2016年 · 第32卷第07期

polycythemia vera (PV) is a myeloproliferative disease (MPD) characterized by clonal proliferation of red blood cells^[1]。 PV complicated with glomerulonephritis is rare, and its pathological form is mostly focal segmental glomerulosclerosis (FSGS)^{[2,3,4,5,6,7]}。 Recently, we diagnosed a case of PV complicated with membranoproliferative glomerulonephritis (MPGN), which is reported below.

Fabry disease, also known as Fabry disease, Anderson-Fabry disease or systemic diffuse somatic angiokeratoma, is a rare hereditary lysosomal storage disease. Due to the GLA mutation of the gene encoding alpha-galactosidase A (alpha-galactosidase A), the partial or complete lack of enzyme activity leads to excessive glycosphingolipid accumulation in cells, thus causing various system and organ lesions^[1]。 A case of Fabry's disease with renal damage as the first symptom admitted to our hospital is reported as follows, and the relevant literature is reviewed.

Bilateral cystic renal carcinoma is rare in clinic, and it is easy to be misdiagnosed as simple cyst before operation. We report the diagnosis and treatment of a patient with bilateral cystic renal carcinoma.

Hyperphosphatemia is one of the common complications of chronic kidney disease (CKD). The decline of renal function causes the weakening of renal excretion capacity of phosphorus, which is the root cause of hyperphosphatemia in CKD patients. Hyperphosphatemia may increase the rate of arteriosclerosis and cardiovascular disease^[1,2,3,4]Accelerate the progression of kidney disease^[5,6,7], is one of the predictors of all-cause death in CKD patients^[8,9]。 Therefore, the current clinical treatment guidelines regard early and effective control of serum phosphorus as one of the important contents in the management of CKD patients. This article will review the relationship between serum phosphorus and prognosis of patients with CKD, and explore the efficacy and safety of phosphorus binding agent in the application of CKD patients before dialysis, so as to provide reference for clinical work.

Diabetic nephropathy (DN) is a disease closely related to metabolic abnormalities. Inositol (MI) is a lipophilic vitamin in vitamin B family. Its main functions include maintaining cell growth and survival, regulating nerve cell development and bone cell metabolism, reducing insulin resistance, increasing high-density cholesterol, reducing low-density cholesterol and triacylglycerol levels, maintaining mitochondrial morphology and function, changing sodium and potassium pump activity, scavenging reactive oxygen species, etc^[1]。 In the study of diabetes, it was found that MI level is related to blood glucose concentration, insulin resistance, and can reduce vascular endothelial cell damage in diabetes by antioxidant^[2]。 It suggests that MI may play an important role in the development of DN. Inositol oxygenase (MIOX) is a key molecule in the glucuronate-xylulose metabolic pathway (P-X pathway) and a rate-limiting enzyme that catalyzes the first key reaction in the only pathway of MI metabolism. MIOX through ferrous/ferrous iron (Fe²⁺/Fe³⁺) catalyzes the oxidation of MI to glucuronic acid to form xylulose 5-phosphate, which plays a key role in the P-X pathway^[3,4]。 In recent years, studies have found that MIOX not only regulates MI metabolism, but also participates in the pathogenesis of DN tubular injury through non-MI metabolic pathways. We reviewed the literature at home and abroad, combined with our previous work, to review the role and mechanism of MIOX expressed specifically in renal tubular epithelial cells in DN tubular injury.

Klotho is an "anti-aging" gene discovered in 1997 and expresses Klotho (KL) protein. In recent years, it has been found that KL has the functions of anti-aging and regulating blood phosphorus^[1]There are also many biological effects closely related to kidney disease, such as antioxidant, anti-inflammatory, inhibiting apoptosis, inhibiting renal fibrosis and inhibiting vascular calcification. Therefore, KL is considered as a newly discovered nephroprotective protein. This paper discusses the renal protective effect and mechanism of KL.