Light chain (AL) amyloidosis is a protein folding disorder that can affect many different organ systems, in addition to the coagulation pathway. D-dimer, a measurement of fibrin degradation, is commonly elevated in hematologic malignancies, but the prevalence and significance of D-dimer elevation in AL amyloidosis is unknown. We conducted an analysis of 921 patients with AL amyloidosis that presented to the Boston University Amyloidosis Center. Baseline characteristics and laboratory data of the 897 patients included in the final cohort were analyzed. Four hundred twenty three patients (47%) had an elevated D-dimer (>0.5 μg/mL). Multivariate analysis demonstrated that a normal D-dimer level of ≤0.5 μg/mL, and a level of >0.5 μg/mL but <1 μg/mL, conferred a lower risk of mortality (HR 0.49 and 0.59, respectively) when compared to a D-dimer level ≥ 1 μg/mL. The increased risk of mortality in patients with a D-dimer level ≥ 1 μg/mL was present in all cardiac stages. The median overall survival based on D-dimer range of ≤0.5, >0.5 but <1, and ≥ 1 μg/mL was 5.86, 4.04, and 2.08 years, respectively (P < .001). This retrospective analysis demonstrates the high prevalence of D-dimer elevation in AL amyloidosis and confirms that this laboratory finding is independently associated with decreased survival.Median age at first visit, y (range)62 (27-90) Males/Females (%) 62/38 Organ involvementRenal, n (%) 661 (74) Cardiac, n (%) 547 (61) Gastrointestinal, n (%) 153 (17) Liver, n (%) 148 (17) Peripheral nervous system, n (%) 132 (15) Autonomic nervous system, n (%) 242 (27) Soft tissue, n (%) 173 (19) Median number of involved organs (range) 2 (1 -7) Cardiac biomarkers Median BNP, pg/mL (range), N = 803 204 (0 -6 026) Median troponin-I, ng/mL (range), N = 466 0.064 (<0.006 -8.915) Boston University Cardiac stage b Stage I, n (%) 77 (18) Stage II, n (%) 199 (47) Stage III, n (%) 75 (18) Stage IIIb, n (%) 69 (16) Renal assessment Median eGFR, mL/min/1.73m 2 (range) 67 (3 -319) Median creatinine, mg/dL (range) 1.1 (0.2 -15.5) Median proteinuria, mg/24 hours (range) 2 643.5 (0-153 613) Renal stage b Stage I, n (%) 423 (50) Stage II, n (%) 302 (36) Stage III, n (%) 115 (14) Hematological parameters Median dFLC, mg/L (range) 123 (0 -10 154) D-dimer, mcg/mL ≤0.5, n (%) 474 (53) >0.5-<1, n (%) 200 (22) ≥1, n (%) 223 (25) Confirmation of venous thrombosis a , n (%) 71 (8) Abbreviations: BNP, B-type Natriuretic Peptide; eGFR, estimated Glomerular Filtration Rate; sFLC, serum free light chain ratio; dFLC, difference between involved and uninvolved free light chains. a Cardiac staging data available for 420 patients and renal staging data available for 840 patients. b Not all patients were evaluated for venous thrombosis.
Two dimensional nanostructures of transition metal dichalcogenides like WS2 have been drawing much attention for biological applications owing to their intrinsic chemical and optical properties with tunable band gap. We have evaluated photocatalytic properties of chemical vapor deposited (CVD) grown WS2 monolayer with a direct band gap of 1.94 eV to disinfect water from Gram‐negative E.coli bacteria under fluorescence light by colony counting method. WS2 monolayer decorated with 10‐15 nm silver nanoparticles is found to enhance the antibacterial activity in comparison with individual components significantly. We have achieved up to 96% of bacterial growth inhibition by increasing the areal coverage of WS2 (upto 42%) coated with Ag nanoparticles in 3 hours, under fluorescent light. Moreover, direct correlation between the water disinfection ability and geometrical surface areal coverage of the monolayer WS2 on SiO2/Si substrate has been established to emphasize on the need of uniform CVD growth of WS2 monolayers over large scale for practical application like rapid and efficient cleaning technology.
Importance: Caregivers of children with cerebral palsy can best help their child if they understand the disorder and the correct terminology. Objective: To assess caregiver understanding of cerebral palsy. Design: This was a cross-sectional study from a large tertiary medical center in Boston, to assess understanding of the term cerebral palsy by primary caregivers of children and adolescents with cerebral palsy. All cases were obtained from hospital electronic medical records. Telephone surveys were conducted. Caregiver understanding of cerebral palsy was assessed by open-ended responses (50%) and success in answering true/false questions about cerebral palsy (50%). Participants: Primary caregivers of children 18 years and younger with cerebral palsy. Results: Thirty-three percent of caregivers denied ever being told that their child had cerebral palsy. Most caregivers identified cerebral palsy as a brain problem (79%), lifelong condition (73%), often caused by a perinatal (60%) or gestational (40%) insult. Fifty-two percent knew that cerebral palsy was nonprogressive. Sixty-two percent of caregivers believed they had a good, very good, or excellent understanding of cerebral palsy, whereas the investigators found 69% of caregivers had a good, very good, or excellent understanding of cerebral palsy (P = .006). Most caregivers rated very good or excellent the setting where cerebral palsy was discussed (58%), the explanations provided (55%), and the amount of time spent (45%), yet using a Pearson correlation coefficient, most important was the time spent ( r = 0.53). Conclusions: Following discussion with their child’s physician, most primary caregivers of children with cerebral palsy have a good, very good, or excellent understanding of cerebral palsy. Most critical to a good understanding of cerebral palsy was the time spent in explaining the diagnosis.
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