[Anatomo-clinical conference. Pitié-Salpêtrière Hospital. Case No. 2-1992. Chronic pneumopathy in a woman with systemic lupus erythematosus and antiprothrombinase].
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Biomedical subjects
Publications and source records attributed to P Cacoub.
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We adapted an immunoblotting technique for the immunochemical characterization of cryoglobulins. We first compared the results from 157 samples with results obtained by immunofixation and immunoelectrophoresis and then with clinical observations in 125 cases. Full identification was possible in 98% of the cases by immunoblotting, in 54% by immunofixation, and in 28% by immunoelectrophoresis. Novel microheterogeneity aspects were observed by immunoblotting and immunofixation in 13% and 6% of the cases studied, respectively. Our results confirm the frequency of autoimmune and infectious diseases associated with mixed cryoglobulinemias, whereas a lymphoproliferative syndrome was observed only in cases of cryoglobulinemia with a monoclonal constituent or a microheterogeneity aspect, which was not always revealed by immunofixation or immunoelectrophoresis. The pathophysiological importance and classification of microheterogeneity aspects is not clear. These observations justify using a sensitive and specific method for identifying cryoglobulins, even when present at low concentrations, and call for long-term studies of these patients.
Twenty-seven cases of chronic constrictive pericarditis seen between 1975 and 1990 in an internal medicine department were analyzed retrospectively. The chronic pericarditis was consecutive to one (n = 5) or several (n = 7) episodes of acute pericarditis. Echography demonstrated the presence of pericardial effusion in 74 percent of the cases, pericardial thickening in 41 percent and/or compression of right heart cavities in 55 percent. Computerized tomography of the chest, performed in 16 cases, showed pericardial effusion in 63 percent of the cases, pericardial thickening in 37 percent and lymph node enlargement in 19 percent. Magnetic resonance imaging of the chest was carried out in 2 patients but showed no abnormality. All 11 patients who underwent cardiac catheterization were found to be adiastolic. The cause of constrictive pericarditis, elicited in 13 patients was neoplasia in 4, sequelae of radiotherapy in 2, injuries in 2, mediastinal and retroperitoneal fibrosis in 2, myocardial infarction in 1, purulent pericarditis in 1 and bacteriologically proven tuberculosis in 1. Medical treatment with corticosteroids (n = 16) and/or antituberculous therapy (n = 15) was successful in 2 patients; 25 patients had to undergo surgery 7 +/- 11 months after constriction was diagnosed. Pericardial drainage (through a pericardiopleural window in 4 cases) proved to be sufficient in 10/15 patients but failed in 5. Pericardectomy was performed initially in 3 cases and after failure of medical treatment and/or drainage in 11 cases. The 4 patients with neoplastic constrictive pericarditis died 10 months on average after the diagnosis, but the remaining 23 patients were alive after à 9 to 48 months (mean: 19 +/- 15) follow-up. These results suggest that the data provided by echocardiography and computerized tomography of the chest usually point to the relevant therapeutic measures without a need for invasive haemodynamic exploration. Idiopathic constrictive pericarditis now accounts for 50 percent of the cases; tuberculosis has become exceptional, but the other, previously exceptional causes (neoplasia, heart surgery, radiotherapy, connective tissue diseases) are more frequent. Corticosteroids should be used in chronic constrictive pericarditis occurring after cardiac surgery or in the course of a connective tissue disease, but they are effective only in highly inflammatory forms of the disease. Modern treatment relies on early surgery, since functional results and patient's survival are closely related to the date of pericardectomy which must be carried out before very important myocardial repercussions develop.
Folate and vitamin B12 are indispensable to normal cell division. High turnover tissues, therefore, are the first to be affected when these vitamins are deficient. Such deficiencies, which are known to result in megaloblastic anaemia at a late stage, may also influence the granulocyte and platelet lines, but pancytopenia rarely occurs. We report 3 cases of pancytopenia associated with folate and/or vitamin B12 deficiency in elderly patients. In two of these patients bone marrow examination showed a misleading "pseudo-leukaemia" due to a significant proportion of strongly dystrophic young cells. Cure was obtained within 30 to 45 days of vitamin therapy, with complete normalization of the haemogram.
