[Ocular manifestations of thrombotic thrombocytopenic purpura]. / Manifestations neuro-ophtalmologiques révélant un purpura thrombotique thrombocytopénique.

We report a case of thrombotic thrombocytopenic purpura (TTP) in a 47-year-old woman, who presented fluctuating visual disturbances which had developed over the last six months. An antiphospholipid syndrome was suspected and intravenous heparin treatment was started. One week later, hemolytic anemia and renal insufficiency occurred. Severe deficiency of von Willebrand factor-cleaving protease was found and a diagnostic of TTP was made. The clinical outcome was favorable after treatment with plasmapheresis and fresh frozen plasma. Diagnosis, etiology and treatment of this life-threatening disease are discussed.

Polyclonal rabbit gamma globulins against a human cytotoxic CD4 T cell clone. II. Use in prevention of rejection in kidney transplantation: a pilot study.

Antiblast globulins (GAB) were prepared by immunization of rabbits with activated T lymphocytes (AT) derived from a rejected kidney allograft. AT consisted of a CD4+ (CD3+, CD2+ TCR alpha+ beta+) clone cytotoxic for HLA DR8-positive targets. The immunizing cells were adapted to industrial growth conditions by repetitive stimulations with an EBV-transformed line from the kidney donor and recombinant IL-2. In the pilot study, GAB (1.0-1.5 mg/kg/day) was given in 12-hr infusions, in association with prednisone (Pred) 1 mg/kg/day and azathioprine (Aza) 2 mg/kg/day, as prophylactic treatment of rejection in 12 kidney-transplanted patients during the first 2 weeks postgrafting. GAB dosage was further adapted according to the level of circulating E-rosette-forming T cells (ERFT). Cyclosporine A (8 mg/kg/day) was given at day 14 as a monotherapy after Pred and Aza were progressively tapered. No patient died, but one kidney was lost from surgical complication. No rejection occurred under GAB treatment; 41% of patients had at least one episode in the first 3 months and 16% from 3 to 9 months. GAB side effects were minor (skin rash: 2, low grade fever: 4) except for one acute serum sickness. Platelet and white blood cell counts were unchanged, but there was a significant decrease in hemoglobin during the 2 weeks of GAB infusions. Few infectious episodes occurred (3 bacterial, 2 viral). GAB monitoring showed a dramatic drop in T11+, T3+, T4+, and T8+ circulating T cells (less than 10% of normal values between days 3 and 14), whereas EFRT cells had a delayed and somewhat lower decrease (less than 10% after day 6 only). Consequently, mean GAB doses had to be raised to 1.3 mg/kg/day at day 4 and 1.6 at days 8 and 14. This pilot study suggests that this new bioreagent should be of major interest in the prophylaxis and treatment of rejection in allograft recipients. A controlled study is in progress.

Direct determination of blood recirculation rate in hemodialysis by a conductivity method.

Blood recirculation is one of the key factors of decreasing dialysis efficiency. Determination of recirculation rate (R) is necessary to optimize effective dialysis delivery and to monitor vascular access function. R can be directly measured by a conductivity method in paired filtration dialysis (PFD), a double-compartment hemodiafiltration system that permits direct access to plasma water via the ultrafiltration stream. Measurement of R, in this system, involves the first of two conductivity sensors integrated in a urea monitor (UMS, BelIco-Sorin, Mirandola, Italy), and two saline injections. The rise in conductivity (deltaC1) induced by a 2.7 ml bolus of hypertonic saline 20% (mg/dl) in the arterial line serves for calibration, and is followed by an equivalent injection into the venous line, giving rise to deltaC2. The ratio deltaC2/deltaC1 equals R. A comparison between R values obtained with this method and with the low-flow technique in 31 chronic dialysis patients during 138 PFD sessions is reported. Mean R+/-SD by the conductivity method was 5.1+/-2.0 and 5.7+/-2.0% after 65 and 155 minutes of PFD (correlation coefficient, r = 0.75), whereas it was 6.4+/-4.9% and 5.5+/-4.6% after 30 sec of low blood pump flow for urea and creatinine markers, respectively (r = 0.35). After 120 sec of low flow, mean R increased to 9.0+/-5.1 and 8.8+/-4.6% for urea and creatinine, respectively (r = 0.45). Considerable discrepancies were found in R values measured simultaneously with the two blood markers. Statistically significant differences were found between the two measurement modalities (blood-side and conductivity); the correlation coefficients (r) varied between 0.28 and 0.41. The observed differences in mean R results do not seem considerable from a clinical perspective. The best agreement between blood-side and conductivity R measurements was obtained with Rcreat after 30 sec of low flow. Overall, a wider distribution was found in R values from blood-side determinations, most likely consequent to variability in the dosing method. The conductivity method appears more accurate and simple in assessing total R, and can be readily automated and integrated into the dialysis machine. The authors, therefore, recommend evaluation of R using methods not based on chemical blood concentration values.

[Kidney transplantation after 60 years of age. Survival of patients and functional results]. / Transplantation rénale après 60 ans. Survie des malades et résultats fonctionnels.

