Clinical and Experimental Pediatrics最新文献

Background: Patients with coarctation of the aorta (CoA) and an interrupted aortic arch (IAA) may present with small mitral valves (MVs) and a reduced left ventricular (LV) volume. Biventricular repair (BVR) in these patients is dependent on adequate size of the left cardiac structures.

Purpose: This study evaluated the impact of the hemodynamic characteristics of atrial septal defects (ASDs) on MV growth following surgical repair.

Methods: We retrospectively reviewed the data of patients diagnosed with CoA or IAA between 2007 and 2024. The z score for MV size measured 6 months postoperatively (Z2) was compared with the preoperative MV size (Z1). The factors associated with MV growth were also studied.

Results: A total of 161 patients with CoA or IAA were included. Transthoracic echocardiography was used to assess the MV and LV dimensions preoperatively and 6 months postoperatively. Of the cohort, 155 (96.3%) underwent initial BVR and 6 underwent single-ventricle palliation. MV z scores significantly increased following BVR (mean change: +0.45±1.35; P<0.001) but decreased after single-ventricle repair (-0.56±0.49, P=0.04). Multivariate analysis identified the initial MV z score and ASD pressure gradient as independent predictors of MV growth (R²=0.39).

Conclusion: Annular growth of the MV was not observed in patients who underwent single-ventricle palliation. In contrast, among patients who achieved BVR, those with a small preoperative MV annulus and low ASD pressure gradient demonstrated subsequent catch-up MV growth, suggesting that adequate left-sided preload is essential for MV development.

Background: Acute necrotizing encephalopathy (ANE) is a rare but devastating neurological disorder in children that is typically triggered by viral infections such as influenza, sudden acute respiratory syndrome coronavirus 2, and human herpesvirus-6. ANE is characterized by cytokine storm and associated with high mortality; however, optimal immunomodulatory strategies remain undefined.

Purpose: To evaluate the effectiveness of multiple immunomodulatory strategies, including high-dose methylprednisolone (MP), plasma exchange (PLEX), and tocilizumab, at reducing short-term mortality among pediatric patients with ANE stratified by disease severity using the ANE severity score (ANE-SS).

Methods: We retrospectively reviewed 65 pediatric patients (median age, 4.8 years; interquartile range, 2.8-7.7 years) diagnosed with ANE at Beijing Children's Hospital from 2016 to 2024. Patients were stratified by ANE-SS at admission into low- to medium-risk (ANE-SS<5) and high-risk (ANE-SS≥5) groups. Immunomodulatory treatments included different doses of MP, intravenous immunoglobulin, PLEX, and tocilizumab. The primary outcome was the 28-day postdischarge mortality. Multivariate logistic regression was used to identify the treatment strategies that were independently associated with improved survival.

Results: The overall 28-day postdischarge mortality rate was 45.9%, significantly higher in patients with ANE-SS ≥5 (65.7%) than in those with ANE-SS<5 (16.7%; P<0.001). A notable decline in mortality has been observed since 2022, coinciding with the increased use of high-dose MP (20 and 30 mg/kg/day) and tocilizumab. The annual mortality rate will drop to 38.9% in 2022, 36.8% in 2023, and 16.7% in 2024. In low- to medium-risk patients (ANE-SS<5), both 20-mg/kg/day and 30-mg/kg/day MP were associated with improved outcomes. In high-risk patients (ANE-SS≥5), the combination of 30-mg/kg/day MP and tocilizumab provided the greatest survival benefit. Multivariable logistic regression analysis identified that this combined therapy was independently associated with reduced mortality (odds ratio, 0.04; 95% confidence interval, 0.01-0.18; P<0.001). No significant survival benefit was observed following PLEX treatment.

Conclusion: In low- to moderate-risk patients, the 20- and 30-mg/kg/day MP regimens were effective. In high-risk patients with ANE, high-dose MP (30 mg/kg/day), particularly when combined with tocilizumab, may improve survival and possibly long-term neurological recovery. These findings support the use of a severity-based immunotherapy strategy for pediatric patients with ANE.

Pneumococcal disease remains the leading cause of morbidity in young children worldwide. Recent advances have expanded pneumococcal conjugate vaccines (PCVs) beyond 13-valent (PCV13) to 15-valent (PCV15) and 20- valent (PCV20) vaccines, which add 2 and 7 serotypes, respectively, thereby broadening their potential protective effects against invasive pneumococcal disease (IPD). Although policies vary among countries, Korea incorporated PCV15 and PCV20 into its National Immunization Program for children in April 2024 and October 2025, respectively, an implementation that is considered within the context of global epidemiology and policy trends. This review discusses evidence of the immunogenicity and safety of PCV15 and PCV20 from clinical trials and early postlicensure data, examines the serotype distribution and IPD burden in Korea alongside data from other regions, and summarizes vaccination recommendations for healthy and high-risk pediatric populations, including considerations for catch-up, interchangeability, and their coadministration. By integrating national updates and global evidence, this review aims to support clinicians and policymakers worldwide optimizing pneumococcal vaccination strategies for children.

