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

Hayward, Rachel M., Nicolin, Gary, Kennedy, Charles, Joy, Harriet and Davies, Justin H. (2011) From idiopathic diabetes insipidus to neurodegenerative Langerhans cell histiocytosis--an unusual presentation and progression of disease Journal of Pediatric Endocrinology and Metabolism, 24, (9-10), pp. 811-814. (doi:10.1515/JPEM.2011.298). (PMID:22145482).

Record type: Article

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 2 1/2-year-old boy who presented initially with apparent idiopathic DI, developed anterior pituitary hormone deficiency and progressive neurological deterioration secondary to neurodegenerative LCH.

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Published date: 2011
Keywords: children, diabetes insipidus, neurodegenerative Langerhans cell histiocytosis
Organisations: Human Development & Health

Identifiers

Local EPrints ID: 207535
URI: http://eprints.soton.ac.uk/id/eprint/207535
ISSN: 0334-018X
PURE UUID: e716d415-efd8-446f-be3a-e378e791307d

Catalogue record

Date deposited: 10 Jan 2012 11:45
Last modified: 18 Jul 2017 10:50

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Contributors

Author: Rachel M. Hayward
Author: Gary Nicolin
Author: Charles Kennedy
Author: Harriet Joy

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