Impact of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Children with Cystic Fibrosis and One or Two F508del Alleles.

BACKGROUND: We recently demonstrated that elexacaftor/tezacaftor/ivacaftor (ETI) improves the lung clearance index (LCI) and abnormalities in lung morphology detected by magnetic resonance imaging (MRI) in adolescent and adult patients with cystic fibrosis (CF). However, real-world data on the effect of ETI on these sensitive outcomes of lung structure and function in school-age children with CF have not been reported. The aim of this study was therefore to examine the effect of ETI on the LCI and the lung MRI score in children with CF and one or two F508del alleles aged 6 to 11 years. METHODS: This prospective, observational, multicenter, post-approval study assessed the longitudinal LCI up to 12 months and the lung MRI score before and three months after initiation of ETI. RESULTS: A total of 107 children with CF including 40 heterozygous for F508del and a minimal function mutation (F/MF) and 67 homozygous for F508del (F/F) were enrolled in this study. Treatment with ETI improved the LCI in F/MF children (-1.0; IQR, -2.0 to -0.1; p<0.01) and F/F children (-0.8; IQR, -1.9 to -0.2; p<0.001) from 3 months onwards. Further, ETI improved the MRI global score in F/MF (-4.0; IQR, -9.0 to 0.0; p<0.01) and F/F children (-3.5; IQR, -7.3 to -0.8; p<0.001). CONCLUSIONS: ETI improves early abnormalities in lung ventilation and morphology in school-age children with CF and at least one F508del alleles in a real-world setting. Our results support early initiation of ETI to reduce or even prevent lung disease progression in school-age children with CF.

Effect of CFTR modulator therapy with elexacaftor/tezacaftor/ivacaftor on pulmonary ventilation derived by 3D phase-resolved functional lung MRI in cystic fibrosis patients.

OBJECTIVES: To investigate whether 3D phase-resolved functional lung (PREFUL)-MRI parameters are suitable to measure response to elexacaftor/tezacaftor/ivacaftor (ETI) therapy and their association with clinical outcomes in cystic fibrosis (CF) patients. METHODS: Twenty-three patients with CF (mean age: 21; age range: 14-46) underwent MRI examination at baseline and 8-16 weeks after initiation of ETI. Morphological and 3D PREFUL scans assessed pulmonary ventilation. Morphological images were evaluated using a semi-quantitative scoring system, and 3D PREFUL scans were evaluated by ventilation defect percentage (VDP) values derived from regional ventilation (RVent) and cross-correlation maps. Improved ventilation volume (IVV) normalized to body surface area (BSA) between baseline and post-treatment visit was computed. Forced expiratory volume in 1 second (FEV1) and mid-expiratory flow at 25% of forced vital capacity (MEF25), as well as lung clearance index (LCI), were assessed. Treatment effects were analyzed using paired Wilcoxon signed-rank tests. Treatment changes and post-treatment agreement between 3D PREFUL and clinical parameters were evaluated by Spearman's correlation. RESULTS: After ETI therapy, all 3D PREFUL ventilation markers (all p < 0.0056) improved significantly, except for the mean RVent parameter. The BSA normalized IVVRVent was significantly correlated to relative treatment changes of MEF25 and mucus plugging score (all |r| > 0.48, all p < 0.0219). In post-treatment analyses, 3D PREFUL VDP values significantly correlated with spirometry, LCI, MRI global, morphology, and perfusion scores (all |r| > 0.44, all p < 0.0348). CONCLUSIONS: 3D PREFUL MRI is a very promising tool to monitor CFTR modulator-induced regional dynamic ventilation changes in CF patients. CLINICAL RELEVANCE STATEMENT: 3D PREFUL MRI is sensitive to monitor CFTR modulator-induced regional ventilation changes in CF patients. Improved ventilation volume correlates with the relative change of mucus plugging, suggesting that reduced endobronchial mucus is predominantly responsible for regional ventilation improvement. KEY POINTS: • 3D PREFUL MRI-derived ventilation maps show significantly reduced ventilation defects in CF patients after ETI therapy. • Significant post-treatment correlations of 3D PREFUL ventilation measures especially with LCI, FEV1 %pred, and global MRI score suggest that 3D PREFUL MRI is sensitive to measure improved regional ventilation of the lung parenchyma due to reduced inflammation induced by ETI therapy in CF patients. • 3D PREFUL MRI-derived improved ventilation volume (IVV) correlated with MRI mucus plugging score changes suggesting that reduced endobronchial mucus is predominantly responsible for regional ventilation improvement 8-16 weeks after ETI therapy.

