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Biomedical subjects

N Brousse

Publications and source records attributed to N Brousse.

At least 253 records · Page 14Linked to original sources

[Cardiac manifestations of the hypereosinophilia syndrome: a report on fifteen cases (author's transl)].

A retrospective study was conducted in fifteen patients with the hypereosinophilia syndrome to assess the cardiac manifestations of the affection. Clinical, electrical, and radiological findings, together with the results of ultrasonography (8 cases), hemodynamic tests (6 cases), and pathological examinations (7 cases) were used to classify the fifteen patients into four groups: 7 cases with endomyocardial fibrosis (EMF), 5 with non-obstructive cardiomyopathy (NOCM), 2 with pre-existing cardiopathies, and 1 with a normal heart. The manifestations in the 7 EMF cases varied according to the extent of the fibrosis present: 2 adiastolies, 3 valve incompetences, and 2 asymptomatic forms. Cardiac insufficiency can occur in the absence of fibrosis, pathological examination in one case demonstrating that it was due to a myocarditis, with infiltration of eosinophils and necrotic foci. The results of this study underline the close relationship between Loëffler's fibroplastic endocarditis and Davies' fibrous endomyocarditis. The presence of vascular lesions in the hypereosinophilia syndrome leads to difficulties in nosological distinction with some forms of necrotizing angitis especially Churg and Strauss' syndrome.

Adult↗

[Angioimmunoblastic lymphadenopathy and Prurigo strophulus (author's transl)].

Angio-immunoblastic lymphadenopathy (A.I.L.) was classified as a special type of malignant lymphoma. However, the same morphologic features can be observed in non-malignant diseases. We report a case of A.I.L. associated with Prurigo Strophulus: pseudo-tumoral adenopathy and cutaneous involvement disappeared with parasiticide treatment.

Aged↗

Cerebromeningeal localizations in acute myeloblastic leukemia of the adult. Clinical and pathological study of 15 cases.

68 consecutive adults were treated for acute myeloblastic leukaemia (A.M.L). Involvement of the central nervous system (CNS) developed in 15 cases. The clinical characteristics of nine of these patients were: symptoms of increased intra-cranial pressure in 5, cranial nerve lesions in 4, peripheral nerve lesions in 5, cord compression in 1, hemispherical symptoms in 7. Lumbar puncture vas performed systematically on seven patients, two of them with evidence of meningeal leukaemia (M.L.). Post-mortem examination was performed on seven patients who died during the first induction treatment: three had evidence of C.N.S. involvement. M.L. developed in three patients during complete hematologic remission (R.C.) In patients younger than 60 years old, the incidence of M.L. was 8/24 (approximately 33%). Medial survival time was reduced:8 months in cases with M.L., compared to 13.5 months. Cerebro-fluid protein levels were increased in six cases without blast cells: in four cases, M.L. occurred within a few months. The frequency of meningeal leukaemia in A.M.I. is under-estimated. Eradication of this frequent complication is difficult. Prophylactic meningeal treatment may be instituted for patients younger than 60, to reduce the incidence of M.L. and to improve the median remission time.

Adolescent↗

Congenital intraspinal lipomas: histological analysis of 234 cases and review of the literature.

The clinical, radiologic, and pathologic data from a series of 234 patients hospitalized in the Pediatric Neurosurgical Department of the Necker-Enfants Malades Hospital, Paris, for congenital intraspinal lipomas and operated on from 1976 to 1995 were examined. Histological studies showed that these lesions may be simple lipomas, similar to those developing elsewhere in the body, or they may be more complex forms including in addition to the lipomatous component a variety of unusual ectopic tissues of ectodermal, mesodermal, and/or endodermal origin. These complex forms indica te the malformative nature of these tumors. When they contain elements that are truly foreign to the region, the possibility of teratoma with a tumoral potential should be considered. Data found in the literature and from Necker-Enfants Malades Hospital are discussed.

Child↗

[Melanotic neurofibroma].

BACKGROUND: Melanotic neurofibromas are rare tumours. The clinical and histological diagnosis is often difficult to make. CASE REPORT: A 41 year-old woman with type-1 neurofibromatosis presented with an old, large (16 cm by 6 cm) pigmented tumour on her left arm. It was initially considered to be a congenital naevus. Partial surgical resection was performed. Histological examination showed a loose proliferation of spindle-cells within the dermis and subcutaneous layers, with multiple foci of melanin-laden cells but no mitotic figures or atypical cells. There was no melanocytic theca. The tumour had immunoreactivity for the S-100 protein, neuron-specific-enolase, neurofilaments, synaptophysin, A-103 and HMB-45. The association of a benign pigmented tumour producing melanin and the presence of Schwann cells and nervous cells, led to the diagnosis of diffuse melanotic neurofibroma. DISCUSSION: Melanotic neurofibromas can occur on their own or be associated with neurofibromatosis. They must be distinguished from classical neurofibromas when pigmentation occurs in the latter. Melanotic neurofibromas usually appear in the second or third decade of life and rarely in childhood. It is worth noting that hairs may overlie a melanotic neurofibroma, mimicking a giant naevus or a neurocristic cutaneous hamartoma. These are the two main differential diagnoses among children. Among adults, the main difficulty is to distinguish melanotic neurofibroma from pigmented dermatofibrosarcoma, because of the clinical and histological similarities between these two.

