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Increased Liver Enzymes in Adult Women with Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency

机译:由于21羟化酶缺乏症的先天性肾上腺皮质增生的成年女性肝脏酶增加

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References(30) Cited-By(8) The aims were assessing liver function tests (LFT) in women with congenital adrenal hyperplasia (CAH) on glucocorticoids. Sixty-one women with genetically verified CAH due to 21-hydroxylase deficiency, aged 18-63 years were compared to 61 controls. Serum alkaline phosphatase (ALP), alanine aminotransferase (ALT ), aspartate aminotransferase (AST) and gamma-glutamyl transpeptidase (GGT), anthropometry and fat mass (dual energy X-ray absorptiometry) were measured. ALT and GGT were higher in the entire patient group (p=0.01and 0.002); AST, GGT and ALP in patients ≥30 years (p=0.007-0.045); all LFT in salt-wasting (p0.001-0.042); GGT in simple virilizing (p=0.008); ALT , GGT and ALP in Null/Null genotype (p=0.018-0.040); ALT and GGT in I2splice genotype (p0.001 and 0.011). Using a recently proposed cut-off level for ALT (0.317 μkat/L), 54% of patients vs 23% of controls had elevated levels (p=0.028). In patients, GGT and ALP correlated with waist circumference and with total body and trunk fat (r=0.274-0.406, p=0.001-0.043). However, ALT, GGT and ALP were increased even in non-obese patients (waist circumference ≤88 cm and body mass index 30 kg/m2) (p=0.012-0.045) mainly attributed to the patients ≥30 years who also demonstrated elevated insulin levels and HOMA-indices. In conclusion, compared with controls, women with CAH have higher LFT, in particular patients ≥30 years and those with severe forms, probably reflecting a higher lifetime glucocorticoid exposure. LFT were positively correlated to measurements of body fat. These women might have increased frequency of NAFLD. The finding of higher LFT also in non-obese patients suggests that not only central obesity but also glucocorticoids per se may influence.
机译:参考文献(30)(8)被引用目的是评估先天性肾上腺皮质激素增生(CAH)妇女的肝功能测试(LFT)。将61位因21-羟化酶缺乏症而经过基因验证的CAH,年龄18-63岁的妇女与61名对照进行了比较。测量血清碱性磷酸酶(ALP),丙氨酸转氨酶(ALT),天冬氨酸转氨酶(AST)和γ-谷氨酰转肽酶(GGT),人体测量法和脂肪量(双能X射线吸收法)。整个患者组的ALT和GGT均较高(p = 0.01和0.002); ≥30岁患者的AST,GGT和ALP(p = 0.007-0.045);所有LFT都在浪费盐(p <0.001-0.042); GGT在简单杀菌中(p = 0.008); Null / Null基因型的ALT,GGT和ALP(p = 0.018-0.040); I2剪接基因型中的ALT和GGT(p <0.001和0.011)。使用最近提议的ALT临界水平(> 0.317μkat/ L),有54%的患者相对于23%的对照组的患者具有升高的水平(p = 0.028)。在患者中,GGT和ALP与腰围以及全身和躯干脂肪相关(r = 0.274-0.406,p = 0.001-0.043)。但是,即使是非肥胖患者(腰围≤88cm,体重指数<30 kg / m2)(p = 0.012-0.045),ALT,GGT和ALP仍升高,这主要归因于≥30岁的患者,他们也表现出升高胰岛素水平和HOMA指标。总之,与对照组相比,患有CAH的女性的LFT较高,特别是≥30岁的患者和重症患者,可能反映了其终生糖皮质激素暴露的增加。 LFT与体内脂肪测量值呈正相关。这些妇女的NAFLD频率可能增加。在非肥胖患者中也发现较高的LFT,这表明不仅中枢性肥胖而且糖皮质激素本身也可能会影响。

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