Comparative analysis of formulae for umbilical venous catheterization depth based on birth weight.

Importance: Umbilical venous catheterization (UVC) is a common procedure for critically ill newborn infants. The insertion depth was estimated before the procedure using various formulae.

Objective: To compare the accuracy of five published formulae based on birth weight (BW).

Clinical evaluation of bispectral index-guided closed-loop infusion of propofol for preschool children: A multi-center randomized controlled study.

Importance: The closed-loop infusion system can automatically adjust and maintain the depth of anesthesia by using the propofol target-controlled infusion (TCI) model under the feedback guidance of the bispectral index (BIS).

Objective: To evaluate the safety and superiority of closed-loop TCI of propofol guided by BIS during maintenance of generalized intravenous anesthesia for preschool children.

Methods: A total of 120 children aged 1-6 years were enrolled and were divided into a closed-loop feedback group (Group C) and an open-loop manual control group (Group O), with 60 participants in each group.

Real-world use of emicizumab in Chinese children with hemophilia A: Retrospective data from a comprehensive care center.

Importance: Emicizumab (EMI) is efficacious and safe for hemophilia A (HA) prophylaxis. However, its high cost poses a challenge in China.

Objective: To explore the possibility of using reduced-dosage EMI in Chinese HA children.

Nicotinamide mononucleotide supplementation rescues mitochondrial and energy metabolism functions and ameliorates inflammatory states in the ovaries of aging mice.

Noninvasive pharmacological strategies like nicotinamide mononucleotide (NMN) supplementation can effectively address age-related ovarian infertility by maintaining or enhancing oocyte quality and quantity. This study revealed that ovarian nicotinamide adenine dinucleotide levels decline with age, but NMN administration significantly restores these levels, preventing ovarian atrophy and enhancing the quality and quantity of ovulated oocytes. Improvements in serum hormone secretion and antioxidant factors, along with decreased expression of proinflammatory factors, were observed.

Neurophenotype and genetic analysis of children with Aicardi-Goutières syndrome in China.

Importance: Aicardi-Goutières syndrome (AGS) is a rare genetic disorder mainly affecting the central nervous system and autoimmunity. However, research on AGS among Chinese patients is limited.

Objective: To summarize the neurologic phenotypes and genetic causes in pediatric AGS patients, providing insights for early recognition and diagnosis in the Chinese population.

Relationship between fundamental motor skills and physical fitness in children with global developmental delay.

Importance: Understanding the significance of motor skills in promoting physical fitness (PF) can offer valuable insights for devising comprehensive intervention and clinical rehabilitation programs for children with global developmental delay (GDD). However, it remains unclear whether fundamental motor skills (FMS) can improve the PF of children with GDD.

Objective: To investigate the correlation between FMS and PF in children with GDD.

Technological Advances Are Associated With Better Clinical Outcomes of Percutaneous Coronary Intervention in Patients With Unprotected Left Main Disease.

Background: Few large-scale studies have evaluated the effectiveness of percutaneous coronary intervention (PCI) technological advances in the treatment of patients with unprotected left main coronary artery disease (LM-CAD). We aim to identify independent factors that affect the prognosis of PCI in patients with unprotected LM-CAD and to assess the impact of PCI technological advances on long-term clinical outcomes.

Methods And Results: A total of 4512 consecutive patients who underwent unprotected LM-CAD PCI at Fuwai Hospital from 2004 to 2016 were enrolled.

Lysinuric protein intolerance with novel mutations in solute carrier family 7A member 7 in a Chinese family.

Introduction: Lysinuric protein intolerance (LPI) is a rare genetic disorder caused by mutations in the solute carrier family 7A member 7 () gene.

Case Presentation: We presented two siblings with LPI, carrying novel mutations of c.776delT (p.

Low-dose immune tolerance induction for severe hemophilia A inhibitor patients: Immunosuppressants are generally not necessary for inhibitor-titer below 200 BU/mL.

Importance: It remained unclear that the efficacy comparison between low-dose immune tolerance induction (LD-ITI) incorporating immunosuppressants (IS) when severe hemophilia A (SHA) patients had inhibitor-titer ≥200 Bethesda Units (BU)/mL (LD-ITI-IS regimen) and LD-ITI combining with IS when SHA patients had inhibitor-titer ≥40 BU/mL (LD-ITI-IS regimen).

Objective: To compare the efficacy of the LD-ITI-IS regimen with that of the LD-ITI-IS regimen for SHA patients with high-titer inhibitors.

