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Biomedical subjects

G Richet

Publications and source records attributed to G Richet.

At least 73 records · Page 4Linked to original sources

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. Ten of fifteen patients with renal failure produced lambda light chains. There was no simple relationship between the isoelectric point and nephrotoxicity. However, light chains with the lowest pI observed in this series were associated with normal renal function in two cases and with acute reversible but severe renal failure requiring dialysis in five cases. By contrast, pI values above 6.0 observed in the remaining patients were associated with moderate renal failure in six patients with recently diagnosed myeloma and with irreversible renal failure, and in two patients in whom myeloma had been evolutive for several years. We thus suggest that further pI measurements may help to identify light chains with different nephrotoxic potentials.

Acute Kidney Injury↗

[Epidemiologic aspects of hypertension in pregnancy. Study of 442 cases].

We studied retrospectively the case records of 442 women who were followed because of hypertension during a pregnancy. Pregnancy hypertension was more frequent in white european than in black or arabic women. It occurred more frequently in women with an important professional activity than it did in housewives. 58% of patients had a familial history of high blood pressure, 35% were obese, 12% had glucose intolerance and 18% had hypercholesterolemia. Those frequencies were out of proportion with those in the control population. Finally, 32% of patients remained hypertensive after pregnancy. It is concluded that pregnancy hypertension occurs mainly in women whose genetic and metabolic background is that of essential hypertension.

Adult↗

[Renal involvement in scleroderma].

Renal biopsies were carried out in 29 patients with scleroderma to study the early vascular lesions and their eventual clinical significance. Haemolytic acute renal failure was present in 9 patients. The biopsies showed early vascular lesions on interlobar arteries. The main biological change was proliferative or fibrous endarteritis. Mucoid infiltration was found in 3 biopsies. The arterioles were spared or only slightly affected without major fibrinoid necrosis. These lesions were therefore distinct from those of malignant hypertension. However, at autopsy of 2 of these cases, the vascular lesions were undistinguishable from those of malignant hypertension. The biopsies in 13 out of 14 patients with scleroderma without obvious renal involvement (9 cases) or with moderate proteinuria and/or hypertension without renal failure (5 cases) showed interlobar endarteritis with associated mucoid infiltration in 3 patients. This lesion was isolated but sometimes extensive even in young patients without hypertension. One patient died within one year, of disseminated colonic carcinoma, and 4 of cardio-respiratory failure due to scleroderma without hypertension, renal failure or proteinuria. Eight of the 9 remaining patients were traced 6 to 16 years after biopsy. Two were moderately hypertensive but none had renal failure. Cutaneous and internal organ scleroderma had regressed in the majority and proteinuria had disappeared in 3 cases.

Acute Kidney Injury↗

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 0.01); (2) pulmonary manifestations, nine of 12 in Group I and two of 22 in Group II (p less than 0.001); (3) cardiac manifestations, eight of 12 in Group I and four of 22 in Group II (p less than 0.01); and (4) renal biopsy, which showed milder lesions in Group I than in Group II (p less than 0.05). Evolution was fatal in four patients in Group I and in none in Group II. It is suggested that in SLE, the presence of anti-Sm antibody is associated with a much higher incidence of vasculitis, resulting in peculiar visceral manifestations, which can be poorly responsive to therapy. Whether there is a direct association between anti-Sm antibody and vasculitis or whether the common denominator is a genetic selection remains to be determined.

Adolescent↗

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. The glomerulonephritis recurred on a renal allograft. This disease is not HLA-linked and no characteristic abnormality of complement profile was seen in the 3 patients.

Adolescent↗

Tamm-Horsfall protein and erythrocyte ghosts immunologically cross-react.

Antisera were raised in the rabbit against human Tamm-Horsfall protein (THP) and erythrocyte ghosts. They were purified and exhausted with serumalbumin. Using a radioimmunoassay, antisera against THP allowed to detect in erythrocyte ghosts 41 ng of a reaction material per mg membrane protein. Ouchterlony double diffusion technique showed a similar corss-reaction and, conversely, a cross-reaction between THP and antisera to erythrocyte ghosts. These data support the view that red cell membrane contains a compound related to THP.

Animals↗

[Hepatic and renal deposits of kappa light chains revealing a dysglobulinemia (author's transl)].

A 38 year old woman presented portal hypertension and proteinuria. Liver biopsy showed peliosis hepatis with non amyloid deposits, reactive with anti-kappa antibody by immunofluorescence, along the sinusoids and in the Disse spaces. There are identical deposits along glomerular and tubular basement membranes. An underlying pleomorphic lymphoplasmocytic malignancy, analogous to Waldenström's macroglobulinemia was found, but without serum or urine monoclonal Ig. Biosynthesis experiments showed that the bone marrow cells synthesized abnormally short light chains with a larger apparent molecular weight when secreted than in the cytoplasmic extracts, presumably because of glycosylation. These results suggest a causal relationship between production of abnormal Ig chains and tissue deposition.

Female↗

[Vascular and obstetrical prognosis after hypertension during pregnancy: value of renal biopsy (author's transl)].

Renal biopsy was performed in 125 patients with severe hypertension during pregnancy. Three groups could be defined: I: normal kidney; II: vascular lesions; III: nephropathy. The histological appearance could not be suspected from clinical criteria (age, parity, symptoms, date of appearance). About 70% of the patients in groups I and II had permanent metabolic disorders (mainly hypercholesterolemia) and had a familial history of hypertension. Recurrence of hypertension during subsequent pregnancies occurred in 70% of cases, identical in groups I and II, and therefore impossible to predict by histological findings. Permanent hypertension developed in 47% of group II patients and 8% of group I (p < 0.001). The authors conclude that hypertension during pregnancy affects a sub-population genetically selected and that renal biopsy is of little value for predicting the prognosis in future pregnancies. The indications for renal biopsy are discussed.

Adult↗