bluuebunny/crossref_metadata_2025_split huggingface dataset:
Megalin is involved in angiotensinogen-induced, angiotensin II-mediated ERK1/2 signaling to activate Na+-H+ exchanger 3 in proximal tubules
Authors: Goto Sawako , Yoshida Yutaka , Hosojima Michihiro , Kuwahara Shoji , Kabasawa Hideyuki , Aoki Hiroyuki , Iida Tomomichi , Sawada Ryuhei , Ugamura Daisuke , Yoshizawa Yuta , Takemoto Kazuya , Komochi Koichi , Kobayashi Ryota , Kaseda Ryohei , Yaoita Eishin , Nagatoishi Satoru , Narita Ichiei , Tsumoto Kouhei , Saito Akihiko 
Published: September 2023

Background:
Kidney angiotensin (Ang) II is produced mainly from liver-derived, glomerular-filtered angiotensinogen (AGT). Podocyte injury has been reported to increase the kidney Ang II content and induce Na+ retention depending on the function of megalin, a proximal tubular endocytosis receptor. However, how megalin regulates the renal content and action of Ang II remains elusive.

Methods:
We used a mass spectrometry-based, parallel reaction-monitoring assay to quantitate Ang II in plasma, urine, and kidney homogenate of kidney-specific conditional megalin knockout (MegKO) and control (Ctl) mice. We also evaluated the pathophysiological changes in both mouse genotypes under the basal condition and under the condition of increased glomerular filtration of AGT induced by administration of recombinant mouse AGT (rec-mAGT).

Results:
Under the basal condition, plasma and kidney Ang II levels were comparable in the two mouse groups. Ang II was detected abundantly in fresh spot urine in conditional MegKO mice. Megalin was also found to mediate the uptake of intravenously administered fluorescent Ang II by PTECs. Administration of rec-mAGT increased kidney Ang II, exerted renal extracellular signal-regulated kinase 1/2 (ERK1/2) signaling, activated proximal tubular Na+-H+ exchanger 3 (NHE3), and decreased urinary Na+ excretion in Ctl mice, whereas these changes were suppressed but urinary Ang II was increased in conditional MegKO mice.

Conclusion:
Increased glomerular filtration of AGT is likely to augment Ang II production in the proximal tubular lumen. Thus, megalin-dependent Ang II uptake should be involved in the ERK1/2 signaling that activates proximal tubular NHE3 in vivo, thereby causing Na+ retention.
A Systematic Literature Review of Disease Progression Reported in RPGR-associated X-linked Retinitis Pigmentosa
Authors: Lam Byron L. , Scholl Hendrik P. N. , Doub Daneal , Sperling Marvin , Hashim Mahmoud , Li Nan 
Published: September 2023

Abstract

Purpose:

RPGR-associated X-linked retinitis pigmentosa (RPGR-XLRP) is a rare and severe form of retinitis pigmentosa (RP) resulting in progressive visual impairment; however, disease progression data are limited. A systematic literature review was conducted to assess available data on disease progression in RPGR-XLRP.

Methods:
PubMed, EMBASE, and select congress abstracts were evaluated through June 2022. Eligible studies included results specific to RPGR-XLRP or populations with ≥80% of RP patients carrying disease-causing RPGR variants. Endpoints of interest included visual acuity (VA), visual field (VF), ellipsoid zone width (EZW), progression to blindness, and patient-reported outcomes (PROs).

Results:
Fourteen studies met ≥1 endpoint of interest. Progressive declines in VA, VF, and EZW were reported across studies. Nearly all publications reported annual declines in VA (3.5%-8.2%). Annual VF declines ranged from 4.2% to 13.3%. Changes in retinal structure were also observed (EZW changes: –177 to –830 μm/y). Most studies measured blindness using VA; VF-based definitions resulted in blindness by age ∼25 years. PRO data were limited.

Conclusion:
Published evidence shows that patients with RPGR-XLRP experience progressive decline in VA, VF, and EZW, eventually resulting in blindness. Additional longitudinal data with standardized endpoints and expanded collection of PROs are needed to assess visual decline in RPGR-XLRP.
PREFOVEAL ADHERENT HEAVY SILICONE OIL AFTER VITRECTOMY: INCIDENCE, CLINICAL FEATURES, AND RISK FACTORS
Authors: Akyol Mehmet , Zewar Ahmed , Lochhead Jonathan 
Published: September 2023

Purpose:
The purpose of this study was to study the optical coherence tomography (OCT) and clinical features of all patients presenting with prefoveal “sticky” heavy silicone oil (HSO) after vitreoretinal surgery and subsequent oil removal.

