title: Pediatric idiopathic intracranial hypertension and the underlying endocrine-metabolic dysfunction: a pilot study.
abstract: OBJECTIVE
  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.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Salpietro
authors.forename: Kshitij
authors.initials: K
authors.lastname: Mankad
authors.forename: Maria
authors.initials: M
authors.lastname: Kinali
authors.forename: Ashok
authors.initials: A
authors.lastname: Adams
authors.forename: Mariella
authors.initials: M
authors.lastname: Valenzise
authors.forename: Gaetano
authors.initials: G
authors.lastname: Tortorella
authors.forename: Eloisa
authors.initials: E
authors.lastname: Gitto
authors.forename: Agata
authors.initials: A
authors.lastname: Polizzi
authors.forename: Valeria
authors.initials: V
authors.lastname: Chirico
authors.forename: Francesco
authors.initials: F
authors.lastname: Nicita
authors.forename: Emanuele
authors.initials: E
authors.lastname: David
authors.forename: Anna Claudia
authors.initials: AC
authors.lastname: Romeo
authors.forename: Carlo Attilio
authors.initials: CA
authors.lastname: Squeri
authors.forename: Salvatore
authors.initials: S
authors.lastname: Savasta
authors.forename: Gian Luigi
authors.initials: GL
authors.lastname: Marseglia
authors.forename: Teresa
authors.initials: T
authors.lastname: Arrigo
authors.forename: Conrad Earl
authors.initials: CE
authors.lastname: Johanson
authors.forename: Martino
authors.initials: M
authors.lastname: Ruggieri
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004700:Endocrine System Diseases / Q000150:complications*; D005260:Female; D006801:Humans; D019586:Intracranial Hypertension / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D008297:Male; D008659:Metabolic Diseases / Q000150:complications*; D010865:Pilot Projects; D012189:Retrospective Studies