title: Incidence of growth hormone deficiency in pediatric-onset Langerhans cell histiocytosis: efficacy and safety of growth hormone treatment.
abstract: We retrospectively studied 61 patients with GH deficiency (GHD), identified among 589 patients with Langerhans cell histiocytosis (LCH) enrolled in a nationwide survey between 1993 and 2001. Overall, 141 patients in the survey developed diabetes insipidus. The median follow-up of the 61 patients with GHD was 12 yr. The 5- and 10-yr risks of GHD among patients with diabetes insipidus were 34.7 +/- 4.5% and 53.7 +/- 5.2%, respectively. Growth velocity decreased soon after LCH diagnosis in patients who developed GHD, and anterior pituitary height, estimated by magnetic resonance imaging, was significantly reduced relative to patients who remained free of GHD. GH replacement therapy was administered to 47 of the 61 patients with GHD. Among GH-treated patients, median final height (-0.8 SD) was significantly greater than median height at GHD diagnosis (-1.6 SD) but remained below midparental (target) height. Among patients with pituitary involvement, the number of LCH disease episodes appeared not significantly influenced by GHD or GH administration, suggesting an absence of deleterious effect of GH therapy on LCH disease activity.
authors.affiliation: Service d'Hémato-Oncologie Pédiatrique, Hopital Trousseau, 75012 Paris, France.
authors.forename: Jean
authors.initials: J
authors.lastname: Donadieu
authors.forename: Maria-Alejandra
authors.initials: MA
authors.lastname: Rolon
authors.forename: Isabelle
authors.initials: I
authors.lastname: Pion
authors.forename: Caroline
authors.initials: C
authors.lastname: Thomas
authors.forename: François
authors.initials: F
authors.lastname: Doz
authors.forename: Mohamed
authors.initials: M
authors.lastname: Barkaoui
authors.forename: Alain
authors.initials: A
authors.lastname: Robert
authors.forename: Anne
authors.initials: A
authors.lastname: Deville
authors.forename: Françoise
authors.initials: F
authors.lastname: Mazingue
authors.forename: Michel
authors.initials: M
authors.lastname: David
authors.forename: Raja
authors.initials: R
authors.lastname: Brauner
authors.forename: Sylvie
authors.initials: S
authors.lastname: Cabrol
authors.forename: Catherine
authors.initials: C
authors.lastname: Garel
authors.forename: Michel
authors.initials: M
authors.lastname: Polak
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002648:Child; D004700:Endocrine System Diseases / Q000150:complications; D005260:Female; D006128:Growth / Q000187:drug effects; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000453:epidemiology; D019382:Human Growth Hormone / Q000009:adverse effects / Q000172:deficiency* / Q000627:therapeutic use*; D006801:Humans; D015994:Incidence; D008279:Magnetic Resonance Imaging; D008297:Male; D010900:Pituitary Diseases / Q000188:drug therapy* / Q000453:epidemiology / Q000209:etiology*; D010903:Pituitary Gland, Anterior; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D019382:Human Growth Hormone