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We report the treatment of 5 patients with crescentic glomerulonephritis by immunoadsorption using a protein A column. Two had systemic vasculitis, 2 antiglomerular basement membrane disease and 1 systemic lupus erythematosus (SLE). In the patients with systemic vasculitis and SLE, there was successful removal of autoantibodies and rapid control of disease; remission was maintained over a mean follow-up of 2 years. Clinical improvement was not seen in 2 patients with anti-glomerular basement membrane (GBM) disease who presented with dialysis-dependent renal failure. There were no apparent clinical side-effects related to the immunoadsorption procedure. Protein A immunoadsorption offers a semiselective alternative to plasma exchange and appears to be safe and efficient in removing pathogenic autoantibodies in crescentic glomerulonephritis without anti-GBM antibodies.

Angiotensin-converting enzyme inhibitors (CEI) have been found effective in numerous cases of arterial hypertension, even non renovascular, and they are now widely used. However, the reference compound, captopril, has exhibited various side-effects, including acute renal failure. Different mechanisms may be responsible for this complication. In some case haemodynamic changes occur which are rapidly reversible after treatment is discontinued. In other, less frequent cases, captopril induces an immunoallergic interstitial nephritis, sometimes accompanied by skin rashes and/or eosinophilia. Since renal biopsies have been performed in only a few cases of CEI-induced nephropathy, we thought that it might be of interest to report a case of acute renal failure due to acute interstitial nephritis with epithelioid granulomas in the interstitium. The favourable outcome was confirmed by two successive follow-up renal biopsies.

The regiment of the Zouaves pontificaux was formed by young Catholic volunteers, chiefly French, Dutch and Belgian, who enrolled for the defence of the Pontifical State from 1861 to 1870 against the scheme of the United Italy planned by King Victor-Emmanuel II and Garibaldi. For ten years fights were scarce. They only took place in 1867 principally in Mentana and in September 1870 when the papal state was invaded and Rome taken. At first the military medical service in the regiment as in the whole army was rather inadequate except for the central hospital. The ambulances system was based largely upon benevolent people. But the organization improved in 1870. On the whole amongst more than 9,000 enlisted volunteers, 476 died (5%). The causes of death were more often diseases (78%) than injuries. As in all the armies of that time, cholera, smallpox, typhoid fever made a lot of victims. Malaria which still reigned in the Latium was responsible of a large morbidity.

The authors report a retrospective series of 217 cases of autosomal dominant renal polycystic disease collected over a period of 30 years in the urology and nephrology departments of Nantes university hospital. They study the incidence of urological complications, observed in 87 patients (40%), consisting of calculi (15%), infection (22%, with 4 deaths), intracystic haemorrhages (3.5%) and urinary tract compression (2%). The diagnostic and therapeutic methods are presented and discussed. The results of renal transplantation are also analysed: 39 patients were transplanted, 72% retained a functioning kidney with a mean follow-up of 44.9 months (range: 12-108 months) and three patients died as a result of infectious complications. The 1-year and 3-year actuarial transplant survival rate of 92% was similar to that of renal transplantations performed for another form of renal disease. Preparation for renal transplantation remains an essential problem: the two major indications for pre-transplantation nephrectomy were the size of the kidneys and the presence of infection.

Angiotensin-converting enzyme inhibitors (CEI) have been found effective in numerous cases of arterial hypertension, even non renovascular, and they are now widely used. However, the reference compound, captopril, has exhibited various side-effects, including acute renal failure. Different mechanisms may be responsible for this complication. In some case haemodynamic changes occur which are rapidly reversible after treatment is discontinued. In other, less frequent cases, captopril induces an immunoallergic interstitial nephritis, sometimes accompanied by skin rashes and/or eosinophilia. Since renal biopsies have been performed in only a few cases of CEI-induced nephropathy, we thought that it might be of interest to report a case of acute renal failure due to acute interstitial nephritis with epithelioid granulomas in the interstitium. The favourable outcome was confirmed by two successive follow-up renal biopsies.

