Donadieu Jean, Rolon Maria-Alejandra, Pion Isabelle, Thomas Caroline, Doz François, Barkaoui Mohamed, Robert Alain, Deville Anne, Mazingue Françoise, David Michel, Brauner Raja, Cabrol Sylvie, Garel Catherine, Polak Michel
Service d'Hémato-Oncologie Pédiatrique, Hopital Trousseau, 75012 Paris, France.
J Clin Endocrinol Metab. 2004 Feb;89(2):604-9. doi: 10.1210/jc.2003-030907.
We retrospectively studied 61 patients with GH deficiency (GHD), identified among 589 patients with Langerhans cell histiocytosis (LCH) enrolled in a nationwide survey between 1993 and 2001. Overall, 141 patients in the survey developed diabetes insipidus. The median follow-up of the 61 patients with GHD was 12 yr. The 5- and 10-yr risks of GHD among patients with diabetes insipidus were 34.7 +/- 4.5% and 53.7 +/- 5.2%, respectively. Growth velocity decreased soon after LCH diagnosis in patients who developed GHD, and anterior pituitary height, estimated by magnetic resonance imaging, was significantly reduced relative to patients who remained free of GHD. GH replacement therapy was administered to 47 of the 61 patients with GHD. Among GH-treated patients, median final height (-0.8 SD) was significantly greater than median height at GHD diagnosis (-1.6 SD) but remained below midparental (target) height. Among patients with pituitary involvement, the number of LCH disease episodes appeared not significantly influenced by GHD or GH administration, suggesting an absence of deleterious effect of GH therapy on LCH disease activity.
我们对61例生长激素缺乏症（GHD）患者进行了回顾性研究，这些患者是在1993年至2001年全国范围内一项针对589例朗格汉斯细胞组织细胞增多症（LCH）患者的调查中确定的。总体而言，该调查中有141例患者发生了尿崩症。61例GHD患者的中位随访时间为12年。尿崩症患者中GHD的5年和10年风险分别为34.7±4.5%和53.7±5.2%。发生GHD的患者在LCH诊断后不久生长速度下降，通过磁共振成像估计的垂体前叶高度相对于未发生GHD的患者显著降低。61例GHD患者中有47例接受了生长激素替代治疗。在接受生长激素治疗的患者中，最终身高的中位数（-0.8标准差）显著高于GHD诊断时的身高中位数（-1.6标准差），但仍低于父母平均（目标）身高。在垂体受累的患者中，LCH疾病发作的次数似乎不受GHD或生长激素治疗的显著影响，这表明生长激素治疗对LCH疾病活动没有有害影响。
