MUCOLIPIDOSIS-II - CORRELATION BETWEEN RADIOLOGICAL FEATURES AND HISTOPATHOLOGY OF THE BONES
MUCOLIPIDOSIS-II - CORRELATION BETWEEN RADIOLOGICAL FEATURES AND HISTOPATHOLOGY OF THE BONES
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DOI：
10.1007/bf02387638
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发表时间：
1989-07-01
影响因子：
2.3
通讯作者：
PAGANI, G
中科院分区：
文献类型：
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作者：
PAZZAGLIA, UE;BELUFFI, G;PAGANI, G
本文对12例广泛累及骨骼的粘脂病II(I细胞病)进行了研究。两个病例的病理结果为了解多发性骨质疏松症的影像特征提供了帮助。干骺端可见生长板软骨钙化抑制和软骨软骨样病变。骨膜下强化重塑和小梁旁纤维化在骨干中也很明显。1例患者甲状旁腺激素水平升高。这一发现支持了这样的假设，即骨骼损伤可能是次要的，至少部分是由于肾脏和/或肝脏等内脏的损害，它们是由维生素D和甲状旁腺素介导的。
Twelve cases of Mucolipidosis II (I-cell disease) with a wide range of severity of skeletal involvement were studied. Pathological findings in two cases provided helpful information in understanding the radiographic features of dysostosis multiplex. Inhibition of the growth plate cartilage calcification and rickets-like lesions were observed in the metaphyses. Enhanced subperiosteal remodelling and paratrabecular fibrosis were also evident in the diaphyses. High levels of parathormone were found in one case. This finding supports the hypothesis that bone lesions may be secondary, at least in part, to damage in such viscera as the kidney and/or the liver and that they are mediated by vitamin D and parathormone.