Phosphoglyceride (PG) crystal deposition disease: A novel acquired disease in which PG crystals are deposited in injured soft tissue and bone with phospholipid metabolism disturbance
Phosphoglyceride (PG) crystal deposition disease: A novel acquired disease in which PG crystals are deposited in injured soft tissue and bone with phospholipid metabolism disturbance
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DOI：
10.1111/j.1440-1827.2004.01776.x
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发表时间：
2004-12-01
影响因子：
2.2
通讯作者：
Tsutsui, Y
中科院分区：
文献类型：
--
作者：
Miura, K;Han, GP;Tsutsui, Y
比较了三例已发表病例和三例新发软组织和骨骼磷酸甘油酯（PG）晶体沉积病病例的临床病史和晶体特征。所有患者（年龄范围，51-64 岁）总体健康，没有先天性免疫缺陷或脂质沉积症的遗传背景。异物肉芽肿生长缓慢，主要发生在术后或重复注射病变处。在两种情况下，晶体沉积在多个位置，在一种情况下，在骨髓中发现了噬脂体积聚。该晶体的特点是溶于乙酸并形成氧气，易溶于碱，并通过异羟肟酸金法显示 PG 阳性染色。通过红外显微镜、质谱和 X 射线微量分析检查的所有晶体都显示出相似的结果，支持了晶体是 PG 的理论。磷酸甘油酯沉积病是一种脂质代谢紊乱疾病，其中PG晶体缓慢沉积，主要在受伤的软组织中，形成异物肉芽肿。诊断可以基于组织学特征。尽管有些病例表现出反复的全身沉积，但预后良好。巨噬细胞中的溶酶体磷酸甘油酯代谢可能受到影响。
The clinical history and crystal characteristics of three published cases and three new cases of phosphoglyceride (PG) crystal deposition disease of soft tissues and bones were compared. All patients (age range, 51-64 years) were generally healthy without a genetic background of congenital immunodeficiency or lipidosis. Foreign body granulomas grew slowly, predominantly at postoperative or repeat injection lesions. In two cases, crystals were deposited in multiple locations, and in one case, lipophage accumulations were found in the bone marrow. The crystals characteristically dissolved in acetic acid with oxygen gas formation, easily dissolved in alkalis and showed positive staining for PG by the gold hydroxamic acid method. All crystals examined by infrared microscopy, mass spectrometry and X-ray microanalysis showed similar results, supporting the theory that the crystals were PG. Phosphoglyceride deposition disease is a lipid metabolic disorder in which PG crystals are slowly deposited, predominantly in injured soft tissues, forming foreign body granulomas. The diagnosis can be based on histological characteristics. The prognosis is favorable, although some cases showed systemic depositions with repetitions. Lysosomal phosphoglyceride metabolism in macrophages might be affected.