Trigeminal neuralgia is a nerve disorder that causes unilateral severe facial pain. The clinical features of trigeminal neuralgia are agonizing, paroxysmal, anticipated in one or more divisions of ...the trigeminal nerve, with repetitive bursts of a few seconds, exacerbated by cutaneous stimuli. Microvascular decompression is proven effective, resulting in a positive outcome. Here, we report two cases of trigeminal neuralgia associated with the vertebral artery, who underwent endoscopic microvascular decompression. This case report aims to show the benefit of computational fluid dynamics evaluation of the neurovascular contact and its effect on change in wall shear stress magnitude of the offending vertebral artery after surgical management with microvascular decompression.
Abstract
Bovine-derived cultured cells, including Madin-Darby bovine kidney cells, are used worldwide; however, lipofection tend to result in low transfection efficiency, which has impeded the ...progress of veterinary research. We performed experiments to confirm the lipofection efficiency of bovine-derived cultured cells, to identify cells that suitable for lipofection. Several bovine tissues (endometrium, testis, ear tissue and foetal muscle) were collected, and primary cultured cells were prepared. Lipofection assay showed that only bovine endometrium (BE)-derived cells could be transfected efficiently (50‒70%). BE cells can be divided into at least two types of cell populations (BE-1 and BE-2). The BE-1 cells, which were suitable for lipofection, were obtained by passages at short intervals and were negative for cytokeratin- and positive for vimentin-expression; the BE-2 cells did not have these characteristics and were not suitable for lipofection. Furthermore, the BE-1 cells and artificially immortalised cells of BE-1, iBE-1 cells, were utilised in a reporter assay requiring the introduction of multiple DNAs. Endometrial tissues can be collected from living cows, and BE-1 cells can be obtained easily by controlling passaging timing. The production of BE-1 cells and sharing the methods required to prepare them will contribute to the development of veterinary research.
•Previous studies reported that intravenous dexmedetomidine use decreased adverse neurocognitive outcomes in adults undergoing cardiovascular surgery.•The effects of dexmedetomidine use on the brain ...and inflammatory markers have been unclear in children with congenital heart disease, although several randomized controlled trials have been reported since 2017.•We performed a systematic review and meta-analysis of randomized controlled trials that assessed the effects of dexmedetomidine on the brain and inflammatory markers in pediatric cardiac surgery.•A total of seven randomized controlled trials with 579 children who received cardiac surgeries were included in the meta-analysis.•Intravenous dexmedetomidine use reduced brain markers (neuron-specific enolase, S-100β protein) and an inflammatory marker (interleukin-6) within 24 hours after pediatric cardiac surgery.
Dexmedetomidine use decreases adverse neurocognitive outcomes in adults undergoing cardiovascular surgery, but its effect has been unclear in children with congenital heart disease.
The authors conducted a systematic review using the PubMed, Embase, and Cochrane Library databases for randomized controlled trials (RCTs) that compared intravenous dexmedetomidine with normal saline during pediatric cardiac surgery under anesthesia. Published randomized controlled trials that evaluated children aged <18 years who underwent congenital heart surgery were included. Nonrandomized trials, observational studies, case series and case reports, editorials, reviews, and conference papers were excluded. The quality of the included studies was assessed using the Cochrane revised tool for assessing risk-of-bias in randomized trials. Meta-analysis was performed to estimate the effects of intravenous dexmedetomidine on brain markers (neuron-specific enolase NSE, S-100β protein) and inflammatory markers (interleukin-6, tumor necrosis factor TNF-α, nuclear factor kappa-B NF-κB) during and after cardiac surgery, using random-effect models for standardized mean difference (SMD).
Seven RCTs involving 579 children were eligible for the following meta-analyses. Most children underwent cardiac surgery for atrial or ventricular septum defects. Pooled analyses (5 treatment groups in 3 RCTs with 260 children) showed that dexmedetomidine use was associated with reduced serum levels of NSE (pooled SMD, −0.54; 95% CI, −0.96 to −0.12) and S-100β (pooled SMD, −0.85; 95% CI, −1.67 to −0.04) within 24 hours after the surgery. Also, dexmedetomidine use was associated with reduced levels of interleukin-6 (pooled SMD, −1.55; 95% CI, −2.82 to −0.27; 4 treatment groups in 2 RCTs with 190 children). In contrast, the authors observed similar levels of TNF-α (pooled SMD, −0.07; 95% CI, −0.33 to 0.19; 4 treatment groups in 2 RCTs with 190 children) and NF-κB (pooled SMD, −0.27; 95% CI, −0.62 to 0.09; 2 treatment groups in 1 RCT with 90 children) between the dexmedetomidine and control groups.
The authors’ findings support the effect of dexmedetomidine on reductions in brain markers among children who undergo cardiac surgery. Further studies would be needed to elucidate its clinically meaningful effects using cognitive functions in the long term, and its effects among children who undergo more complex cardiac surgeries.
