Thrombotic thrombocytopenic purpura in pediatric patients / 中国当代儿科杂志

Although thrombotic thrombocytopenic purpura (TTP) is rarely seen in pediatric patients, failure to recognize this condition often leads to severe consequences and poor outcomes. Classic features of TTP include thrombocytopenia, microangiopathic hemolytic anemia, acute kidney injury, fever, and central nervous system involvement. However, patients suffering from this condition may not present with all of the symptoms simultaneously. Therefore, it is of utmost importance for healthcare providers to have a high index of suspicion. Laboratory investigations may reveal the presence of schistocytes on peripheral blood smear, negative Coombs test, high lactate dehydrogenase levels and severely low platelet counts. The etiology of TTP is mainly due to insufficient cleavage of the large multimers of von Willebrand factor (vWF) secondary to decreased activity of ADAMTS13 (a disintegrin and metalloprotease with Thrombospondin type 1 repeats, member 13). TTP can be broadly classified into familial TTP (Upshaw Schulman syndrome) and non-familial TTP. Familial TTP is due to a congenital deficiency of ADAMTS13. Its mainstay of therapy is initiation of plasmapheresis during the acute phase, followed by regular fresh frozen plasma (FFP) infusions. Alternatively, non-familial TTP is due to a decrease in ADAMTS13 activity secondary to the presence of anti-ADAMTS13 antibodies. Once again, the primary treatment is plasmapheresis; however, recent anecdotal data also supports the use of rituximab in select cases.

Information policy and management framework for environmental protection organization with ecosystem conception

Rapid development of information technology has changed people's attitudes towards information usage. To tender to the public's expectation, information system must feature facilities to increase the efficiency of information usage using modern information technology. Facing this challenge, it is necessary to establish a sustainable information environment, including information policy, data quality regulations and information management framework to deal with the rapidly increasing environmental data and changing behavior related to data/information usage except upgrading the hardware and software devices. Taking the uniqueness and complexity of environmental data into account, this study proposes a systematic framework based on the principle of life cycle assessment to outline the elements and its associated guidance required for a sustainable information environment. Simultaneously, the concept of information ecology is also embedded into such a planning for the purpose of establishing a self-evolutional information environment. Finally, the environmental protection administration of Taiwan is used as a case study to explain the practice of proposed framework