Hyperglycemia During Home Parenteral Nutrition Administration in Patients Without Diabetes

Background: Parenteral nutrition (PN) is a life‐sustaining therapy in appropriate clinical settings. In the hospital setting, some nondiabetic patients develop hyperglycemia and subsequently require long‐term insulin while receiving PN. Whether similar hyperglycemia is seen in the outpatient setting is unclear. Methods: We studied patients enrolled in the Mayo Clinic Home Parenteral Nutrition (HPN) program between January 1, 2010, and December 31, 2012. Patients were excluded if they had diabetes mellitus type 2 (DM2), had previously received HPN, had taken corticosteroids, or were at risk for refeeding syndrome. Results: Of 144 enrolled patients, 93 met inclusion criteria with 39 patients requiring the addition of insulin to HPN. The mean age of the insulin‐requiring group (IR) was higher than that of the non–insulin‐requiring group (NIR) (60.74 ± 13.62 years vs 48.97 ± 17.62 years, P < .001). There were 17 (44%) men in the IR group and 26 (48%) men in the NIR group. Mean blood glucose at baseline before starting the infusion was 131.82 ± 49.55 mg/dL in IR patients and 106.16 ± 59.01 mg/dL in NIR patients (P = .03). In the stepwise multivariate analysis for assessing the risk for developing hyperglycemia, HR for age was 1.020 (1.010–1.031), P < .001. Conclusions: Hyperglycemia is a common finding with the use of PN in both the hospital and ambulatory setting in patients without a previous diagnosis of DM2. Age was the most significant predictor of the requirement of insulin in the present study. When hyperglycemia is managed appropriately with insulin therapy, the long‐term complications can be minimized.     

An insulin protocol for management of hyperglycemia in patients receiving parenteral nutrition is superior to ad hoc management

Background: The authors investigated whether an insulin protocol for parenteral nutrition (PN)–induced hyperglycemia is superior to conventional management relying primarily on sliding‐scale insulin at a large county hospital. Methods: A prospective cohort study with historical controls was completed. Adult patients receiving PN were managed with a protocol that determined insulin doses based on carbohydrate delivery and capillary blood glucose (CBG) if half or more of CBG measurements in the first 24 hours after initiation of PN exceeded 140 mg/dL. Control data were obtained from records of patients who met study eligibility criteria but had been managed before implementation of the insulin protocol. Results: Mean CBG after the start of insulin therapy was 138 ± 37 mg/dL for protocol patients and 159 ± 46 mg/dL for controls (P < .0001). Proportion of CBG values in the target range of 80–140 mg/dL was 60% in the protocol group and 35% in the control group (P < .0001). Hypoglycemia, defined as CBG <80 mg/dL, occurred infrequently but more often in the protocol group (3% vs 1%, P = .012). There was no difference in total daily insulin between groups, although protocol patients received mostly scheduled insulin (93% total daily dose), whereas control patients received predominantly supplemental insulin (66% total daily dose). Conclusions: Protocol‐directed management of PN‐induced hyperglycemia is superior to ad hoc insulin dosing. Linking insulin to carbohydrate in PN leads to improved glycemic control with a low rate of hypoglycemia.     

Iron Deficiency in Long‐Term Parenteral Nutrition Therapy

Background: Iron is not routinely added to parenteral nutrition (PN) formulations in the United States because of the risk of anaphylaxis and concerns about incompatibilities. Studies have shown that iron dextran in non‐lipid‐containing PN solutions is safe. Data are limited on iron status, prevalence of iron deficiency anemia (IDA), and efficacy of intravenous iron infusion in long‐term home PN (HPN). We aimed to determine the incidence of IDA and to examine the effectiveness of parenteral iron replacement in patients receiving HPN. Methods: Medical records of patients receiving HPN at the Mayo Clinic from 1977 to 2010 were reviewed. Diagnoses, time to IDA development, and hemoglobin, ferritin, and mean corpuscular volume (MCV) values were extracted. Response of iron indices to intravenous iron replacement was investigated. Results: Of 185 patients (122 women), 60 (32.4%) were iron deficient. Five patients were iron deficient, and 18 had unknown iron status before HPN. Of 93 patients who had sufficient iron storage, 37 had IDA development after a mean of 27.2 months (range, 2–149 months) of therapy. Iron was replaced by adding maintenance iron dextran to PN or by therapeutic iron infusion. Patients with both replacement methods had significant improvement in iron status. With intravenous iron replacement, mean ferritin increased from 10.9 to 107.6 mcg/L (P < .0001); mean hemoglobin increased from 11.0 to 12.5 g/dL (P = .0001); and mean MCV increased from 84.5 to 89.0 fL (P = .007). Conclusions: Patients receiving HPN are susceptible to IDA. Iron supplementation should be addressed for patients who rely on PN.     

