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MR of the pituitary in patients with Prader-Willi syndrome: Size determination and imaging findings

MR of the pituitary in patients with Prader-Willi syndrome: Size determination and imaging findings 247 26 26 1 1 L. Miller M. Angulo D. Price S. Taneja Department of Radiology Winthrop University Hospital 259 First Street 11501 Mineola NY USA Department of Pediatrics, Division of Endocrinology and Genetics Winthrop University Hospital 259 First Street 11501 Mineola NY USA Abstract Prader-Willi syndrome (PWS) is an unusual genetic disorder characterized by short stature, obesity, hypogonadism, hypotonia, cognitive impairment, and dysmorphic facies. There is an interstitial deletion of the proximal long arm of chromosome 15 in about 70% of patients. Some of these clinical features suggest a central hypothalamic/pituitary dysfunction, and recent investigations have demonstrated a marked impairment in spontaneous growth hormone (GH) secretion. We studied 15 GH-deficient PWS patients by magnetic resonance imaging (MRI) to determine whether there was a diminution in the gross morphological size of the anterior pituitary gland, the site of GH synthesis. We also set out to catalog the pertinent imaging findings in this patient population. Our results indicate that this is the first report documenting pituitary size by MRI in PWS patients. No statistically significant difference was found in the height of the anterior pituitary gland in PWS patients compared with either normal children or children with isolated GH deficiency. An interesting imaging finding is that three of 15 patients (20%) demonstrated complete absence of the posterior pituitary bright spot (PPBS), and a fourth patient demonstrated a small PPBS. These observations reflect an objective physiologic disturbance in the hypothalamus. The clinical and radiologic implications of these findings are discussed. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Pediatric Radiology Springer Journals

MR of the pituitary in patients with Prader-Willi syndrome: Size determination and imaging findings

Pediatric Radiology , Volume 26 (1) – Jan 1, 1996

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References (17)

Publisher
Springer Journals
Copyright
Copyright © 1996 by Springer-Verlag
Subject
Medicine & Public Health; Imaging / Radiology; Pediatrics
ISSN
0301-0449
eISSN
1432-1998
DOI
10.1007/BF01403704
Publisher site
See Article on Publisher Site

Abstract

247 26 26 1 1 L. Miller M. Angulo D. Price S. Taneja Department of Radiology Winthrop University Hospital 259 First Street 11501 Mineola NY USA Department of Pediatrics, Division of Endocrinology and Genetics Winthrop University Hospital 259 First Street 11501 Mineola NY USA Abstract Prader-Willi syndrome (PWS) is an unusual genetic disorder characterized by short stature, obesity, hypogonadism, hypotonia, cognitive impairment, and dysmorphic facies. There is an interstitial deletion of the proximal long arm of chromosome 15 in about 70% of patients. Some of these clinical features suggest a central hypothalamic/pituitary dysfunction, and recent investigations have demonstrated a marked impairment in spontaneous growth hormone (GH) secretion. We studied 15 GH-deficient PWS patients by magnetic resonance imaging (MRI) to determine whether there was a diminution in the gross morphological size of the anterior pituitary gland, the site of GH synthesis. We also set out to catalog the pertinent imaging findings in this patient population. Our results indicate that this is the first report documenting pituitary size by MRI in PWS patients. No statistically significant difference was found in the height of the anterior pituitary gland in PWS patients compared with either normal children or children with isolated GH deficiency. An interesting imaging finding is that three of 15 patients (20%) demonstrated complete absence of the posterior pituitary bright spot (PPBS), and a fourth patient demonstrated a small PPBS. These observations reflect an objective physiologic disturbance in the hypothalamus. The clinical and radiologic implications of these findings are discussed.

Journal

Pediatric RadiologySpringer Journals

Published: Jan 1, 1996

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