Pensler J M, Langman C B, Radosevich J A, Maminta M L, Mangkornkanok M, Higbee R, Molteni A
Department of Surgery, Northwestern University Medical School, Chicago, IL 60614.
J Bone Miner Res. 1990 May;5(5):493-8. doi: 10.1002/jbmr.5650050511.
Children with monostotic and polyostotic bone dysplasias often exhibit localized bone overgrowth. We investigated the presence of nuclear estrogen and nuclear progesterone receptors by solid-phase radioimmunoassay, immunocytochemistry, and radioligand binding in osteoblast cell cultures derived from the areas of overgrowth of membranous bone, noninvolved membranous bone, and normal membranous bone from children undergoing elective craniotomy. Membranous bone of normal children had demonstrable levels of nuclear estrogen and progesterone receptors identified by radioimmunoassay and immunocytochemical assay. Two- to threefold increased levels of these receptors (p less than 0.001 versus normals) were found in cultures derived from the involved bone of two children with monostotic fibrous dysplasia and in one patient with polyostotic dysplasia (McCune-Albright syndrome). The noninvolved bone in our patients with fibrous dysplasia exhibited nuclear sex steroid hormone receptor levels similar to those in the normal children. Radioligand binding studies demonstrated increased sex steroid hormone receptors in cultures derived from involved osteoblasts. The presence of an increased level of sex steroid hormone receptor was accompanied by increased alkaline phosphatase activity and increased production of osteocalcin in vitro compared to normal or noninvolved bone. The mechanisms by which sex steroid hormone receptor levels are increased in the ostotic dysplasias remain to be established.
患有单骨型和多骨型骨发育异常的儿童常表现出局部骨过度生长。我们通过固相放射免疫测定、免疫细胞化学和放射性配体结合技术，对择期开颅手术患儿的膜性骨过度生长部位、未受累膜性骨及正常膜性骨来源的成骨细胞培养物中的核雌激素受体和核孕酮受体进行了研究。通过放射免疫测定和免疫细胞化学测定发现，正常儿童的膜性骨中有可检测到的核雌激素和孕酮受体水平。在两名单骨型纤维发育不良患儿的受累骨来源的培养物以及一名多骨型发育异常（McCune-Albright综合征）患者的培养物中，这些受体水平增加了两到三倍（与正常儿童相比，p<0.001）。我们的纤维发育不良患者的未受累骨中，核性类固醇激素受体水平与正常儿童相似。放射性配体结合研究表明，受累成骨细胞来源的培养物中的性类固醇激素受体增加。与正常或未受累骨相比，性类固醇激素受体水平升高伴随着体外碱性磷酸酶活性增加和骨钙素产生增加。骨发育异常中性类固醇激素受体水平升高的机制尚待确定。
