Primary catastrophic antiphospholipid syndrome in an 8 year-old girl

Antiphospholipid syndrome (APS) is a disease characterized by recurrent arterial and venous thromboses. Rapidly progressive multiple thromboses leading to multiorgan failure occur in less than 1% of patients and named as catastrophic antiphospholipid syndrome (CAPS). We, hereby, describe an 8 year-old-girl with erythematous skin lesions progressing into purpura fulminans. The patient developed CAPS with the findings including proteinuria, microangiopathic hemolytic anemia, thrombocytopenia, arterial and venous thromboses demonstrated on skin biopsies. She was admitted to intensive care unit and received empirical antibiotics, anticoagulants, antiaggregants, steroids and intravenous immunoglobulins. The diagnosis of APS was confirmed by positive lupus anticoagulants, elevated anti beta-2 glycoprotein IgG and antiphospholipid IgG titers. Moreover, other than MTHFRA1298C, MTHFR-C677T, factor V H1299R, beta fibrinogen-455 G>A heterozygosity indicating low risk for thrombophilia, no infectious, rheumatological or malignant etiologies were identified. Family history revealed Raynaud’s phenomenon in a sister, interstitial lung disease, proteinuria and hematuria in paternal grandmother in addition to lupus anticoagulant positivity in father and 2 elder sisters. Her treatment included debridement of necrotic skin tissue, grefting and local mesenchymal stem cell application to upper thigh and lower leg region following oral azathioprine administration

The effect of the American Society of Anesthesiology classification scores on complications associated with percutaneous nephrolithotomy

Objectives: We aimed to evaluate the effect of American Society of Anesthesiology (ASA) classification scoring and age on complications and surgical outcomes during and after percutaneous nephrolithotomy (PCNL) operation. Material and methods: The records of 263 patients, above the age of 18 years, that underwent PCNL surgery between October 2014 and May 2017 were evaluated retrospectively. The patients were divided into three groups based on their ASA risk scores (ASA 1, 2, 3) and into two groups based on their age (younger and older than 65 years). Postoperative complications were assessed according to the ASA groups and age and according to the Clavien classification system. Results: The number of patients in the ASA 1, 2, and 3 groups were 97 (36.8%), 131 (49.8%) and 35 (13.3%), respectively. Four patients in ASA4 were not included in the study. There was no significant difference in ASA 1, 2, 3 groups in terms of changes in Hgb values, mean duration of operation, and mean hospital stay. When ASA1 was compared to ASA3 and ASA2 was compared to ASA3, there was no significant difference in the incidence of all complication rates. There were 159 (60.4%) patients in the young group and 104 (39.5%) patients in the elderly group. Postoperative PCNL complications of these 2 groups were compared according to Clavien classification system and no significant difference was found in incidence of complications. Conclusions: We believe that PCNL operation can be performed effectively and safely in both ASA3 patients and patients above the age of 65 years

Limitations of Current ERP Packages Regarding the New Supply Chain Management Requirements in the New Millennium

The future of Enterprise Resource Planning (ERP).is at risk. The existing structure of ERP is inadequate to meet new Supply Chain Management (SCM) requirements. Handling data on real-time basis, flexibility towards rapidly changing business opportunities and visibility of the entire supply chain are what seems to be missing in ERP. These requirements are forcing ERP to revolutionize itself. As a result, ERP vendors are releasing SCM modules and tools to enhance the existing capabilities of their ERP packages. In this context, our goal is to define the limitations of ERP both conceptually and in a practical sense through the use of extensive research and interviews with experts from various companies (Intel, Nike, LSI Logic and Promatis ). From these methods we will recommend ways to overcome the shortcomings due to the limitation issues.

