With advances in perinatal medicine and improved care for extremely preterm infants, the incidence of severe respiratory complications—such as neonatal respiratory distress syndrome (NRDS)—has risen in recent years. Although the diagnosis and management of NRDS in China have made substantial progress, rates of severe complications, including severe intracranial hemorrhage and bronchopulmonary dysplasia (BPD), remain markedly higher than those observed in high-income countries in Europe and North America. Current clinical practice faces several critical challenges, including difficulties in the early differentiation of NRDS from neonatal acute respiratory distress syndrome (NARDS), which may delay timely and appropriate interventions. Furthermore, key therapeutic decisions lack robust evidence or consensus, such as the optimal timing of pulmonary surfactant administration, selection of invasive ventilation strategies, and post-extubation respiratory support approaches—some guidelines exist but are often based on limited or low-quality data. Leveraging national strategic initiatives and multicenter collaborative platforms like the China Neonatal Network (CHNN), Chinese researchers have recently published a series of high-impact studies on NRDS in leading journals including The BMJ, directly addressing these unresolved clinical questions and generating high-quality, context-specific evidence to improve outcomes. This article reviews the current landscape of NRDS management, highlights pivotal recent findings, and outlines future research priorities, in order to enhance the cognitive level of chinical physicians.

Objective Both preterm and postterm pregnancies can adversely affect neonatal outcomes; however, research on the differences in neonatal outcomes across various gestational ages within the full-term range (37⁺⁰ to 41⁺⁶ weeks) remains limited. Therefore, this study aims to explore the potential association between the gestational week of delivery within full-term pregnancy and adverse perinatal outcomes using a retrospective pregnancy cohort from a single center. Methods A retrospective cohort of pregnant women who delivered at hospital between January 2023 and December 2024, and their neonates, was included. Maternal and neonatal characteristics, as well as adverse outcomes [including large for gestational age (LGA), small for gestational age (SGA), low birth weight (LBW), macrosomia, 5-minute Apgar score≤7, and stillbirth], were compared across different gestational weeks. Restricted cubic spline (RCS) models and multivariable logistic regression were employed to analyze the association between gestational week at delivery and adverse perinatal outcomes. Results A total of 13,123 singleton full-term natural delivery cases were included from 43,502 parturients. They were included and categorized into five groups according to gestational week at delivery: the 37⁺⁰-37⁺⁶ weeks group (n=952), the 38⁺⁰-38⁺⁶ weeks group (n=2,707), the 39⁺⁰-39⁺⁶ weeks group (n=4,920), the 40⁺⁰-40⁺⁶ weeks group (n=4,164), and the 41⁺⁰-41⁺⁶ weeks group (n=380). The proportion of advanced maternal age (≥35 years) decreased with increasing gestational age, declining from 18.2% in the 37⁺⁰-37⁺⁶ weeks group to 10.0% in the 41⁺⁰-41⁺⁶ weeks group. The distribution of pre-pregnancy BMI categories differed significantly among the gestational age groups (P<0.001), with the 37⁺⁰-37⁺⁶ weeks group having higher proportions of overweight and obesity (10.7% and 1.7%, respectively). Gestational weight gain also varied significantly across groups (P<0.001), with the 41⁺⁰-41⁺⁶ weeks group exhibiting greater weight gain during pregnancy. The incidence of gestational diabetes mellitus (GDM) and hypertensive disorders of pregnancy/preeclampsia differed significantly among gestational age groups (P<0.001), both being most prevalent in the 37⁺⁰-37⁺⁶ weeks group (27.2% and 4.3%, respectively). There were 15 stillbirths (0.1%), 13,108 surviving newborns, among whom 423 newborns (3.2%) were LGA, 799 (6.1%) were SGA, 166 (1.3%) were macrosomia, 180 (1.4%) were LBW, and 9 (0.1%) had a low Apgar score at 5 minutes. Restricted cubic spline analysis initially suggested that 39⁺⁰-40⁺⁶ weeks of gestation might represent a window of lower risk for adverse neonatal outcomes. Multivariate logistic regression further confirmed that, compared with delivery at 39⁺⁰-39⁺⁶ weeks, delivery at 37⁺⁰-37⁺⁶ weeks and 38⁺⁰-38⁺⁶ weeks was associated with a significantly increased risk of SGA (P<0.01); delivery at 40⁺⁰-40⁺⁶ weeks and 41⁺⁰-41⁺⁶ weeks was associated with a significantly increased risk of macrosomia (P<0.001); delivery at 37⁺⁰-37⁺⁶ weeks and 38⁺⁰-38⁺⁶ weeks was associated with a significantly increased risk of LBW (P<0.001); the 41⁺⁰-41⁺⁶ weeks group had an increased risk of a 5-minute Apgar score≤7 (P=0.029); and delivery at 37⁺⁰-37⁺⁶ weeks and 38⁺⁰-38⁺⁶ weeks was associated with a significantly increased risk of stillbirth (P<0.01). Conclusions The timing of delivery in full-term pregnancies may affect neonatal outcomes. The period from 39⁺⁰ to 40⁺⁶ weeks of gestation is a window during which the risks of various adverse outcomes are at relatively low levels. This study may have potential value for further optimizing clinical pregnancy monitoring and perinatal management.

