Impact of Fish Intake Frequency on Cardiovascular Disease-Specific Survival in Hemodialysis Patients.

Introduction: Cardiovascular disease (CVD) is the leading cause of death in hemodialysis patients (HPs). As a food source, fish contains both CVD-preventive and CVD-promoting fatty acids; however, there is no consensus on fish consumption as a preventive measure for CVD in HPs. This single-center longitudinal cohort study aims to assess the impact of fish intake frequency (FIF) per week on CVD in Japanese HPs. Methods: Upon the initiation of the study, 148 HPs were evaluated to determine the FIF, and blood samples were analyzed. These patients were then monitored for 6 years.The relationships between each FIF and blood sampling data, CVD-specific survival (CSS), and new CVD-free survival (nCFS) were statistically calculated using Kaplan-Meier survival curves. Results: During the observation period, 65 deaths were reported, 16 of which were attributed to CVD. Further, 53 patients developed new CVD onset, and no association was found between the FIF and blood sampling data. Based on the Kaplan-Meier survival curves, there was a significant difference in the CSS probability rates at 72 months between patients with an FIF of ≥4 (0.719, 95% confidence interval (CI): 0.530-0.842) and those with an FIF of ≤3 (0.930, 95% CI: 0.851-0.968) (p < 0.01). However, the nCFS probability at 72 months did not significantly differ between patients with an FIF of ≥4 and those with an FIF of ≤3. Multivariate Cox proportional hazards regression showed that an FIF of ≥4 (hazard ratio: 3.64, 95% CI: 1.22-10.9, p = 0.02) was an independent predictor of CSS, but not of nCFS. Conclusions: It was suggested that a higher FIF in HPs might be one of the risks for developing CVD with increased mortality.

Pitfalls in the diagnosis and treatment of a hypertensive patient with unilateral primary aldosteronism and contralateral pheochromocytoma: a case report.

BACKGROUND: Primary aldosteronism (PA) is a common cause of secondary hypertension, whereas pheochromocytoma is a rare cause of it. Thus, concomitant PA and pheochromocytoma is a very rare condition. CASE PRESENTATION: A 52-year-old woman was admitted to our hospital with suspected PA based on the presence of hypertension, spontaneous hypokalemia, and a high aldosterone-to-renin ratio. She had no catecholamine excess symptoms other than hypertension. Abdominal computed tomography (CT) showed a right lipid-rich adrenal mass and a left lipid-poor adrenal mass. PA was diagnosed by the captopril challenge test. The 24-h urinary fractionated metanephrines were slightly elevated. Adrenal vein sampling (AVS) confirmed that the right adrenal gland was responsible for aldosterone hypersecretion. Medical therapy with eplerenone was started because the patient refused surgery. Five years later, she requested surgery for PA. The second AVS confirmed right unilateral hyperaldosteronism, as expected. Repeated abdominal CT showed the enlargement of the left adrenal mass. The 24-h urinary fractionated metanephrines had risen to the diagnostic level. 123I- metaiodobenzylguanidine (MIBG) scintigraphy showed a marked tracer uptake in the left adrenal mass with no metastatic lesion. After preoperative management with α-blockade, laparoscopic left partial adrenalectomy was performed. Immunohistochemical examination of the tumor showed chromogranin A positivity leading to the diagnosis of left pheochromocytoma. CONCLUSIONS: We report an extremely rare case of concomitant unilateral PA and contralateral pheochromocytoma. When diagnosing unilateral PA by AVS, especially in cases with a lipid-poor adrenal mass, clinicians should rule out the possibility of the presence of pheochromocytoma before proceeding to undergo unilateral adrenalectomy. Although there is no standard treatment for this rare condition, it is essential to select personalized treatment from the perspective of conserving the adrenal gland.

A case of retroperitoneal venous malformation resected by laparoscopic surgery.

Introduction: Among vascular malformations, venous malformations are the most common type. Among these, retroperitoneal venous malformations are extremely rare. Case presentation: A 60-year-old woman was diagnosed with a retroperitoneal tumor 4.5 cm in diameter by abdominal computed tomographic scan. We had difficulty judging whether the tumor was benign or malignant. We performed laparoscopic surgery in order to remove the tumor and make a precise diagnosis. The pathological diagnosis was a venous malformation. Conclusion: Venous malformation located in the retroperitoneum is very rare, and there were few cases that could be removed by laparoscopic surgery. Laparoscopic surgery may be beneficial both for treatment and diagnosis of patients with a small retroperitoneal venous malformation.

Modeling a human CLP1 mutation in mouse identifies an accumulation of tyrosine pre-tRNA fragments causing pontocerebellar hypoplasia type 10.

Cleavage factor polyribonucleotide kinase subunit 1 (CLP1), an RNA kinase, plays essential roles in protein complexes involved in the 3'-end formation and polyadenylation of mRNA and the tRNA splicing endonuclease complex, which is involved in precursor tRNA splicing. The mutation R140H in human CLP1 causes pontocerebellar hypoplasia type 10 (PCH10), which is characterized by microcephaly and axonal peripheral neuropathy. Previously, we reported that RNA fragments derived from isoleucine pre-tRNA introns (Ile-introns) accumulate in fibroblasts of patients with PCH10. Therefore, it has been suggested that this intronic RNA fragment accumulation may trigger PCH10 onset. However, the molecular mechanism underlying PCH10 pathogenesis remains elusive. Thus, we generated knock-in mutant mice that harbored a CLP1 mutation consistent with R140H. As expected, these mice showed progressive loss of the upper motor neurons, resulting in impaired locomotor activity, although the phenotype was milder than that of the human variant. Mechanistically, we found that the R140H mutation causes intracellular accumulation of Ile-introns derived from isoleucine pre-tRNAs and 5' tRNA fragments derived from tyrosine pre-tRNAs, suggesting that these two types of RNA fragments were cooperatively or independently involved in the onset and progression of the disease. Taken together, the CLP1-R140H mouse model provided new insights into the pathogenesis of neurodegenerative diseases, such as PCH10, caused by genetic mutations in tRNA metabolism-related molecules.

