bluuebunny/crossref_metadata_2025_split huggingface dataset:
Operative management of primary hyperparathyroidism in Europe
Authors: Norén Erik , Nordenström Erik , Bergenfelz Anders O J 
Published: May 2024

Abstract

Background
Multicentre studies have previously reported on national outcomes of surgery for primary hyperparathyroidism, but not investigated whether management and outcome are uniform among countries. This study investigated whether there are differences among European countries in operative management and outcome of surgery for primary hyperparathyroidism.

Methods
Using data from Eurocrine®, a pan-European registry for endocrine surgeries, a retrospective observational cross-sectional multicentre study with 99 participating centres in 14 European countries was performed. Data on age, sex, calcium levels, operative strategy, conversion rate and rate of failed exploration were analysed for patients who underwent initial surgery for sporadic primary hyperparathyroidism. Primary outcome measures were intention to perform limited parathyroidectomy and the rate of hypercalcaemia at first follow-up.

Results
A total of 9548 patients were registered between 2015 and 2020. There were 7642 (80%, range 74.5–93.2%) females. There was intention to perform limited parathyroidectomy in 7320 of 9548 (76.7%) operations, ranging from 498 of 1007 (49.5%) to 40 of 41 (97.6%) among countries. Hypercalcaemia at first follow-up (median time to follow-up 15 days) was found in 416 of 9548 (4.4%) operations, ranging from 0 of 119 (0%) to 3 of 38 (7.9%) among countries.

Conclusion
This study demonstrated large differences in the intention to perform limited parathyroidectomy for primary hyperparathyroidism among European countries, as well as differences in the rate of postoperative hypercalcaemia. Future studies are needed to evaluate the impact of these different healthcare practices on patient outcomes.
Dibutyltin(IV) formulations as potential antitubercular scaffold: DFT assisted emerging advances and mechanism of antitubercular action
Authors: Soni Komal , Kumawat Anita , Saxena Sanjiv , Jain Asha 
Published: May 2024

Abstract
The interplay between N,O-orthometallated, O,O′-chelated, and dibutyltin(IV) groups in biopotent hybrid formulations to combat tuberculosis is deciphered. The present study is concerned with design, generation, spectroscopic, and DFT-assisted structural characterization. It improves understanding of the structure-antitubercular activity relationship and mode of action of some dibutyltin(IV) formulations against Mycobacterium tuberculosis as possible antituberculosis agents. Dibutyltin(IV) formulations of the general formula Bu2SnLL′ (where LH = heterocyclic/non-fluorinated/fluorinated β-diketones and L′H = aminophenol/substituted aminophenols) were generated by the reaction of Bu2SnCl2 with sodium salts of the ligands in 1:1:1 molar ratio. The newly generated dibutyltin(IV) formulations were characterized with the assistance of spectroscopic techniques and mass studies. Computational calculations of some representative complexes (1 and 2) also supported the obtained spectral data. Antituberculosis activity analysis was performed on some representative dibutyltin(IV) complexes (1, 4, and 8). Out of these, complex 4 demonstrated the highest activity against Mycobacterium tuberculosis. The structure-antitubercular activity relationship and mechanism of action of the active complexes against M. tuberculosis were also investigated. An intercluster electron hopping mechanism of mode of action is suggested.
Patient-initiated follow-up for high-risk cutaneous squamous cell carcinoma: how we do it and 2 years of outcome data
Authors: Abdul Gafoor Shafiah Muna , Robinson Sophie , Diskantova Sofya , Woodcock Emma , Yethenpa Sonam , Holloran Sophie , Nelson Toby 
Published: May 2024

Abstract

Background
For patients with high-risk cutaneous squamous cell carcinomas (cSCCs), current guidance suggests we should offer post-treatment follow-up appointments at regular intervals for 24 months. Is this to improve prognosis, provide psychological support or find the next cancer? Recent data confirm that recurrence and metastasis are rarer events, and that perhaps these intense follow-up schedules do not really lead to improved health outcomes.

Objectives
To question whether current follow-up practices are truly needed by introducing an option of patient-initiated follow-up (PIFU).

