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Background: Spontaneous pneumomediastinum (SP) is defined as the presence of free air in the mediastinum without a clear precipitating cause. Whilst uncommon and with a relatively benign course, SP is often thoroughly investigated by surgeons as spontaneous oesophageal perforation (Boerhaave’s syndrome) must be identified early due to high morbidity and mortality. This study aimed to evaluate current practice in the investigation and management of SP at our institution. Methods: All patients with imaging-confirmed pneumomediastinum at a tertiary hospital from 2014 to 2022 were reviewed. Those with precipitating trauma, recent surgery or aerodigestive instrumentation were excluded. Results: 70 patients were included; 4 (5.7%) patients were diagnosed with Boerhaave’s syndrome based on computed tomography (CT) evidence of oesophageal injury alone and were treated with immediate surgery. The remaining 66 patients were diagnosed with SP. In this group, common predisposing factors were young age, smoking, illicit drugs and alcohol. The lead presenting symptoms were dyspnoea, chest pain, vomiting and cough. Of the 13 different admitting specialties, respiratory and upper gastrointestinal surgery were most common. Of those patients diagnosed with SP, 37 (54%) patients underwent imaging to exclude oesophageal injury; 30 (43%) were assessed using CT with oral contrast, 16 (23%) had a fluoroscopic contrast oesophagram, and 9 were investigated with both modalities. Conclusions: SP is uncommon, benign and does not require operative intervention. Multiple specialities manage SP at our institution and the use of imaging modalities differs between teams. We propose a management algorithm to assist clinicians unfamiliar with SP, risk-stratify patients for oesophageal injury and avoid unnecessary investigation.

Pediatric renal tumors are a rare entity and majority of these tumors are accounted for by Wilms tumor. The second most common renal tumor is clear cell sarcoma of the kidney (CSSK). Most of the CSSK have either BCOR-internal tandem duplication (ITD) or YWHAE-NUTM2B/E fusion. The sarcomas with BCOR-CCNB3 fusion are well documented in soft tissue and bone tumors, but are extremely rare in the pediatric renal setting. We are reporting an extremely rare case of pediatric clear cell sarcoma of the kidney (CSSK) with BCOR-CCNB3 fusion, which was a diagnostic challenge on morphological grounds. A final diagnosis could only be reached after multiple reviews and NGS based RNA fusion testing. We have also performed a brief review of literature which revealed eight (8) other cases of this rare entity.

This study aims to assess the Effect of Investment, Labor, Inflation, and Consumption on Gross Regional Domestic Income of North Sumatra for the 2017-2020 period. This research method is descriptive quantitative. The sample of this research is 120 data on the Gross Regional Domestic Income of North Sumatra for the 2017-2020 period. Method of data analysis using multiple linear regression test. The results showed, partially the results of the t test are as follows, a) the investment variable has a tcount value of -0.262, with a sig value of 0.794, and a -tcount > -ttable value, namely -0.262 > -1.980; b) the labor variable has a tcount value of 23.450, with a sig value of 0.000, and a tcount > ttable that is 23.450>1.980; c) the inflation variable has a tcount value of -0.092, with a sig value of 0.927, and a value of -tcount > -ttable that is -0.092>-1.980; d) the consumption variable has a tcount value of 5.122, with a sig value of 0.000, and a tcount > ttable that is 5.122>1.980. The results of the F test are the Fcount value of 230.202, with a sig value of 0.000, and Ftable (120-4-1=115) of 2.45. Partially, investment and inflation variables have no impact on GRDP in North Sumatra during the 2017-2020 period. Meanwhile, partially labor and consumption have an effect on GRDP in North Sumatra during the 2017-2020 period. Simultaneously, investment, labor, inflation, and consumption affect the GRDP in North Sumatra during the 2017-2020 period. Keywords: Inflation, Investment, Consumption, Gross Regional Domestic Product, Labor

