Publications by authors named "Isabelle Zaccaria"

20 Publications

  • Page 1 of 1

Distinction between arterial and venous occlusion with tissue oxygen pressure in a porcine fascio-cutaneous flap model.

Microsurgery 2020 Nov 6;40(8):881-885. Epub 2020 Oct 6.

Service de Chirurgie Maxillo-faciale, Centre Hospitalier d'Annecy Genevois, Epagny Metz-Tessy, France.

Background: In recent years, many devices have been developed to monitor free flaps. The Licox probe, which measures tissue oxygen pressure (PtO ), is one of the available devices. Our aim was to demonstrate that PtO could distinguish arterial from venous occlusion in a porcine fascio-cutaneous flap model.

Materials And Methods: Twenty pigs (Sus scrofa domestica, Youna strain, males) were included in this study. The median weight was 87.6 kg (84.6-90.8). Bilateral fascio-cutaneous flaps based on the superficial inferior epigastric pedicle were harvested from each pig. Thirty-eight flaps were analyzed in this study and were monitored by a Licox system during vascular occlusion. The flaps were randomized into two groups according to the clamped vessel: the arterial group (n = 19) and the venous group (n = 19). After a stabilization period of almost 40 min, vascular clamping (arterial or venous) was performed using a microvascular clamp for almost 20 min. The curve profiles were compared between arterial and venous occlusion.

Results: The inflection point was reached significantly faster in the arterial group: 11 min (9-16) for arterial clamping and 17 min (13-23) for venous clamping (p = .001). A total of 18/19 (95%) pigs in the arterial group and 13/19 (68%) in the venous group (p = .09) reached a level lower than 10 mmHg. The median duration for pressure to drop below 10 mmHg was 9 min (6-12) for arterial clamping and 10 min (9-16) for venous clamping (p = .06).

Conclusion: We showed that PtO decreased faster in cases of arterial occlusion than in cases of venous occlusion in a pig model. Based on this observation, it may be possible to distinguish arterial from venous occlusion.
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http://dx.doi.org/10.1002/micr.30661DOI Listing
November 2020

Variants of the mannose-binding lectin gene in the Benin population: heterozygosity for the p.G57E allele may confer a selective advantage. 2007.

Hum Biol 2009 Dec;81(5-6):899-909

Human mannose- binding lectin (MBL) plays an important role in innate immunity. MBL deficiency is associated with mutations in the promoter region and in exon 1 of the MBL2 gene. Such deficiency has been correlated with elevated incidence of infections in infancy and in immunocompromised adults. We determined the distribution profile of the MBL2 gene variants in the general population of Benin (West Africa) and in a vulnerable subset of children with sickle cell disease (SCD) (SS homozygotes). Five hundred forty-two healthy individuals (274 newborns, 268 adults) and 128 patients with SCD (35 newborns, 93 children) were screened for the common variant alleles in the MBL2 secretor haplotype region (exon 1 and promoter). The p.G57E variant allele was the most frequent allele compared to p.G54D (27.5% vs. 1.6%, respectively). The p.R52C allele was not found in this population. There was no difference in allele or genotype frequencies between healthy newborns and newborns with SCD. Alleles associated with MBL deficiency were more frequent in adults than in newborns (69.8% vs. 57.3%, respectively; p=0.002). This enrichment was exclusively due to an elevated proportion of heterozygotes for the p.G57E allele (47.0% vs. 35.3%,respectively; p=0.004), supporting a potential selective advantage of this genotype. Our results, compared to those reported in other African countries, support the implication of the MBL2 gene in various major infections in Africa, such as meningitis and tuberculosis in HIV- positive patients.
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http://dx.doi.org/10.3378/027.081.0630DOI Listing
December 2009

Genotype-phenotype correlations in fetuses and neonates with autosomal recessive polycystic kidney disease.

Kidney Int 2010 Feb 25;77(4):350-8. Epub 2009 Nov 25.

Assistance Publique-Hôpitaux de Paris, Université Paris Diderot, Hôpital Robert Debré, Laboratoire de Biochimie Génétique, INSERM U722, Paris, France.

The prognosis of autosomal recessive polycystic kidney disease is known to correlate with genotype. The presence of two truncating mutations in the PKHD1 gene encoding the fibrocystin protein is associated with neonatal death while patients who survive have at least one missense mutation. To determine relationships between genotype and renal and hepatic abnormalities we correlated the severity of renal and hepatic histological lesions to the type of PKHD1 mutations in 54 fetuses (medical pregnancy termination) and 20 neonates who died shortly after birth. Within this cohort, 55.5% of the mutations truncated fibrocystin. The severity of cortical collecting duct dilatations, cortical tubule and glomerular lesions, and renal cortical and hepatic portal fibrosis increased with gestational age. Severe genotypes, defined by two truncating mutations, were more frequent in patients of less than 30 weeks gestation compared to older fetuses and neonates. When adjusted to gestational age, the extension of collecting duct dilatation into the cortex and cortical tubule lesions, but not portal fibrosis, was more prevalent in patients with severe than in those with a non-severe genotype. Our results show the presence of two truncating mutations of the PKHD1 gene is associated with the most severe renal forms of prenatally detected autosomal recessive polycystic kidney disease. Their absence, however, does not guarantee survival to the neonatal period.
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http://dx.doi.org/10.1038/ki.2009.440DOI Listing
February 2010

Crohn's disease and early exposure to domestic refrigeration.

