The Experts below are selected from a list of 213 Experts worldwide ranked by ideXlab platform
Alan Norman Langnas - One of the best experts on this subject based on the ideXlab platform.
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Isolated Liver transplantation in infants with end-stage Liver disease due to short bowel syndrome.
Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation S, 2006Co-Authors: Jean F. Botha, Debra L Sudan, Angie Iverson, Byers W Shaw, Wendy J. Grant, Clarivet Torres, Alan Norman LangnasAbstract:Infants with short bowel syndrome (SBS) and associated Liver failure are often referred for combined Liver/intestinal transplantation. We speculated that in some young children, nutritional autonomy would be possible with restoration of normal Liver function. Features we believed to predict nutritional autonomy include history of at least 50% enteral tolerance, age less than 2 yr, and no underlying intestinal disease. This report documents our experience with Liver transplantation alone in children with Liver failure associated with SBS. Twenty-three children with SBS and end-stage Liver disease, considered to have good prognostic features for eventual full enteral adaptation, underwent Isolated Liver transplantation. Median age was 11 months (range, 6.5 to 48 months). Median pretransplant weight was 7.4 kg (range, 5.2 to 15 kg). All had growth retardation and advanced Liver disease. Bowel length ranged from 25 to 100 cm. Twenty-three children underwent 28 Isolated Liver transplants. There were 14 whole Livers and 14 partial grafts (five living donors). Seventeen patients are alive at a median follow-up of 57 months (range, 6 to 121 months). Actuarial patient and graft survival rates at 1 yr are 82% and 75% and at 5 yr are 72% and 60%, respectively. Four deaths resulted from sepsis, all within 4 months of transplantation, and 1 death resulted from progressive Liver failure. Two allografts developed chronic rejection; both children were successfully retransplanted with Isolated Livers. Of 17 surviving patients, three require supplemental intravenous support; the remaining 14 have achieved enteral autonomy, at a median of 3 months (range, 1 to 72 months) after transplantation. Linear growth is maintained and, in many, catch-up growth is evident. Median change in z score for height is 0.57 (range, -4.47 to 2.68), and median change in z score for weight is 0.42 (range, -1.65 to 3.05). In conclusion, Isolated Liver transplantation in children with Liver failure as a result of SBS, who have favorable prognostic features for full enteral adaptation, is feasible with satisfactory long-term survival.
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Isolated Liver transplantation in infants with end stage Liver disease associated with short bowel syndrome
Annals of Surgery, 2002Co-Authors: Simon Horslen, Debra L Sudan, Kishore Iyer, Stuart S Kaufman, Angie Iverson, Byers W Shaw, Alan Norman LangnasAbstract:The ability of patients with intestinal failure resulting from short bowel syndrome (SBS) to achieve nutritional autonomy depends on the capacity of the gut remnant to compensate or adapt. Intestinal adaptation requires increasing the functional absorptive surface by growth in length and diameter as well as in villous height and crypt depth. 1 If fibrotic Liver disease develops before enteral autonomy is achieved, a downward spiral occurs, with decreased enteral tolerance, nutritional impairment, and progression to Liver failure. The deterioration of bowel function is related to several factors, including anorexia, intestinal malabsorption secondary to luminal bile acid deficiency, ascites, bowel wall edema and portal hypertensive exudative enteropathy, and gastrointestinal bleeding. 2 Typically patients are then considered for intestinal or combined Liver and small bowel transplantation. In selected patients, if it were not for the appearance of severe Liver disease, there might still be considerable optimism that adaptation of the bowel to full enteral tolerance could be achieved in time. In these circumstances, is there a role for Isolated Liver transplantation? Anecdotal accounts have circulated that Liver transplantation alone should not be attempted because in these patients cholestasis will inevitably develop in the allograft after transplantation and that the prospect of intestinal adaptation is negligible. Despite these dire warnings, several groups have successfully carried out Isolated Liver transplantation in children with end-stage Liver disease related to SBS, although reports in the literature are few. 2–4 We now report the largest experience with Isolated Liver transplantation in children with Liver failure associated with SBS.
Girish Gupte - One of the best experts on this subject based on the ideXlab platform.
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Isolated Liver transplant in infants with short bowel syndrome: insights into outcomes and prognostic factors.
Journal of pediatric gastroenterology and nutrition, 2009Co-Authors: Dominic Dell-olio, J. De Ville De Goyet, Sue V. Beath, S. Clarke, P. Davies, Christine Lloyd, Susan Protheroe, A. J. Millar, Deirdre A Kelly, Girish GupteAbstract:ABSTRACTObjective:Selected infants with short bowel syndrome (SBS) and progressive intestinal failure associated Liver disease (IFALD) may benefit from Isolated Liver transplantation (iLTx). The aim of the study is to identify risk factors for unfavourable outcome in iLTx.Patients and Methods:A retr
Simon Horslen - One of the best experts on this subject based on the ideXlab platform.
