Long-term Home Parenteral Nutrition Research Articles

Avoiding the use of long-term parenteral support in patients without intestinal failure: A position paper from the European Society of Clinical Nutrition & Metabolism, the European Society of Neurogastroenterology and Motility and the Rome Foundation for Disorders of Gut-Brain Interaction.

The role of long-term parenteral support in patients with underlying benign conditions who do not have intestinal failure (IF) is contentious, not least since there are clear benefits in utilising the oral or enteral route for nutritional support. Furthermore, the risks of long-term home parenteral nutrition (HPN) are significant, with significant impacts on morbidity and mortality. There has, however, been a recent upsurge of the use of HPN in patients with conditions such as gastro-intestinal neuromuscular disorders, opioid bowel dysfunction, disorders of gut-brain interaction and possibly eating disorders, who do not have IF. As a result, the European Society of Clinical Nutrition and Metabolism (ESPEN), the European Society of Neuro-gastroenterology and Motility (ESNM) and the Rome Foundation for Disorders of Gut Brain Interaction felt that a position statement is required to clarify - and hopefully reduce the potential for harm associated with - the use of long-term parenteral support in patients without IF. Consensus opinion is that HPN should not be prescribed for patients without IF, where the oral and/or enteral route can be utilised. On the rare occasions that PN commencement is required to treat life-threatening malnutrition in conditions such as those listed above, it should only be prescribed for a time-limited period to achieve nutritional safety, while the wider multi-disciplinary team focus on more appropriate biopsychosocial holistic and rehabilitative approaches to manage the patient's primary underlying condition.

Association between chronic intestinal failure etiology and eGFR trajectory in adults receiving home parenteral nutrition: A retrospective longitudinal cohort study.

Patients with chronic intestinal failure (CIF) are at increased risk of developing renal impairment. The aim of this study was to evaluate the occurrence of chronic kidney disease (CKD) in patients dependent on home parenteral nutrition (HPN) and assess risk factors for renal impairment, including patients with all mechanisms of CIF. This was a cohort study of patients initiated on HPN between March 1, 2015, and March 1, 2020, at a national UK IF Reference Centre. Patients were followed from their first discharge with HPN until HPN cessation or the end of follow-up on December 31, 2021. There were 357 patients included in the analysis. Median follow-up time was 4.7 years. At baseline, >40% of patients had renal impairment, with 15.4% fulfilling the criteria for CKD. Mean estimated glomerular filtration rate (eGFR) decreased significantly during the first year after initiation of HPN from 93.32 ml/min/1.73 m2 to 86.30 ml/min/1.73 m2 at the first year of follow-up (P = 0.002), with sequential stabilization of renal function. Increased age at HPN initiation and renal impairment at baseline were associated with decreased eGFR. By the end of follow-up, 6.7% patients developed renal calculi and 26.1% fulfilled the criteria for CKD. This is the largest study of renal function in patients receiving long-term HPN. After the first year following HPN initiation, the rate of decline in eGFR was similar to that expected in the general population. These findings should reassure patients and clinicians that close monitoring of renal function can lead to good outcomes.

Parenteral nutrition at home/long-term parenteral nutrition.

Some diseases require that patients receive parenteral nutrition (PN) over a prolonged time period. Long-term administration of PN can further complicate an already complex therapy, posing additional risk of potential complications. This article is based on presentations and discussions held at the International Safety and Quality of PN Summit, providing insights into aspects of home PN (HPN) and examples of good HPN practice. One critical step in the HPN process is when patients transition from a hospital to a home setting, and vice versa. Generally, electronic PN ordering is not feasible in an HPN setting, leading to potential difficulties in communication and coordination. HPN requires that patients (or their home caregivers) administer PN, and thus their education and competency are crucial. Likewise, the choice of PN formulation is of great importance. For example, using more modern intravenous lipid emulsions containing medium-chain triglycerides, olive oil, and/or fish oil can provide benefits in terms of liver function during long-term HPN. Internationally, there are wide variations in delivery of HPN, with compounded PN dominating in some countries while others make greater use of market-authorized multichamber bags (MCBs). Patient-related factors, institutional considerations, and the availability of different MCB formulations, are also contributing factors guiding formulation and delivery system preferences. Education and communication remain key components of a successful HPN process. The information shared here may help to motivate efforts to improve HPN processes and to consider the often-differing perspectives of patients and their healthcare professionals.