Prevention of Subsequent Catheter‐Related Bloodstream Infection Using Catheter Locks in High‐Risk Patients Receiving Home Parenteral Nutrition

Introduction:Catheter‐related bloodstream infection (CRBSI) is a serious complication in patients receiving home parenteral nutrition (HPN). Antibiotic lock therapy (ALT) and ethanol lock therapy (ELT) can be used to prevent CRBSI episodes in high‐risk patients. Methods: Following institutional review board approval, all patients enrolled in the Mayo Clinic HPN program from January 1, 2006, to December 31, 2013, with catheter locking were eligible to be included. Patients without research authorization and <18 years old at the initiation of HPN were excluded. Total number of infections before and after ALT or ELT were estimated in all patients. Results: A total of 63 patients were enrolled during the study period. Of 59 eligible patients, 29 (49%) were female, and 30 (51%) were male. The median duration of HPN was 3.66 (interquartile range, 0.75–8.19) years. The mean age ± SD at initiation of HPN was 49.89 ± 14.07 years. A total of 51 patients were instilled with ALT, and 8 patients were instilled with ELT during their course of HPN. A total of 313 CRBSI episodes occurred in these patients, 264 before locking and 49 after locking (P < .001). Rate of infection per 1000 catheter days was 10.97 ± 25.92 before locking and 1.09 ± 2.53 after locking (P < .001). Discussion: The major findings of the present study reveal that ALT or ELT can reduce the overall rate of infections per 1000 catheter days. ALT or ELT can be used in appropriate clinical setting for patients receiving HPN.     

Computer‐Assisted Glucose Regulation During Rapid Step‐Wise Increases of Parenteral Nutrition in Critically Ill Patients

Background: Early delivery of calories is important in critically ill patients, and the administration of parenteral nutrition (PN) is sometimes required to achieve this goal. However, PN can induce acute hyperglycemia, which is associated with adverse outcome. We hypothesized that initiation of PN using a rapid “step‐up” approach, coupled with a computerized insulin‐dosing protocol, would result in a desirable caloric intake within 24 hours without causing hyperglycemia. Methods: In our surgical intensive care unit (ICU), glucose is regulated by a nurse‐centered computerized glucose regulation program. When adequate enteral feeding was not possible, PN was initiated according to a simple step‐up rule at an infusion rate of 10 mL/h (approximately 10 kcal/h) and subsequently increased by steps of 10 mL/h every 4 hours, provided glucose was <10 mmol/L, until the target caloric intake (1 kcal/kg/h) was reached. All glucose levels and insulin doses were collected during the step‐up period and for 24 hours after achieving target feeding. Results: In all 23 consecutive patients requiring PN, mean intake was 1 kcal/kg/h within 24 hours. Of the 280 glucose samples during the 48‐hour study period, mean ± standard deviation glucose level was 7.4 ± 1.4 mmol/L. Only 4.5% of glucose measurements during the step‐up period were transiently ≥10 mmol/L. After initiating PN, the insulin requirement rose from 1.1 ± 1.5 units/h to 2.9 ± 2.5 units/h (P < .001). Conclusions: This proof of concept study shows that rapid initiation of PN using a step‐up approach coupled with computerized glucose control resulted in adequate caloric intake within 24 hours while maintaining adequate glycemic control.     

