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IntroductionNeurologic manifestations may be presenting signs of celiac disease (CD). Pediatric-onset paroxysmal non-epileptic events (PNEEs) are not included among them.Cases presentationWe report the case of two children who presented with ocular PNEEs in association with mild symptoms evocative for CD, who were subsequently diagnosed with CD and experienced regression of PNEEs on a gluten-free diet. Data from 12 patients undergoing neurological evaluation in 2019–2023 for ocular PNEEs were reviewed: 3 (25%) had a subsequent diagnosis of CD.ConclusionsOcular PNEEs could be a presenting manifestation of CD. In the diagnostic work-up of PNEEs, screening for CD could be included, both to avoid unnecessary tests and to promptly start a gluten-free diet, which might lead to a favorable clinical response.

Racial and ethnic disparities have long been studied in the delivery of healthcare. One such avenue is acute pediatric appendicitis, which continues to be an area of significant and continual research. Because of its routine clinical presentation and standardized management, acute pediatric appendicitis serves as an appropriate proxy for studying discrepancies in healthcare. Our review therefore aims to comprehensively highlight the various dimensions of its clinical management subject to disparities, their collective clinical impact, and future recommendations to mitigate it.

Long-term follow-up for individuals with hypospadias remains a critical area of need, yet evidence-based guidelines for such follow-up are lacking, and the role of involvement of relevant experts is not yet established. Using our hypospadias-specific health-related quality of life conceptual framework and a subsequent qualitative study of prepubertal males and parents of males with hypospadias, we identified potential priorities for long-term follow-up of youth with hypospadias. Using thematic codes from our patient and parent interviews, we searched PubMed for relevant articles and identified the specialties represented by all the authors of these articles. Our search strategy revealed consistent expertise across HRQOL themes and subthemes, including pediatric and adult urology, health psychology, psychiatry, endocrinology, genetics, and social work. Communication experts, as well as patients and families, were also represented in our literature search. Using these findings, we compiled a comprehensive list of potential stakeholders to inform the development of holistic care guidelines for individuals with hypospadias. By engaging these stakeholders, we aim to develop consensus-based, long-term follow-up guidelines and tools to address the evolving physical and psychosocial needs of people with hypospadias over a lifetime. The use of qualitatively derived thematic codes to search for relevant literature is an accessible approach to identifying relevant stakeholders. These findings underscore the importance of involving diverse, multidisciplinary expertise to ensure comprehensive, patient-centered care in complex genitourinary conditions.

BackgroundIdiopathic intracranial hypertension (IIH) is defined as elevated intracranial pressure and consequent symptoms (mainly headache and visual deterioration) occurring in the absence of secondary causes. Surgical and interventional radiology procedures should be considered for refractory IIH and mainly include cerebrospinal fluid (CSF) diversion techniques, optic nerve sheath fenestration (ONSF), and venous sinus stenting (VSS). Our study aims to review the current literature on the application of these techniques in clinical practice.MethodsA systematic literature review on the surgical and interventional radiology treatment of IIH was conducted, focusing on ONSF, VSS, and CSF diversion techniques. According to PRISMA guidelines, all reports published in PubMed in the last 30 years (1993–2023) were considered, and among 722 papers, 48 were included in the present study, resulting in a total study population of 454 children or adolescents (11 months–17 years old).ResultsAmong 454 patients, 193 underwent an invasive approach, divided into CSF diversion (115/193), ONSF (65/193), VSS (11/193), cranial subtemporal decompression (8/193), and internal cranial expansion (9/193). Sixteen of the 193 patients (8%) required reintervention due to relapsing symptoms or surgical complications, particularly those who underwent CSF diversion. Furthermore, 9/115 required shunt revision due to shunt obstruction or malfunction. We extracted data on the outcome of each procedure: of the 193 patients, 71 experienced a positive outcome with symptom resolution or improvement, while 27 demonstrated a negative outcome.Discussion and conclusionsSevere and refractory cases of IIH are eligible for invasive treatments. CSF diversion is the most frequently used technique, despite its high failure risk and need for reintervention. ONSF has shown good results in terms of outcome and safety, particularly in children with visual symptoms. VSS is the most recent approach, indicated in children with stenosis of the venous sinus. In our study population, VSS demonstrated good results in terms of symptom resolution and need for reintervention, but its use remains limited to a few centers.Systematic Review Registrationhttps://www.crd.york.ac.uk/, PROSPERO (CRD42024504244).

IntroductionFluid accumulation, presently defined as a pathologic state of overhydration/volume overload associated with clinical impact, is common and associated with worse outcomes. At times, deresuscitation, the active removal of fluid via diuretics or ultrafiltration, is necessary. There is no consensus regarding deresuscitation in children admitted to the pediatric intensive care unit. Little is known regarding perceptions and practices among pediatric intensivists and nephrologists regarding fluid provision and deresuscitation.MethodsCross-sectional electronic survey of pediatric nephrologists and intensivists from academic societies in the United States designed to better understand fluid management between disciplines. A clinical vignette was used to characterize the perceptions of optimal timing and method of deresuscitation initiation at four timepoints that correspond to different stages of shock.ResultsIn total, 179 respondents (140 intensivists, 39 nephrologists) completed the survey. Most 75.4% (135/179) providers believe discussing fluid balance and initiating fluid deresuscitation in pediatric intensive care unit (PICU) patients is “very important”. The first clinical vignette time point (corresponding to resuscitation phase of early shock) had the most dissimilarity between intensivists and nephrologists (p = 0.01) with regards to initiation of deresuscitation. However, providers demonstrated increasing agreement in their responses to initiate deresuscitation as the clinical vignette progressed. Compared to intensivists, nephrologists were more likely to choose “dialysis or ultrafiltration” as a deresuscitation method during the optimization [10.3 vs. 2.9% (p = 0.07)], stabilization [18.0% vs. 3.6% (p

IntroductionHigh-intensity interval training (HIIT) is an effective strategy for improving a variety of health and fitness outcomes within school settings. Incorporating HIIT into existing physical activity opportunities appears practically feasible, yet the process evaluation and effectiveness of this strategy needs to be further evaluated. Therefore, a PRO-HIIT intervention will be conducted to evaluate the effectiveness of a 12-week school-based HIIT intervention on cardiorespiratory fitness, physical activity, body composition, muscular strength, bone health, cognitive function, wellbeing and academic performance among 12–13-year-olds.Methods and analysisEight classes of year 7 students (12–13-year-olds) from a secondary school in Ningbo, China, will be recruited and randomly allocated into an intervention or control group. While the control group maintains their usual activities, a 6 to 10 min HIIT session will be embedded in the physical education or physical activity lessons five days a week for 12 weeks for the intervention group. Training workshops will be conducted for participants, teachers, and research staff for facilitating the intervention. Outcome data will be collected at three time points: pre- and post-intervention, and two months (summer holiday) upon completion of the intervention. Linear mixed models will be used to analyse the impact of groups (intervention and control), timepoints (pre-, post- and two-month after intervention) and group by time interactions. The implementation process of the intervention will be evaluated using a process evaluation framework.Ethics and disseminationEthics approval is obtained from the Ningbo University Ethics Committee (TY2024002). Results from PRO-HIIT study will be disseminated via peer-reviewed journals, scientific conferences as well as local education system. The study protocol has been retrospectively registered on ClinicalTrials.gov Protocol Registration and Results System (NCT06374732), https://clinicaltrials.gov/study/NCT06374732.

