Pathophysiology and Genetics of Bronchiectasis Unrelated to Cystic Fibrosis
Апстракт
Bronchiectasis is characterized by deregulated inflammatory response and recurrent bacterial infection resulting in progressive lung damage and an irreversible dilatation of bronchi and bronchioles. Generally accepted model of the development of bronchiectasis is the "vicious cycle hypothesis" that proposes compromising of the mucociliary clearance by an initial event, which leads to the infection of the respiratory tract followed by further impairment of mucociliary function, bacterial proliferation, and more inflammation. Bronchiectasis is a very common symptom in patients with cystic fibrosis (CF), while bronchiectasis unrelated to CF is heterogeneous pathology of unknown cause with a large number of potential contributory factors and poorly understood pathogenesis. It is presumed that bronchiectasis unrelated to CF is a multifactorial condition predisposed by genetic factors. Different molecules have been implicated in the onset and development of idiopathic bronchiectasis, as well... as modulation of the disease severity and response to therapy. Most of these molecules are involved in the processes that contribute to the homeostasis of the lung tissue, especially mucociliary clearance, protease-antiprotease balance, and immunomodulation. Evaluation of the studies performed towards investigation of the role these molecules play in bronchiectasis identifies genetic variants that may be of potential importance for clinical management of the disease, and also of interest for future research efforts. This review focuses on the molecules with major roles in lung homeostasis and their involvement in bronchiectasis unrelated to CF.
Кључне речи:
Pathophysiology / Genetics / BronchiectasisИзвор:
Lung, 2018, 196, 4, 383-392Издавач:
- Springer, New York
Финансирање / пројекти:
- Комплексне болести као модел систем за проучавање модулације фенотипа-структурна и функционална анализа молекуларних биомаркера (RS-MESTD-Basic Research (BR or ON)-173008)
DOI: 10.1007/s00408-018-0121-y
ISSN: 0341-2040
PubMed: 29754320
WoS: 000439321500003
Scopus: 2-s2.0-85046799303
Институција/група
Institut za molekularnu genetiku i genetičko inženjerstvoTY - JOUR AU - Nikolić, Aleksandra PY - 2018 UR - https://imagine.imgge.bg.ac.rs/handle/123456789/1114 AB - Bronchiectasis is characterized by deregulated inflammatory response and recurrent bacterial infection resulting in progressive lung damage and an irreversible dilatation of bronchi and bronchioles. Generally accepted model of the development of bronchiectasis is the "vicious cycle hypothesis" that proposes compromising of the mucociliary clearance by an initial event, which leads to the infection of the respiratory tract followed by further impairment of mucociliary function, bacterial proliferation, and more inflammation. Bronchiectasis is a very common symptom in patients with cystic fibrosis (CF), while bronchiectasis unrelated to CF is heterogeneous pathology of unknown cause with a large number of potential contributory factors and poorly understood pathogenesis. It is presumed that bronchiectasis unrelated to CF is a multifactorial condition predisposed by genetic factors. Different molecules have been implicated in the onset and development of idiopathic bronchiectasis, as well as modulation of the disease severity and response to therapy. Most of these molecules are involved in the processes that contribute to the homeostasis of the lung tissue, especially mucociliary clearance, protease-antiprotease balance, and immunomodulation. Evaluation of the studies performed towards investigation of the role these molecules play in bronchiectasis identifies genetic variants that may be of potential importance for clinical management of the disease, and also of interest for future research efforts. This review focuses on the molecules with major roles in lung homeostasis and their involvement in bronchiectasis unrelated to CF. PB - Springer, New York T2 - Lung T1 - Pathophysiology and Genetics of Bronchiectasis Unrelated to Cystic Fibrosis EP - 392 IS - 4 SP - 383 VL - 196 DO - 10.1007/s00408-018-0121-y ER -
@article{ author = "Nikolić, Aleksandra", year = "2018", abstract = "Bronchiectasis is characterized by deregulated inflammatory response and recurrent bacterial infection resulting in progressive lung damage and an irreversible dilatation of bronchi and bronchioles. Generally accepted model of the development of bronchiectasis is the "vicious cycle hypothesis" that proposes compromising of the mucociliary clearance by an initial event, which leads to the infection of the respiratory tract followed by further impairment of mucociliary function, bacterial proliferation, and more inflammation. Bronchiectasis is a very common symptom in patients with cystic fibrosis (CF), while bronchiectasis unrelated to CF is heterogeneous pathology of unknown cause with a large number of potential contributory factors and poorly understood pathogenesis. It is presumed that bronchiectasis unrelated to CF is a multifactorial condition predisposed by genetic factors. Different molecules have been implicated in the onset and development of idiopathic bronchiectasis, as well as modulation of the disease severity and response to therapy. Most of these molecules are involved in the processes that contribute to the homeostasis of the lung tissue, especially mucociliary clearance, protease-antiprotease balance, and immunomodulation. Evaluation of the studies performed towards investigation of the role these molecules play in bronchiectasis identifies genetic variants that may be of potential importance for clinical management of the disease, and also of interest for future research efforts. This review focuses on the molecules with major roles in lung homeostasis and their involvement in bronchiectasis unrelated to CF.", publisher = "Springer, New York", journal = "Lung", title = "Pathophysiology and Genetics of Bronchiectasis Unrelated to Cystic Fibrosis", pages = "392-383", number = "4", volume = "196", doi = "10.1007/s00408-018-0121-y" }
Nikolić, A.. (2018). Pathophysiology and Genetics of Bronchiectasis Unrelated to Cystic Fibrosis. in Lung Springer, New York., 196(4), 383-392. https://doi.org/10.1007/s00408-018-0121-y
Nikolić A. Pathophysiology and Genetics of Bronchiectasis Unrelated to Cystic Fibrosis. in Lung. 2018;196(4):383-392. doi:10.1007/s00408-018-0121-y .
Nikolić, Aleksandra, "Pathophysiology and Genetics of Bronchiectasis Unrelated to Cystic Fibrosis" in Lung, 196, no. 4 (2018):383-392, https://doi.org/10.1007/s00408-018-0121-y . .