[Anti-cytoplasm antibodies to polynuclear neutrophils: specificity in renal vasculitis]. / Anticorps anticytoplasme des polynucléaires neutrophiles: spécificité dans les vascularites rénales.

We present a retrospective study of all patients with a positive anti-neutrophil cytoplasm autoantibodies (ANCA) result from July 1987 to December 1990. The aims of the study were to assess specificity of ANCA in renal vasculitis and to correlate ANCA immunofluorescence subtypes with clinical aspect, diagnosis, severity and outcome. Anti-myeloperoxidase antibody (A-MPO) was sought in 31 cases. Specificity was 86% and false-positive results were 11%. ANCA subtypes varied with the clinical picture. C-ANCA were more often present when renal vasculitis was associated with pulmonary and ear, nose and throat involvement; P-ANCA were more often present when there was no respiratory tract involvement. Thus C-ANCA were preferentially associated with Wegener's granulomatosis and P-ANCA with microscopic polyarteritis and pauci-immune necrotizing and crescentic glomerulonephritis. No correlation was found between ANCA subtypes and severity or outcome. A-MPO were positive in 14 cases (especially in micropolyarteritis and necrotizing glomerulonephritis).

Rapidly progressive glomerulonephritis associated with bacterial endocarditis: efficacy of antibiotic therapy alone.

A 65-year-old woman presented rapidly progressive glomerulonephritis with purpura and mitral insufficiency. Blood cultures grew Streptococcus mitis. By light microscopy, the renal biopsy revealed necrotizing glomerulonephritis 56% associated with cellular crescents and tubulointerstitial changes. By immunofluorescence, deposits of IgM and C3 were found to be present in the mesangium. Electron-microscopic study showed subendothelial and intramembranous deposits. Treatment with antibiotics alone resulted in renal recovery with disappearance of proteinuria, circulating immune complexes and cryoglobulinemia. A 2nd renal biopsy, performed after 3 months, showed segmental sclerosis and tubulointerstitial lesions. Eight months after the first hospitalization, cardiac insufficiency occurred. Four years later, a valve replacement was performed. No abnormal serum creatinine, serum creatinine clearance or urinalysis levels were present. These data suggest that rapidly progressive glomerulonephritis associated with bacterial endocarditis may be treated by antibiotics alone and result in normal and stable renal function.

[The heart in chronic kidney failure patients]. / Le coeur de l'insuffisant rénal chronique.

Risk factors for heart disease in patients with chronic renal failure (CRF) are the same as in general population; moreover CRF and renal replacement therapies (dialysis, immunosuppressive drugs for kidney transplantation) induce further specific cardiac risks. In practice, the commonest heart diseases associated with CRF are coronary artery diseases, myocardiopathies from various aetiologies, valve diseases and arrhythmias. Uremic pericarditis are quite unusual nowadays. Advances in therapy authorize easier control of congestive heart failure, the major complication of heart disease in CRF patients. Furthermore, it was observed that correction of anemia with erythropoietin therapy or kidney transplantation can ameliorate or reverse partially some cardiac diseases.

[Micropolyarteritis]. / Micropolyartérites.

Micropolyarteritis are defined as necrotizing inflammatory lesion of the wall of small vessels affecting different organs. A necrotizing crescentic glomerulonephritis without immuno-deposits is observed in the kidney, frequently involved in this microscopic form of systemic vasculitis. Micropolyarteritis are regrouped with Wegener's syndrome and isolated pauci-immune necrotizing crescentic glomerulonephritis as "ANCA-related vasculitis", whose diagnosis is assessed throw renal biopsy and prognosis dependent on immuno-suppressive treatment. Sixty-one patients treated in our nephrology department for a RPGN during these ten recent years have been retrospectively studied. Among them, twenty-five patients presented clinical and morphologic aspects compatible with the diagnosis of micropolyarteritis. We present their clinical aspects and evolution under treatment.

[Anti-polynuclear neutrophil cytoplasmic antibodies of the perinuclear type or p-ANCA]. / Les anticorps anti-cytoplasme des polynucléaires neutrophiles de type périnucléaire ou P-Anca.

