不同疾病运用不同骨龄评价方法的比较

目的探讨国内常用的骨龄评价方法G-P图谱、中国人骨成熟评价标准-CHN记分法(CHN法)及儿童青少年骨龄评分法(叶氏评分法)的特点及应用选择。方法用此3种方法对不同疾病骨龄评价进行选择。结果3种骨龄评价方法无差异性(P>0.05)。结论G-P图谱、CHN法和叶化评分法可对一骨龄进行评价。     

三种骨龄评价方法在3～17岁儿童临床应用中的一致性比较研究

目的评价Greulich-Pyle图谱法(GP)、中国人手腕骨发育标准-CHN法(CHN)和中国人手腕标准-中华05法(TW3-C RUS)测得的骨龄是否会影响临床医生对儿童骨成熟水平的判断。方法纳入因身材矮小或提前发育或怀疑生长发育问题有必要行X线片评估的3~17岁儿童左手正位X线骨龄片。排除患遗传代谢性、骨软骨发育障碍性疾病的儿童,排除接受过生长激素等治疗的儿童。1名经过骨龄片评估培训的研究生依序对每张X线骨龄片用上述3种方法分别评价,采用Bland-Altman法构建一致性限。结果 567例左手正位X线片进入分析,男童269例,女童298例,男、女童各分3~4、~6、~8、~10、~12、~14和~17年龄组,每组17~78例。男103例和女142例达到青春期发育标准。男、女童所有年龄组测得的骨龄CHN法GP法和TW3-C RUS法;男、女童中除3个年龄组外,余11个年龄组测得骨龄TW3-C RUS法均GP法。男、女童的所有年龄组3种方法评价的最大骨龄与最小骨龄的差值随年龄增加而增大。鉴于TW3-C RUS评价男童最大骨龄为16岁,女童最大骨龄为15岁,去除TW3-C RUS法男童≥16岁(18例)和女童≥15岁(34例)的骨龄片,515例左手正位的X线片(男251例,女264例)进入3种方法一致性限分析。男童3~4岁骨龄和~6岁骨龄TW3-C RUS法与GP法一致性良好,女童3~4岁骨龄CHN法与TW3-C RUS法、~8岁骨龄TW3-C RUS法与GP法一致性良好,女童未发育组TW3-C RUS法与GP法一致性良好,余男、女童不同骨龄和发育情况3种骨龄测量法一致性差。结论 3种骨龄测量方法得出的骨龄结果不能相互替代,临床应用时应明确骨龄测评的方法。     

中枢性性早熟患儿5种骨龄测定方法的比较研究

目的探讨CHN法、TW2法中日英3种标准、TW3法5种骨龄测定方法对中枢性性早熟(CPP)患儿的诊断价值及正常值临界点的确定。方法由两名医师采用盲法回顾性分析CPP患儿61例(病例组),与每一个CPP患儿性别相同,年龄、身高、体质量基本一致的同期体检者61例、8岁以后出现乳房发育的女童6例(均为对照组)治疗前左手腕部X线片,用CHN法、TW2法中英日3种标准和TW3法进行骨龄判定,计算骨龄与年龄的差值,用SPSS13.0统计软件进行受试者工作特征(ROC)分析。结果 (1)两名医师骨龄测定结果的Kappa值为0.776(u=16.128,P<0.05);(2)5种骨龄测定方法的ROC曲线下面积分别为:CHN法0.921±0.024,95%可信区间为0.875～0.967;TW2法中国南方人标准为0.947±0.019(0.910～0.983);TW2法日本人标准为0.937±0.023(0.892～0.982);TW2法英国人标准为0.931±0.022(0.888～0.975);TW3骨龄测定法为0.924±0.023(0.879～0.969);5种方法的诊断价值差异无统计学意义(Z=0.85,P>0.05...     

国人常见的两种葡萄糖-6-磷酸脱氢酶基因突变及其临床表现

目的 探讨中国人中两种常见葡萄糖－６－磷酸脱氢酶（Ｇ６ＰＤ）基因突变与临床表现的关系。方法 应用突变扩增系统（ＡＲＭＳ）法。研究１５例云南籍汉族Ｇ６ＰＤ缺乏症患儿两种常见Ｇ６ＰＤ基因突变,并对其临床表现进行分析,结果 １５例中检出,ｃＤＮＡＧ１３８８Ａ突变９例,ｃＤＮＡＧ１３７６Ｔ突变５例,未定型１例,所有患者都具有急性溶血性贫血,黄疸等临床表现不同程度的血红蛋白尿,Ｇ６酶活性降低,结论 Ｇ６ＰＤ     