AimTo provide a risk prediction for bronchopulmonary dysplasia (BPD) in premature infants under the new diagnostic criteria and establish a prediction model.MethodsIn this study, we retrospectively collected case data on preterm infants admitted to the NICU from August 2015 to August 2018. A lasso analysis was performed to identify the risk factors associated with the development of BPD. A nomogram predictive model was constructed in accordance with the new diagnostic criteria for BPD.ResultA total of 276 preterm infants were included in the study.The incidence of BPD under the 2018 diagnostic criteria was 11.2%. Mortality was significantly higher in the BPD group than the non-BPD group under the 2018 diagnostic criteria (P

ObjectiveNational Institute for Clinical Excellence (NICE) guidance for the management of neonatal sepsis recommends a first-line antibiotic regimen containing an aminoglycoside (gentamicin). Aminoglycoside exposure causes sensorineural hearing loss in individuals with a specific mitochondrial genetic variant (m.1555A>G). This variant can be detected promptly (in G genetic variant at risk of iatrogenic hearing loss. There needs to be integration of point-of-care genetic testing within the neonatal septic screening pathway.

IntroductionConcurrent alterations in the metabolic profile and thyroid dysfunction, including non-thyroidal illness syndrome (NTIS) has been reported in multisystem inflammatory syndrome in children (MIS-C). Considering the influence of thyroid hormones (TH) on lipid metabolism, we explored the relationship between thyroid function and the atherogenic lipid profile in children with MIS-C at admission and during a 12-month follow-up.Patients and methodswe considered children admitted for MIS-C. Total and HDL cholesterol, triglycerides (TG), fasting plasma glucose, fasting plasma insulin as well as free T3 (FT3), free T4 (FT4), and TSH were assessed at diagnosis within 24 h of admission and during follow-up. TG/HDL ratio, no-HDL/HDL ratio and atherogenic index of plasma was also considered as atherogenic risk markers.Resultswe monitored 56 children. On admission, pathological levels of FT3, FT4, TSH, TG, TC, HDL, TG/HDL ratio, no-HDL/HDL ratio, and AIP were detected. Correlation analyses revealed associations between FT3, FT4, and lipid markers and TSH with TG. During monitoring, while complete restoration of TH balance was achieved at 12 months, some patients still exhibited an altered lipid profile, without correlation between thyroid function and lipid markers.Conclusionswe supported a relationship between thyroid function and an atherogenic lipid profile in children with MIS-C. This may result from interactions between adaptive and innate metabolic responses and genetic predisposition. Elucidating the relationship between TH and metabolic pathways during infections could help identify new biomarkers to prevent acute and fatal outcomes, improving patient prognosis and protecting long-term health.

IntroductionThis study aims to estimate the validity and reliability of sound transmission tests using a tuning fork and stethoscope compared with the usual clinical procedures for the diagnosis of developmental dysplasia of the hip in newborns from the state of Guanajuato, Mexico.MethodologyThis is a cross-sectional study of newborns at the Guanajuato General Hospital of the Institute of Public Health from Guanajuato. The population under study comprised newborns born from April to May 2023. All parents of newborns were invited to participate. The Ortolani, Barlow, Peter–Baden, and sound transmission tests were performed with a tuning fork and stethoscope three times by two observers. Ultrasonography using the Graf technique was also applied to both hips. We evaluated the validity and repeatability of clinical procedures and sound transmission tests against ultrasonography as the gold standard. We calculated sensitivity, specificity, and predictive values for validity and Cohen's kappa for repeatability.ResultsThe sample consisted of 100 newborns (56% male and 44% female); 65% born by vaginal delivery. Sound transmission test results for sensitivity, specificity, the positive predictive value, and the negative predictive value were 87.88%, 95.81%, 80%, and 97.53%, respectively. The intra-observer kappa was 0.89 (95% CI = 0.80–0.97) and the inter-observer kappa was 0.85 (95% CI = 0.73–0.97). The validity and repeatability of the Ortolani, Barlow, and Peter–Baden tests were low compared with the sound transmission test.ConclusionThe sound transmission test using a tuning fork and stethoscope is helpful for the diagnosis of developmental dysplasia of the hip.

BackgroundPreventing neonatal mortality and morbidity in developing countries such as Ethiopia requires improved practices and knowledge among healthcare providers. Several studies have been conducted in Ethiopia, but the overall level has not been estimated based on essential newborn care practices, knowledge of health care providers, and associated factors. Therefore, the objective of this review is to assess the overall practice and knowledge of essential newborn care and associated factors among healthcare providers in Ethiopia.Methods and materialsOnly articles published in English were included in this review. Medline/PubMed, Web of Science, Google Scholar, EMBASE, and CINAHL, Scopus, Ethiopian University Repository Online, and the Cochrane Library are the main databases. The review included cross-sectional studies written in English that met the inclusion requirements. Using a random-effects model, the overall practice and knowledge level was estimated. Additionally, funnel plots and Eggers’ test were used to assess publication bias. STATA version 14 was used to perform all statistical analysis.ResultsThis review included 15 studies involving 3,210 health care providers in Ethiopia. In Ethiopia, overall health care providers had a good practice and knowledge level of essential newborn care of 57.38% [95% CI (49.56; 65.20); I2 = 95.3%, P

Aim of the studyTo evaluate the clinical characteristics and treatment efficacy of patients with severe primary IGF-1 deficiency (PSIGFD) using a recombinant IGF-1 (rhIGF-1).Objectives of the studyTo examine the clinical characteristics of patients with PSIGFD before starting treatment with a rIGF-1. To assess the height changes in patients with PSIGFD, before and after treatment with a rhIGF-1. To analyze the clinical characteristics, side effect frequency, and treatment efficacy with a rhIGF-1 analog in patients with PSIGFD.MethodsA retrospective analysis was conducted on patients with PSIGFD treated with the rhIGF-1 (mecasermin). Data were collected from patients’ medical records, focusing on the impact of treatment on their growth and monitoring any side effects.ResultsThe study showed that treatment with rhIGF-1 positively affects growth rate, especially in the first years of treatment. However, the growth rate decreases over time. The change in height from the beginning to the end of the treatment was 0.76 ± 0.64 SD, with the first quartile at 0.29 SD and the third quartile at 1.14 SD. During the treatment period, patients’ average body mass increased by 0.37 ± 1.35 SD, with the first quartile at −0.33 SD and the third quartile at 0.92 SD. Side effects occurred in 50% of patients, with 40% of patients treated with rhIGF-1 experiencing hypoglycemia during treatment.ConclusionsTreatment with rhIGF-1 is effective in treating patients with PSIGFD, causing significant improvement in growth, but requires continuous monitoring and treatment adjustment.

BackgroundResearch on how the physical activity home environment affects fundamental movement skills (FMS) in preschool children in China is limited. However, the role of moderate-to-vigorous physical activity (MVPA) in this relationship is still unclear. This study aims to analyze gender differences in FMS, explore associations between the physical activity home environment, MVPA, and FMS, and investigate MVPA's mediating role in these relationships.MethodsWe recruited 169 preschool children (95 boys, 74 girls; mean age 4.9 years) from four kindergartens in Hengyang, China. The Family Environment Scale on Motor Development for Preschool Urban Children (FESMPD) assessed physical activity home environment. Objective measurement of MVPA used ActiGraph wGT3-BT accelerometers. The Test of Gross Motor Development-3 (TGMD-3) evaluated FMS. Statistical analyses were conducted using the PROCESS macro in SPSS, with sociodemographic variables as controls.ResultsBoys exhibited significantly higher levels of MVPA, parenting style, locomotor skills, and object control skills compared to girls (P

Congenital portosystemic shunts (CPSS) are rare pediatric vascular malformations characterized by abnormal development of the portal vein, which is attributed to incomplete embryonic remodeling of the hepatic and surrounding vasculature. CPSS manifests in two main forms: intrahepatic and extrahepatic. This study details the management of a pediatric patient diagnosed with Congenital Intrahepatic Portosystemic Shunt (CIPS) who was referred to our institution. By using a computer-assisted surgical system, the right hepatectomy was successfully performed, guided by precise intraoperative navigation based on three-dimensional reconstructions of enhanced CT imagery. The patient exhibited a favorable postoperative recovery trajectory, with the absence of complications or recurrence throughout the monitoring period.

