正常青春前期、青春后期儿童血中Leptin水平与生长激素结合蛋白关系的研究

目的研究Ｌｅｐｔｉｎ与营养、生长的关系。方法对４９例正常青春前期及４８例正常青春后期儿童进行身高、体重测量及血Ｌｅｐｔｉｎ、生长激素结合蛋白（ＧＨＢＰ）检测。结果正常青春前期儿童血Ｌｅｐｔｉｎ水平在女孩（４９１±２７９）μｇ／Ｌ,较男孩（３７１±３５５）μｇ／Ｌ略高,但无显著性差异（Ｐ＞００５）;在青春后期女孩（１２７９±７７２）μｇ／Ｌ,较男孩（４３４±３２５）μｇ／Ｌ显著升高（Ｐ＜００１）。无论青春前期还是青春后期男、女孩血中Ｌｅｐｔｉｎ水平均与ＧＨＢＰ、体重指数（ＢＭＩ）显著相关。结论Ｌｅｐｔｉｎ很可能是一种营养与生长间的调节因子并且可能具有多种功能。     

rhG—CSF在小儿ANLL强烈化疗中的应用

为探讨ｒｈＧ－ＣＳＦ对小儿ＡＮＬＬ强烈化疗后粒细胞缺乏的疗效，采用ＡＡＥ方案（ＡＤＭ、Ａｒａ－Ｃ、ＶＰ１６或ＶＭ２６），化疗后当ＷＢＣ〈１×１０＾９／Ｌ或ＡＮＣ〈０．５×１０＾９／Ｌ时，给予ｒｈＧ－ＣＳＦ２００μｇ／ｍ＾２·ｄ（５￣１０μｇ／ｋｇ·ｄ），皮下注射，一般连续５￣１０天。本文１５例ＡＮＬＬ，用ｒｈＧ－ＣＳＦ３０例次。用ｒｈＧ－ＣＳＦ前，ＷＢＣ平均０．７８×１０＾９／Ｌ、ＡＮＣ０．１５×     

甲状腺素结合球蛋白缺乏致婴儿持续性甲低

婴儿甲状腺素结合球蛋白（ＴＢＧ）缺乏的发生率为１／５０００～１／１２０００,可造成甲状腺素（Ｔ４）、三碘甲腺原氨酸（Ｔ３）总量降低,游离甲状腺激素正常,无需另外补充。如新生儿Ｔ４低、促甲状腺素（ＴＳＨ）和游离Ｔ４（ｆＴ４）正常即可确诊。ＴＳＨ测定可发现早期甲低但不能发现ＴＢＧ缺乏。现报告１例ＴＢＧ缺乏致持续性甲低的婴儿。 病例男婴,足月,正常产,生后第２天甲状腺筛查试验异常（Ｔ４４３μｇ／Ｌ）,ＴＳＨ １４．１ＩＵ／Ｌ（先天性甲低早期＞２９Ｉ／Ｌ）。生后第９天 Ｔ４ １８μｇ／Ｌ,ＴＳＨ１２．３ＩＵ…     

保健日光灯照射对婴幼儿血清25羟维生素D_3水平影响的研究

采用保健日光灯照射婴幼儿４４例，并设立未照射组小儿１８例作对照，观察血清２５羟维生素Ｄ３［２５（ＯＨ）Ｄ３］于实验前后的变化。结果：照射组２５（ＯＨ）Ｄ３平均增加（８０６±１９８）μｇ／Ｌ（Ｐ＜００１），而未照射组实验前后差值为（－２６７±１９８）μｇ／Ｌ。两组实验后比较亦有显著性差异。照射天数和有效暴露面积对保健日光灯预防佝偻病的效果亦有明显影响。     

抗生素对小儿呼气氢试验的影响

目的探讨抗生素对呼气氢试验（ＢＨＴ）的影响。方法应用ＨＤ１型呼气氢测定仪,检测抗生素应用前后７５例住院患儿空腹呼气氢（ＦＢＨ）值和其中２３例患儿乳果糖氢呼吸试验（ＬＨＢＴ）的结果,并与２３１例健康儿ＦＢＨ比较。结果抗生素应用后ＦＢＨ［（１９５０±１２４）×百万分浓度（１０－６）］较应用前（３５９４±２９８）×１０－６明显降低（ｔ＝４９５,Ｐ＜０００１）;住院患儿抗生素应用前ＦＢＨ（３５９４±２９８）×１０－６与健康儿（１４５２±０１０）×１０－６比较,（ｕ＝４８３,Ｐ＜００１）;应用抗生素患儿口服乳果糖后呼气氢值无明显变化。结论住院患儿抗生素应用前,ＦＢＨ较健康患儿明显升高;抗生素应用后ＦＢＨ较应用前明显降低,ＬＨＢＴ呈假阴性结果。提示肠道氢产生受抑制。     

