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自身免疫性多腺体综合征 3 型和生长激素缺乏症。

Autoimmune polyglandular syndrome Type 3 and growth hormone deficiency.

机构信息

Department of Pediatric Endocrinology, Rhode Island Hospital - The Warren Alpert Medical School of Brown University, Providence, RI 02903, USA.

出版信息

Pediatr Diabetes. 2010 Sep;11(6):438-42. doi: 10.1111/j.1399-5448.2009.00622.x. Epub 2009 Dec 8.

Abstract

The simultaneous occurrence of prepubertal Graves' disease, type 1 Diabetes Mellitus (DM), and Growth hormone deficiency (GHD) is uncommon. GHD has been reported in Autoimmune Polyglandular Syndrome (APS) Type 1 and Type 2 but not in APS Type 3. We report a 3-yr-old boy who presented simultaneously with type 1 DM and Graves' disease. After he developed urticarial rash to Propylthiouracil and Methimazole with persistent thyrotoxicosis, he received 8 millicuries of (131)I at 5 yr of age. We diagnosed GHD at age 8 yr 8 months because of growth deceleration (from 95 to 25%) and abnormal growth rate (3 cm/yr) despite euthyroidism, fair glycemic control, and normal weight gain. Both insulin-like growth factor (IGF) 1 (90 ng/mL; normal 113-261 ng/mL) and IGFBP3 (1.3 mcg/mL; normal 2.1-4.2 mcg/mL) levels were low and peak growth hormone level measured by RIA was 5.2 ng/mL after L-Dopa and insulin tolerance test. The rest of his pituitary functions and magnetic resonance imaging of the pituitary gland were normal. Growth hormone treatment (0.3 mg/kg/wk) was administered at 8 yr 9 months until near final adult height (FAH). Near FAH (172 cm) was close to midparental target height of 180 cm. GHD may be a component of all APS even though it is rare. Growth in treated children with Graves' disease should be followed closely as catch down growth below genetic height potential may be a harbinger of underlying GHD.

摘要

同时发生的青春期前格雷夫斯病、1 型糖尿病(DM)和生长激素缺乏症(GHD)并不常见。GHD 已在自身免疫性多腺体综合征(APS)1 型和 2 型中报告过,但不在 APS 3 型中报告。我们报告了一名 3 岁男孩,他同时患有 1 型 DM 和格雷夫斯病。在他因持续性甲状腺毒症对丙基硫氧嘧啶和甲巯咪唑出现荨麻疹后,他在 5 岁时接受了 8 毫居里的 (131)I。由于生长速度减慢(从 95%降至 25%)和生长速度异常(3 cm/年),尽管甲状腺功能正常、血糖控制良好且体重正常增加,我们在 8 岁 8 个月时诊断为 GHD。胰岛素样生长因子(IGF)1(90ng/mL;正常 113-261ng/mL)和 IGFBP3(1.3mcg/mL;正常 2.1-4.2mcg/mL)水平均较低,RIA 测量的峰值生长激素水平在 L-多巴和胰岛素耐量试验后为 5.2ng/mL。他的其余垂体功能和垂体磁共振成像均正常。在 8 岁 9 个月时开始给予生长激素治疗(0.3mg/kg/周),直到接近最终成年身高(FAH)。接近 FAH(172cm)接近中亲身高目标 180cm。尽管 GHD 很少见,但它可能是所有 APS 的一个组成部分。患有格雷夫斯病的经治疗儿童的生长情况应密切随访,因为低于遗传身高潜力的追赶生长可能是潜在 GHD 的先兆。

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