新生儿早发型败血症预后不良的预测因素

目的 探讨新生儿早发型败血症（EOS）预后不良的预测因素。方法 收集371例EOS新生儿的临床资料,根据预后分为预后良好组（264例）和预后不良组（107例）。比较两组患儿的围生期特点、临床表现、实验室指标、合并症、治疗过程等,采用多因素logistic回归模型分析EOS预后不良的预测因素。结果 预后不良组患儿的出生体重、胎龄均低于预后良好组（P < 0.05）;预后不良组的早产、低出生体重、极低出生体重及双胎比例高于预后良好组（P < 0.05）;预后不良组母亲产前使用激素、产前使用抗生素比例高于预后良好组（P < 0.05）;预后不良组反应差和呼吸功能低下的发生率高于预后良好组,皮肤黄染发生率低于预后良好组（P < 0.05）;预后不良组WBC<5×10⁹/L、血小板<100×10⁹/L、贫血、凝血功能障碍、肾功能损害、肝功能损害、低蛋白血症及低血糖的发生率高于预后良好组（P < 0.05）;预后不良组新生儿呼吸窘迫综合征、肺出血、坏死性小肠结肠炎、脑出血、脑损伤、肺动脉高压及休克的发生率高于预后良好组（P < 0.05）;预后不良组住院时间和抗生素疗程长于预后良好组（P < 0.05）;预后不良组使用机械通气和血管活性药的比例高于预后良好组（P < 0.05）。多因素logistic回归分析显示,极低出生体重（OR=41.734）、坏死性小肠结肠炎（OR=12.669）、脑损伤（OR=8.372）、休克（OR=5.889）、机械通气（OR=5.456）及肝功能损害（OR=4.075）是新生儿EOS预后不良的独立预测因素（P < 0.05）。结论 极低出生体重、机械通气及合并坏死性小肠结肠炎、脑损伤、休克或肝功能损害对新生儿EOS预后不良有预测价值。     

川崎病患儿治疗前后血清脂源性细胞因子的变化及意义

目的 检测急性期川崎病患儿静脉注射丙种球蛋白（IVIG）治疗前后血清中脂源性细胞因子Omentin-1和Chemerin浓度变化及意义。方法 选取2015年1月至2019年4月确诊为川崎病患儿60例为研究对象,同时选取40例健康儿童和40例急性感染性疾病患儿分别作为健康对照组和感染对照组。根据是否对IVIG治疗敏感将川崎病患儿分为IVIG敏感组（n=51）和IVIG不敏感组（n=9）;根据是否合并冠状动脉损害（CAL）将川崎病患儿分为合并CAL组（n=13）和不合并CAL组（n=47）。ELISA法检测川崎病患儿IVIG治疗前后及两对照组儿童血清Omentin-1和Chemerin水平。结果 川崎病患儿血清Omentin-1和Chemerin水平均明显高于健康对照组和感染对照组（P < 0.05）。经过48 h治疗后,IVIG敏感组患儿血清Chemerin水平较治疗前明显降低（P < 0.05）,血清Omentin-1水平在IVIG敏感组患儿治疗前后比较差异无统计学意义（P > 0.05）。治疗前,IVIG不敏感组患儿血清Chemerin水平明显高于IVIG敏感组（P < 0.05）;合并CAL组血清Chemerin水平明显高于不合并CAL组;而血清Omentin-1水平在IVIG敏感与不敏感组间及合并CAL与不合并CAL组间比较差异均无统计学意义（P > 0.05）。结论 川崎病患儿血清中高表达的Chemerin和Omentin-1可能参与川崎病的发生和发展;Chemerin可能参与川崎病所致CAL过程,且血清Chemerin水平可能成为临床预测IVIG敏感性的新监测指标。     

