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身材矮小患者对人生长激素短期代谢反应的诊断和预后价值

Diagnostic and prognostic value of short-term metabolic response to human growth hormone in short stature.

作者信息

Clayton B E, Tanner J M, Vince F P

出版信息

Arch Dis Child. 1971 Aug;46(248):405-13. doi: 10.1136/adc.46.248.405.

DOI:10.1136/adc.46.248.405
PMID:5565454
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1647762/
Abstract

Metabolic tests of the response to three days of administration of human growth hormone (HGH) have been done on 55 patients aged 6·2 to 20·3 years. 22 had `isolated' growth hormone deficiency (or hyposomatotrophic short stature, HS), 16 CNS tumours or multiple hormone deficiencies, 6 short stature associated with low birthweight, 3 psychosocial short stature, 4 Turner's syndrome, 1 hereditary short stature, and 3 uncertain diagnosis. Height was measured at 3-monthly intervals for a full year, then HGH was given for a full year and the difference in rate, that is the acceleration during the growth hormone year, was calculated. The height measurements were all done by one measurer in 45 of the patients. In the metabolic test there were 5 baseline days followed by 3 days of a single injection of 10 IU HGH, then 2 final days. The means for urinary nitrogen excretion, blood urea, and urinary calcium excretion were calculated for the 5 baseline days and for the last 2 HGH days plus the one subsequent day. The difference between the means is given as a percentage of baseline values. The children with isolated growth hormone (GH) deficiency had a greater decrease in nitrogen excretion (33±3%) than the low birthweight cases and small children (7±5%), but there was overlap between individuals in the isolated deficiency group (range 12 to 66%) and the rest (range -12% to 42%); 2 deficient children were below a dividing line of 20%, and 1 Turner's syndrome and 3 psychosocial short stature children were above it. The tumour and multiple deficiency patients had an average fall of 40±3% and showed no overlap with the low birthweight group. Blood urea and calcium excretion gave a worse separation. Within the isolated deficiency and the tumour and multiple deficiency groups there was no relation between any metabolic parameter and the amount of growth acceleration in the first year of HGH treatment. In the HS, though not in the tumour patients, there was a correlation (-0·60) between the decrease in percentage nitrogen excretion and peak GH level on stimulation and between per cent decrease in urinary nitrogen excretion and per cent decrease in blood urea (0·76). We conclude that the differential diagnostic value of the short-term metabolic test is very limited now that we have tests of GH response; and that in cases of isolated GH or multiple pituitary hormone deficiency the result of the metabolic test does not predict at all the height acceleration obtained on HGH treatment.

摘要

对55名年龄在6.2至20.3岁的患者进行了为期三天的人生长激素(HGH)给药反应的代谢测试。22例为“单纯性”生长激素缺乏(或生长激素不足性矮小症,HS),16例为中枢神经系统肿瘤或多种激素缺乏,6例为出生体重低相关的矮小症,3例为心理社会性矮小症,4例为特纳综合征,1例为遗传性矮小症,3例诊断不明。身高每三个月测量一次,为期一整年,然后给予HGH一整年,并计算生长速度的差异,即生长激素治疗年期间的生长加速情况。45例患者的身高测量均由一名测量者完成。在代谢测试中,先有5个基线日,随后是3天单次注射10 IU HGH,然后是2个终末日。计算5个基线日以及最后2个HGH日加随后1天的尿氮排泄、血尿素和尿钙排泄的平均值。平均值之间的差异以基线值的百分比表示。单纯性生长激素(GH)缺乏的儿童氮排泄量的下降幅度(33±3%)大于低出生体重病例和幼儿(7±5%),但单纯性缺乏组个体(范围为12%至66%)与其他组(范围为-12%至42%)之间存在重叠;2例GH缺乏儿童低于20%的分界线,1例特纳综合征儿童和3例心理社会性矮小症儿童高于该分界线。肿瘤和多种激素缺乏患者平均下降40±3%,与低出生体重组无重叠。血尿素和钙排泄的区分效果较差。在单纯性缺乏组以及肿瘤和多种激素缺乏组中,任何代谢参数与HGH治疗第一年的生长加速量之间均无关联。在HS组中,尽管肿瘤患者中不存在,但氮排泄百分比下降与刺激后GH峰值水平之间以及尿氮排泄百分比下降与血尿素百分比下降之间存在相关性(-0.60和0.76)。我们得出结论,鉴于我们有GH反应测试,短期代谢测试的鉴别诊断价值现在非常有限;并且在单纯性GH缺乏或多种垂体激素缺乏的病例中,代谢测试结果根本无法预测HGH治疗所获得的身高加速情况。

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Effect of human growth hormone treatment for 1 to 7 years on growth of 100 children, with growth hormone deficiency, low birthweight, inherited smallness, Turner's syndrome, and other complaints.对100名患有生长激素缺乏症、低出生体重、遗传性身材矮小、特纳综合征及其他病症的儿童进行1至7年生长激素治疗的效果
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Urinary hydroxyproline in children with growth hormone deficiency. Clinical value in diagnosis and prognosis.生长激素缺乏症患儿的尿羟脯氨酸。在诊断和预后中的临床价值。
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Changes in body composition and energy expenditure after six weeks' growth hormone treatment.六周生长激素治疗后身体成分和能量消耗的变化。
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本文引用的文献

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Growth hormone-induced nitrogen retention in children of short stature.生长激素诱导的身材矮小儿童的氮潴留
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Acute metabolic response to human growth hormone in different types of dwarfism.不同类型侏儒症患者对人生长激素的急性代谢反应。
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The metabolic effect of a small uniform dose of human growth hormone in hypopituitary dwarfs and in control children. I. Nitrogen, alpha-amino-N, creatine-creatinine and calcium excretion and serum urea-N, alpha-amino-N, inorganic phosphorus and alkaline phosphatase.小剂量统一剂量人生长激素对垂体性侏儒症患儿及对照儿童的代谢影响。I. 氮、α-氨基氮、肌酸-肌酐及钙的排泄以及血清尿素氮、α-氨基氮、无机磷和碱性磷酸酶
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