BackgroundDespite the critical role of the clavicle in understanding growth and development in early childhood, there remains a notable paucity of comprehensive studies investigating clavicle growth patterns during this crucial period. This hinders our ability to establish normative growth parameters during these early life stages. Our study sought to measure clavicle dimensions and subsequently construct growth curves spanning from preterm infants to toddlers up to the age of 6 years by measuring routine chest radiographs. Differences between both sides of the body and between the sexes should be analysed. This aimed to provide a nuanced understanding of clavicle growth dynamics and offering a foundation for the establishment of normative values in this understudied context. In this retrospective study, children aged 23 weeks of gestation to 6 years who underwent a chest radiography between January 2010 and June 2020 were enrolled. A total of 5.311 potential radiographs was screened. Clavicle length and width were measured in all radiographs using the Centricity™ Universal Viewer. Statistical analysis was performed using SPSS®.Results1.340 images met the quality criteria to be included in our study. The growth curves of clavicle lengths and widths showed a steadily increasing trend with age. Inclusion of premature infants in the age group of one month resulted in a decrease in this age group. Significant differences between both sides of the body and between the sexes could be shown. Measurements of clavicle length and width, derived from routine chest radiographs, are highly accurate. This accuracy underscores the potential for utilizing thoracic radiographs as a reliable tool for assessing clavicle growth in clinical settings or even forensic analysts. The establishment of reference values derived from our measurements provides a basis for normative growth parameters.

BackgroundHuman rhinoviruses (HRVs) are among the most common pathogens of upper respiratory infections, and they are responsible for the common cold. An increasing number of studies have shown that HRV is associated with more severe illness. However, HRV-associated fulminant myocarditis has rarely been reported.Patient presentationA previously healthy 8-year-old boy developed fever, fatigue, and vomiting for 3 days, with a subsequent exacerbation accompanied by confusion lasting for 9 h. The day before admission, the patient presented with oliguria, confusion, and hypotension, and he was suspected of having myocarditis. The patient was transferred to our hospital for further diagnosis and treatment. On admission, rough and moist rales were detected, and the heart sounds were muffled, accompanied by an irregular heart rhythm and a gallop. An electrocardiogram (EKG) revealed a wide QRS complex, ST-segment depression, premature ventricular contractions, and complete right bundle branch block. Laboratory tests revealed that brain natriuretic peptide (BNP), N-terminal pro BNP (NT-pro BNP), and cardiac biomarkers, such as troponin I, creatinine kinase (CK), and creatinine kinase-MB (CK-MB) were elevated. Additionally, echocardiography revealed an ejection fraction of approximately 28%. The child developed severe cardiac dysfunction and tissue hypoperfusion, and the cardiogenic shock could not be corrected despite active drug therapy. He had indications for ECMO implantation. A rarely reported rhinovirus, namely, A66, was detected in his bronchoalveolar lavage fluid and oropharyngeal swabs via metagenomic next-generation sequencing and a PCR assay. Bacterial culture of all the samples yielded negative results.ConclusionsThis case presents a patient with severe human rhinovirus A66 infection, which is likely responsible for fulminant myocarditis. This report facilitates prompt diagnosis and treatment of fulminant myocarditis. Clinicians should consider rhinovirus as a possible pathogen of fulminant myocarditis, especially when patients present with symptoms or signs of heart involvement.

ObjectiveIt is well-established that overweight/obesity is a major risk factor for left ventricular hypertrophy (LVH) in childhood. However, it is still unclear if reversing from overweight/obesity to normal weight is associated with decreased LVH in children. This study aimed to examine the association between weight status change during four years and LVH among Chinese children based on a prospective cohort study.MethodsData were obtained from the Huantai Childhood Cardiovascular Health Cohort Study in China. A total of 1,178 children without LVH at baseline (mean age: 8.3 years) were included in this study. According to weight status [normal weight or overweight (including obesity)] at baseline (2017) and follow-up (2021), children were divided, based on sex- and age-adjusted body mass index (BMI), into four groups: persistent normal weight (normal weight at both baseline and follow-up), incident overweight (normal weight at baseline but overweight at follow-up), reversal to normal weight (overweight at baseline but normal weight at follow-up), persistent overweight (overweight at both baseline and follow-up).ResultsAfter adjustment for potential confounding factors, children with incident overweight (n = 114, 30.63 ± 4.74 g/m2.7) and those with persistent overweight (n = 363, 31.56 ± 6.24 g/m2.7) had higher left ventricular mass index (LVMI) at the end of the follow-up period than those with persistent normal weight (n = 632, 28.46 ± 7.64 g/m2.7), while those who reversed from overweight to normal weight had a non-significantly lower LVMI (n = 69, 28.51 ± 4.28 g/m2.7). Compared to children with persistent normal weight, those with persistent overweight [odds ratio (OR) = 5.14, 95% confidence interval (CI) = 3.33–7.95] and those with incident overweight (OR = 3.34, 95% CI = 1.77–6.30) had an increased risk of LVH. The risk of LVH tended to decrease, although not significantly, in those who reversed from overweight to normal weight (OR = 0.76, 95% CI = 0.22–2.55).ConclusionOur findings demonstrate a positive association between overweight and left ventricular mass in children and suggest that LVH in childhood could be attenuated by weight loss.

AimsTo investigate the epidemiological and clinical characteristics of children with Mycoplasma pneumoniae pneumonia (MPP) in Hubei, China.MethodsWe retrospectively analyzed inpatients with MPP from 20 hospitals in Hubei, China from January 2021 to December 2022. The co-detected pathogens of Mycoplasma pneumoniae (M. pneumoniae) were investigated using targeted next-generation sequencing (tNGS), and 23S rRNA gene mutations were analyzed to assess the macrolide resistance.ResultsM. pneumoniae infected 20.7% of patients with CAP, with cough (96.59%) and fever (80.28%) being the most prevalent symptoms. The infection rates in children younger than 1, 1–2, 3–6, 7–12, and older than 12 years were 6.17%, 19.98%, 26.97%, 43.93%, and 2.95%, respectively. Among 1,349 patients undergoing tNGS, the overall co-detection rate was 59.45%, with Streptococcus pneumoniae (29.30%), Haemophilus influenzae (23.57%), and Human rhinovirus (17.21%) being the most commonly co-detected pathogens. In 635 patients undergoing the 23S rRNA gene mutation test, 86.30% exhibited positive mutations (A2063G, 98.00%; A2064G, 1.50%; A2067G, 0.50%). Despite a significant age difference (P = 0.037) between macrolide-resistant M. pneumoniae and macrolide-sensitive M. pneumoniae groups, there were no significant differences in symptoms, lab data, or disease severity.ConclusionsIn Hubei Province, the prevalence of exhibited consistent changes during the COVID-19 pandemic. MPP was prevalent year-round, particularly in summer and autumn, with school-age children being more susceptible. Co-detections of viruses and bacteria were frequent in MPP cases, and macrolide resistance exceeded 85%. Ongoing surveillance of M. pneumoniae in children is crucial for understanding the healthcare impact of MPP.

Despite availability of multiple FDA approved therapies, many children with juvenile idiopathic arthritis (JIA) suffer pain and disability due to uncontrolled disease. The term JIA includes a heterogeneous set of conditions unified by chronic inflammatory arthritis, collectively affecting 1:1,000 children. When reviewing treatment options with families the rheumatologist currently refers to the experience of the average patient in relatively small controlled clinical trials, to consensus-based treatment plans, or increasingly the choice is dictated by the formulary restrictions of insurance payers. The current paradigm for treatment selection does not incorporate real-world evidence of treatment effectiveness centered to the individual patients with whom decisions are to be made. Treatment decisions based on the evidence of the average patient are not optimized to reflect the unique clinical characteristics of an individual with JIA and their disease course, nor does it account for heterogeneous treatment effects. To guide treatment choices centered around each patient, we describe a novel concept of utilizing digital health technology to bring patient-centered information into shared decision-making discussions based on comparative effectiveness analysis of electronic health record or observational clinical registry data of patients with similar characteristics. The envisioned digital tool will organize and present data relevant to the individual patient and enable evidence-based individualized treatment decision making when used in a collaborative manner with the patient family and rheumatologist. Capabilities in digital health technology, data capturing, and analytical methodologies are ripe for this endeavor. This brings the concept of a learning health system directly to the point of care.

