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Biomedical subjects

A De Muret

Publications and source records attributed to A De Muret.

At least 19 recordsLinked to original sources
PubMed1998

[Abdominal emergencies in type IV ehlers-Danlos syndrome].

Ehlers-Danlos syndrome denotes a group of inherited connective tissue diseases comprising nine types. Type IV Ehlers-Danlos syndrome is the most life-threatening form. It is characterized by a type III collagen deficiency resulting in arterial fragility and death from vascular rupture or bowel perforation. This disease involves a col 3A1 gene mutation. We report the case of a 44 year-old woman with type IV Ehlers-Danlos syndrome. The medical history of our patient included bowel necrosis and two vascular ruptures. We indicate data required to establish Ehlers-Danlos syndrome diagnosis and guidelines for patient management.

Adult↗
PubMed1998

[Incidental discovery of a giant myxoma].

The authors report a case of very large myxoma of the left atrium, original in terms of its mode of discovery, its very calcified appearance, in a totally asymptomatic elderly patient. A complete ultrasound assessment, catheterization, and coronary angiography allowed assessment of the morphology, topography, blood supply and cardiac repercussions of this tumour. Myxoma of the left auricle is the commonest cardiac tumour, with multiple clinical features and presentations. This benign tumour arises from embryonic vestigial remains, usually in the fossa ovale. The present case is original by its unusual mode of discovery in a totally asymptomatic patient.

Angiocardiography↗
PubMed1997

[Mycobacterium chelonae cutaneous infection: efficacy of prolonged treatment by clarithromycin].

INTRODUCTION: Mycobacterium chelonae is an ubiquitous mycobacterium which is exceptionally pathogenic. It can cause localized skin infections which are difficult to treat because of multiple drug-resistance. CASE REPORT: A 87-year-old woman with bullous pemphigoid was treated with long-term prednisone and developed a skin infection due to M. Chelonae. Treatment with clarithromycin was successful, but relapse occurred 6 months after withdrawal of the antibiotic. DISCUSSION: Clarithromycin is effective in single drug regimen for M. Chelonae infection, but the duration of treatment should be long when immunosuppression cannot be avoided.

Aged↗
PubMed1995

Primary cutaneous medium and large cell lymphomas other than mycosis fungoides. An immunohistological and follow-up study on 54 cases. French Study Group for Cutaneous Lymphomas.

Primary cutaneous medium and large cell lymphomas (MLCL) other than mycosis fungoides (MF) are rare, and their prognosis and treatment are controversial. The clinical, immunohistological and follow-up data of 54 well-documented cases of primary cutaneous MLCL other than MF, seen in our institutions over a 14-year period, were retrospectively reviewed, in order to determine the prognostic factors related to these lymphomas, and to analyse the results obtained with different treatment regimens. Forty-six patients presented with a solitary tumour or with localized lesions, and eight had disseminated cutaneous lesions. According to the updated Kiel classification, 45 cases (83%) corresponded to B-cell lymphomas: centroblastic lymphomas, 32 cases; centroblastic-centrocytic lymphomas, 11 cases; immunoblastic lymphomas, two cases. Nine cases (17%) were classified as T-cell lymphomas: pleomorphic medium and large cell lymphomas, eight cases; anaplastic large cell lymphoma, one case. Four of eight patients with disseminated skin lesions had a T-cell lymphoma, whereas 41 of 46 patients with a solitary tumour had a B-cell lymphoma. Patients with disseminated skin lesions and elevated serum lactate dehydrogenase (LDH) levels had a poor prognosis. Comparison of patients' overall survival, depending on immunohistological subtype, showed that the median survival of patients with pleomorphic T-cell lymphoma was 2.5 years, whereas it was not reached at 12 years for patients with centroblastic-centrocytic and centroblastic lymphoma. The eight patients with disseminated skin lesions were treated with polychemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1995

[Pulmonary fibroma].

The authors report the case of a pulmonary fibroma in a 66 year-old smoker. Intrapulmonary localization of solitary fibrous tumors is rare; these tumors are more frequently described in pleura. Histologically, the presence of spindle cells without nuclear atypy on a collagen background must evoke the diagnosis made difficult by the unusual localization.

Aged↗
PubMed1995

[Spinal cord compression due to thoracic spinal chondroma. A study of 2 case and a review of the literature].

We report two cases of thoracic spine chondroma in two girls aged 14 and 12 years. The lesion was revealed by signs of spinal cord compression and affected T1 and T7 respectively. Chondroma is a benign bone tumour, fairly frequent in the extremities but very rare in the spine. It accounts for 2.8% of benign bone tumours and 12% of all bone tumors. 3.6% to 4% of chondroma are located in the spine. Out of 51 cases reported in the literature 21 had been revealed by spinal cord compression, with an age range of 5 to 76 years. In all cases of spinal cord compression magnetic resonance imaging (MRI) is necessary as first-line examination, but CT scans are still useful in the diagnosis and post-operative follow-up of spinal chondroma because of the osteo-cartilaginous nature of these tumors. The purpose of this study was to report two new cases of spinal cord compression by thoracic spine chondroma and to review the literature.

Adolescent↗
PubMed1995

[Cutaneous candidiasis due to Candida parapsilosis occurring in the course of idiopathic aplastic anemia].

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.

Adrenal Cortex Hormones↗
PubMed1993

Localized atypical pemphigoid on lymphoedema following radiotherapy.