An increasing number of studies in Western countries have shown that healthy dietary patterns may have a protective effect against cognitive decline and dementia. However, information on this ...relationship among non-Western populations with different cultural settings is extremely limited. We aim to examine the relationship between dietary patterns and cognitive function among older Japanese people.
This cross-sectional study included 635 community-dwelling people aged 69-71 years who participated in the prospective cohort study titled Septuagenarians, Octogenarians, Nonagenarians Investigation with Centenarians (SONIC). Diet was assessed over a one-month period with a validated, brief-type, self-administered diet history questionnaire. Dietary patterns from thirty-three predefined food groups energy-adjusted food (g/d) were extracted by factor analysis. Cognitive function was assessed using the Japanese version of the Montreal Cognitive Assessment (MoCA-J). Multivariate regression analysis was performed to examine the relationship between dietary patterns and cognitive function.
Three dietary patterns were identified: the 'Plant foods and fish', 'Rice and miso soup', and 'Animal food' patterns. The 'Plant foods and fish' pattern, characterized by high intakes of green and other vegetables, soy products, seaweeds, mushrooms, potatoes, fruit, fish, and green tea, was significantly associated with a higher MoCA-J score MoCA-J score per one-quartile increase in dietary pattern: β = 0.56 (95% CI: 0.33, 0.79), P for trend <0.001. This association was still evident after adjustment for potential confounding factors β = 0.41 (95% CI: 0.17, 0.65), P for trend <0.001. In contrast, neither the 'Rice and miso soup' nor the 'Animal food' pattern was related to cognitive function. To confirm the possibility of reverse causation we also conducted a sensitivity analysis excluding 186 subjects who reported substantial changes in their diet for any reason, but the results did not change materially.
This preliminary cross-sectional study suggests that a diet with high intakes of vegetables, soy products, fruit, and fish may have a beneficial effect on cognitive function in older Japanese people. Further prospective studies are needed to confirm this finding.
Aim
Portopulmonary hypertension (PoPH) is a well‐known complication of liver cirrhosis. The aim of this study was to clarify the pulmonary hemodynamics and the prevalence and characteristics of PoPH ...in patients with portal hypertension.
Methods
The subjects were 335 patients with portal hypertension diagnosed by hepatic vein pressure gradient (HVPG). Among them, 186 patients received measurements of pulmonary artery pressure (PAP), pulmonary artery wedge pressure (PAWP) and pulmonary vascular resistance (PVR). PoPH was diagnosed by PAP >20 mmHg, PVR ≥3 Wood units (WU) and PAWP ≤15 mmHg.
Results
The Child–Pugh classification was class A in 53, B in 92 and C in 41 patients. Median (range) values of HVPG, PAP, PVR and PAWP were 18.4 (5.5–39.0) mmHg, 12.9 (6.6–40.8) mmHg, 0.8 (0.1–4.5) WU and 7.5 (2.2–15.4) mmHg, respectively. Of six patients with PAP >20 mmHg, four had autoimmune hepatitis or primary biliary cholangitis, with the prevalence being significantly higher than that in patients with PAP ≤20 mmHg. Meanwhile, no significant difference was noted in the hepatic functional reserve or HVPG between patients with PAP >20 mmHg and ≤20 mmHg. Only two patients met the diagnostic criteria of PoPH and both patients were Child–Pugh B. The Child–Pugh score and HVPG were not associated with PoPH.
Conclusions
Our study demonstrated that only two patients were complicated by PoPH. High PAP values were noted in patients with primary biliary cholangitis or autoimmune hepatitis. However, the presence of PoPH and high PAP were not associated with the degree of hepatic functional reserve or HVPG.
An n-6 essential fatty acid, arachidonic acid (ARA) is converted into prostaglandin E2, which is involved in tumour extension. However, it is unclear whether dietary ARA intake leads to cancer in ...humans. We thus systematically evaluated available observational studies on the relationship between ARA exposure and the risk of colorectal, skin, breast, prostate, lung, and stomach cancers.
We searched the PubMed database for articles published up to May 17, 2010. 126 potentially relevant articles from the initial search and 49,670 bibliographies were scrutinised to identify eligible publications by using predefined inclusion criteria. A comprehensive literature search yielded 52 eligible articles, and their reporting quality and methodological quality was assessed. Information on the strength of the association between ARA exposure and cancer risk, the dose-response relationship, and methodological limitations was collected and evaluated with respect to consistency and study design.
For colorectal, skin, breast, and prostate cancer, 17, 3, 18, and 16 studies, respectively, were identified. We could not obtain eligible reports for lung and stomach cancer. Studies used cohort (n = 4), nested case-control (n = 12), case-control (n = 26), and cross-sectional (n = 12) designs. The number of subjects (n = 15 - 88,795), ARA exposure assessment method (dietary intake or biomarker), cancer diagnosis and patient recruitment procedure (histological diagnosis, cancer registries, or self-reported information) varied among studies. The relationship between ARA exposure and colorectal cancer was inconsistent based on ARA exposure assessment methodology (dietary intake or biomarker). Conversely, there was no strong positive association or dose-response relationship for breast or prostate cancer. There were limited numbers of studies on skin cancer to draw any conclusions from the results.
