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朗格汉斯细胞组织细胞增多症患儿的生长情况

Growth of children with Langerhans cell histiocytosis.

作者信息

van den Hoek A C, Karstens A, Egeler R M, Hählen K

机构信息

Sophia Children's Hospital/Erasmus University Rotterdam, The Netherlands.

出版信息

Eur J Pediatr. 1995 Oct;154(10):822-5. doi: 10.1007/BF01959790.

Abstract

Diseases in childhood have an impact on growth. The influence of Langerhans cell histiocytosis (LCH) on growth has never been studied well. Recently a patient with LCH was treated with human growth hormone (GH) because of severe GH deficiency due to LCH involvement of both the hypothalamus and pituitary. This led us to review our charts from 1971 onward for evaluation of the growth patterns in patients with LCH. Here the long-term growth of 22 patients with LCH is reported, the median follow up being 7 years and 1 month. The height data were converted into standard deviation scores (SDS). At diagnosis the mean SDS of patients with isolated LCH at diagnosis was 0.04 and -0.37 in patients with disseminated LCH. Of the total group, 12 patients did not show any influence from the LCH or therapy on their growth. The remaining 10 patients reached, after a minimum of 3 years, a percentile clearly higher than that at diagnosis. However all the ten above mentioned patients, either isolated or disseminated LCH, had a lesion in the facial side of the skull. CONCLUSION. GH deficiency is not a common manifestation of LCH in childhood and GH provocation tests are only indicated when there is a poor or decelerating growth rate. In our patients the number of organs involved and/or the treatment modality did not influence the growth in all but one.

摘要

儿童疾病会影响生长发育。朗格汉斯细胞组织细胞增多症(LCH)对生长发育的影响尚未得到充分研究。最近,一名LCH患者因下丘脑和垂体均受LCH累及导致严重生长激素(GH)缺乏,接受了人生长激素治疗。这促使我们回顾1971年以来的病历,以评估LCH患者的生长模式。本文报告了22例LCH患者的长期生长情况,中位随访时间为7年1个月。身高数据转换为标准差评分(SDS)。诊断时,孤立性LCH患者的平均SDS为0.04,播散性LCH患者为-0.37。在整个研究组中,12例患者的生长未受LCH或治疗的任何影响。其余10例患者在至少3年后,其百分位数明显高于诊断时。然而,上述10例患者,无论是孤立性还是播散性LCH,均在颅骨面部有病变。结论。GH缺乏并非儿童LCH的常见表现,仅在生长速率不佳或生长减速时才需进行GH激发试验。在我们的患者中,除1例患者外,受累器官数量和/或治疗方式均未影响生长。

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