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  • Book
    Peter M. Elias, Mary L. Williams, Debra Crumrine, Matthias Schmuth.
    Summary: The various manifestations of ichthyoses are classified either by their appearance or their molecular genetics. This volume focuses on generalized, inherited disorders of cornification, which constitute an ever-enlarging group of monogenic diseases caused by a large number of genes that affect a broad array of cellular functions. The authors' overview reflects their unique perspective that the clinical phenotype in the inherited ichthyoses mirrors a 'best attempt' by a metabolically compromised epidermis to maintain a barrier sufficiently impermeable for survival in a desiccating external environment. The basis for threats to survival is illuminated, and the systemic problems, including growth failure, also reflect a compromised barrier. A new consensus classification of these disorders is provided, and the distinguishing clinical features of each disorder are described. Further, the latest molecular genetic information is succinctly reviewed with up-to-date and comprehensive references. Yet, the major emphases of this volume are on disease pathogenesis and on the identification of key ultrastructural features. This publication will prove an invaluable aid to dermatologists, pediatric dermatologists and pediatricians dealing with patients with inherited ichthyoses. In addition, clinical geneticists and dermatopathologists will find it interesting reading.

    Contents:
    Inherited clinical disorders of lipid metabolism
    Inherited disorders of accelerated desquamation
    Inherited disorders of corneocyte proteins.
    Digital Access Karger 2010
  • Article
    Jordan RK, Owen JJ, Raff MC.
    Eur J Immunol. 1977 Oct;7(10):736-43.
    There is evidence that peripheral lymphoid organs of nude mice, born from homozygous matings, contain a small proportion of theta-positive lymphocytes indicating that nude mice may not be totally devoid of T cell function. It has been suggested that such lymphocytes may develop within the dysplastic nude thymus itself. While this suggestion receives no support from morphological studies, it has been claimed that on explanation to organ culture the developing nude thymus becomes lymphoid. In this present study we confirm the presence of theta-positive lymphocytes in peripheral lymphoid tissues of homozygous nude mice born of nude parents. However, when we have organ-cultured nude thymus, explained from homozygous nude embryos at days 13, 14, 16 and 18 of gestation, we have found no histological sign of lymphopoiesis nor have we detected any theta-positive cells in such cultured material. On the contrary, the nude thymus in vitro develops into the polycystic structure characteristic of the adult nude thymus. We conclude that the small number of theta-positive cells present in the periphery result from extrathymic differentiation.
    Digital Access Access Options