The available epidemiologic evidence is weak because of the limited number of studies and their methodological limitations, but nonetheless, the results suggest that ARA exposure is not associated with increased breast and prostate cancer risk. Further evidence from well-designed observational studies is required to confirm or refute the association between ARA exposure and risk of cancer.
Human pluripotent stem cells (PSCs) express human endogenous retrovirus type-H (HERV-H), which exists as more than a thousand copies on the human genome and frequently produces chimeric transcripts ...as long-non-coding RNAs (lncRNAs) fused with downstream neighbor genes. Previous studies showed that HERV-H expression is required for the maintenance of PSC identity, and aberrant HERV-H expression attenuates neural differentiation potentials, however, little is known about the actual of function of HERV-H. In this study, we focused on ESRG, which is known as a PSC-related HERV-H-driven lncRNA. The global transcriptome data of various tissues and cell lines and quantitative expression analysis of PSCs showed that ESRG expression is much higher than other HERV-Hs and tightly silenced after differentiation. However, the loss of function by the complete excision of the entire ESRG gene body using a CRISPR/Cas9 platform revealed that ESRG is dispensable for the maintenance of the primed and naïve pluripotent states. The loss of ESRG hardly affected the global gene expression of PSCs or the differentiation potential toward trilineage. Differentiated cells derived from ESRG-deficient PSCs retained the potential to be reprogrammed into induced PSCs (iPSCs) by the forced expression of OCT3/4, SOX2, and KLF4. In conclusion, ESRG is dispensable for the maintenance and recapturing of human pluripotency.
Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) is a late-onset, slow-progressing multisystem neurodegenerative disorder. Biallelic AAGGG repeat expansion in RFC1 has been ...identified as causative of this disease, and repeat conformation heterogeneity (ACAGG repeat) was also recently implied. To molecularly characterize this disease in Japanese patients with adult-onset ataxia, we accumulated and screened 212 candidate families by an integrated approach consisting of flanking PCR, repeat-primed PCR, Southern blotting and long-read sequencing using Sequel II, GridION or PromethION. We identified 16 patients from 11 families, of whom seven had ACAGG expansions (ACAGG)exp/(ACAGG)exp (ACAGG homozygotes), two had ACAGG and AAGGG expansions (ACAGG)exp/(AAGGG)exp (ACAGG/AAGGG compound heterozygotes) and seven had AAGGG expansions (AAGGG)exp/(AAGGG)exp (AAGGG homozygotes). The overall detection rate was 5.2% (11/212 families including one family having two expansion genotypes). Long-read sequencers revealed the entire sequence of both AAGGG and ACAGG repeat expansions at the nucleotide level of resolution. Clinical assessment and neuropathology results suggested that patients with ACAGG expansions have similar clinical features to previously reported patients with homozygous AAGGG expansions, although motor neuron involvement was more notable in patients with ACAGG expansions (even if one allele was involved). Furthermore, a later age of onset and slower clinical progression were implied in patients with ACAGG/AAGGG compound heterozygous expansions compared with either ACAGG or AAGGG homozygotes in our very limited cohort. Our study clearly shows the occurrence of repeat conformation heterogeneity, with possible different impacts on the affected nervous systems. The difference in disease onset and progression between compound heterozygotes and homozygotes might also be suspected but with very limited certainty due to the small sample number of cases in our study. Studies of additional patients are needed to confirm this.
The genotype-phenotype correlation in BRAF variant in cardio-facio-cutaneous (CFC) syndrome is not clearly defined. Here we report a case with a severe clinical phenotype and a novel BRAF variant, ...p.Leu485del. The present case showed severe intellectual disability, impaired awareness, hyperekplexia, involuntary movements, early onset refractory seizures, and delayed myelination on brain magnetic resonance imaging as well as a polycystic and dysplastic kidney, which are previously unreported anomalies in CFC or RAS/mitogen-activated protein kinase syndromes related to BRAF variant. CFC syndrome, especially caused by BRAF variant, should be included in the differential diagnosis of patients with developmental and epileptic encephalopathies and hyperekplexia. Furthermore, we need to keep in mind that missense variants or the deletion of Leucine-485 may be associated with severe symptoms.
A 78-year-old woman with a history of intractable otitis media presented with a fever, hearing impairment, thigh pain, and a skin rash. She had renal dysfunction, positive ...myeloperoxidase-antineutrophil cytoplasmic autoantibody, otitis media, and multiple nodules in both lungs. She was diagnosed with granulomatosis with polyangiitis, crescentic glomerulonephritis, and interstitial nephritis, which was confirmed in a kidney biopsy specimen. Induction therapy with rituximab and avacopan without glucocorticoids promptly resolved her fever and thigh pain and improved her auditory acuity and nodule in the right lung. The patient experienced no adverse effects with rituximab or avacopan.
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