Cunha Heloisa Marcelina, Elias Lucila Leico Kagohara, Camacho-Hübner Cecilia, Moreira Ayrton Custódio, Martinelli Carlos Eduardo
Division of Endocrinology, Department of Paediatrics, School of Medicine of Riberao Preto-USAP, Brazil.
Clin Endocrinol (Oxf). 2004 Jul;61(1):94-101. doi: 10.1111/j.1365-2265.2004.02075.x.
Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD) is characterized by high androgen levels, ambiguous genitalia or premature pubarche, increased height velocity and skeletal maturation. Considering the possibility of changes in the IGF system components depending on the state of clinical control, the objective of the present study was to analyse serum IGF-I, IGF-II and IGFBP levels in children with 21-OHD under two states of clinical control.
We studied 12 prepubertal children with 21-OHD CAH aged 4.0 +/- 0.7 years. They were classified as good (GC) or poor control (PC) based on growth rate, signs of adrenal insufficiency or Cushing syndrome, progression of sexual characteristics and serum androgens levels. Blood samples were obtained from each patient in two different states of clinical control (GC and PC) for biochemical measurements.
IGF-I, IGF-II, IGFBP-1, IGFBP-2 and IGFBP-3 were determined by immunoassays. IGFBPs were also analysed by Western ligand blotting (WLB).
Levels of IGF-I (P = 0.03) and IGFBP-3 (P = 0.01) were higher in GC than in PC while IGFBP-1 (P = 0.004) concentrations were lower in GC patients. A trend towards higher levels of IGF-II (P = 0.08) and lower levels of IGFBP-2 (P = 0.08) was observed in GC children. Increased IGFBP-4 band intensity was observed in GC children (P = 0.03).
Higher levels of IGF-I, IGFBP-3 and IGFBP-4, but lower levels of IGFBP-1, were associated with better control in children with 21-OHD CAH. These findings are different from those observed in children with other causes of increasing androgens levels and are likely to be related to the insufficient glucocorticoid status.
21-羟化酶缺乏症（21-OHD）所致先天性肾上腺皮质增生症（CAH）的特征为雄激素水平升高、生殖器模糊或青春期提前、身高增长速度加快和骨骼成熟加速。鉴于胰岛素样生长因子（IGF）系统成分可能随临床控制状态而变化，本研究的目的是分析处于两种临床控制状态下的21-OHD患儿的血清IGF-I、IGF-II和IGF结合蛋白（IGFBP）水平。
我们研究了12名4.0±0.7岁的青春期前21-OHD CAH患儿。根据生长速度、肾上腺功能不全或库欣综合征体征、性征进展及血清雄激素水平，将他们分为良好控制组（GC）或控制不佳组（PC）。在两种不同的临床控制状态（GC和PC）下，从每位患者采集血样进行生化检测。
采用免疫分析法测定IGF-I、IGF-II、IGFBP-1、IGFBP-2和IGFBP-3。还通过Western配体印迹法（WLB）分析IGFBP。
GC组的IGF-I水平（P = 0.03）和IGFBP-3水平（P = 0.01）高于PC组，而GC组患者的IGFBP-1浓度（P = 0.004）较低。在GC组儿童中观察到IGF-II水平有升高趋势（P = 0.08），IGFBP-2水平有降低趋势（P = 0.08）。在GC组儿童中观察到IGFBP-4条带强度增加（P = 0.03）。
在21-OHD CAH患儿中，较高的IGF-I、IGFBP-3和IGFBP-4水平，但较低的IGFBP-1水平，与更好的控制相关。这些发现与在其他导致雄激素水平升高的患儿中观察到的结果不同，可能与糖皮质激素状态不足有关。
