Immune complexes with cationic antibodies deposit in glomeruli more effectively than cationic antibodies alone.

In previously published studies, highly cationized antibodies alone and in immune complexes bound to glomeruli by charge-charge interaction, but only immune complexes persisted in glomeruli. Because normal IgG does not deposit in glomeruli, studies were conducted to determine whether cationized antibodies can be prepared which deposit in glomeruli when bound to antigen but not when free in circulation. A series of cationized rabbit antiHSA was prepared with the number of added amino groups ranging from 13.

Antibody localization in the glomerular basement membrane may precede in situ immune deposit formation in rat glomeruli.

The administration of cationized antibodies, specific to human serum albumin, into the renal artery of rats caused transient presence of IgG in glomeruli by immunofluorescence microscopy. Intravenous infusion of appropriate doses of antigen after the injection of cationized antibodies resulted in immune deposit formation in glomeruli that persisted through 96 hr. By electron microscopy, these deposits were located in the subepithelial area.

Autosomal dominant polycystic kidney disease: symptoms and clinical findings.

In 259 subjects at risk to have inherited autosomal dominant polycystic kidney disease (PKD), the frequency of symptoms consistent with urinary tract infection, haematuria, back and abdominal pain, hypertension, renal stones, and end-stage renal failure was evaluated. The diagnosis of PKD was made in 140 of these subjects (54 per cent). At the time of the study, 36 per cent of males and 7 per cent of females with PKD were asymptomatic, normotensive, and denied any previous problems.

Precipitating antigen-antibody systems are required for the formation of subepithelial electron-dense immune deposits in rat glomeruli.

This study was conducted to determine whether multivalent, precipitating antigens are required for formation of subepithelial electron-dense immune deposits in glomeruli. 2-nitro-4-azidophenyl (NAP) was conjugated with variable density to human serum albumin (HSA) to yield nonprecipitating (NAP3.1 X HSA and NAP11.

Peripheral arterial thrombosis due to the nephrotic syndrome: the clinical spectrum.

We reviewed the literature concerning arterial thromboses associated with the nephrotic syndrome and the hypercoagulable state. An illustrative case of peripheral arterial thrombosis and pulmonary emboli attributed to the hypercoagulable state of the nephrotic syndrome is included. In our patient, serum levels of antithrombin III, plasminogen, factor VII activity, factor VIII activity, and factor VIII antigen were abnormal.

Fertility and pregnancy complications in women with autosomal dominant polycystic kidney disease.

Fertility and pregnancy complications were assessed in 137 women at risk of having inherited the gene for autosomal dominant polycystic kidney disease. Seventy-six (55%) of these subjects were found to have polycystic kidney disease (multiple renal cysts). The remaining 61 women served as controls.

Rearrangement of immune complexes in glomeruli leads to persistence and development of electron-dense deposits.

Covalently, cross-linked immune complexes were prepared with multivalent 2-nitro-4-azidophenyl X human serum albumin (NAP X HSA) and antibodies to NAP at five times antigen excess. After purification with gel filtration, affinity chromatography with antigen-agarose column, and addition of the hapten, 9.5% of the antibodies dissociated from the complexes by sodium dodecyl sulfate-polyacrylamide gel electrophoresis analysis.

Potential causes and pathogenesis in autosomal dominant polycystic kidney disease.

To evaluate early pathological changes in autosomal dominant polycystic kidney disease (PKD), percutaneous renal biopsy specimens from 14 subjects at risk for PKD were analyzed. The subjects represented 5 unrelated families, ranged in age from 11 to 26 years and had normal excretory urograms. One additional renal tissue specimen was obtained at the time of nephrectomy from a patient with PKD and end-stage renal failure.

Clinical studies of a continuous extracorporeal cyanate treatment system for patients with sickle cell disease.

An extracorporeal carbamylation system was evaluated in two patients with sickle cell disease. Access was achieved with existing veins in one patient and an AV fistula in the second. Modifications in the treatment procedure were made as experience indicated.

Liver cysts in patients with autosomal dominant polycystic kidney disease.

Liver cysts were found in 46 (29 per cent) of 158 patients over 10 years of age with documented autosomal dominant-type polycystic kidney disease (PKD) from 62 unrelated families. Hepatic cysts were not found in any patient at risk for PKD in whom renal cysts were not detected. The prevalence of liver cysts increased with advancing age and with declining rate of glomerular filtration.

Carbamylation distributions in extracorporeal treatment of sickle cell anemia.

The extracorporeal exposure of blood from patients with sickle cell anemia results in the binding of different amounts of cyanate to the hemoglobin of individual erythrocytes. This distribution pattern of carbamylated hemoglobin may affect the efficacy of treatment. A computer model has been developed to predict the carbamylation distribution attained in batch ex vivo exposures.

Severe systemic lupus erythematosus with nephritis in a boy with deficiency of the fourth component of complement.

A young boy with severe systemic lupus erythematosus was found to be totally deficient in the fourth component of complement. Family studies were consistent with an autosomal recessive mode of transmission and with linkage of the gene(s) determining C4 deficiency to the major histocompatibility complex; no disease states were associated with heterozygosity. This patient has had severe multisystem disease and immune complex glomerulonephritis presumably the alternative pathway of complement was utilized in the pathogenesis of his nephritis.

The appearance of nonlinear deposits of immunoglobulins in Goodpasture's syndrome.

A twenty year old woman presented with pulmonary hemorrhage and glomerulonephritis with linear deposition of IgG on the glomerular capillary basement membrane, and high titer of antiglomerular basement membrane antibody in her serum. Without treatment, renal function, which was transiently impaired, has improved, pulmonary hemorrhage subsided, and repeat renal biopsies have revealed the appearance of nonlinear in addition to the linear deposition of IgG on the glomerular capillary basement membrane in the face of diminished serum antiglomerular basement membrane antibody titer.

The adverse effect of high-dose steroids on renal autografts and homografts.

We observed that canine renal homografts preserved for 5 hours with hypothermic pulsatile perfusion to which large doses of methylprednisolone were added showed obliteration of glomerular capillary loops by eosinophilic proteinaceous material immediately after revascularization. The lesion progressed to focal necrosis at 24 hours and diffuse necrosis by 5 days. Urine formation was present 7.

Glycol methacrylate embedding of renal biopsy specimens for light microscopy.

Thirty-five consecutive renal biopsy specimens were evaluated by light histology using paraffin and glycol methacrylate embedding. The clarity of the renal structures was found to be much better, and the renal lesions better defined, using glycol methyacrylate embedding.