Deposition of fibrin-stabilizing factor (F XIIIA and S), fibrinogen-related antigens, fibrinogen degradation products (FDPd and FDPe) and antihemolytic factor (F VIII) in renal disease: analysis of 161 cases by immunofluorescence microscopy.

To see whether or not the fibrin-stabilizing factor is involved in the pathogenesis of renal damage, we analyzed by IF the glomerular deposition of factor XIII (subunits A and S) in 161 patients with various renal diseases. In 4 out of 5 cases of thrombotic microangiopathy (80%), F XIII deposits were found in a continuous subendothelial pattern, in association with deposition of fibrinogen and FDP, suggesting the occurrence of intraglomerular coagulation. In 22 out of 45 patients with membranous GN (idiopathic or SLE-associated), F XIII deposits were found along the capillary walls in a subepithelial location.

Digestive tract and renal small vessel hyalinosis, idiopathic nonarteriosclerotic intracerebral calcifications, retinal ischemic syndrome, and phenotypic abnormalities. A new familial syndrome.

A new familial syndrome that affected 3 of 7 siblings is described. All 3 patients were young women with a very peculiar phenotype, poikilodermia and hair greying, and idiopathic nonarteriosclerotic cerebral calcifications. Pathological studies demonstrated a marked and progressive hyalinosis involving capillaries and often arterioles and small veins of the digestive tract, kidneys, and calcified areas of the brain.

Renal failure in myeloma: relationship with isoelectric point of immunoglobulin light chains.

Renal failure is a frequent but inconstant complication of myeloma related to light chain excretion. Since it has been suggested that cationic light chains (lc) are most likely to induce renal damage, we have studied the isoelectric point (pI) of light chains produced by 17 patients with myeloma and related the results to the type and severity of renal damage assessed clinically and pathologically. In order to do so, we have applied immunoenzymatic techniques which allow identification of light chain types as well as measurement of pI without prior purification.

Absence of 'true' minimal change nephrotic syndrome in African children in South Africa.

Minimal change nephrotic syndrome (MCNS) which is a well defined clinical, histopathological and therapeutic entity accounts for the majority of childhood nephrosis throughout the world except Africa. We describe 15 (13%) of 115 biopsy-confirmed African children with nephrotic syndrome who had minimal change on light microscopy. These are compared to 42 biopsied Indian children with typical MCNS.

Clinical significance of anti-Sm antibodies in systemic lupus erythematosus.

Case records of 34 patients with systemic lupus erythematosus (SLE) were analyzed. Twelve patients had both anti-DNA and anti-Sm antibodies (Group I) and 22 had anti-DNA antibodies only (Group II). The disease patterns were comparable, except for (1) cutaneous vasculitis, which was observed in six of 12 patients in Group I and one of 22 in Group II (p less than 0.

Renal lesions in multiple myeloma: their relationship to associated protein abnormalities.

Renal biopsy and autopsy specimens were studied in 43 patients with renal complications of multiple myeloma and correlated with immunoelectrophoresis (IEP) and other clinical data at the time of biopsy. Lesions specifically related to multiple myeloma fell into two categories, with different patterns of protein excretion. (1) Myeloma Cast Formation: When other lesions which might contribute to renal insufficiency (RI) were excluded, there was a good correlation between the extent of myeloma cast formation and severity of RI.

Drug-induced granulomatous interstitial nephritis.

Among 22 cases of drug-induced acute interstitial nephritis (AIN), noncaseating interstitial granulomas were found in eight cases (36%). Acute renal failure (ARF), oliguric in three patients, appeared within 1-20 days after the beginning of therapy. Clinical symptoms suggesting a hypersensitivity reaction were unusual, marked blood eosinophilia was absent, and immunologic tests were inconstantly positive.

Acute interstitial nephritis due to drug hypersensitivity. An up-to-date review with a report of 19 cases.

The clinical, biologic, and pathologic features and the course and treatment of acute interstitial nephritis (AIN) due to drug hypersensitivity are reviewed. The authors report 19 additional cases of AIN, outlining some particular and unusual features. The drugs most often responsible now are penicillins and cephalosporins, cotrimoxazole, thiazide diuretics, glafenin and its derivatives, and nonsteroid anti-inflammatory agents.

[Acute hematogenic interstitial nephritis of urinary origin: an unrecognized factor in the exacerbation of chronic kidney failure].