The vitamin and trace element status of intestinal failure patients receiving long-term home parenteral nutrition and home parenteral fluids

The vitamin and trace element status of intestinal failure patients receiving long-term home parenteral nutrition and home parenteral fluids

Avoiding the use of long-term parenteral support in patients without intestinal failure: A position paper from the European Society of Clinical Nutrition & Metabolism, the European Society of Neurogastroenterology and Motility and the Rome Foundation for Disorders of Gut–Brain Interaction

The role of long-term parenteral support in patients with underlying benign conditions who do not have intestinal failure (IF) is contentious, not least since there are clear benefits in utilising the oral or enteral route for nutritional support. Furthermore, the risks of long-term home parenteral nutrition (HPN) are significant, with significant impacts on morbidity and mortality. There has, however, been a recent upsurge of the use of HPN in patients with conditions such as gastro-intestinal neuromuscular disorders, opioid bowel dysfunction, disorders of gut-brain interaction and possibly eating disorders, who do not have IF. As a result, the European Society of Clinical Nutrition and Metabolism (ESPEN), the European Society of Neuro-gastroenterology and Motility (ESNM) and the Rome Foundation for Disorders of Gut Brain Interaction felt that a position statement is required to clarify - and hopefully reduce the potential for harm associated with – the use of long-term parenteral support in patients without IF.Consensus opinion is that HPN should not be prescribed for patients without IF, where the oral and/or enteral route can be utilised. On the rare occasions that PN commencement is required to treat life-threatening malnutrition in conditions such as those listed above, it should only be prescribed for a time-limited period to achieve nutritional safety, while the wider multi-disciplinary team focus on more appropriate biopsychosocial holistic and rehabilitative approaches to manage the patient’s primary underlying condition.

Catheter salvage or removal in catheter-related bloodstream infections with Staphylococcus aureus in children with chronic intestinal failure receiving home parenteral nutrition and the use of prophylactic taurolidine catheter lock solution: A descriptive cohort study.

Children with chronic IF require long-term home parenteral nutrition (HPN), administered through a central venous catheter. Catheter-related bloodstream infection (CRBSI) with Staphylococcus aureus is known to be a serious infection with a high mortality rate and risk of complications. A standardized protocol on the management of S aureus CRBSIs in children receiving HPN is lacking. The aim of this study is to evaluate the effectiveness and safety of the current management in an HPN expertise center in the Netherlands. We performed a retrospective descriptive cohort study between 2013 and 2022 on children 0-18 years of age with chronic IF requiring long-term HPN. Our primary outcomes were the incidence of S aureus CRBSI per 1000 catheter days, catheter salvage attempt rate, and successful catheter salvage rate. Our secondary outcomes included complications and mortality. A total of 74 patients (39 male; 53%) were included, covering 327.8 catheter years. Twenty-eight patients (38%) had a total of 52 S aureus CRBSIs, with an incidence rate of 0.4 per 1000 catheter days. The catheter salvage attempt rate was 44% (23/52). The successful catheter salvage rate was 100%. No relapse occurred, and no removal was needed after catheter salvage. All complications that occurred were already present at admission before the decision to remove the catheter or not. No patients died because of an S aureus CRBSI. Catheter salvage in S aureus CRBSIs in children receiving HPN can be attempted after careful consideration by a multidisciplinary team in an HPN expertise center.

