Home enteral nutrition does not decrease oral feeding in children during the first year of nutritional support.

BACKGROUND: Enteral nutrition is necessary when nutritional status is poor and oral intake is insufficient or impossible. Although it has been suspected to reduce spontaneous oral feeding, no study has formally assessed the influence of enteral nutrition on pediatric oral intake. The present study aimed to evaluate variation in oral feeding intake after enteral nutrition initiation, and to identify factors influencing oral feeding. METHODS: This retrospective cohort study included 149 pediatric patients from two French tertiary care hospitals, who received home enteral nutrition from 2009 to 2022. The patients were aged 2 months to 17 years (median age 3 years, interquartile range 1.3-9.2). Oral and enteral intakes were assessed when enteral nutrition was initiated (M0), and again at M3 (n = 123), M6 (n = 129), and M12 (n = 134) follow-ups, based on dieticians' and home services' reports. Oral feeding and body mass index z score variations during follow-ups were evaluated using a linear mixed regression model, including "time" as a fixed effect and "patient" as a random effect. Factors associated with oral feeding changes were assessed using a model interaction term. RESULTS: Oral intake did not vary significantly (P = 0.99) over time and accounted for 47.4% ± 27.4%, 46.9% ± 27.4%, 48.4% ± 28.2%, and 46.6% ± 26.9% of the ideal recommended daily allowance (calculated for the ideal weight for height) at M0, M3, M6, and M12, respectively. Delivery method (nasogastric tube versus gastrostomy), prematurity, underlying disease, history of intrauterine growth retardation, and speech therapy intervention did not influence oral intake. Administration (i.e., exclusively continuous nocturnal infusion versus daytime bolus) led to different oral intake development, although oral intake also differed at M0. CONCLUSIONS: Enteral nutrition, although increasing total energy intake, does not alter oral feeding during the first year of administration. Only the mode of administration might influence oral intake.

"Baby-led weaning" - Progress in infant feeding or risky trend?

Baby-led weaning (BLW), proposed as a new form of complementary feeding, has emerged as a real trend phenomenon in the media. Infants are seated at the family table from the age of 6 months, facing the foods they grab and bring to their mouth: they decide which foods they want to eat and what amount. The consumption of mashed foods and the use of a spoon are totally discouraged. BLW is increasingly used in nurseries and centers of young children. A bibliographic search carried out between 2000 and 2021 found 423 articles, of which 38 were selected. The clinical studies selected are 11 cross-sectional observational studies and two randomized controlled studies. BLW promotes breastfeeding, the early introduction of morsels, the respect of the child's appetite, the use of unprocessed foods, and the choice of "homemade" and friendliness. These benefits can nonetheless be reached with usual complementary feeding (SCF), according to current recommendations. Other benefits are claimed without scientific evidence such as easier achievement of dietary complementary feeding and an optimal growth with prevention of excess weight gain. BLW has some obvious downsides. The infant may not get enough energy, iron, zinc, vitamins, and other nutrients, or too much protein, saturated fat, salt, or sugar. The risk of choking, which must be distinguished from the physiological gagging reflex, has not been ruled out by scientific studies. Currently, the Nutrition Committee of the French Pediatric Society considers that the data published to date in terms of benefits and risks of BLW do not lend themselves to advice for this practice in preference over SCF carried out according to current recommendations.

The role of nutrition in non-alcoholic fatty liver disease treatment in obese children.

Non-alcoholic fatty liver disease (NAFLD) is a highly prevalent chronic liver disease that occurs mostly in the context of insulin resistance and obesity. It has rapidly evolved into the most common cause of liver disease among children. The incidence is high in obese children and a greater risk of disease progression is associated with severe obesity, highlighting the role of nutrition. To date, there is no consensus on NAFLD management. This is a narrative review of clinical studies on the potential benefit of nutritional interventions, including lifestyle modifications, vitamins, docosahexaenoic acid, and probiotics in children with NAFLD. The Comité de nutrition de la Société Française de Pédiatrie (CN-SFP) emphasizes the effect of limiting added sugar intake, i.e., fructose or sucrose-containing beverages, and promoting physical activity in the care of NAFLD.

