BACKGROUND Post-pancreaticoduodenectomy(PD)intestinal failure(IF)is rare and associated with poor outcomes.To our knowledge,the role of intestinal transplantation(ITx)as a rescue treatment for this complication has ne...BACKGROUND Post-pancreaticoduodenectomy(PD)intestinal failure(IF)is rare and associated with poor outcomes.To our knowledge,the role of intestinal transplantation(ITx)as a rescue treatment for this complication has never been reported.CASE SUMMARY A 42-year-old female with a benign neurilemmoma of the duodenum underwent PD.Her superior mesenteric vein(SMV)was injured during surgery and required reconstruction.She experienced SMV thrombosis and bowel gangrene requiring massive bowel resection.Consequently,she developed short gut syndrome and an enterocutaneous fistula,leading to prolonged hospitalization for wound care and total parenteral nutrition(TPN)support.She was referred to our hospital for ITx evaluation.Upon arrival,she had cholestasis due to IF-associated liver disease.After gastrointestinal(GI)reconstruction to restore GI continuity,she was eligible for multi-visceral transplantation(MVTx).The anticipated allograft included the stomach,small intestine,liver,pancreas,and duodenum.She found a suitable donor after two years of waiting.The MVTx procedure was straightforward with signs of immediate function.Enteral feeding was initiated on postoperative day(POD)7.TPN weaning was achieved on POD 28,and the patient was discharged on POD 69.Two years post-MVTx,she is healthy with excellent graft function.To our knowledge,this is the first case report on MVTx as the treatment for fatal post-PD complications and also the first reported case of ITx in Southeast Asia.CONCLUSION Post-PD IF is rare and lethal.Intestinal and MVTx might be a rescue treatment for IF after GI surgery in eligible patients.展开更多
The most common cause of intestinal failure is short bowel syndrome (SBS), occurring as a result of a small functional intestine length, usually less than 200 cm, leading to intestinal malabsorption. A 59-year-old fem...The most common cause of intestinal failure is short bowel syndrome (SBS), occurring as a result of a small functional intestine length, usually less than 200 cm, leading to intestinal malabsorption. A 59-year-old female with a past medical history of Crohns disease status post total colectomy with ileostomy over 20 years ago came to the hospital due to progressive weakness. Despite medical management, the patient had high ileostomy output, leading to electrolyte disbalance, metabolic acidosis, dehydration, and progressive kidney decline. Due to the high dependence on continuous fluid supplementation, it was decided to place a port for parenteral hydration to maintain fluid replacements and homeostasis after discharge. Prompt initiation of parenteral fluid replacement and close follow-up on patients with ileostomy and intestinal failure is strongly recommended to avoid complications and prevent intestinal, liver, or kidney transplants.展开更多
Chronic intestinal failure(CIF)is a rare but feared complication of Crohn’s disease.Depending on the remaining length of the small intestine,the affected intestinal segment,and the residual bowel function,CIF can res...Chronic intestinal failure(CIF)is a rare but feared complication of Crohn’s disease.Depending on the remaining length of the small intestine,the affected intestinal segment,and the residual bowel function,CIF can result in a wide spectrum of symptoms,from single micronutrient malabsorption to complete intestinal failure.Management of CIF has improved significantly in recent years.Advances in home-based parenteral nutrition,in particular,have translated into increased survival and improved quality of life.Nevertheless,60%of patients are permanently reliant on parenteral nutrition.Encouraging results with new drugs such as teduglutide have added a new dimension to CIF therapy.The outcomes of patients with CIF could be greatly improved by more effective prevention,understanding,and treatment.In complex cases,the care of patients with CIF requires a multidisciplinary approach involving not only physicians but also dietitians and nurses to provide optimal intestinal rehabilitation,nutritional support,and an improved quality of life.Here,we summarize current literature on CIF and short bowel syndrome,encompassing epidemiology,pathophysiology,and advances in surgical and medical management,and elucidate advances in the understanding and therapy of CIF-related complications such as catheter-related bloodstream infections and intestinal failure-associated liver disease.展开更多
