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BACKGROUND Few studies have examined the epidemiology and risk factors for the development of outpatient-acquired catheter-related bloodstream infections (CRBSIs) in children receiving home parenteral nutrition. This study aimed to (1) characterize the incidence, clinical presentation, and epidemiology of CRBSIs and (2) identify risk factors for CRBSIs in children receiving home parenteral nutrition. METHODS A longitudinal database approved by our Institutional Review Board was created to prospectively track CRBSIs in the UCLA pediatric population from January to December 2012. Eligible patients included those

BACKGROUND Intravenous fish oil (FO) treats pediatric intestinal failure-associated liver disease (IFALD). There are concerns that a lipid emulsion composed of ω-3 fatty acids will cause an essential fatty acid deficiency (EFAD). This study's objective was to quantify the risk for abnormal fatty acid concentrations in children treated with FO. METHODS Inclusion criteria for this prospective study were children with intestinal failure. Intravenous soybean oil (SO) was replaced with FO for no longer than 6 months. Serum fatty acids were analyzed using linear and logistic models, and compared with age-based norms to determine the percentage of subjects with low and high concentrations. RESULTS Subjects (n = 17) started receiving FO at a median of 3.6 months (interquartile range 2.4-9.6 months). Over time, α-linolenic, linoleic, arachidonic, and Mead acid decreased, whereas docosahexaenoic and eicosapentaenoic acid increased (P

Purpose Damaged central venous catheters (CVCs) are commonly repaired to avoid line replacement and preserve vascular access. However, limited data suggest an increased risk for central line-associated bloodstream infections (CLABSIs) associated with the repair procedure. The purpose of this study was to describe outcomes of CVC repairs among parenteral nutrition (PN) dependent children with intestinal failure (IF). Methods A 2-year retrospective review was performed on children with IF on home PN > 6 months. Outcomes of interest were repair success and postrepair CLABSI incidence. Descriptive statistics included medians and frequencies. Results A total of 36 pediatric IF patients underwent 96 CVC repairs during the study period. The median CVC repair count was 1.5 repairs/patient (range, 1 to 16 repairs/patient) with > 1 repair in half the patients. Ninety-four broken catheters (98%) were successfully repaired with restoration of function. Of the unsuccessful repairs (2%), the two catheters eventually required surgical removal and replacement. One repair (1%) was followed by a CLABSI with Enterococcus faecalis in an immunocompromised patient. Conclusion CVC repair is a highly successful procedure with a low risk for infection. Catheter repair should be considered whenever possible as it may extend the lifetime of the catheter and decrease the risk for vascular access loss. Level of evidence Treatment study; level IV.

Soybean oil (SO) emulsions are associated with intestinal failure-associated liver disease (IFALD); fish oil (FO) emulsions are used to treat IFALD. SO and FO differ with respect to their fatty acid and phytosterol content. In children with IFALD whose SO was replaced with FO, we aimed to (1) quantify changes in erythrocyte fatty acids and plasma phytosterols, cytokines, and bile acids and (2) correlate these changes with direct bilirubin (DB).This study enrolled IFALD children who received 6 months of FO. Blood samples were collected prior to FO, and after 2 weeks and 3 and 6 months of FO. The primary outcome was 3-month vs baseline biomarker concentrations.At study initiation, the median patient age was 3 months (interquartile range, 3-17 months), and mean ± standard deviation DB was 5.6 ± 0.7 mg/dL (n = 14). Cholestasis reversed in 79% of subjects. Eicosapentaenoic and docosahexaenoic acid was greater than baseline (P.001, all time points). Linoleic and arachidonic acid and sitosterol and stigmasterol were less than baseline (P.05, all time points). Three- and 6-month interleukin-8 (IL-8) and total and conjugated bile acids were less than baseline (P.05). Baseline IL-8 was correlated with baseline DB (r = 0.71, P.01). Early changes in stigmasterol and IL-8 were correlated with later DB changes (r = 0.68 and 0.75, P.05).Specific fat emulsion components may play a role in IFALD. Stigmasterol and IL-8 may predict FO treatment response.