IntroductionThere are few reports of severe hematological involvement in children with neonatal lupus erythematosus (NLE) treated with exchange transfusion. In this case report, we present a female patient with NLE admitted to the Children's Hospital of Soochow University. The main clinical manifestations were pancytopenia and congenital heart block (CHB). Her condition was serious and could not be improved by conventional treatment; however, she responded well to exchange transfusion therapy.Case presentationA female infant, aged 1 month and 3 days, was admitted to the Children's Hospital of Soochow University owing to the “discovery of thrombocytopenia over 1 month.” She tested positive for anti-SSA IgG, anti-Ro-52 IgG, and anti-mitochondrial M2 antibodies. In contrast, her mother tested positive for ANA (1:320) and anti-Ro/SSA antibodies. The patient was diagnosed with NLE and presented with pancytopenia and CHB. Her cardiac function was normal and no intervention was performed; however, her hematological involvement was more severe, without significant improvement after steroid, intravenous immunoglobulin, and transfusion treatments. After exchange transfusion therapy, the patient significantly improved, and the short-term follow-up prognosis was good.ConclusionFor patients with NLE presenting with hematological involvement that cannot be improved by conventional treatment or whose condition is serious, exchange transfusion therapy should be considered to reduce antibody titers and improve their condition.

IntroductionSevere acute malnutrition (SAM) is a critical public health concern in Ethiopia, contributing to high morbidity and mortality rates among children. Despite the improvement in hospital coverage and the development of standardized WHO treatment guidelines, recent reviews indicated a wide range in recovery rates (34%–88%) due to several context-specific factors. Understanding the factors influencing the recovery time can help inform targeted interventions and improve the overall management of SAM cases. Therefore, this study aimed to assess the time to recovery and predictors of children aged 6–59 months with severe acute malnutrition in Addis Ababa, Ethiopia, in 2023.MethodsAn institutional-based retrospective follow-up study was conducted among children aged 6–59 months who were admitted to Tirunesh Beijing Hospital (TBH), Addis Ababa, Ethiopia, from July 2019 to June 2023. The Kaplan–Meir estimate and survival curve were used to compare the time to recovery using a log-rank test among different characteristics. A Cox proportional hazard regression analysis model was used to identify significant predictors of time to recovery. Finally, a p-value of

BackgroundChildren with moderate-to-severe obstructive sleep apnea (OSA) require specific management. However, the risk factors associated with this level of severity in pediatric OSA remain poorly defined. This study aimed to identify clinical predictors of moderate-to-severe pediatric OSA.MethodsThis retrospective study enrolled children aged 1–18 years who underwent respiratory polygraphy or nocturnal oximetry between January 2013 and December 2017. Patient history, demographics, and sleep study data were analyzed. Logistic regression analysis was conducted to assess risk factors associated with moderate-to-severe pediatric OSA. The STROBE checklist was followed in reporting this research.ResultsAmong 818 children with a median age of 5 years (IQR: 3, 9), 69.4% were male, and 96.7% were diagnosed with OSA. Of those diagnosed, 542 (66.3%) had moderate-to-severe cases. Independent predictors of moderate-to-severe OSA, with their adjusted odds ratios (95% CI), included: age 1–5 years; 6.16 (3.98–9.53), obesity; 2.08 (1.35–3.19), adenotonsillar hypertrophy; 1.58 (1.05–2.36), frequent snoring (>5 nights/week); 6.86 (4.40–10.67), stopped breathing during sleep; 2.34 (1.50–3.63), awakening during sleep; 2.04 (1.32–3.12), and excessive daytime somnolence; 2.10 (1.28–3.43).ConclusionsChildren referred to a sleep center demonstrated an increased risk of being diagnosed with moderate-to-severe OSA. Key clinical predictors of moderate-to-severe OSA included age 1–5 years, frequent snoring, episodes of stopped breathing or awakening during sleep, excessive daytime somnolence, obesity, and adenotonsillar hypertrophy. Prioritizing early sleep investigations and appropriate management for children exhibiting these predictors may enhance clinical outcomes and mitigate the risk of long-term complications.

PurposeSleep disturbances are prevalent in autistic children. The emergence of telehealth offers new possibilities for remote professional intervention. By combining telehealth with parental support, this study aims to explore a novel family-based model to enhance moderate-to-vigorous physical activity (MVPA) and improve sleep quality in children with autism.MethodsThirty-four autistic children (mean age = 15.7 years) were randomly assigned to either a 6-month intervention group or a control group. Both groups received standard physical education classes at school. The intervention group received additional after-school telehealth support. MVPA and sleep quality were assessed 1 week before the intervention and at the 6-month follow-up.ResultsAfter 6 months, children in the intervention group nearly doubled their daily MVPA compared to the control group (Cohen's d = 8.34, CI95% = 6.17–10.52). Actigraphy-assessed sleep efficiency was notably higher (d = 2.35, CI95% = 1.44–3.26), and there were reductions in wake time (d = 1.65, CI95% = 0.84–2.46), sleep fragmentation (d = 0.80, CI95% = 0.07–1.52), and sleep latency (d = 0.82, CI95% = 0.09–1.54) were all reduced. These improvements in objective sleep metrics were corroborated by subjective assessments using the Sleep Disturbance Scale for Children (d = 0.86, CI95% = 0.13–1.59).ConclusionsTelehealth combined with parental support addresses barriers to enhancing health behaviors at home. This innovative model not only improves after-school MVPA and sleep quality in autistic children but also holds significant potential for benefiting other populations requiring remote support.Clinical Trial Registrationhttps://clinicaltrials.gov/study/NCT06444659?id=NCT06444659&rank=1 (NCT06444659).

BackgroundUnlike in developed countries, neonatal morbidity and mortality are the leading challenges associated with easily preventable and treatable disorders during the neonatal period in low- and middle-income countries. However, evidence-based data about prolonged transitional hypoglycemia and associated factors are highly limited in Ethiopia and resource-limited countries.MethodsAn institution-based prospective cross-sectional study was conducted at public hospitals in South Gondar in neonatal intensive care units (NICUs). The data were entered and analyzed using SPSS version 23. Descriptive statistics were used to summarize maternal characteristics. Multivariate binary logistic regression at a p value

BackgroundPediatric kidney transplantations are rarely performed, and there is limited knowledge about the diversity in current clinical practices across Europe. This study aims to explore the utility of clinical snapshot studies in identifying these disparities, establishing a foundation for future snapshot studies and standardization efforts.MethodsA pilot clinical snapshot study was conducted, with invitations extended to all 109 pediatric kidney transplant centres in Europe. Each participating centre provided pre-, peri-, and postoperative data concerning their most recent thirty transplantations. The primary outcomes encompassed the evaluation of disparities in donor-recipient selection, surgical techniques, post-operative drainage procedures, and immunosuppressive therapy protocols. Secondary outcomes involved the analysis of rejection rates, incidence of infections, and graft survival.ResultsThe study involved 439 patients from fifteen centres (14%) in twelve countries, with varying transplant volumes (range 1–29 transplantations per year) and follow-up periods. Significant differences were found among centres in terms of donor types, cold and warm ischemia time, pre-emptive transplant rates, and kidney transplant drainage methods. The rate of living donors varied between 3% and 90% and the median duration of cold ischemia ranged was 770 min after deceased donation and 147 min after living donation. Basiliximab was the dominant induction therapy, yet steroid withdrawal varied widely. Infection, rejection, and graft survival rates also varied significantly between centres.ConclusionThis study revealed substantial variation in clinical practices among European centres performing pediatric kidney transplantations. These findings could serve as a stimulus for international dialogue and collaboration.