Descriptive Analysis of Renal Function and Potential Renal Acid Load in Adult Patients with Long-term Home Parenteral Nutrition Support: A Retrospective Study

Descriptive Analysis of Renal Function and Potential Renal Acid Load in Adult Patients with Long-term Home Parenteral Nutrition Support: A Retrospective Study

Risk factors for catheter-related bloodstream infections associated with home parental nutrition in children with intestinal failure: A prospective cohort study

Catheter-related bloodstream infection (CRBSI) is the most common, potentially life-threatening complication of long-term parenteral nutrition (PN). We prospectively assessed the incidence and risk factors for CRBSI in children receiving long-term home PN (HPN) for intestinal failure (IF) in a single IF rehabilitation center. Data regarding episodes and potential risk factors for CRBSI in children on HPN were prospectively recorded. Forty-one of 75 children were diagnosed with CRBSI. The overall CRBSI rate was 1.61 per 1000 catheter days. The indications for HPN were gastrointestinal motility disorders in 35%, short bowel syndrome (SBS) in 28% graft versus host disease (GvHD) post bone marrow transplant in 17%, congenital enteropathy in 15%, and severe neurodevelopmental impairment in 5%. Gastrointestinal motility disorders had significantly higher CRBSI rate compared to other groups (p<0.0005; 2.74 in motility group vs 1.54 in GvHD group vs 0.52 in congenital enteropathies vs 0.36 in SBS group vs 0.67 in severe neurodevelopmental delay). Multivariate analysis revealed that enterocutaneous distal stoma (ileostomy or colostomy) (HR 3.35 [95% CI, 1.63-6.86]; p<0.001), age <2 years (HR 0.28 [95% CI, 0.15-0.53]; p<0.0001), male sex (HR 2.28 [95% CI, 1.51-3.43]; p<0.0001), non-use of taurolidine citrate lock (HR 2.70 [95% CI, 1.72-4.11]; p<0.0001) and gastrointestinal motility disorder (HR 3.02 [95% CI, 1.81-4.91]; p<0.001) were independent risk factors for developing CRBSI. Extra care in managing PN connections and disconnections should be taken in children with an underlying gastrointestinal motility disorder, distal enterocutaneous stoma, male sex and those aged <2 years since they are at a significantly higher risk of CRBSI. Early introduction of taurolidine lock should be considered.

Central venous catheter (CVC) salvage in case of central line-associated bloodstream infection (CLABSI): A monocentric prospective study in patients on long-term home parenteral nutrition (HPN)

CLABSI is a major complication in HPN and frequently leads to central venous catheter (CVC) removal. We developed a salvaging attitude in long term HPN patients due to the necessity of venous preservation. The main objective of this study is to determine the prognosis of CLABSI. We followed-up for three months, in an approved HPN centre, a cohort of 250 adult patients receiving HPN with CLABSI from 2018 to 2020. CLABSI was defined by a blood culture growth differential [peripheral blood] - [CVC blood]≥2h. A therapeutic approach to conserve CVC was established according to the department's protocol. The primary endpoint was conservation of CVC with negative CVC and peripheral blood cultures at 3 months without complications. Data from 30 CLABSIs were collected for 22 HPN patients. The incidence rate of CLABSIs was 0.28 infections/1000 catheter days. Sixteen CVCs were removed immediately, with causes due to the type of germ (staphylococcus aureus: n=6, candida parapsilosis: n=4, klebsiella: n=2), chronic colonization (n=4) or initial complications (n=4). Among the 14 non-removed CVC, 11 were maintained at 3 months with blood cultures on CVC and peripheral negative for 9 (80%) of them. 3 CVC were removed during the 3 months follow-up (non-CVC-related sepsis n=2, and resistant pseudomonas aeruginosa n=1). The incidence rate of CLABSIs in an expert HPN centre remains low. In case of CLABSIs, according to specific protocol, approximately 50% of CVC were removed immediately (essentially due to bacteriological characteristics). In case of CLABSIs and without initial complication, 80% of CVCs can be maintained at 3 months. These results justify a conservative attitude according to standardized protocol.