术中判断肠活力三种方法的比较

术中准确地评价肠活力是外科医师面临的一个普遍问题。应用兔肠缺血模型，比较静脉荧光素、表面血氧测定和激光多普勒三种技术判断肠活力的精确性。结果：诊断效率静脉荧光素法为７８％，表面血氧测定为６８％，激光多普勒为９５％。后者精确度显著优于前二者，且操作简便、迅速，具有临床应用前景。     

G—6—PD缺陷致新生儿高未结合胆红素血症不同治疗方法的比较

红细胞葡萄糖-6-磷酸脱氢酶(G-6-PD)缺陷是新生儿高未结合胆红素血症(简称高胆)的常见病因。治疗高胆的目的是预防核黄疸的产生和防止胆红素对神经系统的损害。我科对G-6-PD缺陷的高胆患儿分别进行了换血和光疗的不同处理,均取得了满意效果,现报告如下。     

三种不同危重症评估法对危重新生儿死亡风险的预测价值比较

目的 比较新生儿急性生理评分(score of neonatal acute physiology,SNAP)、新生儿危重病例评分(neonatal critical illness scores,NCIS)及第三代小儿死亡风险评分(pediatric rick of mortality Ⅲscore,PRISM Ⅲ)三种危重症评分法对危重新生儿死亡风险的预测价值,探索适合我院新生儿重症监护室(NICU)应用的评分系统.方法 对2014年10月至2015年6月入住我院NICU的398例患儿同时采用SNAP、NCIS及PRISM Ⅲ三种评分系统进行不同时点评分,以新生儿是否死亡为结局,分别描绘各评分系统各时点受试者工作特征曲线(Receiver Operator Characteristic Curve,ROC),比较各ROC曲线下面积(Ar-ea Under ROC Curve,AUC)的差异.结果 NCIS评分系统的各时点AUC分别为0.769/1d,0.834/3d,0.862/5d;SNAP评分系统的各时点AUC分别为0.849/1d,0.856/2d;PRISM Ⅲ评分系统的各时点AUC分别为0.869/12h,0.878/24h.AUC均大于0.5,可预测疾病死亡风险.三系统各时点AUC的比较:PRISM Ⅲ(24h)、SNAP(1d)两者差异无统计学意义(Z=1.26,P=0.208);NCIS(1d)、NCIS(3d)各与PRISM Ⅲ(24h)及SNAP(1d)比较,前者差异有统计学意义(Z=4.73、2.57;P=0.00001、0.01);后者差异无统计学意义(Z=1.9、0.46;P=0.060、0.645).结论 PRISM Ⅲ、SNAP及NCIS三个评分系统均能预测危重新生儿的死亡风险;PRISM Ⅲ、SNAP早期能预测危重新生儿死亡风险.     

儿童鼻出血三种治疗方法的比较

儿童鼻出血大多数为Ｌｉｔｔｌｅ区出血[1] ,选择安全、有效、迅速、简便的治疗方法 ,对减轻患儿痛苦和减少并发症十分重要。我科对 1999年 1月～ 2 0 0 1年 12月 15 1例 (189侧 )门诊儿童鼻Ｌｉｔｔｌｅ区出血患儿分别采用微波热凝固术、化学烧灼和冷冻治疗等三种方法进行治疗 ,以选择最佳疗法 ,现报告如下。资料与方法一、临床资料　儿童鼻出血 15 1例 (189侧 ) ,男 91例(114侧 ) ,女 6 0例 (75侧 ) ;年龄 4～ 8岁。出血时间 2～ 7ｄ ,反复出血 3～ 9次。鼻Ｌｉｔｔｌｅ区可见糜烂和 (或 )搏动性出血。常规检查排除血液病及其他凝血机制障…     

几种呼吸道合胞病毒检测方法的比较

目的比较不同从临床样本中快速、敏感特异的检测呼吸道合胞病毒（RSV）的方法,以便早期诊断RSV感染。方法应用不同方法对45例急性下呼吸道感染患儿鼻咽分泌物RSV进行检测,包括病毒分离、直接涂片间接免疫荧光法（IFA）、快速细胞培养法、双抗体夹心酶联免疫吸附法（ELISA）及链亲和素-生物素法（LSAB）。结果鼻咽分泌物45例中,病毒分离阳性12例（26.7%）,直接涂片间接免疫荧光法检测出阳性14例（31.1%）,快速细胞培养法阳性20例（44.4%）。双抗体夹心ELISA及LSAB法均仅检出4例阳性,阳性率均为8.9%。结论将直接涂片间接免疫荧光法与快速细胞培养法同时应用于RSV感染的早期诊断,既能及时提供检测结果,又能提高诊断的敏感性,是最适合于RSV感染早期诊断的方法。     

