中国当代儿科杂志最新文献

A 3-month-old female infant was admitted for incessant crying for 3 days. Examination revealed a rapidly growing massive retroperitoneal mass that was difficult to resect. Needle biopsy confirmed infantile fibrosarcoma. Initial chemotherapy with the VAC regimen (vincristine, actinomycin D, and cyclophosphamide) was administered, but the response was poor. The common fusion in infantile fibrosarcoma is ETV6-NTRK3, and next-generation sequencing detected an RBPMS-NTRK3 gene fusion in this patient. To our knowledge, this is the first reported case of infantile fibrosarcoma with RBPMS-NTRK3 fusion in China. Treatment with larotrectinib resulted in marked tumor shrinkage.

Objectives: To develop dynamic prediction models based on multiple postnatal time points to support early diagnosis and individualized intervention for bronchopulmonary dysplasia (BPD) in preterm infants with gestational age < 32 weeks.

Methods: Clinical data of 472 preterm infants with gestational age <32 weeks admitted to the Second Xiangya Hospital of Central South University between January 2016 and November 2020 were retrospectively analyzed. Multivariable logistic regression was applied to develop five independent prediction models at postnatal days 1, 7, 14, 21, and 28. The performance of the models was assessed using the area under the receiver operating characteristic curve (AUC) and the Hosmer-Lemeshow test.

Results: Baseline characteristics such as gestational age and birth weight differed significantly between the BPD group (n=147) and the non-BPD group (n=325) (P<0.05). Predictors of BPD evolved across time points: on day 1, key predictors included gestational age, birth weight, Score for Neonatal Acute Physiology II (SNAP-II), invasive mechanical ventilation, and fraction of inspired oxygen >30%; by day 7, additional variables emerged, including fasting duration >2 days, mean feeding advancement rate <8.5 mL/(kg·d), neonatal respiratory distress syndrome, apnea of prematurity, and positive sputum culture; from day 14 onward, nutrition- and treatment-related indicators were incorporated additionally. The models demonstrated good discrimination at postnatal days 1, 7, 14, 21, and 28, with AUCs of 0.917, 0.927, 0.939, 0.944, and 0.968, respectively, and good calibration (Hosmer-Lemeshow P>0.05). Internal validation showed AUCs ranging from 0.899 to 0.958, indicating robust performance.

Conclusions: Dynamic postnatal prediction models incorporating indicators spanning perinatal factors, respiratory support, nutritional management, and therapeutic interventions demonstrate high predictive performance and facilitate dynamic risk assessment for BPD in preterm infants with gestational age < 32 weeks.

Objectives: To evaluate the efficacy and safety of ibuprofen in treating hemodynamically significant patent ductus arteriosus (hsPDA) in preterm infants of different postnatal ages at treatment initiation.

Methods: Clinical records of infants with gestational age <37 weeks who received ibuprofen for hsPDA in the Department of Neonatology, Fourth Hospital of Shijiazhuang, from January 2020 to December 2023 were retrospectively reviewed. One hundred eligible infants were divided by the postnatal age at the first ibuprofen administration into three groups: group A (≤4 days), group B (5-7 days), and group C (>7 days). Clinical efficacy and safety indicators were compared among groups.

Results: After treatment, cure rates were 92% in group A, 72% in group B, and 60% in group C, and effective rates were 8%, 25%, and 33%, respectively. Differences in clinical efficacy among the three groups were statistically significant (P<0.05). No significant differences were observed among groups in the incidence of pulmonary hemorrhage, gastrointestinal bleeding, cholestasis, bronchopulmonary dysplasia, necrotizing enterocolitis, intracranial hemorrhage, or acute kidney injury (P>0.05).

Conclusions: For hsPDA requiring pharmacologic therapy, earlier oral ibuprofen administration yields a higher ductal closure rate and does not increase the incidence of adverse events.

