本文選題：聯(lián)合性垂體激素 + 缺乏癥��； 參考：《安徽醫(yī)科大學》2013年碩士論文

【摘要】：目的通過對患者門診、住院的病歷資料的研究，觀察兒童聯(lián)合性垂體激素缺乏癥(Combined Pituitary Hormone Deficiency,CPHD)的臨床特點，總結診斷、治療經(jīng)驗，，以指導兒科內(nèi)分泌的臨床工作。 方法將2010年5月～2012年6月之間就診我院的24例聯(lián)合性垂體激素缺乏癥兒童的病歷資料收集、整理，統(tǒng)計數(shù)據(jù)，分析兒童聯(lián)合性垂體激素缺乏癥的臨床特點、治療效果。這些患者均在初診時接受規(guī)范的生長激素激發(fā)試驗、甲狀腺功能、腎上腺皮質(zhì)功能、骨齡、蝶鞍部核磁共振、血生化等檢查，部分患者接受促性腺激素釋放激素類似物（Gonadotropin-releasing Hormone Analogues,GnRHa）激發(fā)試驗。部分患者接受藥物治療，其他有接受手術治療，少數(shù)單純隨訪。主要對藥物治療的患者進行治療后的評估。另外分別于治療前、治療后一年做簡化情緒量表（Simple Mood And Feeling Questionnaire,SMFQ），了解干預前后患者抑郁情緒的變化。 結果24例患者均存在兩種及以上垂體激素的缺乏。生長激素（GrowthHormone,GH）缺乏24例，促甲狀腺激素（Thyroid Stimulating Hormone,TSH）缺乏13例，促腎上腺皮質(zhì)激素（Adrenocorticotropic Hormone,ACTH）缺乏19例；15例行促性腺激素釋放激素類似物激發(fā)試驗，提示促性腺激素（Gonadotropin,GN）缺乏13例。生長激素缺乏占100%，其中大多數(shù)為完全缺乏型，另外促性激素缺乏占相當大的比例。全部患者表現(xiàn)有身材矮小，其他常見表現(xiàn)有青春發(fā)育延遲、外生殖器發(fā)育不良、智力低下等。異常出生史患者常見，其中出生窒息史8例，出生難產(chǎn)史5例，出生窒息史與難產(chǎn)史同時存在3例，早產(chǎn)1例，過期產(chǎn)并母妊娠高血壓病1例。激素替代治療后患者病情好轉(zhuǎn)，1例發(fā)生垂體危象。收集簡化情緒量表12份，因樣本量小，未進行統(tǒng)計學分析，仍在繼續(xù)收集過程中。 結論身材矮小的兒童，需進行系統(tǒng)的下丘腦-垂體-靶腺軸功能及相關影像學檢查，以明確診斷。有異常出生史的矮小患者是聯(lián)合性垂體激素缺乏癥的高發(fā)人群。激素替代治療后患者病情好轉(zhuǎn)。
[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.
【學位授予單位】：安徽醫(yī)科大學
【學位級別】：碩士
【學位授予年份】：2013
【分類號】：R725.8

【共引文獻】

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1 全會標;傅世華;陳文杰;李建軍;;垂體柄中斷綜合征1例[J];重慶醫(yī)學;2010年09期

2 張麗娜;梁立陽;孟哲;陳銳涵;李棟方;何展文;;新生兒垂體柄中斷綜合征1例[J];中國當代兒科雜志;2012年02期

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4 劉夢雨;馮逢;有慧;李明利;金征宇;;垂體柄阻斷綜合征的MRI表現(xiàn)[J];中國醫(yī)學影像學雜志;2011年05期

本文編號：1947207