Stagi Stefano, Del Greco Paolo, Ricci Franco, Iurato Chiara, Poggi Giovanni, Seminara Salvatore, de Martino Maurizio
Department of Health Sciences, University of Florence, Anna Meyer Children's University Hospital, Florence, Italy.
Ital J Pediatr. 2014 Sep 26;40:78. doi: 10.1186/s13052-014-0078-2.
Primary adrenal insufficiency is relatively rare in children and, if unrecognized, may present with cardiovascular collapse, making it a potentially life-threatening entity.
The proposita, 11 months old of age, was admitted for lethargy and severe dehydration. Blood pressure was 62/38 mm Hg, and biochemical measurements showed hyponatraemia, hypochloraemia, hyperkalaemia, and metabolic acidaemia. Renin activity was 1484 μU/mL; cortisol, 1.03 μg/dL (normal, 5-25 μg/dL); and corticotropin (ACTH), 4832 ng/L (normal, 9-52 ng/L). Adrenal deficiency was diagnosed, and replacement therapy with glucocorticoids and mineralocorticoids was initiated. After 40 days, ACTH was 797 ng/L. During follow-up, the patient started taking macrogol twice daily for constipation and experienced a significant increase in ACTH (3262 ng/L), which dropped to 648 ng/L when macrogol was stopped. After arbitrary reintroduction of macrogol, the child presented with hypoglycaemia, lethargy, weakness, and hypotonia; ACTH was 3145 ng/L. After again stopping macrogol, her ACTH was near normalized (323 ng/L).
Hydrocortisone malabsorption may be caused by macrogol use. Because chronic constipation is frequently reported in children, the possibility that macrogol contributes to adrenal crisis should be taken in account.
原发性肾上腺皮质功能减退症在儿童中相对罕见,若未被识别,可能会出现心血管衰竭,成为潜在的危及生命的病症。
本例患者为一名11个月大的婴儿,因嗜睡和严重脱水入院。血压为62/38 mmHg,生化检查显示低钠血症、低氯血症、高钾血症和代谢性酸血症。肾素活性为1484 μU/mL;皮质醇为1.03 μg/dL(正常范围为5 - 25 μg/dL);促肾上腺皮质激素(ACTH)为4832 ng/L(正常范围为9 - 52 ng/L)。诊断为肾上腺功能不全,并开始使用糖皮质激素和盐皮质激素替代治疗。40天后,ACTH为797 ng/L。在随访期间,患者开始每天服用两次聚乙二醇治疗便秘,之后ACTH显著升高(3262 ng/L),停用聚乙二醇后降至648 ng/L。在随意重新使用聚乙二醇后,患儿出现低血糖、嗜睡、虚弱和肌张力减退;ACTH为3145 ng/L。再次停用聚乙二醇后,其ACTH接近正常(323 ng/L)。
聚乙二醇的使用可能导致氢化可的松吸收不良。由于儿童中经常报告慢性便秘,应考虑聚乙二醇导致肾上腺危象的可能性。