Abstract
the association with foregut symptoms. Methods: We conducted a retrospective review of the medical notes and electronic patient records for all children diagnosed with JHS (Beighton score >4/9) referred to the syncope service based at Hammersmith Hospital. The study period was June 2011 to October 2013. Cardiovascular autonomic function was evaluated using the tilt table test. Results: A total of 53 children were recruited with a median age range of 15.2 yrs (range 8.1yrs-18.7 yrs). The gender distribution was 28 females (53%) and 25 males (47%). All patients had autonomic nervous system-related symptoms including postural dizziness or syncope (53%), palpitations (40%), migraine (25%) and chest pain (19%). Of the 53 JHS patients with autonomic symptoms, 48 (91%) patients had GI-related symptoms. 45 (85%) of these patients had confirmed cardiovascular autonomic dysfunction on tilt testing. All these patients also complained of foregut symptoms, predominantly nausea and bloating. Summary: Foregut dysfunction was a co-morbid presentation in all JHS patients with cardiovascular dysautonomia. Conclusion: Children with JHS presenting with foregut symptoms are likely to have associated cardiovascular autonomic dysfunction. Recognition of these symptoms by paediatricians is important as formal evaluation and treatment of the dysautonomia may improve the quality of life for these patients. Further studies are required to evaluate the objective improvement of GI symptoms following treatment for dysautonomia.
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