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Biomedical subjects

J P Girardet

Publications and source records attributed to J P Girardet.

At least 19 recordsLinked to original sources

[Clinical profile and long-term outcome in children with esophageal achalasia].

UNLABELLED: Achalasia of the cardia is rare in children. We report our experience in the management of 20 children with achalasia. PATIENTS: Twenty children (seven girls including two sisters) with achalasia were studied, seven of them had a morbid association. Age at diagnosis ranged from eight months to 18 years (med: 6.4 yrs). Duration of symptoms prior to diagnosis ranged from one to 62 months (med: 8 months). Regurgitations, weight loss and recurrent pneumonias were the most common presenting symptoms. Diagnosis was established using esophageal manometry, which showed aperistalsis throughout the esophageal body with impaired relaxation of lower esophageal sphincter in all children, and chest x-ray and barium esophagram, which were abnormal in 11 and 18 children respectively. OUTCOME: Nifedipine used in nine children was ineffective. Two balloon dilatations were performed in one child with a poor result. Surgery with Heller's myotomy combined with an antireflux procedure was performed in 19 children. One child died six months later due to an hypoglycemic coma. During the follow-up (6 months-21 years) symptoms reappeared in five children. Seventy-two percent of the children had no symptoms one year after the surgery and 45%, five years after. Esophageal manometry performed after surgery in seven children showed a normal lower esophageal sphincter pressure but with impaired relaxation, and an aperistalsis throughout the esophageal body. These results justify prolonged follow-up of children with achalasia of the esophagus.

Adolescent↗

Esophageal replacement in children by an isoperistaltic gastric tube: a 12-year experience.

The colonic segment is the most frequently used material for replacing the esophagus in children; however, the use of a gastric tube has become a reliable alternative operation. Since 1987, we have used an isoperistaltic gastric tube to replace the esophagus in children, and we present a series of 21 patients. Indications for operation included caustic injury (nine), esophageal atresia (eight), peptic stricture (two), congenital stricture (one), and esophageal duplication (one). There was no death or necrosis of the graft during the early postoperative period. The esophagogastric anastomosis leaked in two cases, but both of them closed spontaneously. A temporary dumping syndrome was encountered in two children. Two patients had strictures of their upper anastomosis responding to dilatations. The two patients who had a pharyngogastric anastomosis developed either intractable stricture or nonfunctioning anastomosis. One of them died 9 months later from aspiration pneumonitis. At follow-up, 16 of 21 patients could accept a normal diet (13 were entirely asymptomatic, and three suffered occasional mild dysphagia). Two patients suffered significant dysphagia (one had a durable dilation of his gastric tube), and three needed a feeding jejunostomy. Acid secretion of the gastric tube was proved in nine cases. Two patients were shown to have cervical Barrett's esophagus above the anastomosis. These findings indicate the need for lifelong endoscopic follow-up for these patients.

Child↗

Resting energy expenditure and substrate utilisation rate in children with constitutional leanness or obesity.

BACKGROUND & AIM: The influence of energy expenditure on body weight regulation, in the absence of organic disease, has never been studied in a paediatric population covering a broad range of body weights. The aim of this study was to investigate resting energy metabolism in children with constitutional leanness, normal body weight, or common obesity. METHODS: Fourteen children with constitutional leanness, 16 children with obesity, and controls were studied. Resting energy expenditure and postabsorptive substrate utilisation rate were measured by indirect calorimetry and body composition was assessed from skinfold thicknesses. RESULTS: As compared to the predicted value calculated from the regression equation of resting energy expenditure on fat-free mass in the controls, resting energy expenditure was decreased in lean children (P=0.002), whereas no difference was found in obese children. In obese children and the overall population, fat mass was positively correlated with fat oxidation rate. In each group and in the overall population, fat oxidation rate was positively correlated to resting energy expenditure. CONCLUSIONS: Constitutionally lean children have a low resting metabolic rate, probably adaptive in nature. In obese children, resting energy expenditure is increased in proportion to the fat-free mass, and fat balance is the main determinant of energy balance. These data suggest a constitutional regulation of body weight.

