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Laron syndrome (primary growth hormone resistance or insensitivity): the personal experience 1958-2003.拉龙综合征(原发性生长激素抵抗或不敏感):1958 - 2003年个人经历
J Clin Endocrinol Metab. 2004 Mar;89(3):1031-44. doi: 10.1210/jc.2003-031033.
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Refractive evaluation in children with growth defect.
Curr Eye Res. 2004 Jan;28(1):11-5. doi: 10.1076/ceyr.28.1.11.23491.
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Craniofacial and brain abnormalities in Laron syndrome (primary growth hormone insensitivity).
Eur J Endocrinol. 2002 Apr;146(4):499-503. doi: 10.1530/eje.0.1460499.
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Reappraisal of the human ocular growth curve in fetal life, infancy, and early childhood.对胎儿期、婴儿期和幼儿期人类眼部生长曲线的重新评估。
Br J Ophthalmol. 1996 Oct;80(10):918-21. doi: 10.1136/bjo.80.10.918.
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Body fat in Laron syndrome patients: effect of insulin-like growth factor I treatment.拉龙综合征患者的体脂:胰岛素样生长因子I治疗的效果。
Horm Res. 1993;40(1-3):16-22. doi: 10.1159/000183762.
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Growth hormone receptor deficiency in Ecuador: clinical and biochemical phenotype in two populations.厄瓜多尔的生长激素受体缺乏症:两个群体的临床和生化表型
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Skull changes in pituitary dwarfism and the syndrome of familial dwarfism with high plasma immunoreactive growth hormone--a Roentgenologic study.垂体性侏儒症及伴有高血浆免疫反应性生长激素的家族性侏儒症综合征的颅骨改变——一项X线研究
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拉隆综合征患者的眼球和眼眶。

The globe and orbit in Laron syndrome.

机构信息

Imaging Department, Schneider Children's Medical Center of Israel, Petah Tiqwa, Israel.

出版信息

AJNR Am J Neuroradiol. 2011 Sep;32(8):1560-2. doi: 10.3174/ajnr.A2573. Epub 2011 Jul 14.

DOI:10.3174/ajnr.A2573
PMID:21757529
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7964357/
Abstract

BACKGROUND AND PURPOSE

Patients with LS have an inborn growth hormone resistance, resulting in failure to generate IGF-1. The purpose of this study was to evaluate the size of the eye and orbit in LS.

MATERIALS AND METHODS

We retrospectively reviewed the MR imaging of the brain in 9 patients with LS for the following parameters: axial diameter of the globe, interzygomatic distance, perpendicular distance from the interzygomatic line to margins of the globe, medial-to-lateral diameter of the orbit at the anterior orbital rim, distance from the anterior orbital rim to the anterior globe, maximal distance between the medial walls of the orbits, lateral orbital wall angle, lateral orbital wall length, and mediolateral thickness of the intraorbital fat in the most cranial image of the orbit. All measurements were made bilaterally. Twenty patients referred for MR imaging for unrelated reasons served as control subjects.

RESULTS

Compared with the control group, the patients with LS had a significantly smaller maximal globe diameter and shallower but wider orbits due to a shorter lateral wall, a smaller medial distance between the orbits, and a larger angle of the orbit. The ratio between the most anterior orbital diameter and the globe was greater than that in controls. The position of the globe was more anterior in relation to the interzygomatic line.

CONCLUSIONS

Shallow and wide orbits and small globes relative to orbital size are seen in LS and may be secondary to IGF-1 deficiency.

摘要

背景与目的

LS 患者存在先天性生长激素抵抗,导致 IGF-1 生成失败。本研究旨在评估 LS 患者的眼球和眼眶大小。

材料与方法

我们回顾性分析了 9 例 LS 患者的脑部 MRI 图像,评估了以下参数:眼球的轴向直径、面颧距、面颧线至眼球边缘的垂直距离、眶前缘处眶内的前后径、眶前缘至前眼球的距离、眶内最宽处眶内的内-外侧直径、眶外侧壁角度、眶外侧壁长度以及眶内脂肪的中-外侧厚度。所有测量均为双侧测量。20 例因其他原因行 MRI 检查的患者作为对照组。

结果

与对照组相比,LS 患者的最大眼球直径明显较小,眼眶较浅但较宽,原因是眶外侧壁较短、眶内距离较小、眶角较大。最前眶径与眼球的比例大于对照组。眼球相对于面颧线的位置更靠前。

结论

LS 患者的眼眶较浅且较宽,眼球相对较小,可能与 IGF-1 缺乏有关。