Methods:
A retrospective review of all consecutive patients who underwent vitreoretinal surgery with HSO tamponade over 12 years was performed. All OCT scans were reviewed after HSO removal to determine features consistent with residual prefoveal oil deposits. A detailed analysis was performed to investigate potential clinical features and possible associations.

Results:
Seven cases of prefoveal HSO (pfHSO) were identified from a total of 51 consecutive cases (13.7%). All cases of pfHSO had a tamponade duration exceeding 3 months. The mean tamponade duration in the pfHSO group was 7.8 (3.5–21) months compared with 4.6 (1–9) months in the unaffected group. The use of mixed HSO compared with HSO increases the risk of pfHSO changes (P = 0.024).

Conclusion:
In summary, 7 novel cases of sticky pfHSO remnants with variable OCT dimensions were identified, which could have an impact on the visual prognosis. Removal of pfHSO is extremely challenging and may not always be possible. There were no pfHSO patients identified below 3 months of tamponade duration.
Ophthalmologic Findings in Pediatric Erdheim-Chester Disease: A Literature Review With a Novel Case Report
Authors: Yeager Lauren B. , Grimes Joseph M. , Dal Col Alexis K. , Shah Nirav V. , Bogomolny Dmitry , Debelenko Larisa , Marr Brian P. 
Published: September 2023

Purpose:
The authors aim to describe the ophthalmologic manifestations of pediatric Erdheim-Chester disease (ECD).

Methods:
The authors describe a novel case of ECD presenting as isolated bilateral proptosis in a child and provide a comprehensive review of the documented pediatric cases to observe overall trends and ophthalmic manifestations of disease. Twenty pediatric cases were identified in the literature.

Results:
The mean age at presentation was 9.6 years (1.8–17 years) with a mean time of symptom presentation to diagnosis of 1.6 years (0–6 years). Nine patients (45%) had ophthalmic involvement at diagnosis, 4 who presented with ophthalmic complaints: 3 with observable proptosis and 1 with diplopia. Other ophthalmic abnormalities included eyelid findings of a maculopapular rash with central atrophy on the eyelids and bilateral xanthelasmas, neuro-ophthalmologic findings of a right hemifacial palsy accompanied by bilateral optic atrophy and diplopia, and imaging findings of orbital bone and enhancing chiasmal lesions. No intraocular involvement was described, and visual acuity was not reported in most cases.

Conclusions:
Ophthalmic involvement occurs in almost half of documented pediatric cases. Typically presenting with other symptoms, the case highlights that isolated exophthalmos may be the only clinical sign, and ECD should be included in the differential diagnosis of bilateral exophthalmos in children. Ophthalmologists may be the first to evaluate these patients, and a high index of suspicion and an understanding of the varied clinical, radiographic, pathologic, and molecular findings are critical for prompt diagnosis and treatment of this unusual disease.
Obstructive sleep apnea in the patient with atrial fibrillation: current knowledge and remaining uncertainties
Authors: Lyons Owen D. 
Published: September 2023

Purpose of review
Obstructive sleep apnea (OSA) is highly prevalent in patients with atrial fibrillation and plays a causal role for OSA in the pathogenesis of atrial fibrillation. The presence of OSA in atrial fibrillation is associated with increased symptom burden and increased risk of hospitalizations. Furthermore, untreated OSA is associated with an increased risk of atrial fibrillation recurrence post ablation or cardioversion, and observational studies suggest that continuous positive airway pressure (CPAP) therapy can attenuate this risk. This review describes our current understanding of the relationship between OSA and atrial fibrillation with an emphasis on emerging evidence.

Recent findings
Recent studies have identified novel screening questionnaires, which may be superior to traditional questionnaires in identifying OSA in atrial fibrillation populations. Significant night-to-night variability in OSA severity has been shown in atrial fibrillation patients, which has implications for diagnostic testing. While several small, randomized control trials (RCTs) have not shown CPAP therapy to be effective in reducing atrial fibrillation burden, one RCT did show CPAP can attenuate the atrial substrate with implications for long-term outcomes.