Nutrition Management of Home Parenteral Nutrition Among Patients With Enterocutaneous Fistula in the Sustain Registry

Background: Home parenteral nutrition (HPN) is a vital therapy for patients who have the diagnosis of enterocutaneous fistula (ECF), yet little is known about how these patients are managed. This research compares nutrition management of adults with ECF as the indication for HPN therapy to those with other indications. Methods: This is an analysis of data from adult HPN patients in the Sustain registry enrolled between August 2011 and February 2014 who have the diagnosis of ECF or other indication for HPN who served as the control group. Differences between the ECF and control group were assessed by t test, analysis of variance, or χ² as appropriate. Results: There were 141 HPN patients with ECF and 632 control patients. Patients with ECF were older (55 vs 50 years, P < .001), more frequently had a goal for future surgery (30% vs 15%, P = .010), had greater prevalence of overweight/obesity (33% vs 20%, P = .04), and had a lower serum albumin (2.98 ± 0.65 g/dL vs 3.16 ± 0.66 g/dL, P = .006) than controls. The diet order was more frequently nil per os (NPO) in patients with ECF (48% vs 22%, P < .001), and amino acid content of HPN was greater (111.90 ± 29.11 vs 102.06 ± 27.84, P < .001) than in controls. There were no differences in patterns of weight change by ECF or control groups, although underweight patients gained, normal‐weight patients maintained, and overweight/obese patients lost weight and serum albumin increased similarly. Conclusions: The HPN management of patients with ECF is similar to other HPN patients other than greater provision of protein, more frequent NPO status, and a goal for future surgery.     

Dietary Management of Blood Glucose in Medical Critically Ill Overweight and Obese Patients: An Open‐Label Randomized Trial

Background: Enteral nutrition (EN) increases hyperglycemia due to high carbohydrate concentrations while providing insufficient protein. The study tested whether an EN formula with very high‐protein‐ and low‐carbohydrate‐facilitated glucose control delivered higher protein concentrations within a hypocaloric protocol. Methods: This was a multicenter, randomized, open‐label clinical trial with parallel design in overweight/obese mechanically ventilated critically ill patients prescribed 1.5 g protein/kg ideal body weight/day. Patients received either an experimental very high‐protein (37%) and low‐carbohydrate (29%) or control high‐protein (25%) and conventional‐carbohydrate (45%) EN formula. Results: A prespecified interim analysis was performed after enrollment of 105 patients (52 experimental, 53 control). Protein and energy delivery for controls and experimental groups on days 1–5 were 1.2 ± 0.4 and 1.1 ± 0.3 g/kg ideal body weight/day (P = .83), and 18.2 ± 6.0 and 12.5 ± 3.7 kcals/kg ideal body weight/day (P < .0001), respectively. The combined rate of glucose events outside the range of >110 and ≤150 mg/dL were not different (P = .54, primary endpoint); thereby the trial was terminated. The mean blood glucose for the control and the experimental groups were 138 (?SD 108, +SD 177) and 126 (?SD 99, +SD 160) mg/dL (P = .004), respectively. Mean rate of glucose events >150 mg/dL decreased (Δ = ?13%, P = .015), whereas that of 80–110 mg/dL increased (Δ = 14%, P = .0007). Insulin administration decreased 10.9% (95% CI, ?22% to 0.1%; P = .048) in the experimental group relative to the controls. Glycemic events ≤80 mg/dL and rescue dextrose use were not different (P = .23 and P = .53). Conclusions: A very high‐protein and low‐carbohydrate EN formula in a hypocaloric protocol reduces hyperglycemic events and insulin requirements while increasing glycemic events between 80–110 mg/dL.     

Low‐Dose Parenteral Soybean Oil for the Prevention of Parenteral Nutrition–Associated Liver Disease in Neonates With Gastrointestinal Disorders

Background: Neonates with gastrointestinal disorders (GDs) are at high risk for parenteral nutrition–associated liver disease (PNALD). Soybean‐based intravenous lipid emulsions (S‐ILE) have been associated with PNALD. This study's objective was to determine if a lower dose compared with a higher dose of S‐ILE prevents cholestasis without compromising growth. Materials and Methods: This multicenter randomized controlled pilot study enrolled patients with GDs who were ≤5 days of age to a low dose (~1 g/kg/d) (LOW) or control dose of S‐ILE (~3 g/kg/d) (CON). The primary outcome was cholestasis (direct bilirubin [DB] >2 mg/dL) after the first 7 days of age. Secondary outcomes included growth, PN duration, and late‐onset sepsis. Results: Baseline characteristics were similar between the LOW (n = 20) and CON groups (n = 16). When the LOW group was compared with the CON group, there was no difference in cholestasis (30% vs 38%, P = .7) or secondary outcomes. However, mean ± SE DB rate of change over the first 8 weeks (0.07 ± 0.04 vs 0.3 ± 0.09 mg/dL/wk, P = .01) and entire study (0.008 ± 0.03 vs 0.2 ± 0.07 mg/dL/wk, P = .02) was lower in the LOW group compared with the CON group. Conclusion: In neonates with GDs who received a lower dose of S‐ILE, DB increased at a slower rate in comparison to neonates who received a higher dose of S‐ILE. Growth was comparable between the groups. This study demonstrates a need for a larger, randomized controlled trial comparing 2 different S‐ILE doses for cholestasis prevention in neonates at risk for PNALD.     

