title: Endocrine disorders in pediatric - onset Langerhans Cell Histiocytosis.
abstract: Langerhans Cell Histiocytosis (LCH) is a rare disorder with a great variety of clinical manifestations. The purpose of this retrospective study was to evaluate the pattern and the long-term course of clinical, laboratorial and radiological findings in pediatric-onset LCH. We reviewed 46 children with histological diagnosis of LCH. Ten children (22%) showed endocrine disorders. Central diabetes insipidus (DI) was observed in all ten patients; GH deficiency was confirmed in four and hypogonadism in two children. There were no adrenal, prolactin or thyroid axis abnormalities. Obesity was observed in three patients. Eight patients showed soft tissue infiltration and five bone involvement. The MRI showed a lack of posterior pituitary bright spot in all DI patients; infundibular infiltration (II) associated or not with sellar or supra-sellar mass was observed in 4 patients. We conclude that the investigation of LCH, a multi-systemic disease, should include central nervous system images. The presence of II and/or DI should raise the diagnosis of LCH. Complete endocrine evaluation, allowing an early hormone therapy, is required to obtain a better quality of life in children with LCH.
authors.affiliation: Division of Endocrinology, Department of Internal Medicine, School of Medicine of Ribeirao Preto, University of Sao Paulo, Brazil.
authors.forename: M C M
authors.initials: MC
authors.lastname: Amato
authors.forename: L L K
authors.initials: LL
authors.lastname: Elias
authors.forename: J
authors.initials: J
authors.lastname: Elias
authors.forename: A C D
authors.initials: AC
authors.lastname: Santos
authors.forename: A D
authors.initials: AD
authors.lastname: Bellucci
authors.forename: A C
authors.initials: AC
authors.lastname: Moreira
authors.forename: M
authors.initials: M
authors.lastname: De Castro
mesh_terms: D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004700:Endocrine System Diseases / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D020249:Hormone Replacement Therapy; D006728:Hormones / Q000097:blood; D006801:Humans; D007223:Infant; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D010902:Pituitary Gland / Q000000981:diagnostic imaging / Q000473:pathology; D011859:Radiography; D012189:Retrospective Studies
chemical_list: D006728:Hormones