矮身材儿童应用重组人生长激素治疗前后免疫状态的研究

垂体前叶分泌的生长激素（Growthhormone，GH）对儿童身高增长起关键作用。因垂体前叶生长激素分泌不足所致矮小者称为生长激素缺乏症（Growth hormone deficiency，GHD）。部分矮身材患儿导致矮身材的原因不明，称为非生长激素缺乏性矮小（non—GH deficient short stature，NGHDSS）或特发性矮小（idiopathic short stature，ISS）。在临床工作中我们发现矮身材的儿童，相当一部分易发生感染，食欲低下、体重较轻，而应用rhGH治疗后，食欲增加，体重身高增长满意，同时发生感染的机会明显减少。本研究通过观察32例GHD及15例ISS儿童应用国产rhGH治疗前后的免疫功能变化，探讨GH对免疫功能的调节及作为免疫增强剂应用的前景。     

136例住院矮身材儿童不同干预方法的临床观察

目的:探讨不同干预方法对地处苏北的淮安地区矮身材儿童的影响,为本地区儿童矮身材的防治提供科学依据。方法:回顾性分析南京医科大学附属淮安第一医院2020年12月至2021年12月收治的136例矮身材患儿的临床资料,通过统计学分析了解本地区矮身材患儿常见病因、诊疗情况及重组人生长激素治疗(rhGH)和生活方式干预的疗效差异等。结果:就诊的136例矮身材患儿,男女比例为1.72∶1,病因包括生长激素缺乏(GHD)(69.85%)、特发性矮身材(ISS)(24.26%)、小于胎龄(SGA)(5.15%)及甲状腺功能减退(0.74%)。48例患儿接受rhGH治疗;另87例患儿因家长原因未接受rhGH治疗,给予生活方式干预;1例甲状腺功能减退患儿接受甲状腺激素替代治疗。随访6个月后rhGH治疗组和生活方式干预组患儿身高、身高标准差积分(HtSDS)均明显改善(P<0.05),但rhGH治疗组患儿较生活方式干预组患儿HtSDS差异更大;与正常对照组相比,两组随访6个月后生长速率均明显增加。结论:本地区矮身材儿童就诊人群以男孩居多;病因以GHD及ISS为主;rhGH治疗患儿占比较低;rhGH与生...     

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。     

特发性中枢性性早熟女孩下丘脑垂体核磁共振和血清胰岛素样生长因子-1检测的临床意义

目的 探讨特发性中枢性性早熟(ICPP)女孩下丘脑垂体核磁共振(MRI)检查和血清胰岛素样生长因子-1(IGF-1)测定的临床诊断意义。方法 选取2007年2月至2009年12月在山东大学附属省立医院儿科内分泌门诊就诊的ICPP女孩患者60例,其中早期ICPP组8例,典型ICPP组36例,ICPP合并生长激素缺乏(GHD)组16例,收集所有患者空腹血清。3组均采用放射免疫法(RIA)测定空腹血清IGF-1和生长激素(GH)水平。免疫化学发光法(ICMA)测定空腹血清促卵泡刺激素(FSH)、促黄体生成素(LH)、 雌二醇(E2)、促性腺激素释放激素类似物(GnRHa)刺激后峰值LH及峰值LH/FSH,ICPP合并GHD组加做生长激素(GH)刺激实验。行下丘脑垂体部位的头颅MRI(测量垂体前叶高度并根据Elster方法将其分级)、妇科B超、骨龄(BA)等检查。另外选取30例青春前期女孩作为正常对照组。对3组的上述指标进行比较,分析所测定的血清IGF-1、E2、基值LH、基值LH/FSH、峰值LH、峰值LH/FSH、骨龄与实足年龄差值(BA-CA)与垂体前叶高度的相关性。结果 早期ICPP组血清E2、峰值LH、峰值LH/FSH、CA、BA-CA均显著低于典型ICPP组(P均＜0.01);早期ICPP组和典型ICPP组的垂体前叶高度及血清IGF-1均显著高于正常对照组(P＜0.01),早期ICPP组及典型ICPP组比较无明显差异(P＞0.05); GnRHa对早期ICPP有显著治疗效果;血清中IGF 1、E2、基值LH、基值LH/FSH、峰值LH、峰值LH/FSH、BA-CA分别与垂体前叶高度之间存在显著的正相关(r=0.65,0.54,0.54,0.45,0.58,0.57,0.45,P均＜0.05);典型ICPP组BA-CA、垂体前叶高度、IGF-1均显著高于ICPP合并GHD组(P＜0.01),峰值LH及峰值LH/FSH显著高于ICPP合并GHD组(P＜0.05);垂体前叶高度及IGF-1在ICPP合并GHD组均显著低于正常对照组(P＜0.01)。结论 下丘脑垂体MRI和血清IGF-1对ICPP女孩的早期和鉴别诊断具有重要临床意义,下丘脑垂体MRI与下丘脑-垂体-靶腺轴联系密切。     