Methods
We enrolled 476 patients with cSCC (January 2020–January 2023) who fulfilled the definition of high-risk cSCC based on guidelines in use at the time. Of the total, 59 did not fulful the inclusion criteria and were excluded; 250 (52.5%) did not recontact us during the 2-year period, with no clinical record of complications or recurrences; and 167 (35.1%) utilized the PIFU pathway, of which 119 patients required only one face-to-face appointment. Seven patients (1.5%) developed metastatic disease, 11 (2.3%) developed recurrence and 68 (14.3%) developed cSCC at another site. All lesions were identified by the patient via PIFU. We saved 1250 follow-up appointments from those who did not contact us (n = 250), financially equating to £181 462.50.

Conclusions
Our data imply that PIFU can be considered safe alternative practice for patients with cSCC. Patients independently identified the need for review without scheduled follow-up, making these appointments available to other patient cohorts. Although follow-up appointments may provide mental health support, they can be inconvenient and not the ideal use of our healthcare resources. Our findings support a call for revision of existing skin cancer health policies to cope with and subsequently improve our practices for better patient care.
Inclisiran administration potently and durably lowers LDL-C over an extended-term follow-up: the ORION-8 trial
Authors: Wright R Scott , Raal Frederick J , Koenig Wolfgang , Landmesser Ulf , Leiter Lawrence A , Vikarunnessa Sheikh , Lesogor Anastasia , Maheux Pierre , Talloczy Zsolt , Zang Xiao , Schwartz Gregory G , Ray Kausik K 
Published: May 2024

Abstract

Aims
Data describing the long-term efficacy and tolerability of inclisiran are limited. This was explored in ORION-8, an open-label extension of preceding Phase 2 and Phase 3 placebo-controlled and open-label extension trials.

Methods and results
Following completion of the parent trial, adult patients with atherosclerotic cardiovascular disease (ASCVD), ASCVD risk equivalent, or heterozygous familial hypercholesterolaemia received open-label inclisiran twice yearly (after initial and 3-month doses) until Day 990, followed by an end-of-study visit at Day 1080 or ≥ 90 days after the last dose. The study endpoints included the proportion of patients achieving pre-specified low-density lipoprotein cholesterol (LDL-C) goals [ASCVD: &lt; 1.8 mmol/L (&lt; 70 mg/dL); ASCVD risk equivalent: &lt; 2.6 mmol/L (&lt; 100 mg/dL)], percentage and absolute changes in LDL-C at end-of-study, and safety of inclisiran. Of 3274 patients, 2446 (74.7%) were followed until end-of-study. Mean age was 64.9 ± 9.9 years, 82.7% (n = 2709) had ASCVD, and mean baseline LDL-C was 2.9 ± 1.2 mmol/L. Mean cumulative exposure to inclisiran (including parent trials) was 3.7 years; maximum exposure was 6.8 years. With inclisiran, 78.4% [95% confidence interval (CI): 76.8, 80.0] of patients achieved pre-specified LDL-C goals and mean percentage change in LDL-C was −49.4% (95% CI: −50.4, −48.3). No attenuation of LDL-C lowering over time was observed. Treatment-emergent adverse events at injection site (all mild/moderate) occurred in 5.9% of the patients. Inclisiran-associated anti-drug antibodies were infrequent (5.5%) and had no impact on the efficacy or safety of inclisiran. No new safety signals were identified.

Conclusion
In the largest and longest follow-up to date with &gt;12 000 patient-years exposure, inclisiran demonstrated consistent and effective LDL-C lowering with a favourable long-term safety and tolerability profile.

Trial Registration number
ClinicalTrials.gov identifier: NCT03814187
A case report of superior vena cava/right coronary artery fistula secondary to chronic endocarditis
Authors: Kiel Michaela D , Prathivadhi-Bhayankaram Sruti , Singhal Arun K , Ashwath Mahi L 
Published: May 2024

Abstract

Background
Coronary arteriovenous fistulas present an abnormal connection between the coronary arteries and an adjacent systemic or pulmonary vessel. They are rare, representing 0.002% of the general population. The majority is congenital but may additionally occur related to trauma or interventional cardiac procedures.

Case summary
We present the case of a 48-year-old male with a history of untreated bacterial endocarditis developing a right coronary/superior vena cava fistula. We detail the imaging findings of this rare phenomenon to arrive at this diagnosis. We describe his clinical course and the interventions considered, including surgical extraction. Unfortunately, this patient left against medical advice before completing recommended treatment.

