Veronique Anne Pelletier - Academia.edu (original) (raw)

Papers by Veronique Anne Pelletier

PLOS Global Public Health

Food insecurity (FI) is at a crisis level in some Indigenous communities and impacts many of the ... more Food insecurity (FI) is at a crisis level in some Indigenous communities and impacts many of the half million First Nations Inuit and Métis (FNIM) children across Canada, particularly in isolated northern communities. This can lead to malnutrition and can have significant impacts on the physical, intellectual, emotional and social development of a child, often with lasting effects across the life course. This is a narrative review article with extensive search of the medical literature with input from the FNIM National organizations. The primary cause of FI is an imbalance between the high price of food relative to household income, where poverty is a driving factor. The cost and lack of availability to healthy foods has resulted in a transition to unhealthy market foods. Food security programs need to be prioritized, multi-faceted and multi-tiered within a framework of food sovereignty. Translational science, research, to practice is also important. The use of successful Indigenous...

Paediatrics & Child Health, 2017

Paediatrics & Child Health, 2017

While tobacco is sacred in many Indigenous cultures, the recreational misuse of commercial tobacc... more While tobacco is sacred in many Indigenous cultures, the recreational misuse of commercial tobacco is highly addictive and harmful. Tobacco misuse is the leading preventable cause of premature death in the world. Smoking rates among Canadian Indigenous youth are at least three times higher than for their non-Aboriginal peers, an alarming statistic on many levels. The tolls on health from extensive tobacco use range from disproportionately high individual mortality and morbidity to heavy socioeconomic burdens on Indigenous communities. Paediatric health care providers are uniquely positioned to collaborate with community stakeholders to prevent and treat tobacco misuse in young people and their families, while understanding the cultural value of tobacco for many Indigenous peoples. Targeted interventions can positively impact length and quality of life, improve overall health and decrease the immense social and human costs of tobacco misuse.

American Journal of Preventive Cardiology

Kidney International, Sep 1, 1979

It has been shown that during nephrotoxic serum nephritis (NSN) there is an important increase of... more It has been shown that during nephrotoxic serum nephritis (NSN) there is an important increase of urinary antigens released from the glomerular basement membrane (GBM) in the rabbit [1, 2] and in the rat [3]. This suggests a lysis of the GBM which appears to be due to enzymes from the lysosomes of activated polymorphonuclear leukocytes [1]. The GBM is composed, in part, of a collagen-like glycoprotein that contains a high percentage of disaccharide units a-o-glucopyranosyl-1-2-/3-i-galactopyranosyl-l-O-hydroxylysine (GIc-Gal-Hyl) [4], characteristic of collagen. In the rat, this percentage is 6% of the dry weight of the GBM [5]. Thus, it was interesting to confirm the lysis of the GBM during NSN and to determine if the disaccharide units were affected by the phenomenon using a biochemical assay of Glc-Gal-Hyl and its derivatives, Gal-Hyl and Hyl, in urine. This assay could be of interest for evaluating the degree of GBM destruction in human glomerulonephritis. The confirmation of an increase in disaccharide unit excretion raised the question of whether the biosynthesis of these units was increased in the gbmerular cells to replace the lysed material. This was studied by measuring in isolated gbomeruli the activity of the specific glucosyl transferase catalyzing the reaction [6]: Gal-Hyl + UDP glucose-Glc-Gal-Hyl + UDP Results indicate that biosynthesis of the GBM is certainly of importance in the procedure of healing of the GBM in gbomerulonephritis. Methods Nephrotoxic serum nephritis. Kidney cortices were isolated from 40 Sprague-Dawley male rats (500 g body wt) sacrificed by decapitation. They were washed with 0.15 M sodium chloride at +4° C 329 which has been reported as being very rich in carbohydrates [7]. The sponge was hydrolyzed in 2 N sodium hydroxide, 20 hours at 105° C in poiypropylene tubes with screw caps at a concentration of 15 mg/ml. After neutralization, the hydrolysate was put onto Dowex SOWX4 200-to 400-mesh (140

Clinical Biochemistry, Dec 1, 2008

The MTG-BT estimates the hydrolysis of triacyl-glycerols by pancreatic lipase, and appears attrac... more The MTG-BT estimates the hydrolysis of triacyl-glycerols by pancreatic lipase, and appears attractive for monitoring exogenous lipase requirements in patients with exocrine pancreatic insufficiency. To assess the test's discrimination capacity and repeatability, 9 CF patients with PERT and 10 healthy children underwent the (13)C-MTG-BT twice, at a 2- to 4-week interval. The test distinguished well between patients with severe exocrine pancreatic insufficiency (SEPI) and healthy subjects. However, within-subject variability for postprandial per thousand(13)C-enrichment and postprandial % dose recovery (PDR) was high in both groups. Therefore, the (13)C-MTG-BT seems useful to distinguish between SEPI and normal exocrine pancreatic function, but requires further development to improve its repeatability.

