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儿童颅咽管瘤的内分泌及生长特征:一项单机构研究

Endocrine and growth features in childhood craniopharyngioma: a mono-institutional study.

作者信息

Di Battista Eliana, Naselli Arturo, Queirolo Sara, Gallarotti Federica, Garré Maria Luisa, Milanaccio Claudia, Cama Armando

机构信息

Auxoendocrinological Centre, Paediatric Department, University of Genoa, School of Medicine, Italy.

出版信息

J Pediatr Endocrinol Metab. 2006 Apr;19 Suppl 1:431-7.

Abstract

OBJECTIVE

To evaluate growth and endocrine features in children with craniopharyngioma who were treated and followed up by a single institution between 1976 and 2004.

PATIENTS

The records of 32 children, 18 males and 14 females, were evaluated. The mean follow-up period was 6.3 years.

RESULTS

At presentation, the most common symptoms were headache, nausea and vomiting, visual impairment, and neurological changes. Some patients presented signs or symptoms of isolated or combined endocrine disorder (five polyuria and polydipsia, five growth failure, two precocious puberty, eight obesity or overweight). After tumour treatment, multiple pituitary hormonal deficiencies, especially growth hormone (GH) deficit (GHD) were found and required hormonal replacement therapy. Eight children grew normally without GH despite GHD. Hypothalamic involvement was observed in ten patients; obesity was frequent and was often associated with hyperinsulinism and hyperphagia.

CONCLUSION

Anterior and posterior pituitary deficiencies following surgery are present in all patients. The growth pattern is heterogeneous.

摘要

目的

评估1976年至2004年间在单一机构接受治疗和随访的颅咽管瘤患儿的生长和内分泌特征。

患者

对32例儿童(18例男性和14例女性)的记录进行了评估。平均随访期为6.3年。

结果

就诊时,最常见的症状是头痛、恶心和呕吐、视力障碍及神经功能改变。一些患者出现孤立或合并内分泌紊乱的体征或症状(5例多尿和烦渴、5例生长发育迟缓、2例性早熟、8例肥胖或超重)。肿瘤治疗后,发现多种垂体激素缺乏,尤其是生长激素(GH)缺乏(GHD),需要进行激素替代治疗。8例患儿尽管存在GHD,但未使用GH仍正常生长。10例患者观察到下丘脑受累;肥胖很常见,且常与高胰岛素血症和食欲亢进有关。

结论

所有患者术后均存在垂体前叶和后叶功能减退。生长模式具有异质性。

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