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Journal of Pediatric Endocrinology and Metabolism

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In This Section
Volume 27, Issue 1-2 (Jan 2014)


Pediatric idiopathic intracranial hypertension and the underlying endocrine-metabolic dysfunction: a pilot study

Vincenzo Salpietro
  • Corresponding author
  • Department of Pediatrics, University of Messina, Messina, Italy
  • Email:
/ Kshitij Mankad
  • Department of Pediatrics, University of Messina, Messina, Italy
  • Great Ormond Street Children’s Hospital, London, UK
/ Maria Kinali
  • Chelsea and Westminster Hospital, London, UK
/ Ashok Adams
  • Department of Pediatrics, University of Messina, Messina, Italy
  • Great Ormond Street Children’s Hospital, London, UK
/ Mariella Valenzise
  • Department of Pediatrics, University of Messina, Messina, Italy
/ Gaetano Tortorella
  • Department of Pediatrics, University of Messina, Messina, Italy
/ Eloisa Gitto
  • Department of Pediatrics, University of Messina, Messina, Italy
/ Agata Polizzi
  • National Centre for Rare Diseases, Superior Institute of Health, Rome, Italy
  • Institute of Neurological Sciences, National Research Council (CNR), Catania, Italy
/ Valeria Chirico
  • Department of Pediatrics, University of Messina, Messina, Italy
/ Francesco Nicita
  • Department of Pediatrics, Child Neurology Division, Sapienza University, Rome, Italy
/ Emanuele David
  • Department of Radiology, University of Messina, Messina, Italy
/ Anna Claudia Romeo
  • Department of Pediatrics, University of Messina, Messina, Italy
/ Carlo Attilio Squeri
  • Ophthalmology Unit, Department of Experimental Medical Surgical Sciences and Odontostomatology, University of Messina, Messina, Italy
/ Salvatore Savasta
  • Department of Pediatrics, University of Pavia, IRCCS San Matteo, Pavia, Italy
/ Gian Luigi Marseglia
  • Department of Pediatrics, University of Pavia, IRCCS San Matteo, Pavia, Italy
/ Teresa Arrigo
  • Department of Pediatrics, University of Messina, Messina, Italy
/ Conrad Earl Johanson
  • Department of Pediatrics, University of Messina, Messina, Italy
  • Department of Neurosurgery, Brown University, Providence, Rhode Island, USA
/ Martino Ruggieri
  • Department of Pediatrics, University of Messina, Messina, Italy
  • Department of Educational Sciences, University of Catania, Catania, Italy
  • Unit of Neurosurgery, University of Catania, Catania, Italy
Published Online: 2013-09-11 | DOI: https://doi.org/10.1515/jpem-2013-0156


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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About the article

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:

Received: 2013-04-21

Accepted: 2013-08-06

Published Online: 2013-09-11

Published in Print: 2014-01-01

Citation Information: Journal of Pediatric Endocrinology and Metabolism, ISSN (Online) 2191-0251, ISSN (Print) 0334-018X, DOI: https://doi.org/10.1515/jpem-2013-0156. Export Citation

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