Autoimmune bullous diseases have been reported associated with different causal factors: drugs, mechanical trauma and physical trauma, particularly ultraviolet light and radiotherapy. In these cases different hypotheses regarding the pathogenesis of blister formation can be supported. In this observation, we report a localized cicatricial pemphigoid with unusual clinical presentation. Moreover, it appeared 9 years after radiotherapy for breast carcinoma and it was preferentially localized on an upper limb lymphoedema. Because of the long time between the treatment of carcinoma with radiotherapy and the onset of pemphigoid, we assume that lymphoedema played a major etiological role in this particular cicatricial pemphigoid.

Aged↗
PubMed1993

[Decrease of haptoglobin serum level in patients with chronic viral hepatitis C].

Serum levels of haptoglobin, an acute phase protein, in usually increased in patients with inflammation and decreased in patients with intravascular hemolysis. Changes in haptoglobin serum levels were also observed in patients with liver disease. The aim of this study was to evaluate the serum level of haptoglobin in patients with chronic viral hepatitis C. Haptoglobin serum levels were measured by rate nephelometry in 34 patients with histologically proven chronic viral hepatitis C and in 30 controls. Haptoglobin serum levels were lower in patients than in controls (0.56 +/- 0.36 versus 0.90 +/- 0.35 g/L, P < 0.001). In patients with chronic hepatitis but without cirrhosis, haptoglobin serum levels were decreased as well. Haptoglobin serum levels were positively correlated to prothrombin index (P < 0.001) and negatively correlated to the histology activity index of Knodell (P < 0.001), and especially to scores of fibrosis (P < 0.001) and periportal necrosis (P < 0.05). Red cell count, indirect bilirubin level, and reticulocyte count, as markers of hemolysis, were similar in patients and controls. We conclude that determination of haptoglobin serum levels may be useful in the evaluation and the follow-up of patients with chronic viral hepatitis C.

Adult↗
PubMed1992

A primary cutaneous multi-lobed B-cell lymphoma.

Multi-lobed non-Hodgkin's lymphoma (NHL) has recently been recognized as a NHL variant. A patient presented with a scalp nodule which, upon skull X-Ray, was seen to be associated with a bone defect. Immunophenotyping clearly demonstrated that this was a B-cell proliferation. Histologically the B-lymphocytes were closely related to centroblasts. There were no other extra cutaneous localizations. The present report emphasizes the importance of this clinico anatomical entity which shows prominent extra-nodal involvement, large lymphoid cells with multi-lobed nuclei and a good response to chemotherapy. Multi-lobed NHL may be a T-cell lymphoma, or a B-cell lymphoma closely related to centroblastic NHL. Although multi-lobed lymphomas have a predilection for cutaneous localizations, our case is the first primary cutaneous multi-lobed B-NHL, proven by immunophenotyping.

Female↗
PubMed1992

Schnitzler's syndrome (urticaria and macroglobulinemia) associated with pseudoxanthoma elasticum.

Schnitzler's syndrome, first described in 1974, is defined by chronic non-pruritic urticaria, osteocondentation, and a monoclonal IgM dysproteinemia, but without criteria of lymphoproliferative disease. We report a patient with chronic urticaria and macroglobulinemia. In addition, he had double monoconal dysproteinemia IgM kappa (31.3 g/l) and IgA lambda, osteocondensation, and some cutaneous lesions of pseudoxanthoma elasticum. Only 20 cases of Schnitzler's syndrome have been reported hitherto. This is the first case associated with pseudoxanthoma elasticum, which was localized and discovered at the same time as Schnitzler's syndrome. We discuss the possible role of monoclonal immunoglobulin in the occurrence of localized elastorrexhis.

Aged↗
PubMed1992

[Sclerosing intravascular bronchiolo-alveolar tumor].

We report the case of a 56 year old female who was admitted to hospital in 1980 for assessment of disseminated nodular pulmonary opacities. The histological examination of a prescalene node biopsy showed features which were compatible with sarcoidosis. No treatment was given. In 1987 a discrete increase in the pulmonary nodules was noted. In 1989 effort dyspnoea occurred and there were pains in the right lower chest and an evening fever. The chest X-ray showed a right sided pleurisy and an increase in the size of the nodules with mediastinal lymphadenopathy. A histological examination of a pulmonary nodule, which was taken at an open lung biopsy enabled the diagnosis of a broncho-alveolar sclerosing intravascular tumour to be established. This is a rare tumour which has a slow evolution occurring most often in middle aged females. In 50% of cases the discovery is fortuitous. Besides patients present with non-specific symptoms. The radiological examination reveals multiple nodular opacities which are diffuse and 0.5 to 1.5 cm in diameter. Currently these tumours do not benefit from any particular therapy.

Diagnosis, Differential↗
PubMed1990

Iododerma and acute respiratory distress with leucocytoclastic vasculitis following the intravenous injection of contrast medium.

A 72-year-old woman with chronic renal failure requiring haemodialysis developed acute iododerma twice, after receiving iodide contrast dye for radiological procedures. Iododerma was localized to the face, scalp and elbows and was associated with papular purpura of the legs. Histopathology of the skin lesions showed acute necrotizing vasculitis. During the second skin eruption the patient developed acute respiratory distress, which was treated with corticosteroids. During the first eruption asymptomatic infiltrates were present on chest X-ray which disappeared 2 months later. At the present time iododerma seems more frequent in patients with renal failure. Iodides may also be responsible for pulmonary abnormalities, which are sometimes asymptomatic. All these features may be due to leucocytoclastic vasculitis following iodide ingestion.

Acute Disease↗