Discussion
We present the first documentation of a right coronary/superior vena cava fistula secondary to chronic untreated bacterial endocarditis. Clinicians should be aware of this rare complication.
A case report of refractory multivessel coronary spasm associated with hypereosinophilic syndrome: one cell, one disease?
Authors: Godo Shigeo , Takagi Hidenobu , Komaru Kohei , Takahashi Jun , Yasuda Satoshi 
Published: May 2024

Abstract

Background
Hypereosinophilic syndrome (HES) is characterized by moderate to severe eosinophilia, exclusion of neoplastic or secondary origins of eosinophilia, and systemic involvement with end-organ damage. Coronary arteries can be affected to cause vasospastic angina (VSA); however, the association of the two diseases is not well recognized.

Case summary
A 55-year-old woman who had a history of multiple allergic disease such as bronchial asthma and chronic sinusitis with nasal polyps was hospitalized due to attacks of chest pain at rest. During a spontaneous attack of chest pain, ECG revealed ST-segment elevation in the inferior leads and emergency coronary angiography showed focal spasms of the right and left anterior descending coronary arteries, both of which were relieved after intracoronary administration of nitroglycerine. She was diagnosed with VSA according to the Japanese Circulation Society guidelines. Despite conventional vasodilator therapies, her resting angina remained refractory. Laboratory findings were notable for moderate eosinophilia. A comprehensive evaluation to uncover the underlying cause of refractory VSA led to the diagnosis of HES, concomitant with eosinophilic pneumonia and eosinophilic chronic rhinosinusitis. Pericoronary inflammation by fat attenuation index (FAI) was increased in the proximal segment of the right coronary artery. Treatment was initiated with oral prednisolone at a starting dose of 30 mg/day. The response to treatment was rapid, with her symptoms disappearing and a regression of eosinophilia observed the following day.

Discussion
Hypereosinophilic syndrome manifests with refractory VSA, and eosinophil-suppressing corticosteroid therapy proves effective in improving both conditions along with reduction of the pericoronary inflammation by FAI.
Coronary artery fistula following surgical myectomy for hypertrophic obstructive cardiomyopathy: a case report
Authors: Bowles James , Martin Joshua , Russell Penni L , Bailey Amy , Holland David J 
Published: May 2024

Abstract

Background
Coronary artery fistula is a rare, but recognized complication of surgical myectomy. Although most communicate with the right heart, a large fistula into the left ventricular cavity may result in a shunt haemodynamically analogous to aortic regurgitation. Understanding the variable presentation of iatrogenic coronary fistulae and the optimal evaluation strategy is critical to obtaining a timely diagnosis and instituting treatment.

Case summary
We report the case of a 57-year-old renal transplant recipient admitted for evaluation of presyncope, one-year post-surgical myectomy for hypertrophic obstructive cardiomyopathy. An iatrogenic coronary artery fistula was suspected by transthoracic echocardiography, and later confirmed with both non-invasive and invasive coronary angiography.

Discussion
We highlight various cardiac imaging modalities that confirmed the diagnosis of coronary artery fistula and helped to determine the clinical significance. We report the tailored approach often required to determine the anatomic and haemodynamic characteristics of coronary fistulae and outline potential management strategies.
Bilateral isolated coronary ostial stenosis in a middle-aged premenopausal woman with vasospastic angina: a case report
Authors: Takahashi Koji , Kodama Akihiro , Uemura Shigeki , Okura Takafumi 
Published: May 2024

Abstract

Background
Vasospastic angina (VSA) is uncommon in premenopausal women who have less chronic endothelial injury causing vascular remodelling, considered to play a primary role in the pathogenesis for coronary vasospasms. Furthermore, vasospasms rarely occur in the bilateral coronary ostia. Isolated coronary ostial stenosis (ICOS), which often causes severe effort angina and requires surgical intervention, is more commonly reported in middle-aged women, with causes including fibromuscular dysplasia (FMD) and large-vessel vasculitis. However, ICOS associated with VSA is extremely rare.