IGF-Ⅰ和IGFBP-3在诊断儿童生长激素缺乏症中的价值

目的:评价IGF-Ⅰ和IGFBP-3在诊断GHD中的价值.方法:采用化学发光免疫法检测60例青春发育前期矮小儿童的血清生长激素(GH),酶联免疫法(ELISA)检测IGF-Ⅰ和IGFBP-3.根据GH峰值≤5 μg/L为完全性生长激素缺乏(CGHD),＞5 μg/L～10 μg/L为部分性生长激素缺乏,＞10 μg/L为特发性矮身材(ISS),并与20例健康儿童对照.结果:CGHD组IGF-Ⅰ和IGFBP-3值较其它三组明显降低(P＜0.05).CGHD患儿IGF-Ⅰ的敏感性为100%,特异性65.6%,试验正确性80%;IGFBP-3的敏感性为96.4%,特异性71.8%,试验正确性83.3%,IGF-Ⅰ和IGFBP-3同时检测,其敏感性为96.4%,特异性75%,试验正确性85%.结论:GHD者IGF-Ⅰ低于正常,尤以IGFBP-3为著,两者同步检测较单独检测更具诊断的正确性.     

南通地区矮身材儿童不同病因的运动、学习状况调查研究

目的：探究南通地区矮身材儿童常见病因及运动、学习状况,并讨论两者之间的关系。方法回顾性分析108例矮身材儿童的相关资料,通过详尽病史采集、体格检查、实验室检查,诊断矮小以明确病因。采用病例对照研究,对108例矮身材儿童进行运动、学习状况的问卷调查。应用多因素Logistic 回归分析、列联系数分析等统计学方法,分析研究南通地区矮身材儿童的运动和学习情况,及其与病因的相关性。对照组按年龄成组匹配的原则选出具有代表性的108名儿童。结果在确诊的108例矮身材患儿中,生长激素缺乏症(GHD)61例,占56．5%；非生长激素缺乏矮身材(NG-HD)47例,占43．5%。多因素 Logistic 回归分析显示,参加体育锻炼则为保护因素；好动不安且注意力不集中为危险因素,经常好动不安且注意力不集中所起作用更大( P <0．001,OR =7．483,95% CI =2．620~21．374)；常见病因与运动和学习情况之间无显著相关性(P >0．05)。结论各种类型的运动(篮球、羽毛球、跑步、骑脚踏车)均为儿童矮身材的保护因素,好动不安且注意力不集中为儿童矮身材的危险因素；无论是否存在生长激素缺乏,矮身材儿童都应当尽量增加正确的运动,避免或减少上述危险因素的暴露。     

垂体MRI诊断小儿矮小症的价值观察

目的 研究垂体磁共振成像(MRI)诊断小儿矮小症的应用价值。方法 回顾性分析2018年7月至2019年9月本院临床诊断为矮小症的121例患儿的临床资料,均进行垂体MRI检查及生长激素激发试验检查,汇总分析MRI检查结果 及生长激素激发试验结果 。结果 121例患儿中垂体未见异常有72(59.50%)例,垂体异常有49(40.50%)例,其中垂体发育不良35例(其中合并空蝶鞍4例,Rathke囊肿1例),单纯性Rathke囊肿13例,颅咽管瘤1例。生长激素水平未见异常19例,部分性生长激素缺乏61例,缺乏41例。MRI检查垂体未见异常情况下,生长激素部分缺乏或缺乏率为80.6%,MRI检查垂体发育不良情况下,生长激素部分缺乏或缺乏率为85.7%,MRI检查垂体病变(Rathke囊肿、肿瘤)情况下,生长激素部分缺乏或缺乏率为100%。结论 垂体MRI在诊断小儿矮小症中有良好应用效果,可协助患儿诊断及病因的了解。     