TO THE EDITORWe read with great interest the article by Ding LA and LiJS, which aimed to review the current knowledge on the physiology of normal intestinal barrier function and highlight the role of intestinal failur...TO THE EDITORWe read with great interest the article by Ding LA and LiJS, which aimed to review the current knowledge on the physiology of normal intestinal barrier function and highlight the role of intestinal failure after various injurious insults in the development of septic complications or multiple organ failure with subsequent rapid clinical deterioration or even death.展开更多
Short bowel syndrome(SBS)with intestinal failure(IF)is a rare but severe complication of Crohn’s disease(CD),which is the most frequent benign condition that leads to SBS after repeated surgical resections,even in th...Short bowel syndrome(SBS)with intestinal failure(IF)is a rare but severe complication of Crohn’s disease(CD),which is the most frequent benign condition that leads to SBS after repeated surgical resections,even in the era of biologics and small molecules.Glucagon-like peptide-2 analogues have been deeply studied recently for the treatment of SBS-IF.These drugs have a significant intestinotrophic effect and the potential to reduce the chronic dependence of SBSIF patients on parenteral support or nutrition.Teduglutide has been approved for the treatment of SBS-IF,and apraglutide is currently in clinical development.The use of these drugs was examined with a focus on their use in CD patients.展开更多
Inflammatory bowel disease and Crohn’s disease in particular, is a common cause of intestinal failure. Current therapeutic options include home parenteral nutrition and intestinal transplantation. For most...Inflammatory bowel disease and Crohn’s disease in particular, is a common cause of intestinal failure. Current therapeutic options include home parenteral nutrition and intestinal transplantation. For most patients, home intravenous therapy including parenteral nutrition, with a good probability of long-term survival, is the favoured choice. However, in selected patients, with specific features that may shorten survival or complicate home parenteral nutrition, intestinal transplantation presents a viable alternative. We present survival, complications, quality of life and economic considerations that currently influence individualised decision-making between home parenteral nutrition and intestinal transplantation.展开更多
To investigate the fatty acid-based functional lipidomics of patients on long-term home parenteral nutrition receiving different intravenous lipid emulsions.METHODSA cross-sectional comparative study was carried out o...To investigate the fatty acid-based functional lipidomics of patients on long-term home parenteral nutrition receiving different intravenous lipid emulsions.METHODSA cross-sectional comparative study was carried out on 3 groups of adults on home parenteral nutrition(HPN),receiving an HPN admixture containing an olive-soybean oil-based intravenous lipid emulsion(IVLE)(OO-IVLE;n=15),a soybean-medium-chain triacylglycerol-olive-fish oil-based IVLE(SMOF-IVLE;n=8)or HPN without IVLE(No-IVLE;n=8)and 42 healthy controls(HCs).The inclusion criteria were:duration of HPN≥3 mo,current HPN admixtures≥2 mo and HPN infusions≥2/wk.Blood samples were drawn 4-6 h after the discontinuation of the overnight HPN infusion.The functional lipidomics panel included:the red blood cell(RBC)fatty acid(FA)profile,molecular biomarkers[membrane fluidity:saturated/monounsaturated FA ratio=saturated fatty acid(SFA)/monounsaturated fatty acid(MUFA)index;inflammatory risk:n-6/n-3 polyunsaturated fatty acid(PUFA)ratio=n-6/n-3 index;cardiovascular risk:sum of n-3 eicosapentaenoic acid(EPA)and docosahexaenoic acid(DHA)=n-3 index;free radical stress:sum of FA trans isomers=%trans index]and FA pathway enzyme activity estimate(delta-9-desaturase=D9D;delta-6-desaturase=D6D;delta-5-desaturase=D5D;elongase=ELO).Statistics were carried out using nonparametric tests.The amount of each FA was calculated as a percentage of the