Journal of Nutrition, Dec 1, 1989

The purpose of this paper is to attempt to define the upper limit of the safe range of protein in... more The purpose of this paper is to attempt to define the upper limit of the safe range of protein intake, with particular reference to the protein content of prepared fixed-formulas used for feeding healthy, term infants. For discussion purposes we use the current upper limit proposed by the U.S. Food and Drug Administration (FDA), namely, 4.5 g protein per 100 kcal, as our initial reference level. To help reach a conclusion, the concept and definitions of nutritional adaptation and accommodation are considered, followed by a brief review of selected studies in full-term infants fed varying levels of protein intake. Based on growth and blood biochemical data, principally plasma free amino acid levels, we conclude that the currently proposed FDA upper limit is probably too high. The available data support a recommendation for lowering the value to about 3.5 g of protein per 100 kcal. Concerns for renal solute load (RSL) may require a further, desirable refinement in the value proposed. Indeed RSL should perhaps serve as the primary basis for establishing a rational and safe upper limit for the protein content of prepared fixed-formula diets for the very young, healthy infant.

The Journal of Pediatrics, 1986

Four infants had noninfectious intractable diarrhea, vomiting, anasarca, hepatomegaly, hypoglycem... more Four infants had noninfectious intractable diarrhea, vomiting, anasarca, hepatomegaly, hypoglycemia, and malnutrition within the first 3 months of life. Their parents originated from the same Northeastern part of Quebec, and consanguinity was found in two kindreds. Diarrhea was secretory in three infants (mean stool volume 87 ml/kg/day, Na + 108 mEq/L, CI-85 mEq/L). Hypoalbuminemia (mean 2.0 gm/dl), present in all infants, appeared to be secondary to a protein-losing enteropathy, which was documented in two infants. Histologic examination of the upper small intestine showed only mild to moderate villous atrophy. The remarkable findings were those of cystic dilation of the crypts and acute inflammation of crypts and lamina propria, all of which were most prominent in the colon and terminal ileum; the changes were progressive over time. Mild lymphangiectasia was found in all of the patients. Congenital hepatic fibrosis, present in all, was associated in one patient with a nonfunctional multicystic kidney. Prolonged total parenteral nutrition, intravenously administered albumin, antisecretory agents, and antibiotics were unsuccessful in controlling the disease. Although a total colectomy was followed by a temporary decrease in stool output and normalization of serum albumin concentration in one infant, the patients died between 4 and 21 months of age.

Pediatric Research, Apr 1, 1985

The American Journal of Clinical Nutrition, 1993

The fate of dietary leucine and phenylalanine was studied in five healthy, young adult men, by us... more The fate of dietary leucine and phenylalanine was studied in five healthy, young adult men, by using a dual, stable isotope-tracer infusion approach to estimate amino acid fluxes, splanchnic (Sp) uptake, and dietary of absorbed amino acid to the peripheral circulation. Subject received two, 4-h tracer infusions of [1-13C]leucine and [15N]phenylalanine infused through a feeding tube placed in the duodenum, and [5,5,5-2H3]leucine, [ring-2H5]phenylalanine, and [6,6-2H2]glucose infused simultaneously by vein. In one experiment subjects received an amino acid mixture (83 mg amino acid.kg-1.h-1) via the feeding tube and in the other experiment amino acids were supplied with carbohydrate (CHO) (167 mg.kg-1.h-1). Sp uptake of dietary leucine decreased with added dietary CHO (29% of ingested leucine for amino acids alone vs 20% with CHO; P < 0.05) but was not different for phenylalanine (P > 0.05). Addition of CHO decreased both release of leucine via protein breakdown and leucine oxidation and increased body leucine balance (P < 0.05).

