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Background The most severely ill neonates and infants with AKI who need kidney replacement therapy have had to rely upon peritoneal dialysis, or adaptations of veno-venous continuous kidney replacement therapy (CKRT) devices for adults. Data from the Prospective Pediatric CRRT (ppCRRT) registry observed children 10 kg (44% vs. 64%, p < 0.001). A CKRT device designed specifically for small children could improve outcomes. The Cardio-Renal Pediatric Dialysis Emergency Machine (CARPEDIEM[TM]) is specifically dedicated to providing CKRT for newborns and small infants. Methods We performed a retrospective cohort analysis comparing patient severity of illness and outcomes between the ppCRRT and CARPEDIEM registries, involving 6 Italian pediatric intensive care units. Thirty-eight subjects from the CARPEDIEM registry and 84 subjects from the ppCRRT registry < 10 kg were screened for comparison. We compared patient outcomes with a weight-matched cohort (< 5 kg) of 34 patients from the CARPEDIEM registry and 48 patients from the ppCRRT registry. Results The ppCRRT subjects had higher rates of vasoactive medication at CKRT initiation. Survival to CKRT termination was higher for CARPEDIEM subjects (33/34 vs. 21/48, p < 0.0001). Multivariable logistic regression showed that CARPEDIEM registry cohort was the only variable to retain an association with survival to CKRT discontinuation. Conclusions We suggest children receiving CKRT using CARPEDIEM have excellent survival. Our data should be interpreted with caution given the retrospective comparison across two eras more than a decade apart. Graphical abstract, Author(s): Stuart L. Goldstein [sup.1] , Enrico Vidal [sup.2] , Zaccaria Ricci [sup.3] , Fabio Paglialonga [sup.4] , Licia Peruzzi [sup.5] , Mario Giordano [sup.6] , Nicola Laforgia [sup.6] , [...]

Background: Exclusive breastfeeding during postpartum hospitalization is very important for ensuring the success of breastfeeding at home. The aim of the study is to determine if the on-site nurse in rooming in improves exclusive breastfeeding ratio. Methods: We conducted a prospective observational cohort study to evaluate exclusive breastfeeding during the first three months of life in two Neonatology Units in the South of Italy with different hospital settings: Ente Ecclesiastico Miulli of Acquaviva delle Fonti with on-site nurse h24 (on-site group) and Policlinico of Bari with nurse available on call h24 from Neonatology Unit (on-call group). Results: A total of 564 mother-baby dyads were admitted from 3 January to 31 March 2018 (299 in on-site group and 265 in on-call group). In the overall population, exclusive breastfeeding rate was 76.4% at 90-days, confirming the role of nurse and rooming in, independently of modality of setting. Considering the way of delivery, in infants from cesarean section there were higher rates for exclusive breastfeeding at 30 and 90 days of life in on-site group. Conclusions: We can assume that the presence of a nurse h24 could better identify breastfeeding problems. Our study suggests the role of on-site nurse during rooming in to encourage exclusive breastfeeding until three months of life in mothers who underwent caesarean section. [ABSTRACT FROM AUTHOR]

Background: Positional plagiocephaly is an asymmetrical flattened skull deformity whose incidence increased significantly in the last decades. Osteopathic treatment has been suggested to tackle early deformational sequences, in order to ensure the correct development of the child. The aim of the study was to assess the effectiveness of osteopathic treatment of positional cranial deformities in infants. Methods: Retrospective observational study carried out at the Section of Neonatology and Neonatal Intensive Care Unit of the Department of Interdisciplinary Medicine of University of Bari, Italy in collaboration with a specialized pediatric osteopath. Results: 424 infants were enrolled. Isolated positional plagiocephaly affected the vast majority of infants (n. 390, 91.98%); 34 patients (8.02%) were diagnosed with positional brachycephaly. Both infant groups (positional plagiocephaly and positional brachycephaly) had a median severity score of 3 (IQR: 3 – 3 and 2 – 3, respectively) and benefited from a median of 3 osteopathic sessions (IQR 3–4 and 2–4, respectively). Higher severity scores of positional asymmetries were significantly more common in preterm neonates (Pearson chi2: 11.58; p-value: 0.021) and in males (Pearson chi2: 10.06; p-value: 0.039). Conclusions: Significant improvements in positional cranial deformations of children were obtained after only five osteopathic treatments provided in the first months of life. The osteopathic treatment could positively impact the clinical history of patients with positional plagiocephaly and positional brachycephaly. Implication for practice: • Positional plagiocephaly is increasingly common among infants and may cause moderate to severe neurodevelopmental adverse effects. • Osteopathic treatment may tackle early deformational sequences, in order to ensure the correct development of the child. • Our study reveals that cranial asymmetry of infants with positional plagiocephaly is significantly reduced after only five osteopathic treatments provided in the first months of life. • Osteopathic treatment should be offered as a first line approach to young infants diagnosed with positional plagiocephaly. [ABSTRACT FROM AUTHOR]

