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(1) Introduction: New technologies that leverage gas hydrates phenomena include carbon capture and sequestrations. These processes are often semi-continuous and require regulation of the system's flow properties for proper operation. Accurate computational models for the viscosity of carbon dioxide hydrate systems at pre-nucleation conditions can be important for process design and control of such technologies. (2) Methods: This work validates the viscosity predictions of molecular dynamics simulations using previously measured experimental data. The TIP4P/Ice force field was used to model water, while the EPM2, TraPPE, and Zhang force fields were used for carbon dioxide. The Green-Kubo and Einstein formulations of viscosity and diffusivity were used in this work. (3) Results: All force fields overpredicted viscosity when compared to experimental data, but EPM2 resulted in lower discrepancies. Additionally, EPM2 was determined to model molecular behavior expected from the macroscopic trends in viscosity with respect to temperature and pressure. (4) Conclusions: The EPM2 force field more accurately predicted the viscosity of carbon dioxide hydrates systems at pre-nucleation conditions relative to TraPPE and Zhang.

Psoriasis is a chronic immune-mediated disease involving complex interaction of T cells and keratinocytes. The comprehensive pathogenesis of psoriasis is not fully understood but the IL-23/Th17 axis is a central pathway in driving disease development. Guselkumab is the first treatment of moderate-to-severe psoriasis that specifically targets the p19 subunit of IL-23. The benefit of guselkumab has been established by a number of clinical trials including demonstration of greater long-term efficacy in recent comparator trials. This review addresses the results of head-to-head trials (ECLIPSE, IXORA-R, and POLARIS) that compared guselkumab to secukinumab, ixekizumab, and fumaric acid esters. The previously demonstrated long-term efficacy of guselkumab has been corroborated by many recently published studies. The effective and safe profile, convenient dosing, and improved quality of life in patients make gulselkumab a viable first-line treatment option for moderate-to-severe psoriasis.

Hypothesis: Dilated cardiomyopathy (DCM) is an inevitable complication of Duchenne muscular dystrophy (DMD). Late gadolinium enhancement (LGE) on MRI occurs in DMD-related DCM, indicating myocyte death and remodeling. We hypothesize that patients with earlier onset of LGE develop a more rapid decline in heart function.Methods: We conducted a retrospective chart review, reviewing clinical data for DMD patients identified between January 2009 and July 2013. Subjects were cohorted by presence of LGE before age 14. We excluded patients in whom LGE status prior to age 14 was indeterminate. Results: Of the 41 subjects with complete data, 15 demonstrated LGE before age 14 (“early LGE”), and 26 had no LGE by age 14 (“controls”). Those with early LGE exhibited more rapid decline in LV fractional shortening (p=0.028). Patients with early LGE were younger at age of initiation of ACE inhibition (p=0.025), mineralocorticoid receptor antagonism (p= 0.0024), and beta blockade (p=0.0017), suggesting aggressive clinical management in response to abnormal MRI. There were no significant differences in LV dilation between the two groups (p=0.1547). Early LGE was not associated with obesity (p=0.32), age at loss of ambulation (p=0.31), or type of mutation. Conclusion: Early onset of myocardial fibrosis demonstrated by LGE on cardiac MRI is associated with earlier progression of cardiomyopathy despite earlier medication therapy. Identifying this risk, observed in 34% of our cohort during preadolescence, may guide medical therapy and counseling about cardiomyopathy progression. We suggest obtaining at least one MRI in DMD patients prior to age 14 to risk stratify patients.