Adipose Tissue↗

[Drug treatment of acute infectious diarrhea in infants and children].

In this paper written by the Groupe francophone d'hépatologie, gastroentérologie et nutrition pédiatriques, recommendations are given on the indications of drugs in infant and child infectious acute diarrhea, based upon the current scientific knowledge on their effectiveness and tolerance. This paper complements an article on nutritional treatment of acute diarrhea written by the Comité de nutrition de la Société française de pédiatrie, and published in the same issue of the Archives de Pédiatrie.

Acute Disease↗

Presence of increased stiffness of the common carotid artery and endothelial dysfunction in severely obese children: a prospective study.

BACKGROUND: Epidemiological studies suggest that obesity-induced atherosclerosis may start in childhood, but this process has never been demonstrated. We looked for arterial changes and investigated their relation to cardiovascular risk factors in obese children. METHODS: Non-invasive ultrasonographic measurements were made in 48 severely obese children and 27 controls to investigate arterial mechanics and endothelial function. Plasma lipid concentrations, indices of insulin resistance, and body composition were assessed in the obese children. FINDINGS: The obese children had significantly lower arterial compliance than the healthy controls (median 0.132 [0.022-0.273] vs 0.143 [0.112-0.237] mm(2).mm Hg; p=0.02) and lower distensibility (0.60 [0.10-1.00] vs 0.70 [0.50-1.10] mm Hg(-1).10(-2); p=0.0001). Conversely, the obese children had higher values than the controls for wall stress (3.36 [2.00-5.01] vs 2.65 [2.13-3.54] mm Hg.10(2); p=0.0001) and incremental elastic modulus (1.68 [0.72-10.8] vs 0.96 [0.64-1.47]; p=0.0001). Endothelium-dependent and independent function were also lower in the obese than in the control children. An android fat distribution was positively correlated with indices of insulin resistance and plasma triglyceride concentrations and was negatively correlated with plasma HDL-cholesterol concentration and arterial compliance. Endothelial dysfunction was correlated with low plasma apolipoprotein A-I and with insulin resistance indices. INTERPRETATION: Severe obesity in children is associated with arterial wall stiffness and endothelial dysfunction. Low plasma apolipoprotein A-I, insulin resistance, and android fat distribution may be the main risk factors for these arterial changes, which are of considerable concern as possible early events in the genesis of atheroma.

Adolescent↗

[Obesity in children: a disease that risks the vital prognosis].

UNLABELLED: Drug consumption by adolescents causes social and health problem. OBJECTIVE: This prospective study was designed to examine reasons for and against using substances such as tobacco, alcohol, and marijuana and to discover whether or not motives to use drugs vary with the number of drugs used. METHOD: The participants were 886 adolescents from a suburban high school (Nancy, France), who answered a self-report questionnaire. RESULTS: The response rate was 95% (655 males and 175 females, mean age 17.5 +/- 2.5 years). The prevalence of current tobacco use, former tobacco use and never used was 40.7, 21.3 and 31.7%, respectively. For alcohol consumption, the results wre 85.8, 3.5 and 0.7% respectively and for marijuana consumption 34.4, 18.3 and 47.3%, respectively. Ecstasy had already been used by the respondens. The main reasons given by the adolescents to explain why they did or did not use these substances varied with the mumber of drugs used. CONCLUSION: This work shows an increasing deterioration of youths' social relations (with his/her parents and with him/herself) that seem to depend onthe number of drugs used. Further research is necessary to improve these results.

Adult↗

Efficacy and tolerability of racecadotril in acute diarrhea in children.

