Hoshiro Madoka, Harada Takeshi, Iwai Hiroshi, Miyatake Toshiyuki, Nishimura Akiyoshi, Ohno Yasuhiro, Aoki Norihiko
Department of Endocrinology, Metabolism and Diabetes Mellitus, Kinki University School of Medicine, Osaka-Sayama 589-8511, Japan.
Endocr J. 2003 Aug;50(4):385-92. doi: 10.1507/endocrj.50.385.
We report two cases of monostotic Paget's disease which were effectively treated with bisphosphonate. Case 1 was a 60-year-old female. Medical examination revealed high alkaline phosphatase (ALP) levels making her visit our clinic. Hematological examination showed high levels of ALP isozyme 3 and bone metabolism markers, and bone scintigraphy demonstrated strong accumulation of 99mTc on the skull. With the diagnosis of monostotic Paget's disease of the skull, treatment with bisphosphonate (etidronate) was started. The response to etidronate was good and after 12 weeks of treatment, the ALP levels decreased to about 26% of the levels before treatment, without the appearance of any symptoms or lesion development. One year and three months later, ALP increased again, and etidronate administration was resumed. However, four years after the diagnosis of the disease, etidronate became ineffective and oral administration of alendronate, a stronger bisphosphonate, was started at 5 mg/day. The patient responded favorably to the bisphosphonate and is still under observation. Case 2 was a 71-year-old female. High ALP levels were found during the follow-up of type 2 diabetes, and the case was diagnosed as monostotic Paget's disease of the pelvis based on bone metabolism markers and bone scintigraphy. Etidronate treatment at 200 mg/day resulted in the improvement of bone metabolism markers and bone scintigraphy findings. When she died of colon cancer twelve months later, with no marked progress of the Paget's disease of bone observed clinically.
我们报告了两例经双膦酸盐有效治疗的单骨型佩吉特病病例。病例1是一名60岁女性。体格检查发现碱性磷酸酶(ALP)水平升高,促使她前来我们诊所就诊。血液学检查显示ALP同工酶3和骨代谢标志物水平升高,骨闪烁显像显示99mTc在颅骨上强烈聚集。诊断为颅骨单骨型佩吉特病后,开始用双膦酸盐(依替膦酸)治疗。对依替膦酸的反应良好,治疗12周后,ALP水平降至治疗前水平的约26%,且未出现任何症状或病变进展。一年零三个月后,ALP再次升高,于是恢复依替膦酸给药。然而,疾病诊断四年后,依替膦酸变得无效,开始口服更强效的双膦酸盐阿仑膦酸钠,剂量为5毫克/天。患者对双膦酸盐反应良好,目前仍在观察中。病例2是一名71岁女性。在2型糖尿病随访期间发现ALP水平升高,根据骨代谢标志物和骨闪烁显像,该病例被诊断为骨盆单骨型佩吉特病。每天200毫克依替膦酸治疗使骨代谢标志物和骨闪烁显像结果得到改善。十二个月后她死于结肠癌,临床上未观察到骨佩吉特病有明显进展。