1/9. Primary synovial sarcoma of the kidney: a case report with literature review.We describe a case of primary renal synovial sarcoma (SS) in a 48-year-old man. The patient presented with hematuria and was found to have a large tumor in his left kidney on computed tomography scan. histology revealed a highly cellular spindle cell neoplasm with minimal pleomorphism. The major differential diagnoses included leiomyosarcoma, hemangiopericytoma, and SS. The presence of focal areas with a biphasic pattern, uniformly positive immunostain for bcl-2, focally positive immunostains for epithelial membrane antigen and cytokeratin, and negative immunostains for CD-34, smooth muscle actin and S-100 established the diagnosis. This was subsequently confirmed by molecular testing for t(X;18) translocation. Since the existence of primary SS in the kidney was first suggested in 1999, to the best of our knowledge a total of 19 cases including the present case have been reported to date. Although primary renal SS is rare, these findings indicate that it should be included in the differential diagnosis of spindle cell tumors of the kidney.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/9. Papillary adenoma of the prostatic urethra: report of two cases.Papillary adenoma of the prostatic urethra is a rare cause of hematuria, hemospermia and lower urinary tract obstruction, especially in young adults. We report two cases of papillary adenoma and their endoscopic views. One of the patients had a lesion located on the verumontanum and presented with hematuria, hemospermia and symptoms of lower urinary tract obstruction. The other patient had papillary lesions located in the paramontanal gutter and presented with persistent painless hematuria. The origin of both lesions was proven to be prostatic epithelial differentiation using immunohistochemical identification of prostate-specific antigen. Papillary adenoma is believed to be benign, therefore, both lesions were carefully excised and fulgurated transurethrally.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/9. IgA nephropathy: acute renal failure, acute tubular necrosis, and features of postinfectious acute glomerulonephritis.From 1976 to 1987 on our Nephrological Unit, 57 patients with IgA nephropathy (IgAN) proven by renal biopsies were found. Three of those presented with acute tubular necrosis (ATN) and glomerulitis, without extrarenal predisposing cause in two; and showed, as prominent manifestation, a severe acute renal failure syndrome (ARFS), needing dialytic treatment. All three had hematuria, which was macroscopic in two and microscopic in one. Thus the prevalence of the association of glomerulitis and ATN was about 5.2%. There was complete recovery of renal functions in all three patients, but the usual symptomatology of IgAN. Two patients presented polymorphonuclear neutrophils infiltration of glomerular capillaries and in one of them, electron-dense deposits on the epithelial side of glomerular basement membrane ("humps") were observed, as well as those identified in the mesangial area. The glomerular polymorphonuclear neutrophils infiltration and endothelial cells proliferation (cases 1 and 3), the presence of "humps" (case 1), high antistreptolysin O (ASO) titers (cases 1 and 2), and low serum complement levels (case 1), suggest the possibility that antigens able to cause postinfectious glomerulonephritis (streptococcal or not) could induce in some individuals, by another immunopathogenetic route, mixed histopathological and clinical features of IgAN and postinfectious glomerulonephritis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/9. nephrolithiasis and hematuria--sometimes a stony road to diagnosis.We report a case of a young man with a history of kidney stones. Occurrence of gross hematuria several months after the extracorporeal shock wave, lithotripsy (ESWL) treatment lead to hospitalization. By ultrasound and abdominal CT scan, the urologist could exclude post-renal causes of the gross hematuria and acute renal failure. After transfer to a department of nephrology hemodialysis was started, an immediate kidney biopsy was performed and prednisolone was administered on the same day. The kidney biopsy revealed an anti-glomerular basement membrane (GBM) disease. The renal function did not recover and the patient remained on hemodialysis. In the literature it has been hypothesized that ESWL-treated patients are prone to develop anti-GBM disease by liberation of glomerular basement antigen through the ESWL high energy shock waves. An additional hypothesis considering the higher susceptibility for anti-GBM disease among certain HLA-tissue types is discussed with regard to our case. Unfortunately, the prolonged track to diagnosis and delayed immunosuppressive treatment could not prevent poor clinical outcome. Although anti-GBM disease is a rather rare disease, it should be included as a differential diagnosis for hematuria--especially months after ESWL treatment. Otherwise early diagnosis may be missed and as in our patient immunosuppressive treatment will remain unsuccessful to recover renal function.