Nutritional risks of ARFID (avoidant restrictive food intake disorders) and related behavior.

Avoidant/restrictive food intake disorder (ARFID) has recently been added to the DSM V (Diagnostic and Statistical Manual of Mental Disorders, 5th edition) as a new class of eating disorders (EDs). ARFID is characterized by a lack of interest in eating or avoiding specific types of foods because of their sensory characteristics. This avoidance results in decreased nutritional intake, eventually causing nutritional deficiencies. In severe cases, ARFID can lead to dependence on oral nutritional supplements, which interferes with psychosocial functioning. The prevalence of ARFID can be as high as 3% in the general population, and it is often associated with gastrointestinal symptoms and mainly appears in children with anxiety disorders. Given the high prevalence of ARFID, a rapid and systematic nutrition survey should be conducted during every pediatric consultation. Its treatment should also be adapted depending on the severity of the nutritional problem and may involve hospitalization with multidisciplinary care (pediatrician, nutritional therapist, dietitian, psychologists, and speech therapists).

TTC7A mutation must be considered in patients with repeated intestinal atresia associated with early inflammatory bowel disease: Two new case reports and a literature review.

TTC7A mutations cause multiple neonatal intestinal atresias with early inflammatory bowel disease and severe combined immunodeficiency. There are no treatment protocols for this rare disease. Two new cases are described for which radical early treatment measures - total enterectomy, home parenteral nutrition, immunoglobulin therapy and intravenous antibiotic prophylaxis - have allowed both patients to develop optimally.

Single, short in-del, and copy number variations detection in monogenic dyslipidemia using a next-generation sequencing strategy.

Optimal molecular diagnosis of primary dyslipidemia is challenging to confirm the diagnosis, test and identify at risk relatives. The aim of this study was to test the application of a single targeted next-generation sequencing (NGS) panel for hypercholesterolemia, hypocholesterolemia, and hypertriglyceridemia molecular diagnosis. NGS workflow based on a custom AmpliSeq panel was designed for sequencing the most prevalent dyslipidemia-causing genes (ANGPTL3, APOA5, APOC2, APOB, GPIHBP1, LDLR, LMF1, LPL, PCSK9) on the Ion PGM Sequencer. One hundred and forty patients without molecular diagnosis were studied. In silico analyses were performed using the NextGENe software and homemade tools for detection of copy number variations (CNV). All mutations were confirmed using appropriate tools. Eighty seven variations and 4 CNV were identified, allowing a molecular diagnosis for 40/116 hypercholesterolemic patients, 5/13 hypocholesterolemic patients, and 2/11, hypertriglyceridemic patients respectively. This workflow allowed the detection of CNV contrary to our previous strategy. Some variations were found in previously unexplored regions providing an added value for genotype-phenotype correlation and familial screening. In conclusion, this new NGS process is an effective mutation detection method and allows better understanding of phenotype. Consequently this assay meets the medical need for individualized diagnosis of dyslipidemia.

[Food protein-induced enterocolitis syndrome: A case report of diarrhea with hypovolemic shock and methemoglobinemia]. / Syndrome d'entérocolite induite par les protéines alimentaires (SEIPA) : à propos d'une observation de diarrhée avec choc hypovolémique et méthémoglobinémie.

We report on the case of a young infant with chronic diarrhea that worsened and turned into hypovolemic shock with methemoglobinemia. We underline and discuss the main features of food protein-induced enterocolitis syndrome (FPIES). FPIES is a non-IgE-mediated food allergy involving tumor necrosis factor-alpha (TNF-α). Many triggering foods exist but cow's milk, as in the case reported herein, is one of the most frequent. It can start early or be delayed and start around the average age of 5 months. Symptoms are nonspecific with diarrhea and vomiting, but in the presence of methemoglobinemia, the diagnosis must be seriously considered. The oral food challenge remains the gold standard to confirm the diagnosis if there is still a doubt. Treatment of FPIES associates emergency treatment of acute dehydration with the prevention of relapses by avoiding the suspected protein.

