矮身材儿童垂体MRI检查的临床意义

目的探讨矮身材儿童垂体MRI检查的应用价值。方法回顾性分析34例生长激素缺乏症(GHD)儿童和22例非生长激素缺乏性矮身材(ISS)儿童的临床及MRI检查结果,观察MRI检查中垂体形态及异常改变。结果GHD儿童垂体MRI检查发现垂体高度缩小21例(61.7%),垂体柄移位2例,ISS儿童发现垂体高度缩小5例(22.7%),GHD组异常检出率高于ISS组(χ2=4.5,P<0.05)。GHD儿童MRI异常组诊断时的年龄、身高(标准差)、骨龄和GH最大峰值均较MRI正常组偏低(t=2.23~3.76,P<0.05)。结论垂体MRI检查可协助矮身材儿童的诊断及垂体病变程度的评价。     

儿童矮小症67例病因和治疗分析

目的探讨儿童矮小症的病因与治疗特点,为临床诊断治疗提供参考依据。方法对67例儿童矮小症患儿的临床资料进行分析,并使用重组人生长激素（rh—GH）和其他相关治疗。结果67例矮小症儿童中,生长激素（GH）缺乏性矮小23例,特发性矮小21例,家族性矮小9例,体质性青春发育延迟4例,宫内发育迟缓3例,Turner综合征、甲状腺功能减退、21-三体综合征各2例,先天性软骨发育不良1例。rh—GH对生长激素缺乏症（GHD）疗效显著,对特发性矮小、家族性矮小、宫内发育迟缓效果满意,非GH缺乏甲状腺功能低下者予以甲状腺素片治疗,生长增速。结论矮小原因很多,及时发现病因,寻求最佳治疗手段非常重要。     

矮小儿童生长激素检测的临床意义分析

目的评价矮小儿童血清中的生长激素（GH）水平,了解生长激素缺乏（GHD）与特发性矮小（ISS）患儿生长激素激发试验后GH峰值分布的差异及临床意义。方法采用化学发光法对80例矮小儿童进行生长激素联合激发试验（可乐定,精氨酸）,检测用药前及用药后30、60、90、120min时的血清GH水平。以测得的GH最高值为峰值,峰值≥10μg/L为GH不缺乏,10μg/L〉峰值≥5μg/L为GH部分缺乏;峰值〈5μg/L为完全缺乏。结果 80例患儿中,GHD占45.0%,其中部分GHD占31.25%;GHD与ISS患儿无性别差异（P〉0.05）。激发后GH峰值出现的时间以0～120分钟为主,占总数的81.25%,激发试验的GH峰值出现时间ISS患儿与GHD患儿无差异（P〉0.05）。结论矮小儿童中由GHD缺乏造成的矮小所占比例较高,且以部分缺乏为主,ISS也是引起矮小的主要原因。生长激素联合激发试验GHD和ISS患儿GH峰值出现的时间段无差异。     

91例儿童血清IGF1水平测定的临床研究

目的 监测身材矮小儿童血清胰岛素样生长因子1（IGF1）水平与生长激素（GH）水平，探讨是否可以通过测定IGF1水平来替代GH激发试验诊断生长激素缺乏症（GHD）；方法 对91例儿童进行空腹血清IGF1水平测定，并对61例矮小儿童进行清晨空腹及可乐定，精氨酸＋左旋多巴激发试验血清GH水平测定，将GHD儿童与正常健康儿童的IGF1水平进行对照分析；结果 GH缺乏的矮小儿童的血清IGF1明显低于正常对照组，GH不缺乏的矮小儿童的血清IGF1与正常对照组无显著差异。结论 血清IGF1水平与GH水平相关，可作为GH分泌正常与否的初筛指标。     

生长激素治疗对GHD患儿免疫功能的影响

对生长激素缺乏症（GHD）患儿生长激素（GH）治疗前后的免疫功能改变进行了观察。结果显示：（1）GHD患儿NK细胞活性明显降低，经GH治疗3个月后恢复到正常水平；（2）GHD患儿治疗前IL－1a和IL－2活性偏低，治疗后两者有逐渐增高的趋势；（3）治疗前后CD细胞亚群、sIL－2R和LPS诱生的TNFa含量均无明显变化。认为GH缺乏症患儿存在一定的免疫功能缺陷，而GH有调节其免疫功能的作用。     

