[Kappa light chain disease. Apropos of 2 anatomical cases of myeloma].
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Biomedical subjects
Publications and source records attributed to H Beaufils.
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Four cases of amiodarone-induced restrictive, hypoxaemic lung disease are described. The cumulative dosages of amiodarone were relatively low (30 to 100 g). Alveolar lavage studies showed a lymphocytosis and study of the lymphocytic sub-populations showed an increase in the OKT8 group, and an inversion of the OKT4/OKT8 ratio. The outcome was favourable on withdrawal of amiodarone and steroid therapy. The immunological origin of this form of lung disease was confirmed. Two patients had renal failure; in the first case, hypercalcaemia, hyperphosphoremia and renal calcification were observed. The second patient had endo- and extracapillary glomerulonephritis with C3 deposits and circulating immune complexes. Renal failure regressed in both cases on withdrawal of amiodarone and with steroid therapy.
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Signs of glomerulopathy, especially a nephrotic syndrome can occur in cancer patients, but the exact frequency of glomerular lesions is not well known in these patients. To define this frequency in a given type of malignancy we have studied the nephrectomy kidneys in 40 patients with renal cell carcinoma. Proteinuria, which was present in 7 cases, ranged from 0.15 to 1.5 g per 24 h. Reduction of the creatinine clearance greater than 50% was observed in 5 patients. Circulating immune complexes were detected in 11 of the 15 patients studied. Carcinoembryonic antigens were noted in 2 of 9 patients investigated. Research of alpha 1 foetoprotein carried out in 12 patients was always negative. HBs antigen or Hbs antibodies were detected in 6 of 29 patients studied. Light microscopic examination of the normal uninvolved kidney tissue showed obvious glomerular lesions (mesangial hypertrophy with or without deposits, with or without cell proliferation) in 7 patients (17.5%). Amyloid deposits were never observed. Immunofluorescence study revealed mesangial deposits in 35% of patients versus 5.4% of control subjects (P less than 0.0001). These deposits included C3 and/or IgM in 13 cases, IgA and C3 in one case. No fixation was observed, neither on tubules of normal tissue nor on carcinoma lesions. This report demonstrates that glomerular deposits are usually found in approximately one third of patients with renal cell carcinoma and that these deposits are located in the mesangial areas and not in the subepithelial space as it is often observed when glomerulonephritis is expressed by clinical symptoms.
Visceral involvement in systemic scleroderma is frequent, the most commonly affected organs being the kidney, the lung, the heart and the gastro-intestinal tract; associating different degrees of sclerosis and vasculitis depending on which organ is involved. However, this is not always clinically obvious, and angiospastic phenomena may be assumed to be responsible for certain ischaemic lesions. The diagnosis is also difficult because some visceral lesions may occur at an early stage and appear to be of primary origin. Extensive investigation, including biopsies to demonstrate small vascular changes then becomes essential.
A 39-year-old woman with documented sarcoidosis and membranous glomerulonephritis (GN) with renal failure received a kidney from her identical twin. A few months after transplantation a proliferative GN appeared with hematuria, proteinuria and progressive renal failure over 2 years. Indeed the occurrence of GN in patients with sarcoidosis raises the question of whether the relationship between these two conditions is causal or fortuitous. This report suggests that GN is secondary to sarcoidosis.
Acute glomerulopathy due to pneumococcal infection occurred in a patient with chronic discoid lupus. The onset of renal involvement in this context suggested that cutaneous lupus may have developed into systemic lupus erythematosus. Renal biopsy established the correct diagnosis. The physiopathology of glomerular involvement in lupus and infections is discussed. Attention is drawn to the significance of complement activation, either by the classical or the alternative pathways according to the etiology.
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Sera from 105 adult patients with idiopathic chronic glomerulonephritis (GN) were investigated to assess the diagnostic and prognostic value of cryoglobulin (CG) detection. CG+ sera were found in 49% of cases but also in 34% of normal donors. CG composition in these two groups was different. Concentrations of CG greater than micron g/ml were considered as abnormal, since concentrations did not exceed this value in the controls. Using this criterion 30% of patients with GN had CG; most had membrano-proliferative GN, GN with mesangial IgA deposits, and membranous GN. CG occurred with the same frequency but at higher concentrations in the group of patients with diffuse proliferative GN than in other groups; however, there was no difference in the frequency and concentration of CG when GN was thought to be mediated by immune complexes (IC) than when it was not. No relationship was observed with disease activity. In conclusion low CG might represent an in vitro artifact of a common in vivo immunoregulatory mechanism in normal donors. Although they might be a marker for the presence of IC in adult patients with GN not related to systemic disease, CG detection appears to be of little help in diagnosis, assessment of disease activity, or therapeutic monitoring.