Studies have suggested that when intravenous (IV) soybean oil (SO) is replaced with fish oil (FO), direct hyperbilirubinemia is more likely to resolve. The necessary duration of FO has not been established. This study seeks to determine if 24 weeks of FO is an effective and safe therapy for intestinal failure-associated liver disease (IFALD).This is a clinical trial using patients with IFALD between the ages of 2 weeks and 18 years. SO was replaced with FO (1 g/kg/d) in 10 patients who were receiving most of their calories from parenteral nutrition (PN). Patients were compared with 20 historic controls receiving SO. SO for both groups was prescribed by the primary medical team at variable doses. The primary outcome was time to reversal of cholestasis. Secondary outcomes were death, transplant, and full enteral feeds. Safety measurements included growth, essential fatty acid deficiency, and laboratory markers to assess bleeding risk.The Kaplan-Meier method estimated that 75% in the FO group would experience resolution of cholestasis by 17 weeks vs 6% in the SO group (P.0001). When compared with the SO group, the FO group had decreased serum direct bilirubin concentrations at weeks 8 (P = .03) and 12, 16, 20, and 24 weeks (P.0001). Although length z score at the end of the study increased in the FO group compared with baseline (P = .03), there were no significant differences in other outcomes.A limited duration of FO appears to be safe and effective in reversing IFALD.

BACKGROUND Advances in intestinal transplantation (ITx) have resulted in improved survival and the opportunity to examine nutritional outcomes. The aim of this study was to describe detailed, long-term nutritional results and identify positive predictors of growth and weight gain following pediatric ITx. METHODS A single-center retrospective, Institutional Review Board-approved review of a prospective database was conducted. Inclusion criteria were ITx recipients 18 years or younger with survival of 6 months or more. Outcomes included anthropometric measurements and biochemical markers at 6, 12, 24, 36, and 48 months post-ITx. More than 25 ITx-related variables were analyzed as potential predictors of growth and weight gain. Statistical analysis was performed using chi-square test, t test, and analysis of variance. RESULTS Between November 1991 and April 2007, 50 children received 55 ITx; 33 patients met eligibility criteria. Median age at ITx was 2.2 years, follow-up time was 3.8 years, and time from ITx to cessation of total parenteral nutrition was 31 days. The most common micronutrient deficiencies post-ITx were zinc, iron, and copper. Serum protein levels improved significantly over time. Weight gain occurred within 6 months and vertical growth within 12 months, although limited catch-up growth was seen. Early predictors of weight gain and growth included shorter hospitalization and absence of rejection. Long-term predictors were low steroid dosage, infrequent hospitalization, and the use of peptide-based formulas. CONCLUSIONS This represents one of the largest and most comprehensive long-term studies on nutritional outcomes in pediatric ITx. Overall, positive growth and weight gain were seen as were micronutrient deficiencies. Numerous long-term nutritional challenges exist which require a multidisciplinary approach and future prospective studies.

Objectives In a large cohort of children with intestinal failure (IF), we sought to determine the cumulative incidence of achieving enteral autonomy and identify patient and institutional characteristics associated with enteral autonomy. Study design A multicenter, retrospective cohort analysis from the Pediatric Intestinal Failure Consortium was performed. IF was defined as severe congenital or acquired gastrointestinal diseases during infancy with dependence on parenteral nutrition (PN) >60 days. Enteral autonomy was defined as PN discontinuation >3 months. Results A total of 272 infants were followed for a median (IQR) of 33.5 (16.2-51.5) months. Enteral autonomy was achieved in 118 (43%); 36 (13%) remained PN dependent and 118 (43%) patients died or underwent transplantation. Multivariable analysis identified necrotizing enterocolitis (NEC; OR 2.42, 95% CI 1.33-4.47), care at an IF site without an associated intestinal transplantation program (OR 2.73, 95% CI 1.56-4.78), and an intact ileocecal valve (OR 2.80, 95% CI 1.63-4.83) as independent risk factors for enteral autonomy. A second model (n = 144) that included only patients with intraoperatively measured residual small bowel length found NEC (OR 3.44, 95% CI 1.36-8.71), care at a nonintestinal transplantation center (OR 6.56, 95% CI 2.53-16.98), and residual small bowel length (OR 1.04 cm, 95% CI 1.02-1.06 cm) to be independently associated with enteral autonomy. Conclusions A substantial proportion of infants with IF can achieve enteral autonomy. Underlying NEC, preserved ileocecal valve, and longer bowel length are associated with achieving enteral autonomy. It is likely that variations in institutional practices and referral patterns also affect outcomes in children with IF.

