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To study the clinicopathologic features of collagen III glomerulopathy and its cause, pathogenesis and prognosis.Five cases of collagen III glomerulopathy that collected from 2005 to 2014 were observed by renal biopsy. The morphologic characteristics were studied by light microscopy, immunofluorescence, immunohistochemical and electron microscopy.The glomerular mesangium became expansion but no hypercellularity, basement membrane appeared thickened. The glomeruli showed collagen type III deposit by immunohistochemistry method, and collagen fibers increased by electron microscopy. The patients often show serious proteinuria, nephrotic syndrome and renal function damage.Collagen III glomerulopathy is an idiopathic glomerular disease, characterized by massive accumulation of collagen type III within the glomerular mesangial areas and basement membrane. Collagen III glomerulopathy is extremely rare. The etiology and pathogenesis may relate to the abnormality of collagen III gene. There is no specific treatment for it and its prognosis is poor.

Tissue transglutaminase (tTG) is a Ca(2+)-dependent enzyme which stabilizes the extracellular matrix (ECM) through post-translational modification, and may play an important role in the pathogenesis of focal and segmental glomerulosclerosis (FSGS). Here, we have investigated whether tTG contributes to the glomerular ECM expansion in the puromycin aminonucleoside (PAN)-injection-induced experimental rat model of FSGS. The localization and expression of tTG, MMP-9 gelatinase, and the ECM component fibronectin (FN) in kidneys was determined by immunohistochemistry and measured by semi-quantitative analysis. Protein levels of tTG and MMP-9 were also analyzed by Western blotting.In situtransglutaminase activity was assayed by measurement of incorporated substrate and the immunofluorescence staining for the cross-linking product, epsilon-(gamma-glutamyl) lysine. Prominent proteinuria, a typical pathological feature of FSGS, was observed in PAN injection group rats. tTG immunoreactivity was located markedly in glomeruli and the levels of this protein in whole-kidney homogenates of PAN injection group rats were significantly increased (361+/- 106% control, P0.05). Similarly, transglutaminase activity and epsilon-(gamma-glutamyl) lysine were also predominately located within glomeruli and were much more intense in the PAN-injected group than that in control animals. MMP-9 was also located primarily within glomeruli. In PAN-injected kidneys, protein levels of active MMP-9 were significantly reduced (59+/- 27% control, P0.01), while pro-MMP-9 levels increased (148+/- 42% control, P0.05). Remarkable expression of glomerular fibronectin (FN) was found in PAN injection group rats. Semi-quantitative analysis demonstrated this increased intensity of FN staining in the PAN-injected rats was 149+/- 23% of the control values (P0.05). Enhanced cross-linking of ECM by tissue transglutaminase and decreased degradation due to reduced active MMP-9 expression may be at least partially responsible for the deposition of FN within injured glomeruli in experimental FSGS.

Mycophenolate mofetil (MMF) is a potential new treatment for diffuse proliferative lupus nephritis. This study examines the clinical and histopathological effects, and potential mechanisms, of combination MMF/prednisone therapy in diffuse proliferative lupus nephritis. Nine patients with diffuse proliferative lupus nephritis confirmed by renal biopsy received MMF/prednisone for six months when repeat biopsies were performed. Clinical and histopathological parameters of activity and chronicity were studied. Collagens were detected by Sirus red staining; leucocyte phenotype, osteopontin (OPN), fibrinectin (FN), a-smooth muscle actin (a-SMA) and TGF-b1 were detected by immunohistochemistry. The changes of clinical and histopathologic parameters were assessed and compared to histopathologic indicators. Eight of the nine patients achieved clinical remission; renal function deteriorated in one. Histopathological activity indices reduced significantly (9.56 + 2.83 versus 5.22 + 1.86, P < 0.01); however, the chronicity indices did not change (3.56 + 1.42 versus 3.22 + 1.20). T-cell and monocyte/macrophage infiltration, OPN expression and the percentage of proliferative cells in both glomerulus and tubulo-interstitiumdecreased significantly. Other features of chronic lesions, except for glomerular collagen deposition, did not change. In conclusion, MMF/prednisone therapy was effective for our patients with proliferative lupus nephritis. The active inflammatory lesions could be ameliorated through reduction of lymphocyte and monocyte/macrophage infiltration, inhibition of cell proliferation and downregulation of adhesive molecules. However, the chronic fibrotic lesions could not be significantly reduced.

