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作 者:洪雪莲 路慈 张婷[1] 吴华香[1] Hong Xuelian;Lu Ci;Zhang Ting;Wu Huaxiang(Department of Rheumatology,the Second Affiliated Hospital of Zhejiang University,School of Medicine,Hangzhou 310009,China)
机构地区:[1]浙江大学医学院附属第二医院风湿科,杭州310009
出 处:《中华内科杂志》2021年第8期760-763,共4页Chinese Journal of Internal Medicine
基 金:浙江省重点研发项目(2020C03044)。
摘 要:探讨以肌肉肿块为首发症状的结节型肌肉结节病的临床特点。回顾性分析2014年5月至2020年10月浙江大学医学院附属第二医院收治的10例以肌肉肿块为首发症状的结节型肌肉结节病患者的临床资料。结果显示,10例均为女性,2例单侧下肢可及肿块,7例双侧下肢可及肿块,1例双侧下肢及上肢可及肿块。10例均累及肺组织,9例纵隔淋巴结肿大。6例患者血清血管紧张素转化酶57~118 IU/ml。B超显示肿块无特异性表现。磁共振成像显示肿块可有典型“黑色星芒征”及“三层条纹征”。肿块组织活检病理为横纹肌内非坏死性肉芽肿性炎症。3例患者未治疗,7例患者使用0.5~1 mg·kg^(-1)·d^(-1)泼尼松治疗,其中3例患者初始联合免疫抑制剂,4例随访期间加用免疫抑制剂。随访7~62个月,1例患者稳定,6例患者好转,3例患者复发。至随访结束,5例患者仍糖皮质激素联合免疫抑制剂治疗。To investigate clinical characteristics of muscular sarcoidosis presenting muscle mass as initial symptom.The clinical data of 10 patients diagnosed with muscular sarcoidosis and admitted in the Second Affiliated Hospital of Zhejiang University School of Medicine from May 2014 to October 2020 were collected and retrospectively analyzed.All patients were female,including 2 patients with unilateral involvement of lower extremities,7 patients with bilateral involvement of lower extremities,and 1 patient with bilateral involvement of upper and lower extremities.Lung lesions were presented in all patients,and 9 cases had mediastinal lymphadenopathy.Serum angiotensin-converting enzyme(sACE)was 57-118 IU/ml.No specific characteristics were found by ultrasound.Typical“dark-star”sign and“three-stripe”sign were shown in the magnetic resonance imaging(MRI).The pathology of striated muscle was non-necrotizing granulomatous inflammation.Three patients were not treated.In 7 patients treated with prednisone 0.5-1 mg·kg^(-1)·d^(-1),immunosuppressants were combined in 3 patients at initial and the other 4 patients later on.During the follow-up of 7-62 months,1 patient was stable,6 improved and 3 relapsed.Till now,5 patients are still on the treatment of glucocorticoid and immunosuppressant.
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