PubMed Health⌕ Search

Biomedical subjects

C Ricour

Publications and source records attributed to C Ricour.

At least 37 records · Page 2Linked to original sources

Up-to-date evolution of small bowel transplantation in children with intestinal failure.

PURPOSE: The aim of the authors was to report an up-to-date review of their experience with 26 intestinal transplantations in children since 1987. METHODS: A retrospective study was conducted of 26 patients with a mean age of 5 years (range, 0.3 to 14 years). Three groups were isolated. In group A (1987 to 1990), seven patients received nine isolated intestinal transplants for short bowel syndrome. Immunosuppression therapy consisted of cyclosporine, aziathioprine, and corticosteroids. In group B (1994-current), nine patients received nine isolated intestinal transplants for short bowel syndrom (n = 2), intestinal pseudoobstruction (n = 2), neonatal intractable diarrhea (n = 3), and Hirschsprung' disease (n = 1); hepatic biopsy results showed weak cholestasis or fibrosis. In group C (1994-current), 10 patients received 10 combined liver-small bowel transplants for short bowel syndrome (n = 3), neonatal intractable diarrhea (n = 4), and Hirschsprung' disease (n = 3); hepatic cirrhosis related to total parenteral nutrition (TPN) was shown in all cases. Groups B and C received immunosupressive treatment consisting of tacrolimus, aziathioprine, and corticosteroids. Posttransplant follow-up included intestinal biopsies of the small bowel twice a week and more frequently or combined with liver biopsy if rejection was suspected. RESULTS: Overall patient survival (PS) and graft survival (GS) are 61% (16 of 26) and 50% (13 of 26), respectively. In group A, severe intestinal allograft rejection occurred in six patients leading to graft removal (GS, 11%). Five patients died of TPN complications after graft removal (PS, 28%). One survivor is off TPN, and one currently is waiting for a second graft. In group B, six patients survived (PS, 66%). Causes of death include hepatic failure (n = 1), renal and liver failure (n = 1), and systemic infection (n = 1). Severe intestinal allograft rejection occurred in five patients, which neccessitated aggressive immunosuppression (antilymphocyte serum) leading to an incomplete functional recovery of the graft. Only two patients currently are off TPN. In group C, eight patients survived (PS, 80%) all of which are currently off TPN. One patient died during the procedure, and one died of severe systemic infection. Intestinal graft rejection occurred in six patients; rejection of the liver allograft occurred in five patients, yet all rejections were weak and successfully treated by corticosteroids (GS, 80%). CONCLUSIONS: Intestinal transplantation is a valid therapeutic option for children with definitive intestinal failure and not only for short bowel syndrome. Tacrolimus improves graft and patient survival (group A v group B). The lower severity of graft rejection in combined liver-small bowel transplantation improves functional results of intestinal transplantation in children without additional mortality or morbidity (group B vgroup C).

Adolescent↗

Chronic intestinal pseudo-obstruction syndrome in pediatric patients.

The aim of this study was to report the presentation and outcome of 22 consecutive children (13 female) who presented with a syndrome of chronic intestinal pseudo-obstruction with or without urinary tract involvement. We analyse the main clinical and histopathological features and discuss therapeutic management. Ten patients had signs of intestinal obstruction at birth, in which 6 presented antenatally with megacystis on ultrasound. Six children presented with constipation and/or obstruction between 1 and 6 months of age and in 6 other patients diagnosis was made between the ages of 1 and 12 years. There was a family history in 4 patients. Investigations showed diffusely dilated gut on x-ray with slow transit on small bowel follow through. Absent or abnormal motor migrating complex with low amplitude contractions were demonstrated on duodeno-jejunal manometry in 12/13. Megacystis occurred in 15/21 and megaureter in 2/21. Full thickness biopsies (n = 22) revealed involvement of muscle layers in 8, and abnormal myenteric plexus on histochemistry in 13. In 1, the biopsies were inconclusive. Recurrent urinary tract infections occurred in all with structural urinary tract abnormality and most had bacterial overgrowth. Severe recurrent episodes of obstruction which required parenteral nutrition (PN) occurred in all patients. Drugs were unhelpful and decompression ileostomies or colostomies were performed in 20/22. Five children died from sepsis (n = 3) or sudden death. Eleven patients remain partially or totally dependent on PN despite decompression ileostomy in 10/11. Six patients underwent colectomy and ileorectal pull-through, 2 of which remain on long-term PN, while the others are totally orally fed. Despite careful histological study pointing to 2 main forms, myopathy and neuropathy, the etiology of primary intestinal pseudoobstruction syndromes remains unknown. It may present antenatally while most of the time the gut and the urinary tract are diffusely involved. The condition has a high morbidity with a percentage requiring long-term PN. Although the mortality rate is high (23%), careful treatment of urinary tract infections and bacterial overgrowth, decompression surgery and judicious use of PN allows survival to adult life.

Child↗

Long-term efficacy and safety of a new olive oil-based intravenous fat emulsion in pediatric patients: a double-blind randomized study.

