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Kawasaki Disease in the Time of COVID-19 and MIS-C: The International Kawasaki Disease Registry
No full textBackground: Patients with multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) have overlapping clinical features. We compared demographics, clinical presentation, management, and outcomes of patients according to evidence of previous SARS-CoV-2 infection. Methods: The International Kawasaki Disease Registry (IKDR) enrolled KD and MIS-C patients from sites in North, Central, and South America, Europe, Asia, and the Middle East. Evidence of previous infection was defined as: Positive (household contact or positive polymerase chain reaction [PCR]/serology), Possible (suggestive clinical features of MIS-C and/or KD with negative PCR or serology but not both), Negative (negative PCR and serology and no known exposure), and Unknown (incomplete testing and no known exposure). Results: Of 2345 enrolled patients SARS-CoV-2 status was Positive for 1541 (66%) patients, Possible for 89 (4%), Negative for 404 (17%) and Unknown for 311 (13%). Clinical outcomes varied significantly among the groups, with more patients in the Positive/Possible groups presenting with shock, having admission to intensive care, receiving inotropic support, and having longer hospital stays. Regarding cardiac abnormalities, patients in the Positive/Possible groups had a higher prevalence of left ventricular dysfunction, and patients in the Negative and Unknown groups had more severe coronary artery abnormalities. Conclusions: There appears to be a spectrum of clinical features from MIS-C to KD with a great deal of heterogeneity, and one primary differentiating factor is evidence for previous acute SARS-CoV-2 infection/exposure. SARS-CoV-2 Positive/Possible patients had more severe presentations and required more intensive management, with a greater likelihood of ventricular dysfunction but less severe coronary artery adverse outcomes, in keeping with MIS-C
Sodium-Glucose Co-Transporter 2 Inhibitors in Patients With Heart Failure With Preserved Ejection Fraction: Proposed Mechanisms, Recent Evidence, and Clinical Implications
No full textHeart failure (HF) affects approximately 6 million Americans and is projected to increase in prevalence as the population ages. While progress has been made in the treatment of heart failure with reduced ejection fraction (HFrEF), treatments for heart failure with preserved ejection fraction (HFpEF) are few and far between. Since HFpEF makes up half of all heart failure cases, its management may be the biggest unmet need in cardiology. Results from the recent EMPEROR-Preserved trial are encouraging. After 26 months, patients with HFpEF who used the sodium-glucose co-transporter 2 inhibitor empagliflozin had a lower risk of hospitalization for HF than patients taking a placebo. However, the trial did not show empagliflozin to reduce the risk of cardiovascular death, unlike the EMPEROR-Reduced trial, in which empagliflozin was associated with a reduced risk of both cardiac death and hospitalization for patients with HFrEF. The outcomes of these trials highlight the dissimilarities between the two diseases. While HFrEF is mainly a disease of cardiomyocyte injury and systolic dysfunction, HFpEF is a multifactorial syndrome of inflammation and endothelial dysfunction stemming from chronic diseases like hypertension, diabetes and obesity. While trials of empagliflozin for HFpEF did not show a mortality benefit, sodium-glucose co-transporter 2 inhibitors are promising additions to the management of HFpEF for their effects on the disease\u27s risk factors through weight loss, natriuresis, blood pressure lowering, and glycemic control
Systemic Sclerosis Is Associated With Increased In-Patient Mortality in Patients Hospitalized for Heart Failure
