중추성 요붕증이 동반된 랑게르한스 세포 조직구증 1예 = Langerhans Cell Histiocytosis with Central Diabetes Insipidus: A Case Report

다국어 초록 (Multilingual Abstract)

Central diabetes insipidus (DI) is a syndrome characterized by thirst, polydipsia and polyuria. Langerhans cell histiocytosis is one of the etiologies of DI. Recently we experienced a central DI associated with Langerhans cell histiocytosis. The 44 years old female patient complained right hip pain polydipsia and polyuria. We carried out water deprivation test. After vasopressin injection, urine osmotic pressure was increased form 109mOsmol/Kg to 327mOsmol/Kg (300%). Brain MRI showed a thickened pituitary stalk and at hot bone CT.CT guided biopsy revealed abnormal histiocytes proliferation and abundant lymphocytes, The final diagnosis was central DI associated with systemic Langerhans cell histiocytosis invading hip bone, L-spine and pituitary stalk. Desmopressin and etoposide chemotherapy were performed to the patient.