DIAGNOSTIC VALUE OF SERUM INSULIN—LIKE GROWTH FACTOR BINDING PROTEIN—3 IN CHILDREN WITH OR WITHOUT GROWTH HORMONE DEFICIENCY

Conventionally，thediagnosisofgrowthhormonedeficiency（GHD）requiresatleasttwogrowthhormone（GH）stimulationtestswithinsulin，arginine，orlev－odopaandsoon．Thesetestsareverycomplicated，andeachtestneedsfivebloodsamplestakenatdifferenttimeandtheresultsare     

生长激素缺乏儿童的酸碱平衡研究

目的 :探讨生长激素缺乏 (GHD)和特发性矮身材 (ISS)儿童血浆实际碳酸氢盐浓度 (AB)差异 ,以及碳酸氢盐标准差评分 (SDS)在 GHD诊断中的价值。方法 :对 4 7例矮小症儿童作血气分析及电解质测定及 GH激发试验 ,根据 GH激发试验的峰值分为 GHD和 ISS两组 ,比较两组血浆 AB、碳酸氢盐 SDS、阴离子间隙 (AG)的差异。结果 :GHD儿童血浆 AB、碳酸氢盐 SDS分别为 (2 2 .6 0± 1.2 9) mmol/L、- 0 .2 7± 0 .98,明显低于 ISS组 [(2 3.80±0 .95 ) mmol/L、0 .6 4± 0 .73],P<0 .0 1;而 AG GHD组为 (11.73± 4 .5 2 ) mmol/L高于 ISS组 [(7.87± 1.70 ) mmol/L],P<0 .0 1。在碳酸氢盐 SDS≤ 1s的矮小儿童中 ,72 % (31/4 3)患儿为 GHD。结论 :GHD儿童血浆 AB、碳酸氢盐SDS明显低于 ISS儿童 ,血浆碳酸氢盐 SDS≤ 1s者应高度怀疑 GHD。     

尿液生长激素测定对生长落后儿童的诊断意义

为正确评价生长落后患儿生长激素（ＧＨ）分泌情况，本文用放免法测定６７例受试者夜间１２ｈ尿ＧＨ含量。其中生长落后儿童３４例，包括经可乐定和左旋多巴两种刺激试验证实的ＧＨ缺乏患儿１２例和刺激试验正常的生长落后儿童２２例，另３３例为正常儿童。结果：１２例ＧＨ缺乏患儿尿ＧＨ排出量明显低于正常儿童，而刺激试验正常的生长落后儿童尿ＧＨ排出量则变动很大，约半数以上处于ＧＨ缺乏患儿同样的低水平状态。本资料表明，尿ＧＨ排出量测定可用以测定ＧＨ内源性分泌情况，是诊断ＧＨ缺乏症的一种非损伤性实用方法。     

吡啶斯的明对生长激素分泌及其释放激素的激发与增效作用

观察了吡啶斯的明(PD)对正常儿童、垂体性和下丘脑性生长激素(GH)缺乏病儿GH分泌的激发作用和对生长激素释放激素(GHRH)的增效作用。结果表明,PD有良好的激发GH分泌作用,可作为GH缺乏的筛选试验。PD对正常儿童和下丘脑性GH缺乏病儿有明显的增加GHRH引起的GH释放效应。PD可能对某些GH缺乏病儿及其他原因所致的身材矮小病儿有较好的治疗效果。     

