In-situ Neutron Tomography on Mixing Behavior of Supercritical Water and Room Temperature Water in a Tubular Flow Reactor

We have synthesized metal oxide nanoparticles through hydrothermal reaction at around 400 °C and 25 MPa by mixing the stream of metal ion solution at room temperature with another stream of supercritical water in a continuous flow-type reactor. In order to visualize the mixing behavior of the two streams, we performed neutron tomography measurements. By performing tomography measurements while rotating the mixing piece with supplying supercritical water and room temperature water, we succeeded in obtaining the three dimensional distribution of neutron attenuation. The results clearly showed how the two streams mix, which serves as a reference for numerical simulation

Isolated congenital megacystis without intestinal obstruction: A mild variant of chronic intestinal pseudoobstruction syndrome?

Megacystis is frequently involved with chronic intestinal pseudoobstruction syndrome; however, isolated megacystis without intestinal obstruction is extremely rare. We present the case of a female patient with isolated congenital megacystis without severe intestinal obstruction. In this case, barium enema did not reveal any significant findings; however, histologic evaluation of her rectum showed hypoganglionosis of the submucous and myenteric plexuses. These findings indicate that this case may be a mild variant of chronic intestinal pseudoobstruction syndrome. The presence of megacystis should alert the physician to the possibility of chronic intestinal pseudoobstruction syndrome. © 2011 Elsevier Inc. All rights reserved

Case report: A family of atypical hemolytic uremic syndrome involving a CFH::CFHR1 fusion gene and CFHR3-1-4-2 gene duplication

Mutations in the complement factor H (CFH) gene are associated with complement dysregulation and the development of atypical hemolytic uremic syndrome (aHUS). Several fusion genes that result from genomic structural variation in the CFH and complement factor H-related (CFHR) gene regions have been identified in aHUS. However, one allele has both CFHR gene duplication and CFH::CFHR1 fusion gene have not been reported. An 8-month-old girl (proband) presented with aHUS and was treated with ravulizumab. Her paternal grandfather developed aHUS previously and her paternal great grandmother presented with anti-neutrophil cytoplasmic antibody-associated vasculitis and thrombotic microangiopathy (TMA). However, the proband’s parents have no history of TMA. A genetic analysis revealed the presence of CFH::CFHR1 fusion gene and a CFHR3-1-4-2 gene duplication in the patient, her father, and her paternal grandfather. Although several fusion genes resulting from structural variations of the CFH–CFHR genes region have been identified, this is the first report of the combination of a CFH::CFHR1 fusion gene with CFHR gene duplication. Because the CFH–CFHR region is highly homologous, we hypothesized that CFHR gene duplication occurred. These findings indicate a novel pathogenic genomic structural variation associated with the development of aHUS

BCS and generalized BCS superconductivity in relativistic quantum field theory. I. formulation

We investigate the BCS and generalized BCS theories in the relativistic quantum field theory. We select the gauge freedom as U(1), and introduce a BCS-type effective attractive interaction. After introducing the Gor'kov formalism and performing the group theoretical consideration of the mean fields, we solve the relativistic Gor'kov equation and obtain the Green's functions in analytical forms. We obtain various types of gap equations.Comment: 31 page

Impact of Chronic Kidney Disease on the Presence and Severity of Aortic Stenosis in Patients at High Risk for Coronary Artery Disease

