Donos Mădălina Andreea, Țarcă Elena, Cojocaru Elena, Țarcă Viorel, Butnariu Lăcrămioara Ionela, Bernic Valentin, Popovici Paula, Roșu Solange Tamara, Tîrnovanu Mihaela Camelia, Ionescu Nicolae Sebastian, Trandafir Laura Mihaela
Pediatrics Department, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania.
Department of Surgery II, Pediatric and Orthopedic Surgery, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania.
Diagnostics (Basel). 2025 Aug 2;15(15):1940. doi: 10.3390/diagnostics15151940.
Cystic fibrosis (CF) is a genetic condition affecting several organs and systems, including the pancreas, colon, respiratory system, and reproductive system. The detection of a growing number of variants and genotypes has contributed to an increase in the CF population which, in turn, has had an impact on the overall statistics regarding the prognosis and outcome of the condition. Given the increase in life expectancy, it is critical to better predict outcomes and prognosticate in CF. Thus, each person's choice to aggressively treat specific disease components can be more appropriate and tailored, further increasing survival. of our narrative review is to summarize the most recent information concerning the value and significance of clinical parameters in predicting outcomes, such as gender, diabetes, liver and pancreatic status, lung function, radiography, bacteriology, and blood and sputum biomarkers of inflammation and disease, and how variations in these parameters affect prognosis from the prenatal stage to maturity. : A methodological search of the available data was performed with regard to prognostic factors in the evolution of CF in children and young adults. We evaluated articles from the PubMed academic search engine using the following search terms: prognostic factors AND children AND cystic fibrosis OR mucoviscidosis. We found that it is crucial to customize CF patients' care based on their unique clinical and biological parameters, genetics, and related comorbidities. : The predictive significance of more dynamic clinical condition markers provides more realistic future objectives to center treatment and targets for each patient. Over the past ten years, improvements in care, diagnostics, and treatment have impacted the prognosis for CF. Although genotyping offers a way to categorize CF to direct research and treatment, it is crucial to understand that a variety of other factors, such as epigenetics, genetic modifiers, environmental factors, and socioeconomic status, can affect CF outcomes. The long-term management of this complicated multisystem condition has been made easier for patients, their families, and physicians by earlier and more accurate identification techniques, evidence-based research, and centralized expert multidisciplinary care.
囊性纤维化(CF)是一种影响多个器官和系统的遗传性疾病,包括胰腺、结肠、呼吸系统和生殖系统。越来越多的变异体和基因型被检测出来,这导致了CF患者数量的增加,进而对该疾病的预后和转归的总体统计数据产生了影响。鉴于预期寿命的延长,更好地预测CF的转归和预后至关重要。因此,每个人积极治疗特定疾病成分的选择可以更加恰当且具有针对性,从而进一步提高生存率。我们叙述性综述的目的是总结有关临床参数在预测转归方面的价值和意义的最新信息,这些参数包括性别、糖尿病、肝脏和胰腺状况、肺功能、影像学、细菌学以及炎症和疾病的血液及痰液生物标志物,以及这些参数的变化如何影响从产前阶段到成年期的预后。方法:针对儿童和青年CF病情发展中的预后因素,对现有数据进行了方法学检索。我们使用以下检索词在PubMed学术搜索引擎中评估文章:预后因素、儿童、囊性纤维化或黏液黏稠病。我们发现,根据CF患者独特的临床和生物学参数、遗传学及相关合并症来定制护理至关重要。结果:更具动态性的临床状况标志物的预测意义为针对每位患者的治疗和目标提供了更现实的未来目标。在过去十年中,护理、诊断和治疗方面的改善对CF的预后产生了影响。尽管基因分型为CF的分类提供了一种指导研究和治疗的方法,但必须明白,多种其他因素,如表观遗传学、基因修饰因子、环境因素和社会经济地位,都可能影响CF的转归。通过更早、更准确的识别技术、循证研究以及集中的专家多学科护理,这种复杂的多系统疾病的长期管理对于患者、其家人和医生来说变得更加容易。