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Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumor originating in the smooth muscle of the media. Although rare, it is the most common malignancy in the IVC. One hundred and six cases have been reported thus far in the world literature, usually as isolated case reports. Clinical, radiologic, and therapeutic management and follow-up, including 7 additional cases, have been reviewed and summarized. Clinical manifestations are dependent upon the location of the tumor. The main symptom was a palpable mass for a tumor in segment I, abdominal pain for segment II, the presence of Budd-Chiari syndrome for segment III. Segment II was the most frequent site of leiomyosarcoma of the IVC, alone (n = 41) or with other segments (n = 39). Before laparotomy, clinical recognition was difficult or impossible. Recently, however, newer imaging modalities including ultrasound and CT scan have permitted earlier diagnosis. Metastases, when diagnosed, were either present at diagnosis (n = 20) or appeared as the disease progressed (n = 18). Metastatic disease frequently involved the liver, lung, lymph nodes, or bone. The small number of patients alive without metastases (16/113) must be analyzed all the more carefully because these patients were followed for less than 2 years. When prolonged follow-up is possible, the number of patients alive without neoplastic disease is significantly reduced. We found the prognosis of patients with LMS of the IVC to be poor. Diagnosis was made at autopsy for 27 patients. Among the 86 patients with follow-up information, 59 died within a mean of 16 months, and 26 were alive 25 months after the diagnosis. The main prognostic factor is topography, particularly the highest level of extension of the tumor. The upper-segment tumors have the poorest prognosis. The best therapeutic management is difficult to recommend because most of the cases in the literature did not include a sufficient follow-up. Given the very small number of patients completely free of neoplastic disease after sufficient follow-up, it seems unlikely that leiomyosarcoma of the IVC can now be cured. Patients who received a combination of surgery, radiotherapy and chemotherapy remained free of disease for longer periods. The unanswered question is: what is the best timing for each of these treatments? We recommend diagnosis of leiomyosarcoma of the IVC through biopsy guided by ultrasonography or computed tomographic scan. Therapeutic management should include large doses of chemotherapy preoperatively with or without radiotherapy to reduce tumor size.(ABSTRACT TRUNCATED AT 250 WORDS)
The authors sought to evaluate renal tolerance for ioxaglate sodium meglumine used as a contrast agent in patients with chronic renal failure. Eight male patients (mean age, 55 years +/- 5) with chronic renal insufficiency (glomerular filtration rate less than 60 mL/min) who underwent diagnostic cardiac catheterization were enrolled. Renal clearance of inulin and rho-aminohippuric acid and urinary enzyme excretion were studied 1 day before and 1 day after administration of 167 mL +/- 43 of ioxaglate. None of the patients experienced any adverse reactions. All the patients had markedly depressed renal clearance values before angiography. Mean serum creatinine level, glomerular filtration rate, effective renal plasma flow, and urinary beta 2-microglobulin excretion were unaltered by angiography. After the procedure, only one patient had an increase in serum creatinine level of more than 10% (from 115 to 159 mumol/L [1.3 to 1.8 mg/dL]), with a decrease in glomerular filtration rate from 34 to 27 mL/min. In this patient, serum creatinine level and glomerular filtration rate normalized within 72 hours. Using accurate and sensitive renal function tests, the authors have shown that ioxaglate may be used safely in patients with chronic renal failure.
Cardiac localisation of hydatid cysts is rare representing 0.5 to 2% of all clinical forms of this condition. Hydatid cysts are usually observed in the left ventricle and involvement of the interventricular septum is exceptional. The principal diagnostic and preoperative investigations are serology and imaging by echocardiography and computed tomography, which can give conflicting results. The authors report a case of hydatid cyst of the interventricular septum in which computed tomography was non-contributory whereas echocardiography (the key investigation in all cases of hydatid cyst) associated with nuclear magnetic resonance imaging provided particularly accurate preoperative information. The latter investigation also allowed diagnosis of pulmonary, hepatic, splenic and renal involvement of the disease.