Objective To investigate the clinical characteristics and risk factors for plastic bronchitis (PB) in children with macrolide-resistant Mycoplasma pneumoniae pneumonia (MRMPP). Methods A retrospective analysis was conducted on 156 children hospitalized with MRMPP in the Department of Respiratory between October 2023 and January 2024. Based on electronic bronchoscopy findings, patients were divided into a PB group (n=44) and a non-PB group (n=112). Clinical characteristics and laboratory indicators were compared between the two groups. Continuous variables showing statistical significance in univariate analysis were analyzed using receiver operating characteristic (ROC) curves to determine optimal cut-off values, which were then used to convert these variables into categorical variables. Independent risk factors were identified via multivariate binary logistic regression analysis. Results Univariate analysis revealed that the PB group had significantly higher incidences of pleural effusion, severe pneumonia, oxygen therapy support, as well as significantly longer hospitalization duration, older age, longer fever duration, and higher levels of C-reactive protein, procalcitonin, D-dimer, lactate dehydrogenase (LDH), and M. pneumoniae DNA in bronchoalveolar lavage fluid (BALF) compared to the non-PB group (all P<0.05). ROC curve analysis determined the optimal cut-off values for D-dimer, LDH, and BALF M. pneumoniae DNA to be 1.655 mg/L, 364.5 IU/mL, and 428500 copies/mL, respectively. Multivariate logistic regression analysis identified D-dimer>1.655 mg/L (OR=14.002, 95% CI: 4.063-48.251), LDH>364.5 IU/mL (OR=6.865, 95% CI: 2.251-20.933), and BALF M. pneumoniae DNA>428500 copies/mL (OR=12.306, 95% CI: 3.586-42.236) as independent risk factors for PB in MRMPP (all P<0.001). Conclusion Elevated levels of D-dimer, LDH, and M. pneumoniae DNA in BALF are independent risk factors for PB in children with MRMPP. Monitoring these indicators is crucial for the early identification of children at risk of developing PB.

Objective To analyze the clinical characteristics and genetic phenotypes of children and adolescents with metastatic pheochromocytoma and paraganglioma (PPGL) at initial diagnosis and recurrence or metastasis. Methods A retrospective analysis was conducted on the clinical data, catecholamine levels, and genetic characteristics of 41 children and adolescents with metastatic PPGL at the initial diagnosis and after recurrence and metastasis, who were admitted to the hospital from January 2014 to October 2024. Results Hypertension was the most common manifestation at both the initial diagnosis (76.9%) and recurrence and metastasis (80.6%) of PPGL in children and adolescents. However, paroxysmal hypertension was more common at the initial diagnosis (56.7%), while persistent hypertension was more prevalent at recurrence and metastasis (80.0%), with a statistically significant difference (P=0.008). The incidence rates of headache, typical triad, nausea/vomiting, and fatigue were significantly different between the initial diagnosis and recurrence and metastasis stages (all P<0.05). Laboratory tests showed that abnormal urine catecholamines were most prominently characterized by elevated urine norepinephrine (NE) at both the initial diagnosis (86.5%) and recurrence and metastasis (90.3%), and the proportion of patients with two or more abnormal urine catecholamines was over 50% in both stages (54.1% and 54.8%, respectively). Lymph nodes were the most common site of recurrence and metastasis (68.3%), and 80.5% of the patients had two or more sites of recurrence and metastasis. 64.5% of the PPGL patients carried pathogenic gene variations, with SDHB mutations being the most common (45.2%). The median progression-free survival (PFS) of the group with clear gene variations was 42 months, significantly shorter than that of the group without detected gene variations (115 months), with a statistically significant difference (χ²=4.91, P=0.027). Conclusion Hypertension is the core manifestation of PPGL in children and adolescents at both the initial diagnosis and recurrence and metastasis stages, but the types of hypertension are significantly different. Recurrence and metastasis often present as multifocal and multi-site. Pathogenic gene variations may be related to the long-term prognosis of children, and further validation is needed through larger sample sizes and longer follow-up periods in future cohort studies.