total FA content(relative%).RESULTSIn the OO-IVLE group,the percentage of oleic acid in the RBCs was positively correlated with the weekly load of OO-IVLE(r=0.540,P=0.043).In the SMOF-IVLE cohort,the RBC membrane EPA and DHA were positively correlated with the daily amount of SMOF-IVLE(r=0.751,P=0.044)and the number of HPN infusions per week(r=0.753;P=0.046),respectively.The SMOF-IVLE group showed the highest EPA and DHA and the lowest arachidonic acid percentages(P<0.001).The RBC membrane linoleic acid content was lower,and oleic and vaccenic acids were higher in all the HPN groups in comparison to the HCs.Vaccenic acid was positively correlated with the weekly HPN load of glucose in both the OO-IVLE(r=0.716;P=0.007)and the SMOF-IVLE(r=0.732;P=0.053)groups.The estimated activity of D9D was higher in all the HPN groups than in the HCs(P<0.001).The estimated activity of D5D was lower in the SMOF-IVLE group than in the HCs(P=0.013).The SFA/MUFA ratio was lower in all the HPN groups than in the HCs(P<0.001).The n-6/n-3 index was lower and the n-3 index was higher in the SMOF-IVLE group in comparison to the HCs and to the other HPN groups(P<0.001).The%trans index did not differ among the four groups.CONCLUSIONThe FA profile of IVLEs significantly influenced the cell membrane functional lipidomics.The amount of glucose in the HPN may play a relevant role,mediated by the insulin regulation of the FA pathway enzyme activities.展开更多
Clinical-nutritional autonomy is the ultimate goal of patients with intestinal failure(IF).Traditionally,patients with IF have been relegated to lifelong parenteral nutrition(PN)once surgical and medical rehabilitatio...Clinical-nutritional autonomy is the ultimate goal of patients with intestinal failure(IF).Traditionally,patients with IF have been relegated to lifelong parenteral nutrition(PN)once surgical and medical rehabilitation attempts at intestinal adaptation have failed.Over the past two decades,however,outcome improvements in intestinal transplantation have added another dimension to the therapeutic armamentarium in the field of gut rehabilitation.This has become possible through relentless efforts in the standardization of surgical techniques,advancements in immunosuppressive therapies and induction protocols and improvement in postoperative patient care.Four types of intestinal transplants include isolated small bowel transplant,liver-small bowel transplant,multivisceral transplant and modified multivisceral transplant.Current guidelines restrict intestinal transplantation to patients who have had significant complications from PN including liver failure and repeated infections.From an experimental stage to the currently established therapeutic modality for patients with advanced IF,outcome improvements have also been possible due to the introduction of tacrolimus in the early 1990s.Studies have shown that intestinal transplant is cost-effective within 1–3 years of graft survival compared with PN.Improved survival and quality of life as well as resumption of an oral diet should enable intestinal transplantation to be an important option for patients with IF in addition to continued rehabilitation.Future research should focus on detecting biomarkers of early rejection,enhanced immunosuppression protocols,improved postoperative care and early referral to transplant centers.展开更多
Intestinal failure is characterized by loss of enteral function to absorb necessary nutrients and water to sustain life.Parenteral nutrition(PN)is a lifesaving therapeutic modality for patients with intestinal failure...Intestinal failure is characterized by loss of enteral function to absorb necessary nutrients and water to sustain life.Parenteral nutrition(PN)is a lifesaving therapeutic modality for patients with intestinal failure.Lifelong PN is also needed for patients who have short bowel syndrome due to extensive resection or a dysmotility disorder with malabsorption.However,prolonged PN is associated with short-term and long-term complications.Parenteral nutrition-associated liver disease(PNALD)is one of the long-termcomplications associated with the use of an intravenous lipid emulsion to prevent essential fatty acid deficiency in these patients.PNALD affects 30–60%of the adult population on long-term PN.Further,PNALD is one of the indications for isolated liver or combined liver and intestinal transplantation.There is no consensus on how to manage PNALD,but fish oil-based lipid emulsion(FOBLE)has been suggested to play an important role both in its prevention and reversal.There is significant improvement in liver function in those who received FOBLE as lipid supplement compared with those who received soy-based lipid emulsion.Studies have also demonstrated that FOBLE reverses hepatic steatosis and reduces markers of inflammation in patients on long-term PN.Future prospective studies with larger sample sizes are needed to further strengthen the positive role of FOBLE in PNALD.展开更多