Noble gases are widely used as physically based climate proxies, notably in dissolved water samples as tracers of past recharge temperature in groundwater and air-sea gas exchange processes in seawater. Recent advances in measuring large volume samples of dissolved noble gas isotopic ratios at high precision have expanded the range of climate parameters that can be interpreted.We build on prior methods for measuring noble gas stable isotopes at high precision with a new large volume equilibration (LVE) method wherein sample gases are equilibrated in the sample flask between the dissolved phase and the headspace. The original dissolved gas composition is determined by measuring the headspace gases and correcting for the equilibrium dissolved gas content of the discarded water using known solubilities and fractionation factors. We evaluate the accuracy and precision of this method with air-equilibrated water standards of known noble gas composition RESULTS: Replicate air-equilibrated water standards and field measurements demonstrate that the LVE method achieves comparable precision to prior methods, with major advantages of measuring the Ne content as a constraint on excess air and allowing for long term sample storage. Isotope ratios measured with the LVE method in replicate samples are consistent between two laboratories, and LVE elemental noble gas abundances agree closely with replicate samples measured using established copper tube methods and static noble gas mass spectrometry CONCLUSIONS: The new LVE method enables reconstruction of past water table depths at ±1 m precision along with excess air, recharge temperature, and age and hydrogeochemical indicators. It has wide application to investigating climate signals and physical gas exchange processes in groundwater and seawater.

Treatment for acute coronary syndrome (ACS) in women during pregnancy is challenging. Current standard treatment for ACS includes coronary angioplasty with guideline-directed medical therapy including aspirin, P2Y12 inhibitors, beta-blockers, angiotensin converting enzyme inhibitors, which may portend adverse effects to the fetus. ACS increases ischemic and obstetric complications during pregnancy and the postpartum period. Management of these patients necessitates balancing the potential risks and benefits to both maternal and fetal health. We present a case of a 37-year-old female with a background of hypertension and hyperlipidemia who presented with Non-ST segment elevation myocardial infarction (NSTEMI). The urine pregnancy test that was performed turned positive with an estimated gestational age of four weeks. After counselling on the potential risks and benefits, a diagnostic angiogram was performed which revealed triple vessel disease with critical stenosis in all three vessels. Percutaneous coronary intervention (PCI) was performed successfully with precautions taken to minimize radiation exposure to the fetus. In such cases, there is a fundamental trade-off between maternal and fetal health in the treatment of NSTEMI. Medications used for the treatment of acute coronary syndrome will need to be balanced against the potential risks to the fetus. Invasive coronary angiogram results in exposure to potentially teratogenic ionizing radiation and hence all efforts must be made to minimize exposure. Thus, risks and benefits of treatment ought to be discussed with patients and measures need to be taken to minimize potential harm to both the mother and fetus.

Over the past decade, several distinct novel renal epithelial neoplasms driven by underlying tuberous sclerosis comples ( TSC)/ mammalian target of rapamycin (MTOR) pathway mutations have been described. We report herein two distinctive TSC2 -mutated renal cell carcinomas which do not fit any previously described entity. The two renal carcinomas occurred in young patients (ages 10 and 31 y), and were characterized by highly permeative growth within the kidney with metastases to perirenal lymph nodes. The neoplastic cells were predominantly large, multinucleated giant cells having variably eosinophilic to xanthomatous cytoplasm with basophilic stippling and frequent vacuolization. While the discohesive nature of the neoplastic cells, xanthomatous cytoplasm, immunoreactivity for histiocytic markers and minimal immunoreactivity for conventional epithelial markers raised the possibility of a histiocytic neoplasm, multifocal immunoreactivity for cytokeratin 20 helped establish their epithelial nature. Despite the aggressive growth pattern of these neoplasms and lymph node metastases, mitotic figures were rare and Ki-67 indices were low (1%). One patient with follow-up shows no evidence of disease seven years after nephrectomy with no adjuvant therapy. Next-generation sequencing demonstrated TSC2 mutations in each case. By immunohistochemistry, downstream markers of mTOR pathway activation S6K1, 4EBP1, and glycoprotein nonmetastatic melanoma protein B were all highly expressed in these neoplasms, suggesting mTOR pathway activation as the neoplastic driver. While the cytokeratin 20 immunoreactivity and focal basophilic cytoplasmic stippling suggest a relationship to eosinophilic solid and cystic renal cell carcinoma, and cytoplasmic vacuolization suggests a relationship to eosinophilic vacuolated tumor, these neoplasms appear to be distinctive given their permeative growth patterns and predominant xanthomatous giant cell morphology. Addition of cytokeratin 20 to a panel of epithelial markers helps avoid misdiagnosis in such cases.

Children with low-grade gliomas have excellent long-term survival outcomes. The development of therapies targeted to the driver mutations along the Mitogen Activated Protein (MAP) kinase signalling pathway are providing long-term stability for many patients with these tumours. Given the frequency of these tumours residing within or near the suprasellar region, our patients commonly suffer from hormone deficiencies. In Australia, the Pharmaceutical Benefits Scheme currently restricts growth hormone therapy to patients who are not being actively treated for cancer, including those receiving targeted therapies. This viewpoint hopes to facilitate an important discussion amongst our colleagues as to whether this should be changed to allow growth hormone to become available to children on chronic tumour suppressive therapy.

