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目的探讨分析原发性色素沉着性结节性肾上腺皮质病(primary pigmented nodular adrenocortical disease,PPNAD)的临床特征、辅助检查结果及治疗方法,以增强对该病的认识。方法采用回顾性分析,对本科收治的2例患者及国内报道的23例患者从性别比例、发病年龄、临床特征、实验室检查、影像学特征及治疗方法进行综合分析。结果 25例患者中男性9例,女性16例,确诊年龄11-53岁,年龄在40岁以上者仅1例。23例有典型库欣表现,伴Carney综合征者12例,12例肾上腺CT表现为结节样增粗,13例无异常改变。肾上腺病理可见色素沉积的结节样改变。双侧肾上腺切除后予激素替代是本病有效治疗方法。结论对影像学检查无明显异常改变的非ACTH依赖性库欣综合征患者,尤其是青少年患者,要常规将PPNAD列入鉴别诊断。双侧肾上腺切除后予激素替代治疗是本病的最佳治疗方法,术后应密切随访。
Objective To explore the clinical features of primary pigmented nodular adrenocortical disease (PPNAD), to assist in the examination of the results and treatment methods to enhance the understanding of the disease. Methods A retrospective analysis of two cases admitted to our hospital and 23 cases reported in our country from the sex ratio, age of onset, clinical features, laboratory tests, imaging features and treatment methods for a comprehensive analysis. Results Among the 25 patients, 9 were males and 16 females, with a definite diagnosis of 11-53 years of age and only 1 case of patients over the age of 40 years. Twenty-three patients had typical Cushing’s manifestations, 12 patients with Carney’s syndrome, 12 patients with adrenal CT showed nodular thickening, and 13 patients without abnormal changes. Adrenal pathological changes in pigmented nodules. Bilateral adrenalectomy after hormone replacement is an effective treatment of this disease. CONCLUSIONS: Non-ACTH-dependent Cushing’s syndrome patients, especially adolescents with no abnormal changes in imaging studies, should be routinely included in the differential diagnosis of PPNAD. Bilateral adrenalectomy after hormone replacement therapy is the best treatment for this disease, should be followed up after surgery.