Some neutrophil cytoplasmic auto-antibodies produce perinuclear immuno staining of alcohol-fixed neutrophils called P-Anca pattern. These autoantibodies show chiefly reactivity with myeloperoxidase in Elisa but other specificities have been detected (elastase, cathepsin, lactoferrin). These P-Anca anti-MPO are more frequent with renal angiitis but P-Anca (anti-MPO negative) are associated with other diseases without renal failure (rheumatoid arthritis, Gougerot-Sjogren and ulceritis colitis).

[The European experience of liver-kidney transplantation for primary hyperoxaluria type I. Prevention of recurrent intrarenal oxalate deposits]. / Expérience européenne de transplantation hépato-rénale dans l'hyperoxalurie primitive de type I. Prévention de la récidive des dépôts d'oxalate au niveau du rein.

Following simultaneous liver-kidney transplantation for primary hyperoxaluria type I, although the missing enzyme is provided by the liver transplant, there is a risk of recurrent calcium oxalate crystal formation in the implanted kidney. The necessary kidney protection methods are extrarenal blood purification, principally haemodialysis and haemofiltration, and an abundant diuresis ensured by copious fluid intakes together with prescription of diuretics. These therapeutic measures reduce the oxalate concentrations in both blood and urine. Oxalates are excreted in large amounts over a long period, owing to the formation of important tissue deposits during systemic oxalosis.

Pharmacokinetics of a low molecular weight heparin (Fraxiparine) in various stages of chronic renal failure.

This study investigates the pharmacokinetics of a low molecular weight heparin (Fraxiparine) after a single bolus intravenous injection of 100 antifactor Xa IC U.kg-1 in 3 groups of patients affected by chronic renal insufficiency of various severity: group A (n = 7) was composed of hemodialyzed patients; groups; B (n = 7) had a creatinine clearance ranging from 10 to 20 ml.min-1 and group C (n = 5) from 30 to 50 ml.min-1. There was no significant difference between the pharmacokinetic parameters determined in the 3 groups of patients and no correlation between these parameters and the creatinine clearance. However, when compared to the values established in a group of 12 healthy volunteers, the half-life of disappearance of the antifactor Xa activity was significantly prolonged. Therefore it is advised to monitor antifactor Xa activity in patients affected by chronic renal insufficiency of any severity to avoid a possible accumulation phenomenon.

[Plasmapheresis and acute attacks of uveitis]. / Plasmaphérèses et poussées aiguës d'uvéite.

Our study concerns the effects of plasma exchange in acute attacks of posterior or total chronic uveitis. The treatment undertaken in 10 patients consisted of 8 plasma exchanges in a 2 weeks period, associated with a corticosteroid and immunosuppressive therapy. Immediately after the plasma exchanges, there was a significant improvement in visual acuity and a decrease in inflammatory activity in most patients (70%). Plasma exchange seems to be an interesting adjuvant in the difficult treatment of some uveitis.

[Segmental renal hypoplasia of an adult originating from pseudo-tumor hyperreninism]. / Hypoplasie rénale segmentaire de l'adulte à l'origine d'un hyperréninisme pseudo-tumoral.

A 38 year-old man was admitted for severe hypertension with hypokalemia. Blood pressure was 180-120 mmHg, funduscopic examination revealed grade II retinopathy and left ventricular hypertrophy was present. Laboratory data disclosed: natremia = 140 mmol/ml, kalemia = 2.8 mmol/l, chloride = 105 mmol/l, bicarbonate = 30 mmol/l, creatinine clearance = 100 ml/mn, natriuresis = 140 mmol/day, kaliuresis = 80 mmol/day. Intravenous pyelography was normal. Angiography revealed a defect in the mid third of the right kidney without arterial abnormalities. Study of renin angiotensin aldosterone system showed: plasma renin activity: peripheric blood = 36 ng/ml/h (normal range I to 2), right renal vein = 30 ng/ml/h. Left renal vein = 18 ng/ml/h, inferior cava vein = 19 ng/ml/h. Plasma aldosterone level = 86 ng/100 ml (normal range 10 to 15). Captopril acute administration was followed by a fall of BP to 70-50 mmHg at 2 hours. Right nephrectomy was performed and revealed an ischemic retracted cortical area without necrosis nor tumoral aspect. The day after BP was 140/80 mmHg. Eight days after, kaliemia was 4.2 mmol/l, PRA was 0.5 ng/ml/h. Light microscopy showed that affected area was sharply delimited from surrounding normal tissue. In this area, glomeruli were present and seemed more numerous as usual; interstitial fibrosis and infiltrates of inflammatory cells were also noted. The main fact was tortuosities of intralobular arteries, thickened, with intimal proliferation. There was a pelvic recess near this cortical tissue. Immunofluorescence findings: antirenin serum fixed on JGA but also on interlobular arterial walls and on peritubular interstitium.(ABSTRACT TRUNCATED AT 250 WORDS)