不同生长激素分泌状态的矮身材儿童血脂水平的研究

目的 探讨不同生长激素分泌状态下矮身材儿童血脂水平的差异,为生长激素缺乏对儿童体脂代谢的影响提供理论依据。方法 收集矮身材儿童188例,依据生长激素药物激发试验峰值分为生长激素完全缺乏(cGHD)组、生长激素部分缺乏(pGHD)组、非生长激素缺乏性(nGHD)组,研究对象均禁食禁水10 h后空腹测定总胆固醇(TC)、三酰甘油(TG)、低密度脂蛋白胆固醇(LDL)和高密度脂蛋白胆固醇(HDL)4项血脂水平。结果 3组儿童HDL水平比较差异无统计学意义(P>0.05);3组儿童TC、TG、LDL、non-HDL水平比较差异均有统计学意义(P<0.05);组间两两比较,cGHD组TC、LDL、non-HDL水平较其他组明显升高(P<0.05),cGHD组TG水平较nGHD明显升高(P<0.05),与pGHD组比较差异无统计学意义(P>0.05)。pGHD、nGHD组高TC、高LDL及高non-HDL的发生率明显低于cGHD组(P<0.05),nGHD组临界高LDL的发生率明显低于cGHD组(P<0.05)。而3组间TG、HDL的异常发生率及TC、TG...     

IGF-1生成试验在ISS患儿生长激素治疗前的疗效评估

目的:探讨IGF-1生成试验在ISS患儿生长激素治疗前的疗效评估,以期为ISS患儿的治疗提供一定的理论依据。方法:选取2012年1月-2015年1月期间于本院经左旋多巴激发试验及精氨酸激发试验诊断为ISS及GHD的青春期前患儿共80例,其中ISS患儿50例,GHD患儿30例。对50例ISS患儿进行IGF-1生成试验,根据IGF-1结果,将IGF-1增长2倍及以上者分为ISS-A组(30例),增长不足2倍者分为ISS-B组(20例)。将30例GHD设为GHD组。对三组患者均予以重组人生长激素治疗2年,比较三组患者的疗效。结果:治疗后,第一年三组患儿增加的平均身高比较差异无统计学意义(P0.05);第二年ISS-A组与GHD组患儿增加的平均身高分别为(9.12±1.58)、(9.23±1.66)cm,明显高于ISS-B组(7.18±1.43)cm,比较差异有统计学意义(P0.05),ISS-A组与GHD组间比较差异无统计学意义(P0.05)。ISS-A组与GHD组的身高SDS分别为(-2.11±0.28)、(-2.06±0.31),明显高于ISS-B组(-2.45±0.39),比较差异有统计学意义(P0.05),ISS-A组与GHD组间比较差异无统计学意义(P0.05)。ISS-A组与GHD组的IGF-1水平分别为(328.64±58.47)、(332.59±60.83)ng/mL,明显高于ISS-B组(279.83±50.63)ng/mL,比较差异有统计学意义(P0.05),ISS-A组与GHD组间比较差异无统计学意义(P0.05)。三组患者的BMI及IGFBP-3水平比较差异均无统计学意义(P0.05)。结论:重组人生长激素治疗IGF-1生成试验中IGF-1增长2倍及以上患儿的临床疗效与GHD患儿差异不明显,但显著优于IGF-1增长不足2倍的患儿,故IGF-1生成试验在ISS患儿生长激素治疗前的疗效评估中具有重要意义。     

