• Archives of Plastic Surgery
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• v.32 no.5
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• pp.648-652
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• 2005

In 1940, Kasabach and Merritt first described the association of a large vascular tumor and thrombocytopenia and termed this Kasabach-Merritt(KM) syndrome. It is characterized by a rapidly enlarging vascular anomaly and consumptive coagulopathy with thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, hypofibrinogenemia, and the presence of D-dimer and fibrin split product, with or without microangiopathic hemolytic anemia. This is a potentially life-threatening condition with mortality rates from 20 to 30% as a result of severe sepsis, coagulopathy, or invasion of vital organs. Treatment modalities are corticosteroids, interferon alfa-2a or 2b, chemotherapy(vincristine, cyclophosphamide, etc.), aspirin, dipyridamole, com- pression, radiation therapy, embolization of feeding vessels and surgical excision. A standard treatment regimen for KM syndrome has not been established and most reports on definitive management of these complex vascular lesions have been anecdotal, involving small numbers of patients. The authors have successfully treated a patient of KM syndrome with actively bleeding huge hemangioma by surgical excision. They present it with the review of articles.

• Childhood Kidney Diseases
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• v.24 no.2
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• pp.120-125
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• 2020

Gorham-Stout syndrome is a rare bone disorder characterized by progressive massive osteolysis and proliferation of vascular and lymphatic vessels. A 15-year-old boy was initially diagnosed with Gorham-Stout at the age of 8 years based on clinical and radiological findings. Following diagnosis, he was treated with pamidronate, interferon alfa, propranolol, oral corticosteroids, and sirolimus. He developed proteinuria at the age of 15 and progressed into the nephrotic range 2 years later. A renal biopsy revealed focal segmental glomerulosclerosis, not otherwise specified variant. The sequential increase in proteinuria associated with medications suggested that the focal segmental glomerulosclerosis may be caused by pamidronate and sirolimus, but cannot completely rule out the possibility of kidney involvement of GSS itself.

• Proceedings of the CALSEC Conference
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• 2002.01a
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• pp.86-106
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• 2002

Informatization is a process that corporation's external environmental factors and internal environmental factors influence as complex. is a phenomenon that appears via this process. To evaluate that informatization was propeled well or informatization level is high can be dangerous work extremely by only once-over-lightly some factors, organization information ability is superior or infrastructure is constructed well. Therefore, an evaluation for industrial information systems that consider corporation's external environment and internal environment configurationally and objective estimation through this is required in national dimension. This research sorted types of business using types of business classification of 2001 EIII(Evaluation Indices of Industrial Informatization) laying stress on corporation's product and product production process for reflecting various industrial classification. And we are dividing whole our country corporations by manufacture industry, the construction industry, distribution industry, service industry, banking industry 5 types of business. To see such classed types industry classification from consistent viewpoint, we saw them within new framework, purchase, operation, physical distribution, marketing and sale. service etc. laying stress on primary businesses except support businesses of planning, financial management etc. To draw special quality of business center from primary business of each types of business, we draw industry classification Key Capability that centers when plans corporation's corporate strategy and information strategy. And we deducted industrial classification key production business connected with industry classification Key Capability. After drawing an evaluation items for industrial information systems in informatization analysis viewpoint laying stress on drawn businesses. Finally we did Case Study by making out an evaluation for industrial information systems questionnaire that considers special quality of manufacturing industry. Through EIII that consider the industrial classification, we could know that it explains the corporation's purchase, production, distribution in general and detail.