Long-term prednisone treatment causes fungal microbiota dysbiosis and alters the ecological interaction between gut mycobiome and bacteriome in rats.

Glucocorticoids (GCs) are widely used in the treatment of immune-mediated diseases due to their anti-inflammatory and immunosuppressive effects. Prednisone is one of the most commonly used GCs. However, it is still unknown whether prednisone affects gut fungi in rats.

Integrative Analysis of Gut Microbiota and Fecal Metabolites in Rats after Prednisone Treatment.

Prednisone (PRED) is a synthetic glucocorticoid (GC) widely used in immune-mediated diseases for its immunosuppressive and anti-inflammatory properties. The effects of GC are achieved by genomic and nongenomic mechanisms. However, the nongenomic effects are largely unknown.

Haploidentical hematopoietic stem cell transplantation for pediatric patients with chronic active Epstein-Barr virus infection: a retrospective analysis of a single center.

Background: This study aimed to evaluate the feasibility and clinical effect of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) for the treatment of pediatric patients with chronic active Epstein-Barr virus infection (CAEBV).

Methods: Children with CAEBV who did not have matched donors and underwent haplo-HSCT in Beijing Children's Hospital, Capital Medical University, from October 2016 to June 2020 were analyzed retrospectively. Data relating to the clinical manifestations, engraftment, and prognosis of the children were extracted from medical records.

Diagnosis and treatment of pediatric anaplastic lymphoma kinase-positive large B-cell lymphoma: A case report.

Background: Anaplastic lymphoma kinase-positive (ALK+) large B-cell lymphoma (LBCL) is a rare type of lymphoma with high invasiveness and rapid progression. It occurs in all age groups, but is extremely rare in children. The lesions mainly involve the lymph nodes and may present with extra-nodal involvement.

Responsible genes in children with primary vesicoureteral reflux: findings from the Chinese Children Genetic Kidney Disease Database.

Background: Primary vesicoureteral reflux (VUR) is a common congenital anomaly of the kidney and urinary tract (CAKUT) in childhood. The present study identified the possible genetic contributions to primary VUR in children.

Methods: Patients with primary VUR were enrolled and analysed based on a national multi-center registration network (Chinese Children Genetic Kidney Disease Database, CCGKDD) that covered 23 different provinces/regions in China from 2014 to 2019.

TMT-based proteomic analysis reveals the effects of chloroquine on human podocytes.

Chloroquine (CQ) is an antimalarial drug widely used in rheumatic, immunological and infectious diseases. CQ is also a well-known autophagy inhibitor. It slows the progression of renal injury in patients with rheumatology diseases.

Haploidentical Hematopoietic Stem Cell Transplantation for XIAP Deficiency: a Single-Center Report.

Purpose: X-linked inhibitor of apoptosis (XIAP) deficiency caused by mutations in the XIAP/BIRC4 gene is a rare inherited primary immunodeficiency also known as X-linked lymphoproliferative syndrome type 2 (XLP2). Hematopoietic stem cell transplantation (HSCT) is currently the only curative strategy available. However, few studies of haploidentical HSCT have been published regarding the outcomes in patients with this syndrome.

Value of micro-proteinuria in combination with ultrasonography of the left renal vein in the diagnosis of orthostatic proteinuria.

Background: This study aimed to evaluate the clinical value of micro-proteinuria in combination with ultrasonography of the left renal vein (LRV) in the diagnosis of orthostatic proteinuria (OP).

Methods: The patients with suspected OP received West test, upright lordotic position test, Robinson test, ultrasonography of the LRV, and detection of morning urine micro-proteinuria and micro-proteinuria after activity. The sensitivity (Se), specificity (Sp), positive and negative predictive values (PPV and NPV), positive and negative likelihood ratios (PLR and NLR) and Youden's index (YI) for micro-proteinuria, ultrasonography of the LRV and both of them in the diagnosis of OP were analyzed.

Compositional alterations of gut microbiota in children with primary nephrotic syndrome after initial therapy.

Background: Primary nephrotic syndrome (PNS) is a common glomerular disease in children. T cell dysfunction plays a crucial role in the pathogenesis of PNS. Moreover, dysbiosis of gut microbiota contributes to immunological disorders.