IGF-1和IGFBP-3在生长激素缺乏的矮小儿童诊断中的意义和价值

目的 探讨胰岛素样生长因子1(IGF-1)和胰岛素样生长因子结合蛋白3(IGFBP-3)在诊断儿童生长激素缺乏中的价值.方法 采用化学发光法检测50例正常儿童,52例生长激素缺乏(GHD)患儿及45例特发性矮小(ISS)患儿的血清IGF-1和IGFBP-3的水平并进行两两比较.结果 GHD组、ISS组和正常儿童组间比较均有显著性差异(P＜0.05).CGHD患儿IGF-1的敏感性为95.5%,特异性73.1%,试验的正确性83.5%;IGBP-3的敏感性为97.7%,特异性82.6%,试验的正确性89.7%.IGF-1和IGFBP-3同时检测的敏感性为95.5%,特异性84.6%,试验的正确性89.7%.结论 GHD患儿的IGF-1和IGFBP-3均低于正常,两者同时检测比单独检测可能更具有诊断的正确性,可以作为诊断GHD的指标之一.     

Turner 综合征垂体MRI 特征 及与激素、染色体的关系

目的 分析Turner 综合征患儿的垂体磁共振成像（MRI）表现特征及与相关激素、染色体的关系, 探讨垂体改变的原因。方法 选取2010 年6 月－ 2018 年6 月于青岛大学附属医院神经内分泌儿科诊治的43 例 Turner 综合征患儿,回顾性分析垂体MRI 大小与相关激素、染色体的相关性。结果 患儿垂体MRI 多见垂 体正常（22 例,51.2%）和垂体增大（18 例,41.9%）;垂体正常组促卵泡激素、黄体生成素低于垂体增大组 （P <0.05）,雌二醇高于垂体增大组（P <0.05）。结论 部分Turner 综合征患儿垂体形态异常,主要表现为垂 体增大,可能与靶腺（卵巢）功能低下引起负反馈抑制作用减弱,导致垂体代偿性增生有关。     

空蝶鞍综合征20例的临床和MRI分析

分析２０例空蝶鞍综合征（ＥＳＳ）的ＭＲＩ表现和其中１６例的内分泌改变。结果１６例ＥＳＳ均有内分泌功能异常。ＭＲＩ表现有蝶鞍充满长Ｔ＿１和长Ｔ＿２的脑脊液;垂体受压至鞍底,冠状位呈“锚”状,矢状位呈“新月”状;垂体柄延长。结果表明ＥＳＳ常具有内分泌功能紊乱,ＭＲＩ对ＥＳＳ具有特征性诊断意义。     

儿童垂体疾病的MRI特征性表现与临床分析

目的探讨儿童垂体疾病特征性MRI表现,提高对儿童垂体疾病的影像学认识和诊断水平。方法回顾分析420匆垂体1．5T检查患儿,垂体有特征性形态、信号改变29例,分析其影像学及临床表现。结果垂体柄阻断12例,甲减致垂体增生5例,LCH浸润垂体4例,空泡蝶鞍综合征7例,垂体巨腺瘤1例。结论MRI能准确显示儿童垂体形态、信号改变,临床与影像特点相结合可以正确诊断该类病变,为早期诊断和治疗提供帮助。     

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.     

MEASUREMENTS OF URINARY HUMAN GROWTH HORMONE IN SUBJECTS WITH NORMAL AND ABNORMAL GROWTH:A CRITICAL OF ITS POTENTIAL CLINICAL UTILITY

ＭＥＡＳＵＲＥＭＥＮＴＳＯＦＵＲＩＮＡＲＹＨＵＭＡＮＧＲＯＷＴＨＨＯＲＭＯＮＥＩＮＳＵＢＪＥＣＴＳＷＩＴＨＮＯＲＭＡＬＡＮＤＡＢＮＯＲＭＡＬＧＲＯＷＴＨ：ＡＣＲＩＴＩＣＡＬＯＦＩＴＳＰＯＴＥＮＴＩＡＬＣＬＩＮＩＣＡＬＵＴＩＬＩＴＹＣｈｅｎｇＹｏｕｈｏ...     

