Urokinase prophylactic anticoagulation in children with nephrotic syndrome: a multicenter retrospective cohort study.

Objective: To analyze the clinical effect of urokinase on the prevention of thrombosis in children with primary nephrotic syndrome.

Methods: A total of 370 children diagnosed with primary nephrotic syndrome (PNS) in the Children's Hospital of Soochow University and Zibo Maternal and Child Health Hospital from January 2018 to December 2022 were selected as the research objects. The patients were divided into a urokinase adjuvant therapy group and non-urokinase adjuvant therapy group according to the application of drugs.

Reduction of renal interstitial fibrosis by targeting Tie2 in vascular endothelial cells.

Background: Tie2, a functional angiopoietin receptor, is expressed in vascular endothelial cells and plays an important role in angiogenesis and vascular stability. This study aimed to evaluate the effects of an agonistic Tie2 signal on renal interstitial fibrosis (RIF) and elucidate the underlying mechanisms.

Methods: We established an in vivo mouse model of folic acid-induced nephropathy (FAN) and an in vitro model of lipopolysaccharide-stimulated endothelial cell injury, then an agonistic Tie2 monoclonal antibody (Tie2 mAb) was used to intervent these processes.

Sintilimab treatment for chronic active Epstein-Barr virus infection and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children.

Background: Chronic active Epstein-Barr virus infection (CAEBV) and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) are rare but life-threatening progressive diseases triggered by EBV infection. Glucocorticoid/immunosuppressants treatment is temporarily effective; however, most patients relapse and/or progress. Hematopoietic stem cell transplantation (HSCT) is a potentially curative therapy; however, there are risks of transplantation-associated complications.

Clinical features and management of atypical hemolytic uremic syndrome patient with gene variants: a case report.

Background: Atypical hemolytic uremic syndrome (aHUS) with diacylglycerol kinase epsilon () gene variant is a rare variant of thrombotic microangiopathy (TMA). The information on the clinical features, management and long-term outcomes of -aHUS patients have not yet been fully elucidated. The aim of this study was to report a novel variant of the gene in a Chinese population with aHUS.

The role of PD-1 signaling in health and immune-related diseases.

Programmed cell death 1 receptor (PD-1) and its ligands constitute an inhibitory pathway to mediate the mechanism of immune tolerance and provide immune homeostasis. Significantly, the binding partners of PD-1 and its associated ligands are diverse, which facilitates immunosuppression in cooperation with other immune checkpoint proteins. Accumulating evidence has demonstrated the important immunosuppressive role of the PD-1 axis in the tumor microenvironment and in autoimmune diseases.

Incidence of thromboembolism in children with primary nephrotic syndrome: a systematic review and metaanalysis.

Objective: To estimate the incidence of thromboembolism in children with primary nephrotic syndrome with Meta-analysis.

Methods: Relevant studies published from January 1, 1980 to December 31, 2021 were retrieved from Pubmed, Web of science, Cochrane library, China National Knowledge Infrastructure (CNKI), China Science and Technology Journal Database(VIP) and Wangfang Database. Quality evaluation of the literatures included was conducted according to Agency for Healthcare Research and Quality(AHRQ) assessment tool, followed by data extraction and Meta-analysis with software RevMan 5.

Clinical characteristics and outcomes of chronic nonbacterial osteomyelitis in children: a multicenter case series.

Objective: The aim of this study was to evaluate demographic, clinical, laboratory, imaging, histopathology characteristics, and treatment responses of children with Chronic nonbacterial osteomyelitis (CNO).

Methods: Retrospective multi-center case series study of pediatric patients diagnosed with CNO treated at five tertiary centers in south China.

Results: Totally there were 18 patients diagnosed as CNO between 2014 and 2020.

Acute pancreatitis and macrophage activation syndrome in pediatric systemic lupus erythematosus: case-based review.

Pancreatitis is uncommon in systemic lupus erythematosus (SLE) and is rarely reported in children, possibly being related to macrophage activation syndrome (MAS). The incidence of MAS in children with lupus pancreatitis is unknown, as is their prognosis. In this case-based review, we report a pediatric patient with SLE complicated with pancreatitis and MAS, and performed a literature review.