Kraus Mareen Sarah, Teufel Matthias, Esser Michael, Kiefer Lena Sophie, Fleischer Sabrina, Graepler-Mainka Ute, Hector Andreas, Tsiflikas Ilias, Schaefer Jürgen F
Radiology, Eberhard-Karls-University Tübingen, Tübingen, Germany.
Community practice for Diagnostic Radiology and Nuclear Medicine, Klinikum Schwäbisch Gmünd, Mutlangen, Germany.
Rofo. 2020 Jun;192(6):567-575. doi: 10.1055/a-1088-3537. Epub 2020 Feb 6.
In cystic fibrosis (CF) the phenotypic expression of complaints varies widely. Genotypes with sufficient pancreatic function (PS) exhibit milder lung disease compared to CF patients with insufficient pancreatic function (PI). The purpose of this study was to evaluate structural lung disease (SLD) in CF patients with differing pancreatic status but similar results on pulmonary function testing using a pulmonary magnetic resonance imaging score (MR-CF score).
In this retrospective study, 20 patients in our single-center CF database were included: 10 with PS (mean age 12.5 years; six male; BMI 17.4 kg/m; FeV 102 %) were matched by gender, age and lung function with 10 PI patients. Experienced observers semi-quantitatively assessed SLD for each lung lobe. The established MR-CF score measures the extent and the severity of bronchiectasis and bronchial wall thickening, mucus plugging, centrilobular opacity, consolidation, sacculation, and air trapping. The total score and sub-score values were compared to the pancreatic status.
Patients with CF-PS had overall statistically significant lower MR-CF scores (p = 0.024), and therefore milder SLD, compared to CF-PI. The differences were most significant for bronchiectasis (p = 0.0042) and air trapping (p = 0.0304). SLD was more severe in the upper lobes in all patients. However, differences between CF-PS and CF-PI patients were present in both the upper and lower lung areas (p = 0.0247 and p = 0.0196, respectively).
Our results demonstrated that CF patients with impaired pancreatic function show more severe lung pathology detected by MRI, especially bronchiectasis and air trapping.
· Pulmonary MRI offers morphological and functional details without using ionizing radiation. · CF patients with pancreatic insufficiency show more severe pulmonary structural impairment. · Bronchiectasis and air trapping are the most common structural lung changes with predominance in the upper lung lobes..
· Kraus MS, Teufel M, Esser M et al. Differing Pulmonary Structural Abnormalities Detected on Pulmonary MR Imaging in Cystic Fibrosis Patients with Varying Pancreatic Function. Fortschr Röntgenstr 2020; 192: 567 - 575.
在囊性纤维化（CF）中，症状的表型表达差异很大。与胰腺功能不全（PI）的CF患者相比，具有足够胰腺功能（PS）的基因型患者的肺部疾病较轻。本研究的目的是使用肺部磁共振成像评分（MR-CF评分）评估胰腺状态不同但肺功能测试结果相似的CF患者的结构性肺病（SLD）。
在这项回顾性研究中，纳入了我们单中心CF数据库中的20名患者：10名PS患者（平均年龄12.5岁；6名男性；BMI 17.4kg/m²；FEV1 102%）在性别、年龄和肺功能方面与10名PI患者相匹配。经验丰富的观察者对每个肺叶的SLD进行半定量评估。既定的MR-CF评分用于测量支气管扩张、支气管壁增厚、黏液嵌塞、小叶中心性混浊、实变、囊状扩张和气陷（空气潴留）的程度和严重程度。将总分和子分值与胰腺状态进行比较。
与CF-PI患者相比，CF-PS患者的MR-CF总分在统计学上显著更低（p = 0.024），因此SLD更轻。支气管扩张（p = 0.0042）和气陷（p = 0.0304）的差异最为显著。所有患者的上叶SLD更严重。然而，CF-PS和CF-PI患者在上肺区和下肺区均存在差异（分别为p = 0.0247和p = 0.0196）。
我们的结果表明，胰腺功能受损的CF患者通过MRI检测出的肺部病理改变更严重，尤其是支气管扩张和气陷。
· 肺部MRI无需使用电离辐射即可提供形态和功能细节。· 胰腺功能不全的CF患者肺部结构损伤更严重。· 支气管扩张和气陷是最常见的肺部结构改变，以上肺叶为主。
· Kraus MS, Teufel M, Esser M等。不同胰腺功能的囊性纤维化患者肺部磁共振成像检测到的不同肺部结构异常。Fortschr Röntgenstr 2020; 192: 567 - 575。
