Case Report: Pediatric myeloid/lymphoid neoplasm with eosinophilia and PDGFRA rearrangement: The first case presenting as B-lymphoblastic lymphoma

According to the latest WHO classification of hematopoietic malignancies, myeloid and lymphoid neoplasms with eosinophilia and gene rearrangements include three specific rare diseases and one provisional entity. Myeloid/lymphoid neoplasms with platelet-derived growth factor receptor alpha (PDGFRA) rearrangements are the most frequent of these disorders and are usually present in adult males with a median age of the late 40s. Patients usually have chronic eosinophilic leukemia but can occasionally manifest as acute myeloid leukemia or extramedullary T- or B-lineage lymphoblastic lymphoma. We report a case of a previously healthy 2-year-old girl who presented with a right supraorbital swelling with no associated lymphadenopathy. Peripheral blood smear evaluation at initial presentation revealed microcytic hypochromic red blood cells and leukocytosis with marked eosinophilia, occasional myelocytes, and occasional blasts. Whole-body CT scans and PET scans revealed hypermetabolic potentially lymphomatous mass in the superior medial aspect of the right orbit in addition to splenomegaly but no evidence of hypermetabolic mediastinal, hilar, abdominal, or pelvic lymph nodes. Bone marrow aspirate and biopsy revealed hypercellular bone marrow with quantitatively decreased erythroid precursors and increased granulocytic precursors with 60% of the cells being eosinophilic cells in different stages of maturation. The diagnosis of myeloid neoplasm with eosinophilia and rearrangement of PDGFRA was made following confirmation by fluorescence in situ hybridization (FISH) test for FIP1L1-PDGFRA gene fusion. An incisional biopsy of the supraorbital mass revealed B-cell lymphoblastic lymphoma (B-LBL). FISH test for FIP1L1-PDGFRA gene fusion was positive in 70% of the cells studied. Thus, the final diagnosis was B-cell lymphoblastic lymphoma arising in the setting of myeloid/lymphoid neoplasm with eosinophilia and PDGFRA rearrangement. The patient was started on imatinib with concomitant therapy for B-LBL per the Children Oncology Group (COG) standard therapy for localized B-LBL and demonstrated a favorable outcome in the 2.5-year follow-up period. To our knowledge, this is the first pediatric case of myeloid/lymphoid neoplasm with PDGFRA rearrangement presenting with synchronous myeloproliferative disease and B-LBL. We present our diagnostic and management approach of this patient and review prior relevant pediatric cases of myeloid/lymphoid neoplasms with PDGFRA rearrangement

Enterprise Architecture Specification Case Study

A graduate course in enterprise architecture had a team project component in which a real-world business case, provided by an industry sponsor, formed the basis of the project charter and the architecture statement of work. The paper aims to share the team project experience on developing the architecture specifications based on the business case of an accountable health care organization. Students collaborated as a team in various roles to develop the architecture specifications for a new business initiative of the sponsoring organization, XYZ ACO. The teaching case describes the case study approach and the architecture approach adopted for the architecture process, and is accompanied by Teaching Case Notes which provide a selection of the models developed by members of the project team towards the architecture specifications. The course started with coverage of enterprise architecture theory, best practices and standards, and the team project gave students the opportunity to apply their theoretical knowledge and “learn by doing”. Students were challenged to interpret the business case, the project charter and project requirements, and each team member was allocated an architecture viewpoint and a role to play. The Teaching Case presents a summary of the team project and the lessons learned in performing the project

SCSAP: Spiral Clustering Based on Selective Activation Protocol for industrial tailored WSNs