The incidence of pediatric inflammatory bowel disease (IBD) is rising, with an especially high proportion of early-onset cases in Asia. Conventional treatments such as glucocorticoids and immunosuppressants have limited efficacy and notable adverse effects, whereas biologic therapies substantially improve remission rates and quality of life. Therapeutic drug monitoring (TDM), by assessing trough concentrations and anti-drug antibodies, enables individualized dose optimization, reduces immunogenicity, and prolongs treatment persistence. However, challenges remain, including insufficient standardization and the lack of pediatric-specific concentration thresholds. This review summarizes recent advances in biologics and TDM in pediatric IBD to inform precision treatment.

Objectives: To explore the effect of rituximab on cellular immunity and cytokines in children with new-onset steroid-sensitive nephrotic syndrome (SSNS).

Methods: Clinical data of 60 children with new-onset SSNS treated at Xuzhou Children's Hospital from December 2021 to March 2023 were retrospectively analyzed. Children were allocated according to rituximab use into a control group (no rituximab) and an observation group (rituximab). The relapse rate, T-lymphocyte subsets and cytokines before and after treatment, and the incidence of adverse reactions were compared between groups.

Results: The relapse rate was lower in the observation group than in the control group (27% vs 73%, P<0.05). After treatment, CD3⁺ and CD4⁺ T-lymphocyte counts, the CD4⁺/CD8⁺ ratio, and serum interleukin-2 increased in the observation group and were higher than in the control group (P<0.05). Interleukin-6 and tumor necrosis factor-α levels decreased after treatment in the observation group and were lower than in the control group (P<0.05). After treatment, CD8⁺ T-lymphocyte counts decreased, interferon-γ increased, and interleukin-10 decreased in both groups, with no significant differences between the two groups (P>0.05). The incidence of adverse reactions did not differ significantly between the two groups (P>0.05).

Conclusions: Rituximab can reduce the relapse rate in children with new-onset nephrotic syndrome and shows good safety. Its therapeutic effect is achieved by regulating the number and function of T cells and by modulating the anti-inflammatory effects of cytokines.

With continuous advancements in neonatal care in China, the survival rate of extremely preterm infants has markedly increased in recent years. However, the proportion of voluntary withdrawal of treatment for extremely preterm infants remains relatively high. To further improve the overall treatment success rate for extremely preterm infants in Hunan Province, the Hunan Neonatal Medical Quality Control Center, in collaboration with the Perinatal Committee of the Hunan Medical Association, organized experts to develop recommendations on antenatal counseling for extremely preterm infants. These recommendations aim to standardize antenatal counseling procedures, enhance the scientific rigor and consistency of clinical decision-making, and improve survival outcomes.

Objectives: To summarize the clinical and genetic characteristics of children with sodium taurocholate co-transporting polypeptide (NTCP) deficiency.

Methods: Clinical data of children with NTCP deficiency diagnosed and treated at the First People's Hospital of Yunnan Province from July 2022 to March 2025 were retrospectively analyzed.

Results: A total of 14 children were included (6 males, 8 females), all with normal growth and development. Reasons for initial consultation included elevated serum bile acids in 7 cases, jaundice in 4 cases, cholestatic hepatitis in 1 case, and one case each of pneumonia and cow's milk protein allergy. At the first visit, all patients had elevated serum total bile acids beyond the normal range, with a mean of 152.5 μmol/L. Elevated alanine aminotransferase was observed in 1 case, elevated aspartate aminotransferase in 2 cases, and elevated total bilirubin in 10 cases. Genetic sequencing revealed that all children carried the homozygous SLC10A1 variant c.800C>T (p.Ser267Phe), classified as likely pathogenic.

Conclusions: NTCP deficiency often lacks obvious clinical symptoms and signs. Some children present with transient hyperbilirubinemia, cholestasis, or other liver function abnormalities. Persistent isolated elevation of serum bile acids warrants suspicion for this disease. Biallelic pathogenic variants in SLC10A1 constitute the basis for definitive diagnosis. There is no specific treatment for this disease, and management is mainly symptomatic.

Objectives: To explore the relationship between frontal alpha asymmetry (FAA) assessed by electroencephalogram (EEG) and emotion dysregulation (ED) in children with attention deficit hyperactivity disorder (ADHD).

