ALBERT

Description: Abundances of the life stages of zooplankton taxa were calculated in the Arctic Ocean during the Hakon Mosby cruise to Fram Strait in 1987. Samples were collected in obliquely hauled nets with a 1m diameter mesh size: 118 µm). The water volume filtered was measured with a flowmeter attached in the net. Further details on sampling and analysis methodology can be found at Smith et al. (1988). The zooplankton samples were taken as part of the international Marginal Ice Zone Experiment (MIZEX) that was designed to study the atmospheric, oceanic, and ice interactions in the Fram Strait region of the Greenland Sea.

Description: Abundances of the life stages of zooplankton taxa were calculated in the Arctic Ocean during Polarstern cruise ARK-II/2. Samples were collected in vertically hauled nets with a Multinet midi (Hydrobios, Kiel), which is equipped with five nets (mesh size: 200 µm). The water volume filtered was measured with a flowmeter attached in the net. Further details on sampling and analysis methodology can be found at Smith et al. (1986). The zooplankton samples were taken as part of the international Marginal Ice Zone Experiment (MIZEX) that was designed to study the atmospheric, oceanic, and ice interactions in the Fram Strait region of the Greenland Sea.

Description: Abstract Deltas are important coastal sediment accumulation zones in both marine and lacustrine settings. However, currents derived from tides, waves or rivers can transfer that sediment into distal, deep environments, connecting terrestrial and deep marine depozones. The sediment transfer system of the Rhone River in Lake Geneva is composed of a sublacustrine delta, a deeply incised canyon and a distal lobe, which resembles, at a smaller scale, deep‐sea fan systems fed by high discharge rivers. From the comparison of two bathymetric datasets, collected in 1891 and 2014, a sediment budget was calculated for eastern Lake Geneva, based on which sediment distribution patterns were defined. During the past 125 years, sediment deposition occurred mostly in three high sedimentation rate areas: the proximal delta front, the canyon‐levée system and the distal lobe. Mean sedimentation rates in these areas vary from 0·0246 m year−1 (distal lobe) to 0·0737 m year−1 (delta front). Although the delta front–levées–distal lobe complex only comprises 17·0% of the analysed area, it stored 74·9% of the total deposited sediment. Results show that 52·5% of the total sediment stored in this complex was transported toward distal locations through the sublacustrine canyon. Namely, the canyon–levée complex stored 15·9% of the total sediment, while 36·6% was deposited in the distal lobe. The results thus show that in deltaic systems where density currents can occur regularly, a significant proportion of riverine sediment input may be transferred to the canyon‐lobe systems leading to important distal sediment accumulation zones.

Description: Energy & Fuels DOI: 10.1021/ef4014983

Description: Disease severity and healthcare utilization varies widely among persons with sickle cell disease (SCD). Hydroxyurea (HU) has been demonstrated to reduce rates of pain and acute chest syndrome, the leading causes of inpatient utilization in patients with Hb SS and S β°-thalassemia in clinical trials. We recently reported that HU was clinically effective in reducing rates of pain and acute chest syndrome in patients who initiate treatment. Use of hydroxyurea in SS/S β°-thalassemia has increased markedly since 2010. Thus we sought to detemine trends of hospital utilization for acute illness during an 8 yr in which HU utilization increased markedly (2010-2017). Data from years 2010-201 were obtained from the SCD database and patient records at Children's Healthcare of Atlanta (CHOA). Utilization data were restricted to acute care admissions. Admissions for elective procedures, non-SCD related discharge diagnoses, rare SCD genotypes, and patients who had undergone bone marrow transplant were excluded. Patients were compared based on number of hospitalizations, age, sex, SCD genotype (SS/S β°-thalassemia vs Hb SC/S β+thalassemia), and discharge diagnosis. A total of 3,116 patients had at least one encounter between 2010 and 2017; 2,947 patients met inclusion criteria. From 2010-2017 the total number of active patients per year increased from 1,546 patients to 1,789 patients (+16%), while the total number acute care admissions increased from 1,295 admissions to 1,609 admissions (+24%). There were no significant differences in the proportion of patients with genotypes SS/S β° thalassemia genotypes (67.0% vs 63.9%, p=0.06). Overall patients with SS/S β° thalassemia had higher admission rates compared to SC/S β+ thalassemia patients (0.94 vs 0.57 admissions per patient per year). During the study period. overall admission rates in SCD (acute illness hospitalizations/patient/yr) increased from a low of 0.74 in 2011 to a high of 0.90 in 2017. The proportion of admissions attributed to SS/S β°-thalassemia patients decreased (79.2% in 2010 vs 72.3% in 2017, p90% of patients over age 8 years), and had greater percentages of admissions for pain and acute chest syndrome then non-SHHU. In conclusion, during a period in which HU utilization in SS/S β°-thalassemia increased significantly, hospital utilization for acute illness in SS/S β° thalassemia decreased as expected. However, during the same period there was an unexpected increase in overall hospital utilization for acute illness in SCD. This increase in hospital utilization was the result of 1) a marked increase in SHHU and 2) an overall increased utilization in SC/S β+ thalassemia. Disclosures Dampier: Pfizer: Research Funding.

