Lodish Maya B, Sinaii Ninet, Patronas Nicholas, Batista Dalia L, Keil Meg, Samuel Jonelle, Moran Jason, Verma Somya, Popovic Jadranka, Stratakis Constantine A
Section on Endocrinology Genetics, Program on Developmental Endocrinology Genetics, National Institute of Child Health and Human Development, NIH, Building 10, CRC, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA.
J Clin Endocrinol Metab. 2009 Jun;94(6):2002-8. doi: 10.1210/jc.2008-2694. Epub 2009 Mar 17.
Hypertension (HTN) has been reported in up to 60% of children with Cushing syndrome (CS), but its course, side effects, and potential differences among various causes of CS have not been adequately studied.
The objective of the study was to measure blood pressure in pediatric patients with CS before and after transphenoidal surgery or adrenalectomy and identify side effects and rates of residual HTN.
Data from 86 children with corticotropinomas [Cushing disease (CD)] and 27 children with ACTH-independent CS (AICS) were analyzed.
Patients with CD and AICS had significant HTN before surgery; more patients with AICS had systolic HTN (SHTN) than with CD (74 vs. 44%, P = 0.0077), but the rate of diastolic HTN (DHTN) was similar. Both groups experienced significant decreases in SHTN immediately after transphenoidal surgery and adrenalectomy. One year postoperatively, both SHTN and DHTN were lower than the preoperative values in all patients, but as many as 16 and 4% of the patients with CD and 21 and 5% of the patients with AICS still had SHTN and DHTN, respectively. Higher blood pressure preoperatively correlated with cortisol levels. Two patients suffered serious side effects: one with multiple infarcts and another with hypertensive encephalopathy.
Children with CS are at risk for residual HTN despite a significant improvement after surgical cure. HTN appears to correlate with the degree of hypercortisolemia. Serious HTN-related side effects, although rare, may occur during the perioperative period.
据报道，高达60%的库欣综合征（CS）患儿患有高血压（HTN），但其病程、副作用以及CS各种病因之间的潜在差异尚未得到充分研究。
本研究的目的是测量经蝶窦手术或肾上腺切除术前、后儿童CS患者的血压，并确定副作用和残余HTN的发生率。
分析了86例患有促肾上腺皮质激素瘤[库欣病（CD）]的儿童和27例不依赖促肾上腺皮质激素的CS（AICS）儿童的数据。
CD和AICS患者术前均有明显的HTN；AICS患者中收缩期HTN（SHTN）的患者比CD患者多（74%对44%，P = 0.0077），但舒张期HTN（DHTN）的发生率相似。两组在经蝶窦手术和肾上腺切除术后SHTN均显著下降。术后1年，所有患者的SHTN和DHTN均低于术前值，但仍分别有多达16%和4%的CD患者以及21%和5%的AICS患者存在SHTN和DHTN。术前血压较高与皮质醇水平相关。两名患者出现严重副作用：一名患有多发性梗死，另一名患有高血压脑病。
尽管手术治愈后有显著改善，但CS患儿仍有残余HTN的风险。HTN似乎与高皮质醇血症的程度相关。严重的HTN相关副作用虽然罕见，但可能在围手术期发生。
