Biomedical subjects
Publications and source records attributed to A Meyrier.
A case of diffuse primary interstitial lung fibrosis associated with familial benign hypercalcaemia is reported. This, and the 3 cases previously published, suggests that both diseases share a common genetic mode of transmission.
PURPOSE: Atheroma as a cause of renal failure has been largely overlooked. We wanted to report our experience with atheromatous renal disease over a 12-year period. PATIENTS AND METHODS: Observations on 32 cases of various forms of renal failure in patients with atheromatous renal disease are presented. These patients had been hypertensive for an average of 10.2 +/- 9.2 years. The length of deterioration was an average of 17 months, and at presentation renal insufficiency was severe, with serum creatinine levels of (mean +/- SD) 616 +/- 358 mumol/liter (6.8 +/- 4.0 mg/dl). At this stage, the clinical picture was indistinguishable from other common causes of chronic renal failure in the elderly. Thus, the precise diagnosis would have been overlooked without an aggressive diagnostic workup. All patients underwent angiography and six patients underwent renal biopsy. RESULTS: In 22 cases, renal insufficiency was mainly due to atheromatous stenosis of renal arteries. In six of six patients, the results of renal biopsy showed cholesterol crystal embolism. In four additional cases, there was clinical or histologic evidence of extrarenal cholesterol embolism. In eight, renal artery plaques coexisted with cholesterol embolism. In two patients, renal failure was due only to cholesterol embolism. Renal atheromatous stenoses were developing, as shown on serial angiographies performed in five cases. In seven cases, stenoses involved both the main trunks of renal arteries and several intrarenal branches of too small a diameter to allow reconstructive surgery or percutaneous transluminal angioplasty. In addition, the general condition of most patients was so poor as to preclude surgery. Dialysis was begun in 11 patients, four other patients died, and renal failure was managed conservatively in 11. When undertaken, reconstructive surgery was successful in five of six patients. CONCLUSIONS: Atheromatous renal disease is a frequent and easily overlooked cause of chronic renal insufficiency. It is not only due to renal artery stenosis but also to complex intrarenal lesions, with multiple stenoses of intrarenal vasculature and cholesterol embolism. It should be diagnosed by early angiography and renal biopsy, before the stage of multivisceral complications and at a time when surgery can still be undertaken.
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Severe hypertension developed in 4 patients who had sustained blunt renal trauma 2-32 years previously. A protracted period of normal blood pressure separated the renal injury from the onset of hypertension. Angiography disclosed lesions which had been underestimated by intravenous pyelography. These lesions consisted of traumatic dissection of branches of the renal artery, leading to renal infarct. In 3 cases, unilateral high renin secretion was demonstrated, and in 1 case this was confirmed by immunofluorescence with an anti-renin antiserum which showed abundant renin granules within the infarcted zone. In all patients, total or polar nephrectomy was followed by return of blood pressure to normal or near-normal levels. Blunt renal trauma is an underestimated cause of renovascular hypertension.