The American Journal of Clinical Nutrition, 1991

Recent estimates of the leucine requirement ofadult men based an '3C-tracer studies are substanti... more Recent estimates of the leucine requirement ofadult men based an '3C-tracer studies are substantially higher than those proposed by FAO/WHO/UNU (1985). To explore whether leucine oxidation and requirements are affected by the dietary amount ofvaline, 1 1 healthy young adult men received, in random order, for 5 d, one offour L-amino acid diets providing 40 or 1 5 mg leucine kg 'd ' together with variable amounts mgkg d of valine and isoleucine in the following cambinations (Val:Ile): 80:62 and 20:62 (six subjects; phase 1); 20:62 and 20:20 (five subjects, phase 2). On the morning of day 6, a continuous intravenous infusion of L-[l-'3C]leucine was given for 7-8 h; the subject was in the fasting state for the initial 2.5 or 3 h and in the fed state for the remainder of the time. Also, [2H3]leucine was added to the diet. Leucine oxidation was similar for all diet groups in the fasted state. During the fed state, leucine oxidation was not affected by the Val:Ile pattern. Thus, changes in the pattern of branched-chain amino acid intake within a physiological range do not affect isotopically derived estimates of the leucine requirement.

The Journal of Pediatrics, 2003

To study the effectiveness of dimethylglycine (DMG) on oxygen consumption (VO(2)) in children wit... more To study the effectiveness of dimethylglycine (DMG) on oxygen consumption (VO(2)) in children with Saguenay-Lac-Saint-Jean cytochrome-c oxidase (SLSJ-COX) deficiency (OMIM 220111). In a crossover randomized double-blind clinical trial, 5 children with SLSJ-COX deficiency, who were stable and old enough to comply with VO(2) measurement, were treated with placebo or DMG for 3 days, and with the alternate treatment after a 2-week washout period. VO(2) was measured by indirect calorimetry before and after treatment. Dietary caloric intake was calculated for 3 days before each measurement. Mean caloric intakes per day were 1562 and 1342 kcal x m(-2) before and during placebo, 1,336 and 1,380 before and during DMG, respectively. DMG was well tolerated and, in all cases, resulted in markedly increased blood DMG levels (617 + 203 mmol x L(-1)), versus 0 to 2 mmol x L(-1) without treatment. Mean VO(2) was lower after administration of either DMG (-1 +/- 3 mL x min(-1) x m(-2)) or placebo (-6 +/- 4), but neither difference was statistically significant. There was no detectable effect of DMG treatment on blood levels of lactate, pyruvate, bicarbonate, or pH. VO(2) values of patients (range, 101-135 mL x min(-1) x m(-2)) were lower than published norms (150-160 mL x min(-1) x m(-2)). This study suggests that treatment with DMG does not substantially change VO(2) in children with SLSJ-COX deficiency.

Journal of Medical Genetics, 2002

Clinical Infectious Diseases, 2003

Kidney International, 1979

It has been shown that during nephrotoxic serum nephritis (NSN) there is an important increase of... more It has been shown that during nephrotoxic serum nephritis (NSN) there is an important increase of urinary antigens released from the glomerular basement membrane (GBM) in the rabbit [1, 2] and in the rat [3]. This suggests a lysis of the GBM which appears to be due to enzymes from the lysosomes of activated polymorphonuclear leukocytes [1]. The GBM is composed, in part, of a collagen-like glycoprotein that contains a high percentage of disaccharide units a-o-glucopyranosyl-1-2-/3-i-galactopyranosyl-l-O-hydroxylysine (GIc-Gal-Hyl) [4], characteristic of collagen. In the rat, this percentage is 6% of the dry weight of the GBM [5]. Thus, it was interesting to confirm the lysis of the GBM during NSN and to determine if the disaccharide units were affected by the phenomenon using a biochemical assay of Glc-Gal-Hyl and its derivatives, Gal-Hyl and Hyl, in urine. This assay could be of interest for evaluating the degree of GBM destruction in human glomerulonephritis. The confirmation of an increase in disaccharide unit excretion raised the question of whether the biosynthesis of these units was increased in the gbmerular cells to replace the lysed material. This was studied by measuring in isolated gbomeruli the activity of the specific glucosyl transferase catalyzing the reaction [6]: Gal-Hyl + UDP glucose-Glc-Gal-Hyl + UDP Results indicate that biosynthesis of the GBM is certainly of importance in the procedure of healing of the GBM in gbomerulonephritis. Methods Nephrotoxic serum nephritis. Kidney cortices were isolated from 40 Sprague-Dawley male rats (500 g body wt) sacrificed by decapitation. They were washed with 0.15 M sodium chloride at +4° C 329 which has been reported as being very rich in carbohydrates [7]. The sponge was hydrolyzed in 2 N sodium hydroxide, 20 hours at 105° C in poiypropylene tubes with screw caps at a concentration of 15 mg/ml. After neutralization, the hydrolysate was put onto Dowex SOWX4 200-to 400-mesh (140