Histological observations of 20 occluded vas segments obstructed by the standard procedure of medical polyurethane-vas occlusion in 10 volunteers requesting vasectomy and of 20 vas segments with plugs removed from 10 vas-occluded men requesting vas reversal were carried out in order to elucidate the mechanism of vas occlusion by medical polyurethane (MPU). Twenty vas deferens in vasectomy group were ruptured, only a small amount of MPU elastomer remained within the vas lumen, most having leaked through to encircle the ruptured vas. Histomorphology of the removed vas segments from 10 vas-occluded men, who had undergone MPU-vas occlusion 4 years earlier, showed diffuse proliferation of connective tissue, fibrosis, or hyalinization of fibroplastic tissue and local infiltration of lymphocytes and macrophages. The vas lumen both proximal and distal to the plugs was completely blocked. Sperm granuloma, foreign body granuloma, proliferation of nerve fibers and local infiltration of lymphocytes were found in four vas-occluded men with painful nodules. These results suggest that the contraceptive mechanism of MPU-vas occlusion could be the result of secondary obstruction due to tissue proliferation by MPU irritation after the rupture of the vas deferens.

To investigate the role of electron microscopy (EM) and immunoelectron microscopy (IEM) in the diagnosis of light chain deposition diseases (LCDD) and primary amyloidosis (AL) of the kidney. METHODS Renal biopsy specimens collected in our hospital from Jan. 1994 to Feb. 2002 were studied by EM, and ultrastructural immunogold labeling for light chains (kappa, Lambda) were made on the cases suggested of LCDD and early stage AL in the preliminary diagnosis.Six cases were diagnosed as LCDD, and punctate, granular, electron-dense material was identified in the outside of tubular basement membrane (TBM), inner layer of glomerular basement membrane (GBM) and vascular walls by EM. Two of which showed interrupted segmental localization of granular material in subendothelial areas of GBM. The granular material was labeled by monotypical light chains (4 cases of kappa chain, 2 cases of Lambda chain). Thirty-six cases were diagnosed as AL. EM revealed fibrillary material distributed in glomerular mesangium, GBM and vascular walls; 15 of them showed segmental distribution of fibrils in mesangium and/or GBM, and were further studied by IEM labeling for light chains. The fibrillary material was labeled by monotypical light chains (4 cases of kappa chain, 11 cases of Lambda chain). CONCLUSION The diagnosis of renal LCDD and early stage of AL depended on EM, IEM is a unique diagnostic tool in early stage of LCDD and AL.

To study the relationship between monocyte/macrophage (MC/MP) accumulation and tubulointerstitial fibrosis.The renal tubulointerstitial fibrosis model in Wistar rats was established by unilateral renal vein ligature. The rats were fed for 25 days. The kidneys were obtained every 5 days by killing the rats. The morphological changes of tubulointerstitial fibrosis were observed by light microscopy with HE, PAS, PASM and Masson staining. Immunohistochemistry and double immunohistochemistry were used to observe MC/MP accumulation and proliferation. In situ hybridization and immunochemistry were used to observe MCP-1 and M-CSF expression in experimental renal tissue. The MCP-1 protein expression was inspected by Western blot. All the data were analyzed statistically.The pathological changes of tubulointerstitial fibrosis were typical. There were many MC/MPs accumulsated in the interstitial space at the early stage and some of them were PCNA positive. At the late stage both accumulation and proliferation of MC/MPs were decreased. The portion of monocyte proliferation was high correlated with the MC/MP accumulation. In situ hybridization showed the positive signals of MCP-1 and M-CSF were mainly located in the cytoplasm of degenerated tubular epithelium and they were strong at the early stage, weak at the late stage. MCP-1 by immunochemistry and Western blot were consistent with in situ hybridization. The MC/MP accumulation was high correlated with the expression of MCP-1 and tubular epithelium degeneration. The portion of monocyte proliferation was high correlated with the expression of M-CSF.There was obvious accumulation of MC/MP at the early stage of tubulointerstitial fibrosis. The accumulation came from infiltration and proliferation which were regulated by degenerated tubular epithelial cells producing MCP-1 and M-CSF. MC/MP accumulation was highly correlated with tubular degeneration. MC/MP promoted tubulointerstitial fibrosis and damaged tubular epithelium by secreting a variety of cytokines.