BACKGROUND & AIMS: Oral rehydration therapy is the only treatment recommended by the World Health Organization in acute diarrhea in children. Antisecretory drugs available could not be used because of their side effects, except for racecadotril, which is efficient in acute diarrhea in adults. METHODS: The efficacy and tolerability of racecadotril (1.5 mg/kg administered orally 3 times daily) as adjuvant therapy to oral rehydration were compared with those of placebo in 172 infants aged 3 months to 4 years (mean age, 12.8 months) who had acute diarrhea. The treatment groups were comparable in terms of age, duration of diarrhea, number of stools, and causative microorganism at inclusion. RESULTS: During the first 48 hours of treatment, patients receiving racecadotril had a significantly lower stool output (grams per hour) than those receiving placebo. The 95% confidence interval was 43%-88% for the full data set (n = 166; P = 0.009) and 33%-75% for the per-protocol population (n = 116; P = 0.001). There was no difference between treatments depending on rotavirus status. Significant differences between treatment groups were also found after 24 hours of treatment: full data set (n = 167; P = 0.026) and per-protocol population (n = 121; P = 0.015). Tolerability was good in both groups of patients. CONCLUSIONS: This study demonstrates the efficacy (up to 50% reduction in stool output) and tolerability of racecadotril as adjuvant therapy to oral rehydration solution in the treatment of severe diarrhea in infants and children.

Acute Disease↗

Mutational analysis of melanocortin-4 receptor, agouti-related protein, and alpha-melanocyte-stimulating hormone genes in severely obese children.

OBJECTIVE: To search for mutations in melanocortin pathway elements, that is, the melanocortin-4 receptor (MC4R ), agouti-related protein (AGRP ), and (alpha-melanocyte-stimulating hormone (alpha MSH ) genes in children with severe obesity. STUDY DESIGN: Direct sequencing of the MC4R encoding sequence and single-strand polymorphism conformation analysis of AGRP and alpha MSH genes were performed in 63 severely obese children. Polymerase chain reaction (PCR) assays of restriction fragment length polymorphism were used to assess the frequency of each newly discovered mutation in 283 non-obese control subjects. RESULTS: Four dominantly inherited, heterozygous, missense MC4R mutations (Val50Met, Ser58Cys, Ile102Ser, and Ile170Val) were identified in 4 unrelated children and none of the control subjects. Expression of the obese phenotype was variable in mutation-positive family members. Clinical and laboratory features were similar in the obese children with and without an MC4R mutation. Two polymorphisms were detected in the AGRP -encoding sequence (a silent mutation in exon 1 and Ala67Thr in exon 2), with similar frequencies in the obese and control groups. No mutations were found in the alpha MSH gene. CONCLUSIONS: MC4R mutations may be a non-negligible cause of severe obesity in children with variable expression and penetrance. Mutations in AGRP and alpha MSH genes were not among the causes of obesity in our population.

Adolescent↗

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↗

Arterial mechanical changes in children with familial hypercholesterolemia.

Atherosclerosis is preceded by a phase of changes in the arterial wall that could have functional consequences even before the appearance of atheromatous changes. We hypothesized that early alterations of the mechanical properties of the arterial wall could precede clinical and echographic modifications. We used an automatic, computerized, ultrasonic procedure to evaluate geometric and mechanical characteristics of the common carotid artery (CCA) in normotensive children with primary familial class IIA hypercholesterolemia (FH; n=30; mean+/-SD age, 11+/-2 years old; mean+/-SD systolic/diastolic blood pressure, 109+/-9/55+/-7 mm Hg). These subjects were compared with age-matched, nonobese control subjects (n=27; 11+/-3 years old; 112+/-10/55+/-7 mm Hg). Noninvasive ultrasonic measurements were performed by the same investigator to measure the CCA luminal systolic and diastolic diameters and intima-media thickness (IMT). The cross-sectional compliance, cross-sectional distensibility, and the incremental elastic modulus of the CCA wall were then calculated. Finally, we assessed the degree of reactive hyperemia in the brachial artery produced after distal cuff occlusion and release. The changes in brachial arterial diameter in response to reactive hyperemia (endothelium-dependent dilation) and to glyceryltrinitrate (endothelium-independent dilation) were then measured. In patients with FH, we observed a significant reduction of systodiastolic variations in diameter (by 20%, P:<0.001) without a significant difference in IMT. Cross-sectional compliance and cross-sectional distensibility were significantly reduced in FH subjects (by 15%, P:<0.05 and 19%, P:<0.01, respectively). In parallel, the incremental elastic modulus was significantly increased (by 27%, P:<0.01) in children with FH. No correlation was evident between the carotid incremental modulus and either IMT or plasma low density lipoprotein cholesterol level. There was no difference in diameter of the brachial artery at rest in control and FH subjects (3.0+/-0.5 versus 3.0+/-0.4 mm). The reactive hyperemia and glyceryltrinitrate dilation were also similar in the 2 groups. However, the flow-mediated dilation of the brachial artery was smaller in the FH subjects (4.2+/-2.9%) than in controls (9.0+/-3.1%, P:<0.001). In FH, endothelium-dependent dilation was negatively correlated with the plasma low density lipoprotein cholesterol level (P:<0.04). These results indicate that increased stiffness of the CCA wall in children with FH is independent of blood pressure and could be related to endothelial dysfunction. Thus, alterations in CCA wall mechanics could be early and easily measurable markers of atheromatous changes in the arterial wall.