The ingestion of foreign bodies and caustic substances represents a significant clinical concern in pediatric populations, particularly among children aged 1-5 years. These events can result in a wide spectrum of complications ranging from acute, life-threatening emergencies to delayed sequelae with long-term morbidities. The severity and clinical course are influenced by multiple factors, including ingested material's nature, size, shape, and chemical composition, as well as its anatomical location, particularly when esophageal impaction occurs. High-risk foreign bodies- such as button batteries, high-powered magnets, sharp objects, superabsorbent polymers, and items containing toxic substances-pose an elevated risk of rapid tissue injury or perforation. Similarly, the ingestion of caustic agents, whether acidic or alkaline, carries the potential for extensive mucosal damage, with the degree of injury dictated by the pH, volume ingested, and inherent toxicity. Clinical management requires a highly individualized, case-by-case approach guided by the clinical presentation, imaging findings, and endoscopic evaluation findings. Endoscopy plays a pivotal role in the diagnostic assessment of mucosal injury and is a therapeutic modality for foreign body retrieval. Timely endoscopic interventions are strongly associated with improved outcomes and reduced complication rates. A high index of clinical suspicion remains critical to ensuring early diagnosis, prompt intervention, and the prevention of both acute and long-term complications, including strictures, perforations, and impaired quality of life. Comprehensive, multidisciplinary management is essential for optimizing patient outcomes in these potentially complex clinical scenarios.

Background: Associations have been suggested between prenatal exposure and allergic diseases in children as well as between respiratory allergies and maternal sleep disorders during pregnancy.

Purpose: This study aimed to examine the association between maternal sleep disorders during pregnancy and allergic diseases, including respiratory, skin, and ocular allergies, in their children.

Methods: This study was based on the Tohoku Medical Megabank Project Birth and Three-Generation Cohort Study. Sleep disorders during pregnancy were defined as an Athens Insomnia Scale score of ≥6. Allergic diseases in children up to 5 years of age were assessed by maternal self-report on "bronchial asthma," "atopic dermatitis," "food allergy," and "allergic conjunctivitis/allergic rhinitis/hay fever." Hazard ratios (HRs) with 95% confidence intervals (CIs) were calculated using a Cox proportional hazards model.

Results: A total of 11,123 mother-child pairs were included. The mean gestational age at registration was 19.6±7.6 weeks. During pregnancy, 4,115 women (37.0%) experienced sleep disorders. Additionally, 53.7% of the participants had a history of parity, 8.8% worked night shifts, and 0.4% used sleep medications. In the crude models, maternal sleep disorders during pregnancy were associated with all examined allergic diseases in children. After the adjustment for all confounders, the associations remained significant for atopic dermatitis and allergic conjunctivitis/allergic rhinitis/hay fever (HR [95% CI], 1.09 [0.97-1.23] for bronchial asthma, 1.17 [1.05-1.31] for atopic dermatitis, 1.11 [0.98-1.26] for food allergy, and 1.25 [1.13-1.39] for allergic conjunctivitis/allergic rhinitis/hay fever).

Conclusion: Maternal sleep disorders during pregnancy were associated with atopic dermatitis as well as allergic conjunctivitis/allergic rhinitis/hay fever in children.

Background: Immunoglobulin A vasculitis (IgAV) is a systemic vasculitis characterized by potential recurrence.

Purpose: This study aimed to explore the prevalence of and factors associated with IgAV recurrence. The clinical phenotypes of childhood- and adolescent-onset IgAV were described and compared.

Methods: This retrospective cohort analysis included patients aged ≤18 years and diagnosed with IgAV treated in a tertiary academic center between January 2010 and December 2022. Recurrence was classified by the reappearance of purpuric/petechiae or other features that reappeared after at least 1 asymptomatic month.

Results: The 361 patients with IgAV had a mean age of 7.7±3.9 years; 53.2% of them were female. All patients with IgAV exhibited skin manifestations. Gastrointestinal (GI) and musculoskeletal (MSK) features were observed in 214 (59.3%) and 219 patients (60.7%), respectively. Renal involvement was observed in 82 patients (22.7%). The prevalence of childhood- and adolescent-onset IgAV was 269 (74.5%) and 92 (25.5%), respectively. Patients with childhood-onset IgAV had significant GI manifestations (P=0.01), had MSK features (P=0.002), and required hospitalization (P=0.004). In contrast, patients with adolescent-onset IgAV had significant renal involvement (P<0.001) and required a longer duration of corticosteroids treatment (P<0.001). Of the study population, 119 patients (35.6%) experienced recurrence. During the 12-month follow-up period, 98 patients (27.1%) had recurrent IgAV at a median 1.9 months (interquartile range, 1.2-4.8 months). On the multivariable Cox proportional hazards regression analysis, corticosteroids treatment was significantly associated with IgAV recurrence (hazard ratio, 1.99; 95% confidence interval, 1.266-3.157; P=0.003).

Conclusion: Renal involvement was more prevalent in adolescent-onset IgAV, whereas MSK and GI involvement were more common in childhood-onset IgAV. Recurrence was noted in 35.6% of the population. Therefore, vigilant monitoring for recurrence is necessary.