ObjectiveWe aimed to investigate the clinical features of children with plastic bronchitis caused by pneumonia, and assess the risk factors for pleural effusion (PE) in plastic bronchitis.MethodsA retrospective study was conducted on children with plastic bronchitis and hospitalized in Xiamen Children’s Hospital from January 2017 to April 2023. Participants were categorized based on the presence of PE. Their clinical manifestations and laboratory findings were analyzed.ResultsSixty-nine children with plastic bronchitis were enrolled: 34 cases (49.27%) in the non-PE group and 35 cases (50.72%) in the PE group. No significant differences were found in sex, age, and etiology between the two groups. Significant differences were found in fever duration, C-reactive protein (CRP), albumin and lactate dehydrogenase (LDH) (p31.66 mg/L or LDH >551 U/L. The prediction of PE was performed with the combination of CRP >31.66 mg/L and LDH >551 U/L, using multivariate logistic regression analysis. The area under the curve (AUC) for logistic regression was 0.797. Elevated CRP increased the risk of PE (odds ratio [OR] 8.358, 95% confidence interval [CI] 2.179–42.900, p=0.0042), elevated LDH increased the risk of PE (OR 5.851 [95% CI 1.950–19.350], p=0.0023).ConclusionThe combined detection of CRP and LDH helps predict the risk of PE in children with plastic bronchitis caused by pneumonia.

IntroductionJuvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease which is commonly monitored by a combination of history, physical examination, bloodwork, and imaging. The COVID-19 pandemic prompted a rapid shift to telemedicine to ensure that patients continued to receive healthcare. The shift to telemedicine changed the methodology and ability of healthcare providers to monitor their patients' progress, as they were unable to perform direct hands-on assessments. The following survey sought to understand the impact of switching pediatric rheumatology healthcare delivery from in-person to telemedicine modality. Specifically, it sought to examine the rate of collection of critical data elements (CDE) for monitoring JIA disease activity and outcomes, barriers and facilitators to its collection, opinions on difficulty and importance of collecting CDE over telemedicine, tools and electronic medical record modifications that facilitated CDE collection, and other data elements that were important to collect during telemedicine visits.MethodsA cross-sectional survey was sent to healthcare providers at all PR-COIN centers who saw patients using telemedicine. Qualitative data was analyzed using descriptive statistics and qualitative data was analyzed using an inductive approach.ResultsSurvey respondents reported that they documented the CDE at least 75% of the time. Barriers to assessing and documenting critical data elements included (1) the inability to palpate or visualize all joints over telemedicine, (2) connectivity issues, and (3) forgetfulness with collecting all CDE. Respondents suggested using reminders within the electronic medical record to prompt documentation completeness and improve reliability. They also suggested including medication adherence, quality of life, and patient/caregiver satisfaction with their telemedicine experience as part of their documentation. A few centers reported that they had established processes to assist with data collection in advance of the telemedicine visit; however, the variation in responses reflects the need to standardize the process of providing care over telemedicine.DiscussionMultiple barriers and facilitators to collecting CDE during telemedicine visits exist. Given that a proportion of the population will continue to be seen over telemedicine, teams need to adapt their practices to consistently provide high-quality care over virtual platforms, ensuring that patients at any institution receive a standardized level of service.

BackgroundHemolytic uremic syndrome (HUS) is the most common cause of acute kidney injury in children. It is mainly caused by Shiga toxin-producing enterohemorrhagic Escherichia coli (EHEC; STEC-HUS) and is more rarely caused by uncontrolled complement activation (cHUS). Renal replacement therapy is frequently required and kidney function recovers in the majority of patients. Ultrasound (US) is the preferred imaging modality for the evaluation of any renal failure. The aim of this study is the evaluation of US diagnostics in both HUS types at disease onset and in the course of the disease.Materials and methodsClinical, laboratory, and US data from the digital patient records of children admitted as inpatients with a diagnosis of HUS were recruited for a monocentric, retrospective analysis. STEC-HUS and cHUS were diagnosed when, in addition to the laboratory constellation, EHEC infection and complement system activation were verified, respectively. US examinations were performed by pediatricians with certified pediatric US experience.ResultsIn total, 30 children with STEC-HUS (13/25 male; median age of disease onset 2.9 years; most prevalent EHEC serotype was O157) and cHUS (2/5 male; median age of disease onset 5.4 years; 3/5 with proven pathogenic variation) were included. Renal replacement therapy proportions were comparable in the STEC-HUS and cHUS patients (64% vs. 60%). The resistance index (RI) was elevated at disease onset in the patients with STEC-HUS and cHUS (0.88 ± 0.10 vs. 0.77 ± 0.04, p = 0.13) and was similar in the STEC-HUS subcohorts divided based on dialysis requirement (yes: 0.86 ± 0.1; no: 0.88 ± 0.1; p = 0.74). Total kidney size at disease onset displayed a positive correlation with dialysis duration (R = 0.53, p = 0.02) and was elevated in both HUS types (177% ± 56 and 167% ± 53). It was significantly higher in the STEC-HUS subcohort which required dialysis (200.7% vs. 145%, p

IntroductionLacticaseibacillus rhamnosus GG (LGG) is a well-studied probiotic with a history of safe use.MethodsIn this double-blind, prospective study, growth and tolerance were evaluated in healthy term infants randomized to: marketed, routine intact cow's milk protein-based formula (Control, n = 172) or a similar investigational formula with added LGG (INV-LGG, n = 179; 106 CFU LGG®/g powder) from 14 to 120 days of age. Anthropometrics, stool characteristics, fussiness, and gassiness were evaluated through Day 120. Medically confirmed adverse events were recorded throughout the study period. The primary outcome was rate of weight gain from Day 14–120.ResultsOf 351 infants enrolled, 275 completed (Control, n = 131; INV-LGG, n = 144). No significant group differences in rate of weight gain from Day 14–120 were detected. Study formula acceptance and tolerance was good with no significant differences in study discontinuation due to study formula or parent-reported gassiness, stool frequency, or stool consistency; however mean fussiness relative to normal was significantly lower for INV-LGG vs Control at Days 60 and 90.DiscussionIn healthy term infants, a routine intact cow's milk protein-based formula with added LGG supported adequate growth and was well tolerated. Further studies are needed to evaluate potential benefits for fussiness and efficacy outcomes. Clinical Trial RegistrationClinicaltrials.gov, identifier (NCT01897922).

IntroductionMany studies have investigated the impact of congenital heart defects (CHD) on child development. However, because CHD not only affects the child and his or her development but, also the entire family, family functioning after pediatric cardiac surgery is of increasing research interest. This prospective childhood-adolescence case-control study aimed to examine differences and changes in parenting behavior and mother-child relationship quality after early surgical repair of an isolated ventricular septum defect (VSD) compared to non-affected controls.Patients and methods39 affected children (M = 7.3 years) with surgically repaired VSD and their mothers were compared with a matched, non-affected control group of 39 mother-child-dyads (M = 7.3 years) during primary school age (t1). At child early adolescence, 24 affected children (M = 12.4 years) and 24 children of the control group (M = 13.2 years) were examined again (t2). Parenting behavior characteristics (t1: mother report; t2: mother- and child report) and mother-child relationship quality (t2: child report) were measured by standardized questionnaires.ResultsThe mother-rated parenting behavior dimensions Involvement (p

ObjectiveTo retrospectively analyze the clinical characteristics and independent risk factors of severe influenza combined with febrile seizures, and to provide more basis for early clinical intervention.MethodsA total of 161 children with severe influenza were collected as study subjects and divided into convulsive (FC) group (40 cases) and non-convulsive (NFC) group (121 cases) according to whether they had febrile seizures. The demographic characteristics and clinical data of the two groups were analyzed. Multivariate logistic regression was used to analyze the risk factors of severe influenza combined with febrile seizures. The predictive efficacy was evaluated by receiver operating characteristic (ROC) curve analysis.Results(1) Multiple logistic regression analysis revealed that C-reactive protein (CRP) levels, Serum interleukin 6 (IL-6) levels, Days from onset of Flu symptoms to hospitalization, cerebrospinal fluid protein (CSF-TP) levels and the influenza virus type A (FluA) infection rate were found to be independent risk factors for severe influenza combined with febrile seizures in children. (2) ROC curve analysis showed that the cut-off value of CRP, Serum IL-6, Days from onset of Flu symptoms to hospitalization and CSF-TP were 7.57 mg/L, 9.84 pg/ml, 4.5 days and 194.8 mg/L, respectively.ConclusionChildren with severe influenza with CRP ≥ 7.57 mg/L, Serum IL-6 ≥ 9.84 pg/ml, Days from onset of Flu symptoms to hospitalization ≤4.5 days, CSF-TP ≥ 194.8 mg/L and FluA had a significantly increased risk of febrile seizures. It is useful for clinicians to determine the risk of severe influenza combined with febrile seizures, to adjust the early treatment plan, and to reduce the incidence of critically ill patients.

