[Proceedings: Changes of systolic time intervals in hypertension].
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Biomedical subjects
Publications and source records attributed to K Shioda.
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Two patients and a healthy blood donor were found to be Lan negative and to have anti-Lan in their sera. These are the first three examples of the Lan-negative phenotype to be described in Japanese. The families of two of the propositi were studied: the parents of both were first cousins and one of the propositi had a Lan-negative sibling. No Lan-negative examples were found in 15,000 Japanese donors screened with anti-Lan.
We report a sibling case of Nasu disease. A 35-year-old housewife, whose parents were consanguineous and whose sister died of the same disease, developed dementia, followed by bone fracture, incontinence and convulsions. She died at age 41. Pathologically, characteristic membranocystic changes of the fat cells in bone marrow and adipose tissues were observed. Neuropathologically, there was demyelination associated with intense gliosis and numerous axonal spheroids in the cerebral white matter. At the electron microscope level, these spheroids were an accumulation of various cell organelles. In addition, some had Hirano bodies. Incontinence was correlated with reduction of the number of nerve cells in Onuf's nucleus of the sacral cord.