Dr. von Hauner Children’s Hospital; Ludwig Maximilian University, Department of Paediatrics, Division of Paediatric Endocrinology, Munich, Germany
Ludwig Maximilian University, Department of Medicine IV, Munich, Germany
J Clin Res Pediatr Endocrinol. 2023 Nov 22;15(4):380-389. doi: 10.4274/jcrpe.galenos.2023.2023-3-5. Epub 2023 May 23.
Patients with congenital adrenal hyperplasia (CAH) require lifelong therapy with glucocorticoids to suppress androgen excess and substitute for deficient cortisol. An important aspect of care is the prevention of metabolic sequelae. In infants, potentially lethal nocturnal hypoglycaemia has been described. In adolescence, visceral obesity, hypertension, hyperinsulinism and insulin resistance are reported. To date, systematic studies of glucose profiles in this age group with CAH are lacking.
This was a monocentric, prospective, observational study to determine the glucose profiles under different treatment regimens in a cohort of young patients with CAH. The continuous glucose monitoring device used was the latest generation FreeStyle Libre 3 sensor in blinded mode. Therapeutic and auxological data were obtained.
The cohort consisted of 10 children/adolescents with a mean age of 11 years. Three patients exhibited morning fasting hyperglycaemia. Overall, 6 out of 10 patients had unacceptably few total values in the desired range of 70-120 mg/dL. Tissue glucose values above 140-180 mg/dL were found in 5 of 10 patients. The mean value for glycosylated haemoglobin for the cohort was of 5.8%. All pubertal adolescents with reverse circadian regimens had significantly higher glucose levels at night. Two adolescents showed asymptomatic nocturnal hypoglycaemia.
Most of the patients exhibited abnormalities in glucose metabolism. Two-thirds had elevated total 24h glucose values outside the age-appropriate reference values. Thus, this aspect may need to be addressed early in life by adjusting the doses, treatment regimen or dietary measures. Consequently, reverse circadian therapy regimens should be critically indicated and closely monitored due to the potential metabolic risk.
患有先天性肾上腺皮质增生症（CAH）的患者需要终身接受糖皮质激素治疗以抑制雄激素过多并替代皮质醇缺乏。护理的一个重要方面是预防代谢后遗症。在婴儿中，已描述了潜在致命的夜间低血糖。在青春期，报道了内脏肥胖，高血压，高胰岛素血症和胰岛素抵抗。迄今为止，缺乏针对该年龄段 CAH 患者的葡萄糖谱的系统研究。
这是一项单中心，前瞻性，观察性研究，旨在确定在 CAH 年轻患者队列中不同治疗方案下的葡萄糖谱。使用的连续葡萄糖监测设备是盲法的最新一代 FreeStyle Libre 3 传感器。获得了治疗和生长数据。
该队列包括 10 名患有 CAH 的儿童/青少年，平均年龄为 11 岁。有 3 名患者出现清晨空腹高血糖。总体而言，10 名患者中有 6 名患者的总数值在 70-120mg/dL 的理想范围内无法接受。10 名患者中有 5 名患者的组织葡萄糖值高于 140-180mg/dL。该队列的平均糖化血红蛋白值为 5.8%。所有具有逆昼夜节律方案的青春期青少年夜间血糖水平均显着升高。两名青少年出现无症状性夜间低血糖。
大多数患者的葡萄糖代谢异常。三分之二的患者的 24 小时总葡萄糖值高于相应年龄的参考值。因此，通过调整剂量，治疗方案或饮食措施，可能需要尽早解决这一方面。因此，由于潜在的代谢风险，应慎重指示并密切监测逆昼夜节律治疗方案。
