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OBJECTIVE@#To investigated the clinical and pathological characteristics of related-renal damage in patients with POEMS syndrome.@*METHODS@#Five patients diagnosed as POEMS syndrome in our hospital were selected. Their clinical manifestation, pathological characteristics of kidney and laboratory examination were analyzed retrospectively. Among the 5 patients, three males and two females with a median age of 50 years old. The mean interval before diagnosis was 13.0±7.2 months.@*RESULTS@#All the patients showed neuropathy, endocrinopathy, monoclonal plasma cell-proliferative disorder, skin changes and extravascular volume overload, in which 4 patients showed organomegaly. Proteinuria was found in 5 patients, and microhematuria was found in 4 patients. Moreover, 4 patients showed an elevated blood urea, while 2 patients showed creatinine elevation. 1 patient at chronic kidney disease (CKD)-G1 stage, 2 patients at CKD-G2 stage, and 1 patient at CKD-G3b stage, moreover, 1 patient at CKD-G5 stage. Endothelial injury and mesangial lesion were the main characteristics of renal pathology. 3 patients were pathologically diagnosed as thrombotic microangiopathy kidney damage, while 2 patients as light chain amyloidosis.@*CONCLUSION@#POEMS syndrome is a multi-systemic disease with complex clinical manifestations. 5 patients had different degrees of renal insufficiency. Endothelial injury and mesangial lesion are the main features of renal pathology.
Subject(s)
Female , Humans , Male , Middle Aged , Kidney , POEMS Syndrome , Paraproteinemias , Renal Insufficiency , Retrospective StudiesABSTRACT
MASP3 is one of the mannan-binding lectin-associated serine proteases(MASPs)of the complement lectin pathway.It is the alternative splice product of MASP1/3 gene.MASP3 does not participate in the activation of the lectin pathway and it has no cleavage activity towards C2,C4 and C3.Recently,a novel MASP3 function has been discovered that it may play an important role in the alternative pathway through direct activation of factor D.Besides,studies on the murine disease model suggested that MASP3 might be a new therapeutic target for the treatment of alternative pathway-mediated diseases.This review present an overall description of the coding gene and protein structure of MASP3 protein and provide recent research progress on its function and especially in its role in the alternative pathway-mediated diseases.
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Objective To determine the expression of endothelin in kidneys of rats with unilateral ureteral obstruction (UUO). Methods Rats were evenly divided into two groups. Some were subjected to complete ligation of left ureter and the others with sham operation were used as control group.Four weeks later, we examined the mRNA expressions of ET-1 in kidneys of all these rats using reverse transcription-polymerase chain reaction (RT-PCR) method.The immunohistochemistry analysis was carried out to investigate the protein level of ET-1. The urinary concentration of ET-1 was determined by radioimmunoassay.Results In UUO group, the urinary concentration of ET-1 increased substantially;both gene expression and protein level of ET-1 were much higher than those in control group. At the same time, tubulointerstitial fibrosis was present in the kidneys of rats with UUO. Conclusion ET-1 is involved in the pathogenesis of the injury of UUO.
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Objective To research the effects of bosentan in on unilateral ureteral obstruction (UUO) rats. Methods Wistar rats were randomly divided into three groups. UUO group was subjected to complete ligation of left ureter, sham operation group with sham operation was used as control. Bosenten group was subjected to complete ligation of left ureter and to stomach with bosentan suspension. Three groups were killed at 5,10,15,20 d. We examined renal pathological changes and the expressions of transforming growth factor-?1 (TGF-?1) in kidney of all rats using immunohistochemistry analisis method. The plasma and ureteral obstructive kidney urinary concentration of endothelin - 1 (ET - 1) was determined by radioimmunoassay. Results In Bos and UUO groups, the urinary concentration of ET - 1 increased substantially and the expression of TGF - ?1 were much higher than those in the S group. At the same time.tubulointerstitial fibrosis was present in the kidney of rats in Bos and UUO group. But the expression of TGF- ?1 and tubu-lointerstitial fibrosis in Bos group were lower than those of UUO group. Conclusion Bosentan can effectively amelirate tubulointerstitial fibrosis in rats with UUO by ET and TGF - ?1 machanism.