A Method to Identify Variability in Knowledge Intensive Processes

One of the most important features of an Knowledge Intensive Process (KIP) is the variability: different activities can be performed by certain factors, i.e., certain behaviors lead to different directions at runtime. Despite the variability to be a key point in PIC, the various existing proposals in the literature are not treated satisfactorily aspects of reasons why the behavior of processes to be modified. This article aims to present the KIP-Organon method, designed to identify the elements that can cause variability in Knowledge Intensive Processes

Cuttlefish capsule: An effective shield against contaminants in the wild

Increasing anthropogenic pressures in estuaries are responsible for the rise of contaminants in several compartments of these ecosystems. Species that benefit from the nursery services provided by estuaries are exposed to such contaminants (e.g. metals and metalloids). It is therefore relevant to understand if marine invertebrates that use these areas as spawning grounds accumulate contaminants in their tissues throughout embryogenesis. This study aimed to quantify As, Co, Cr, Cu, Mn, Ni, Se, Pb, V and Zn concentrations in both capsule and embryos of the common cuttlefish (Sepia officinalis) in Sado Estuary (Portugal). Moreover, embryos at their initial, intermediate and final stage of development were collected in sites subjected to different anthropogenic pressures. In general, the capsule accumulated higher element concentration throughout embryogenesis which indicates that the capsule acts as an effective barrier against contaminants uptake by the embryo. Although the capsule becomes thinner throughout embryogenesis, embryo's protection does not seem to be compromised at later development stages. Additionally, the higher concentrations of As, Cu, Se and Zn in the embryo in comparison to the capsule suggests important biological roles during the embryogenesis of this cephalopod mollusc.info:eu-repo/semantics/publishedVersio

Cuttlefish capsule: an effective shield against contaminants in the wild

Increasing anthropogenic pressures in estuaries are responsible for the rise of contaminants in several compartments of these ecosystems. Species that benefit from the nursery services provided by estuaries are exposed to such contaminants (e.g. metals and metalloids). It is therefore relevant to understand if marine invertebrates that use these areas as spawning grounds accumulate contaminants in their tissues throughout embryogenesis. This study aimed to quantify As, Co, Cr, Cu, Mn, Ni, Se, Pb, V and Zn concentrations in both capsule and embryos of the common cuttlefish (Sepia officinalis) in Sado Estuary (Portugal). Moreover, embryos at their initial, intermediate and final stage of development were collected in sites subjected to different anthropogenic pressures. In general, the capsule accumulated higher element concentration throughout embryogenesis which indicates that the capsule acts as an effective barrier against contaminants uptake by the embryo. Although the capsule becomes thinner throughout embryogenesis, embryo’s protection does not seem to be compromised at later development stages. Additionally, the higher concentrations of As, Cu, Se and Zn in the embryo in comparison to the capsule suggests important biological roles during the embryogenesis of this cephalopod mollusc

The impact of JAK2 and MPL mutations on diagnosis and prognosis of splanchnic vein thrombosis: a report on 241 cases

Myeloproliferative diseases (MPDs) represent the commonest cause of splanchnic vein thrombosis (SVT), including Budd-Chiari syndrome (BCS) and portal vein thrombosis (PVT), but their diagnosis is hampered by changes secondary to portal hypertension, while their influence in the outcome of SVT remains unclear. We assessed the diagnostic and prognostic value of JAK2 and MPL515 mutations in 241 SVT patients (104 BCS, 137 PVT). JAK2V617F was found in 45% of BCS and 34% of PVT, while JAK2 exon 12 and MPL515 mutations were not detected. JAK2V617F was found in 96.5% of patients with bone marrow (BM) changes specific for MPD and endogenous erythoid colonies, but also in 58% of those with only one feature and in 7% of those with neither feature. Stratifying MPD diagnosis first on JAK2V617F detection would have avoided BM investigations in 40% of the patients. In BCS, presence of MPD carried significantly poorer baseline prognostic features, required hepatic decompression procedures earlier, but had no impact on 5-year survival. Our results suggest that JAK2V617F testing should replace BM investigations as initial test for MPD in patients with SVT. Underlying MPD is associated with severe forms of BCS, but current therapy appears to offset deleterious effects of MPD on the medium-term outcome

Outcomes and mutational analysis of patients with lower-risk non-del5q myelodysplastic syndrome treated with antithymocyte globulin with or without ciclosporine A

International audienceImmunosuppressive treatment is a disease-modifying therapy for lower-risk myelodysplastic syndromes (MDS). However, IST is relatively rarely used and long-term outcomes of patients are seldom reported. We retrospectively studied outcomes of 20 patients with lower-risk non del 5q MDS with transfusion dependency, with horse or rabbit antithymocyte globulin ± ciclosporine A, and frontline eltrombopag in two of them. IPSS-R was low, intermediate and high in 30%, 55% and 10% of the patients, respectively. Fifty-five percent of the patients had hypocellular bone marrow (BM). Baseline mutations were detected in 31.5% of the patients and were more frequent in patients with normo/hypercellular MDS than in patients with hypocellular MDS. Transfusion independence rate for both red blood cells (RBC) and platelets was achieved in 45% of patients. RBC transfusion duration ≤6 months, B-cell counts >0.2 G/L and, marginally, BM blasts ≤2% were associated with higher transfusion independence rate. Age and cellularity did not influence the response rate. Median transfusion independence duration was 53 months. Cumulative incidence of progression to a more aggressive myeloid disease was 0 in patients without baseline mutations and 33% in patients with baseline mutations (P = .008). Median progression-free and overall survival after treatment onset and median overall survival after loss of transfusion independence were 45.5 months, 68 months and not reached, respectively. In conclusion, antithymocyte globulin ± ciclosporine A results in durable responses in MDS, irrespective of age, in patients with lower-risk disease without B-cell lymphopenia and treated early in the course of the disease

The impact of JAK2 and MPL mutations on diagnosis and prognosis of splanchnic vein thrombosis: A report on 241 cases

Myeloproliferative diseases (MPDs) represent the commonest cause of splanchnic vein thrombosis (SVT), including Budd- Chiari syndrome (BCS) and portal vein thrombosis (PVT), but their diagnosis is hampered by changes secondary to portal hypertension, while their influence in the outcome of SVT remains unclear. We assessed the diagnostic and prognostic value of JAK2 and MPL515 mutations in 241 SVT patients (104 BCS, 137 PVT). JAK2V617F was found in 45% of BCS and 34% of PVT, while JAK2 exon 12 and MPL515 mutations were not detected. JAK2V617F was found in 96.5% of patients with bone marrow (BM) changes specific for MPD and endogenous erythoid colonies, but also in 58% of those with only one feature and in 7% of those with neither feature. Stratifying MPD diagnosis first on JAK2V617F detection would have avoided BM investigations in 40% of the patients. In BCS, presence of MPD carried significantly poorer baseline prognostic features, required hepatic decompression procedures earlier, but had no impact on 5-year survival. Our results suggest that JAK2V617F testing should replace BM investigations as initial test for MPD in patients with SVT. Underlying MPD is associated with severe forms of BCS, but current therapy appears to offset deleterious effects of MPD on the medium-term outcome