Incidence,Prevention, and Treatment of Parenteral Nutrition–Associated Cholestasis and Intestinal Failure–Associated Liver Disease in Infants and Children

Background: Cholestasis is a significant life‐threatening complication in children on parenteral nutrition (PN). Strategies to prevent/treat PN‐associated cholestasis (PNAC) and intestinal failure–associated liver disease (IFALD) have reached moderate success with little supporting evidence. Aims of this systematic review were (1) to determine the incidence of PNAC/IFALD in children receiving PN for ≥14 days and (2) to review the efficacy of measures to prevent/treat PNAC/IFALD. Methods: Of 4696 abstracts screened, 406 relevant articles were reviewed, and studies on children with PN ≥14 days and cholestasis (conjugated bilirubin ≥ 2 mg/dL) were included. Analyzed parameters were (1) PNAC/IFALD incidence by decade and by PN length and (2) PNAC/IFALD prevention and treatment (prospective studies). Results: Twenty‐three articles (3280 patients) showed an incidence of 28.2% and 49.8% of PNAC and IFALD, respectively, with no evident alteration over the last decades. The incidence of PNAC was directly proportional to the length of PN (from 15.7% for PN ≤1 month up to 60.9% for PN ≥2 months; P < .0001). Ten studies on PNAC met inclusion criteria. High or intermediate‐dose of oral erythromycin and aminoacid‐free PN with enteral whey protein gained significant benefits in preterm neonates (P < .05, P = .003, and P < .001, respectively). None of the studies reviewed met inclusion criteria for treatment. Conclusions: The incidence of PNAC/IFALD in children has no obvious decrease over time. PNAC is directly correlated to the length of PN. Erythromycin and aminoacid‐free PN with enteral whey protein have shown to prevent PNAC in preterm neonates. There is a lack of high‐quality prospective studies, especially on IFALD.     

Canadian home parenteral nutrition (HPN) registry: validation and patient outcomes

Background: In Canada, there are an estimated 400 home parenteral nutrition (HPN) patients. In 2006, a registry was created to gather patient outcome information. The aim of this study was to validate the registry and report on HPN patient outcomes. Methods: Several demographic, clinical parameters were collected. For the validation, paired t test and intraclass correlation coefficient (ICC) were used to assess agreement between repeat entries. For the outcome report, paired t test was used to assess changes, and survival analysis was performed using the Kaplan‐Meier method. Results are expressed as mean ± SEM. Results: On validation, there was high correlation/agreement (P < .05) for most parameters except vascular access/line sepsis, liver disease (ultrasound, biopsy, diagnoses), and hospitalizations. For the outcome report, 96 patients had their data entered at 2.24 ± 0.11 years after baseline. Over the period, there was a significant reduction in PN calories (P = .001) and proteins (P < .001). There were no significant changes in nutrition parameters and laboratory results except for lower platelet counts (P = .028), lower plasma potassium (P = .030), and a trend toward an increase in bilirubin from 19.29 ± 4.65 to 29.06 ± 8.73 µmol/L (P = .071). The QOL decreased significantly over time (P < .001) and the survival on HPN was 17.67 ± 1.89 years. Conclusions: The registry is a valid tool to assess several clinical parameters. On follow‐up, HPN patients maintain good nutrition status while PN is reduced but do have a reduced quality of life.     

