Syncope is a common symptom, with 15% to 25% of children and adolescents experiencing at least one syncopal episode by young adulthood. In most cases, syncope is a symptom of benign diseases however may be a symptom of severe cardiac disease that results in sudden death. The purpose of this study is to analyze the etiologies and clinical characteristics of syncope in children and adolescents. We retroprospectively analyzed 51 patients with syncope. A total of 51 patients were included in the study and the ratio of males to female was 1: 1.04. The mean patient age was 12.7±3.1 years. Abrupt standing was the most common state of patients with syncopal attack. The etiologies of syncope were noncardiac syncope (60.7%), cardiac syncope (2%), neuropsychiatric syncope (25.5%), and unknown (11.8%). Abnormality of the head-up tilt test was more common in noncardiac syncope than the others [38.7% (12/31) vs 5% (1/20) (p=0.007)]. Further, EEG is a useful diagnostic test for neuropychiatric syncope [41.6% (5/12) vs 2.7% (1/36) (p=0.002)]. Therefore, detailed history taking and physical examinations were useful tools to diagnose the etiology of sycope. The head-up tilt test is an effective diagnostic test in noncardiac syncope and EEG is a valuable test in neuropychiatric syncope, especially in seizure.

XX male syndrome is a genetic disease associated with gender dysplasia and characterized by a spectrum of clinical presentations, which range from ambiguous to normal male genitalia. The incidence of XX male syndrome is about 1 per 20,000- 25,000. The majority of XX males with SRY gene have normal genitalia, whereas most SRY-negative cases have ambiguous genitalia. Here, we report a case of a SRY-negative XX male with ambiguous genitalia.