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Biomedical subjects

O A Bodamer

Publications and source records attributed to O A Bodamer.

33 records · Page 2Linked to original sources
PubMed2000

Resting energy expenditure in disorders of propionate metabolism.

OBJECTIVES: During intercurrent illness children with methylmalonic acidemia were found to have increased resting energy expenditure (REE). We measured REE in children with disorders of propionate metabolism (methylmalonic and propionic acidemia) when they were well and compared the values with those predicted by the Schofield equation. STUDY DESIGN: Prospective study in tertiary care facility. REE was measured with open-circuit indirect calorimetry under standardized conditions. Predicted REE values were calculated with the Schofield equation. Fourteen subjects with propionic acidemia (n = 3) and methylmalonic acidemia (n = 11) were studied. RESULTS: The median REE was 690 kcal/d (range 186 to 1687 kcal/d), which is significantly reduced, representing 80% +/- 18% of that predicted by the Schofield height and weight equation (P <.01). REE was significantly lower in female compared with male patients for unknown reasons. There were no differences with age or neurologic state. REE was not further reduced in those with chronic renal failure. CONCLUSION: REE in patients with disorders of propionate metabolism is reduced when they are well.

Amino Acid Metabolism, Inborn Errors↗
PubMed2000

Measurement of carbon dioxide production in very low birth weight babies.

BACKGROUND: CO2 production is most commonly measured by using indirect calorimetry to quantify elimination of CO(2) in breath (VCO2). An alternative is to measure the rate at which CO2 appears in the body pool (RaCO2) by infusing a (13)C labelled bicarbonate tracer. VCO2 and RaCO2 generally differ but are related by c, a factor that adjusts for the incomplete recovery of infused tracer in the breath. The literature relating to human studies cites a wide range of values for c but the only neonatal study to determine c empirically estimated a mean value of 0.77. AIM: To estimate fractional recovery rate, c, in very low birthweight babies, and assess the feasibility of using the isotopic technique to measure CO2 production during mechanical ventilation. METHOD: Eleven spontaneously breathing, continuously fed, very low birthweight infants (median birth weight 1060 g, median gestational age 29 weeks) were studied. RESULTS: Mean (SD) VCO2 was 9.0 (2.0) ml/min (standard temperature and pressure dry, STPD) and mean (SD) RaCO2 was 9.6 (2.1) ml/min (STPD). The mean (SD) value of c was estimated as 0.95 (0.13). The 95% confidence intervals of the mean were 0.87-1.03. CONCLUSIONS: The results emphasise the importance of measuring c for a given study population rather than assuming a value based on adult studies. The close approximation of RaCO2 and VCO2 in this group of babies implies that the labelled bicarbonate infusion technique could be used to measure simply CO2 production during mechanical ventilation.

Breath Tests↗
PubMed1998

Effects of fish oil supplementation on apolipoprotein B100 production and lipoprotein metabolism in normolipidaemic males.

OBJECTIVE: To evaluate the effects of four weeks of fish oil supplementation on apolipoprotein B100 production and lipoprotein metabolism in normolipidaemic males. DESIGN AND SUBJECTS: Very low density lipoprotein (VLDL) apolipoprotein B100 (apoB100) kinetics in ten healthy, white males, aged 22-43 y (mean 32 y) were investigated using 13C-leucine technique and gas chromatography-mass spectrometry before and after fish oil supplementation. INTERVENTION: All subjects received 10 g (1.8 g EPA, 1.2 g DHA)/d of fish oil concentrate for four weeks. RESULTS: Fish oil supplementation resulted in a decrease of total plasma VLDL (mean +/- s.d. 1.11 +/- 0.41 vs 0.87 +/- 0.28 mmol/l, P < 0.05) and triacylglycerol concentrations (0.74 +/- 0.27) vs 0.48 +/- 0.21 mmol/l, P < 0.01). VLDL apoB100 pool size was decreased without alteration of the fractional synthetic rate but a significant decrease of apoB100 production (2.23 +/- 0.90 vs 1.54 +/- 0.52 mg/dl/h, P < 0.02). Following fish oil supplementation plasma concentrations of glucose and insulin as well as lipoprotein and hepatic lipase activities were unchanged. Fasting plasma concentrations of non-esterified fatty acid (NEFA) were decreased (0.45 +/- 0.12 vs 0.33 +/- 0.10 mmol/l, P < 0.05). CONCLUSIONS: Dietary supplementation with fish oil in healthy males results in decreased VLDL-triacylglycerol concentrations through a decrease in VLDL particle synthesis. The decrease in NEFA substrate supply also contributes.