[Nutritional status in pediatric intermediate care: Assessment at admission, progression during the stay and after discharge]. / État nutritionnel en unité de surveillance continue pédiatrique : évaluation à l'admission, en cours de séjour et à distance.

INTRODUCTION: Malnutrition, known as a cause of morbidity, has not been studied in children admitted to pediatric intermediate care units. This study aimed to describe the association between patients' nutritional status and the relative severity of illness that characterizes this population. PATIENTS AND METHODS: In this prospective longitudinal study, a dedicated nutrition support team assessed the nutritional status of all children, aged 5 days to 18 years, admitted to our university pediatric intermediate care unit (Hôpital Femme-Mère-Enfants, Lyon, France) over one year (2012-2013). Weight, height, body mass index, weight-for-age ratio, height-for-age ratio, and the analysis of growth curves were collected at admission. We monitored patients' weight, which allowed us to detect malnutrition occurrence during the stay, and its progression up to 3 months after discharge. RESULTS: A total of 459 patients were enrolled. Based on the analysis integrating all nutritional indices and the progression of growth curves, malnutrition at admission was detected in 23.8% of children (20.5% and 6.8% suffered from acute and chronic malnutrition, respectively). Based only on the body mass index, malnutrition was detected in 15.5% of children. Chronic disease appeared as a risk factor for malnutrition at admission (P=0.0001) and young age for acute malnutrition (P=0.04). The incidence of acquired malnutrition during the stay (in children with a length of stay > 5 days) was up to 26%, and dyspnea was the only risk factor identified. This population recovered with a normal nutritional status late (66% after 2 months and 16% after 3 months). CONCLUSIONS: The prevalence of malnutrition is high in our pediatric intermediate care unit. The occurrence of acquired malnutrition during the stay is frequent. All children should benefit from systematic nutritional assessment at admission as well as careful monitoring during the stay and after discharge, to adapt early and individualized nutritional support.

Plasma fatty acid composition in French-Canadian children with non-alcoholic fatty liver disease: Effect of n-3 PUFA supplementation.

Non-alcoholic fatty liver disease (NAFLD) represents one of the most common causes of liver disease worldwide. As the NAFLD pathogenesis is associated with diet and lifestyle, the aims of the present work are to assess fatty acid (FA) composition in NAFLD young French-Canadian, to determine whether treatment with n-3 FA improves the plasma FA profile, and to define the time on the effectiveness of n-3 FA supplementation. Baseline characteristics of the NAFLD subjects show increased, anthropometric and biochemical parameters. Their plasma FA composition is characterized by a percent increase in total n-6 FA and a high proportion of saturated and total monounsaturated FA, as well as a decrease in Δ5 and increase in Δ6 desaturases. In conclusion, our results document for the first time the composition of plasma FAs in NAFLD young French Canadian and the efficacy of 3-month supplementation to improve the proportion of n-3 FA in their plasma.

Implementation of a computerized system in pediatric wards to improve nutritional care: a cluster randomized trial.