We report a case in which lesions were limited to muscle of the neck, simulating a meningeal syndrome, with large calcifications visible on radiography, 99 Tc phosphate scanning, and CT scanning

34 cadaveric donor grafts were randomized in a blind study of the effect of pretreatment of 5 g each of methylprednisolone and cyclophosphamide on kidney graft outcome. There was no difference in overall survival or functioning after 3, 6 or 12 months between grafts from pretreated (33 kidneys) or control (29 kidneys) cadaveric donors. In addition, this pretreatment protocol did not modify the recipient immune response against B-lymphocyte alloantigens which developed in unsuccessful transplants. Our data, thus, neither confirm the high rate of kidney graft survival attributed to cadaveric donor treatment nor the supposition that treatment is effective in suppressing recipient antidonor B-lymphocyte antibodies.

The authors present the first case of cardiac arrest, occurring at the time of vessel clamps removal, of a kidney transplantation cooled prior to grafting with Collin's solution. Monitoring of cardiac (K) has demonstrated the role of Collin's solution (K = 141 mEq/l) in the genesis of this incident. The mean +/- SD increase of the K concentration in the right atrium within the seconds following the removal of the graft vessels clamps was 2.6 +/- 1.5 mEq. Acute electrocardiographic abnormalities primarily represented by sudden apparition of hyperkalemic T waves and extrasystols occurs in more than 50% of the cases. The effects of progressive blood release in the graft and other preventive manipulations are studied and discussed.

Peripheral blood lymphocytes (PBL) of patients with Hodgkin's disease were studied for their capacity to produce interleukin 2 upon in vitro phytohemaglutinin stimulation in the presence or absence of either interleukin 1 or indomethacin (2 micrograms/ml); eight patients were studied at the discovery of their disease before receiving any therapy (onset HD; OHD). Seventeen patients were tested in long-term (greater than 3 yr) remission (remission HD; RHD); most RHD were treated with both chemotherapy and irradiation. Fourteen healthy individuals served as controls. PBL from OHD have a significant (P less than 0.01) defect in the production of PHA-induced IL-2. Indomethacin and IL-1 had no effect on IL-2 yield. PBL from RHD yield intermediate levels of IL-2, which are nevertheless significantly lower (P less than 0.02) than control values. RHD recover the capacity of normal PBL to increase their production of IL-2 in indomethacin-supplemented culture medium. Interestingly, PHA responsiveness was significantly decreased only in RHD, thus not explaining the low IL-2 yield obtained in supernatants. In addition, 4-day PHA-blasts from both HD patients and control individuals increase their thymidine incorporation in the presence of purified lectin-free IL-2 to a similar degree, suggesting that their IL-2 receptors are unimpaired. Finally, OHD sera significantly inhibit PHA-induced IL-2 yield of normal PBL, suggesting that a seric component(s) may play a role in some cases. We conclude that defective IL-2 production may play a role in the well-documented deficient cellular immunity seen in Hodgkin's disease.

Resume Les auteurs utilisent l'intervalle de temps qui separe les deux reponses musculaires du Reflexe de Hoffmann pour apprecier la valeur conductrice des fibres nerveuses impliquees dans le trajet reflexe. Ils definissent un Index H (≠ 100 chez le sujet normal) qui relie cet intervalle de temps a la taille du sujet. Cette methode permet une surveillance iterative, indolore, objective et chiffree des neuropathies peripheriques. Les auteurs presentent les resultats obtenus depuis 2 ans chez 52 insuffisants renaux chroniques traites par hemodialyse au long cours.

The authors studied the MLC blocking effect of anti-DRW anti-sera. These sera were harvested from multiparous women and their anti-DRW activities were defined (after exhaustive platelets absorption) on a panel of 73 B-lymphocytes. All the six tested sera were strongly inhibitor until dilution greater than 1/500 when they were present in the MLC for all the culture period but without specificity for their relevant target cell. The MLC inhibitory activity was still found in IgG fraction. A significant specific inhibition could be found only when the cultured cells were independently preincubated with the corresponding anti-DRW serum prior culture. In this last case inhibition occured when the target cell (bearing the DRW antigen recognized by the studied sera) was used as the stimulator cell in MLC. A decrease of the inhibition was found when the anti-DRW was absorbed with its relevant target cell but also with non-target cell, as assessed by B-cell cytotoxicity, suggesting that non cytotoxic antibodies directed against "public" determinant are involved in MLC inhibition.

