库欣综合征的定义是长期接触糖皮质激素,导致发病率和死亡率过高。由于临床体征的特异性低,这种罕见病理的诊断很困难,临床表现的不同严重程度,以及与诊断方法相关的解释困难。法国内分泌学会和法国儿科内分泌学和糖尿病学学会的38位专家的本共识论文首先旨在详细说明建议诊断的情况和生物学诊断工具,以及它们对阳性诊断和根据ACTH独立和依赖机制进行病因诊断的解释。其次,使诊断复杂的情况(怀孕,强烈的皮质醇增多症,波动库欣综合征,儿科形式和遗传确定的形式)进行了详细说明。最后,最后一节讨论了复发的监测和诊断方法。
Cushing\'s syndrome is defined by prolonged exposure to glucocorticoids, leading to excess morbidity and mortality. Diagnosis of this rare pathology is difficult due to the low specificity of the clinical signs, the variable severity of the clinical presentation, and the difficulties of interpretation associated with the diagnostic methods. The present
consensus paper by 38 experts of the French Society of Endocrinology and the French Society of Pediatric Endocrinology and Diabetology aimed firstly to detail the circumstances suggesting diagnosis and the biologic diagnosis tools and their interpretation for positive diagnosis and for etiologic diagnosis according to ACTH-independent and -dependent mechanisms. Secondly, situations making diagnosis complex (pregnancy, intense hypercortisolism, fluctuating Cushing\'s syndrome, pediatric forms and genetically determined forms) were detailed. Lastly, methods of surveillance and diagnosis of recurrence were dealt with in the final section.