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Background: Objective tools are needed to improve pain assessment in newborns. The aim of this study was to assess the correlation between the Newborn Infant Parasympathetic Evaluation (NIPE) index and two pain scales during a painful procedure in premature infants.Method: Each baby born at least at 26 weeks of gestational age (GA) undergoing a planned painful procedure in the Neonatal Intensive Care Unit (NICU) was eligible. NIPE index, heart rate variability (HRV) indices and Neonatal Acute Pain scale (DAN) were recorded across three periods: the first at rest 5 min before the painful procedure (T1), the second during it (T2) and the third 3 min after the end of it (T3). The Premature Infant Pain Profile-Revised (PIPP-R) pain scale was recorded at T2.Results: Sixty-four recordings were performed in 29 preterm infants (mean GA = 29.9 ± 4.2 weeks). Twenty-eight tachograms were coupled to NIPE for analysis. We did not find a correlation between the NIPE index and DAN and PIPP-R at the pain time T2. Between T1 and T2, heart rate was higher (159 ± 16 vs. 169 ± 12, p < 0.001). Considering the linear HRV indices, we did not observe a modification in parasympathetic or sympathetic activity, while for the nonlinear HRV indices (H exponent, Approximate and conditional Entropy), a significant change towards a loss of physiological chaotic cardiac behaviour was detected.Conclusions: The NIPE index seems to be not reliable to assess acute pain in the preterm infant, but other HRV indices could be explored as additional tools next to pain scales in NICUs.Significance: The NIPE monitor was developed for objective pain assessment in neonates based on HFnu variations, but it does not seem reliable enough for assessing acute pain in real time in preterm neonates. Pain assessment in preterm babies still relies on pain scales. [ABSTRACT FROM AUTHOR]

Objective: Despite recent progress in caring for patients born with esophageal atresia (EA), undernutrition and stunting remain common. Our study objective was to assess nutritional status in the first year after birth with EA and to identify factors associated with growth failure., Study Design: We conducted a population-based study of all infants born in France with EA between 2010 and 2016. Through the national EA register, we collected prenatal to 1 year follow-up data. We used body mass index and length-for-age ratio Z scores to define patients who were undernourished and stunted, respectively. Factors with P < 0.20 in univariate analyses were retained in a logistic regression model., Results: Among 1,154 patients born with EA, body mass index and length-for-age ratio Z scores at 1 year were available for about 61%. Among these, 15.2% were undernourished and 19% were stunted at the age of 1 year. There was no significant catch-up between ages 6 months and 1 year. Patients born preterm (41%), small for gestational age (17%), or with associated abnormalities (55%) were at higher risk of undernutrition and stunting at age 1 year ( P < 0.05). Neither EA type nor surgical treatment was associated with growth failure., Conclusion: Undernutrition and stunting are common during the first year after birth in patients born with EA. These outcomes are significantly influenced by early factors, regardless of EA type or surgical management. Identifying high-risk patient groups with EA (i.e., those born preterm, small for gestational age, and/or with associated abnormalities) may guide early nutritional support strategies., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential of interest., (Copyright © 2022 Depoortere, Lapillonne, Sfeir, Bonnard, Gelas, Panait, Rabattu, Guignot, Lamireau, Irtan, Habonimana, Breton, Fouquet, Allal, Elbaz, Talon, Ranke, Abely, Michel, Lirussi Borgnon, Buisson, Schmitt, Lardy, Petit, Chaussy, Borderon, Levard, Cremillieux, Tolg, Breaud, Jaby, Grossos, De Vries, Arnould, Pelatan, Geiss, Laplace, Kyheng, Nicolas, Aumar and Gottrand.)

Objective: To identify predictors of and factors associated with the performance of antireflux surgery during the first year of life in children born with esophageal atresia., Study Design: All patients were included in a French registry for esophageal atresia. All 38 multidisciplinary French centers completed questionnaires about perinatal characteristics and one-year outcome for children born with esophageal atresia., Results: Of 835 infants with esophageal atresia born in France from 2010 to 2014, 682 patients, excluding those with long-gap esophageal atresia, were included. Three patients had type I, 669 had type III, and 10 had type IV esophageal atresia. Fifty-three children (7.8%) received fundoplication during the first year of life. The median age at the time of the end-to-end esophageal anastomosis was 1.1 day (range 0-15). Multivariate analysis identified three perioperative factors that predicted the need for early antireflux surgery: anastomotic tension (P = .004), associated malformations (P = .019), and low birth weight (P = .018). Six other factors, measured during the first year of life, were associated with the need for antireflux surgery: gastroesophageal reflux (P < .001), anastomotic stricture (P < .001), gastrostomy (P < .001), acute life-threatening event (P = .002), respiratory complications (P = .045), and poor nutritional status (P < .001)., Conclusions: Gastroesophageal reflux disease, low birth weight, poor nutrition, and surgical anastomosis difficulties predicted the performance of antireflux surgery in the first year of life in infants with esophageal atresia., (Copyright © 2019 Elsevier Inc. All rights reserved.)

