Rituximab and pericardiectomy with waffle procedure in constrictive pericarditis due to IgG4-related disease: A case report.

We should consider IgG4-related disease (IGRD) as one of the potential causes of constrictive pericarditis. In patients with constrictive pericarditis due to IGRD, the combination of surgical treatment and immunosuppressive therapy may be an effective strategy.

[Left Main Coronary Artery-to-left Atrium Fistula with Aneurysmal Dilatation:Report of a Case].

Coronary artery fistula (CAF) is a relatively rare disease, many of which are asymptomatic and are not indicated for surgery. However, CAF's patients with angina, heart failure due to shunt, and fistula hemangiomas are usually indicated for surgical treatment. A 49-year-old woman with severe congestive heart failure was found to have a fistula from the main trunk of the left coronary artery to the left atrium, and a fistula aneurysm. This patient had heart failure due to shunt blood flow, and also had a fistula aneurysm, so surgical treatment was indicated. During surgery, the fistula aneurysm was incised and the origin and opening of the fistula were closed without any problems. The postoperative course is good, and as of three years after the operation, the patient is being followed up at an outpatient clinic without recurrence of shunt flow and heart failure.

A flat-lying dimer as a key intermediate in NO reduction on Cu(100).

The reaction of nitric oxide (NO) on Cu(100) is studied by scanning tunneling microscopy, electron energy loss spectroscopy and density functional theory calculations. The NO molecules adsorb mainly as monomers at 64 K, and react and dissociate to yield oxygen atoms on the surface at ∼70 K. The temperature required for the dissociation is significantly low for Cu(100), compared to those for Cu(111) and Cu(110). The minimum energy pathway of the reaction is via (NO)2 formation, which converts into a flat-lying ONNO and then dissociates into N2O and O with a considerably low activation energy. We propose that the formation of (NO)2 and flat-lying ONNO is the key to the exceptionally high reactivity of NO on Cu(100).

Metallic conduction through van der Waals interfaces in ultrathin [Formula: see text] films.

While the van der Waals (vdW) interface in layered materials hinders the transport of charge carriers in the vertical direction, it serves a good horizontal conduction path. We have investigated electrical conduction of few quintuple-layer (QL) [Formula: see text] films by in situ four-point probe conductivity measurement. The impact of the vdW (Te-Te) interface appeared as a large conductivity increase with increasing thickness from 1 to 2 QL. Angle-resolved photoelectron spectroscopy and first-principles calculations reveal the confinement of bulk-like conduction band (CB) state into the vdW interface. Our analysis based on the Boltzmann equation showed that the conduction of the CB has a long mean free path compared to the surface-state conduction. This is mainly attributed to the spatial separation of the CB electrons and the donor defects located at the Bi sites.

X-ray Diffraction Studies on the Structural Origin of Dynamic Tension Recovery Following Ramp-Shaped Releases in High-Ca Rigor Muscle Fibers.

It is generally believed that during muscle contraction, myosin heads (M) extending from myosin filament attaches to actin filaments (A) to perform power stroke, associated with the reaction, A-M-ADP-Pi → A-M + ADP + Pi, so that myosin heads pass through the state of A-M, i.e., rigor A-M complex. We have, however, recently found that: (1) an antibody to myosin head, completely covering actin-binding sites in myosin head, has no effect on Ca2+-activated tension in skinned muscle fibers; (2) skinned fibers exhibit distinct tension recovery following ramp-shaped releases (amplitude, 0.5% of Lo; complete in 5 ms); and (3) EDTA, chelating Mg ions, eliminate the tension recovery in low-Ca rigor fibers but not in high-Ca rigor fibers. These results suggest that A-M-ADP myosin heads in high-Ca rigor fibers have dynamic properties to produce the tension recovery following ramp-shaped releases, and that myosin heads do not pass through rigor A-M complex configuration during muscle contraction. To obtain information about the structural changes in A-M-ADP myosin heads during the tension recovery, we performed X-ray diffraction studies on high-Ca rigor skinned fibers subjected to ramp-shaped releases. X-ray diffraction patterns of the fibers were recorded before and after application of ramp-shaped releases. The results obtained indicate that during the initial drop in rigor tension coincident with the applied release, rigor myosin heads take up applied displacement by tilting from oblique to perpendicular configuration to myofilaments, and after the release myosin heads appear to rotate around the helical structure of actin filaments to produce the tension recovery.

