Questionnaire-Based Survey Conducted in 2011 concerning Endoscopic Management of Barrett's Esophagus in East Asian Countries

Background/Aims: Endoscopic definitions and management of Barrett's esophagus vary widely among countries. To examine the current situation regarding diagnosis, epidemiology, management and treatment of Barrett's esophagus in East Asian countries using a questionnaire-based survey. Methods: Representative members of the Committee of the International Gastrointestinal Consensus Symposium developed and sent a questionnaire to major institutions in China, South Korea, Japan, Thailand, Indonesia, and the Philippines. Results: A total of 56 institutions in the 6 countries participated in the survey. We found that the presence of specialized columnar metaplasia is considered to be important for diagnosing Barrett's esophagus in East Asian countries except for Japan. C&M criteria have not been well accepted in East Asia. The palisade vessels are mainly used as a landmark for the esophagogastric junction in Japan. The prevalence of long segment Barrett's esophagus is extremely low in East Asia, while the prevalence of short segment Barrett's esophagus is very high only in Japan, likely due to different diagnostic criteria. Conclusion: Among East Asian countries, we found both similarities and differences regarding diagnosis and management of Barrett's esophagus. The findings in the present survey are helpful to understand the current situation of Barrett's esophagus in East Asian countries.     

Recessively inherited severe aortic aneurysm caused by mutated EFEMP2

Familial aortic aneurysm (AA) is mostly inherited as an autosomal dominant disorder. However, recessively inherited AA has also been observed but in association with skin manifestations of cutis laxa, which is caused by a mutated EFEMP2 gene. In the present study, we recruited 9 patients, from 4 unrelated consanguineous families, with recessively inherited AA. The index cases, their parents, and siblings underwent clinical evaluation and cardiac imaging. In the affected subjects, the clinical presentation ranged from sweating and cyanosis at 3 months of age to incidental findings in an asymptomatic adult. The echocardiogram revealed a wide spectrum of severity of the AA, with a Z-score varying from 5 to 33. Intrafamilial variability was also evident; 2 unrelated subjects were detected at 17 and 20 years of age through family screening. The skin manifestations of cutis laxa were not found in any patient. In 1 family, genome-wide single-nucleotide polymorphism analysis detected a homozygous block, shared by 2 affected siblings, on chromosome 11 at q13. Sequence analysis of EFEMP2, located on chromosome 11 at q13, identified a novel homozygous mutation (p.E161K) in all 9 affected subjects. In this largest cohort of reported patients with a mutated EFEMP2 gene, we illustrate the phenotypic spectrum of inherited AA due to a novel EFEMP2 mutation. In conclusion, our work suggests that in families with apparently recessively inherited AA, molecular analysis of EFEMP2 gene might be warranted.     

Finite element analysis of different surgical approaches in various occlusal loading locations for zygomatic implant placement for the treatment of atrophic maxillae

The aim of this study was to compare two different types of surgical approaches, intrasinus and extramaxillary, for the placement of zygomatic implants to treat atrophic maxillae. A computational finite element simulation was used to analyze the strength of implant anchorage for both approaches in various occlusal loading locations. Three-dimensional models of the craniofacial structures surrounding a region of interest, soft tissue and framework were developed using computed tomography image datasets. The implants were modelled using computer-aided design software. The bone was assumed to be linear isotropic with a stiffness of 13.4GPa, and the implants were assumed to be made of titanium with a stiffness of 110GPa. Masseter forces of 300N were applied at the zygomatic arch, and occlusal loads of 150N were applied vertically onto the framework surface at different locations. The intrasinus approach demonstrated more satisfactory results and could be a viable treatment option. The extramaxillary approach could also be recommended as a reasonable treatment option, provided some improvements are made to address the cantilever effects seen with that approach.     