上下气道呼出气一氧化氮与哮喘控制水平的相关性研究

目的 探讨联合检测呼出气一氧化氮（FeNO）及鼻呼出气一氧化氮（FnNO）的临床价值及其与哮喘控制水平的关系。方法 选取2018年1~6月诊断为哮喘并处于慢性持续期的患儿120例为研究对象。所有患儿进行儿童哮喘控制测试（C-ACT）,得分 > 23分为控制组,20~23分为部分控制组,≤ 19分为未控制组,每组各40例;同时依据哮喘患儿有无合并过敏性鼻炎分为未合并鼻炎组（n=55）和合并鼻炎组（n=65）;同期收集健康体检儿童40例为对照组。哮喘患儿及对照组儿童均检测FeNO与FnNO水平。结果 不同水平控制组内FeNO值以未控制组最高,部分控制组其次,控制组最低（P < 0.05）,其中未控制组及部分控制组FeNO值高于对照组（P < 0.05）;未控制组与部分控制组FnNO值高于完全控制组及对照组（P < 0.05）,完全控制组FnNO值高于对照组（P < 0.05）。合并鼻炎组FeNO与FnNO值均高于未合并鼻炎组（P < 0.05）。结论 FeNO可用来评估哮喘控制情况,与FnNO联合可评估上下气道炎症情况,为上下气道联合治疗提供依据。     

血红蛋白H病患儿血清可溶性转铁蛋白受体水平的研究

目的 分析小儿血红蛋白H病（HbH病）血清可溶性转铁蛋白受体（sTfR）水平,探讨其与HbH病贫血程度的关系。方法 纳入55例HbH病患儿以及30例正常健康儿童（对照组）为研究对象,回顾性分析缺失型、非缺失型HbH病组以及对照组的血液学指标与血清sTfR水平。结果 55例HbH病中,缺失型39例,非缺失型16例。缺失型与非缺失型HbH组血红蛋白（Hb）、平均红细胞体积（MCV）、平均血红蛋白含量（MCH）均低于对照组（P < 0.05）,血清sTfR水平均高于对照组（P < 0.05）。非缺失型HbH病组红细胞（RBC）、Hb水平低于缺失型HbH病组（P < 0.05）,而MCV、MCH以及血清sTfR水平高于缺失型HbH病组（P < 0.05）。HbH病患儿血清sTfR水平与RBC、Hb水平呈负相关（r分别为-0.739、-0.667,均P < 0.05）;而与MCV、MCH呈正相关（r分别为0.750、0.434,均P < 0.05）。结论 血清sTfR水平与HbH病患儿贫血程度相关,可能是HbH病治疗的一个靶点。     

肾功能亢进对耐甲氧西林金黄色葡萄球菌感染患儿万古霉素血药浓度及治疗效果的影响分析

目的 研究肾功能亢进（ARC）对万古霉素治疗儿童耐甲氧西林金黄色葡萄球菌（MRSA）感染时的血药浓度、细菌学疗效及临床疗效的影响。方法 回顾性研究2013年1月至2017年7月期间因明确MRSA感染使用万古霉素,并进行血药浓度监测的60例危重患儿的病例资料,根据肾小球滤过率（eGFR）分为ARC组（n=19）和肾功能正常组（n=41）,对两组患儿在万古霉素使用、血药浓度及治疗效果等方面进行统计学比较分析。结果 ARC组的年龄主要分布在1~12岁,其体重和体表面积明显大于肾功能正常组（P < 0.05）。ARC组初始万古霉素血药谷浓度明显低于肾功能正常组,且ARC组达有效血药谷浓度（10~20 mg/L）比例低于肾功能正常组（P < 0.05）。两组在细菌学疗效评价和临床疗效评价方面比较差异均无统计学意义（P > 0.05）,但ARC组的儿童重症监护室（PICU）住院时间及总住院时间明显长于肾功能正常组（P < 0.05）。结论 ARC明显降低MRSA感染患儿的万古霉素血药谷浓度,延长PICU住院时间及总住院时间。临床上应注意对ARC患儿施行个体化给药治疗。     