A 17-year-old with severe hypertrophic cardiomyopathy (HCM) presented to the emergency department with symptoms of cough, shortness of breath, chest pain, and tactile fevers. She was initially admitted to the cardiac floor, and later transferred to the cardiothoracic intensive care unit on day 5 of illness with deterioration over the next week from BiLevel positive airway pressure to endotracheal intubation. The patient met criteria for severe acute respiratory distress syndrome (ARDS). Standard ARDS lung-protective strategies were refined in consideration of complications caused by her HCM. Such complications included dynamic cardiac outflow obstruction, myocardial ischemia with tachycardia, elevated pulmonary vascular resistance from diastolic dysfunction, and narrow fluid balance window to reduce pulmonary edema while maintaining adequate left ventricular preload. The patient remained refractory despite broad-spectrum antibiotics requiring multiple vasoactive medications, aggressive ventilator management, and inhaled nitric oxide. Social history revealed “vaping” cannabis with butane hash oil prior to symptom onset. Corticosteroids were initiated 2 weeks after initial presentation (day 9 of mechanical ventilation) with rapid recovery and resolution of illness. Acute respiratory distress syndrome is an aggressive disease in the intensive care unit. E-cigarette or vaping product use–associated lung injury is increasingly recognized as a cause of ARDS in adolescents and adults. A complete social history is essential and must be obtained early in all such patients presenting with symptoms of acute respiratory distress and revisited throughout the hospital stay if no other reason for the ARDS is discovered. Disease progression may be subacute with a long interval between onset of symptoms and peak symptoms. The risk of barotrauma is high despite lung-protective ventilation strategies. Management is supportive with resolution over days to weeks. However, other clinical factors may considerably complicate management in cases of underlying comorbidities.

Introduction The role of accountability in promoting patient adherence to medication is not well characterized. An accountability measurement tool (AMT) has been developed to quantify accountability but has not been investigated for use with patients with skin disease. We aim to test the reliability and validity of the AMT for patients with psoriasis. Methods A 12-item AMT was used for patients with psoriasis. English-speaking adults with psoriasis who were expected to continue their medication were asked to complete the questionnaire. Reliability was measured using Cronbach’s alpha. Validity was measured using Pearson’s correlation coefficient and t-tests between the AMT and other validated scales measuring fear of negative evaluation and self-regulation. Results A total of 30 patients were recruited for this study. The AMT demonstrated excellent internal consistency (Cronbach’s \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\alpha$$\end{document}α = 0.86). Total accountability positively correlated with fear of negative evaluation (r = 0.59), autonomous motivation (r = 0.46), introjected regulation (r = 0.60), and external regulation (r = 0.57), demonstrating good convergent validity. Divergent validity was supported by nonsignificant associations between psoriasis accountability and age, gender, race, education level, years with physician, and amotivation. Conclusions The AMT has been further validated for measuring accountability in patients with psoriasis. Electronic Supplementary Material The online version of this article (10.1007/s13555-020-00376-w) contains supplementary material, which is available to authorized users.

Medical therapy for pediatric heart failure is based on a detailed mechanistic understanding of the underlying causes, which are diverse and unlike those encountered in most adult patients. Diuresis and improved perfusion are the immediate goals of care in the child with acute decompensated heart failure. Conversion to maintenance oral therapy for heart failure is based on the results of landmark studies in adults, as well as recent pediatric clinical trials and heart failure guidelines. There will continue to be an important role for newer drugs, some of which are in active trials in adults, and some of which are already approved for use in children. The need to plan for clinical trials in children during drug development for heart failure is emphasized.

MEK inhibitors (MEKi) have shown efficacy in pediatric low-grade glioma as well as plexiform neurofibroma. MEKi have been associated with acute cardiac dysfunction in adults. Cardiac consequences in children are unknown. We performed a single center retrospective cohort study evaluating cardiac function by echocardiography (echo) in children and young adults 21 years receiving MEKi between October 2013 and May 2018. Blinded assessment of left ventricular function by fractional shortening (FS) and ejection fraction (EF) was performed on all available echocardiograms performed before, during, and following therapy, as well as after re-initiation of therapy. Twenty-six patients underwent MEKi therapy with echo follow-up during the study period. Twenty-four of these had complete echo data. Median follow-up was 12 months. Borderline EF (EF 53-57.9%) occurred in 12 (50%) patients; and 3 (12.5%) progressed to abnormal EF (EF 53%). Cardiac dysfunction, when it occurred, was mild (lowest documented EF was 45%, and lowest FS was 24.4%). EF abnormalities typically fluctuated during therapy, resolved off therapy, and recurred with MEKi re-initiation. No clinical or demographic differences were detected between those who maintained normal cardiac function and those who developed borderline or overt cardiac dysfunction. Symptomatic heart failure did not occur. In this cohort of children and young adults, MEKi use was associated with a high (50%) incidence of borderline or mildly decreased left ventricular function. There was no evidence of permanent cardiac dysfunction. Further evaluation in larger prospective trials is needed.

