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Disturbance of fluid and electrolyte transport in cystic fibrosis epithelia. 囊性纤维化上皮中液体和电解质运输的紊乱。
Pub Date : 1989-01-01 DOI: 10.1111/apa.1989.78.s363.10
J Bijman
Studies of the disease CF suggest that the basic defect is related to impaired electrolyte movement in the epithelia of a variety of organs with exocrine function. The disturbances of electrolyte secretion in the organs classically involved in CF range from 1) a decrease in secretion‐ or uptake of chloride ions in all the organs studied; 2) an increase in sodium uptake in nasal airway epithelium and 3) a decrease in bicarbonate output of the pancreas. In this review an overview is presented of the expression of the CF defect, the abnormalities of fluid and electrolyte secretion in each CF affected organ are considered in more detail with particular emphasis on the hormonal and neuronal (dys)regulation of ion transport systems in epithelial cells.
对CF疾病的研究表明,这种基本缺陷与多种具有外分泌功能的器官上皮中电解质运动受损有关。典型的与CF有关的器官中电解质分泌的紊乱包括:1)在所研究的所有器官中氯离子的分泌或摄取减少;2)鼻气道上皮钠摄取增加,3)胰腺碳酸氢盐输出减少。在这篇综述中概述了CF缺陷的表达,更详细地考虑了每个CF影响器官中液体和电解质分泌的异常,特别强调了上皮细胞中铁运输系统的激素和神经元(天)调节。
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引用次数: 5
Disease-specific growth charts--do we need them? 特定疾病生长图表——我们需要它们吗?
Pub Date : 1989-01-01 DOI: 10.1111/j.1651-2227.1989.tb11236.x
M B Ranke
A physician’s training and experience leads him to follow a particular path when confronted with a problem. The complaints are discussed, the history is taken, symptoms are investigated, the diagnosis is made, treatment is recommended and, finally, the prognosis is given. When the patient’s problems are related to growth, there is no reason to take a different approach. The medical pathway, however, is never one-dimensional. Like any hormonally regulated metabolic pathway, there is feedback interaction and interdependence between various factors. The situation is made even more complex when time is taken into consideration. In view of such a complex situation, the question of whether disease-specific growth charts (DSGCs) are needed requires a more sophisticated answer than yes or no. The exact nature of the role that disease-specific growth charts may play in the interactional process from presentation to prognosis needs to be considered.
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引用次数: 27
Long-term growth in small-for-date children. 长期成长的小约会的孩子。
Pub Date : 1989-01-01 DOI: 10.1111/j.1651-2227.1989.tb17164.x
P M Fitzhardinge, S Inwood

The growth patterns of 158 infants with significant intrauterine growth retardation (IUGR) were studied for the first 2 years of life. Eighty-four infants were born after 36 completed weeks. All these full-term infants survived; complete follow-up data were obtained for 78. Acceleration of growth in weight began soon after birth and continued for an average of 6 months. Acceleration of linear growth began somewhat later, but was limited to the first 9 months. Twenty-three infants (29%) were still below the 5th centile for both weight and height by 2 years of age. There was a negative correlation between the neonatal ponderal index and length at 18 months for females only. Seventy-four infants were born prematurely, before 37 weeks' gestation. Mortality in this group was 18% and complete follow-up data were obtained for 49 of the 61 survivors. Birth weight was regained on average at 11 days; accelerated weight velocity began 4-6 weeks before the expected date of delivery (term date). The potential for catch-up growth lasted up to 9 months after the term date. By 18 months, however, 44% of these pre-term infants were still below the 5th centile for weight. Size at 18 months post-term was correlated with weight at the term date and length at 3 months post-term, but not with the degree of IUGR or with the ponderal index.