Long-Term Use of Mixed-Oil Lipid Emulsion in Soybean Oil–Intolerant Home Parenteral Nutrition Patients

Background: Although home parenteral nutrition (HPN) is lifesaving for patients with chronic intestinal failure (IF), long-term use can be associated with complications such as infections, metabolic abnormalities, and IF–associated liver disease (IFALD). The key to treatment of many of these complications is prevention. Guidelines recommend avoidance of overfeeding, use of oral/enteral nutrition if possible, cyclic PN, and maintaining dose of soybean oil (SO) intravenous lipid emulsion (ILE) <1 g/kg/day as preventive strategies for IFALD. Additionally, with development of IFALD, ω-6/ω-3 polyunsaturated fatty acid ratio should be decreased in ILE. The newly available mixed-oil (MO) ILE offers such an opportunity; however, there is a paucity of long-term data available. Methods: The current study reports our long-term experience with MO ILE use in HPN patients. Results: Seventeen patients (8 female and 9 male) with an average age of 47 ± 12 years and median HPN duration of 4.6 years (1.1–32.1 years) have utilized MO ILE for >12 months after being transitioned from SO ILE because of intolerance. Use of MO ILE allowed an increase in ILE energy from 8% ± 8% to 22% ± 8% while reducing dextrose energy from 66% ± 8% to 54% ± 5%, maintaining stability in alkaline phosphatase and triglyceride levels, and achieving improvement in aspartate aminotransferase, alanine aminotransferase, total bilirubin, and α-tocopherol levels. Conclusion: In this HPN cohort with SO ILE intolerance, MO ILE was well tolerated and allowed an improvement in macronutrient composition while improving some liver parameters over a 12-month period.     

Plasma Aluminum Concentrations in Pediatric Patients Receiving Long‐Term Parenteral Nutrition

Background: Patients receiving long‐term parenteral nutrition (PN) are at increased risk of aluminium (Al) toxicity because of bypass of the gastrointestinal tract during PN infusion. Complications of Al toxicity include metabolic bone disease (MBD), Al‐associated encephalopathy in adults, and impaired neurological development in preterm infants. Unlike the United States, there are no regulations regarding Al content of large‐ and small‐volume parenterals in Canada. We, therefore, aimed to present our data on plasma Al concentration and Al intake from our cohort of pediatric patients receiving long‐term PN. Methods: Plasma Al concentration was retrospectively gathered from the patient charts of all 27 patients with intestinal failure (IF) receiving long‐term PN at The Hospital for Sick Children, Toronto, Canada, and compared with age‐ and sex‐matched controls recruited for comparison. In addition, Al concentration was measured in PN samples collected from 10 randomly selected patients with IF and used to determine their Al intake. Results: The plasma Al concentration of patients with IF receiving long‐term PN was significantly higher than that of control participants (1195 ± 710 vs 142 ± 63 nmol/L; P < .0001). In the subgroup of 10 patients for whom Al intake from their PN solution was determined, mean ± SD Al intake from PN was 15.4 ± 15 µg/kg, 3 times the Food and Drug Administration upper recommended intake level, and Al intake was significantly related to plasma Al concentration (P = .02, r² = 0.52). Conclusion: Pediatric patients receiving long‐term PN for IF in Canada are at risk for Al toxicity.     

Innate Mucosal Immune System Response of BALB/c vs C57BL/6 Mice to Injury in the Setting of Enteral and Parenteral Feeding