Biomechanical Phenomena↗

Efficacy of cyclosporin A in children with type 2 autoimmune hepatitis.

The conventional treatment of autoimmune hepatitis (AIH) with prednisone and azathioprine induces remission in most cases but is often associated with poorly tolerated side effects. We carried out a retrospective study to evaluate the efficacy of and the tolerance to cyclosporin treatment in 15 children and adolescents with type 2 AIH. Eight children received cyclosporin as primary immunosuppression because of risk factors for poor tolerance of steroids. Five other patients with relapsing AIH refused to resume treatment with steroids and were treated with cyclosporin. In both groups alanine aminotransferase activity returned to normal within 6 months. Side effects were minimal and well tolerated. No relapse occurred in 10 patients after 1 to 6 years. Cyclosporin was withdrawn in 3 patients after 1, 2, and 3 years and replaced by low doses of prednisone in combination with azathioprine. In 2 other children with acute liver failure, which progressed despite treatment with steroids and azathioprine, the addition of cyclosporin was followed by normalization of prothrombin time.

Adolescent↗

Stability and compatibility of teicoplanin in parenteral nutrition solutions used in pediatrics.

AIMS: To investigate teicoplanin added to pediatric parenteral nutrition solutions in terms of its stability, its compatibility with parenteral nutrition solution components, and its diffusion through an antibacterial filter material. METHODS: Three binary solutions with and without teicoplanin were studied. Different solution compositions and teicoplanin concentrations were used: A (98.3 +/- 8.2 mg/l), B (116.3 +/- 12.4 mg/l), and C (162.7 +/- 16.2 mg/l). Concentrations of teicoplanin and of solution components, osmolality, and pH of each solution were measured at H0, after 24 h at room temperature, after 24 h at +4 degrees C followed by 24 h at room temperature, and after 144 h at +4 degrees C followed by 24 h at room temperature (H168). Teicoplanin concentrations were also measured before and after passage of each solution through a 0.22 micro filter. RESULTS: Teicoplanin concentrations remained unchanged from H0 to H168 in solutions A (99.6 +/- 8.3 mg/l), B (116.9 +/- 12. 3 mg/l), and C (162.4+12.9 mg/l). During the H0-H168 interval, iron and methionine were the only components that showed significant decreases, which were similar in solutions without teicoplanin [iron, -6.1% (A), -6.8% (B), and -4.5% (C); methionine, -7.3% (A) and -8. 7% (B)] and in those with teicoplanin [iron, -6.2% (A), -7.1% (B), and -4.0% (C, nonsignificant); methionine, -10.5% (A) and -10.7% (B)], indicating that they were not dependent on the presence of teicoplanin. Teicoplanin levels after filtration were identical to prefiltration values in solutions A (86.4 +/- 5.0 vs 89.8 +/- 3.4 mg/l) and B (112.6 +/- 4.3 vs 115.3 +/- 9.0 mg/l) but were 10.0% lower in solution C (161.6 +/- 3.9 vs 145.4 +/- 4.0; P << 0.001). CONCLUSIONS: Teicoplanin can be added to pediatric parenteral nutrition solutions to treat central venous catheter-related infections due to teicoplanin-susceptible organisms since its concentrations and those of solution components remain stable over time.

Anti-Bacterial Agents↗