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Isolated Liver transplantation in infants with end stage Liver disease associated with short bowel syndrome
Annals of Surgery, 2002Co-Authors: Simon Horslen, Debra L Sudan, Kishore Iyer, Stuart S Kaufman, Angie Iverson, Byers W Shaw, Alan Norman LangnasAbstract:The ability of patients with intestinal failure resulting from short bowel syndrome (SBS) to achieve nutritional autonomy depends on the capacity of the gut remnant to compensate or adapt. Intestinal adaptation requires increasing the functional absorptive surface by growth in length and diameter as well as in villous height and crypt depth. 1 If fibrotic Liver disease develops before enteral autonomy is achieved, a downward spiral occurs, with decreased enteral tolerance, nutritional impairment, and progression to Liver failure. The deterioration of bowel function is related to several factors, including anorexia, intestinal malabsorption secondary to luminal bile acid deficiency, ascites, bowel wall edema and portal hypertensive exudative enteropathy, and gastrointestinal bleeding. 2 Typically patients are then considered for intestinal or combined Liver and small bowel transplantation. In selected patients, if it were not for the appearance of severe Liver disease, there might still be considerable optimism that adaptation of the bowel to full enteral tolerance could be achieved in time. In these circumstances, is there a role for Isolated Liver transplantation? Anecdotal accounts have circulated that Liver transplantation alone should not be attempted because in these patients cholestasis will inevitably develop in the allograft after transplantation and that the prospect of intestinal adaptation is negligible. Despite these dire warnings, several groups have successfully carried out Isolated Liver transplantation in children with end-stage Liver disease related to SBS, although reports in the literature are few. 2–4 We now report the largest experience with Isolated Liver transplantation in children with Liver failure associated with SBS.
Dominic Dell-olio - One of the best experts on this subject based on the ideXlab platform.
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Isolated Liver transplant in infants with short bowel syndrome: insights into outcomes and prognostic factors.
Journal of pediatric gastroenterology and nutrition, 2009Co-Authors: Dominic Dell-olio, J. De Ville De Goyet, Sue V. Beath, S. Clarke, P. Davies, Christine Lloyd, Susan Protheroe, A. J. Millar, Deirdre A Kelly, Girish GupteAbstract:ABSTRACTObjective:Selected infants with short bowel syndrome (SBS) and progressive intestinal failure associated Liver disease (IFALD) may benefit from Isolated Liver transplantation (iLTx). The aim of the study is to identify risk factors for unfavourable outcome in iLTx.Patients and Methods:A retr
Byers W Shaw - One of the best experts on this subject based on the ideXlab platform.
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Isolated Liver transplantation in infants with end-stage Liver disease due to short bowel syndrome.
Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation S, 2006Co-Authors: Jean F. Botha, Debra L Sudan, Angie Iverson, Byers W Shaw, Wendy J. Grant, Clarivet Torres, Alan Norman LangnasAbstract:Infants with short bowel syndrome (SBS) and associated Liver failure are often referred for combined Liver/intestinal transplantation. We speculated that in some young children, nutritional autonomy would be possible with restoration of normal Liver function. Features we believed to predict nutritional autonomy include history of at least 50% enteral tolerance, age less than 2 yr, and no underlying intestinal disease. This report documents our experience with Liver transplantation alone in children with Liver failure associated with SBS. Twenty-three children with SBS and end-stage Liver disease, considered to have good prognostic features for eventual full enteral adaptation, underwent Isolated Liver transplantation. Median age was 11 months (range, 6.5 to 48 months). Median pretransplant weight was 7.4 kg (range, 5.2 to 15 kg). All had growth retardation and advanced Liver disease. Bowel length ranged from 25 to 100 cm. Twenty-three children underwent 28 Isolated Liver transplants. There were 14 whole Livers and 14 partial grafts (five living donors). Seventeen patients are alive at a median follow-up of 57 months (range, 6 to 121 months). Actuarial patient and graft survival rates at 1 yr are 82% and 75% and at 5 yr are 72% and 60%, respectively. Four deaths resulted from sepsis, all within 4 months of transplantation, and 1 death resulted from progressive Liver failure. Two allografts developed chronic rejection; both children were successfully retransplanted with Isolated Livers. Of 17 surviving patients, three require supplemental intravenous support; the remaining 14 have achieved enteral autonomy, at a median of 3 months (range, 1 to 72 months) after transplantation. Linear growth is maintained and, in many, catch-up growth is evident. Median change in z score for height is 0.57 (range, -4.47 to 2.68), and median change in z score for weight is 0.42 (range, -1.65 to 3.05). In conclusion, Isolated Liver transplantation in children with Liver failure as a result of SBS, who have favorable prognostic features for full enteral adaptation, is feasible with satisfactory long-term survival.
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Isolated Liver transplantation in infants with end stage Liver disease associated with short bowel syndrome
Annals of Surgery, 2002Co-Authors: Simon Horslen, Debra L Sudan, Kishore Iyer, Stuart S Kaufman, Angie Iverson, Byers W Shaw, Alan Norman LangnasAbstract:The ability of patients with intestinal failure resulting from short bowel syndrome (SBS) to achieve nutritional autonomy depends on the capacity of the gut remnant to compensate or adapt. Intestinal adaptation requires increasing the functional absorptive surface by growth in length and diameter as well as in villous height and crypt depth. 1 If fibrotic Liver disease develops before enteral autonomy is achieved, a downward spiral occurs, with decreased enteral tolerance, nutritional impairment, and progression to Liver failure. The deterioration of bowel function is related to several factors, including anorexia, intestinal malabsorption secondary to luminal bile acid deficiency, ascites, bowel wall edema and portal hypertensive exudative enteropathy, and gastrointestinal bleeding. 2 Typically patients are then considered for intestinal or combined Liver and small bowel transplantation. In selected patients, if it were not for the appearance of severe Liver disease, there might still be considerable optimism that adaptation of the bowel to full enteral tolerance could be achieved in time. In these circumstances, is there a role for Isolated Liver transplantation? Anecdotal accounts have circulated that Liver transplantation alone should not be attempted because in these patients cholestasis will inevitably develop in the allograft after transplantation and that the prospect of intestinal adaptation is negligible. Despite these dire warnings, several groups have successfully carried out Isolated Liver transplantation in children with end-stage Liver disease related to SBS, although reports in the literature are few. 2–4 We now report the largest experience with Isolated Liver transplantation in children with Liver failure associated with SBS.