BACKGROUND: A new intravenous lipid emulsion (ILE) prepared from a mixture of soybean and olive oils contains only long-chain triacylglycerols, with a low proportion (20%) of polyunsaturated fatty acids and 60% monounsaturated fatty acids. OBJECTIVE: The goal of this randomized, double-blind clinical trial was to assess in children the efficacy and safety of this new ILE compared with a control group receiving a soybean-oil emulsion. DESIGN: Eighteen children received for 2 mo 24% of nonprotein energy (1.80 g kg (-)(1) d(-)(1)) either as the new ILE or a soybean oil-based emulsion. Assessments were performed on days -30, 0, 30, and 60 and the changes (day 60 - day 0) assessed by analysis of variance. RESULTS: There were no significant differences in triacylglycerol, apolipoproteins A-I and B, or HDL cholesterol between the 2 groups, whereas total and LDL cholesterol were higher in the soybean oil group on day 60. The pattern of 20:4n-6 in erythrocyte membranes did not change significantly, nor did the ratio of 20:3n-9 to 20:4n-6. On day 60, 18:1n-9 was significantly higher in the olive oil group, the ratio of Sigma(n)-6 > C(18) + 18:3n-6 to 18:2n-6 was 2.20 +/- 0.09 in the olive oil group and 1.33 +/- 0.16 in the soybean-oil group, and Sigma(n)-3 > C(18) was 3.83 +/- 0.30 in the olive oil group and 4. 03 +/- 0.33 in the soybean-oil group. The peroxidation index was lower after the olive oil treatment. CONCLUSIONS: The olive oil-based emulsion was well tolerated, maintained a normal EFA status, and may be more suitable for prevention of lipid peroxidation than the soybean-oil-based emulsion.

Child↗

[Can intestinal transplantation constitute treatment for intestinal failure?].

The management of patients with intestinal failure has benefited from progress in parenteral nutrition (PN), especially home-based parental nutrition. Intestinal transplantation is now possible and in some conditions, constitutes the logical treatment option. Since 1985, more than 300 small-bowel grafts have been performed, involving the isolated small bowel with or without the colon (45%), the liver + small bowel (40%) or several organs (15%). 2/3 of recipients were under 20 years of age, and indications were short-bowel syndrome (64%), severe intractable diarrhea (13%), abdominal cancer (13%), or chronic intestinal pseudo-obstruction syndrome (8%). 51% of patients survived > 2 years after the graft. Patient and graft survival depends on the type of immunosuppression, i.e. Cyclosporine or FK 506. The results must be interpreted carefully as they represent the first experience in numerous centers using different immuno-suppressive protocols, without any randomization. The results from the largest of these centers more closely reflect the current situation and may exceed a 70% 2-year survival rate. 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 persistently dependent 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 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 case of life-threatening progressive cirrhogenic liver disease.

Adolescent↗

Enteral ornithine alpha-ketoglutarate enhances intestinal adaptation to massive resection in rats.

Ornithine alpha-ketoglutarate (OKG) has been advocated in the treatment of critically ill patients for its anabolic effect on protein metabolism. Since OKG is a precursor of glutamine, arginine, and polyamines, key substrates of intestinal metabolism and function, we investigated the influence of OKG on intestinal adaptation and trophicity and on glutamine status after small bowel resection. After massive (80%) small bowel resection, rats were enterally fed for 7 days with a standard diet supplemented with either OKG (2 g/kg/d) or an isonitrogenous amount of glycine. OKG induced an adaptative hyperplasia of the villi, demonstrated in the jejunum by an increase in the villus height to crypt depth ratio (OKG v control, 4.3+/-0.4 v 3.3+/-0.5, P < .01) along with an increase (P < .05) in ornithine decarboxylase (ODC) activity (+80%) and ornithine content (+102%). Plasma glutamine (+25%) and muscle glutamine (anterior tibialis [AT], +43%; extensor digitorum longus [EDL], +54%) and protein (AT, +32%) were significantly higher (P < .05) after OKG administration, supporting its role in the restoration of glutamine pools. In summary, enterally administered OKG, which enhances intestinal adaptation after massive resection and improves muscle glutamine and protein content, could contribute significantly to nutritional management after small bowel resection.

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↗

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↗

t(3;17)(q21;q25) in Epstein-Barr virus associated peripheral T-cell lymphoma: a paediatric case.

We describe an immunocompetent 8-year-old boy with a serological profile indicating chronic infection by Epstein-Barr virus (EBV) who developed subcutaneous and pulmonary lesions related to a peripheral T-cell proliferation. No clonal rearrangement of T-cell receptor or immunoglobulin genes was seen. However, the finding of a t(3;17)(q21;q25) in 44 metaphases from one skin lesion demonstrated a clonal origin. We also showed that the proliferative T cells contained EBV genome leading to the diagnosis of EBV-associated peripheral T-cell lymphoma. Further cytogenetic and molecular studies are needed to identify genes implicated in the pathogenesis of this haematological malignancy.

Child↗

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↗