Journal of Pediatric Endocrinology and Metabolism
Editor-in-Chief: Kiess, Wieland
Ed. by Bereket, Abdullah / Cohen, Pinhas / Darendeliler, Feyza / Dattani, Mehul / Gustafsson, Jan / Luo, Feihong / Mericq, Veronica / Roth, Christian / Toppari, Jorma
Editorial Board Member: Battelino, Tadej / Buyukgebiz, Atilla / Cassorla, Fernando / Chrousos, George P. / Cutfield, Wayne / Fideleff, Hugo L. / Hershkovitz, Eli / Hiort, Olaf / LaFranchi, Stephen H. / Lanes M. D., Roberto / Mohn, Angelika / Root, Allen W. / Rosenfeld, Ron G. / Werther, George / Zadik, Zvi
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Pediatric idiopathic intracranial hypertension and the underlying endocrine-metabolic dysfunction: a pilot study
1Department of Pediatrics, University of Messina, Messina, Italy
2Chelsea and Westminster Hospital, London, UK
3National Centre for Rare Diseases, Superior Institute of Health, Rome, Italy
4Institute of Neurological Sciences, National Research Council (CNR), Catania, Italy
5Department of Pediatrics, Child Neurology Division, Sapienza University, Rome, Italy
6Department of Radiology, University of Messina, Messina, Italy
7Ophthalmology Unit, Department of Experimental Medical Surgical Sciences and Odontostomatology, University of Messina, Messina, Italy
8Department of Pediatrics, University of Pavia, IRCCS San Matteo, Pavia, Italy
9Department of Educational Sciences, University of Catania, Catania, Italy
10Unit of Neurosurgery, University of Catania, Catania, Italy
11Great Ormond Street Children’s Hospital, London, UK
12Department of Neurosurgery, Brown University, Providence, Rhode Island, USA
Citation Information: Journal of Pediatric Endocrinology and Metabolism. Volume 27, Issue 1-2, Pages 107–115, ISSN (Online) 2191-0251, ISSN (Print) 0334-018X, DOI: 10.1515/jpem-2013-0156, September 2013
- Published Online:
Aim: To unravel the potential idiopathic intracranial hypertension (IIH) endocrine-metabolic comorbidities by studying the natural (and targeted drug-modified) history of disease in children. IIH is a disorder of unclear pathophysiology, characterized by raised intracranial pressure without hydrocephalus or space-occupying lesion coupled with normal cerebrospinal fluid (CSF) composition.
Methods: Retrospective study (years 2001–2010) of clinical records and images and prospective follow-up (years 2010–2013) in 15 children (11 girls, 4 boys; aged 5–16 years) diagnosed previously as “IIH”, according to the criteria for pediatric IIH proposed by Rangwala, at four university pediatric centers in northern, central, and southern Italy.
Results: We identified six potential endocrine-metabolic comorbidities including, weight gain and obesity (n=5), recombinant growth hormone therapy (n=3), obesity and metabolic syndrome (n=1), secondary hyperaldosteronism (n=1), hypervitaminosis A (n=1), and corticosteroid therapy (n=1). Response to etiologically targeted treatments (e.g., spironolactone, octreotide) was documented.
Conclusions: IIH is a protean syndrome caused by various potential (risk and) associative factors. Several conditions could influence the pressure regulation of CSF. An endocrine-metabolic altered homeostasis could be suggested in some IIH patients, and in this context, etiologically targeted therapies (spironolactone) should be considered
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