Adult↗

Intestinal transplantation in children: preliminary experience in Paris.

From November 1994 to November 1998, 20 children (2.5 to 14 years) received a jejunoileal graft alone (SBTx; n = 10) or in combination with the liver (SBLTx; n = 10 and/or the right colon (5 SBTx). Indications were intractable diarrhea of infancy (n = 8), short bowel syndrome (n = 6), extensive Hirschsprung disease (n = 4), and chronic intestinal pseudoobstruction (n = 2). Immunosuppression included tacrolimus, methylprednisolone, and azathioprine. Current follow-up ranges from 6 to 54 months. Five patients died (3 SBTx) within the first 2 months. Acute liver rejection occurred in 5 patients during the first 2 months. Sixteen episodes of intestinal rejection during the first 3 months in 11 patients (8 in 4 SBTx) were successfully treated in all but 3 by increasing tacrolimus dose and/or a 3-day methyprednisolone bolus or required antilymphoglobulins in 3 cases. Surgical complications occurred 8 times after SBLTx and 3 after SBTx. Infectious complications were more frequent in SBLTx recipients. Reversible Epstein-Barr virus-related posttransplant lymphoproliferative disease occurred in 3 recipients. Five presented cytomegalovirus infection. The SB graft was removed in 5 recipients (3 chronic rejection). All patients were started with oral and/or enteral feeding from the 7th postoperative day by using either normal food or protein hydrolysate diet. Currently, 10 of 11 children (8 SBLTx) achieved digestive autonomy after 5 to 30 weeks. All recipients gained weight; however, growth velocity remained reduced during the first 6 months because of the steroid therapy. Overall graft and patient survival is higher after SBLTx. Intestinal transplantation is indicated for patients with permanent intestinal failure. However, because parenteral nutrition is generally well tolerated, even for long periods, each indication for transplantation must be weighed carefully in terms of risk and quality of life.

Adolescent↗

[Eosinophilic myositis and Shulman syndrome (author's transl)].

Three cases with eosinophilic infiltration of fascia and muscle are described. In every case at least one relapse occurred. One patient exhibited clinically the definite features of eosinophilic fasciitis. About two cases a tentative diagnosis of Shulman's syndrome is discussed. In these patients, all known causes of eosinophilic myositis had been excluded.

Diagnosis, Differential↗

[Osteosclerotic myeloma. Report on three cases and review of the literature (author's transl)].

Three cases of osteosclerotic myeloma are reported. Clues for diagnosis are agarose gel electrophoresis and sclerotic bone biopsy. Review of 50 cases of literature points out frequency of peripheral neuropathy and Ig A type Immunoglobulin. Iliac crest biopsy shows thickening of bone structures and plasmocytosis in medullary spaces. Main X-Ray signs are mentioned and pathogenesis is discussed.

Aged↗

[Intrasplenic transplantation of hepatocytes in spf-ash mice with congenital ornithine transcarbamylase deficiency].

The only curative treatment for enzymatic deficiency such as Ornithine transcarbamylase (OTC) deficiency is liver transplantation. We have studied hepatocyte transplantation as alternative therapy in spfash mouse, the animal mode of OTC deficiency. Hepatocytes were obtained from C57BL/6J strain mice (normal OTC activity). About 2 x 10(6) hepatocytes in 0.1 ml were injected in the spleen of recipient mouse. We then studied: ammonemia, urinary orotate, spleen OTC activity, liver OTC activity, histological presence of hepatocytes in the spleen, immunohistochemical OTC staining in liver and spleen hepatocytes. In syngeneic hepatocyte transplantation (C57 > C57) functional transplanted hepatocytes were found in the spleen over 5 months after transplantation. In allogeneic transplantation (C57 > spfash) without immunosuppression, hepatocytes were rejected in 3 days. With cyclosporine immunosuppression, hepatocyte survived 12 days and showed OTC activity. A few transplanted hepatocytes with OTC activity migrated from spleen of liver. Metabolic disorder were not corrected. This technically easy method (compared to whole liver transplantation) demonstrated 2 problems: reject in allogeneic transplantation and the small amount of injected enzyme.

Animals↗