Clinical and Experimental Pediatrics最新文献

Adenomatous Polyposis Coli (APC) is a tumor suppressor gene expressed throughout the body. APC mutations increase the risk of malignancy and are often characterized by syndromes that encompass a spectrum of neoplastic manifestations, such as familial adenomatous polyposis (FAP). We present a rare case of palatal peripheral nerve sheath tumor in the context of APC gene mutation. A 17-year-old male with a significant history of FAP presented to our clinic for with globus sensation for 5 months with increasing discomfort. Flexible nasolaryngoscopy revealed a pedunculated lesion attached to the posterior surface of the soft. Imaging was obtained and confirmed a soft tissue homogenous mass contiguous with the soft palate. Endoscopic-assisted transoral resection was performed and pathologic features were consistent with schwannoma. We also discuss the spectrum of benign neoplastic lesions. Current literature fails to describe pharyngeal masses in the setting of APC gene mutations. The purpose of this case report is to describe a patient presentation of a symptomatic pharyngeal tumor with a known APC gene mutation and explore the differential diagnoses that must be considered.

Functional gastrointestinal disorders (FGID) and their association with anxiety disorders in children significantly impact a child's functioning and treatment response. This study aimed to scope the evidence of anxiety disorders manifesting as FGID in children up to 16 years old. A comprehensive search strategy was conducted on Embase (1974-2024), Medline (via EBSCOHost 1946-2024), and APA PsycINFO (via EBSCOHost 1967-2024). Articles were retrieved, screened, and assessed for bias using the GRADE system. Our initial search yielded 1984 articles. After screening titles and abstracts, 53 articles remained. Full-text screening further narrowed this to four eligible studies. The first study found that anxiety indirectly influenced abdominal pain severity in children with irritable bowel syndrome (IBS). The second study reported an association between anxiety and abdominal pain but found that anxiety might not predict abdominal pain in later childhood. The third study suggested FGID could be a risk factor for anxiety, with higher anxiety rates in children with FGID compared to those without. The fourth study found no significant difference in pain intensity between children with functional abdominal pain disorders (FAPD) alone and those with FAPD and anxiety. The reviewed studies indicate a relationship between anxiety and FGID but lack clarity on directionality or causation. The limited number of studies calls for more research, including case-control studies with large sample sizes and longitudinal cohort studies to investigate the incidence and causation.

Background: Febrile neutropenia (FN) remains an important complication of cytotoxic chemotherapy for which an urgent and appropriate evaluation is imperative.

Purpose: To assess the diagnostic and prognostic roles of mid-regional pro-adrenomedullin (MR-ProADM) levels in predicting infection in patients with FN.

Methods: This comparative cross-sectional study included 137 patients with chemotherapy-induced FN. Complete blood count, C-reactive protein (CRP), procalcitonin, and MR-ProADM were evaluated on the 1st day of FN. Chest computed tomography (CT) was performed on the 5th day.

Results: MR-ProADM levels were significantly higher in patients with FN than in controls. CRP and MR-ProADM levels were significantly higher and ANC was significantly lower in patients with versus without bacterial infections. CRP, procalcitonin, and MR-ProADM levels were significantly negatively correlated with absolute neutrophil count (ANC). CRP, procalcitonin, and MR-ProADM levels were significantly and positively correlated with FN degree, FN duration, and hospital stay length. A multivariate regression analysis showed that a longer FN duration and hospital stay length, along with elevated CRP, procalcitonin, and MR-ProADM levels, were significant risk factors for mortality.

Conclusion: MR-ProADM is a reliable prognostic and diagnostic tool for predicting infection in patients with FN.

Background: Patients with systemic lupus erythematosus (SLE) are susceptible to infectious diseases owing to various immunosuppressive treatments and disease characteristics. Meningococcal infections progress rapidly with a high incidence of severe complications and mortality; therefore, meningococcal vaccination is needed. However, there is limited evidence regarding the immunity and immunogenicity of patients with SLE.

Purpose: This study aimed to analyze the serum bactericidal activity against meningococci in patients with SLE in two domestic institutions in Korea.

Methods: Serum samples were collected from patients diagnosed with SLE (age < 19 years) at Seoul National University Children's Hospital and Ewha Womans University Mokdong Hospital in 2016-2018. Serum bactericidal activity against the four meningococcal serogroups was analyzed using a serum bactericidal assay with rabbit serum. The patients' demographic information, diagnostic history, and disease activity status were obtained from electronic medical records.

Results: The mean age of the 41 included patients was 20.3±5.4 (range, 10-35) years. All but one patient received steroids. The sera of most of the patients (34/41 [82.9%]) lacked bactericidal activity against serogroup A. Some patients showed bactericidal activity against serogroups C, W-135, and Y (63.4%, 56.1%, and 61.0%, respectively). There were no significant differences in the geometric mean indices based on complement consumption state or anti-double-stranded DNA antibody positivity.

Conclusion: Although the sera of some patients exhibited serum bactericidal activity against meningococci, most remained seronegative. It is important that patients with SLE at risk of meningococcal infection receive appropriate vaccinations. Our findings serve as baseline serological data for meningococcal vaccination policies for patients with SLE.

Background: Febrile seizures (FSs) are the most common form of childhood seizures. Determining the role of trace elements in the pathophysiology of FSs will contribute to the management of FSs by pediatricians.

Purpose: This study aimed to investigate the effects of zinc and selenium on the nervous system and how they may influence the risk of FSs.

