Clinical and Experimental Pediatrics最新文献

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.

Background: Thrombocytopenia in preterm infants born to mothers with systemic lupus erythematosus (SLE) is poorly characterized, despite its potential link to adverse outcomes. Our understanding of platelet dynamics, risk factors, and clinical outcomes in this population is limited, necessitating further investigation.

Purpose: This study aimed to characterize the incidence, timing, and severity of thrombocytopenia in this population; to identify associated maternal and neonatal risk factors; and to evaluate its association with adverse outcomes.

Methods: We included 154 preterm infants born to mothers with SLE who were admitted to Shanghai Children's Medical Center within 24 hours of birth between 2014 and 2024. Logistic regression was used to identify risk factors and outcomes associated with neonatal thrombocytopenia.

Results: Thrombocytopenia (platelet count < 150 ×109/L) occurred in 32.5% of infants, and severe form (< 50 ×109/L) occurred in 4.6%. The condition peaked on postnatal days 4-5, and 16% of affected infants required intervention. Late preterm infants (adjusted odds ratio [aOR], 0.15; 95% confidence interval [CI], 0.05-0.42), moderate preterm infants (aOR, 0.26; 95% CI, 0.09-0.76), and maternal hydroxychloroquine use (aOR, 0.19; 95% CI, 0.07-0.52) were protective factors. In contrast, maternal hypertensive disorders of pregnancy (HDP; aOR, 3.41; 95% CI, 1.06-10.93) increased the risk. Infants with thrombocytopenia had significantly higher risks of intracranial hemorrhage (aOR, 4.27; 95% CI, 1.65-11.00) and late-onset sepsis (aOR, 11.00; 95% CI, 1.23-98.14).

Conclusion: Preterm infants exposed to maternal SLE frequently developed thrombocytopenia, but most cases were self-limited. Key risk modulators included gestational age, maternal HDP, and hydroxychloroquine use. Furthermore, thrombocytopenia was significantly associated with neonatal morbidity.

Background: Multiple perinatal factors influence hemodynamically significant patent ductus arteriosus (HS PDA) in preterm infants.

Purpose: This study aimed to identify the risk factors associated with HS PDA in very low birth weight infants (VLBWIs) and determine the predictors of surgical ligation.

Methods: This retrospective study included VLBWIs born at 23-32 weeks' gestation whose HS PDA properties could be identified using echocardiography. The infants were stratified into 2 groups based on gestational age (23- 27 and 28-32 weeks).

Results: Among the 496 included VLBWIs, 171 had no PDA, 90 had non-HS PDA, and 235 had HS PDA. In infants born at 23-27 weeks' gestation, risk factors for HS PDA included low birth weight, the absence of histological chorioamnionitis, and premature rupture of membranes. For VLBWIs born at 28-32 weeks' gestation, HS PDA was associated with lower birth weight, frequent surfactant treatment, and maternal hypertension. Within the HS PDA group, infants with a lower birth weight or who received incomplete antenatal steroid administration had an increased likelihood of requiring surgical ligation, whereas those with a small-for-gestational-age status had a decreased need for surgical ligation.

Conclusion: Recognizing these risk factors can aid the development of targeted treatment strategies for HS PDA in VLBWIs, enabling early ligation and potentially reducing the need for surgical management.

Background: Cerebral hypoxia-ischemia impairs brain development in extremely preterm infants and is associated with poor neurological outcomes. Near-infrared spectroscopy (NIRS) is a noninvasive continuous monitoring method for regional cerebral oxygen saturation (rcSO2).

Purpose: This study evaluated the clinical feasibility and neurological impact of a neurocritical care bundle that incorporates prolonged multidisciplinary hemodynamic monitoring and a stepwise management algorithm.

Methods: Preterm infants with a gestational age (GA) ≤28 weeks or birth weight (BW) ≤1,000 g were prospectively enrolled in a bundle group subjected to NIRS for rcSO2, electrical cardiometry for cardiac output, and daily brain and cardiac echography during the first 72 hours of life. Monitoring was repeated weekly in the first month and then monthly until discharge or the term-equivalent age (TEA) was reached. We implemented a stepwise management algorithm for treating cerebral hypoxia. The primary outcome was a composite of mortality and adverse neurological events (structural abnormalities or electroencephalogram-confirmed seizures) before discharge. The secondary outcomes were the physiological pattern of rcSO2 within the initial 72 hours and up to discharge or TEA.

