[IgM anti-MAG neuropathy with involvement of the cranial nerves disclosing B-cell lymphoma].
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Biomedical subjects
Publications and source records attributed to E Diot.
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Diagnosis and treatment of lower limbs arterial disease do not depend exclusively on functional staging. Fontaine's classification has to be replaced by a classification based on clinical and complementary investigations to understand better the evolutive risks, and guide management. Distal systolic pressures along with symptom analysis and clinical examination allow a more precise diagnosis. Complementary investigations aim at assessing, the localization and extension of atherosclerotic lesions; the severity of tissue ischaemia. The concept of critical ischaemia has tried to insure greater diagnostic homogeneity for patients with rest ischaemia. The role of complementary investigation can only increase for the diagnosis and management sclerotic arterial disease. The detection of the other localizations of atherosclerosis and arteriosclerosis are essential to prevent complications which are responsible for the prognosis of the disease. Sclerotic arterial diseases represent approximately 90 to 95% of the causes of arterial diseases. Identification of these other causes remains, however, essential as it can greatly influence prognosis.
The authors report the case of a 40 year-old man with POEMS syndrome (peripheral neuropathy, organomegaly, endocrinopathy, M-protein and skin changes). Pathological studies of the peroneal nerve showed a microangiopathy of the vasa nervorum. Several authors have already reported microvascular abnormalities that could be responsible for some systemic manifestations of the POEMS syndrome, involving the skin, the kidney and the peripheral nerves. This new case enhances this hypothesis. The relation between microangiopathy and plasma cell dyscrasia is unknown.
Candida parapsilosis is a very common and saprophytic fungus. Its pathogenic role has been reported in intensive care units after invasive instrumentations. We report a case of dermal candidiasis due to Candida parapsilosis occurring in an immunocompromised patient suffering from aplastic anemia treated with corticosteroids. The histopathological aspect was non specific, showing a polymorphous granulomatous reaction in the middermis. Findings of periodic acid-Schiff and Grocott-Gomori stains revealed a large number of pseudohyphae and spores. Candida parasilosis was cultured from a skin biopsy. The route of skin infection is discussed: direct inoculation or septic localization after fungemia.
High resolution computed tomography (HRCT) was recently demonstrated to be as good as open lung biopsy for the diagnosis of pulmonary involvement in patients with scleroderma. Nevertheless, in view of its price and related irradiation, HRCT cannot be recommended as a screening test. Serum III procollagen (sPIIINP) is an aminopropeptide of type III collagen, which is released during conversion into collagen by specific proteases. Increased levels of sPIIINP have been observed in patients with scleroderma. The aim of the present study was to assess the relationship between sPIIINP measurement and pulmonary involvement defined according to HRCT and pulmonary function tests (PFT) with single-breath carbon monoxide transfer capacity (TL,CO) in 28 patients suffering from scleroderma. Patients were divided into two groups for analysis, Group A comprising 16 patients without pulmonary scleroderma and Group B comprising 12 patients with pulmonary scleroderma. All patients had stable cutaneous disease and normal renal and hepatic function. The level of sPIIINP was determined by radioimmunoassay (RIA-gnost P-III-P, Prod. Nr. ODMT; Behring, Marburg, Germany). Mean +/- SD sPIIINP level in Group A was 0.85 +/- 0.21 U.mL-1. Individual values ranged 0.6-1.3 U.mL-1. Mean +/- SD sPIIINP value was 1.30 +/- 0.40 U.mL-1 in Group B and individual values ranged 0.7-1.9 U.mL-1. The difference in mean sPIIINP level between Group A and Group B was significant. Using a cut-off at 1.1 U.mL-1, sensitivity of sPIIINP was 0.66, specificity 0.94, positive predictive value 0.89, negative predictive value 0.79, false positive rate 0.06, and false negative rate 0.33. The value of sPIIINP correlated with HRCT score but not with PFT. This study confirms the relationship between sPIIINP and scleroderma with interstitial lung disease. We suggest that sPIIINP could be measured in patients with scleroderma to screen those patients requiring HRCT. Further studies are necessary to determine the value of sPIIINP in terms of prognosis and follow-up of patients under treatment.
