Calcified Carcinoma of the Gallbladder with Calcified Nodal Metastasis Presenting as a Porcelain Gallbladder: A Case Report

Porcelain gallbladder is regarded as a risk factor of gallbladder cancer. A porcelain gallbladder with calcified regional lymph nodes was found using computed tomography (CT) and magnetic resonance imaging (MRI) in a 43-year-old man who presented with nausea, vomiting, and abdominal pain. His cholecystectomy specimen showed diffuse wall thickening and contained small gallstones. Histological examination revealed diffuse infiltrative adenocarcinoma with extensive intratumoral calcification (calcified carcinoma). The majority of the calcified material was located within or replaced the tumor glands, and was not found in the stroma. A lymph node was totally replaced with a calcified metastatic adenocarcinoma. To the best of our knowledge, only one case of calcified lymph node metastasis from a calcified carcinoma of the gallbladder has been previously reported in the literature. We herein add a case of calcified carcinoma of the gallbladder with calcified lymph node metastasis, presenting as a porcelain gallbladder on CT and MRI

Angiographic analysis of the lateral intercostal artery perforator of the posterior intercostal artery: anatomic variation and clinical significance

PURPOSEKnowledge of the anatomic variations of the posterior intercostal artery (PICA) and its major branches is important during transthoracic procedures and surgery. We aimed to identify the anatomic features and variations of the lateral intercostal artery perforator (LICAP) of the PICA with selective PICA arteriography.METHODSWe retrospectively evaluated 353 PICAs in 75 patients with selective PICA arteriography for the following characteristics: incidence, length (as number of traversed intercostal spaces), distribution at the hemithorax (medial half vs. lateral half), and size as compared to the collateral intercostal artery of the PICA.RESULTSThe incidence of LICAPs was 35.9% (127/353). LICAPs were most commonly observed in the right 8th–11th intercostal spaces (33%, 42/127) and in the medial half of the hemithorax (85%, 108/127). Most LICAPs were as long as two (35.4%, 45/127) or three intercostal spaces (60.6%, 77/127). Compared to the collateral intercostal artery, 42.5% of LICAPs were larger (54/127), with most of these observed in the right 4th–7th intercostal spaces (48.8%, 22/54).CONCLUSIONWe propose the clinical significance of the LICAP as a potential risk factor for iatrogenic injury during posterior transthoracic intervention and thoracic surgery. For example, skin incisions must be as superficial as possible and directed vertically at the right 4th–7th intercostal spaces and the medial half of the thorax. Awareness of the anatomical variations of the LICAPs of the PICA will allow surgeons and interventional radiologists to avoid iatrogenic arterial injuries during posterior transthoracic procedures and surgery

The strong association of left-side heart anomalies with Kabuki syndrome

PurposeKabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center.MethodsA retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome.ResultsThe median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome.ConclusionPediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling

Pathogenicity and antigenicity of a new variant of Korean nephropathogenic infectious bronchitis virus

Despite the existence of an active vaccination program, recently emerged strains of nephropathogenic infectious bronchitis virus (IBV) in Korea have caused significant economic losses in the poultry industry. In this study, we assessed the pathogenic and antigenic characteristics of a K-IIb type field strain of IBV that emerged in Korea since 2003, such as Kr/Q43/06. Specific pathogen free 1-week-old chickens exhibited severe respiratory symptoms (dyspnea) and nephropathogenic lesions (swollen kidneys with nephritis and urate deposits) following challenge with the recent IBV field strain. The antigenic relatedness (R value), based on a calculated virus neutralization index, of the K-IIb type field strain and K-IIa type strain KM91 (isolated in 1991) was 30%, which indicated that the recent strain, Kr/Q43/06, is a new variant that is antigenically distinct from strain KM91. This report is the first to document the emergence of a new antigenic variant of nephropathogenic IBV in chicken from Korea

The outcome of percutaneous stent implantation in congenital heart disease: experience of a single institute

PurposeThe efficacy of percutaneous stent implantation for congenital heart disease (CHD) in Korea, where stent availability is limited, has not been determined. This study evaluated the acute and midterm results of stent implantation in different CHD subgroups.MethodsStents were implanted in 75 patients with 81 lesions: (1) pulmonary artery stenosis (PAS) group, 56 lesions in 51 patients; (2) coarctation of the aorta (CoA) group, 5 lesions in 5 patients; (3) Fontan group, 13 lesions in 12 patients; (4) ductal stent group, 3 lesions in 3 patients; and (5) other CHD group, 4 lesions in 4 patients. Mean follow-up duration was 2.1 years (0.1–4 years). Medical records were reviewed retrospectively.ResultsThe minimum lumen diameter (MLD) in PAS and CoA increased from 5.0±1.9 mm and 8.4±1.6 mm to 10.1±3.6 mm and 12.3±2.5 mm, respectively (P<0.01). In the PAS group, pressure gradient decreased from 25.7±15.6 mmHg to 10.4±10.1 mmHg, and right ventricular to aortic pressure ratio from 0.56±0.21 to 0.46±0.19. In the CoA group, the pressure gradient decreased from 50±33 mmHg to 17±8 mmHg. In the ductal stent group, the MLD of the ductus increased from 2.3 mm to 4.3 mm and arterial oxygen saturation from 40%–70% to 90%. No deaths were associated with stent implantation. Stent migration occurred in 3 patients, but repositioning was successful in all. Stent redilation was performed successfully in 26 cases after 29±12 months.ConclusionPercutaneous stent implantation was safe and effective, with acceptable short and mid-term outcomes in Korean CHD patients

