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Purpose: To evaluate the diagnostic performance and feasibility of a modified computed tomography (CT) scan protocol, we performed a serial assessment of the computed tomography angiography for pulmonary artery (CTA-P) and systemic artery (CTA-S) (CTA-PS) using a reduced contrast agent dose to diagnose systemic artery-to-pulmonary artery shunts (SPSs). Materials and methods: Twenty-five patients who underwent multiphase contrast-enhanced chest CT and conventional chest angiography were included. Three image sets (CTA-P, CTA-S, and CTA-PS) were evaluated by two board-certified radiologists. The visualization of the CT image findings associated with SPSs, such as filling defects and enhancement in the pulmonary arteries, was evaluated using a 5-point scale. Results: The diagnostic performance (sensitivity, specificity, and accuracy) of CT imaging findings associated with SPSs in CTA-P and CTA-PS were as follows: CTA-P, 57.1%, 87.5%, and 62.0%; CTA-PS, 81.0%, 100.0%, and 84.0%. CT findings associated with SPSs in CTA-P were significantly sensitive to the CTA-PS protocol. There were no significant differences between the CTA-S and CTA-PS protocols. The area under the curve (AUC) of the CT imaging findings associated with SPSs in the CTA-P and CTA-PS groups was 0.835 and 0.911, respectively (P = 0.191). The AUC of the CT imaging findings associated with SPSs in CTA-S and CTA-PS were 0.891 and 0.926, respectively (P = 0.373). Conclusion: CTA-PS using a reduced contrast agent dose protocol could improve the overall diagnostic confidence of SPSs, owing to better visualization of CT imaging findings associated with SPSs compared to individual assessments of CTA-P or CTA-S. Therefore, CTA-PS can be used as an alternative preembolization evaluation modality to conventional angiography in patients with hemoptysis suspected of having SPSs. [ABSTRACT FROM AUTHOR]

Purpose: This study evaluated major vascular injury risk in distal femoral osteotomy (DFO) via the subvastus approach and examined the relationship between the posterior border of the vastus medialis (VM) and the superficial femoral artery (SFA) and vein (SFV). Methods: Eighty limbs from 80 patients were evaluated using lower extremity computed tomographic venography. The positional relationship between the VM, SFA, and SFV was evaluated by measuring the angle between each structure and the horizontal reference point (VMA: VM angle, FAA (femoral arterial angle), FVA (femoral venous angle) and the distance between VM and the SFA and SFV (VMAD: VM-arterial distance, VMVD: VM-venous distance) in each axial slice (0/10/20/30/40 mm) proximal to the level of the superior patellar margin. The proximity of the posterior border of the VM and the SFA/SFV as a vertical distance, measured between the slice of the superior border of the patella and the slice where the posterior border of the VM contacted the SFA was evaluated. Single and multiple regression analyses were performed using the vertical distance as the objective variable. Results: As the slice shifted proximally, the VMA significantly increased (24.9 ± 8.5, 36.3 ± 8.8, 47.4 ± 11.8, 59.9 ± 14, 70.3 ± 13.7 degrees, respectively, p < 0.001 between all slices) and moved posteromedially from a medial direction. FAA (94.2 ± 7, 86.9 ± 9.2, 78.4 ± 9.7, 71.4 ± 9.8, 66.6 ± 10.5 degree, respectively, p < 0.001 between all slices) and FVA (100.6 ± 4.9, 98.3 ± 5.9, 93.7 ± 7.5, 88 ± 9.2, 81.1 ± 10.5 degrees, respectively, p < 0.001 between all slices) decreased and moved from a posterolateral to a posteromedial direction, while VMAD (35.4 ± 7.8, 24.1 ± 7.3, 14.3 ± 6, 8.4 ± 7, 6.2 ± 6.3 mm, respectively, p < 0.001 between all slices) and VMVD significantly decreased (42.7 ± 7.3, 32 ± 7.4, 22.4 ± 6.8, 14.5 ± 10.6, 8.7 ± 7.1 mm, respectively, p < 0.001 between all slices). The average vertical distance was 36 ± 9.3 mm (range 18.6–61.5 mm). The body height and the patellar length significantly affected the vertical distance. Conclusion: The posterior border of the VM shifted posteromedially from distal to proximal and contacted the SFA at an average of 36 mm from the suprapatellar border. Surgeons should be aware of the risk of major vascular injury during exposure and osteotomy. Level of evidence: Case series, Level IV. [ABSTRACT FROM AUTHOR]

