儿童颅咽管瘤。早期生长衰竭和术后体重迅速增加。

R Sorva
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引用次数: 72

摘要

分析了22例颅咽管瘤患儿术前和术后的生长情况。在19名儿童中,生长衰竭在诊断前平均提前了4年。6例患儿术前肥胖。术后前3个月,14/21的患儿相对体重增加超过10%(1例手术死亡)。手术一年后,13/21的患者肥胖。肿瘤的大小和手术方式都不是导致肥胖的决定性因素。我们评估了4例生长激素缺乏症(GHD)患儿的血清胰岛素和胰岛素样生长因子I (IGF-I),这些患儿在颅咽管瘤手术后生长正常,没有GH替代。其中一名体重正常,胰岛素和igf - 1水平正常;另一组肥胖,胰岛素和igf - 1水平均异常。四名未替代GHD患儿中的一名和另外两名达到最终身高SDS -0.8, -2.0和-2.4。1例术后生长激素反应正常的患儿最终身高SDS达到-0.7。6/11名儿童替代生长激素后获得的最终身高SDS大于或等于-2.5。与7/11期间亲本身高的期望高度SDS相比,偏差大于2.0 SD。对儿童生长发育的充分监测将导致早期诊断,并可能获得更好的结果。
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Children with craniopharyngioma. Early growth failure and rapid postoperative weight gain.

Pre- and postoperative growth was analyzed in 22 children with craniopharyngioma. In 19 children a growth failure preceded the diagnosis by a mean of 4 years. Six children were obese preoperatively. During the first 3 postoperative months relative weight increased greater than 10% in 14/21 children (there was one surgical death). One year after surgery 13/21 were obese. Neither the size of the tumor nor the mode of surgery was decisive in the development of the obesity. Serum insulin and insulin-like growth factor I (IGF-I) were assessed in four children with growth hormone deficiency (GHD) who, after surgery for craniopharyngioma, were growing normally without GH substitution. One of them was normal in weight and had normal insulin and IGF-I levels; the others were obese and had supranormal insulin and subnormal IGF-I levels. One of the four and two other children with unsubstituted GHD reached final height SDS -0.8, -2.0 and -2.4. One child with normal postoperative GH response reached final height SDS -0.7. Final height SDS greater than or equal to -2.5 was gained with GH substitution by 6/11 children. It was greater than 2.0 SD below the height SDS expected from the heights of the parents in 7/11. An adequate monitoring of children's growth would lead to earlier diagnosis and probably better outcome.

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