Summary
Further RCTs, appropriately powered, and focused on well defined cohorts, are required to guide management decisions regarding screening and treatment of OSA in atrial fibrillation populations.
Early versus delayed laparoscopic cholecystectomy after endoscopic retrograde cholangiopancreatography: A meta-analysis
Authors: Qi Sheng , Xu Jie , Yan Chao , He Yanan , Chen Yao 
Published: September 2023

Purposes:Patients with cholecysto- and choledocholithiasis usually undergo endoscopic retrograde cholangiopancreatography (ERCP)/-endoscopic sphincterotomy followed by laparoscopic cholecystectomy (LC). However, differences in the timing of LC after the ERCP may alter the post-operative outcomes. The aim of this study was to compare the effect of early (≤3 days) or delayed LC (>3 days) following ERCP on the post-operative outcomes.Methods:A comprehensive search of the 3 databases PubMed, EMBASE and the Cochrane Library was performed. Articles related to LC at different time-points after ERCP were retrieved. Dichotomous and continuous outcomes were analyzed by risk ratio (RR) and mean difference, and RevMan was used to analyze each group.Results:A total of 7 studies, including 5 randomized controlled studies and 2 retrospective studies, involved a total of 711 patients. There were 332 patients in early LC group and 379 in delayed LC group. The conversion rate was lower in the early LC group compared to the delayed LC group (RR 0.38, 95% confident interval 0.19 to 0.74,P= .005, I2= 0%). Early LC resulted in a shorter operation time (RR −6.2, 95% CI −27.2 to −5.2,P= .004, I2= 97%) and fewer complications (RR 0.48, 95% CI 0.29 to 0.79,P= .004, I2= 17%). Subgroup analysis found that there were no significant differences in the conversion rate (RR 0.61, 95% CI 0.25 to 1.45,P= .26, I2= 0%) or complications between the early LC group and the delayed group who underwent LC after 1 month.Conclusion:Early LC after ERCP is the preferred treatment for patients with concurrent cholecysto- and choledocholithiasis due to improved clinical outcomes as compared to those who undergo delayed LC.
Long-term cognitive dysfunction after the COVID-19 pandemic: a narrative review
Authors: Shariff Sanobar , Uwishema Olivier , Mizero Jocelyn , Devi Thambi Vimala , Nazir Abubakar , Mahmoud Ashraf , Kaushik Ikshwaki , Khayat Saadeddine , Yusif Maigoro Abdulkadir , Awde Sara , Al Maaz Zeina , Alwan Iktimal , Hijazi Mahdi , Wellington Jack , Soojin Lee 
Published: September 2023

Introduction:
SARS-CoV-2, the virus responsible for the COVID-19 pandemic, has brought a conglomerate of novel chronic disabling conditions described as ‘Long COVID/Post-COVID-19 Syndrome’. Recent evidence suggests that the multifaceted nature of this syndrome results in both pulmonary and extrapulmonary sequelae,chronic dyspnoea, persistent fatigue, and cognitive dysfunction being the most common, debilitating symptoms. Several mechanisms engender or exacerbate cognitive impairment, including central nervous system and extra-central nervous system causes, although the exact mechanism remains unclear. Both hospitalized and non-hospitalized patients may suffer varying degrees of cognitive impairment, ranging from fatigue and brain fog to prolonged deficits in memory and attention, detrimental to the quality-of-life years post-recovery. The aim of this review is to understand the underlying mechanisms, associations, and attempts for prevention with early intervention of long-term cognitive impairment post-COVID-19.

Methodology:
A systematic search was conducted through multiple databases such as Medline, National Library of Medicine, Ovid, Scopus database to retrieve all the articles on the long-term sequalae of cognitive dysfunction after SARS-CoV-2 infection. The inclusion criteria included all articles pertinent to this specific topic and exclusion criteria subtracted studies pertaining to other aetiologies of cognitive dysfunction. This search was carefully screened for duplicates and the relevant information was extracted and analysed.

Results/discussion:
To date, the exact pathogenesis, and underlying mechanisms behind cognitive dysfunction in COVID-19, remain unclear, hindering the development of adequate management strategies. However, the proposed mechanisms suggested by various studies include direct damage to the blood-brain barrier, systemic inflammation, prolonged hypoxia, and extended intensive care admissions. However, no clear-cut guidelines for management are apparent.

Conclusion:
This review of the COVID-19 pandemic has elucidated a new global challenge which is affecting individuals’ quality of life by inducing long-term impaired cognitive function. The authors have found that comprehensive evaluations and interventions are crucial to address the cognitive sequelae in all COVID-19 patients, especially in patients with pre-existing cognitive impairment. Nevertheless, the authors recommend further research for the development of relevant, timely neurocognitive assessments and treatment plans.
Calciphylaxis during the course of psoriatic arthritis patient. Is it coincidence? A case report
Authors: Khalayli Naram , Hodifa Yara , Hodaifa Amr , Alsamarrai Omar , Kudsi Maysoun 
Published: September 2023

Introduction and importance:
Calciphylaxis manifests clinically by skin ischemia and necrosis and histologically by calcification of dermal arterioles. Usually, it occurs in patients with end-stage renal disease on dialysis or in patients who had a kidney transplant. Here, the authors present a case of calciphylaxis occurring in a patient with psoriasis and psoriatic arthritis.

