Catli Gonul, Abaci Ayhan, Demir Korcan, Ulusoy Emel, Altincik Ayca, Buyukgebiz Atilla, Bober Ece
Department of Pediatric Endocrinology, Dokuz Eylul University, Izmir, Turkey.
J Pediatr Endocrinol Metab. 2012;25(5-6):499-502. doi: 10.1515/jpem-2012-0006.
The aim of this study is to evaluate the clinical, anthropometric, hormonal, and radiological characteristics of children with central diabetes insipidus (DI).
Case records of 34 children (22 boys and 12 girls) with documented central DI referred to the Pediatric Endocrinology and Adolescent Clinic of Dokuz Eylul University Faculty of Medicine were reviewed. The mean age at diagnosis was 6.4 +/- 5.6 years (range, 0.08-16 years). All patients underwent anterior pituitary function assessment and magnetic resonance imaging of pituitary at diagnosis. The median duration of follow-up was 7.9 +/- 4.5 years.
The etiology of central DI was organic in 22 (64.7%) patients, trauma in 2 (5.9%) patients, and idiopathic in 10 (29.4%) patients. Organic causes consisted of craniopharyngioma in 7 patients, Langerhans cell histiocytosis in 4 patients, germinoma in 4 patients, holoprosencephaly in 3 patients, astrocytoma in 1 patient, cavernous hemangioma in 1 patient, Rathke's cleft cyst in 1 patient, and autoimmune polyendocrinopathy in 1 patient. Anterior pituitary hormone deficiencies were documented in 18 (53%) patients. Organic central DI group had a greater prevalence of anterior pituitary hormone deficiency when compared with the idiopathic group (66% and 10%, respectively; p = 0.007). The final height of patients with organic etiology were significantly lower than the idiopathic group (155 and 178, cm respectively; p = 0.021).
Etiological diagnosis is possible in a significant proportion (70.6%) of children with central DI. Findings of this study suggest that accompanying anterior pituitary hormone deficiencies and short stature may be considered as indicators of organic etiology.
本研究旨在评估中枢性尿崩症（DI）患儿的临床、人体测量学、激素及影像学特征。
回顾了转诊至多库兹艾吕尔大学医学院儿科内分泌与青少年诊所的34例确诊为中枢性DI的患儿（22例男孩和12例女孩）的病例记录。诊断时的平均年龄为6.4±5.6岁（范围0.08 - 16岁）。所有患者在诊断时均接受了垂体前叶功能评估及垂体磁共振成像检查。随访的中位时长为7.9±4.5年。
中枢性DI的病因中，22例（64.7%）为器质性，2例（5.9%）为创伤性，10例（29.4%）为特发性。器质性病因包括7例颅咽管瘤、4例朗格汉斯细胞组织细胞增多症、4例生殖细胞瘤、3例全前脑畸形、1例星形细胞瘤、1例海绵状血管瘤、1例拉克氏囊肿及1例自身免疫性多内分泌腺病。18例（53%）患者记录有垂体前叶激素缺乏。与特发性组相比，器质性中枢性DI组垂体前叶激素缺乏的患病率更高（分别为66%和10%；p = 0.007）。器质性病因患者的最终身高显著低于特发性组（分别为155厘米和178厘米；p = 0.021）。
相当比例（70.6%）的中枢性DI患儿能够做出病因诊断。本研究结果提示，伴随的垂体前叶激素缺乏和身材矮小可被视为器质性病因的指标。
