There is no information concerning renal functioning. races or locations. The reports contribute Rabbit polyclonal to ZNF287 to a better understanding of this disease, which although not so prevalent, should be considered as an importantly differential diagnostic of cases of proteinuria. Background Collagenofibrotic Glomerulopathy (CG) is a rare and recently defined entity characterized by deposition in the mesangial glomerulus and in the subendothelial space of type III collagen fibers [1]. It clinically manifests itself with proteinuria, hematuria, hypertension and variable degrees of renal failure in adults and children [2,3]. Type III collagen inside the basal membrane of the glomeruli is already part of the identification of another disease, known as Nail-Patella Syndrome. This syndrome is characterized by bone and nail abnormalities, associated with proteinuria of variable degrees. Publication of articles related to this new entity began in the late 70’s, and it was made by a team of Japanese doctors who considered this disease to be either a variation of Nail-Patella Syndrome or a completely new one [4]. Based on Upamostat the archive of renal biopsies at Nephopathology Service at General Pathology at the Federal University of Triangulo Mineiro (UFTM), we have identified three cases of CG that occurred from 2000 to 2007. There hadn’t been any cases reported in South America until that time, since the great majority of cases had occurred in Japan [5]. Case Presetation Case 1 Female, 55 years old, hypertensive for the last 20 years. In the last 5 years, she had been showing microscopic hematuria associated with leukocyturia Upamostat and cylindruria. The patient presented proteinuria (1.18 g/24 hours). Clearance of creatinine: 52 ml/min/1.73 m2. No changes to the clinical test. The patient underwent renal biopsy on December 12, 2000. One fragment was taken, because the patient showed severe hypertension during the performing of the biopsy. This fragment was processed by the electronic microscopy scanning, and consequently, there were no fragments for immunofluorescence microscopy. Renal Biopsy (semi-thin slices): There were eight glomeruli, and two of them were globally sclerotic. The other six glomeruli showed global expansion of the mesangium, thickening of capillary walls and no substantial hypercellularity. The capillary lumina were narrowed but not occluded. Foci of interstitial fibrosis and arteriolar hyaline deposits were found. Electronic microscopy scanning demonstrated expansion of the glomerular mesangium and subendothelial space by dense and curvilinear structures (banded fibrillar material). There were rare “calcium-like” deposits in subendothelial spaces. Upamostat The dense lamina of the glomerular capillary basement membranes seemed normal (Figure ?(Figure1A1A and ?and1B1B). Open in a separate window Figure 1 Case 1 (2001): Electronic micrographs sections show expansion of the glomerular mesangium and subendothelial space by dense and curvilinear structures (banded fibrillar material. (Original magnification: A 3000; B 8500). Case 2 Female, 21 Upamostat years old, white, previously healthy and presenting no symptoms, no family background related to renal diseases. The patient presented proteinuria (1.6 g/24 hours) for a year, associated with microscopic hematuria. There is no information concerning renal functioning. The patient underwent a renal biopsy on May 31, 2005. Renal Biopsy: In the light microscopy, there were ten glomeruli, one of them was totally sclerotic. The rest presented mesangial hypercellularity which could go from mild to moderate, with apparent increase of the mesangial matrix. Staining with picrosyrius showed mesangial expansion with reddish positive material and with greenish birefringence under a polarized light microscopy. Tubules and interstice.
