Frontiers in Pediatrics最新文献

Introduction: Infants with congenital diaphragmatic hernia can suffer severe respiratory insufficiency and pulmonary hypertension after birth. Aerating the lungs before removing placental support (physiologically based cord clamping, PBCC) increases pulmonary blood flow (PBF) and reduces pulmonary vascular resistance (PVR) in lambs with a diaphragmatic hernia (DH). We hypothesized that these benefits of PBCC persist for at least 8 h after birth.

Methods: At ∼138 days of gestation age (dGA), 21 lambs with a surgically induced left-sided DH (∼86 dGA) were delivered via cesarean section. The umbilical cord was clamped either before ventilation onset (immediate cord clamping, ICC, n = 9) or after achieving a tidal volume of 4 ml/kg, with a maximum delay of 10 min (PBCC, n = 12). The lambs were ventilated for 8 h, initially with conventional mechanical ventilation, but were switched to high-frequency oscillatory ventilation after 30 min if required. Ventilatory parameters, cardiopulmonary physiology, and arterial blood gases were measured throughout the study.

Results: PBF increased after ventilation onset in both groups and was higher in the PBCC DH lambs than the ICC DH lambs at 8 h (5.2 ± 1.2 vs. 1.9 ± 0.3 ml/min/g; p < 0.05). Measured over the entire 8-h ventilation period, PBF was significantly greater (p = 0.003) and PVR was significantly lower (p = 0.0002) in the PBCC DH lambs compared to the ICC DH lambs. A high incidence of pneumothoraces in both the PBCC (58%) and ICC (55%) lambs contributed to a reduced sample size at 8 h (ICC n = 4 and PBCC n = 4).

Conclusion: Compared with ICC, PBCC increased PBF and reduced PVR in DH lambs and the effects were sustained for at least 8 h after ventilation onset.

Introduction: Musicality is an innate capability and the fundamental architectures necessary for music processing are present from birth. However, there is a notable gap in pediatric specific music neuroscience research and research that employs ecologically valid musical stimuli.

Methods: This pragmatic feasibility study aimed to assess the utility of EEG collected via pre-existing clinical monitoring to describe the processing of familiar song as an ecologically valid stimulus, in the underrepresented pediatric population. Three comparative auditory conditions (song, speech, and noise) were utilized to assess the changes in EEG across these conditions compared to a baseline silence.

Results: Analysis of EEG data from a pilot sample of four children revealed distinct changes in the underlying frequency components of the EEG during the song condition that were not observed in either the speech or noise conditions. To extend this analysis, a uniquely hypothesis-driven, multivariate statistical analysis method (generalized eigendecomposition [GED]) was employed, however in this study we did not isolate a consistent source responsible for the observed changes in the frequency components of the EEG during the song condition.

Discussion: The study is limited by the small sample size but nevertheless demonstrated feasibility of collecting EEG data in the imperfect auditory environment of an acute clinical setting to describe a response to an ecologically valid stimulus in the underrepresented pediatric population. Further research with a more restrictive study design and greater participant numbers is needed to extend these preliminary findings.

In adults, esophageal cancers are a global health concern. Esophageal squamous cell carcinoma (ESCC) accounts for approximately 90% of esophageal carcinomas. The prognosis of esophageal cancers remains dismal, with a five-year survival rate below 20%. It typically affects older patients, and for now, ESCC after esophageal atresia has not been reported in patients younger than 18 years. We present an exceptional case of an ESCC in a 13-year-old boy with a history of esophageal atresia and corrective surgery in infancy. After the surgery the patient was lost to surgical follow up for over ten years and then presented to our emergency department with respiratory distress requiring antibiotic therapy and supplemental oxygen. Radiologic imaging revealed a volume reduction of the right lung with bronchiectasis, as well as esophageal stenosis at the level of the previous anastomosis, with an adjacent abscess in the right lung. These changes may have arisen due to a chronic fistula from the esophagus to the right lung. Initial interventional therapy with a stent implantation had no lasting success and, in an effort to prevent further aspiration into the right lung, a cervical esophagus stoma was established, and the patient received prolonged antibiotic treatment. However, a thoracic CT scan performed 4 months later revealed a large, retrospectively progressive prevertebral mass originating from the distal portion of the esophagus below the stenosis, compressing the trachea and the right main bronchus. The patient's condition rapidly worsened and he developed respiratory failure, requiring veno-venous extracorporeal membrane oxygenation. Unfortunately, an endoscopic biopsy revealed an advanced ESCC. With no rational treatment options available, we changed the goals of care to a palliative setting. The key message of this case is that in adolescents with chronic infections, an abscess can potentially mask a malignant transformation. Therefore, in adolescents, with an history of corrective surgery for esophageal atresia and chronic complications, consideration should also be given to the possibility of squamous cell carcinoma of the esophagus.

Objective: To explore the efficacy of sandplay therapy in intervening social communication deficits in children with autism spectrum disorders (ASD), and whether this efficacy is influenced by the age of the children and the dosage of sandplay therapy intervention.

Methods: Following the PICOS principle, randomized controlled trials (RCTs) related to sandplay therapy for social communication deficits in ASD children were retrieved from seven databases: PubMed, WOS, The Cochrane Library, Embase, CNKI, Wanfang, and VIP, from the inception of each database to November 10, 2023. Two experimenters independently conducted study screening and excluded studies with concomitant diseases, incomplete data, unextractable data, and non-randomized controlled trials. The PEDro scale was used for methodological quality assessment, and the GRADEprofiler method was employed to evaluate the quality of evidence. Stata17 software was used for meta-analysis, subgroup analysis, sensitivity analysis, and publication bias testing. The standardized mean difference (SMD) and 95% confidence interval (CI) were used as the effect statistics.

Results: A total of 12 RCTs (791 cases) were included. Sandplay therapy had a positive impact on the social communication deficits of ASD children [SMD = -1.42, 95%CI (-1.79, -1.04), P < 0.001]. Subgroup analysis revealed that sandplay therapy administered during the early school age (449 cases, SMD = -1.44, P < 0.05), for a duration of 22-28 weeks (208 cases, SMD = 1.69, P < 0.05), and with a frequency of once per week (218 cases, SMD = -1.67, P < 0.05) was most effective in improving on social communication deficits of ASD children.

Discussion: The quality of evidence in this study was rated as high, with good methodological quality, including 12 studies with better quality and no detection of bias risk. The study had high heterogeneity, which was attributed to the measurement tools and intervention duration through subgroup analysis, with no inconsistency found. Additionally, no downgrade factors related to imprecision, publication bias, or indirectness were identified. In conclusion, sandplay therapy is an effective measure to improve social communication deficits in children with ASD, and current evidence recommends early intervention using an individual sandplay therapy or integrated sandplay therapy intervention program once a week for 22-28 weeks, which can serve as evidence-based clinical guidance.

Systematic review registration: www.crd.york.ac.uk, identifier (CRD420234821750).

Objective: To examine the measurements on enteral feeding intolerance (EFI) in critically ill children.

Methods: The Joanna Briggs Institute methods for conducting a scoping review were followed. Articles published since 2004 which assessed EFI in critically ill children were identified. A full search strategy was executed in seven English databases (MEDLINE, EMBASE, PubMed, Web of Science, Cochrane Central Register of Controlled Trials, JBI EBP, CINAHL) and four Chinese databases (CNKI, VIP, Wanfang, Sinomed). Two reviewers screened records according to our inclusion and exclusion criteria, and conducted a full-text review of selected articles. The reference lists of all studied selected were screened for additional sources. Relevant data was extracted using a researcher-developed tool.

Results: Of the 627 articles identified, 32 were included in this scoping review. Most articles focused on the measurement of high gastric residual volume (n = 22), followed by diarrhea (n = 20), and vomiting (n = 9). Most of the studies were of observational-analytic design (13/32) and experimental design (8/32).

