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布鲁姆综合征伴生长激素缺乏:一种罕见的关联。

Bloom's syndrome with growth hormone deficiency: a rare association.

机构信息

Endocrinology, Gandhi Medical College and Hospital, Secunderabad, Telangana, India

Endocrinology, Gandhi Medical College and Hospital, Secunderabad, Telangana, India.

出版信息

BMJ Case Rep. 2020 Oct 29;13(10):e235238. doi: 10.1136/bcr-2020-235238.

Abstract

We report a case of a 5-year-old boy presenting to us with short stature. He was born of consanguineous parentage and was small for gestational age. He had severe short stature, with height Z score of -6.2 SD Score, markedly delayed skeletal age, low level of insulin-like growth factor 1, unstimulated growth hormone and hypoplastic anterior pituitary gland on MRI. He was advised growth hormone (GH) replacement at 2 years of age, but he did not receive it . Later on, he developed photosensitive telangiectatic lesions over face and required multiple hospital admissions for recurrent systemic infections. Genetic analysis confirmed the diagnosis of Bloom's syndrome. The present case report illustrates the need for high vigilance for conditions like Bloom's syndrome in growth hormone deficiency (GHD), in whom GH treatment could potentially be harmful. Bloom's syndrome with GHD is an exceedingly rare association.

摘要

我们报告了一例 5 岁男孩因身材矮小就诊。他出生于近亲家庭,且为小于胎龄儿。他身材矮小,身高 Z 评分-6.2,骨骼年龄明显延迟,胰岛素样生长因子 1 水平低,MRI 显示垂体前叶发育不良。他在 2 岁时被建议接受生长激素(GH)替代治疗,但并未接受。后来,他面部出现光敏性毛细血管扩张性皮损,需要多次住院治疗反复发生的全身感染。基因分析确诊为布卢姆综合征。本病例报告说明了在生长激素缺乏症(GHD)中对布卢姆综合征等疾病保持高度警惕的必要性,因为 GH 治疗可能有害。Bloom 综合征合并 GHD 极为罕见。

相似文献

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Bloom's syndrome in an Indian man in the UK.一名在英国的印度男子患布卢姆综合征。
BMJ Case Rep. 2016 Jan 5;2016:bcr2015212297. doi: 10.1136/bcr-2015-212297.

本文引用的文献

1
Study of Pituitary Morphometry Using MRI in Indian Subjects.印度受试者垂体形态测量的磁共振成像研究
Indian J Endocrinol Metab. 2018 Sep-Oct;22(5):605-609. doi: 10.4103/ijem.IJEM_199_18.
6
Evaluation of short stature, carbohydrate metabolism and other endocrinopathies in Bloom's syndrome.
Horm Res. 2006;66(3):111-7. doi: 10.1159/000093826. Epub 2006 Jun 9.
9
Leukemia in growth-hormone-treated patients: an update, 1992.
Horm Res. 1992;38 Suppl 1:56-62. doi: 10.1159/000182571.

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