The magnitude of global cooling during the Last Glacial Maximum (LGM, the coldest multimillennial interval of the last glacial period) is an important constraint for evaluating estimates of Earth's climate sensitivity1,2. Reliable LGM temperatures come from high-latitude ice cores3,4, but substantial disagreement exists between proxy records in the low latitudes1,5-8, where quantitative low-elevation records on land are scarce. Filling this data gap, noble gases in ancient groundwater record past land surface temperatures through a direct physical relationship that is rooted in their temperature-dependent solubility in water9,10. Dissolved noble gases are suitable tracers of LGM temperature because of their complete insensitivity to biological and chemical processes and the ubiquity of LGM-aged groundwater around the globe11,12. However, although several individual noble gas studies have found substantial tropical LGM cooling13-16, they have used different methodologies and provide limited spatial coverage. Here we use noble gases in groundwater to show that the low-altitude, low-to-mid-latitude land surface (45degrees south to 35degrees north) cooled by 5.8±0.6degrees Celsius (mean±95% confidence interval) during the LGM. Our analysis includes four decades of groundwater noble gas data from six continents, along with new records from the tropics, all of which were interpreted using the same physical framework. Our land-based result broadly supports a recent reconstruction based on marine proxy data assimilation1 that suggested greater climate sensitivity than previous estimates5-7.

Introduction Gastroesophageal reflux disease (GERD) is associated with accelerated decline in lung health in children with cystic fibrosis (CF). Thus, antireflux surgery (ARS) is offered to a selected CF cohort with refractory GERD, but outcomes remain poorly investigated. This study aimed to determine the incidence of GERD in children with CF and to evaluate complications and outcomes of ARS. Materials and Methods A systematic literature-based search was conducted using various online databases according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The number of GERD cases in pediatric CF cohorts who underwent diagnostic investigation(s) was recorded. Data on postoperative complications and outcomes (including symptoms, lung function, and nutritional status) following ARS were analyzed. Results Ten articles (n = 289 patients) met the defined inclusion criteria (51% male; age range, 0.5 month–36 years). The overall incidence of GERD was 46% (range, 19–81%), derived from seven studies (n = 212 patients). Four publications (n = 82 patients) reported on ARS due to uncontrolled GERD. All ARSs were Nissen fundoplication (majority with gastrostomy placement). Major postoperative complications occurred in 15 (18%) patients, two required redo-ARS. Median follow-up time was 2 years (range, 3 months–6 years); 59% showed symptom improvement, and pulmonary exacerbations and decline in lung function were reduced. Nutritional status mainly improved in milder CF cases. There were no deaths related to ARS. Conclusion Approximately half of pediatric CF patients have GERD. Published data for children with CF are limited and heterogeneous in terms of GERD diagnosis and outcomes following ARS. However, ARS has shown to slow the deterioration of lung function in CF.