Relapsing Polychondritis with Tracheobronchial Involvement: A Detailed Description of Two Pediatric Cases and Review of the Literature.

Relapsing polychondritis (RP) is a rare immune-mediated disease that primarily affects the cartilaginous structures of the ears, nose and airways. The clinical spectrum ranges from mild to severe disease characterized by progressive destruction of cartilage in the tracheobronchial tree leading to airway obstruction and acute respiratory failure. Early diagnosis is crucial to prevent irreversible airway damage and life-threatening complications. Due to its rarity and variability of symptoms, the diagnosis of RP is often delayed particularly in childhood. To address this and increase awareness of this rare disease, we present a detailed case report of two adolescent females affected by RP. We aim to describe the clinical findings, consequences of a delayed diagnosis and provide a review of the current literature.

European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (1) pre-transplant evaluation.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment in end-stage liver disease. Imaging is a key element for successful organ-transplantation to assist surgical planning. So far, only limited data regarding the best radiological approach to prepare children for liver transplantation is available. OBJECTIVES: In an attempt to harmonize imaging surrounding pediatric liver transplantation, the European Society of Pediatric Radiology (ESPR) Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phase. This paper reports the responses on preoperative imaging. MATERIAL AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted and 22 institutions from 11 countries returned the survey. From 2018 to 2020, the participating centers collectively conducted 1,524 transplantations, with a median of 20 transplantations per center per annum (range, 8-60). RESULTS: Most sites (64%) consider ultrasound their preferred modality to define anatomy and to plan surgery in children before liver transplantation, and additional cross-sectional imaging is only used to answer specific questions (computed tomography [CT], 90.9%; magnetic resonance imaging [MRI], 54.5%). One-third of centers (31.8%) rely primarily on CT for pre-transplant evaluation. Imaging protocols differed substantially regarding applied CT scan ranges, number of contrast phases (range 1-4 phases), and applied MRI techniques. CONCLUSION: Diagnostic imaging is generally used in the work-up of children before liver transplantation. Substantial differences were noted regarding choice of modalities and protocols. We have identified starting points for future optimization and harmonization of the imaging approach to multicenter studies.

European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (2) intraoperative imaging.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of intraoperative and postoperative complications. So far, only limited data regarding the best radiological approach to monitor children during liver transplantation is available. OBJECTIVE: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra- and postoperative phase. This paper reports the responses related to intraoperative imaging. MATERIALS AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey. RESULTS: Intraoperative ultrasound (US) is used by all sites to assess the quality of the vascular anastomosis in order to ensure optimal perfusion of the liver transplant. Vessel depiction is commonly achieved using color Doppler (95.3%). Additional US-based techniques are employed by fewer centers (power angio mode, 28.6%; B-flow, 19%; contrast-enhanced US, 14.3%). Most centers prefer a collaborative approach, with surgeons responsible for probe handling, while radiologists operate the US machine (47.6%). Less commonly, the intraoperative US is performed by the surgeon alone (28.6%) or by the radiologist alone (23.8%). Timing of US, imaging frequency, and documentation practices vary among centers. CONCLUSION: Intraoperative US is consistently utilized across all sites during pediatric liver transplantation. However, considerable variations were observed in terms of the US setup, technique preferences, timing of controls, and documentation practices. These differences provide valuable insights for future optimization and harmonization studies.