ACE2 is a mono-carboxypeptidase with remarkable vasculo-protective properties, and its expression in the human placenta plays a central role in blood pressure homeostasis and fetal perfusion. Therefore, an alteration in the placental expression of ACE2 could be responsible for reduced placental perfusion and infantile hemangioma (IH) development. Study placentae were collected from patients affected by IHs who were referred to our Dermatology Clinic from 2016 to 2022, while control placentae were randomly collected while matching cases for gestational age. Immunohistochemical investigations were performed with a recombinant anti-ACE2 rabbit monoclonal antibody. A total of 47 placentae were examined, including 20 study placentae and 27 control ones. The mean placental weight was significantly lower in the study group (380.6 g vs. 502.3 g; p = 0.005), while subclinical chorioamnionitis occurred more frequently in the study group (20% vs. 0%, p = 0.03). The mean ACE2 expression was dramatically lower in the study group (χ2 = 42.1 p < 0.001), and the mean placental weight was significantly lower when ACE2 was not expressed compared to the 25–75% and >75% classes of expression (p < 0.05). This study demonstrated that ACE2, as a marker for tissue hypoxia, is dramatically hypo-expressed in placentae belonging to mothers who delivered one or more babies with IH compared to the controls. [ABSTRACT FROM AUTHOR]

Multiple acyl-CoA dehydrogenase deficiency (MADD) is a rare inborn error of metabolism affecting fatty acid and amino acid oxidation with an incidence of 1 in 200,000 live births. MADD has three clinical phenotypes: severe neonatal-onset with or without congenital anomalies, and a milder late-onset form. Clinical diagnosis is supported by urinary organic acid and blood acylcarnitine analysis using tandem mass spectrometry in newborn screening programs. MADD is an autosomal recessive trait caused by biallelic mutations in the ETFA, ETFB, and ETFDH genes encoding the alpha and beta subunits of the electron transfer flavoprotein (ETF) and ETF-coenzyme Q oxidoreductase enzymes. Despite significant advancements in sequencing techniques, many patients remain undiagnosed, impacting their access to clinical care and genetic counseling. In this report, we achieved a definitive molecular diagnosis in a newborn by combining whole-genome sequencing (WGS) with RNA sequencing (RNA-seq). Whole-exome sequencing and next-generation gene panels fail to detect variants, possibly affecting splicing, in deep intronic regions. Here, we report a unique deep intronic mutation in intron 1 of the ETFDH gene, c.35-959A>G, in a patient with early-onset lethal MADD, resulting in pseudo-exon inclusion. The identified variant is the third mutation reported in this region, highlighting ETFDH intron 1 vulnerability. It cannot be excluded that these intronic sequence features may be more common in other genes than is currently believed. This study highlights the importance of incorporating RNA analysis into genome-wide testing to reveal the functional consequences of intronic mutations. [ABSTRACT FROM AUTHOR]

Group B Streptococcus (GBS; Streptococcus agalactiae) is a notable cause of sepsis and meningitis in infancy (1). Intrapartum antimicrobial prophylaxis (IAP) has substantially reduced the rates of early-onset disease (EOD; [...]

Background Providing extracorporeal renal support to neonates and infants involves a number of technical and clinical issues, possibly discouraging early utilization. This report aims to describe a multicenter experience of continuous kidney replacement therapy (CKRT) delivery to small infants using a device specifically designed for this age group. Methods A retrospective cohort analysis of all patients treated with the Carpediem[TM] machine (Bellco-Medtronic, Mirandola, Italy) in 6 centers between June 2013 and December 2016. Results Twenty-six neonates and small infants received 165 CKRT sessions in convective modality. Median age at neonatal intensive care unit admission 1 day (IQR 1-11), median body weight 2.9 kg (IQR 2.2-3.6). Median circuit duration 14 h (IQR 10-22), with delivered/prescribed time ratio of 84%. CKRT was conducted using 4 Fr (27%), 5 Fr (35%), 6.5 Fr (11%), and 7 Fr (3%) vascular access, and with umbilical and peripheral accesses (11% each) allowing overall median blood flow of 4.5 ml/kg/min (IQR 3.4-6) and median effluent flow rate 35 ml/kg/h (IQR 28-42). Circuits were primed with normal saline in 58% of treatments, colloids in 31%, and packed red blood cells in 11%. No serious adverse events directly related to machine application were reported by any center. Twenty-five (96%) patients survived their CKRT course and 13 patients (50%) survived to ICU discharge. Conclusions CKRT in neonates was easy to initiate and conduct when performed with small central vascular accesses coupled with this device. A dedicated technology for infant CKRT delivery enables patients to be safely treated avoiding technical complications., Author(s): Francesco Garzotto [sup.1] , Enrico Vidal [sup.2] , Zaccaria Ricci [sup.3] , Fabio Paglialonga [sup.4] , Mario Giordano [sup.5] , Nicola Laforgia [sup.6] , Licia Peruzzi [sup.7] , Massimo [...]