Épidémiologie de la nutrition parentérale à domicile chez l’adulte en France

ObjectiveFrench epidemiological data regarding home parenteral nutrition (HPN) are not well known and give rise to imprecise estimates. This study targets the adult population to obtain accurate data especially by trying to determine the pathologies associated with the HPN. MethodsA retrospective and observational study covering the year 2017 (from 01/01 to 31/12/2017) has been carried out, with the data of the adult population aged over 15 years, by defining three age groups: 15–39 years, 40–64 years, 65 years and over. The databases of the health insurance allowed to obtain on the one hand, the number of patients who started HPN and on the other hand, the number of patients followed in HPN. The target population (health insurance beneficiary population) being known, these data allowed to calculate incidence and prevalence, respectively. The data were also crossed with the thirty long-term diseases file of health national insurance (ALD 30), allowing patients to be classified into six categories: cancer, neurology, chronic intestinal inflammatory diseases, organ deficiency, others pathologies, and undetermined pathologies. ResultsData from 92.1% of adult national population were obtained, totaling 50.8 millions adults. The average incidence and prevalence of HPN were estimated at 20.8/100,000 inhabitants/yr and 26.0/100,000 inhabitants, respectively. Prevalence of long-term HPN (>12 weeks) was 6.0/100,000 inhabitants. The main pathology was cancer. Inflammatory bowel diseases were the second cause of HPN in the age range 15–39 years. ConclusionThis is the first national study on the epidemiology of HPN in adults in France, based on data from health insurance. The projection of these figures to the entire French adult population leads to an estimate of around 14,300 patients per year benefiting from national health insurance for HPN, mainly for cancer diseases.

Epidemiology, survival, costs, and quality of life in adults with short bowel syndrome.

Short bowel syndrome (SBS) is a rare disorder with known physical, psychosocial, and economic burdens and significant morbidity and mortality. Many individuals with SBS require long-term home parenteral nutrition (HPN). The incidence and prevalence of SBS is difficult to determine because it is often based on HPN usage and may not account for those who receive intravenous fluids or achieve enteral autonomy. The most common etiologies associated with SBS are Crohn's disease and mesenteric ischemia. Intestinal anatomy and remnant bowel length are prognostic for HPN dependency, and enteral autonomy confers a survival advantage. Health economic data confirm that PN-related costs are higher for hospitalizations than at home; yet significant healthcare resource utilization is necessary for successful HPN, and patients and families report substantial financial distress that impacts quality of life (QOL). An important advancement in QOL measurement is the validation of HPN- and SBS-specific QOL questionnaires. In addition to the known factors negatively impacting QOL, such as diarrhea, pain, nocturia, fatigue, depression, and narcotic dependency, research has shown that the volume and number of PN infusions per week is associated with QOL. Although traditional QOL measurements describe how underlying disease and therapy influence life, they do not assess how symptoms and functional limitations affect the QOL of patients and caregivers. Patient-centered measures and conversation focused on psychosocial issues helps patients with SBS and HPN dependency better cope with their disease and treatment. This article presents a brief overview of SBS, including epidemiology, survival, costs, and QOL.

COVID-19 infection in patients on long-term home parenteral nutrition for chronic intestinal failure