Clinical Biochemistry, 2008

The MTG-BT estimates the hydrolysis of triacyl-glycerols by pancreatic lipase, and appears attrac... more The MTG-BT estimates the hydrolysis of triacyl-glycerols by pancreatic lipase, and appears attractive for monitoring exogenous lipase requirements in patients with exocrine pancreatic insufficiency. To assess the test's discrimination capacity and repeatability, 9 CF patients with PERT and 10 healthy children underwent the (13)C-MTG-BT twice, at a 2- to 4-week interval. The test distinguished well between patients with severe exocrine pancreatic insufficiency (SEPI) and healthy subjects. However, within-subject variability for postprandial per thousand(13)C-enrichment and postprandial % dose recovery (PDR) was high in both groups. Therefore, the (13)C-MTG-BT seems useful to distinguish between SEPI and normal exocrine pancreatic function, but requires further development to improve its repeatability.

The Journal of Pediatrics, 1986

Four infants had noninfectious intractable diarrhea, vomiting, anasarca, hepatomegaly, hypoglycem... more Four infants had noninfectious intractable diarrhea, vomiting, anasarca, hepatomegaly, hypoglycemia, and malnutrition within the first 3 months of life. Their parents originated from the same Northeastern part of Quebec, and consanguinity was found in two kindreds. Diarrhea was secretory in three infants (mean stool volume 87 ml/kg/day, Na + 108 mEq/L, CI-85 mEq/L). Hypoalbuminemia (mean 2.0 gm/dl), present in all infants, appeared to be secondary to a protein-losing enteropathy, which was documented in two infants. Histologic examination of the upper small intestine showed only mild to moderate villous atrophy. The remarkable findings were those of cystic dilation of the crypts and acute inflammation of crypts and lamina propria, all of which were most prominent in the colon and terminal ileum; the changes were progressive over time. Mild lymphangiectasia was found in all of the patients. Congenital hepatic fibrosis, present in all, was associated in one patient with a nonfunctional multicystic kidney. Prolonged total parenteral nutrition, intravenously administered albumin, antisecretory agents, and antibiotics were unsuccessful in controlling the disease. Although a total colectomy was followed by a temporary decrease in stool output and normalization of serum albumin concentration in one infant, the patients died between 4 and 21 months of age.

e-SPEN, the European e-Journal of Clinical Nutrition and Metabolism, 2009

Background & aims: As critically ill children are at high risk of malnutrition, nutritional statu... more Background & aims: As critically ill children are at high risk of malnutrition, nutritional status at admission, prescribed and delivered calories and factors confounding the actual intake were assessed in a pediatric intensive care unit (PICU). Methods: Mechanically ventilated children with an expected PICU stay !72 h were included from May 1 to August 1, 2005. Anthropometric measurements, prescribed and delivered calories and the main reasons for feeding interruptions were prospectively recorded. Main results: 10/49 patients (20%) showed moderate or severe malnutrition at admission. Median estimated energy requirements (EER) per patient was 90 kcal/kg/day (95% CI: 82;90), prescribed calories were 75 kcal/kg/day (95% CI: 45;98), and actual delivered calories were 58 kcal/kg/day (95% CI: 35;85). Full EER was prescribed in 50% of the patients within almost 6 days and actually delivered within 9 days. A difference !10 kcal/kg/day between prescribed and delivered calories was observed on 165/576 recorded days (29%). The major barriers to the provision of prescribed enteral nutrition were procedural interruptions. Conclusions: Despite a high prevalence of malnutrition, EER are not achieved partially due to enteral feeding interruptions for procedures during PICU stay. Physicians are challenged to address the nondelivery of prescribed nutrition.

The Journal of nutrition, 1989

The purpose of this paper is to attempt to define the upper limit of the safe range of protein in... more The purpose of this paper is to attempt to define the upper limit of the safe range of protein intake, with particular reference to the protein content of prepared fixed-formulas used for feeding healthy, term infants. For discussion purposes we use the current upper limit proposed by the U.S. Food and Drug Administration (FDA), namely, 4.5 g protein per 100 kcal, as our initial reference level. To help reach a conclusion, the concept and definitions of nutritional adaptation and accommodation are considered, followed by a brief review of selected studies in full-term infants fed varying levels of protein intake. Based on growth and blood biochemical data, principally plasma free amino acid levels, we conclude that the currently proposed FDA upper limit is probably too high. The available data support a recommendation for lowering the value to about 3.5 g of protein per 100 kcal. Concerns for renal solute load (RSL) may require a further, desirable refinement in the value proposed. ...

