Clinical and Experimental Pediatrics最新文献

Background: Gut-lung crosstalk is a pathway involving interactions between the gastrointestinal, respiratory, and immune systems. The immune responses of the gut and lungs are intricately linked, and previous studies demonstrated that the gut microbiota can influence systemic immune responses in the respiratory system as well as bronchopulmonary dysplasia (BPD).

Purpose: To analyze the composition of the gut microbiota in very low birth weight infants with versus without BPD.

Methods: Secondary data from a previous randomized controlled trial were analyzed. Microbiomes were analyzed using QIIME 2 software. Gut microbiota diversity and abundance were compared between groups.

Results: Fifty-one neonates were classified into the BPD (n=24) and non-BPD (n=27) groups, between which no differences were noted in the alpha and beta diversities of the gut microbiota. In both groups, Proteobacteria, Gammaproteobacteria, and Klebsiella were the predominant phylum, class, and genus in gut microbiota, respectively. Enterococcus, Acinetobacter, Elizabethkingia, Clostridium sensu stricto 1, Bacteroides, Streptococcus, and Serratia were more abundant, whereas Klebsiella, Faecalibacterium, Escherichia-Shigella, Enterobacter, Bifidobacterium, Veillonella, Staphylococcus, and Enterobacteriaceae were less abundant in the BPD versus non-BPD group. Faecalibacterium, Roseburia, Clostridium, Eubacterium, and Coprococcus were significantly more abundant in the non-BPD versus BPD group.

Conclusion: The alpha and beta diversities of the gut microbiota did not differ significantly between the BPD and non-BPD groups. However, in terms of relative abundance, the presence of common respiratory pathogens was notable in the BPD group. Conversely, the non-BPD group had a significantly higher prevalence of anaerobic taxa known for their capacity to produce butyrate, a key component of postbiotics.

Background: Postinfectious bronchiolitis obliterans (PIBO) is a rare chronic obstructive pulmonary disease that occurs after a respiratory infection. Its diagnosis is generally based on clinical history, respiratory symptoms, and computed tomography (CT) findings.

Purpose: Here we evaluated the frequency of exacerbations, clinical progress, and inhaled corticosteroid (ICS) usage in children diagnosed with PIBO with or without comorbid bronchopulmonary dysplasia (BPD).

Methods: This retrospective observational study was conducted in Rouen, France. The inclusion criteria were as follows: child diagnosed with PIBO (history of respiratory infection, airway obstruction with no or poor response to bronchodilation treatment, and/or mosaic pattern or trapping on chest high-resolution CT) in 2009-2024 treated with intravenous corticosteroid pulses.

Results: Fifty-seven patients were included: 13 (23%) with BPD and 44 (77%) without BPD. The mean age at diagnosis was 7.0±3.6 months, with no significant intergroup difference. We observed a significant reduction in exacerbations following corticosteroid pulse treatment as soon as 6 months (P<0.001), with persistent effects observed up to 24 months (P=0.02). We also noted a reduced daily ICS dose starting at 12 months (P=0.03). Respiratory syncytial virus is the most commonly identified causative virus, followed by rhinoviruses and adenoviruses. The viral codetection rates were 18% and 61% in the BPD and non-BPD groups, respectively.

Conclusion: In our cohort, intravenous corticosteroid pulse treatment effectively treated PIBO, with a rapid and long-lasting reduction in exacerbations and ICS requirements. BPD was a significant comorbidity of PIBO.

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 with globus sensation for 5 months with increasing discomfort. Flexible nasolaryngoscopy revealed a pedunculated lesion attached to the posterior surface of the soft palate. 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.

In some children, atopic manifestations begin with atopic dermatitis and progress to allergic asthma and allergic rhinitis; of them, a small subset experience food allergies as well. This progression shares genetic and environmental predisposing factors and immunological features, such as allergen-specific T-helper type 2 responses, that manifest as specific immunoglobulin E production and eosinophil activation. Eosinophil-derived neurotoxin (EDN), which is released by eosinophils during this activation, shows promise as a reliable and accurate biomarker. EDN levels are elevated in a subset of patients with atopic march-associated conditions. Elevated EDN levels predict allergic disease development, demonstrating that EDN is a good biomarker for the prognosis, diagnosis, treatment, and monitoring of allergic diseases comprising atopic march. The early measurement of EDN would help identify those who are more likely to develop allergic diseases later in life. Thus, the early detection and treatment of elevated EDN could lead to better outcomes, including halting atopic march.

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 (PCT), and MR-ProADM were evaluated on the 1st day of FN. Chest computed tomography 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 absolute neutrophil count (ANC) was significantly lower in patients with versus without bacterial infections. CRP, PCT, and MR-ProADM levels were significantly negatively correlated with ANC. CRP, PCT, 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, PCT, 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.