To investigate the incidence and the severity of COVID-19 infection in patients enrolled in the database for home parenteral nutrition (HPN) for chronic intestinal failure (CIF) of the European Society for Clinical Nutrition and Metabolism (ESPEN). Period of observation: March 1st, 2020 March 1st, 2021. patients included in the database since 2015 and still receiving HPN on March 1st, 2020 as well as new patients included in the database during the period of observation. Data related to the previous 12 months and recorded on March 1st 2021: 1) occurrence of COVID-19 infection since the beginning of the pandemic (yes, no, unknown); 2) infection severity (asymptomatic; mild, no-hospitalization; moderate, hospitalization no-ICU; severe, hospitalization in ICU); 3) vaccinated against COVID-19 (yes, no, unknown); 4) patient outcome on March 1st 2021: still on HPN, weaned off HPN, deceased, lost to follow up. Sixty-eight centres from 23 countries included 4680 patients. Data on COVID-19 were available for 55.1% of patients. The cumulative incidence of infection was 9.6% in the total group and ranged from 0% to 21.9% in the cohorts of individual countries. Infection severity was reported as: asymptomatic 26.7%, mild 32.0%, moderate 36.0%, severe 5.3%. Vaccination status was unknown in 62.0% of patients, non-vaccinated 25.2%, vaccinated 12.8%. Patient outcome was reported as: still on HPN 78.6%, weaned off HPN 10.6%, deceased 9.7%, lost to follow up 1.1%. A higher incidence of infection (p=0.04), greater severity of infection (p<0.001) and a lower vaccination percentage (p=0.01) were observed in deceased patients. In COVID-19 infected patients, deaths due to infection accounted for 42.8% of total deaths. In patients on HPN for CIF, the incidence of COVID-19 infection differed greatly among countries. Although the majority of cases were reported to be asymptomatic or have mild symptoms only, COVID-19 was reported to be fatal in a significant proportion of infected patients. Lack of vaccination was associated with a higher risk of death.

Long-term home parenteral nutrition in systemic sclerosis-related intestinal failure is feasible but unveils occult cardiac disease

ObjectiveThe aim of this study was to compare safety and efficacy of long-term home parenteral nutrition between patients with systemic sclerosis and intestinal failure (IF) and controls with IF from another etiology. MethodsA retrospective study was conducted in a referral center for systemic sclerosis (SSc) in Montpellier, France. Patients followed between 1985 and 2020 with SSc-related IF were included and compared with control patients with IF from another etiology. The patients included had to be treated for ≥4 wk by home parenteral nutrition (HPN). Primary outcome was occurrence of HPN-related complications. Secondary outcomes included duration of parenteral nutrition, body mass index at 12 mo, and survival. ResultsCumulative duration of HPN was 23 397 catheter days. HPN resulted in body mass index increase in both groups. There was no statistical difference regarding catheter-related bloodstream infections and thrombosis between the groups, despite use of immunosuppressive drugs and autologous hematopoietic stem cell transplantation in patients with SSc. However, the patients with SSc had significantly more HPN-related cardiac overload than the controls (P < 0.0001). Overloads occurred in SSc patients with and without cardiac disease, arguing for comprehensive hemodynamic screening in this condition. ConclusionLong-term HPN in SSc-related IF is feasible but unveils occult cardiac disease.

Management of long-term home parenteral nutrition: Historical perspective, common complications, and patient education and training.

Intestinal failure (IF) is a rare disease that requires ongoing intravenous supplementation to sustain growth and health. Advancements in parenteral nutrition (PN) and central venous access in the 1960s and 1970s transformed a life-limiting disease to a chronic one in which patients are able to administer hydration, electrolytes, micronutrients, and macronutrients in the comfort of their home. However, despite ongoing advancements in the field of home PN (HPN), complications-whether related to central venous catheters or PN itself-remain common and can be associated with significant morbidity and mortality. Central venous access can be associated with thrombosis, central line-associated bloodstream infection, or damage and can result in loss of access over time. PN can be associated with IF-associated liver disease or hyperglycemia. The key to preserving central venous access and quality of life and maintaining health for patients with chronic IF (CIF) is education focused on prevention and prompt management of CIF complications as they arise. This education typically takes place at the time of initiation of HPN, either in the hospital setting or in the patient's home. The present manuscript describes the historical progression of HPN, prevalence and characteristics of CIF, and an in-depth discussion of the most common catheter-related and PN-related complications and their management, along with a discussion of our education and training process.

