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Introduction: Infantile oxalosis is the most severe form of primary hyperoxaluria type 1 (PH1), with onset of end-stage kidney disease (ESKD) during infancy. We aimed to analyze the outcome of these patients as our current understanding is limited owing to a paucity of reports. Methods: A retrospective registry study was conducted using data from the OxalEurope registry. All PH1 patients with ESKD onset at age

Summary Background & aims Ornithine alphaketoglutarate (OKG) is known for its anabolic and anticatabolic effects. Little is known about its effect on proteolysis. We studied urea formation and nitrogen excretion rates following OKG supplementation in healthy volunteers. Methods Sixteen male subjects were kept on a diet stable in protein (1 g kg−1 d−1) and aimed at maintaining body weight. They received oral supplementation of OKG (10 g) or an isonitrogenous placebo ( l -alanine) from days 4 to 6 in single daily doses in a randomised, blinded fashion. Urea kinetics were studied on day 4 from plasma urea enrichments following an oral [13C]urea bolus. Nitrogen excretion was assessed from 24-h urine collections prior to supplementation, on day 4 and day 7. Results Mean urea rate of appearance was 380±86 and 353±120 μmol kg−1 h−1 in the OKG and placebo group, respectively (p=0.62). There were no significant differences in 24-h nitrogen excretion data between groups, neither at separate time points, in overall values (p=0.40) nor in development over time (p=0.53). Conclusions A single oral dose of OKG did not affect urea production rate differently than the alanine placebo. OKG supplementation led to a temporary increase in nitrogen excretion, as did administration of placebo.

OBJECTIVE: Longitudinal comparison of prescribed energy, actually administered energy, and energy expenditure (EE) predicted by Schofield's equations to actual EE, as determined by daily indirect calorimetry measurements in critically ill children during the first 7 days following admission.DESIGN: Observational study.SETTING: Pediatric intensive care unit, high and medium care wards, in a university hospital.PATIENTS: Forty-six mechanically ventilated and spontaneously breathing infants and children (0-18 yrs) who were admitted with sepsis or following major abdominal or thoracic surgery or trauma.INTERVENTIONS: Daily indirect calorimetry measurements and assessment of energy balance.MEASUREMENTS AND MAIN RESULTS: Energy balance studies were performed for a total of 298 admission days in 13 sepsis, 27 surgery, and 6 trauma patients. Indirect calorimetry measurements were performed on 89% of the days. Mean measured EE was 44.6 +/- 15 kcal/kg.d and equaled predicted EE (44.2 +/- 12 kcal/kg.d; p = .56). Measured EE did not change over time, neither overall nor in diagnostic subgroups. Overall, median (range) administered energy was 31.1 (0-119) kcal/kg.d, which was significantly lower than measured EE (p < .001) and predicted EE (p < .001). Patients were underfed on 60% of days and overfed on 28% of days. Administered energy rose significantly in the course of admission, independently of diagnostic category, and did not differ from prescribed energy (p = .42). Energy intake was significantly higher in sepsis patients than in surgery and trauma patients during the whole course of the study (p < .01). The cumulative energy balance was positive only in sepsis patients. The administration of parenteral feeding was the single significant factor determining energy intake in mixed-effect modeling.CONCLUSIONS: Measured EE was stable and not significantly different from predicted values over the course of hospitalization. Underfeeding was frequently present and mainly due to prescription and administration of energy amounts inferior to measured EE values in enterally fed patients.

