Pathology in family practice. Instraspinal dermoid cyst.
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Biomedical subjects
Publications and source records attributed to E Gilbert-Barness.
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We have reviewed 35 deaths of infants who were found face down on polystyrene-filled beanbags or their equivalents. Most of the deaths were recorded as sudden infant death syndrome. One-half of the children died before the age of 2 months. It is suggested that the bags played a major factor in the babies' deaths and are dangerous for young children. Mechanisms involved in the deaths of infants on beanbags may also be applicable to deaths of infants on other types of bedding.
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The clinical features, neuroimaging, and neuropathologic findings of a new syndrome, characterized by onset in early infancy, progressive course, choreiform movements, hypotonia, and dysphagia, are described in 2 siblings originating from a consanguineous marriage. The serial neuroimaging studies indicated progressive loss of volume of both caudate nuclei and change in signal intensity in putamina. Pathologically, there was severe neuronal loss and gliosis in the striatum and thalamus. This pathologic pattern in association with clinical and radiologic correlates, to our knowledge, has not been previously described. It appears that this syndrome is an autosomal recessive disorder.
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A male infant was born with a massive orbitocranial tumor without evidence of metastasis. On light microscopy, the histologic pattern of the tumor was that of a largely necrotic and highly undifferentiated small round cell neoplasm of uncertain origin. Ultrastructural features of the primitive cells included a rare tight junction and myofibril. Immunohistochemical studies showed positive staining for cytokeratin, vimentin, muscle-specific actin, neuron-specific enolase, and S100 protein and negativity for desmin and leukocyte common antigen. We believe this case represents an example of a polyphenotypic small-cell tumor of childhood with epithelial, rhabdomyoblastic, and neuroectodermal differentiation.
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