三种宫内发育迟缓大鼠模型方法的比较

目的　对比常用的三种建立宫内发育迟缓 (IUGR)大鼠模型的方法 ,选择适用于儿科研究IUGR对生后生长发育和代谢远期影响的动物实验模型。方法　 1 5只孕鼠平均分为 3组采用子宫动脉结扎法、更生霉素腹腔注射法、被动吸烟法建立IUGR动物模型 ,以自然分娩为结束妊娠的方式 ,比较仔鼠的体重 ,IUGR发生率及围产期死亡率。结果　三个实验组的仔鼠平均体重分别为 5 .5 46g ,5 .377g ,5 .1 0 3g ,以正常对照组平均体重减 2个标准差为IUGR的标准 ,孕鼠产出的IUGR仔鼠发生率分别为 2 7.8% ,42 .2 % ,6 1 .6 % ,仔鼠围产期死亡率分别为 6 1 .1 % ,1 7.8% ,6 .7%。结论　被动吸烟法及更生霉素腹腔注射法均能成功建立IUGR大鼠模型 ,尤以前者更适用于儿科研究。     

Craniofacial and acral growth responses in growth hormone-deficient children treated with growth hormone

OBJECTIVES: To investigate the effects of growth hormone (GH) therapy on craniofacial growth and body proportions in growth hormone deficient children. STUDY DESIGN: By using a cross-sectional study design, we investigated GH effects on craniofacial growth with photographic facial morphometrics, head circumference, and hand and foot size in 52 children with GH deficiency (GHD) treated with GH (0.27 mg/kg/wk) for 0.19 to 15.5 years, compared with untreated children with GHD and normal first-degree relatives. To detect disproportion and to correct for stature, age and height age (HA) SD scores were analyzed. RESULTS: Untreated subjects with GHD had retarded facial height and width (P values=.001) compared with normal controls; small head circumference for age and HA (P=.001); small hands for age (P<.001) that were large for HA (P=.003); and small feet for age (P<.001) that were normal for HA. When compared with normal controls, GH-treated subjects had proportional facial heights but narrower facial widths. Head circumference, however, increased disproportionately to height (P=.001), becoming large for stature, and increasing with duration of therapy and cumulative GH dose (P<.001). Hands and feet grew proportionately to height. CONCLUSION: Growth hormone treatment with conventional doses partially corrects craniofacial deficits and does not adversely affect hand and foot growth but appears to result in excessive head circumference growth.     

Comparison of growth hormone releasing hormone therapy and growth hormone therapy in growth hormone deficiency

Seven children with growth hormone deficiency of hypothalamic origin responded to an i.v. bolus of growth hormone releasing hormone (GHRH) (1–29)-NH2 with a mean serum increase of 10.7 ng/ml growth hormone (GH) (range 2.5–29.3 ng/ml). Continuous s.c. administration of GHRH of 4–6 g/kg twice daily for at least 6 months did not improve the growth rate in five of the patients. One patient increased his growth rate from 1.9 to 3.8 cm/year and another from 3.5 to 8.2 cm/year; however, the growth rate of the latter patient then decreased to 5.4 cm/year. When treatment was changed to recombinant human growth hormone (rhGH) in a dose of 2 U/m² daily, given s.c. at bedtime, the growth rate improved in all patients to a mean of 8.5 cm/year (range: 6.2 to 14.6). Presently GHRH cannot be recommended for the routine therapy of children with growth hormone deficiency since a single daily dose of rhGH produced catch-up growth which GHRH therapy did not.Abbreviations GH growth hormone - GHD growth hormone deficiency - GHRH growth hormone releasing hormone - hGH human growth hormone - rhGH recombinant human growth hormone - SM C/IGF I somatomedin C/insulin-like growth factor I On the occasion of the 85th birthday of Prof. Dr.Dr.h.c. mult. Adolf Butenandt     

Prediction of growth response in prepubertal children treated with growth hormone for idiopathic growth hormone deficiency