European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (3) postoperative imaging.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of postoperative complications. So far, limited data is available regarding the best radiologic approach to monitor children after liver transplantation. OBJECTIVE: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phases. This paper reports the responses related to postoperative imaging. MATERIALS AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey. RESULTS: All sites commence ultrasound (US) monitoring within 24 h after liver transplantation. Monitoring frequency varies across sites, ranging from every 8 h to 72 h in early, and from daily to sporadic use in late postoperative phases. Predefined US protocols are used by 73% of sites. This commonly includes gray scale, color Doppler, and quantitative flow assessment. Alternative flow imaging techniques, contrast-enhanced US, and elastography are applied at 31.8%, 18.2%, and 63.6% of sites, respectively. Computed tomography is performed at 86.4% of sites when clarification is needed. Magnetic resonance imaging is used for selected cases at 36.4% of sites, mainly for assessment of biliary abnormalities or when blood tests are abnormal. CONCLUSION: Diagnostic imaging is extensively used for postoperative surveillance of children after liver transplantation. While US is generally prioritized, substantial differences were noted in US protocol, timing, and monitoring frequency. The study highlights potential areas for future optimization and standardization of imaging, essential for conducting multicenter studies.

The influence of MRI-based pelvimetric measurements in mother's choice of delivery in fetal breech position.

INTRODUCTION: At term, about 3-4% of all singleton pregnancies present as breech. MRI-based pelvimetry is a valuable tool to support selection of adequate candidates for a trial-of-labor in women expecting term breech babies. Shared decision-making is playing an increasingly important role in obstetrics. Since the divergent existing knowledge of breech term delivery needs to be discussed with the pregnant woman, we examined the influence of MRI results on the shared decision-making process in women with term breech presentation. METHODS: Between 08/2021 and 12/2022, anamnestic and clinical parameters were collected from singleton pregnancies expecting term breech babies resulting in birth at the Hanover Medical School. After information, written consent and inclusion, clinical parameters, the course of birth and the maternal and fetal outcome were collected retrospectively. 32 women participated in a postpartum questionnaire study on inquiry. The subsequent acquisition of information and the arguments in the decision-making process were determined. In addition, the sense of security and self-determination was asked both before and during birth. RESULTS: 50% of the respondents had not decided for a mode of delivery before having MRI pelvimetry. After imaging and information, about the own pelvic dimensions and predictors for a successful vaginal birth, 80% of this subgroup decided to give birth vaginally. Over 40% of the collective descripted that they made a decision based on the result of MRI pelvimetry. None of the women felt to be insecure after having talked about the MRI results. The elective cesarean section group and the group of those who delivered vaginally were approximately equally highly satisfied with their feeling of self-determination of the birth mode. Overall, the study population had a very positive birth experience. The group of women who had delivered by elective cesarean showed a wider range in their assessment and appeared to perceive the experience more negative than the group of women who had a vaginal birth or emergency cesarean. Fetal and maternal outcomes did not differ between the groups. DISCUSSION: MRT pelvimetry measurements can be used as a predictor for a successful vaginal breech delivery. The additional information obtained from the MRI measurements can be used in the shared decision-making process to decide more easily on the mode of delivery while improving women's awareness and safety. A balanced education on rare and frequently adverse events of vaginal delivery and cesarean section and patient expectations about labor processes must be taken into account.

Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles.

Rationale: We recently demonstrated that triple-combination CFTR (cystic fibrosis transmembrane conductance regulator) modulator therapy with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) improves CFTR function in airway and intestinal epithelia to 40-50% of normal in patients with cystic fibrosis (CF) with one or two F508del alleles. In previous studies, this improvement of CFTR function was shown to improve clinical outcomes; however, effects on the lung clearance index (LCI) determined by multiple-breath washout and abnormalities in lung morphology and perfusion detected by magnetic resonance imaging (MRI) have not been studied. Objectives: To examine the effect of ELX/TEZ/IVA on LCI and lung MRI scores in patients with CF and one or two F508del alleles aged ⩾12 years. Methods: This prospective, observational, multicenter, postapproval study assessed LCI and lung MRI scores before and 8-16 weeks after initiation of ELX/TEZ/IVA. Measurements and Main Results: A total of 91 patients with CF, including 45 heterozygous for F508del and a minimal function mutation (MF) and 46 homozygous for F508del, were enrolled in this study. Treatment with ELX/TEZ/IVA improved LCI in F508del/MF (-2.4; interquartile range [IQR], -3.7 to -1.1; P < 0.001) and F508del homozygous (-1.4; IQR, -2.4 to -0.4; P < 0.001) patients. Furthermore, ELX/TEZ/IVA improved the MRI global score in F508del/MF (-6.0; IQR, -11.0 to -1.3; P < 0.001) and F508del homozygous (-6.5; IQR, -11.0 to -1.3; P < 0.001) patients. Conclusions: Our data demonstrate that improvement of CFTR function by ELX/TEZ/IVA improves lung ventilation and abnormalities in lung morphology, including airway mucus plugging and wall thickening, in adolescent and adult patients with CF and one or two F508del alleles in a real-world, postapproval setting. Clinical trial registered with www.clinicaltrials.gov (NCT04732910).

