László A, Karcsú S, Havass Z
Department of Paediatrics, University Medical School, Szeged, Hungary.
Acta Paediatr Hung. 1987;28(3-4):163-73.
On the basis of electronmicroscopic examinations of the peripheral lymphocytes and polymorphonuclear leukocytes (PMNL) in mucopolysaccharidosis of types I and II in Gaucher and Nieman-Pick diseases, in metachromatic leukodystrophy and in hyperlipoproteinemia, the ultrastructural characteristics are described. Pathological findings with vacuoles formations were observed in Gaucher disease and in metachromatic leukodystrophy against the preliminary literature. The ultrastructural pathological changes are reported from the first ultrastructural PMNL examinations in hyperlipoproteinemias. Electronmicroscopic analysis of the leukocytes is considered to give information equivalent in value to that from liver biopsy studies, but is advantageous in view of its non-invasive nature.
基于对戈谢病、尼曼-匹克病Ⅰ型和Ⅱ型粘多糖贮积症、异染性脑白质营养不良及高脂蛋白血症患者外周淋巴细胞和多形核白细胞（PMNL）的电子显微镜检查，描述了其超微结构特征。与之前的文献报道不同，在戈谢病和异染性脑白质营养不良中观察到了有空泡形成的病理表现。本文首次报道了高脂蛋白血症患者PMNL的超微结构病理变化。白细胞的电子显微镜分析被认为能提供与肝脏活检研究同等价值的信息，但鉴于其非侵入性，具有一定优势。
