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We report our local experience of two patients with type 1 primary hyperoxaluria (PH1) who received successful isolated cadaver kidney transplantation. The indication of isolated renal transplantation exists for PH1, but it must probably be restricted to the less severe forms of this disease. Recipient and donor selection is crucial.

Anti-CD3 monoclonal antibodies suppress immune responses by transient T-cell depletion and antigenic modulation of the CD3/T-cell receptor complex. Anti-CD3 treatment of adult nonobese diabetic (NOD) mice, a spontaneous model of T-cell-mediated autoimmune insulin-dependent diabetes mellitus, significantly inhibits the autoimmune process. Short-term low-dose anti-CD3 treatment (5 micrograms/day i.v. for 5 consecutive days) prevented the occurrence of an accelerated form of the disease induced by cyclophosphamide. More unexpectedly, when applied to adult NOD females within 7 days of the onset of full-blown diabetes, the same anti-CD3 regimen induced a complete remission of overt disease (i.e., a return to permanent normoglycemia) in 64-80% of mice. This remission was durable (> 4 months) and was not associated with the disappearance of insulitis (mononuclear cell infiltration of the islets). The immunosuppression was apparently specific for beta-cell-associated antigens, since mice showing anti-CD3-induced remission rejected histoincompatible skin grafts normally, whereas they did not destroy syngeneic islet grafts, unlike control untreated overtly diabetic NOD females. These results open major therapeutic perspectives. They strongly suggest that self-tolerance can be restored in adult mice once autoimmunity is fully established and confirm that this effect can be obtained by transient targeting of the CD3/T-cell receptor without massive T-cell debulking.

The accuracy of color Doppler US (CDUS) for the detection of main renal transplant artery stenoses is excellent (Se 92%; SP 99%). Whereas the sensitivity of CDUS is still unsatisfactory for the detection of arterial branch stenosis (Se 70%), it has become higher than previously reported because of the ability of color flow images to identify hemodynamic changes at the site of stenosis and focal downstream repercussions in the case of tight stenosis. Color Doppler US is a valuable method in the detection of segmental infarction or large areas of cortical necrosis. However, small superficial cortical perfusion defects are usually undetectable by color Doppler. Contrast-enhanced MRI appears to be more accurate than color Doppler US; it is useful in confirming the diagnosis of infarction and detecting small infarcts missed by color Doppler US, and provides an accurate evaluation of the extent of the infarct; Spectral features (reflux during the whole diastole) obtained from renal arteries in case of acute renal vein thrombosis are suggestive but not specific since they can be observed in the case of severe acute rejection with cortical necrosis. Such findings associated with a lack of venous Doppler signals in the whole kidney are highly suggestive of renal transplant vein thrombosis. Post-biopsy arteriovenous fistulas and false arterial aneurysms are accurately detected by color Doppler Imaging. Color Doppler appears to be the primary Imaging modality for early detection of renal allograft vascular complications.(ABSTRACT TRUNCATED AT 250 WORDS)

Between 1968 and 1991, 36 pregnancies were reported in 28 patients who had undergone kidney transplantation at the Necker hospital, Paris. Pregnancies with favourable graft outcome were compared with those accompanied by significant deterioration of graft function and requiring haemolysis. The risk factors for renal function deterioration during pregnancy in kidney-transplanted women were found to be an initial nephropathy induced or aggravated by a previous pregnancy, an alteration, even mild, of graft function, hypertension, even controlled by treatment, an HLA mismatch and several episodes of acute graft rejection soon after kidney transplantation.

Anti-CD3 monoclonal antibodies are potent immunosuppressants widely used in clinical transplantation to prevent or treat acute allograft rejection. We have used a hamster monoclonal antibody (145 2C11) specific for the epsilon chain of the murine CD3 complex to treat autoimmune insulin-dependent diabetes appearing spontaneously by 15 to 30 weeks of age in female Non Obese Diabetic (NOD) mice. Mice showing overt disease (glycosuria and glycemia > or = 4 g/l) were randomized in two groups receiving either anti-CD3 (5 micrograms/day i.v. for 5 consecutive days) or an identical dose of hamster polyclonal immunoglobulins. Progressive remission of disease was observed, 3 to 6 weeks after the end of treatment, in 80% of anti-CD3 treated mice as compared to 6% of mice in the control group. This remission was maintained long term namely, during the 4 to 5 months (after the end of treatment) observation period. These results open interesting perspectives on the possibility to treat recently diagnosed diabetic patients with therapy showing long term efficacy and no chronic toxicity.

A 24-year-old white woman with a past history of recurrent venous thromboses of the lower extremities was admitted for hypertension and renal failure. She had a chronic cutaneous ulcer on the anterior side of the left leg and oral ulcers of the palatum. Laboratory tests demonstrated rapidly progressive renal failure and the presence of an anticardiolipin antibody (ELISA). Thrombosis of the inferior vena cava was shown by phlebocavography. Renal biopsy revealed typical thrombotic microangiopathy. Tissue-type plasminogen activator (tPA) was visualized by immunofluorescence in endothelial cells of renal arterioles and glomeruli. Normal plasma levels of tPA, urokinase and plasminogen activator inhibitor 1 were found by ELISA, and tPA antigen levels rose after desmopressin acetate infusion. Thus, in this case, the diffuse thrombotic process was not related to defective circulating or renal fibrinolytic systems and could be promoted by the procoagulant effect of antiphospholipid antibodies.

The X-ray scanning symptoms of dysthyroidal ophtalmopathy are so typical that they are enough to make the diagnosis in most of cases. Their determination through the FEMEN system increases the sensitivity of the method. The investigation of 60 cases of oculomotor palsies with a FEMEN score greater than 10 confirms that they are certainly or probably subsequent to an endocrinopathy in 93% ot the cases.