[Is JAK inhibition an option in the treatment of interstitial lung disease in rheumatoid arthritis?] / Ist JAK-Hemmung eine Option in der Behandlung der interstitiellen Lungenerkrankung bei einer rheumatoiden Arthritis?

A 69-year-old male patient with seropositive erosive rheumatoid arthritis (RA) presented to our clinic due to progressive dyspnea. High-resolution computed tomography (HRCT) and immunological bronchioalveolar lavage revealed ground-glass opacities and a lymphocytic alveolitis caused by interstitial lung disease (ILD) in RA. Considering previous forms of treatment, disease-modifying antirheumatic drug (DMARD) treatment was switched to tofacitinib. Tofacitinib treatment demonstrated a 33% reduction in ground-glass opacities by artificial intelligence-based quantification of pulmonary HRCT over the course of 6 months, which was associated with an improvement in dyspnea symptoms. In conclusion, tofacitinib represents an effective anti-inflammatory therapeutic option in the treatment of RA-ILD.

Ultrashort echo time MRI of the lung in children and adolescents: comparison with non-enhanced computed tomography and standard post-contrast T1w MRI sequences.

OBJECTIVES: To compare the diagnostic value of ultrashort echo time (UTE) magnetic resonance imaging (MRI) for the lung versus the gold standard computed tomography (CT) and two T1-weighted MRI sequences in children. METHODS: Twenty-three patients with proven oncologic disease (14 male, 9 female; mean age 9.0 + / - 5.4 years) received 35 low-dose CT and MRI examinations of the lung. The MRI protocol (1.5-T) included the following post-contrast sequences: two-dimensional (2D) incoherent gradient echo (GRE; acquisition with breath-hold), 3D volume interpolated GRE (breath-hold), and 3D high-resolution radial UTE sequences (performed during free-breathing). Images were evaluated by considering image quality as well as distinct diagnosis of pulmonary nodules and parenchymal areal opacities with consideration of sizes and characterisations. RESULTS: The UTE technique showed significantly higher overall image quality, better sharpness, and fewer artefacts than both other sequences. On CT, 110 pulmonary nodules with a mean diameter of 4.9 + / - 2.9 mm were detected. UTE imaging resulted in a significantly higher detection rate compared to both other sequences (p < 0.01): 76.4% (84 of 110 nodules) for UTE versus 60.9% (67 of 110) for incoherent GRE and 62.7% (69 of 110) for volume interpolated GRE sequences. The detection of parenchymal areal opacities by the UTE technique was also significantly higher with a rate of 93.3% (42 of 45 opacities) versus 77.8% (35 of 45) for 2D GRE and 80.0% (36 of 45) for 3D GRE sequences (p < 0.05). CONCLUSION: The UTE technique for lung MRI is favourable in children with generally high diagnostic performance compared to standard T1-weighted sequences as well as CT. Key Points • Due to the possible acquisition during free-breathing of the patients, the UTE MRI sequence for the lung is favourable in children. • The UTE technique reaches higher overall image quality, better sharpness, and lower artefacts, but not higher contrast compared to standard post-contrast T1-weighted sequences. • In comparison to the gold standard chest CT, the detection rate of small pulmonary nodules small nodules ≤ 4 mm and subtle parenchymal areal opacities is higher with the UTE imaging than standard T1-weighted sequences.

Bronchial artery diameter in massive hemoptysis in cystic fibrosis.