Background Allergic contact dermatitis (ACD) may occur secondary to topical antifungals containing potential allergens in their vehicles. Variation of allergenic ingredients among commonly used antifungal creams (AFCs) has not been well characterized. Objective The study goal was to assess the frequency of allergenic ingredients in 4 commonly used topical AFCs. Methods Topical AFCs (clotrimazole, ketoconazole, miconazole, and terbinafine) were selected, and the ingredient lists for these products were obtained from the US Food and Drug Administration's Online Label Repository via a proprietary name search. A systematic literature review was performed using the ingredient name on MEDLINE (PubMed) database to identify reports of ACD confirmed by patch testing. Results Of the 20 ingredients analyzed, 6 had frequent allergenic potential. Propylene glycol was the most common cause of ACD identified in the literature and is an ingredient in ketoconazole 2% and miconazole nitrate 2%. Ketoconazole 2% and miconazole nitrate 2% creams contained the highest number of potential allergens (n = 3) among the 4 creams analyzed. Conclusions Of the 4 creams, terbinafine hydrochloride 1% and clotrimazole 1% contained the least number of potential allergenic ingredients. Awareness of the allergenic potential of commonly used AFCs may help health care providers when evaluating patients with ACD.

Background Pediatric dilated cardiomyopathy is an uncommon diagnosis. Although significant surgical and medical advances have improved the care of patients with dilated cardiomyopathy, it still carries significant morbidity and mortality. It remains the most common diagnosis requiring pediatric heart transplantation. Aim of Review The etiology of dilated cardiomyopathy, the co-morbidity profile, and response to medications are all vastly different in the adult and pediatric populations. Current medical management is largely extrapolated from adult studies, which is wrought with limitations. Though much progress has been made, there continue to be considerable areas for expansion of knowledge and subsequent management of pediatric dilated cardiomyopathy. This review summarizes the current areas of improvement in risk stratification and acute management and highlights areas for future opportunity, as summarized in the Fifth International Conference on Cardiomyopathy in Children. Key Scientific Concepts of Review This review will describe current echocardiographic and laboratory tools used for risk stratification and prognostication, with a focus on the need for further refinement in their use in determining prognosis and timing of attempts to transition medical therapy. We will also focus on differences in pediatric and adult responses to pharmacotherapy and how this affects the treatment of pediatric decompensated heart failure. Lastly, we will describe surgical interventions for pediatric dilated cardiomyopathy. Mechanical ventricular assist devices are increasingly utilized in end-stage pediatric dilated cardiomyopathy, however, there remains considerable institutional practice variation in device candidacy, transplant listing after device implantation, and assessment for recovery while on device support. Although ventricular assist devices are the current standard of practice once a patient fails medical management, pulmonary artery banding as a temporizing palliative strategy that is emerging as a possible strategy in a select patient population.

Outcomes after cardiac transplantation have improved over past decades, but long-term graft survival remains limited in part because of uncertainty regarding clinical implications of donor-specific antibodies (DSAs). The purpose of this review is to consolidate recent advances in knowledge on the topic of DSA and their potential to impact long-term prognosis after heart transplantation.The presence of persistent DSA increases the risk of poor outcome after heart transplantation, including development of antibody-mediated rejection (AMR), graft failure, cardiac allograft vasculopathy, and mortality. Importantly, different DSA vary in clinical significance. DSA capable of activating the complement cascade portend a higher risk of developing AMR. human leukocyte antigen class I and class II antigens are expressed differently within the heart, and so, clinical manifestations of class I and class II DSA vary accordingly. Further, compared with class I, class II DSA carry an increased risk of graft loss and mortality. When comparing preexisting DSA with formation of de-novo DSA, de-novo DSA are associated with worse outcome.DSAs are generally associated worse long-term prognosis after heart transplantation but vary in their clinical significance. Recognition of specific risk profiles is essential for guiding posttransplant antibody management.

Allergic contact dermatitis (ACD) is a frequent problem, often caused from repeated exposure to an object or substance related to the patient's routine activities. We present a case of a well-demarcated, erythematous, scaly plaque on a finger caused from reading with an e-book device. Although metal from mobile devices can cause ACD, mobile device cases may cause irritation or contain additives that can also cause contact dermatitis. Similar presentations of contact dermatitis may become more common as technology use increases.