BACKGROUND/OBJECTIVES: Malnutrition occurs frequently in hospitalized children. We aimed to assess whether a computerized system could lead to improved clinical practices in malnourished children. SUBJECTS/METHODS: Healthcare workers (242) from six departments in a pediatric university hospital participated in a cluster randomized trial, studying 1457 malnourished children hospitalized from September 2009 to August 2011. Following a baseline observational pre-intervention period, all departments were randomized into either intervention or control arms. A computerized malnutrition-screening system was implemented in the intervention group to automatically trigger a dietetic referral in real time. Furthermore, the nutrition support team conducted an awareness campaign with healthcare workers and a leadership-based strategy to reinforce the message during the entire study period. Adherence to practice guidelines (daily weights, investigation of etiology for malnutrition, management by a dietitian and application of refeeding protocols) was compared between pre- and post-intervention periods in both the intervention and trial arms. RESULTS: When compared with the pre-intervention period, the clinical practices were significantly improved within the intervention arm for every outcome (P<0.01), whereas remained unchanged in the control arm. In addition, during the post-intervention period, malnutrition etiology investigation by physicians (adjusted odds ratio (OR) of 4.4, 95% confidence interval (CI) 1.7-11.8, P=0.003) and management by a dietitian (OR 2.7, 95% CI 1.0-6.9, P=0.046) occurred more frequently in the intervention clusters. CONCLUSIONS: Implementation of an electronic system to detect malnutrition in real time was associated with a rapid improvement in clinical practices for better care of hospitalized children.

Prevalence of liver complications in children receiving long-term parenteral nutrition.

BACKGROUND/OBJECTIVES: The hepatic prognosis of long-term home total parenteral nutrition (TPN)-dependent children is poorly documented. The objective was to study outcome data in home TPN-dependent children and to describe precisely their liver biopsies in the attempt to analyze risk factors for biochemical and histological hepatic abnormalities. SUBJECTS/METHODS: Medical records of 42 children receiving home TPN for more than 2 years between January 1998 and December 2007 in a single approved home total parenteral center were reviewed. Hepatic biochemical abnormalities were analyzed. Hepatic biopsies were classified by two independent pathologists. RESULTS: Duration of TPN was 7.9±0.8 years (mean±s.e.m.), with an average age at onset of 1.5±0.5 years. A total of 24 patients (57%) developed biochemical liver abnormalities in an average of 2.9±0.4 years after starting TPN. Risk factors for biochemical abnormalities were younger age at TPN commencement, longer duration of TPN, higher rate of catheter-related infections and higher volume and energy content of TPN. Liver biopsies were carried out in 43% of patients (mean age 3.2±0.9 years). Almost all patients had fibrosis (94%). Risk factors were dependent on each histological abnormality: fibrosis was significantly associated with a shorter length of bowel and a longer duration of TPN; cholestasis correlated with a lower percentage of total parenteral energy intake due to lipids; and steatosis had no risk factor identified. CONCLUSION: Our study reports a high rate of histological liver abnormalities and analyzes risk factors in children who underwent very long-term home TPN.

Anderson's disease (chylomicron retention disease): a new mutation in the SARA2 gene associated with muscular and cardiac abnormalities.

Anderson's disease (AD) or chylomicron retention disease (CMRD) is a rare hereditary lipid malabsorption syndrome linked to SARA2 gene mutations. We report in this study a novel mutation in two sisters for which the Sar1b protein is predicted to be truncated by 32 amino acids at its carboxyl-terminus. Because the SARA2 gene is also expressed in the muscle, heart, liver and placenta, extraintestinal clinical manifestations may exist. For the first time, we describe in this study in the two sisters muscular as well as cardiac abnormalities that could be related to the reported expression of SARA2 in these tissues. We also evaluated six other patients for potential manifestations of the SARA2 mutation. The creatine phosphokinase levels were increased in all patients [1.5-9.4 x normal (N)] and transaminases were moderately elevated in five of the eight patients (1.2-2.6 x N), probably related to muscle disease rather than to liver dysfunction. A decreased ejection fraction occurred in one patient (40%, N: 60%). The muscle, liver and placental tissues that were examined had no specific abnormalities and, in particular, no lipid accumulation. These results suggest that myolysis and other extraintestinal abnormalities can occur in AD/CMRD and that the clinical evaluation of patients should reflect this.

Asymmetrical regulation of scavenger receptor class B type I by apical and basolateral stimuli using Caco-2 cells.