新生儿缺氧缺血性脑病血清LDH、CK与同功酶活性的变化及其临床意义

目的探讨新生儿缺氧缺血性脑病（ＨＩＥ）患儿血清乳酸脱氢酶（ＬＤＨ）、肌酸激酶（ＣＫ）及其同功酶活性检测的临床应用价值。方法对４７例ＨＩＥ患儿和３９例正常新生儿采用比色法和电泳法测定其血清中总ＬＤＨ、ＣＫ及其同功酶活性。结果中至重度ＨＩＥ血清总ＬＤＨ、ＣＫ及同功酶ＬＤＨ３、ＬＤＨ４、ＬＤＨ５、ＣＫ－ＢＢ较正常儿明显增高（Ｐ＜００５）,ＬＤＨ１、ＬＤＨ２、ＣＫ－ＭＭ、ＣＫ－ＭＢ仅在重度ＨＩＥ组较正常儿显著增高（Ｐ＜００１）;ＨＩＥ治疗后７天血清ＬＤＨ３、ＬＤＨ４、ＬＤＨ５、ＣＫ－ＭＭ、ＣＫ－ＭＢ、ＣＫ－ＢＢ较出生４８ｈ内明显下降（Ｐ＜００５）,但血清总ＬＤＨ、ＬＤＨ１、ＬＤＨ２、ＬＤＨ３、ＣＫ－ＭＢ、ＣＫ－ＢＢ仍高于同时相正常组（Ｐ＜００５）。结论ＨＩＥ时血清ＬＤＨ、ＣＫ及同功酶,特别是ＬＤＨ３、ＣＫ－ＢＢ可作为评价ＨＩＥ脑损害程度的敏感指标。     

一氧化氮与Ⅰ型辅助细胞因子在过敏性紫癜中的作用

目的观察过敏性紫癜（ＨＳＰ）患儿急性期的一氧化氮（ＮＯ）及Ⅰ型辅助细胞（ＴＨ１）因子水平,以探讨其在该病全身血管炎发病机制中的作用。方法用镉还原法测定血浆ＮＯ水平,用ＥＬＩＳＡ法检测血浆及外周血单个核细胞（ＰＢＭＣ）培养上清中白细胞介素－２（ＩＬ－２）及干扰素－γ（ＩＦＮ－γ）水平。结果ＨＳＰ急性期血浆ＮＯ水平较对照组明显增高（Ｐ＜００５）,血浆ＩＬ－２及ＩＦＮ－γ与正常对照组比较无显著性差异（Ｐ＞００５）,但患儿的ＰＢＭＣ培养上清的ＩＬ－２及ＩＦＮ－γ明显低于正常对照组（Ｐ＜００５）。结论ＮＯ及ＴＨ１细胞因子在ＨＳＰ全身血管炎的发生、发展中起作用     

应激状态下新生儿对不同剂量外源性脂肪代谢的研究

为了探讨应激状态下新生儿应用两种不同剂量脂肪乳剂时的脂肪代谢情况，以指导临床合理应用。将１７例胎龄＞３５周，手术后需ＴＰＮ＞１周的新生儿，随机分成低剂量脂肪乳剂组（１ｇ·ｋｇ~（－１）／ｄ）８例，普通剂量对照组（２ｇ·ｋｇ~（－１）／ｄ）９例。在ＴＰＮ前、ＴＰＮ第３天和第７天分别观察ＴＧ、ＴＣ、ＰＬ、ＦＦＡ、ＨＤＬ－Ｃ、ＬＤＬ－Ｃ、Ａｐｏ－Ａ_１和Ａｐｏ－Ｂ变化，结果低剂量组ＴＰＮ１周期间，ＴＧ、ＴＣ、ＰＬ变化不明显，而对照组ＴＧ、ＴＣ在ＴＰＮ第３天升高（但＜１．５０ｇ／Ｌ），第７天回到基值；ＰＬ持续升高，Ａｐｏ－Ａ_１／Ａｐｏ－Ｂ的比值１周变化为：低剂量组１．４７→１．６０→１．４１，对照组１．２０→０．８５→０．９６，它的变化与ＴＧ、ＴＣ等血脂变化相一致。从研究中得出，足月新生儿ＴＰＮ时选用１ｇ·ｋｇ~（－１）／ｄ对血脂几乎没有影响，用２ｇ·ｋｇ~（－１）／ｄ虽然有一过性升高，但仍＜１．５０ｇ／Ｌ，是安全的。     

