囊性纤维化相关的肝病的病理生理学
Anna Palmiotti1, Anna Bertolini2, Romina Fiorotto1
1Section of Digestive Diseases, Department of Internal Medicine, Yale University, New Haven, Connecticut, USA.
Current opinion in gastroenterology
|December 29, 2025
概括
晚期囊性纤维化肝病 (aCFLD) 涉及肠肝轴功能障碍和血管变化,而不仅仅是胆汁问题. 针对这些新见解的多方面的治疗对于管理这种并发症至关重要.
科学领域:
- 肝病学 肝病学是一种肝病学.
- 胃肠病学 胃肠病学
- 肺部病理学 肺部病理学
背景情况:
- 囊性纤维化肝病 (CFLD) 影响5-10%的囊性纤维化患者.
- 先进的CFLD (aCFLD) 呈现出临床显著的门性高血压.
- 本综述侧重于aCFLD的病原和治疗点.
研究的目的:
- 总结最近对CFLD病变的机制性见解.
- 确定潜在的针对性治疗策略,以治疗aCFLD.
- 为了突出aCFLD的临床意义.
主要方法:
- 关于CFLD病变的最新科学文献的综述.
- 对超出胆汁分泌的促成因素的新兴证据的分析.
- 专注于血管变化和aCFLD的肠肝轴.
主要成果:
- aCFLD的发病包括缺陷的胆汁分泌,免疫失调,肠道失调和肠道屏障功能障碍.
- 肝炎炎症是由这些肠道衍生因素促进的.
- 非肝硬化门高血压是aCFLD的关键临床特征,独立于肝硬化.
结论:
- aCFLD的发病因子是多因素的,涉及因素的复杂相互作用.
- 针对肠肝轴和血管贡献提供了新的治疗途径.
- 未来的研究应该验证这些发现,并评估囊性纤维化转膜导电性调节器调节器和针对微生物的治疗方法.
相关概念视频
Cystic Fibrosis: Pathogenesis
676
Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
676
Cystic Fibrosis: Management
438
Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
Sinus disease and chronic...
438
Chronic Pancreatitis I: Introduction
644
The pancreas, an elongated and flat gland situated behind the stomach, serves a vital function in digesting food and managing blood sugar levels.
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
644
Effect of Hepatic Disease on Pharmacokinetics: Drug Dosing and Hepatic Blood Flow
197
Chronic liver disease significantly impacts drug metabolism due to alterations in hepatic blood flow and enzyme accessibility. This disruption affects the body's pharmacokinetics—the movement and processing of drugs within the system. Key enzymes crucial for metabolizing medications become less accessible, changing how drugs are processed and utilized. Furthermore, liver disease influences the synthesis of plasma proteins, such as albumin and globulins, which play critical roles in drug...
197
Chronic Obstructive Pulmonary Disease-II: Pathophysiology
4.2K
Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
Chronic Inflammation
4.2K
Diseases of the Liver and Gallbladder
1.8K
Liver and gallbladder diseases are a significant health concern, with prominent conditions including cirrhosis, hepatitis, non-alcoholic fatty liver disease (NAFLD), and gallstones. Jaundice is a common manifestation of liver and biliary disease.
Cirrhosis is characterized by the scarring of hepatic lobules in the liver, which are replaced by fibrous tissue, affecting the liver's normal functioning. NAFLD, on the other hand, is caused by an excessive build-up of fat in the liver, not...
Cirrhosis is characterized by the scarring of hepatic lobules in the liver, which are replaced by fibrous tissue, affecting the liver's normal functioning. NAFLD, on the other hand, is caused by an excessive build-up of fat in the liver, not...
1.8K