Benign and malignant breast lesions in children and adolescents are rare compared to adults. Most tumors are benign. Malignant breast lesions are extremely rare. Fibroadenomas are the most common, accounting for 95% of all lesions. Diagnosis is based on history and physical examination of the breast and armpit. Imaging studies include ultrasound, mammography, and magnetic resonance imaging. Ultrasound is the most commonly used imaging test. Other tests are used in cases of diagnostic doubt. Core needle biopsy should be considered for appropriate diagnostic management. Excisional biopsy should be considered for complex clinical conditions and imaging studies. Except in doubtful situations in children and adolescent girls, a conservative approach and observation of the lesions along with periodic ultrasound examination initially every 6–12 months is advisable. Management of malignant breast lesions in children typically involves a multidisciplinary team consisting of pediatric oncologists, surgeons, radiation oncologists, pathologists, and other specialists and depends on the clinical condition of the patient. An important aspect is the experience of the clinician and radiologist in the treatment of breast lesions, as well as increasing patient and family awareness of possible breast lesions and self-examination. This review aims to provide a scoping overview of the available literature on benign and malignant lesions of the breast in pediatric and adolescent populations to assist physicians and surgeons in making decisions regarding the appropriate diagnosis and management of pediatric breast disease.

BackgroundPersistent pulmonary hypertension of the newborn (PPHN) is a serious condition that affects 1–2 per 1,000 newborns. Scientific data report the existence of neurological developmental abnormalities between 10 and 30%, but the description of these disorders linked with this situation of cerebral hypoxia and haemodynamic failure remains poorly documented.ObjectiveThe main goal of this study was to describe the prevalence of neuro-psychomotor developmental disorders in children aged between one and five years old who have been hospitalised at birth in a neonatal intensive care unit for the management of PPHN.MethodsAll of the newborns ≥34 weeks of gestational age (WGA) with PPHN, treated with inhaled nitric oxide in our neonatal intensive care unit between January 2015 and December 2019 were retrospectively enrolled. An ASQ-3 standardised questionnaire, adapted to the appropriate age (12, 24, 36, 48 and 60 months) was performed by the parents.ResultsFifty-five children (81% of answers) with a median age of 36 months (11–68), whose real age was close to the one of the questionnaire (12, 24, 36, 48 and 60 months), have been included in this study. There was 47% of pathological score [borderline: less than 1 standard deviation (SD) or suspect: less than 2SD] in at least one of the five studied domains, mainly in communication (25%) and individual and social skills (22%), despite a high overall score of 250 [220; 285] out of 300 that improved with age.ConclusionThis study showed a significant prevalence of neuro-psychomotor developmental disorders which justifies making more accessible a prolonged and adapted follow-up for early and multidisciplinary screening and management of these children with PPHN history. Larger cohorts are needed to better explore long term outcome of these vulnerable term neonates.

This case report presents a comprehensive evaluation of the complex balance of therapeutic benefits and potential risks associated with the cystic fibrosis transmembrane conductance regulator (CFTR) modulator elexacaftor/tezacaftor/ivacaftor (ETI) therapy in managing an eight-year-old male with cystic fibrosis (CF) and exocrine pancreatic insufficiency (EPI). While ETI therapy significantly enhanced exocrine pancreatic function, it led to hepatotoxicity, necessitating therapy discontinuation. Attempts to restart ETI at reduced doses were unsuccessful due to persistent hepatic dysfunction. Reduced ETI dosing frequency, implemented due to hepatic dysfunctions, did not result in substantial therapeutic benefits. Clinical markers showed a resurgence of severe EPI and sustained need for gastrostomy tube feeds, with only modest improvement in hepatic function compared to the period following ETI cessation or during prior use of CFTR modulator therapy with lumacaftor/ivacaftor. This case underscores the importance of personalized therapeutic approaches, biomarker-guided monitoring, and multidisciplinary insights to optimize CF management while also highlighting the ongoing need for research to mitigate hepatotoxicity risks and ensure long-term therapeutic efficacy.

BackgroundC3 Glomerulopathy (C3G) is a complement-mediated disease, with predominant C3 deposits, where pathogenic genetic variants in complement system components and circulating autoantibodies result in loss of control of the alternative pathway, have been described. A high incidence of disease recurrence including graft failure has been reported after kidney transplantation (KTx). Currently treatment modalities for preventing and treating post KTx C3G recurrence (plasma exchange, rituximab and eculizumab) in adults have yielded inconsistent results. Data on post KTx C3G recurrence in childhood-onset C3G is still unknown.MethodsA comprehensive case study of patients diagnosed with C3G as children or adolescents, who underwent KTx between the years 2015–2023. Data collected included complement workup, treatment modalities, and outcomes.Results19 patients with C3G were identified during the study period. Five patients developed ESRD and received a kidney transplant. C3G recurrence was diagnosed post KTx in 100% of patients. Graft function improved in 3 of these patients (two with anti-factor H antibodies) after eculizumab treatment, one patient reached graft failure 9 months after transplantation despite eculizumab, recieved a second successful transplantation with pre-emptive eculizumab treatment and one patient showed histologic signs of disease recurrence without clinical signs.ConclusionsC3G recurrence after KTx in patients diagnosed as children or adolescents may be higher than previously described. Treatment with eculizumab is beneficial in some patients. New treatments are needed for improving post-transplant outcome in patients with C3G.

IntroductionHereditary Elliptocytosis (HE) comprises clinically and genetically heterogeneous red cell membranopathies resulting from defects in the horizontal linkage between red blood cell (RBC) membrane and cytoskeletal proteins, which affect mechanical stability and deformability, thereby reducing RBC lifespan. The principal defect in HE is due to dysfunction or deficiency of RBC cytoskeletal proteins.Case descriptionThis study reported a case of severe hemolysis occurring within one day after birth in a term newborn. High-throughput sequencing was used to characterize the pathogenic gene variation in this child and to study the correlation between the identified variation and its corresponding phenotypic characteristics.ConclusionHE is caused by monoallelic mutations, which justify the phenotypic heterogeneity observed in patients. Furthermore, molecular analysis using high-throughput sequencing enables diagnosis in disorders with highly variable heterogeneity. HE can also present with severe hemolysis during the neonatal period.

Acute lymphoblastic leukemia (ALL) treatment, involving chemotherapy, radiotherapy, and pharmacotherapy (antibiotics, antineoplastics) perturbs the gut microbiota in pediatric patients, with enduring effects post-treatment. ALL treatments diminish microbial richness and diversity, favoring pathogenic bacteria. Probiotics may offer promise in mitigating these disruptions and associated side effects. This mini-review explores the impact of ALL treatment on the gut microbiota and the potential benefits of probiotics in pediatric oncology. Probiotics have shown promise in restoring gut microbial balance, reducing treatment-associated side effects, and potentially improving quality of life. However, potential adverse effects, particularly in immunocompromised patients, warrant caution. Notably, there's emerging interest in probiotics’ role in bone health and mineral bioaccessibility. Further research is needed to elucidate probiotics’ mechanisms and their broader impact on pediatric health. Integration of probiotics into ALL treatment and post-treatment regimens offers significant potential for improving patient outcomes and reducing treatment-related complications and long-lasting disruptions, although careful monitoring is essential.

