[To a new definition of malignant histiocytosis in children].
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Biomedical subjects
Publications and source records attributed to C Nézelof.
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The author reconsiders Stanford classification which separates 3 degrees of rejection of transplanted hearts: minimal, moderate or severe rejection. This classification, widely used, was enriched with J. Kennitz's sub-varieties, based on more than 4,000 endomyocardial biopsies, performed on 132 patients who underwent heart transplantation. Interpretation of the histopathologic results of endomyocardial biopsies requires careful knowledge of simultaneous clinical or biochemical data. However, heart biopsy remains indispensible since it is the only test providing information on the presence or absence of cellular infiltrate and on myocardial fibers changes. The difficulties result from the very small size of all samples. Several types of rejection are illustrated and commented. However, the author's experience is presently restricted and hardly allows separating moderate rejections from minimal or severe rejections.
Ultrastructural and immunohistochemical analysis of six specimens revealed for the first time a small number of Langerhans cells in non-neoplastic bronchial epithelium. These cells were usually found either interspersed perpendicularly between columnar epithelial cells or just above the basal lamina with extending cytoplasmic processes. Usually few Birbeck granules, especially located in the Golgi region, were present throughout the cytoplasm. Immunohistochemical studies were performed on semi-thin sections with a polyclonal anti-S-100 protein. The population of S-100 positive cells represented about 1% of all the epithelial cells. Not all ultrastructurally identified Langerhans cells were shown to be positive for S-100 antigen. Our results suggest that Langerhans cells could be a constant cellular constituent for the normal bronchial epithelium. The exact function of Langerhans cells in the respiratory epithelium remains to be investigated, but they may have an immunologic function, such as antigen presentation to T lymphocytes.
A case of multinodular pigmented adrenocortical hyperplasia occurring in a child aged 4 years is reported. The disease suggested an autonomously functioning lesion with low plasma ACTH. At surgery both adrenals presented with brown or yellow patches on the surface corresponding to multiple nodules. Unilateral adrenalectomy was followed by complete recovery and normal ACTH response to IV lysine vasopressin with a follow-up of 3 years. The patient also presented mild spastic diplegia and retarded mental development of unexplained origin.
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Clinico-pathological case report of a 11 year-old girl who successively presented persistent abdominal pain, pneumoperitoneum, repeated intestinal hemorrhages, superficial intestinal ulcerations, first localized on the jejunum and later extended to the all gut. After a course of 13 months, the child died from diffuse and repeated hemorrhages. The morphologic studies revealed a diffuse and homogeneous cellular infiltrate made of large round "lymphomatous" cells scattered within the lamina propria and submucosa. Initially this superficial cellular infiltrate was overshadowed by accompanying inflammatory cells and was not recognized as a tumoral process. Later on the dissemination of abnormal cells to the entire ileon, mesenteric lymph nodes, spleen and liver and the cellular appearance confirmed its neoplastic nature and allowed to consider this process as a malignant histiocytosis of the intestine as described by Isaacson and Wright. This case seems to be the first case reported in childhood.
The availability of drugs that are active against Pneumocystis carinii has renewed interest in and underlined the difficulties of the early diagnosis pneumocystis pneumonia. A retrospective study of 33 cases was undertaken to define the optimal management. It is necessary to take into consideration the high mortality of the untreated condition and the risks of investigation and of treatment. Pneumocystis pneumonia affects only those patients who are immunologicaly incompetent, particularly those with defects of cellular immunity. Early symptoms are common but they are insidious. Indirect immunofluorescent tests were positive in 75% of our cases and were useful as screening tests. However it is necessary to identify the parasite (in our cases it was detected in 29 patients) and this requires invasive techniques such as lung puncture, lung biopsy or bronchial brushing. The best method for the rapid diagnosis of pneumocystis is endobronchial brushing, because it is simple, effect and has few complications.
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The case of a 14-year-old boy of North-African origin, presenting a phagocytic sinus histiocytosis is reported. The main features of this now classical disease are illustrated: its localization to the neck, its chronicity, the fact that it is well tolerated, the pseudotumoral appearance of the adenomegaly as well as the intensive phagocytic (essentially lymphocytophagic) activity of the sinus macrophages. The bringing to light in a picture of hyperimmunity of an elevated percentage of antibodies against the measles and EB viruses, associated with a temporary depression of the cellular immunity, suggest that this lymphophagocytosis could be controlled and facilitated by preferential opsonization of lymphoid cells carrying on their membrane the antigen of the virus or viruses initially responsible.
A case of systemic hyalinosis is reported and the main features of this unusual affection are quoted. The systemic hyalinosis is a syndrome exhibiting the following symptoms : a distressing arthogryposis syndrome, a diffusely stiff skin, some subcutaneous and submucosal fibrotic nodular lymps, repeated suppurative original infections and a facial dysmorphy. Biopsies of nodules show an active proliferation of the connective tissue associated with some peculiar hyalin changes of the collagen fibres. Most cases including the present one die in the first years. The pathogenesis is presently unknown. The systemic hyalinosis seems to be an autosomic recessive heritable disorder and is reasonably settled in the large group of the hereditary diseases of the connective tissue.
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Histochemical study combined with differential extractions of lipids from biopsy fragments in three cases of eosinophil granuloma reveals a great amount and variety of lipidic categories existing in histiocytosis X cells : sterids, fatty-acids, triacylglycerols, phosphoglycerids, cholino-phospholipids, sphingomyelins, glycolipids. Comparison of the lipidic content of histiocytosis X cells and that of normal histiocytes, showed a preponderance of cholesterol esters in histiocytosis X contrary to the normal macrophages and alteration in phospholipidic control of cholesterol esterification. This study confirms that the histiocytosis X cells have an active and probably preferential lipid metabolism, explaining the frequent xanthomatous changes of the lesions and the intense production of various cytoplasmic membranous structures.
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