Impact of the Nationwide Intravenous Selenium Product Shortage on the Development of Selenium Deficiency in Infants Dependent on Long-Term Parenteral Nutrition.

BACKGROUND: For patients dependent on parenteral nutrition (PN), selenium must be supplemented intravenously. A nationwide intravenous selenium shortage began in April 2011. The impact of this shortage on PN-dependent infants was evaluated by examining the provision of selenium, development of biochemical deficiency, and costs associated with the shortage. MATERIALS AND METHODS: This single-center, retrospective study included PN-dependent infants aged ≤1 year who weighed ≤30 kg, received PN for ≥1 month, and had ≥1 serum selenium measurement. The primary outcome was the incidence of biochemical selenium deficiency. Secondary outcomes included severity of biochemical deficiency, clinical manifestations, costs, and relationship between serum selenium levels and selenium dose. RESULTS: The average selenium dose decreased 2-fold during the shortage (2.1 ± 1.2 µg/kg/d; range, 0.2-4.6 µg/kg/d) versus the nonshortage period (3.8 ± 1 µg/kg/d; range, 2.4-6 µg/kg/d; P < .001). A linear relationship between serum selenium concentration and selenium dose was observed (r(2) = 0.42), with a dose of 6 µg/kg/d expected to result in normal serum levels in most cases. Similar proportions of patients developed biochemical deficiency in both groups: shortage period, 59.1%; nonshortage, 66.7%; P = .13. The severity of biochemical deficiency was similar between groups. A significant increase in incremental cost during the shortage was observed. CONCLUSION: This is the first study examining the impact of the intravenous selenium shortage on PN-dependent infants. Both groups exhibited similarly high incidences of biochemical selenium deficiency, suggesting higher empiric doses may benefit this population. However, ongoing shortages limit the ability to provide supplementation.

Preliminary findings of long-term neurodevelopmental outcomes of infants treated with intravenous fat emulsion reduction for the management of parenteral nutrition-associated cholestasis.

INTRODUCTION: Parenteral nutrition-associated cholestasis (PNAC) is linked with the administration of soybean-based intravenous fat emulsion (IVFE). IVFE reduction (IFER) may be an effective management strategy for PNAC; however, long-term associated neurodevelopmental outcomes (NDOs) for infants undergoing IFER have not been measured previously. This single-institution, prospective study examined the risk for negative NDOs and key predictors of NDOs associated with IFER. METHODS: Patients (2-5 years) treated with soybean-based IFER as neonates underwent NDO measurements, including Ages and Stages Questionnaires-3 (ASQ-3), Parents' Evaluations of Developmental Status (PEDS), and Behavior Assessment System for Children, Second Edition Preschool, Parent (BASC-2 PRS-P). The relationship between NDOs and predictive variables was evaluated. RESULTS: A total of 25 children had a complete PEDS survey, and 17 were found to be "not at risk." The BASC-2 PRS-P evaluation (n = 18 patients) showed that all 4 composite domains fell within the normative developmental range, and 67%-89% of patients were observed to be "typically developing." For the primary outcome measure, ASQ-3, 82.4%-94.4% of patients were "not at risk." Logistical regression analyses were performed to examine risk factors contributing to negative NDOs. Of children completing all NDO studies, IFER-related variables (eg, development of essential fatty acid deficiency, duration of IFER, and mean IVFE dose) were not found to be predictors of adverse NDOs. CONCLUSIONS: This study represents the first report of NDOs in pediatric patients treated with IFER. IFER-treated patients score within the normative range most of the time. IFER-related variables were not found to be associated with negative NDOs. The results set the stage for a larger, multicenter, prospective study.

Dosing and monitoring of trace elements in long-term home parenteral nutrition patients.

BACKGROUND: Trace elements (TEs) dosing and monitoring in home parenteral nutrition (PN) patients vary with their underlying conditions. METHODS: This retrospective observational study evaluated parenteral TE dosing, serum TE concentrations and monitoring, and dose-concentration relationships between TE doses and serum TE concentrations in 26 adult and adolescent home PN patients. RESULTS: There was a total of 40,493 PN days. Average parenteral zinc doses of 9.1 mg/d and 7.6 mg/d resulted in the majority of serum zinc concentrations (90%) within normal range in patients with and without short bowel syndrome (SBS), respectively. Selenium at about 70 mcg/d resulted in about 60% of serum selenium concentrations within normal range, with 38% of values below normal in patients with and without SBS alike. Copper at 1 mg/d resulted in 22.5% of serum copper concentrations above the normal range. The majority of serum manganese (94.6%) and chromium (96%) concentrations were elevated. Serum TE concentrations were infrequently monitored. Significant relationships existed between doses and serum concentrations for zinc (P < .0001), manganese (P = .012), and chromium (P < .0001) but not for selenium or copper. CONCLUSIONS: TE doses in home PN should be individualized and adjusted based on regular monitoring of TE status. In long-term home PN patients, higher zinc and selenium doses may be necessary to maintain their normal serum concentrations. Lower copper doses and restrictions of manganese and chromium supplementation may be needed to avoid their accumulation. Relationships between TE doses and serum TE concentrations vary for each TE and underlying clinical conditions.

Survival outcomes of pediatric intestinal failure patients: analysis of factors contributing to improved survival over the past two decades.

BACKGROUND: Intestinal failure (IF) is associated with significant and life-threatening complications. Recent studies suggest that treatments for IF in the pediatric population are improving over time. Based on this, we examined whether pediatric IF survival rates have improved in our patient population over the past two decades, and secondarily examined which aspects of patient care contributed to changes in survival. METHODS: We conducted a retrospective chart review of all pediatric patients with IF at our children's hospital from 1990 through 2009. Cox regression analyses were used to determine change in survival rates over time (5-y cohorts), and we examined multiple covariates to determine their potential influence on survival rates over time. RESULTS: A significant improvement in survival of 171 children with IF was noted over the past two decades. Children with an onset of IF in the 1990-1994 cohort had significantly decreased survival compared with children in all subsequent cohorts (P = 0.011). The only intervention that was identified between this time period and future periods was the establishment of a comprehensive intestinal failure clinical care team. While the latter three cohorts were not significantly different, progressively increased survival was noted. Ability to wean off parenteral nutrition (PN) and small bowel length greater than 10% of the expected length were significantly associated with improved survival over this time period (P < 0.01). Other tested covariates, including the more recent use of ethanol lock therapy (to prevent catheter sepsis) and lipid reduction strategies (to treat PN-associated cholestasis) failed to show a significant impact on improved survival. CONCLUSIONS: Despite a striking improvement in survival of children with IF over the past two decades, the only identified intervention that significantly impacted survival was establishment of a comprehensive care team. These findings emphasize the need for multi-disciplinary efforts to care for such complex and challenging children.