6~18岁智力障碍人群的孤独症谱系障碍样症状分析

目的 了解智力障碍（ID）人群的孤独症谱系障碍（ASD）样症状的发生情况。方法 选取2017年1~6月就读于上海市某特殊学校的6~18岁ASD人群和ID人群,以及同期在上海市某普通学校就读的同年龄段普通（TD）人群,由父母或其他监护人填写社交反应量表（SRS）,分别对其进行ASD样症状评估。结果 共纳入69例ASD、74例ID和177例TD研究对象。ID组SRS量表检查阳性率（47.3%）显著高于TD组（1.7%）（P < 0.001）,低于ASD组（87.0%）（P < 0.001）。ASD组、ID组和TD组SRS量表总分分别为114±26、80±24、38±19分,其中ID组SRS量表总分显著高于TD组得分（P < 0.05）,以社交认知维度差异最为显著（Cohen's d值为2.00）。轻-中度ID亚组和重-极重度ID亚组的SRS总分及各维度得分差异无统计学意义（P > 0.05）,且SRS得分与IQ之间无明显相关性（P > 0.05）。结论 6~18岁ID人群较普通人群存在更显著的ASD样症状,应对ID人群尽早进行ASD筛查并给予干预。     

丙酸氟替卡松、孟鲁斯特钠、酮替芬治疗儿童咳嗽变异性哮喘的临床疗效观察

目的 观察丙酸氟替卡松（Flu）、孟鲁斯特钠（Mon）与酮替芬（Ket）的不同联合用药方案治疗儿童咳嗽变异性哮喘（CVA）的效果。方法 将2015年6月至2018年1月于呼吸科门诊收治的280例CVA患儿随机分为Flu+Mon+Ket组、Flu+Mon组、Flu+Ket组、Mon+Ket组、Flu组、Mon组、Ket组（n=40）。每组根据各自的用药方案给予相应药物,疗程均为3个月。评估患儿在治疗后2个月及3个月时的咳嗽情况及评分、肺功能和药物不良反应,并随访复发情况。结果 随治疗时间,7组患儿咳嗽评分均呈下降趋势,第1秒用力呼气容积占预计值的百分比（FEV1%）、最大呼气流量占预计值的百分比（PEF%）均呈上升趋势。治疗2个月后,Flu+Mon+Ket组咳嗽评分均低于其他组,FEV1%、PEF%均高于其他组（P < 0.05）;治疗2个月后及治疗3个月后,Ket组咳嗽评分明显高于其他组,FEV1%、PEF%明显低于其他组（P < 0.05）;其他各组在治疗3个月后,组间咳嗽评分、FEV1%、PEF%比较差异均无统计学意义（P > 0.05）。7组不良反应发生率低且差异无统计学意义（P > 0.05）。Ket组咳嗽复发率明显高于其他组（P < 0.001）,其他各组间咳嗽复发率比较差异无统计学意义（P > 0.0024）。结论 Flu、Mon、Ket三药联合使用治疗儿童CVA在2个月时的疗效较两药联用及单药使用显著,且用药安全;但用药3个月后,单用Flu或单用Mon的疗效不差于联合用药;单用Ket疗效不佳,且停药后复发率较高。     

哮喘高危婴幼儿喘息发作期病毒病原及过敏原检测分析

目的分析哮喘高危婴幼儿喘息发作期病毒病原学、过敏原分布，为喘息患儿的早期诊断与干预治疗提供帮助。方法选取2016年4月至2017年8月因喘息性支气管炎和喘息性支气管肺炎住院的135例哮喘高危婴幼儿为研究对象。采用荧光探针PCR法检测患儿鼻咽部抽吸物标本甲型流感病毒（Flu-A）、呼吸道合胞病毒（RSV）、腺病毒（ADV）、副流感病毒（PinF）、人鼻病毒（HRV）、人偏肺病毒（hMPV）、博卡病毒（HBoV）感染情况；采用ImmunoCAP技术检测患儿吸入性变应原、食物性变应原及总IgE浓度。结果 135例患儿中，鼻咽部抽吸物标本病毒检出阳性率为49.6%，各病毒检出阳性率由高到低依次为HRV 25.2%、HBoV 9.6%、RSV 8.1%、PinF 5.9%、Flu-A 3.7%、ADV 1.5%、hMPV 0.7%。HRV在1~3岁年龄组检出率高于<1岁组（P < 0.05）。过敏原筛查试验阳性率为59.3%，吸入性过敏原阳性率为44%，食物性过敏原阳性率为89%；吸入性过敏原中阳性率由高到低依次为尘螨77%、霉菌37%、花粉26%、动物皮屑9%；食物性过敏原中阳性率由高到低依次为鸡蛋白73%、牛奶68%。<1岁组吸入性过敏原阳性率大于1~3岁组（P < 0.05）；1~3岁组T-IgE水平明显高于<1岁组（P < 0.05）。病毒检出组吸入性过敏原阳性率大于病毒未检出组（P < 0.05）。第2次喘息患儿吸入性、食物性过敏原阳性率及T-IgE水平均高于第1次喘息患儿（P < 0.05）；吸入性过敏原尘螨、霉菌在第2次喘息患儿中阳性率高于第1次喘息患儿（P < 0.05）。结论早期HRV感染和吸入性过敏原阳性与哮喘高危婴幼儿喘息发生密切相关。     

