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Pituitary height and neuroradiological alterations in patients with Prader-Labhart-Willi syndrome

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Abstract

Prader-Willi syndrome (PWS), a genetic disorder due to an alteration in the paternally derived long arm of chromosome 15, is characterized by a complex clinical picture (short stature, obesity, hypogonadism) that seems to be referable to as a central hypothalamic/pituitary dysfunction. To determine whether there is any diminution in the anterior pituitary gland or other neuroradiological alterations, we retrospectively analysed 91 patients with PWS (42 females, 49 males; age range: 0.7–16.8 years) by cerebral magnetic resonance imaging (MRI). Of these 91 patients, MRI analysis showed a reduction in pituitary height in 45 patients (49.4%), a complete absence of the posterior pituitary bright spot in six patients (6.6%) and other neuroradiological alterations in ten patients (11%). Altogether, neuroradiological alterations were present in 61 of the 91 (67%) patients. Our results indicate that neuroradiological alterations are more frequent in PWS patients than has been reported to date.

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Authors and Affiliations

  1. Department of Pediatrics, University of Modena and Reggio Emilia, Via del Pozzo, 71-41.100, Modena, Italy

    L. Iughetti, B. Predieri, P. Bruzzi & G. Caselli

  2. Department of Pediatrics, Institute Hospital San Raffaele, University Vita e Salute, Milan, Italy

    L. Bosio

  3. Department of Pediatrics, University of Turin, Turin, Italy

    A. Corrias

  4. A.S.L. Treviglio, Treviglio, Italy

    L. Gargantini

  5. IRCCS Oasi, Troina, Italy

    L. Ragusa

  6. Department of Pediatrics, University of Pavia, Pavia, Italy

    C. Livieri

  7. Istituto Auxologico Italiano, Hospital San Giuseppe IRCCS, Verbania, Italy

    G. Grugni

Authors
  1. L. Iughetti
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  2. L. Bosio
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  3. A. Corrias
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  4. L. Gargantini
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  5. L. Ragusa
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  6. C. Livieri
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  7. B. Predieri
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  8. P. Bruzzi
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  9. G. Caselli
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  10. G. Grugni
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Corresponding author

Correspondence to L. Iughetti.

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Iughetti, L., Bosio, L., Corrias, A. et al. Pituitary height and neuroradiological alterations in patients with Prader-Labhart-Willi syndrome. Eur J Pediatr 167, 701–702 (2008). https://doi.org/10.1007/s00431-007-0555-3

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  • DOI: https://doi.org/10.1007/s00431-007-0555-3

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