C. Lambe

Outcome of home parenteral nutrition in 251 children over a 14-y period: report of a single center.

BACKGROUND Parenteral nutrition (PN) is the main treatment for intestinal failure. OBJECTIVE We aimed to review the indications for home parenteral nutrition (HPN) in children and describe the outcome over a 14-y period from a single center. DESIGN We conducted a retrospective study that included all children who were referred to our institution and discharged while receiving HPN between 1 January 2000 and 31 December 2013. The indications for HPN were divided into primary digestive diseases (PDDs) and primary nondigestive diseases (PNDDs). We compared our results to a previous study that was performed in our unit from 1980 to 2000 and included 302 patients. RESULTS A total of 251 patients were included: 217 (86%) had a PDD. The mean ± SD age at HPN onset was 0.7 ± 0.3 y, with a mean duration of 1.9 ± 0.4 y. The indications for HPN were short bowel syndrome (SBS) (59%), PNDD (14%), congenital enteropathies (10%), chronic intestinal pseudo-obstruction syndromes (9%), inflammatory bowel diseases (5%), and other digestive diseases (3%). By 31 December 2013, 52% of children were weaned off of HPN, 9% of the PDD subgroup had intestinal transplantation, and 10% died mostly because of immune deficiency. The major complications of HPN were catheter-related bloodstream infections (CRBSIs) (1.7/1000 d of PN) and intestinal failure-associated liver disease (IFALD) (51 children; 20% of cohort). An increased rate of CRBSIs was observed compared with our previous study, but we saw a decreasing trend since 2012. No noteworthy deceleration of growth was observed in SBS children 6 mo after weaning off HPN. CONCLUSIONS SBS was the major indication for HPN in our cohort. IFALD and CRBSIs were potentially life-threatening problems. Nevertheless, complication rates were low, and deaths resulted mostly from the underlying disease.

Intravenous lipid emulsions in pediatric patients with intestinal failure

The incidence of cholestatic liver disease (CLD) in pediatric patients suffering intestinal failure (IF) is not well established. Due to persistent portal inflammation, about 20% of these patients will progress to end-stage intestinal failure associated liver disease (IFALD) leading to liver transplant or death. Purpose of review Premature babies as well as infants with short bowel syndrome (SBS) and repeated sepsis (catheter or small intestinal bacterial overgrowth related) are at risk of developing CLD. Clinical data in SBS infants focused on intravenous lipid emulsion (ILE) as an important factor of CLD. Recent findings Compared to the last generation of composite ILE containing fish oil (FO), soybean oil (SO) based ILE, have marked differences in term of oil source, omega-3 fatty acids (FAs) composition, vitamin E (&agr;-tocopherols) and plant sterols contents, that may explain CLD and CLD reversal. Randomized controlled trials and meta-analysis allow the following recommendations. Summary In pediatric patients with developing or established CLD or IFALD, potential causes should be explored and pure SO ILE should be avoided. A reduction of the ILE dosage and/or the use of the new composite FO based ILE, may be recommended along with the treatment and management of other risk factors. The 10% pure FO ILE should not be used as a sole provision of IV lipids in paediatric patients on total PN but can only serve as a short-term rescue treatment.

Strategies to Reduce Catheter-Related Bloodstream Infections in Pediatric Patients Receiving Home Parenteral Nutrition: The Efficacy of Taurolidine-Citrate Prophylactic-Locking

BACKGROUND Catheter-related bloodstream infections (CRBSIs) remain a major issue in patients who are receiving home parenteral nutrition (HPN). The aim of this interventional study was to assess the impact of a new strategy using taurolidine-citrate (T-C) prophylactic locks on the CRBSI rate in children with intestinal failure who are receiving HPN. METHODS The rate of CRBSIs was monitored every calendar year in a prospective cohort of 195 children with intestinal failure. T-C locks were initiated from October 2011 in children with recurring CRBSIs (≥2 episodes per year). RESULTS In the whole cohort, the median annual CRBSI rate per 1000 catheter days decreased significantly from 2.07 in 2008 to 2010 to 1.23 in 2012 to 2014 (P < .05). T-C locks were used in 40 patients. No adverse events were reported. In taurolidine-treated patients, the CRBSI rate per 1000 catheter days decreased from 4.16 to 0.25 (P < .0001). The cumulative percentage of patients free of CRBSI at 18 months was 92% (95% confidence interval [CI]: 71-98) on T-C lock vs 61% (95% CI: 49-72) in controls (P = .01). In multivariate analysis, factors associated with CRBSI were immune deficiency (adjusted hazard ratio 3.49; 95% CI: 1.01-12.17) and the young age of the parents (adjusted hazard ratio 4.79, 95% CI: 2.16-10.62), whereas T-C locks were protective (adjusted hazard ratio 0.22, 95% CI: 0.06-0.74). CONCLUSION This study confirms the efficacy of T-C catheter locks in decreasing the incidence of CRBSIs in children with intestinal failure who are receiving HPN.

