Risk factors of necrotizing enterocolitis in neonates with sepsis: A retrospective case-control study.

Sepsis, a severe infectious disease in the neonatal period, is considered a risk factor for necrotizing enterocolitis (NEC). To investigate the specific risk factors for NEC in septic infants, septic infants admitted to our center from January 2010 to April 2018 were included. Septic neonates with proven NEC (Bell's stage ⩾II) were enrolled in the NEC group, and those without NEC were enrolled in the control group.

Mixed type of total anomalous pulmonary venous connection: diagnosis, surgical approach and outcomes.

Purpose: To summarize the diagnosis and treatment of 13 patients with mixed-type total anomalous pulmonary venous connection (TAPVC) and propose another classification for mixed TAPVC.

Methods: A retrospective review of 13 patients with mixed TAPVC undergoing repair at a single institution was conducted between January 2010 and November 2019. The diagnosis of mixed-type TAPVC was made in all patients using echocardiography combined with computed tomography angiography.

Nurse education to reduce physical restraints use in ICU: A scoping review.

Background: Physical restraints are defined as a manual approach to reduce a patient's physical movement and has been regarded as a protective nursing measure in the intensive care unit (ICU) to avoid unplanned extubation, falls, and other unexpected events. However, the limitations and changes associated with physical restraints have been verified by several studies. Restraint minimization has been advocated by studies worldwide; however, the most effective interventions are still being explored.

Do children with left ventricular noncompaction and a noncompaction-to-compaction ratio < 2 have a better prognosis?

Background: Ultrasonography is commonly used to diagnose left ventricular noncompaction (LVNC). A ratio of noncompacted to compacted myocardium (NC/C ratio) > >2 is often used to diagnose LVNC. However, a large proportion of patients with noncompact myocardium have NC/C < 2, and the prognosis of these patients have not been studied.

Corrigendum to "Novel biallelic mutations lead to atypical SIFD and multiple immune defects" [Genes Dis 7 (1) (2020) 128-137].

[This corrects the article DOI: 10.1016/j.gendis.

[Status survey and training efficiency of food allergy knowledge among pediatric medical worker].

To investigate the status and influencing factors of diagnosis and treatment of food allergy knowledge in pediatric medical workers, and formulate a targeted training course for pediatricians to evaluate its training effect. A web-based questionnaire survey was conducted to investigate the current situation of food allergy knowledge among some pediatric medical workers nationwide from April 22 to 24, 2019. Then a 2.

Acetabular roof lesions in children: a descriptive study and literature review.

Background: Acetabular roof lesions (ARLs) in children are uncommon and may involve a variety of diseases. The acetabular roof is the main weight-bearing area of the hip joint, and lesions affecting the acetabular roof lead to fluid accumulation in the hip joint, causing hip pain and claudication. Methods for diagnosing and treating ARLs and the prognosis after treatment are rarely reported.

The deubiquitinase USP44 promotes Treg function during inflammation by preventing FOXP3 degradation.

The transcription factor forkhead box P3 (FOXP3) is essential for the development of regulatory T cells (Tregs) and their function in immune homeostasis. Previous studies have shown that in natural Tregs (nTregs), FOXP3 can be regulated by polyubiquitination and deubiquitination. However, the molecular players active in this pathway, especially those modulating FOXP3 by deubiquitination in the distinct induced Treg (iTreg) lineage, remain unclear.

Tumor necrosis factor-alpha blockade ameliorates inflammatory response in two children with chronic infantile neurological, cutaneous and articular syndrome.

Chronic infantile neurological, cutaneous and articular (CINCA) syndrome is a rare autoinflammatory disease caused by monogenic defects in the NLRP3 gene. Pro-inflammatory cytokines such as interleukin (IL)-1β play a crucial role in the pathogenesis, and IL-1 receptor antagonists have been regarded as the mainstay therapy. Endogenous tumor necrosis factor (TNF)-α was found recently to be involved in the onset of the disease.

Trehalose Inhibits Aβ Generation and Plaque Formation in Alzheimer's Disease.

Alzheimer's disease (AD) is the most common neurodegenerative disease, and there has been no disease-modifying treatment for AD. Recent studies suggest that trehalose may have beneficial effect on neurodegenerative diseases through regulating autophagy and facilitating aggregated protein clearance. However, the effects of trehalose on AD-related neuropathologies are still unknown.

Refractive status and optical components of premature babies with or without retinopathy of prematurity at 7 years old.

Background: This study aimed to investigate the refractive status and optical components of premature babies with or without retinopathy of prematurity (ROP) at 7 years old and to explore the influence of prematurity and ROP on the refractive status and optical components.

Methods: From January 2009 to February 2011, premature babies receiving fundus photographic screening (FPS) were recruited and divided into non-ROP group and ROP group. Full-term babies matched in age were recruited as controls.

A case report of Noonan syndrome-like disorder with loose anagen hair 2 treated with recombinant human growth hormone.

Protein phosphatase 1 catalytic subunit beta (PPP1CB) is a disease-causing gene of Noonan-like syndrome, which acts via the RAS/MAPK pathway. To date, only 17 patients diagnosed with PPP1CB-related Noonan-like syndrome have been reported around the world, with few reports in Asia. Twelve reported patients are of short stature and only one patient was treated with growth hormone (GH); however, follow-up data is lacking.

CD8 T-cell senescence and skewed lymphocyte subsets in young Dyskeratosis Congenita patients with PARN and DKC1 mutations.

