Endocrine disorders in pediatric - onset Langerhans Cell Histiocytosis
| Autor: | Jorge Elias, Lucila Leico Kagohara Elias, A. D. Bellucci, Marcelo Campos Moraes Amato, Ariany C. Santos, M. de Castro, Ayrton Custódio Moreira |
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| Rok vydání: | 2006 |
| Předmět: |
Male
medicine.medical_specialty Pathology Hormone Replacement Therapy Endocrinology Diabetes and Metabolism medicine.medical_treatment Clinical Biochemistry Endocrine System Diseases Biochemistry Endocrinology Langerhans cell histiocytosis Posterior pituitary Internal medicine medicine Endocrine system Humans Age of Onset Child Lung Retrospective Studies business.industry Biochemistry (medical) Infant Retrospective cohort study General Medicine medicine.disease Magnetic Resonance Imaging Hormones Radiography Histiocytosis Histiocytosis Langerhans-Cell medicine.anatomical_structure Child Preschool Pituitary Gland Diabetes insipidus Disease Progression Female Hormone therapy Age of onset business |
| Zdroj: | Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme. 38(11) |
| ISSN: | 0018-5043 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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