Matoussi N, Aissa K, Fitouri Z, Hajji M, Makni S, Bellagha I, Ben Becher S
Service de pédiatrie, urgences et consultations externes, hôpital d'enfants de Tunis, 1007, rue Jabbari-Bab-Saâdoun, Tunis, Tunisie.
Ann Endocrinol (Paris). 2008 Jun;69(3):231-9. doi: 10.1016/j.ando.2007.11.003. Epub 2008 May 19.
Central diabetes insipidus is rare in children. Characteristic features include polyuria and polydipsia due to arginine vasopressin deficiency. The differential diagnosis of polyuric states may be difficult. Etiologic diagnosis of central diabetes insipidus may be an equally difficult task.
To specify the difficulties encountered in the diagnosis of central diabetes insipidus and to point out features of the etiologic work-up and of long-term follow-up of children with idiopathic central diabetes insipidus.
A retrospective study of 12 children admitted with a polyuria/polydipsia syndrome to the pediatric - consultation and emergency unit of the children's hospital of Tunis between 1988 and 2005. Children with acquired nephrogenic central diabetes insipidus were excluded. Fourteen-hour fluid restriction test and/or desmopressin test were used without plasma vasopressin measurement.
Eight patients were classified as having central diabetes insipidus, which was severe in seven children and partial in one girl. One patient was classified as having primary polydipsia. The diagnosis remains unclear in three patients. The etiological work-up in eight patients with central diabetes insipidus enabled the identification of Langerhan's-cell histiocytosis in two patients and neurosurgical trauma in one. The cause was considered idiopathic in five patients. The median follow-up of the five patients with idiopathic central diabetes insipidus was five years two months plus or minus six years seven months (range five months, 14.5 years). During this follow-up, neither brain magnetic resonance imaging scans findings nor anterior pituitary function have changed.
Fluid restriction and desmopressin tests did not enable an accurate distinction between partial diabetes insipidus and primary polydipsia. Regular surveillance is warranted in patients with idiopathic central diabetes insipidus to identify potential etiologies.
中枢性尿崩症在儿童中较为罕见。其特征性表现包括因精氨酸加压素缺乏导致的多尿和烦渴。多尿状态的鉴别诊断可能较为困难。中枢性尿崩症的病因诊断同样可能是一项艰巨的任务。
明确中枢性尿崩症诊断中遇到的困难，并指出特发性中枢性尿崩症患儿病因检查及长期随访的特点。
对1988年至2005年间在突尼斯儿童医院儿科咨询及急诊科因多尿/烦渴综合征入院的12名儿童进行回顾性研究。排除获得性肾性中枢性尿崩症患儿。采用14小时禁水试验和/或去氨加压素试验，未测定血浆加压素。
8例患者被诊断为中枢性尿崩症，其中7例为重度，1例为部分性，为1名女孩。1例患者被诊断为原发性烦渴。3例患者的诊断仍不明确。8例中枢性尿崩症患者的病因检查发现2例患有朗格汉斯细胞组织细胞增多症，1例有神经外科创伤。5例患者病因被认为是特发性。5例特发性中枢性尿崩症患者的中位随访时间为5年2个月±6年7个月（范围5个月至14.5年）。在此随访期间，脑磁共振成像扫描结果及垂体前叶功能均未改变。
禁水试验和去氨加压素试验无法准确区分部分性尿崩症和原发性烦渴。对特发性中枢性尿崩症患者进行定期监测以识别潜在病因是必要的。
