Mid-term results in patients having tricuspidization of the quadricuspid aortic valve

BACKGROUND: Quadricuspid aortic valve (QAV) is a rare congenital anomaly. We investigate the mid-term results of aortic valve reconstruction by tricuspidization in patients with QAV. METHODS: We analyzed the outcome of eight consecutive patients who underwent aortic valve reconstruction surgery (AVRS) with pericardial leaflets with symptomatic quadricuspid aortic valve (QAV) disease between December 2007 and May 2012. AVRS consists of leaflet reconstruction and fixation of the sino-tubular junction in order to maintain coaptation of the new valve. RESULTS: Six males and two females were included; ages ranged from 19 to 63 years (mean age, 51 years). According to Hurwitz and Roberts’s classification, three patients had type A, three patients had type B, one patient had type C, and one patient had type E. All patients had significant aortic regurgitation (AR): moderate in three patients, moderate to severe in one patient, and severe in four patients. Concomitant ascending aorta wrapping with an artificial vascular graft was performed in one case. There was no occurrence of mortality during the follow-up period (42.4 ± 18.0 months). No redo-operation was required. The NYHA functional class showed improvement from 2.1 ± 0.2 to 1.1 ± 0.2 (p = 0.008). The latest echocardiograms showed AR absent or trivial in seven patients, and mild in one patient. The aortic valve orifice area index (AVAI) was 1.03 ± 0.49 cm(2)/m(2). Compared with preoperative echocardiograms, the left ventricular (LV) ejection fraction showed improvement from 57.6 ± 17.0 to 63.7 ± 13.2% (p = 0.036); the end-diastolic and end-systolic LV dimensions showed a significant decrease, from 63.5 ± 9.6 to 49.5 ± 3.1 mm (p = 0.012) and 43.6 ± 11.8 to 32.1 ± 5.4 mm (p = 0.012), respectively. CONCLUSION: In patients with QAV, AVRS with tricuspidization showed satisfactory early and mid-term results. Long-term follow-up will be necessary in order to study the durability of AVRS; however, it can be considered as a potential standard procedure

Serum BDNF levels in patients with gambling disorder are associated with the severity of gambling disorder and Iowa Gambling Task indices

Background and aims Gambling disorder (GD) shares many similarities with substance use disorders (SUDs) in clinical, neurobiological, and neurocognitive features, including decision-making. We evaluated the relationships among, GD, decision-making, and brain-derived neurotrophic factor (BDNF), as measured by serum BDNF levels. Methods Twenty-one male patients with GD and 21 healthy sex- and age-matched control subjects were evaluated for associations between serum BDNF levels and the Problem Gambling Severity Index (PGSI), as well as between serum BDNF levels and Iowa Gambling Task (IGT) indices. Results The mean serum BDNF levels were significantly increased in patients with GD compared to healthy controls. A significant correlation between serum BDNF levels and PGSI scores was found when controlling for age, depression, and duration of GD. A significant negative correlation was obtained between serum BDNF levels and IGT improvement scores. Discussion These findings support the hypothesis that serum BDNF levels constitute a dual biomarker for the neuroendocrine changes and the severity of GD in patients. Serum BDNF level may serve as an indicator of poor decision-making performance and learning processes in GD and help to identify the common physiological underpinnings between GD and SUDs

Sleep Duration, Sleep Quality, and the Development of Nonalcoholic Fatty Liver Disease:A Cohort Study

INTRODUCTION: The longitudinal relationship between sleep duration, sleep quality, and the risk of nonalcoholic fatty liver disease (NAFLD) is unknown. We aimed to examine the association between sleep duration, sleep quality, and NAFLD development.METHODS: Using the Pittsburgh Sleep Quality Index, sleep duration and quality were evaluated for 143,306 NAFLD-free Korean adults with a mean age of 36.6 years, who were followed for an average of 4.0 years. Hepatic steatosis (HS) was assessed using ultrasonography and liver fibrosis by the fibrosis-4 index (FIB-4) or the NAFLD fibrosis score. Flexible parametric proportional hazard models were used to determine the hazard ratios (HRs) and 95% confidence intervals.RESULTS: There were 27,817 subjects with incident HS, of whom 1,471 had incident HS plus intermediate/high FIB-4. Multivariable-adjusted HRs (95% confidence intervals) for incident HS comparing sleep durations of ≤5, 6, 8, and ≥ 9 hours with 7 hours were 1.19 (1.14-1.23), 1.07 (1.04-1.10), 0.98 (0.94-1.02), and 0.95 (0.87-1.03), respectively. The corresponding HRs for incident HS plus intermediate/high FIB-4 were 1.30 (1.11-1.54), 1.14 (1.01-1.29), 1.11 (0.93-1.33), and 1.08 (0.71-1.63). The association between sleep duration and HS plus intermediate/high FIB-4 was inverse in individuals with good sleep quality but tended to be U-shaped in those with poor sleep quality. The results were similar if FIB-4 was replaced by the NAFLD fibrosis score.DISCUSSION: In young adults, short sleep duration was independently associated with an increased risk of incident NAFLD with or without intermediate/high fibrosis score, suggesting a role for inadequate sleep quantity in NAFLD risk and severity.</p

