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Clin Pediatr Hematol Oncol. 2006 Apr;13(1):72-79. Korean. Case Report.
Han DK , Kook H , Baek HJ , Song ES , Kang HS , Kim MJ , Kim SJ , Kim SH , Kim CJ , Hwang TJ .
Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea. hoonkook@chonnam.ac.kr
Abstract

Central diabetes insipidus (CDI) is the most common manifestation of central nervous system involvement in Langerhans cell histiocytosis (LCH). Therefore, in patients with endocrinologically proven CDI, a comprehensive diagnostic evaluation is crucial to identify possible extracranial sites of LCH. However, once established, DI is mostly associated with life-long dependence on antidiuretic hormone (ADH) replacement therapy. We here report 3 cases of CDI in association with LCH, diagnosed by a water deprivation test. Two patients who presented with CDI as the initial manifestation showed thickened pituitary stalk on the sellar MRI. The 3rd patient developed CDI as a late sequela of LCH. Regression of pituitary thickness on MRI did not correlate with the clinical recovery of CDI, as shown in 2 cases.

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