Mucopolysaccharidosis (MPS) is a group of genetic disorders characterized by defective lysosomal enzyme activity that can result in growth abnormalities and other complications. Hematopoietic stem cell transplantation (HSCT), especially bone marrow transplantation (BMT), aims to restore enzyme function and improve growth parameters in patients with MPS. This systematic review evaluates the impact of HSCT on growth outcomes, including height, weight, body mass index (BMI), head circumference, and pubertal development, in pediatric patients with MPS. Using the PRISMA (Preferred Reporting Items for Systematic reviews and Meta-analyses) guidelines, we systematically searched the PubMed, Embase, Scopus, and Web of Science databases. The retrieved studies focused on the growth outcomes of patients with MPS treated with HSCT emphasizing the role of BMT. Study quality was assessed using the Newcastle-Ottawa Scale and Joanna Briggs Institute checklist. The study protocol was registered in PROSPERO (registration no. CRD42024571488). These findings indicate that HSCT improves height, weight, and BMI, and better outcomes were observed in patients who underwent early transplantation. However, many patients still experience declining height z scores, resulting in short stature in adulthood, an elevated BMI, disproportionate head growth, and, in some cases, precocious puberty or pubertal arrest. Therefore, ongoing monitoring and personalized care are necessary to address these long-term growth challenges.

Background: Diagnosing and predicting neonatal sepsis is challenging because of its nonspecific symptoms, lack of diagnostic criteria consensus, and absence of early, sensitive, and specific diagnostic laboratory tests.

Purpose: To evaluate the diagnostic and prognostic potential of adrenomedullin (ADM), interleukin-6 (IL-6), and C-reactive protein (CRP) in late-onset neonatal sepsis (LOS).

Methods: We studied 53 neonates with culture-proven LOS by sampling at admission and on antibiotic treatment days 3 and 7. These data were compared with those of 22 healthy full-term controls sampled on day 3 before hospital discharge. Survivors and nonsurvivors in the sepsis group were analyzed separately.

Results: Coagulase-negative Staphylococcus was the most commonly detected pathogen. ADM (cutoff, 0.5 ng/mL) and CRP (cutoff, <5 mg/L) values aligned with manufacturer recommendations, while IL-6 levels (cutoff, 10 pg/mL) were higher than expected, likely due to labor stress. The median biomarker levels significantly distinguished neonates with sepsis from controls (P<0.0001) at all time points with ADM and IL-6 levels elevated at admission, indicating their potential as early diagnostic markers. CRP level was diagnostically useful starting on day 3. Prognostically, IL-6 (P<0.001) and ADM (P<0.05) differentiated survivors from nonsurvivors; however, only IL-6 consistently predicted mortality at all time points (area under the curve [AUC] >0.90). ADM and CRP levels showed poor prognostic value (AUC<0.70). ADM and IL-6 demonstrated strong diagnostic utility in early LOS, whereas CRP became relevant later. IL-6 was the only reliable biomarker for predicting mortality, supporting its integration into clinical protocols. Combining IL-6 with CRP may enhance early detection and management, potentially improving neonatal outcomes.

Conclusion: IL-6 is a robust biomarker for the early diagnosis and prognosis of LOS. Incorporating IL-6 into clinical practice with CRP could improve early neonatal LOS diagnosis and patient outcomes.

Background: Coronavirus disease 2019 (COVID-19) vaccination remains an essential strategy for reducing disease burden. Specific guidelines for vaccinating children with systemic lupus erythematosus (SLE) are currently unavailable, highlighting the gap in tailored recommendations for this population.

Purpose: This study aimed to estimate parental intention to vaccinate children with SLE against COVID-19 and identify factors associated with this intention. It also explored parents' attitudes toward the vaccine.

Methods: Seventy-four parents of patients aged 5-21 years who were diagnosed with SLE before 18 years of age were surveyed regarding their willingness to further vaccinate their children with SLE against COVID-19. The parents were categorized into vaccine acceptance (VA) and vaccine hesitancy (VH) groups and completed a validated 6-item questionnaire designed to gauge their attitudes toward the vaccine. Vaccine hesitancy scale (VHS) scores were calculated with higher scores indicating increased VH. The adjusted odds ratios (aOR) (95% confidence interval [CI]) for VA-associated factors were determined using multivariate analysis.

Results: Twenty-five parents (33.8%) were diagnosed with VH. Compared with the VH group, the VA group showed a higher frequency of previous COVID-19 vaccine uptake, completed immunization in children, and parental willingness to be vaccinated themselves. Children were older in the VA versus VH group. The mean total VHS score was significantly higher in the VH versus VA group. In a multivariate model of factors differing significantly between the VA and VH groups, parental willingness to vaccinate themselves (aOR, 5.0; 95% CI, 1.2-20.4), patient age (aOR, 1.4; 95% CI, 1.1-1.9), and VHS score on vaccine efficacy belief (aOR, 0.1 [0.0-0.5]) were significantly associated with VA.

Conclusion: A significant proportion of parents were hesitant to vaccinate their children with SLE against COVID-19. These insights underscore the importance of developing targeted educational interventions to address specific parental concerns and improve vaccine uptake in children with SLE.