Decrease in sleep duration and poor sleep quality over time is associated with an increased risk of incident non-alcoholic fatty liver disease

The impact of changes in sleep duration and sleep quality over time on the risk of nonalcoholic fatty liver disease (NAFLD) is not known. We investigated whether changes in sleep duration and in sleep quality between baseline and follow-up are associated with the risk of developing incident NAFLD. The cohort study included 86,530 Korean adults without NAFLD and with a low fibrosis score at baseline. The median follow-up was 3.6 years. Sleep duration and quality were assessed using the Pittsburgh Sleep Quality Index. Hepatic steatosis (HS) and liver fibrosis were assessed using ultrasonography and the fibrosis-4 index (FIB-4). Cox proportional hazard models were used to determine hazard ratios (HRs) and 95% confidence intervals (Cis). A total of 12,127 subjects with incident HS and 559 with incident HS plus intermediate/high FIB-4 was identified. Comparing the decrease in sleep duration of &gt;1 h, with stable sleep duration, the multivariate-adjusted HR (95% CIs) for incident HS was 1.24 (1.15–1.35). The corresponding HRs for incident HS plus intermediate/high FIB-4 was 1.58 (1.10–2.29). Comparing persistently poor sleep quality with persistently good sleep quality, the multivariate-adjusted HR for incident HS was 1.13 (95% CI, 1.05–1.20). A decrease in sleep duration or poor sleep quality over time was associated with an increased risk of incident NAFLD, underscoring an important potential role for good sleep in preventing NAFLD risk.</p

Surgical Treatment of Adult Degenerative Scoliosis

The rapid increase of elderly population has resulted in increased prevalence of adult scoliosis. Adult scoliosis is divided into adult idiopathic scoliosis and adult degenerative scoliosis. These two types of scoliosis vary in patient age, curve pattern and clinical symptoms, which necessitate different surgical indications and options. Back pain and deformity are major indications for surgery in adult idiopathic scoliosis, whereas radiating pain to the legs due to foraminal stenosis is what often requires surgery in adult degenerative scoliosis. When selecting a surgical method, major symptoms and underlying medical diseases should be carefully evaluated, not only to relieve symptoms but also to minimize postoperative complications. Surgical options for adult degenerative scoliosis include: decompression alone; decompression and limited short fusion; and decompression coupled with long fusion and correction of deformity. Decompression and limited short fusion can be applied to patients with a small Cobb's angle and normal sagittal imbalance. For those with a large Cobb's angle and positive sagittal imbalance, long fusion with correction of deformity is required. When long fusion is applied, a careful decision regarding the extent of fusion level should be made when selecting L5 or S1 as the distal fusion level and T10 or the thoracolumbar junction as the proximal fusion level. For the fusion extending to the sacrum, restoration of sagittal balance and rigid fixation with additional iliac screws should be considered. Any surgical procedures for adult degenerative scoliosis are known to have relatively high occurrences of complications; therefore, risks and benefits should be meticulously considered before selecting a surgical procedure

A Case of Intramuscular Hemangioma Presenting with Large-angle Hypertropia

Purpose: To report the case of a patient with large-angle hypertropia of an intramuscular hemangioma of the right superior rectus muscle (SR). Methods: A 63-year-old man with progressive vertical deviation of the right eye for the past 6 months visited our strabismus department; his condition was not painful. An examination indicated that he had 60PD of right hypertropia at distance and near in primary gaze. Additionally, a significant limitation of his downgaze was noted. The right eye appeared mildly proptotic, and the upper and lower eyelids were slightly edematous. Corrected vision was 20/20 in both eyes. Results: Orbital magnetic resonance imaging (MRI) studies revealed fusiform enlargement of the right superior rectus muscle, with prominent but irregular enhancement following gadolinium administration. Incisional biopsy revealed an intramuscular hemangioma in the superior rectus muscle with cavernous-type vessels. Conclusions: This case demonstrates that intramuscular hemangioma should be considered in the differ-ential diagnosis of isolated extraocular muscle enlargement and unusual strabismus. Korean Journal o

The lipoxygenase gene family: a genomic fossil of shared polyploidy between Glycine max and Medicago truncatula

