2021 英国临床实践共识指南:特发性垂体柄增厚、中枢性尿崩症或两者兼有的儿童和青少年的管理

2021-09-01 英国儿科内分泌和糖尿病协会 The Lancet Child & Adolescent Health

不明原因或特发性垂体柄增厚或中枢性尿崩症不仅在 40% 的病例中隐藏着罕见的隐匿性恶性肿瘤,而且还可以反映良性先天性缺陷。

中文标题:

2021 英国临床实践共识指南:特发性垂体柄增厚、中枢性尿崩症或两者兼有的儿童和青少年的管理

英文标题:

Management of children and young people with idiopathic pituitary stalk thickening, central diabetes insipidus, or both: a national clinical practice consensus guideline

发布日期:

2021-09-01

简要介绍:

不明原因或特发性垂体柄增厚或中枢性尿崩症不仅在 40% 的病例中隐藏着罕见的隐匿性恶性肿瘤,而且还可以反映良性先天性缺陷。2014 年至 2019 年间,英国的一个多学科专家国家指南制定小组系统地制定了管理流程图和临床实践指南,以告知专科护理并改善患有特发性垂体柄增厚、中枢神经系统疾病的儿童和年轻人(<19 岁)的预后。尿崩症,或两者兼而有之。所有这些特发性垂体柄增厚和中枢性尿崩症都需要动态垂体功能测试、专业垂体成像、血清β-人绒毛膜促性腺激素和甲胎蛋白浓度的测量、胸部X光检查、腹部超声检查验光和隐匿性疾病的骨骼检查。视交叉处的茎秆增厚 4 mm 或以上,垂体插入处的茎秆增厚3 mm 或以上,或两者兼而有之,可能是病理性的,尤其是在内分泌疾病或视力障碍并存的情况下。在本指南中,我们定义了监测、脑脊液肿瘤标志物、全身成像、适应症、茎部活检的时间和风险以及出院标准。我们鼓励对结果进行登记以验证本指南中描述的系统方法和研究,以建立典型的儿科茎大小以及新型生物标志物、成像技术或两者在诊断中的可能作用。

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