The Expression Patterns of FAM83H and PANX2 Are Associated With Shorter Survival of Clear Cell Renal Cell Carcinoma Patients

FAM83H is primarily known for its role in amelogenesis; however, recent reports suggest FAM83H might be involved in tumorigenesis. Although the studies of FAM83H in kidney cancer are limited, a search of the public database shows a significant association between FAM83H and pannexin-2 (PANX2) in clear cell renal cell carcinomas (CCRCCs). Therefore, we evaluated the clinicopathological significance of the immunohistochemical expression of FAM83H and PANX2 in 199 CCRCC patients. The expression of FAM83H and PANX2 were significantly associated with each other. In univariate analysis, individual, and co-expression pattern of FAM83H and PANX2 was significantly associated with shorter overall survival (OS) and relapse-free survival (RFS) of CCRCC patients: nuclear expression of FAM83H (OS; P &lt; 0.001, RFS; P &lt; 0.001), cytoplasmic expression of FAM83H (OS; P &lt; 0.001, RFS; P &lt; 0.001), nuclear expression of PANX2 (OS; P &lt; 0.001, RFS; P &lt; 0.001), cytoplasmic expression of PANX2 (OS; P &lt; 0.001, RFS; P &lt; 0.001), co-expression pattern of nuclear FAM83H and nuclear PANX2 (OS; P &lt; 0.001, RFS; P &lt; 0.001). In multivariate analysis, nuclear expression of FAM83H (OS; P &lt; 0.001, RFS; P = 0.003) and the co-expression pattern of nuclear FAM83H and PANX2 (OS; P &lt; 0.001, RFS; P &lt; 0.001) were independent indicators of shorter survival of CCRCC patients. Cytoplasmic expression of FAM83H was associated with shorter RFS (P = 0.030) in multivariate analysis. In Caki-1 and Caki-2 CCRCC cells, knock-down of FAM83H decreased PANX2 expression and cell proliferation, and overexpression of FAM83H increased PANX2 expression and cell proliferation. These results suggest that FAM83H and PANX2 might be involved in the progression of CCRCC in a co-operative manner, and their expression might be used as novel prognostic indicators for CCRCC patients

Genome-wide analysis of DNA methylation patterns in horse

Background: DNA methylation is an epigenetic regulatory mechanism that plays an essential role in mediating biological processes and determining phenotypic plasticity in organisms. Although the horse reference genome and whole transcriptome data are publically available the global DNA methylation data are yet to be known. Results: We report the first genome-wide DNA methylation characteristics data from skeletal muscle, heart, lung, and cerebrum tissues of thoroughbred (TH) and Jeju (JH) horses, an indigenous Korea breed, respectively by methyl-DNA immunoprecipitation sequencing. The analysis of the DNA methylation patterns indicated that the average methylation density was the lowest in the promoter region, while the density in the coding DNA sequence region was the highest. Among repeat elements, a relatively high density of methylation was observed in long interspersed nuclear elements compared to short interspersed nuclear elements or long terminal repeat elements. We also successfully identified differential methylated regions through a comparative analysis of corresponding tissues from TH and JH, indicating that the gene body regions showed a high methylation density. Conclusions: We provide report the first DNA methylation landscape and differentially methylated genomic regions (DMRs) of thoroughbred and Jeju horses, providing comprehensive DMRs maps of the DNA methylome. These data are invaluable resource to better understanding of epigenetics in the horse providing information for the further biological function analyses.open1

The PEX7-Mediated Peroxisomal Import System Is Required for Fungal Development and Pathogenicity in Magnaporthe oryzae

In eukaryotes, microbodies called peroxisomes play important roles in cellular activities during the life cycle. Previous studies indicate that peroxisomal functions are important for plant infection in many phytopathogenic fungi, but detailed relationships between fungal pathogenicity and peroxisomal function still remain unclear. Here we report the importance of peroxisomal protein import through PTS2 (Peroxisomal Targeting Signal 2) in fungal development and pathogenicity of Magnaporthe oryzae. Using an Agrobacterium tumefaciens-mediated transformation library, a pathogenicity-defective mutant was isolated from M. oryzae and identified as a T-DNA insert in the PTS2 receptor gene, MoPEX7. Gene disruption of MoPEX7 abolished peroxisomal localization of a thiolase (MoTHL1) containing PTS2, supporting its role in the peroxisomal protein import machinery. ΔMopex7 showed significantly reduced mycelial growth on media containing short-chain fatty acids as a sole carbon source. ΔMopex7 produced fewer conidiophores and conidia, but conidial germination was normal. Conidia of ΔMopex7 were able to develop appressoria, but failed to cause disease in plant cells, except after wound inoculation. Appressoria formed by ΔMopex7 showed a defect in turgor generation due to a delay in lipid degradation and increased cell wall porosity during maturation. Taken together, our results suggest that the MoPEX7-mediated peroxisomal matrix protein import system is required for fungal development and pathogenicity M. oryzae