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Oral manifestations of alpha-mannosidosis: report of a case with ultrastructural findings
Authors:Tetsuro Ishigami  rea Schmidt-Westhausen  Hans Peter Philipsen  Sergei I. Baiborodin  Hans Gelderblom  Peter A. Reichart
Affiliation:First Department of Oral and Maxillofacial Surgery, Kagoshima University Dental School, Kagoshima, Japan;Abteilung für Oralchirurgie und Zahnärztliche Röntgenologie, Charité, Humboldt-Universität zu Berlin, Berlin, Germany;Oral Biology Unit, University of Hong Kong, Hong Kong;Institute of Cytology and Genetics, Academy of Sciences, Siberian Division, Novosibirsk, Russia;Robert Koch Institut, Bundesgesundheitsamt, Berlin, Germany
Abstract:Alpha-mannosidosis is a rare storage disease with distinct biochemical, clinical, histological and ultrastructural features. The oro-facial findings in a 26-year-old man are described. Gingival and oral mucosal hyperplasias were studied using histology and transmission electron microscopy (TEM). The TEM findings were comparable to those of other tissues examined in previous reports, consisting of histiocytic cells containing storage vacuoles with fine reticule-granular material.
Keywords:giant cells    histiocytes    hyperplasia    lysosomal storage disease    mannosidosis
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