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Biomedical subjects

Frédéric Gottrand

Publications and source records attributed to Frédéric Gottrand.

At least 19 recordsLinked to original sources
PubMed2006

Nutritional status at 2 years in former infants with bronchopulmonary dysplasia influences nutrition and pulmonary outcomes during childhood.

Improved survival rates for extreme prematurity have been accompanied by an increase in the incidence of bronchopulmonary dysplasia (BPD). The objective of this study was to assess factors associated with long-term nutritional and pulmonary function outcomes. The study was a cross-sectional study of 52 children who had been born prematurely, had experienced BPD, and were 4-8 y old at the time of the study. Undernutrition was defined as a Z score for weight-for-height of <-2 SD. Body composition and lung function were evaluated. Resting energy expenditure (REE) was measured using indirect calorimetry. Stepwise logistic regression was used to test for factors associated with undernutrition and pulmonary function. Eighteen children (35%) with BPD, predominantly girls, were undernourished. Undernutrition occurred within the first months of life and was associated with high REE. Multivariate analysis showed that factors significantly associated with undernutrition were female sex and undernutrition at age 2 y. Thirty-one children (60%) had abnormal lung function tests. Multivariate analysis showed that undernutrition at age 2 y was the only factor associated with the risk of developing distension of the airways. Nutritional status at age 2 y in children who had BPD in infancy influences nutritional and pulmonary outcomes in childhood.

Bronchopulmonary Dysplasia↗
PubMed2006

Management of patients with biliary atresia in France: results of a decentralized policy 1986-2002.

This study analyzed the results of the decentralized management of biliary atresia (BA) in France, where an improved collaboration between centers has been promoted since 1997. Results were compared to those obtained in England and Wales, where BA patients have been centralized in three designated centers since 1999. According to their birth dates, BA patients were divided into two cohorts: cohort A, with patients born between 1986 and 1996, had 472 patients; and cohort B, with patients born between 1997 and 2002, had 271 patients. Survival rates were calculated according to the Kaplan-Meier method and compared by using the log rank test and the Cox model. Four-year overall BA patient survival was 73.6% (95% CI 69.5%-77.7%) and 87.1% (CI 82.6%-91.6%) in cohorts A and B, respectively (P < .001). Median age at time of the Kasai operation was 61 and 57 days in cohorts A and B, respectively (NS). Four-year survival with native liver after the Kasai operation was 40.1% and 42.7% in cohorts A and B, respectively (NS): 33.9% (cohort A) and 33.4% (cohort B) in the centers with two or fewer caseloads a year, 30.9% (cohort A) and 44.5% (cohort B) in the centers with 3-5 cases/year, 47.8% (cohort A) and 47.7% (cohort B) in the center with more than 20 caseloads a year. In cohorts A and B, 74 (15.7%) and 19 (7%) patients, respectively, died without liver transplantation (LT). Four-year survival after LT was 75.1% and 88.8% in cohorts A and B, respectively (P = .006). In conclusion, BA patients currently have the same chance of survival in France as in England and Wales. The early success rate of the Kasai operation remains inferior in the centers with limited caseloads in France, leading to a greater need for LTs in infancy and early childhood.

Adolescent↗
PubMed2006

[Complexity of pathological interpretation in megacystis-microcolon-intestinal hypoperistalsis syndrome].

Megacystis-microcolon-intestinal hypoperistalsis syndrome is very rare, and is the most severe of the chronic intestinal pseudoobstructions. Diagnosis is usually made in the neonatal period, is clinical and radiological, and is confirmed by manometric studies. Microscopic abnormalities are variable, inconstant and nonspecific. They involve the smooth muscle more often than the intrinsic innervation of the gut and the bladder. A girl, currently seven years old, presented with megacystis observed on prenatal ultrasound at 21 weeks of gestation. At first, amniotic fluid volume was appropriate for gestational age, and then hydramnios appeared at 30 weeks of gestation. Microcolon was discovered at birth, with microileum, dilatation of the duodenum and proximal jejunum, intestinal malposition, and severe hypoperistalsis of the entire gastrointestinal tract, which indicated enterostomy and total parenteral nutrition from birth. At pathological examination, rectal biopsy and enteric nervous plexuses were normal. There was hypoplasia of the external longitudinal layer of the muscularis propria in the colon and ileum. Cajal cells could not be demonstrated immunohistochemically in the colon. This case highlights the complexity and difficulties of pathological interpretation in this syndrome, and the necessity of a large study of controls at different ages and different levels of the digestive tract and the bladder.