OBJECTIVE: Troponin-I (TN-I) levels are elevated following pediatric cardiac surgery with speculation that particular patterns may have prognostic significance. There is lack of procedure-specific data regarding postoperative TN-I levels in infants undergoing cardiac surgery. We hypothesized that TN-I elevation varies with type of surgery and persistent elevation predicts poor prognosis. METHODS: We prospectively measured serial TN-I levels (preoperatively, 4, 8, 12, 24, and 48 hours postoperatively) in 90 infants (age < 1 year) undergoing cardiac surgery: off cardiopulmonary bypass (CPB) (n = 15), on CPB (n = 43), and on CPB with ventricular incision (CPB with ventricular incision) (n = 32). RESULTS: All patients had undetectable baseline TN-I levels. The area under the curve (AUC) of TN-I levels over the 48-hour period was significantly different among the surgical groups (p < 0.002), and highest in patients with CPB with ventricular incision. Generally, TN-I levels peaked by 4 hours after surgery and returned to near-normal levels within 48 hours. A persistent TN-I rise beyond 8 hours after surgery was a strong predictor of postoperative hypoperfusion injury (defined as a composite endpoint of end-organ injury resulting from inadequate perfusion, OR 21.5; p = 0.001) and mortality (30% in those with persistently high TN-I, compared with 3.5% in the remaining patients; p < 0.001), independent of patient age, anatomy/complexity of surgery, and level of postoperative support. CONCLUSIONS: Our data provide benchmark values for TN-I levels following cardiac surgery in infants. Extent of TN-I elevation correlates with type of surgery. Persistent TN-I elevation beyond 8 hours after surgery is strongly associated with postoperative hypoperfusion injury and mortality.

The outcomes of pediatric ventricular assist device support in patients with diastolic heart failure have not been well described. This study reviews the North American experience with Berlin Heart EXCOR

A 2-week-old infant with normal intracardiac anatomy presented to the emergency department in a hypertensive crisis with acute cardiac failure. Despite extensive evaluation, no underlying disease was found. The patient’s hypertension and cardiac dysfunction resolved after 1 week of supportive care in the PICU, and she was discharged within 2 weeks of presentation. The patient’s history revealed transplacental exposure to the α-adrenergic agonist methyldopa for 10 weeks before delivery. Her age at presentation and the self-limited nature of cardiac sequelae with complete resolution of cardiac dysfunction suggest withdrawal effects from this exposure. Whereas the rebound hypertensive effects of α-adrenergic agonists are well established in the adult population, this report shows an unusual adverse outcome of in utero exposure to methyldopa.

Anomalous origin of a pulmonary artery from the ascending aorta is a congenital defect that can be complicated by pulmonary arterial hypertension, typically due to vascular disease if the anomaly is left uncorrected past 6 months of age. We describe a unique case of severe pulmonary arterial hypertension with this defect in a 1-month-old infant unexpectedly caused instead by bronchial compression from her dilated left pulmonary artery.

Dilated cardiomyopathy (DCM) inevitably afflicts patients with Duchenne muscular dystrophy (DMD) as a consequence of cell death induced by unguarded calcium influx into cardiomyocytes. This mechanism may also inhibit muscle relaxation in early stages of cardiomyopathy. ACE inhibition (ACEi) is known to delay the onset and slow the progression of DCM in DMD. The objective of this study is to use echocardiography to assess for preclinical cardiac changes consistent with intracellular calcium dysregulation before the onset of overt ventricular dysfunction, and to evaluate how prophylactic ACEi may alter these pre-cardiomyopathic changes in the pediatric DMD population. We examined 263 echocardiograms from 70 pediatric patients with DMD. We defined abnormal tonic contraction (TC) as left ventricular internal dimension in diastole (LVIDd) Z-score < -1.5. In our cohort, we found that TC is detectable as early as 8 years of age, and most commonly affects patients between 11 and 15 years. This effect was independent of LV mass and systolic function. Prophylactic ACEi decreased the incidence of TC (p = 0.007) and preserved cardiac function (p < 0.0001). Left ventricular TC often precedes DCM in DMD, most commonly affecting the 11- to 15-year-old age range. TC is not related to ventricular hypertrophy, but rather may be a clinical correlate of the "calcium hypothesis" of DMD pathophysiology. LV TC is thus a promising biomarker for early detection of cardiomyopathy in DMD. ACEi prophylaxis suppresses LV TC and delays the development of DCM in DMD.