EFFECTS OF CHINA-MADE RECOMBINANT HUMAN GROWTH HORMONE ON THE TREATMENT OF GROWTH HORMONE DEFICIENCY

Objective To evaluate the therapeutic effect of China-made recombinant human growth hormone (r-hGH) in children with growth hormone deficiency (GHD) and to investigate the utilities of various biochemical parameters in GHD diagnosis and treatment.Methods Our study comprises of 30 normal children and 71 GHD children treated with China-made r-hGH substitution 3 (IGFBP-3), bone turnover markers (Ost, ICTP), and anti-growth hormone antibody (GHAb) were detected before and after r-hGH treatment.Results After the first 3 and 6 months of treatment, growth velocities of GHD children were significantly increased (13.1 3.7 and 12.6 ± 3.6 cm/year) compared with pretreatment values (2.9 ± 0.8 cm/year, P ＜ 0.01). GHD Children had obviously reduced serum levels of IGF-1, IGFBP-3, and bone turnover markers (Ost, ICTP) compared with normal controls(P ＜ 0.01), and these biochemical parameters improved significantly after treatment (P ＜ 0.01). Growth hormone antibodies were positive in 17 of 45 cases after treatment by binding capacity detection. The binding percentage of growth hormone antibody which was increased more than 30% after the treatment showed a negative correlation with growth velocity (P ＜ 0.01).Conclusions (1) The growth stimulating effect and safety were confirmed in using China-made r-hGH in the treatment of GHD children for 6 months. (2) The measurements of serum IGF-1 and IGFBP-3 may serve as useful parameters in the diagnosis of GHD. (3) Serum Ost and ICTP are useful laboratory criteria for evaluating the effect of r-hGH therapy in the early stage. (4) It is necessary to monitor serum levels of GHAb during r-hGH therapy.     

儿童矮小症病因分析

目的:探讨儿童症的病因。方法:对50例矮小儿童进行全面的病史采集和体格检查及相关实验室检查。结果:生长激素缺乏症(growth hormone deficienty,GHD)20例,占40%;体质性青春发育延迟1例(2.0%)例;宫内发育迟缓3例(6.0%);营养不良性矮小4例(8.0%);原因不明5例(10.0%);Turner's氏综合征者6例(12.0%);铅中毒6例(12.5%);特发性矮小症5例(10.0%)。结论:矮小儿童病因中,内分泌疾病占大多数,其中以生长激素缺乏症最为多见。对矮小患儿应明确病因,给予对因治疗。     

Prevalence of growth hormone deficiency of children in Beijing.

103,753 (male 51,994, female 51,759) primary and middle school students aged 6-15 years in two districts in Beijing city were surveyed from October 1987 to April 1989. The heights of the students were measured. According to the height standard of northern cities in China, 202 students with heights below the 3rd percentile for age were requested for detailed history, physical examination, screening GH test bone age, T4, SGPT, chest X-ray, routine urine test and sex chromatin (in female). If GH less than 10 micrograms/L, two provocative tests (L-dopa or clonidine and insulin hypoglycaemia test) were done. Then the heights of the short students were observed for 1/2-2 years. GHD was diagnosed in 12 cases based on the GH peak levels less than 10 micrograms/L in two provocative tests, whose growth velocity was slower than that for students of the same age and sex. Of these subjects with GHD, total GHD (GH less than 5 micrograms/L) was present in 7 and partial GHD (GH = 5-9.9 micrograms/L) in 5. The 12 GHD students (male 9, female 3) aged 8.9-15.7 years accounted for 1/8,646 in the total surveyed students. The male and female GHD accounted for 1/5,777 and 1/17,253 in the total males and females respectively.

     

苏州地区矮小儿童164例病因及相关因素分析

目的对164例身材矮小儿童进行病因及其相关分析。方法对164例矮小儿童进行全面的病史采集和体格检查及相关实验室检查,另选20名健康儿童为对照组。结果164例中,特发性矮小（ISS）86例（占52.4%）,其中包括体质性青春发育延迟（CDGP）及家族性矮小（FSS）;生长激素缺乏症（GHD）57例（占34.8%）,其IGF-1及IGFBP-3水平较正常对照显著降低,差异有统计学意义（P〈0.01）;宫内发育迟缓者（IUGR）15例（占9.1%）;Turner综合征6例（占3.7%）。结论就诊的矮小儿童中以特发性矮小最为多见,其次为生长激素缺乏症;测定IGF-1及IGFBP-3水平可作为筛查和诊断GHD有价值的指标。对矮小患儿应明确病因,给予对因治疗。     

