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Background The association of cardiorenal syndrome (CRS) with mortality in children with dilated cardiomyopathy (DCM) is unknown. Methods With a modified Schwartz formula, we estimated glomerular filtration rates (eGFR) for children ≥1 year of age with DCM enrolled in the Pediatric Cardiomyopathy Registry at the time of DCM diagnosis and annually thereafter. CRS was defined as an eGFR of <90 mL/min/1.73 m 2 . Children with and without CRS were compared on survival and serum creatinine concentrations (SCr). The association between eGFR and echocardiographic measures was assessed with linear mixed-effects regression models. Results Of 285 eligible children with DCM diagnosed at ≥1 year of age, 93 were evaluable. CRS was identified in 57 of these 93 children (61.3 %). Mean (standard deviation) eGFR was 62.0 (22.6) mL/min/1.73 m 2 for children with CRS and 108.0 (14.0) for those without ( P  < 0.001); median SCr concentrations were 0.9 and 0.5 mg/dL, respectively ( P  < 0.001). The mortality hazard ratio of children with CRS versus those with no CRS was 2.4 (95 % confidence interval 0.8–7.4). eGFR was positively correlated with measures of left ventricular function and negatively correlated with age. Conclusions CRS in children newly diagnosed with DCM may be associated with higher 5-year mortality. Children with DCM, especially those with impaired left ventricular function, should be monitored for renal disease.

The National Asthma Education and Prevention Program published their most recent guidelines for management of acute asthma exacerbation, and methylxanthines such as aminophylline were not recommended.6In the emergency department, aminophylline appears to provide no additional benefit to optimal short-acting beta agonists and increases the frequency of adverse effects.7Even as failing to show a reduction in nebulised treatments or length of hospital stay in children, studies have actually shown increased toxicity in those being treated with methylxanthine for severe asthma exacerbation.8Therefore, although the data from Axelrod et al would suggest that there is no additional arrhythmia risk in postoperative cardiac patients, the combination of a possible arrhythmia risk in the asthmatic population, coupled with limited clinical benefit, would leave the authors to question the use of aminophylline in patients with asthma exacerbations.

Arrhythmias are common in patients admitted to the paediatric intensive care unit. We sought to identify the rates of occurrence and types of arrhythmias, and determine whether an arrhythmia was associated with illness severity and paediatric intensive care unit length of stay. This is a prospective, observational study of all patients admitted to the paediatric intensive care unit at the Children's Hospital at Montefiore from March to June 2012. Patients with cardiac disease or admitted for the treatment of primary arrhythmias were excluded. Clinical and laboratory data were collected and telemetry was reviewed daily. Tachyarrhythmias were identified as supraventricular tachycardia, ventricular tachycardia, and arrhythmias causing haemodynamic compromise or for which an intervention was performed. A total of 278 patients met the inclusion criteria and were analysed. There were 97 incidences of arrhythmia in 53 patients (19%) and six tachyarrhythmias (2%). The most common types of arrhythmias were junctional rhythm (38%), premature atrial contractions (24%), and premature ventricular contractions (22%). Tachyarrhythmias included three supraventricular tachycardia (50%) and three ventricular tachycardia (50%). Of the six tachyarrhythmias, four were related to placement or migration of central venous lines and two occurred during aminophylline infusion. Patients with an arrhythmia had longer duration of mechanical ventilation and paediatric intensive care unit stay (p<0.001). In multivariate analysis, central venous lines (odds ratio 3.1; 95% confidence interval 1.3-7.2, p=0.009) and aminophylline use (odds ratio 5.1; 95% confidence interval 1.7-14.9, p=0.003) were independent predictors for arrhythmias. Arrhythmias were common in paediatric intensive care unit patients (19%), although tachyarrhythmias occurred rarely (2%). Central venous lines and use of aminophylline were identified as two clinical factors that may be associated with development of an arrhythmia.

