The incidence of endogenous Cushings syndrome (CS) is ~25 new cases per million people per year; ~10% of these new cases occur in children. The most common cause of endogenous CS in children is an ACTH-secreting pituitary adenoma (~75% of all cases in children older than 7 years). CS is still diagnostic and therapeutic challenge, especially in childhood. One of the most important diagnostic steps is thinking about it. The evaluation of children with the clinical manifestations of CS is more complex because many of the diagnostic tests are based on studies in adults, and then extrapolated for children. One major concern is the appropriate screening test for obese children to exclude CS. We report 16 years old obese girl (BMI>97.percentile), with hirsurtism, amenorrhea and short stature. She is referred the first time to pediatric endocrinologist after 3 years of ambulatory treatment for obesity. The first suspicion is raised after detail history and clinical examination. After differential diagnostic approach, diagnosis of morbus Cushing is established. The transsphenoidal surgery by experienced pituitary neurosurgeon performed. Follow up shown total success: appropriate weight, high velocity and normal menstrual cycle. This girl is the first child with CS in our institution. Besides all tests and neuroimaging techniques, this case reminds us that the old skills as detail history and clinical examination represent the most important diagnostic step in all conditions.
Prague, Czech Republic
24 - 28 Apr 2010
European Society of Endocrinology