Advances in the regulation of autosomal dominant polycystic kidney disease by Hippo signaling pathway / 中华肾脏病杂志

The Hippo signaling pathway is highly conserved in evolution, and participates in the regulation of cell proliferation, differentiation, and tissular dynamic balance, and plays an important role in regulating tissue, organ size, and cell number. Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease and one of the most common causes of end-stage renal disease. Emerging studies have identified the Hippo signaling pathway is closely related to the occurrence and development of ADPKD. The abnormal activity and expression of the main members of the pathway affect the cilia and cell polarity of renal tubular epithelial cells and induce the formation of renal cysts. The review summarizes the potential mechanism of the Hippo pathway in the pathogenesis of ADPKD, the crosstalk with other signaling pathways, and the variances in different species, and discusses the strategies for the treatment of ADPKD based on the Hippo signaling pathway to provide new strategies for the treatment.

The renal boipsy data analysis of 107 patients at high altitude area in Tibet / 医学研究生学报

[Abstract ] Objective Chronic renal disease,a common and frequent disease,is the most cause inducing chronic renal failure. There is an important guiding significance for renal biopsy in the diagnosis,treatment and prognosis of renal disease.The aim of this study was to analyze the characteristics of renal biopsy and the relation between pathological types and clinical presentation in Tibet. Methods Between June 2011 and December 2013 in General Hospital of Tibetan Military Command, Lhasa, percutaneous renal biopsy were per-formed in 107 renal patients.In this study, the gender, age, pathologic entities, and clinical manifestation were analyzed retrospectively. The final diagnosis was made by the clinical manifestation, laboratory examination,and pathologic results. Results The mean age at renal biopsy was 29.8 ±12.2(10~66)years in 47 male cases (43.9%) and 69 female cases (56.1%).This includes 75 Tibetan cases (70.1%), 31 Han cases (28.9%), and Bai cases (1.0%).The primary glomerular disease was 95.3%and the secondary glomerular disease was 4.7%in the total 107 cases.Fourty-four point three percent of the primary glomerular disease were male cases and 100%of secondary glomerular disease were female cases.The main pathological type of the primary glomerular disease was podocyte nephropathy (43.9%), followed by membranous nephropathy (18.7%), IgA nephropathy (11.2%), focal segmental glomerulosclerosis (9.3%) and mesangiocapillary glomerulonephritis (5.6%).The clinical manifestations of 107 cases were classified as syndrome of nephrotic syn-dromn(69.1%),urinary abnormalities(14.9%),isolated macrographic haematuria (3.7%),chronic renal failure(2.8%).Among the nephrotic syndrome,podocyte disease was 63.5%,membranous glomerulopathy was 18.9%,focal segmental glomeruolsclerosis was 8.1%,and membranoproliferative glomerulonephritis was 5.4%. Conclusion The primary glomerulonephritis was the most common glomerular diseases at high altitude area in Tibet.The most frequent type of pri-mary glomerular nephritis was podocyte disease, and the most frequent type of secondary glomerular nephritis was Henoch-Schonlein purpura glomerulonephritis.