To analyze the outcome of children with short bowel syndrome (SBS) who required long-term parenteral nutrition (PN).Retrospective analysis of children (n=78) with SBS who required PN3 months from 1975 to 2000.univariate analysis, Kaplan-Meier method, and Cox proportional regression model were used.We identified 78 patients. Survival was better with small bowel length (SBL)38 cm, intact ileocecal valve (ICV), intact colon, takedown surgery after ostomy (all P.01), and primary anastomosis (P.001). PN-associated early persistent cholestatic jaundice (P.001) and SBL of15 cm (P.01) were associated with a higher mortality. Intestinal adaptation was less likely if SBL15 cm (P.05), ICV was removed, colonic resection was done (both P.001),50% of colon was resected (P.05), and primary anastomosis could not be accomplished (P.01). Survival was 73% (57), and 77% (44) of survivors had intestinal adaptation.SBL, intact ICV, intestinal continuity, and preservation of the colon are important factors for survival and adaptation. Adaptation usually occurred within the first 3 years. Need for long-term PN does not preclude achieving productive adulthood. Patients with ICV even with15 cm of SBL and patients with SBL15 cm without ICV have a chance of intestinal adaptation.

Home parenteral nutrition (PN) is a lifesaving therapy for children with intestinal failure (IF). Our aims were to describe the prevalence of micronutrient deficiencies (vitamin D, zinc, copper, iron, selenium) in a diverse population of children with IF receiving PN and to identify and characterize risk factors associated with micronutrient deficiencies, including hematologic abnormalities.Data were collected on 60 eligible patients through retrospective chart review between May 2012 and February 2015. Descriptive statistics included frequencies, medians, interquartile ranges (IQRs), and odds ratios (ORs). Statistical analyses included χPatients were primarily young (median age, 3.3 years; IQR, 0.7-8.4), Latino (62%), and male (56%), with short bowel syndrome (70%). Of 60 study patients, 88% had ≥1 deficiency and 90% were anemic for age. Of 51 patients who had all 5 markers checked, 59% had multiple deficiencies (defined as ≥3). Multivariate analysis shows multiple deficiencies were associated with nonwhite race (OR, 9.4; P = .012) and higher body mass index z score (OR, 2.2; P = .016). Children with severe anemia (hemoglobin8.5 g/dL) made up 50% of the cohort. Nonwhite race (OR, 6.6; P = .037) and zinc deficiency (OR, 11; P = .003) were multivariate predictors of severe anemia.Micronutrient deficiency and anemia are overwhelmingly prevalent in children with IF using chronic PN. This emphasizes the importance of universal surveillance and supplementation to potentially improve quality of life and developmental outcomes. Future research should investigate how racial disparities might contribute to nutrition outcomes for children using chronic PN.

Vitamin D plays important roles in both skeletal and nonskeletal health. Limited data suggest that patients with intestinal failure (IF) receiving home parenteral nutrition (PN) are at risk for vitamin D deficiency due to inadequate oral intake, poor absorption, and chronic illness. The purpose of this study was to document vitamin D status in pediatric patients with IF receiving home PN.We performed a 2-year retrospective review of children with IF followed at our center who had been on home PN for ≥6 months and had ≥1 serum 25-hydroxyvitamin D (25-OHD) level checked as part of routine clinical care. Patients were then categorized as deficient (20 ng/mL), insufficient (20-29 ng/mL), or normal (≥30 ng/mL) based on their lowest vitamin D level. Demographic data and clinical characteristics were also assessed.Eleven of 27 children (41%) had ≥1 insufficient 25-OHD level, including one child with vitamin D deficiency. Diagnosis of short bowel syndrome (compared with dysmotility or malabsorption syndromes) was associated with decreased likelihood of suboptimal vitamin D status, with an odds ratio of 0.12 (95% confidence interval, 0.02-0.8, P = .028). Osteopenia was noted in 59% of the cohort. There was a trend toward higher risk for osteopenia in patients with low 25-OHD levels compared with those with normal 25-OHD levels (82% vs 44%, P = .109).Suboptimal 25-OHD levels are common in children with IF on home PN. This emphasizes the critical importance of routine surveillance of serum vitamin D levels and consideration of enteral supplementation when indicated.

Intravenous fish oil (FO) has changed the management of intestinal failure associated liver disease (IFALD). This report describes two IFALD patients who received FO for 5 and 10 months, respectively and reports on their 3-year follow-up.

