Segoe Hisato, Nakamura Akie, Uetake Kimiaki, Hishimura Nozomi, Kaneko Naoya, Morikawa Shuntaro, Nakamura-Utsunomiya Akari, Yamaguchi Takeshi
Department of Pediatrics, Obihiro-Kosei Hospital, Obihiro, Japan.
Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Clin Pediatr Endocrinol. 2024;33(3):163-168. doi: 10.1297/cpe.2024-0001. Epub 2024 Apr 22.
Adipsic hypernatremia is typically caused by congenital dysplasia of the hypothalamus and pituitary or brain tumors. However, cases of adipsic hypernatremia without underlying organic abnormalities are rare, and some cases have been reported to be complicated by hypothalamic-pituitary dysfunction. The patient in this case was a 9-yr-old boy who was referred to our hospital because of hypernatremia. His growth chart revealed that he had rapidly become obese since infancy, with growth retardation since the age of seven. His hands and feet were very cold, and he had erythema on his abdomen, indicating possible autonomic dysregulation due to hypothalamic dysfunction. Several hormone load tests showed severe GH deficiency (GHD) and marked hyperprolactinemia (peak: 302.8 ng/mL). Magnetic resonance imaging revealed no organic abnormalities in the hypothalamus and pituitary gland. GH replacement therapy was initiated. Although his growth rate improved, obesity persisted. To the best of our knowledge, this is the first report of adipsic hypernatremia without organic intracranial abnormalities that was treated with GH. Moreover, the patient's prolactin levels were higher than those reported in previous studies. In conclusion, adipsic hypernatremia requires the evaluation of pituitary function and appropriate therapeutic interventions.
无渴感高钠血症通常由下丘脑和垂体的先天性发育异常或脑肿瘤引起。然而,无潜在器质性异常的无渴感高钠血症病例较为罕见,且有一些病例据报道合并下丘脑 - 垂体功能障碍。本病例患者为一名9岁男孩,因高钠血症转诊至我院。他的生长图表显示,自婴儿期起他迅速肥胖,7岁起生长发育迟缓。他的手脚非常冰冷,腹部有红斑,提示可能因下丘脑功能障碍导致自主神经调节异常。多项激素负荷试验显示严重生长激素缺乏(GHD)和显著高催乳素血症(峰值:302.8 ng/mL)。磁共振成像显示下丘脑和垂体无器质性异常。开始进行生长激素替代治疗。尽管他的生长速度有所改善,但肥胖持续存在。据我们所知,这是首例无颅内器质性异常的无渴感高钠血症接受生长激素治疗的报告。此外,该患者的催乳素水平高于先前研究报道的水平。总之,无渴感高钠血症需要评估垂体功能并采取适当的治疗干预措施。