N-terminal pro brain natriuretic peptide predicts both all-cause and cardiovascular disease mortality in Japanese hemodialysis patients.

BACKGROUND: The association between N-terminal pro brain natriuretic peptide (NT-proBNP) level and long-term mortality in Japanese hemodialysis patients has not been fully assessed. METHODS: This prospective, multicenter study included 1428 hemodialysis outpatients. Baseline NT-proBNP levels were measured at the first hemodialysis session of the week and participants were followed for 5 years. The areas under the curve were calculated from receiver operating characteristic curves. Groups determined by quartiles of baseline NT-proBNP level were assessed by the Kaplan-Meier method and log-rank test. The association between NT-proBNP level and mortality was assessed using multivariate Cox proportional hazards models. RESULTS: During the 5-year follow-up, we observed 370 deaths and 256 censored cases. The areas under the curve of pre-hemodialysis NT-proBNP for all-cause mortality and cardiovascular disease mortality after 1 year were 0.75 and 0.78, respectively, and significantly greater than the areas under the curve at the 3- and 5-year follow-up. Cut-off values for all-cause mortality and cardiovascular disease mortality after 1 year were 4550 and 5467 ng/L, respectively (sensitivity: 82% and 81%; specificity: 59% and 64%). Kaplan-Meier survival analysis showed that the group with pre-hemodialysis NT-proBNP ≥ 8805 ng/L had increased all-cause mortality (P < 0.001) and cardiovascular disease mortality (P < 0.001). Finally, multivariate Cox analysis showed that NT-proBNP level was associated with all-cause mortality (P < 0.001) and cardiovascular disease mortality (P = 0.004) independently from other clinical parameters. CONCLUSION: NT-proBNP is a useful marker to predict both all-cause and cardiovascular disease mortality in hemodialysis patients.

Hypo-attenuated leaflet thickening and reduced leaflet motion after the implantation of the Perceval S sutureless bioprosthesis.

Hypo-attenuated leaflet thickening (HALT) is gaining attention as a relatively common issue after surgical or transcatheter aortic valve replacement (AVR). However, only a few reports have described HALT in sutureless bioprosthesis, which has emerged as a promising tool with excellent hemodynamics and enhanced implantability. We herein report a 75-year-old woman who underwent quintuple coronary artery bypass grafting and sutureless AVR with a Perceval S bioprosthesis (LivaNova PLC, London, UK). Despite an uneventful perioperative course, her recovery was slow with persistent pleural effusion. Echocardiography revealed an increased transvalvular pressure gradient, and HALT was confirmed by computed tomography. The patient received aggressive anticoagulation therapy with resolution of the HALT and made an uneventful recovery. Current guidelines provide no specific recommendations for peri-procedural antithrombotic therapy for sutureless AVR. However, HALT is not rare after sutureless AVR and can lead to significant clinical consequences. In this case, aggressive anticoagulation therapy with systemic heparinization was effective as HALT treatment following early post-sutureless AVR. Further investigation is required to determine the optimal antithrombotic strategy for sutureless AVR.

Sutureless aortic valve replacement with concomitant aortic arch reconstruction.

Sutureless aortic valve replacement (AVR) offers an alternative approach to the standard AVR in aortic valve disease. We herein report a case of an 82-year-old woman with severe aortic insufficiency and a persistent type 1 endoleak following a thoracic endovascular aortic repair, who underwent successful combined aortic arch reconstruction and sutureless AVR. The bioprosthesis, Perceval (LivaNova PLC, London, UK), a self-anchoring, self-expanding, sutureless valve, which can be implanted in selected patients with aortic insufficiency was used. Although the patient was frail and at a high risk of open-heart surgery, she had an uneventful postoperative course. Hence, Perceval may be a useful option for combined aortic arch reconstruction and aortic valve surgery in high-risk elderly patients.

A case of immunotactoid glomerulopathy with false-negative IgG staining.

BACKGROUND: Immunotactoid glomerulopathy (ITG) is a rare glomerulonephritis characterized by microtubular deposits. Immunofluorescence findings are necessary to differentiate ITG from other proliferative glomerular diseases. The characteristic tubular structure on electron microscopy is essential for a definitive diagnosis, and the diameter of the structure has been traditionally used for differentiating between ITG and other types of glomerulonephritis with organized deposits. In recent years, the disease concept of monoclonal gammopathy of renal significance, which is associated with M protein produced by plasma cell tumors, has been proposed. CASE PRESENTATION: This was a peculiar case of ITG with underlying monoclonal gammopathy in which IgG showed a false-negative result with immunofluorescence using frozen sections. Additional examinations using a different clone of the anti-IgG antibody revealed typical IgG staining. C4d was strongly positive, consistent with immune complex type glomerulonephritis. CONCLUSIONS: This case highlights unusual features of ITG, and provides a practical hint to avoid a diagnostic pitfall.