BACKGROUND: Massive hemoptysis is a rare but potentially life-threatening condition of patients with cystic fibrosis (CF) and advanced pulmonary disease. Hypertrophied bronchial arteries are understood to cause massive hemoptysis when rupturing. Risk factors to predict massive hemoptysis are scarce and bronchial artery diameters are not part of any scoring system in follow-up of patients with CF. Aim of this study was to correlate bronchial artery diameter with massive hemoptysis in CF. METHODS: Bronchial artery and non-bronchial systemic artery diameters were measured in contrast enhanced computed tomography (CT) scans in patients with massive hemoptysis and compared to patients with end-stage CF and no history of hemoptysis. Demographic and clinical data and side of bronchial artery/non-bronchial systemic artery hypertrophy and coil embolization were documented. RESULTS: In this retrospective multicenter study 33 patients with massive hemoptysis were included for bronchial artery/non-bronchial systemic artery diameter measurements, (13 female, 20 male, median age 30 years (18-55)). Bronchial artery diameters were significantly larger in the case group than in the control group with median 4 mm (2.2-8.2 mm), and median 3 mm (1-7 mm), respectively (p = 0.002). Sensitivity of bronchial arteries ≥ 3.5 mm to be associated with hemoptysis was 0.76 and specificity 0.71 with ROC creating an area under the curve of 0.719. If non-bronchial systemic arteries were present, they were considered culprit and embolized in 92% of cases. CONCLUSION: Bronchial arteries ≥ 3.5 mm and presence of hypertrophied non-bronchial systemic arteries correlate with massive hemoptysis in patients with CF and might serve as risk predictor for massive hemoptysis. Therefore, in patients with advanced CF we propose CT scans to be carried out as CT angiography to search for bronchial arteries ≥ 3.5 mm and for hypertrophied non-bronchial systemic arteries as possible risk factors for massive hemoptysis.

The First 4 Years - Outcome of Children Identified by Newborn Screening for CF in Germany.

BACKGROUND: Newborn screening (NBS) has been shown to improve cystic fibrosis (CF) disease course and has been widely implemented worldwide. This monocentric study compared children diagnosed by NBS vs. a cohort preceding the implementation of NBS in Germany in 2016 to evaluate ascribed benefits of NBS. METHODS: We compared all children with confirmed CF diagnosis (n=19, "NBS group") out of all children presenting with positive NBS at our center after implementation of NBS (n=100) to children diagnosed with CF at our center within 4 years before NBS implementation (n=29, "pre-NBS group") for outcomes of anthropometry, gastrointestinal and pulmonary disease manifestations and respiratory microbiology. RESULTS: Children diagnosed by NBS had a lower incidence of initial difficulty to thrive (15 vs. 41%) and showed higher mean z-scores for Body-Mass-Index (BMI), weight and length at diagnosis and during study period. Children in the pre-NBS group displayed higher proportions of oxygen-dependent pulmonary exacerbations (10 vs. 0%). They show a significantly lower amount of normal bacterial flora (p=0.005) along with a significantly higher number of throat swab cultures positive for Pseudomonas aeruginosa (p=0.0154) in the first year of life. Yet, pulmonary imaging did not reveal less pulmonary morbidity in the NBS group. CONCLUSIONS: Our results confirm that NBS for CF leads to earlier diagnosis and improves nutritional outcomes in early childhood. Although trajectories of structural lung damage at early age were unaffected by NBS, NBS positive CF patients at preschool age displayed less pulmonary exacerbations and pathological bacteria in throat swabs.

[Chest X-rays in children and adolescents : Indications and limitations]. / Röntgenuntersuchungen des Thorax bei Kindern und Jugendlichen : Indikationen und Limitationen.

CLINICAL ISSUE: Chest X­ray is the most commonly performed X­ray examination in children and adolescents. The aim of this review is to present the benefit of this radiologic modality, but also its limitations. METHODS: Compared with older children, most X­ray examinations of the chest were performed in newborns. After the neonatal period, this review focusses on the diagnosis of inflammatory pulmonary changes, foreign body aspiration, detection of pulmonary nodules, and cystic fibrosis. METHODOLOGICAL INNOVATIONS: The radiation exposure of X­ray examinations is continuously decreasing due to technical innovations. However, other imaging modalities were also continuously being optimized; therefore, alternatives without radiation exposure, i.e., magnetic resonance imaging [MRI] and ultrasound, should be considered in case of specific clinical indications. PRACTICAL RECOMMENDATION: Even if the diagnostic performance of chest X­ray examinations is often minor compared to computed tomography or MRI, chest X­ray still has a high value in children and adolescents, due to its ubiquitous availability and the relatively simple acquisition.

