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Biomedical subjects

Y Pirson

Publications and source records attributed to Y Pirson.

At least 91 records · Page 5Linked to original sources

Outcome of thirty patients with Alport's syndrome after renal transplantation.

Graft antiglomerular basement membrane nephritis in patients with Alport's syndrome (AS) is a unique complication related to the glomerular basement membrane (GBM) abnormality characteristic of the disease. Its prevalence and clinical significance however remain unknown. We used strict criteria of AS to select 30 patients (26 men, 4 women), aged 17 to 44 years (m: 27) in whom 35 grafts (30 first, 5 second) had been performed at our center between 1968 and 1988. Patient and graft survival were, respectively, 96 and 75% at 5 years, 77 and 42% at 10 years. Graft survival and function, as well as the incidence of rejection episodes in the AS group were not different from those of a control group without AS, matched for age, sex, graft origin, and immunosuppressive regimen. Fifteen grafts were examined by immunofluorescence at least 3 months after TP: linear IgG deposits along GBM were present in 5 cases in the absence of signs of crescentic glomerulonephritis. Circulating anti-GBM antibodies detected in one of these cases 8 months post-TP had disappeared 24 months later. The presence of linear IgG did not seem to influence graft survival and function. We conclude: (1) the overall outcome of TP in AS patients does not differ from a control group without AS; (2) appearance of linear glomerular IgG is frequent but is not necessarily associated with a poor graft outcome; (3) the course of de novo graft anti-GBM disease may be benign; and (4) the aggressivity of the disease could be determined by the degree of immunosuppression and/or by the specificity of the anti-GBM antibodies.

Adult↗

Antiglomerular basement membrane nephritis induced by IgA1 antibodies.

A 62-year-old man was admitted for acute renal failure due to crescentic rapidly progressive glomerulonephritis. Kidney biopsy showed intense staining for IgA, IgA1 and C3 in a linear pattern along the glomerular basement membrane (GBM). Serologic tests for detecting anti-GBM antibodies were positive for IgA class and IgA1 subclass and negative for IgG and IgA2. Serum IgA anti-GBM antibodies appeared essentially in the polymeric form, an observation in agreement with recent studies demonstrating polymeric IgA response after parenteral immunization. There was no sign of pulmonary involvement. This patient is the first to present with an isolated nephritis due to anti-GBM antibodies restricted to the IgA class (IgA1 subclass). Routine tests for circulating anti-GBM antibodies should also include the IgA class.

Acute Kidney Injury↗

Myocardial revascularization in patients on renal replacement therapy.

Coronary disease is the main cause of mortality and morbidity among long-term survivors on renal replacement therapy (RRT). Despite the additional risk factors, myocardial revascularization has been recently attempted with various success in some patients on RRT. We report on 26 patients (13 dialyzed and 13 transplanted, mean age: 50 years [range 38-66]) who have undergone either surgical aorto-coronary bypass (CABG) (n = 16) with mammary artery grafts, or percutaneous coronary angioplasty (PTCA) (n = 9), or both procedures (n = 2). Indication was angina pectoris in all but three patients with painless ischemia. Eight patients had unstable angina (NYHA class IV). A previous myocardial infarction was documented in 11 cases. Coronary angiography disclosed mainly multiple vessel disease (81%). Post CABG complications consisted of severe intrathoracic bleeding (n = 3) resulting in death in 2 cases. PTCA entailed no major complication. After the critical postoperative period, the long-term survival was the same as that of non-uremic patients and the clinical improvement, according to the NYHA classification, was highly satisfactory at 6 months and persists up to 2 years. We conclude that coronary angiography and myocardial revascularization should be considered in patients on long-term RRT developing coronary disease.

Adult↗

Unrelated living donor kidney transplantation.