Background: Outbred mice exhibit increased airway and intestinal immunoglobulin A (IgA) following injury when fed normal chow, consistent with humans. Parenteral nutrition (PN) eliminates IgA increases at both sites. Inbred mice are needed for detailed immunological studies; however, specific strains have not been evaluated for this purpose. BALB/c and C57BL/6 are common inbred mouse strains but demonstrate divergent immune responses to analogous stress. This study addressed which inbred mouse strain best replicates the outbred mouse and human immune response to injury. Methods: Intravenously cannulated mice received chow or PN for 5 days and then underwent sacrifice at 0 or 8 hours following controlled surgical injury (BALB/c: n = 16–21/group; C57BL/6: n = 12–15/group). Bronchoalveolar lavage (BAL) was analyzed by enzyme‐linked immunosorbent assay for IgA, tumor necrosis factor–α (TNF‐α), interleukin (IL)–1β, and IL‐6, while small intestinal wash fluid (SIWF) was analyzed for IgA. Results: No significant increase in BAL IgA occurred following injury in chow‐ or PN‐fed BALB/c mice (chow: P = .1; PN: P = .7) despite significant increases in BAL TNF‐α and SIWF IgA (chow: 264 ± 28 vs 548 ± 37, P < .0001; PN: 150 ± 12 vs 301 ± 17, P < .0001). Injury significantly increased mucosal IgA in chow‐fed C57BL/6 mice (BAL: 149 ± 33 vs 342 ± 87, P = .01; SIWF: 236 ± 28 vs 335 ± 32, P = .006) and BAL cytokines. After injury, PN‐fed C57BL/6 mice exhibited no difference in BAL IgA (P = .9), BAL cytokines, or SIWF IgA (P = .1). Conclusions: C57BL/6 mice exhibit similar airway responses to injury as outbred mice and humans, providing an appropriate model for studying mucosal responses to injury. The BALB/c mucosal immune system responds differently to injury and does not replicate the human injury response.     

Nutrition therapy for the critically ill surgical patient: we need to do better!

Background: To identify opportunities for quality improvement, the nutrition adequacy of critically ill surgical patients, in contrast to medical patients, is described. Methods: International, prospective, and observational studies conducted in 2007 and 2008 in 269 intensive care units (ICUs) were combined for purposes of this analysis. Sites provided institutional and patient characteristics and nutrition data from ICU admission to ICU discharge for maximum of 12 days. Medical and surgical patients staying in ICU at least 3 days were compared. Results: A total of 5497 mechanically ventilated adult patients were enrolled; 37.7% had surgical ICU admission diagnosis. Surgical patients were less likely to receive enteral nutrition (EN) (54.6% vs 77.8%) and more likely to receive parenteral nutrition (PN) (13.9% vs 4.4%) (P < .0001). Among patients initiating EN in ICU, surgical patients started EN 21.0 hours later on average (57.8 vs 36.8 hours, P < .0001). Consequently, surgical patients received less of their prescribed calories from EN (33.4% vs 49.6%, P < .0001) or from all nutrition sources (45.8% vs 56.1%, P < .0001). These differences remained after adjustment for patient and site characteristics. Patients undergoing cardiovascular and gastrointestinal surgery were more likely to use PN, were less likely to use EN, started EN later, and had lower total nutrition and EN adequacy rates compared with other surgical patients. Use of feeding and/or glycemic control protocols was associated with increased nutrition adequacy. Conclusions: Surgical patients receive less nutrition than medical patients. Cardiovascular and gastrointestinal surgery patients are at highest risk of iatrogenic malnutrition. Strategies to improve nutrition performance, including use of protocols, are needed.     

High Rates of Mortality and Morbidity Occur in Infants With Parenteral Nutrition–Associated Cholestasis

Background: Extremely few data are available about the natural history of parenteral nutrition (PN)–associated cholestasis. The authors evaluated a cohort of infants at a large center to determine the outcome of PN‐associated cholestasis in infants with some gastrointestinal function. Methods: The authors reviewed the records of all infants admitted to a level 3 neonatal intensive care unit over a 16‐month period who had the diagnosis of PN‐associated cholestasis. Records were reviewed in these infants for course of cholestasis, laboratory values, outcome, and infection rate. Results: Sixty‐six patients were admitted who met the study criteria. There were 10 deaths and 1 referral for liver transplant (Death/TPlant) (17%) in the first year of life. All Death/TPlant infants had at least 1 positive blood culture after the onset of cholestasis. Maximum conjugated bilirubin (MaxCB) in Death/TPlant infants was 15.7 ± 2.2 (SEM) compared to 8.4± 1.0 mg/dL in babies who recovered. Of 21 infants with a MaxCB≥ 10.0, Death/TPlant occurred in 8/21 (38%). Of 40 babies with positive blood cultures, 11 were in the Death/TPlant group vs no deaths among the 25 without positive blood cultures. Average time to resolution from the MaxCB to a CB <2.0 mg/dL was 66 ± 7 days (n = 49). Conclusions: Infants with PN‐associated cholestasis have high rates of mortality despite the presence of some gastrointestinal function. These data support further evaluation and the development of novel forms of therapy for babies with parenteral‐associated CB ≥2 mg/dL with emphasis on interventions for infants with a CB >10 mg/dL.     

