Cushing’s syndrome is the set of clinical manifestations secondary to a chronic excess of glucocorticoids. Bilateral macronodular adrenal hyperplasia with subclinical cortisol secretion is the most common, but its pr...Cushing’s syndrome is the set of clinical manifestations secondary to a chronic excess of glucocorticoids. Bilateral macronodular adrenal hyperplasia with subclinical cortisol secretion is the most common, but its prevalence remains unknown. We describe a case of bilateral macronodular adrenal hyperplasia. This is a 36-year-old female patient who had been consulting for secondary amenorrhea and developing asthenia for 4 months. The clinical examination noted an overweight patient with high blood pressure, facio-trunk obesity, hirsutism and purple stretch marks in the abdomen and thighs. Biologically, hypokalemia at 2.9 meq/l (3.5 - 5.4), normal calcemia at 90 mg/l (85 - 104), fasting blood sugar was 0.84 g/l (0.7 - 1), the tests for minute, low and high dexamethasone suppression test revealed insufficient suppression of cortisol. The cortisoluria collected from the second day to the third day of the high dexamethasone suppression test was at 186 μg/24 h (<60), the ACTH (Pg/ml) was undetectable (6.4 - 49.8). The diagnosis of an independent adrenocorticotrophin (ACTH) Cushing syndrome was made and the adrenal CT scan revealed bilateral macronodular hyperplasia. A bilateral adrenalectomy was performed and a complete remission of Cushing syndrome was achieved. We prescribed to her, hydrocortisone 20 mg/day and alpha-fludrocortisone 25 to 50 μg/day, This medical observation showed that macro-nodular adrenal hyperplasia with overt Cushing syndrome can occur in the third decade of life. Bilateral adrenalectomy has resulted in a complete cure for Cushing’s syndrome, but may be fraught with complications.展开更多
目的探索一种治疗原发性双侧大结节性肾上腺增生(PBMAH)的新方法并分析其疗效。方法回顾性分析郑州大学第一附属医院在2010年3月—2021年4月收治的20例确诊为PBMAH患者的临床资料。20例患者均采用腹腔镜下带蒂肾上腺背部皮下移位术治疗...目的探索一种治疗原发性双侧大结节性肾上腺增生(PBMAH)的新方法并分析其疗效。方法回顾性分析郑州大学第一附属医院在2010年3月—2021年4月收治的20例确诊为PBMAH患者的临床资料。20例患者均采用腹腔镜下带蒂肾上腺背部皮下移位术治疗,术后定期监测患者的临床症状、血浆游离皮质醇、促肾上腺皮质激素(ACTH)、24 h尿游离皮质醇(UFC)。结果术后19例患者获得随访,1例患者失联,随访时间18~120个月,中位值60个月。术后3个月复查血浆游离皮质醇、ACTH、24 h UFC,1例出现血浆游离皮质醇下降、ACTH升高,但患者未出现皮质激素低下症状,观察3个月后复查血浆游离皮质醇、ACTH,均恢复正常。术后6~48个月复查血浆游离皮质醇、ACTH、24 h UFC,19例患者均恢复正常,临床症状消失。结论腹腔镜下带蒂肾上腺背部皮下移位术是治疗PBMAH新的有效治疗方法,可长期缓解库欣综合征患者的症状,目前尚未发现明显不良反应。展开更多
文摘Cushing’s syndrome is the set of clinical manifestations secondary to a chronic excess of glucocorticoids. Bilateral macronodular adrenal hyperplasia with subclinical cortisol secretion is the most common, but its prevalence remains unknown. We describe a case of bilateral macronodular adrenal hyperplasia. This is a 36-year-old female patient who had been consulting for secondary amenorrhea and developing asthenia for 4 months. The clinical examination noted an overweight patient with high blood pressure, facio-trunk obesity, hirsutism and purple stretch marks in the abdomen and thighs. Biologically, hypokalemia at 2.9 meq/l (3.5 - 5.4), normal calcemia at 90 mg/l (85 - 104), fasting blood sugar was 0.84 g/l (0.7 - 1), the tests for minute, low and high dexamethasone suppression test revealed insufficient suppression of cortisol. The cortisoluria collected from the second day to the third day of the high dexamethasone suppression test was at 186 μg/24 h (<60), the ACTH (Pg/ml) was undetectable (6.4 - 49.8). The diagnosis of an independent adrenocorticotrophin (ACTH) Cushing syndrome was made and the adrenal CT scan revealed bilateral macronodular hyperplasia. A bilateral adrenalectomy was performed and a complete remission of Cushing syndrome was achieved. We prescribed to her, hydrocortisone 20 mg/day and alpha-fludrocortisone 25 to 50 μg/day, This medical observation showed that macro-nodular adrenal hyperplasia with overt Cushing syndrome can occur in the third decade of life. Bilateral adrenalectomy has resulted in a complete cure for Cushing’s syndrome, but may be fraught with complications.
文摘目的探索一种治疗原发性双侧大结节性肾上腺增生(PBMAH)的新方法并分析其疗效。方法回顾性分析郑州大学第一附属医院在2010年3月—2021年4月收治的20例确诊为PBMAH患者的临床资料。20例患者均采用腹腔镜下带蒂肾上腺背部皮下移位术治疗,术后定期监测患者的临床症状、血浆游离皮质醇、促肾上腺皮质激素(ACTH)、24 h尿游离皮质醇(UFC)。结果术后19例患者获得随访,1例患者失联,随访时间18~120个月,中位值60个月。术后3个月复查血浆游离皮质醇、ACTH、24 h UFC,1例出现血浆游离皮质醇下降、ACTH升高,但患者未出现皮质激素低下症状,观察3个月后复查血浆游离皮质醇、ACTH,均恢复正常。术后6~48个月复查血浆游离皮质醇、ACTH、24 h UFC,19例患者均恢复正常,临床症状消失。结论腹腔镜下带蒂肾上腺背部皮下移位术是治疗PBMAH新的有效治疗方法,可长期缓解库欣综合征患者的症状,目前尚未发现明显不良反应。