Methods: A prospective cohort study on patients receiving LD-ITI-IS compared to those receiving LD-ITI-IS from January 2021 to December 2023.

Somatic variants in a cohort of Chinese children with phakomatosis pigmentovascularis.

Importance: Postzygotic mutations in the genes, which encode the G-protein nucleotide binding protein alpha subunits, have been identified in patients with phakomatosis pigmentovascularis (PPV). However, little is known about the Chinese population.

Objective: To identify pathogenic mutations in pediatric patients with PPV within the Chinese population.

Long noncoding nuclear enriched abundant transcript 1_2 is a promising biomarker for childhood-onset systemic lupus erythematosus.

Importance: Systemic lupus erythematosus (SLE) is a diffuse connective tissue disease with complex clinical manifestations and prolonged course. The early diagnosis and condition monitoring of SLE are crucial to disease prognosis.

Objective: To assess the diagnostic value of long noncoding RNA (lncRNA) nuclear enriched abundant transcript 1 (NEAT1) in childhood-onset SLE (cSLE).

The clinical characteristics and risk factors for necrotizing soft tissue infection in children.

Importance: Necrotizing soft tissue infection (NSTI) is a serious infectious disease. However, the early clinical manifestations and indicators of NSTI in children are still unclear.

Objective: The purpose of this study was to analyze the clinical characteristics and risk factors of NSTI in pediatric patients.

Current situation and prospect for the diagnosis and treatment of pediatric critical rare diseases in China.

The onset of critical rare diseases (RDs) in children is rapid and dangerous, accompanied by a high mortality rate, which brings a heavy burden to both families and society. Multiple malformations, neuromuscular diseases, metabolic diseases, and heart diseases are the most common types of RDs in children of China, often manifesting with multiple organ dysfunction. At present, the diagnosis and treatment of critical RDs in children face challenges such as prolonged diagnosis time, a high misdiagnosis rate, limited treatment modalities, and a significant disease burden.

Importance of sample size determination for randomized controlled clinical trials for coronavirus disease 2019 antiviral therapies.

For the calculation of sample size for a randomized controlled clinical trial, choosing a proper, medium clinically meaningful difference in the size of the effect of the test and control interventions is important for avoiding too small or too large sample sizes.

Diagnose and treatment for Type D congenital esophageal atresia with tracheoesophageal fistula.

Importance: Type D esophageal atresia (EA) with tracheoesophageal fistula (TEF) is characterized by EA with both proximal and distal TEFs. It is a rare congenital anomaly with a very low incidence.

Objective: To investigate diagnostic and treatment strategies for this rare condition.

Spotlight on eltrombopag concentration in pediatric immune thrombocytopenia: A single-center observational study in China.

Importance: Eltrombopag has been recommended for pediatric immune thrombocytopenia (ITP). Response and adverse drug reactions (ADRs) varied widely between individuals, even at the same dose of eltrombopag. The appropriate eltrombopag concentration in ITP has not been reported.

Hepatoblastoma with neonatal necrotizing enterocolitis: Two case reports.

We report two children with hepatoblastoma (HB) with a history of neonatal necrotizing enterocolitis (NEC). Case 1 was diagnosed with HB at 5 months of age. Liver enlargement was found during the NEC operation at 3 months of age and then was clinically diagnosed by imaging.

Multilevel omics for the discovery of biomarkers in pediatric sepsis.

Severe sepsis causes organ dysfunction and continues to be the leading reason for pediatric death worldwide. Early recognition of sepsis could substantially promote precision treatment and reduce the risk of pediatric death. The host cellular response to infection during sepsis between adults and pediatrics could be significantly different.

Dwell time and bloodstream infection incidence of umbilical venous catheterization in China.

Importance: Central line-associated bloodstream infection (CLABSI) is one of the most serious complications of central venous access devices. Reducing the risk of CLABSI is of utmost significance in efforts to improve neonatal mortality rates and enhance long-term prognosis.

Objective: To determine the dwell time and incidence of CLABSI of umbilical venous catheterization (UVC) for preterm infants in China.

Recurrent infection triggered encephalopathy syndrome in a pediatric patient with mutation and severe acute respiratory syndrome coronavirus 2 infection.

Introduction: Acute necrotizing encephalopathy (ANE), a fatal subtype of infection-triggered encephalopathy syndrome (ITES), can be triggered by many systemic infections. gene mutations were associated with recurrent ANE.

Case Presentation: Here we report a 1-year-old girl with recurrent ITES and mutation.