title: Diabetes insipidus and Langerhans cell histiocytosis: a case report of reversibility with 2-chlorodeoxyadenosine.
abstract: Diabetes insipidus (DI) is the most common manifestation of central nervous system involvement in Langerhans cell histiocytosis (LCH). Patients with LCH involving the head and neck region are reported to have about a 40% lifetime chance of developing DI. The clinical and biochemical diagnosis of DI is sometimes supported by the absence of the posterior pituitary bright signal on magnetic resonance images. Cladribine (2-chlorodeoxyadenosine, 2-CDA) has been reported as an active drug in children and adults with relapsed or refractory LCH. The authors report the successful reversal of DI in a 3-year-old child with established LCH using 2-CDA.
authors.affiliation: The Neuro-oncology Program of the New York University Cancer Institute and the Department of Pediatrics, New York University School of Medicine, 317 East 34th Street, 10th Floor, New York, NY 10016, U.S.A. jonathan.finlay@msnyuhealth.org
authors.forename: Fabio
authors.initials: F
authors.lastname: Ottaviano
authors.forename: Jonathan L
authors.initials: JL
authors.lastname: Finlay
mesh_terms: D017338:Cladribine / Q000627:therapeutic use*; D003919:Diabetes Insipidus / Q000175:diagnosis* / Q000188:drug therapy; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D017338:Cladribine