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相关概念视频

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

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, but...
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

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 sinusitis...
Chronic Pancreatitis II: Pathophysiology01:21

Chronic Pancreatitis II: Pathophysiology

Chronic pancreatitis is a progressive and irreversible inflammation of the pancreas, most often caused by long-term alcohol abuse, but it can also be related to ductal obstruction, smoking, or genetic factors.Chronic pancreatitis occurs when the pancreas is repeatedly exposed to harmful agents like alcohol, smoking, ductal obstruction, or genetic predisposition. These factors lead to the release of toxic metabolites and inflammatory cytokines, sustaining chronic inflammation in the pancreatic...
Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features01:24

Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features

Chronic bronchitis is a key phenotype of chronic obstructive pulmonary disease (COPD), characterized by airway-centered inflammation and mucus overproduction. It develops from long-term exposure to harmful particles or gases, most commonly cigarette smoke, which triggers a persistent inflammatory response.Cellular and Structural ChangesInflammation initially affects the large bronchi and later the smaller airways, with infiltration by immune cells, including neutrophils, macrophages, and...
Chronic Pancreatitis I: Introduction01:25

Chronic Pancreatitis I: Introduction

Chronic pancreatitis is a long-standing, relapsing inflammation of the pancreas, characterized by irreversible damage to the gland. It results in progressive destruction of the pancreatic parenchyma, fibrosis, and eventual loss of both exocrine and endocrine function. The disease may evolve gradually after multiple episodes of acute pancreatitis or develop independently.EtiologyChronic pancreatitis can arise from a variety of causes:Alcohol use is the leading cause, accounting for 70–80% of...
Chronic Pancreatitis I: Introduction01:24

Chronic Pancreatitis I: Introduction

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...

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A multi-center study of non-invasive colorectal cancer evaluation in cystic fibrosis (NICE-CF).

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Study Design Considerations for the Evaluation of Pancreatic Enzyme Replacement Therapy (PERT) Products in People with Cystic Fibrosis: An Update.

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Increased gastrointestinal cancer risk in cystic fibrosis: Screening, prevention, and future directions.

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Longitudinal changes in bone mineral density after initiation of elexacaftor-tezacaftor-ivacaftor in youth and adults with cystic fibrosis: PROMISE-ENDO.

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Corrigendum to "Results of a clinical trial of ANG003, a non-porcine pancreatic enzyme replacement therapy, in people with cystic fibrosis" [Journal of Cystic Fibrosis Original Article Articles in Press July 31, 2025].

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相关实验视频

Updated: Jun 23, 2026

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
05:56

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Published on: August 29, 2025

囊性纤维化症是什么

Brian P O'Sullivan1, Steven D Freedman

  • 1Department of Pediatrics, University of Massachusetts Medical School, Worcester, MA 01655, USA. osullivb@ummhc.org

Lancet (London, England)
|May 1, 2009
PubMed
概括

囊性纤维化 (CF) 护理因早期诊断和针对基本缺陷的先进疗法而得到改善. 新的治疗方法正在出现,为患有这种常见致命遗传疾病的患者提供了希望.

科学领域:

  • 遗传学 是一个遗传学.
  • 肺部病理学 肺部病理学
  • 分子生物学分子生物学

背景情况:

  • 囊性纤维化是高加索人群中普遍存在的致命遗传疾病.
  • 患者治疗结果的显著改善归因于早期诊断,密集治疗和专业护理中心.
  • 了解CF分子基础的进展为新的治疗策略铺平了道路.

研究的目的:

  • 审查目前的囊性纤维化临床护理标准.
  • 讨论了解CF病变的最新突破.
  • 突出新生儿查方面的进展和针对潜在分子缺陷的治疗方法的开发.

主要方法:

  • 临床护理实践的文献综述.
  • 分析关于囊性纤维化病原体的最新研究.
  • 检查新兴的新生儿查协议.
  • 评估新的治疗方法,包括高血压盐水和基本缺陷的治疗方法.

主要成果:

  • 患有囊性纤维化症的患者前景随着时间的推移显示出一致的改善.
  • 更深入地了解驱动CF的分子缺陷正在促进新的治疗途径.
  • 高血压盐水是一种成熟的治疗方法,其他新疗法正在先进的开发中.

更多相关视频

Cystic Fibrosis Aggregate Biofilm Model to Study Infection-relevant Gene Expression
08:58

Cystic Fibrosis Aggregate Biofilm Model to Study Infection-relevant Gene Expression

Published on: April 18, 2025

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
08:00

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study

Published on: April 11, 2018

相关实验视频

Last Updated: Jun 23, 2026

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
05:56

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Published on: August 29, 2025

Cystic Fibrosis Aggregate Biofilm Model to Study Infection-relevant Gene Expression
08:58

Cystic Fibrosis Aggregate Biofilm Model to Study Infection-relevant Gene Expression

Published on: April 18, 2025

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
08:00

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study

Published on: April 11, 2018

结论:

  • 了解囊性纤维化病原体的持续进展至关重要.
  • 加强新生儿查和向治疗正在改变CF管理.
  • 解决基本分子缺陷的治疗方法的开发对改善患有囊性纤维化个体的生活具有重大前景.