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We undertook a review of the literature concerning idiopathic NS in the adult (with MGL or due to FSGS) in an effort to assess the results obtained with corticosteroid and/or conventional immunosuppressive treatments. We found few usable published works and we were left with an impression of great imprecision. This imprecision stems both from frequently deficient definition of glomerular lesions and from different evaluations of responses to treatment. The almost total absence of controlled series, due to the rarity of nephrosis in the adult, hinders any firm conclusions with regard to the effect of treatments. It limits evaluation to mere tendencies, formulated in such vague terms as "complete remission," "partial remission," and "treatment failure," in a disease whose natural history is sometimes so whimsical that the same clinical case, over a period of years, can be both a success and a failure of the same treatment. In the adult, there is a significant difference in the incidence of complete remissions obtained by corticosteroids according to whether the NS is due to MGL or to FSGS. Nevertheless, the published data are difficult to interpret because dosage and length of treatment vary among authors. A high percentage of corticoresistant, corticodependent, or multirelapsing nephroses responds to immunosuppressive treatment. Efficacy has been documented for three drugs. Chlorambucil is easy to use but entails some long-term oncogenic hazards. Cyclophosphamide has more short-term side effects, but its long-term risk of malignancy appears less. Both of these alkylating agents may definitively compromise fertility. Azathioprine, easy to use but prescribed surprisingly little in NS, may have considerable advantages over alkylating drugs, which would justify reconsidering its use in nephrosis. An open, cooperative study was launched to determine the efficacy and the tolerance of cyclosporine in 45 patients who had been considered as failures of corticosteroid and/or conventional immunosuppressive therapy. The preliminary results of this trial showed that two thirds of the patients with cases of MGL and half of those with FSGS had total or partial remission after long-term treatment with cyclosporine. It is not yet known whether this may eventually lead to lasting remission after treatment is progressively tapered to a stop, or only represent reappearance of low-dose corticosensitivity. In case of dependence on this new immunosuppressive drug, long-term tolerance remains to be determined.(ABSTRACT TRUNCATED AT 400 WORDS)
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The "syndrome of inappropriate calcitriol secretion" may be observed in diseases with disseminated granulomas. The main examples are sarcoidosis and tuberculosis, but it can also be observed in fungal infections, in granulomas due to foreign bodies and in lymphomas. The syndrome is due to autonomous production of 1 alpha hydroxylase by granulomas. The insuing synthesis of calcitriol escapes normal regulation by serum calcium and phosphate levels. The syndrome includes hypercalcemia, hypercalciuria, high 1,25(OH)2D3 serum levels and reduced PTH secretion. It can supervene in anephric or hypoparathyroid patients. The notion that calcitriol may be secreted extrarenally is new. It could have important bearings on several issues in nephrology, immunology and oncology.
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Nephrotic syndrome in minimal change lipoid nephrosis and focal segmental glomerulosclerosis may be due to alteration of glomerular anionic sites by a lymphokine. Six adults with nephrotic syndrome who were resistant to treatment with corticosteroids and immunosuppressants were treated with cyclosporin A. In three patients with minimal change lipoid nephrosis who had been nephrotic for 3.5 to 23 years proteinuria resolved within 12 to 42 days. Subsequently, these patients became dependent on cyclosporin A. In three patients with focal segmental glomerulosclerosis who had been nephrotic for four to six years mean (SD) 24 hour urinary protein decreased from 14.7 (8.4) g to 3.6 (0.6) g within 20 to 25 days, serum albumin concentration rose, and oedema subsided. One patient died of myocardial infarction when still in partial remission after 11 weeks' treatment. Two patients remained proteinuric despite continuing treatment with cyclosporin A, but control of sodium balance was easy and serum albumin concentrations remained higher than without cyclosporin A. In all patients renal function improved during treatment. These preliminary results show that cyclosporin A may be effective in the treatment of patients with nephrotic syndrome that resists every other form of treatment and especially in the treatment of those with minimal change lipoid nephrosis. The results are in keeping with a T lymphocyte mediated mechanism of minimal change lipoid nephrosis and focal segmental glomerulosclerosis, but they also suggest that minimal change lipoid nephrosis and focal segmental glomerulosclerosis are separate entities.
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The authors studied clinical and biological data occurring in 165 patients observed during 23 years and afflicted with polyarteritis nodosa. Hypertension was present in 52 patients (31.5%) and seven of them suffered from malignant hypertension (4%). Mean age of patients (6 male, 1 female), with malignant hypertension was 38 +/- years old. Mean follow up was 49 +/- 28 months including 26 +/- 21 months after discontinuation of treatment of polyarteritis nodosa. Malignant hypertension occurred during the first year of evolution of polyarteritis nodosa. Renal insufficiency was present in 5 of 7 patients. Proteinuria was greater than 1 gr/d in 4 cases. Renal arteriography was performed in 6 patients and showed in every case renal ischemia and microaneurysms in five. In 4 patients measurements of plasma renin activity and of aldosterone were obtained. A stimulation of those hormones was demonstrated. Some symptoms of polyarteritis nodosa were present with a high incidence in case of malignant hypertension: digestive signs (6/7), orchitis (3/6). HBs antigen was present in 6 cases and hepatitis in 5. Captopril was effective in every case, alone or associated with other treatments. Follow up of hypertension went from 8 months to 4 years. At present time 6 patients are alive and one is lost of follow up. A treatment is necessary in 6 of 7 patients. Creatininemia is greater than 300 micromol/l in 4 patients. A successful kidney transplantation was performed in one case. Our study shows a close relation between malignant hypertension observed in polyarteritis nodosa, vascular nephropathy, digestive and urologic signs. Hepatitis B virus could be responsible of those manifestations.