Case presentation:
A 66-year-old Syrian male with a history of psoriatic arthritis presented for evaluation of 2-month nonpainful ulcers on his feet and hands that were treated with warfarin. Biopsies confirmed the diagnosis of calciphylaxis. The patient received sodium thiosulfate, zoledronic acid, intralesional sodium thiosulfate injections, and an intravenous infusion of vitamin K with dramatic improvement. At the 3-month follow-up, his wounds had been completely remitted.

Discussion:
Nonuremic calciphylaxis occurs in many cases, like vitamin D administration, vitamin K antagonists’ administration, chronic inflammation, and others. The association between calciphylaxis and psoriasis was reported only in four cases in the literature; meanwhile, this was the first case that described calciphylaxis in the setting of psoriatic arthritis.

Conclusion:
A suspicion of calciphylaxis should be maintained in patients with underlying inflammatory mechanism diseases.
Visualization of Thromboinflammation by 18F-DPA-714 PET in a Stroke Patient
Authors: Lin Qianqian , Wang Youliang , Zhao Wenlong , Yao Shaobo 
Published: September 2023

Abstract
A 44-year-old man who presented with progressive right limb weakness was diagnosed with ischemic stroke. He was referred for 18F-DPA-714 PET/CT for evaluation of the disease. 18F-DPA-714 PET/CT showed increased uptake of the intracranial thrombus. This DPA-714–avid thrombus highly suggested the involvement of immune cells in the extension of the clot resulting in neurological deterioration. This present case suggested that 18F-DPA-714 PET might be a promising tracer in visualizing thromboinflammation in vivo.
How should tranexamic acid be administered in haemorrhagic shock? - continuous serum concentration measurements in a swine model
Authors: Lynghaug Trine , Bakke Håkon Kvåle , Fuskevåg Ole Martin , Nielsen Erik Waage , Dietrichs Erik Sveberg 
Published: September 2023

Abstract

Background
Tranexamic acid (TXA) reduces mortality in trauma patients. Intramuscular (i.m.) administration could be advantageous in low-resource and military settings. Achieving the same serum concentration as i.v. administration is important to achieve equal mortality reduction. Therefore, we aimed to investigate whether dividing an i.m. dose of TXA between two injection sites, and whether an increase in dose, would lead to serum concentrations comparable to those achieved by i.v. administration.

Methods
Norwegian landrace pigs (n = 29) from a course in haemostatic emergency surgery were given tranexamic acid 1 h after start of surgery. Blood samples were drawn at 0, 5,10, 15, 20, 25, 35, 45, 60 and 85 min. The samples were centrifuged and serum TXA concentrations quantified with liquid chromatography–tandem mass spectrometry (LC–MS/MS). The use of two injection sites was compared to distributing the dose on one injection site, and a dose of 15 mg/kg was compared to a dose of 30 mg/kg. All i.m. groups were compared to i.v. administration.

Results
The groups were in a similar degree of shock. Increasing the i.m. dose from the standard of 15 mg/kg to 30 mg/kg resulted in significantly higher serum concentrations of TXA, comparable to those achieved by i.v. administration. Distributing the i.m. dose on two injection sites did not affect drug-uptake, as shown by equal serum concentrations.

Conclusions
For i.m. administration of TXA, 30 mg/kg should be the standard dose. With a short delay, i.m. administration will provide equal serum concentrations as i.v. administration, above what is considered necessary to inhibit fibrinolysis.
Differential Impact of the UNOS Simultaneous Liver-kidney Transplant Policy Change Among Patients With Sustained Acute Kidney Injury
Authors: Tanaka Tomohiro , Lentine Krista L. , Shi Qianyi , Vander Weg Mark , Axelrod David A. 
Published: September 2023

Background.
Simultaneous liver-kidney transplant (SLK) allocation policy in the United States was revised in August 2017, reducing access for liver transplant candidates with sustained acute kidney injury (sAKI) and potentially adversely impacting vulnerable populations whose true renal function is overestimated by commonly used estimation equations.

Methods.
We examined national transplant registry data containing information for all liver transplant recipients from June 2013 to December 2021 to assess the impact of this policy change using instrumental variable estimation based on date of listing.

