Simforoosh Nasser, Razzaghy Azar Maryam, Soltani Mohmmad Hossein, Nourbakhsh Mona, Shemshaki Hamidreza
Professor, Department of Urology, Shahid Labbafi Nejad Hospital, Urology and Nephrology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
H. Aliasghar Children's Hospital, Iran University of Medical Sciences, Tehran, Iran.
Urol J. 2017 Aug 29;14(5):5030-5033.
ACTH-independent Cushing's syndrome is an uncommon disorder in children. While laparoscopic adrenalectomy is well-established in adults, it is rarely used in infants and is associated with some concerns. A seven-month infant was referred to our hospital due to progressive signs and symptoms of Cushing's syndrome. Laboratory data confirmed ACTH-independent hypercortisolism. No history of exogenous corticosteroid contact was observed. The patient underwent left transperitoneal laparoscopic adrenalectomy when she was 7 months old, nevertheless,complete response was not seen. The patient underwent right laparoscopic adrenalectomy (contra-lateral adrenal gland) when she was 20 months old. The signs and symptoms of Cushing's syndrome began to resolve and serum and urine cortisol levels became normal 3 months after the second surgery. laparoscopic adrenalectomy is safe and feasible in infants, and in this case, relieved patient of the symptoms and saved her life.
促肾上腺皮质激素(ACTH)非依赖性库欣综合征在儿童中是一种罕见的疾病。虽然腹腔镜肾上腺切除术在成人中已广泛应用,但在婴儿中很少使用,且存在一些问题。一名7个月大的婴儿因库欣综合征的进行性体征和症状被转诊至我院。实验室数据证实为ACTH非依赖性高皮质醇血症。未发现有外源性皮质类固醇接触史。该患者7个月大时接受了经腹腔左侧腹腔镜肾上腺切除术,但未见完全缓解。患者20个月大时接受了右侧腹腔镜肾上腺切除术(对侧肾上腺)。第二次手术后3个月,库欣综合征的体征和症状开始缓解,血清和尿皮质醇水平恢复正常。腹腔镜肾上腺切除术在婴儿中是安全可行的,在本例中,缓解了患者的症状并挽救了她的生命。