ARC syndrome (arthrogryposis - renal dysfunction - cholestasis) is a rare lethal multisystemic autosomal recessive disease. A newborn of consanguineous parents of Algerian descent presented cholestatic jaundice, dehydration, and Fanconi syndrome at 10 days of life. The blood smear showed a very characteristic gray appearance of platelets. A homozygous mutation was evidenced in the VPS33B gene. This gene codes for a protein involved in trafficking of intracellular vesicles. The mutation (c.604-2A>G) present in the heterozygous state in the parents affects an invariant base of the splice acceptor site and to our knowledge has not been reported yet. This child died at the age of 3 months. Prenatal diagnosis was offered to the family; another pregnancy was carried to completion and a girl was born without the disease. The combination of cholestasis and proximal tubulopathy should suggest the diagnosis in a newborn with orthopedic problems. A blood smear greatly facilitates diagnosis., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Congenital short-bowel syndrome (CSBS) is a rare neonatal pathology associated with poor prognosis and high mortality rate. We describe a newborn presenting CSBS intestinal malrotation and chronic intestinal pseudo-obstruction syndrome (CIPS), compound heterozygous for two previously unreported heterozygous mutations in Coxsackie and adenovirus receptor-like membrane protein (CLMP) gene, one in intron 1 (c.28+1G>C), the other on exon 4 (c502C>T, p.R168X). Both mutations are predicted to be pathogenic, leading to impaired splicing and the appearance of a premature stop codon, respectively. Our case is remarkable in that it concerns two heterozygous truncating mutations associated with a good clinical prognosis with a favorable cerebral and gastrointestinal outcome and a substantial enteral input at 8 months of age, despite a small intestine measuring only 35cm., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Severe combined immune deficiencies (SCIDs) are a heterogeneous group of severe cellular immunodeficiencies. Early diagnosis is essential to allow adapted care before life-threatening systemic infections or complications associated with live vaccines. Adenosine deaminase 1 deficiency (ADA1) is an inborn error of metabolism leading to severe lymphopenia and characteristic bone lesions. Herein, we present the typical case of a child in whom ADA SCID was diagnosed at 2 months of life, revealed by lung involvement and extreme lymphopenia. Immune restoration in terms of peripheral lymphocyte count with enzyme replacement therapy, namely pegylated bovine ADA, is satisfactory so far. The search for a compatible donor is underway. Correcting the genetic defect by gene transfer is also being considered. The phenotype of this very rare condition is described. A severe peripheral lymphopenia in a young child is a finding of utmost importance for the diagnosis of a primary cellular immunodeficiency., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Primary Objective: To estimate the incidence of acute gastroenteritis (AGE) and rotavirus acute gastroenteritis (RVAGE) in children less than 5 years of age seeking medical care in primary care, emergency department, and hospital settings., Secondary Objectives: To compare the clinical profile of RVAGE and non-RVAGE and to describe the distribution of RV serotypes among RVAGE cases., Methods: A prospective primary care, emergency ward and hospital-based observational study was conducted during 1 year in a selected city of France with 250,000 inhabitants. Children less than 5 years of age presenting with symptoms of AGE were included. Rotavirus was identified using an Elisa test in stools., Results: The estimated annual incidence of RVAGE was 1.56% for AGE and 0.87% for RVAGE in hospital, 5.87% for AGE and 2.65% for RVAGE in emergency-wards, 7.39% for AGE and 1.45% for RVAGE in primary care. Total incidence was 14.82% for AGE and 4.96% for RVAGE among children less than 5 years of age. RVAGE were more clinically severe than the AGE: dehydration (26.8% vs. 14.7%, p<0.0001), vomiting 84.9% vs. 60.9%, p<0.0001), fever (74.3% vs. 44.4%, p<0.0001), lethargy (84.9% vs. 70.2%, p<0.0001). G9 serotype was the most frequent serotype encountered (54.7%) during the study period followed by G3 serotype (33.6%) and G2 serotype (7.9%)., Conclusion: In this study, RVAGE, caused by serotypes G9 and G3, represented about 1/3 of AGE and were more severe than non-RV AGE with twice as high dehydration rate. These results underline the need for continued promotion on the use of oral rehydration fluids and provide some arguments on the benefits of vaccination against rotavirus and also permanent virological monitoring of circulating serotypes.