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Biomedical subjects

Y de Prost

Publications and source records attributed to Y de Prost.

At least 91 records · Page 5Linked to original sources
PubMed1987

[Digestive involvement in dystrophic recessive epidermolysis bullosa. Apropos of 6 cases and review of the literature].

Six personal cases of digestive tract involvement in dystrophic recessive epidermolysis bullosa are reported, and the relevant literature is reviewed. The study deals with the clinical aspects of these cases (buccal and dental lesions, digestive symptoms, effects on nutritional status; table I), as well as with their biochemical (table II), radiological and endoscopic aspects (table III, fig. 1 and 2). All patients presented with bucco-dental lesions, including two cases of congenital abnormalities: one with malposition and dysgenesis of the teeth, the other with dysplasia of the enamel in a patient whose dystrophic skin disease was proven by electron microscopic study. The oesophagus was involved in six cases, with tight concentric stenosis (2 cases), retrocricoidal stenosis (1 case) and oesophagitis (2 cases). No gastro-duodenal or intestinal lesions were detected. A case of constipation was related to anal involvement. The patients' nutritional status was investigated clinically and biochemically. A search for intestinal malabsorption by means of specific tests was conducted in 2 patients and proved negative. A study of the literature provided data on the nature and specificity of dental lesions. The morphological features, complications and physiopathology of oesophageal stenoses are described The existence of gastrointestinal lesions is discussed. Nutritional repercussions are presented and their causes are discussed. Attention is paid to the medical and surgical treatments of these lesions.

Adolescent↗
PubMed1986

Cutaneous pheomorphic T cell lymphoma. Immunologic, virologic, and T-cell receptor gene rearrangement studies in one European case with initial pseudolymphoma presentation.

An unusual case of cutaneous nodular T cell lymphoma evolving for 4 years with massive eosinophilia and greatly increased IgE levels is discussed. Repeated histologic and immunohistologic examinations could not ascertain malignancy because tumors were composed of a granuloma-like, highly polymorphic cellular infiltrate with mature immunotype and no significant nuclear abnormalities nor epidermotropism. T cell lymphoma was evidenced by the T cell receptor beta-chain gene study, which showed a clonal rearrangement. Final histologic classification was "pleomorphic T cell lymphoma" because further biopsy samples displayed numerous lymphoid cells with pleomorphic convoluted nuclei. The T-cell receptor gene probe is a major tool for the early diagnosis of some T cell lymphomas. The case we report shares many features with the smoldering type of human T cell lymphotropic virus type I-associated Japanese endemic pleomorphic T cell lymphoma. Our virologic study confirms that in nonendemic Western countries, pleomorphic T cell lymphomas do not show evidence of a retrovirus association.

Antibodies, Monoclonal↗
PubMed1985

[Severe combined immune deficiency with hypereosinophilia. Immunologic study of 5 cases].

We herein report five new cases of severe combined immunodeficiency with hypereosinophilia, the so-called familial reticuloendotheliosis first described by Omenn. It is characterized by erythroderma, polyadenopathy, hepatosplenomegaly, severe and repeated infections, protracted diarrhoea with failure to thrive. There is marked eosinophilia as well as a profound immunodeficiency. The immunologic abnormalities consist of an increase in T cell number, a B cell lymphopenia and a complete lack of humoral and cellular immune responses to antigens. A deficiency of lymphocytes 5'-nucleotidase has been inconstantly found. Histologic findings are characteristic, consisting of severe T and B lymphocyte depletion in lymphoid organs with infiltration by histiocytes and, to a lesser extent, eosinophils. The outcome was uniformly fatal within the first year of life. Treatment by a combination of parenteral nutrition, steroids and epipodophyllotoxin was effective in obtaining the complete remission of clinical manifestations due to the histiocytic and eosinophilic infiltration in two patients. However, the treatment failed to correct the immunologic defect. These results indicate that the histiocytic infiltration is possibly not responsible for the immunologic detect observed in this condition.

5'-Nucleotidase↗