Endocrine, cardiac and neuropsychological aspects of adult congenital adrenal hyperplasia

IF 3 3区 医学 Q2 ENDOCRINOLOGY & METABOLISM
Lukas Ochsner Ridder, Camilla Mains Balle, Anne Skakkebæk, Marie Lind-Holst, Mette Mølby Nielsen, Pernille Hermann, Stinus Hansen, Dorte Guldbrand Nielsen, Sine Knorr, Niels Holmark Andersen, Mette Hansen Viuff, Agnethe Berglund, Claus Højbjerg Gravholt
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引用次数: 0

Abstract

Objective

To investigate the metabolic, cardiovascular, and neuropsychological phenotype, quality of life (QoL), and hormonal regulation in individuals with congenital adrenal hyperplasia (CAH), a group of autosomal recessive disorders characterized by impaired synthesis of cortisol in the adrenal cortex and, if untreated compensatory hyperandrogenism. CAH is associated with an increased cardiovascular and metabolic morbidity, possibly due to overtreatment with glucocorticoids, leading to weight gain, insulin resistance, and metabolic syndrome.

Design, Participants, Measurements

Thirty-seven individuals with CAH and 33 age- and sex-matched controls were evaluated at a single centre at Aarhus University Hospital with echocardiography, electrocardiogram, 24-h blood pressure, biochemistry, anthropometrics, and autism spectrum, anxiety, depression, personality, cognitive failures, and QoL were assessed using questionnaires.

Results

CAH individuals had lower height than controls (170.5 vs. 182.9 cm in males and 160.2 vs. 170.1 cm in females, p < 0.01). Compared with female controls, females with CAH had higher haemoglobin (8.8 vs. 8.2 mmol/L, p = 0.003) and BMI (29.7 vs. 25.5 kg/m2, p = 0.006), reduced insulin sensitivity (HOMA-IR): 2.7 vs. 1.9, p = 0.018), prolonged E-wave deceleration time (193 vs. 174 cm, p = 0.015), and E/é ratios (5.4 vs. 4.5, p = 0.017), and lower self-reported QoL. Males with CAH had more cognitive complaints (p = 0.034) and higher autistic scores (19.9 vs. 14.9; p = 0.068) compared with male controls. More individuals with CAH than controls reported writing problems.

Conclusion

A sex-specific comorbidity profile is evident in CAH, with females presenting with decreased metabolic and overall self-reported health, whereas males with CAH presented with increased cognitive complaints and autistic traits.

Abstract Image

成人先天性肾上腺皮质增生症的内分泌、心脏和神经心理学问题
目的研究先天性肾上腺皮质增生症(CAH)患者的代谢、心血管和神经心理学表型、生活质量(QoL)和激素调节。CAH 与心血管和新陈代谢发病率增加有关,这可能是由于过度使用糖皮质激素导致体重增加、胰岛素抵抗和新陈代谢综合征。设计、参与者、测量在奥胡斯大学医院的一个中心对 37 名 CAH 患者和 33 名年龄和性别匹配的对照组进行了评估,包括超声心动图、心电图、24 小时血压、生物化学、人体测量,并使用问卷对自闭症谱、焦虑、抑郁、人格、认知失败和 QoL 进行了评估。结果 自闭症患者的身高低于对照组(男性为 170.5 厘米对 182.9 厘米,女性为 160.2 厘米对 170.1 厘米,p < 0.01)。与女性对照组相比,患有 CAH 的女性血红蛋白(8.8 对 8.2 mmol/L,p = 0.003)和体重指数(29.7 对 25.5 kg/m2,p = 0.006)较高,胰岛素敏感性降低(HOMA-IR):2.7 vs. 1.9,p = 0.018),E 波减速时间延长(193 vs. 174 cm,p = 0.015),E/é 比值降低(5.4 vs. 4.5,p = 0.017),自我报告的 QoL 降低。与男性对照组相比,CAH 男性患者有更多的认知抱怨(p = 0.034)和更高的自闭症评分(19.9 vs. 14.9; p = 0.068)。结论 CAH明显存在性别特异性合并症,女性患者的新陈代谢和整体自我报告健康状况下降,而男性患者的认知抱怨和自闭症特征增加。
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来源期刊
Clinical Endocrinology
Clinical Endocrinology 医学-内分泌学与代谢
CiteScore
6.40
自引率
3.10%
发文量
192
审稿时长
1 months
期刊介绍: Clinical Endocrinology publishes papers and reviews which focus on the clinical aspects of endocrinology, including the clinical application of molecular endocrinology. It does not publish papers relating directly to diabetes care and clinical management. It features reviews, original papers, commentaries, correspondence and Clinical Questions. Clinical Endocrinology is essential reading not only for those engaged in endocrinological research but also for those involved primarily in clinical practice.
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