Al-Rayess Heba, Fleissner Kristin, Jaber Mu'taz, Brundage Richard C, Sarafoglou Kyriakie
Department of Pediatrics, Division of Endocrinology, University of Minnesota, Minneapolis, Minnesota.
Department of Experimental and Clinical Pharmacology, University of Minnesota, Minneapolis, Minnesota.
J Endocr Soc. 2020 Jul 5;4(8):bvaa091. doi: 10.1210/jendso/bvaa091. eCollection 2020 Aug 1.
Currently there are no commercially available hydrocortisone formulations for the treatment of children with congenital adrenal hyperplasia (CAH) that allow for smaller doses (0.1-1.25 mg) and incremental adjustments needed to control excess androgen production and avoid the negative effects of overtreatment. This lack of availability has led physicians to recommend dividing hydrocortisone 5-mg tablets into 4 to 6 pieces, compounding capsules or hydrocortisone suspension, or crushing 5- or 10-mg tablets in 5 or 10 mL of water. We report a case of iatrogenic Cushing syndrome in a 6-year 11-month-old girl with salt-wasting CAH treated with hydrocortisone tablets that were administered after crushing and dispersing into water to obtain the prescribed dose. She presented with poor growth, increasing body mass index (BMI), excess downy hair, round facies, and gastric ulcers. Her hydrocortisone dose was 8.1 mg/m/day. Results for all adrenal steroid concentrations were undetectable at 8 am, 12 hours after her last dose. The year prior to presentation her parents began dissolving 10 mg of hydrocortisone in 10 mL of water and using this preparation over the course of 24 hours, which coincided with rapid increase of BMI. We switched her to a pharmacy-compounded alcohol-free hydrocortisone suspension with total daily doses ranging from 6.5 to 8.2 mg/m/day, which resulted in resolution of her cushingoid features, a decrease in BMI, and catch-up growth. Our case highlights that manipulation of hydrocortisone tablets by parents can result in great variability in dosing and the need for commercially available pediatric formulations allowing for smaller dosing required in young children.
目前，尚无用于治疗先天性肾上腺皮质增生症（CAH）患儿的市售氢化可的松制剂，这些制剂需要较小剂量（0.1 - 1.25毫克）并进行递增调整，以控制雄激素分泌过多并避免过度治疗的负面影响。由于缺乏此类制剂，医生建议将5毫克氢化可的松片剂分成4至6片、配制胶囊或氢化可的松混悬液，或将5毫克或10毫克片剂碾碎于5或10毫升水中。我们报告了一例医源性库欣综合征病例，患儿为一名6岁11个月大的患有失盐型CAH的女孩，使用碾碎并溶于水后服用的氢化可的松片剂进行治疗，以获得规定剂量。她出现生长发育迟缓、体重指数（BMI）增加、汗毛过多、满月脸和胃溃疡。她的氢化可的松剂量为8.1毫克/平方米/天。末次服药12小时后的上午8点，所有肾上腺类固醇浓度检测结果均为未检出。在出现症状的前一年，她的父母开始将10毫克氢化可的松溶于10毫升水中，并在24小时内使用该制剂，这与BMI的快速增加同时出现。我们将她改用药房配制的无酒精氢化可的松混悬液，每日总剂量为6.5至8.2毫克/平方米/天，这使她的库欣样特征得到缓解，BMI下降，并实现了追赶生长。我们的病例强调，家长对氢化可的松片剂的处理可能导致给药剂量差异很大，并且需要有市售的儿科制剂，以满足幼儿所需的较小剂量。