Identification of single-layer metallic structure of indium on Si(1 1 1).

We have studied the In/Si(1 1 1) ([Formula: see text])-hex and striped phases by low-energy electron diffraction (LEED) and angle-resolved photoelectron spectroscopy (ARPES). The two phases are formed by different processes, and hence the different names have been used conventionally. We, however, found that LEED I-[Formula: see text] curves of the two phases agree with each other, indicating that they have an identical atomic structure. Our ARPES measurement revealed that the ([Formula: see text])-hex phase has metallic surface states. The observed Fermi surface was found to be very similar to the theoretical one calculated for an indium single-layer model.

Atomic-scale study of the formation of sodium-water complexes on Cu(110).

We observed individual sodium (Na) atoms and their complexes with water molecules on Cu(110) with scanning tunneling microscopy at 6 K. We induced the reaction of a Na adatom with one or two water molecules, which yielded two kinds of Na-water complexes. Density functional theory calculations were performed to study the structure of the complexes, which revealed that the water molecules are bonded to a Na atom along the [11[combining macron]0] direction via an oxygen atom with the hydrogen atoms pointing toward the Cu atoms of the surface. The 1 : 1 Na-water complex is stablized by 225 meV upon bond formation, and the ligand water moves back and forth around the Na atom. The complex can accommodate another water molecule to yield a 1 : 2 Na-water complex with an energy gain of 214 meV. The atomic-scale identification of the alkali-water complexes would give fundamental insights into the hydration process of alkali cations and their specific adsorption onto metal electrodes.

Tension Recovery following Ramp-Shaped Release in High-Ca and Low-Ca Rigor Muscle Fibers: Evidence for the Dynamic State of AMADP Myosin Heads in the Absence of ATP.

During muscle contraction, myosin heads (M) bound to actin (A) perform power stroke associated with reaction, AMADPPi → AM + ADP + Pi. In this scheme, A • M is believed to be a high-affinity complex after removal of ATP. Biochemical studies on extracted protein samples show that, in the AM complex, actin-binding sites are located at both sides of junctional peptide between 50K and 20K segments of myosin heavy chain. Recently, we found that a monoclonal antibody (IgG) to the junctional peptide had no effect on both in vitro actin-myosin sliding and skinned muscle fiber contraction, though it covers the actin-binding sites on myosin. It follows from this that, during muscle contraction, myosin heads do not pass through the static rigor AM configuration, determined biochemically and electron microscopically using extracted protein samples. To study the nature of AM and AMADP myosin heads, actually existing in muscle, we examined mechanical responses to ramp-shaped releases (0.5% of Lo, complete in 5ms) in single skinned rabbit psoas muscle fibers in high-Ca (pCa, 4) and low-Ca (pCa, >9) rigor states. The fibers exhibited initial elastic tension drop and subsequent small but definite tension recovery to a steady level. The tension recovery was present over many minutes in high-Ca rigor fibers, while it tended to decrease quickly in low-Ca rigor fibers. EDTA (10mM, with MgCl2 removed) had no appreciable effect on the tension recovery in high-Ca rigor fibers, while it completely eliminated the tension recovery in low-Ca rigor fibers. These results suggest that the AMADP myosin heads in rigor muscle have long lifetimes and dynamic properties, which show up as the tension recovery following applied release. Possible AM linkage structure in muscle is discussed in connection with the X-ray diffraction pattern from contracting muscle, which is intermediate between resting and rigor muscles.

Adsorption and reaction of H2S on Cu(110) studied using scanning tunneling microscopy.