Since 1966, we have performed 41 renal transplants from unrelated living donors (ULD), 39 of which were "emotionally related". All donor-recipient pairs included in the present series were AB0-compatible. Recipients included 37 with primary and 4 with secondary transplants; 2 of the latter were diabetics. We compared these results to those of 41 recipients of cadaver donor kidneys matched for age, sex, immunosuppressive regimen, rank, and year of transplant, focusing our attention on the subgroups of patients under cyclosporin A (CyA) therapy (n = 24). We found that ULD transplantation was as successful as cadaver transplantation with good HLA matching: at 3 years, graft survival rates were 81% in ULD versus 86% in the control group under CyA. Moreover, grafts from ULD functioned more rapidly (no post-transplant dialysis and 70% of the patients with serum creatinine below 2 mg/dl within 3 days post-transplant). Graft tolerance was equivalent in both groups (50% of the patients experienced no rejection). We conclude that despite poor HLA matching, ULD transplantation with CyA as the basic immunosuppressive agent offers good results: benefiting from the quality of living donor kidney grafts, it helps to alleviate the persistent shortage of cadaver donors.

Adult↗

Outcome of patients with tuberous sclerosis after renal transplantation.

The fate of tuberous sclerosis (TS) patients after renal transplantation (RT) for end-stage renal failure remains to be defined. We report three patients with a posttransplantation follow-up averaging 54 months and review 6 previously published cases. Three women, aged 27-46 years, received a cadaver kidney 26-67 months after starting dialysis. None had mental retardation, 2 had suffered from seizures during infancy and 2 had intracranial calcification; neurological involvement was equally mild in the 6 reported patients. Currently, 16-84 months after RT, our 3 patients are fully rehabilitated with a well-functioning graft (serum creatinine 1.2-1.7 mg/dl). Results of RT are also satisfactory in the 4 other reported cases for whom a follow-up is available, except for 1 death unrelated to the initial disease. Neurologic disorders did not progress. Renal cell carcinoma was discovered in one removed kidney, and cells suggestive of malignant transformation in another case. No metastases were discovered up to 4 years later. No neoplastic transformation was observed up to 7 years after RT in the 3 patients who retained their native kidneys. TS patients with end-stage renal failure are good candidates for RT. The probably small risk of neoplastic transformation of native kidneys warrants a close monitoring by CT scan of the few patients who have not undergone bilateral nephrectomy.

Adult↗

The "low TBG syndrome" in patients on chronic hemodialysis: evolution after renal transplantation.

Patients on chronic hemodialysis may have a spuriously low TBG level, tentatively ascribed to uremia-induced changes in TBG immunoreactivity. In order to test this hypothesis, the effect of restoration of a normal renal function on TBG immunoreactivity was evaluated. TBG concentration was measured in 20 patients both by radioimmunoassay and by a T4-binding capacity assay, before, 4 and 10 months after a successful renal transplantation. Mean pretransplant TBG value measured by RIA was in the low normal range (1.60 +/- 0.14 mg/dl; normal limits 1.6-2.4 mg/dl). Twelve patients (60%) had TBG levels below 1.6 mg/dl (group I), while T4-binding capacity of TBG (TBC) measured in 7 of them was normal, so that the TBG/TBC ratio was characteristically reduced. The 8 remaining patients (group II) had TBG levels within or slightly above the normal range (mean value 2.26 +/- 0.17 mg/dl) and in the 5 tested patients, TBC and TBG/TBC ratio were normal. Four months after transplantation, mean TBG increased significantly in group I from 1.15 +/- 0.03 mg/dl before transplantation to 1.54 +/- 0.12 mg/dl (p less than 0.01). TBC increased also from 269 +/- 37.5 to 335 +/- 24 nmol/l (p less than 0.01) but to a significantly lesser extent than TBG (24% vs 48%, p less than 0.05). As a result, the TBG/TBC ratio returned to normal values, rising from 0.79 +/- 0.07 to 0.95 +/- 0.07 (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Treatment of renal graft artery stenosis. Comparison between surgical bypass and percutaneous transluminal angioplasty.

In order to compare saphenous bypass (SB) and percutaneous transluminal angioplasty (PTA) as treatment of renal graft artery stenosis (GAS), we have reviewed the results of both procedures in 33 patients treated consecutively by either SB (n = 16) or PTA (n = 17). All patients had become hypertensive within the first year after transplantation despite triple hypotensive drug therapy. SB was performed 17 (range 3-55) and PTA 19 (range 2-96) months after transplantation. SB failed in only 1 patient as a result of vascular thrombosis with graft loss. PTA was technically unsuccessful in 3 patients and was complicated by vascular branch thrombosis in 1 patient. Blood pressure decrease was similar in both groups: from 179/114 before SB to 147/90 (n = 15, P less than .001) at 6 months and 150/93 (n = 14, P less than .005) at 12 months after SB and from 177/110 before PTA to 149/93 (n = 13, P less than .01) at 6 months and 150/95 (n = 10, P less than .02) at 12 months. At 1 year, control of BP was improved in 85% of SB group patients and 74% of PTA group patients. Recurrent stenosis was documented in 3 PTA group patients: subsequently 1 had a successful SB and the 2 others a repeated PTA--successful in 1, unsuccessful in the other. We conclude that both methods are equally effective for BP control but that PTA entails a higher rate of initial failure and a significant rate of restenosis. However, because of technical ease and better tolerance, PTA emerges as the first-choice treatment of GAS, SB remaining indicated when PTA is not feasible or has failed.

