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12th International Symposium on Growth and Growth Disorders. Geneva, 25-26 October 1991. 第十二届生长和生长障碍国际研讨会。1991年10月25日至26日,日内瓦。
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引用次数: 0
10th International Symposium on Growth and Growth Disorders. Proceedings of a meeting. Madrid, Spain, 19-20 October 1990. 第十届生长和生长障碍国际研讨会。会议记录。1990年10月19日至20日,西班牙马德里。
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引用次数: 0
The carbohydrate-deficient glycoprotein syndrome. A new inherited multisystemic disease with severe nervous system involvement. 碳水化合物缺乏糖蛋白综合征。一种新的遗传性多系统疾病,严重累及神经系统。
J Jaeken, H Stibler, B Hagberg
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引用次数: 0
Prevention and treatment of primary malnutrition in developing and industrialized countries. Papers presented at the Milupa Nutrition Workshop. Cairo, February 18-20, 1990. 在发展中国家和工业化国家预防和治疗初级营养不良。在Milupa营养研讨会上发表的论文。开罗,1990年2月18日至20日。
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引用次数: 0
Growth hormone treatment in short children with chronic renal failure and after renal transplantation: combined data from European clinical trials. The European Study Group. 生长激素治疗矮个子儿童慢性肾功能衰竭和肾移植后:来自欧洲临床试验的综合数据欧洲研究小组。
A Van Es

Growth retardation is common in children with chronic renal failure (CRF). To investigate the efficacy and safety of recombinant human growth hormone treatment in such children and in children after renal transplantation, 43 prepubertal children with CRF, and 30 prepubertal and 25 pubertal patients with a renal transplant were studied. Data are reported for 31, 26 and 17 of these patients, respectively. Median height velocity increased from 4.2 to 9.8 cm/year during the first year of treatment, and to 6.8 cm/year during the second year of treatment in the patients with CRF. In the prepubertal transplant group, median height velocity changed from 3.5 to 8.4 cm/year during the first year and to 5.4 cm/year during the second year. In the pubertal transplant group, median height velocity increased from 3.2 to 6.6 cm/year during the first year and was 4.5 cm/year during the second year. The gain in height SDS for the prepubertal children in both the CRF and transplant groups was approximately 1 SD over 2 years. Treatment was well tolerated, and renal function did not change significantly in any group.

生长迟缓在慢性肾功能衰竭(CRF)患儿中很常见。为了探讨重组人生长激素治疗慢性肾功能衰竭儿童及肾移植后儿童的疗效和安全性,我们对43例青春期前慢性肾功能衰竭儿童、30例青春期前肾移植患者和25例青春期前肾移植患者进行了研究。分别报道了其中31例、26例和17例患者的数据。在治疗的第一年,CRF患者的中位高度速度从4.2增加到9.8 cm/年,在治疗的第二年增加到6.8 cm/年。在青春期前移植组中,平均身高速度在第一年从3.5到8.4 cm/年变化,第二年为5.4 cm/年。在青春期移植组中,平均身高速度在第一年从3.2增加到6.6 cm/年,第二年增加到4.5 cm/年。在CRF组和移植组中,青春期前儿童的身高SDS在2年内增加了大约1个SD。治疗耐受性良好,两组患者肾功能均无明显变化。
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引用次数: 0
Growth response in prepubertal children with idiopathic growth hormone deficiency during the first two years of treatment with human growth hormone. Analysis of the Kabi Pharmacia International Growth Study. 特发性生长激素缺乏症的青春期前儿童在人类生长激素治疗的前两年的生长反应。Kabi Pharmacia国际生长研究分析。
M B Ranke, O Guilbaud

From the large database of patients enrolled in the Kabi Pharmacia International Growth Study (KIGS), 289 prepubertal patients with idiopathic growth hormone deficiency (GHD), treated for 2 years with growth hormone (GH) substitution therapy, were selected. A multiple regression analysis was performed to determine both the auxological factors characterizing the patients at the beginning of the first and second years on GH therapy and the respective treatment modalities relevant to the magnitude of the growth response. It was observed that during the first year on GH therapy the magnitude of the growth response was negatively correlated with chronological age and height SDS, and positively correlated with target height SDS, GH dose (IU/kg/week) and frequency of GH injections. During the second year the growth response was negatively correlated with chronological age and the first-year GH dose (IU/kg/week), and positively correlated with height velocity during the first year, GH dose (second year), and injection frequency (second year). The data suggest that the forces of 'catch-up'--auxologically entrenched within the distance between target height SDS and height SDS--no longer prevail during the second year of GH therapy. The inverse influence of the first-year GH dose in the two yearly phases of growth suggests that optimizing GH treatment must be attempted by analysing growth in response to GH over longer periods of time and considering that the growth process is influenced by interactive factors.