Background and Objectives:Home parenteral nutrition(HPN)is a life sustaining therapy for patients with chronic intestinal failure.Reported outcomes for Asian HPN patients are scarce.We aim to review the clinical outco...Background and Objectives:Home parenteral nutrition(HPN)is a life sustaining therapy for patients with chronic intestinal failure.Reported outcomes for Asian HPN patients are scarce.We aim to review the clinical outcomes of adult and paediatric HPN patients in our cohort which caters for 95%of Singaporean HPN patients.Methods and Study Design:This is a retrospective review of HPN patients from an adult(2002-2017)and paediatric cohort(2011-2017)from the largest tertiary PN centres in Singapore.Patient demographics and clinical outcomes were reviewed.Results:There were 41 adult and 8 paediatric HPN patients.Mean age was 53.0(±15.1)(adults)and 8(±1.8)years-old(paediatrics).Mean duration of HPN was 2.6(±3.5)and 3.5(±2.5)years.Leading indications for adult HPN were short bowel syndrome(SBS)(n=19,46.3%),mechanical obstruction(n=9,22.0%),and gastrointestinal dysmotility disorders(GID)(n=5,12.2%).Thirteen adult(31.7%)patients had underlying malignancy,with seven(17.3%)receiving palliative HPN.Indications for HPN amongst paediatric patients was GID(n=5,62.5%)and SBS(n=3,37.5%).Central line-associated bloodstream infection(CLABSI)/1000catheter-days was 1.0(±2.1)and 1.8(±1.3).Catheter associated venous thrombosis(CAVT)/1000catheter-days was 0.1(±0.4)and 0.7(±0.8).Biochemical Intestinal Failure Associated Liver Disease(IFALD)was found in 21.9% and 87.5%.For adults,median overall survival was 90-months(4.3,175.7,95%CI),with actuarial survival of 70.7%(1-year)and 39.0%(5-years).Median survival for adult patients with malignancy was 6-months(4.2,7.7,95%CI),actuarial survival of 85.7%(3-months)and 30.7%(1-year).One adult patient died from PN related complications.No paediatric deaths were noted.Conclusions:Whilst patient numbers were modest,we report comparable complication and survival rates to other international centres in both our adult and paediatric cohorts.展开更多
文摘BACKGROUND Post-pancreaticoduodenectomy(PD)intestinal failure(IF)is rare and associated with poor outcomes.To our knowledge,the role of intestinal transplantation(ITx)as a rescue treatment for this complication has never been reported.CASE SUMMARY A 42-year-old female with a benign neurilemmoma of the duodenum underwent PD.Her superior mesenteric vein(SMV)was injured during surgery and required reconstruction.She experienced SMV thrombosis and bowel gangrene requiring massive bowel resection.Consequently,she developed short gut syndrome and an enterocutaneous fistula,leading to prolonged hospitalization for wound care and total parenteral nutrition(TPN)support.She was referred to our hospital for ITx evaluation.Upon arrival,she had cholestasis due to IF-associated liver disease.After gastrointestinal(GI)reconstruction to restore GI continuity,she was eligible for multi-visceral transplantation(MVTx).The anticipated allograft included the stomach,small intestine,liver,pancreas,and duodenum.She found a suitable donor after two years of waiting.The MVTx procedure was straightforward with signs of immediate function.Enteral feeding was initiated on postoperative day(POD)7.TPN weaning was achieved on POD 28,and the patient was discharged on POD 69.Two years post-MVTx,she is healthy with excellent graft function.To our knowledge,this is the first case report on MVTx as the treatment for fatal post-PD complications and also the first reported case of ITx in Southeast Asia.CONCLUSION Post-PD IF is rare and lethal.Intestinal and MVTx might be a rescue treatment for IF after GI surgery in eligible patients.
