Department of Endocrinology, Sahlgrenska University Hospital and University of Gothenburg, Gröna Stråket 8, Gothenburg, Sweden.
Eur J Endocrinol. 2012 Apr;166(4):593-600. doi: 10.1530/EJE-11-0942. Epub 2012 Jan 18.
Cushing's disease (CD) and non-functioning pituitary adenoma (NFPA) are rare in paediatric patients. The aim of this study was to describe long-term consequences in adults with GH deficiency (GHD) treated for CD or NFPA during childhood.
DESIGN, PATIENTS AND METHODS: This was a retrospective analysis of data from KIMS (Pfizer International Metabolic Database). Background characteristics, anthropometry and comorbidity were studied in 47 patients diagnosed with childhood-onset (CO)-CD and 62 patients with CO-NFPA. Data from 100 ACTH-sufficient patients with CO-idiopathic hypopituitarism (CO-Idio) were used for comparison. Cardiovascular risk profile was analysed at baseline and at 1 year on GH treatment in a subgroup of patients (17 CO-CD, 24 CO-NFPA and 55 CO-Idio) not receiving GH treatment at study entry.
The median age at diagnosis of pituitary tumour was 14.0 years (range 10-17) in patients with CO-CD and 13.7 years (range 8-17) in CO-NFPA. In addition to GHD, 41% of patients with CO-CD had three or four other pituitary hormone deficiencies compared with 78% of patients with CO-NFPA (P<0.001). Eighty-nine per cent of patients with CO-CD had height SDS lower than 0 compared with 61% of patients with CO-NFPA (P=0.002). Hypertension was more common in CO-CD compared with CO-Idio (23 vs 9%, P=0.018). At 1 year on GH treatment, total- and low-density lipoprotein-cholesterol decreased significantly in CO-CD but not in CO-NFPA.
Adult patients with GHD following treatment for paediatric CD and NFPA have long-term adverse consequences. Despite more severe hypopituitarism in CO-NFPA, patients with CO-CD have more frequently compromised final stature.
库欣病（CD）和无功能垂体腺瘤（NFPA）在儿科患者中较为罕见。本研究旨在描述儿童期接受生长激素缺乏症（GHD）治疗的 CD 或 NFPA 成年患者的长期后果。
设计、患者和方法：这是对辉瑞国际代谢数据库（KIMS）中数据的回顾性分析。对 47 例儿童期发病的库欣病（CO-CD）患者和 62 例 CO-NFPA 患者进行了背景特征、人体测量学和合并症的研究。为了进行比较，使用了 100 例有 ACTH 分泌功能的儿童特发性垂体功能减退症（CO-Idio）患者的数据。在未接受 GH 治疗的患者亚组（17 例 CO-CD、24 例 CO-NFPA 和 55 例 CO-Idio）中，分析了心血管风险概况，基线和 GH 治疗 1 年后。
CO-CD 患者的垂体肿瘤诊断中位年龄为 14.0 岁（范围 10-17 岁），CO-NFPA 患者为 13.7 岁（范围 8-17 岁）。除 GHD 外，41%的 CO-CD 患者有 3 种或 4 种其他垂体激素缺乏症，而 CO-NFPA 患者有 78%（P<0.001）。89%的 CO-CD 患者身高 SDS 低于 0，而 61%的 CO-NFPA 患者（P=0.002）。CO-CD 患者高血压的发生率高于 CO-Idio（23%比 9%，P=0.018）。在 GH 治疗 1 年后，CO-CD 患者的总胆固醇和低密度脂蛋白胆固醇显著降低，但 CO-NFPA 患者没有。
接受儿童 CD 和 NFPA 治疗的成年 GHD 患者有长期不良后果。尽管 CO-NFPA 患者的垂体功能减退症更为严重，但 CO-CD 患者的最终身高更常受到影响。
