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Biomedical subjects

G Hirbec

Publications and source records attributed to G Hirbec.

At least 19 recordsLinked to original sources
PubMed1986

[Lipoid nephrosis of toxic origin. 2 cases].

Classically, the histological lesion observed in a drug-or heavy metal-induced nephrotic syndrome is membranous glomerulonephritis. We report two cases of "toxic" nephrotic syndrome with unusual histological features. One was secondary to mercury intoxication and the other, to D-penicillamine in a patient with rheumatoid arthritis. In both cases, renal biopsy revealed minimal glomerular changes. The proteinuria rapidly disappeared after exposure to the toxic agent was discontinued. Genetic factors and a disregulation of the immune system with lymphokine production may be responsible for these renal changes. This study demonstrates that renal biopsy is necessary in this clinical setting.

Adult↗
PubMed1986

Coincidence of familial systemic lupus erythematosus and the fragile X syndrome.

The coincidence of fragile X syndrome (fra(X] and systemic lupus erythematosus (SLE) in the same family is reported here for the first time. A 16-year-old boy with typical fra(X) had a severe SLE with multiple organ involvement. His 12-year-old sister of normal intelligence had circulating antinuclear antibodies and proliferative glomerulonephritis. The fra(X) was not found in her karyotype. Except for abnormalities due to immunosuppressive treatment during pregnancy, the association of SLE and chromosome abnormalities has been only reported in Klinefelter's syndrome. The possible pathogenic role of sex hormonal abnormalities due to an extra X chromosome has been suggested in the occurrence of SLE.

Fragile X Syndrome↗
PubMed1985

Focal glomerulosclerosis associated with Charcot-Marie-Tooth disease.

This report describes a new case of the rare association of focal glomerulosclerosis with a peroneal muscular atrophy of the Charcot-Marie-Tooth type. The young patient was admitted with a nephrotic syndrome. A terminal renal failure rapidly developed. Numerous chronic nephropathies were known in the patient's family. The ultrastructural study of the renal biopsy revealed a focal fusion of the epithelial foot processes, thickened and pleated mesangial basal laminae, vacuolated podocytes and small intranuclear clear inclusions. The Charcot-Marie-Tooth disease was of the hypertrophic type. According to a review of the literature and to further information concerning the follow-up of the 5 previously reported cases, the prognosis of the nephropathy is guarded with progression to end-stage renal disease in a few years. This case represents the 6th reported case of focal glomerulosclerosis associated with Charcot-Marie-Tooth disease.

Adolescent↗
PubMed1984

Intraglomerular metastases. Report of two cases.

Glomerular metastases are rarely observed. Two cases of such metastases are reported. In one patient, proteinuria was detected and the diagnosis was made by kidney needle biopsy; light microscopy, immunofluorescence, and electron microscopy studies were performed. The second case was an autopsy finding. The histologic patterns were, respectively, an intracapillary metastasis and associated intra- and extracapillary metastases. These findings raise a number of questions concerning the early detection of such neoplastic diseases, the nature of primary tumors, and the mechanisms of malignant dissemination and glomerular localization.

Autopsy↗
PubMed1984

[Incorporation of L-azetidine-2-carboxylic acid into collagen of the skin. Structural changes].

As previously shown by two dimensional thin-layer chromatography L-azetidine-2-carboxylic-acid (L-Az) is incorporated into type I skin collagen instead of proline when 3 week old mice are fed with a 0,1% solution of L-Az orally. Ultrastructural investigations did not reveal significant changes in collagen periodicity and on fibril diameter. The collagen fibrils of the upper papillary dermis seemed to be packed more densely, sometimes only one electron dense lamina was seen instead of basal lamina and plasma membrane. The glycosaminoglycane-induced fibrillogenesis was not changed in contrary to the collagen-heat-gelation fibrillogenesis at 37 degrees C, where no gel aggregation could be seen. The reconstruction of native fibres from collagen solutions was disturbed too, several finer precipitated fibrils being detectable. On infrared spectroscopy significant differences in absorption spectra were detected. Correlating with previous results of reduced tensile strength and normal melting point of L-Az collagen we can conclude that L-Az might cause rather intermolecular than intramolecular disturbances of crosslinking.

Animals↗
PubMed1984

Silent lupus nephritis among patients with discoid lupus erythematosus.

A kidney biopsy was performed in 7 hypocomplementemic discoid lupus erythematosus patients despite the absence of overt renal involvement. Five patients had glomerular immune deposits and 2 patients with disseminated discoid lupus erythematosus exhibited definite proliferative glomerulonephritis. Those findings show that silent lupus nephritis may be encountered in discoid as well as in systemic lupus erythematosus, providing additional evidence supporting the unity of the disease. We suggest that hypocomplementemic patients with discoid lupus erythematosus must be carefully screened for renal disease by periodic urinalysis examinations.

Complement C3↗
PubMed1984

[Biological study of spontaneous amyloidosis in PS mice].