Tyrosine pre-transfer RNA fragments are linked to p53-dependent neuronal cell death via PKM2.

Fragments of transfer RNA (tRNA), derived either from pre-tRNA or mature tRNA, have been discovered to play an essential role in the pathogenesis of various disorders such as neurodegenerative disease. CLP1 is an RNA kinase involved in tRNA biogenesis, and mutations in its encoding gene are responsible for pontocerebellar hypoplasia type-10. Mutation of the CLP1 gene results in the accumulation of tRNA fragments of several different kinds. These tRNA fragments are expected to be associated with the disease pathogenesis. However, it is still unclear which of the tRNA fragments arising from the CLP1 gene mutation has the greatest impact on the onset of neuronal disease. We found that 5' tRNA fragments derived from tyrosine pre-tRNA (5' Tyr-tRF) caused p53-dependent neuronal cell death predominantly more than other types of tRNA fragment. We also showed that 5' Tyr-tRF bound directly to pyruvate kinase M2 (PKM2). Injection of zebrafish embryos with PKM2 mRNA ameliorated the neuronal defects induced in zebrafish embryos by 5' Tyr-tRF. Our findings partially uncovered a mechanistic link between 5' Tyr-tRF and neuronal cell death that is regulated by PKM2.

CLP1 acts as the main RNA kinase in mice.

CLP1 plays an essential role in the protein complex involved in mRNA 3'-end formation and polyadenylation as well as in the tRNA splicing endonuclease (TSEN) complex involved in the splicing of precursor tRNAs. NOL9 localizes in the nucleolus of cells and plays an essential role in ribosomal RNA maturation. Both CLP1 and NOL9 are RNA kinases that phosphorylate the 5' end of RNAs. From the evidence that phosphorylation of the 5' end of a siRNA is essential for its efficient RNA cleavage, it was expected that CLP1 and NOL9 would be corresponding molecules. However, there had been no direct evidence that this is the case. In this study, murine NOL9 showed no apparent RNA kinase activity in cells or even in an RNA kinase assay using recombinant murine NOL9 protein. Although siRNA efficiency was decreased in CLP1 kinase-dead (Clp1K/K) cells, it was not influenced by NOL9 overexpression. These findings indicate that in mouse cells it is CLP1 that mainly acts to phosphorylate the 5' end of RNAs in the siRNA pathway, with no apparent involvement of NOL9.

Pfannenstiel laparoendoscopic reduced-port radical nephrectomy.

INTRODUCTION: We previously reported cases of laparoendoscopic single-site nephrectomy performed through an umbilical or pararectal incision. To improve cosmesis and operability, we performed three Pfannenstiel laparoendoscopic reduced-port nephrectomies. MATERIALS AND SURGICAL TECHNIQUE: In the first case, a GelPOINT access was placed through a 2-cm umbilical incision, and two additional 3-mm trocars were inserted. The specimen was extracted through a 4-cm Pfannenstiel incision. In the second and third cases, a GelPOINT access was placed through a 5-cm Pfannenstiel incision, and two additional 3-mm trocars were inserted. The specimens were extracted without additional skin incisions. In all cases, the endoscope and vessel-sealing device were inserted through the GelPOINT access. We used 3-mm scissors, dissecting forceps, and bipolar forceps. DISCUSSION: The operating time and estimated blood loss were 228, 280, and 155 min and 10, 410, and 5 mL, respectively. There were no intraoperative or postoperative complications. The 3-mm forceps showed similar efficacy as the conventional 5-mm forceps. Therefore, a Pfannenstiel reduced-port nephrectomy using 3-mm working trocars is a safe and feasible procedure with good cosmesis.

[MASSIVE HEMORRHAGE FROM THE FISTULA FORMATION BETWEEN CUTANEOUS URETEROSTOMY AND INFERIOR EPIGASTRIC ARTERY: A CASE REPORT].

A 87-year-old man received radical nephroureterectomy for right renal pelvic cancer in 2009 and left cutaneous ureterostomy after radical cystectomy for bladder cancer in 2013. He visited the hospital for exchanging a 7 or 8 Fr single-J catheter every 2 to 4 weeks. Eleven months after the 2nd operation, massive bleeding from the stoma occurred when ureteral catheter was exchanged. Contrast-enhanced computed tomography showed that left inferior epigastric artery was located close to left ureter. Angiography of the left inferior epigastric artery didn't show an obvious fistula, but revealed the stoma was surrounded by ramified new blood vessels from left inferior epigastric artery. We suspected a rupture of the vessels and performed embolization for the branch of inferior epigastric artery to left ureter. This embolization made it possible for the bleeding to be controlled. Massive bleeding from the branch of inferior epigastric artery is very rare, and we report the case and review the literature.