内皮细胞微粒在过敏性紫癜患儿中的表达及意义

目的 探讨内皮细胞微粒（EMPs）在过敏性紫癜患儿中的表达及意义。方法 100例初治过敏性紫癜患儿分为紫癜性肾炎组（HSPN组,40例）和非肾炎组（60例）,并以30例健康体检儿童为对照组。采用流式细胞术或ELISA方法检测各组血清EMPs、Th17及IL-17的表达或含量。结果 Th17及IL-17在HSP的肾炎组和非肾炎组均高于对照组,以HSPN组最高,差异有统计学意义（P < 0.05）。HSP肾炎组和非肾炎组的EMPs水平较对照组升高,以HSPN组最高,差异有统计学意义（P < 0.05）。紫癜性肾炎组的Th17、IL-17水平与EMPs水平呈正相关（r=0.830、0.644,P < 0.05）。结论 EMPs在过敏性紫癜的发病机制中起一定作用,EMPs升高可能是过敏性紫癜患儿肾脏受累的原因之一。     

重庆地区202例儿童慢性咳嗽的病因研究

目的 探讨儿童慢性咳嗽的病因构成。方法 对2015年5月至2017年11月于重庆医科大学附属儿童医院住院治疗的202例慢性咳嗽患儿的临床资料进行回顾性分析。结果 202例患儿的病因分布为：感染后咳嗽81例（40.1%）,咳嗽变异性哮喘71例（35.1%）,上气道咳嗽综合征43例（21.3%）,异物吸入3例（1.5%）,胃食管反流性咳嗽1例（0.5%）,多发性抽动症2例（1.0%）,先天性呼吸道疾病1例（0.5%）。119例（58.9%）慢性咳嗽是单病因所致,83例（41.1%）有多种病因。不同年龄组（< 1岁、1岁~、3岁~、6~14岁）和不同咳嗽性质组（湿咳、干咳）慢性咳嗽病因构成差异均有统计学意义（P < 0.01）。结论 儿童慢性咳嗽的前三位主要病因为：感染后咳嗽、咳嗽变异性哮喘和上气道咳嗽综合征;不同年龄阶段及不同咳嗽性质的患儿其慢性咳嗽的主要病因存在差异。     

不同病因矮身材儿童TW2-R、C、T骨龄评分特征研究

目的　探讨不同病因矮身材儿童TW2-R、C、T骨龄评分特征, 为矮身材的病因诊断提供参考。方法　以363例未经治疗的矮身材儿童为研究对象, 根据病因分为4组：生长激素缺乏症(GHD, 27例)、特发性矮小(ISS, 280例)、小于胎龄儿(SGA, 41例)、Turner综合征(TS, 15例)。拍摄左手腕骨骨龄片, 应用TW-2骨龄评分法对各组患儿R骨龄、C骨龄及T骨龄进行评分, 将各序列骨龄与年龄对比分析。结果　GHD组男、女儿童表现为R骨龄、C骨龄及T骨龄均较年龄落后2岁以上。ISS组男童R骨龄、C骨龄及T骨龄较年龄落后约1岁; ISS组女童各序列骨龄与年龄比较无显著差异。SGA组男女儿童各序列骨龄与年龄比较无显著差异。TS组R骨龄及T骨龄较年龄显著落后, C骨龄与年龄比较无显著差异。结论 不同病因所致的矮身材儿童具有不同的TW-2 R、C、T各序列骨龄特点。TW-2 R、C、T各序列骨龄的评估对于矮身材儿童病因的诊断具有辅助作用。     