Intratubular deposits of calcium oxalate crystals can be responsible for acute renal failure. The present report concerns two cases for which none of the known causes of oxalate nephropathy were found. Both patients had common features: chronic alcoholism and denutrition. Except for early lumbar and abdominal pain, the renal failure picture was without any peculiarity. Renal biopsy showed tubular epithelium alterations with marked luminal deposition of birefringent crystals consistent with calcium oxalate. In one patient serum oxalate level was high, and in the other urinary oxalate excretion rose above normal when diuresis resumed. Renal function recovered spontaneously (follow-up of four years for one patient). Neither intoxication nor intestinal disease could be detected. Given the key role of pyridoxine in oxalate metabolism, we suggest that vitamin B6 deficiency secondary to alcoholism and denutrition could cause a rise in oxalemia leading to oxalate nephropathy. Experiments in animals support this hypothesis.

Hyperparathyroidism developed in 8 of 76 patients two months to 4 years after functional Kidney transplants, the most constant manifestation being a hypercalcemia. Raised PTH levels were present in all 7 patients in whom they were measured. The 8 patients were treated by total parathyroidectomy, followed in 7 cases by an autograft of a parathyroid fragment into the muscle off one forearm, according to the technique described by wells. Blood calcium levels became normal in all cases after one month. PTH levels also returned to normal except in one case where rejection of the Kidney graft caused a reduction in renal function. Comparing PTH levels in the venous blood on the side of the autograft with that on the opposite side demonstrated that the parathyroid graft was actively secreting.

The authors report a case of relapsing polychondritis, in a 41 year-old man, with renal complication. Immunofluorescence study of this segmental necrotizing glomerulonephritis showed an important fibrin fixation without immunoglobulins. Selective renal arteriography, preliminary to the biopsy, showed lobular arterial aneurysms. Because of other exceptional cases of literature, relation between periarteritis nodosa and relapsing polychondritis is discussed.

In view of their beneficial effect on the functional survival of transplanted kidneys, pretransplantation blood transfusions of haemodialysed patients have become routine procedure. At the same time, the number of hyperimmune patients awaiting transplantation has been increasing, as these are often excluded on account of positive cross-match. Cross-matching is usually done by testing recipients' sera against total donor's lymphocytes, so that positive cross-match due to specific anti-T cell cytotoxic antibodies cannot be differentiated from that due to anti-B cell antibodies. Differential anti-T cell and anti-B cell cross-matching showed no significant difference between the survival of 34 transplants with B cell-positive cross-match and that of 126 transplants with B cell-negative cross-match (67% and 60% respectively at 12 months). It also showed that anti-B cell antibodies had no adverse effect on the survival of transplants. The presence of antibodies or auto-antibodies with optimal cytotoxicity at 4 degrees C did not appear to correlate with a significantly longer transplant survival as compared to other transplanted patients. Finally, a high degree of a anti-T cell immunization or a strongly positive B cell cross-match on transplantation day did not seem to jeopardize transplant survival in a B-cell-positive cross-match population of transplanted patients. The differential cross-matching method has made it possible to successfully transplant a substantial number of hyperimmune patients: at least 62% of patients with B cell-positive cross-match also had positive total lymphocyte cross-match which would have precluded renal transplantation.

We describe six cases of kidney graft performed against a positive anti-B lymphocyte cross-match. These antibodies were found after retrospective and systematic screening of the effect of the recipient sera upon donor frozen B lymphocytes. As opposed to the consequences of anti-HLA "SD" preimmunisation, none of the transplantation rapported here failed by acute rejection. The benefic role of anti-B lymphocyte antibodies "Ia like" present at the time of grafting is then suggested.

This analysis involves 93 cases of transplants of non-related cadaver kidneys. The result was better (p = 0.02 at 1 year) in recipients transfused before the graft (73 cases) than in those who had not been trasfused (20 cases). However, this latter group had a shorter average period of haemodialysis (p = 0.05 than the first. The benefits of pre-immunisation appear from 1 to 2 transfusions or 1 pregnancy onwards (p = 0.02 at 1 year). Subjects transfused during the 6 months prior to the transplant had a graft which was functional more often than those who had been more than 6 months before the operation (p = 0.01 at 6 months). These results would be in favour of routine small quantity transfusions, approximately every six months in patients undergoing haemodialysis and on a waiting list for transplantation.

