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), 811-814. (doi:10.1515/JPEM.2011.298). (PMID:22145482).

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Description/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.

Item Type: Article
ISSNs: 0334-018X (print)
2191-0251 (electronic)
Keywords: children, diabetes insipidus, neurodegenerative Langerhans cell histiocytosis
Subjects: Q Science > QH Natural history > QH426 Genetics
R Medicine > R Medicine (General)
Divisions: Faculty of Medicine > Human Development and Health
ePrint ID: 207535
Date Deposited: 10 Jan 2012 11:45
Last Modified: 27 Mar 2014 19:49
URI: http://eprints.soton.ac.uk/id/eprint/207535

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