Favorable effect of bortezomib in dense deposit disease associated with monoclonal gammopathy: a case report.

BACKGROUND: Complement component 3 (C3) glomerulopathy, which includes dense deposit disease (DDD) and C3 glomerulonephritis, is caused by dysregulation of the alternative complement pathway. In most cases, C3 glomerulopathy manifests pathologically with membranoproliferative glomerulonephritis-like features. An association between C3 glomerulopathy and monoclonal gammopathy was recently reported in several cases, raising the possibility that C3 glomerulopathy is the underlying pathological process in monoclonal gammopathy of renal significance. CASE PRESENTATION: We herein report a case of monoclonal gammopathy-induced DDD that improved histologically and clinically with chemotherapy including bortezomib. Our case is the first in which treatment response can be linked to the histological response. Potential pathological insights are also discussed. CONCLUSIONS: Rapid and efficient chemotherapy has the potential to limit renal damage in monoclonal gammopathy-associated DDD.

Surgical repair of an aortoesophageal fistula after salvage thoracic endovascular aortic repair: a case report.

BACKGROUND: An aortoesophageal fistula can prove to be fatal. Salvage thoracic endovascular aortic repair as a bridging therapy and radical surgery with thoracotomy should be considered while treating aortoesophageal fistula without spontaneous closure. Moreover, it is essential to select a technique that reduces the risk of reinfection. Here we report a rare case of a ruptured thoracic aortic aneurysm related to esophageal perforation by a fish bone that led to massive hematemesis and shock, and the surgical treatment of an aortoesophageal fistula that developed after salvage thoracic endovascular aortic repair. CASE PRESENTATION: A 70-year-old Japanese female patient was admitted with hematemesis, thoracic pain, and shock related to esophageal perforation of a ruptured descending aortic aneurysm caused by fish bone aspiration and esophageal perforation 1 month previously. An emergency thoracic endovascular aortic repair was performed. Postoperatively, an aortoesophageal fistula that remained open and a food intake-related increase in the inflammatory response was noted. Radical blood-vessel prosthesis implantation and fistula closure were performed. The patient's postoperative course was favorable and the patient was discharged 22 days after the blood vessel prosthesis implantation. CONCLUSION: Such a case of rupture of a descending aortic aneurysm related to perforation by a fish bone and an aortoesophageal fistula is considerably rare. Thus, we report the therapeutic strategy of this particular case and review the relevant literature.

Characteristics of Physicochemical Properties of Chalky Grains of Japonica Rice Generated by High Temperature during Ripening.

Global warming has caused devastating damage to starch biosynthesis, which has led to the increase in chalky grains of rice. This study was conducted to characterize the qualities of chalky rice grains and to develop the estimation formulae for their quality damage degree. We evaluated the chalkiness of 40 Japonica rice samples harvested in 2019, in Japan. Seven samples with a high ratio of chalky rice grains were selected and divided into two groups (whole grain and chalky grain). As a results of the various physicochemical measurements, it was shown that the surface layer hardness (H1) of cooked rice grains from chalky grains was significantly lower, and their overall hardness was significantly lower than those from the whole grains. In addition, α- and ß-amylase activities, and sugar contents of the chalky rice grains were significantly higher than those of the whole rice grains. The developed estimation formula for the degree of retrogradation of H1 based on the α-amylase activities and pasting properties, showed correlation coefficients of 0.84 and 0.81 in the calibration and validation tests, respectively. This result presents the formula that could be used to estimate and to characterize the cooking properties of the rice samples ripened under high temperature.

Off-Pump Resection of an Epicardial Cyst by Using a Minimally Invasive Approach.

A 25-year-old man presented with palpitations and subsequently received a diagnosis of a large epicardial cyst (6.8 × 3.8 cm) originating from the left ventricle. The cyst compressed the left atrium and ventricle and led to left ventricular diastolic dysfunction. Contrast-enhanced chest computed tomography revealed that the circumflex artery passed over or through the cyst. We successfully resected the cyst without using cardiopulmonary bypass through a left mini-thoracotomy with thoracoscopic assistance. The diastolic dysfunction improved after the procedure. Most epicardial cysts may be treated in this fashion if the cyst is located in the left side of the heart.

Graft Traction Resolved Left Ventricular Outflow Tract Obstruction.