BACKGROUND: J001X is an acylated poly-galactoside isolated from the membrane of Klebsiella and able to interact with macrophages, mainly in their activated state. The aim of the present study was to determine the ability of 99m-labelled technetium (99mTc)-J001X scintigraphy to image pulmonary disease, defined by high resolution computed tomographic scanning and pulmonary function tests, in patients suffering from scleroderma. METHODS: Patients were considered to have pulmonary disease when they had at least two positive signs on high resolution computed tomography, or a decrease in lung volume and single breath carbon monoxide transfer, or both, with no disease process other than scleroderma in their medical history. Positive J001X scintigraphic imaging was defined by symmetrical bilateral pulmonary fixation three and five hours after inhalation of 99mTc-J001X. J001X scintigraphic results were compared with disease activity as indicated by bronchoalveolar lavage (BAL) fluid lymphocytosis. RESULTS: Seventeen patients were studied, in 12 of whom J001X scintigraphy was positive. There was no correlation between BAL lymphocytosis and J001X scintigraphic findings, nor between BAL and pulmonary scleroderma. This was not surprising because of the high specificity of macrophage targeting by J001X. CONCLUSIONS: Follow up of a larger population over a longer period is needed to establish whether there is a prognostic value for positive J001X scintigraphic findings in scleroderma.
Non-neoplastic pancreatic disorders may cause pleuro-pulmonary signs. In acute pancreatitis respiratory disorders are very frequent and may cause simply an hypoxia or the adult respiratory distress syndrome (SDRA). There is no single mechanism: mechanical hypoventilation, alteration of the vessel walls and interstitial oedema, stasis of leucocytes, alterations in surfactant and intravascular coagulation may all contribute. These disturbances may be made by direct action of enzymes or by the intermediary of the activation of the kinine system, of complement activation and of coagulation mechanisms. In chronic pancreatitis the problem is to diagnose the cause of the pleural effusion which may often appear to be primary. The cause is suggested by a raised pleural amylase, type P. Although neoplastic pleural effusions may sometimes be rich in amylase it is of type S. The diagnosis rests on echos, computerised scans and retrograde cholangeopancreatography. The pathophysiology of these effusions is through the passage on the enzymes by the lymphatic route, by the oesophageal and aortic route or by a pancreatico-pleural fistula.
Raynaud's phenomenon is a common, paroxysmal acrosyndrome, affecting about 4% of the general population. Most of the time it is a purely functional condition: Raynaud's disease. However, in rare cases, there is an organic cause and it is then known as Raynaud's phenomenon. In view of its prevalence, a simple, atraumatic, low cost and reliable work-up should be performed to guide diagnosis. This is based on the findings of clinical examination, capillaroscopy, antinuclear factor antibody tests and X ray of chest and hands. Only a few cases will require more specialised investigation with arteriography and autoimmune studies. The commonest causes are arterial and connective tissue diseases, especially scleroderma. However, occupational hazards, toxic and iatrogenic drug-induced aetiologies should not be overlooked. With the exception of patients who have a curable underlying disease, most cases require vasodilator therapy, the keystone of which over the last decade has been the calcium channel blockers. Only patients who are severely handicapped by their acrosyndrome should be given drug therapy.
We report the case of a 72 year old woman treated for seven months for Horton's disease presenting with a cavitating pneumonia of the left axillary segment associated with a deterioration of the general state, at the time of which the steroid therapy was in the course of being decreased. The radiological appearance and the clinical context of immune depression had led to a trial of a quadruple anti-tuberculous therapy in spite of the absence of any bacteriological proof. This rapid aggravation of the condition under treatment led to the suggestion of a pulmonary localisation of Horton's disease. The rapid regression of the lesions after increasing the dose of steroids and the negative cultures on Lowenstein medium pleaded in favour of this hypothesis. However the continuation of anti-tuberculous treatment seems justified in such cases in so far as the diagnosis of Horton's disease of the lung often cannot be maintained other than retrospectively in the absence of histological proof.
