Lena Wucherpfennig

Background: In a previous trial, magnetic resonance imaging (MRI) showed stabilisation of structural and functional lung abnormalities in children with cystic fibrosis (CF) after up to 96 weeks of Lumacaftor/Ivacaftor (LUM/IVA) therapy. However, real-world evidence on long-term effects in preschool and school-age children is limited. Methods: Forty-nine children with CF homozygous for F508del (mean age 5.0 ± 3.5years, range 1-12years) underwent chest MRI before (MRI1) and annually up to four years on LUM/IVA (MRI2-5, mean 3.5 ± 1.1 examinations; mean follow-up 2.6 ± 1.1years). Treatment effects were evaluated using the chest MRI score, lung clearance index (LCI) and percent predicted forced expiratory volume in one second (ppFEV1). Results: MRI global and morphology scores significantly decreased from MRI1 to MRI2 (15.9 ± 6.8 vs. 13.7 ± 6.5, p < 0.05; and 11.6 ± 4.8 vs. 10.6 ± 4.8, p < 0.05, respectively), while MRI perfusion score showed a non-significant decrease (4.7 ± 2.7 vs. 3.7 ± 2.6, p = 0.200). From MRI2 through MRI5, MRI global and perfusion scores remained stable ( p = 0.308–0.999), whereas MRI morphology score increased at MRI4 and MRI5 to the level of MRI1 ( p = 0.990 and p = 0.999 vs. MRI1). LCI was stable between MRI1 and MRI2 (9.0 ± 2.4 vs. 8.2 ± 2.2, p = 0.253) and was lower from MRI3 onward compared to MRI1 ( p < 0.05), while ppFEV1 was stable ( p = 0.355–0.999). Improvements in MRI global score between MRI1 and MRI2 correlated with improvements in LCI ( r = 0.42, p < 0.05). Conclusions: Longitudinal chest MRI demonstrates initial improvement and second-year stabilization of morphological and functional lung abnormalities in preschool and school-age children with CF homozygous for F508del under LUM/IVA therapy, with increasing morphological abnormalities toward pre-treatment levels during the third year of therapy.

Background: Previous magnetic resonance imaging (MRI) studies demonstrated a high prevalence of bronchial artery dilatation (BAD) and its association with disease severity in people with cystic fibrosis (pwCF). More recently, dilated bronchial arteries (BA) were shown to decrease under modulator therapy. However, systematic imaging studies on the extent and development of BAD in pwCF are lacking. Methods: 174 pwCF (mean age 27.8±7.9 years) undergoing standardised chest MRI including MR angiography (MRA) were included; 71 pwCF contributed ≥2 MRI. BAD prevalence and number of dilated BA were assessed visually, and disease severity using the chest MRI score. BA were segmented to measure mean lumen diameter (MLD) and mean lumen area (MLA). Percent predicted forced expiratory volume in 1 s (ppFEV1), hemoptysis, and throat swabs were recorded. Results: At baseline, 93% of pwCF had ≥1 dilated BA, 41% had two, and 27% three (mean 2.1±1.0). MLD and MLA were 3.8±0.9 mm and 9.5±7.1 mm ² , and correlated with ppFEV1 and MRI global, morphology, and perfusion scores (r=−0.22–0.34, pP. aeruginosa infection, the number of dilated BA was higher (2.3±0.8 versus 1.9±1.1, p=0.018). Hemoptysis occurred solely in pwCF with BAD (15%) and was associated with higher MLA (10.6±3.6 mm ² versus 9.2±4.4 mm ² , p=0.029) and a higher proportion of tortuous vessels (p=0.046). During follow-up, 16% developed a new dilated BA, associated with an increase in MRI global score (17.2±7.1 versus 20.5±6.6, p=0.001). Conclusions: MRA sensitively detects BAD in pwCF. Higher disease severity is associated with BAD development, and larger size of dilated BA with hemoptysis.

