Manzke E, Gruber H, Baylink D, Hiness R
Radiologe. 1982 Apr;22(4):175-80.
Morphokinetic measurements after tetracycline labeling were performed on bone specimens of the iliac crest in a 30-year-old man and a 38-year-old women. On radiographs, the osteopetrosis (=marble bone disease Albers-Schönberg) was characterized by an almost homogenous osteosclerosis in the man and by variable zones of high and normal density in the woman. Histologically, the man revealed increased rates of bone formation and mineralisation as signs of hyperactivity of the osteoblasts accompanied, biochemically, by an elevation of serum alkaline phosphatase and a high urinary output of hydroxyproline. In the woman increased resorbing bone seams, and elevation of serum acid phosphatase and a slightly lowered urinary output of hydroxyproline were apparent as signs of an osteoclastic deficiency. The cause of osteopetrosis has been generally ascribed to decreased bone resorption. This study shows that the accumulation of bone mass in osteopetrosis may be due also to increased bone formation and that by pathomechanism, probably, two forms of osteopetrosis in the adult may be differentiated.
在一名30岁男性和一名38岁女性的髂嵴骨标本上进行了四环素标记后的形态动力学测量。在X线片上，骨硬化症（=大理石骨病，阿尔伯斯-尚伯格病）在男性中表现为几乎均匀的骨质硬化，在女性中表现为高密度区和正常密度区的可变区域。组织学上，男性显示骨形成和矿化率增加，这是成骨细胞活性亢进的迹象，生化方面伴有血清碱性磷酸酶升高和羟脯氨酸尿排出量增加。在女性中，可见骨吸收缝增加、血清酸性磷酸酶升高以及羟脯氨酸尿排出量略有降低，这是破骨细胞功能缺陷的迹象。骨硬化症的病因通常归因于骨吸收减少。本研究表明，骨硬化症中骨量的积累也可能是由于骨形成增加，并且从发病机制来看，成人的骨硬化症可能有两种形式。