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Despite its poor specificity, CA 125 is a widely used tumoral marker. Significant rises in serum CA 125 levels are found mainly in ovarian cancer, but they also occur in other malignant or benign pathologies such as chronic liver diseases. However, in benign pathologies the CA 125 concentrations hitherto reported did not exceed 850 U/ml. The authors report two cases of chronic constrictive pericarditis with ascites and pleural effusions in which a very high increase of serum CA 125 levels (above 1,200 U/ml) was directly related to the presence of pleural effusions.
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These studies were designed to test the hypothesis that adenosine and calcium are important in mediating radiocontrast-media-associated reduction in renal blood flow (RBF) in the dog. Intravenous verapamil (V) and diltiazem (DTZ) infusion significantly attenuated the magnitude of the vasoconstrictor response observed after each intrarenal contrast media (CM) injection. (First injection: -47 +/- 8% control vs. -14 +/- 3% V, p less than 0.03; -38 +/- 4% control vs. -19 +/- 3% DTZ, p less than 0.02. Second injection: -33 +/- 6% control vs. -12 +/- 1% V, p less than 0.03; -32 +/- 5% control vs. -17 +/- 2% DTZ, p less than 0.03. Third injection: -32 +/- 6% control vs. -11 +/- 5% V, p less than 0.03; -38 +/- 5% control vs. -10 +/- 5% DTZ, p less than 0.02). Furthermore, V and DTZ almost completely abolished the increase in renal vascular resistance (RVR) induced by each CM administration. Theophylline also significantly attenuated the magnitude of the vasoconstrictor response observed after CM injection (first injection: -31 +/- 3% control vs. -12 +/- 3% theophylline, p less than 0.05; second injection: -26 +/- 3% control vs. -12 +/- 3% theophylline, p less than 0.03). Similarly, theophylline blunted the increase in RVR induced by CM injection. In addition, theophylline inhibited exogenous adenosine-induced decrease in RBF (-61 +/- 10% and -26 +/- 1% decrease in RBF without and with theophylline, respectively). In contrast, dipyridamole significantly enhanced the vasoconstriction induced by CM (first injection: 25 +/- 3% control vs. 49 +/- 4% dipyridamole; second injection: 31 +/- 3% control vs. 48 +/- 4% dipyridamole p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)
The purpose of the present study was to assess the plasma levels of atrial natriuretic peptide (ANP) in chronically uremic patients not submitted to dialysis and to determine the predialysis plasma concentration of ANP, the effect of ultrafiltration on plasma levels of ANP (hemodialysis, (HD), and the HD clearance of ANP in a population of adult patients treated with maintenance HD. The mean plasma ANP concentration (pg/ml) in HD was 370.2 +/- 35.5 pg/ml (mean +/- SEM) before HD and decreased to 165.3 +/- 15.2 after HD (p less than 0.01). Both values were significantly higher than in controls (28 +/- 2; n = 39). The changes in plasma ANP levels correlated inversely with those in plasma protein concentration (r = -0.53; p less than 0.03; y = 48.6 +/- 0.8 x). ANP clearance across the cuprophan membrane averaged 13 +/- 6.4 ml/mn. Resting plasma ANP values in the 16 uremic patients ranged between 16 and 277 pg/ml (124 +/- 11 pg/ml). These levels were significantly higher than those observed in controls (p less than 0.01). In these patients there was a highly significant correlation between serum creatinine and plasma ANP concentrations (p less than 0.01; r = 0.75; y = 0.2x + 3). Furthermore we report the results of the determination of the renal clearance of ANP in normal dogs. In all dogs a fall in plasma ANP concentration was recorded between the aorta (28.6 +/- 4.5 pg/ml) and the renal vein (14.2 +/- 2.7 pg/ml). The renal extraction ratio averaged 51.3 +/- 3.7%. Mean ANP renal clearance was 38.2 +/- 5.2 ml/mn.(ABSTRACT TRUNCATED AT 250 WORDS)
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Three cases of AIDS patients hospitalized for Pneumocystis carinii (PNC.C) pneumonia are reported. These cases are of interest because of the unusual radiological semiology of these pneumocytoses, i.e., their partially or totally pitted nodules. In addition, 4 related facts merit attention: the occurrence of pneumocytosis in 1 patient under prophylactic aerosol pentamidine therapy: the rarity, unusual in AIDS, of PNC.C in the bronchoalveolar lavage fluid from 2 patients; the association of pitted nodules with mediastinal adenopathies due to PNC.C in 1 patient; and the favorable outcome in all cases.
