Nutritional profile and management of patients with primary immunodeficiencies (PID) undergoing hematopoietic stem cell transplant (HSCT) has not been described in the literature. We aim to report the nutritional challenges and practices peculiar to this population before and after HSCT and suggest clinical pathways for their management. We conducted a single-centre retrospective study. Inclusion criteria were children aged less than 20 years with a diagnosis of PID who have undergone HSCT at the Royal Children's Hospital Melbourne since April 2014 with a minimal follow-up of 1 year. Nutritional parameters were collected in the pre-transplant period, at conditioning, and at 1, 3, 6 and 12 months post-HSCT. Descriptive analysis were used. Between April 2014 and December 2018, 27 children received 31 HSCT. Before transplant, 33% had a weight and/or height ≤ -2 standard deviations (SD). Forty percent required nutritional support before transplant: 33% had enteral nutrition (EN) while 7% required long-term parenteral nutrition (PN) due to intestinal failure. After transplant, although most children were started on EN, 82% required PN with a mean duration of 67 days. Mean time to full oral diet was 154 days. Pre-transplant mean weight and height were -0.57 SD and -0.88 SD respectively. After a decrease in anthropometric parameters the first 3 months post-transplant, progressive catch up was noticeable for weight (-0.27 SD) with no catch up for height at 1 year (-0.93 SD). Our work highlights the nutritional challenges and specificities of children with PID in the peri-transplant period. An approach to nutrition assessment and management in the pre- and post-transplant period is proposed.

Monitoring of long-term parenteral nutrition in children with intestinal failure.

Pediatric intestinal failure (IF) is a rare and complex condition associated with significant morbidity and mortality. It is defined as the reduction of gut mass or function below the minimal needed for absorption of nutrients and fluid to sustain life and growth. Since the advent of specialized multidisciplinary intestinal rehabilitation centers, IF management has considerably evolved in the last years, but serious complications of long-term parenteral nutrition (PN) can occur. Main complications include intestinal failure-associated liver disease, growth failure, body composition imbalance, central venous access complications, micronutrient deficiencies and toxicities, metabolic bone disease, small intestinal bacterial overgrowth, and renal disease. With improvement in survival rates of patients over the last 20 years, emphasis should be on limiting IF-related comorbidities and improving quality of life. Close monitoring is pivotal to ensuring quality of care of these patients. The care of children with chronic IF should involve a comprehensive monitoring plan with flexibility for individualization according to specific patient needs. Monitoring of children on long-term PN varies significantly across units and is mainly based on experience, although few guidelines exist. This narrative review summarizes the current knowledge and practices related to monitoring of children with IF. The authors also share their 20-year experience at the Royal Children's Hospital in Melbourne Australia on this topic.

Trace Element Provision in Parenteral Nutrition in Children: One Size Does Not Fit All.

Routine administration of trace elements is recognised as a standard of care in children requiring parenteral nutrition. However, there is a lack of global consensus regarding trace elements provision and dosing in pediatric parenteral nutrition. This review provides an overview of available evidence regarding trace elements supply and posology in parenteral nutrition in neonates and children. Trace elements provision in children should be tailored to the weight and clinical condition of the child with emphasis on those at risk of toxicity or deficiency. Based on current evidence, there is a need to review the formulation of commercial solutions that contain multiple-trace elements and to enable individual trace elements additives to be available for specific indications. Literature supports the removal of chromium provision whereas manganese and molybdenum supplementation are debated. Preterm neonates may have higher parenteral requirements in iodine, selenium and copper than previously recommended. There is growing support for the routine provision of iron in long-term parenteral nutrition. Further studies on trace elements contamination of parenteral nutrition solutions are needed for a range of trace elements.

A novel LAMB2 gene mutation associated with a severe phenotype in a neonate with Pierson syndrome.

BACKGROUND: Pierson syndrome (PS) is a rare autosomal recessive disorder, caused by mutations in the laminin ß2 (LAMB2) gene. It is characterized by congenital nephrotic syndrome, microcoria, and neurodevelopmental deficits. Several mutations with genotype-phenotype correlations have been reported, often with great clinical variability. We hereby report a novel homozygous nonsense mutation in the LAMB2 gene, associated with a severe phenotype presentation. CASE DIAGNOSIS: We describe a term male infant born from consanguineous parents. The mother previously lost three children in the neonatal period, secondary to undefined renal disease, had two spontaneous abortions, and gave birth to one healthy daughter. The index case presented at birth with bilateral microcoria, severe hypotonia, respiratory distress, and congenital nephrotic syndrome associated with anuria and severe renal failure requiring peritoneal dialysis. The patients' clinical follow-up was unfavorable, and the newborn died at 7 days of life, after withdrawal of life support. Genetic analysis revealed a homozygous nonsense mutation at position c.2890C>T causing a premature stop codon (p.R964*) in LAMB2 gene. CONCLUSION: We here describe a novel nonsense homozygous mutation in LAMB2 gene causing a severe neonatal presentation of Pierson syndrome. This new mutation expands the genotype-phenotype spectrum of this rare disease and confirms that truncating mutations might be associated with severe clinical features.