Halioui-Louhaichi S, Dridi Y, Azzabi O, Selmi I, Fetni I, Siala N, Maherzi A
Service de pédiatrie, hôpital Mongi Slim La Marsa, faculté de médecine de Tunis, université de Tunis El Manar, La Marsa, Tunisie.
Service de pédiatrie, hôpital Mongi Slim La Marsa, faculté de médecine de Tunis, université de Tunis El Manar, La Marsa, Tunisie.
Arch Pediatr. 2016 Jan;23(1):61-5. doi: 10.1016/j.arcped.2015.09.018. Epub 2015 Nov 6.
Cushing syndrome (CS) is a rare feature of McCune-Albright syndrome. Treatments consist of bilateral adrenalectomy followed by lifelong glucocorticoid and mineralocorticoid treatment. However, cases of spontaneous remission of CS have been reported in the literature. We report a case of McCune-Albright syndrome with CS treated with metyrapone for 30 months with prolonged remission after a 12-year follow-up. Adrenalectomy may be avoided in some cases of CS caused by McCune-Albright syndrome. Metyrapone could be a good alternative to surgical treatment.
库欣综合征(CS)是McCune - Albright综合征的一种罕见特征。治疗方法包括双侧肾上腺切除术,随后进行终身糖皮质激素和盐皮质激素治疗。然而,文献中已有CS自发缓解的病例报道。我们报告一例患有CS的McCune - Albright综合征患者,用甲吡酮治疗30个月,经过12年随访后获得长期缓解。在某些由McCune - Albright综合征引起的CS病例中,肾上腺切除术可能可以避免。甲吡酮可能是手术治疗的一个良好替代方案。