Case summary
A 50-year-old premenopausal Japanese woman presented with a complaint of typical chest pain due to angina during light exertion daily in the early morning hours since 3 years. Coronary angiography (CAG) revealed bilateral mild-to-moderate ICOS in addition to multi-vessel spasms involving the bilateral coronary ostia confirmed by the vasospasm provocation test using intracoronary acetylcholine injection. Tests to determine the cause of ICOS did not identify FMD or any other disease. The angina attacks alleviated after calcium channel blocker (CCB) administration without intervention for bilateral ICOS for 24 years since the first presentation. Moreover, coronary computed tomography angiography (CTA) performed 24 years after the first presentation showed no ICOS.

Discussion
In our patient with typical and frequent VSA symptoms, CAG revealed both mild-to-moderate ICOS and the vasospasms in the bilateral coronary ostia. Fibromuscular dysplasia or large-vessel vasculitis was ruled out as the causes of ICOS. Vasospastic angina rarely occurred after the prescription of CCB, and coronary CTA 24 years after the first presentation showed no ICOS. Bilateral ICOS in our patient might be VSA related.
Syncope or seizure: that is the question—case report of a young patient with convulsive cardioinhibitory syncope treated with cardioneuroablation
Authors: Papa Andrea , Fisch Urs , Bassetti Stefano , Badertscher Patrick , Krisai Philipp 
Published: May 2024

Abstract

Background
Differentiation of syncope from seizure is challenging and has therapeutic implications. Cardioinhibitory reflex syncope typically affects young patients where permanent pacing should be avoided whenever possible. Cardioneuroablation may obviate the need for a pacemaker in well-selected patients.

Case summary
A previously healthy 24-year-old woman was referred to the emergency department after recurrent episodes of transient loss of consciousness (TLOC). The electrocardiogram (ECG) and the echocardiogram were normal. An electroencephalogram (EEG) showed intermittent, generalized pathological activity. During EEG under photostimulation, the patient developed a short-term TLOC followed by brachial myocloni, while the concurrent ECG registered a progressive bradycardia, which turned into a complete atrioventricular block and sinus arrest with asystole for 14 s. Immediately after, the patient regained consciousness without sequelae. The episode was interpreted as cardioinhibitory convulsive syncope. However, due to the pathological EEG findings, an underlying epilepsy with ictal asystole could not be fully excluded. Therefore, an antiseizure therapy was also started. After discussing the consequences of pacemaker implantation, the patient agreed to undergo a cardioneuroablation and after 72 h without complications, she was discharged home. At 10 months, the patient autonomously discontinued the antiepileptics. The follow-up EEG displayed unspecific activities without clinical correlations. An implantable loop recorder didn’t show any relevant bradyarrhythmia. At 1-year follow-up, the patient remained asymptomatic and without syncopal episodes.

Discussion
Reflex syncope must be considered in the differential diagnosis of seizures. The cardioneuroablation obviated the need for a pacemaker and allowed for the withdrawal of anticonvulsants, originally started on the premise of seizure.
Sex-specific cardiac magnetic resonance pulmonary capillary wedge pressure
Authors: Garg Pankaj , Grafton-Clarke Ciaran , Matthews Gareth , Swoboda Peter , Zhong Liang , Aung Nay , Thomson Ross , Alabed Samer , Demirkiran Ahmet , Vassiliou Vassilios S , Swift Andrew J 
Published: May 2024

Abstract

Aims
Heart failure (HF) with preserved ejection fraction disproportionately affects women. There are no validated sex-specific tools for HF diagnosis despite widely reported differences in cardiac structure. This study investigates whether sex, as assigned at birth, influences cardiac magnetic resonance (CMR) assessment of left ventricular filling pressure (LVFP), a hallmark of HF agnostic to ejection fraction.

Methods and results
A derivation cohort of patients with suspected pulmonary hypertension and HF from the Sheffield centre underwent invasive right heart catheterization and CMR within 24 h of each other. A sex-specific CMR model to estimate LVFP, measured as pulmonary capillary wedge pressure (PCWP), was developed using multivariable regression. A validation cohort of patients with confirmed HF from the Leeds centre was used to evaluate for the primary endpoints of HF hospitalization and major adverse cardiovascular events (MACEs). Comparison between generic and sex-specific CMR-derived PCWP was undertaken. A total of 835 (60% female) and 454 (36% female) patients were recruited into the derivation and validation cohorts respectively. A sex-specific model incorporating left atrial volume and left ventricular mass was created. The generic CMR PCWP showed significant differences between males and females (14.7 ± 4 vs. 13 ± 3.0 mmHg, P &gt; 0.001), not present with the sex-specific CMR PCWP (14.1 ± 3 vs. 13.8 mmHg, P = 0.3). The sex-specific, but not the generic, CMR PCWP was associated with HF hospitalization (hazard ratio 3.9, P = 0.0002) and MACE (hazard ratio 2.5, P = 0.001) over a mean follow-up period of 2.4 ± 1.2 years.

