儿童联合性垂体激素缺乏症临床特点观察

发布时间：2018-05-28 15:15

本文选题：联合性垂体激素 + 缺乏症　；参考：《安徽医科大学》2013年硕士论文

【摘要】：目的通过对患者门诊、住院的病历资料的研究，观察儿童联合性垂体激素缺乏症(Combined Pituitary Hormone Deficiency,CPHD)的临床特点，总结诊断、治疗经验，，以指导儿科内分泌的临床工作。方法将2010年5月～2012年6月之间就诊我院的24例联合性垂体激素缺乏症儿童的病历资料收集、整理，统计数据，分析儿童联合性垂体激素缺乏症的临床特点、治疗效果。这些患者均在初诊时接受规范的生长激素激发试验、甲状腺功能、肾上腺皮质功能、骨龄、蝶鞍部核磁共振、血生化等检查，部分患者接受促性腺激素释放激素类似物（Gonadotropin-releasing Hormone Analogues,GnRHa）激发试验。部分患者接受药物治疗，其他有接受手术治疗，少数单纯随访。主要对药物治疗的患者进行治疗后的评估。另外分别于治疗前、治疗后一年做简化情绪量表（Simple Mood And Feeling Questionnaire,SMFQ），了解干预前后患者抑郁情绪的变化。结果24例患者均存在两种及以上垂体激素的缺乏。生长激素（GrowthHormone,GH）缺乏24例，促甲状腺激素（Thyroid Stimulating Hormone,TSH）缺乏13例，促肾上腺皮质激素（Adrenocorticotropic Hormone,ACTH）缺乏19例；15例行促性腺激素释放激素类似物激发试验，提示促性腺激素（Gonadotropin,GN）缺乏13例。生长激素缺乏占100%，其中大多数为完全缺乏型，另外促性激素缺乏占相当大的比例。全部患者表现有身材矮小，其他常见表现有青春发育延迟、外生殖器发育不良、智力低下等。异常出生史患者常见，其中出生窒息史8例，出生难产史5例，出生窒息史与难产史同时存在3例，早产1例，过期产并母妊娠高血压病1例。激素替代治疗后患者病情好转，1例发生垂体危象。收集简化情绪量表12份，因样本量小，未进行统计学分析，仍在继续收集过程中。结论身材矮小的儿童，需进行系统的下丘脑-垂体-靶腺轴功能及相关影像学检查，以明确诊断。有异常出生史的矮小患者是联合性垂体激素缺乏症的高发人群。激素替代治疗后患者病情好转。
[Abstract]:Objective to observe the clinical characteristics of combined pituitary hormone deficiency (CPHD) and summarize the experience of diagnosis and treatment in pediatric endocrinology through the study of medical records of outpatient and inpatient patients in order to guide the clinical work of pediatric endocrine. Methods the medical records of 24 children with combined pituitary hormone deficiency from May 2010 to June 2012 were collected, sorted and statistically analyzed. The clinical features and therapeutic effects of the combined pituitary hormone deficiency in children were analyzed. At first visit, these patients underwent standard growth hormone stimulation tests, thyroid function, adrenal cortex function, bone age, Sella turcica MRI, blood biochemistry, etc. Some patients received gonadotropin-releasing Hormone Analogues-GnRHa-stimulation test. Some patients received drug therapy, others received surgical treatment, and a few patients were followed up. The patients treated with drugs were evaluated after treatment. In addition, a simple Mood And Feeling questionnaire was made before and one year after treatment to understand the changes of depression in patients before and after intervention. Results there were two or more kinds of pituitary hormone deficiency in 24 patients. There were 24 cases of growth hormone (GH) deficiency, 13 cases of thyroid stimulating hormone (Thyroid Stimulating) deficiency, and 19 cases of adrenocorticotropic hormone (Adrenocorticotropic Hormotropic ACTH) deficiency, which suggested that gonadotropin (Gonadotropin GNN) was deficient in 13 cases. Growth hormone deficiency accounted for 100%, most of which were completely deficient, and sex hormone deficiency accounted for a large proportion. All patients showed short stature, other common manifestations were youth delay, external genitalia dysplasia, mental retardation and so on. Abnormal birth history was common in 8 cases of birth asphyxia, 5 cases of dystocia, 3 cases of birth asphyxia and dystocia, 1 case of premature delivery and 1 case of pregnancy hypertension. One patient developed pituitary crisis after hormone replacement therapy. Twelve samples of simplified emotion scale were collected. Because of small sample size and no statistical analysis, the collection process is still in progress. Conclusion the function of hypothalamus-pituitary-target gland axis and related imaging examination should be carried out in children with short stature to make a definite diagnosis. Dwarf patients with a history of abnormal birth are associated with a high incidence of pituitary hormone deficiency. The patient's condition improved after hormone replacement therapy.
【学位授予单位】：安徽医科大学
【学位级别】：硕士
【学位授予年份】：2013
【分类号】：R725.8

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