Amyloid Deposits in a Functionally Unicuspid Stenotic Aortic Valve.

Amyloidosis concomitant to aortic stenosis usually occurs with myocardial infiltration by the transthyretin protein. To our knowledge, this is the first report of localized amyloidosis of indeterminate type in a severely calcified and functionally unicuspid aortic valve. Isolated dystrophic valvular amyloidosis is believed to be related to fibrocalcific valve disease. In light of the literature on this topic, the present case raises new hypotheses on pathophysiology and further supports the contributory role of unusual non-tricuspid valve morphology in the development of dystrophic amyloid, likely secondary to altered hemodynamic stress.

Une amyloïdose associée à une sténose aortique survient généralement avec infiltration du myocarde par la protéine transthyrétine. Le cas que nous décrivons est, à notre connaissance, le premier cas rapporté d'amyloïdose localisée de type indéterminé dans une valve aortique sévèrement calcifiée et fonctionnellement unicuspide. L'amyloïdose valvulaire dystrophique isolée serait liée à l'atteinte fibrocalcique de la valve. À la lumière de la littérature à ce sujet, le cas décrit ici permet de soulever de nouvelles hypothèses physiopathologiques et appuie le lien entre une morphologie valvulaire inhabituelle (non tricuspide) et l'apparition de substances amyloïdes dystrophiques, probablement secondaire à une altération des contraintes hémodynamiques.

Barriers to telemedicine for patients with heart failure: Who are the patients being left behind?

Heart failure is associated with high rates of hospitalization, which are more prevalent in frail patients, impacting the quality of life and clinical outcomes. Telemedicine is considered cost-effective for improving patient self-management and hospitalization. However, socioeconomic deprivation and frailty could hinder access to virtual care. We investigated if frailty and socioeconomic factors were associated with telemedicine access among heart failure patients. For this cross-sectional analysis of Continuum study, 35 patients were allocated to the "able to use" group (had a smart device and were able to use it) or the "not able to use" group. Socioeconomic deprivation was determined according to the deprivation index. Frailty was assessed using the Fried criteria. The mean age was 69.9 ± 9 years, 74% were in New York Heart Association class II. A total of 14 patients (39%) were physically frail. Patients considered not able to use the app were more socioeconomically deprived (p = 0.011) and frail (p = 0.036). There was no correlation between frailty score and socioeconomic deprivation (r = 0.15, p = 0.411). Telemedicine use seems to be independently associated with frailty and socioeconomic deprivation in heart failure patients. More efforts should be made to foster the inclusion of vulnerable patients and improve global telemedicine access.

Focused review on nutritional status of patients with immunoglobulin light chain amyloidosis.

BACKGROUND: Immunoglobulin light chain (AL) amyloidosis is a complex disease marked by a poor clinical portrait and prognosis generally leading to organ dysfunction and shortened survival. We aimed to review the available evidence on whether AL amyloidosis can lead to malnutrition, thus having a negative impact on quality of life (QoL) and survival. MATERIALS: We searched Pubmed for studies that assessed malnutrition in amyloidosis patients, with no restrictions to the year of publication or language. Retrospective or prospective, observational, and interventional studies that reported data regarding AL amyloidosis and nutritional status were included. RESULTS: From 62 articles retrieved, 23 were included. Malnutrition was prevalent in up to 65% of patients with AL Amyloidosis. Prevalence of weight loss of 10% or more ranged from 6 to 22% of patients, while a body mass index of < 22 kg/m2 was found in 22 to 42%. Weight loss, lower BMI and other indicators of poor nutritional status were negatively associated with quality of life and survival. Only one RCT focused on nutritional counseling was found and reported positive results on patients QoL and survival. CONCLUSION: Despite inconsistencies across assessment criteria, the available data reveal that weight loss and malnutrition are common features in patients with AL amyloidosis. This review reinforces the premise that an impaired nutritional status can be negatively associated with QoL and survival in patients with AL amyloidosis, and therefore should be further investigated.

Arbitrary arbitration: diverting juveniles into the justice system--a reexamination after 22 years.

This article reports on a study of a juvenile diversion program based in California. It replicates a similar 1986 study of a Florida-based juvenile diversion program. Both studies examine the appropriateness of the actual juvenile population serviced in comparison with the declared and described programs' target populations. Findings in the current research support earlier findings that the diversion program services self-serving, low-risk populations, rather than their intended "first-time nonviolent," more seriously at risk of continued delinquent behavior, target population. This finding is examined in terms of its relationship to issues of labeling and net widening, and suggests how the current study diversion program manages to create a façade that more seriously at-risk youth are being properly targeted by the program.