Using low-temperature scanning tunneling microscopy (STM), the adsorption and reaction of hydrogen sulfide (H2S) and its fragments (SH and S) on Cu(110) are investigated at 5 K. H2S adsorbs molecularly on the surface on top of a Cu atom. With voltage pulses of STM, it is possible to induce sequential dehydrogenation of H2S to SH and S. We found two kinds of adsorption structures of SH. The short-bridge site is the most stable site for SH, while the long-bridge site is the second. Density functional theory calculations show that the S-H axis is inclined from the surface normal for both species. The reaction of H2S with OH and O was directly observed to yield SH and S, respectively, providing a molecular-level insight into catalyst poisoning.

Controlled switching of single-molecule junctions by mechanical motion of a phenyl ring.

Mechanical methods for single-molecule control have potential for wide application in nanodevices and machines. Here we demonstrate the operation of a single-molecule switch made functional by the motion of a phenyl ring, analogous to the lever in a conventional toggle switch. The switch can be actuated by dual triggers, either by a voltage pulse or by displacement of the electrode, and electronic manipulation of the ring by chemical substitution enables rational control of the on-state conductance. Owing to its simple mechanics, structural robustness, and chemical accessibility, we propose that phenyl rings are promising components in mechanical molecular devices.

Differing relationship between hypercholesterolemia and a bicuspid aortic valve according to the presence of aortic valve stenosis or aortic valve regurgitation.

OBJECTIVES: To assess the difference in hyperlipidemia between patients with bicuspid aortic valve (BAV) and those with a normal aortic valve (NAV), and to compare aortic valve stenosis (AS), with aortic valve regurgitation (AR). METHODS: Among 32 patients with BAV and 142 patients with NAV who underwent aortic valve replacement, 81 patients had AR and 91 patients had AS. The preoperative clinical characteristics were compared between the BAV and NAV patients. Patients with replacement of the ascending aorta were included, and those who underwent combined valvular surgery, coronary artery bypass grafting, or statin treatment were excluded. RESULTS: The proportions of females patients (p = 0.42), patients with diabetes (p = 0.26) and patients on dialysis (p = 0.69) were similar in the two groups. Mean age was significantly lower, the mean diameter of the ascending aorta was significantly larger, and the rate of surgical intervention for the ascending aorta was significantly higher in the BAV group than in the NAV group (all p < 0.0001). The mean levels of low-density lipoprotein cholesterol (LDL) (p < 0.0001) and total cholesterol (TC) (p = 0.0003) were significantly higher in the BAV group than in the NAV group, in the analysis of only patients with AS, whereas these levels did not differ significantly between the groups, when only patients with AR were considered. CONCLUSION: BAV with AS is associated with hypercholesterolemia. However, BAV with AR was not associated with hypercholesterolemia.

Minimally invasive direct coronary artery bypass surgery with right gastroepiploic artery for redo patients.

Coronary artery bypass grafting (CABG) has been widely performed for coronary artery disease. Therefore, cases requiring reoperative CABG are increasing. We performed a minimally invasive direct coronary artery bypass (MIDCAB) procedure on four patients, as reoperative CABG surgery for the right coronary artery (RCA), employing the right gastroepiploic artery (RGEA). The target sites were the distal RCA in two patients and the posterior descending (PD) branch in the other two. Complete revascularization was accomplished in all patients without sternotomy, cardiopulmonary bypass (CPB), or blood transfusion. The mean operative time was 3.0 h (range: 2.4-3.7 h). Postoperative coronary angiography showed all grafts to be patent. All patients were discharged without postoperative complications and remained free from cardiac events during a mean follow-up period of 1.5 years (range: 0.5-3.0 years). MIDCAB for the RCA, employing the RGEA via a subxiphoid incision showed, excellent revascularization in redo CABG cases. This technique is a safe and effective method for redo cases.

A case of IgG4-related kidney disease complicated by eosinophilic lung disease.