Isotopic techniques enable to determine the best conditions to aerosolize pentamidine as a preventive treatment of pneumocystis. An original labelling technique of pentamidine isethionate has led into a feasibility study of pentamidine scintigraphy in the monkey, and then to a clinical study. A tracer dose of 2 mg of pentamidine isethionate was diluted into 200 mg of pentamidine mesylate. An ultrasonic nebulizer (TV 6000) and a jet nebulizer (Venticis II) were first compared in four baboons. With the two nebulisers the curves of pulmonary kinetic activity showed a peak 10-15 minutes after the beginning of inhalation and then a plateau. With the ultrasonic TV6000 the pulmonary dose was on average 0.8 mg or 0.4% of the pentamidine aerosolized and the gastric contamination 3.1%. With the Venticis II compressed air apparatus the amount of pentamidine delivered to the deep lung was a mean of 4.2 mg or 2.1% of the aerosolized dose whereas the gastric contamination was only 0.5%. The delivery of the aerosol by the ultrasonic inhaler TV 6000 was then compared with two compressed air inhalers Venticis II and Respirgard II in four patients infected with HIV virus who were submitted to a preventative therapy against pneumocystis. With the ultrasonic TV 6000 the pulmonary dose was 0.8 mg or 0.3%-0.5% of the dose of pentamidine aerosolized and the digestive contamination was 3.1%. The performance of the two compressed air nebulizers Venticis II and Respirgard II were similar. The amount of pentamidine delivered to the deep lung was respectively 12 and 14 mg or 6 and 8% of the dose of pentamidine aerosolized and the digestive activity was 0.2%.(ABSTRACT TRUNCATED AT 250 WORDS)
Bromocriptine, a derivative of ergot of rye, is used in high doses for the treatment of Parkinson's disease and may be responsible for pleuro-pulmonary complications. We report a case of a 72 year old patient treated for 2 years for Parkinson's disease with a daily dose of 30 mgms. of bromocriptine and in whom a pleural effusion, initially unilateral then bilateral, was discovered with a deterioration in the general physical state. The effusion was a transudate and examination did not give any clues as to aetiology, there was a rapidly favourable outcome following the cessation of treatment. Amongst the respiratory complications of bromocriptine it is the pleuro-pulmonary fibrosis which is most frequently reported. The cases of isolated pleural effusions are rare and are generally exudates. Several hypotheses have been put forward to explain the pathogenesis of these disorders: there is a theory that the molecule induces a toxic fibrogenesis, there is a vascular theory analogous with the secondary effects of methysergide and finally immunological theory. The frequency of the disorder is probably underestimated and these drug reactions justify a close follow up for any respiratory problems in patients who are subjected to treatment with bromocriptine.
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We have studied 12 reports of pneumonitis associated with nilutamide (Anandron) and notified to the French regional ADR monitoring centers between November 1987 and June 1990. The mean age of the patients was 71.5 +/- 9.5 years, and 35% (5/12) had a history of lung disease. All 12 patients developed dyspnea, cough and fever, 4.7 +/- 6 months after starting nilutamide. Ten patients (83%) required admission to hospital. The symptoms revolved in 11 cases when the drug was withdrawn. In the other case, they disappeared when another drug was stopped and the dose of nilutamide was reduced. Six patients were treated with glucocorticoids. The severity of nilutamide-associated pneumonitis appears to be related to the time between the onset of dyspnea and consultation.
The authors report the case of a 65-year-old man who had an evolving case of Schnitzler's syndrome with lytic bone lesions which was unresponsive to several drugs, and which dramatically improved with small quantities of corticosteroids. Schnitzler's syndrome, first described in 1974, is defined by chronic non-pruritic urticaria, osteocondensation, and monoclonal IgM dysproteinemia without features of lymphoproliferative disease, and is associated with fever, deterioration of the general health and biological signs of inflammation. Only 22 cases of Schnitzler's syndrome have been reported hitherto and there is no known effective treatment, H1 and H2 blockers, dapsone, colchicine, chloroquine, cyclophosphamide, chlorambucil and azathioprine having proven useless in the few patients treated. Corticosteroids may be effective, however, especially against the urticaria.
The physiopathology of Graves' ophthalmopathy is poorly understood. Therefore, the best treatment of Graves' ophthalmopathy is still a matter of debate. Steroids, X-ray therapy, surgery and more recently cyclosporine, plasma exchange and somatostatine analogs have been tried. There is no doubt that we are dealing with an autoimmune process. The use of immunoglobulins has been shown to be safe and efficient.