Padidela Raja, Hindmarsh Peter C
London Centre for Paediatric Endocrinology and Metabolism, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH, UK.
Int J Pediatr Endocrinol. 2010;2010:656925. doi: 10.1155/2010/656925. Epub 2010 May 4.
Approximately 75%-80% of patients with Congenital Adrenal Hyperplasia (CAH) fail to synthesize sufficient mineralocorticoids to maintain salt and water balance. In most instances genotype can predict mineralocorticoid deficiency in CAH. Early recognition and replacement with 9alpha-fludrocortisone and salt supplements will prevent development of potentially lethal salt losing crises. In infancy a relative state of aldosterone resistance exists and replacement dose of 9alpha-fludrocortisone based on body surface area is higher during infancy compared to childhood and adults. Salt supplementation is generally not required after weaning is started. Regular monitoring of blood pressure and measurements of plasma electrolytes and renin are required to prevent complications of under or over dosage.
大约75%-80%的先天性肾上腺皮质增生症（CAH）患者无法合成足够的盐皮质激素来维持水盐平衡。在大多数情况下，基因型可以预测CAH患者的盐皮质激素缺乏情况。早期识别并用9α-氟氢可的松和补充盐分进行替代治疗，可预防潜在致命的失盐危象的发生。在婴儿期，存在相对的醛固酮抵抗状态，与儿童及成人相比，基于体表面积的9α-氟氢可的松替代剂量在婴儿期更高。开始断奶后通常不需要补充盐分。需要定期监测血压以及测量血浆电解质和肾素水平，以预防剂量不足或过量的并发症。