文摘The most common cause of intestinal failure is short bowel syndrome (SBS), occurring as a result of a small functional intestine length, usually less than 200 cm, leading to intestinal malabsorption. A 59-year-old female with a past medical history of Crohns disease status post total colectomy with ileostomy over 20 years ago came to the hospital due to progressive weakness. Despite medical management, the patient had high ileostomy output, leading to electrolyte disbalance, metabolic acidosis, dehydration, and progressive kidney decline. Due to the high dependence on continuous fluid supplementation, it was decided to place a port for parenteral hydration to maintain fluid replacements and homeostasis after discharge. Prompt initiation of parenteral fluid replacement and close follow-up on patients with ileostomy and intestinal failure is strongly recommended to avoid complications and prevent intestinal, liver, or kidney transplants.
文摘Chronic intestinal failure(CIF)is a rare but feared complication of Crohn’s disease.Depending on the remaining length of the small intestine,the affected intestinal segment,and the residual bowel function,CIF can result in a wide spectrum of symptoms,from single micronutrient malabsorption to complete intestinal failure.Management of CIF has improved significantly in recent years.Advances in home-based parenteral nutrition,in particular,have translated into increased survival and improved quality of life.Nevertheless,60%of patients are permanently reliant on parenteral nutrition.Encouraging results with new drugs such as teduglutide have added a new dimension to CIF therapy.The outcomes of patients with CIF could be greatly improved by more effective prevention,understanding,and treatment.In complex cases,the care of patients with CIF requires a multidisciplinary approach involving not only physicians but also dietitians and nurses to provide optimal intestinal rehabilitation,nutritional support,and an improved quality of life.Here,we summarize current literature on CIF and short bowel syndrome,encompassing epidemiology,pathophysiology,and advances in surgical and medical management,and elucidate advances in the understanding and therapy of CIF-related complications such as catheter-related bloodstream infections and intestinal failure-associated liver disease.
文摘TO THE EDITORWe read with great interest the article by Ding LA and LiJS, which aimed to review the current knowledge on the physiology of normal intestinal barrier function and highlight the role of intestinal failure after various injurious insults in the development of septic complications or multiple organ failure with subsequent rapid clinical deterioration or even death.
文摘Short bowel syndrome(SBS)with intestinal failure(IF)is a rare but severe complication of Crohn’s disease(CD),which is the most frequent benign condition that leads to SBS after repeated surgical resections,even in the era of biologics and small molecules.Glucagon-like peptide-2 analogues have been deeply studied recently for the treatment of SBS-IF.These drugs have a significant intestinotrophic effect and the potential to reduce the chronic dependence of SBSIF patients on parenteral support or nutrition.Teduglutide has been approved for the treatment of SBS-IF,and apraglutide is currently in clinical development.The use of these drugs was examined with a focus on their use in CD patients.
文摘Inflammatory bowel disease and Crohn’s disease in particular, is a common cause of intestinal failure. Current therapeutic options include home parenteral nutrition and intestinal transplantation. For most patients, home intravenous therapy including parenteral nutrition, with a good probability of long-term survival, is the favoured choice. However, in selected patients, with specific features that may shorten survival or complicate home parenteral nutrition, intestinal transplantation presents a viable alternative. We present survival, complications, quality of life and economic considerations that currently influence individualised decision-making between home parenteral nutrition and intestinal transplantation.
