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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

1 / Kshitij Mankad11 / Maria Kinali2 / Ashok Adams11 / Mariella Valenzise1 / Gaetano Tortorella1 / Eloisa Gitto1 / Agata Polizzi3, 4 / Valeria Chirico1 / Francesco Nicita5 / Emanuele David6 / Anna Claudia Romeo1 / Carlo Attilio Squeri7 / Salvatore Savasta8 / Gian Luigi Marseglia8 / Teresa Arrigo1 / Conrad Earl Johanson12 / Martino Ruggieri9, 10

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

Corresponding author: Vincenzo Salpietro, MD, Unit of Genetics and Pediatric Immunology, Department of Pediatrics, University of Messina, Policlinico Universitario “Gaetano Martino”, Via Consolare Valeria, 1 98125 Messina, Italy, Phone: +39 090 2213115, Fax: +39 090 2217029, E-mail:

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

Publication History

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

Keywords: aldosterone; childhood obesity; children; idiopathic intracranial hypertension; magnetic resonance imaging; metabolic syndrome; pseudotumor cerebri; spironolactone

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