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/9. Intravenous desmopressin acetate in children with sickle trait and persistent macroscopic hematuria.Persistent gross hematuria associated with sickle hemoglobinopathy that fails to respond to conventional supportive therapy represents a difficult management dilemma. Two such patients with protracted, often painful, sickle trait macrohematuria are described. Both patients had normal renal anatomy and vasculature and had failed to respond to bed rest, intravenous hydration, and a trial of oral epsilon-aminocaproic acid. Patient 1 had normal coagulation function. Patient 2 had von Willebrand disease (decreased factor viii antigen and quantitative ristocetin cofactor activity). Patient 1 responded to intravenous desmopressin acetate at a dose of 0.3 microgram/kg with a 155% increase in factor viii clotting activity and a 135% increase in ristocetin cofactor and cessation of her macrohematuria within 18 hours after completion of the desmopressin infusion. She remained free of gross hematuria for 5 months with the exception of short-lived trauma-induced hematuria (in three voids) 6 weeks after desmopressin therapy. Patient 2 did not respond to intravenous desmopressin infusion despite a 234% and a 360% increase in factor viii clotting activity and ristocetin cofactor, respectively. Intravenous desmopressin acetate may be helpful in halting protracted significant macrohematuria associated with sickle trait hemoglobinopathy in some patients when conventional management fails.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/9. sickle cell trait and hematuria associated with von Willebrand syndromes.A von Willebrand syndrome was present in four patients with sickle cell trait and hematuria. The first two patients had severe anemia and active bleeding and received cryoprecipitate, with prompt cessation of hemorrhage. All of the patients had repetitive laboratory and clinical features; that is, reduced, but detectable, factor viii-related antigen, heterogeneity of, and incongruities within, the coagulation studies performed by consistently defective platelet aggregation to ristocetin correctable only with normal plasma. Bleeding outside the genitourinary tract never was observed. Because of the focal nature of the hemorrhage, the hematuria may not have been perceived as part of a general hemorrhagic disorder and the diagnosis not pursued. These observations suggest that when sickle cell trait and hematuria occur together, a von Willebrand syndrome should be a major diagnostic consideration that ultimately may point toward a rational, effective, easily administered, and clinicially acceptable form of treatment with cryoprecipitate.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/9. Hemorrhagic urethritis in female-to-male transsexual. Possible androgen-related phenomena.This article describes a case of hematuria and urethritis related to androgen stimulation in a female-to-male transsexual receiving testosterone cypionate. biopsy of the affected urethral tissue revealed periurethral glands which demonstrated a strong positive reaction with immunoperoxidase staining for prostatic specific antigen. When the androgen stimulus was reduced, the patient's symptoms resolved. This report gives evidence for possible androgen-induced pathology in the female urethra.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/9. Evidence for Pseudomonas antigen in immune complexes in Pseudomonas osteomyelitis.In infections associated with immune complex disease, microbial antigens have rarely been found in the complexes. Using an enzyme-linked immunoassay, we studied the immune complexes of a patient who had hematogenous pseudomonas aeruginosa osteomyelitis associated with palpable purpura, arthritis, and microscopic hematuria. After separation of the complexes into high and low molecular weight fractions, a 6-fold selective concentration of P aeruginosa antibody was found in the low molecular weight fraction compared with the concentration of the serum. Following disruption of immunoglobulin, the high molecular weight fraction competed with solid-phase P aeruginosa antigen for P aeruginosa antibody from another source. After successful treatment of the infection, the patient's symptoms resolved, and the complexes disappeared. These findings strongly suggest that immune complexes in this patient contained P aeruginosa antigen and antibody that may have been pathogenetic in his disease.- - - - - - - - - - ranking = 7keywords = antigen (Clic here for more details about this article) |
9/9. Renal medullary carcinoma: a potential sickle cell nephropathy of children and adolescents.An extremely aggressive malignant epithelial neoplasm of the kidney has recently been described and named renal medullary carcinoma. The finding of this tumor is highly predictive of drepanocytes (sickle cells) in tissue sections and thus the presence of sickle hemoglobin, specifically sickle cell trait, in the patient. We present a case report of this rare tumor in a 10-year-old male. The tumor displayed a variable histologic architecture including gland-like areas with intra- and extracytoplasmic material resembling mucin with hematoxylin and eosin stain. This material was negative with periodic acid-Schiff and mucicarmine stains, stained only weakly with alcian blue, and was positive using antibodies against peanut agglutinin. Tumor cells stained positively with antibodies to epithelial membrane antigen, cytokeratin, vimentin, and ulex europaeus lectin. The luminal face of tumor cells stained with peanut agglutinin. Stains using antibodies against carcinoembryonic antigen and alpha-fetoprotein were negative. Ultrastructurally, the tumor cells were characterized by short microvilli lining the luminal surface and lateral complex infoldings of adjacent plasma membranes. We discuss the relationship of this neoplasm to another renal pelvic neoplasm, collecting duct carcinoma, which may rarely occur in children. Renal medullary carcinoma should be included in the differential diagnosis of gross hematuria, which is most commonly benign self-limited hematuria, in young patients with sickle cell trait.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |