title: Endocrine changes in histiocytosis of the hypothalamic-pituitary axis.
abstract: Histiocytosis is characterized by proliferation of cells from the mononuclear phagocyte system, and may be divided into Langerhans cell histiocytosis (LCH) and non-Langerhans cell histiocytosis (including Erdheim-Chester disease [ECD]). While diabetes insipidus (DI) is the most common hypothalamic-pituitary consequence, anterior pituitary deficiencies are less known. This study analyzed the frequency and progression of pituitary hormone deficiencies and the radiographic findings in 9 patients (7 with LCH and 2 with ECD) with hypothalamic-pituitary (HP) axis. Eighty-nine percent of patients had DI (62% at diagnosis), and 78% had one or more anterior pituitary deficiencies (71% at diagnosis). HP involvement is relatively common in patients diagnosed with histiocytosis and hormone deficiencies may be present at diagnosis or appear gradually during the course of disease. Regular monitoring of these patients is recommended.
authors.affiliation: Servicio de Endocrinología y Nutrición, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, España. Electronic address: storo@bellvitgehospital.cat.
authors.forename: Silvia
authors.initials: S
authors.lastname: Toro Galván
authors.affiliation: Servicio de Endocrinología y Nutrición, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, España.
authors.forename: Alejandra
authors.initials: A
authors.lastname: Planas Vilaseca
authors.affiliation: Servicio de Endocrinología y Nutrición, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, España.
authors.forename: Theodora
authors.initials: T
authors.lastname: Michalopoulou Alevras
authors.affiliation: Servicio de Neurocirugía, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, España.
authors.forename: Alberto
authors.initials: A
authors.lastname: Torres Díaz
authors.affiliation: Servicio de Endocrinología y Nutrición, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, España.
authors.forename: Javier
authors.initials: J
authors.lastname: Suárez Balaguer
authors.affiliation: Servicio de Endocrinología y Nutrición, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, España.
authors.forename: Carles
authors.initials: C
authors.lastname: Villabona Artero
mesh_terms: D000328:Adult; D000368:Aged; D003919:Diabetes Insipidus / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D006111:Graves Disease / Q000150:complications; D015614:Histiocytosis / Q000150:complications / Q000503:physiopathology*; D006801:Humans; D006966:Hyperprolactinemia / Q000209:etiology; D007018:Hypopituitarism / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D007030:Hypothalamo-Hypophyseal System / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010908:Pituitary Hormones, Anterior / Q000097:blood / Q000172:deficiency; D010913:Pituitary-Adrenal System / Q000503:physiopathology*; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D010908:Pituitary Hormones, Anterior
keywords: Chester-Erdheim disease; Diabetes insipidus; Diabetes insípida; Enfermedad de Chester-Erdheim; Hipopituitarismo; Histiocitosis de células de Langerhans; Hypopituitarism; Langerhans cell histiocytosis