关键词: GH deficiency adipsic hypernatremia hyperprolactinemia hypothalamic-pituitary dysfunction obesity

来  源:   DOI:10.1297/cpe.2024-0001   PDF(Pubmed)

Abstract:
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.
摘要:
腹水性高钠血症通常由下丘脑和垂体或脑肿瘤的先天性发育不良引起。然而,没有潜在器质性异常的脂肪性高钠血症病例很少见,据报道,一些病例并发下丘脑-垂体功能障碍。本例中的患者是一个9岁的男孩,由于高钠血症而被转诊到我们医院。他的成长图显示他从婴儿期开始就迅速变得肥胖,从7岁开始发育迟缓。他的手和脚很冷,他的腹部有红斑,表明下丘脑功能障碍可能导致自主神经失调。几项激素负荷测试显示严重的GH缺乏症(GHD)和明显的高催乳素血症(峰值:302.8ng/mL)。磁共振成像显示下丘脑和垂体无器质性异常。开始GH替代疗法。虽然他的增长率有所提高,肥胖持续存在。据我们所知,这是用GH治疗的无器质性颅内异常的脂肪性高钠血症的首例报告.此外,患者的催乳素水平高于之前研究报告的水平.总之,脂肪性高钠血症需要评估垂体功能和适当的治疗干预措施。
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