[Sudden infant death. Should the definition be changed?].
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Biomedical subjects
Publications and source records attributed to C Rambaud.
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This report illustrates a lingual localization of an inclusion body fibromatosis, the so-called Reye tumor or infantile digital fibromatosis (IDF). The light microscopic features were identical to those found in IDF, showing eosinophilic perinuclear inclusions located in spindle-shaped cells arranged in interlacing fascicles. The immunocytochemical and ultrastructural findings suggested a fibroblastic and/or myofibroblastic nature of the proliferative cells. However, the inclusions in our case were strongly stained with vimentin and their ultrastructural appearance was in keeping with intermediate filaments. These findings have never been described in other reports of fibromatosis. Whereas most reviews state that IDF occurs exclusively on the digits, this unique case describes its possible occurrence in the tongue.
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Over the last 20 years, the sudden infant death syndrome has become the leading cause of death in infants aged one month to one year in developed countries. The SIDS Referral Centers set up in France have been assigned the task of performing thorough clinical, metabolic, infectious and histologic studies. This post-mortem evaluation, whose results are difficult to interpret, is undertaken in an attempt to discriminate between the multiple causes of conditions present at the time of death. This classification task will improve the definition of a number of risk factors. Among these factors, prematurity, perinatal distress requiring resuscitation, and an unfavorable sociocultural environment are often mentioned. Other factors, including intrauterine growth retardation, dysmorphic disorders, impaired regulation of ventilation, heart rhythm anomalies, and inherited defects in fatty acid metabolism are still under study since they are all infrequent. Various combinations of these factors may result in increased vulnerability to stress during the first months of life, the period when SIDS is most common. This ongoing research is indispensable for providing advice and support of the family and developing appropriate individual preventive measures for newborn SIDS siblings.
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In order to evaluate the effect of laser coagulation on placental vessels and to define the optimal power density that should be used for this procedure, coagulation of placental vessels and histological examination were performed on freshly delivered placentae. This study suggests that 45- to 55-watt (6,000-7,666 J/cm2) shots can cause adequate placental injury. The extent of vascular lesions depends upon incident beam energy, fiber diameter, distance and angle to the vessel.
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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.