<p>Abstract</p> <p>Background</p> <p>Soybean lipoxygenases (<it>Lxs</it>) play important roles in plant resistance and in conferring the distinct bean flavor. <it>Lxs </it>comprise a multi-gene family that includes <it>GmLx1</it>, <it>GmLx2 </it>and <it>GmLx3</it>, and many of these genes have been characterized. We were interested in investigating the relationship between the soybean lipoxygenase isozymes from an evolutionary perspective, since soybean has undergone two rounds of polyploidy. Here we report the tetrad genome structure of soybean <it>Lx </it>regions produced by ancient and recent polyploidy. Also, comparative genomics with <it>Medicago truncatula </it>was performed to estimate <it>Lxs </it>in the common ancestor of soybean and <it>Medicago</it>.</p> <p>Results</p> <p>Two <it>Lx </it>regions in <it>Medicago truncatula </it>showing synteny with soybean were analyzed. Differential evolutionary rates between soybean and <it>Medicago </it>were observed and the median Ks values of Mt-Mt, Gm-Mt, and Gm-Gm paralogs were determined to be 0.75, 0.62, and 0.46, respectively. Thus the comparison of Gm-Mt paralogs (Ks = 0.62) and Gm-Mt orthologs (Ks = 0.45) supports the ancient duplication of <it>Lx </it>regions in the common ancestor prior to the <it>Medicago</it>-<it>Glycine </it>split. After speciation, no <it>Lx </it>regions generated by another polyploidy were identified in <it>Medicago</it>. Instead tandem duplication of <it>Lx </it>genes was observed. On the other hand, a lineage-specific duplication occurred in soybean resulting in two pairs of <it>Lx </it>regions. Each pair of soybean regions was co-orthologous to one <it>Lx </it>region in <it>Medicago</it>. A total of 34 <it>Lx </it>genes (15 <it>MtLxs </it>and 19 <it>GmLxs) </it>were divided into two groups by phylogenetic analysis. Our study shows that the <it>Lx </it>gene family evolved from two distinct <it>Lx </it>genes in the most recent common ancestor.</p> <p>Conclusion</p> <p>This study analyzed two pairs of <it>Lx </it>regions generated by two rounds of polyploidy in soybean. Each pair of soybean homeologous regions is co-orthologous to one region of <it>Medicago</it>, demonstrating the quartet structure of the soybean genome. Differential evolutionary rates between soybean and <it>Medicago </it>were observed; thus optimized rates of Ks per year should be applied for accurate estimation of coalescence times to each case of comparison: soybean-soybean, soybean-<it>Medicago</it>, or <it>Medicago</it>-<it>Medicago</it>. In conclusion, the soybean <it>Lx </it>gene family expanded by ancient polyploidy prior to taxon divergence, followed by a soybean- specific duplication and tandem duplications, respectively.</p

Diffusion-Weighted MR Imaging of Unusual White Matter Lesion in a Patient with Menkes Disease

We report here on the diffusion-weighted imaging of unusual white matter lesions in a case of Menkes disease. On the initial MR imaging, the white matter lesions were localized in the deep periventricular white matter in the absence of diffuse cortical atrophy. The lesion showed diffuse high signal on the diffusion-weighted images and diffuse progression and persistent hyperintensity on the follow up imaging. Our case suggests that the white matter lesion may precede diffuse cortical atrophy in a patient with Menkes disease

Serum alanine aminotransferase levels are closely associated with metabolic disturbances in apparently healthy young adolescents independent of obesity

Purpose Liver metabolism plays a pivotal role in the development of metabolic disorders. We aimed to investigate the clinical and laboratory risk factors associated with alanine aminotransferase (ALT) levels in young adolescents from an urban population in Korea. Methods A population of 120 apparently healthy adolescents aged 12–13 years was included in the cross-sectional design study; 58 were overweight or obese and 62 were of normal weight. We estimated anthropometric and laboratory measurements, including waist-to-height ratio, blood pressure, insulin sensitivity, aspartate aminotransferases (AST), ALT, and lipid profiles. Results The mean ages of the overweight or obese and normal weight participants were 12.9±0.3 and 13.0±0.3 years, respectively. Height, weight, body mass index, waist circumference, waist-to-height ratio, systolic and diastolic blood pressure, AST, ALT, total cholesterol, low-density lipoprotein-cholesterol, triglyceride, insulin, and the homeostatic model assessment of insulin resistance (HOMA-IR) score were significantly higher and the high-density lipoprotein-cholesterol and quantitative insulin-sensitivity check index were significantly lower in the overweight/obese participants in comparison to the normal-weight participants (all P<0.05). In multivariate linear regression analysis, waist-to-height ratio, systolic blood pressure, and HOMA-IR score were independently and positively associated with serum ALT levels. Conclusion Screening for ALT levels in adolescents may help to differentiate those at risk of metabolic abnormalities and thus prevent disease progression at an early age