BackgroundRetardation among children is a persistent global health concern. Vitamin deficiency in childhood may contribute to growth retardation; however, its causal effects are not fully understood.ObjectiveHere, we aimed to explore the prediction values of fat-soluble vitamin levels on GR in children aged 1–6 years.Methods614 children aged from 1 to 6 years at Nantong Maternal and Child Care Health Hospital between January 2021 and December 2022 in this retrospective observational study participated in the assessment of developmental status and blood detection of vitamin from peripheral blood (PB). The relationship between vitamin levels and GR was analyzed by Multivariable logistic regression analysis.ResultsDevelopmental assessment results showed that 132 cases from 614 with growth retardation (21.50%). Statistical analysis showed children with GR were more likely to be males (59.45% vs. 40.55%, p = 0.191). The age of children with GR was significantly higher than those without GR (p

Immunoglobulin A vasculitis (IgAV), previously known as Henoch-Schönlein purpura, is the most common form of systemic vasculitis in childhood. The primary organs involved are the skin, gastrointestinal (GI) tract, joints, and kidneys. The spectrum of GI involvement in IgAV ranges from being mild and self-limited to severe manifestations often requiring surgical intervention. Galactose-deficient IgA1 on the immunoglobulin hinge region and its immune complexes are thought to play a central pathogenetic role in IgAV, however, an association between such molecules and specific GI mucosal damage remains unclear. GI endoscopy (both upper and lower) shows a variety of mucosal findings, many of which are not specific for IgAV. In upper GI endoscopy, however, the mucosal features can be diagnostic when found localized in the more distal part of upper GI tract (second and/or third parts of the duodenum). Abdominal computed tomography and capsule endoscopy have demonstrated that the small intestine is most commonly involved in IgAV. The GI mucosal involvement when evaluated microscopically shows IgA deposition which is histologically diagnostic. Conversely, leukocytoclastic vasculitis is less useful. Since the 1960s, cases of duodenojejunitis, in which IgAV was suspected but evident purpura was not dermatologically present, have often been labeled as “idiopathic”. In a pediatric case series, IgA enteropathy, without dermatological manifestations (i.e., purpura), was reported to have similar symptoms, as well as endoscopic characteristics and immunohistological findings as in IgAV. Subsequently, several case reports provide additional supportive evidence that IgA enteropathy must be a variant of IgAV. Thus, the immunologically driven auto-immune vasculitis results in the symptom complex dependent on the organ system involved, and the subsequent clinical features which are manifested. Present classification criteria are useful and universally available for diagnosing IgAV. However, based upon current knowledge including IgA enteropathy, minor modification of the IgAV criteria is proposed in the review.

BackgroundHypotension is a common entity in the neonatal intensive care unit (NICU) and is reported in 24%–50% of preterm infants with birth weight less than 1,500 g. Rapid diagnosis and aggressive management is crucial to reduce its detrimental effects on end-organs especially the brain. Physicians often rely on blood pressure alone as a reliable indicator of tissue perfusion, but variations exist in the definition of this crucial parameter. There are also practice variations in the use of diagnostic tools and management modalities among physicians.MethodologyA physician-based cross-sectional survey of management practices of hypotension in preterm neonates in Indian NICUs was conducted using an online survey tool. The questionnaire addressed diagnostic criteria used, utility of echocardiography for the assessment of hypotension, and management strategies used, such as volume expansion, inotropes and steroids.ResultsThree hundred and twenty physicians, working predominantly in Level III NICUs, responded to the survey. The practice of delayed cord clamping was followed in the units of 78% respondents. Only 44% respondents had an institutional written protocol for the management of hypotension. The criteria for the diagnosis of hypotension varied, with 52% using mean blood pressure (BP) less than gestational age as the criteria. Capillary refill time, blood pressure and heart rate were the most common clinical criteria used. 85% respondents used echocardiography in the NICU, but only 73% utilised it for assessment of a hypotensive neonate. Physicians preferred a ‘volume-inotrope-echo-steroid’ strategy, with 85% respondents using volume expansion. Dopamine was the preferred first line inotrope, followed by norepinephrine and low-dose epinephrine.ConclusionThis survey reflects significant variations in practice amongst neonatal physicians in India. Bedside targeted echocardiography needs to be better utilised as a vital tool to determine the pathophysiology of disease and hemodynamic monitoring in the management of hypotension in neonates. While further research is needed on outcome-oriented objectives, awareness and dissemination of already existing guidelines would be useful to standardize clinical practice.

IntroductionThe objective of this review is to know the existing scientific evidence about the effects of mechanical ventilation (MV) on neurological development in low-birth-weight premature pediatric patients after 12 months of life, taking as background the direct impact that ventilation has on the central nervous system in the newborn during the first days of life.MethodsA systematic search was carried out between 2003 and 2024 in the data bases of: PUBMED, Cochrane Library Plus, PEDro, CINAHL, and SciELO, and two investigators scored the articles according to the Newcastle-Ottawa Assessment scale.ResultsWere found 129 non-replicated articles, and 10 cohort and cross-sectional studies were selected that performed an assessment of neurodevelopment in the three spheres after 12 months of life in corrected age of premature infants exposed to ventilator support and related the two variables independently.ConclusionsMechanical ventilation is an independent neurodevelopmental risk factor in low-birth-weight preterm infants. The time of exposure and the type of ventilation were the variables with the most scientific evidence.Systematic Review Registrationhttps://www.crd.york.ac.uk/, Identifier CRD42023446797.

Newborn screening (NBS) for severe combined immunodeficiency (SCID) has been widely implemented to enable early detection and intervention. Trisomy 21, commonly known as Down syndrome (DS), poses unique challenges in NBS due to its frequent association with T and/or B cell lymphopenia. The pilot NBS screening program recently conducted in Russia was aimed to identify both severe T and B cell deficiencies by measuring TREC and KREC. This study aims to evaluate the incidence of DS in newborns who participated in the pilot program, assess their TREC and KREC values, and determine the proportion of DS newborns potentially identifiable through T/B lymphopenia NBS. We conducted a retrospective analysis of the data obtained during the pilot NBS program, involving 202,908 newborns from eight regions of Russia. The study identified 157 patients with trisomy 21 among the screened cohort, resulting in a DS birth prevalence of 1:1,284. Median TREC and KREC values did not significantly differ between full-term and pre-term subgroups of DS patients. TREC values in DS newborns were decreased and comparable to those of the extremely preterm newborns. DS newborns also demonstrated significant differences in KREC values as compared to the general cohort regardless of gestational age. Our data suggests abnormalities of T- and B-cell lineages development and requires further investigation. This article highlights the need for increased awareness of the intrinsic immunological defects associated with DS. The findings underscore the importance of continued follow-up and comprehensive support by healthcare teams for individuals with DS.

BackgroundAntiphospholipid syndrome (APS) is a systemic autoimmune disorder that can manifest as thrombosis in the pediatric population, characterized by persistently positive antiphospholipid antibodies. APS is infrequently observed in children and could represent non-criteria manifestations.Case presentationA six-year-old Chinese female presented with jaundice and dark urine, leading to a diagnosis of hemolytic anemia. Prednisone therapy initially improved her complexion, but she later developed neurological symptoms. Further laboratory tests showed intravascular hemolysis, coagulation abnormalities, and a positive lupus anticoagulant (LA) test result. Magnetic resonance imaging (MRI) scan revealed abnormal signals in the pons and cerebellar hemispheres, and an occluded part of the basilar artery. She was subsequently diagnosed with autoimmune encephalitis and received IG(immunoglobulin) and high-dose glucocorticoid (GC) treatment, leading to improvement in her clinical symptoms. However, the symptoms of hemolytic anemia worsened after two years. Subsequent laboratory assessments demonstrated the presence of intravascular hemolysis, coagulation abnormalities, and positive tests of anticardiolipin, LA, and anti-beta2 glycoprotein I antibodies. Elevated troponin I and N-terminal pro-brain natriuretic peptide levels, along with electrocardiogram and echocardiogram findings, indicated a myocardial infarction and a thrombus-like mass in the left auricle. Brain MRI showed multifocal infarction and cerebrovascular obstruction. She was diagnosed with APS accompanied by hemolytic anemia, cerebrovascular obstruction, and myocardial infarction. After several weeks of treatment with GC, IG, rituximab, hydroxychloroquine alone with low-molecular-weight heparin sodium, and warfarin, there was a marked improvement in the patient's condition.ConclusionPediatricians should be familiar with various presentations of pediatric APS to promptly detect possible aPL-related complications and initiate appropriate management strategies early on.

