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Living-Related Liver Transplantation with Heterozygote Carrier Graft in Children with Wilson Disease (윌슨병 환아에서 이형 접합 보인자 공여간을 이용한 혈연간 생체 부분 간이식)

  • Kim, Jin-Taek;Chang, Soo-Hee;Choi, Bo-Hwa;Kim, Kyung-Mo;Yoo, Han-Wook;Lee, Young-Joo;Lee, Sung-Gyu
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.6 no.2
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    • pp.161-166
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    • 2003
  • Purpose: The purpose of this study was to evaluate the efficiency of treatment of living-related liver transplantation (LRLT) with the parental heterozygote carrier graft in children with Wilson disease. Methods: We retrospectively evaluated 7 children with Wilson disease who had received liver transplantation from 1994 to 2002 at Asan Medical Center. All the donors were parental. Liver functions, Kayser-Fleischer ring, and other factors regarding to copper metabolism were analyzed. Results: Of the 7 children, 5 had fulminant hepatitis and 2 had decompensated liver cirrhosis irresponsive to medical therapy. All donors being parental, all grafts came to be heterozygote carrier grafts. Survival rate was 100% in those 7 children, 87% in all children with liver transplantation in the same period, and 84% in children with non-metabolic liver disease. After liver transplantation, all 7 children could stop low copper diet and penicillamine therapy and their AST, total bilirubin and prothrombin time were recovered to normal. After liver transplantation, ceruloplasmin and serum copper levels were also recovered to normal. A marked reduction in 24 hr-urinary copper excretion was observed in all recipients after transplantation. During follow-up, Kayser-Fleischer rings resolved completely after LRLT in 5 children and partially in 1 child. Conclusion: We concluded that living-related liver tranplantation in children with Wilson disease with parental heterozygote carrier graft is an effective treatment modality.

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Review of Production, Husbandry and Sustainability of Free-range Pig Production Systems

  • Miao, Z.H.;Glatz, P.C.;Ru, Y.J.
    • Asian-Australasian Journal of Animal Sciences
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    • v.17 no.11
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    • pp.1615-1634
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    • 2004
  • A review was undertaken to obtain information on the sustainability of pig free-range production systems including the management, performance and health of pigs in the system. Modern outdoor rearing systems requires simple portable and flexible housing with low cost fencing. Local pig breeds and outdoor-adapted breeds for certain environment are generally more suitable for free-range systems. Free-range farms should be located in a low rainfall area and paddocks should be relatively flat, with light topsoil overlying free-draining subsoil with the absence of sharp stones that can cause foot damage. Huts or shelters are crucial for protecting pigs from direct sun burn and heat stress, especially when shade from trees and other facilities is not available. Pigs commonly graze on strip pastures and are rotated between paddocks. The zones of thermal comfort for the sow and piglet differ markedly; between 12-22$^{\circ}C$ for the sow and 30-37$^{\circ}C$ for piglets. Offering wallows for free-range pigs meets their behavioural requirements, and also overcomes the effects of high ambient temperatures on feed intake. Pigs can increase their evaporative heat loss via an increase in the proportion of wet skin by using a wallow, or through water drips and spray. Mud from wallows can also coat the skin of pigs, preventing sunburn. Under grazing conditions, it is difficult to control the fibre intake of pigs although a high energy, low fibre diet can be used. In some countries outdoor sows are fitted with nose rings to prevent them from uprooting the grass. This reduces nutrient leaching of the land due to less rooting. In general, free-range pigs have a higher mortality compared to intensively housed pigs. Many factors can contribute to the death of the piglet including crushing, disease, heat stress and poor nutrition. With successful management, free-range pigs can have similar production to door pigs, although the growth rate of the litters is affected by season. Piglets grow quicker indoors during the cold season compared to outdoor systems. Pigs reared outdoors show calmer behaviour. Aggressive interactions during feeding are lower compared to indoor pigs while outdoor sows are more active than indoor sows. Outdoor pigs have a higher parasite burden, which increases the nutrient requirement for maintenance and reduces their feed utilization efficiency. Parasite infections in free-range pigs also risks the image of free-range pork as a clean and safe product. Diseases can be controlled to a certain degree by grazing management. Frequent rotation is required although most farmers are keeping their pigs for a longer period before rotating. The concept of using pasture species to minimise nematode infections in grazing pigs looks promising. Plants that can be grown locally and used as part of the normal feeding regime are most likely to be acceptable to farmers, particularly organic farmers. However, one of the key concerns from the public for free-range pig production system is the impact on the environment. In the past, the pigs were held in the same paddock at a high stocking rate, which resulted in damage to the vegetation, nutrient loading in the soil, nitrate leaching and gas emission. To avoid this, outdoor pigs should be integrated in the cropping pasture system, the stock should be mobile and stocking rate related to the amount of feed given to the animals.