In a 66-year-old female patient with a past history of tuberculosis, prolonged fever at 38 degrees C accompanied by a strong increase in ESR ultimately led to the diagnosis of renal cyst. Biopsies of renal tissue and of lymph nodes taken during surgery showed renal granulomatosis without caseum. Despite negative bacterial findings and anergy to tuberculin, an anti-tuberculous treatment was administered and the patient recovered.
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The clinical value of the immunological tests performed routinely has been evaluated in 91 patients with various histological types of glomerulonephritis. An antigen possibly involved in the pathogeny of renal disease was found in 24% cases. However, in most instances no direct link between the presence of this given antigen and the disease could be observed. Therefore systematic investigations for antigens should be restricted to dosages of antistreptolysin and antinuclear antibodies and to determination of Hbs antigen. Immune complexes, which were observed in many cases, were of no discriminant value for the clinician. Dosages of the complement fractions and the determination of cryoglobulins can be helpful for diagnostic, follow-up and treatment. Our results justify a restricted selection of the routine immunological tests used in patients with glomerulonephritis. More specific investigations remain useful when they are clinically oriented.
Full renal investigation were conducted in 11 patients with Behçet's syndrome. Biological tests revealed the presence of mild proteinuria in 5 of them. Needle biopsy showed extra-membranous or mesangial fibrinoid deposits in 6 cases, associated in 1 case with segmental and focal glomerulitis lesions. Immunofluorescence studies demonstrated glomerular deposits of mesangial and extra-membranous C3, sometimes with immunoglobulins (IgA-AgG). Ultrastructural examination was carried out in 7 cases and showed "hump" deposits attached to the external face of the basal membrane and rounded nodules of 7 to 15 mu included in the mesangium. The presence of immune complexes in the renal glomeruli, therefore, is not all unusual in this disease, which is probably auto-immune in nature, and in fact appears to be a quite frequent occurrence. These deposits, which were probably well-tolerated by most of the patients in this series, might be involved in the production of progressive glomerulopathies in chronic cases, as observed in one of the cases studied.
The pathogenicity of primary non-obstructive myocardiopathies remains unknown at the present time. Immunological disturbances have been demonstrated but their role is open to discussion. A comparison between 37 such patients and 37 control subjects with cardiac insufficiency, similar according to age and sex, gave the following results: circulating immune complexes levels, anti-organ or anti-tissue antibody levels, and the levels of serum complement and its fractions were the same in both groups. Serum IgA levels were significantly higher in the "patient" group, and this appears to be partly related to alcoholism. Finally, an antimyocardial cellular immunity exists. These results would appear to demonstrate that conventional immunological techniques are of limited value in primary cardiac insufficiency.
The study involved 298 cases of chronic glomerulonephritis (GN) in adults. The results of renal biopsy were used to classify the patients into four groups: Membranous GN, 81 cases; focal glomerulo sclerosis, 80 cases; Membrano-proliferative GN, 62 cases; GN with mesangial deposits of IgA, 75 cases. The patients were observed over a period ranging from 1 month to 36 years. The average period of surveillance for each category was between 4 and 6 years. The course in each histological type was assessed on the basis of actuarial tates of renal death, of moderate renal insufficiency (plasma creatinine greater than 1.5 mg%( and of hypertension. Renal survival at 10 years was was arounds 90% for membranous GN. 85% for GN with mesangial deposits of IgA, 70% for focal glomerulo sclerosis and 50% for membrano-proliferative GN. The prognosis should be based upon a combination of histological and clinical findings. Severity of prolonged nephrotic syndrome, regardless of the histological type of the nephropathy, is worthy of emphasis. In the group fo focal glomerulo sclerosis, prognosis differs greatly in relation to the presence or absence of a nephrotic syndrome. Complete remission may be seen in the group of focal glomerulo sclerosis, and in membrano-proliferative GN despite the persistence or worsening of histological lesions seen on repeated biopsies.
70 cases of focal glomerulosclerosis (FGS) followed for periods ranging from 1 to 24 years are reported. 39 patients had a nephrotic syndrome. 31 patients had asymptomatic proteinuria. 97 renal biopsies were performed. At least one biopsy specimen from every patient showed focal and segmental involvement of glomeruli, but no lesions were observed on the first biopsy in 6 patients. 26 of the 35 patients with a nephrotic syndrome were treated with steroids alone and/or chemotherapy and/or indomethacin. Resistance to treatment was encountered in 21 patients. Complete remission was observed in 6 cases, despite persistence or accentuation of histological lesions on serial biopsies in 3 cases. Actuarial renal survival rate at 10 years was 45% in the group with a nephrotic syndrome versus 91% in patients with proteinuria. FGS with a persistent nephrotic syndrome represents the 'malignant' form of the disease. Recurrence of the disease was observed after transplantation in 2 cases and was absent in 1.