Description: Abstract 845 Background: Transfusion therapy can effectively treat many complications associated with sickle cell disease (SCD), but iron overload will develop without iron chelation therapy. Despite long-term transfusion requirements, long-term data for iron chelation in SCD are limited. The oral iron chelator, deferasirox, effectively reduced iron burden in SCD patients aged ≥2 years during 1 year of treatment (Vichinsky et al. Br J Haematol 2007). This 5-year follow-up is the first report of long-term deferasirox treatment in SCD patients. Methods: Eligible patients that completed the 1-year core study (randomized to deferasirox [deferasirox cohort] or deferoxamine [crossover cohort]) entered the 4- year extension. All received deferasirox while continuing their regular transfusion regimen. Deferasirox dose in the extension was initially based on serum ferritin (SF) trends in the core study (deferasirox cohort) or transfusion requirements (crossover cohort). Dose adjustments were based on monthly SF and safety assessments (investigator-reported adverse events [AEs] and centrally processed lab parameters). Growth and sexual development (Tanner staging) were assessed annually. Results: Of the patients in the deferasirox (n=132) and crossover (n=53) cohorts that received ’1 deferasirox dose during the core or extension, 43 (33%) and 19 (36%) patients, respectively, completed the extension. Reasons for discontinuation included withdrawal of consent (n=44, 23.8%), loss to follow-up (n=17, 9.2%) and AEs (n=14, 7.6%). Three deaths occurred, all in the extension (intraventricular hemorrhage, n=2; intracranial bleed post liver transplantation, n=1); none considered by investigators to be deferasirox-related. Mean dose during the study was 18.7 ± 6.5 and 21.2 ± 5.3 mg/kg/day in the deferasirox and crossover cohorts, respectively. Investigator-assessed drug-related AEs (≥5% overall) included nausea (14.6%), diarrhea (10.8%), increased blood creatinine (5.9%) and vomiting (5.4%). Generally, these AEs were manageable and transient, and decreased in frequency over time. Serious AEs were reported in 70.8% of patients overall and were mostly related to the underlying disease. Serious investigator-assessed drug-related AEs were reported in 8 patients (6.1%) in the deferasirox cohort and 1 patient (1.9%) in the crossover cohort. In the deferasirox and crossover cohorts respectively, 9 (6.8%) patients and 1 (1.9%) patient had 2 consecutive serum creatinine level increases 〉33% above baseline and 〉upper limit of normal (ULN). Median creatinine clearance remained stable within normal range throughout the study. One patient from each cohort had alanine aminotransferase (ALT) 〉10 × ULN on 2 consecutive visits; both had ALT values ≤ULN at the start of deferasirox treatment. In 37 patients with data available before and after dose increases to ≥30 mg/kg/day, no clinically relevant differences were observed in AE profile or laboratory parameters before and after dose increase. Deferasirox had no adverse effect on pediatric growth and adolescent sexual development. Overall, median SF levels in patients who received deferasirox treatment for ≥4 years decreased significantly from 3410 to 3108 ng/mL at end of study (median absolute change, –591 ng/mL, P=0.027; n=67). Decreases in SF were more pronounced when mean deferasirox dose increased to 〉20 mg/kg/day (Figure 1). In the deferasirox cohort, the median absolute change in SF levels from start of deferasirox to end-of-study was greater in patients aged ≥16 than 2–20 mg/kg/day, to achieve negative iron balance. Disclosures: Vichinsky: Novartis: Consultancy, Research Funding, Speakers Bureau; Apotex: Consultancy, Research Funding; Hemaquest: Consultancy, Membership on an entity's Board of Directors or advisory committees. Bernaudin:Novartis: Investigator for SCD deferasirox (Exjade). Forni:Novartis: Research Funding. Gardner:Novartis: Research Funding. Hassell:Novartis: Research Funding. Heeney:Novartis: Research Funding. Kutlar:Novartis: Research Funding. Lane:Novartis: Research Funding. Mathias:Novartis: Research Funding. Porter:Novartis: Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau. Tebbi:Novartis: Speakers Bureau. Wilson:Novartis: Honoraria, Research Funding, Speakers Bureau. Griffel:Novartis: Employment. Deng:Novartis: Employment. Giannone:Novartis: Employment. Coates:Novartis: Research Funding, Speakers Bureau.