文摘To investigate the fatty acid-based functional lipidomics of patients on long-term home parenteral nutrition receiving different intravenous lipid emulsions.METHODSA cross-sectional comparative study was carried out on 3 groups of adults on home parenteral nutrition(HPN),receiving an HPN admixture containing an olive-soybean oil-based intravenous lipid emulsion(IVLE)(OO-IVLE;n=15),a soybean-medium-chain triacylglycerol-olive-fish oil-based IVLE(SMOF-IVLE;n=8)or HPN without IVLE(No-IVLE;n=8)and 42 healthy controls(HCs).The inclusion criteria were:duration of HPN≥3 mo,current HPN admixtures≥2 mo and HPN infusions≥2/wk.Blood samples were drawn 4-6 h after the discontinuation of the overnight HPN infusion.The functional lipidomics panel included:the red blood cell(RBC)fatty acid(FA)profile,molecular biomarkers[membrane fluidity:saturated/monounsaturated FA ratio=saturated fatty acid(SFA)/monounsaturated fatty acid(MUFA)index;inflammatory risk:n-6/n-3 polyunsaturated fatty acid(PUFA)ratio=n-6/n-3 index;cardiovascular risk:sum of n-3 eicosapentaenoic acid(EPA)and docosahexaenoic acid(DHA)=n-3 index;free radical stress:sum of FA trans isomers=%trans index]and FA pathway enzyme activity estimate(delta-9-desaturase=D9D;delta-6-desaturase=D6D;delta-5-desaturase=D5D;elongase=ELO).Statistics were carried out using nonparametric tests.The amount of each FA was calculated as a percentage of the total FA content(relative%).RESULTSIn the OO-IVLE group,the percentage of oleic acid in the RBCs was positively correlated with the weekly load of OO-IVLE(r=0.540,P=0.043).In the SMOF-IVLE cohort,the RBC membrane EPA and DHA were positively correlated with the daily amount of SMOF-IVLE(r=0.751,P=0.044)and the number of HPN infusions per week(r=0.753;P=0.046),respectively.The SMOF-IVLE group showed the highest EPA and DHA and the lowest arachidonic acid percentages(P<0.001).The RBC membrane linoleic acid content was lower,and oleic and vaccenic acids were higher in all the HPN groups in comparison to the HCs.Vaccenic acid was positively correlated with the weekly HPN load of glucose in both the OO-IVLE(r=0.716;P=0.007)and the SMOF-IVLE(r=0.732;P=0.053)groups.The estimated activity of D9D was higher in all the HPN groups than in the HCs(P<0.001).The estimated activity of D5D was lower in the SMOF-IVLE group than in the HCs(P=0.013).The SFA/MUFA ratio was lower in all the HPN groups than in the HCs(P<0.001).The n-6/n-3 index was lower and the n-3 index was higher in the SMOF-IVLE group in comparison to the HCs and to the other HPN groups(P<0.001).The%trans index did not differ among the four groups.CONCLUSIONThe FA profile of IVLEs significantly influenced the cell membrane functional lipidomics.The amount of glucose in the HPN may play a relevant role,mediated by the insulin regulation of the FA pathway enzyme activities.
文摘Clinical-nutritional autonomy is the ultimate goal of patients with intestinal failure(IF).Traditionally,patients with IF have been relegated to lifelong parenteral nutrition(PN)once surgical and medical rehabilitation attempts at intestinal adaptation have failed.Over the past two decades,however,outcome improvements in intestinal transplantation have added another dimension to the therapeutic armamentarium in the field of gut rehabilitation.This has become possible through relentless efforts in the standardization of surgical techniques,advancements in immunosuppressive therapies and induction protocols and improvement in postoperative patient care.Four types of intestinal transplants include isolated small bowel transplant,liver-small bowel transplant,multivisceral transplant and modified multivisceral transplant.Current guidelines restrict intestinal transplantation to patients who have had significant complications from PN including liver failure and repeated infections.From an experimental stage to the currently established therapeutic modality for patients with advanced IF,outcome improvements have also been possible due to the introduction of tacrolimus in the early 1990s.Studies have shown that intestinal transplant is cost-effective within 1–3 years of graft survival compared with PN.Improved survival and quality of life as well as resumption of an oral diet should enable intestinal transplantation to be an important option for patients with IF in addition to continued rehabilitation.Future research should focus on detecting biomarkers of early rejection,enhanced immunosuppression protocols,improved postoperative care and early referral to transplant centers.