从Kabi Pharmacia国际生长研究(KIGS)的大型患者数据库中,选择289例特发性生长激素缺乏症(GHD)的青春期前患者,接受生长激素(GH)替代疗法治疗2年。进行了多元回归分析,以确定患者在生长激素治疗的第一年和第二年开始时的生长学因素特征,以及与生长反应程度相关的各自的治疗方式。我们观察到,在生长激素治疗的第一年,生长反应的大小与实足年龄和身高SDS呈负相关,与目标身高SDS、生长激素剂量(IU/kg/周)和生长激素注射频率呈正相关。在第二年,生长反应与实足年龄和第一年GH剂量(IU/kg/周)呈负相关,与第一年身高速度、GH剂量(第二年)和注射频率(第二年)呈正相关。数据表明,在生长激素治疗的第二年,“追赶”的力量——在生理上根深蒂固的目标身高SDS和身高SDS之间的距离——不再盛行。第一年生长激素剂量对两个年度生长阶段的反向影响表明,必须通过分析较长时间内生长对生长激素的反应,并考虑到生长过程受相互作用因素的影响,来尝试优化生长激素治疗。
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引用次数: 0
11th International Symposium on Growth and Growth Disorders. Proceedings of a meeting, Stockholm, Sweden, 26-27 April 1991. 第11届生长和生长障碍国际研讨会。1991年4月26日至27日,瑞典斯德哥尔摩,会议记录。
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引用次数: 0
The infancy-childhood growth spurt. 婴儿期和儿童期的生长突增。
J Karlberg

Early normal human linear growth is unstable and oscillates owing to an increase in growth rate between 6 and 12 months of age. This defines the onset of the infancy-childhood growth spurt (IC spurt). The onset is identical with the onset of the childhood component of the infancy-childhood-puberty growth model. The IC spurt is completed when the infancy component of this model has virtually ceased, at around 3 years of age. Additional observations suggest that its onset represents the age at which growth hormone (GH) begins to regulate growth significantly. The negative impact of late onset of the IC spurt on attained height is illustrated and discussed. A delayed spurt is observed in 30-50% of children with growth-related disorders and of infants living in a poor area of a developing country. The IC spurt is absent in children with GH deficiency who receive no hormonal therapy.

早期正常的人类线性生长是不稳定的,并且由于6至12个月之间生长速度的增加而振荡。这就定义了婴幼儿生长突增的开始。发病与婴儿期-儿童期-青春期生长模型中儿童期成分的发病相同。当这个模型的婴儿期几乎停止时,在大约3岁时,IC爆发就完成了。另外的观察表明,它的发病代表了生长激素(GH)开始显著调节生长的年龄。迟发的IC冲刺对达到的高度的负面影响进行了说明和讨论。在30-50%患有生长相关疾病的儿童和生活在发展中国家贫困地区的婴儿中,观察到发育迟缓。没有接受激素治疗的生长激素缺乏症儿童不存在IC爆发。
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引用次数: 0
Children with epilepsy as adults: outcome after 30 years of follow-up. 成人癫痫患儿:30年随访后的结果
M Sillanpää

The study dealt with a representative sample of the epileptic population in southwestern Finland, followed up prospectively for 23-39 years. At the end of the follow-up period, 55.5% of the original sample, i.e. 63.2% of the subjects who participated in the last follow-up evaluation, or 76.4% of those who were alive at that time, had not had epileptic attacks for at least the previous three years. A total of 60% lived independently; 21% of subjects, on the other hand, were not gainfully employed and lived in institutions. The intermediate group was the smallest (less than 18%); these were receiving a disability pension but had not been institutionalized. A certain polarization thus seemed to take place here: the epileptic patient either lead a normal life or was in institutional care. The intermediate group showed a steady decline in size in the course of the follow-up.