No full textAims: We aimed to analyse the characteristics and in-hospital outcomes of patients hospitalized for heart failure (HF) with co-morbid systemic sclerosis (SSc) and compare them to those without SSc, using data from the National Inpatient Sample from years 2016 to 2019. Methods and results: International Classification of Diseases, Tenth Revision diagnosis codes were used to identify hospitalized patients with a primary diagnosis of HF and secondary diagnoses of SSc from the National Inpatient Sample database from 2016 to 2019. Patients were divided into two groups: those with and without a secondary diagnosis of SSc. Baseline characteristics including demographics and co-morbidities, outcomes of mortality, length of stay (LOS), and costs were compared between the two groups. Multivariable logistic regression analysis was performed to adjust for confounders and assess the impact of SSc on in-hospital mortality, cost, and LOS. A total of 4 709 724 hospitalizations for HF were identified, with 8150 (0.17%) having a secondary diagnosis of SSc. These patients were predominantly female (82.3% vs. 47.8%; P = 0.01), younger (mean age of 67.4 vs. 71.4; P \u3c 0.01), and had significantly lower rates of traditional cardiovascular risk factors such as coronary artery disease (35.8% vs. 50.6%; P \u3c 0.01), hyperlipidaemia (39.1% vs. 52.9%; P \u3c 0.01), diabetes (22.5% vs. 49.1%; P \u3c 0.01), obesity (13.2% vs. 25.0%; P \u3c 0.01), and hypertension (20.2% vs. 23.8%; P \u3c 0.01). Higher rates of co-morbid pulmonary disease in the form of interstitial lung disease (23.1% vs. 2.0%; P \u3c 0.01) and pulmonary hypertension (36.6% vs. 12.7%; P \u3c 0.01) were noted in the SSc cohort. Unadjusted in-hospital mortality was significantly higher in the HF with SSc group [5.1% vs. 2.6%; odds ratio: 1.99; 95% confidence interval (CI): 1.60–2.48; P \u3c 0.001]. Unadjusted mortality was also higher among female (86.7% vs. 47.0%; P \u3c 0.01), Black (15.7% vs. 13.0%; P \u3c 0.01), and Hispanic (13.3% vs. 6.9%; P \u3c 0.01) patients in the SSc cohort. After adjusting for potential confounders, SSc remained independently associated with higher in-hospital mortality (adjusted odds ratio: 1.81; 95% CI: 1.44–2.28; P \u3c 0.001). Patients with HF and SSc also had longer LOS (6.4 vs. 5.4; adjusted mean difference [AMD]: 0.37, 95% CI: 0.05–0.68; P = 0.02) and higher hospitalization costs (57 128; AMD: 198.9; 95% CI: −4780 to 5178; P = 0.93). Conclusions: In patients hospitalized for HF, those with SSc were noted to have higher odds of in-hospital mortality than those without SSc. Patients with HF and SSc were more likely to be younger, female, and have higher rates of co-morbid interstitial lung disease and pulmonary hypertension at baseline with fewer traditional cardiovascular risk factors
Navigating Post-Traumatic Osteoporosis: A Comprehensive Review of Epidemiology, Pathophysiology, Diagnosis, Treatment, and Future Directions
No full textPost-traumatic osteoporosis (PTO) presents a significant challenge in clinical practice, characterized by demineralization and decreased skeletal integrity following severe traumatic injuries. This literature review manuscript addresses the knowledge gaps surrounding PTO, encompassing its epidemiology, pathophysiology, risk factors, diagnosis, treatment, prognosis, and future directions. This review emphasizes the complexity of the etiology of PTO, highlighting the dysregulation of biomineralization processes, inflammatory cytokine involvement, hormonal imbalances, glucocorticoid effects, vitamin D deficiency, and disuse osteoporosis. Moreover, it underscores the importance of multidisciplinary approaches for risk mitigation and advocates for improved diagnostic strategies to differentiate PTO from other musculoskeletal pathologies. This manuscript discusses various treatment modalities, including pharmacotherapy, dietary management, and physical rehabilitation, while also acknowledging the limited evidence on their long-term effectiveness and outcomes in PTO patients. Future directions in research are outlined, emphasizing the need for a deeper understanding of the molecular mechanisms underlying PTO and the evaluation of treatment strategies’ efficacy. Overall, this review provides a comprehensive overview of PTO and highlights avenues for future investigation to enhance clinical management and patient outcomes
Resolution of Fenfluramine-Associated Pulmonary Arterial Hypertension in Lennox-Gastaut Syndrome: A Case Report and Literature Review