Limitations of Current ERP Packages Regarding the New Supply Chain Management Requirements in the New Millennium

The future of Enterprise Resource Planning (ERP).is at risk. The existing structure of ERP is inadequate to meet new Supply Chain Management (SCM) requirements. Handling data on real-time basis, flexibility towards rapidly changing business opportunities and visibility of the entire supply chain are what seems to be missing in ERP. These requirements are forcing ERP to revolutionize itself. As a result, ERP vendors are releasing SCM modules and tools to enhance the existing capabilities of their ERP packages. In this context, our goal is to define the limitations of ERP both conceptually and in a practical sense through the use of extensive research and interviews with experts from various companies (Intel, Nike, LSI Logic and Promatis ). From these methods we will recommend ways to overcome the shortcomings due to the limitation issues.

Possible negative effects of snoring and increased sleep fragmentation on developmental status of preschool children

Limited studies have investigated the effects of snoring although harmful neurocognitive effects of obstructive sleep apnea syndrome are relatively well known. The aim of the present study was to investigate the effects of snoring and fragmented sleep on mental development in preschool children. The study population consisted of 212 healthy preschool children (age range 3.1-6 years; mean = 5.4 +/- 0.6 years; 112 boys, 100 girls). The developmental status of the children was investigated by using the Ankara Developmental Screening Inventory (ADSI) and Peabody Picture Vocabulary Test (PBT). Children's sleep and snoring frequency were assessed by using parent completed Children's Sleep Habits Questionnaire (CSHQ). Snoring children had lower scores of language/cognitive skills, social/activities of daily living skills, and general development compared to controls (P 0.05). Children with fragmented sleep had lower scores of language/cognitive skills, fine motor skills, social/activities of daily living skills, and general development compared to controls (P 0.05). Present study suggested that healthy preschool children who have habitual snoring or sleep fragmentation may have reduced scores of various mental development compared to controls. Uninterrupted sleep may be more important than sleep duration for the development of the various mental abilities in healthy preschool children. More studies investigating snoring and sleep fragmentation on mental development in children are warranted for a definite conclusion

A data-driven decision support system with smart packaging in grocery store supply chains during outbreaks

The unexpected emergence of the COVID-19 pandemic has changed how grocery shopping is done. The grocery retail stores need to ensure hygiene, quality, and safety concerns in-store shopping by providing “no-touch” smart packaging solutions for agri-food products. The benefit of smart packaging is to inform consumers about the freshness level of a packaged product without having direct contact. This paper proposes a data-driven decision support system that uses smart packaging as a smart product-service system to manage the sustainable grocery store supply chain during outbreaks to prevent food waste. The proposed model dynamically updates the price of a packaged perishable product depending on freshness level while reducing food waste and the number of rejected customers and maximising profit by increasing the inventory turnover rate of grocery stores. The model was tested on a hypothetical but realistic case study of a single product. The results of this study showed that stock capacities, freshness discount rate, freshness period, and quantity discounts significantly affect the performance of a grocery store supply chain during outbreaks

Internet Service Provider Decision Making Model

There are several companies that offer access to the Internet. The connection type that these companies offer will determine the speed or bandwidth that the customer will have to the ability to access the information highway. The applications have been growing and the Internet is becoming graphic intensive. For this reason the connection types have evolved and now the customers have several options that go from dial-up modem (56 Kb per second) to broadband connections such as DSL (25 times faster than regular modem connections). New technologies are arising and the newest type of connection is the satellite that could be accessed from anywhere in the world with a considerable speed that has an average of 10 times the dial-up speed. The study showed that the different companies . in the search for differentiating their products have developed several packages or plans to meet the diverse needs of the customers. Some of the packages that are offered include: different speed connections and prices, email access, content website and technical support that could be accessed from the server web page. The following study will give the customers a model to help them make a wise decision when choosing from different ISP\u27s. The decision will have a subjective input based on the preferences of the customer over the features that the different companies have to offer. The different companies, AOL, MSN, AT&T, Verizon, Qwest, Earthlink and Freelnternet, were picked based on the most popular ones for the Portland area that were shown in Cnet\u27s website