Abnormalities, Multiple↗
PubMed2006

Estimating body composition in children with Duchenne muscular dystrophy: comparison of bioelectrical impedance analysis and skinfold-thickness measurement.

BACKGROUND: Duchenne muscular dystrophy (DMD) is often associated with obesity, which worsens the handicap early in the course of the disease. Nutritional assessment, however, can be difficult and often misleading in DMD. OBJECTIVE: Two methods of estimating body composition in DMD, skinfold-thickness (ST) measurement and bioelectrical impedance analysis (BIA), were compared with a reference method, labeled water dilution (WD). DESIGN: Body composition was estimated by using ST measurements and BIA (50 kHz, 800 mAmp), as well as the WD method (1 mL H2(18)O/kg) in 11 DMD patients with a mean (+/-SD) age of 10.0 +/- 2.5 y. RESULTS: When compared with the WD method, ST measurement significantly (P < 0.01) overestimated fat-free mass (FFM) (mean +/- SD ST: 24.5 +/- 5.9 kg; mean +/- SD WD: 18.2 +/- 2.5 kg), which led to an underestimation of the percentage of fat mass (%FM) (ST: 23.3 +/- 10.4%; WD: 40.1 +/- 17.1%; P < 0.05). In contrast, estimates obtained with BIA (FFM: 21.5 +/- 4.5 kg; %FM: 31.3 +/- 13.9%) did not differ from those obtained with WD. The difference from the reference method was less for BIA (mean: 3.3 kg; 95% CI: 0.8, 4.9 kg) than for ST (6.3 kg; 2.2, 8.6 kg). WD and BIA defined 73% and 55%, respectively, of the children as obese (%FM associated with body mass index cutoffs for obesity), whereas ST measurements defined 9% as obese (P < 0.01). CONCLUSIONS: Body-composition estimates by BIA are closer to those by WD than are those by ST measurement. Early detection of fat accumulation and longitudinal monitoring of nutritional care are 2 relevant applications of BIA to prevent obesity and hence lessen the burden of DMD.

Body Composition↗
PubMed2006

Oral glutamine and amino acid supplementation inhibit whole-body protein degradation in children with Duchenne muscular dystrophy.

BACKGROUND: Glutamine has been shown to acutely decrease whole-body protein degradation in Duchenne muscular dystrophy (DMD). OBJECTIVE: To improve nutritional support in DMD, we tested whether oral supplementation with glutamine for 10 d decreased whole-body protein degradation significantly more than did an isonitrogenous amino acid control mixture. DESIGN: Twenty-six boys with DMD were included in this randomized, double-blind parallel study; they received an oral supplement of either glutamine (0.5 g . kg(-1) . d(-1)) or an isonitrogenous, nonspecific amino acid mixture (0.8 g . kg(-1) . d(-1)) for 10 d. The subjects in each group were not clinically different at entry. Leucine and glutamine metabolisms were estimated in the postabsorptive state by using a primed continuous intravenous infusion of [1-(13)C]leucine and [2-(15)N]glutamine before and 10 d after supplementation. RESULTS: A significant effect of time was observed on estimates of whole-body protein degradation. A significant (P < 0.05) decrease in the rate of leucine appearance (an index of whole-body protein degradation) was observed after both glutamine and isonitrogenous amino acid supplementation [x +/-SEM: 136 +/- 9 to 124 +/- 6 micromol . kg fat-free mass (FFM)(-1) . h(-1) for glutamine and 136 +/- 6 to 131 +/- 8 micromol . kg FFM(-1) . h(-1) for amino acids]. A significant (P < 0.05) decrease in endogenous glutamine due to protein breakdown was also observed (91 +/- 6 to 83 +/- 4 micromol . kg FFM(-1) . h(-1) for glutamine and 91 +/- 4 to 88 +/- 5 micromol . kg FFM(-1) . h(-1) for amino acids). The decrease in the estimates of whole-body protein degradation did not differ significantly between the 2 supplemental groups. CONCLUSION: Oral glutamine or amino acid supplementation over 10 d equally inhibits whole-body protein degradation in DMD.

Administration, Oral↗
PubMed2006

Dramatic changes in home-based enteral nutrition practices in children during an 11-year period.