No full textFenfluramine is a medication originally approved for weight loss before being withdrawn for an association with the development of pulmonary arterial hypertension (PAH) and cardiac valvulopathy. Interest in fenfluramine at lower doses has re-emerged for treatment of drug-resistant epilepsy (DRE). Here, we present a case of a patient with Lennox-Gastaut Syndrome (LGS) treated with fenfluramine with development of PAH and tricuspid regurgitation that resolved upon discontinuation. A 4-year-old female with LGS refractory to anti-seizure medications and neuromodulation was screened by echocardiogram and started on fenfluramine due to persistent seizures. At 6 months, the patient developed asymptomatic PAH and tricuspid regurgitation. The medication was discontinued, and 2 months post-cessation, an echocardiogram showed PAH and cardiac valvulopathy resolution. The patient was restarted on fenfluramine due to seizures, and follow up exam with echocardiogram 6 months following retrial showed no PAH recurrence. We present a case of LGS treated with fenfluramine complicated by PAH until cessation, with subsequent retrial tolerated. This is the first pediatric patient to develop cardiac abnormalities during treatment with fenfluramine, which has been demonstrated to be efficacious and safe in DRE at lower doses than those utilized for weight loss. With appropriate screening, fenfluramine should be considered as an adjunct treatment option for DRE in select patients, and PAH as a complication of the medication may be reversible and non-recurrent on retrial
Infant Sitting and Multi-Directional Reaching Skill
No full textWe tested twenty-one 6- to 10-month-old infants with a wide range of sitting experience in forward and rightward reaching during unsupported sitting on the floor. Sessions were video-recorded for further behavioral and machine learning-based kinematic analyses. All infants, including novice sitters, successfully touched and grasped toys in both directions. Infant falls, hand support, and base of support changes were rare. Infants with more sitting experience showed better upright posture than novice sitters. However, we found no differences in trunk displacement or reaching kinematics between directions or across sitting experience. Thus, multi-directional reaching is functional in both novice and experienced infant sitters. We suggest that trunk and arm stability in sagittal and frontal planes is integral to learning to sit
Inpatient Outcomes of Cerebral Venous Thrombosis in Patients With Malignancy Throughout the United States
No full textBackground and Purpose Cerebral venous thrombosis (CVT) is associated with a high degree of morbidity and mortality. Our objective is to elucidate characteristics, treatments, and outcomes of patients with cancer and CVT (CA-CVT). Methods The 2016–2019 National Inpatient Sample (NIS) database was queried for patients with a primary diagnosis of CVT. Patients with a currently active diagnosis of malignancy (CA-CVT) were then identified. Demographics and comorbidities were compared between CA-CVT and CVT patients. Subgroup analyses explored patients with hematopoietic cancer and non-hematopoietic cancers. Stroke severity and treatment were explored. Inpatient outcomes studied were discharge disposition, length of stay, and mortality. Results Between 2016 and 2019, 6,140 patients had a primary diagnosis code of CVT, and 370 (6.0%) patients had a coexisting malignancy. The most common malignancy was hematopoietic (n=195, 52.7%), followed by central nervous system (n=40, 10.8%), respiratory (n=40, 10.8%), and breast (n=40, 10.8%). These patients tended to be older than non-CA-CVT and were more likely to have coexisting comorbidities. CA-CVT patients had higher severity scores on the International Study of Cerebral Vein and Dural Sinus Thrombosis Risk Score (ISCVT-RS) and increased complications. In a propensity-score matched cohort, there were no differences in inpatient outcomes. Conclusion Malignancy occurs in 6% of patients presenting with CVT and should be considered a potential comorbidity in instances where clear causes of hypercoagulabilty have not been identified. Malignancy was linked to higher mortality rates. Nonetheless, after adjusting for the severity of CVT, the outcomes for inpatients with cancer-associated CVT were comparable to those without cancer, indicating that the increased mortality associated with malignancy is probably due to more severe CVT conditions