Unsuitable value of abdominal fat tissue aspirate examination for the diagnosis of amyloidosis in long-term hemodialysis patients.

Abdominal fat tissue aspiration was used in 22 long-term hemodialysis patients (5-17 years). Fourteen of these patients had carpal tunnel syndrome and amyloid deposits of beta 2-microglobulin in the synovium. One patient had a spontaneous rupture of the spleen with amyloid deposits in spleen vessels. Seven other patients presented carpal tunnel syndrome and/or articular pains, and radiological lytic lesions in bone, strongly suggesting an amyloid origin. As a control group, in 22 patients with biopsy-proven amyloidosis, abdominal fat tissue aspirates were performed and were studied under the same conditions: by light microscopy these tissues were stained with Congo red and examined with a polarizing microscope; these specimens were also studied by electron microscopy. In all hemodialyzed patients, no amyloid deposit was present in fat tissue with Congo red staining and by electron microscopy. On the contrary, amyloid was observed in 17 of 22 cases in other types of amyloidosis. It seems that this method which has been proved to be simple and sensitive for the diagnosis of systemic amyloidosis is not a good marker for the presence of amyloid in long-term hemodialysis patients.

[Alleviation of ocular complications of the hyperviscosity syndrome in Waldenström's macroglobulinemia using plasma exchange]. / Amélioration des complications oculaires du syndrome d'hyperviscosité de la macroglobulinémie de Waldenström par les échanges plasmatiques.

Two patients presenting ocular complications of hyperviscosity syndrome in Waldenström's macroglobulinemia have been treated by plasmapheresis after inefficiency of immunosuppressive therapy. Plasma exchange seems an useful treatment as shown by clinic and angiographic improvement. In each case improvement of retinopathy was correlated with decrease of serum IgM level and blood viscosity measured by isotopic method. Fluorescein angiography, easily repeated, seems a very good method for following hyperviscosity syndrome in Waldenström's macroglobulinemia.

Low haematocrit and prolonged bleeding time in uraemic patients: effect of red cell transfusions.

This study demonstrates that a low haematocrit is the main determining factor of the prolonged bleeding time often encountered in uraemic haemodialysed patients. Thirty-three patients submitted to regular haemodialysis and having a platelet count greater than 100 X 10(9)/l were investigated with the following tests: simplate bleeding time, blood cell count, platelet aggregation induced by ADP, collagen and sodium arachidonate, arachidonate induced MDA synthesis, tests for detection of an acquired storage pool disease, and factor VIII complex level. The results were compared to two other groups; one of uraemic patients not yet subjected to haemodialysis and another of healthy volunteers. The results were basically identical in the two groups of uraemic patients. The only consistent abnormality was a 30-35% reduction in the platelet MDA synthesis in comparison with control subjects. There was a negative correlation between the log bleeding time and the haematocrit (r = 0.78, P less than 0.01). Fourteen uraemic patients having a prolonged bleeding time were submitted to a red cell transfusion programme and were investigated a second time under identical conditions. There was no change in any of the platelet function tests or in the factor VIII complex level, but the bleeding time was normalized when the post-transfusion haematocrit was over 26% (nine patients). This study emphasizes the role of anaemia in the pathogenesis of the prolonged bleeding time in uraemia and suggests that red cell transfusion can be a long-term efficient therapeutic measure to stop bleeding in these patients.