Several multiple regression models have been developed to predict the first-year growth response to human growth hormone (hGH) in children with growth hormone deficiency (GHD). It was the aim of this study to analyse the significance of various growth parameters for a height prediction model. Data from 148 prepubertal children with idiopathic GHD were evaluated. The prediction model was developed by means of univariate and stepwise linear regression analysis and an “all possible” regression approach using Mallow's C(p) statistics. Six out of eight selected variables had a significant influence on the first-year growth rate. The most important parameter was the difference between target height SDS and height SDS at the start of therapy (THSDS - HSDSC0), accounting for 23.95% and 25.74% of the variability. No other single variable or combination of variables was more informative than the variable THSDS - HSDSC0 alone. From these data, growth velocity for the first year of hGH treatment was estimated as 1.106 (THSDS - HSDSC0) + 6.8 cm/y ± 2.2 cm (SE), allowing a prediction for different intervals between THSDS and HSDSC0. This equation was validated in a small group of 18 GHD patients demonstrating a predicted vs. observed first-year growth rate of 9.4 ± 1.1 vs. 9.5 ± 2.6 cm/y. We conclude that the difference between THSDS and height SDS at the start of therapy is an important predictor of the first-year growth response in children treated with hGH for idiopathic GHD. Unlike in previous studies, additional parameters did not increase predictability.     

Chemotherapy-induced growth hormone deficiency in children with cancer

Background. Chemotherapy (CT) may produce growth impairment, however, the pathogenesis is still unclear. Methods. A series of 25 patients mean age 13.3 years (6.3–19.8), previously treated for malignant solid tumours with only CT and surgery were studied. Growth hormone (GH) reserve was assessed by two different provocative stimuli (Clonidine and L-Dopa). Mean time between completion of treatment and GH evaluation was 18.5 months (2–74 months). At that time, all patients were in complete remission. Results. GH deficiency (GHD), defined by an impaired GH response to both provocative tests was observed in 11 out of 25 patients (44%). At diagnosis, mean standing height was +0.23 ± 1.42 SDS in the GHD group (GHD-g) and +0.18 ± 1.23 SDS in the non-GHD group (n-GHD-g). At the end of therapy, the mean standing height in the GHD-g was ?0.31 ± 1.22 SDS and ?0.17 ± 1.41 in the n-GHD-g, differing from the former group (P = 0.05). For a mean follow-up of 30 months from the end of treatment, the mean standing height was ?0.48 ± 1.23 SDS in the GHD-g and ?0.24 ± 1.51 SDS for the n-GHD-g (P = 0.03). Growth rate at the end of treatment was +0.13 ± 1.54 in the GHD-g and +0.21 ± 1.75 in the n-GHD-g. For a mean follow-up of 30 months from the end of treatment, the growth rate was different between GHD-g and n-GHD-g (?0.31 ±2.72 vs. ?0.21 ± 1.93, P < 0.05). Conclusions. 1) Growth impairment in children treated because of malignant diseases has a multifactorial etiology, but CT-induced GH deficiency is one potential adverse factor. 2) An endocrine follow-up should be introduced in order to detect and treat hormonal deficiencies as early as possible. © 1995 Wiley-Liss, Inc.     

Changes with growth hormone treatment in growth hormone deficient children

A total of 54 previously untreated patients (15 girls, 39 boys) with poor growth due to idiopathic growth hormone deficiency (IGHD) were treated with human growth hormone (hGH), continuously up to 4 years. All of the patients had a peak hGH level which was below 10 ng/mL after at least two pharmacological tests and/or blunted physiologic hGH secretion, and their height was below ?2.5 s.d. for age and gender. After the 1st year of therapy, height velocity (HV) increased significantly when compared with baseline (from 3.18 ±0.76 cm/year to 9.17±1.03 cm/year; P <0.001), declined during the 2nd year and then remained significantly higher than pretreatment HV. When considering improvement in height expressed by height standard deviation score (SDS), during the therapy all of the patients showed a significant gain ± 1.72±1.09 (from ?4.11±0.61 to ?2.21±0.48). The height values were significantly higher than pretreatment, but remained below ?2 s.d. after 4 years of hGH therapy in our patients. Increased height velocity has been sustained, but height improvement after therapy was inversely correlated to height SDS for chronological age of patients at the start of therapy. In conclusion post-treatment height has been shown to be related to height deficit at the beginning of therapy. Therapy was well tolerated with no local or systemic adverse effects or acceleration of bone age.     