<p>Abstract</p> <p>Objective</p> <p>We evaluated the impact of chronic kidney disease (CKD) on the presence and severity of aortic stenosis (AS) in patients at high risk for coronary artery disease (CAD).</p> <p>Methods</p> <p>One hundred and twenty consecutive patients who underwent invasive coronary angiography were enrolled. Aortic valve area (AVA) was calculated by the continuity equation using transthoracic echocardiography, and was normalized by body surface area (AVA index).</p> <p>Results</p> <p>Among all 120 patients, 78% had CAD, 55% had CKD (stage 3: 81%; stage 4: 19%), and 34% had AS (AVA < 2.0cm²). Patients with AS were older, more often female, and had a higher frequency of CKD than those without AS, but the prevalence of CAD and most other coexisting conventional risk factors was similar between patients with and without AS. Multivariate linear regression analysis indicated that only CKD and CAD were independent determinants of AVA index with standardized coefficients of -0.37 and -0.28, respectively. When patients were divided into 3 groups (group 1: absence of CKD and CAD, n = 16; group 2: presence of either CKD or CAD, n = 51; and group 3: presence of both CKD and CAD, n = 53), group 3 had the smallest AVA index (1.19 ± 0.30*# cm²/m², *p < 0.05 vs. group 1: 1.65 ± 0.32 cm²/m², and #p < 0.05 vs. group 2: 1.43 ± 0.29* cm²/m²) and the highest peak velocity across the aortic valve (1.53 ± 0.41*# m/sec; *p < 0.05 vs. group 1: 1.28 ± 0.29 m/sec, and #p < 0.05 vs. group 2: 1.35 ± 0.27 m/sec).</p> <p>Conclusion</p> <p>CKD, even pre-stage 5 CKD, has a more powerful impact on the presence and severity of AS than other conventional risk factors for atherosclerosis in patients at high risk for CAD.</p

CHAPTER 9: AORTIC STENOSIS

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Aortic Stenosis

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Pulmonary Hypertension with Valvular Heart Disease: When to Treat the Valve Disease and When to Treat the Pulmonary Hypertension.

PURPOSE OF REVIEW: This article will review the current guidelines for therapeutic intervention in (pulmonary hypertension) PH related to left heart disease (PH-LHD). RECENT FINDINGS: The 6th World Symposium on Pulmonary Hypertension (WSPH) recommended that the mean pulmonary artery pressure (mPAP) should be lowered to 20 mmHg. In several randomized controlled trials performed in patients with PH-LHD, pulmonary arterial hypertension (PAH)-specific drug therapy demonstrated no evidence of beneficial effects. Furthermore, in the sildenafil for improving outcomes after valvular correction (SIOVAC) trial, the use of sildenafil in the context of PH post-valvular heart disease (VHD) intervention is associated with an increased risk of clinical deterioration and death. Therefore, medical therapy such as PAH-specific drugs is still not recommended in PH-LHD. The principle of PH-LHD therapy is the treatment of underlying VHD. It is crucial to undergo surgical intervention at an appropriate time prior the development of potentially irreversible PH. Stress echocardiography (SE) is helpful to define symptoms and can be useful to assess the systolic pulmonary artery pressure (SPAP) and stratify severity of VHD. This comprehensive review of the literature highlights the role of SE imaging to assess VHD and is needed for the asymptomatic patients with severe VHD or symptomatic patients with non-severe VHD in the context of PH-LHD. The focus of patient evaluation should be on identifying patients with significant underlying valvular heart disease and referring in a timely manner for VHD treatment per society guidelines as pharmacologic pulmonary vasodilator therapy for PH-LHD has not shown efficacy as seen in other forms of PH

Exercise Testing in Mitral Regurgitation.

Mitral regurgitation (MR) is the second most common valvular heart disease referred for corrective surgery. Diagnostic and management dilemmas are not uncommon when dealing with MR patients. Exercise testing plays an important role in sorting out some of these clinical challenges. In primary asymptomatic MR, exercise testing allows symptom assessment, confident link of symptoms to valve disease severity, safe deferral of surgery for the next 1-year in patients with preserved exercise capacity, insights into the mechanism of exercise-induced dyspnea and helps in individual risk stratification. Moreover, exercise testing in the form of exercise stress echocardiography is also useful in the evaluation of patients with secondary ischemic MR for risk stratification as well as for the detection of patients with moderate ischemic MR in whom mitral valve repair at the time of surgical revascularization may add benefit