Objective X-linked myotubular myopathy (XLMTM) caused by MTM1 gene variation is a rare neuromuscular disease. Currently, the systematic summary of the early natural history and diagnostic clinical characteristics of XLMTM patients in China is still incomplete. Methods We retrospectively reviewed clinical data, including perinatal findings, neonatal phenotype, genetic results, and outcomes, of male neonates with genetically confirmed severe XLMTM who were admitted to the neonatal intensive care units (NICUs) in two hospitals, between January 2018 and August 2024. And the early life natural history of this disease was systematically summarized. Results Six male patients were included in this cohort. The average gestational age was (37.5±2.6) weeks. Three cases were indicated with polyhydramnios by prenatal ultrasonography, one case was found with a racquet-shaped placenta at 24 weeks of gestation, and two cases had a family history of multiple early deaths among male infants within the same lineage. Neonatal asphyxia occurred immediately after birth in 6 children, with Apgar scores ranging from 3 to 6 at 1 minute and 4 to 7 at 5 minutes. All the patients presented with severe neonatal respiratory failure and characteristic profound hypotonia/floppy, and required continuous mechanical ventilation, nutritional support and nasogastric feeding throughout the course. All the patients had secondary hole-type atrial septal defect (ASD type II), with defect diameters ranging from 2.0 to 4.5 mm. Associated findings included cryptorchidism, micrognathia and slender limbs. All patients died within 6 months, with a median survival of 32 (3-150) days. The leading causes of death included respiratory failure, infection, and withdrawal of care. Genetic analysis revealed five maternally inherited variants and one de novo mutation in the MTM1 gene; five variants were located in the Rac1-induced recruitment domain (RID), and one in the protein tyrosine phosphatase (PTP) catalytic domain. Conclusions Severe XLMTM neonates exhibit a highly uniform clinical phenotype. The core features of its natural history, including neonatal asphyxia, respiratory failure, characteristic profound hypotonia, specific dysmorphic features and limb anomalies, serve as key diagnostic pointers. This cohort suggests that the natural history of XLMTM neonates in China may differ from international reports, and different types of MTM1 gene variations can all lead to extremely severe phenotypes. Given that most patients show early warning signs such as polyhydramnios during the perinatal period, and the disease progresses rapidly with an extremely poor prognosis, the importance of early genetic screening and diagnosis for high-risk pregnant women and their fetuses is underscored.

Objective To investigate the clinical features and immune mechanisms of post-viral encephalitis autoimmune encephalitis (PVEAE) caused by human herpesvirus 7 (HHV-7) encephalitis in children. Methods A retrospective analysis of clinical manifestations, cranial MRI, electroencephalogram (EEG), and other clinical data from four pediatric patients during the viral encephalitis (VE) phase and post-viral encephalitis with autoimmune encephalitis (PVEAE) phase. Clinical indicators including cytokines and antibodies associated with autoimmune encephalitis in cerebrospinal fluid (CSF) during the PVEAE phase were measured. The clinical characteristics and immune mechanisms of HHV-7 encephalitis complicated by PVEAE were analyzed, and diagnostic and therapeutic experiences were summarized. Results The median age of onset in the 4 pediatric patients was 7 years. The average interval between the onset of HHV-7 encephalitis and the emergence of PVEAE symptoms was 17 days. All 4 patients exhibited symptoms during the PVEAE phase, including fever, seizures, psychiatric and behavioral abnormalities, cognitive impairment, and speech disorders. During the PVEAE phase, 3 patients tested positive for anti-NMDAR antibodies and 1 for anti-neurexin-3a antibodies. CSF levels of IL-6 and IL-8 were significantly elevated in all cases. New lesions were identified on cranial MRI in 3 patients during the PVEAE phase, primarily involving the frontal-parietal lobes, commissural region of the corpus callosum, right thalamus, and left hippocampus. Four patients received first-line immunosuppressive therapy. All demonstrated favorable outcomes at 6-to 12-month follow-up, though mild neurobehavioral sequelae persisted, including attention deficits and memory impairment. One patient developed mild enuresis and refractory epilepsy. Conclusion In children with HHV-7 encephalitis, paradoxical deterioration during the remission phase warrants consideration of concurrent PVEAE. During the PVEAE phase, CSF cytokine levels of IL-6 and IL-8 significantly increase, indicating that immune-inflammatory responses constitute the primary pathogenesis of PVEAE.

