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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]

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.