本文对158例明显宫内生长迟缓(IUGR)婴儿出生后2年的生长模式进行了研究。84名婴儿在36周后出生。所有这些足月婴儿都存活了下来;78例获得完整随访资料。出生后不久体重就开始加速增长,平均持续6个月。线性增长的加速开始稍晚,但仅限于前9个月。23名婴儿(29%)在2岁时体重和身高仍低于第5百分位数。仅雌性在18月龄时,新生儿膀胱指数与体长呈负相关。74名婴儿在妊娠37周之前早产。该组的死亡率为18%,61名幸存者中有49名获得了完整的随访数据。出生体重平均在第11天恢复;体重加速速度在预产期前4-6周开始。在任期结束后的9个月里,追赶型增长的潜力依然存在。然而,到18个月时,44%的早产儿体重仍低于第5百分位。分娩后18个月的体重与分娩时的体重和分娩后3个月的体长相关,但与IUGR程度和ponderal指数无关。
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引用次数: 122
Growth hormone treatment of short stature: state-of-the-art in 1989. Proceedings of a meeting. Stockholm, October 19-20, 1989. 生长激素治疗矮小:1989年最先进的。会议记录。斯德哥尔摩,1989年10月19日至20日。
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引用次数: 0
Investigation of growth hormone secretion in patients with intrauterine growth retardation. 宫内生长迟缓患者生长激素分泌的研究。
Pub Date : 1989-01-01 DOI: 10.1111/j.1651-2227.1989.tb17167.x
P Rochiccioli, M Tauber, V Moisan, C Pienkowski

Growth hormone (GH) deficiencies have rarely been reported in intrauterine growth retardation (IUGR). This study has investigated GH secretion using GH provocation tests, 24-hour GH secretory profiles, and insulin-like growth factor I (IGF-I) measurements in 24 children with intrauterine growth retardation. The criteria for diagnosis were a birth length and weight below the 10th percentile for gestational age. The average age at investigation was 5.5 years, and the average growth retardation was -3.3 SD. Twenty children had shown catch-up growth between the ages of 6 months and 3 years, followed by varying decreases in growth velocity. Studies of GH secretion demonstrated GH deficiency in 16 patients, with neurosecretory dysfunction in six. Treatment with pituitary GH in nine children increased mean growth velocity from 3.5 cm/year to 7 cm/year. GH therapy should thus be effective in improving the height prognosis of children with intrauterine growth retardation.

生长激素(GH)缺乏很少报道在宫内生长迟缓(IUGR)。本研究利用生长激素激发试验、24小时生长激素分泌谱和胰岛素样生长因子I (IGF-I)测量24例宫内生长迟缓儿童的生长激素分泌情况。诊断标准是出生长度和体重低于胎龄的第10个百分位数。调查时平均年龄为5.5岁,平均生长发育迟缓为-3.3 SD。20名儿童在6个月到3岁之间表现出追赶性生长,随后生长速度出现不同程度的下降。生长激素分泌的研究表明,生长激素缺乏的16例患者,神经分泌功能障碍的6例。9例儿童垂体激素治疗使平均生长速度从3.5 cm/年增加到7 cm/年。因此,生长激素治疗对改善宫内生长迟缓患儿的身高预后是有效的。
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引用次数: 43
Host response to Pseudomonas aeruginosa. 宿主对铜绿假单胞菌的反应。
Pub Date : 1989-01-01 DOI: 10.1111/apa.1989.78.s363.37
G Döring

Patients with cystic fibrosis (CF) do not reveal a primary immune defect and respond with high numbers of functional polymorphonuclear leukocytes (PMN) and specific antibodies to lung infection with Pseudomonas aeruginosa. The mucoid character of P. aeruginosa, an altered epithelial cell surface, and high concentrations of PMN-derived lysosomal enzymes contribute to impaired bacterial lung clearance and result in chronic infection. Released PMN-elastase inactivates exotoxin A, the major toxin of P. aeruginosa, thus reducing its virulence. The imbalance between PMN-proteinases and their inhibitors leads to lung tissue damage, impaired opsonophagocytosis, and T-cell and B-cell imbalance. New therapeutical concepts in CF therefore combine anti-inflammatory drugs with effective antibiotics.