IgG4-related disease is a systemic chronic inflammatory disorder characterized by a high blood level of IgG4 and the organ injuries by marked infiltration of IgG4-positive plasma cells and fibrosis. A 71-year-old male was hospitalized for a cough, malaise and anorexia. IgG4-related disease was suspected due to marked elevation of the serum IgG4 level. However, on lung biopsy, only eosinophil infiltration was demonstrated with no plasma cell infiltration. Otherwise abdominal contrast-enhanced CT showed mild enlargement of the bilateral kidneys and many differed contrasted areas and FDG PET-CT. Moreover, renal biopsy specimens showed typical tubulointerstitial nephritis with a large number of IgG4-positive plasma cells infiltration (the IgG4/IgG-positive cell rate, 89 %) and fibrosis. We diagnosed this patient as typical IgG4-related kidney disease. He was treated by the moderate dose of prednisolone (0.8 mg/kg/day) alone, and showed prompt response in the clinical condition, and both the lung and kidney lesions. In this case, it was useful for diagnosis of IgG4-related diseases to evaluate an image such as abdominal contrast-enhanced CT and FDG PET-CT. Our case might be one of the possible patterns of IgG4-related lung diseases. In addition, we thought that there might be an association between hypereosinophilia and IgG4-related kidney disease.

Clinical significance of serum and urinary soluble urokinase receptor (suPAR) in primary nephrotic syndrome and MPO-ANCA-associated glomerulonephritis in Japanese.

BACKGROUND: The soluble urokinase receptor (suPAR) has been implicated as a cause of primary focal segmental glomerulosclerosis (FSGS). However, the clinical significance of suPAR in glomerular diseases currently remains unclear. METHODS: In this retrospective single-center cohort study, we investigated serum (s-) and urinary (u-) suPAR in patients with primary nephrotic syndrome (NS) (serum/urine: 37/32 cases) and MPO-ANCA-associated glomerulonephritis (ANCA-GN) (serum/urine: 13/11 cases). RESULTS: In pretreatment s- and u-suPAR, no significant differences were observed between the primary NS and ANCA-GN groups or among the pathological types of primary NS. An inverse correlation was noted between pretreatment s-suPAR and eGFR in the primary NS and ANCA-GN groups. A positive correlation was noted between pretreatment u-suPAR and proteinuria in the primary NS group. Furthermore, time-course changes in s- and u-suPAR over 2 months after therapy were associated with the therapeutic responsiveness of primary NS, particularly the differentiation of MCNS from FSGS (s-suPAR: AUC-ROC = 0.905, p = 0.007; u-suPAR: AUC-ROC = 0.816, p = 0.048). In the ANCA-GN group, a positive correlation was found between pretreatment s-suPAR and clinical severity or crescent formation, whereas u-suPAR was not correlated with these parameters. CONCLUSION: S- and u-suPAR after therapy may serve as clinical markers to judge the treatment response of untreated NS and differentiate MCNS from FSGS, but not in pretreatment patients. S-, but not u-suPAR may predict the severity of and crescent formation in ANCA-GN.

[Familial Annulo-aortic Ectasia without Marfan Syndrome].

Marfan syndrome is a hereditary disease that presents ocular, skeletal, and cardiovascular abnormalities. In recent years, there have been several reports of patients with familial cardiovascular disease but no physical features of Marfan syndrome. We encountered 3 cases of familial annulo-aortic ectasia (AAE). Their father had also had aortic regurgitation, and died during surgery 10 years before. No case demonstrated any physical characteristics of Marfan syndrome or any other connective tissue disease. All cases were operated successfully. One case showed cystic medial necrosis, and 2 cases showed degenerative change. The present report suggests that familial AAE may be caused by weakness of the aortic wall related to heredity. If AAE is left untreated, it can lead to aortic dissection. Thus, we recommend that patients with familial AAE should undergo screening and follow-up similar to patients with Marfan syndrome.

Clinicopathological characteristics of M-type phospholipase A2 receptor (PLA2R)-related membranous nephropathy in Japanese.