家族性矮小症GH1基因突变的检测

目的 检测家族性单纯性生长激素缺乏症(IGHD)及非GHD家族性矮小症患者GH1的基因突变。方法 用巢式聚合酶链式反应及限制性内切酶酶切方法,检测8例IGHD患者和17例非GHD家族性矮小症患者的GH1基因。结果 8例IGHD患者GH1基因均未发现已知突变317例非GHD家族性矮小症患者中,7例为GH1基因第二外显子G→A突变的杂合子。结论 GH1基因杂合突变能引起非GHD家族性矮小症的临床表型。     

矮小儿童943例的病因分析

目的探讨矮小儿童常见病因。方法对943例矮小患儿的病史、体格检查、相关实验室检查进行回顾性分析。结果内分泌疾病导致矮小868例,其中特发性矮小（ISS）501例（53.1%）,生长激素缺乏（GHD）240例（25.4%）,其他内分泌疾病127例（13.4%）;非内分泌疾病75例（7.9%）。结论矮小病因繁多,以ISS、GHD为常见,需通过相关实验室检查明确病因,以便及早治疗。     

Serum ghrelin and leptin levels in adult growth hormone deficiency syndrome

BACKGROUND: In spite of the increasing information that has recently been accumulated on the involvement of ghrelin and leptin in the control of energy balance, the relationship between ghrelin and leptin and the growth hormone (GH)-insulin-like growth factor 1 (IGF-1) axis in the pathological condition characterized by GH deficiency has been poorly clarified. Therefore, we performed this study to examine the correlation of the plasma levels of ghrelin and leptin with the anthropometric and biochemical markers in GH-deficient (GHD) adults as compared to their healthy cohorts. METHODS: In 60 male adults (GHD; n = 12, healthy control; n = 48, average age: 54 years), we investigated the correlations between the serum leptin and ghrelin levels with the anthropometric and biochemical factors in the control group, as compared to the GHD patients. The diagnosis of GH deficiency was made when peak response for serum GH was <5 microg/L to a GH-provocative test (L-dopa test). All subjects underwent assessment of waist circumference, body mass index (BMI) and percentage body fat for their body composition. Plasma ghrelin, leptin, insulin, GH and IGF-1 were measured. RESULTS: Groups were matched for age, BMI, waist circumference and percent of body fat. Ghrelin and leptin levels were not significantly different between the two groups. There was no correlation between the peak GH level or the GHAUC and the ghrelin concentrations in the GHD subjects. Plasma leptin correlated positively with percentage of body fat, total cholesterol and LDL-cholesterol, but it had no correlation with the peak GH or area under the curve for growth hormone (GHAUC) in the GHD subjects. Plasma ghrelin concentrations were not correlated with the biochemical and anthropometric markers in the subjects with GHD, and ghrelin showed no significant differences in the GHD and control subjects. Leptin concentrations were positively correlated with body fat, but they were not correlated with the levels of either IGF-1 or GH in the GHD patients. CONCLUSIONS: It is possible that ghrelin concentrations appeared normal in the GHD subjects because of the opposing influences of increased adiposity, which reduce ghrelin secretion, and GHD, which may increase it. Further studies are needed to clarify these controversies about the relation of ghrelin and leptin with the GH and IGF-1 levels.     