Various expert bodies have recommended that the daily parental intake of chromium in children receiving total parenteral nutrition (TPN) should be 0.20 micrograms/kg. To test whether this recommendation is appropriate, we assessed chromium intake, serum chromium concentrations, and renal function in 15 children receiving TPN. The median duration of TPN use was 9.5 (range 1.3-14) years. The children's glomerular filtration rate (GFR), measured by plasma clearance of indium-111-DTPA was lower than that of non-TPN controls (70 [SD 17] vs 110 [10] ml/min per 1.73 m2). The daily chromium intake averaged 0.15 (0.09) micrograms/kg daily but the serum chromium concentration was 20 (4 to 42) times higher than that of the controls (2.1 [1.2] vs 0.10 [0.03] micrograms/l; p less than 0.0001). GFR was significantly inversely correlated with serum chromium concentration (r = -0.60, p less than 0.02), daily chromium intake (r = -0.69, p less than 0.01), cumulative parenteral chromium intake (r = -0.72, p less than 0.01), and TPN duration (r = -0.52, p less than 0.05). We discontinued chromium supplementation of TPN solutions and reassessed the children a year later. Contaminating chromium concentrations were 1.0-1.8 micrograms/l in TPN solutions and 0.9 micrograms/l in fat emulsions. Drinking water contained 4.3-5.7 micrograms/l. Thus, the chromium intake without supplementation was only 0.05 (0.01) micrograms/kg daily. The mean serum chromium concentration fell to 0.50 (0.30) micrograms/l but was still significantly higher than that in the controls (p less than 0.01). The GFR did not change significantly (65 [14] ml/min per 1.73 m2). No patient has shown signs of chromium deficiency. Although our patients were receiving less than the recommended chromium intake during supplementation, their high serum concentrations suggested excessive intake. The recommended parenteral chromium intake for children should be lowered.

Use of long-term total parenteral nutrition (TPN) is often presumed to be associated with serious hepatic dysfunction. In this retrospective study, we reviewed the complete charts of patients who had received TPN for more than 2.5 years, starting in infancy or childhood, for evidence of liver dysfunction. There were 16 male and 10 female patients with a total of 254.5 patient years on TPN. Seventeen patients have been on TPN since birth or early infancy. Thirteen of 26 patients deriveor = 90% of their calorie intake from TPN. Six patients had hepatomegaly; two of them also had splenomegaly. Twenty-one patients had normal transaminases, nine have had past episodes of raised enzymes ranging from 2.5 to 7.5 times normal. Seventeen patients always had normal bilirubin levels, five had past episodes of hyperbilirubinaemia, while four patients had persistently raised bilirubin levels (range 1.5-20.7 g/dl). Alkaline phosphatase was normal for age in all patients except two. Hepatic synthetic function, as measured by albumin, pre-albumin levels and prothrombin time, was within the normal range in all patients except one. Liver biopsies were performed in eight patients. Two biopsies showed cirrhosis, one showed chronic active hepatitis (CAH) with cholestasis, two patients had fibrosis, one showed cholestasis and two biopsies were normal. One patient with cirrhosis and one with CAH were positive for hepatitis C antibody. Another asymptomatic patient was positive for hepatitis B. Only the patient with CAH had hepatic decompensation. We conclude that clinical hepatic failure is uncommon in our group of patients on long-term TPN for 2.5 years or more. Cirrhosis and fibrosis, when found, could not be solely attributed to TPN.

Purpose To evaluate the outcomes of patients undergoing intestinal transplantation (IT). Methods Retrospective review was undertaken using existing medical records and database. Results Between November 1991 and May 2003, 114 patients were referred for consideration for IT, of which 33 patients received 37 intestinal allografts. All patients had intestinal failure and all patients had significant complications from total parenteral nutrition (TPN). TPN was the predominant cause of liver failure (63%). Combined liver intestinal grafts were used in the majority of patients. Overall 1- and 3-year patient survival is 77% and 52% with patients transplanted since 1999 having a 1- and 3-year survival of 94% and 73%, respectively. The most common cause of death was sepsis. No graft or patient was lost to cytomegalovirus or Epstein-Barr virus disease. Twenty-seven percent of allografts were lost to rejection. Long-term TPN independence is 82% for grafts more than 30 days after IT. Statistical analysis revealed several important factors impacting outcome. Conclusions Successful IT defined as prolonged patient and graft survival and TPN independence can be readily achieved in select patients with IF and complications related to TPN therapy. Outcomes have improved with experience gained and control of viral infections and rejection.

