Cystic fibrosis related liver disease and endocrine considerations

IF 4.2 Q1 ENDOCRINOLOGY & METABOLISM Journal of Clinical and Translational Endocrinology Pub Date : 2022-03-01 DOI:10.1016/j.jcte.2021.100283
Jordan S. Sherwood , Jagdeesh Ullal , Katherine Kutney , Kara S. Hughan
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引用次数: 3

Abstract

Cystic fibrosis-liver disease (CFLD) is one of the most common non-pulmonary complications in the CF population, is associated with significant morbidity and represents the third leading cause of mortality in those with CF. CFLD encompasses a broad spectrum of hepatobiliary manifestations ranging from mild transaminitis, biliary disease, hepatic steatosis, focal biliary cirrhosis and multilobular biliary cirrhosis. The diagnosis of CFLD and prediction of disease progression remains a clinical challenge. The identification of novel CFLD biomarkers as well as the role of newer imaging techniques such as elastography to allow for early detection and intervention are active areas of research focus. Biliary cirrhosis with portal hypertension represents the most severe spectrum of CFLD, almost exclusively develops in the pediatric population, and is associated with a decline in pulmonary function, poor nutritional status, and greater risk of hospitalization. Furthermore, those with CFLD are at increased risk for vitamin deficiencies and endocrinopathies including CF-related diabetes, CF-related bone disease and hypogonadism, which can have further implications on disease outcomes and management. Effective treatment for CFLD remains limited and current interventions focus on optimization of nutritional status, identification and treatment of comorbid conditions, as well as early detection and management of CFLD specific sequelae such as portal hypertension or variceal bleeding. The extent to which highly effective modulator therapies may prevent the development or modify the progression of CFLD remains an active area of research. In this review, we discuss the challenges with defining and evaluating CFLD and the endocrine considerations and current management of CFLD.

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囊性纤维化相关的肝脏疾病和内分泌的考虑
囊性纤维化-肝脏疾病(CFLD)是CF人群中最常见的非肺部并发症之一,发病率高,是CF患者死亡的第三大原因。CFLD包括广泛的肝胆表现,包括轻度转膜炎、胆道疾病、肝脂肪变性、局灶性胆汁性肝硬化和多小叶性胆汁性肝硬化。CFLD的诊断和疾病进展的预测仍然是一个临床挑战。新的CFLD生物标志物的鉴定以及新的成像技术(如弹性成像)的作用,使得早期检测和干预成为研究的重点领域。胆汁性肝硬化合并门脉高压是CFLD最严重的一种,几乎只发生在儿科人群中,并与肺功能下降、营养状况不良和住院风险增加有关。此外,CFLD患者患维生素缺乏症和内分泌疾病(包括cf相关糖尿病、cf相关骨病和性腺功能减退)的风险增加,这可能对疾病结局和管理产生进一步影响。CFLD的有效治疗仍然有限,目前的干预措施侧重于优化营养状况,识别和治疗合并症,以及早期发现和管理CFLD特异性后遗症,如门静脉高压或静脉曲张出血。高效调节疗法在多大程度上可能阻止CFLD的发展或改变CFLD的进展仍然是一个活跃的研究领域。在这篇综述中,我们讨论了定义和评估CFLD的挑战,以及CFLD的内分泌考虑和当前的管理。
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CiteScore
6.10
自引率
0.00%
发文量
24
审稿时长
16 weeks
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