Seronegative spondyloarthropathies and IgA glomerulonephritis: a report of four cases and a review of the literature.
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Biomedical subjects
Publications and source records attributed to G Lagrue.
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To estimate food sensitization, the human basophil degranulation test (HBDT), usually used for allergy diagnosis, was performed in 34 idiopathic nephrotic syndrome (INS) patients, classified as minimal change glomerular disease, with or without atopic manifestations, and in 19 blood donors; five food allergens (wheat flour, cow's milk, whole egg, beef and pork) were investigated. The HBDT was positive in 22 out of the 34 INS (64%) and in 1 out of the 19 blood donors (p less than 0.001). The test was 9 times positive for wheat flour, 9 times for pork, 6 times for cow's milk, 6 times for beef and 5 times for whole egg. Moreover in 13 cases, the basophil sensitization was present for two or three of these food allergens in the same patient. A direct causal relationship between food sensitization and INS cannot be drawn from this study: it might be demonstrated only by elemental or oligoantigenic diet prior to specific avoidances.
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A morphometric technique is reported that uses a new selective staining of the elastic system fibers in skin biopsy specimens to facilitate the quantitative evaluation of the volume fraction occupied by these elastic fibers in the tissue. The study of elastic fibers in the dermis of 30 patients, before and after six months of treatment with Colchicin, was carried out with a Quantimet 720 system. Preelastic (oxytalan and elaunin) fibers and mature elastic fibers were quantitated separately. Compared to the average volume fraction (surface occupied by the elastic fibers) before treatment with Colchicin (1.449 +/- 0.64%), the mean values after treatment were significantly increased (2.076 +/- 0.61%). The same results were found for the preelastic fibers: 0.807 +/- 0.51% before treatment and 1.025 +/- 0.54% after treatment. These results demonstrate the advantages of our monochromatic staining method for automatic quantitation of elastic fibers as well as the possibilities of the quantitative study of the elastic fibers in human dermis. This methodology should be applicable to other inherited or acquired diseases affecting skin elastic fibers as well as to other tissues containing elastic fibers.
The protein C-protein S natural anticoagulant system was evaluated in 32 patients with proteinuria, 11 without nephrotic syndrome (NS) and 21 with NS of different grade. Antithrombin III (AT III), factors II, VII, X were also measured in the same groups of patients. In addition to plasma levels of these proteins, urinary loss of protein C was evaluated in concentrated urine specimens from 17 of the 21 patients with NS. The protein C antigen level was found to be normal or high in NS, but the difference between the control group and nephrotic patients was statistically significant only in severe NS. For all proteinuric patients, the plasma concentration of protein C correlated positively with the degree of proteinuria, cholesterol level, triglyceride level, and correlated inversely with serum albumin concentration; 17/17 patients with NS exhibited urinary loss of protein C and the degree of protein C excretion correlated positively with the degree of proteinuria. Plasma protein S antigen was measured in only 11 patients with NS and was found to be significatively increased, with a negative correlation with serum albumin concentration. AT III did not differ between the control group and the proteinuric patients with or without NS. Factors II and X were in the normal range for all patients. Factor VII was increased even in mild NS. Thus, the plasma levels of protein C and protein S antigens were normal or increased in patients with NS and this is probably related to an increased liver synthesis rate of these proteins, which is secondary to proteinuria.(ABSTRACT TRUNCATED AT 250 WORDS)
Primary thrombocythemia may cause vascular thrombosis; it has been rarely involved in coronary atherosclerosis with myocardial infarction. We report three cases of renal arteries atherosclerosis occurring in association with primary thrombocythemia. These cases are three young women (20, 40 and 42 years old) with severe hypertension secondary to atherosclerosis with stenosis of renal arteries, one or both sided, and in association in one case with diffuse arterial stenosis. Systematic investigation revealed thrombocytosis with latent myeloproliferative syndrome of megacaryocytic colony. Thrombocytosis was previously present as attested by a blood count one year before (in one case) and by long-term peripheral vasomotor troubles, electively improved by aspirin (in two cases). In none of these three cases, vascular risk factors, nor hereditary vascular diseases were present. So we assume that platelets high levels are responsible for this early atherosclerosis, in keeping with the well-know role of platelets in atherosclerosis pathogenesis. Platelets investigations must be done in case of renovascular hypertension, occurring without any classical vascular risk factors.
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High prevalence of HLA DR7 was well documented in idiopathic nephrotic syndrome (INS) of childhood. Our data in 43 INS, child and adult patients, did not show any significant difference from control group, specially for HLA DR7. Moreover a significant difference occurred when the data was compared in child and adult INS patients. HLA DR7 was more frequent when the nephrotic syndrome appeared before 15 years old. There was no correlation between the presence of HLA DR7 and those of allergy history or increased serum IgE level, on the opposite with previous data. The presence of DR7 would favor its onset at an earlier age. Alternatively, the difference in DR7 prevalence could be an additional argument for considering that the corticosensitivity and negative histological criteria might collect, under the same name, different glomerulopathies.
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.
The effect of colchicine was studied in 51 hypertensive subjects with several other vascular risk factors. Colchicine was administered for 3-4 months in a daily dose of 1 mg per os. The treatment did not change the lipid content in the blood and in skin biopsies, and had no effect on systolic and diastolic blood pressure. On the contrary, colchicine treatment significantly improved the conjunctival biomicroscopy score, the duration of the dicrotic wave and the peripheral resistance index. The results show the improvement of the microcirculatory parameters (elasticity of arteries) without changes of serum and tissue lipid parameters in the patients treated with colchicine.
The biosynthesis of proteins and glycosaminoglycans (GAGs) was determined in skin biopsies from atherosclerotic patients treated perorally for 3 months with 1 mg/day colchicine. The biopsies were incubated with 3H-glucosamine and 14C-proline for 5 h and subsequently digested with pronase. In an aliquot of the pronase digest, the specific radioactivity of 14C-proline and 14C-hydroxyproline were determined. The 3H-glucosamine-labeled GAGs were identified by specific enzymic assay and quantified after electrophoretic separation. 3 months treatment with colchicine did not modify the total amounts of proline and hydroxyproline in skin proteins, but diminished the amount of the GAGs as expressed by uronic acid content. Colchicine treatment decreased also the specific radioactivity of proline and hydroxyproline, which reflects a decrease of total protein and collagen synthesis. The incorporation of 3H-glucosamine in the 3H-GAGs was also decreased, mainly in hyaluronic acid. These results suggest that peroral administration of colchicine modifies the synthesis of extracellular matrix proteins and polysaccharides by skin fibroblasts.