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孤立性生长激素缺乏症的磁共振成像(MRI)表现

Magnetic resonance imaging (MRI) findings in isolated growth hormone deficiency.

作者信息

Kandemir N, Cila A, Besim A, Yordam N

机构信息

Department of Pediatrics, Hacettepe University, Faculty of Medicine, Ankara.

出版信息

Turk J Pediatr. 1998 Jul-Sep;40(3):385-92.

PMID:9763902
Abstract

In this study the presence of pituitary-hypothalamic abnormalities was searched by magnetic resonance (MR) imaging in 30 children (18 males and 12 females, aged 7.4 to 23 years) with isolated growth hormone deficiency (IGHD). Small anterior pituitary was demonstrated in 18 patients and ectopic posterior pituitary (EPP) in four of them. Pituitary stalk was found to be thin in two patients with anterior pituitary hypophasia and EPP and was visible only in post-gadolinium images. In one patient, a hypothalamic mass was found and the bright spot of the posterior pituitary was found without diabetes insipidus, possibly due to a variation in the intensity of the bright signal. Eight patients had normal pituitary imaging suggesting functional damage. In all five patients with familial growth hormone deficiency the anterior pituitary was hypoplastic. We conclude that a high percentage of patients with IGHD had anomalies of the hypothalamo-pituitary region, which could be demonstrated by MR imaging. Furthermore, the low frequency of perinatal abnormalities in these patients suggested developmental defect as the cause of the morphostructural abnormalities. The presence of the familial cases with the same defect pointed to the genetic origin in some instances.

摘要

在本研究中,通过磁共振(MR)成像对30例孤立性生长激素缺乏症(IGHD)儿童(18例男性和12例女性,年龄7.4至23岁)进行垂体 - 下丘脑异常检查。18例患者显示垂体前叶小,其中4例有异位后叶(EPP)。在2例垂体前叶发育不全和EPP的患者中发现垂体柄细,且仅在钆增强图像中可见。1例患者发现下丘脑肿块,后叶亮点存在但无尿崩症,可能是由于亮信号强度的变化。8例患者垂体成像正常,提示存在功能损害。在所有5例家族性生长激素缺乏症患者中,垂体前叶发育不全。我们得出结论,高比例的IGHD患者存在下丘脑 - 垂体区域异常,可通过MR成像显示。此外,这些患者围产期异常的低发生率提示发育缺陷是形态结构异常的原因。存在相同缺陷的家族性病例表明在某些情况下存在遗传起源。

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