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Biomedical subjects

O Goulet

Publications and source records attributed to O Goulet.

At least 37 records · Page 2Linked to original sources

[Nutritional treatment of acute diarrhea in an infant and young child].

This paper written by the Comité de nutrition de la Société française de pédiatrie is specially devoted to the nutritional treatment of infant and child acute diarrhea, i.e. oral rehydration with salts solution and feeding. It complements an article on drug therapy of child acute diarrhea written by the Groupe francophone d'hépatologie, gastroentérologie et nutrition pédiatriques, and published in this same issue of the Archives de pédiatrie.

Acute Disease↗

Indications and results of surgery in patients with Crohn's disease with onset under 10 years of age: a series of 18 patients.

In order to examine the presentation and course of Crohn's disease (CD) with an onset of CD before the age of 10 and to assess indications and effects of surgery, a retrospective study was performed in 18 children. One hundred and twenty-two children with CD have been followed over the last 22 years in our institution. Thirty of them had the first onset of disease before 10 years of age. Eighteen out of these 30 underwent intestinal resection (IR). The mean age at the onset of CD was 6.4 years and the average duration of follow-up was 11.1 years. Most common symptoms were abdominal pain (72 %), diarrhoea (72 %), and growth retardation (50 %). Colon and ileum were the most frequently involved areas. Most of the children (94.5 %) received extensive medical and or nutritional treatment before surgery. We performed 26 IRs and 14 other operations in 18 patients. Indications for IR were chronic intestinal obstruction (13 cases) and chronic intestinal disability (13 cases). Improvements in the general clinical state were obtained in 17 cases out of 18. IR is beneficial for chronically ill children with ineffective medical therapy. A disease-free interval after surgery may provide an important time for improved growth and pubertal development and a return to nearly normal life for a long time.

Adolescent↗

Prepubertal growth in children with long-term parenteral nutrition.

In children who depend on long-term parenteral nutrition (PN), a major goal is to obtain optimal growth. The aim of this retrospective study was to analyze growth in children on long-term cyclic nocturnal home PN, over at least 8 years before puberty. Nine boys and 7 girls were studied. Their mean age at the time of study was 11 years with a mean PN duration of 10.5 (8.6-16.4) years. Diseases were short bowel syndrome (5), intractable diarrhea (4), chronic intestinal pseudo-obstruction (4) and long segment Hirschsprung's disease (3). In each child, periods of at least 2 years were analyzed: either periods of regular growth (R: height gain >50th percentile), or slow growth (S: height gain < or =25th percentile). Results were expressed as mean +/- SD. Comparisons were performed using either Student's test for unpaired data or Wilcoxon's test for paired data. PN provided a mean of 224 +/- 80 mg nitrogen/kg/day and 43 +/- 14 kcal/kg/day equivalent to 50% of recommended supplies. At the time of study, the population presented with weight (W) = -0.7 +/- 0.8 SD and height (H) = -1.5 +/- 1.3 SD. The difference between W and expected W for H (W/H) was significant (p < 0.002). W/H ratio was 105 +/- 11%. For the total PN duration, weight gain was +0.2 +/- 1.5 SD and height loss was -0.75 +/- 1.4 SD. An excess weight gain occurred in parallel with the deflection of height gain. Of the 16 children, regular prepubertal growth was achieved in 4 only. The other 12 showed alternate periods of R and S. In 8 of them, 26.5 years of R and 33.5 years of S were compared, each child being his own control. PN nitrogen and energy supplies were significantly higher during R periods than during S periods. In the absence of any disease or treatment explaining the failure to thrive, inadequate PN supplies, especially in terms of nitrogen supply, are thought to be responsible for a negative nitrogen balance and slowed growth. In case of any deflection away from the individual growth curve, it is recommended to adjust the PN supply early, especially nitrogen supply.

Adolescent↗

[Infant formulas and soy protein-based formulas: current data].

For many years soy bean-based formulas (SBBF) were the only dietary product used for infants with cow's milk intolerance. At the present time, their place in infant nutrition is reduced as a result of the availability of new dietary products without lactose and/or cow's milk proteins and the recognition of soy bean protein allergy. There is no evidence that SBBF have any efficiency in infant colic. SBBF have no indication in the prevention of allergy, nor in premature infants' nutrition. Their main indication is the feeding of infants of vegetarian parents who do not want to use cow's milk products. Studies have shown that SBBF contain large quantities of phytoestrogens, particularly isoflavone. Because of experimental data suggesting a possible deleterious effect of phytoestrogens on the neuroendocrine maturation, the reduction of their content in SBBF must be considered.

Colic↗

[Pediatric gastrointestinal endoscopy: which sedation?].

Through an analysis of the French experience of digestive endoscopists in adult patients and of their own 25 years practice of pediatric digestive endoscopy, the authors militate in favour of anesthetic sedation in order to reduce painfulness and to obtain better acceptation of these procedures by children and their parents.

Anesthesia, General↗

X-linked neonatal diabetes mellitus, enteropathy and endocrinopathy syndrome is the human equivalent of mouse scurfy.

To determine whether human X-linked neonatal diabetes mellitus, enteropathy and endocrinopathy syndrome (IPEX; MIM 304930) is the genetic equivalent of the scurfy (sf) mouse, we sequenced the human ortholog (FOXP3) of the gene mutated in scurfy mice (Foxp3), in IPEX patients. We found four non-polymorphic mutations. Each mutation affects the forkhead/winged-helix domain of the scurfin protein, indicating that the mutations may disrupt critical DNA interactions.

