title: Clinical characteristics of central diabetes insipidus in Taiwanese children.
abstract: OBJECTIVE
  Data on the clinical features of children with central diabetes insipidus (CDI) are lacking in Taiwan. This study investigated the clinical manifestations and etiology of CDI in Taiwanese children.
  
  
  METHODS
  From 1983 to 2012, 62 children with permanent diabetes insipidus were enrolled in the study. They were diagnosed at the Department of Pediatrics of National Taiwan University Hospital. Their medical records were thoroughly reviewed and their clinical symptoms and signs, laboratory data, and etiologies were analyzed.
  
  
  RESULTS
  The patients' median age at diagnosis was 10 years and the median interval between initial manifestations and diagnosis was 0.5 years. The most common symptoms and signs were polyuria, polydipsia, nocturia, and growth retardation. Most patients had low urine osmolality and elevated plasma osmolality on diagnosis. Absence of a posterior pituitary hyperintense signal and thickening of the pituitary stalk were common findings on magnetic resonance imaging. Approximately 80% of the patients had anterior pituitary hormone deficiency and all patients had growth hormone deficiency. Approximately 60% of patients had intracranial lesions, the most common causes of which were germ cell tumor and Langerhans cell histiocytosis. Two patients were initially believed to have idiopathic CDI but intracranial lesions were detected during the follow-up period.
  
  
  CONCLUSIONS
  Because a delayed diagnosis of CDI is common in Taiwanese children, a high index of suspicion is important. The underlying etiology of CDI in children may not initially be obvious. Long-term surveillance is therefore necessary, especially for the early detection of evolving treatable intracranial lesions.
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan.
authors.forename: Shih-Yao
authors.initials: SY
authors.lastname: Liu
authors.forename: Yi-Ching
authors.initials: YC
authors.lastname: Tung
authors.forename: Cheng-Ting
authors.initials: CT
authors.lastname: Lee
authors.forename: Hon-Man
authors.initials: HM
authors.lastname: Liu
authors.forename: Shinn-Forng
authors.initials: SF
authors.lastname: Peng
authors.forename: Mu-Zon
authors.initials: MZ
authors.lastname: Wu
authors.forename: Meng-Fai
authors.initials: MF
authors.lastname: Kuo
authors.forename: Wen-Yu
authors.initials: WY
authors.lastname: Tsai
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D020790:Diabetes Insipidus, Neurogenic / Q000097:blood / Q000209:etiology* / Q000652:urine*; D005260:Female; D013006:Growth Hormone / Q000172:deficiency; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications; D006801:Humans; D007018:Hypopituitarism / Q000150:complications; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000150:complications*; D009994:Osmolar Concentration; D010902:Pituitary Gland / Q000503:physiopathology; D010908:Pituitary Hormones, Anterior / Q000172:deficiency; D013624:Taiwan; D014556:Urine / Q000737:chemistry
chemical_list: D010908:Pituitary Hormones, Anterior; D013006:Growth Hormone
keywords: Langerhans cell histiocytosis; central diabetes insipidus; germ cell tumor; hypopituitarism