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Licensed Unlicensed Requires Authentication Published by De Gruyter August 11, 2011

From idiopathic diabetes insipidus to neurodegenerative Langerhans cell histiocytosis – an unusual presentation and progression of disease

  • Rachel M. Hayward , Gary Nicolin , Charles Kennedy , Harriet Joy and Justin H. Davies EMAIL logo
From the journal Journal of Pediatric Endocrinology and Metabolism
https://doi.org/10.1515/JPEM.2011.298

Abstract

Diabetes insipidus (DI) is rare in childhood and has a wide-ranging aetiology including the involvement of uncontrolled proliferation of dendritic cells in the hypothalamic-pituitary axis, characteristic of Langerhans cell histiocytosis (LCH). DI may manifest as a sequela of multisystem LCH disease involving skin, bone, liver, spleen and lymph nodes. In very rare cases patients diagnosed with LCH exhibit neurodegenerative changes, such as severe ataxia, tremor, dysarthria and intellectual impairment. We report a 2½-year-old boy who presented initially with apparent idiopathic DI, developed anterior pituitary hormone deficiency and progressive neurological deterioration secondary to neurodegenerative LCH.

Keywords: children; diabetes insipidus; neurodegenerative Langerhans cell histiocytosis
Corresponding author: Dr. J.H. Davies, Consultant in Paediatric Endocrinology, Child Health Directorate, MP 43, Southampton University Hospital Trust, Tremona Road, Southampton SO16 6YD, UK Phone: +44 2380 796985, Fax: +44 2380 795230
Received: 2011-6-4
Accepted: 2011-6-18
Published Online: 2011-08-11
Published in Print: 2011-10-1

©2011 by Walter de Gruyter Berlin Boston

From the journal
Journal of Pediatric Endocrinology and Metabolism
Journal of Pediatric Endocrinology and Metabolism
Volume 24 Issue 9-10
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