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Purpose Lymphangioleiomyomatosis (LAM) is a rare, progressive, frequently lethal cystic lung disease that almost exclusively affects women. Prognostic information in LAM has been limited by small numbers and heterogeneous study methodology. Early retrospective cohorts cited 5- and 10-year mortality of 40 and 80%, respectively. More recently, mortality at 10 years has been estimated to be approximately 10-20% from the onset of symptoms and 30% at 10 years from the time of lung biopsy but varies widely in individual patients. Given the heterogeneous disease course, it would be useful to establish which clinical characteristics are associated with survival to develop prediction models for disease outcome. Methods The LAM Foundation maintains a population-based registry of 1,149 registered self-identified LAM patients. Of these, 590 have completed a 'General Information/Clinical History Questionnaire' with limited demographic and clinical data, 410 of whom were identified as U.S. residents and provided date of birth. Vital status was obtained on all 410 participants through December 31, 2007 by linking patient identifiers and the National Death Index. Survival time was calculated as the time since first lung-related symptom or physician diagnosis until censoring (still alive, received lung transplant, or died). Cox proportional hazard analysis evaluated the association of demographic and clinical features with survival. Results Among the 410 subjects, there were 50 deaths and 55 lung transplantations during a median of 10.4 years of observation time. The estimated median transplant-free survival time for LAM patients in the United States is 29 years from symptom onset and 23 years from diagnosis. The estimated 10-year survival transplant-free was 86%. Age at disease onset, smoking status, race, presence of tuberous sclerosis, occurrence of pneumothorax, and pregnancy did not demonstrate an association with survival or transplant. Greater age at presentation and presence of angiomyolipoma were associated with less risk of mortality. Treatment with hormonal therapy was associated with an increased risk of death/transplant (hazard ratio (HR) 2.93; 95% confidence interval (CI), 1.54-5.58; p = 0.001), particularly progesterone therapy (HR 2.17; 95% CI 1.26-3.75, p = 0.005), and may represent confounding by indication. Patients who required oxygen therapy had a worse outcome (HR 4.53; 95% CI 2.76-7.42; p < 0.001). Conclusions Our population-based study showed that the median survival in patients with LAM from the onset of symptoms or diagnosis is much longer than previously described. This has important implications for life choices and treatment decisions regarding medication use and lung transplantation for patients with LAM. Keywords Lymphangioleiomyomatosis * Epidemiology * Survival * Cystic lung disease, Introduction Lymphangioleiomyomatosis (LAM) is a rare, progressive, frequently lethal cystic lung disease that almost exclusively affects women [1, 2]. It occurs in up to 40% of women with the tuberous [...]

OBJECTIVE: The aim of this study was to identify specifically which biochemical indices predict excessive weight gain over time in a cohort of pre-pubertal children. SUBJECTS: Fifty nine healthy pre-pubertal children (age: 6.3–9.8 y). MEASUREMENTS: Children were defined anthropometrically and biochemically at baseline. Height and weight measurements were then repeated after six (n=52) and 12 months (n=37). RESULTS: Weight change after six months (defined by a change in body mass index (BMI) z-score from baseline) demonstrated no correlation with fasting plasma levels of leptin, insulin, insulin:glucose (IG) ratio, cholesterol, triglyceride or high density lipoprotein (HDL) cholesterol. However, after 12 months there was a significant negative correlation between BMI z-score change and initial plasma leptin (r=-0.35, P=0.048) and this relationship strengthened when adjusted for body fat (from bio-electrical impedance; r=-0.46, P=0.009). In addition, there was a significant positive relationship between plasma total cholesterol and BMI z score change (r=0.38, P=0.03) and this relationship remained unchanged when adjusted for body fat. No relationship was observed between weight change after 12 months and plasma levels of insulin, IG ratio, HDL cholesterol or triglyceride. CONCLUSION: Plasma leptin and total cholesterol were found to be predictive of weight gain over 12 months in a cohort of pre-pubertal children. These two potential predictors can be readily measured in clinical practice and these findings may represent a method of defining the ‘at risk of obesity’ state in childhood. [ABSTRACT FROM AUTHOR]

