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
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普瑞德-威利综合征患者的垂体 MR：大小测定和影像学表现
DOI：
10.1007/bf01403704
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发表时间：
2005
影响因子：
2.3
通讯作者：
S. Taneja
中科院分区：
文献类型：
--
作者：
L. J. Miller;M. Angulo;D. Price;S. Taneja
普瑞德威利综合征 (PWS) 是一种罕见的遗传性疾病，其特征为身材矮小、肥胖、性腺功能减退、肌张力减退、认知障碍和面容畸形。约 70% 的患者存在 15 号染色体近端长臂间质性缺失。其中一些临床特征表明中枢下丘脑/垂体功能障碍，最近的研究表明自发生长激素（GH）分泌明显受损。我们通过磁共振成像 (MRI) 对 15 名 GH 缺乏的 PWS 患者进行了研究，以确定垂体前叶（GH 合成部位）的总体形态尺寸是否缩小。我们还着手对这一患者群体的相关影像学结果进行分类。我们的结果表明，这是第一份通过 MRI 记录 PWS 患者垂体大小的报告。 PWS 患者的垂体前叶高度与正常儿童或单纯 GH 缺乏症儿童相比，没有发现统计学上的显着差异。一个有趣的影像学发现是，15 名患者中的 3 名 (20%) 表现出垂体后叶亮点 (PPBS) 完全缺失，第四名患者表现出小 PPBS。这些观察结果反映了下丘脑的客观生理紊乱。讨论了这些发现的临床和放射学意义。
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.
影响因子：
8
作者：
V. A. Holm;S. Cassidy;M. Butler;J. M. Hanchett;L. R. Greenswag;B. Whitman;F. Greenberg
通讯作者：
V. A. Holm;S. Cassidy;M. Butler;J. M. Hanchett;L. R. Greenswag;B. Whitman;F. Greenberg
健康志愿者垂体形态的 MR 评估：年龄和性别相关差异。
DOI：
--
发表时间：
1992
期刊：
AJNR. American journal of neuroradiology
影响因子：
--
作者：
Doraiswamy,PM;Potts,JM;Axelson,DA;Husain,MM;Lurie,SN;Na,C;Escalona,PR;McDonald,WM;Figiel,GS;EllinwoodJr,EH
通讯作者：
EllinwoodJr,EH