KMID : 0387820060130010072
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Clinical Pediatric Hematology-Oncology 2006 Volume.13 No. 1 p.72 ~ p.79
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Central Diabetes Insipidus as a Manifestation of Langerhans Cell Histiocytosis: Report of 3 Cases with Literature Review
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Han Dong-Kyun
Kook Hoon Baek Hee-Jo Song Eun-Song Kang Hyun-Soo Kim Mi-Jung Kim Seok-Joo Kim Sun-Hui Kim Chan-Jong Hwang Tai-Ju
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Abstract
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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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KEYWORD
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Langerhans cell histiocytosis, Diabetes insipidus, Pituitary stalk thickening, MRI
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