PLOS Global Public Health

Food insecurity (FI) is at a crisis level in some Indigenous communities and impacts many of the ... more Food insecurity (FI) is at a crisis level in some Indigenous communities and impacts many of the half million First Nations Inuit and Métis (FNIM) children across Canada, particularly in isolated northern communities. This can lead to malnutrition and can have significant impacts on the physical, intellectual, emotional and social development of a child, often with lasting effects across the life course. This is a narrative review article with extensive search of the medical literature with input from the FNIM National organizations. The primary cause of FI is an imbalance between the high price of food relative to household income, where poverty is a driving factor. The cost and lack of availability to healthy foods has resulted in a transition to unhealthy market foods. Food security programs need to be prioritized, multi-faceted and multi-tiered within a framework of food sovereignty. Translational science, research, to practice is also important. The use of successful Indigenous...

Paediatrics & Child Health, 2017

Paediatrics & Child Health, 2017

While tobacco is sacred in many Indigenous cultures, the recreational misuse of commercial tobacc... more While tobacco is sacred in many Indigenous cultures, the recreational misuse of commercial tobacco is highly addictive and harmful. Tobacco misuse is the leading preventable cause of premature death in the world. Smoking rates among Canadian Indigenous youth are at least three times higher than for their non-Aboriginal peers, an alarming statistic on many levels. The tolls on health from extensive tobacco use range from disproportionately high individual mortality and morbidity to heavy socioeconomic burdens on Indigenous communities. Paediatric health care providers are uniquely positioned to collaborate with community stakeholders to prevent and treat tobacco misuse in young people and their families, while understanding the cultural value of tobacco for many Indigenous peoples. Targeted interventions can positively impact length and quality of life, improve overall health and decrease the immense social and human costs of tobacco misuse.

American Journal of Preventive Cardiology

Kidney International, Sep 1, 1979

It has been shown that during nephrotoxic serum nephritis (NSN) there is an important increase of... more It has been shown that during nephrotoxic serum nephritis (NSN) there is an important increase of urinary antigens released from the glomerular basement membrane (GBM) in the rabbit [1, 2] and in the rat [3]. This suggests a lysis of the GBM which appears to be due to enzymes from the lysosomes of activated polymorphonuclear leukocytes [1]. The GBM is composed, in part, of a collagen-like glycoprotein that contains a high percentage of disaccharide units a-o-glucopyranosyl-1-2-/3-i-galactopyranosyl-l-O-hydroxylysine (GIc-Gal-Hyl) [4], characteristic of collagen. In the rat, this percentage is 6% of the dry weight of the GBM [5]. Thus, it was interesting to confirm the lysis of the GBM during NSN and to determine if the disaccharide units were affected by the phenomenon using a biochemical assay of Glc-Gal-Hyl and its derivatives, Gal-Hyl and Hyl, in urine. This assay could be of interest for evaluating the degree of GBM destruction in human glomerulonephritis. The confirmation of an increase in disaccharide unit excretion raised the question of whether the biosynthesis of these units was increased in the gbmerular cells to replace the lysed material. This was studied by measuring in isolated gbomeruli the activity of the specific glucosyl transferase catalyzing the reaction [6]: Gal-Hyl + UDP glucose-Glc-Gal-Hyl + UDP Results indicate that biosynthesis of the GBM is certainly of importance in the procedure of healing of the GBM in gbomerulonephritis. Methods Nephrotoxic serum nephritis. Kidney cortices were isolated from 40 Sprague-Dawley male rats (500 g body wt) sacrificed by decapitation. They were washed with 0.15 M sodium chloride at +4° C 329 which has been reported as being very rich in carbohydrates [7]. The sponge was hydrolyzed in 2 N sodium hydroxide, 20 hours at 105° C in poiypropylene tubes with screw caps at a concentration of 15 mg/ml. After neutralization, the hydrolysate was put onto Dowex SOWX4 200-to 400-mesh (140

Clinical Biochemistry, Dec 1, 2008

The MTG-BT estimates the hydrolysis of triacyl-glycerols by pancreatic lipase, and appears attrac... more The MTG-BT estimates the hydrolysis of triacyl-glycerols by pancreatic lipase, and appears attractive for monitoring exogenous lipase requirements in patients with exocrine pancreatic insufficiency. To assess the test's discrimination capacity and repeatability, 9 CF patients with PERT and 10 healthy children underwent the (13)C-MTG-BT twice, at a 2- to 4-week interval. The test distinguished well between patients with severe exocrine pancreatic insufficiency (SEPI) and healthy subjects. However, within-subject variability for postprandial per thousand(13)C-enrichment and postprandial % dose recovery (PDR) was high in both groups. Therefore, the (13)C-MTG-BT seems useful to distinguish between SEPI and normal exocrine pancreatic function, but requires further development to improve its repeatability.

