Horm Metab Res 2006; 38(11): 746-751
DOI: 10.1055/s-2006-955086
Original Clinical

© Georg Thieme Verlag KG Stuttgart · New York

Endocrine Disorders in Pediatric - Onset Langerhans Cell Histiocytosis

M. C. M. Amato 1 , L. L. K. Elias 1 , J. Elias 2 , A. C. D. Santos 2 , A. D. Bellucci 2 , A. C. Moreira 1 , M. De Castro 1
  • 1Division of Endocrinology
  • 2Division of Radiology, Department of Internal Medicine, School of Medicine of Ribeirao Preto, University of Sao Paulo, Sao Paulo, Brazil
Further Information

Publication History

Received 27 March 2006

Accepted after revision 7 June 2006

Publication Date:
16 November 2006 (online)

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.

References

Correspondence

Margaret de Castro, M.D., Ph.D. 

Department of Internal Medicine·School of Medicine of Ribeirao Preto·University of Sao Paulo

Av Bandeirantes 3900

14049-900 Ribeirao Preto

Sao Paulo

Brazil

Phone: +55/16/602/26 54

Fax: +55/16/633/66 95

Email: castrom@fmrp.usp.br