Clinical Efficacy and Safety of Low-Dose Pemafibrate in Patients With Severe Renal Impairment: A Retrospective Study.

BACKGROUND: The management of hypertriglyceridemia in patients with chronic kidney disease (CKD) is important. Pemafibrate, a novel selective peroxisome proliferator-activated receptor-alpha modulator with less toxic effects on liver and kidney function than those of other fibrates, has recently been approved for the treatment of patients with an estimated glomerular filtration rate (eGFR) lower than 30 mL/min/1.73 m2. However, the efficacy and safety of pemafibrate in patients with severe renal impairment have not yet been established. METHODS: This single-center, retrospective observational study included 12 outpatients with CKD and hypertriglyceridemia, who were newly started on low-dose pemafibrate (0.1 mg/day) treatment between December 2021 and May 2023 and whose eGFRs were less than 30 mL/min/1.73 m2 at baseline. Blood samples were collected before and at 12 weeks after pemafibrate treatment. RESULTS: After 12 weeks of treatment, the serum triglyceride level was significantly decreased, whereas the high-density lipoprotein cholesterol level was significantly increased. The serum alanine aminotransferase, alkaline phosphatase, gamma-glutamyl transpeptidase, and uric acid levels were also significantly decreased, without worsening of the eGFR and serum creatinine levels. In the subgroup analysis, there were no significant differences in the changes in clinical parameters regardless of statin use and CKD stage at baseline. CONCLUSIONS: Low-dose pemafibrate administration in patients with severe renal impairment resulted in significant improvements in triglyceride, high-density lipoprotein cholesterol, and serum uric acid levels, and liver function, without adverse events.

Reducing the Sodium Intake of Patients With Chronic Kidney Disease Through Education and Estimating Salt Excretion: A Propensity Score Matching Analysis.

BACKGROUND: Japanese people traditionally consume high quantities of salt. This study aimed to investigate the effects of educating patients with chronic kidney disease (CKD) on simple methods for reducing their daily dietary salt intake. METHODS: This single-center, retrospective observational study included 115 outpatients with CKD at Kawashima Hospital (Tokushima, Japan). One physician routinely recommended that patients should reduce their salt intake and provided tips for salt restriction. The physician estimated the patients' daily salt intake using spot urine samples at each medical examination (education group; n = 61). The other physicians' outpatients only received dietary guidance on recommended salt intake (control group; n = 54). The estimated 24-hour urinary sodium excretion (24hUNaV) and 24-hour potassium excretion (24hUKV) were calculated using Tanaka's equation. RESULTS: Estimated 24hUNaV was positively correlated with body mass index (BMI), estimated 24hUKV, and urinary Na/K ratio. The patients in the education group were younger and had a lower BMI, higher estimated glomerular filtration rate, and lower systolic blood pressure (SBP). Using 38 pairs of patients obtained by propensity score matching with these variables, estimated 24hUNaV, estimated 24hUKV, and diastolic blood pressure (DBP) after one year were significantly reduced in the education group. CONCLUSION: A simple salt reduction education may reduce salt intake in outpatients with CKD.

X-linked Alport Syndrome with Type IV Collagen α5 Chain Staining Revealing Normal Expression in the Glomerular Basement Membrane and Negative on Bowman's Capsule and Distal Tubular Basement Membrane: A Case Report.

X-linked Alport syndrome is a hereditary progressive renal disease resulting from the disruption of collagen α3α4α5 (IV) heterotrimerization caused by pathogenic variants in the COL4A5 gene. This study aimed to report a male case of X-linked Alport syndrome with a mild phenotype accompanied by an atypical expression pattern of type IV collagen α5 [α5 (IV)] chain in glomerulus. A 38-year-old male presented with proteinuria (2.3 g/day) and hematuria. He has been detected urinary protein and occult blood since childhood. A renal biopsy was performed at the age of 29 years; however, a diagnosis of Alport syndrome was not considered. A renal biopsy 9 years later revealed diffuse thinning and lamellation of the glomerular basement membrane. Α staining for α5 (IV) revealed a normal expression pattern in the glomerular basement membrane and a complete negative expression in Bowman's capsule and distal tubular basement membrane. Using next-generation sequencing, we detected a COL4A5 missense variant within exon 35 (NM_000495.5: c.3088G>A, p. G1030S). The possibility of X-linked Alport syndrome should be considered when negative expression of α5 (IV) staining on Bowman's capsule was observed.

