{Reference Type}: Journal Article
{Title}: Systemic and endocrine manifestations of Langerhans' cell histiocytosis: current concepts in diagnosis and management.
{Author}: Makras P;Piaditis G;Kaltsas GA;
{Journal}: Expert Rev Endocrinol Metab
{Volume}: 2
{Issue}: 6
{Year}: Nov 2007
暂无{DOI}: 10.1586/17446651.2.6.773
{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.