Objective To investigate the clinical phenotypes, pathological characteristics, genetic variants, and treatment outcomes in children with C3 glomerulopathy (C3G), and to provide evidence for precise diagnosis and management. Methods Clinical, pathological, and genetic data from four pediatric patients diagnosed with C3G at the Department of Nephrology between July 2021 and March 2024 were retrospectively analyzed. Additionally, published cases of C3G in Chinese children from both domestic and international literature were systematically reviewed and summarized to contextualize our findings. Results Four pediatric patients with C3G were included in the study, comprising two boys and two girls aged 10 to 12 years. All presented with nephrotic syndrome and hematuria, and three exhibited renal insufficiency at initial presentation. Laboratory evaluation revealed reduced serum complement C3 levels (0.9-1.8 g/L) in all patients, decreased C4 in two, and elevated membrane attack complex (C5b-9) in all. Renal histopathology demonstrated diffuse mesangial cell and matrix proliferation in all cases. Immunofluorescence showed predominant C3 deposition, and electron microscopy confirmed a diagnosis of C3 glomerulonephritis (C3GN) in three patients and dense deposit disease (DDD) in one. Whole exome sequencing was performed in three patients, identifying a single missense variant in the C3 gene (c.4887G>C, p.Glu1629Asp) inherited from a phenotypically healthy mother; this variant was classified as having uncertain clinical significance according to ACMG criteria. All patients received immunosuppressive therapy combining glucocorticoids with other agents; eculizumab was added in two refractory cases. With follow-up of 3 to 8 months, all three patients with initial renal insufficiency achieved normalization of renal function, and proteinuria decreased in all four. Two patients attained complete remission, one achieved partial remission, and one showed no response. Conclusions Pediatric C3G predominantly manifests as nephrotic syndrome with persistent hypocomplementemia. Key pathological features include diffuse mesangial proliferation and dominant C3 deposition on immunofluorescence. Genetic variants are infrequently detected, suggesting potential non-monogenic mechanisms in many cases. Immunosuppressive regimens based on glucocorticoids remain the mainstay of therapy, while eculizumab may offer therapeutic benefit in selected refractory cases. Early integration of renal biopsy, complement profiling, and genetic analysis facilitates timely and accurate diagnosis. Long-term outcomes require further assessment through extended follow-up and larger cohort studies.

Objective Cardiovascular aneurysm is an extremely rare and serious complication of Behcet syndrome (BS) in children, lacking clinical specificity and with very few studies. This study aims to explore the clinical characteristics and treatment strategies of BS complicated with aneurysms in children. Methods A retrospective analysis was conducted on the clinical data of 3 children with BS complicated with aneurysms who were admitted to hospital from December 2018 to March 2023. Results A total of 36 children with BS were included during the same period, among whom 3 patients (8.3%) developed aneurysms, including 2 boys and 1 girl. The age range of BS onset was 5 to 13 years old, and the time of aneurysm discovery was 2 months to 5 years after the onset of BS. Two cases involved pulmonary artery aneurysms (PAAs) with in-situ thrombosis, while one had an aneurysm at the right coronary sinus of the aorta. All patients presented with concurrent vascular pathologies (deep vein thrombosis, pulmonary arterial thrombosis, and aortic dissection). At aneurysm detection, all exhibited fever and elevated inflammatory markers, with no gastrointestinal involvement. All three patients were treated with corticosteroids combined with immunosuppressants (cyclophosphamide/methotrexate) and biologics (TNF-α inhibitors/tocilizumab), among whom two patients received surgical intervention (left lower lobectomy in Case 1 and Bentall procedure in Case 2). Following medical therapy, Cases 1 and 3 achieved extravascular stability, but PAAs showed no regression. During corticosteroid tapering, Cases 1 and 2 experienced recurrence with new organ damage (Case 1 involved the nervous system, and Case 2 involved the skin, heart, and intestines). Conclusions In children with BS, pulmonary artery aneurysms are the most common type of arterial aneurysm involvement. The condition often has an insidious onset and is frequently associated with other vascular lesions. Regular vascular imaging screening is recommended for children with elevated inflammatory markers and no gastrointestinal involvement. Treatment should be individualized, potentially combining immunosuppressive therapy and surgical intervention, as some cases exhibit steroid dependence.