Journal of Nutrition, Dec 1, 1989

The purpose of this paper is to attempt to define the upper limit of the safe range of protein in... more The purpose of this paper is to attempt to define the upper limit of the safe range of protein intake, with particular reference to the protein content of prepared fixed-formulas used for feeding healthy, term infants. For discussion purposes we use the current upper limit proposed by the U.S. Food and Drug Administration (FDA), namely, 4.5 g protein per 100 kcal, as our initial reference level. To help reach a conclusion, the concept and definitions of nutritional adaptation and accommodation are considered, followed by a brief review of selected studies in full-term infants fed varying levels of protein intake. Based on growth and blood biochemical data, principally plasma free amino acid levels, we conclude that the currently proposed FDA upper limit is probably too high. The available data support a recommendation for lowering the value to about 3.5 g of protein per 100 kcal. Concerns for renal solute load (RSL) may require a further, desirable refinement in the value proposed. Indeed RSL should perhaps serve as the primary basis for establishing a rational and safe upper limit for the protein content of prepared fixed-formula diets for the very young, healthy infant.

The Journal of Pediatrics, 1986

Four infants had noninfectious intractable diarrhea, vomiting, anasarca, hepatomegaly, hypoglycem... more Four infants had noninfectious intractable diarrhea, vomiting, anasarca, hepatomegaly, hypoglycemia, and malnutrition within the first 3 months of life. Their parents originated from the same Northeastern part of Quebec, and consanguinity was found in two kindreds. Diarrhea was secretory in three infants (mean stool volume 87 ml/kg/day, Na + 108 mEq/L, CI-85 mEq/L). Hypoalbuminemia (mean 2.0 gm/dl), present in all infants, appeared to be secondary to a protein-losing enteropathy, which was documented in two infants. Histologic examination of the upper small intestine showed only mild to moderate villous atrophy. The remarkable findings were those of cystic dilation of the crypts and acute inflammation of crypts and lamina propria, all of which were most prominent in the colon and terminal ileum; the changes were progressive over time. Mild lymphangiectasia was found in all of the patients. Congenital hepatic fibrosis, present in all, was associated in one patient with a nonfunctional multicystic kidney. Prolonged total parenteral nutrition, intravenously administered albumin, antisecretory agents, and antibiotics were unsuccessful in controlling the disease. Although a total colectomy was followed by a temporary decrease in stool output and normalization of serum albumin concentration in one infant, the patients died between 4 and 21 months of age.

Pediatric Research, Apr 1, 1985

The American Journal of Clinical Nutrition, 1993

The fate of dietary leucine and phenylalanine was studied in five healthy, young adult men, by us... more The fate of dietary leucine and phenylalanine was studied in five healthy, young adult men, by using a dual, stable isotope-tracer infusion approach to estimate amino acid fluxes, splanchnic (Sp) uptake, and dietary of absorbed amino acid to the peripheral circulation. Subject received two, 4-h tracer infusions of [1-13C]leucine and [15N]phenylalanine infused through a feeding tube placed in the duodenum, and [5,5,5-2H3]leucine, [ring-2H5]phenylalanine, and [6,6-2H2]glucose infused simultaneously by vein. In one experiment subjects received an amino acid mixture (83 mg amino acid.kg-1.h-1) via the feeding tube and in the other experiment amino acids were supplied with carbohydrate (CHO) (167 mg.kg-1.h-1). Sp uptake of dietary leucine decreased with added dietary CHO (29% of ingested leucine for amino acids alone vs 20% with CHO; P < 0.05) but was not different for phenylalanine (P > 0.05). Addition of CHO decreased both release of leucine via protein breakdown and leucine oxidation and increased body leucine balance (P < 0.05).