The Association Between Home Parenteral Nutrition and Patients With FAP‐Associated Intra‐Abdominal Desmoids

Background: Intra‐abdominal desmoid tumors (IADTs) are a common complication of familial adenomatous polyposis (FAP). Treatment is not standardized for advanced disease. Medical and surgical treatments may be ineffective in preventing complications, which can cause intestinal failure. Home parenteral nutrition (HPN) can be a life‐saving treatment in these patients. The aim of this study was to investigate the association with HPN in FAP‐IADTs. Methods: A retrospective review of FAP patients with IADTs at the Cleveland Clinic (CC) between 1980 and 2009 was performed. Patients and tumor characteristics were retrieved from the CC Jagelman Registry for Inherited Neoplasms and CC HPN database. Inclusion criteria were FAP‐IADTs and 6‐month follow up at CC. Exclusion criteria were <6‐month follow‐up, lack of 3‐dimensional lesion or sheet desmoid, and/or incomplete medical records. Kaplan‐Meier curves were analyzed for HPN and non‐HPN groups. Results: One hundred fifty‐four patients were included and divided into 2 groups: HPN (n = 41, 26.6%) and non‐HPN (n = 113, 73.4%). The HPN group was more likely to have advanced‐stage disease and significantly higher incidence of chronic abdominal pain, narcotic dependency, bowel obstruction, ureteral obstruction, deep vein thrombosis, pulmonary embolism, fistulae, and sepsis (P < .05). The need for HPN represented a strong predictor of mortality (5‐year survival HPN = 72% vs non‐HPN = 95%), but duration of HPN did not affect mortality. Conclusion: HPN, although a life‐saving treatment, is an independent poor prognostic factor associated with high morbidity and mortality.     

Effect of Discontinuation of Manganese Supplementation From Home Parenteral Nutrition Solutions on Whole‐Blood Levels and Magnetic Resonance Imaging of the Brain: A 5‐Year Cohort Study

Background: Manganese (Mn) is included in current premixed multiple trace element (TE) additives for home parenteral nutrition (HPN). However, there is a risk of oversupplementation of Mn due to contamination from PN additives. Oversupplementation can produce Mn toxicity with neurologic symptoms and abnormalities on brain magnetic resonance imaging (MRI). In 2009, we reported that whole‐blood Mn levels were above the upper limit of normal in 16 HPN patients, with 81% having MRI findings. Subsequently, we removed Mn supplementation from all our HPN patients. We present a 5‐year follow‐up here. Methods: This is a prospective cohort study on 11 of the surviving 16 patients on HPN. All patients had Mn removed from PN and had yearly monitoring of blood Mn levels. Eight patients had a repeat MRI to evaluate for resolution of basal ganglia deposits. Patient demography, clinical history, and bloodwork were recorded. Results: Five of 6 patients who initially had elevated Mn levels had normal levels on follow‐up. All patients who had Mn levels measured serially had a decrease in levels; the mean percent decrease of Mn was 38.1% (range, 10.1%–53.8%). Two patients had elevated Mn despite the absence of supplementation. Six of 8 patients who had repeat MRIs had complete resolution abnormalities. Conclusions: Removal of Mn as an additive in HPN solutions resulted in resolution of MRI abnormalities in most patients. Over 5 years, all patients except for 1 maintained normal blood Mn levels. Therefore, Mn levels should be monitored and supplementation be individualized.     

Parenteral Fish Oil as Monotherapy Improves Lipid Profiles in Children With Parenteral Nutrition–Associated Liver Disease