Background: Dyskeratosis congenita (DC) is a syndrome resulting from defective telomere maintenance. Immunodeficiency associated with DC can cause significant morbidity and lead to premature mortality, but the immunological characteristics and molecular hallmark of DC patients, especially young patients, have not been described in detail.

Methods: We summarize the clinical data of two juvenile patients with DC.

[Clinical and immunological characteristics of a case with activated phosphoinositide 3-kinase δ syndrome 2].

To analyze the clinical and immunological characteristics of a patient with activated phosphoinositide 3-kinase δ syndrome 2 (APDS2). A retrospective analysis of clinical data, immune-related gene sequencing, imaging and laboratory findings of a patient with APDS2 admitted to Children's Hospital of Chongqing Medical University was performed. The absolute and relative numbers of peripheral lymphocyte subsets, immune cell subsets and phenotypes were detected by flow cytometry with the age matched healthy child or the patient's father as a control.

Clinical features and management of post-necrotizing enterocolitis strictures in infants: A multicentre retrospective study.

To explore the clinical features and management of post-necrotizing enterocolitis strictures.Clinical data from 158 patients with post-necrotizing enterocolitis strictures were summarized retrospectively in 4 academic pediatric surgical centers between April 2014 and January 2019. All patients were treated conservatively in the internal medicine department.

Risk factors for mechanical ventilation in children with Guillain-Barré syndrome.

Background: We assessed clinical predictors of mechanical ventilation in children with Guillain-Barré syndrome (GBS) to help identify patients who require mechanical ventilation.

Methods: We retrospectively collected the clinical, laboratory, and electrophysiological data of 103 children with GBS. Patients were categorized into two groups based on the requirement for mechanical ventilation.

Phenotypic characterization of patients with activated PI3Kδ syndrome 1 presenting with features of systemic lupus erythematosus.

Activated phosphoinositide 3-kinase δ syndrome 1 (APDS1) is a primary immunodeficiency disease caused by gain-of-function mutations in . Clinical features of autoimmune disease have been reported in patients with APDS1. In this study, we reported three patients with APDS1 presenting with systemic lupus erythematosus (SLE) phenotype.

TRPV1 Contributes to the Neuroprotective Effect of Dexmedetomidine in Pilocarpine-Induced Status Epilepticus Juvenile Rats.

To investigate the antiepileptic and neuroprotective effects of dexmedetomidine (Dex) in pilocarpine- (Pilo-) induced status epilepticus (SE) juvenile rats, rats were randomly assigned to the following six groups ( = 20): normal, normal+Dex, SE, SE+Cap, SE+Dex, and SE+Dex+Cap. The rats were treated with either diazepam (i.p.

Bioinformatic identification of hub genes and key pathways in neutrophils of patients with acute respiratory distress syndrome.

Acute respiratory distress syndrome (ARDS) is characterized as a neutrophil-dominant disorder without effective pharmacological interventions. Knowledge of neutrophils in ARDS patients at the transcriptome level is still limited. We aimed to identify the hub genes and key pathways in neutrophils of patients with ARDS.

[Role of mitochondrial DNA in acute lung injury/acute respiratory distress syndrome induced by sepsis].

Sepsis is a common disease the field of critical care medicine. It is easy to be complicated by acute lung injury (ALI) or even acute respiratory distress syndrome (ARDS), which lead to high mortality. Although domestic and foreign scholars have carried out a large number of research on the pathogenesis of sepsis-related ALI/ARDS, it has not been fully elucidated so far.

Overexpression of ubiquitin-conjugating enzyme E2 L3 in hepatocellular carcinoma potentiates apoptosis evasion by inhibiting the GSK3β/p65 pathway.

UBE2L3 is a ubiquitin-conjugating protein belonging to the E2 family that consists of 153 amino acid residues. In this study, we found that UBE2L3 was generally upregulated in clinical HCC samples compared to non-tumour samples and that there was a strong association between high UBE2L3 expression and tumour size, clinical grade and prognosis in HCC patients. UBE2L3 depletion inhibited the proliferation and induced the apoptosis of HCC cells.

Vitamin A regulates neural stem cell proliferation in rats after hypoxic-ischemic brain damage via RARɑ-mediated modulation of the β-catenin pathway.

Our previous experiments found that a suitable dose of vitamin A (VA) can affect neuronal apoptosis after hypoxic-ischemic brain damage (HIBD) by binding to RARα to activate the PI3K/AKT signaling pathway; however, the other neuroprotective effects of VA after HIBD, for example, whether it promotes neural stem cell (NSC) proliferation, remain unclear. In this study, in vivo and in vitro experiments revealed that VA regulates β-catenin signaling through RARɑ to affect NSC proliferation after HIBD and to improve neurocognitive outcomes. Because of the accumulation and suspended growth characteristics of NSCs, we performed in vitro experiments with PC12 cells to mimic NSCs.

Novel biallelic mutations lead to atypical SIFD and multiple immune defects.

Mutations in the gene encoding transfer RNA (tRNA) nucleotidyltransferase, CCA-adding 1 (TRNT1), an enzyme essential for the synthesis of the 3'-terminal CCA sequence in tRNA molecules, are associated with a rare syndrome of congenital sideroblastic anemia, B cell immunodeficiency, periodic fevers, and developmental delay (SIFD). Clinical manifestations and immunological phenotypes were assessed in a Chinese patient with novel compound heterozygous mutations in . The patient required multiple hospitalizations starting at the age of 2 years for recurrent fevers without an infective cause.