Korrelation verschiedener radiologischer Untersuchungsverfahren und Verfahren der Lungenfunktionsuntersuchung bei Patienten mit zystischer Fibrose

Kratzer, Vivien Laura (2024): Korrelation verschiedener radiologischer Untersuchungsverfahren und Verfahren der Lungenfunktionsuntersuchung bei Patienten mit zystischer Fibrose. Dissertation, LMU München: Medizinische Fakultät

Abstract

Abstract: Cross-sectional study Cystic fibrosis (CF) is the most common life-threatening autosomal recessive disease among the Caucasian population with the first pulmonary damages already occurring in early childhood. There is a very diverse course of disease during childhood and adolescence due to a varying degree of therapy adherence in that group. Therefore, a high variability of disease severity must be expected when treating CF patients in early adulthood. To assess the actual, this study aims to evaluate the state of disease of young adults suffering from CF based on high-resolution computed tomography (HRCT) -examinations of the lungs and pulmonary function tests (PFTs) through a cross-sectional study. The study used a dataset from 1998 to 2018 of ninety-one patients from age sixteen to twenty-two and was conducted in two steps. First, HRCT-examinations of the lungs were retrospectively evaluated by two observers (O1 and O2) using the Brody score to measure the degree of pulmonary damage. Second, the Brody score was correlated with the three spirometry parameters vital capacity (VC), forced expiratory volume in one second (FEV1), and the Tiffeneau index, as well as with the body-mass-index (BMI) and the genetic mutation. Overall, the severity of pulmonary damages exhibited a wide range (Brody score ranging from 0 to 144 (O1) / 175 (O2)), with a high interobserver reproducibility (rs=0.69). The associated PFTs presented a heterogeneous distribution as well. The Brody score showed an excellent correlation on a statistically highly significant level (p<0.001) with the three spirometry parameters VC (rs=-0.75 (O1); rs=-0.60 (O2)), FEV1 (rs=-0.81 (O1); rs=-0.69 (O2)), and the Tiffeneau index (rs=0.68 (O1); rs=0.67 (O2)), as well as with the BMI (rs=-0.76 (O1); rs=-0.67 (O2)). There was no statistically significant difference between male and female CF patients. A correlation between the Brody score and the genetic mutation status could not be found. A promising avenue for future research would be analysing the causes of these vast differences in the state of disease, like, the influence of genetic mutations or the patient's compliance during childhood and teenage age. Abstract: Cohort study Cystic fibrosis (CF) is the most common life-threatening autosomal recessive disease among the Caucasian population. As there is still no cure for CF, it is essential to prevent or at least delay pulmonary damage to conserve lung function. This aim can be achieved through regular monitoring using high-resolution computed tomography (HRCT) -examinations and pulmonary function tests (PFTs) to be able to intervene, if necessary, as soon as possible. Strict monitoring is especially essential in the case of young adults while getting more independent from their parents since they tend to have less disease knowledge than their parents. This often results in less therapy adherence which negatively influences the course of disease. To evaluate whether there had been any changes in pulmonary damage within the first four years in CF patients after reaching adulthood, a retrospective cohort study was conducted. The study used a dataset of twenty-nine patients having an HRCT-examination at the age of sixteen to twenty-two with a follow-up HRCT-examination after approximately four years and was conducted in three steps. First, the degree of pulmonary damage for the initial and the follow-up HRCT-examinations of the lungs was retrospectively evaluated by two observers (O1 and O2) using the Brody score. Second, the initial Brody score was correlated with the difference between the initial and the follow-up scores. Third, the associated spirometry parameters of the initial and the follow-up examinations were compared and correlated with the Brody score. The results revealed a dependency between the magnitude of change in pulmonary damages within the four years and their initial state; the fewer pulmonary damages in the initial HRCT-examination, the bigger the increase of the pulmonary damages within the four-year period (rs=-0.60 (O1) / rs=-0.39 (O2)). This increase can be mainly ascribed to the sub-scores bronchiectasis, mucous plugging, and peribronchial thickening. Reduction of pulmonary damages was exclusively found in patients with a medium or high Brody score at the initial HRCT-examination. The spirometry parameters did not show a statistically significant change over the four years. There was no statistically significant correlation between the changes in the Brody score and the changes of the spirometry parameters (p>0.05). Statistically significant differences in the course of disease between male and female and ∆-F-508-homozygous and heterozygous CF patients could not be found. Future research should focus on analysing the impact of different monitoring procedures between CF patients with initially low and high-grade disease severity.

• Dokumententyp: Dissertationen (Dissertation, LMU München)
• Keywords: Cystic Fibrosis, Pulmonary Damage, Brody Score, HRCT, Pulmonary Function Test
• Themengebiete: 600 Technik, Medizin, angewandte Wissenschaften ; 600 Technik, Medizin, angewandte Wissenschaften > 610 Medizin und Gesundheit
• Fakultäten: Medizinische Fakultät
• Sprache der Hochschulschrift: Englisch
• Datum der mündlichen Prüfung: 14. November 2024
• 1. Bericht­erstatter:in: Müller-Lisse, Ullrich