Kurnaz Erdal, Erdeve Şenay Savaş, Özgür Senem, Keskin Melikşah, Özbudak Pınar, Çetinkaya Semra, Aycan Zehra
Pediatric Endocrinology Clinic, Dr. Sami Ulus Obstetrics and Gynecology and Pediatrics Training and Research Hospital, Ankara, Turkey.
Pediatric Cardiology Clinic, Dr. Sami Ulus Obstetrics and Gynecology and Pediatrics Training and Research Hospital, Ankara, Turkey.
Turk J Pediatr. 2019;61(5):791-793. doi: 10.24953/turkjped.2019.05.022.
Kurnaz E, Savaş Erdeve Ş, Özgür S, Keskin M, Özbudak P, Çetinkaya S, Aycan Z. Congenital long-QT syndrome in type 1 diabetes: a unique association. Turk J Pediatr 2019; 61: 791-793. In contrast to acquired long QT syndrome (LQTS), congenital LQTS is a relatively rare channelopathy with an incidence of 1/2,500. We describe a patient found to have a prolonged QTc in the setting of newly diagnosed Type 1 DM. To the best of our knowledge, this unique association has not been previously reported. Currently, it is shown that glucose ingestion aggravated cardiac repolarization disturbances in LQT2 patients and prolonged the cardiac repolarization phase in healthy controls. Our case presented to the hospital with syncope after increased glucose level. Therefore, it seems that increased glucose level may have prolonged QTc interval and aggravated cardiac repolarization disturbances in the presented case. By this report, we want to emphasize the importance of hyperglycaemia in congenital LQTS.
库尔纳兹E、萨瓦斯·埃尔德韦Ş、奥兹居尔S、凯斯金M、奥兹布达克P、切廷卡亚S、艾扬Z。1型糖尿病中的先天性长QT综合征：一种独特的关联。《土耳其儿科学杂志》2019年；61: 791 - 793。与获得性长QT综合征（LQTS）不同，先天性LQTS是一种相对罕见的通道病，发病率为1/2500。我们描述了一名在新诊断的1型糖尿病背景下发现QTc延长的患者。据我们所知，这种独特的关联此前尚未见报道。目前，研究表明，摄入葡萄糖会加重LQT2患者的心脏复极紊乱，并延长健康对照者的心脏复极期。我们的病例在血糖水平升高后因晕厥入院。因此，在该病例中，血糖水平升高似乎可能延长了QTc间期并加重了心脏复极紊乱。通过本报告，我们想强调高血糖在先天性LQTS中的重要性。