Background: Implantable cardioverter defibrillator (ICD) acceptance and shock anxiety are issues that clinicians should address to improve quality of life among device recipients. Previous findings have indicated that younger patients experience poorer device adjustment. The purpose of this study was to examine age and ICD-specific quality-of-life outcomes in a large sample of Canadian ICD patients. We tested the hypothesis that patient age is related to device acceptance and shock anxiety in an Alberta (Canada) ICD population. Methods: The Florida Patient Acceptance Survey (FPAS) and Florida Shock Acceptance Survey (FSAS) were completed by ICD patients attending the Cardiac Implantable Electrical Device Clinics in Alberta. The population was dichotomized into those aged ≤ 65 years (younger) and those aged > 65 years (older). Sex, ICD shock history, and remote monitoring use were also examined. Results: Surveys were completed by 126 younger (53 ± 11 years; 79% male) and 216 older (74 ± 6 years; 85% male) patients. Younger, compared with older, patients had greater device-related distress (P < 0.001) and more body-image concerns (P < 0.001), but no differences in return to function or positive appraisal. Younger patients reported lower total device acceptance (P = 0.001) and greater total shock anxiety (P < 0.001) compared with older patients. Conclusions: ICD patients aged ≤ 65 years reported poorer device acceptance and greater shock anxiety than older patients. Younger patients may require targeted interventions addressing adjustment to the ICD, and impact of the ICD on body image. Moreover, education about the relatively low probability of shocks may alleviate shock anxiety in younger patients. Résumé: Contexte: L’acceptation d’un défibrillateur cardioverteur implantable (DCI) et la crainte des chocs de DCI sont des problèmes auxquels les cliniciens devraient s’attaquer pour améliorer la qualité de vie des patients qui sont porteurs d’un DCI. Selon des observations antérieures, les plus jeunes patients ont davantage de mal à s’adapter au dispositif. L’étude avait pour objet l’examen des paramètres de la qualité de vie particuliers à l’âge et au port d’un DCI dans un vaste échantillon de patients canadiens porteurs de ce dispositif. Nous avons testé l’hypothèse voulant que l’âge du patient soit lié à l’acceptation du dispositif et à la crainte des chocs chez des Albertains porteurs d’un DCI. Méthodologie: Les patients des cliniques de l'Alberta (Canada) des dispositifs cardiaques électroniques implantables qui étaient porteurs d’un DCI ont rempli les questionnaires FPAS (Florida Patient Acceptance Survey) et FSAS (Florida Shock Acceptance Survey). Les patients ont été partagés en deux groupes selon leur âge, soit ceux de 65 ans et moins (plus jeunes patients) et ceux de plus de 65 ans (patients âgés). Nous avons aussi examiné les facteurs suivants : sexe, chocs de DCI antérieurs et utilisation d’une télésurveillance. Résultats: Les questionnaires ont été remplis par 126 plus jeunes patients (53 ± 11 ans; 79 % d’hommes) et 216 patients âgés (74 ± 6 ans; 85 % d’hommes). Chez les plus jeunes patients, par rapport aux patients âgés, la détresse causée par le dispositif était plus grande (p < 0,001) et il y avait davantage de préoccupations liées à l’image corporelle (p < 0001), mais il n’y avait pas de différences pour ce qui est de la reprise des activités ou de l’évaluation positive du dispositif. Chez les plus jeunes patients, l’acceptation totale du dispositif était moindre (p = 0,001) et la crainte des chocs était plus grande (p < 0,001) que chez les patients âgés. Conclusions: Chez les porteurs d’un DCI de 65 ans et moins, le dispositif était moins bien accepté, et la crainte des chocs était plus grande que chez les patients âgés. Chez les plus jeunes patients, des interventions ciblées axées sur l’adaptation au DCI et les répercussions du DCI sur l’image corporelle pourraient être nécessaires. De plus, l’éducation à propos de la probabilité relativement faible de subir des chocs pourrait réduire cette crainte chez les plus jeunes patients.

Constraining the magnitude of past hydrological change may improve understanding and predictions of future shifts in water availability. Here we demonstrate that water-table depth, a sensitive indicator of hydroclimate, can be quantitatively reconstructed using Kr and Xe isotopes in groundwater. We present the first-ever measurements of these dissolved noble gas isotopes in groundwater at high precision (≤0.005‰ amu

Background Frequent syncope is linked to poorer health-related quality of life (HRQoL). Recurrent syncope has been observed to reduce in all groups after seeing a syncope expert and enrolling in a clinical trial. It is unknown if HRQoL improves with this reduction in syncope recurrence. Objectives We examined the change in HRQoL over time in vasovagal syncope (VVS) patients seen by a syncope expert and enrolled in a trial. We also explored whether change differed with treatment or the frequency of fainting during follow up. Methods The Short Form Health Survey (SF36) was completed at baseline (BL), 6 m, and 12 m post-enrollment by VVS patients in the 1st and 2nd Prevention of Syncope Trials, which were multi-centered, randomized, placebo-controlled trials of metoprolol (POST) and fludrocortisone (POST2). Differences in HRQoL at BL, 6 m, and 12 m were analyzed and compared by faints in follow-up and randomization group. Results Complete study data were available for 143 VVS patients (40 ± 17 years, 62% F). Over 12 months, patients reported improvement in all SF36 dimensions except for bodily pain. Post hoc analyses indicated that differences first occurred between BL and 6 m for all but general health. Fainting in follow-up or drug randomization group did not diminish the improvements. The baseline syncope burden was not different whether patients' HRQoL improved or not. Conclusion HRQoL of VVS patients improves over time after enrolling in a clinical trial, even with recurrent faints or randomization to placebo. Improvements may result from alternative factors, such as interaction with experts or patient adjustment.