Background: Flat-detector computed tomography (FDCT) is increasingly used for periinterventional cerebral imaging. The recently introduced Sine Spin FDCT (S-FDCT) aims to improve cerebral soft tissue contrast compared with conventional FDCT (C-FDCT). Reliable visualization of healthy brain parenchyma is essential for detecting pathological changes. This study compared gray–white matter differentiation between intraindividually acquired S-FDCT and C-FDCT. Methods: A retrospective analysis of a prospectively maintained database included patients with ischemic stroke treated by mechanical thrombectomy who underwent both S-FDCT and C-FDCT within the same interventional session on a latest-generation angiography system. Quantitative image quality was assessed using the contrast-to-noise ratio (CNR). Qualitative image quality was evaluated using a five-point scale at basal ganglia and supratentorial cortex. Analyses focused on healthy brain parenchyma contralateral to infarction. Radiation dose was assessed using entrance-skin dose and dose-area product (DAP). Results: Forty patients (74.2 ± 15.3 years) were analyzed. S-FDCT demonstrated a higher CNR than C-FDCT (mean CNR ± SD: 2.62 ± 1.36 versus 1.03 ± 0.38; p < 0.001). Qualitative ratings were higher for S-FDCT at the basal ganglia (2.45 ± 0.71 versus 1.54 ± 0.53; p < 0.001) and supratentorial cortex (3.01 ± 0.80 versus 1.71 ± 0.60; p < 0.001). Inter-reader agreement was good (κ = 0.798). S-FDCT showed a moderately higher radiation dose than C-FDCT (DAP: 50.6 ± 3.10 versus 46.89 ± 2.86 Gy·cm², p < 0.001). Conclusion: S-FDCT improves cerebral soft tissue visualization compared with C-FDCT in periinterventional imaging. These findings highlight the potential of Sine Spin technology to enhance FDCT image quality and support its role in advanced angiography-suite–based neuroimaging.

Three-dimensional rotational angiography (3D-RA) is a key modality in endovascular neurointerventions but often provides limited visibility of small, clinically relevant vessels such as perforating arteries. This study aimed to evaluate the performance of a novel, dedicated vessel enhancement software in improving vascular visibility in 3D-RA datasets. In this retrospective analysis of a prospectively maintained institutional database, fifty consecutive patients with unruptured anterior circulation aneurysms underwent standardized 3D-RA acquisitions on the ARTIS icono angiographic system (Siemens Healthineers). Each dataset was reconstructed with and without vessel enhancement. Quantitative analysis comprised contrast-to-noise ratio (CNR) measurements in the M1 segment of the middle cerebral artery and in perforating branches. Qualitative image quality was independently assessed by two experienced readers on a five-point scale. Vessel enhancement yielded consistent improvements across all analyses. CNR increased in the M1 segment (mean CNR ± standard deviation (SD), no vessel enhancement (N-VE): 25.15±5.42 vs. VE: 27.85±5.41, p<0.001) and in perforating arteries (mean CNR ± SD, N-VE: 22.26±4.57 vs. VE: 24.80±4.75, p<0.001). Overall qualitative ratings improved from "very good" to "excellent" diagnostic quality, with higher scores for vessel-enhanced reconstructions compared with unenhanced images (mean score ± SD, N-VE: 3.90±0.65 vs. VE: 4.66±0.52, p<0.001). Improvements were particularly evident for the continuity and visibility of fine vascular structures. Inter-reader agreement was good (κ=0.72), supporting the robustness of the findings. Dedicated vessel enhancement software improves the depiction of both larger arteries and small perforating branches in cerebral 3D-RA. By enabling clearer visualization of fine vascular structures, this approach may strengthen diagnostic confidence and procedural safety in complex endovascular neurointerventions.

Rationale: The progression of lung changes in cystic fibrosis (CF) from infancy through adolescence remains poorly understood as a result of limited longitudinal imaging data. Objectives: To assess changes in lung morphology and perfusion in children with CF through the pediatric age range by longitudinal chest magnetic resonance imaging (MRI). Methods: 1,112 annual chest MRI scans were performed in 226 patients with CF aged 0–18 years. MRI was assessed using a validated MRI scoring system. Results: The MRI global score continuously increased from 5.5 ± 4.6 at infancy (0 yr) to 17.9 ± 8.5 at adolescence (12–18 yr), and the MRI morphology score increased from 5.0 ± 3.9 to 12.3 ± 6.1 (P < 0.001). Bronchiectasis/wall thickening prevalence increased from 89.1% at infancy to approximately 100% at preschool age (1–5 yr), and the subscore increased from 3.1 ± 1.9 at infancy to 6.6 ± 2.1 at adolescence (P < 0.001). Mucus plugging prevalence increased from 55.4% at infancy to 83.0% at adolescence, and the subscore increased from 1.2 ± 1.6 to 3.7 ± 2.6 in the same period (P < 0.001). Perfusion abnormalities were found in 44.4% at infancy, and increased to approximately 90% at preschool age (P < 0.001). The MRI perfusion score increased from 1.1 ± 1.6 at infancy to 5.6 ± 3.0 at adolescence (P < 0.001). Chronic Pseudomonas aeruginosa infection was associated with higher MRI scores at school age (6–11 yr; P < 0.05–0.001). Conclusions: This is the first study to assess longitudinal changes in lung morphology and perfusion in CF throughout the pediatric age range, providing percentiles as age-specific references for lung disease severity. Our data may facilitate the use of MRI as an endpoint in clinical trials in children with CF. Clinical trial registered with www.clinicaltrials.gov (NCT00760071 and NCT02270476).