The association of nephronophthisis and tapeto-retional degeneration was described by both Senior and Loken in 1961, but prior to 1974 only 28 cases had been published. This report describes 8 new cases in 27 members of 5 families. The severe juvenile type produces blindness in infancy and death from renal failure before the age of ten. The adult type is characterized by later onset, slower progression of the renal disease and milder ocular manifestations. The eye disease may be congenital amaurosis of Leber type, pigmentary retinal degeneration or retinitis punctata albescens and the electroretinogram (ERG) is of value in the diagnosis of these varieties of hereditary tapeto-retinal degeneration. Renal involvement is often asymptomatic. Defective urinary concentration leading to polyuria and polydipsia is the earliest sign. Proteinuria is inconstant and urinary sediment is often normal. Two patients had aminoaciduria. The disease progresses inexorably to chronic renal failure. One patient has been successfully transplanted and two others are on chronic hemodialysis. Renal histological changes are those of nephronophthisis with tubulointerstitial lesions and multiple cysts. Senior-Loken syndrome appears to be transmitted by a single autosomal recessive pleotropic gene of variable expression. Degeneration of neuroepithelium and renal tubular epithelium, both tissues of ectodermal origin, may represent a genetically determined enzyme abnormality.

In view of the theory of autoregulation of cell multiplication in a growth organ, we attempted to inhibit renal tubular cell hyperplasia in the remaining kidney 48 hours after controlateral nephrectomy. To do this we drew up a method of measurement of hyperplasia by measuring the rate of uptake of tritiated thymidine in the organ undergoing compensatory growth. We then injected renal cytosol. Whereas the rate of uptake of tritiated thymidine doubles in the growing kidney compared with control animals, the intravenous administration of renal cytosol reduces the renal uptake of the tracer to its basal level. We correlated this observation with the microscopic count of mitotic figures, and noted the existence of parallel fluctuations. Our results seem to support the initial working hypothesis but they do not exclude the possibility of a more complex regulatory mechanism in which serum stimulant factors may also play a role.

In order to demonstrate in the uninephrectomised rat possible circulating serum factors which might induce an increase in the rate of synthesis of DNA in the remaining kidney, we injected intravenously to 125 g rats serum from similar rats uninephrectomised 24 to 48 hours previously. The injections preceded the administration of tritiated thymidine by 13 hours (1.5 ml), 5 hours (1 ml), or 2 hours (1 ml) depending on each group. The parameter studied was the radioactivity of the acid-insoluble fraction of 100 mg of kidney. We observed in uninephrectomised animals receiving no injection of serum that the rate of uptake of the tracer in the remaining kidney doubles 48 hours after nephrectomy but this uptake by the kidneys of unoperated rats is not modified by the injection of serum from similar rats whether uninephrectomised or not. Furthermore the addition of serum from uninephrectomised rats to slices of normal kidney under incubation does not modify the rate of synthesis of DNA. All these results are not in favour of a serum factor regulating compensatory renal hypertrophy.

Since June 1977, a systematic blood transfusion (BT) policy (160 ml of leukocyte-poor washed erythrocytes given every 6 months) has been applied to 126 hemodialyzed patients awaiting a first kidney graft. Only patients who had anti-T or B lymphocyte (T or BLY) antibodies (Ab) killing fewer than 10 or 20% of the panel cells, respectively, entered the protocol. Screening of anti-T and BLY was performed 8, 15, and 21 days after each BT. Patients were removed from the protocol if they developed Ab against more than 10 or 20% of the T or BLY panel cells. The cumulative immunization (all Ab types) averaged 90% after four BTs. Anti-BLY (63%) were more frequent than anti-TLY (49%) after for BTs. No anti-HLA-DR specificity could be attributed to the anti-BLY, whereas 20% of the anti-TLY displayed a particular anti-HLA-A,B specificity. Patients that had had BTs or pregnancies before entering the protocol had a higher degree of immunization. The kinetics of the anti-B or TLY pattern differed greatly both at the level of their detection after 8, 15, and 21 days following one BT and in their development after repeated BTs. Forty-three patients received transplants at various stages of the protocol. Recipients grafted without Ab had the best graft outcome (87 versus 66 actuarial percentage at 3 months), even though their HLA (A,B and DR) matching was inferior. There was no significant difference in recipients who had different subgroups of Ab. These data indicate that immunization is very high even after a few BTs when careful controls are performed and they suggest that BTs do not act via active enhancement.