Introduction: With the advent of effective systemic therapy, transarterial chemoembolization (TACE) is established as a highly effective locoregional treatment modality for carefully selected patients with hepatocellular carcinoma (HCC). This randomized controlled trial was conducted to clarify whether selective TACE with drug-eluting beads (DEB-TACE) loaded with epirubicin or selective conventional TACE (cTACE) with epirubicin-ethiodized oil might be more effective for obtaining complete response(CR) in patients with HCC. Methods: Between March 2016 and May 2019, Child-Pugh class A or B patients with unresectable HCC who were scheduled to receive selective TACE were randomly assigned at a 1:1 ratio to the DEB-TACE arm or the cTACE arm. The primary endpoint was the CR rate at 3 months, as evaluated according to the modified Response Evaluation Criteria in Solid Tumors by an independent review committee, and the secondary endpoints were the CR rate at 1 month and incidences of adverse events. Results: A total of 200 patients (DEB-TACE, 99 patients; cTACE, 101 patients) were enrolled in the study. The CR rates at 3 months and 1 month were significantly higher in the cTACE arm (75.2%, 84.2%) as compared with the DEB-TACE arm (27.6%, 35.7%). However, the frequencies of adverse events of any grade, including pyrexia (DEB-TACE vs. cTACE, 19.4% vs. 45.5%, p = 0.0001), fatigue (5.1% vs. 15.8%, p = 0.0194), malaise (11.1% vs. 25.7%, p = 0.0103), appetite loss (12.1% vs. 28.7%, p = 0.0048), abdominal pain (12.1% vs. 23.8%, p = 0.0423), increased serum bilirubin (22.2% vs. 48.5%, p = 0.0002), hypoalbuminemia (43.4% vs. 60.3%, p = 0.0154), increased serum aspartate aminotransferase (35.7% vs. 81.2%, p < 0.0001), and increased serum alanine aminotransferase (35.7% vs. 77.2%, p < 0.0001), were also significantly higher in the cTACE arm than in the DEB-TACE arm. Conclusions: Selective cTACE appeared to have higher CR rates for local tumor control as compared to selective DEB-TACE for HCC. However, the frequency of postembolization syndrome was also significantly higher in the cTACE group than in the DEB-TACE group. Thus, to achieve CR, cTACE may be selected over DEB-TACE in patients who can be expected to tolerate postembolization syndrome. [ABSTRACT FROM AUTHOR]

Cardiospondylocarpofacial syndrome (CSCF; OMIM#157800) is characterized by growth impairment, failure to thrive in infancy, multiple valvular disease, carpal and tarsal fusions, vertebral fusions, and joint hypermobility. It is caused by pathogenic variants of MAP3K7, which encodes transforming growth factor‐β activated kinase 1 (TAK1), a member of the mitogen‐activated protein kinase kinase kinase family (MAPKKK). Only eight individuals with molecularly confirmed CSCF have been reported. Here, we report the first Asian CSCF male with a novel missense variant of MAP3K7 (NM_145331.3: c.467A > T: p.Asp156Val). We compared and reviewed the clinical and molecular findings in previously reported CSCF cases and the present case to better delineate the phenotype of CSCF. In addition to the main symptoms of CSCF, the present case had a mixed phenotype of Ehlers–Danlos syndrome (EDS) and Noonan syndrome. Taking this case together with the previously reported cases, CSCF may overlap with the phenotypes of EDS and Noonan syndrome, suggesting that this finding may contribute to diagnosing CSCF. Another major achievement of this research is to successfully capture the process of carpal fusion in a CSCF case radiographically. This work may expand the phenotypic spectrum of CSCF. [ABSTRACT FROM AUTHOR]

Elsahy–Waters syndrome (EWS; OMIM#211380) is a rare autosomal recessive disorder that is caused by loss‐of‐function variants in CDH11, which encodes cadherin 11. EWS is characterized by brachycephaly, midface hypoplasia, characteristic craniofacial morphology, cervical fusion, cutaneous syndactyly in 2–3 digits, genitourinary anomalies, and intellectual disability. To the best of our knowledge, there have been only six patients of molecularly confirmed EWS. We report the first patient of EWS in East Asia in a Japanese man with a novel splice site homozygous variant of CDH11. We reviewed the clinical and molecular findings in previously reported individuals and the present patient. In addition to the previously reported clinical features of EWS, the present patient had unreported findings of atlantoaxial instability due to posterior displacement of dens, thoracic fusion, thoracic butterfly vertebra, sacralization of the lumbar vertebra (L5), and multiple perineural cysts. The spinal findings in this patient could represent a new spectrum of skeletal phenotypes of EWS. It remains to be clarified whether the multiple perineural cysts in the patient were associated with EWS or coincidental. The current observation might contribute to an expanded understanding of the clinical consequences of loss‐of‐function of cadherin 11. [ABSTRACT FROM AUTHOR]