Results: Thirty preterm infants (GA, 27.1±2.0 weeks; BW, 830±225 g) were enrolled in the bundle group. The mean time-averaged rcSO2 (66.8%±10.3%) was not associated with GA or BW. However, postnatal age appeared to influence physiological rcSO2 changes, given that rcSO2 values were higher during the initial 72 hours than at subsequent intervals. Seven infants (23.3%) had poor outcomes and significantly lower time-averaged rcSO2 (51.1% [50.0%-65.2%] vs. 71.8% [67.1%-73.1%], P=0.002). Multivariate regression indicated that a lower rcSO2 was an independent risk factor, and a 65% threshold showed an optimal predictive value for poor outcomes.

Conclusion: The neurocritical care bundle helped identify preterm infants at risk of cerebral hypoxia, and lower rcSO2 was an independent risk factor for composite mortality and adverse neurological outcomes.

Background: The rising prevalence of severe obesity among children and adolescents poses a major public health challenge.

Purpose: In this study, we examined the differences in body composition and physical fitness between obese and severely obese Asian youth and evaluated their responses to a customized lifestyle intervention.

Methods: A total of 136 overweight and obese participants (mean age, 11.5±3.0 years) were enrolled in an individualized lifestyle modification program. The participants were stratified by obesity severity, with severe obesity defined as a body mass index (BMI)-for-age ≥120% of the 95th percentile. Body composition and physical fitness were assessed at baseline and after 3 interventional stages. The results were compared between the severely obese group and nonseverely obese group, and the relationships between the changes were analyzed.

Results: Among the obese participants, 46% met the criteria for severe obesity. At baseline, those with severe obesity demonstrated a lower performance percentile in the 1-minute sit-up test (22.1±25.2 vs. 47.9±28.0, P=0.002) and the standing long jump (8.5±14.6 vs. 26.8±23.2, P= 0.003) than their nonseverely obese peers. Participants in both groups showed significant reductions in body fat percentage and preserved skeletal muscle mass after the intervention. Those in the severely obese group achieved greater reductions in weight, BMI, BMI z score, and fat mass, particularly during the first 2 interventional stages, indicating a stronger response to the program.

Conclusion: The severely obese youth showed poorer baseline physical fitness levels but greater improvements in key obesity-related measures following the lifestyle interventions. These findings highlight the potential benefits of early targeted interventions for this high-risk group.

Background: We aimed to investigate differences in gut microbiota between patients with Hirschsprung disease (HSCR) and healthy children; assess longitudinal changes in the microbiota of patients with HSCR from diagnosis through postoperative period; and identify microbial markers predictive of postoperative HSCR-associated enterocolitis (HAEC).

Purpose: To investigate alterations in the gut microbiota of patients with HSCR by assessing longitudinal microbiome changes after surgery and identifying microbial signatures predictive of postoperative HAEC.

Methods: A case-control study of 20 patients with HSCR and 20 controls was conducted at Maharaj Nakorn Chiang Mai Hospital. Fecal specimens were collected from patients with HSCR at initial diagnosis and from age-matched controls. Additional samples were obtained from patients intraoperatively and at 1 and 6 months postoperatively. A microbial analysis was performed using 16S rRNA gene sequencing (V3-V4 hypervariable regions).

Results: Compared to controls, patients with HSCR exhibited gut dysbiosis characterized by reduced microbial diversity and altered community composition as determined by Analysis of Compositions of Microbiomes with Bias Correction. Increased relative abundances of Robinsoniella, Fusobacterium, Cutibacterium, Citrobacter, and Eubacterium fissicatena were observed in patients with HSCR, whereas NK4A214, Lachnospiraceae XPB1014 groups, Acinetobacter and Acetitomaculum were decreased (q< 0.05). Alpha diversity in patients with HSCR was significantly increased at 6 months postoperatively versus at theinitial diagnosis (P<0.05). Longitudinal changes in Eubacterium and Eubacteriales suggest their potential use as markers of treatment efficacy. In patients who developed postoperative HAEC, Olsenella was enriched in the proximal intestine, whereas Holdemanella, Corynebacterium, Collinsella, and CAG-352 were elevated in the distal intestine (q<0.05).

Conclusion: Patients with HSCR exhibited distinct alterations in the gut microbiota, with significant shifts observed between the pretreatment period and 6 months postoperatively. Specific bacterial taxa were identified as potential markers for HAEC development. Future microbiome- targeted.