Adult↗

Evidence that atrial natriuretic factor is the humoral factor by which volume loading or mannitol infusion produces an improved renal function after acute ischemia. An experimental study in dogs.

This experimental study in dogs was designed to investigate whether maximal loading produces atrial natriuretic factor (ANF) release and whether this physiological peptide is involved in the improvement of the early renal function recovery after acute ischemia. The experimental protocol included a renal artery occlusion for 45 min in uninephrectomized dogs and the measurement of various parameters of renal function over 2-hr period after declamping. There were 3 experimental groups. In the control group (I) (n = 10), the dogs received, after ischemia, an isotonic saline solution infusion at a rate of 0.2 ml/kg/min. In group II, (n = 10) the animals underwent acute volemic expansion (1 ml/kg/min) with whole blood (hematocrit approximately equal to 25%) during the ischemic period, and after declamping, an isotonic saline infusion (NaCl 0.9%) infusion at the same rate as in the control group. In group III, (n = 8) the dogs only received NaCl 0.9% (0.2 ml/kg/min) before ischemia and alpha human ANF (3.6 ng/kg/min) dissolved in saline after ischemia and during the 2 hr of the renal recovery period. Volemic expansion induced a highly significant increase of the cardiac filling pressures concomitant with a prompt but transient 5-6-fold increase in ANF levels (357 +/- 92 pg/ml versus 60 +/- 4.1 pg/ml in controls at the time of declamping [P less than 0.05]). With these higher plasma ANF levels in overloaded animals, we observed, 2 hr after declamping, considerably improved renal function recovery in terms of glomerular filtration rate--37.5% +/- 8.7 versus 11.8 +/- 3.9%; urinary sodium excretion rate--53.89 mu eq/min versus 5.36 +/- 1.2 mu eq/min (P less than 0.01); total Na reabsorption rate--1.2 +/- 0.23 meq/min versus 0.28 +/- 0.09 meq/min (P less than 0.01) (group II vs. controls, respectively). A 1-28 alpha ANF infusion after the ischemic insult allowed a comparable but more significant improved recovery of renal function--indeed, 2 hr after declamping, the GFR reached 73.7 +/- 14% of the preoperative GFR values. The urinary sodium excretion rate was 15-fold higher than in controls, and the total and fractional sodium reabsorption rates followed a similar increase. These beneficial effects of ANF were obtained with low doses of synthetic ANF (3.6 ng/kg/min) inducing plasma levels slightly higher (120 pg/ml) than in controls and comparable to the levels reached in the overloading group. In addition, maximal loading or ANF infusion produces an inhibition of the aldosterone rise occurring after the ischemic insult.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Effect of renal transplantation on the radiological signs of dialysis amyloid osteoarthropathy.

We have assessed the fate of amyloid bone cysts after a successful renal transplantation in 5 patients who had been on dialysis for 7 to 15 years. The size of 34 cysts, typical of amyloid osteoarthropathy, was monitored on annual X-rays for 29 to 75 (mean 47) months after TP. In sharp contrast to their progression during dialysis, cysts did not increase in size and no new cysts appeared during follow-up. However, despite a successful graft, none of the 34 cysts regressed significantly. The stability of the bone cysts may reflect either the poor solubility of the beta 2-m amyloid deposits or the limited accessibility of bone deposits to lytic factors. A longer follow-up will perhaps be necessary to document a significant regression of the cysts. Strikingly, transplantation resulted within a few days in an almost complete relief of articular complaints; in the absence of evidence of amyloid regression, this probably reflects the anti-inflammatory effect of steroids.

Adult↗