Background: Implantable cardioverter defibrillator (ICD) therapy is lifesaving; however, real-world data regarding the proportion of patients eligible for a primary prevention ICD and subsequent use remain sparse. This study evaluated rates of primary prevention ICD eligibility and use among patients in heart function clinics (HFCs) and to identify reasons for nonimplantation. Methods: A retrospective study was performed of patients seen at HFCs in Alberta, Canada, from 2013 to 2015. Demographics, comorbidities, clinical indications, and reasons for nonimplantation were abstracted. Eligibility was defined according to the 2008 American College of Cardiology/American Heart Association/Heart Rhythm Society ICD, 2012 American College of Cardiology/American Heart Association/Heart Rhythm Society Focused Update, and 2013 Canadian Cardiovascular Society Cardiac Resynchronization Therapy guidelines. Logistic regression was used to calculate an odds ratio (OR) and 95% confidence interval (CI) for predictors of nonimplantation. Results: Among 1239 patients in HFCs, the median age was 70 years (interquartile range, 59-80), 67% were male, and the median left ventricular ejection fraction was 0.40 (interquartile range, 0.28-0.53). Overall, 45% of patients (n = 553) met guideline criteria for an ICD, and of those, 36% (n = 198) received a device. Among device nonrecipients, 52% (n = 185) had no documented reason for nonimplantation. The most common reason for nonimplantation among nonrecipients was patient preference (48%). Predictors associated with nonimplantation were age more than 75 years (OR, 1.92; 95% CI, 1.31-2.82) and history of cancer (OR, 2.26; 95% CI, 1.07-4.78). At 3 years follow-up, 27% of nonrecipients were deceased. Conclusions: We found that one-third of patients who met guideline criteria received an ICD and that documentation for nonimplantation was poor. Résumé: Contexte: Le défibrillateur cardioverteur implantable (DCI) sauve des vies. Or, les données recueillies dans la pratique réelle concernant le pourcentage de patients admissibles à l’implantation d’un tel dispositif en prévention primaire et l’utilisation subséquente de ce dispositif sont très limitées. Cette étude a évalué le taux de patients admissibles à la pose d’un DCI en prévention primaire et le taux d’utilisation de ce dispositif chez des patients traités en clinique de cardiologie. Elle a également recensé les motifs de non-implantation. Méthodologie: Une étude rétrospective a été réalisée chez des patients traités de 2013 à 2015 dans plusieurs cliniques de cardiologie de l’Alberta, au Canada. Les données relatives aux caractéristiques démographiques, aux comorbidités, aux indications cliniques et aux motifs de la non-implantation ont été extraites. L’admissibilité était définie en fonction des lignes directrices de 2008 de l’American College of Cardiology, de l’American Heart Association et de la Heart Rhythm Society sur le DCI, de leur mise à jour ciblée en 2012 et des lignes directrices de 2013 de la Société canadienne de cardiologie sur la thérapie de resynchronisation cardiaque. Une régression logistique a été utilisée pour calculer le risque relatif approché (RRA) et l’intervalle de confiance (IC) à 95 % associés aux facteurs de prédiction de la non-implantation. Résultats: L’âge médian des 1 239 patients traités en clinique de cardiologie était de 70 ans (plage interquartile : 59 – 80 ans); 67 % d’entre eux étaient des hommes, et la fraction d’éjection ventriculaire gauche médiane était de 0,40 (plage interquartile : 0,28 – 0,53). D’une manière générale, 45 % des patients (n = 553) répondaient aux critères énoncés dans les lignes directrices pour la pose d’un DCI, et 36 % d’entre eux (n = 198) ont reçu un dispositif. Parmi les patients qui n’ont pas reçu de dispositif, aucun motif justifiant la non-implantation n’a été documenté chez 52 % des patients (n = 185); chez les 48 % des patients restants, le motif le plus courant pour justifier la non-implantation a été la préférence du patient. Les facteurs de prédiction associés à la non-implantation ont été l’âge (plus de 75 ans; RRA : 1,92; IC à 95 % : 1,31 – 2,82) et les antécédents de cancer (RRA : 2,26; IC à 95 % : 1,07 – 4,78). Après 3 ans de suivi, 27 % des patients qui n’avaient pas reçu de dispositif étaient décédés. Conclusions: Nous avons constaté que le tiers des patients qui répondaient aux critères énoncés dans les lignes directrices ont reçu un DCI et que les motifs justifiant la non-implantation étaient mal documentés.