Electrolyte abnormalities and stress dosing predict illness-related hospitalizations among infants and toddlers with congenital adrenal hyperplasia.
Electrolyte abnormalities and stress dosing predict illness-related hospitalizations among infants and toddlers with congenital adrenal hyperplasia.
复制标题
电解质异常和应激剂量可预测患有先天性肾上腺增生的婴儿和幼儿因疾病相关的住院治疗。
DOI：
10.1111/cen.14876
复制
发表时间：
2023
影响因子：
3.2
通讯作者：
Kim,MimiS
中科院分区：
文献类型：
--
作者：
Tseng,Teresa;Seagroves,Amy;Tanawattanacharoen,VeerayaK;Liang,MarkC;Koppin,ChristinaM;Keenan,Madison;Davidowitz,Elana;Nguyen,Eugene;Chand,Sanjay;Geffner,MitchellE;Chang,ToddP;Kim,MimiS
目的：婴幼儿先天性肾上腺增生症（CAH）是危及生命的肾上腺危象的高危发病/死亡人群。CAH急性疾病的管理需要了解在生命的最初几年导致急诊科（ED）访问和住院的因素。因此，我们检查了患有CAH的幼儿院前和ED阶段的肾上腺危机与医疗结果的关系。患者和设计回顾性队列研究39例因21 -羟化酶缺乏而患有CAH的儿童（0-4岁）和27例年龄匹配的对照组。测量访问，急性疾病症状（发烧，呕吐，腹泻）和其他特征（住院，服用应激剂量氢化可的松，电解质异常）。结果scah婴幼儿ED就诊率（0.50[0.25-0.88]/人-年）显著高于对照组（0[0 - 0]/人-年；p< .001）。此外，6个月以下的CAH儿童与年龄较大的儿童相比，ED就诊率明显更高。在CAH患儿中，只有50%（51/102）的疾病相关ED就诊前由父母给予口服（46/51）或肌肉注射（11/51）压力给药。共有10.8%的急诊科就诊导致住院。控制诊断时的年龄和17 -羟孕酮，电解质异常和肠外注射氢化可的松显著预测ED住院。在ED前接受氢化可的松注射是电解质异常的重要预测因子。结论典型CAH的婴幼儿急性疾病和住院的风险较高，且通常在ED前没有得到足够的应激剂量。
ObjectiveInfants and toddlers with classical congenital adrenal hyperplasia (CAH) are at high risk for morbidity/mortality arising from life‐threatening adrenal crisis. Management of acute illnesses in CAH requires an understanding of factors leading to emergency department (ED) visits and hospitalizations in the first few years of life. We, therefore, examined adrenal crisis at prehospital and ED stages of illness in young children with CAH as they related to medical outcomes.Patients and DesignRetrospective cohort study of 39 children with CAH due to 21‐hydroxylase deficiency (0–4 years of age) and 27 age‐matched controls.MeasurementsED visit, acute illness symptoms (fever, vomiting, diarrhoea) and other characteristics (hospitalizations, administration of stress‐dose hydrocortisone, electrolyte abnormalities).ResultsCAH infants and toddlers had significantly higher rates of ED visits (0.50 [0.25–0.88] per person‐year) than controls (0 [0–0] per person‐year;p< .001). Moreover, CAH children under 6 months old had significantly higher rates of ED visits compared with older ages. Only 50% (51/102) of illness‐related ED visits in CAH children were preceded by the administration of either oral (46/51) or intramuscular (11/51) stress dosing by parents. A total of 10.8% of ED visits resulted in hospital admission. Controlling for age and 17‐hydroxyprogesterone at diagnosis, electrolyte abnormalities and administration of parenteral hydrocortisone in the ED significantly predicted hospital admission. Receiving a hydrocortisone injection before the ED was a significant predictor of having electrolyte abnormalities.ConclusionsInfants and toddlers with classical CAH are at high risk for acute illness and hospitalizations and often do not receive adequate stress dosing before the ED.