Conclusion
Accounting for sex improves precision and prognostic performance of CMR biomarkers for HF.
Name-Based Estimators of Intergenerational Mobility
Authors: Santavirta Torsten , Stuhler Jan 
Published: May 2024

Abstract
Recent studies use names—first and surnames—to estimate intergenerational mobility in sources that lack direct family links. While generating novel evidence on intergenerational transmission processes, it remains unclear how different estimators compare and how reliable they are. This paper evaluates the most popular name-based methods, using newly digitised records from Finland and US census data. We illustrate that their interpretation depends on sampling properties of the data, such as the overlap between the parent and child samples, which differ widely across studies. We correct for the attenuation bias from limited overlap and address other common problems encountered in applications.
Brugada syndrome in Japan and Europe: a genome-wide association study reveals shared genetic architecture and new risk loci
Authors: Ishikawa Taisuke , Masuda Tatsuo , Hachiya Tsuyoshi , Dina Christian , Simonet Floriane , Nagata Yuki , Tanck Michael W T , Sonehara Kyuto , Glinge Charlotte , Tadros Rafik , Khongphatthanayothin Apichai , Lu Tzu-Pin , Higuchi Chihiro , Nakajima Tadashi , Hayashi Kenshi , Aizawa Yoshiyasu , Nakano Yukiko , Nogami Akihiko , Morita Hiroshi , Ohno Seiko , Aiba Takeshi , Krijger Juárez Christian , Mauleekoonphairoj John , Poovorawan Yong , Gourraud Jean-Baptiste , Shimizu Wataru , Probst Vincent , Horie Minoru , Wilde Arthur A M , Redon Richard , Juang Jyh-Ming Jimmy , Nademanee Koonlawee , Bezzina Connie R , Barc Julien , Tanaka Toshihiro , Okada Yukinori , Schott Jean-Jacques , Makita Naomasa 
Published: May 2024

Abstract

Background and Aims
Brugada syndrome (BrS) is an inherited arrhythmia with a higher disease prevalence and more lethal arrhythmic events in Asians than in Europeans. Genome-wide association studies (GWAS) have revealed its polygenic architecture mainly in European populations. The aim of this study was to identify novel BrS-associated loci and to compare allelic effects across ancestries.

Methods
A GWAS was conducted in Japanese participants, involving 940 cases and 1634 controls, followed by a cross-ancestry meta-analysis of Japanese and European GWAS (total of 3760 cases and 11 635 controls). The novel loci were characterized by fine-mapping, gene expression, and splicing quantitative trait associations in the human heart.

Results
The Japanese-specific GWAS identified one novel locus near ZSCAN20 (P = 1.0 × 10−8), and the cross-ancestry meta-analysis identified 17 association signals, including six novel loci. The effect directions of the 17 lead variants were consistent (94.1%; P for sign test = 2.7 × 10−4), and their allelic effects were highly correlated across ancestries (Pearson’s R = .91; P = 2.9 × 10−7). The genetic risk score derived from the BrS GWAS of European ancestry was significantly associated with the risk of BrS in the Japanese population [odds ratio 2.12 (95% confidence interval 1.94–2.31); P = 1.2 × 10−61], suggesting a shared genetic architecture across ancestries. Functional characterization revealed that a lead variant in CAMK2D promotes alternative splicing, resulting in an isoform switch of calmodulin kinase II-δ, favouring a pro-inflammatory/pro-death pathway.

Conclusions
This study demonstrates novel susceptibility loci implicating potentially novel pathogenesis underlying BrS. Despite differences in clinical expressivity and epidemiology, the polygenic architecture of BrS was substantially shared across ancestries.