Dynamic endocrine testing and magnetic resonance imaging in the long-term follow-up of childhood langerhans cell histiocytosis.
Dynamic endocrine testing and magnetic resonance imaging in the long-term follow-up of childhood langerhans cell histiocytosis.
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动态内分泌检测和磁共振成像在儿童朗格汉斯细胞组织细胞增多症长期随访中的应用。
DOI：
10.1210/jcem.83.9.5108
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发表时间：
1998
期刊：
影响因子：
--
通讯作者：
Maurizio Aricò
中科院分区：
文献类型：
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作者：
Mohamad Maghnie;Grazia Bossi;C. Klersy;Gianluca Cosi;Eugenio Genovese;Maurizio Aricò
接受朗格汉斯细胞组织细胞增多症 (LCH) 治疗的儿童面临短期和长期内分泌后遗症的风险，但特定缺陷的生物学预测因素尚不明确。我们评估了长期随访期间 LCH 相关内分泌缺陷的频率和进展，并评估了动态内分泌检测识别有晚期垂体前叶或后叶激素功能障碍风险的患者的能力。这 17 名患者（5 名男性和 12 名女性）在诊断为单系统 (n = 6) 或多系统 (n = 11) 疾病后接受了平均 10 年的随访。研究评估在诊断后平均 4.1 年进行，包括垂体激素对适当挑战的反应、7 小时禁水测试、3% 高渗盐水输注和磁共振成像 (MRI)。评估时 6 名 GH 缺乏症患者对 GHRH 的 GH 反应显着低于其他患者 [中位峰值，7.3 vs. 21.5 微克/升 (P = 0.03)；曲线下面积中位数，4.7 与 13.5 微克/升 (P = 0.03)]；后一组的水平与 20 名年龄和性别匹配的先天性或家族性身材矮小对照者的水平没有显着差异。两名患者在 2.8 岁和 9.5 岁时对 GHRH 的 GH 反应分别为 20.6 ng/mL 和 23 ng/mL，分别在 6.5 岁和 11.2 岁时出现 GH 缺乏症。 3 名患者的 TSH 对 TRH 的反应低于 10 mU/L，其中两人后来出现中枢性甲状腺功能减退症。所有病例中 ACTH 和皮质醇对 CRF 的反应以及 PRL 对 TRH 的反应均正常，LH 和 FSH 对 GnRH 的反应与青春期相一致。仅在 4 名患有尿崩症 (DI) 的患者中观察到精氨酸加压素对缺水或高渗盐水输注的反应异常；一名后来出现 DI 的患者检查结果正常。在标准 MRI 上，仅 DI 患者不存在垂体后叶高信号。 7 名患者出现垂体柄增厚，其中 3 名患者没有 DI，但精氨酸加压素反应正常。两名患者的动态 MRI 出现后部和前部延迟增强，两人后来均出现中枢性甲状腺功能减退症。单系统疾病患者 5 年 LCH 再激活概率较低（多系统疾病患者为 41%，P = 0.21；P = 0.21），内分泌功能障碍风险也显着降低（P = 0.007）。在本系列中，垂体功能的动态评估并不是晚期内分泌后遗症的有用预测因子，但 TSH 对 TRH 的反应逐渐降低可能是个例外。同样，标准 MRI 不能预测，尽管动态成像可能提供有关不断发展的垂体激素缺乏的信息。
Children treated for Langerhans cell histiocytosis (LCH) are at risk for short and long term endocrine sequelae, but biological predictors of specific deficits are not well defined. We evaluated the frequency and progression of LCH-related endocrine deficits during long term follow-up and assessed the ability of dynamic endocrine testing to identify patients at risk for late anterior or posterior pituitary hormone dysfunction. The 17 patients (5 males and 12 females) were followed a median of 10 yr after diagnosis of single system (n = 6) or multisystem (n = 11) disease. Study evaluations, performed a median of 4.1 yr after the diagnosis, comprised pituitary hormone responses to the appropriate challenge, 7-h water deprivation test, 3% hypertonic saline infusion, and magnetic resonance imaging (MRI). The six patients with GH deficiency at the time of evaluation had a significantly lower GH response to GHRH than the other patients [median peak, 7.3 vs. 21.5 micrograms/L (P = 0.03); median area under the curve, 4.7 vs. 13.5 micrograms/L (P = 0.03)]; levels in the latter group did not differ significantly from those in 20 age- and sex-matched controls with constitutional or familial short stature. Two patients who had GH responses to GHRH of 20.6 and 23 ng/mL at 2.8 and 9.5 yr of age developed GH deficiency at 6.5 and 11.2 yr of age, respectively. The TSH response to TRH was less than 10 mU/L in three patients, two of whom later developed central hypothyroidism. ACTH and cortisol responses to CRF, and PRL responses to TRH were normal in all cases, and LH and FSH responses to GnRH were compatible with pubertal stage. Abnormalities in arginine vasopressin responses to water deprivation or hypertonic saline infusion were seen only in four patients who had preexisting diabetes insipidus (DI); one patient who later developed DI had normal findings. On standard MRI, posterior pituitary hyperintensity was absent only in the patients with DI. Pituitary stalk thickening was seen in seven patients, including three who did not have DI and had normal arginine vasopressin responses. Delayed posterior and anterior enhancement on dynamic MRI was present in two patients, both of whom later developed central hypothyroidism. Patients with single system disease had a lower 5-yr probability of LCH reactivation (41% vs. 83% for those with multisystem disease; P = 0.21) and a significantly lower risk of endocrine dysfunction (P = 0.007). In this series, dynamic evaluation of pituitary function was not a useful predictor of late endocrine sequelae, with the possible exception of the progressively decreasing TSH response to TRH. Similarly, a standard MRI was not predictive, although dynamic imaging may be informative regarding evolving pituitary hormone deficiency.