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垂体功能不全的生长激素治疗:颅咽管瘤和髓母细胞瘤患儿的特定病例

Growth hormone treatment in pituitary insufficiency: selected cases of children with craniopharyngioma and medulloblastoma.

作者信息

Kedzia A, Obara-Moszyńska M, Korman E, Rabska-Pietrzak B, Kopinski P, Trojan J, Goździcka-Józefiak A

机构信息

Dept. Developmental Endocrinology and Diabetology, Institute of Pediatry, Medical University, Poznań, Poland.

出版信息

Rocz Akad Med Bialymst. 2003;48:28-33.

Abstract

PURPOSE

The work concerns the substitution treatment with growth hormone (GH) in hypopituitary children, including cases that occurred in the course of tumor disease, craniopharyngioma (CP) and medulloblastoma (MB).

MATERIAL AND METHODS

The studied population concerned 117 children who presented either somatotropic or polyhormonal pituitary insufficiency (the average age was 12.6 years for girls and 13.6 years for boys). The diagnosis of somatotropic pituitary insufficiency (SPI) was based on insulin and clonidin stimulation tests evaluating GH reserve of hypophysis. The computer tomography (CT) and nuclear magnetic resonance (NMR) examinations were carried out before GH substitution in all children. The tumors (four CP cases and one case of MB) were all found in boys and they were treated with surgery and/or radiotherapy. All studied children, including CP and MB operated patients were treated with human GH (hGH)--Genotropin 16 IU, administered in subcutaneous injections. The daily dose was calculated as 0.5 IU/kg/week.

RESULTS

The annual increase of children height before GH therapy was about 3.2 cm. In the first year of GH therapy the difference in children growth between the CP/MB group as compared with the rest of patients was less than 1.0 cm: 9.4 and 10.2 cm/year, resp. During the second year of hormone substitution the growth became slower: average values were 8.2 cm and 7.4 cm/year, resp. In CP and MB patients the height increase calculated as SDS values was significant (2.7 and 1.0 resp.). Control NMR examination performed in CP/MB patients treated with surgery with subsequent hGH therapy did not demonstrate any recurrence of tumor.

CONCLUSIONS

After two years of hGH therapy the final height of hypopituitary children, including CP patients, nearly reached the values observed in healthy children. GH therapy did not induce a recurrence of neoplasm in CP and MB patients.

摘要

目的

本研究关注生长激素(GH)替代治疗垂体功能减退儿童,包括肿瘤疾病(颅咽管瘤(CP)和髓母细胞瘤(MB))过程中出现的病例。

材料与方法

研究对象为117例患有生长激素或多种激素垂体功能减退的儿童(女孩平均年龄12.6岁,男孩平均年龄13.6岁)。生长激素垂体功能减退(SPI)的诊断基于胰岛素和可乐定刺激试验,评估垂体的GH储备。所有儿童在进行GH替代治疗前均进行了计算机断层扫描(CT)和核磁共振(NMR)检查。肿瘤(4例CP和1例MB)均在男孩中发现,他们接受了手术和/或放疗。所有研究儿童,包括接受CP和MB手术的患者,均接受了人生长激素(hGH)——健高素16 IU,皮下注射给药。每日剂量计算为0.5 IU/kg/周。

结果

GH治疗前儿童身高的年增长约为3.2 cm。在GH治疗的第一年,CP/MB组儿童与其他患者的生长差异小于1.0 cm,分别为9.4 cm/年和10.2 cm/年。在激素替代治疗的第二年,生长速度变慢:平均值分别为8.2 cm/年和7.4 cm/年。在CP和MB患者中,以SDS值计算的身高增长显著(分别为2.7和1.0)。对接受手术并随后进行hGH治疗的CP/MB患者进行的对照NMR检查未显示肿瘤复发。

结论

经过两年的hGH治疗,垂体功能减退儿童(包括CP患者)的最终身高几乎达到了健康儿童的水平。GH治疗未导致CP和MB患者肿瘤复发。

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