囊性纤维化(CF)患者不表现出原发性免疫缺陷,并对铜绿假单胞菌肺部感染产生大量功能性多形核白细胞(PMN)和特异性抗体。P. aeruginosa的粘液特性,上皮细胞表面的改变,以及高浓度的pmn衍生的溶酶体酶有助于细菌肺部清除受损并导致慢性感染。释放的pmn弹性酶使铜绿假单胞菌的主要毒素外毒素A失活,从而降低其毒力。pmn蛋白酶及其抑制剂之间的不平衡导致肺组织损伤,调理吞噬功能受损,t细胞和b细胞失衡。因此,CF的新治疗理念是将抗炎药物与有效的抗生素结合起来。
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引用次数: 6
Cell multiplication and differentiation. 细胞增殖和分化。
Pub Date : 1989-01-01 DOI: 10.1111/j.1651-2227.1989.tb17161.x
D J Hill
Embryonic and fetal growth involves a complex interaction of mitogenesis, tissue induction, cell migration and differentiation which occurs within a temporal programme. These tissue interactions are controlled by the extracellular matrix, cell-cell recognition systems, and the release of hormonal messengers such as peptide growth factors. The growth factors include peptides which potentiate growth, such as the insulin-like growth factors (IGFs), and also growth inhibitors, such as transforming growth factor-p. Additionally, some growth factors potentiate and some block tissue differentiation. Studies of whole embryos have shown that growth factors are present and are withdrawn in particular structures at predetermined times. However, discrepancies may exist between sites of synthesis and action due to growth factor distribution being influenced by cellular binding mechanisms. A picture emerges of a paracrine system of growth control which may be controlled by external influences, including insulin and nutritional metabolites. The most obvious parameter of embryonic and fetal growth is the size of the conceptus. However, increasing mass is only one aspect of a complex series of events that constitute early growth. During blastulation inductive effects cause the appearance of the mesoderm from the primitive ectoderm. From this point on the growth of the embryo is asymmetrical, some tissues remaining in a rapidly growing and relatively undifferentiated state while others are differentiated and functionally active. For instance, the anterior somite blocks form and differentiate while the posterior somites have yet to condense. The events of mitogenesis, induction and differentiation therefore take place within the additional developmental dimension of time. At least three primary control mechanisms have been identified; the deposition and subsequent modification of extracellular matrix molecules, the expression and withdrawal of cell-cell recognition molecules, such as the cadherins, and the selective expression of humoral intercellular messengers, such as peptide growth factors.
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引用次数: 2
The Wessex Growth Study: first report. 威塞克斯成长研究:第一份报告。
Pub Date : 1989-01-01 DOI: 10.1111/j.1651-2227.1989.tb17171.x
L Voss, J Walker, H Lunt, T Wilkin, P Betts

The Wessex Growth Study is a community-based longitudinal survey of short children recruited from two Health Districts in Wessex during 1985-86 (cohort I) and 1986-87 (cohort II). Screening of new school entrants during 1985-86 identified only 1.3% who were at or below the 3rd centile for height as defined by Tanner and Whitehouse, suggesting a strong secular trend and an urgent need for updated height charts. After exclusion of the small number of children with underlying organic pathology and those from ethnic minorities, there were 84 children in cohort I on whom this report is based. These apparently normal, short children were sex- and age-matched with normal controls (10th-90th centile) from the same school class. Forty-two per cent of cohort I had a delayed bone age, and 34% lay above the 3rd centile after correction for parental height. Forty-four per cent were of low birth weight. The correlation between two successive height velocities measured at 6 and 12 months was only -0.14 for cohort I and -0.15 for their controls. Twelve-month mean height velocity SD scores (SDS) were -0.45 and +0.25, respectively, corresponding to the 33rd and 60th centiles, and rather higher than the 25th and 50th centiles expected in view of the heights of these children. Thirty-eight per cent of cohort I had a 12-month height velocity below the 25th centile, and in 16% height velocity was below the 10th centile. As a group, the children in cohort I grew more slowly than their controls, but the height velocity in 88% of cases lay within the control range.(ABSTRACT TRUNCATED AT 250 WORDS)