Hypertension, phosphate retention, hyperfiltration hyalinosis and the natural course of the underlying are well known factors leading to progression of chronic renal failure. Acute bacterial interstitial nephritis occurring in a previously diseased kidney, although well documented in experimental animals, has not been shown to aggravate chronic renal failure in man. We report on 3 cases of acute suppurative interstitial nephritis, due to E.

Acute endocapillary glomerulonephritis in adults: a histologic and clinical comparison between patients with and without initial acute renal failure.

30 renal biopsies were performed in 20 adult patients with acute diffuse endocapillary glomerulonephritis. 11 patients who presented with acute renal failure (ARF) and 9 patients who had normal or mildly altered renal function were compared in order to look for clinical or pathologic features peculiar to each group and possible differences in outcome. All patients underwent early renal biopsy, and 8 had repeat biopsies.

Immunopathological studies of polyarteritis nodosa and Wegener's granulomatosis: a report of 43 patients with 51 renal biopsies.

Although it is generally considered that vasculitis of the polyarteritis nodosa (PAN) group and Wegener's granulomatosis (WG) is immune complex (IC) mediated, there are no simultaneous data on circulating IC, complement levels and deposits of Ig and complement in the kidney. Therefore we have performed a retrospective study of 43 patients suffering from PAN and WG. Ig glomerular deposits were uncommon and scanty, except in two patients with WG; C3 deposits were detected in 12 patients, whereas fibrinogen was constantly found when lesions were recent and active.

[Protective effect of procyanidolic oligomers on the heterologous phase of glomerulonephritis induced by anti-glomerular basement membrane antibodies].

Treatment by procyanidolic oligomers can significantly decrease the proteinuria indiced in the Rat by intravenous injection of anti glomerular basement membranes antibodies. Immunohistological analysis shows that procyanidolic oligomers do not interfere with the mechanisms of immunopathological injury involved in this model (antibody binding to glomerular basement membrane, complement activation, glomerular influx of polymorphonuclear leucocytes). Their protective effect may be due to an increased resistance of the glomerular capillary to the inflammatory mediators released by neutrophils.

Severity of glomerulonephritis induced in different strains of suckling mice by infection with lymphocytic choriomeningitis virus: correlation with amounts of endogenous interferon and circulating immune complexes.

Renal lesions due to neonatal infection with lymphocytic choriomeningitis virus were studied in three different strains of mice known to produce different amounts of viral interferon. Very severe ultrastructural lesions similar to those induced by exogenous interferon were found as early as day 8 in C3H mice which produced the highest amount of interferon. Further studies could not be performed in these mice since all died by day 14.

[Light chain or monoclonal immunoglobulin deposition disease: physiopathogenic concepts].

Although recently identified, this disease is by no means exceptional. It is characterized by the deposition in various organs of an amorphous substance which differs from the amyloid substance and contains monoclonal immunoglobulin determinants: either a light kappa or lambda chain, or a light and a heavy chain. The severity of the disease is due to various organs being involved, notably the kidneys.

Circulating immune complexes and severe sepsis: duration of infection as the main determinant.

The relation between the duration of bacterial infection and circulating immune complexes (CIC) level was evaluated using the C1q binding assay in a group of patients with well defined clinical sepsis. Fifty-four patients with endocarditis and 35 with post-open heart surgery mediastinitis were prospectively studied over a period of 2 years. CIC were detected in 42% of patients studied.

[Bacterial endocarditis on a mitral prosthesis with negative blood cultures. Case evolving over a 3 year period in the absence of fever].

A 38 year-old man, operated upon two years previously for combined mitral and tricuspid valve disease of rheumatic origin, presented with signs of a severe biological inflammatory syndrome, and a mild proliferative glomerulonephritis (normal complement with exclusively mesangial deposits of IgM, Clq, and C3 on immunofluorescence), which developed slowly over a period of 3 years in the absence of any fever. Blood cultures were always negative, and prolonged antibiotic therapy on two occasions had no effect on the clinical or biological picture. Mitral prosthesis replacement was necessary 6 months after the onset of the affection, a second leakage 16 months later leading to rapidly fatal heart failure.

A new form of familial glomerulonephritis.

An unusual familial glomerular disease, characterized by the presence of diffuse round mesangial deposits of C3, is described in 2 siblings (1 male and 1 female) and their mother. The clinical picture in the 3 patients was a long-lasting proteinuria. An acute hemolytic uremic syndrome with malignant hypertension developed in the male at the age of 24 years, requiring bilateral nephrectomy.