Conclusion: This scoping review addressed the complexity and diversity of EFI measurements. Given the importance of adequacy of enteral nutrient intake, we highlighted the necessary to develop individual measurements of EFI, taking the age of children and disease condition into consideration. Further studies can also investigate accurate and objective physiological measurements of EFI to advance EN and improve outcomes in critically ill children.

Introduction: Recently, researchers have introduced the concept of 'early term' infants, defined as infants born at 37 or 38 weeks of gestation, and their outcome has been discussed. Although the complications experienced by early term are less severe than those in preterm infants, this group accounts for a much larger proportion of newborns, making the assessment of outcomes important in clinical practice.

Methods: This observational study of term infants born at Tokyo Metropolitan Toshima Hospital aimed to understand the short-term outcomes in early term infants. Data extracted from the medical records were analyzed.

Results: Among 4,669 eligible participants, 463 (9.9%) were born at 37 weeks and 1,270 (27.2%) were born at 38 weeks. The remaining 2,936 infants were born after 39 weeks of gestation. Logistic regression analysis showed higher odds ratio of hospitalization (1.56, 95% CI: 1.37-1.79, p < 0.05), apnea (2.23, 95%CI: 1.08-4.60, p < 0.05), and hypoglycemia (3.13, 95%CI: 1.95-5.03, p < 0.05) in early term infants. In detail, infants born at 37 weeks of gestational age had higher odds ratio for hospitalization (2.07, 95%CI: 1.68-2.35, p < 0.05) and hypoglycemia (4.11, 95%CI 2.22-7.60, p < 0.05) than infants born at 38 weeks of gestational age (1.40, 95%CI: 1.20-1.62, p < 0.05, and 2.78, 95%CI: 1.66-4.67, p < 0.0 respectively).

Discussion: This study revealed complicated clinical course in early term infants, and represents one of the largest contributions to understanding the outcomes of early term infants, and could help to determine strategies for elective cesarean section. According to this result, elective cesarean sections would be better planned at 38 weeks of gestational age. Moreover, in clinical practice, it is important to be aware of the complicated clinical course in early term infants.

Background: The exact mechanism of hyperammonemia is thought to be multifactorial, but is not yet fully understood. No studies have yet reported hyperammonemia combined with Fanconi syndrome caused by deferasirox.

Case presentation: A 10-year-old girl was admitted for vomiting and altered consciousness. Blood testing revealed hyperammonemia and normal liver and coagulation functions. During hospitalization, the patient also exhibited hyperchloremic metabolic acidosis, hypokalemia, hyponatremia, and hypophosphatemia. Additionally, urinalysis revealed glucose and protein levels clinically consistent with Fanconi syndrome. The patient had a history of severe beta-thalassemia and had received intermittent blood transfusions for approximately ten years. The patient had been administered oral deferasirox at a 400 mg/day dose at the age of four, which had been gradually increased to the current 750 mg/day dosage. Upon admission, deferasirox was discontinued and treatment including mechanical ventilation, continuous blood purification therapy for ammonia reduction and acidosis, and electrolyte imbalance corrections was administered. Subsequently, serological markers returned to normal, urine test findings improved. To the best of our knowledge, this is the first report of a case of hyperammonemia with Fanconi syndrome owing to deferasirox.

Conclusions: For effective management and long-term follow-up of chronic diseases in children, pediatricians must master standardized treatments and the adverse reactions of various drugs. When symptoms are difficult to explain clinically, we must trace the source and adjust the treatment plan to maximize improving the patient's prognosis.

Background: Functional constipation in children is a worldwide problem that impacts both children's gastrointestinal function and the quality of family life. The treatment of this condition often depends on parental involvement to administer rectal interventions to their children to stimulate defecation. However, adherence to rectal interventions is currently suboptimal. We sought to explore the factors that facilitate and hinder parents from adherence to rectal interventions.

Methods: A descriptive qualitative study was conducted involving semi-structured interviews with parents of infants and young children with functional constipation requiring rectal interventions from March to May 2023. The data were analyzed using content analysis.

Results: Fourteen parents participated in the study. Parents reported the main facilitators of adherence to prescribed rectal interventions as recognition of illness severity, support from family and friends, and medical resource support and e-health literacy. Parents reported the primary barriers as information barriers, family conflict, cognitive misalignment, and difficulties in accessing healthcare services.