We report a photovoltaic effect in ferroelectric BiFeO3 thin films. The all-oxide heterostructures with SrRuO3 bottom and tin doped indium oxide top electrodes are characterized by open-circuit voltages ∼0.8–0.9 V and external quantum efficiencies up to ∼10% when illuminated with the appropriate light. Efficiencies are at least an order of magnitude larger than the maximum efficiency under sunlight (AM 1.5) thus far reported for ferroelectric-based devices. The dependence of the measured open-circuit voltage on film thickness suggests contributions to the large open-circuit voltage from both the ferroelectric polarization and band offsets at the BiFeO3/tin doped indium oxide interface. [ABSTRACT FROM AUTHOR]

We report a photovoltaic effect in ferroelectric BiFeO3 thin films. The all-oxide heterostructures with SrRuO3 bottom and tin doped indium oxide top electrodes are characterized by open-circuit voltages ∼0.8–0.9 V and external quantum efficiencies up to ∼10% when illuminated with the appropriate light. Efficiencies are at least an order of magnitude larger than the maximum efficiency under sunlight (AM 1.5) thus far reported for ferroelectric-based devices. The dependence of the measured open-circuit voltage on film thickness suggests contributions to the large open-circuit voltage from both the ferroelectric polarization and band offsets at the BiFeO3/tin doped indium oxide interface. [ABSTRACT FROM AUTHOR]

Background Appropriate Use Criteria (AUC) for echocardiography are a useful tool to deliver quality healthcare. Our quality-based interventional study was designed to assess the trends in appropriate utilization rates for echocardiography in our institution and improve adherence to the AUC criteria for transthoracic echocardiograms (TTE). Methodology A prospective, time series analysis was conducted at the Upstate University Hospital for the months of July 2019 and August 2020. A chart analysis was performed on 620 consecutive inpatients who underwent TTE for the month of July 2019. We assessed the trends of the appropriate ordering of TTEs. We then updated our order form incorporating the 42 most common appropriate indications. A post-intervention chart analysis was performed on all inpatient TTEs ordered for the month of August 2020 (n = 410). The appropriateness of the TTE for the entire group was determined based on the true indication per chart review. The primary outcome was the proportion of appropriate and inappropriate TTEs ordered. Secondary outcomes included assessing for concordance between the indication on the order requisition form and by chart review. A p-value <0.05 was considered significant. Results Using the 2011 AUC for the entire group, 81% of the pre-intervention TTEs and 79.5% of the post-intervention TTEs were appropriate (p = 0.55). There was a statistically significant reduction in the number of discordant TTE orders before and after the intervention (p < 0.01). In addition, we noted increased appropriateness of TTEs in the concordant group both pre and post-intervention. Conclusions Our study demonstrates a significant increase in the concordance between the TTE order sheet and actual indication per chart review with the intervention. This can translate into improved scanning and physician reading quality and time, thereby increasing focus on areas of interest according to the true indication. There was no significant increase in the appropriate TTEs ordered., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Paulraj et al.)

Mitral valve prolapse (MVP) affects approximately 170 million people worldwide; however, phenotypically, there is a wide variety of heterogeneity. In particular subsets, the incidence of sudden cardiac death is calculated to be 998 per 100,000 person-years, which is significantly increased when compared with the general population of MVP patients. Individuals with high-risk features have been identified as young females with bileaflet MVP and electrocardiogram findings of frequent complex ectopy, ST-T wave changes, and inferior T wave inversions. Supplemental imaging modalities in this subgroup demonstrate redundant leaflets and chordae on 2-dimensional transthoracic echocardiography along with varying severity of mitral annular disjunction. Detailed morphologic assessment by 3-dimensional echocardiography provides a quantitative assessment of annular disjunction along with left ventricular longitudinal and basal circumferential strain patterns. Late gadolinium enhancement on cardiac magnetic resonance imaging identifies diffuse and isolated left ventricle fibrosis involving the fascicles and papillary muscles, which has been visualized in isolation during autopsy. Findings of this review propose that sudden cardiac death as a result of malignant arrhythmias arises from automaticity, complex ectopy, and reentry at the level of the fascicles and papillary muscles. The repetitive mechanical stress provides a nidus for the development of both micro- and macrofibrosis easily identified by late gadolinium enhancement on cardiac magnetic resonance imaging. Escalation to electrophysiology studies and early intervention could provide new targeted lifesaving therapies., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)