Repolarization variability, evaluated by TWV, is independently related to the risk of death in ChD. This noninvasive methodology could facilitate the identification of patients who may benefit from more aggressive therapeutic strategies.

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Periodic fever with pharyngitis, aphthous stomatitis and cervical adenitis (PFAPA syndrome) is a common cause of periodic fever in children and usually manifests as episodes of fever recurring with a clockwork periodicity. Although rare after adolescence, adult patients with PFAPA syndrome may present with a wider range of symptoms and may lack the clockwork periodicity of fever. A 24-year-old patient presented with a 4-year history of periodic fever with pharyngitis and cervical adenitis. She also complained of vomiting, fatigue and sporadically presented with aphthous stomatitis. During crises, laboratory evaluation showed a moderate elevation of inflammatory markers. Blood cultures and ANA titres were negative. Immunoglobulins and serum ferritin levels were normal. After other causes of periodic fever had been excluded, a diagnosis of PFAPA syndrome was made. LEARNING POINTS PFAPA syndrome is characterized by periodic fever accompanied by pharyngitis, aphthous stomatitis and cervical adenitis. It is a common cause of periodic fever in children but can also present in adults with a wider range of clinical manifestations. Establishing a firm diagnosis of PFAPA syndrome may avoid excessive work-up and potentially harmful treatment.