Clinical and Laboratory Risk Factors of Early Poor Outcome in Patients With Childhood-Onset Lupus Nephritis-A Single-Center Retrospective Study.

Objective: Childhood-onset lupus nephritis (LN) tends to be more severe than in adults. A significant correlation between remission at 3 months of induction therapy and remission after 3 years was found in adults. While few studies on the risk factors of poor early prognosis in children with LN were made.

Overproduction of Mitochondrial Fission Proteins and Mitochondrial Fission in Podocytes of Lupus Nephritis Patients.

Background: The glomerular injury is associated with different pathogeneses, and podocyte damage is common in various ISN/RPS class lupus nephritis (LN). In podocyte, mitochondrial morphological changes are observed in lupus nephritis (LN) in our previous study. This study aimed to explore mitochondrial fission proteins expression in podocytes using bioinformatics analysis and further to investigate the associations between mitochondrial fission proteins and laboratory features in LN.

Efficacy of initial combination with belimumab in newly diagnosed childhood-onset lupus nephritis: a single-centre historical control study.

Objective: To explore the efficacy of initial treatment of newly diagnosed childhood-onset lupus nephritis (cLN) with combination of belimumab and either cyclophosphamide, mycophenolate mofetil, tacrolimus or multitargeted therapy.

Methods: A historical control study was conducted on children aged 5-17 years with newly diagnosed cLN. All patients recruited met the 2012 Systemic Lupus International Collaborating Clinics and/or 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for SLE, and the 2003 International Society of Nephrology/Renal Pathology Society histopathological criteria for LN.

Juvenile dermatomyositis with central nervous system involvement: two case reports from a retrospective single-center cohort, with literature review.

Background: Juvenile dermatomyositis (JDM) is a systemic autoimmune disease primarily involving the muscles and skin; it can also affect the central nervous system (CNS). The relevant literature provides limited information regarding the characteristics of JDM with CNS involvement.

Method: We reviewed patients with JDM who were hospitalized at our center between January 2016 and August 2023, with a focus on those with CNS involvement.

Monogenic autoinflammatory disease-associated cardiac damage.

Introduction: Autoinflammatory diseases (AIDs) constitute several disorders that are characterized by the presence of recurrent episodes of unprovoked inflammation due to dysregulated innate immune system in the absence of autoantibodies or infections. Most of them have a strong genetic background, with mutations in single genes involved in inflammation referred to monogenic AIDs. In this article, we will review the cardiac manifestations in various monogenic AIDs.

Identifying circRNA-associated-ceRNA networks in juvenile spondyloarthropathies patients.

Background: Juvenile spondyloarthropathies (JSpA) are defined as a heterogeneous group of diseases that start before the age of 16. The study aimed to identify key genes and pathways that are influenced by circRNAs and to screen potential therapeutic agents for JSpA. The study involved the analysis of circRNA expression profiles, identification of circRNA-miRNA-mRNA regulatory networks, and functional annotation of differentially expressed genes.

Identification of a circRNA-miRNA-mRNA network to explore the effects of circRNAs on pathogenesis and treatment of systemic lupus erythematosus.

Objectives: Systemic lupus erythematosus (SLE) is an autoimmune disease characterised by immune inflammation. It involves multiple organs. Many studies have demonstrated that circRNAs are closely associated with SLE.

Symptomatic femoral head necrosis in patients with rheumatoid arthritis: A retrospective case-control study.

Objective: We conducted a retrospective case-control study to investigate the risk factors for osteonecrosis of the femoral head (ONFH) in rheumatoid arthritis (RA) patients.

Methods: The clinical data of patients diagnosed with RA at Fujian Provincial Hospital from January 2013 to December 2020 were retrospectively collected and evaluated. Twenty-two patients with ONFH were identified.

Glomerular Expression of S100A8 in Lupus Nephritis: An Integrated Bioinformatics Analysis.

Introduction: Lupus nephritis (LN) is a major risk factor of morbidity and mortality. Glomerular injury is associated with different pathogeneses and clinical presentations in LN patients. However, the molecular mechanisms involved are not well understood.

Five years follow-up of juvenile lupus nephritis: a single-center retrospective cohort study.

Background: We analyze the clinical manifestations and 5 years of follow-up outcomes of children with lupus nephritis (LN) and provide a reference for clinicians.

Methods: The clinical data of children diagnosed with LN (n=62) from January 2012-2015 were collected and analyzed.

Results: The median age at the diagnosis was 12.

Antiphospholipid antibodies and osteonecrosis in systemic lupus erythematosus: a meta-analysis.

: The present meta-analysis aimed to assess the relationship between antiphospholipid antibodies (aPLs) or antiphospholipid antibody syndrome (APS) and the incidence of osteonecrosis (ON) in systemic lupus erythematosus (SLE) patients.: MEDLINE/Pubmed, EMBASE, Web of science, the Chinese Biomedical Literature Database (CBM), the Wan-Fang Database, and the China National Knowledge Infrastructure (CNKI) were searched from their inception up until 26 December 2020. Studies in English were included.

Single-Center Overview of Pediatric Monogenic Autoinflammatory Diseases in the Past Decade: A Summary and Beyond.

Monogenic autoinflammatory diseases (AIDs) are inborn disorders caused by innate immunity dysregulation and characterized by robust autoinflammation. We aimed to present the phenotypes and genotypes of Chinese pediatric monogenic AID patients. A total of 288 pediatric patients clinically suspected to have monogenic AIDs at the Department of Pediatrics of Peking Union Medical College Hospital between November 2008 and May 2019 were genotyped by Sanger sequencing, and/or gene panel sequencing and/or whole exome sequencing.

The association of MEFV gene mutations with the disease risk and severity of systemic juvenile idiopathic arthritis.

Background: Systemic juvenile idiopathic arthritis (sJIA) has many clinical features overlapping with familial Mediterranean fever (FMF), which is caused by mutations in MEFV gene. And FMF patients were easily misdiagnosed as sJIA in China. So we speculate that MEFV is critical genetic background for sJIA and influences patients' severity.

[Clinical features and TTC21B genotype of a child with nephronophthisis type 12].

Nephronophthisis (NPHP) is a group of autosomal recessive tubulointerstitial cystic kidney disorders. This article reports a case of NPHP type 12 caused by TTC21B mutations. The girl had an insidious onset, with moderate proteinuria, renal dysfunction, stage 2 hypertension, situs inversus, and short phalanges when she visited the hospital for the first time at the age of 3 years and 6 months.

Overproduction of Mitochondrial Fission Proteins in Membranous Nephropathy in Children.

Background/aims: The molecules involved in nephrotic syndrome (NS) have not been fully clarified. Mitochondrial fission proteins are found to be involved in podocyte injury in vitro. Increased glomerular expression of mitochondrial fission proteins was found in adriamycin nephropathy in our previous study.

IPR-Grp75-VDAC1-MCU calcium regulation axis antagonists protect podocytes from apoptosis and decrease proteinuria in an Adriamycin nephropathy rat model.

Background: The mechanism of podocyte apoptosis is not fully understood. In addition, the role of the inositol 1,4,5-triphosphate receptor (IPR)/glucose-regulated protein 75 (Grp75)/voltage-dependent anion channel 1 (VDAC1)/mitochondrial calcium uniporter (MCU) calcium regulation axis, which is located at sites of endoplasmic reticulum (ER) mitochondria coupling, in the mechanism of podocyte apoptosis is unclear. This study aimed to understand the roles of this axis in podocyte apoptosis and explore potential targets for podocyte protection.