Background: A peritumoral hypointense rim (PTHR) is sometimes observed around hepatocellular carcinoma (HCC) on T2*-weighted images (T2*WIs). We aimed to investigate the association between the PTHR and histopathologic findings on T2*WIs. Methods: We assessed the presence of a PTHR on T2*WIs in 39 pathologically proven HCCs from April 2012 to December 2013. Prussian blue staining was performed, and iron deposition was evaluated by semiquantitative and quantitative methods. Optical density was used in the quantitative methods. The associations between a PTHR on T2*WI and histopathologic peritumoral or background liver iron deposition were analyzed. Results: A PTHR on T2*WI was observed in 23 of 39 (59%) HCCs. There was no significant difference in the histopathologic fibrous capsule findings (P = 0.394). In the semiquantitative methods, both peritumoral and background liver iron deposition grade were significantly higher in HCCs with a PTHR compared with HCCs without a PTHR (P < 0.001). The mean optical density in HCCs with a PTHR was significantly higher compared with HCCs without a PTHR, in the quantitative peritumoral (42,244.1 ± 20,854.9 vs. 18,739.1 ± 12,258.7, respectively; P < 0.001) and background liver iron deposition analyses (35,554.7 ± 19,854.8 vs. 17,292.4 ± 11,605.8, respectively; P < 0.001). Tumor size (P = 0.005), etiology (P = 0.001), and degree of fibrosis (P = 0.042) were significantly associated with the presence of a PTHR. Conclusions: A PTHR in HCCs on T2*WIs was strongly associated with peritumoral iron deposition in the iron-deposited background liver but not with the fibrous capsule. [ABSTRACT FROM AUTHOR]

Purpose: To compare the visualization of hemodynamic imaging findings of hypervascular hepatocellular carcinoma (HCC) on dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) using radial volumetric imaging breath-hold examination with k-space-weighted image contrast reconstruction (r-VIBE-KWIC) versus dynamic computed tomography during hepatic arteriography (dyn-CTHA).Materials and Methods: We retrospectively reviewed the databases of preoperative DCE-MRI using r-VIBE-KWIC, dyn-CTHA, and postoperative pathology of resected specimens. Fourteen patients with 14 hypervascular HCCs underwent both DCE-MRI and dyn-CTHA. The imaging findings of the tumor and adjacent liver parenchyma were assessed on both modalities by two readers. The tumor enhancement time was also compared between the two modalities.Results: On DCE-MRI/dyn-CTHA, early staining, peritumoral low-intensity or low-density bands, corona enhancement, and washout of HCC were observed in 14/14 (100%), 10/12 (83%), 11/14 (78%), and 4/14 (29%) patients, respectively. Pathologically, four HCCs with low-density bands on dyn-CTHA had no fibrous capsules. The median tumor enhancement time on DCE-MRI and dyn-CTHA was 24 (9-24) and 23 (8-35) s, respectively. The correlation coefficient between the two groups was 0.762 (P < 0.002).Conclusions: DCE-MRI using r-VIBE-KWIC has diagnostic potential comparable with that of dyn-CTHA in the hemodynamic evaluation of hypervascular HCC except for the washout phenomenon. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the difference in the time-intensity curves (TICs) of the abdominal aorta on dynamic contrast-enhanced MRI (DCE-MRI) between Gd-DTPA and Gd-EOB-DTPA. Materials and methods: Ten healthy volunteers underwent DCE-MRI three times with the following protocol: group A, Gd-DTPA at an injection rate of 3 ml/s; group B, Gd-EOB-DTPA, 3 ml/s; group C, Gd-EOB-DTPA, 1.5 ml/s. Signal intensities (SIs) of the abdominal aorta were measured, and the contrast enhancement ratio (CER) was calculated. Time-to-CER curves were compared among the three groups. The differences in maximum CER (CER) and time-to-peak of CER were analyzed. Results: The time-to-CER curve showed a double peak pattern in group A and single-peak pattern in groups B and C. The mean time between the first and the second peak was 6.2 s. The mean CER of each group was 4.50, 4.52 and 4.27, respectively. In group A, B and C, the mean time-to-peak was 14.6, 10.6 and 12.6 s, respectively. There was a significant difference between group A and B ( P < 0.01). Conclusion: To set up the optimal protocol for abdominal DCE-MRI, it should be noted that TIC in the Gd-DTPA and Gd-EOB-DTPA group showed different patterns, and a slower injection rate showed a less abrupt SI change in the Gd-EOB-DTPA group than in the Gd-DTPA group. [ABSTRACT FROM AUTHOR]

A 58-year-old woman with chronic hepatitis C developed interstitial pneumonia (IP) while undergoing pegylated interferon (PEG IFN)-α-2a and ribavirin (RBV) therapy. Serum levels of sialylated carbohydrate antigen KL-6 (KL-6), a known marker of disease activity in fibrosing lung disorders, had been regularly measured once a month for early detection of IP, and had begun rising noticeably from 12 weeks to 540 U/mL at 33 weeks of treatment. On examination, remarkable fine crackles were detected by dorsal auscultation and bilateral ground-glass opacities and reticular shadows were depicted by computed tomography. The patient successfully recovered from her early-stage pneumonia by immediate discontinuation of therapy, which indicates that regular monitoring of serum KL-6 may be effective for avoidance of IP progression induced by PEG IFN and RBV therapy. [ABSTRACT FROM AUTHOR]

The article presents a case study of asymptomatic arteriovenous fistula (AVF) that was found in a patient after five years of renal biopsy through Doppler ultrasonography. Topics discussed include the patient history showing mesangial proliferative glomerulonephritis and focal glomerulosclerosis, treatment with Japanese herbal medicine saireito and use of transcatheter arterial embolization in the diagnosis.