To investigate the effects of low bovine serum, high glucose and high albumin on the phenotypic transformation of cultured renal tubular epithelial cells to mesenchymal cells.The human kidney proximal tubular cells were cultured for 7 days in the presence of low bovine serum (0.2 mol.L-1), high glucose (4.5 g.L-1) and high albumin (15 g.L-1), and morphological changes were assessed by electronic microscopy, immunohistochemistry stain and Western blot to show the phenotypic changes, including the expressions of cytokeratin, vimentin, collagen I and III, alpha-SMA, and TGF beta 1. The expression of collagen I mRNA was assessed by in situ hybridization.Renal tubular epithelial cells cultured in low bovine serum, high glucose and high albumin, showed obvious morphologic changes, including elongated shape, reducing of microvillus and mitochondria, increasing of rough endoplasmic reticulum, and the appearance of actin microfilaments. Immunohistochemistry stain and Western blot showed the low expression of cytokeratin and high expression of vimentin, collagen types I and III, alpha-SMA, and TGF beta 1. The increasing expression of collagen I mRNA was assessed by in situ hybridization.Lower bovine serum, high glucose and high albumin can induce the expression of TGF beta, and lead to the phenotypic transformation of tubular epithelial cells to mesenchymal cells.

To study the diagnosis and differentiation of primary small round cell tumors of bone.Thirty-nine cases were studied by light microscopy and immunochemistry of O13, NSE, S-100, Actin and LCA.20/22 cases of EW/PNET were positive for O13.16/22 cases of EW/PNET and one case of small cell osteosarcoma were positive for NSE. S-100 was positive in EW/PNET, small cell osteosarcoma and mesenchymal chondrosarcoma. All malignant lymphomas were positive for LCA. 6 cases of small cell osteosarcoma have osteoid production. Islands of cartilage were found in mesenchymal chondrosarcoma.O13 is a specific valuable marker for the diagnosis of EW/PNET. Histologic features of the osteoid in osteosarcoma and the cartilage in mesenchymal chondrosarcoma are important for the diagnosis.

The clinicopathologic and immunohistochemical features of 11 pseudomyxoma peritonei (PMP) cases were studied to determine pathologic diagnosis, site of origin and prognosis.Clinical files of 11 cases of PMP were reviewed with follow up. The changes in mucinous tumors of peritoneum and primary tumors under microscope and immunostaining were reviewed.Eleven cases (8 women, 3 men) are reported. The patients age ranged from 36 to 76 (average 56). One died 2 years after operation, and one case was lost. The remaining 8 cases were alive 1 to 60 months postoperatively. Of the 11 cases, 8 cases had appendiceal mucinous neoplasm of the 11 cases, and 5 women had synchronous ovarian mucinous tumors; colon mucinous adenocarcinoma was present in one case with synchronous ovarian mucinous tumor; simple ovarian mucinous tumors were present in two cases. Immunostainings were consistent on mucinous tumors of appendix, ovary and peritoneum in the same case.To diagnose the PMP, the type of tumor should be considered whether it is benign, low malignant or malignant. The appendix neoplasm is closely related to PMP. The prognosis depends greatly on the growth speed of the mucinous neoplasm.

To investigate the morphological features, immunohistochemical speciality of the gastrointestinal stromal tumors (GISTs), and its histogenesis as well.The morphologic characteristics of GISTs were studied in 65 cases using light microscopy and 17 cases by electron microscopy. The expression of c-kit (CD117), CD34, vimentin, SMA, actin, desmin, S-100 and MBP were detected in all the cases with EnVision trade mark staining.Among 65 cases of GISTs, 46 were spindle cell type, 6 epithelioid cell type and 13 mixture type, equivalent to 85.5% (65 of 76) of all of the mesenchymal tumors of gastrointestinal tract admitted in the same period. The epithelioid cell type tumors composed of mainly the epithelioid cells, predominantly short spindle, oval or round in pattern, with an overall eosinophilic cytoplasm by hematoxylin-eosin stain. Focal cytoplasmic vacuolization was often seen. Sometimes signet-ring like cells and cells with a clear cytoplasm were seen in the epithelioid stromal tumor. The tumor cells arranged in interlacing fascicles forming whorls or sometimes cell clusters. Electronic microscopy showed the presence of interdigitating cell processes, in some areas, synapse-like structure and numerous desmosome-like junctions as well as a few gap junctions and small round neurosecretory granules. There were also abundant intermediate filaments and thin filaments of actin-type with longitudinal condensations (dense bodies). All of the 65 stromal tumors were strongly positive for vimentin (100%), 61 out of 65 tumors positive for CD117 (c-kit) (93.8%) and 51 out of 65 positive for CD34 (78.5%). Some cases also expressed SMA, actin, S-100 and MBP.GISTs were the most frequent mesenchymal tumor seen in the gastrointestinal tract. Under light microscope, the morphology of stromal tumors looks sometimes like a leiomyoma and Schwannoma. The application of immunohistochemical markers (particularly CD117 and CD34) and ultrastructural study are considered necessary for the differential diagnosis. GISTs may originate from the pluripotential precursor cells like the interstitial cells of Cajal.