BackgroundFailure of successful transition to adult care for adolescents and young adults with chronic rheumatic diseases negatively impacts their health and wellbeing. Transition of care is a vital and complex process within pediatric rheumatology that can be difficult to execute. Use of quality improvement (QI) and clinical informatics (CI) can help implement transition programs.Local problemDespite efforts to improve transition of care within our pediatric rheumatology clinic, it has been difficult to implement and sustain good transition practices including assessment of transition readiness. Using QI methodology and CI, this study aimed to improve transition readiness assessment from 12 to 30% and sustain for one year by surveying transitioning patients yearly.MethodsA transition-focused QI team utilized methods endorsed by the Institute for Healthcare Improvement and leveraged CI to improve survey completion. Control charts of survey completion rates were tracked monthly. Descriptive statistics were used to analyze survey responses.InterventionsInterventions focused on automation of patient surveys at regularly scheduled clinic visits.Results1,265 questionnaires were administered to 1,158 distinct patients. Survey completion rose from a baseline of 12% to greater than 90% and was sustained over 18 months. Identified educational needs included health insurance, scheduling appointments, obtaining care outside of rheumatology clinic business hours, Electronic Health Record messaging, and refilling medications.ConclusionsBy leveraging CI and QI methodology, we were able to assess transition readiness in more than 90% of our patients and identify gaps in self-management. Process automation can create sustainable transition practices.

IntroductionSepsis is the leading cause of child death worldwide, with the majority of these deaths occurring in low- and middle-income countries (LMICs). The aim of this systematic review and meta-analysis was to describe clinical prognostic scores and models for pediatric sepsis outcomes and assess the performance of these scores for predicting mortality in LMICs.MethodsOvid Medline, CINAHL, Cochrane Library, EBSCO Global Health, and Web of Science, were searched through September 2022 for citations related to the development or validation of a clinical prognostic score or model among children with sepsis, conducted in LMIC. Titles, abstracts, and full texts were screened by two independent reviewers and data extracted included population characteristics, variables included, outcomes, and model performance. Risk of bias was assessed with the Prediction Model Risk of Bias Assessment Tool (PROBAST).Results4,251 titles/abstracts and 315 full-text studies were screened, with 12 studies meeting inclusion criteria. Study countries included India, China, Egypt, Indonesia, Tanzania, and a multi-site study in Latin America. Prognostic scores/models included existing scores such as PELOD-2, pSOFA, PRISM, P-MODS, refractory shock criteria. There was high risk of bias in all studies. Meta-analysis was possible for pSOFA, PELOD-2, PRISM, and P-MODS, with pooled area under the receiver-operator characteristic curve of 0.86 (95%CI 0.78–0.94), 0.83 (95% CI 0.76–0.91), respectively.ConclusionRelatively few clinical scores and models have been externally validated for prognostication and risk-stratification among children with sepsis in diverse LMIC settings. Notably there were no studies from low-income countries. Some potentially relevant studies were excluded due to lack of clarity regarding the presence of sepsis in the study populations. More widespread and standardized use of sepsis criteria may aid in better understanding the burden of sepsis and prognostic model performance at the bedside among children in LMICs. Further research to externally validate, implement and adapt these models is needed to account for challenges in use of these scores in resource-limited settings.Systematic Review Registrationhttps://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42022340126, PROSPERO [CRD42022340126].

ObjectiveMethicillin-resistant Staphylococcus aureus (MRSA) infection in children has been on the rise, which poses a serious threat to their health and life in China. The purpose of this study was to determine the molecular characteristics, risk factors, and clinical outcomes of MRSA infections among critically ill pediatric patients.MethodsA retrospective case-control study was performed in the pediatric intensive care unit (PICU) of a tertiary university teaching hospital. All children infected with culture-positive S. aureus in the PICU between January 2016 and December 2021 were included. Univariate and multivariable logistic regression analyses were used to identify potential risk factors for MRSA infection and clinical outcomes of S. aureus infection. All S. aureus isolates were characterized based on antimicrobial resistance, multilocus sequence typing (MLST) and Staphylococcal protein A (spa) typing.ResultsOf 3,974 patients admitted to the PICU, 280 were diagnosed with a S. aureus infection during the 6-year study period. Among them, 43.2% (121/280) were MRSA. All MRSA isolates showed significantly higher rates of resistance to penicillin, erythromycin, clindamycin and tetracycline than MSSA strains. The MRSA strains consisted of 45 spa types and 20 sequence types (STs) (20 clonal complexes), among which the most frequently represented were ST59-t437and ST398-t034. Multivariable logistic regression revealed vaginal delivery, respiratory failure, co-infection with a virus, C-reactive protein (CRP) > 8 mg/L as significant risk factors for MRSA infection. There was no significant difference in all-cause mortality during hospitalization between the MRSA group and the MSSA group. Furthermore, independent predictors for mortality in patients with S. aureus infections were the presence of hypoproteinemia, hematopathy, septic shock, respiratory failure, fever, and white blood cell (WBC) > 15.0 × 109/L.ConclusionsThe study revealed a high proportion of MRSA infections among critically ill pediatric patients, and found significant risk factors for MRSA infection and poor prognosis of S. aureus infection. Methicillin resistance did not contribute to the mortality in the current study. These findings will provide evidence-based practices to make the strategies of prevention and rational use of antibiotics for pediatric patients with S. aureus infection in the ICU.

BackgroundA personalised approach to the treatment of acute myeloid leukemia (AML) in children and adolescents, as well as the development of supportive therapies, has significantly improved survival. Despite this, some patients still die before starting treatment or in an early phase of therapy before achieving remission. The study analysed the frequency, clinical features and risk factors for early deaths (ED) and treatment related deaths (TRD) of children and adolescents with AML.MethodsFrom January 2005 to November 2023, 646 children with AML treated in the centers of the Polish Pediatric Leukemia and Lymphoma Study Group according to three subsequent therapeutic protocols were evaluated: AML-BFM 2004 Interim (385 children), AML-BFM 2012 Registry (131 children) and AML-BFM 2019 (130 children).ResultsOut of 646 children, early death occurred in 30 children, including 15 girls. The median age was 10.7 years (1 day to 18 years). More than half of the patients (53%) were diagnosed with acute myelomonocytic leukemia (M5) and 13% with acute promyelocytic leukemia (M3). The ED rate for the three consecutive AML-BFM protocols was 4.9% vs. 5.3% vs. 3.1%, respectively. In 19 patients, death occurred before the 15th day of treatment, in 11 between the 15th and 42nd day. The most common cause of death before the 15th day (ED15) was leukostasis and bleeding, whereas between the 15th and 42nd day (ED15-42), infections, mainly bacterial sepsis. A significant association was found between ED15 and high leukocyte count (>10 × 109/L), M3 leukemia (p 100 × 109/L, was a significant predictor of early death. The overall TRD for the entire study period was 3.4%. The main cause of death were infections, mainly bacterial sepsis (10 children out of 22, 45.4%).ConclusionsHyperleukocytosis remains significant factor of early mortality in patients with AML, despite the introduction of various cytoreductive methods. Infections are still the main cause of treatment related deaths. A more individualized approach by using new targeted drugs may be the therapeutic option of choice in the future.