• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• Journal of Distribution Science
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• v.9 no.1
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• pp.17-27
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• 2011

As the world becomes more globalized, business competition becomes fiercer, while consumers' needs for less expensive quality products are on the increase. Business operations make an effort to secure a competitive edge in costs and services, and the logistics industry, that is, the industry operating the storing and transporting of goods, once thought to be an expense, begins to be considered as the third cash cow, a source of new income. Logistics centers are central to storage, loading and unloading of deliveries, packaging operations, and dispensing goods' information. As hubs for various deliveries, they also serve as a core infrastructure to smoothly coordinate manufacturing and selling, using varied information and operation systems. Logistics centers are increasingly on the rise as centers of business supply activities, growing beyond their previous role of primarily storing goods. They are no longer just facilities; they have become logistics strongholds that encompass various features from demand forecast to the regulation of supply, manufacturing, and sales by realizing SCM, taking into account marketability and the operation of service and products. However, despite these changes in logistics operations, some centers have been unable to shed their past roles as warehouses. For the continuous development of logistics centers, various measures would be needed, including a revision of current supporting policies, formulating effective management plans, and establishing systematic standards for founding, managing, and controlling logistics centers. To this end, the research explored previous studies on the use and effectiveness of logistics centers. From a theoretical perspective, an evaluation of the overall introduction, purposes, and transitions in the use of logistics centers found issues to ponder and suggested measures to promote and further advance logistics centers. First, a fact-finding survey to establish demand forecast and standardization is needed. As logistics newspapers predicted that after 2012 supply would exceed demand, causing rents to fall, the business environment for logistics centers has faltered. However, since there is a shortage of fact-finding surveys regarding actual demand for domestic logistic centers, it is hard to predict what the future holds for this industry. Accordingly, the first priority should be to get to the essence of the current market situation by conducting accurate domestic and international fact-finding surveys. Based on those, management and evaluation indicators should be developed to build the foundation for the consistent advancement of logistics centers. Second, many policies for logistics centers should be revised or developed. Above all, a guideline for fair trade between a shipper and a commercial logistics center should be enacted. Since there are no standards for fair trade between them, rampant unfair trades according to market practices have brought chaos to market orders, and now the logistics industry is confronting its own difficulties. Therefore, unfair trade cases that currently plague logistics centers should be gathered by the industry and fair trade guidelines should be established and implemented. In addition, restrictive employment regulations for foreign workers should be eased, and logistics centers should be charged industry rates for the use of electricity. Third, various measures should be taken to improve the management environment. First, we need to find out how to activate value-added logistics. Because the traditional purpose of logistics centers was storage and loading/unloading of goods, their profitability had a limit, and the need arose to find a new angle to create a value added service. Logistic centers have been perceived as support for a company's storage, manufacturing, and sales needs, not as creators of profits. The center's role in the company's economics has been lowering costs. However, as the logistics' management environment spiraled, along with its storage purpose, developing a new feature of profit creation should be a desirable goal, and to achieve that, value added logistics should be promoted. Logistics centers can also be improved through cost estimation. In the meantime, they have achieved some strides in facility development but have still fallen behind in others, particularly in management functioning. Lax management has been rampant because the industry has not developed a concept of cost estimation. The centers have since made an effort toward unification, standardization, and informatization while realizing cost reductions by establishing systems for effective management, but it has been hard to produce profits. Thus, there is an urgent need to estimate costs by determining a basic cost range for each division of work at logistics centers. This undertaking can be the first step to improving the ineffective aspects of how they operate. Ongoing research and constant efforts have been made to improve the level of effectiveness in the manufacturing industry, but studies on resource management in logistics centers are hardly enough. Thus, a plan to calculate the optimal level of resources necessary to operate a logistics center should be developed and implemented in management behavior, for example, by standardizing the hours of operation. If logistics centers, shippers, related trade groups, academic figures, and other experts could launch a committee to work with the government and maintain an ongoing relationship, the constraint and cooperation among members would help lead to coherent development plans for logistics centers. If the government continues its efforts to provide financial support, nurture professional workers, and maintain safety management, we can anticipate the continuous advancement of logistics centers.