[15岁男孩的库欣氏病,在青春期前表现出来]。

Y L Skorodok, I Y Ioffe, E V Plotnikova, N Y Kalinchenko, I I Nagornaya, A V Kozhevnikova, N V Kazachenko, O V Filippova
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引用次数: 0

摘要

库欣病(CD)是青少年内源性高皮质醇症(EH)的主要形式(75-80%)。尽管临床表现明显,但由于需要多种研究方法,假阳性和假阴性结果的风险,解释困难以及儿童mr成像比例低,CD的诊断比较复杂。本例患者自10岁起体重过度增加,回顾性发现生长速度下降,14.5岁时出现绝对身材矮小。15岁时的检查显示昼夜皮质醇节律反转(9:00 378.4 nmol/l, 23:00 598.9 nmol/l), 23:00唾液皮质醇水平升高(20.32 nmol/l),尿液皮质醇水平升高(981.5 mcg/天)。夜间地塞米松试验(ODT)阳性(皮质醇44.26 nmol/l), ACTH处于“灰色地带”(22.19 pg/ml)。磁共振造影显示垂体结构不均匀的征象。15.5岁复查时,患者皮质醇昼夜节律反转伴有绝对高皮质醇血症(9:00 843.4 nmol/l, 23:00 929.4 nmol/l), ODT变为阴性(皮质醇235 nmol/l), ACTH水平维持在“灰色地带”(25.1 pg/ml)。MRI示垂体腺瘤6×4 mm。左岩下窦与外周血间ACTH水平梯度为13.3,证实CD。经蝶窦腺瘤切除术后,皮质醇水平为39.4 nmol/l,表明病情缓解,复发风险低;随后注意到EH症状的反向发展。术后尿崩症和原发性甲状腺功能减退需要替代治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Cushing's disease in a 15-year-old boy, manifesting in prepubertal age].

Cushing's disease (CD) is the leading form (75-80%) of the endogenous hypercortisolism (EH) in adolescents. Despite the obvious clinical manifestations, the diagnosis of CD is complicated because of the need of several research methods, the risk of false-positive and false-negative results, difficulties in interpretation and the low percentage of MR-imaging in children. The present patient had noted excessive weight gain since the age of 10 years, a decrease in growth rate was detected retrospectively, absolute short stature developed by the age of 14.5 years. An examination at the age of 15 revealed an inversion of the circadian cortisol rhythm (9:00 378.4 nmol/l, 23:00 598.9 nmol/l), an increase in the cortisol level in saliva at 23:00 (20.32 nmol/l) and excretion in urine (981.5 mcg/day). The overnight dexamethasone test (ODT) was positive (cortisol 44.26 nmol/l), ACTH was in the "gray zone" (22.19 pg/ml). MRI with contrast showed signs of a heterogeneous pituitary gland structure. When patient was re-examined at 15.5 years the inversion of the circadian rhythm of cortisol was accompanied by absolute hypercortisolemia (9:00 843.4 nmol/l, 23:00 929.4 nmol/l), ODT became negative (cortisol 235 nmol/l), ACTH level remained in the "gray zone" (25.1 pg/ml). MRI with contrast showed pituitary adenoma 6×4 mm. An ACTH level gradient between the left inferior petrosal sinus and peripheral blood of 13.3 confirmed CD. After transsphenoidal adenomectomy, a cortisol level of 39.4 nmol/l indicated remission with a low risk of relapse; subsequently a reverse development of EH symptoms were noted. Postoperative diabetes insipidus and primary hypothyroidism required replacement therapy.

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