Krane S M, Simon L S
Clin Orthop Relat Res. 1987 Apr(217):26-36.
High rates of bone resorption, bone formation, and marrow fibrosis are characteristic of Paget's disease of bone. This excessive bone turnover is reflected by increased fluxes of calcium ions out of and into the skeleton. The rates of these fluxes are highly geared to each other such that calcium balances are close to zero in the absence of fracture or significant immobilization. An increased turnover of bone matrix is also evident by increased urinary excretion of collagen breakdown products (oligopeptides of hydroxyproline, hydroxylysine, and hydroxylysine glycosides) as well as products (peptides of higher molecular weight) related to collagen synthesis. Increased circulatory levels of procollagen extension fragments reflect increased synthesis of Type I collagen (bone matrix) and Type III collagen (marrow fibrosis). Increased levels of bone gamma-carboxyglutamic acid-protein presumably reflect primarily bone matrix synthesis but bone resorption as well. When bone resorption is suppressed pharmacologically, the abnormal levels of these markers of matrix turnover and osteoblastic activity (alkaline phosphatase) also decrease, presumably as a result of coupling of resorption and formation.
高骨吸收、骨形成和骨髓纤维化发生率是骨佩吉特病的特征。这种过度的骨转换表现为钙离子进出骨骼的通量增加。这些通量的速率彼此高度相关，以至于在没有骨折或显著固定的情况下钙平衡接近零。骨基质周转率增加还表现为胶原分解产物（羟脯氨酸、羟赖氨酸和羟赖氨酸糖苷的寡肽）以及与胶原合成相关的产物（更高分子量的肽）的尿排泄增加。前胶原延伸片段循环水平升高反映了I型胶原（骨基质）和III型胶原（骨髓纤维化）合成增加。骨γ-羧基谷氨酸蛋白水平升高可能主要反映骨基质合成，但也反映骨吸收。当通过药物抑制骨吸收时，这些基质转换和成骨细胞活性标志物（碱性磷酸酶）的异常水平也会降低，这可能是吸收与形成偶联的结果。
