title: Analysis of 43 cases of Langerhans cell histiocytosis (LCH)-induced central diabetes insipidus registered in the JLSG-96 and JLSG-02 studies in Japan.
abstract: To determine the ability of recent systemic chemotherapy protocols to reduce the incidence of central diabetes insipidus (CDI) in Langerhans cell histiocytosis (LCH), 43 CDI cases that belonged to a cohort of 348 pediatric patients with multi-focal LCH who were treated with the JLSG-96/-02 protocols were analyzed. The overall incidence of CDI was 12.4%, but in 24 cases CDI was already present at the time LCH was diagnosed. Thus, CDI developed during or after systemic chemotherapy over a follow-up period of 5.0 (0.2-14.7) years in only 19 patients (5.9%), with 7.4% at 5-year cumulative risk by Kaplan-Meier analysis. In two cases, complete resolution of CDI was noted. Anterior pituitary hormone deficiency was detected in 13 cases, while CDI-associated neurodegenerative disease was observed in six cases. The JLSG-96/-02 protocol appears to effectively reduce the occurrence of CDI. However, novel therapeutic measures are required to reverse pre-existing CDI and to prevent CDI-associated neurological complications.
authors.affiliation: Division of Pediatric Oncology, National Center for Child Health and Development, Tokyo, Japan. shioda-y@ncchd.go.jp
authors.forename: Yoko
authors.initials: Y
authors.lastname: Shioda
authors.forename: Souichi
authors.initials: S
authors.lastname: Adachi
authors.forename: Shinsaku
authors.initials: S
authors.lastname: Imashuku
authors.forename: Kazuko
authors.initials: K
authors.lastname: Kudo
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Imamura
authors.forename: Akira
authors.initials: A
authors.lastname: Morimoto
mesh_terms: D000293:Adolescent; D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D020790:Diabetes Insipidus, Neurogenic / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D004359:Drug Therapy, Combination; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D015994:Incidence; D007223:Infant; D053208:Kaplan-Meier Estimate; D008279:Magnetic Resonance Imaging; D008297:Male; D019636:Neurodegenerative Diseases / Q000150:complications; D010908:Pituitary Hormones, Anterior / Q000172:deficiency; D016896:Treatment Outcome
chemical_list: D010908:Pituitary Hormones, Anterior