Causes of short stature in children in relation to their midparental height

Introduction: It is considered that the evaluation of a child's height strongly depends on the evaluation of his/her midparental height. Aim of study: Analysis of causes of short stature in children in relation to midparental height. Material and methods: The study included 452 children with short stature, aged 3-18 yrs. The group of children included 178 girls and 274 boys. The children's measurements were standardized using the arithmetic mean and standard deviation for the Institute of Mother and Child norms. Midparental height was evaluated according to standards for 18-year-olds. The average height deficit was -2.5 SDS±0.65. The average midparental height was 166.07 cm±4,63 and in SDS it was -0.93±0,74. Results: Growth hormone deficiency (GHD) or multihormonal pituitary deficiency was found in 34.3% of the patients (50 girls and 105 boys). In 22 girls (12.48) the Turner syndrome (TS) was diagnosed. Other causes of short stature were observed in 37 children. No hormonal disorders were found in the remaining group (275 children). 7% of those children were characterized by significant height deficit (-3 SDS). The difference between midparental height of GHD children and healthy children was not statistically significant. Body height of 20% of GHD children was consistent with their midparental height. The midparental height of girls with TS was significantly higher than the midparental height of the other children. The body height of 50% of girls with TS differed from their midparental height. The body height of 40% of healthy short children was consistent with their midparental height. Conclusions: 1. Children with short stature are a very heterogenous group of patients. 2. Comprehensive evaluation of physical development in children should not be restricted to the widely used criterion of midparental height.     

Evaluation of Quality of Life in Children with GH Deficiency and Idiopathic Short Stature Using the Child Behavior Checklist

The quality of life (QoL) of short children is an important issue that has been studied in Western countries, but not fully in Japan. We assessed the psychosocial profiles of Japanese children with short stature using the Japanese version of the Child Behavior Checklist (CBCL). A higher score in the CBCL means a lower QoL. A total of 116 children with idiopathic short stature (ISS) and 127 children with GH deficiency (GHD), aged 4 to 15 yr, were enrolled in the study. The total CBCL scores of the children in the GHD/ISS group were found to be higher than those of the normal children group. The QoL subscales for social problems and attention problems of the young (4–11 yr) children in the GHD/ISS group were significantly higher than those of the group of children of normal height. The proportion of children with GHD/ISS classified into the borderline/abnormal range was significantly higher than that of normal children. Children with ISS tended to have higher total scores and more subscale problems, and a greater proportion of these children was classified in the borderline/abnormal range than the children with GHD, although the difference was not significant. These results suggest that QoL is impaired in Japanese children due to short stature.     

Growth failure starts from early infancy in children with short stature at age 6

We compared the growth of 183 children with short stature (≤ 2SD) and 73 children of normal height at age six who were visiting the Tanaka Growth Clinic. We classified these short children as suffering from either idiopathic short stature (ISS, n = 119), GH deficiency (GHD, n = 33) or small-for-gestational-age short stature (SGASS, n = 31) on the basis of subsequent test results and other factors. We also conducted a retrospective study of changes in their height, wt and nutritional intake over time. The mean changes in height SD score from birth to 6 yr were –0.24 SD in normal height children with a normal birth length and +2.27 SD in normal height children with a low birth length. In short children, these changes were –1.93 SD for children with ISS, –2.41 SD for those with GHD and +0.58 for those with SGASS. The mean changes from birth to 6 mo were –0.84 SD, −1.03 SD and +0.38 SD in children with ISS, GHD and SGASS, respectively. The mean change in height SD score from birth to age 1 yr was –1.07 SD, –1.44 SD and +0.35 SD, respectively. The decrease in height SD score from birth to 6 mo accounted for 43.5% of the decrease in height SD score from birth to 6 yr in children with ISS and it accounted for 42.6% of the decrease in children with GHD. Only 19% of short children bottle-fed well, and 53% fed poorly, as opposed to 56% and 16% of normal height children who fed well and poorly, respectively. Post weaning, only 22% of short children ate well, and 56% fed poorly, as opposed to 53% and 17% of normal height children who fed well and poorly, respectively. These findings demonstrated that growth failure started from early infancy in ISS and GHD children. It was suggested that poor nutritional intake in infancy and early childhood was a partial cause of short stature at age 6.     