Successful treatment of nephrotic syndrome due to pregnancy-related crescentic IgA nephropathy: a case report.

BACKGROUND: Crescentic immunoglobulin A (IgA) nephropathy, defined as > 50% of the glomeruli with crescents, often has a poor renal prognosis. Because of the high prevalence of pre-eclampsia in the second trimester of pregnancy, we often fail to investigate the new onset of glomerulonephritis and the aggravation of subclinical nephropathies. We report a case of nephrotic syndrome suggestive of crescentic IgA nephropathy possibly triggered by pregnancy. CASE PRESENTATION: A 33-year-old multipara was referred for persistent proteinuria, hematuria, and hypoalbuminemia two months postpartum. The patient was diagnosed with proteinuria for the first time at 36 weeks of gestation. The patient was normotensive during pregnancy. Renal biopsy revealed crescentic IgA nephropathy, with cellular crescents in 80% of the glomeruli and no global sclerosis. After treatment with pulse steroids followed by high-dose oral glucocorticoids and tonsillectomy, a gradual improvement was seen in proteinuria, hematuria, and hypoalbuminemia. CONCLUSION: Although the precise mechanism remains unclear, pregnancy possibly triggered the new onset of crescentic IgA nephropathy or the aggravation of subclinical IgA nephropathy.

A complicated case of Serratia marcescens catheter-related bloodstream infection misdiagnosed as hypersensitivity reactions to bicarbonate dialysate containing acetate.

Recurrent fever during/post-dialysis can occur due to infectious or non-infectious causes. We present the case of a 79-year-old patient who had persistent post-dialysis fever after long-term tunneled central venous catheterization with acetate-containing bicarbonate dialysate. Drug-induced lymphocyte stimulation test (DLST) was positive for acetate dialysate, and he was suspected of having acetate dialysate-induced hypersensitivity reaction. However, switching to acetate-free dialysate did not attenuate the fever. Since Serratia marcescens had been isolated twice from the blood, catheter-related bloodstream infection (CRBSI) was suspected. The culture of the catheter tip confirmed CRBSI caused by S. marcescens. Elevation of ß-d-glucan levels and appearance of pulmonary nodular shadow on chest computed tomography images indicated complicated fungal infections. Administration of antibiotics and antifungals led to resolution of the pulmonary nodular shadow with attenuation of fever and C-reactive protein levels. DLST for acetate dialysate was negative, and its reuse did not aggravate the symptoms; hence, the first result was considered false-positive. An indwelling catheter is a risk factor for S. marcescens-related CRBSI, which leads to post-dialysis fever. Hypersensitivity reactions to dialysates must be diagnosed considering the clinical course and DLST results.

Successful Treatment of Nephrotic Syndrome Due to Collapsing Focal Segmental Glomerulosclerosis Accompanied by Acute Interstitial Nephritis.

A 39-year-old woman was hospitalized for nephrotic syndrome. Laboratory test results showed increased serum creatinine levels and urinary excretions of beta-2-microglobulin, and N-acetyl-beta-D-glucosaminidase. A renal biopsy revealed collapsing focal segmental glomerulosclerosis (FSGS) and acute interstitial nephritis. Despite treatment with pulse steroid followed by oral high-dose glucocorticoids and cyclosporines, heavy proteinuria persisted. After low-density lipoprotein apheresis (LDL-A) therapy was initiated, her proteinuria gradually decreased, leading to complete remission. A repeat renal biopsy after treatment revealed no collapsing glomeruli. Immediate LDL-A should be performed to treat cases of collapsing FSGS poorly responding to other treatments.