Ultra Short Bowel Syndrome (USBS) in Children: Long-Term Parenteral Nutrition in Comparison with Intestinal Transplantation.: Abstract# D3071

D3070 The Addition of Splenectomy to Simultaneous Liver Kidney Transplantation Improves Class II DSA Clearance in Sensitized Patients. R. Redfield, K. McCune, D. Foley, L. Fernandez, A. D’Alessandro, A. Musat, A. Djamali, T. Ellis, J. Mezrich. University of Wisconsin, Madison. Background: The liver allograft clears class I donor specifi c antibody (DSA) more effectively than class II DSA. Persistent class II DSA in simultaneous liver kidney (SLK) transplantation has been associated with inferior graft outcomes. Splenectomy is known to decrease DSA in some sensitized kidney recipients. We therefore sought to determine if splenectomy at the time of SLK reduces class II DSA. Methods: This is a single center retrospective study of sensitized SLK recipients (2008-2014). Results: 34 SLK transplants were performed over the study period at the University of Wisconsin. Of these, 13 patients had preformed class II DSA. 5/13 patients were sensitized with a max class II MFI>5000. 2 of these highly sensitized patients underwent a splenectomy and had complete eradication of their class II DSA (DR, DQ, DP) post-transplant (pre-transplant max MFI: 21,559 and 14,496). The other 3 did not undergo a splenectomy and had persistent class II DSA (post-transplant max MFI 10,508, 13,175, and 14,001), with 2 of these patients experiencing acute antibody mediated rejection. One additional patient with preformed class II DSA (pre-transplant max MFI 21,511) successfully underwent liver transplant and splenectomy, however kidney transplant was aborted secondary to intraoperative instability. This patient cleared most class II DSA, specifi cally complete eradication of most DP and DR specifi cities, and had signifi cant reductions in DQ, but remained highly sensitized to other non-donor specifi c class II loci. Conclusions: In our experience, the addition of a splenectomy at the time of SLK appears to reduce the level of class II DSA. Further study will be required to understand the mechanisms behind this observation and to determine if such a strategy improves graft outcomes. Abstract# D3071 Ultra Short Bowel Syndrome (USBS) in Children: Long-Term Parenteral Nutrition in Comparison with Intestinal Transplantation. S. Artru,1 F. Lacaille,1 C. Lambe,1 C. Talbotec,1 B. Pigneur,1 F. Ruemmele,1 C. Chardot,2 V. Colomb,1 O. Goulet.1 1Pediatric Gastroenterology, Necker-Enfants Malades Hospital, Paris, France; 2Pediatric Surgery, Necker-Enfants Malades Hospital, Paris, France. INTRODUCTION Intestinal transplantation (ITx) or combined liver and intestinal transplantation (LITx) represent the fi nal option for USBS patients with cirrhosis or loss of venous access resulting from thrombosis. This study aims to compare survival, PN dependency and nutritional status of USBS children on long term parenteral nutrition (LTPN) with USBS children who have been transplanted. POPULATION AND METHODS: This retrospective study includes 41 children and young adults followed since 1990. After extensive resection ultrashort bowel (USB) was defi ned according to the intraoperative measure of residual bowel length being ≤ 30cm with ileocaecal valve (ICV), or ≥ 40cm and ≤ 80 cm without ICV. Patients were enrolled in a LTPN program, 24 have received an intestinal graft. RESULTS: In the LTPN group (n=17), all children survived and are currently 14.6 ± 6 years old. Five children have been weaned from PN after 6.2 ± 5.5 years. For the 12 children still on LTPN, the average duration of PN is 10.6 ± 6.2 years, the average number of weekly infusion is 4.0 ± 1.8 days /7 while PN provides 49 ± 26 % of the recommended daily allowance (RDA).In the ITx group (n=24), 15 children were transplanted for extensive vascular thrombosis (5 of them with colon) and 9 had a combined transplantation(5 of them a combined bowel + liver and 4 a combined bowel + liver + duodenum + pancreas). The average age at transplantation was 4.7 ± 2.3 years. ITx group’s survival is 58% (67% for isolated ITx ± colon and 45 % in the combined transplantation group). Death occurred 10 ± 26 months after Tx. Among surviving transplanted children 100% have been weaned from PN, 69 ± 82 days after Tx. CONCLUSION: Even if 100% of the transplanted children who survived have been weaned from PN, survival rate is signifi cantly lower in the ITx group (p<0.05). A multidisciplinary approach by a specialized medical team may allow a total weaning of PN before adulthood. For those children still remaining PN dependent, PN infusions provide less than 50% of RDA, while the reduced number of week’s days of infusions makes their quality of life more acceptable. Extensive vascular thrombosis and/or severe liver disease occur remain indications for transplantation. D3071 Ultra Short Bowel Syndrome (USBS) in Children: Long-Term Parenteral Nutrition in Comparison with Intestinal Transplantation. S. Artru,1 F. Lacaille,1 C. Lambe,1 C. Talbotec,1 B. Pigneur,1 