Department of Pediatric Neurology, Faculty of Medicine, Akdeniz University, Antalya, Turkey.
Horm Res Paediatr. 2010;74(6):444-8. doi: 10.1159/000315477. Epub 2010 Oct 29.
Melatonin, a major photoperiod-dependent hormone, regulates circadian rhythms and biological rhythms and acts as a prominent sleep promoter. Symptoms related to hypermelatoninemia have been reported in individuals supplemented with melatonin. However, spontaneous endogenous hypermelatoninemia has not been reported previously. A 6-year-old girl previously diagnosed with Shapiro's syndrome was admitted to our hospital on several occasions during a 1-year period with complaints of altered consciousness, syncope, hypothermia and episodes of sweating. The episodes occurred daily and during sleep and lasted for 1-6 h. During these episodes, she sweated profusely and felt faint and her skin was pale and cool. Other complaints included recurrent abdominal pain, urge incontinence and myopia. She was shown to have hypermelatoninemia (>1,000 pg/ml, normal range 0-150 pg/ml) during these episodes. The duration of her attacks decreased with phototherapy and she was successfully treated with propranolol. To our knowledge, this is the first case of hypermelatoninemia without any detectable organic pathology. We did not determine the exact mechanism of hypermelatoninemia in this patient; however, it might have been related to irregular control of pinealocytes by the suprachiasmatic nucleus or related pathways. Hypermelatoninemia should be considered in patients with spontaneous periodic hypothermia and hyperhidrosis, and also in patients with Shapiro's syndrome.
褪黑素是一种主要的光周期依赖性激素，它调节昼夜节律和生物节律，并作为一种重要的睡眠促进剂。在补充褪黑素的个体中，已经报道了与高褪黑素血症相关的症状。然而，以前没有报道过自发性内源性高褪黑素血症。一名 6 岁女孩曾被诊断患有 Shapiro 综合征，在 1 年期间因意识改变、晕厥、体温过低和出汗发作多次入住我院。这些发作每天都发生，在睡眠中发生，持续 1-6 小时。在此期间，她大量出汗，感到头晕，皮肤苍白且凉爽。其他症状包括反复发作的腹痛、尿急失禁和近视。在这些发作期间，她的褪黑素水平升高（>1000pg/ml，正常范围 0-150pg/ml）。随着光疗的进行，她的发作持续时间缩短，并用普萘洛尔成功治疗。据我们所知，这是首例无任何可检测到的器质性病变的高褪黑素血症病例。我们没有确定该患者高褪黑素血症的确切机制；然而，它可能与松果体细胞的不规则受下丘脑视交叉上核或相关通路的控制有关。自发性周期性体温过低和多汗症患者，以及 Shapiro 综合征患者，都应考虑高褪黑素血症的可能性。