We have tested the effects of calcium channel blockade with diltiazem on methotrexate-induced nephrotoxicity in patients with biopsy-proven malignant disease. The patients were randomized in a cross-over fashion to receive MTX (4 g.m-2 body surface area) with or without Diltiazem (360 mg per day orally) during two consecutive periods separated by a 3-week interval. Methotrexate caused reversible acute renal failure, with an increase in serum creatinine from 89 (7)mumol.l-1 on day 0 to 150 (6)mumol.l-1 on Day 6. The patterns of beta 2-microglobulin and N-acetyl glucosaminidase urinary excretion were similar, with a sharp increase from Day 0 to Day 3. Urinary beta 2-microglobulin excretion increased from 161 (57) micrograms.l-1 on Day 0 to 1160 (840) micrograms.l-1 on Day 3 and fell to 918 (530) micrograms.l-1 on Day 10. Urinary N-acetyl glucosaminidase excretion increased from 250 (100) mmol.h-1 per mg of creatinine on Day 0 up to 655 (261) on Day 3 and fell to 285 (82) on Day 10. The evolution of renal function was not influenced by diltiazem. In patients receiving diltiazem, serum creatinine increased from 93 (0) mumol.l-1 on Day 0 to 151 (68) mumol.l-1 on Day 6 (p = NS when compared with control values). Urinary enzyme excretion also markedly increased from Day 0 to Day 3 to the same extent as in the group not receiving diltiazem. Our data indicate that acute deterioration in renal function caused by methotrexate is accompanied by tubular damage. Diltiazem was ineffective in preventing the acute renal failure induced by methotrexate.(ABSTRACT TRUNCATED AT 250 WORDS)
Disopyramide is a group I antiarrhythmic drug which is mainly used for the treatment of ventricular and supraventricular rhythm disturbances. Commonest side effects result from disopyramide's anticholinergic activity. Other side effects such as hypoglycemia have been reported less frequently. We report one observation of disopyramide induced hypoglycemia, and a review of the literature is presented. Including our observation, 14 cases (9 men and 5 women, aged from 41 to 88) have so far been reported. Doses of disopyramide ranged from 200 to 1,200 mg per day, administered from one day to one year. Symptomatology was mainly neurologic (12 patients) and two patients were clinically asymptomatic. The outcome was favorable in all but the 2 patients who died with persistent hypoglycemia after a single dose of 250 mg in one patient and after 400 mg daily during 4 days in the other (without stopping the drug). Renal function was markedly impaired in 9 patients, two of these patients being on a long term dialysis therapy. Blood levels of disopyramide were measured in 7 patients and ranged from 1 to 11.4 ng/ml. In five patients it was in the normal range (1-4 ng/ml). Three patients were rechallenged for disopyramide: hypoglycemia occurred in all, without clinical symptoms in two of them. The main risk factors of disopyramide induced hypoglycemia are a preexisting chronic renal failure, advanced age, and malnutrition. In these patients normally non toxic disopyramide blood levels, as defined in normal subjects, seem to be inappropriately high. We suggest that in patients at risk, disopyramide blood levels should be maintained at the lower range of therapeutic level.(ABSTRACT TRUNCATED AT 250 WORDS)