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Biomedical subjects

O Goulet

Publications and source records attributed to O Goulet.

At least 73 records · Page 4Linked to original sources

Short bowel syndrome in pediatric patients.

The treatment of infants and children with short bowel syndrome aims at restoring the intestinal continuity and at improving the physiological process of gut adaptation. Mucosal hyperplasia allows the remaining gut to ensure an adequate digestion and an absorption process leading to intestinal autonomy. During the period of adaptation, appropriate parenteral and/or enteral feeding must be directed at maintaining an optimal nutritional status. Delay of intestinal autonomy depends on the characteristics of the residual intestine: length, presence of the ileocecal valve and colon, and motor function. Bacterial overgrowth compromises intestinal adaptation and increases the risk of liver disorders. Few patients will remain long-term dependent on parenteral nutrition. All approaches aimed at achieving intestinal autonomy should be tried: use of trophic factors, intestinal tapering, and lengthening. In a few residual patients, permanent intestinal failure or extreme short bowel syndrome require intestinal transplantation.

Adaptation, Physiological↗

[Protein and energy needs of the infant with severe malnutrition. Application in a hospital environment for the treatment of malnutrition caused by deficient intake].

Severe malnutrition is defined by a weight for height below 70% of international standards or by presence of oedema in a clinically undernourished child. Severe malnutrition associated with oedema is called kwashiorkor. The origin of oedemas of kwashiorkor is still debated, but its relation with protein deficiency is strongly questioned. The same dietary management is now recommended for malnutrition with or without oedema. Present recommendations are based, as for well nourished children, on the separate estimation of nutritional requirements for maintenance and growth. Total requirements vary between 0.7 g/kg/day in the first few days of treatment to 5 g/kg/day or more when weight gain is maximum. As a result of high energy requirement during catch-up growth, protein requirements never exceed 10 to 12% of total energy needs.

Child Nutrition Disorders↗

Assessment of nutritional status in clinical practice.

As most diseases can have nutritional consequences, the assessment of nutritional status may help to detect an underlying disease, to identify nutritional disorders related to a given disease, and to quantify the impact of nutritional therapy. The aims and methods used for nutritional assessment depend on the circumstances in which nutritional assessment is performed. Whatever the context or aim, nutritional status is assessed through a simple, mainly clinical approach, based on the past history, dietary intake, auxological analysis, anthropometric measurements, body compartment and biological parameters. Accurate techniques for measuring body compartments are available in children such as dual-energy X-ray absorptiometry to assess fat body mass or bioelectrical impedance analysis for body water and lean body mass. Measuring energy expenditure allows for a more accurate monitoring of the patient's energy needs and decreases the risks associated with underfeeding or overfeeding. In clinical practice, the analysis should be longitudinal and take into account situations carrying a risk of malnutrition. Preventive use of nutritional assessment allows nutritional support to be introduced in a timely fashion, thereby avoiding morbidity/mortality and limiting the long-term impact of malnutrition on growth and development.

Absorptiometry, Photon↗

Nutritional support in malnourished paediatric patients.

An imbalance between a person's energy requirements and his or her dietary protein and caloric supply is the source of protein energy malnutrition (PEM), which compounds the problems of any underlying disease. Malnutrition may occur quite rapidly in critically ill patients, particularly those suffering from sepsis, setting up a vicious cycle with worsening of the PEM. This chapter examines the main consequences of PEM, the means whereby appropriate nutrition may be provided, and risks for severely malnourished paediatric patients in hospital. If the gastrointestinal tract can be used for refeeding, it should be used. When the gastrointestinal tract is unable to meet the protein and energy requirements, parenteral nutrition (PN) is required. PN is efficient but carries a high risk of metabolic complications known as the refeeding syndrome and directly related to the homeostatic changes secondary to severe PEM. Catch-up growth may be achieved by using appropriate nutritional support. Changes in body composition have to be assessed during the course of renutrition.

Body Composition↗

Home enteral and parenteral nutrition in children.

The prevalence of home enteral and parenteral nutrition programmes is rising rapidly all over the world, in children as in adults. Home artificial nutrition, especially parenteral nutrition, is an expensive technology but is life-saving for many patients. The only possible alternative to home treatment is keeping patients in hospital, and cost-benefit studies have demonstrated that home nutrition is about 70% more cost-effective than hospital-based therapy. Although home nutrition is usually considered by children and families to lead to an improvement in their quality of life, the complications of these techniques, including psychological consequences, have to be carefully assessed and prevented.