高频振荡通气结合一氧化氮吸入对急性肺损伤兔氧合的影响

目的　判断高频振荡通气（ＨＦＯ）伴低浓度一氧化氮（ＮＯ） 吸入对急性肺损伤家兔气体交换和血液动力学参数的影响。方法　用３７ ℃温盐水反复进行支气管灌洗（按每次３０ ｍｌ／ｋｇ）制成急性肺损伤模型。应用高频振荡呼吸机和１×１０－６ＮＯ吸入对１０ 只急性肺损伤家兔进行实验性治疗,并测定肺力学、血液动力学等参数。结果　ＨＦＯ＋ ＮＯ 与急性肺损伤基础值比较：ＰａＯ２／ＦｉＯ２ 比值由１５６±３２ 上升至４０５±４８（Ｐ＜ ０－０１）、氧合指数（ＯＩ）和肺内动静脉分流量（Ｑｓ／Ｑｔ） 分别由７－８ ±１－７ 和０－４１±０－０６ 下降至３－７ ±０－８ 和０－３０±０－０５（ Ｐ均＜ ０－０１）。ＨＦＯ＋ ＮＯ 与单纯ＨＦＯ 比较：ＰａＯ２／ＦｉＯ２ 比值由３７９±８６ 上升至４０５±４８（ Ｐ＜ ０－０５） 、ＯＩ和Ｑｓ／Ｑｔ 分别由５－８ ±１－９ 和０－３５ ±０－０６ 下降至３－７ ±０－８ 和０－３０±０－０５（Ｐ均＜０－０５） 。结论　ＨＦＯ＋ ＮＯ吸入可改善急性肺损伤兔的氧合,肺内分流减少,吸入１×１０－６ ＮＯ有效。应用本系统质量流量控制器,可以在高频振荡通气时提供稳定浓度的ＮＯ气体。     

特发性身材矮小儿童GH治疗对成年后身高的影响

已有一些对身材矮小、不伴生长激素（ＧＨ）缺乏、归类为特发性矮小身材的儿童应用ＧＨ治疗的报道，但结果不一。短期治疗其生长率及身高标准差得分（ＳＤＳ）增加，但长期应用ＧＨ对成年身高的影响尚不清楚。该研究报道特发性身材矮小儿童应用生长激素治疗２～１０年后对成年身高的影响． 病人与方法研究包括１２１例特发性身材矮小儿童，治疗前身高在第３百分位以下，生长率低，生长激素刺激试验血清ＧＨ ≥ １０μｇ／／Ｌ。给予ＧＨ治疗（每周０．３ｍｇ／ｋｇ） ２～ １０年，其中 ８０例已达成年身高（男 ５７例，女２３例），男孩骨…     

Spontaneous growth in turner syndrome: Evidence for a minor pubertal growth spurt

Spontaneous growth of 141 untreated girls with Turner syndrome was analysed. Of the patients 25% were born prematurely; their weight and height were normal when compared to prematurely born healthy infants. However, birth weight and height was significantly retarded in Turner patients born at term. A curve for height and growth velocity for the age range 0–16 years was constructed with a sensitive statistical method. By use of a mathematical model equations were created for calculating z-scores and the related percentiles for the height of individual patients at given age. median height of 18 untreated patients at 18 years was 143.8 cm. Analysis of growth velocity revealed a minor but significant growth spurt at the age of 12.5 years. This growth spurt was also detectable in patients without signs of spontaneous puberty and occurred later in patients with 45,X0 karyotype. Bone age progression was linear up to the age of 7.5 years and decelerated thereafter.     