The American Journal of Clinical Nutrition, 1991

Recent estimates of the leucine requirement ofadult men based an '3C-tracer studies are substanti... more Recent estimates of the leucine requirement ofadult men based an '3C-tracer studies are substantially higher than those proposed by FAO/WHO/UNU (1985). To explore whether leucine oxidation and requirements are affected by the dietary amount ofvaline, 1 1 healthy young adult men received, in random order, for 5 d, one offour L-amino acid diets providing 40 or 1 5 mg leucine kg 'd ' together with variable amounts mgkg d of valine and isoleucine in the following cambinations (Val:Ile): 80:62 and 20:62 (six subjects; phase 1); 20:62 and 20:20 (five subjects, phase 2). On the morning of day 6, a continuous intravenous infusion of L-[l-'3C]leucine was given for 7-8 h; the subject was in the fasting state for the initial 2.5 or 3 h and in the fed state for the remainder of the time. Also, [2H3]leucine was added to the diet. Leucine oxidation was similar for all diet groups in the fasted state. During the fed state, leucine oxidation was not affected by the Val:Ile pattern. Thus, changes in the pattern of branched-chain amino acid intake within a physiological range do not affect isotopically derived estimates of the leucine requirement.

The Journal of Pediatrics, 2003

To study the effectiveness of dimethylglycine (DMG) on oxygen consumption (VO(2)) in children wit... more To study the effectiveness of dimethylglycine (DMG) on oxygen consumption (VO(2)) in children with Saguenay-Lac-Saint-Jean cytochrome-c oxidase (SLSJ-COX) deficiency (OMIM 220111). In a crossover randomized double-blind clinical trial, 5 children with SLSJ-COX deficiency, who were stable and old enough to comply with VO(2) measurement, were treated with placebo or DMG for 3 days, and with the alternate treatment after a 2-week washout period. VO(2) was measured by indirect calorimetry before and after treatment. Dietary caloric intake was calculated for 3 days before each measurement. Mean caloric intakes per day were 1562 and 1342 kcal x m(-2) before and during placebo, 1,336 and 1,380 before and during DMG, respectively. DMG was well tolerated and, in all cases, resulted in markedly increased blood DMG levels (617 + 203 mmol x L(-1)), versus 0 to 2 mmol x L(-1) without treatment. Mean VO(2) was lower after administration of either DMG (-1 +/- 3 mL x min(-1) x m(-2)) or placebo (-6 +/- 4), but neither difference was statistically significant. There was no detectable effect of DMG treatment on blood levels of lactate, pyruvate, bicarbonate, or pH. VO(2) values of patients (range, 101-135 mL x min(-1) x m(-2)) were lower than published norms (150-160 mL x min(-1) x m(-2)). This study suggests that treatment with DMG does not substantially change VO(2) in children with SLSJ-COX deficiency.

Journal of Medical Genetics, 2002

Clinical Infectious Diseases, 2003

Kidney International, 1979

It has been shown that during nephrotoxic serum nephritis (NSN) there is an important increase of... more It has been shown that during nephrotoxic serum nephritis (NSN) there is an important increase of urinary antigens released from the glomerular basement membrane (GBM) in the rabbit [1, 2] and in the rat [3]. This suggests a lysis of the GBM which appears to be due to enzymes from the lysosomes of activated polymorphonuclear leukocytes [1]. The GBM is composed, in part, of a collagen-like glycoprotein that contains a high percentage of disaccharide units a-o-glucopyranosyl-1-2-/3-i-galactopyranosyl-l-O-hydroxylysine (GIc-Gal-Hyl) [4], characteristic of collagen. In the rat, this percentage is 6% of the dry weight of the GBM [5]. Thus, it was interesting to confirm the lysis of the GBM during NSN and to determine if the disaccharide units were affected by the phenomenon using a biochemical assay of Glc-Gal-Hyl and its derivatives, Gal-Hyl and Hyl, in urine. This assay could be of interest for evaluating the degree of GBM destruction in human glomerulonephritis. The confirmation of an increase in disaccharide unit excretion raised the question of whether the biosynthesis of these units was increased in the gbmerular cells to replace the lysed material. This was studied by measuring in isolated gbomeruli the activity of the specific glucosyl transferase catalyzing the reaction [6]: Gal-Hyl + UDP glucose-Glc-Gal-Hyl + UDP Results indicate that biosynthesis of the GBM is certainly of importance in the procedure of healing of the GBM in gbomerulonephritis. Methods Nephrotoxic serum nephritis. Kidney cortices were isolated from 40 Sprague-Dawley male rats (500 g body wt) sacrificed by decapitation. They were washed with 0.15 M sodium chloride at +4° C 329 which has been reported as being very rich in carbohydrates [7]. The sponge was hydrolyzed in 2 N sodium hydroxide, 20 hours at 105° C in poiypropylene tubes with screw caps at a concentration of 15 mg/ml. After neutralization, the hydrolysate was put onto Dowex SOWX4 200-to 400-mesh (140

