Polycystic kidney disease and polycythemia vera. Occurrence in a patient receiving hemodialysis.

Symptomatic erythrocytosis developed in a 59-year-old man with polycystic kidney disease (PKD) while he was receiving maintenance hemodialysis. Major clinical and laboratory data suggested a diagnosis of polycythemia vera (PV), despite a normal serum alkaline phosphatase level and leukocyte count. Secondary erythrocytosis, related to chronic hypoxemia and increased erythropoietin production, was excluded by appropriate laboratory studies. Despite previous documentation of secondary erythrocytosis in patients receiving hemodialysis, to my knowledge, PV has not been described in this population.

Small cell anaplastic carcinoma of the lung with mesangial proliferative glomerulonephritis.

Mesangial proliferative glomerulonephritis is an uncommon manifestation of renal injury associated with neoplastic disease. A 50-year-old woman with small cell anaplastic cancer of the lung and nephrotic syndrome had renal biopsy findings that were consistent with diffuse mesangial cell proliferation. Electron microscopic evaluation of renal tissue demonstrated numerous intramesangial and paramesangial dense deposits. Resolution of the nephrotic syndrome with improvement in renal function was noted after a response of the patient's tumor to combination chemotherapy.

Effect of aminonucleoside nephrosis on immune complex localization in autologous immune complex nephropathy in rats.

The effect of increased capillary permeability on glomerular immune complex localization was studied in rats immunized with proximal tubular antigen (Fx1A) to induce autologous immune complex nephropathy (AICN). AICN rats were made proteinuric by injection or unilateral renal perfusion with aminonucleoside of puromycin (PA) before developing subepithelial complex deposits. Control AICN kidneys developed diffuse granular deposits of IgG and Fx1A on the subepithelial surface of the glomerular basement membrane (GBM) at 3 wk by immunofluorescence and electron microscopy, and deposits increased in subsequent weekly biopsies. In contrast, PA-nephrotic AICN kidneys developed few or no GBM deposits and a significant increase in mesangial localization of IgG and Fx1A during the period of PA-induced proteinuria. These alterations in complex localization were documented both in rats with PA nephrosis and in unilaterally PA-nephrotic kidneys compared with contralateral controls in the same animals, thus excluding any effect of PA on the immunopathogenetic mechanism in AICN as an explanation for these findings. The absence of GBM deposits closely correlated with reduced staining for polyanionic glomerular sialoprotein in proteinuric kidneys, since PA-perfused kidneys studied 2 wk after resolution of proteinuria demonstrated return of normal staining for sialoprotein and development of subepithelial complex deposits similar to those in contralateral control kidneys. These studies demonstrate that properties of the glomerulus itself play an important role in determining the site of complex deposition in experimental AICN and suggest that electrophysical characteristics of the glomerular capillary wall may influence complex localization on the GBM.

Complement-independent nephrotoxic nephritis in the guinea pig.

Immunologic mechanisms of proteinuria were investigated in guinea pigs (GP) injected with sheep antiserum (NTS) to GP glomerular basement membrane (GBM). Linear deposition of sheep gamma 1 and gamma 2 IgG led to a prompt but transient (36 hr) increase in albumin excretion from control values of 0.026 +/- 0.013 mg/hr to maximal values of 26+/-12.1 mg/rh at six hours without detectable histologic or electron microscopic changes except for decreased staining for glomerular polyanion and epithelial cell foot process fusion. GBM permeability to anionic ferritin was not increased during proteinuria. Anti-GBM antibody deposits did not fix GP C3 or C4 in vivo or in vitro. NTS-induced proteinuria was the same in guinea pigs that were normal, greater than 95% depleted of C3 through C9, genetically deficient in C4, and depleted of circulating polymorphonuclear leukocytes (PMN). Prior administration of antihistamines, steroids, azathioprine, colchicine, indomethacin, heparin, aprotinin (Trasylol), and niridazole also failed to reduced proteinuria. Initial proteinuria subsided by 36 hr, did not recur despite linear deposition of GP gemma 1 and gemma 2 after day seven, and antibody to GMB-bound sheep globlin. In the GP nephrotoxic nephritis model, anti-GBM antibody deposits apparently mediate increased permeability to albumin by a currently undefined mechanism which is independent of complement, PMN, and other know mediators of inflammation.