Conclusion: Rectal interventions are often essential in managing constipation in young children, with parental compliance being crucial for effective treatment. Healthcare providers must consider the psychosocial aspects of parents' perceptions, adhere to guidelines to standardize communication, and ensure comprehensive education to improve medication literacy.

Introduction: Patients with childhood-onset systemic lupus erythematosus (c-SLE) have higher rates of depression than their peers, which has been associated with worse medical outcomes. Therefore, it is imperative that their mental health be addressed. We utilized quality improvement (QI) methodology to automate mental health screening for patients with lupus within a pediatric rheumatology clinic. The retrospective cohort study aims to evaluate the association between mental health screening outcomes and demographics, medications, and disease activity measures in patients with childhood lupus.

Methods: The mental health QI team at a quaternary pediatric rheumatology center implemented an automated process for mental health screening in patients with c-SLE. Patients seen between 2017 and June 2023 with a diagnosis of c-SLE were identified using International Classification of Disease -Clinical Modification (ICD-CM) codes. Disease activity was assessed with the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2K). Medications were identified on outpatient and inpatient orders for conventional synthetic and biologic disease-modifying anti-rheumatic drugs, hydroxychloroquine, corticosteroids, and aspirin. Mental health screening was accomplished with the Patient Health Questionnaire (PHQ). Descriptive statistics, univariate and multivariate linear regression were used.

Results: Between January 2017 and June 2023, 117 patients with c-SLE (41% with lupus nephritis) completed 534 total screenings. Each patient completed PHQ screenings, a median of 5 [interquartile range 2, 6] times. Screening increased when the screening process was automated. Those who were Black, female, or prescribed leflunomide, mycophenolate, and corticosteroids had higher PHQ scores.

Conclusions: Mental health support is essential for patients with chronic rheumatologic diseases such as SLE. Sustainable processes for quickly identifying depression are needed for optimal care of patients with SLE. Our process of automated, streamlined mental health screening successfully increased the screening of patients with SLE at every visit and led to timely interventions for positive PHQ scores. Higher PHQ scores were correlated with patients on leflunomide, mycophenolate, and corticosteroids. Future research should identify modifiable risk factors for high PHQ scores that the medical team can target.

Background: The effects of long-term glucocorticoid (GC) treatment on bone mineral density (BMD) in patients with congenital adrenal hyperplasia (CAH) remain controversial.

Objectives: This cross-sectional study aimed to evaluate BMD in relation to genotype, growth, vitamin D status, cumulative GC doses, and other relevant factors in youths with CAH.

Methods: Thirty-two patients with classical CAH (13 males; mean age 26.0 ± 7.1 years) were compared with 32 healthy controls matched by age and sex. BMD was measured using dual-energy x-ray absorptiometry, and statistical analyses, including the Mann-Whitney U-test and Spearman's correlation coefficient, were performed to evaluate differences and associations.

Results: Median whole-body and lumbar BMD Z-scores were similar between CAH patients and controls (p = 0.27 and 0.15, respectively). Low bone density was observed in 12.5% of CAH patients and 18.75% of controls (p = 0.5), and osteoporosis was confirmed in 12.5% of CAH patients and 0% of controls (p = 0.04). BMD did not correlate with cumulative GC doses, estradiol, renin, phosphate, sodium levels, or anthropometric parameters in CAH patients. There was no significant difference in BMD between severe and non-severe genotypes of CAH. However, a positive correlation was found between the whole-body BMD Z-score and growth velocity during infancy (r = 0.776, p = 0.021) in CAH patients. Vitamin D deficiency was noted in 56.25% of CAH patients, although vitamin D levels did not correlate with BMD or genotype. No history of bone fractures was reported among study participants.

Conclusions: CAH patients are at risk of developing osteoporosis, but in this study, BMD Z-scores were not associated with cumulative GC doses. The study did not identify an association between genotype and BMD. Poor growth during infancy was linked to decreased BMD in adulthood.