Clinical, endocrinological and radiological courses in patients who
was initially diagnosed as idiopathic central diabetes insipidus

Chung, Seung Joon; Lee, Seong Yong; Shin, Choong Ho; Yang, Sei Won

Original Article

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

정승준^¹^, 이성용^²^, 신충호^¹^, 양세원^¹

^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