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We evaluated plasma sulfur amino acid concentrations in children with short gut syndrome receiving home parenteral nutrition (n = 6). Cysteine HCl addition to solutions formulated with a pediatric amino acid product will increase plasma taurine concentrations to within the normal reference range.

Fourteen infants requiring long-term total parenteral nutrition but able to tolerate small quantities of enteral feedings were randomized into carnitine treatment and placebo control groups. All infants had received nutritional support devoid of carnitine. Plasma carnitine levels and observed plasma lipid indices were not different before supplementation. Under standardized, steady-state conditions, 0.5 g/kg fat emulsion (intralipid) was administered intravenously over 2 hours both before and after infants received 7 days of continuous nasogastric or gastric tube L-carnitine (50 mumol/kg/day) or placebo. Plasma triglyceride, free fatty acid, acetoacetate, beta-hydroxybutyrate, and carnitine concentrations were observed at 0 (start of lipid infusion), 2, and 4 hours for pre- and post-treatment periods, and in addition at 6 and 8 hours after carnitine supplementation. Infants receiving carnitine had significantly greater beta-hydroxybutyrate plasma concentrations (P less than 0.05) and carnitine (P less than 0.001) at 0, 2, 4, 6, and 8 hours, and greater plasma acetoacetate concentrations (P less than 0.05) at 2, 4, 6, and 8 hours, compared with controls. Twenty-four-hour urinary carnitine excretion was very low for both groups before supplementation; after supplementation, excretion was higher (P less than 0.05) in the carnitine group. No significant differences were found between groups for plasma triglyceride or free fatty acid concentrations at any observation period. This study demonstrated enhanced fatty acid oxidation, as evidenced by increased ketogenesis, with L-carnitine supplementation in infants receiving long-term total parenteral nutrition.

The relationships among plasma total carnitine concentration, postnatal age, and fatty acid metabolism were evaluated in 57 infants receiving parenteral nutrition. Concentrations of plasma carnitine, triglycerides, free fatty acids, acetoacetate, and beta-hydroxybutyrate were determined before and at 2 and 4 hours from the beginning of a standardized 2-hour lipid infusion. Plasma carnitine concentrations declined with increasing postnatal age. There were no significant differences in gestational age or triglyceride concentrations between infants less than or equal to 4 weeks of age and those greater than 4 weeks of age, whereas free fatty acid concentrations were lower and acetoacetate and beta-hydroxybutyrate concentrations were higher in the younger infants. Infants less than or equal to 4 weeks of age were further grouped according to plasma carnitine concentration greater than 13 nmol/ml (group 1) and less than or equal to 13 nmol/ml (group 2) and were then compared with infants greater than 4 weeks of age (group 3). There were no significant differences in triglyceride concentrations among the three groups; free fatty acids, acetoacetate, and beta-hydroxybutyrate concentrations for group 2 patients were similar to those of group 1 patients or fell between values for group 1 and group 3 patients. These results demonstrate decreasing plasma carnitine concentrations and possibly for more than 4 weeks.

The influence of 11 variables on the infiltration of peripheral venous lines (PVLs) was evaluated during 151 infusions in patients younger than 1 year of age. Infusions were followed from the time of cannula placement to discontinuation. Fifty-eight percent of PVLs were infiltrated by 36.30 +/- 33.53 hours (mean +/- SD; median 40 hours, range 10 to 187 hours). No difference between infiltrated and noninfiltrated PVLs was noted with regard to patient age, gender, weight, cannula type, cannula gauge, cannula site, infusion device, potassium or dextrose concentration, medications, or rate of solution administration (P greater than 0.05). Infiltration was observed more often in black than in white infants (P = 0.03) and in patients with lower controller solution head heights (P = 0.01). The time to infiltration was decreased significantly for steel verus Teflon cannulas (P = 0.02), for administration of intravenous medication versus no administration of medication (P = 0.03), for peripheral parenteral nutrition solutions compared with 5% or 10% dextrose solutions (P = 0.014), and with increasing cannula gauge (P = 0.05). The time to infiltration did not differ significantly for gravity-controlled versus positive-pressure infusion device delivery (P = 0.51) or for potassium concentrations less than or equal to 20 mEq/L versus greater than 20 mEq/L (P = 0.13). Infusion device occlusion alarms were associated with only 19% of infiltrations. No sloughing of skin or necrosis of tissue occurred related to infiltration.