A remarkably severe spontaneous amyloidosis involving multiple organs has characterized the inbred PS mouse strain since the 25th generation. The amyloid substance was extracted with H2O and purified by successive gel filtrations on Sephadex G100, Sephadex G10 and Biogel P4. It was submitted to biochemical, immunochemical and histochemical analysis in order to determine its origin. Potassium permanganate resistance of the affinity for Congo Red dye, aminoacid composition, cross-reactivity with anti-mouse light chains antisera suggested an amyloidogenic process comparable to that described for the AL substance in man. However, even if abnormal lymphoid infiltrates were present in several organs, the presence of M component could not be demonstrated in serum or urine of these mice. This indicated either a limited tumoral mass or a tumor poorly secreting a precursor that would be strongly amyloidogenic. Alternatively, the existence of a so far unidentified precursor could not be excluded.

Amyloid↗
PubMed1979

Hereditary C2 deficiency associated with non-systemic glomerulonephritis.

A patient with non-systemic idiopathic glomerulonephritis was found to have a complete deficiency of C2, the second component of complement. The clinical course, histological findings and serological abnormalities are reported in detail. The renal disease was a mild glomerulonephritis with mesangial and subendothelial immune deposits comprising IgG, IgM and C3, increased mesangial matrix without significant cell proliferation. An immunogenetic analysis of the patient's family was carried out. It was demonstrated that the homozygous C2 deficiency was associated with heterozygotism for HLA-A, B and D. Only one of the C2 deficient genes was associated with the expected HLA-A10, B18 haplotype and the propositus was HLA-D2 negative. This report confirms the fact that non-systemic glomerulonephritis should be included in the variety of immunological disorders associated with a complement deficient state. However, C2 deficiency does not seem to be related specifically to a given histological variety of glomerulonephritis.

Adult↗
PubMed1976

[Action of pyridinol carbamate on hetero-immune Masugi nephritis in the rat (author's transl)].

Pyridinol-carbamate (P.C.) is a new substance with various properties including an anti-inflammatory (anti-kinin) and an antiplatelet aggregation activity. Since a coagulation process has been demonstrated in Masugi nephritis in Rats, we investigated the effect of P.C. in this experimental model. P.C. (150 mg/kg/day) was given orally from day 1 to day 28. It prevented partially the G.N.: proteinuria was significantly lower than in nephritic untreated animals with a reduction of seromucoid blood levels and B.U.N. Histological examination revealed that glomerular injury was limited in treated animals specially with regards to G.B.M. alterations and deposits.

Animals↗
PubMed1975

[Hypokalemic quadriplegia revealing Fanconi syndrome and myeloma].

The authors report a case of multiple myeloma presenting in an unusual fashion as a hypokalaemic quadriparesis secondary to a renal proximal tubulopathy. The tubular functional disturbance appeared to be related to the presence of kappa-type light chains. Electron microscopical study demonstrated in the cells of the concoluted tubules abnormalities which apparently constitute the anatomical substratum of this abnormality. Study of tubular function revealed glycosuria, proximal type tubular acidosis and altered reabsorption of phophorus and uric acid.

Acidosis, Renal Tubular↗
PubMed1975

A vascular permeability factor elaborated from lymphocytes. I. Demonstration in patients with nephrotic syndrome.

P.H.A. and Concanavalin A stimulated lymphocytes culture supernatants were comparatively studied in 23 volontary normals and in 46 patients with nephrotic syndrome. Intradermal injections to guinea-pig demonstrated a permeability increasing and pro-inflammatory factor. Vascular permeability (immediate reaction) is determinated by blue Evans technique. Delayed reaction is assesed by the cutaneous inflammatory process (erythema, induration); pathologically it is defined as polymorphonuclear and mononuclear infiltration. Differences are highly significant between nephrotic patients and normal subjects. This biological activity is not observed with control supernatants (medium plus normal human serum and P.H.A. or Conca A). Positive results were also found with M.E.M. eagle culture supernatants of nephrotic stimulated lymphocytes. To date lymphokines have been studied especially in animals. In human, they have been found only after concentration. Variations in lymphokine production may be present in some pathologic states.

Animals↗
PubMed1975

[Study of several parameters of hemostasis in Masugi's rabbit nephritis].

During experimental Masugi nephritis in the rabbit, were demonstrated various disturbances in hemostasis: a) during the initial stage: immediate, severe and transient fall in the platelet count without any change in Factor V;b)during the secondary stage, from the 7th to the 8th day onwards, increase in platelets and fibrinogen, in relation with the intensity of the nephrotic syndrome; c) in parallel, appearance of urinary fibrinogen split products in relation to the intensity of the glomerular lesions, evidence for the presence of intraglomerular fibrin. These facts confirm the role played by platelets in coagulation phenomena secondary to the immune reaction. They indicate, furthermore, the existence of hemostasis disorders during the nephrotic syndrome.

Animals↗