Cholesterol uptake and the mechanisms that regulate cholesterol translocation from the intestinal lumen into enterocytes remain for the most part unclear. Since scavenger receptor class B type I (SR-BI) has been suggested to play a role in cholesterol absorption, we investigated cellular SR-BI modulation by various potential effectors administered in both apical and basolateral sides of Caco-2 cells. With differentiation, Caco-2 cells increased SR-BI protein expression. Western blot analysis showed the ability of cholesterol and oxysterols in both cell compartments to reduce SR-BI protein expression. Among the n-3, n-6, and n-9 fatty acid families, only eicosapentaenoic acid was able to lower SR-BI protein expression on both sides, whereas apical alpha-linolenic acid decreased SR-BI abundance and basolateral arachidonic acid (AA) raised it. Epidermal growth factor and growth hormone, either in the apical or basolateral medium, diminished SR-BI cellular content, while insulin displayed the same effect only on the basolateral side. In the presence of proinflammatory agents (LPS, TNF-alpha, IFN-gamma), Caco-2 cells exhibited differential behavior. SR-BI was downregulated by lipopolysaccharide on both sides. Finally, WY-14643 fibrate diminished SR-BI protein expression when it was added to the apical medium. Biotinylation studies in response to selected stimuli revealed that regulatory modifications in SR-BI protein expression occurred for the most part at the apical cell surface irrespective of the effector location. Our data indicate that various effectors supplied to the apical and basolateral compartments may impact on SR-BI at the apical membrane, thus suggesting potential regulation of intestinal cholesterol absorption and distribution in various intracellular pools.

Mechanisms of lipid malabsorption in Cystic Fibrosis: the impact of essential fatty acids deficiency.

Transport mechanisms, whereby alimentary lipids are digested and packaged into small emulsion particles that enter intestinal cells to be translocated to the plasma in the form of chylomicrons, are impaired in cystic fibrosis. The purpose of this paper is to focus on defects that are related to intraluminal and intracellular events in this life-limiting genetic disorder. Specific evidence is presented to highlight the relationship between fat malabsorption and essential fatty acid deficiency commonly found in patients with cystic fibrosis that are often related to the genotype. Given the interdependency of pulmonary disease, pancreatic insufficiency and nutritional status, greater attention should be paid to the optimal correction of fat malabsorption and essential fatty acid deficiency in order to improve the quality of life and extend the life span of patients with cystic fibrosis.

New insights into the postnatal growth of infants born 'idiopathic' small for gestational age.

Infants born small for gestational age (SGA) are defined as those with a birth weight and/or birth length below two standard deviations for gestational age. Postnatal catch-up growth is absent in 8-12% of children born SGA and these children achieve adult heights far below their target height. The objectives of our retrospective study were to confirm the prevalence of catch-up growth and to refine the kinetics of the catch-up process. The 'Lyon series' of patients consisted of 179 children with 'idiopathic' SGA, of whom 8% did not achieve catch-up growth (i.e. their height remained below -2 SD throughout the study). The number of days that the mother was hospitalized before delivery was found to be significantly correlated with lack of catch-up growth. Maternal hypertension, term delivery and postnatal parenteral nutrition were also associated with lack of catch-up growth, but the relationship was not significant. In addition, an oscillatory growth velocity pattern was observed in boys and girls born SGA, with alternating periods of growth acceleration and deceleration. This suggests that catch-up growth is regulated in a sophisticated way by the hypothetical 'Somatostat', although further confirmation of this process is needed.

Cytomegalovirus infection may cause ureteral necrosis.

Cytomegalovirus (CMV) infection has protean presentation among immunocompromised patients, but the urinary tract is rarely involved. We report a case of extensive ureteral necrosis in a renal transplant, 12-year-old patient with typical histological feature of CMV inclusions. The role of CMV was confirmed by immunohistochemical analysis and concomitant CMV DNA detection in peripheral blood leukocytes by polymerase chain reaction analysis. CMV infection can, therefore, be regarded as a possible cause of ureteral necrosis in renal transplant recipients.