AIMS: To assess validity and reliability of energy expenditure measurements with a short Douglas bag protocol compared to the standard metabolic monitor in a paediatric intensive care setting.METHODS: 51 paired measurements were performed in 14 ventilated patients (age 0-18 years) with sepsis, trauma or following major surgery. Measured data were compared mutually and compared to Schofield equations using Bland-Altman analysis.RESULTS: Comparing Douglas bag (3.21 +/- 1.43 MJ/day) and metabolic monitor (3.15 +/- 1.49 MJ/day) we found bias in energy expenditure of -0.06 (equal to -2%, NS) with limits of agreement of -0.5 to 0.4 MJ/day (equal to -16% to +13%). Intra-measurement variability (coefficient of variation) was within 10% for both methods. Both the metabolic monitor and Douglas bag showed significant bias compared to Schofield equations (3.39 +/-1.64 MJ/day) of -7% (P < 0.01) and -5% (P < 0.05), respectively, with wide limits of agreement: metabolic monitor vs. Schofield: -37% to +22%, Douglas bag vs. Schofield: -37% to +26%.CONCLUSIONS: The Douglas bag method compared favourably to the metabolic monitor where Schofield equations failed to predict individual energy expenditure. Considering its low cost, this renders the short and simple Douglas bag method a robust measure and a routinely applicable instrument for tailored nutritional assessment in critically ill children.

We describe a GC-MS and GC-c-IRMS method for the determination of labeled urea tracer enrichments in plasma as a result of combined 13C- and 15N(2)-urea infusion experiments in piglets. Urea was converted into 2-methoxypyrimidine, a stable derivative, suited for analyses by both GC-MS and GC-c-IRMS. Using calibration curves for the respective working ranges (13C-urea: 0-1% APE; 15N(2)-urea: 0-7% MPE) enrichments were established in single point measurements; for 15N(2)-urea as values+/-0.15% MPE (95% confidence interval); for 13C-urea as values+/-0.02% APE (95% confidence interval). 15N(1)-urea enrichments were determined by measurement of the same sample with GC-c-IRMS and GC-MS. Subtraction of the 13C specific GC-c-IRMS data from the nondiscriminating GC-MS data for the sum of 13C- and 15N(1)-urea resulted in 15N(1)-urea enrichments+/-0.15% MPE (95% confidence interval). Application of the method in a combined 13C-urea bolus and 15N(2)-urea primed constant infusion experiment in piglet was demonstrated.

Background & aims: An oral [13C]urea protocol may provide a simple method for measurement of urea production. The validity of single pool calculations in relation to a reduced sampling protocol was assessed. Methods: In eight fed and five fasted piglets, plasma urea enrichments from a 10 h sampling protocol were measured following an intragastric [13C]urea bolus. Blood [13C]bicarbonate was measured to trace gut [13C]urea oxidation. Two-compartment and regression (single pool) computations were performed. Pool sizes were compared to urea distribution over total body water (TBW). Shorter protocol duration was tested in regression simulations. Results: Differences in urea kinetics between fed and fasted piglets did not reach statistical significance. Mean (±SE) urea pool from TBW times plasma urea concentration was 2.2±0.16 mmol kg−1. Two-compartment modelling yielded similar results for pool size (despite the oxidation of a small amount of urea tracer). Urea appearance rate was 306±18 μmol kg−1 h−1. Regression calculations overestimated urea appearance rate vs. compartmental model (). When samples

OBJECTIVE: To study current strategies in nutritional management of pediatric intensive care units (PICUs) in Europe, focusing on energy requirements.DESIGN AND SETTING: Survey by a 35-item questionnaire sent to representatives of 242 PICUs in 28 countries. Addresses were obtained from national PICU associations and the members' list of the European Society of Pediatric and Neonatal Intensive Care.PARTICIPANTS: Staff members of 111 European PICUs (46%) from 24 countries.MEASUREMENTS AND RESULTS: Predominantly physicians were reported to be responsible for nutritional support. In 73% of PICUs a multidisciplinary nutritional team was available. In most PICUs daily energy requirements were estimated using weight, age, predictive equations and correction factors. In 17% of PICUs energy expenditure was regularly measured by indirect calorimetry. Nutritional status was mostly assessed by weight, physical examination, and a wide range of biochemical blood parameters. Approximately 70% of PICUs used dedicated software for nutritional support. A similar percentage of PICUs regarded "nutrition" as a research topic and part of the residents' training program.CONCLUSIONS: Most European PICUs regard nutritional support as an important aspect of patient care, as shown by the presence of nutritional teams, software, research, and education. However, energy requirements of pediatric intensive care patient were based predominantly on estimations rather than on measurements.