Adolescent↗

Anti-Saccharomyces cerevisiae mannan antibodies in familial Crohn's disease.

OBJECTIVE: Anti-Saccharomyces cerevisiae mannan antibodies (ASCA) are associated with Crohn's disease. The aim of this study was to determine the prevalence of ASCA in families in which at least two members were affected with Crohn's disease. METHODS: A total of 20 families including two (n=15) or more (n=5) patients with Crohn's disease were tested for ASCA with use of an ELISA method. Overall, 51 affected members, 66 healthy first degree relatives, and 163 healthy control subjects were studied. RESULTS: ASCA were detected in 35 of 51 (69%) patients with Crohn's disease and in 13 of 66 (20%) healthy relatives versus one of 163 healthy control subjects (p < 0.0001 and p < 0.001). ASCA-positive relatives were distributed in 12 of 20 families. ASCA were present in eight healthy parents and four healthy siblings. The prevalence of ASCA in relatives did not depend on the ASCA status of affected members. CONCLUSION: ASCA in 20% of healthy first degree relatives of patients with Crohn's disease suggest that these antibodies might be a subclinical marker for Crohn's disease in families. Whether ASCA reflect environmental or genetic factors or a combination of both is unknown.

Adolescent↗

Lipid requirements in infants with digestive diseases with references to short bowel syndrome.

The treatment of infants with short bowel syndrome aims at restoring the intestinal continuity and at improving the physiological process of gut adaptation. Appropriate parenteral and enteral feeding must be directed at maintaining an ptimal nutritional status. Due to a varying degree of long-chain triglyceride malabsorption, part of the dietary fat needs to be supplied in the form of medium-chain triglycerides in most patients. The dietary fat composition must also meet the needs for essential fatty acids and take into account the positive effects of long-chain lipids on gut trophicity. Appropriate dietary manipulations are of great benefit for infants and children with chronic digestive diseases.

Animals↗

Distribution of cell adhesion molecules in infants with intestinal epithelial dysplasia (tufting enteropathy).

BACKGROUND & AIMS: Intestinal epithelial dysplasia, or tufting enteropathy, is a newly described clinicopathologic entity with refractory diarrhea in infants. Histological abnormalities include villous atrophy, disorganization of the surface epithelium, and basement membrane abnormalities. The aim of this study was to examine defects in intestinal epithelial cell adhesion, differentiation, or proliferation in the pathogenesis of epithelial dysplasia. METHODS: Histological, immunohistochemical, and ultrastructural characteristics of epithelial dysplasia in a group of 6 children were compared with those groups with normal small bowel and other villous atrophy (celiac sprue and microvillous inclusion disease). Distribution of adhesion molecules, markers of cell polarization and proliferation, and the phenotype of intraepithelial lymphocytes were determined. RESULTS: Alterations suggestive of abnormal cell-cell and cell-matrix interactions were present in patients with epithelial dysplasia. They included abnormal distribution of alpha 2 beta 1 integrin along the crypt-villus axis, increased immunohistochemical expression of desmoglein, and ultrastructural changes of desmosomes increased in length and number. No evidence for abnormalities in epithelial cell polarization, proliferation, or T-cell activation was found. CONCLUSIONS: This study strongly suggests a role played by alterations of cell-cell and cell-matrix interactions in the pathogenesis of epithelial dysplasia.

Antigens, Differentiation↗

Energy cost of fever in children on total parenteral nutrition.

The aim of the study was to measure the changes in energy expenditure (EE) and respiratory quotient (RQ) induced by fever in children receiving total parenteral nutrition (TPN). Ten children aged 1-16 years (weight for height: 104.5 +/- 13%) were included. They underwent two EE recordings of 3 h duration using indirect calorimetry, during a febrile (38.9 +/- 0.5 degrees C) and afebrile conditions, after parameters (nutritional intake, ambient temperature) being equal. The EE, (febrile phase) was significantly higher than the EE(2) (afebrile) (2.13 +/- 0.48 vs 1.78 +/- 0.42 kcal/kg/h; P < 0.01). Increase in EE was significantly correlated with body temperature (r = 0.92, P < 0.001). The slope of the regression line indicates an increase of 16.2% in EE per degree of fever. By dividing the individual increase in EE by the individual increase in temperature, the energy expenditure during fever is 11.3% per unit rise ( degrees C). During the febrile phase urinary nitrogen excretion was significantly higher (280 +/- 90 vs 210 +/- 70 mg/kg fat free mass/day, P < 0.02). The RQ during the febrile phase (0.90 +/- 0.13) did not differ significantly from mean RQ during the afebrile phase (0.95 +/- 0.07). Fever due to infection or inflammatory process is partly responsible for an hypermetabolic state including increased EE.