BACKGROUND: Recent studies have suggested that assessments of serum antibodies against M-type phospholipase A2 receptor (PLA2R) and the glomerular expression of PLA2R antigen in biopsy specimens are useful for the diagnosis of primary membranous nephropathy (MN). In this study, we assessed both of them and investigated the clinicopathological characteristics of PLA2R-related Japanese MN. METHODS: We retrospectively enrolled 22 primary and 3 secondary Japanese patients whose serum samples and renal specimens were collected before treatment. According to the findings of serum antibodies and antigen in glomeruli, the primary MN patients were classified into PLA2R-related or -unrelated MN. We compared their clinicopathological findings, including IgG subclass staining, and electron microscopic findings, and evaluated the predictors of proteinuria remission. RESULTS: In primary MN, 16 patients (73 %) were classified into the PLA2R-related group, and 6 patients into the PLA2R-unrelated group. There was no significant difference in baseline laboratory data and electron microscopic findings, except for eGFR and serum IgG levels. IgG4-dominant deposition was more common in the related group (63 vs. 0 %). The 10 PLA2R-related patients with dominant IgG4 deposition had a lower rate and prolonged time in remission compared with the 6 PLA2R-related patients with non-dominant IgG4 (log-rank, p = 0.032). Furthermore, dominant IgG4 deposition was an unfavorable predictor of remission by multivariable Cox proportional hazard analysis. CONCLUSIONS: Assessments of both serum PLA2R antibodies and PLA2R antigen in glomeruli were more sensitive for the diagnosis of PLA2R-related MN, and among affected Japanese patients, those with dominant IgG4 deposition had worse clinical outcomes.

A case of high-output heart failure caused by a femoral arteriovenous fistula after endovenous laser ablation treatment of the saphenous vein.

Endovenous laser ablation treatment has become the less invasive therapeutic choice for the treatment of superficial venous insufficiency and varicose veins. A 64-year-old woman presented at our hospital with varicose veins and prior endovenous laser ablation treatment. The patient had high-output heart failure caused by a right femoral arteriovenous fistula. She was treated medically and underwent an open repair of the right superficial femoral artery and the right femoral vein with complete resolution of the arteriovenous fistula and heart failure. Here, we have reviewed the literature and discussed possible causes of the complication of arteriovenous fistula after endovenous laser ablation treatment.

[Redo double valve replacement in a patient with bioprosthetic valve malfunction by pannus formation; report of a case].

A 76-year-old female, who had received Carpentier-Edwards perimount (CEP) pericardial bioprostheses for aortic and mitral valves 6 years before, was diagnosed with reduced mobility of the valvular cusps by echocardiogram 2 years after the surgery. The symptoms of heart failure gradually aggravated. Significant stenosis and regurgitation were observed of the prosthetic valves, and a second surgery was performed. Calcification and severe pannnus formation were observed of both CEP valves that were excised.

Imaging molecular interaction of NO on Cu(110) with a scanning tunneling microscope.

Molecular interaction on metal surfaces is one of the central issues of surface science for the microscopic understanding of heterogeneous catalysis. In this Personal Account, I review the recent studies on NO/Cu(110) employing a scanning tunneling microscope (STM) to probe and control the molecule-molecule interaction on the surface. An individual NO molecule was observed as a characteristic dumbbell-shaped protrusion, visualizing the 2π* orbital. By manipulating the intermolecular distance with the STM, the overlap of the 2π* orbital between two NO molecules was controlled. The interaction causes the formation of the bonding and antibonding orbitals below and above the Fermi level, respectively, as a function of the intermolecular distance. The 2π* orbital also plays a role in the reaction of NO with water molecules. A water molecule donates a H-bond to NO, giving rise to the down-shift of the 2π* level below the Fermi level. This causes electron transfer from the substrate to NO, weakening, and eventually rupturing, the N-O bond. The facile bond cleavage by water molecules has implications for the catalytic reduction of NO under ambient conditions.