摘要
生长激素相关性肝硬化(GHRC)是指垂体前叶功能减退症患者长期生长激素缺乏引起的肝硬化及其并发症。疾病以垂体前叶功能减退、生长激素缺乏和肝硬化为主要临床特征。生长激素和胰岛素样生长因子-1(IGF-1)缺乏导致肝内脂肪积累是引发GHRC主要始动机制。生长激素生理剂量补充治疗可改善症状,逆转疾病进展。GHRC严重损害患者生活质量,甚至危及生命,但当前绝大多数临床医师对此认识不足,误诊误治率极高。因此各科医师对于隐源性肝硬化患者,应警惕GHRC可能。开展垂体前叶激素尤其是生长激素和IGF-1水平测定,精准识别GHRC并及时开始生长激素补充治疗,以逆转病情,挽救生命,避免不必要的肝移植。知晓和提高对GHRC的理解和认识,具有重要临床和理论价值,并可能彻底改变此类患者的诊治和预后。鉴于此,本共识汇聚了国内内分泌科、神经外科、消化科、呼吸科、血液科和肝移植科等领域50余位专家,以推荐意见分级评估、制订与评价(GRADE)系统证据质量分级作为依据,围绕GHRC的诊断、高危因素、生长激素补充治疗风险和获益等关键科学问题,制订本专家共识。再结合德尔菲法评价意见,最终确定10条推荐意见,以帮助临床医师合理应对GHRC。
Growth hormone-related cirrhosis(GHRC)refers to cirrhosis and its complications caused by long-term growth hormone deficiency in patients with anterior hypopituitarism.The primary clinical characteristics include anterior pituitary hypofunction,growth hormone deficiency,and cirrhosis.The main pathogenic mechanism of GHRC is hepatic fat accumulation due to the lack of growth hormone and insulin-like growth factor-1(IGF-1).The replacement therapy with physiological doses of growth hormone can improve symptoms.GHRC severely impairs patient quality of life and can be life-threatening.Yet current clinical awareness is inadequate,leading to high rates of misdiagnosis and mistreatment.Clinicians should be vigilant for GHRC in patients with cryptogenic cirrhosis.Conducting screenings for anterior pituitary hormones especially growth hormone and IGF-1 to accurately identify GHRC and promptly initiate growth hormone replacement therapy,with the aim of reversing the disease condition,saving lives,and avoiding unnecessary liver transplantation.Enhancing understanding and awareness of GHRC holds significant clinical and theoretical value and could revolutionize the diagnosis,treatment,and prognosis of these patients.This consensus statement was developed by over 50 experts from various fields,including endocrinology,neurosurgery,gastroenterology,respiratory medicine,and hematology,based on the Grading of Recommendations Assessment,Development and Evaluation(GRADE)system for clinical evidence levels.It addressed key scientific issues such as the diagnosis,risk factors,and risks and benefits of growth hormone replacement therapy.Integrating Delphi method feedback,the consensus ultimately formulated 10 recommendations to guide clinicians in managing GHRC.
作者
中国老年保健协会生长发育和性腺疾病委员会
中国民族卫生协会内分泌代谢分会
伍学焱
茅江峰
Pubertal Development and Gonadal Disorders Committee from Chinese Aging Well Association;Society of Endocrinology and Metabolic Disease China National Health Association;Wu Xueyan(不详;Department of Endocrinology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China)
出处
《中华医学杂志》
北大核心
2025年第27期2266-2275,共10页
National Medical Journal of China
关键词
肝硬化
生长激素缺乏症
垂体柄中断综合征
颅咽管瘤
颅内生殖细胞瘤
多种垂体前叶激素缺乏
肝肺综合征
诊断
治疗
Liver cirrhosis
Growth hormone deficiency
Pituitary stem interruption syndrome
Craniopharyngioma
Intracranial germinoma
Multiple anterior pituitary hormone deficiencies
Hepatic pulmonary syndrome
Diagnosis
Treatment