韦塞克斯生长研究是一项以社区为基础的纵向调查,调查对象是1985-86年(队列I)和1986-87年(队列II)期间从韦塞克斯两个卫生区招募的矮个子儿童。1985-86年期间对新入学儿童的筛查发现,只有1.3%的人身高在Tanner和Whitehouse定义的第3个百分点或以下,这表明长期趋势很强,迫切需要更新身高图表。在排除了少数有潜在器质性病理的儿童和少数民族儿童后,本报告所依据的队列1中有84名儿童。这些看起来正常、矮小的孩子在性别和年龄上与来自同一学校班级的正常对照(第10 -90百分位)相匹配。队列1中42%的人骨龄延迟,34%的人在父母身高校正后超过了第3百分位。44%的婴儿出生时体重过轻。在6个月和12个月时测量的两次连续高度速度之间的相关性在队列I中仅为-0.14,在对照组中为-0.15。12个月平均身高速度SD评分(SDS)分别为-0.45和+0.25,对应于第33和60百分位,而考虑到这些儿童的身高,预期的第25和50百分位要高得多。队列1中有38%的人12个月的身高速度低于第25百分位,16%的人身高速度低于第10百分位。作为一个群体,队列1的孩子比对照组长得慢,但88%的病例的身高速度在控制范围内。(摘要删节250字)
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引用次数: 38
Growth survey of schoolchildren in Akita Prefecture: preliminary report. 秋田县学童成长调查:初步报告。
Pub Date : 1989-01-01 DOI: 10.1111/j.1651-2227.1989.tb17172.x
T Okamura

This report presents the results of a preliminary epidemiological study of growth in Akita prefecture. Data were collected from 5672 pupils from nine elementary schools in the region. Previous epidemiological investigations of growth have set the screening standard for growth retardation at -2.58 SD below the mean. On the basis of this figure it was estimated that 22 out of the 5672 pupils screened in this preliminary study (0.39%) would suffer from growth retardation. When this standard is applied to all 102,193 elementary school students in Akita prefecture, 396 children in the region are estimated to be growth retarded. An epidemiological investigation in Niigata prefecture in 1985 found that 10.5% of growth-retarded patients suffered from growth hormone deficiency. While this percentage is known to vary between different studies and with different methods of statistical analysis, application of this rate to the estimated number of 396 growth-retarded patients in Akita prefecture suggests that there may be 41 or 42 patients with growth hormone deficiency.

本报告介绍了秋田县生长的初步流行病学研究结果。数据来自该地区9所小学的5672名学生。以往的生长流行病学调查将生长迟缓的筛查标准定为比平均值低-2.58 SD。根据这一数字,在初步研究中筛选的5672名学生中,估计有22名(0.39%)患有生长迟缓。如果以秋田县全部102193名小学生为对象,则该地区有396名发育迟缓儿童。1985年在新泻县进行的流行病学调查发现,10.5%的生长迟缓患者患有生长激素缺乏症。虽然这一比例在不同的研究和不同的统计分析方法中有所不同,但将这一比例应用于秋田县396名生长迟缓患者的估计数字表明,可能有41或42名患者患有生长激素缺乏症。
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引用次数: 2
Key issues in child growth: some new developments. Proceedings from a one-day research workshop on human growth and development. Exeter, England, July 1st 1986. 儿童成长的关键问题:一些新的发展。为期一天的人类生长与发展研究研讨会的会议记录。1986年7月1日,英国埃克塞特。
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引用次数: 0
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Acta paediatrica Scandinavica. Supplement
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