Splenic abscess diagnosed following relapsing sterile peritonitis in a peritoneal dialysis patient: A case report with literature review.

Peritoneal dialysis (PD)-related peritonitis is sometimes complicated with other infections; however, few cases of splenic abscess have been reported. We present the case of a 64-year-old PD patient with complicated splenic abscesses diagnosed following relapsing sterile peritonitis. After PD induction, he presented with turbid peritoneal fluid and was diagnosed with PD-related peritonitis. A plain abdominal computed tomography (CT) did not reveal any intra-abdominal focus of infection. After empiric intravenous antibiotics, the peritoneal dialysate was initially cleared, with a decrease in dialysate white blood cells (WBC) to 20/µL. However, WBC and C-reactive protein (CRP) levels remained elevated. A contrast-enhanced abdominal CT showed two areas of low-density fluid with no enhancement in a mildly enlarged spleen, making it difficult to distinguish abscesses from cysts. Due to relapsing sterile peritonitis, we performed an abdominal ultrasonography, and suspected splenic abscesses due to rapid increase in size. Repeated imaging tests were useful in establishing a diagnosis of splenic abscesses. Considering the persistent elevation of WBC and CRP levels, imaging findings, and episodes of relapsing peritonitis, we comprehensively formed the diagnosis, and performed a splenectomy as a rescue therapy. We should consider the possibility of other infectious foci with persistent inflammation after resolving PD-related peritonitis.

Clinical Characteristics and Risk Factors for Mortality due to Bloodstream Infection of Unknown Origin in Hemodialysis Patients: A Single-Center, Retrospective Study.

INTRODUCTION: Hemodialysis patients are at a high risk of bloodstream infection (BSI). The risk factors for BSI-associated mortality, especially of unknown origin, remain uncertain. BSI of unknown origin is highly prevalent and related to high mortality. The present study aimed to investigate the clinical and microbiological characteristics of BSI and risk factors for BSI-associated mortality, including BSI of unknown origin, in hemodialysis patients. METHODS: This study was a single-center, retrospective study conducted from August 2012 to July 2019 in hemodialysis patients with BSI at Kawashima Hospital. Data related to demographics, clinical parameters, BSI sources, causative microorganisms, and initial treatments were collected from the medical records. The predictors for mortality associated with BSI were evaluated by logistic regression. RESULTS: Among 174 patients, 55 (30.9%) had the infection from unknown origin. The most frequent bacterium was Staphylococcus aureus. Low serum albumin level was an independent predictor of mortality due to BSI (odds ratio [OR]: 0.28, 95% confidence interval [CI]: 0.13-0.59). A lower serum albumin level (≤2.5 g/dL) was associated with poorer mortality. Methicillin-resistant Staphylococcus aureus (MRSA) was independently associated with mortality due to BSI of unknown origin (OR: 6.20, 95% CI: 1.04-37.1); 87.5% cases with BSI of unknown origin due to MRSA were not initially administrated anti-MRSA antibiotics, and in such patients, the mortality rate was 85.7%. CONCLUSIONS: Serum albumin level of 2.5 g/dL is a cutoff value, which could predict the mortality due to BSI in hemodialysis patients. Considering the high mortality rate of MRSA-associated BSI of unknown origin, wherein no focus of infection was identified in the present study, initial empiric treatment should be considered for MRSA-associated BSI of unknown origin.

Parathyroidectomy for tertiary hyperparathyroidism after second kidney transplantation: a case report.