Background: Parenteral nutrition (PN) is a life‐saving therapy but has been associated with dyslipidemia. Because fish oil has been shown to have positive effects on lipid profiles, the authors hypothesize that a parenteral fish oil lipid emulsion will improve lipid profiles in children who are PN dependent. Methods: The authors examined the lipid profiles of a unique cohort of 10 children who were exclusively administered a fish oil–based lipid emulsion while on PN for a median duration of 14 weeks. Longitudinal data analysis with a generalized estimating equations approach was used to determine the sterol and bilirubin levels based on duration of the fish oil–based lipid emulsion. Results: After 14 weeks of fish oil monotherapy, children had a 24% increase in high‐density lipoprotein. Compared to baseline, serum low‐density lipoprotein, very low‐density lipoprotein, total cholesterol, and triglyceride levels all significantly decreased by 22%, 41%, 17%, and 46%, respectively. Eight children had their bilirubin improved with a decreased direct bilirubin from 6.9 mg/dL (range, 4.4‐10.7) at baseline to 2.3 mg/dL (range, 1.3‐4.0) after 14 weeks, and a decrease in total bilirubin from 8.7 mg/dL (range, 5.5‐13.7) to 3.8 mg/dL (range, 2.2‐6.5). Conclusion: A fish oil–based lipid emulsion used as monotherapy in children who exclusively depended on PN for survival was associated with significant improvement in all major lipid panels as well as improvement of hyperbilirubinemia. Parenteral fish oil may be the preferred lipid source in children with dyslipidemia.     

Effect of Early Full‐Calorie Nutrition Support Following Esophagectomy: A Randomized Controlled Trial

Background: Early use of enteral nutrition (EN) is indicated following surgical resection of esophageal cancer. However, early EN support does not always meet the optimal calorie or protein requirements, and the benefits of supplementary parenteral nutrition (PN) remain unclear. We aimed to evaluate the efficacy and safety of early supplementary PN following esophagectomy. Materials and Methods: We enrolled 80 consecutive patients who underwent esophagectomy. Resting energy expenditure and body composition measurements were performed in all patients preoperatively and postoperatively. EN was administered after surgery, followed by randomization to either EN+PN or EN alone. The amount of PN administered was calculated to meet the full calorie requirement, as measured by indirect calorimetry, and 1.5 g protein/kg fat‐free mass (FFM) per day was added as determined by body composition measurement. The clinical characteristics were compared between the 2 groups. Results: Patients in the EN+PN group but not in the EN group preserved body weight (0.18 ± 3.38 kg vs ?2.15 ± 3.19 kg, P < .05) and FFM (1.46 ± 2.97 kg vs ?2.08 ± 4.16 kg) relative to preoperative measurements. Length of hospital stay, postoperative morbidity rates, and standard blood biochemistry profiles were similar. However, scores for physical functioning (71.5 ± 24.3 vs 60.4 ± 27.4, P < .05) and energy/fatigue (62.9 ± 19.5 vs 54.2 ± 23.5, P < .05) were higher in the EN+PN group 90 days following surgery. Conclusion: Early use of supplemental PN to meet full calorie requirements of patients who underwent esophagectomy led to better quality of life 3 months after surgery. Moreover, increased calorie and protein supplies were associated with preservation of body weight and FFM.     

Longitudinal Bone Mineralization Assessment in Children Treated With Long‐Term Parenteral Nutrition for Severe Intestinal Failure

Background: Metabolic bone disease is common in children receiving home parenteral nutrition (HPN) for intestinal failure (IF). Long‐term evolution of bone mass in pediatric IF is poorly documented. The aims of this study were (1) to determine the prevalence of low bone mass (LBM) in children receiving HPN for IF, (2) to evaluate the evolution of total bone mineral content (TBMC) during HPN with dual‐energy x‐ray absorptiometry (DXA), and (3) to identify related factors. Methods: All children referred in our HPN center from 2004 to 2014 were eligible. Inclusion criteria were HPN dependence due to noninflammatory IF, at least 2 TBMC assessments, and HPN duration of at least 2 years at last DXA. TBMC was expressed in z score for ideal weight for height (WFH). LBM was defined by a TBMC WFH z score ≤–2 standard deviations (SD). Results: A total of 175 DXAs for 31 children were performed, mean of 5.6 ± 2.9 assessments per child. The median time between first and last DXA recorded was 6.2 years (0.7–16.6). At the first DXA, 14 children (45%) had a LBM. TBMC increased by +0.1 ± 0.04 SD per year of HPN (P = .012). The risk of LBM decreased with an odds ratio of 0.9 per year of HPN (95% confidence interval, 0.92–0.99; P = .018). Lean mass z score and calcium parenteral intakes were related to the TBMC improvement. Conclusion: LBM is common in pediatric IF, but bone status could improve during HPN in these children.