Background: Gestational diabetes mellitus (GDM) is a common complication of pregnancy associated with various perinatal risks in mothers and heightened risks of long-term obesity and metabolic syndrome in their children. Understanding the effect of GDM on infant health is crucial. Infant gut colonization has generated significant interest owing to its profound impact on health and potential role in later disease development.

Purpose: Here we conducted a thorough analysis of the microbiota and metabolome of neonatal meconium to understand how GDM in mothers affects microbial colonization in the early lives of their offspring.

Methods: This study included 49 healthy-term neonates born to mothers with GDM (n=29) and normoglycemic mothers (n=20) between March 2022 and February 2023 at Chang Gung Memorial Hospital (Linkou branch). Fecal samples were collected in sterilized containers before the infants reached 5 days of age. To analyze the meconium microbiota, 16S rRNA gene sequencing was performed, and proton nuclear magnetic resonance was used to examine the metabolome.

Results: Neonates born to mothers with diet-controlled GDM exhibited a notable decrease in α-diversity and a shift in β-diversity compared to those born to normoglycemic mothers. A functional analysis revealed increased peroxisome proliferator-activated receptor and adipocytokine signaling pathway activation in the GDM group. Metabolomic analysis revealed significant changes in the fumarate and succinate levels, indicating metabolic shifts associated with maternal GDM.

Conclusion: These findings highlight the potential effects of pregnancy-related complications on the establishment of gut bacteria in neonates. Further comprehensive studies are required to understand the long-term implications of these microbial changes on infant health.

Background: Intravenous cannulation (IVC) is a routine yet distressing procedure in pediatric patients, often provoking significant anxiety and procedural pain. Although eutectic mixtures such as eutectic mixture of local anesthetic cream are widely used, their delayed onset limits their applicability in time-sensitive settings. Ethyl chloride vapocoolant spray and 10% lignocaine spray have been proposed as rapid-onset alternatives, yet direct comparative evidence in children is scarce.

Purpose: This study aimed to compare the analgesic efficacy, onset of action, and ease of IVC between ethyl chloride vapocoolant spray and 10% lignocaine spray in pediatric patients undergoing elective surgery.

Methods: A prospective, randomized, double-blinded trial was conducted in a tertiary care hospital between 2022 and 2024 following ethics approval (IEC:279/2022) and trial registration (CTRI/2023/08/056580). Sixty children aged 8-17 years (American Society of Anesthesiologists [ASA] I-II) scheduled for elective procedures were randomized to receive either 3 sprays of 10% lignocaine (group L) or ethyl chloride spray (group E) before IVC. Pain was assessed using the visual analogue scale (ASA=0-100 mm), ease of cannulation with a 4-point Likert scale, and procedural time was recorded. Failed cannulations, adverse events, and rescue analgesia requirements were noted. Data were analyzed using IBM SPSS Statistics ver. 22.0 with a significance threshold of P<0.05.

Results: Group E demonstrated significantly lower VAS scores (30.43±0.9 mm) compared to group L (70.03±1.07 mm, P<0.001). Mean cannulation time was shorter in group E (16.07±2.41 seconds) than group L (24.57±4.42 seconds, P<0.001). Ease of cannulation was superior in group E, with 100% of patients reporting no difficulty, whereas only 67.8% in group L reported the same (P= 0.002). No adverse effects or serious complications were observed in either group.

Conclusion: Ethyl chloride vapocoolant spray provides significantly superior dermal analgesia, faster onset, and improved ease of IVC compared to 10% lignocaine spray in children aged 8-17 years. Its rapid action and safety profile make it a valuable alternative in pediatric anesthesia practice, particularly in time-sensitive clinical settings.

Pubertal induction in males with hypogonadotropic hypogonadism (HH) remains challenging. Various treatment strategies using testosterone or gonadotropins have been developed; however, the optimal approach for initiating and sustaining puberty remains uncertain. A comprehensive PubMed search was conducted in July 2024 using the keyword "puberty induction in males" for studies published between January 2004 and July 2024. The inclusion criteria were publication in English including male patients under 18 years of age with HH. Animal studies, adult cohorts, and non-HH groups were excluded. Of the 134 retrieved records, 18 met the inclusion criteria and were analyzed for therapeutic regimens, efficacy, and outcomes. Both testosterone- and gonadotropin-based therapies effectively induced puberty in males with HH. Intramuscular testosterone esters remain the most commonly used approach because of their accessibility and cost-effectiveness, whereas newer long-acting transdermal formulations offer improved tolerability. Gonadotropin-based regimens, including human chorionic gonadotropin, alone or in combination with follicle-stimulating hormone, demonstrated effective virilization and increased testicular growth and spermatogenesis, suggesting potential benefits for future fertility. However, treatment protocols vary widely and no standardized guidelines are currently available. Pubertal induction in HH should aim to mimic physiological puberty and consider psychological and somatic well-being as well as future fertility potential. Although testosterone effectively promotes virilization, gonadotropin therapy enhances testicular development and spermatogenesis. Their formulations, dosages, treatment durations, and modes of administration show considerable heterogeneity. Further multicenter studies are required to establish optimal regimens and clarify long-term fertility outcomes associated with different therapeutic strategies.