Successful kidney transplantation usually resolves secondary hyperparathyroidism (SHPT). However, some patients fail to normalize, and their condition is often referred to as tertiary hyperparathyroidism (THPT). Surgical consensus on the timing of post-transplant parathyroidectomy (PTX) for THPT has not been reached. Herein, we report a case of a 58-year-old post-transplant woman, considering the concrete timing of PTX for both SHPT and THPT. She initiated hemodialysis with end-stage renal disease at the age of 24, and underwent first kidney transplantation at the age of 28. When peritoneal dialysis (PD) was induced due to the worsening kidney function at the age of 50, the serum intact parathyroid hormone (iPTH) level remarkably increased (2332 pg/mL). Although cinacalcet was administered, the patient's iPTH levels were not sufficiently suppressed for seven years. Diagnostic images including ultrasound, computed tomography, and 99mTc-methoxyisobutylisonitrile scintigraphy indicated THPT as the reason for prolonged post-transplant hypercalcemia. Therefore, PTX was performed 14 months after the second transplantation. Histology showed nodular hyperplasia of all parathyroid glands, indicating autonomous secretion of parathyroid hormone. In general, patients with more severe THPT are recognized with more severe SHPT prior to transplantation during the dialysis period. We should consider a referral for surgery based on the individual risk factors. We recommend to perform parathyroidectomy earlier, before the kidney transplantation in the clinical suspicion of severe SHPT.

[Thoracic Endovascular Aortic Repair and Abdominal Aorta Replacement to Treat Stanford Type B Acute Aortic Dissection Extending through an Abdominal Aortic Aneurysm;Report of a Case].

A 69-year-old man developed sudden-onset chest and back pain and was brought to our hospital. Enhanced computed tomography (CT) revealed acute Stanford type B aortic dissection extending from the distal aortic arch to a 72 mm abdominal aortic aneurysm( AAA). The acute phase was managed by antihypertensive therapy, and the patient was followed up. Twenty days after the onset of aortic dissection, entry closure of aortic dissection by thoracic endovascular aortic repair and abdominal aorta replacement were performed simultaneously. Aorta remodeling was confirmed by postoperative CT, and the patient's postoperative course was uneventful. In the treatment of patients with acute aortic dissection and AAA, surgical intervention timing and strategy must be considered carefully.

[Resection of Anterior Mediastinal Tumor Followed by Left Brachiocephalic Vein Reconstruction;Report of a Case].

A 65-year-old man was referred to our hospital because of an abnormal shadow on a chest radiogram. Swelling of the face and upper limbs were noted. Enhanced computed tomography showed a 62×101 mm mass in the anterior mediastinum with invasion to the superior vena cava (SVC) and the right upper lobe of the lung. Surgical resection through a sternotomy was performed. The mediastinal tumor was resected along with the left brachiocephalic vein, the part of SVC wall and the partial right upper lobe of the lung with a clamp on the proximal SVC, followed by a left brachiocephalic vein reconstruction. There has been no evidence of recurrence after 1 year. This procedure may be an efficacious technical option in case of anterior mediastinal invasive tumor.

[Operative Results of Total Arch Replacement with Arch First Technique using a Trifurcated Graft].

In aortic arch surgery, we reconstruct branches before opening the arch to avoid cerebral embolism of the debris derived from the aortic wall. We made a trifurcated graft for branch reconstruction using 10 mm and 8 mm polyester grafts. Cardiopulmonary bypass started under right axillary perfusion and venous drainage through the right atrium. While cooling, the left subclavian artery was clamped and anastomosed to the end of the trifurcated graft. Then the ascending aorta was cross-clamped and cardioplegic solution was infused. At 25 degrees centigrade of the tympanic temperature, the left carotid artery is clamped and anastomosed to the branch of the trifurcated graft with or without perfusion into the left carotid artery. Subsequently the brachiocephalic artery was reconstructed in the same manner. After antegrade cerebral perfusion was established through the trifurcated graft via right axillary perfusion, distal anastomosis of the aortic arch was done with the open distal technique. Graft-graft anastomosis was followed to reperfuse the lower half of the body. Finally proximal anastomosis was performed to complete total arch replacement. Forty-four patients underwent total arch replacement in this technique. In-hospital mortality was 4.5%. Cerebral infarction occurred in 4.5% of the patients probably due to embolization of the debris derived from the branches of the aortic arch.

