【摘要】：目的分析矮身材兒童的病因及治療效果,積累該類患兒的診療經(jīng)驗。 方法回顧性分析廣西醫(yī)科大學第一附屬醫(yī)院2011年1月至2012年12月就診的147例矮身材兒童的臨床資料,總結(jié)其病因及治療特點。 結(jié)果 (1)、符合納入標準矮身材兒童共147例,其中男98例(66.67%),女49例(33.33%)；年齡6月-14.7歲。 (2)、生長激素缺乏癥(GHD)92例,占62.59%；特發(fā)性矮小(ISS)28例,占19.05%；骨骼發(fā)育障礙8例,占5.44%；甲狀腺功能減退癥5例,占3.40%；Turner's綜合征4例,占2.72%；其他疾病共10例,占6.8%。 (3)、Turner's綜合征中,1例單體型體征典型,3例嵌合體型僅表現(xiàn)為矮身材。 (4)、130例患兒進行顱腦MRI檢查,異常者61例,占46.92%,垂體發(fā)育不良占異常者的90.16%。 (5)、GHD組和工SS組患兒就診時身高標準差積分(HtSDS)平均值分別為-3.05±1.38、-2.23±1.09,GHD組明顯矮于ISS組(P=0.006)；GHD組與ISS組IGF-1及IGFBP-3水平比較無明顯差異。4例身材極矮GHD患兒血清IGF-1、IGFBP-3水平極低。 (6)、40例患兒應用rhGH治療,GHD組34例,rhGH劑量為0.10IU/Kg/d,ISS組6例,rhGH劑量為0.15IU/Kg/d。垂體區(qū)域異常、治療前身高不影響患兒對rhGH治療反應的敏感性,GHD組與工SS組比較治療效果無明顯差異；rhGH治療前半年(0-6月)的身高增值、生長速度大于治療后半年(6-12月)；rhGH治療過程中,僅1例患兒使用rhGH后出現(xiàn)顏面部皮疹,伴瘙癢,未觀察到其他副作用。 結(jié)論 (1)、內(nèi)分泌疾病所致矮身材占大多數(shù),生長激素缺乏癥最多見,部分GHD及ISS患兒存在垂體發(fā)育不良。 (2)、外周血染色體核型分析對矮身材女童診斷有重要意義。 (3)、血清IGF-1、IGFBP-3水平明顯低于正常,有助于診斷嚴重的GHD,但依賴IGF-1、IGFBP-3水平測定無法鑒別GHD與工SS。 (4)、rhGH治療不良反應事件發(fā)生率低,安全性高。 (5)、矮身材兒童應明確病因,對因治療,并長期隨訪。
[Abstract]:Objective to analyze the etiology and therapeutic effect of children with short stature and to accumulate experience in diagnosis and treatment. Methods the clinical data of 147 children with short stature from January 2011 to December 2012 in the first affiliated Hospital of Guangxi Medical University were retrospectively analyzed and the etiology and treatment characteristics were summarized. Results (1) there were 147 children with short stature according to the inclusion criteria, including 98 males (66.67%) and 49 females (33.33%), aged from 6 months to 14.7 years old. (2) 92 cases of growth hormone deficiency (GHD), accounting for 62.59%, 28 cases of idiopathic dwarf (ISS) (19.05%). There were 8 cases of skeletal dysplasia (5.44%), 5 cases of hypothyroidism (3.40%), 4 cases of Turners syndrome (2.72%), 10 cases of other diseases, (3) one case of haplotype sign typical sign 3 cases of chimerism only showed short stature. (4) 130 cases of children with craniocerebral MRI were examined by craniocerebral MRI, 61 cases were abnormal. In 46.92 cases, pituitary dysplasia accounted for 90.16% of the abnormal ones. (5) the mean value of standard deviation (HtSDS) of height standard deviation in GHD group and SS group was -3.05 鹵1.38 鹵-2.23 鹵1.09g / d, respectively, which was significantly lower than that in ISS group (P0. 006). There was no significant difference in the levels of IGF-1 and IGFBP-3 between the GHD group and the ISS group. (6) there was no significant difference in the level of serum IGF-1,IGFBP-3 between the GHD group and the ISS group. (6) the serum IGF-1,IGFBP-3 level was very low in 40 patients with GHD. (6) the dose of rhGH was 0.10 IUU / kg / d in the rhGH group. There was no significant difference in the therapeutic effect between the rhGH group and the SS group. The growth rate was higher than that in the first half year (0-6 months), and the growth rate was higher than that in the first half year (6-12 months). In the course of rhGH treatment, only one child developed facial rash with itching and no other side effects after using rhGH. Conclusion (1) the majority of the patients with endocrine diseases are short stature, and the growth hormone deficiency is the most common. Some children with GHD and ISS have pituitary dysplasia. (2) the karyotype analysis of peripheral blood chromosome has important significance for the diagnosis of short girl. (3) the level of serum IGF-1,IGFBP-3 is significantly lower than that of normal. It is helpful to diagnose severe GHD, but can not distinguish between GHD and SS. (4). The incidence of adverse events is low and the safety is high. (5) Children with short stature should know the cause of the disease and follow up for a long time.
【學位授予單位】：廣西醫(yī)科大學
【學位級別】：碩士
【學位授予年份】：2013
【分類號】：R725.8

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本文編號：2222544