Angiotensin Ⅱ receptor blockers(ARB) are recently studied as renin-angiotensin system (RAS) inhibitors. They were firstly used in hypertension therapy, while now are widely administered to reduce urinary protein and slow the progression of proteinuric nephropathy to end-stage renal failure in nephropathy treatment. They are considered effective in IgA nephropathy according to its character. This review briefly discusses the mechanism, function, and tissue distribution of ARBs, and the corresponding advances in IgAN treatment.

Glucocorticoid is one of the widely used drugs in clinic,especially in nephrotic syndrome.This paper mainly summarizes its use in nephrotic syndrome in clinic,focusing on its classification,different dosage,mechanisms,basic principles of application,the inhibition of adrenal axis and precautions.

Chronic kidney disease-mineral and bone disorder (CKD-MBD) refers to a series of clinical symptoms and biochemical and imaging abnormalities caused by minerals and calcium phosphorus metabolic disorder,which is associated with chronic kidney disease (CKD).Since 2006 ,the concept of CKD-MBD was put forward for the first time,doctors are increasingly paying more attention to it.This review introduces the epidemiological characteristics, clinical manifestation, examination methods, diagnostic criteria and the research progress of treatment and prevention of CKD-MBD.

Mannose-binding lectin(MBL) is a key component of the innate immune system that plays a pluripotent role in natural defense.MBL genetic variants are associated with increased susceptibility to and the severity and prognosis of various diseases.The association between low MBL-producing allelic variants and disease risk and/or severity is particularly strong in infectious and autoimmune diseases.Enhanced risk for disease by low MBL producers is particularly the case for children and immunocompromised patients,especially when primary and secondary immune deficiencies coexist.Therefore the use of MBL testing and replacement therapy has reached the threshold of personalized medicine.The author presents a review of the role of MBL in health and diseases,the advances in MBL testing methodology and MBL related therapies.

Immune factor is one of the main mechanism of children glomerular diseases. Tacrolimus has a strong and effective immunosuppressive action comparing with CsA. In recent years,the curative effect of ta-crolimus in the treatment of children with kidney disease has attracted doctors attention. Tacrolimus in the treat-ment for refractory nephrotic syndrome ( steroid-dependent nephrotic syndrome, steroid-resistent nephrotic syn-drome and the frequent recurrence of glomerular syndrome) has obtained the good effect. This article reviews the application of tacrolimus in nephrotic syndrome,focal segmental glomerular sclerosis,mesangial proliferative glo-merulonephritis,membranous nephropathy,lupus nephritis( LN) in the children with kidney disease.

MicroRNAs (miRNAs) are short non-coding RNAs that regulate gene expression at the post-transcriptional lev-el and then affect important physiological and pathological processes. Many microRNAs are tissue or organ-speciifc which make it possible for them to become potential biomarkers. Similarly, microRNAs are important for physiological functions of kidney and they are involved in various renal disorders. The review summarizes current information about microRNA effect on various kidney diseases, in order to provide references for the diagnosis and treatment of kidney disease in the future.

Objective The article was to analyze the features of gene mutation and clinical phenotype in Alport syndrome. Methods Next-generation sequencing was applied to capture the exons of COL4A3, COL4A4, COL4A5 genes in 30 cases of children with suspected or confirmed diagnosis of Alport syndrome and Sanger method was used to identify gene mutations of related family mem-bers.Provean database was applied in protein function prediction.We collected and analyzed clinical data of AS patients on the basis of gene mutation. Results All 30 children were diagnosed with AS by gene sequencing, among whom 4 boys were autosomal reces-sive inheritance, 16 boys and 10 girls were X-linked Alport syndrome.Next-generation sequencing detected 35 different gene mutations of COL4A3, COL4A4, COL4A5, including 19 missense mutations, 2 synonymous mutations, 4 splice-site mutations, 3 truncating mu-tations, 2 insertion mutations, 4 deletion mutations and 1 compound mutations.It was observed by Sanger sequencing that 20 mutations were inherited from the mother, 8 from the father, homozygous mutation in 1 propositus from the parents respectively, 8 novel mutations and 1 with unidentified source.All the 30 children had an onset of hematuria or proteinuria, 17 cases had a positive family history, 1 case had hearing loss, and no pathogenesis or renal insufficiency was found in the children.Renal biopsy was performed on 23 children, 13 minimal change disease ( MCD) and 10 mesangial proliferative glo-merulonephritis ( MsPNG) by light microscope.Extensive lamination and split of glomerular basement membrane dense layers were found in 9 children by electron microscope. Conclusion XLAS ac-counts for most AS patients and missense mutation is the main type in pathogenic mutations.Altogether, 31 mutations without disease notification were found.Most of children showed MCD in renal biopsy, with atypical electron microscope manifestations and rare extra renal manifestations.

Acute kidney injury is a clinical common disease,but it is a dangerous illness with higher o-verall mortality.Even mild renal insufficiency carries complications.Therefore,it is very important for the early diagnosis of acute kidney injury,which is based on high sensitivity,strong specificity of biomarkers.

Methylmalonic acidemia(MMA) is one of the most common diseases of autosomal recessive inherited organic acid metabolic disorder,which can cause multiple organ involvement.It can be found in infants or even in the neonatal period and be manifested as feeding difficulty,repeated vomiting,convulsions,abnormal muscle tension,mental retardation,hemolytic uremic syndrome(HUS),etc.In which HUS caused by MMA is better and better known by pediatricians when treating renal diseases while the diagnosis rate is increasing year by year.This article reviews the epidemiology,etiology,pathogenesis,clinical manifestations and treatment of HUS associated with MMA.

Hereditary glomerular disease is an important part of kidney diseases .In recent years , hereditary glomerular disea-ses had a high incidence and poor prognosis .Thus, the studies involving hereditary glomerular diseases such as Alport syndrome , he-reditary nephritis syndrome and thin basement membrane disease , etc.have significant implications .This review mainly focuses on the pathogenesis , clinical features , diagnosis and treatment of Alport syndrome and hereditary nephritis syndrome .