We admitted a 76-year-old woman for treatment of an ascending aortic aneurysm with left ventricular outflow tract (LVOT) obstruction and systolic anterior motion (SAM) of the mitral valve. Echocardiography showed an elevated velocity of the LVOT flow with a sigmoid septum. Mild mitral regurgitation was also detected due to SAM. We performed a graft replacement of the ascending aorta, after which the LVOT obstruction and SAM were resolved. We report a case in which the traction of a graft likely released the compression on the aortic root and ventricular septum.

Repair of pacemaker lead-induced right ventricular perforation via a left mini-thoracotomy.

Cardiac perforation is a rare but serious and life-threatening complication of permanent pacemaker implantation, with an incidence of 0.1-6%. Surgery is usually performed through a median sternotomy; however, sternotomy-related morbidity remains a concern. Herein, we report a case of surgical repair performed via a left mini-thoracotomy for a right ventricular perforation caused by implantation of a permanent pacemaker lead in a 56-year-old woman. Through the left fifth intercostal space, the pacemaker lead was observed to have penetrated the left ventricular myocardium, reaching the pericardium. The lead had passed through the right ventricle and the inferior ventricular septum and protruded from the left ventricular myocardium. After pacemaker lead removal, a dark blow-out type hemorrhage occurred; hence, repair was performed using a pair of pledgeted Mattress sutures. In conclusion, left mini-thoracotomy provides an adequate surgical field and has less impact on hemodynamics when operating at the cardiac apex. .

Perceval sutureless aortic valve replacement after ascending aortic replacement.

In patients with a narrow sinotubular junction, small sinus of Valsalva, or extensibility loss in the aortic root, aortic valve replacement (AVR) with a standard valve is challenging due to limited surgical field. Detailed preoperative measurements of the aortic root render performing AVR using the Perceval valve easy.

Inactive large cerebral hemorrhage is not exacerbated by the adjustment of anticoagulation post open-heart surgery.

Optimal timing of open-heart surgery for the treatment of patients with cerebral hemorrhage remains controversial because systemic heparinization may lead to catastrophic bleeding. Several recent reports have shown that patients who undergo open-heart surgery .within a few weeks of cerebral hemorrhage have a much lower risk of exacerbated bleeding than previously considered. Herein, we report a case of left atrial myxoma and large hemorrhagic embolic stroke, which was successfully operated on with no exacerbation of cerebral hemorrhage. Careful assessment of time-course changes in cerebral hemorrhage by neurological imaging and adjustment of anticoagulation can help prevent the exacerbation of postoperative cerebral hemorrhage and neurological deterioration. .

Acute tubulointerstitial nephritis and IgM deposits on glomerular capillary walls after immunotherapy with nivolumab for metastatic renal cell carcinoma.

Nivolumab is an anti-programmed cell death-1 antibody that is utilized as an immune checkpoint inhibitor for several malignancies. However, this agent is associated with immune-related adverse events (irAEs), mainly in the spectrum of autoimmune disease including interstitial pneumonia, colitis, type 1 diabetes, and renal impairment. We herein present the case of a 59-year-old man with renal cell carcinoma who developed worsening renal function approximately 4 months after initiation of nivolumab. Urinalysis showed proteinuria and microscopic hematuria along with increase levels of N-acetyl-ß-D-glucosaminidase. Renal biopsy revealed acute tubulointerstitial nephritis and thickening of the glomerular basement membranes. Immunofluorescence showed granular IgM deposits in capillary loops. We initiated high-dose prednisolone therapy with nivolumab, which improved renal function and achieved complete remission of proteinuria. Although renal irAEs are considered to be rare and glomerulonephropathy is not typical presentation, physicians need the close monitoring of renal function and urinalysis in patients under immunotherapy with this agents. In addition, our case provides a possible link between nivolumab and immune-mediated glomerulonephropathy.

Novel Asp511Thr mutation in McArdle disease with acute kidney injury caused by rhabdomyolysis.

McArdle disease (glycogen storage disease type V) is a rare hereditary metabolic myopathy. It can be overlooked clinically because it often presents as chronic asymptomatic hypercreatine phosphokinasemia (hyperCKemia). However, vigorous exercise or infections can trigger severe rhabdomyolysis. We present the case of a patient with long-term idiopathic hyperCKemia who, after contracting an upper respiratory tract infection, developed severe rhabdomyolysis and acute kidney injury. Upon hemodialysis, his renal function recovered and CK levels fell to below baseline, and maintenance therapy with vitamin B6 was also started. A molecular diagnosis of McArdle disease was subsequently made. Whole-exome sequencing revealed homozygous c1538delG (p.Asp511Thr fs*28) mutations in the PYGM gene, which was a novel mutation. Therefore, when investigating idiopathic hyperCKemia, glycogen storage disorders should also be considered.