Between January 1, 1984 and October 1, 1988, 36 cadaveric kidney transplantations were performed in 60 to 66 years' old patients (mean: 62.4 years). Induction immunosuppression consisted of rabbit antithymocyte globulins for 10 days or 33B3.1, an anti RIL2 monoclonal antibody (n = 6), with azathioprine and corticosteroids (less than 1 mg/kg/day). Cyclosporin A (CSA) was given on day 10 in doses of 8 mg/kg/day. Steroids were withdrawn on day 45. Actuarial patient and graft survivals were 66 per cent and 80.5 per cent respectively at 1 and 2 years. Mean +/- SD serum creatinine level was 178 +/- 77 mumol/l at one year. The mean CSA dose adjusted to total blood CSA levels was 4.75 +/- 1.7 mg/kg. Five patients died in the first quarter, 2 of opportunistic infections, 2 of cardiovascular disease and 1 of gastric haemorrhage. The prevalence of rejection episodes during the first 3 months was lower than in the general population (22 per cent vs 27 per cent); all were reversible with bolus injections of corticosteroids. These results indicate that kidney transplantation can successfully be performed in elderly patients with a low rejection rate and an excellent long-term graft survival. However, the mortality was higher than in younger recipients.

[Dent's syndrome. Nephrology follow-up of four patients of the same family]. / Le syndrome de Dent. Suivi néphrologique de quatre cas de la même famille.

Dent's syndrome is a rare inherited tubulopathy. Factors influencing renal function in this disease are not well known. The aim of our study is to investigate the evolution of the Dent's syndrome in renal plan. The study was retrospective and conducted in 2006, concerning four brothers. The genetic defect was a mutation S244L missense in exon 6 of gene CLCN5. Various parameters were studied. Patients were 8.5 to 21-years-old at the beginning of the follow-up. Two of them had chronic renal insufficiency (CRI) which evolved, at least 7 years of moderate to terminal stage. Tubular signs were made of hypokalemia, hypercalciuria, hypophosphatemia and proteinuria mostly ß2 microglobulin. Improvement of these abnormalities was obtained with symptomatic treatment which has not always been well tolerated. A case of beginner nephrocalcinosis was observed. There was size and weight delay at the beginning of patient monitoring. Dent's syndrome may be complicated by CRI. It seems to appear in the second decade of life and hypercalciuria would contribute to it. Our CRI patients had significant bone disease. The hypercalciuria and proteinuria are factors over which we try to act. Clinical trials are needed to evaluate the efficacy of treatment on the reduction of CRI or its progression by reducing these factors in patients with Dent's syndrome.

[Late onset cystinosis. Apropos of a case]. / Cystinose de révélation tardive--a propos d'une observation.

This report concerns a new case of late onset cystinosis with heavy proteinuria. Renal biopsy showed focal segmental glomerular hyalinosis and the usual tubular and glomerular lesions of cystinosis. The pathogenesis of glomerular hyalinosis remains unclear.

[Extra-capillary proliferation complicating the development of an extramembranous idiopathic glomerulonephritis]. / Prolifération extra-capillaire compliquant l'évolution d'une glomérulonéphrite extra-membraneuse idiopathique.

Occurrence of crescentic formation superimposed to idiopathic membranous glomerulopathy worsens severely its prognosis with evolution to end stage renal disease. We report the case of a 65 years old man with nephrotic idiopathic membranous glomerulopathy who subsequently developed an acute renal failure secondary to crescentic formation. Pulse of high dose methylprednisolone administration improved renal function. Seven similar observations are analysed and pathogenic mechanisms discussed.

[Pulmonary infection from Rhodococcus equi after renal transplantation. Review of the literature]. / Infection pulmonaire à Rhodococcus equi après transplantation rénale. Revue de la littérature.

Rhodococcus Equi, a strictly aerobic Gram positive coco-bacillus, is a pathogen for horses and foals. It may induce opportunistic infections and is described in AIDS infected patients. We report the case of a 47-year old man, breeder of horses, with kidney transplant who has presented, 8 years after his graft, an impairment of health, a fever and evidence of pulmonary disease. The pulmonary biopsy under scanner guidance and microbiology study, has displayed the diagnosis of Rhodococcus equi infection. The evolution has been favorable with double antibiotherapy (follow-up 27 months). Ten comparable observations have been published after organ transplantation: (kidney: 8; heart: 1; liver: 1). Pulmonary locations are widely predominant. The animal contact is found only in 30% of cases. The presentation of the sickness has been compared to pulmonary tuberculosis or to nocardiosis, pathologies often observed in this context of immunosuppression. The antibiotic treatment is difficult and should required two bactericidal antibiotics. A surgical lobectomy can be envisaged in case of relapse. The mortality is 30%.

[Overload histiocytosis in 2 hemodialyzed patients. Possible role of dextran 40]. / Histiocytose de surcharge chez deux hémodialysés. Rôle possible du dextran 40.

The authors report two cases of singular overloading histiocytosis, observed in patients treated with regular dialysis and undergoing feverish cachectic disease. The observations of the two patients show a same histiocytosis infiltration of ganglions, liver, muscles, lung, pleura and pericardium, consisting in an accumulation of PAS positive intracytoplasmic substance, refringent at polarisation, microcrystalline on electron microscopy and on ionic analysis. Clinical likeness appears with PVP thesaurismosis but the histochemical findings are different. A favorable issue occurred with corticosteroïd treatment. Nature of PAS positive substance is uncertain. An exogenous origin is undoubted, and some elements call for the responsibility of dextran 40.