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重型地中海贫血症青春期前多次输血的矮小儿童的昼夜生长激素分泌情况

Circadian growth hormone secretion in short multitransfused prepubertal children with thalassaemia major.

作者信息

Katzos G, Harsoulis F, Papadopoulou M, Athanasiou M, Sava K

机构信息

Department of Paediatrics, Aristotle University, Hippokration General Hospital, Thessaloniki, Greece.

出版信息

Eur J Pediatr. 1995 Jun;154(6):445-9. doi: 10.1007/BF02029352.

Abstract

UNLABELLED

Growth hormone (GH) secretion was determined by evaluating circadian GH profiles for 24 h and GH responses to clonidine stimulation test and insulin-stimulated hypoglycaemia (ITT), in nine prepubertal children with beta-thalassaemia major (TM) and 17 with non-GH deficient short stature (NGHDSS). The TM children were multitransfused and had early and intensive chelation therapy. All patients had normal hypoglycaemia to ITT, with peak GH levels of 15.71 +/- 5.86 ng/ml for children with NGHDSS and 13.91 +/- 7.20 ng/ml for children with TM. Peak GH levels during a clonidine test were 17.54 +/- 5.30 and 17.15 +/- 1.38 ng/ml, respectively. The GH peak parameters such as the number of peaks, the integrated GH concentration and the area under the curve (AUC) were similar in both groups of children and reflected the total 24-h secretion and the daily and nocturnal secretion separately. An abnormal 24-h GH profile compatible with the diagnosis of endogenous neurosecretory GH dysfunction was found in only two out of nine children with TM and in four out of seven children with NGHDSS.

CONCLUSION

Our data suggest that growth hormone neurosecretory dysfunction is not a universal finding in children with thalassaemia major but might depend on the degree of iron deposit in the pituitary.

摘要

未标记

通过评估9名重型β地中海贫血(TM)青春期前儿童和17名非生长激素缺乏性身材矮小(NGHDSS)儿童的24小时昼夜生长激素(GH)谱以及GH对可乐定刺激试验和胰岛素刺激低血糖(ITT)的反应来确定GH分泌情况。TM儿童接受了多次输血并进行了早期强化螯合治疗。所有患者对ITT的低血糖反应均正常,NGHDSS儿童的GH峰值水平为15.71±5.86 ng/ml,TM儿童为13.91±7.20 ng/ml。可乐定试验期间的GH峰值水平分别为17.54±5.30和17.15±1.38 ng/ml。两组儿童的GH峰值参数,如峰值数量、GH浓度积分和曲线下面积(AUC)相似,分别反映了24小时总分泌量以及白天和夜间分泌量。仅9名TM儿童中的2名和7名NGHDSS儿童中的4名发现了与内源性神经分泌性GH功能障碍诊断相符的异常24小时GH谱。

结论

我们的数据表明,生长激素神经分泌功能障碍在重型地中海贫血儿童中并非普遍存在,可能取决于垂体中铁沉积的程度。

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