Depressive tendency in children with growth hormone deficiency

Aim:   This study assessed changes in depressive tendency of children with growth hormone deficiency.
Methods:   The 41 children with growth hormone deficiency were assessed using the Depression Self-Rating Scale for Children. A score of 16 or more indicated a depressive tendency. The first assessment was carried out before growth hormone treatment, and the second one was carried out at 6 months or longer after the beginning of growth hormone treatment.
Results:   The Depression Self-Rating Scale for Children improved significantly from 9.7 ± 6.1 points before treatment to 6.9 ± 4.6 points after treatment ( P = 0.0013). A depressive tendency was observed in six patients (15%) before growth hormone treatment, and in two patients (5%) after treatment. No significant relationship was observed between the decrease in the score and the length of the treatment. A significant improvement was observed for 6 of the 18 items in the Depression Self-Rating Scale for Children.
Conclusion:   A depressive tendency was relatively common in children with growth hormone deficiency, and the Depression Self-Rating Scale for Children was decreased after growth hormone treatment. These results suggest that growth hormone treatment may have positive effects on the psychosocial aspects in children with growth hormone deficiency.     

生长激素缺乏症患儿重组人生长激素治疗前、后肾上腺皮质功能的变化

目的观察生长激素缺乏症(GHD)患儿重组人生长激素(rh GH)治疗前、后肾上腺皮质功能的变化。方法选取72例确诊GHD并接受rh GH治疗6个月以上的患儿,其中32例伴促肾上腺皮质激素(ACTH)缺乏,回顾性分析其在接受rh GH治疗前及治疗后3、6个月时清晨空腹血皮质醇(COR)、ACTH水平的变化。结果 32例伴ACTH缺乏患儿通过外源性补充氢化可的松(HC)使COR达正常水平后,再开始rh GH治疗,治疗前COR水平和使COR达正常下限时的HC剂量呈显著负相关(r=-0.899,P0.01)。单纯HC治疗1个月后COR水平较治疗前明显增高,ACTH水平明显下降,差异均有统计学意义(P0.001);经rh GH和HC替代治疗后3、6个月后COR及ACTH水平与单纯HC治疗1个月差异无统计学意义(P0.05)。40例无ACTH缺乏患儿在rh GH治疗后COR水平显著降低,与治疗前比较差异有统计学意义(P0.01),其中10例MRI显示下丘脑-垂体异常患儿表现为COR水平低下。结论 GHD患儿在rh GH治疗过程中可出现肾上腺皮质功能减低,特别是MRI显示垂体异常的患儿,应注意监测肾上腺皮质功能,及早干预。     

磁共振成像在生长激素缺乏症中的应用

磁共振成像(MRI)技术可观察患儿垂体大小、形态、结构的变化及与周围结构的关系,结合多种激素检查,对于生长激素缺乏症的精确诊断、合理治疗及其预后的判断均有较大价值.生长激素缺乏所致的侏儒症是儿童矮小最常见的原因之一,常见原因为原发性垂体发育不良、空蝶鞍、颅咽管瘤.     

生长激素缺乏儿童在生长激素治疗中血清瘦素水平的改变

为了观察生长激素对血清瘦素 (leptin)的影响 ,用酶联法测定33例青春期前正常儿童和12例生长激素缺乏(GHD)患儿在治疗前、治疗后血清leptin水平 ;同时观察了患儿BMI与leptin的关系。结果显示 ,正常青春期前儿童血清leptin水平为 (1.22±0.94)ng/ml;治疗前GHD患儿血清leptin水平为 (3.079±2.407)ng/ml,与对照组相比差异无显著性(P>0.05) ;GHD患儿治疗后1、3、6个月血清leptin水平分别为 (1.643±1.367)ng/ml、(1.571±1.397)ng/ml和 (1.349±0.893)ng/ml,与治疗前相比差异有显著性 (P均<0.001)。提示生长激素对leptin的表达有抑制作用     

Long-term response to human growth hormone in 36 children with idiopathic growth hormone deficiency

The results of long term treatment with human growth hormone (Crescormon^®, 12.4 IU/m²/week) in 36 patients with idiopathic growth hormone deficiency are given. Birth trauma—in particular assisted breech delivery (30%)—is the major aetiological cause. Twelve patients had isolated growth hormone deficiency (IGHD), 24 had multiple pituitary hormone deficiencies (MPHD) of which 19 were treated with additional thyroid hormones. The results were judged by the criteria of height velocity, total height gain and change of height prediction (TW₂, age based).It is concluded that the growth hormone dose chosen in many cases is insufficient to maintain high growth rates after the first year of treatment, when catch-up no longer takes place. The tendency of patients supplemented with thyroid hormone to grow better—without additional bone-age advancement—calls for careful search for hypothyroidism and suggests the use of thyroxin in cases of doubt.