神经生长因子-酪氨酸激酶A途径参与人体体温和应激调节:对遗传性感觉异常和自发性Ⅳ型神经病变患者的研究
Nerve growth factor- tyrosine kinase A pathway is involved in thermore gulation and adaptation to stress: Studies on patients with hereditary sensory and autonomic neuropathy type IV作者机构：Pediatric Department Soroka Medical Center POB 151 Beer Sheva 84101 Israel Dr.
出 版 物：《世界核心医学期刊文摘（儿科学分册）》 (Dkgest of the World Latest Medical Information)
年 卷 期：2005年第1卷第8期
页 面：60-61页
学科分类：1002[医学-临床医学] 100204[医学-神经病学] 10[医学]
主 题：感觉异常 神经生长因子 人体体温 内分泌系统 TrkA 内分泌腺体 水平比对 高亲和力受体 肾上腺功能 低体温
摘 要：Hereditary sensory and autonomie neuropathy type IV (HSAN IV) is caused by mu tations in the tyrosin kinase A (TrkA) gene, encoding for the high- affinity re ceptor of nerve growth factor (NGF). The NGF- TrkA system is expressed in many endocrine glands. We hypothesized that HSAN IV represents a natural model for im paired NGF effect on the neuroendocrine system in humans. We have documented the clinical outcome of 31 HSAN IV patients in a single medical center, and investi gated their basal endocrine system status. The endocrine system response to thir st was compared between six patients and six healthy children. High rates of mor tality (22% ) and severe morbidity (30% ) have been found in HSAN IV patients. Hypothermia was noted in 40% of the patients and unexplained fever was observ ed in 56% . Subnormal adrenal function was demonstrated in six (30% ) of the p atients studied. Furthermore, we found lower plasma norepinephrine (NE) levels i n six HSAN IV patients compared with a control group after the thirst test. Our findings emphasize the importance of NGF- TrkA pathway in the physiology of the neuroendocrine system and its response to stress. Inadequate response to stress might contribute to the observed significant mortality, morbidity, and temperat ure instability in HSAN IV patients.
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