儿童短肢型遗传性骨代谢性疾病的临床分析及基因诊断

目的：分析软骨发育不全（ACH）、软骨发育低下（HCH）及假性软骨发育不全（PSACH）3种短肢型遗传性骨代谢性疾病的临床表现、骨骼X线表现及基因结果。方法：对基因确诊的10例短肢型遗传性骨代谢性疾病患儿（其中4例为ACH,3例为HCH,3例为PSACH）的临床特点、骨骼X线表现及基因结果进行分析。结果：10例患儿的平均身高为-3.69±1.79 SD,平均坐高/身高比值为0.65±0.03,平均指间距/身高比值为0.93±0.04。4例ACH患儿及3例PSACH患儿具有典型骨骼X线表现,3例HCH患儿中1例表现为坐骨大切迹变小,1例表现为椎弓根间距未增宽。4例ACH患儿中3例检测到FGFR3基因G380R突变,1例检测到Y278C突变;3例HCH患儿均检测到FGFR3基因N540K突变;3例PSACH患儿检测到COMP基因的杂合突变。结论：ACH及PSACH患儿的矮小程度及骨骼畸形程度较HCH患儿重,HCH患儿临床表现轻,不典型;骨骼X线及基因分析有助于3种疾病的诊断及鉴别诊断;3种疾病涉及2个基因,分别有各自的突变热点,有利于临床基因诊断。     

Effect of Growth Hormone Treatment on Quality of Life in Japanese Children with Growth Hormone Deficiency: An Analysis from a Prospective Observational Study

The aim of this study was to assess changes in quality of life (QoL) in Japanese children with GH deficiency (GHD) after 12 mo of GH treatment or with idiopathic short stature (ISS) after 12 mo without treatment. Children with GHD were treated with GH after enrollment. Outcome measures included the parent-rated Child Behavior Checklist (CBCL), the Youth Self-Report Form (YSR), and height standard deviation scores (SDS). Total CBCL scores significantly decreased in children with GHD (n = 152, mean change (standard deviation [SD]) = –3.42 [11.21]) and ISS (n = 129, mean change = –4.82 [10.09]) after 12 mo (p < 0.001). Total YSR scores (mean change = –9.21 [14.07]) and height SDS (mean change = 0.35 [0.38]) significantly decreased in children with GHD (p < 0.001), but were unchanged in children with ISS. The change in total YSR score was significantly correlated with the change in height SDS in children with GHD (r = –0.516, p = 0.003). Our findings demonstrate that GH treatment can improve QoL in Japanese children with GHD. The correlation between the changes in total YSR score and height SDS demonstrated that increased height resulted in improved QoL.     

A practical approach to growth hormone deficiency

Short stature is one of the most frequent reasons for referral to the paediatric endocrinologist. Familial short stature is the commonest cause; however, a child who is unexpectedly small for their family requires careful evaluation. Short stature in itself is not a disease, but growth failure can be a sensitive sign of underlying health issues in children and adolescents. Differentiating short stature due to a non-endocrine cause from an endocrine cause is critical. Of the endocrine causes, growth hormone deficiency (GHD) is rare with a prevalence of approximately 1:4,000 to 1:10,000 but is an important condition warranting careful evaluation and management. In children with GHD, replacement growth hormone (GH) treatment can be highly effective in normalizing height during childhood and in achieving an adult height within the genetic target range. Whilst GHD, once diagnosed, is relatively easy to treat, the diagnosis still remains a challenge. There is no consensus with respect to a gold standard diagnostic test for GHD, and this is usually based upon a combination of clinical, biochemical and radiological data. This article provides a stepwise guide to diagnosing GHD in children.     

