Executive Summary: Clinical Practice Guideline for Autosomal Dominant Polycystic Kidney Disease in China.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, with a prevalence of 1/2,500-1/1,000, and it affects 1.25 million people in China. ADPKD is responsible for nearly 5% of end-stage renal disease cases, which leads to a major burden on public health. In 2016, the Chinese working group developed guidelines for the diagnosis and treatment of ADPKD, which promoted the clinical management of ADPKD in China. In the last 3 years, Chinese clinicians have deepened their understanding and standardized the management of ADPKD, and several basic and clinical studies on ADPKD have been conducted. In combination with international guidelines and research results, the working group updated the ADPKD guidelines in China. This guideline includes 5 chapters: introduction, diagnosis, kidney disease progression monitoring, treatment, and family planning. We highlight the main recommendations and suggestions of the ADPKD guidelines in this summary.

[The expression and significance of nephrin in hepatitis B virus-associated membranous nephropathy].

OBJECTIVE: To observe the expression of nephrin in hepatitis B virus-associated membranous nephropathy (HBV-MN), and investigate the impairment and significance of podocyte in HBV-MN. METHODS: The protein expression of nephrin in renal biopsy specimens in 35 patients, who were diagnosed as HBV-MN by renal biopsy, was determined by immunohistochemistry and tested by semi-quantitative method. The relationship between the expression of nephrin and clinicopathological data was analyzed. RESULTS: Among the 35 cases with HBV-MN, 6 were in MN phase I, 20 in MN phase II and 9 in MN phase III. A strong intensity expression of nephrin in normal glomerulus was found along capillary loop of glomerulus, while its expression in HBV-MN patients decreased obviously. There was no significantly difference in the expression of nephrin among the different stages of HBV-MN (P > 0.05). The expression of nephrin in different clinical types was significantly different(P < 0.05). The expression of nephrin in patients with nephrotic syndrome was significantly lower than that in patients without nephrotic syndrome (P < 0.01). The expression of nephrin in different grades of 24-hour urinary protein excretion quantity was significantly different(P < 0.05). There was negative correlation between the expression of nephrin and 24-hour urinary protein excretion quantity(r = -0.378, P < 0.05). In the patients with HBV-MN phase II, the expression of nephrin in patients with nephrotic syndrome was also significantly lower than that in patients without nephrotic syndrome (P < 0.01). CONCLUSIONS: The damage of podocytes emerge in the early stage of HBV-MN and the expression of nephrin in HBV-MN patients, especially in patients with nephrotic syndrome, are significantly down regulated. The descended expression of nephrin in HBV-MN patients may promote the production of proteinuria.