Purpose We demonstrate the complexities of managing pediatric patients on extracorporeal membrane oxygenation (ECMO) therapy requiring neurosurgery, focusing on systemic anticoagulation, cardiac function, and medically refractory intracranial pressure (ICP). Methods A 3.5-year-old female with Tetralogy of Fallot developed severe ischemic cerebral edema following post-operative cardiac arrest and required ECMO. This case, along with four additional cases of children requiring neurosurgery while on ECMO, was examined. Results Emergency neurosurgical intervention in the primary case led to significant improvement, highlighting the delicate balance between managing ECMO-induced anticoagulation and urgent neurosurgical needs. The additional cases had variable outcomes, emphasizing the challenges of caring for these critically ill patients. Conclusion Successful management of children requiring ECMO support and neurosurgical intervention requires thoughtful multidisciplinary care. This report illustrates some of the nuances in such decision-making, and demonstrates one potential path to a good outcome.

PurposeFollowing radical orchiectomy, surveillance and primary retroperitoneal lymph node dissection (RPLND) are acceptable options for the management of early stage pure testicular teratoma in adult patients; however, there is no uniform consensus. The aim of this study was to investigate survival outcomes of adults with early stage pure testicular teratoma based on management strategy.MethodsData was extracted from the National Cancer Database (NCDB) from testicular cancer patients diagnosed with clinical stage (CS) I pure teratoma (pT1-4N0M0S0) between 2004 and 2014. Kaplan-Meier and Cox regression analyses were used to assess clinical outcomes based on management strategy.ResultsOf the 61,167 patients diagnosed with testicular cancer, 692 (1.1%) had pure teratoma. Only individuals with CS I disease were considered (n = 237). The median age was 28 (23–35) years. Overall, 43 (18%) patients underwent RPLND and 194 (82%) patients were managed with surveillance. There was an increase in surveillance for CS I teratoma during the study period. Increasing distance from residence to treatment facility was an unadjusted predictor for undergoing primary RPLND (p < 0.001). Median follow-up was 54 months and there was no significant difference in overall survival between CS I teratoma patients managed with RPLND and those managed with surveillance (p = 0.13).ConclusionsThere has been a trend toward increasing adoption of surveillance for the management of early stage pure testicular teratoma in adults. Our findings suggest that surveillance provides comparable survival outcomes to primary retroperitoneal lymph node dissection in this setting.

Aydin Y, Koksal AR, Thevenot P, et al. J Hepatocell Carcinoma. 2020;15:1771-1786. The authors have advised due to an error that occurred inadvertently at the time of figure assembly, Figure 3 on page 1587 is incorrect. The high magnification images of Huh-7.5 and HLE should be interchanged. The correct Figure 3 is shown below. Figure 3 Characterization of exosomes released from an autophagy-deficient and an autophagy-competent HCC cell line. (A) Cryogenic transmission electron microscopy (cryoTEM) images of exosomes purified from culture supernatants of autophagy-deficient (Huh-7.5) and autophagy-competent (HLE) HCC lines. Left panel (low magnification). Right panel (high magnification). (B) Top panel: Representative image of exosome Brownian motion in liquid phase using Nanoparticle tracking analysis (NTA). Bottom panel: Size distribution profile of exosomes with mean and mode of the population diameter ± standard error. (C) Exosome concentration in the culture supernatant quantified by NTA. The results are expressed as the mean ± standard deviation and analyzed by Student's t-test. *P < 0.05. (D) Representative Western blot for TSG101 and CD9 in cell lysates and exosomes isolated from autophagy-deficient Huh-7.5 and autophagy-competent HLE cell cultures. All experiments were performed in triplicate. The authors apologize for the error and advise it does not affect the results and conclusion of the paper.