Methods: Children with ADHD admitted to Fuyang Women and Children's Hospital from September 2021 to December 2024 were prospectively enrolled (n=104). Based on the Achenbach Child Behavior Checklist (CBCL), participants were classified into an ED group (sum of three subscales <180; n=41) and a non-ED group (sum ≥180; n=63). Clinical data were collected, and the Chinese ADHD SNAP-IV parent version and the Weiss Functional Impairment Rating Scale-Parent Report were used. FAA was measured by EEG. Correlations between FAA in different regions and CBCL score were analyzed, and the predictive value of FAA for ED was evaluated with multivariable logistic regression and receiver operating characteristic curves.

Results: Compared with the non-ED group, the ED group had a higher proportion of the predominantly inattentive ADHD subtype, higher SNAP-IV total score, higher Weiss Functional Impairment Rating Scale-Parent Report total score, and higher FP1/FP2-FAA and C3/C4-FAA (P<0.05). FP1/FP2-FAA and C3/C4-FAA were negatively correlated with CBCL score (P<0.05). The multivariable logistic regression analysis showed that FP1/FP2-FAA and C3/C4-FAA were closely associated with ED in children with ADHD (P<0.05). The areas under the curve for predicting ED using FP1/FP2-FAA, C3/C4-FAA, and their combination were 0.827, 0.685, and 0.917, respectively (P<0.05), and the combined prediction had a higher area under the curve than either single marker (P<0.05). The FP1/FP2 FAA value in hyperactive-impulsive ADHD was lower than in combined-type ADHD and predominantly inattentive ADHD (P<0.05).

Conclusions: FP1/FP2-FAA and C3/C4-FAA are reliable neural markers of emotion dysregulation in children with ADHD, and their combination shows superior predictive performance. ADHD subtypes show distinct patterns of FAA-functional impairment associations.

Objectives: To study the clinical characteristics, imaging findings, pathological features, treatment methods, and prognosis of nasopharyngeal carcinoma in children.

Methods: The clinical data of 6 children (<18 years) with nasopharyngeal carcinoma admitted to the Department of Pediatrics, Beijing Tongren Hospital from March 2021 to February 2025 were retrospectively reviewed, and outcomes were followed up.

Results: All 6 patients were male. Age at onset ranged from 11 to 15 years, with a median of 12.5 years. The interval from onset to diagnosis was 1-6 months. The main clinical symptoms were cervical mass (4 cases, 67%) and headache (3 cases, 50%); some patients also had nasal congestion and rhinorrhea, epistaxis, or limited mouth opening. TNM staging was stage III in 4 cases and stage IVa in 2 cases. All patients had non-keratinizing undifferentiated nasopharyngeal carcinoma on pathology. Treatments included radiotherapy, chemotherapy, surgery, targeted therapy, and immunotherapy. By July 1, 2025, after comprehensive treatment, 5 patients (83%) achieved complete remission, and 1 patient (17%) experienced recurrence and progression and died.

Conclusions: Comprehensive treatment based on combined radiotherapy and chemotherapy is the mainstay for childhood nasopharyngeal carcinoma, overall prognosis is favorable, and long-term follow-up is required. Molecular targeted therapy and immunotherapy are expected to improve the prognosis of advanced nasopharyngeal carcinoma and require further research for validation.

Objectives: To evaluate and integrate evidence on the management of sleep disorders in children with attention deficit hyperactivity disorder (ADHD).

Methods: Literature was retrieved based on the 6S model, and evidence related to sleep disorder management in children with ADHD was extracted from the included references.

Results: A total of 17 studies were included, from which 16 pieces of evidence were extracted. Of these, 6 were classified as Level 1 evidence and 10 as Level 5. The evidence covered screening, assessment, non-pharmacological interventions, pharmacological interventions, follow-up, and multidisciplinary collaboration.

Conclusions: This study integrated evidence on the management of sleep disorders in children with ADHD using an evidence-based approach, providing an evidence-based foundation for managing sleep disorders in this population.