Korean Journal of Pediatrics 2007;50(11):1110-1115.
Published online November 15, 2007.
Clinical, endocrinological and radiological courses in patients who was initially diagnosed as idiopathic central diabetes insipidus
Seung Joon Chung1, Seong Yong Lee2, Choong Ho Shin1, Sei Won Yang1
1Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
2Department of Pediatrics, Seoul Metropolitan Boramae Hospital, Seoul, Korea
초기에 특발성 중추성 요붕증으로 진단된 환자에서 임상,내분비학 및 방사선학적 경과
정승준1, 이성용2, 신충호1, 양세원1
1서울대학교 의과대학 소아과학교실
2서울특별시립 보라매병원
Correspondence: 
Choong Ho Shin, Email: chshinpd@snu.ac.kr
Abstract
Purpose
: Idiopathic central diabetes insipidus (CDI) is defined in CDI patients without definite etiology. Some patients initially diagnosed as idiopathic CDI progressed to organic causes. We reviewed clinical, endocrinological, and radiological courses of 20 patients who was initially diagnosed as idiopathic CDI, to assess the predicting factors for progression to brain tumors.
Methods
: We reviewed the medical data and followed up their clinical courses in 20 CDI patients who had no definite organic etiology, such as malformation, tumor, at the time of diagnosis.
Results
: Our study included 15 males and 5 females. Mean age of CDI diagnosis was 7.8?.6 (2.1- 14.7) years. Mean follow-up duration was 8.6?.1 (1.5-18) years. Six (30%) patients were diagnosed as brain tumor during follow-up. Ten (50%) of 20 patients had growth hormone deficiency. Multiple pituitary hormone deficiencies were found more frequently in brain tumor patients than idiopathic patients (60% vs 7%, P=0.037). Pituitary stalk thickening (PST) and loss of posterior pituitary signal were observed in 9 patients (47%), respectively. The newly development of PST was observed in patients diagnosed as brain tumor.
Conclusion
: About 30% of idiopathic CDI patients progress to organic disease such as germ cell tumor or histiocytosis. If there are multiple anterior pituitary hormone deficiency or newly development of PST, more close and careful follow-up is needed.
Key Words: Central diabetes insipidus, Growth hormone deficiency, Cerebral tumor, Germinoma, Langerhans cell histiocytosis


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