Background: More than a quarter of children who are affected by severe acute undernutrition reside in Sub-Saharan Africa. Incorrect feeding practices have a negative impact on a child's health in both the short and the long term, and the interval from conception to two years is the most critical for the development of undernutrition-related complications. These first 1000 days of life also represent an "opportunity window" for early interventions, hence, having a clear insight into dietary habits and the determinants of diet quality is fundamental to improving nutritional counseling practices. Objectives: To investigate incorrect feeding practices, dietary diversity determinants and nutritional status in children aged 6–23 months. Methods: Prospective quali-quantitative observational study conducted at the Missionary Catholic Hospital of Chiulo (Angola) from March to April 2023. Results: Of 250 children, global acute malnutrition affected 25.2% and was associated with starting complementary feeding at <4 months of age (p = 0.007) and not achieving the minimum meal frequency (p < 0.0001). Minimum dietary diversity was found in 11.2%, minimum meal frequency was experienced by 72.8%, and the minimum acceptable diet was found in 11.2% of participants. The minimum dietary diversity was reached only by households with access to food from five or more major food groups (p = 0.007) or the money to buy food from five or more major food groups (p = 0.008) and was higher in households where the householder had a higher educational level (p = 0.002). Regarding the determinants linked to family traditions and beliefs, the main religion-associated beliefs concerned the impurity of pork (n = 25) and fish (n = 8), while eggs (n = 19) and cow milk (n = 8) were the main food types that were deemed harmful for children. Conclusions: Although some factors (economic and religious) may be difficult to overcome, other factors linked to erroneous beliefs (dangerous foods) or incorrect feeding practices (early weaning and an incorrect frequency of meals) can be targeted, to improve the effectiveness of nutritional counseling practices. [ABSTRACT FROM AUTHOR]

The autophagy process recycles dysfunctional cellular components and protein aggregates by sequestering them in autophagosomes directed to lysosomes for enzymatic degradation. A basal level of autophagy is essential for skeletal muscle maintenance. Increased autophagy occurs in several forms of muscular dystrophy and in the merosin-deficient congenital muscular dystrophy 1A mouse model (dy3k/dy3k) lacking the laminin-α2 chain. This pilot study aimed to compare autophagy marker expression and autophagosomes presence using light and electron microscopes and western blotting in diagnostic muscle biopsies from newborns affected by different congenital muscular myopathies and dystrophies. Morphological examination showed dystrophic muscle features, predominance of type 2A myofibers, accumulation of autophagosomes in the subsarcolemmal areas, increased number of autophagosomes overexpressing LC3b, Beclin-1 and ATG5, in the merosin-deficient newborn suggesting an increased autophagy. In Duchenne muscular dystrophy, nemaline myopathy, and spinal muscular atrophy the predominant accumulation of p62+ puncta rather suggests an autophagy impairment. [ABSTRACT FROM AUTHOR]

Background: Bronchiolitis is a major cause of hospitalization in infants, particularly in the first six months of life, with approximately 60–80% of admissions due to respiratory syncytial virus (RSV) infection. Currently, no prophylactic options are available for healthy infants. The present study aimed at describing the demographic, clinical, and epidemiological characteristics of infants hospitalized for bronchiolitis in the Apulia region of Italy in 2021. Methods: From January to December 2021, data on children aged 0–12 months admitted for bronchiolitis in nine neonatal or pediatric units covering 61% of pediatric beds of hospitals in the Apulia region of Italy were analyzed. Demographic data, comorbidities, need for oxygen support, length of hospital stay, palivizumab administration, and outcomes were collected. For the purpose of the analysis, patients were divided into those aged 0–3 months and > 3 months. A multivariate logistic regression model was used to explore associations between the need for oxygen support and sex, age, comorbidities, history of prematurity, length of hospital stay, and palivizumab administration. Results: This study included 349 children aged 0–12 months admitted for bronchiolitis, with a peak of hospitalization in November (7.4 cases/1,000 children). Of these patients, 70.5% were RSV positive, 80.2% were aged 0–3 months, and 73.1% required oxygen support. Moreover, 34.9% required observation in the sub-intensive care unit, and 12.9% in the intensive care unit. Of the infants who required intensive care, 96.9% were aged 0–3 months and 78.8% were born at term. Three patients required mechanical ventilation and one, who required Extra Corporeal Membrane Oxygenation, died. Children aged 0–3 months were more likely to show dyspnea, need oxygen support, and have a longer hospital stay. Conclusions: The present study showed that almost all of the children who required intensive care support were aged ≤ 3 months and most were born at term. Therefore, this age group remains the highest risk group for severe bronchiolitis. Preventive measures such as single-dose monoclonal antibody immunoprophylaxis, and maternal and childhood vaccination against RSV, may reduce the high public health burden of bronchiolitis. [ABSTRACT FROM AUTHOR]