Liver Tumours in Children: The Hepatologist's View.

ABSTRACT: Diagnostic and therapeutic innovations have changed the way we now approach liver tumours in children and adolescents. Novel imaging tools, increasing awareness, and surveillance has led to early diagnosis of benign and malignant liver tumours. Multidisciplinary interventions have favourably altered the natural course in some liver tumours. The role of liver transplantation is expanding and has become fully integrated into today's therapeutic algorithms. Transarterial locoregional and ablation therapies have been successful in adults and are being explored in children to facilitate resectability and improve outcome. For the first time, North American, Japanese, and European experts have designed a global trial to optimize management of malignant liver tumours and aim to find signature molecular profiles that will translate to individualised treatment strategies.This article aims to offer an overview of recent advances in our understanding of liver tumours in children. It focuses on the paediatric hepatologist's view and their role in the multidisciplinary management of benign and malignant liver tumours.

Improvement of image quality and diagnostic confidence using Smart MAR - a projection-based CT protocol in patients with orthopedic metallic implants in hip, spine, and shoulder.

BACKGROUND: In computed tomography (CT) scans, artifacts caused by metallic orthopedic implants still hamper the visualization of important, periprosthetic tissues. Smart MAR metal artifact reduction tool is a promising three-stage, projection-based, post-processing algorithm. PURPOSE: To determine whether the Smart MAR tool improves subjective and objective image quality and diagnostic confidence in patients with orthopedic implants of the hip, spine, and shoulder. MATERIAL AND METHODS: Seventy-two patients with orthopedic screws, hip/shoulder replacement, or spine spondylodesis were included. CT scans were performed on a single-source multislice CT scanner, raw data were post-processed using Smart MAR. Image quality was evaluated both quantitatively (ROI-based) and qualitatively (rater-based) and compared to iterative reconstructions (ASIR V). As comparative standard for artificial prosthetic breaks or loosening, follow-up examinations were used. RESULTS: Smart MAR reconstructions of the hip (n = 23), spine (n = 26), and shoulder (n = 23) showed a significantly reduced attenuation and noise of regions adjacent to metallic implants (P<0.002). Subjective image quality (P<0.005, shoulder P = 0.038/P = 0.046) and overall diagnostic confidence were higher in Smart MAR (all regions P<0.002). Signal-to-noise ratio (SNR; P = 0.72/P = 0.96) was not improved. Compared to standard ASIR V new, artificial metal extinctions (up to 50%) or periprosthetic hem lines (48%-73%) were introduced by Smart MAR. CONCLUSION: Smart MAR improved image quality of the hip, spine, and shoulder CT scans resulting in higher diagnostic confidence in evaluation of periprosthetic soft tissues. As shown for spine implants, it should be used with caution and as a complementary tool for evaluation of periprosthetic loosening or integrity of metal implant, as in many cases it introduced new artifacts.

[Abdominal imaging in cystic fibrosis]. / Abdomenbildgebung bei zystischer Fibrose.

CLINICAL ISSUE: Abdominal complications are often the first indications for cystic fibrosis (CF), a multiorgan disease. A broad range of abdominal manifestations are associated with the disease, including gastrointestinal abnormalities (such as meconium ileus in newborns and distal intestinal obstruction syndrome in older children) and hepatobiliary alterations (e.g., cholelithiasis, microgallbladder, hepatosteatosis, biliary cirrhosis). A characteristic finding is pancreatic involvement, which leads to exocrine and over the course of time to endocrine insufficiency. STANDARD RADIOLOGICAL METHODS: Ultrasonography is the preferred and often sole modality for a precise diagnosis of abdominal CF manifestations. However, all imaging modalities can be used, depending on the pathology: X­ray, fluoroscopic examinations, computed tomography, magnetic resonance imaging (also with application of magnetic resonance cholangiopancreatography). METHODICAL INNOVATIONS/PERFORMANCE: Scoring systems are useful for standardized diagnostics. Sonographic findings, described using a scoring system, correlate with clinical symptoms, such as pancreatic lipomatosis with abdominal pain (p = 0.018), flatulence (p = 0.006), and gastroesophageal reflux (p = 0.006). EVALUATION/PRACTICAL RECOMMENDATIONS: A standardized approach with structured reporting is important due to the numerous abdominal CF manifestations. To enable precise follow-up analyses, scoring systems based on sonographic findings are excellent.