Results.
Posttransplant survival was compared for propensity-matched patients with sAKI who were only eligible for liver transplant alone (LTA_post; n = 638) after the policy change but would have been SLK-eligible before August 2017, with similar patients who were previously able to receive an SLK (SLK; n = 319). Overall posttransplant patient survival was similar at 3 y (81% versus 80%; P = 0.9). However, receiving an SLK versus LTA increased survival among African Americans (87% versus 61% at 3 y; P = 0.029). A trend toward survival benefit from SLK versus LTA, especially later in the follow-up period, was observed in recipients ≥ age 60 (3-y survival: 84% versus 76%; P = 0.2) and women (86% versus 80%; P = 0.2).

Conclusions.
The 2017 United Network for Organ Sharing SLK Allocation Policy was associated with reduced survival of African Americans with end-stage liver disease and sAKI and, potentially, older patients and women. Our study suggested the use of race-neutral estimation of renal function would ameliorate racial disparities in the SLK arena; however, further studies are needed to reduce disparity in posttransplant outcomes among patients with liver and kidney failure.
Impact of Tele-Triage Pathways on Short-Stay Admission after Transfer to a Regional Burn Center for Acute Burn Injury
Authors: Clark Nina M , Agoubi Lauren L , Gibbs Sarah , Stewart Barclay T , De Grauw Xinyao , Vavilala Monica S , Rivara Frederick P , Arbabi Saman , Pham Tam N 
Published: September 2023

Background:
Regionalized care for burn-injured patients requires accurate triage. In 2016, we implemented a tele-triage system for acute burn consultations. We evaluated resource utilization following implementation, hypothesizing that this system would reduce short stay admissions and prioritize inpatient care for those with higher burn severity.

Methods:
We conducted a retrospective study of all transferred patients with acute burn injuries from 1/1/2010-12/31/2015 and 1/1/2017-12/31/2019. We evaluated proportions of short stay admissions (discharges <24 hours without operative intervention, ICU admission, or concern for non-accidental trauma) among patients transferred before (2010-2015) and after (2017-2019) triage system implementation. Multivariable Poisson regression was used to evaluate factors associated with short stay admissions. Interrupted time series analysis was used to evaluate the effect of the triage system.

Results:
There were 4688 burn transfers (3244 pre- and 1444 post-implementation) in the study periods. Mean age was higher post-implementation (32 vs 29 years, p<0.001). Median hospital LOS and ICU LOS were both 1 day higher, more patients underwent operative intervention (19% vs. 16%), and median time to first operation was 1 day lower post-implementation. Short stay admissions decreased from 50% (n=1624) to 39% (n=561), and patients were 17% less likely to have a short stay admission after implementation (aRR 0.83, 95% CI 0.8-0.9). Pediatric patients <15 years old composed 43% of all short stay admissions and were much more likely than adult patients to have a short stay admission independent of transfer timing (aRR 2.36, 95% CI 1.84-3.03).

Conclusions:
Tele-triage burn transfer center protocols reduced short-stay admissions and prioritized inpatient care for patients with more severe injuries. Pediatric patients remain more likely to have short stay admission after transfer.
Natural variation in the zinc-finger-encoding exon ofPrdm9affects hybrid sterility phenotypes in mice
Authors: AbuAlia Khawla FN , Damm Elena , Ullrich Kristian K , Mukaj Amisa , Parvanov Emil , Forejt Jiri , Odenthal-Hesse Linda 
Published: September 2023

AbstractPRDM9-mediated reproductive isolation was first described in the progeny ofMus musculus musculus(MUS) PWD/Ph andMus musculus domesticus(DOM) C57BL/6J inbred strains. These male F1-hybrids fail to complete chromosome synapsis and arrest meiosis at prophase I, due to incompatibilities between thePrdm9gene and hybrid sterility locusHstx2. We identified fourteen alleles ofPrdm9in Exon 12, encoding the DNA-binding domain of the PRDM9 protein in outcrossed wild mouse populations from Europe, Asia, and the Middle East, eight of which are novel. The samePrdm9allele was found in all mice bearing introgressedt-haplotypes, encompassingPrdm9and inversions preventing recombination with wildtype Chr 17. We asked whether seven novelPrdm9alleles in MUS populations and thet-haplotype allele in one MUS and three DOM populations inducePrdm9-mediated reproductive isolation. The results show that only combinations of thedom2allele of DOM origin and the MUSmsc1allele ensure complete infertility of intersubspecific hybrids outside the context of inbred mouse strains. The results further indicate that the erasure of PRDM9msc1binding motifs may be shared by MUS mice from populations with differentPrdm9alleles, implicating that erased PRDM9 binding motifs may be uncoupled from their corresponding PRDM9 zinc finger arrays at the population level. Our data corroborate the model ofPrdm9-mediatedhybrid sterility beyond inbred strains of mice and suggest that sterility alleles ofPrdm9may be rare.