An accessory mitral valve leaflet causing left ventricular outflow tract obstruction and associated with severe aortic incompetence

This case report describes a 20-year-old woman with Turner's syndrome who presented with reduced effort tolerance limited by dyspnea. She had previously been on pediatric cardiology follow-up for congenital subvalvular aortic stenosis first diagnosed at age 7. Unfortunately she defaulted after two visits before any intervention could be done. Transthoracic echocardiography demonstrated severe aortic incompetence (AI) with a membrane-like structure in the left ventricular outflow tract (LVOT). The mean pressure gradient across the LVOT on continuous wave Doppler was 41 mmHg. The membranous interventricular septum appeared aneurysmal and it was observed that the "subaortic membrane" had a connection to the anterolateral papillary muscle via a strand of chordal tissue. Further images were captured using two-dimensional and three-dimensional transthoracic and transesophageal echocardiography (iE33, Philips Medical Systems, Andover, MA, USA). After a review of the literature it was concluded that this appeared to be an accessory mitral valve (AMV) leaflet causing LVOT obstruction associated with AI. AMV tissue is a rare congenital malformation causing LVOT obstruction. Because it is so unusual, it may not be immediately recognizable even in a high volume echocardiography laboratory. The clue which helped with the diagnosis was the strand of chordal tissue which connected the mass to the papillary muscle. This anomaly is often associated with LVOT obstruction.     

Open Versus Closed Rhinoplasty with Primary Cheiloplasty: A Comparative Study

The repair of unilateral cleft lip nose deformity remains a challenging endeavor for reconstructive surgeons for many reasons, one of which is the timing of rhinoplasty, whether to be synchronous or staged with cleft lip repair and the technique for rhinoplasty. Many authors now favor primary rhinoplasty with the cleft lip repair. Various surgical techniques have been used, most commonly the closed and open rhinoplasty techniques. In this randomized controlled prospective study, we compare the closed rhinoplasty technique with open rhinoplasty during primary unilateral cleft lip repair. Thirty-six patients with unilateral complete cleft lip and nose deformity were selected. Out of this 19 patients were assigned randomly and operated with open rhinoplasty and 17 patients with closed rhinoplasty. The cleft lip repair was done using modified, Millard’s rotation-advancement technique in both the groups. Follow-up assessment was done after 6 months. Quantitative and qualitative analysis were done. Statistical analysis of the data was done using SPSS 11.0. Post-operatively, the alar base width difference between the open and closed rhinoplasty techniques was statistically significant. There was no statistically significant difference in other parameters compared.     

HIV type 1 Gag as a target for antiviral therapy

The Gag proteins of HIV-1 are central players in virus particle assembly, release, and maturation, and also function in the establishment of a productive infection. Despite their importance throughout the replication cycle, there are currently no approved antiretroviral therapies that target the Gag precursor protein or any of the mature Gag proteins. Recent progress in understanding the structural and cell biology of HIV-1 Gag function has revealed a number of potential Gag-related targets for possible therapeutic intervention. In this review, we summarize our current understanding of HIV-1 Gag and suggest some approaches for the development of novel antiretroviral agents that target Gag.     

Peripheral blood gene expression profile of atherosclerotic coronary artery disease in patients of different ethnicity in Malaysia

The molecular basis of coronary artery disease (CAD) has been widely studied in the western world but there is no published work on the Malaysian population. This study looked at the global gene expression profiling of the peripheral blood of patients with CAD from the 3 main ethnic groups in Malaysia. Male subjects selected were based on angiographically confirmed CAD (≥50% stenosis) and normal control subjects (0% stenosis) with age range of 55.6±5.3 and 51.0±5.5years, respectively. The global gene expression of 12 angiographically documented CAD patients and 11 matched control subjects were performed. The combined group samples identified 6 up regulated differential expression (DE) genes (GHRL, LTA, CBS, HP, ITGA2B, and OLR1) and 12 down regulated DE genes (IL18R1, ITGA2B, IL18RAP, HP, OLR1, SOD2 ITGB3, IL1B, MMP9, PLA2G7, UTS2, and CBS) to be involved in CAD at the fold change of 1.3 with fault discovery rate (FDR) of 1%. Three genes, MMP9, IL1B, and SOD2 were down regulated in all the 3 ethnic groups making them potential biomarker candidates for CAD across all three ethnicities. Further verification in a cohort study is needed.     

What's your diagnosis?: Skin lesions in a young female with Behcet's disease

A young female with known Behcet's disease presented with skin lesions over lower limbs. The lesions were biopsied and found to be pyoderma gangrenosum. The lesions responded to oral steroids for 6 weeks.