Methods: In this case-control study, there were 60 children in the simple FS group and 40 children in the complex FS group. The control groups comprised 50 children with fever but without seizures and 50 healthy children. Blood samples were collected within the first hour after FS.

Results: Zinc and selenium levels were significantly lower in children with fever but without seizures versus healthy children (p<0.001). Serum zinc levels were lower in children with FSs (simple and complex FSs) than in healthy children (p<0.001) but higher than in children with fever but without seizures (p<0.001). Serum selenium levels in children with FSs (simple and complex) were lower than in healthy children but higher than in the children with fever but without seizures. However, these differences were not statistically significant (p>0.05).

Conclusions: Serum zinc levels are significantly decreased during infection, whereas they show a statistically significant increase within the first hour after FS activity. This indicates that the body secretes zinc during FSs to restore homeostasis, reduce oxidative stress, and increase the seizure threshold. Therefore, zinc supplementation during febrile periods may effectively prevent FSs in high-risk children.

Background: Human breast milk (HBM) is an important source of tolerogenic immune mediators that influence the infant immune system. HBM-derived immune components are affected by various factors; however, few studies have examined the relationship between parity and immune cell profiles of HBM.

Purpose: This study aimed to clarify the effects of parity on HBM immune cell heterogeneity and gene expression by integrating and analyzing publicly available single-cell RNA sequencing datasets.

Results: The proportion of innate immune cells was significantly higher in the primiparous versus multiparous group, whereas the proportion of adaptive immune cells was significantly higher in the multiparous group (p = 0.021). The two immune clusters were re-annotated and classified into monocyte, T/B cell, and CD45 groups. The proportions of monocytes and T/B cells were higher in the primiparous and multiparous groups, respectively. In a gene set enrichment analysis of monocytes, genes with a direct role in the infant immune system and immune response-related genes were more highly expressed in the primiparous group.

Conclusion: Our results support the parity-dependent differences in gene expression between innate and adaptive immune cells.

Background: The oropharyngeal administration of colostrum (OAC) in neonates has several benefits.

Purpose: To investigate the short-term outcomes of OAC in preterm neonates.

Methods: We performed this 2-arm, double-blind, placebo-controlled randomized trial at a tertiary neonatal center in Iran in 2021-2023. The intervention and control arms received 0.2 mL of their mother's colostrum or distilled water via oropharyngeal administration every 6 hours for 3 days starting from birth until 72 hours of age. The main study outcomes were neonatal death, the incidence of necrotizing enterocolitis, sepsis, retinopathy of prematurity (ROP), length of hospital stay, and period to full enteral feeding. A regression analysis was used to adjust for possible confounders.

Results: A total of 126 neonates (mean gestational age, 30.05 weeks) were randomized to the intervention and placebo groups (n=63 each) and had a mean±standard deviation weight of 1,247±193 g versus 1,156±215 g (P=0.013) and 1- and 5-min Apgar scores of 6.35 versus 5.38 (P=0.003) and 7.84 versus 7.13 (P=0.001), respectively. The mortality rate was 12.7% in the intervention group versus 14.3% in the placebo group (P=0.794). The necrotizing enterocolitis rate was significantly lower in the intervention versus placebo arm (11.1% vs. 28.6%, respectively, P=0.010), as was the clinically suspected sepsis rate (15.9% vs. 39.7%, respectively, P=0.004). The ROP and bronchopulmonary dysplasia rates did not differ significantly between groups after the adjustment for confounders. The mean length of hospital stay was shorter in the intervention group (26.1 days vs. 37.32 days, P=0.023). Moreover, the mean duration of antibiotic therapy and period to full feeding were significantly shorter in the intervention group.

Conclusion: OAC could effectively decrease the incidence of complications in preterm infants and facilitate earlier patient discharge.

Cultivating a 24-hour movement behavioral profile conducive to health and well-being, marked by adequate levels of physical activity, limited screen time, and sufficient sleep, has emerged as an important avenue for promoting pediatric health. We aimed to provide evidence of this potential, this multiphase, multimethod, and integrative review comprehensively investigated the global trends in health promotion initiatives and status of the Korean pediatric population. This integrative review comprised 3 phases employing various review methods: an environmental scan of existing guidelines for 24-hour movement behaviors worldwide, a rapid review of physical activity-related indicators from Global Matrix data, and a systematic review of 24-hour movement behaviors among children and adolescents in South Korea. The growing recognition of a holistic approach to the 24-hour movement behavior paradigm in pediatric health promotion has led to the establishment and adoption of 24-hour movement guidelines in different countries and by the World Health Organization (WHO). Korean health authorities have also responded to this need by releasing a translated report of the WHO guidelines for physical activity and sedentary behavior as well as the 2024 update of the Physical Activity Guidelines for Koreans. Accordingly, actions must be followed in policy, research, and practice. This is critical considering that Korean children and adolescents exhibit an unfavorable profile of 24-hour movement behaviors compared to the global average. Among those aged 0-6 years, the weighted averages for guideline adherence were 18.2% for physical activity, 38.1% for sedentary behavior, and 76.2% for sleep. For those aged 12-18 years, the weighted averages for guideline adherence were 20.4% for physical activity, 55.9% for sedentary behavior, and 26.2% for sleep. Building on these current findings, this integrative review provides 3 priorities for pediatric health promotion policy and practice and 3 recommendations for future research.