The case of a 71 year old woman with an idiopathic hypereosinophilic syndrome is reported. Bidimensional ultrasonography was in favor of endomyocardial involvement. This case was original as nephrotic syndrome developed, due to membranous glomerulopathy which was complicated with renal vein thrombosis. Both eosinophilia and nephrotic syndrome greatly improved with corticosteroid treatment. Nonetheless, the patient died suddenly after 2 years, and this demise is in keeping with the overall poor prognosis of idiopathic eosinophilic syndrome.
Sarcoidosis is frequently attended by hypercalciuria and sometimes by hypercalcaemia. The type of hypercalciuria and its relationships with disease extension and activity have rarely been investigated. In order to clarify these issues we undertook an investigation by a calcium absorption test in 39 patients with untreated thoracic sarcoidosis, and tried to establish correlations with vitamin D3 metabolism and some features of the disease. We found three types of responses. Group I (n = 12) with a normal test had normal 1,25-(OH)2D3 and rare extrathoracic localisations. Group II (n = 14) with absorptive hypercalciuria had higher serum calcium; 1,25-(OH)2D3 (p less than 0.001) and the free 1,25-(OH)2D3 index (p less than 0.05) were raised. Sarcoidosis was more often inflammatory, developing and disseminated. Group III (n = 13) had resorptive hypercalciuria, and hypercalcaemia was frequent. 1,25-(OH)2D3 (p less than 0.01) and the free 1,25-(OH)2D3 index (p less than 0.05) were raised but to the same degree as in Group II. Sarcoidosis was more disseminated and developing than in Groups I and II. In the 39 patients, iPTH and nephrogenous cAMP were low. Post-calcium load urinary calcium/creatinine (Ca/Cr) and 1,25-(OH)2D3 were correlated (p less than 0.05). Extrathoracic extension was associated with higher fasting urinary Ca/Cr (p less than 0.001), and development with higher post-load urinary Ca/Cr (p less than 0.001). Thus, absorptive hypercalciuria is related to the development of sarcoidosis and can be explained by high free 1,25-(OH)2D3, while resorptive hypercalciuria seems to be linked with disease extension. In such a case, the mechanism of osteolysis is not solely accounted for by high 1,25(OH)2 vitamin D3 serum levels, and we postulate that some other factor is at work, related to the extent of the granulomatous process.
A 59 year-old male developed the nephrotic syndrome in the course of squamous-cell lung carcinoma with metastases. Renal biopsy disclosed amyloid deposits. Immunofluorescence was positive with an anti-SAA antiserum. Amyloidosis complicates exceptionally lung tumors, despite extremely high serum levels of SAA in this type of malignancy. This infrequency shows that determinants other than high SAA serum levels are necessary to create tissue amyloid deposits.
Two patients, one with ulcerative colitis, the other with Crohn's disease, developed glomerulonephritis due to IgA mesangial deposits. Following symptomatic treatment of the intestinal disease, haematuria subsided in both patients while a second renal biopsy showed disappearance of the mesangial proliferation and IgA deposits. It is suggested that the intestinal and the renal diseases may be linked by abnormal secretion of IgA by the intestinal mucosa with formation of immune complexes, subsequently trapped in the glomerular mesangium.
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