OBJECTIVES: Experience with pediatric home-based enteral nutrition (HEN), in particular series including children only, has been reported only rarely. We investigated the evolution of pediatric HEN activity during an 11-year period. METHODS: All patients aged 17 years or younger who started HEN between January 1990 and December 2000 were included in this retrospective study. RESULTS: The annual number of patients treated with HEN increased dramatically from 16 in 1990 to 200 in 2000, with more than 65 new patients every year since 1999 (P < 0.0001). The mean age at the commencement of HEN decreased from 6.2 +/- 1.4 (SEM) to 4.8 +/- 0.7 years (P = 0.006). The use of nasogastric tubes decreased from 63% in 1990 to 35% in 1998 (P = 0.009), and the use of gastrostomy increased from 50% to 60% from 1994 onward. The proportion of patients with digestive diseases commencing HEN in each year decreased from more than 40% before 1996 to less than 32% in 2000 (P = 0.009). Commercially manufactured pediatric diets were used increasingly (P = 0.0006). CONCLUSIONS: The evolution of HEN was marked by changes in the population treated and the modes of treatment after the emergence of gastrostomy and commercial diets. This justified the creation of a multidisciplinary, pediatric artificial nutrition unit.

Adolescent↗
PubMed2005

Long-chain polyunsaturated fatty acids modulate lung inflammatory response induced by Pseudomonas aeruginosa in mice.

Polyunsaturated fatty acid (PUFA) immunomodulatory properties have been studied extensively in chronic infections. Few studies have focused on acute infection; thus, PUFA effects in a mouse model of Pseudomonas aeruginosa (PA)-induced lung injury were evaluated. C57BL/6 mice were randomized to be fed for 3 wk with an eicosapentaenoic acid (EPA) diet, an arachidonic acid (AA) diet, or a control diet [saturated fatty acids]. Lung injury was induced by intratracheal instillation of 10(7) CFU of PA per mouse. In each diet group, animals were studied either without or after PA-inducing lung injury. Evaluation criteria were early mortality; inflammatory response assessed with tumor necrosis factor-alpha (TNF-alpha), IL-1beta, IL-6 and IL-10 levels in bronchoalveolar lavage; lung injury evaluation; and extravascular lung water, assessed 24 h after the injury. After PA-induced lung injury, no difference in early mortality was observed; TNF-alpha level was significantly higher in the EPA diet than in the other two diet groups. No difference for the other cytokines was found among the groups. Lung edema was also more important in the EPA group, consistent with the variations of TNF-alpha levels. Our study clearly shows that in PA-induced acute lung injury, n-3 PUFA induces differences in the inflammatory response with a higher level of lung edema. Modulation of the inflammatory response with n-3 PUFA can influence the response to a bacterial challenge.

Animals↗
PubMed2005

Relative expression of Pseudomonas aeruginosa virulence genes analyzed by a real time RT-PCR method during lung infection in rats.

The bacterial aspects of the events occurring during the first days of lung colonization by Pseudomonas aeruginosa are still unknown. The aim of this study was to develop a real time RT-PCR method for the direct quantification of the transcripts of three P. aeruginosa virulence genes: exoS, lasI and algD, during the first seven days of a rat lung infection. Our results document differences in bacterial gene expression throughout P. aeruginosa infection. ExoS transcripts levels were very high in the first days of infection, but a significant decrease was then progressively observed. Transcription of algD occurred on the 4th day and increased regularly over time suggesting a balance in the transcription of exoS and algD. The strong expression of exoS during the first 3 days was correlated to 29% of mortality among infected rats. On the contrary, the increase of algD expression after this period was associated to the development of a chronic infection with no further mortality. LasI transcription remained more constant throughout the infection.

ADP Ribose Transferases↗
PubMed2005

Home enteral nutrition in children: an 11-year experience with 416 patients.

BACKGROUND & AIMS: We report our experience of paediatric home enteral nutrition, as there is little detailed evidence published. METHODS: All patients younger than 18 years commencing treatment between January 1990 and December 2000 were included in this retrospective study. RESULTS: The study covered 416 children and adolescents, corresponding to a total of 243,844 days of home enteral nutrition (HEN). The mean (+/-SD) age of patients commencing treatment was 5.4+/-5.3 years (range 0.1-17.8). Indications were digestive disorders in 35% of patients, neurological and muscular disorders in 35%, malignancy in 11%, failure to thrive in 8%, and miscellaneous ailments in 9%. Enteral feeding comprised commercially available paediatric industrial diets in 36%, adult-type diet in 35% and infant formulas in 29%. Children received enteral feeding by nasogastric tube (53%), or gastrostomy (41%). A mechanical pump was used in 98% of the patients. The mean duration of treatment was 595+/-719 days. CONCLUSIONS: HEN can be used while treating a large group of chronic diseases of children. It can be started very early in life and is often prolonged over several years.