Background Arrhythmia symptoms are frequent complaints in children and often require a pediatric cardiology evaluation. Data regarding the clinical utility of wearable technologies are limited in children. We hypothesize that an Apple Watch can capture arrhythmias in children. Methods We present an analysis of patients ≤18 years-of-age who had signs of an arrhythmia documented by an Apple Watch. We include patients evaluated at our center over a 4-year-period and highlight those receiving a formal arrhythmia diagnosis. We evaluate the role of the Apple Watch in arrhythmia diagnosis, the results of other ambulatory cardiac monitoring studies, and findings of any EP studies. Results We identify 145 electronic-medical-record identifications of Apple Watch , and find arrhythmias confirmed in 41 patients (28%) [mean age 13.8 ± 3.2 years]. The arrythmias include: 36 SVT (88%), 3 VT (7%), 1 heart block (2.5%) and wide 1 complex tachycardia (2.5%). We show that invasive EP study confirmed diagnosis in 34 of the 36 patients (94%) with SVT (2 non-inducible). We find that the Apple Watch helped prompt a workup resulting in a new arrhythmia diagnosis for 29 patients (71%). We note traditional ambulatory cardiac monitors were worn by 35 patients (85%), which did not detect arrhythmias in 10 patients (29%). In 73 patients who used an Apple Watch for recreational or self-directed heart rate monitoring, 18 (25%) sought care due to device findings without any arrhythmias identified. Conclusion We demonstrate that the Apple Watch can record arrhythmia events in children, including events not identified on traditionally used ambulatory monitors. Plain language summary Wearable devices, such as smart watches, have become popular for the monitoring of health, particularly for people with heart conditions. Wearable devices have been well-studied in adults, however there is less information available on their effectiveness in monitoring children’s health. We reviewed the heart electrical recordings of a group of children who submitted recordings obtained from their Apple Watches during moments when they felt as though their heart’s rhythm was abnormal. The Apple Watches captured rhythm abnormalities that matched the diagnoses obtained using heart monitors used clinically. This study shows that use of Apple Watches can enable clinicians to identify abnormalities that many traditional at-home monitoring devices do not detect. Thus, wearable devices, such as the Apple Watch, could be used to help identify heart rhythm disorders in children. Zahedivash et al. undertake a single center retrospective analysis of patients less than 18 years of age with history of an arrhythmia to determine whether a wearable device can capture arrhythmias. Arrhythmias are identified in 28% of patients, mainly the difficult to identify supraventricular tachycardias.

Hydrophobins are small proteins, characterised by the presence of eight positionally conserved cysteine residues, and are present in all filamentous asco- and basidiomycetes. They are found on the outer surfaces of cell walls of hyphae and conidia, where they mediate interactions between the fungus and the environment. Hydrophobins are conventionally grouped into two classes (class I and II) according to their solubility in solvents, hydropathy profiles and spacing between the conserved cysteines. Here we describe a novel set of hydrophobins from Trichoderma spp. that deviate from this classification in their hydropathy, cysteine spacing and protein surface pattern. Phylogenetic analysis shows that they form separate clades within ascomycete class I hydrophobins. Using T. atroviride as a model, the novel hydrophobins were found to be expressed under conditions of glucose limitation and to be regulated by differential splicing.

Hepatitis C virus (HCV) infection compromises the natural defense mechanisms of the liver leading to a progressive end stage disease such as cirrhosis and hepatocellular carcinoma (HCC). The hepatic stress response generated due to viral replication in the endoplasmic reticulum (ER) undergoes a stepwise transition from adaptive to pro-survival signaling to improve host cell survival and liver disease progression. The minute details of hepatic pro-survival unfolded protein response (UPR) signaling that contribute to HCC development in cirrhosis are unknown. This study shows that the UPR sensor, the protein kinase RNA-like ER kinase (PERK), mediates the pro-survival signaling through nuclear factor erythroid 2-related factor 2 (NRF2)-mediated signal transducer and activator of transcription 3 (STAT3) activation in a persistent HCV infection model of Huh-7.5 liver cells. The NRF2-mediated STAT3 activation in persistently infected HCV cell culture model resulted in the decreased expression of hepatocyte nuclear factor 4 alpha (HNF4A), a major liver-specific transcription factor. The stress-induced inhibition of HNF4A expression resulted in a significant reduction of liver-specific microRNA-122 (miR-122) transcription. It was found that the reversal of hepatic adaptive pro-survival signaling and restoration of miR-122 level was more efficient by interferon (IFN)-based antiviral treatment than direct-acting antivirals (DAAs). To test whether miR-122 levels could be utilized as a biomarker of hepatic adaptive stress response in HCV infection, serum miR-122 level was measured among healthy controls, and chronic HCV patients with or without cirrhosis. Our data show that serum miR-122 expression level remained undetectable in most of the patients with cirrhosis (stage IV fibrosis), suggesting that the pro-survival UPR signaling increases the risk of HCC through STAT3-mediated suppression of miR-122. In conclusion, our data indicate that hepatic pro-survival UPR signaling suppresses the liver-specific HNF4A and its downstream target miR-122 in cirrhosis. These results provide an explanation as to why cirrhosis is a risk factor for the development of HCC in chronic HCV infection.

This cross-sectional study examines content policies and assesses behaviors of consumer chatbots in response to adolescent health crises.