矮小儿童血清胰岛素样生长因子-1 和瘦素水平的表达及临床意义

目的了解不同病因的矮小儿童血清胰岛素样生长因子-1（IGF-1）和瘦素水平变化,探讨IGF-1和瘦素在生长激素缺乏症（GHD）诊断中的价值。方法选择深圳市妇幼保健院就诊的矮小儿童96例,年龄5-11岁,根据身高低于同性别同年龄均值-2～3SD,排除其他可致身材矮小疾病,根据生长激素激发试验,GH峰值〈10ng／mL为GHD者共67例（GHD组）、GH峰值≥10ng／mL为特发性矮小（ISS）者共29例（ISS组）,另选择同期体检的生长发育正常儿童23名作为对照组。采集所有研究对象的外周静脉血3mL,分离血清后,使用双抗体一步夹心法酶联免疫吸附实验（ELISA）测定血清IGF-1、瘦素浓度。结果GHD组血清IGF-1及瘦素水平[（45．7±19．02）μg／L、（4．25±0．63）mg／L]低于ISS组[（114．07±24．45）μg／L、（4．69±0．69）mg／L]和对照组[（164．61±46．22）μg／L、（6．27±0．89）mg／L]）,组间比较,差异均有高度统计学意义（P〈0．01）;诊断GHD,IGF-1的敏感度为100％,特异度为98％;瘦素的敏感度为88％,特异度为71％。结论IGF-1和瘦素的检测可能可作为筛查和诊断GHD有价值的指标,并对GHD与ISS的鉴别诊断也有着一定的临床意义。     

Growth hormone deficiency in adults and clinical use of recombinant human growth hormone

OBJECTIVE: To review the modern recognition of growth hormone deficiency (GHD) in adults and the beneficial effects of growth hormone (GH) treatment in such cases. DATA SOURCES AND METHODS: Most published original articles about GH and GHD in recent domestic and world wide related literatures were available. STUDY SELECTION: More than 65 originally identified articles were reviewed and 29 were selected that especially addressed the stated purpose. RESULTS: Treatment of GHD in adult human beings became an option following the development of rhGH and the numerous reports on the effect of rhGH therapy in such patients. The syndrome of GH deficiency in adults principally comprises abnormalities in body composition, cardiovascular risk factor, and psychological well-being. In comparison with normal individuals, these patients have increased total-body fat mass (particularly visceral adiposity), reduced muscle mass, reduced muscle strength exercise performance, and reduced bone mass. The psychological dysfunction comprises self-reported reduction in energy, mood, and sleep, along with objective reductions in marital and socioeconomic performance. CONCLUSION: The evidence that rhGH administration may be beneficial for the prevention, as well as treatment, of various clinical situations.     

生长激素缺乏症儿童血清瘦素、骨钙素水平变化及其相关性分析

目的 观察生长激素缺乏症（GHD）JL童血清瘦素（LEP）、骨钙素（OC）水平的变化及两者相关性分析。方法 分别测定32例GHD儿童重组人生长激素（rhGH）替代治疗前、治疗后3个月和35例性别、年龄匹配的正常健康儿童的血清LEP、OC，比较各组差异，并对LEP和OC进行相关性分析。结果 GHD治疗前组血清LEP水平显著高于正常对照组（P〈0．05），rhGH治疗3个月后LEP较基线值显著性降低。而GHD治疗前组血清OC水平显著低于正常对照组（P〈0．05），rhGH治疗3个月后OC较基线值显著性增高，血清LEP、OC无显著相关性（r=0．281，P〉0．05）。结论 GHD儿童LEP水平的改变依赖于GH引起的体脂量的变化；GHD儿童骨代谢的调控很可能与LEP无关。     

中山市723例矮小症的病因分析

目的探讨身材矮小儿童的病因。方法回顾性总结矮小儿童共723例,男433例（59．89％）,女290（40．11％）例,年龄2～19岁,平均年龄8．36岁,对其进行全面的病史采集和体格检查及相关实验室检查、影像学检查。结果引起矮小症的病因达19种,分别为：生长激素缺乏症（GHD）280例（38．7％）;生长激素神经分泌障碍（GHND）129例（17．8％）;病因分布比例在5％～10％的是特发性矮小、小于胎龄儿、甲状腺功能减低症、家族性矮小;性早熟、体质性青春发育延迟、Turner综合征、精神心理性身材矮小、锌缺乏症、地中海贫血所占比例在1％～5％;其余病因在723例患儿中所占比例小于1％。结论矮小症病因复杂,其中生长激素缺乏症是最常见疾病,对矮小症的发病因素需进一步的研究。