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磁共振成像在生长激素缺乏症诊断中的应用

Magnetic resonance imaging in the diagnosis of growth hormone deficiency.

作者信息

Argyropoulou M, Perignon F, Brauner R, Brunelle F

机构信息

Paediatric Endocrinology Unit, INSERM U30, Hôpital et Faculté Necker-Enfants Malades, Paris, France.

出版信息

J Pediatr. 1992 Jun;120(6):886-91. doi: 10.1016/s0022-3476(05)81955-9.

Abstract

Forty-six patients with idiopathic growth hormone deficiency were examined by magnetic resonance imaging at a mean (+/- SEM) age of 9 +/- 1 years (range 15 days to 20 years). They were classified into two groups according to MRI images: group 1 (n = 29) had pituitary stalk interruption syndrome and group 2 (n = 17) had normal pituitary anatomy. All patients with pituitary stalk interruption had a pituitary height at less than -2 SD for age; three had no visible anterior pituitary lobe. By contrast, the pituitary height was less than normal in only 10 patients (60%) with normal pituitary anatomy. Growth hormone deficiency was transient in one of the seven patients with normal pituitary anatomy and height. The group with pituitary stalk interruption had the first symptom of growth hormone deficiency at an earlier age (2.8 +/- 0.6 vs 5.5 +/- 1.2 years; p less than 0.001), were of smaller stature (-4 +/- 0.2 vs -3 +/- 0.2 SD; p less than 0.01) and had lower GH peak response to provocative testing (3 +/- 0.4 vs 5 +/- 0.5 ng/ml; p less than 0.001) than did the group with normal pituitary anatomy. Their pituitary gland was also shorter (2.5 +/- 0.2 vs 3.5 +/- 0.2 mm; p less than 0.01). All the patients with multiple pituitary deficiencies except one (n = 19) belonged to this group. One girl with pituitary stalk interruption and deficiencies in growth hormone and thyroid-stimulating hormone had advanced central precocious puberty. We conclude that the evaluation of the shape and height of the pituitary gland by MRI is an additional tool for the diagnosis of growth hormone deficiency. The presence of pituitary stalk interruption confirms this diagnosis and is predictive of multiple anterior pituitary deficiencies. The lack of a significant increase in perinatal abnormalities in this group and the association of pituitary stalk interruption with microphallus and with facial or sella abnormalities suggest that this appearance may have an early antenatal origin. The finding of a familial case of pituitary stalk interruption suggests a genetic origin.

摘要

46例特发性生长激素缺乏症患者接受了磁共振成像检查,平均(±标准误)年龄为9±1岁(范围15天至20岁)。根据磁共振成像图像将他们分为两组:第1组(n = 29)患有垂体柄中断综合征,第2组(n = 17)垂体解剖结构正常。所有垂体柄中断的患者垂体高度低于同年龄-2标准差;3例患者未见明显的垂体前叶。相比之下,垂体解剖结构正常的患者中只有10例(60%)垂体高度低于正常。垂体解剖结构和高度正常的7例患者中有1例生长激素缺乏是暂时性的。垂体柄中断组生长激素缺乏的首发症状出现年龄更早(2.8±0.6岁 vs 5.5±1.2岁;p<0.001),身材更矮小(-4±0.2标准差 vs -3±0.2标准差;p<0.01),对激发试验的生长激素峰值反应更低(3±0.4 ng/ml vs 5±0.5 ng/ml;p<0.001),与垂体解剖结构正常组相比。他们的垂体也更短(2.5±0.2 mm vs 3.5±0.2 mm;p<0.01)。除1例(n = 19)外,所有患有多种垂体功能减退的患者均属于该组。1例患有垂体柄中断且生长激素和促甲状腺激素缺乏的女孩出现了中枢性性早熟。我们得出结论,通过磁共振成像评估垂体的形态和高度是诊断生长激素缺乏的一项辅助手段。垂体柄中断的存在证实了这一诊断,并可预测多种垂体前叶功能减退。该组围产期异常情况无显著增加,以及垂体柄中断与小阴茎、面部或蝶鞍异常的关联提示这种表现可能有早期产前起源。垂体柄中断家族病例的发现提示有遗传起源。

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