该研究涉及芬兰西南部癫痫人群的代表性样本,前瞻性随访23-39年。在随访期结束时,55.5%的原始样本,即参加最后一次随访评估的63.2%的受试者,或76.4%的当时活着的受试者,至少在过去三年没有癫痫发作。60%的人独立生活;另一方面,21%的受试者没有工作,住在机构里。中间组最小(小于18%);这些人领取残疾养恤金,但尚未制度化。因此,这里似乎出现了某种两极分化:癫痫病人要么过着正常的生活,要么在医疗机构接受治疗。在随访过程中,中间组的大小稳步下降。
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引用次数: 0
Rett syndrome in Sweden. Neurodevelopment--disability--pathophysiology. 在瑞典的Rett综合征。神经发育,残疾,病理生理学。
I W Engerström

Rett syndrome (RS), a complex and severely disabling neuromotor disorder affecting young girls, was not internationally recognized until in the middle eighties. In Austria and Sweden only, there are clinical experiences since the sixties. The aims of this study were to 1) define the nucleus group of RS in Sweden, 2) develop a frame of defined stages for describing the clinical progress of disease, 3) identify early developmental patterns and their predictivity, 4) give the natural history of neuromotor impairments and disability in adults, 5) analyse the period of clinical regression as to developmental profiles, patterns and progression of symptoms, 6) search informative neurophysiologic correlates to clinical symptoms and signs. By a 6 year (1984-90), nationwide search procedure, comprising an information and tracing program, a travelling consultant program, and a neuropediatric and laboratory examination program, 88 females were added to those 17, observed in Sweden during the sixties and seventies. By March, 1990, 105 females, aged 19 months-44 years (median 17 1/2 years), had entered this series, which was considered representative for the nucleus group of RS in Sweden. 97/105 females (92.3%) fulfilled the criteria for classic RS, according to internationally accepted clinical criteria, and 8 (7.7%), represented so called "formes frustes". The prevalence for classic RS in the south-west region of Sweden was 1:12.000-13.000 girls, the birth years 1965-76 and 1960-84. Based on 29 females with RS, aged 13-28 years, a clinical staging system was constructed and evaluated in 1985, emphasizing the main different phases of the disorder throughout life. This staging system was found a valuable tool for clinical assessment, documentation, and research approach, and has become internationally accepted and widely used. Further experiences have given rise to minor adjustments, which are proposed. By scrutinizing early documentation from 10 girls, at referral aged 20 months-6 1/4 years (median 3 1/4 years) and retrospectively applying the Denver Developmental Screening Test frames, early achievements, signs and symptoms were reconstructed in 1986. Although characteristic neuromotor and neurobehavioural patterns of help for early suspicion, and even subtle signs, possibly reflecting a disorder already during the first months of life, could be identified, predictive clinical manifestations of diagnostic value were not found.(ABSTRACT TRUNCATED AT 400 WORDS)

Rett综合征(RS)是一种影响年轻女孩的复杂且严重致残的神经运动障碍,直到80年代中期才得到国际承认。仅在奥地利和瑞典,自60年代以来就有临床经验。本研究的目的是:1)确定瑞典RS的核心群体;2)为描述疾病的临床进展制定明确的阶段框架;3)确定早期发育模式及其预测性;4)给出成人神经运动障碍和残疾的自然史;5)分析发育概况、模式和症状进展的临床退行期;6)寻找与临床症状和体征相关的信息丰富的神经生理学。通过为期6年(1984- 1990年)的全国搜索程序,包括信息和追踪方案、旅行顾问方案以及神经儿科和实验室检查方案,在60年代和70年代在瑞典观察到的17名女性中增加了88名女性。截至1990年3月,共有105名女性入组,年龄19个月~ 44岁(中位17.1 /2岁),被认为是瑞典RS核心组的代表。根据国际公认的临床标准,105名女性中有97名(92.3%)符合经典RS的标准,8名(7.7%)代表所谓的“frustes”。瑞典西南地区典型RS的患病率为1:12 000- 13000女孩,出生年份为1965-76年和1960-84年。1985年对29例年龄13 ~ 28岁的RS女性患者进行临床分期系统的构建和评价,强调RS在一生中的主要不同阶段。该分期系统被认为是临床评估、文献记录和研究方法的有价值的工具,并已被国际上接受和广泛使用。根据进一步的经验,提出了一些小的调整。通过仔细检查10名转诊时年龄在20个月至61又1/4岁(中位数为31又1/4岁)的女孩的早期文件,并回顾性地应用丹佛发育筛查试验框架,重建了1986年的早期成就、体征和症状。虽然可以识别出有助于早期怀疑的特征性神经运动和神经行为模式,甚至可能反映出生命最初几个月已经出现的疾病的细微迹象,但没有发现具有诊断价值的预测性临床表现。(摘要删节为400字)
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Acta paediatrica Scandinavica. Supplement
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