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。     

MAGNETIC RESONANCE IMAGING CHARACTERISTICS AND SURGICAL RESULTS OF ADRENOCORTICOTROPIN-SECRETING PITUITARY ADENOMAS

Objective To evaluate magnetic resonance imaging （MRI） characteristics and surgical results of adrenocorticotropin （ACTH） -secreting pituitary adenomas. Methods MRI characteristics and relationship between MRI positive rate and surgical results of 266 patients with pathologically confn＇med Cushing＇s disease were analyzed retrospectively. All patients underwent thin-section sagittal and coronal scans of the pituitary gland before and after administration of gadolinium-diethylene triamine pentaacetic acid （Gd-DTPA） on a 1. 5 Tesla MR/scanner, and dynamic enhanced MR/was performed in 39 patients. All patients underwent transsphenoidal adenomectomy. Endocrinological examinations and assessments were performed. Results Preoperative MR/revealed normal results in 41 （ 15.4% ） cases, microadenoma in 179 （67. 3% ）, macroadenoma in 42 （ 15.8% ）, and huge adenoma in 4 （ 1.5% ）. Pituitary apoplexy was found in 13 （4.9%） cases. Positive rate of ACTH-secreting adenomas was 84.6% （225/266） on MR/scans, and that of small microadenomas was 87.2% （34/39） on dynamic enhanced MRI scans. Preoperative endocrinological tests of 199 cases supported the diagnosis of typical Cushing＇s disease, while the other 67 cases had atypical endocrinological results. The endocrinological cure rate, remission rate, and inefficacy rate were 85.7%, 7.9%, and 6. 4%, respectively. There was no difference in the initial endocrinological cure rate between the patients with positive and normal MRI results （90% vs. 87. 8%, P =0. 904）. Conclusions Enhanced coronal pituitary MR/ is helpful for preoperative localization of ACTH-secreting pituitary microadenoma. Dynamic enhanced MR/may improve detection rate of microadenoma. There is no marked difference in the surgical results for patients with preoperative MR/results indicating presence or absence of microadenoma.     

Abnormal enlargement of pituitary gland during pregnancy remitted spontaneously after delivery

Ovulation induction is seldom reported to cause pituitary abnormality and the physiological enlargement of the pituitary gland during pregnancy is asymptomatic. We report a woman who became pregnant after ovulation induction. She had symptomatic pituitary enlargement with a pituitary height of 2 cm on cranial magnetic resonance imaging (MRI) in the second trimester. Her symptoms (headache and blurred vision) improved greatly after delivery but she had absence of lactation. The height of the pituitary gland decreased to 1 cm on the second cranial MRI 40 days after delivery and her third cranial MRI only revealed a mild central bulge in the pituitary gland eight months after delivery. This article shows that the abnormal enlargement of the pituitary gland during pregnancy might remit spontaneously after delivery and these patients might have higher risk of postpartum hypopituitarism. Neurological symptoms and signs should be closely monitored during pregnancy and neurosurgery may not be necessary. Endocrine surveys should be performed regularly after delivery to detect hypopituitarism as early as possible.     

MAGNETIC RESONANCE IMAGES OF THE HYPOTHALAMIC-PITUITARY AREA IN IDIOPATHIC GROWTH DEFICIENCY

In order to describe the magnetic resonance imaging (MRI) findings in hypothalamic-pituitary area and its clinical relevance in patients with idiopathic growth hormone deficiency (IGHD), the MR imagings of 26 patients with IGHD were analyzed. On MRI, 24 out of 26 cases (92. 3%) showed apparent pituitary upper margin depression; 8 out of 26 cases (30. 8%) showed definite pituitary stalk transection; 22 out of 26 cases (84. 6%) showed absence of the normal posterior pituitary bright spot. The bright lipidlike signal on T1W1 images at the median eminence distal to the breaking point (so-called ectopic posterior lobe) was found in 4 out of 26 cases (15. 4%). According to the MRI findings of the pituitary stalks, the 26 cases were divided into three groups; group A of 8 cases (31%) characterized by the definite transaction of stalk; group B of 13 cases (50%) defined by the possible stalk transection; and group C of 5 cases (19%) with no definite stalk transection.MRI findings were consistent with the clinica