Amino Acid Sequence↗

Saccharomyces boulardii does not stimulate mucosal hyperplasia after intestinal resection in the rat.

BACKGROUND: Polyamines have been shown to be important regulators of the intestinal adaptation process after massive bowel resection. Saccharomyces boulardii is yeast that has the ability to synthesize polyamines. Therefore. S. boulardii may be useful in the treatment of short bowel syndrome. METHODS: Twenty 150-g male Sprague-Dawley rats were subjected to 80% jejunoileal resection. Another 20 animals received transection and closure and served as pair fed controls. One half of the resected rats and one half of the controls were given S. boulardii 25 mg/day. RESULTS: After 2 weeks, mucosal mass (mg/cm bowel) did not differ between treated and non-treated animals despite the presence of a marked resection effect. Mucosal DNA, protein, and sucrase activity likewise did not differ. Subsequently, the experiment was repeated four times the original dose (100 mg/day) and found comparable results. In the proximal bowel, mucosal mass was 92+/-6 mg/cm in treated animals versus 107+/-8 mg/cm in untreated rats. In the distal small bowel, comparable values were 85+/-5 mg/cm and 88+/-4 mg/cm. Again, mucosal DNA, protein, and sucrase activity levels paralleled these results. CONCLUSIONS: Although S. boulardii may stimulate polyamine synthesis, it does not seem to be helpful in augmenting gut adaptation in this animal model of short bowel syndrome.

Adaptation, Physiological↗

Apolipoprotein B Arg3500Gln mutation prevalence in children with hypercholesterolemia: a French multicenter study.

BACKGROUND: Familial defective apolipoprotein B-100, a dominantly inherited form of hypercholesterolemia caused by a single Arg3500Gln mutation, is silent in childhood but may confer a high risk of cardiovascular disease in adulthood. The objective was to determine the prevalence of familial defective apolipoprotein B-100 in hypercholesterolemic French children and to provide a basis for targeting screening efforts in this population. METHODS: One hundred ninety children attending 13 pediatric clinics distributed throughout France were included based on the presence of type IIa hypercholesterolemia with a plasma low-density lipoprotein-cholesterol level of more than 130 mg/dL. The Arg3500Gln mutation was detected in dried blood spots using a polymerase chain reaction assay combined with enzymatic restriction. RESULTS: Three hyperlipidemia phenotypes were found: monogenic dominant pure hypercholesterolemia (n = 117), polygenic hypercholesterolemia (n = 43), and combined hyperlipidemia (n = 11). Three unrelated children were heterozygous for the Arg3500Gln mutation; all three had monogenic dominant pure hypercholesterolemia (3/94 families; 3.2%), yielding a prevalence of 1.83% (3/164) in hypercholesterolemic children, which is similar to prevalences reported in European adults. CONCLUSIONS: The familial defective apolipoprotein B-100 mutation was common (1/31) in children with a phenotype of familial hypercholesterolemia, supporting screening in this population with the goal of preventing premature cardiovascular events.

Adolescent↗

A novel syndrome with dwarfism, poorly muscled build, absent clavicles, humeroradial fusion, slender bones, oligodactyly and micrognathia.

We report on a 15-year-old girl with severe pre- and post-natal growth retardation, poorly muscled build, micrognathia, ulnar ray oligodactyly, absent clavicles, abnormal scapulae, humeroradial fusion, hip dislocation, small iliac wings, slender tubular bones and normal intelligence. An extensive search has failed to ascribe this association to a known condition. This child shared some clinical and radiological features with the Yunis-Varon syndrome but the normal intelligence and the ulnar ray oligodactyly of our patient does not support this diagnosis.

Abnormalities, Multiple↗

[Iodine nutrition in the infant. Committee on Nutrition of the French Society of Pediatrics].

Iodine is a trace element essential for the synthesis of thyroid hormones. It is present in the human body in minute amounts (15-20 mg in adults). The thyroid is very sensitive to iodine deficiency in newborns and infants because of its very low iodine content. Daily iodine requirements in humans vary from 40 micrograms in neonates to 150 micrograms in adults. Iodine deficiency represents the first cause of avoidable mental deficiency in developed countries; it has not yet disappeared in Europe, especially in the East, where it is responsible for a high prevalence of goiter. Iodine deficiency during pregnancy increases the risk of neonatal transient hypothyroidism, with a high recall rate in programs of systematic screening for congenital hypothyroidism. Data available in France suggest that screening for iodine deficiency should be performed during pregnancy, and that the minimal iodine concentration in formula milk should be increased to 10 micrograms/100 kcal for term infants and 20 micrograms/100 kcal for premature infants. Iodine deficiency is ideally prevented by the use of iodized salt. Because of the risk of iodine overexposure and secondary transient hypothyroidism, the use of iodinated antiseptics must be avoided in premature babies and neonates as well as in pregnant and lactating women. The fight against iodine deficiency, associated with oral stable preventive iodine administration, decreases sharply the risk of thyroid cancer in case of nuclear exposure, by diminishing thyroid uptake of iodine radioactive isotopes.

Adult↗