Cases reported "Lipidoses"

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1/3. Ultrastructural study of so-called curvilinear bodies and fingerprint structures in lymphocytes in late-infantile amaurotic idiocy.

    Peripheral lymphocytes of 6 patients with late-infantile amaurotic idiocy were examined with the electron microscope for the occurrence of curvilinear bodies and fingerprint structures. In 3 of the patients predominantly curvilinear bodies were found; in 1 case they contained some fingerprint profiles. In the remaining 3 patients curvilinear bodies were relatively scarce, whereas fingerprint structures prevailed. Moreover, pleomorphic bodies with rectilinear profiles and parallel tubular inclusion bodies were present. No lymphocytes with vacuoles were observed in any of the patients. The sural nerve biopsies of all patients revealed curvilinear bodies. This tissue consequently may be considered to give more reliable information in comparison with the lymphocyte. The likelihood of transition of one type inclusion body into another, the specificity of the curvilinear body and, to our mind, the rigid classification of the amaurotic idiocy into a curvilinear and a fingerprint type, are discussed.
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ranking = 1
keywords = fingerprint
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2/3. The protracted form of juvenile neuronal ceroid-lipofuscinosis.

    Clinical and ultrastructural findings consisting of curvilinear and fingerprint residual bodies, in a protracted juvenile form of NCL are reported from a woman who died at the age of 35 years. Homochrony and homotypy of her brother's illness emphasize intrafamilial similarities within subgroups of lysosomal disorders.
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ranking = 0.125
keywords = fingerprint
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3/3. Ultrastructure of peripheral lymphocytes in generalized ceroid-lipofuscinosis. Report of a case.

    Electron microscopic observation was made on the peripheral blood cells obtained from a 4-year-old boy with generalized ceroid-lipofuscinosis (GCL), and two kinds of significant inclusions were found in the cytoplasm of lymphocytes. One was ceroid-lipofuscin accompanied with the fingerprint pattern, and the other was a lamellar body which showed the concentric lamellar structure or the myelin figure. ceroid-lipofuscin was found in 40% of peripheral lymphocytes. It is important in diagnosing GCL that ceroid-lipofuscin can be detected with high incidence in lymphocytes of peripheral blood.
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ranking = 0.125
keywords = fingerprint
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