Total parenteral nutrition (TPN) has made survival beyond infancy possible for many infants who have sustained small intestinal loss as a result of gastroschisis or omphalocele. The length and quality of life in these patients have often been limited by the development of late sequelae secondary both to the protracted use of TPN and the long-term complications of a shortened gut. This study was undertaken to determine what factors influence the morbidity and mortality of short-bowel syndrome (SBS) due to gastroschisis or omphalocele. A retrospective chart review of 850 infants who received TPN from January 1977 through December 1999 was carried out. All infants were treated at one academic medical center; those who had receivedor =3 months of TPN were further segregated and their diagnosis, surgical procedures, length of bowel, ability to wean from TPN, follow-up weight and height, and developmental progress were recorded. Seventeen children were identified with SBS and either gastroschisis or omphalocele. Tight primary or secondary closure of the abdominal wall was believed to be a major cause of bowel necrosis and SBS in at least ten of the 17 patients. Overall survival was 76 per cent (13/17); survival was correlated with length of remaining bowel and was 86 per cent in patients having more than 15 cm of small bowel remaining but only 33 per cent in patients with less than 15 cm of small bowel remaining (P = 0.05). A longer length of residual small bowel resulted in a significantly shorter duration of TPN with a mean duration of 1.0 year for survivors having38 cm and 10.0 years for survivors with38 cm of bowel remaining (P = 0.03). Hepatic dysfunction with progressive failure resulting from TPN was related to death in three of the four nonsurvivors. The presence or absence of an ileocecal valve appeared unrelated both to the success of TPN weaning and to the length of time on TPN (P0.2). Eight of the 13 survivors have no ileocecal valve; five have undergone50 per cent colonic resection. Nine of the survivors have adapted to enteral feedings (mean 36 +/- 60 months) during which time weaning from TPN occurred. The mean age of survivors is 7.9 +/- 5.1 years. Near-normal weights (defined as exceeding the fifth percentile for weight) were achieved for 92 per cent of the patients (12/13) with 46 per cent of the patients (6/13) exceeding the 50th percentile. Near-normal heights (exceeding the fifth percentile) were achieved for 77 per cent of the patients (10/13) with 15 per cent of the patients (2/13) exceeding the 50th percentile. Quality of life was measured on the basis of return to public school: nine of ten school-age survivors attend school and eight are normal without signs of developmental delay. Tight abdominal closure of gastroschisis or omphalocele may cause bowel necrosis and SBS. TPN has improved the long-term survival and quality of life in infants with SBS.

In 18 children receiving long-term total parenteral nutrition (TPN) without iodide supplements, thyroid function test results were normal but serum iodide levels were greater than in control subjects (p less than 0.01). Iodine contamination of TPN solutions and fat emulsions accounted for only half of the recommended parenteral intake. Skin absorption of topical iodinated disinfectant may explain the adequate, if not excessive, iodine intake. We conclude that iodine is an unnecessary supplement in TPN solutions.

Background This study sought to describe the long-term nutritional outcomes of children after intestinal transplant (SBT). Methods Between 1991 and March 2005, 30 children received 33 SBT at a single center. Eligibility criteria included patient and graft survival >6 months. Weight, height, albumin, prealbumin, zinc (Zn), and essential fatty acid (EFA) levels were reviewed retrospectively. Results The 19 patients who met inclusion criteria had a median age at SBT of 2.9 years. The majority of patients were male, Latino, transplanted for necrotizing enterocolitis and received combined liver-SBT. All patients were weaned off total parenteral nutrition to elemental formula at a mean of 39 days post-SBT. Seventeen of 19 patients were Zn deficient and four patients were EFA deficient post-SBT. Conclusions Pre-SBT most subjects were significantly deficient in anthropometric and biochemical parameters. Post-SBT the mean Z score for weight and height improved significantly at year 1, then leveled off in year 2. Serum protein levels improved from pre-SBT, yet remained low–normal. Zn deficiency was seen frequently after SBT and is under investigation. Children who developed EFA deficiency were on the same formula, receiving inadequate EFA supplementation. Successful SBT was associated with growth and maintenance of serum nutritional parameters but not with significant catch-up growth.