Long-term home parenteral nutrition and profile of amino acids in serum and their loss in urine.

Amino acids are an essential part of parenteral nutrition. This study aimed to determine the serum profile of amino acids and their loss in urine in patients with long-term home parenteral nutrition (HPN) during 12 h of infusion in comparison with similar parameters in the remaining 12 h as well as in healthy participants. We enrolled forty-five patients with long-term HPN for 6-75 (median, 33) months. The indication for HPN was short bowel syndrome secondary to radical resection of the small intestine following complications of Crohn's disease. HPN was administered via two-chamber all-in-one bags prepared in a hospital pharmacy overnight for 12 h each day. The average dose of amino acids, carbohydrates and fats administered was 1·5, 3·4 and 0·68 g/kg per d, respectively, at an infusion rate of 0·11, 0·28 and 0·06 g/kg per h, respectively. The levels of essential amino acids in the serum of the patients were not significantly different from those in healthy individuals; however, of the non-essential amino acids, cystine and glutamine levels were lower and glycine and ornithine levels were higher in the patients (P < 0·05). Excretion of amino acids in the urine during 12 h of infusion at an infusion rate of 0·11 g/kg per h was 301 mg, while it was 104 mg during the remaining 12 h (P < 0·0001). Our patients on long-term HPN had a normal serum profile of essential amino acids. The total urinary excretion of amino acids during 12 h of infusion accounted for only 0·34 % (0·23-0·46) of the administered dose.

HPN Standard of Care and Long-Term Outcomes of CIF Pediatric Patients: Twenty-Eight Years' Experience in a Reference Center.

Background and AimsChronic intestinal failure (CIF) therapy changed significantly in recent decades, and both survival and complication rates improved over time. International guidelines claim that early referral of long-term home parenteral nutrition (HPN) patients to an expert center with specific standards of care may positively affect long-term outcomes. Herein, we retrospectively analyse the long-term outcomes of a cohort of pediatric patients with CIF followed-up since our Pediatric Intestinal Failure Unit foundation, in 1989.MethodsData of the 120 children followed up at Pediatric Intestinal Failure Unit during the last 28 years were retrospectively collected. Patients' and HPN characteristics, as well as dependence, survival, and complication rates, were described.ResultsIncidence and prevalence of CIF increased during the study period particularly due to the increase of HPN for non-digestive disease (NDD) CIF (47.5% of the study sample). Catheter-related bloodstream infection (CRBSI) rate decreased over the study period: 0.33 episodes/1,000 catheters days before 2011 and 0.19 episodes/1,000 catheters days afterwards. Only 1 patient out of 12 died because of HPN complications. The survival rate of patients with PDD was 98.4% at 1 year from the beginning of HPN, 96.5% at 2 years, and 93.8% from the fifth year onwards. Concerning the dependence rate, 70.6% of patients were still on HPN 1 year after the start of HPN, 63.7% at 2 years, 52.4% at 5 years, and 40.8% from the 9th year onwards, with no significant difference according to the underlying intestinal pathology. The survival rate of NDD patients was 91.2% at 1 year from the beginning of HPN, 87.4% at 2 years, and 81.9% from the third year onwards. For what concerns the enteral autonomy, it was regained by 56.7% 1 year after the start of HPN, 74.5% at 2 years, and 95.0% in the 5th year.ConclusionsOur data confirmed the importance of appropriate standards of care and suggest that applying a specific set of standards and protocols may further improve patients' outcomes and survival. Indeed, both primary and non-digestive diseases HPN showed good outcomes.

Outcomes of the First 54 Pediatric Patients on Long-Term Home Parenteral Nutrition from a Single Brazilian Center.