文摘Intestinal failure is characterized by loss of enteral function to absorb necessary nutrients and water to sustain life.Parenteral nutrition(PN)is a lifesaving therapeutic modality for patients with intestinal failure.Lifelong PN is also needed for patients who have short bowel syndrome due to extensive resection or a dysmotility disorder with malabsorption.However,prolonged PN is associated with short-term and long-term complications.Parenteral nutrition-associated liver disease(PNALD)is one of the long-termcomplications associated with the use of an intravenous lipid emulsion to prevent essential fatty acid deficiency in these patients.PNALD affects 30–60%of the adult population on long-term PN.Further,PNALD is one of the indications for isolated liver or combined liver and intestinal transplantation.There is no consensus on how to manage PNALD,but fish oil-based lipid emulsion(FOBLE)has been suggested to play an important role both in its prevention and reversal.There is significant improvement in liver function in those who received FOBLE as lipid supplement compared with those who received soy-based lipid emulsion.Studies have also demonstrated that FOBLE reverses hepatic steatosis and reduces markers of inflammation in patients on long-term PN.Future prospective studies with larger sample sizes are needed to further strengthen the positive role of FOBLE in PNALD.
文摘Background and Objectives:Home parenteral nutrition(HPN)is a life sustaining therapy for patients with chronic intestinal failure.Reported outcomes for Asian HPN patients are scarce.We aim to review the clinical outcomes of adult and paediatric HPN patients in our cohort which caters for 95%of Singaporean HPN patients.Methods and Study Design:This is a retrospective review of HPN patients from an adult(2002-2017)and paediatric cohort(2011-2017)from the largest tertiary PN centres in Singapore.Patient demographics and clinical outcomes were reviewed.Results:There were 41 adult and 8 paediatric HPN patients.Mean age was 53.0(±15.1)(adults)and 8(±1.8)years-old(paediatrics).Mean duration of HPN was 2.6(±3.5)and 3.5(±2.5)years.Leading indications for adult HPN were short bowel syndrome(SBS)(n=19,46.3%),mechanical obstruction(n=9,22.0%),and gastrointestinal dysmotility disorders(GID)(n=5,12.2%).Thirteen adult(31.7%)patients had underlying malignancy,with seven(17.3%)receiving palliative HPN.Indications for HPN amongst paediatric patients was GID(n=5,62.5%)and SBS(n=3,37.5%).Central line-associated bloodstream infection(CLABSI)/1000catheter-days was 1.0(±2.1)and 1.8(±1.3).Catheter associated venous thrombosis(CAVT)/1000catheter-days was 0.1(±0.4)and 0.7(±0.8).Biochemical Intestinal Failure Associated Liver Disease(IFALD)was found in 21.9% and 87.5%.For adults,median overall survival was 90-months(4.3,175.7,95%CI),with actuarial survival of 70.7%(1-year)and 39.0%(5-years).Median survival for adult patients with malignancy was 6-months(4.2,7.7,95%CI),actuarial survival of 85.7%(3-months)and 30.7%(1-year).One adult patient died from PN related complications.No paediatric deaths were noted.Conclusions:Whilst patient numbers were modest,we report comparable complication and survival rates to other international centres in both our adult and paediatric cohorts.