Inhibition of periarticular bone loss is associated with clinical remission and ACR70-Response in rheumatoid arthritis.

The aim of this study, based on a post hoc analysis of the data set used in the RAPID 1 trial, focuses on the associations between metacarpal bone mineral density, as estimated by digital X-ray radiogrammetry (DXR), and clinical remission as well as ACR70-Response in rheumatoid arthritis (RA) patients treated with certolizumab pegol (CZP). The trial evaluates a total of 345 RA patients treated with methotrexate versus CZP 200 mg versus CZP 400 mg. All patients underwent X-rays of the hand at baseline and week 52 as well as computerized calculations of bone mineral density (BMD) by DXR. Clinical remission was defined as DAS28 < 2.6. ACR70-Response was also evaluated. The radiological assessment of disease progression was estimated using the modified total Sharp Score. The mean difference for DAS28 was observed for patients treated with CZP 400 mg (median: - 3.53, minimum: - 6.77; maximum: + 0.48) and CZP 200 mg (median: - 3.13, minimum: - 6.37; maximum: - 0.52) compared to the methotrexate group (median - 2.41, minimum: - 4.76; maximum: + 0.31). The DXR-BMD showed a minor bone loss for the treatment groups undergoing therapy with CZP 200 mg (median: - 0.009 g/cm2, minimum: - 0.059 g/cm2; maximum: + 0.095 g/cm2) and CZP 400 mg (median: - 0.008 g/cm2, minimum: - 0.064 g/cm2; maximum: + 0.080 g/cm2). The methotrexate group presented an advanced periarticular metacarpal bone loss as measured by DXR-BMD (median: - 0.024 g/cm2, minimum: - 0.102 g/cm2; maximum: + 0.057 g/cm2). In the case of clinical remission and ACR70-Response, no significant change of the DXR-BMD was observed for both CZP groups. The study highlights that patients treated with CZP show a less accentuated periarticular bone loss as estimated by DXR in comparison to patients with methotrexate plus placebo. In addition, patients with clinical remission and ACR70-Response revealed no periarticular demineralisation.

Tailored CT angiography in follow-up after endovascular aneurysm repair (EVAR): combined dose reduction techniques.

Background Endovascular aneurysm repair (EVAR) requires lifelong surveillance by computed tomography angiography (CTA). This is attended by a substantial accumulation of radiation exposure. Iterative reconstruction (IR) has been introduced to approach dose reduction. Purpose To evaluate adaptive statistical iterative reconstruction (ASIR) at different levels of tube voltage concerning image quality and dose reduction potential in follow-up post EVAR. Material and Methods One hundred CTAs in 67 patients with EVAR were examined using five protocols: protocol A (n = 40) as biphasic standard using filtered back projection (FBP) at 120 kV; protocols B (n = 40), C (n = 10), and D1 (n = 5) biphasic using ASIR at 120, 100, and 80 kV, respectively; and protocol D2 (n = 5) with a monophasic splitbolus ASIR protocol at 80 kV. Image quality was assessed quantitatively and qualitatively. Applied doses were determined. Results Applied doses in ASIR protocols were significantly lower than FBP standard (up to 75%). Compared to protocol A, signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) (e.g. arterial CNR intra-/extra-stent lumen: A = 35.4 ± 13.5, B = 34.2 ± 10.0, C = 29.6 ± 6.8, D1 = 32.1 ± 6.3, D2 = 40.8 ± 23.1) in protocol B were equal and in protocols C and D equal to partially inferior, however not decisive for diagnostic quality. Subjective image quality ratings in all protocols were good to excellent without impairments of diagnostic confidence (A-D2: 5), with high inter-rater agreement (60-100%). Conclusion ASIR contributes to significant dose reduction without decisive impairments of image quality and diagnostic confidence. We recommend an adapted follow-up introducing ASIR and combined low-kV in the long-term surveillance after EVAR.