PDF(Pubmed)
Abstract：
UNASSIGNED: Paget\'s disease of the bone refers to a chronic cumulative disorder characterized by enhanced osteoclastic function followed by a secondary surge in osteoblastic activity. The condition can manifest as a polyostotic or monostotic bone disease with most patients having an asymptomatic presentation, although some may complain of pain localized to the affected bone while others express symptoms of nerve compression. A pagetic bone is predisposed to develop pathological fractures, bony deformities, and a rare yet detrimental transformation into osteosarcoma. Detection is often accidental when performing radiographic tests for other indications or when elevated blood levels of alkaline phosphatase (ALP) are detected. Treatment with third-generation bisphosphonates is helpful in preventing further bone resorption and, additionally, reduces bony pains that are believed to be caused by excessive metabolic activity. Here, we present a case of a middle-aged asymptomatic female with elevated serum ALP levels up to 1537 IU/L (reference range 40-150 U/L) during her pre-operative evaluation for elective cholecystectomy.99m Tc-methylene diphosphonate bone scintigraphy revealed diffuse uptake in the skull and, hence, was diagnosed as a case of isolated Paget\'s disease of the skull.
UNASSIGNED: The rarity of this disease in Southeast-Asians, its uncontrived detection, and the isolated skull involvement, imparts high clinical relevance on this case. Early detection and management of this disease can help prevent the development of life-threatening complications in affected patients, hence decreasing the morbidity.
UNASSIGNED: The rarity of this disease in Southeast-Asians, its uncontrived detection, and the isolated skull involvement, imparts high clinical relevance on this case. Early detection and management of this disease can help prevent the development of life-threatening complications in affected patients, hence decreasing the morbidity.
摘要：
■佩吉特骨病是指一种慢性累积疾病，其特征是破骨细胞功能增强，随后成骨细胞活动继发性激增。这种情况可以表现为多骨或单骨骨病，大多数患者无症状表现，尽管有些人可能抱怨疼痛局限于受影响的骨骼，而另一些人则表现出神经压迫的症状。骨头容易发生病理性骨折，骨畸形,和罕见但有害的转化为骨肉瘤。在进行其他适应症的影像学检查或检测到血液中碱性磷酸酶（ALP）水平升高时，检测通常是偶然的。第三代双膦酸盐治疗有助于防止进一步的骨吸收，此外，减少骨痛，被认为是由过度的代谢活动引起的。这里,我们介绍了一例无症状的中年女性，在进行选择性胆囊切除术的术前评估期间，血清ALP水平升高至1537IU/L（参考范围40-150U/L）.99mTc-亚甲基二膦酸盐骨闪烁显像显示颅骨弥漫性摄取，因此，被诊断为孤立的Paget颅骨病。
■这种疾病在东南亚人中很少见，它毫无根据的检测，和孤立的头骨参与，对这种情况具有很高的临床相关性。这种疾病的早期发现和管理可以帮助预防受影响患者危及生命的并发症的发展，从而降低发病率。
■这种疾病在东南亚人中很少见，它毫无根据的检测，和孤立的头骨参与，对这种情况具有很高的临床相关性。这种疾病的早期发现和管理可以帮助预防受影响患者危及生命的并发症的发展，从而降低发病率。