F. Ruemmele,1 C. Chardot,2 V. Colomb,1 O. Goulet.1 1Pediatric Gastroenterology, Necker-Enfants Malades Hospital, Paris, France; 2Pediatric Surgery, Necker-Enfants Malades Hospital, Paris, France. INTRODUCTION Intestinal transplantation (ITx) or combined liver and intestinal transplantation (LITx) represent the fi nal option for USBS patients with cirrhosis or loss of venous access resulting from thrombosis. This study aims to compare survival, PN dependency and nutritional status of USBS children on long term parenteral nutrition (LTPN) with USBS children who have been transplanted. POPULATION AND METHODS: This retrospective study includes 41 children and young adults followed since 1990. After extensive resection ultrashort bowel (USB) was defi ned according to the intraoperative measure of residual bowel length being ≤ 30cm with ileocaecal valve (ICV), or ≥ 40cm and ≤ 80 cm without ICV. Patients were enrolled in a LTPN program, 24 have received an intestinal graft. RESULTS: In the LTPN group (n=17), all children survived and are currently 14.6 ± 6 years old. Five children have been weaned from PN after 6.2 ± 5.5 years. For the 12 children still on LTPN, the average duration of PN is 10.6 ± 6.2 years, the average number of weekly infusion is 4.0 ± 1.8 days /7 while PN provides 49 ± 26 % of the recommended daily allowance (RDA).In the ITx group (n=24), 15 children were transplanted for extensive vascular thrombosis (5 of them with colon) and 9 had a combined transplantation(5 of them a combined bowel + liver and 4 a combined bowel + liver + duodenum + pancreas). The average age at transplantation was 4.7 ± 2.3 years. ITx group’s survival is 58% (67% for isolated ITx ± colon and 45 % in the combined transplantation group). Death occurred 10 ± 26 months after Tx. Among surviving transplanted children 100% have been weaned from PN, 69 ± 82 days after Tx. CONCLUSION: Even if 100% of the transplanted children who survived have been weaned from PN, survival rate is signifi cantly lower in the ITx group (p<0.05). A multidisciplinary approach by a specialized medical team may allow a total weaning of PN before adulthood. For those children still remaining PN dependent, PN infusions provide less than 50% of RDA, while the reduced number of week’s days of infusions makes their quality of life more acceptable. Extensive vascular thrombosis and/or severe liver disease occur remain indications for transplantation. Abstract# D3072 Human Islet Transplantation: An Overview. B. Sangalli,1 E. Barros,2 P. Rainkober,3 L. D’Albuquerque,4 E. Chaib.5 1Liver Transplantation Division, University of Santo Amaro School of Medicine, Sao Paulo, SP, Brazil; 2Liver Transplantation Division, University of Santo Amaro School of Medicine, Sao Paulo, SP, Brazil; 3Liver Transplantation Division, University of Santo Amaro School of Medicine, Sao Paulo, SP, Brazil; 4Liver Transplantation Division, University of Sao Paulo School of Medicine, Sao Paulo, SP, Brazil; 5Liver Transplantation Division, University of Sao Paulo School of Medicine, Sao Paulo, SP, Brazil. Our aim is to compare human islet transplantation across North America (USA/ Canada) and Europe. A literature search was conducted using Medline/Pubmed and Scielo with terms about islet transplantation from 2000 to 2013. We found 60 articles, but only 30 articles were selected. We look at sites of islet transplantation, number of islets, patient survival rate, islet survival rate, complications and immunossupression. We have found 268 patients (USA/Canada) and 104 patients (Europe). North America: site of transplantation portal vein infusion 262 (97.7%); mean number of islet transplant – 11,725 EI/Kg; 1 year and 3-5 years PSR and ISR – 100 (37.3%) and 16 (6%), respectively. The commonest complication was mouth ulcerations 119 (44.4%); immunossupression – TAC+SIR+DAC 227 (84.7%); corticosteroids 44 (16.4%). Europe: site of transplantation portal vein infusion 99 (95.2% ); mean number of islet transplant – 10,898 EI/Kg; 1 year and 3-5 years PSR and ISR – 43 (41.34%) and 24 (23.07%), respectively. The commonest complication was anemia – 23 (8.6%); immunossupression – TAC + SIR + MYC 87 (83.6%); corticosteroids 40 (38.5%).In both continents 1 year PSR (37.3% / 41.34%) was similar, although 3-5 years PSR is higher in Europe than North America. Main transplantation technique was portal vein infusion in both continents. Mouth ulceration and anemia prevailed in NA and Europe as complications, respectively. The association of Tacrolimus, Sirolimus and Daclizumab (NA) / Mycophenolate (Europe) without corticosteroid was the standard immunossupression. D3072 Human Islet Transplantation: An Overview. B. Sangalli,1 E. Barros,2 P. Rainkober,3 L. D’Albuquerque,4 E. Chaib.5 1Liver Transplantation Division, University of Santo Amaro School of Medicine, Sao Paulo, SP, Brazil; 2Liver Transplantation Division, University of Santo Amaro School of Medicine, Sao Paulo, SP, Brazil; 3Liver Transplantation Division, University of Santo Amaro School of Medicine, Sao Paulo, SP, Brazil; 4