The article of "The association of gestational age at term and adverse perinatal outcomes: a retrospective study based on 43502 Chinese pregnant women" by Liu Xiaoyang and co-authors published in this issue, was based on the clinical data of 13,123 singleton full-term and natural delivery mothers from West China Second University Hospital of Sichuan University. It systematically analyzed the impact of different delivery timings within 37⁺⁰ to 41⁺⁶ weeks on neonatal outcomes, and pointed out that 39⁺⁰ to 40⁺⁶ weeks is a "window period" with a relatively low risk of adverse outcomes. This study had a clear research idea and advanced statistical methods, especially in handling the non-linear relationship between gestational age and outcomes, it adopted the restricted cubic spline model, which had high clinical reference value. However, as a retrospective study, it also had certain limitations in causal inference, confounding control, and generalizability. This article, in combination with the review of this research report, makes a summary analysis of the value and limitations of retrospective studies.

Respiratory distress syndrome (RDS) represents a core clinical challenge in the management of extremely preterm infants. While the incidence of ventilator-associated lung injury has declined substantially, the failure rate of conventional nasal continuous positive airway pressure (NCPAP) remains persistently high among extremely preterm infants with very low gestational age. In recent years, noninvasive high-frequency oscillatory ventilation (NHFOV), as an emerging non-invasive ventilation modality, has garnered attention owing to its potential advantages in facilitating carbon dioxide elimination and preserving functional residual capacity. However, high-level evidence-based medical data supporting its efficacy and safety as an initial respiratory support strategy for extremely preterm infants are still lacking. Recently, the team led by Professor Shi from the Children's Hospital Affiliated to Chongqing Medical University published findings of a multicenter randomized controlled trial in The BMJ. This trial enrolled 342 preterm infants diagnosed with RDS, with gestational ages ranging from 24⁺⁰ to 28⁺⁶ weeks, across 20 neonatal intensive care units in China, thereby providing critical evidence addressing this pivotal clinical question. This article not only summarizes the landmark implications of the study but also identifies its inherent limitations, while offering commentary on its guiding value for clinical practice and future research endeavors.

Maintaining a normal body temperature is a core principle of preterm infant care during the delivery. Hypothermia, a common complication in preterm infants, is closely associated with adverse clinical outcomes. Studies have confirmed that the incidence of hypothermia in preterm infants is as high as 73.2%, with preterm infants born at a gestational age < 32 weeks being at particularly high risk. This condition is closely linked to maternal factors, mode of delivery, and delivery room environment. Future efforts should focus on strengthening the management of environments in delivery rooms, identifying high-risk factors for hypothermia, and implementing standardized body temperature maintenance during and after delivery to further improve the prognosis of preterm infants.

Acute upper respiratory tract infection (AURI) is the most prevalent infectious disease among children. However, standardized methods for evaluating symptoms have not garnered the necessary attention. Standardized assessment plays a pivotal role in evaluating the natural history of diseases and the efficacy of medications, particularly in clinical trials. Currently, in clinical trials focusing on pediatric AURI, unverified subjective symptom rating scales or self-developed Traditional Chinese Medicine (TCM) syndrome scales are frequently employed. These tools exhibit limited scientific validity and lack comparability across different studies. This article systematically reviews and compares four validated efficacy assessment scales for pediatric AURI, analyzing their structural frameworks, reliability, validity, and applicable scenarios, thereby providing evidence-based guidance for researchers and clinicians in selecting evaluation indicators. Additionally, this paper notes that TCM research still relies on syndrome rating scales without unified standards. It is proposed that future efforts should integrate evaluation frameworks from both TCM and Western medicine, and develop age-specific, multi-dimensional efficacy evaluation systems for pediatric AURI, with the aim of enhancing the standardization and clinical application value of related research.

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease with unknown etiology. Member of DNAJ heat shock protein family B9 (DNAJB9) protein may be involved in its pathogenesis. The clinical manifestations of FGN are mostly nephrotic syndrome, which can be combined with hematuria, hypertension and renal insufficiency. A few patients show rapid progressive glomerulonephritis. It has important diagnostic value by electron microscopic examination of renal tissue combined with immunohistochemistry of DNAJB9 in FGN patients.Unfortunately, there is no specific therapy at present, and the prognosis is poor. In the future, it is urgent to explore the potential therapeutic targets of FGN to improve the quality of life of FGN patients. This article reviews the possible pathogenesis and clinical characteristics of FGN.