Journal Article↗

Inappropriate iron intake in children on long-term parenteral nutrition: outcome after iron withdrawal.

Inappropriate parenteral iron intake in children on long-term parenteral nutrition can be responsible for iron overload. This study was carried out to monitor iron status changes when iron parenteral intake was stopped in case of iron overload. Seven children with serum ferritin concentrations above 800 ng/ml (6 with documented liver iron overload) were prospectively studied after total discontinuation of parenteral iron intake and without chelation therapy. Iron status was assessed, by means of ferritin and iron plasma concentrations, 8-15 months (T(1)) and 24-30 months (T(2)) after withdrawal of parenteral iron. Ferritin and iron concentrations declined at T(1), or T(2) in all but two children. At T(2) ferritin and iron concentrations were significantly lower (P < 0.05) than before iron parenteral discontinuation with a yearly reduction of 22 +/- 15% and 15 +/- 16%, respectively, for ferritin concentration and iron concentration. This fall in serum ferritin concentration is comparable to thalassemic subjects after bone marrow transplantation. The total withdrawal of parenteral iron intake improves iron status in children with iron overload. Nevertheless, iron overload related parenteral nutrition should be avoided by lowering iron intake in case of long-term total parenteral nutrition and by careful monitoring.

Journal Article↗

Tolerance to starvation in children on long-term total parenteral nutrition.

To evaluate the consequences of long-term cyclic total parenteral nutrition (TPN) on metabolic pathways which contribute to energy metabolism, adaptation to starvation was studied in a group of seven children 2-7 years old, on long-term cyclic TPN. In addition to clinical monitoring, the following biological parameters were measured: blood levels of glucose, free fatty acids, ketone bodies and carnitine, and urinary excretion of dicarboxylic acids. Five of the seven children had good clinical tolerance up to 30 h of fasting. This indicated that metabolic changes arising from prolonged cyclic TPN are easily reversed when such children are fasting. The other two children had to be refed after 22 and 24 h, respectively. Both had metabolic signs of impaired hepatic fatty acid oxidation or ketogenesis. These metabolic changes might reflect the liver failure caused by TPN in these children, and stresses the fact that prolonged starvation has to be carefully monitored in patients with liver dysfunction secondary to TPN.

Journal Article↗

Small-intestinal transplantation.

The management of patients with intestinal failure has benefited from progress in parenteral nutrition (PN), especially home-based PN. Intestinal transplantation is therefore possible and is now, in some conditions, the logical therapeutic option. Since 1985, more than 180 small-bowel grafts have been done, involving the isolated small bowel with or without the colon (38%), the liver-small bowel (46%) or several organs (16%). Two-thirds of recipients were under 20 years of age, and indications were short-bowel syndrome (64%), severe intractable diarrhoea (13%), abdominal cancer (13%) or chronic intestinal pseudo-obstruction syndrome (8%). Of the patients, 51% survived > 2 years after the graft. Patient and graft survival depends on the type of immunosuppression, i.e. cyclosporine or FK506. The results must be interpreted carefully as they represent the first experience in numerous centres using different immunosuppressive protocols, without any randomization. The results from the largest of these centres reflect the current situation more closely. Functional grafts lead to gastrointestinal autonomy (weaning of PN) while maintaining satisfactory nutritional status and normal growth in childhood. Intestinal transplantation is theoretically indicated for all patients permanently or dependent for a long time on PN. However, as PN is generally well tolerated, even for long periods, each indication for transplantation must be carefully weighed up in terms of the iatrogenic risk and quality of life. When PN has reached its limits, especially in those associated with vascular, infectious, hepatic or metabolic complications, intestinal transplantation must be undertaken. Transplantation of the small bowel alone remains the first option, as combined liver-small bowel grafting is only indicated in the case of life-threatening progressive cirrhogenic liver disease.

Graft Rejection↗