[Complications of graft infection after endovascular aortic aneurysm repair].

OBJECTIVE: The endovascular approach to aortic aneurysm repair is widely spreading as an alternative to open repair. However infectious complications may be devastating. We described the experiences with infected aortic endografts and reviewed treatment and outcomes. METHODS: Eight patients were treated due to infected aortic endografts between June 2008 and March 2014. Seven males and 1 female with median age of 66 years( range, 38-84years) had 5 infected thoracic endovascular aortic repairs (EVARs) [5/142:3.5%] and 3 endovascular aortic repairs (EVARs)[3/387:0.8%]. Median time from repair to presentation was 12 months (range, 2-27 months). As the factors associated with infection, hemodialysis due to chronic kidney disease in 1, surgical treatment for the huge atheroma in 1, dental treatment in 2, treatment for infected aneurysm in 1, urinary tract infection after surgery for prostate in 1, aortoenteric fistula after TEVAR in 2 were considered. RESULTS: Five patients were treated with endograft explantation. The surgical mortality was 40% (2/5), during a follow-up, one more patient died with intracranial hemorrhage. The remaining 3 patients who were considered too high risk to remove the infectious endografts were treated conservatively with antibiotics. In 2 of them 1 died of respiratory failure, and multiple organ failure in 1. CONCLUSIONS: Infection of the endograft is a rare but devastating complication after endovascular repair of aortic aneurysms. Surgical removal of the infected prosthesis is accompanied with higher mortality, but antibiotics therapy cannot eradicate or cure the infection. In all cases, the factors associated with infection were calculated, and degenerative or infectious thrombi around the endovascular stent grafts were noted. Therefore, we consider that prophylactic antibiotic treatment may be necessary to prevent the bacteremia in the case of other surgical therapy or dental treatment.

Intraoperative migration of open stent graft detected by transesophageal echocardiography: report of two cases.

We report two cases of graft migration during open stent grafting, detected by transesophageal echocardiography (TEE). The incidence was 3.7% in our series. The length of landing zone was reduced from 45 mm to 25 mm in case 1 and from 50 mm to 22 mm in case 2 before chest closure. Aneurysmal protrusion on the greater curvature with thin mural thrombus were findings common in both cases. Although additional intervention was not done based on the TEE findings of no endoleak and thrombus formation in the aneurysm, and postoperative course was uneventful, meticulous imaging check-up was needed.

[Pacemaker infection in a child; report of a case].

In the treatment for pacemaker infection, removal of infected devices and intensive antibiotics therapy are in principle and new devices should be implanted apart from the infected site. However, there are some problems in the case of children:it is not easy to remove infected devices because epicardial leads are often used for them. If endocardial leads are chosen as a new system, extension of the lead would be concerned with their growth. We report a pediatric case of infection of pacemaker using epicardial leads. It was difficult to cure infection by repeated local treatment leaving epicardial leads and antibiotics therapy was obliged to continue for 9 years to keep infection under control. After growing up, we implanted endocardial leads for her and removed infected devices to cure infection completely.

Cardiac calcified amorphous tumor stuck in the aortic valve that mimicked a chameleon's tongue: report of a case.

A calcified amorphous tumor (CAT) is a rare intracardiac mass that carries a risk of embolism. We herein present the case of a club-shaped CAT that originated from the calcified mitral annulus. Echocardiography indicated a pendular motion of the mass and repeated entrapment by a stenotic aortic valve that was sustained for several beats, mimicking a chameleon's tongue. An emergency operation was performed because of the risk of embolism, as well as potential progression of cardiac failure due to worsening aortic valve stenosis. The histological findings were consistent with the diagnosis of a CAT. This report describes a case of an intracardiac tumor that showed unique motion like a chameleon's tongue.

