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Biomedical subjects

G Hirbec

Publications and source records attributed to G Hirbec.

At least 37 records · Page 2Linked to original sources
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↗
PubMed1975

[ Products of fibrin degradation in the urine during experimental and human glomerulonephritis].

Intravascular coagulation localized in glomeruli is of pathologic importance in human and experimental GN. The measure of fibrinogen related antigen (FRA) in serum and urine after concentration (Merskey's technique) was used to detect and estimate this phenomenon. In Rabbit Masugi GN, FRA were detected in urine 5 to 20 mg/24 h, in close correlation with the amount of proteinuria, the intesity of histological changes and the presence of fibrin deposits in glomeruli. In human GN, urine FRA were detected in many cases (0,5-10 mg/24 h) in correlation with the histological type of lesions (FRA + in primary or secondary proliferative GN) and with the evolutivity of disease (FRA + in cases with rapidly progressive kidney function deficiency). Urine FRA are also in correlation with intraglomerular fibrin deposits : this suggests that urine FRA originate from lysis of fibrin deposited within glomeruli. So urine FRA appears to be an indicator of type and severity of GN and probably of therapeutic measures, indicating anticoagulant and/or antithrombic therapy : the variations of urine FRA during treatment is of value to assess the effects of these drugs and to establish the prognosis of the disease.

Animals↗