Three immunosuppressive regimens have been compared: conventional treatment including anti-thymocyte globulin (ATG) (32 patients) with cyclosporine (Cys) alone (21 patients), sequential combination of ATG with Cys (35 patients). Actuarial graft survivals were: ATG 73 per cent, Cys alone 88 per cent at nine months and ATG/Cys 92 per cent at one to two years. Transplant function was significantly worse with Cys as initial treatment compared with that in controls, while it was similar with ATG/Cys. The Cys dose used was low and no severe infection nor immunoglobulin abnormalities were noticed. Corticosteroids were withdrawn with both Cys protocols, except for rejection treatment.

Interstitial Nephritis (IN) with or without epithelioid granulomas is the most frequent form of renal impairment in sarcoidosis. Nevertheless, few studies have provided conclusions about its treatment and long-term outcome. We report 22 cases collected over a 20-year period in 9 nephrologic departments of the west of France. We discuss the criteria which permit sarcoidosis to be distinguished from other causes, particularly of IN drugs. Twenty patients were treated with corticoids. In 18 the result could be evaluated on at least 12 months. In 11 there was a decrease of plasma creatinine (Pc) and in 8 of these cases this decrease reached 50%. Of the 12 who were followed from 1 to 17 years, 4 obtained a reduction of Pc of at least 50%. These improvements occurred even in patients whose Pc was initially higher than 300 mumol/l. Only 2 patients reached end-stage renal failure and hemodialysis within the observation period, one as early as the first month, the other after 6 years. Five relapses were observed after stopping treatment but 4 responded again favorably upon resumption. Two patients who received no treatment at all nevertheless had stabilization or improvement of their renal function with a follow-up of 5 years for one case. It may be concluded that the long-term prognosis of sarcoidosis IN can be fairly good but there is no proof that it depends mainly on corticosteroid therapy.

We report a case of partial lipodystrophy (P.L.D.) of the cephalothoracic type associated with low C3 serum level. In spite of the absence of renal symptom a renal biopsy was performed which demonstrated dense deposits disease characterized by the lack of cellular proliferation and highly segmentary deposits similar to those observed in remission after renal transplantation for these disease. A review of the literature shows the frequent occurrence of this pathological association. In general P.L.D. and complement abnormalities are thought to precede nephropathy. However chronological relationship between the former and the later must be interpreted cautiously as our case shows that dense deposits disease can be associated with P.L.D. in the lack of clinical evidence of renal disorder.

In a series of 76 functional renal transplantations we noted 8 cases of hyperparthyroidism occurring in periods ranging from 2 months to 4 years. Hypercalcaemia was the most constant manifestation. PTH was raised in the 7 subjects in whom it was measured. Eight patients underwent total parathyroidectomy followed in 7 of them by auto-graft of a fragment of parathyroid into a forearm muscle according to the technique of Wells. The operation was followed in all cases by normalisation in blood calcium levels after the 1st month. Serum PTH levels also returned to normal apart from in one case where renal function was impaired because of rejection of the renal graft. Comparative estimations of PTH in venous blood from the side of the auto-graft and the opposite side showed that the parathyroid graft was functioning.

In order to evaluate the prognosis of IgA nephropathy semi-quantitative analysis of tubulo-interstitial and vascular alterations was performed. Three groups of patients are described. In the first group the prognosis has remained favourable until the present time. The prognosis is variable in the second group. In the last group the prognosis is always unfavourable. However, the limited duration of the study may elicit certain reservations concerning the long-term prognosis for the first and second groups.