Rationale: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are characterized by inherited impaired mucociliary clearance leading to chronic progressive lung disease as well as chronic rhinosinusitis (CRS). The diseases share morphological and functional commonalities on magnetic resonance imaging (MRI) of the lungs and paranasal sinuses, but comparative MRI studies are lacking. Objectives: To determine whether PCD shows different associations of pulmonary and paranasal sinus abnormalities on MRI and lung function test results in children (infants to adolescents) compared with children with CF. Methods: Eighteen children with PCD (median age, 9.5 [IQR, 3.4–12.7] yr; range, 0–18 yr) and 36 age-matched CF transmembrane conductance regulator modulator–naive children with CF (median age, 9.4 [3.4–13.2] yr; range, 0–18 yr) underwent same-session chest and paranasal sinus MRI as well as spirometry (to determine forced expiratory volume in 1 s percent predicted) and multiple-breath washout (to determine lung clearance index z-score). Pulmonary and paranasal sinus abnormalities were assessed using previously validated chest MRI and CRS-MRI scoring systems. Results: Mean chest MRI global score was similar in children with PCD and CF (15.0 [13.5–20.8] vs. 15.0 [9.0–15.0]; P = 0.601). Consolidations were more prevalent and severe in children with PCD (56% vs. 25% and 1.0 [0.0–2.8] vs. 0.0 [0.0–0.3], respectively; P < 0.05). The chest MRI global score correlated moderately with forced expiratory volume in 1 second percent predicted in children with PCD and children with CF (r = −0.523 and −0.687; P < 0.01) and with lung clearance index in children with CF (r = 0.650; P < 0.001) but not in PCD (r = 0.353; P = 0.196). CRS-MRI sum score and mucopyocele subscore were lower in children with PCD than in children with CF (27.5 [26.3–32.0] vs. 37.0 [37.8–40.0] and 2.0 [0.0–2.0] vs. 7.5 [4.8–9.0], respectively; P < 0.01). CRS-MRI sum score did not correlate with chest MRI score in PCD (r = 0.075–0.157; P = 0.557–0.788) but correlated moderately with MRI morphology score in CF (r = 0.437; P < 0.01). Conclusions: MRI detects differences in lung and paranasal sinus abnormalities between children with PCD and those with CF. Lung disease does not correlate with CRS in PCD but correlates in CF.

Rationale: Magnetic resonance imaging (MRI) detects improvements in mucus plugging and bronchial wall thickening, but not in lung perfusion in patients with cystic fibrosis (CF) treated with elexacaftor/tezacaftor/ivacaftor (ETI). Objectives: To determine whether bronchial artery dilatation (BAD), a key feature of advanced lung disease, indicates irreversibility of perfusion abnormalities and whether BAD could be reversed in CF patients treated with ETI. Methods: A total of 59 adults with CF underwent longitudinal chest MRI, including magnetic resonance angiography twice, comprising 35 patients with CF (mean age, 31 ± 7 yr) before (MRI1) and after (MRI2) at least 1 month (mean duration, 8 ± 4 mo) on ETI therapy and 24 control patients with CF (mean age, 31 ± 7 yr) without ETI. MRI was assessed using the validated chest MRI score, and the presence and total lumen area of BAD were assessed with commercial software. Results: The MRI global score was stable in the control group from MRI1 to MRI2 (mean difference, 1.1 [−0.3, 2.4]; P = 0.054), but it was reduced in the ETI group (−10.1 [−0.3, 2.4]; P < 0.001). In the control and ETI groups, BAD was present in almost all patients at baseline (95% and 94%, respectively), which did not change at MRI2. The BAD total lumen area did not change in the control group from MRI1 to MRI2 (1.0 mm2 [−0.2, 2.2]; P = 0.099) but decreased in the ETI group (−7.0 mm2 [−8.9, −5.0]; P < 0.001). This decrease correlated with improvements in the MRI global score (r = 0.540; P < 0.001). Conclusions: Our data show that BAD may be partially reversible under ETI therapy in adult patients with CF who have established disease.