Evaluation of insulin-like growth factor (IGF)-I and IGF binding protein-3 generation test in short stature

BACKGROUND: Differentiation between growth hormone deficiency (GHD) and idiopathic short stature (ISS) based on GH tests and basal IGF-I and IGFBP-3 levels may be difficult. The aim of this study was to evaluate the role of pharmacological GH tests, IGF-I and IGFBP-3 generation test and height velocity off-treatment in the evaluation of GHD and ISS. METHODS: Thirty-three (17 M, 16 F) prepubertal short (height SDS < -2) children were divided into two groups: Group 1 (n = 19) with peak GH level <10 tg/l (GHD) and Group 2 (n = 14) GH > or =10 microg/l in two sex steroid primed pharmacological GH tests. Having excluded other diagnoses, Group 2 was regarded as having ISS. The generation test was performed concomitantly (0.1 IU/kg GH s.c. for 4 days) with IGF-I and IGFBP-3 measured on the 4th day in both groups. The patients were followed for a year for height velocity (HV). RESULTS: Group 1 and 2 had comparable height SDS (-2.3 +/- 0.4 and -2.3 +/- 0.3) at comparable ages (7.8 +/- 2.8 and 7.0 +/- 2.7 yr). Although the deltaIGF-I response was low (<2.0 nmol/l 115 ng/ ml]) in seven (37%) children in the GHD group, all GHD patients with low height velocity had adequate (> or =14 nmol/I [400 ng/ml]) deltaIGFBP-3 response. deltaIGFBP-3 in the generation test showed a negative correlation with HV (p = 0.021, r = -0.570) and also with basal IGFBP-3 (p <0.001, r = -0.743) in the GHD group. In the ISS group, deltaIGF-I and deltaIGFBP-3 responses were low in 31% and 7%, respectively, and the correlation between basal IGF-I, IGFBP-3 and HV and between delta values in the generation test were significantly positive, pointing to a difference in the growth response of these children. CONCLUSION: In the GHD group, based on pharmacological tests, an adequate deltaIGFBP-3 response in the generation test predicts poor height velocity at follow up and thus strengthens the diagnosis of true GHD.     

The role of insulin like growth factor (IGF) — 1 and IGF — binding protein-3 in diagnosis of Growth Hormone Deficiency in short stature children

Objective  The purpose of this study was to evaluate the role of IGF-1 and IGFBP-3 in diagnosis of short stature children and adolescents in whom Growth Hormone Deficiency (GHD) was found. Methods  In this cross sectional study the referred short stature children and adolescents to Namazi Hospital in Shiraz- Iran, in 2003–2005 were studied. The inclusion criteria were proved short stature based on the physical examination, weight, height, standard deviation score (SDS) of height < −2, with considering stage of puberty and predicted height in children without any genetic or chronic disorders. The exclusion criteria were any positive physical or laboratory data suggesting hypothyroidism, rickets or liver disorders. For all patients a provocative growth hormone test was performed with propranolol and L-dopa and serum IGF-1 and IGFBP-3 were measured. GHD defined as peak(cutoff) serum GH level under 10 ìg/L and low IGF-1 and IGFBP-3 considered as cutoff serum level under −2 standard deviation. Results  Eighty one short stature patients (39 boys and 42 girls) with mean age of 10.6 ± 3.5 years completed the study. Seventeen patients with GHD were found and in 18 patients IGF-1 level were low. Only in 6 patients both GH and IGF-1 were low and 2 of them had low IGFBP-3. There were no correlations between the levels of GH,IGF-1 and IGFBP-3 in children with short stature due to GHD. The sensitivity and specifity of IGF-1 and IGFBP-3 in assessment of GHD were 35% and 81% for IGF-1 and 12% and 94% for IGFBP-3, respectively. Conclusion  No correlations were found between GH level and serum levels of IGF-1 and IGFBP-3 in short patients and the sensitivity of those tests in assessment of GHD were poor.     

Idiopathic short stature: definition and treatment

Short stature is the leading cause of consultation in Pediatric Endocrinology. Decreased growth velocity and abnormally short height are characteristic of several different nosologic entities. Some are well characterized, while others correspond to what is known as idiopathic short stature (ISS). ISS includes children who grow less than 2 SD of the mean height values corresponding to their peers of similar age and the same sex, in whom the known causes of short stature have been ruled out. The diagnosis of ISS does not include children who only present a constitutional delay in growth and development. Several clinical trials have demonstrated the efficacy of growth hormone (rhGH) treatment in achieving catch-up growth in these children. Therefore, ISS should be kept in mind in the diagnosis of patients with short stature and abnormal growth patterns, who may benefit from rhGH treatment.