Hypothalamic adipsia without demonstrable structural lesion.
Hypothalamic adipsia without demonstrable structural lesion.
复制标题
下丘脑无明显的结构性病变。
作者：
A. Hayek;G. Peake
本文报告一名5岁男童罹患原发性高钠血症症候群之临床及实验室资料。在四年的随访中，没有发现明显的下丘脑结构性病变。回顾文献发现了四个类似的情况下，提出了一个独特的综合征改变下丘脑功能。该综合征的特征为：厌食-低饮（5/5例患者）、复发性高钠血症（5/5例）、肥胖（4/5例）、不能排泄水负荷（5/5例）、缺乏对刺激刺激的生长激素释放（4/4例）、促甲状腺激素释放激素反应迟钝（3/4例）、甲状腺功能减退（2/4例）和与高钠血症危象相关的高钠血症（1/1例）。其中一名患者的综合征被归因于阿片肽系统的紊乱。
The clinical and laboratory data of a 5-year-old boy with the syndrome of essential hypernatremia are presented. In a four-year follow-up, no demonstrable hypothalamic structural lesion has been identified. Review of the literature has uncovered four similar cases, suggesting a distinct syndrome of altered hypothalamic function. The syndrome is characterized by: adipsia-hypodipsia (5/5 patients), recurrent hypernatremia (5/5), obesity (4/5), inability to excrete a water load (5/5), lack of growth hormone release in response to provocative stimuli (4/4), blunted thyrotropin releasing hormone responses (3/4), hypothyroidism (2/4), and hyperlipemia associated with hypernatremic crisis (1/1). In one of the patients the syndrome has been attributed to a disturbance of the opioid-peptide system.