Objectives:Data on multidisciplinary programs dedicated to home parenteral nutrition (HPN) in Latin America are limited. This study describes the results of the first multidisciplinary pediatric intestinal rehabilitation program for HPN at a public tertiary hospital in Brazil.Methods:We retrospectively reviewed patients aged 0–18 years with intestinal failure (IF) who required parenteral nutrition (PN) for >60 days between January/2014 and December/2020.Results:Fifty-four patients were discharged on HPN (15 achieved enteral autonomy, 34 continued on HPN at the end of the study, 1 underwent intestinal transplantation, and 4 died). The median (IQR) age at the study endpoint of patients who achieved enteral autonomy was 14.1 (9.7–19) versus 34.7 (20.4–53.9) months in those who did not achieve enteral autonomy. Overall prevalence of catheter-related thrombosis was 66.7% and catheter-related bloodstream infection rate was 0.39/1000 catheter-days. Intestinal failure-associated liver disease (IFALD) was present in 24% of all patients; none of the patients who achieved enteral autonomy had IFALD. All patients showed significant improvement in anthropometric parameters during the HPN period. The sociodemographic characteristics of the patients’ family members were mothers less than 20 years old (7.5%), schooling time more than 10 years (55.5%), and household income between 1 and 3 times the minimum wage (64.8%). The 5-year survival rate for HPN is 90%, and 27.7% of patients achieve enteral autonomy.Conclusion:The treatment of pediatric patients with IF followed by a multidisciplinary pediatric intestinal rehabilitation program with HPN is feasible and safe in the Brazilian public health system.

Stratification of cardiovascular disease risk in adults receiving long‑term home parenteral nutrition.

Stratification of cardiovascular disease risk in adults receiving long‑term home parenteral nutrition.

Erythrocyte fatty acid membrane composition in children on long-term parenteral nutrition enriched with ω-3 fatty acids

Composite lipid emulsions containing soybean oil (30%), medium-chain triglycerides (30%), olive oil (25%), and fish oil (15%) (SMOF) are now widely used. We aimed to evaluate the tolerance, the efficiency, and the erythrocyte fatty acid (FA) profile for children on long-term home parenteral nutrition (HPN) receiving a composite fish oil-based emulsion (FOLE). At baseline, children (n=46) with severe intestinal failure highly dependent on parenteral nutrition (PN) for ≥1 y were included in the study when they had received the composite FOLE for >6 mo. Out of this baseline group, only 25 children remained highly PN-dependent (SMOF1, n=25) and could be assessed a second time, 2.4 y later (SMOF2, n=25). An independent control group ("weaned off PN" group; n=24) included children who had been weaned off PN for >2 y (median: 4 y). RBC-FA composition was established by GC-MS. Growth parameters, plasma citrulline, conjugated bilirubin, FA profiles, and the Holman ratio (20:3ω-9/20:4ω-6) were compared between groups. No difference for growth parameters, citrulline, and bilirubin was observed between the SMOF groups after 2.4 y (0.2<P<0.8). The weaned-off group did not differ from the SMOF groups for growth parameters (0.2<P<0.4) but citrulline was higher (P<0.0001) and conjugated bilirubin lower (P<0.01). The composite FOLE induced higher proportions of EPA (20:5n-3) (8.4%±2.9%) and DHA (22:6n-3) (11.7%±2.2%) than what was observed in weaned-off children (0.8%±0.4% and 6.6%±2.3%, respectively) but lower proportions of arachidonic acid (20:4n-6). However, the Holman ratio did not vary between groups (P=0.9), whereas the PUFA concentrations varied widely. Long-term use of the composite FOLE was well tolerated in HPN-dependent children. The RBC-FA profile alterations were consistent with the ω-3 PUFA-enriched composition of this emulsion without evidence of essential FA deficiency.

A Case of Allied Disorders of Hirschsprung’s Disease With Septic Pulmonary Embolism During Long-Term Home Parenteral Nutrition Using a Central Venous Port System

A Case of Allied Disorders of Hirschsprung’s Disease With Septic Pulmonary Embolism During Long-Term Home Parenteral Nutrition Using a Central Venous Port System