Wireless Sensor Networks (WSNs) are generally deployed with low-cost devices that have low power consumption and small-size sensor nodes. They can be used in several monitoring and control applications such as; health, agriculture, and environment. With the advent of Industry 4.0 and Artificial Intelligence (AI), WSNs attracted many industrial applications and specific implementation scenarios. In general, the sensor nodes compromising of WSNs consume vast energy during tracking and monitoring. This poses a challenge especially since most of WSNs are battery-operated and have very limited energy sources. Therefore, it is important to optimize power consumption and prolong the lifetime of WSNs. Many protocols have been proposed with emphasis on data forwarding and routing. These protocols, however, have not been designed to address interference and transmissions issues, such as communication link instability and packet drops. In addition, a quiet substantial amount of energy would be consumed during transmission, which leads to a degradation in network performance. In this research, an effective solution is proposed based on Spiral Clustering Based on Selective Activation Protocol (SCSAP). The objective is to enhance energy consumption and improve network performance, which makes it a good candidate for several Industrial tailored WSNs. In this solution, the network nodes are classified into two types; super and normal. Super nodes have a significant power source and are utilized to construct clusters and act as Cluster Heads (CHs). They have pre-defined locations with an optimized number of nodes selected for the best coverage that is determined in the Stable Election Protocol (SEP). As a result, the power consumption is reduced in the dynamic formatting of clusters. Normal nodes, on the other hand, are static. They have limited power and are connected to the super nodes. Normal nodes operate in two modes; active and sleep. In the active mode, they send data to the CHs and the Time Division Multiple Access (TDMA) is used to guarantee that the transmitted data are correctly received by the other nodes and that the power consumption of re-transmission is largely reduced. The simulation results of SCSAP have shown a 40% enhancement of network lifetime over the Threshold-sensitive Stable Election Protocol (TSEP). They have also shown that SCASP’s remaining power has descended slower than the Low-energy Adaptive Clustering Hierarchy (LEACH) protocol

The Synergistic Protective Effect of γ-Oryzanol (OZ) and N-Acetylcysteine (NAC) against Experimentally Induced NAFLD in Rats Entails Hypoglycemic, Antioxidant, and PPARα Stimulatory Effects

This study estimated that the combined effect of γ-Oryzanol and N-acetylcysteine (NAC) against high-fat diet (HFD)-induced non-alcoholic fatty liver disease (NAFLD) in rats also estimated some of their mechanisms of action. Adult male rats were divided into seven groups (n = 8 each) as control, control + NAC, control + γ-Oryzanol, HFD, HFD + NAC, HFD + γ-Oryzanol, and HFD + NAC + γ-Oryzanol. NAC was administered orally at a final concentration of 200 mg/kg, whereas γ-Oryzanol was added to diets at a concentration of 0.16. All treatments were conducted for 17 weeks and daily. Both NAC and γ-Oryzanol were able to reduce final body weights, fat weights, fasting glucose, fasting insulin, serum, and serum levels of liver function enzymes as well as the inflammatory markers such as tumor necrosis factor-α (TNF-α), interleukine-6 (IL-6), and leptin in HFD-fed rats. They also improved hepatic structure and glucose tolerance, increased adiponectin levels, and reduced serum and hepatic levels of triglycerides (TGs) and cholesterol (CHOL) in these rats. These effects were concomitant with a reduction in the hepatic levels of lipid peroxides (MDA) and serum levels of LDL-C, but also with an increment in the hepatic levels of superoxide dismutase (SOD) and glutathione (GSH). Interestingly, only treatment with γ-Oryzanol stimulated the mRNA levels of proliferator-activated receptor alpha (PPARα) and carnitine palmitoyltransferase 1 (CPT1) in the liver and white adipose tissue (WAT) of rats. Of note, the combination therapy of both drugs resulted in maximum effects and restored almost normal liver structure and basal levels of all the above-mentioned metabolic parameters. In conclusion, a combination therapy of γ-Oryzanol and NAC is an effective therapy to treat NAFLD, which can act via several mechanisms on the liver and adipose tissue

SARS-CoV-2 vaccination modelling for safe surgery to save lives: data from an international prospective cohort study

Background: Preoperative SARS-CoV-2 vaccination could support safer elective surgery. Vaccine numbers are limited so this study aimed to inform their prioritization by modelling. Methods: The primary outcome was the number needed to vaccinate (NNV) to prevent one COVID-19-related death in 1 year. NNVs were based on postoperative SARS-CoV-2 rates and mortality in an international cohort study (surgical patients), and community SARS-CoV-2 incidence and case fatality data (general population). NNV estimates were stratified by age (18-49, 50-69, 70 or more years) and type of surgery. Best- and worst-case scenarios were used to describe uncertainty. Results: NNVs were more favourable in surgical patients than the general population. The most favourable NNVs were in patients aged 70 years or more needing cancer surgery (351; best case 196, worst case 816) or non-cancer surgery (733; best case 407, worst case 1664). Both exceeded the NNV in the general population (1840; best case 1196, worst case 3066). NNVs for surgical patients remained favourable at a range of SARS-CoV-2 incidence rates in sensitivity analysis modelling. Globally, prioritizing preoperative vaccination of patients needing elective surgery ahead of the general population could prevent an additional 58 687 (best case 115 007, worst case 20 177) COVID-19-related deaths in 1 year. Conclusion: As global roll out of SARS-CoV-2 vaccination proceeds, patients needing elective surgery should be prioritized ahead of the general population