Clinical Biochemistry, 2008

The MTG-BT estimates the hydrolysis of triacyl-glycerols by pancreatic lipase, and appears attrac... more The MTG-BT estimates the hydrolysis of triacyl-glycerols by pancreatic lipase, and appears attractive for monitoring exogenous lipase requirements in patients with exocrine pancreatic insufficiency. To assess the test's discrimination capacity and repeatability, 9 CF patients with PERT and 10 healthy children underwent the (13)C-MTG-BT twice, at a 2- to 4-week interval. The test distinguished well between patients with severe exocrine pancreatic insufficiency (SEPI) and healthy subjects. However, within-subject variability for postprandial per thousand(13)C-enrichment and postprandial % dose recovery (PDR) was high in both groups. Therefore, the (13)C-MTG-BT seems useful to distinguish between SEPI and normal exocrine pancreatic function, but requires further development to improve its repeatability.

The Journal of Pediatrics, 1986

Four infants had noninfectious intractable diarrhea, vomiting, anasarca, hepatomegaly, hypoglycem... more Four infants had noninfectious intractable diarrhea, vomiting, anasarca, hepatomegaly, hypoglycemia, and malnutrition within the first 3 months of life. Their parents originated from the same Northeastern part of Quebec, and consanguinity was found in two kindreds. Diarrhea was secretory in three infants (mean stool volume 87 ml/kg/day, Na + 108 mEq/L, CI-85 mEq/L). Hypoalbuminemia (mean 2.0 gm/dl), present in all infants, appeared to be secondary to a protein-losing enteropathy, which was documented in two infants. Histologic examination of the upper small intestine showed only mild to moderate villous atrophy. The remarkable findings were those of cystic dilation of the crypts and acute inflammation of crypts and lamina propria, all of which were most prominent in the colon and terminal ileum; the changes were progressive over time. Mild lymphangiectasia was found in all of the patients. Congenital hepatic fibrosis, present in all, was associated in one patient with a nonfunctional multicystic kidney. Prolonged total parenteral nutrition, intravenously administered albumin, antisecretory agents, and antibiotics were unsuccessful in controlling the disease. Although a total colectomy was followed by a temporary decrease in stool output and normalization of serum albumin concentration in one infant, the patients died between 4 and 21 months of age.

e-SPEN, the European e-Journal of Clinical Nutrition and Metabolism, 2009

Background & aims: As critically ill children are at high risk of malnutrition, nutritional statu... more Background & aims: As critically ill children are at high risk of malnutrition, nutritional status at admission, prescribed and delivered calories and factors confounding the actual intake were assessed in a pediatric intensive care unit (PICU). Methods: Mechanically ventilated children with an expected PICU stay !72 h were included from May 1 to August 1, 2005. Anthropometric measurements, prescribed and delivered calories and the main reasons for feeding interruptions were prospectively recorded. Main results: 10/49 patients (20%) showed moderate or severe malnutrition at admission. Median estimated energy requirements (EER) per patient was 90 kcal/kg/day (95% CI: 82;90), prescribed calories were 75 kcal/kg/day (95% CI: 45;98), and actual delivered calories were 58 kcal/kg/day (95% CI: 35;85). Full EER was prescribed in 50% of the patients within almost 6 days and actually delivered within 9 days. A difference !10 kcal/kg/day between prescribed and delivered calories was observed on 165/576 recorded days (29%). The major barriers to the provision of prescribed enteral nutrition were procedural interruptions. Conclusions: Despite a high prevalence of malnutrition, EER are not achieved partially due to enteral feeding interruptions for procedures during PICU stay. Physicians are challenged to address the nondelivery of prescribed nutrition.

The Journal of nutrition, 1989

The purpose of this paper is to attempt to define the upper limit of the safe range of protein in... more The purpose of this paper is to attempt to define the upper limit of the safe range of protein intake, with particular reference to the protein content of prepared fixed-formulas used for feeding healthy, term infants. For discussion purposes we use the current upper limit proposed by the U.S. Food and Drug Administration (FDA), namely, 4.5 g protein per 100 kcal, as our initial reference level. To help reach a conclusion, the concept and definitions of nutritional adaptation and accommodation are considered, followed by a brief review of selected studies in full-term infants fed varying levels of protein intake. Based on growth and blood biochemical data, principally plasma free amino acid levels, we conclude that the currently proposed FDA upper limit is probably too high. The available data support a recommendation for lowering the value to about 3.5 g of protein per 100 kcal. Concerns for renal solute load (RSL) may require a further, desirable refinement in the value proposed. ...