Adult↗
PubMed1997

Assessment of energy expenditure in metabolic disorders.

The assessment of energy expenditure is valuable for the management of children with various conditions such as obesity and failure to thrive. Total daily energy expenditure (TDEE) includes resting energy expenditure (REE), energy expenditure during physical activity, dietary thermogenesis and growth. TDEE can be assessed by using the double-labelled water technique, but it has complex pitfalls and potential sources of errors and is impractical for everyday use. As REE is a substantial part of TDEE (65%-70%) and computerised indirect calorimeters have become recently available, this non-invasive, relatively cheap and easy to use technique is valuable for the assessment of short-term changes in energy metabolism. This can be used to assess REE of children with inborn errors of metabolism, whilst well and during episodes of metabolic decompensation and therefore to accurately determine energy intake.

Calorimetry, Indirect↗
PubMed1997

Measurement of glucose turnover--implications for the study of inborn errors of metabolism.

Aspects of glucose metabolism have been investigated quantitatively employing stable isotopes for 20 year. Use of non-recycling [6,6-2H2] or [U-13C] glucose labels provides a value for total hepatic glucose production (glycogenolysis plus gluconeogenesis). Quantitation of gluconeogenesis with isotopic tracers has itself recently been revisited employing protocols and analytical options that purport to overcome the isotope exchanges (dilution) experienced at the level of oxaloacetate when the rate of incorporation of label into glucose from infused alanine, lactate or pyruvate is monitored. Labelled glucose has been employed to investigate metabolic disturbances of glycogen storage disease type I (GSD-I) and type III (GSD-III). Endogenous glucose production and glucose recycling have been studied in both these storage diseases employing a primed-continuous infusion of D-[U-13C] glucose and quantitation of isotope enrichments and isotopomer distribution observed in plasma glucose either by mass spectrometry or nuclear magnetic resonance spectroscopy. General aspects of glucose metabolism as investigated with stable isotopes are presented.

Fructose Intolerance↗
PubMed1997

Dietary treatment in late-onset acid maltase deficiency.

Late-onset acid maltase deficiency or glycogen storage disease type II (GSD II) is a rare disorder of intralysosomal glycogen metabolism, resulting in progressive myopathy that is secondary to increased muscle protein breakdown. Stable isotope studies in the postabsorptive state have confirmed that mean protein breakdown in GSD II is increased by 31% compared to control subjects. 6.86 versus 4.69 g/kg per day, that mean protein balance is reduced is GSD II -1.32 versus -1.06 g/kg per day. Indirect calorimetry has demonstrated an increase in mean resting energy expenditure in GSD II, 41.8 versus 31.2 kcal/kg per day. Compliance following the introduction of a high-protein diet is often poor due to the large quantities of protein necessary and to the high caloric intake with the consequent weight gain. Only 25% of all reported subjects with GSD II showed an improvement of muscle or respiratory function after a high-protein diet. Careful evaluation of the underlying pathophysiological changes in GSD II is necessary to develop more logical and therefore more beneficial forms of dietary treatment.

Adolescent↗
PubMed1997

Protein turnover in critically ill children.

There is a paucity of data documenting the metabolic response to catabolic stress in childhood in general and about protein turnover in critically ill children in particular. Despite a high overall morbidity and mortality rate there is little information on which to base decisions to improve the management either by dietary therapy or by use of growth factors. Protein turnover is a key metabolic process that significantly alters during the catabolic state. Protein kinetics are easy to quantify using various stable isotope models, with some having advantages in the critically ill child. The 1-13C leucine technique is the most widely used and best validated model to date, requiring accurate estimation of CO2 production. There is also uncertainty about the bicarbonate kinetics and pool sizes in ventilated children whose respiratory function is severely impaired. The value of the 15N glycine (end product) technique is more limited because the time to achieve isotopic equilibrium is lengthy and considerable concerns about the validity of the model exist. The ring-D5 phenylalanine technique has the advantage of not requiring the measurement of CO2 production or 13C enrichment, but the model has not yet been validated in critically ill children. Despite it is of obvious value to measure protein turnover, few studies in critically ill children have been done.

Adolescent↗