Adolescent↗
PubMed2005

Clinical, biochemical and morphological features of hepatocerebral syndrome with mitochondrial DNA depletion due to deoxyguanosine kinase deficiency.

BACKGROUND/AIMS: The aim of this study was to delineate the specific clinical, biological and liver morphological alterations of the hepatocerebral syndrome due to alterations in the deoxyguanosine kinase gene, a rare and severe form of mitochondrial DNA depletion syndrome. METHODS: We report seven cases from three unrelated families with the same mutation in the deoxyguanosine kinase gene. RESULTS: All the patients presented in the first weeks of life with hepatomegaly and progressive liver failure that led to death few months later. Major psychomotor delay and multidirectional nystagmus were reported shortly after onset of the disease. Severe hyperlactacidaemia was constant. Histological examination of the liver disclosed a multifocal injury of hepatocytes with irregular foamy steatosis, cholestasis, and fibrosis, associated with different degrees of hepatosiderosis and glycogen depletion. Liver respiratory chain activities were abnormal in all analysed patients and the amount of liver mitochondrial DNA was severely decreased. An identical homozygous 4bp GATT duplication was identified in the deoxyguanosine kinase gene of all the cases. CONCLUSIONS: These patients, together with patients reported in the literature, permit to delineate the specific features of the hepatocerebral form of mitochondrial DNA depletion syndrome and to differentiate them from other causes of neonatal liver failure.

Biopsy↗
PubMed2005

Satisfaction in different life domains in children receiving home parenteral nutrition and their families.

OBJECTIVE: To assess the quality-of-life (QOL) of children receiving home parenteral nutrition (HPN). STUDY DESIGN: A national multicenter study of 72 patients (median age 4 years) presenting with a digestive disease requiring HPN, and 90 siblings, 67 fathers, and 69 mothers of these children. Median duration of HPN was 2 years (3 months-18 years). QOL was measured using validated, nondisease-specific questionnaires appropriate to the children's ages. RESULTS: The QOL scores were high in patients of all ages and were not significantly different from scores in a reference population of healthy children and adolescents. Lower QOL scores were recorded in the domains related to hospital, health, doctors, medications, and obligations. The QOL was not affected in siblings but was significantly impaired in parents, especially in mothers, who showed a lower level of satisfaction than did fathers for items related to work, inner life, and freedom. Presence of an ileostomy was the only factor that influenced QOL, especially of adolescents. CONCLUSIONS: QOL of HPN-dependent children and siblings is not different from that of healthy children, suggesting that these children actively use effective coping strategies. In contrast, the QOL of parents of HPN-dependent children is low.

Adolescent↗
PubMed2004

[Synaptophysin expression abnormalities in Hirschsprung's disease].

Hirschsprung's disease is defined by the congenital absence of ganglion cells in enteric plexuses. Immunostaining of synaptophysin after formalin fixation may be used to identify hyperplasia of nerve fibers and rarefaction of neuromuscular junctions in Hirschsprung's disease. The aim of the study was to evaluate semi-quantitatively the expression of synaptophysin in Hirschsprung's disease, in correlation with morphologic features. This retrospective study included 3 controls, 42 surgical rectal biopsies performed for suspicion of Hirschsprung's disease in children presenting with lower digestive occlusion or severe chronic constipation, including 18 Hirschsprung's disease, and 23 surgical specimens of Hirschsprung's disease. In the absence of Hirschsprung's disease, synaptophysin-positive fibers were numerous but thin in the muscularis mucosae, thin and scarce in the mucosa and submucosa. Neuromuscular junctions were thin and numerous in the muscularis propria. In Hirschsprung's disease, synaptophysin-positive fibers were coarse, and increased in number on each side of the muscularis mucosae. Plexuses were enlarged, weakly stained, and associated in the connective tissue of the muscularis propria with coarse and intensely stained fibers. In conclusion, staining for synaptophysin could be useful to demonstrate abnormalities of enteric innervation in rectal biopsies performed for suspected Hirschsprung's disease in the absence of acetylcholinesterase staining on frozen sections, in transmural biopsies performed for guiding surgery in Hirschsprung's disease, and in cases of extensive Hirschsprung's disease.

Acetylcholinesterase↗