Development of a new selective coronary perfusion cannula.

The two most common types of coronary perfusion cannulae currently being used are the "balloon type", with a balloon at the tip, and the "fenestrated type", which has holes along the side near the tip. However, on occasion an unusually high perfusion pressure or a considerable amount of leakage is encountered during infusion of the cardioplegic solution. We have examined the properties of a newly developed Kochi Medical School (KMS)-type cannula and compared these to the properties of the balloon-type and fenestrated-type cannulae in an ex vivo experimental model that contains ostia of 4, 3, or 2 mm in diameter. Ejected flow velocity, circuit pressure, and the amount of leakage were measured at an infusion rate of 100 and 200 mL/min, with the latter two parameters measured under the counterpressure of 0 and 50 cmH2O to examine the influence of coronary vascular resistance. Without counterpressure, the balloon type presented with the highest flow velocity (263 cm/s at 200 mL/min) and perfusion pressure (64 mmHg at 200 mL/min) but without leakage. The fenestrated type yielded a considerable amount of leakage (40 % at an ostium size of 2 mm). The KMS type showed a lower flow velocity and circuit pressure with less leakage. Under 50 cmH2O counterpressure, however, only the KMS-type cannula could inject the water to any ostium size at both flow rates. These results suggest that the concept of the KMS-type cannula may be advantageous to achieving a secure infusion to a diseased coronary ostium.

Intraoperative continuous hemodiafiltration with selective venous drainage of ischemic limbs.

We report two cases of acute limb ischemia with threatened myonephropathic metabolic syndrome (MNMS) in which continuous hemodiafiltration (CHDF) was started before revascularization with selective drainage from the clamped femoral vein of ischemic limb and return of processed blood into the contralateral femoral vein. It was aimed to optimise the removal of metabolites which were produced by myolysis following reperfusion as well as to minimize the deviation of metabolites into the systemic circulation. Both cases had uneventful postoperative course without MNMS and the limbs were salvaged.

A surgical case report of off-pump onlay patch grafting for pseudoaneurysm with diffusely calcified coronary artery.

Coronary pseudoaneurysm usually occurs after catheter-based intervention as a result of traumatic dissection or perforation of a coronary artery in 4%-5% of cases. Here, we report the successful case of pseudoaneurysm occurring after percutaneous coronary angioplasty (PTCA) against the severely calcified coronary artery treated with off-pump long onlay patch bypass technique using internal thoracic arteries. We report the case of a 59-year-old woman treated with off-pump onlay patch grafting for pseudoaneurysm after coronary angioplasty against the diffusely calcified coronary lesion. The portion of the endoarterectomized left anterior descending coronary artery proximal to the resected aneurysm extending 5cm distally was successfully reconstructed with long onlay patch using the internal thoracic arterial graft.

[Introduction, designs and assessment of a suture set for standard cardiovascular surgery].

A packing system for threads and needles has been introduced with the aim of efficient operations in the field of cardiovascular surgery. To provide competent and safe surgery to patients, a suture set was developed according to the standard cardiovascular surgery procedure with cooperation between doctors and nurses at Kochi University Hospital. This suture set was introduced to all surgeries for adults conducted under cardiopulmonary bypass. Herein, we describe problems before the introduction, the process of the introduction, and designs of the suture set. In addition, we assessed the present condition since the introduction of this suture set. Many positive opinions were obtained from both doctors and nurses, and the suture set thus yielded a satisfactory assessment. Psychological stability and safety provided by the suture set may lead not only to cost reduction but also to an increased level of surgery as a whole. In addition, it may now be possible to provide simpler and more efficient cardiovascular surgery with this set. This suture set will serve as a useful model for thread and needle packing systems to be developed at various facilities in the future.