营养不良性生长迟缓生长追赶的相关因素分析

目的　了解营养不良性生长迟缓儿生长追赶的影响因素。方法　观察２８ 例营养不良性生长迟缓儿，在调整营养供应后至少４ 个月的身高体重增长与基础体格指标、骨龄和遗传因素的关系。结果　身高Ｚ评分增长与身高别体重Ｚ评分增长正相关（ Ｐ＜ ０－０１） ，与基础身高别体重Ｚ评分负相关（ Ｐ＜ ０－０１） ，与基础骨龄正相关（ Ｐ＜ ０－０５）；与年龄及遗传身高Ｚ评分不相关。体重Ｚ评分增长与基础身高别体重Ｚ评分负相关（ Ｐ＜ ０－０１） ，与骨龄正相关（ Ｐ＜０－０１）。身高增长速度可超过其年龄应有生长能力。部分患儿恢复期仍有高生长激素血症（ 营养不良所致促生长素轴代谢代偿重整状态的持续）。结论　对营养不良性生长迟缓应尽早供给足够热量和蛋白，以充分利用促生长素轴的代谢代偿转为促生长效应，获得最大追赶。     

Malignant thymoma in a patient with growth hormone deficiency during growth hormone therapy

Malignant thymoma was found in an 8-year-old Japanese boy with growth hormone (GH) deficiency who had received GH therapy for 3 years and 5 months. There may be a possible relationship between the occurrence of malignant thymoma and GH therapy.     

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     

Excessive growth in a child with craniopharyngioma and growth hormone deficiency

In a 5-year-old boy presenting with clumsiness and excessive growth, a large craniopharngioma was diagnosed. Biochemically, there was a deficiency of growth hormone, a hypothalamic hypothyroidism and hypocorticalism, a thyroxine binding globulin elevation, an abnormal gonadotropin secretion and a mild hyperprolactinaemia. After removal of the tumour growth stopped almost completely. Plasma insulin-like growth factor (IGF)-I was in the lower normal range. Plasma IGF-II decreased after tumour removal. It is speculated that the tumour produced a growth factor causing excessive growth.Abbreviations GH growth hormone - IGF insulin-like growth factor - TSH thyroid-stimulating hormone - SDS standard deviation score - TBG thyroxine binding globulin - LH luteinizing hormone     

Multicentric study of efficacy and safety of growth hormone use in growth hormone deficient children in India

Growth Hormone being very expensive in India data on use of recombinant human growth hormone (rhGH) is scarce. The authors studied the effect and safety of one year of therapy with rhGH on growth velocity and predicted final height in Indian patients with growth hormone deficiency (GHD). A multicentric, prospective, open trial with rhGH was performed on 15 patients. Patients received rhGH in a dose of 0.7 IU (0.23 mg)/Kg/week. The mean pretreatment height was 111.2cms {SD 12.4}, height velocity was 3.1 cms per year {1.2} and predicted height was 146.5 cms {10.4} at a mean age of 12.0 (2.8). At the end of therapy mean height was 123.4 {11.9}, height velocity was 12.1 cms per year {2.8} and the predicted height was 153.0 cm {9.4}. The increase in predicted height was thus 6.5cm (4.2). The increment in height velocity with growth hormone therapy was statistically significant (p value= 0.001). The present study shows that children with growth hormone deficiency in India also benefit from therapy with rhGH even when treatment is started late as compared to the published Western data and there is a potential for increased final height.     

Circulating immunoreactive growth hormone releasing hormone concentrations and growth hormone response to growth hormone releasing hormone in short children