[Association between IL-10 Gene -592(C→A) (rs1800872) SNP and GVHD after Allogeneic Hematopoietic Stem Cell Transplantation in Children].

Objective: To study the association between IL-10 gene-592(C→A) (rs1800872) single nucleotide polymorphism (SNP) and the graft versus host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children.

Methods: Ninety-seven childhood patients and seventy-one donors in the Hematology Oncology Center of Beijing Children's Hospital from Jan 2011 to Jul 2017 were enrolled in this study. The genomic DNA was extracted from peripheral blood cells and the SNP genotype was analyzed using TaqMan SNP genotyping assay.

Chylopericardium in a child with IgA nephropathy: a case report.

Background: Chylopericardium effusion is characterized by the accumulation of milky effusion in the pericardium. It is often idiopathic but it can be secondary to trauma, chest radiation, tuberculosis and malignancy. If cardiac tamponade ensues, it becomes life-threatening.

A single-center analysis of Henoch-Schonlein purpura nephritis with nephrotic proteinuria in children.

Background: In children with Henoch-Schonlein purpura nephritis (HSPN), the degree of proteinuria has been proven to be not only a sign of kidney damage, but also an accelerator of kidney disease progression. Nephrotic proteinuria at disease onset has been proposed as a predictor of a poor renal outcome. This study aims to assess the clinical and pathological features of HSPN with nephrotic proteinuria in a single center.

Single dose of rituximab in children with steroid-dependent minimal change nephrotic syndrome.

Rituximab (RTX) can be used in children with nephrotic syndrome, particularly in those with steroid-dependent nephrotic syndrome (SDNS). However, at present there is no unified standard of how to use RTX, with regard to the amount of doses and frequency, in children with nephrotic syndrome. The study aimed to investigate the therapeutic efficacy of a single dose of RTX in children with steroid-dependent minimal change nephrotic syndrome (SD-MCNS).

Haploidentical parental hematopoietic stem cell transplantation in pediatric refractory Langerhans cell histiocytosis.

Children with MS-LCH that fail to respond to conventional chemotherapy have poor outcomes. HSCT represents a potential salvage approach. It has been applied in over 50 cases in recent years.

[Allogeneic stem cell transplantation for 8 patients with malignant infantile osteopetrosis in China].

Objective: Osteopetrosis is a rare genetic disorder and the malignant infantile osteopetrosis (MIOP) is the worst subtype of this disease. Seventy percent of patients die in six years of life without proper treatment. Hematopoietic stem cell transplantation (HSCT) offers the only chance of cure for MIOP.

[Tolerability of 6-mercaptopurine in children with acute lymphoblastic leukemia].

Objective: 6-Mercaptopurine (6-MP) has been the backbone of maintenance chemotherapy for acute lymphoblastic leukemia (ALL), the response to 6-MP is highly variable, adverse events leading to discontinuation or dose-reduction (children intolerant) of 6-MP occur in many children with ALL. The aim of this study was to investigate the tolerability of 6-MP and to optimize thiopurine use.

Methods: The authors evaluated in a prospective manner the tolerance of 6-MP in ALL children from Oct.

[A prospective multicenter clinical control trial on treatment of refractory nephrotic syndrome with mycophenolate mofetil in children].

Objective: To evaluate the efficacy and safety of mycophenolate mofetil (MMF) plus prednisone on refractory nephrotic syndrome (RNS) in children.

Methods: One hundred and forty-two children with RNS from ten clinical trial centers were divided into two groups: MMF (n=87) and control (n=55). The MMF group patients were administered with oral MMF (30-40 mg/kg daily) for at least 6 months.

Efficacy of urine screening at school: experience in Shanghai, China.

To explore the prevalence of hematuria or proteinuria in school children in Shanghai and to evaluate the screening methods, we conducted urine screening in more than 40,000 school children between 2003 and 2005. Children were tested with dipsticks read manually (method A) or dipsticks read by machines (method B) combined with a sulfosalicylic acid test or microscopy. Some children were tested once, and others who had abnormal results in the first screening were tested again 2 weeks later.