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儿科肾上腺疾病的异常线性生长:发病机制、患病率和管理。

Abnormal linear growth in paediatric adrenal diseases: Pathogenesis, prevalence and management.

机构信息

Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy.

Department of Pediatrics, IRCCS San Raffaele Hospital, Milan, Italy.

出版信息

Clin Endocrinol (Oxf). 2020 Feb;92(2):98-108. doi: 10.1111/cen.14131. Epub 2019 Dec 5.

Abstract

Abnormal adrenal function can interfere with linear growth, potentially causing either acceleration or impairment of growth in paediatric patients. These abnormalities can be caused by direct effects of adrenal hormones, particularly glucocorticoids and sex steroids, or be mediated by indirect mechanisms such as the disturbance of the growth hormone-insulin-like growth factor-1 axis and aromatization of androgens to oestrogens. The early diagnosis and optimal treatment of adrenal disorders can prevent or minimize growth disturbance and facilitate improved height gain. Mechanisms of growth disturbance in the following abnormal states will be discussed; hypercortisolaemia, hyperandrogenaemia and obesity. Prevalence and features of growth disturbance will be discussed in ACTH-dependent and ACTH-independent Cushing's syndrome, adrenocortical tumours, premature adrenarche, congenital adrenal hyperplasia and adrenal insufficiency disorders. Recommendations for management have been included.

摘要

肾上腺功能异常可干扰线性生长,有可能导致儿科患者的生长加速或受损。这些异常可能是由肾上腺激素的直接作用引起的,特别是糖皮质激素和性激素,也可能是通过间接机制介导的,如生长激素-胰岛素样生长因子-1 轴的紊乱以及雄激素向雌激素的芳香化。早期诊断和最佳治疗肾上腺疾病可以预防或最小化生长障碍,并促进更好的身高增长。以下异常状态下生长障碍的机制将被讨论;皮质醇过多症、高雄激素血症和肥胖症。ACTH 依赖性和 ACTH 非依赖性库欣综合征、肾上腺皮质肿瘤、性早熟、先天性肾上腺增生和肾上腺功能不全疾病中的生长障碍的患病率和特征将被讨论。已经包括了管理建议。

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