关键词: Langerhans’ cell histiocytosis diabetes insipidus hypothalamus pituitary

来  源:   DOI:10.1586/17446651.2.6.773   PDF(Sci-hub)

Abstract:
Langerhans\' cell histiocytosis (LCH) is a rare disease of unknown etiology and variable biologic behavior and course. Almost every organ can be affected; however, it frequently involves the bones, lungs, skin and lymph nodes. LCH exhibits a particular predilection for involvement of the hypothalamo-pituitary axis resulting in diabetes insipidus and/or other anterior pituitary deficiencies. Although LCH can sometimes resolve spontaneously, it usually follows a chronic course and systemic treatment is mandatory for the management of multisystem disease. However, a significant number of patients will develop at least one permanent sequela as a consequence of the disease, with diabetes insipidus being the most common. A multidisciplinary approach is always needed in diagnosis, treatment and follow-up of all patients with LCH.
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