To study the role of peripheral immunoreactive growth hormone releasing hormone (ir-GHRH) concentrations and the GHRH test in the evaluation of growth hormone (GH) secretion in short stature, 46 children with a mean age of 9.4 years (range 1.6–16.3 years) and a mean relative height score of –3.2 SD (range –5.0–2.1 SD) were investigated. The children were divided into prepubertal (n=35) and pubertal (n=11) and the prepubertal children further into three groups based on their maximal GH responses to insulin-induced hypoglycaemia (IIH) and clonidine: (1) GH deficient subjects (maximal GH<10 g/l in both test); (2) discordant responders (maximal GH<10 g/l in one test and 10 g/l in the other); and (3) normal responders (maximal GH10 g/l in both test). Peripheral ir-GHRH concentrations were measured during the IIH test by radioimmunoassay after purification of plasma samples on Sep-pak cartridges. Among the prepubertal children 10 fell into group 1, 16 into group 2 and 9 into group 3. Children in group 1 were older, than those in group 3. There were no significant differences in relative heights and weights or absolute and relative growth velocities between the groups. Subjects in groups 1 and 2 had lower maximal GH responses to GHRH than those in group 3. There were no significant differences in the basal plasma ir-GHRH concentrations between the groups. Nine children (19.6%) had somatotrophs with a poor response to a single dose of exogenous GHRH (maximal GH<10 g/l). These subjects had increased basal plasma ir-GHRH concentrations. All of them had a decreased GH response to IIH and/or clonidine. Pubertal children had higher circulating ir-GHRH levels than the prepubertal subjects. There was an inverse correlation (r=–0.46;P<0.001) between the maximal GH response to GHRH and calendar age in the whole series. These observations suggest that: (1) a substantial proportion of short children have a heterogenous GH response to pharmacological stimuli necessitating complementary evaluation of their spontaneous GH secretion; (2) a poor response to exogenous GHRH is associated with increased ir-GHRH levels in the peripheral circulation; (3) all children with normal GH responses in pharmacological tests respond normally to GHRH and (4) the pituitary sensitivity to GHRH decreases with increasing age. Peripheral ir-GHRH concentrations do not differentiate between short children with growth hormone deficiency (GHD) and those with undefined short stature. The GHRH test is of limited value in the diagnosis of GHD, since a normal GH response does not exclude GHD, although a subnormal response appears to reflect dysfunctional GH secretion.     

Postnatal growth failure in preterm infants: recovery of growth and body composition after term

BACKGROUND: Many preterm infants are significantly growth restricted at hospital discharge and are at increased risk for long-term growth failure. AIMS: To compare growth and weight gain composition after term between preterm infants who were growth retarded and those who were not. STUDY DESIGN: An observational longitudinal study was conducted. SUBJECTS: 35 preterm infants who showed growth retardation at term (group 1) and 26 preterm infants who did not (group 2). OUTCOME MEASURES: Growth and body composition were assessed at term and at 1, 2, 3, 4 and 5 months of corrected age. RESULTS: At term, and at 1, 2, and 3 months of corrected age, growth-retarded infants showed significantly lower body weight and fat mass than infants who did not develop growth retardation. The mean energy and protein intakes did not differ significantly between the two groups. Daily increases in body weight and fat mass between term and three months did not differ between the groups. However, during the fourth and fifth months, daily gains of body weight and fat mass were significantly greater in growth-retarded than in non-growth-retarded infants, and as a result, body weight and fat mass were comparable between the two groups at 4 and 5 months of corrected age. CONCLUSIONS: In terms of growth parameters and body composition, growth-retarded preterm infants recovered from postnatal growth failure within the fourth month of corrected age.     

Rheumatoid arthritis and growth retardation in children: Treatment with human growth hormone

Twenty patients with rheumatoid arthritis or Still's disease associated with growth failure were treated with human growth hormone, 7.5 to 17 U/m² body surface per week.Five patients did not respond with better growth. In the remainder the mean growth rate increased from 1.9 cm/year (range: 0 to 3.3) to 6.2 cm/year (range: 3.6 to 12) over 5 to 7 months. Twelve patients treated for longer periods increased their mean growth rate from 2.3 cm/year (range: 0.7 to 5.7) to 6.3 cm/year (range: 2.4 to 9.7) and continued to grow during a second year of treatment. Growth velocity decreased in 6 patients when the hGH therapy was discontinued.The causes for this improvement in growth are possibly multifactorial: the growth rate is depressed by the severity of the disease and high-dose glucocorticoid therapy. Increases of growth rate occured during improvements in the disease, reduction of steroid medication, as a result of therapy with human growth hormone, and because of puberty in some patients.Human growth hormone seemed to improve the underlying condition of four of the patients but had no influence on the disease in the remaining children.On the occasion of the 75th birthday of Prof. Dr. Dr. h. c. Adolf Butenandt     

Insulin secretion in children with growth retardation

The effect of tolbutamide administration on insulin secretion was studied in 69 children with growth retardation. Diminished insulin secretion was found in all the patients, compared to the control group. This insulin deficit was most evident in patients with isolated, total GH deficiency and least evident in children with idiopathic short stature. Intermediate values were found in dwarfism due to isolated, partial GH deficiency.These results favour the hypothesis that hypoinsulinism contributes to the somatotropin deficiency in causing growth retardation.Abbreviations PD pituitary dwarfism - FSS familial short stature - GR growth retardation - GH growth hormone - ISS idiopathic short stature - tbt tolbutamide - GHtd isolated, total GH deficiency - GHpd isolated, partial GH deficiency