The outcomes of pediatric ventricular assist device support in patients with diastolic heart failure have not been well described. This study reviews the North American experience with Berlin Heart EXCOR® ventricular assist device implants in children with such physiology. The Berlin Heart clinical database was reviewed. Patients with primary diastolic dysfunction are included in this study. Twenty pediatric patients with restrictive cardiomyopathy (n = 13), hypertrophic cardiomyopathy (n = 3), or congenital heart disease with restrictive physiology (n = 4) who were supported with EXCOR® were identified. Of these, nine (45%) were successfully bridged to transplant, one (5%) weaned from support, and 10 (50%) died after support was withdrawn. Of patients under age 3 years (n = 13), 38.5% survived, whereas those aged 3 or older (n = 7) had 71.4% survival (P = .35). Biventricular assist device (BiVAD) patients experienced a 27.3% survival, vs 77.8% for patients with left ventricular assist device only (P = .07). Primary causes of death included stroke, infection, acidosis, multisystem organ failure, and bleeding. Pediatric patients with diastolic heart failure comprise a high-risk population for mechanical circulatory support. However, half of patients with this physiology have been successfully supported to explant with EXCOR® . The trends toward higher mortality for younger patients and those receiving BiVAD support warrant consideration.

Pediatric HTs account for 13% of all HTs with >60% of recipients surviving at least 10 years post-HT. The purpose of this systematic review is to synthesize the literature on exercise capacity of pediatric HT recipients to improve understanding of the mechanisms that may explain the decreased exercise capacity. Six databases were searched for studies that compared the exercise capacity of HT recipients ≤21 years old with a control group or normative data. Sixteen studies were included. Pediatric HT recipients, as compared to controls or normative data, exhibit significantly higher resting HR, and at peak exercise exhibit significantly decreased HR, VO2 , power, work, minute ventilation, and exercise duration. Peak VO2 appears to improve within the first 2.5 years post-HT; peak work remains constant; and there is inconclusive evidence that peak HR, HR recovery, and HR reserve improve with time since HT. These results are discussed in the context of the mechanisms that may explain the impaired exercise capacity of pediatric HT recipients, including chronotropic incompetence, graft dysfunction, side effects of immunosuppression therapy, and deconditioning. In addition, the limited literature on rehabilitation after pediatric HT is summarized.

Mechanical circulatory support has been used for more than 30 yr to allow the heart to recover from ischemia and injury. There are limited pediatric data, however, on the efficacy of ECMO in the setting of post-transplantation support for primary graft dysfunction or rejection. Data from all patients at our university-affiliated, tertiary care children's hospital who underwent OHT between 1998 and 2010 and required subsequent ECMO support were analyzed. The primary outcome measure was survival to hospital discharge. Two hundred and three pediatric patients underwent OHT between 1998 and 2010 at our institution. Twenty-nine of these patients experienced post-transplantation cardiac failure requiring ECMO support, 18 of whom survived to hospital discharge (62%). Survival in the rejection and allograft vasculopathy group was 75%, and survival in patients with primary graft failure was 53% after ECMO support (p = 0.273). Patient survival to hospital discharge was not associated with ischemic time or duration of ECMO. ECMO provides hemodynamic support in the setting of cardiac failure and can be used successfully after pediatric OHT for primary graft dysfunction or rejection.

In the spectrum of mitral valve anomalies, unguarded mitral orifice is an exceedingly rare malformation, with only four cases described in the current literature. All previously reported cases have been associated with discordant atrioventricular connections. We describe the first known case of unguarded mitral valve orifice, in the setting of atrioventricular concordance, in a newborn with hypoplastic left heart syndrome.

Dilated cardiomyopathy (DCM) inevitably afflicts patients with Duchenne muscular dystrophy (DMD). Understanding of the molecular pathophysiology of DCM in DMD has advanced, but correlation of these advances with clinical disease is lacking. We sought to identify echocardiographic changes that occur