PLoS One 2009 29;4(1):e4288. Epub 2009 Jan 29.

Digestive Disease Research Center, Medical Sciences, University of Tehran, Tehran, Iran.

Background: Environmental risk factors playing a causative role in Crohn's Disease (CD) remain largely unknown. Recently, it has been suggested that refrigerated food could be involved in disease development. We thus conducted a pilot case control study to explore the association of CD with the exposure to domestic refrigeration in childhood.

Methodology/principal Findings: Using a standard questionnaire we interviewed 199 CD cases and 207 age-matched patients with irritable bowel syndrome (IBS) as controls. Cases and controls were followed by the same gastroenterologists of tertiary referral clinics in Tehran, Iran. The questionnaire focused on the date of the first acquisition of home refrigerator and freezer. Data were analysed by a multivariate logistic model. The current age was in average 34 years in CD cases and the percentage of females in the case and control groups were respectively 48.3% and 63.7%. Patients were exposed earlier than controls to the refrigerator (X2 = 9.9, df = 3, P = 0.04) and refrigerator exposure at birth was found to be a risk factor for CD (OR = 2.08 (95% CI: 1.01-4.29), P = 0.05). Comparable results were obtained looking for the exposure to freezer at home. Finally, among the other recorded items reflecting the hygiene and comfort at home, we also found personal television, car and washing machine associated with CD.

Conclusion: This study supports the opinion that CD is associated with exposure to domestic refrigeration, among other household factors, during childhood.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0004288PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2629547PMC
May 2009

Effect of transfusion therapy on cerebral vasculopathy in children with sickle-cell anemia.

Haematologica 2009 Jan 4;94(1):123-6. Epub 2008 Dec 4.

Assistance Publique-Hôpitaux de Paris, Service d'Hématologie Pédiatrique, Hôpital Necker, Paris, France.

This retrospective study assessed the long-term effect of transfusional exchange therapy on MRA/MRI abnormalities in 24 homozygous sickle-cell anemia (HbSS) children presenting with abnormal brain MRA. The median time elapsed from baseline to last available MRA was 29 months. Follow-up MRAs showed improvement, stabilization or worsening of cerebrovascular lesions in 11, 6 and 7 patients respectively. Complete normalization of MRA was observed in 6 patients within a mean time of 1.4 years, but stenosis recurred at the same location in the 4 patients in whom transfusion therapy was discontinued. Baseline severe stenosis/occlusion of large cerebral arteries and occurrence of moyamoya syndrome were significantly associated with an absence of improvement of the cerebral vasculopathy. These data emphasize the heterogeneity of the course of cerebrovasculopathy in SS children receiving chronic transfusion. Further studies are needed to determine whether different therapeutic approaches have to be considered according to these different evolutive patterns in SS children.
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http://dx.doi.org/10.3324/haematol.13610DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2625404PMC
January 2009

Inter-observer variability in chest radiograph reading for diagnosing acute lung injury in children.

Pediatr Pulmonol 2008 Oct;43(10):987-91

Service de Réanimation et Surveillance Continue Pédiatriques, Hôpital Robert-Debré, Assistance Publique-Hôpitaux de Paris et Université Paris Diderot-Paris VII, Paris, France.

Acute lung injury (ALI), including its most serious form called acute respiratory distress syndrome (ARDS), is a devastating disease that can occur at any age. ALI/ARDS accounts for only 5-8% of admissions to pediatric intensive care units (PICUs) but is fatal in 30-60% of cases. International multicenter prospective studies are needed to better understand pediatric ALI/ARDS. However, a reproducible definition of ALI/ARDS is crucial to ensure that study populations are homogeneous. We designed a retrospective review to test the inter-observer variability of chest radiograph interpretation for presence of the American-European Consensus Conference (AECC) radiographic criterion for ALI/ARDS. The medical files of 24 children ventilated for ALI/ARDS in our PICU between January 1993 and December 2002 were reviewed. Five pediatric radiologists and five pediatric intensivists interpreted one frontal chest radiograph (FCR) per patient taken on the day of ALI/ARDS diagnosis. Each reader indicated whether the radiograph showed the AECC radiographic criterion for ALI/ARDS. Data analysis involved comparing each reader to all the others based on the raw agreement and Kappa coefficient (kappa). Features in the 24 patients were consistent with earlier studies. Global inter-observer agreement beyond chance was fair (kappa = 0.29 +/- 0.02) among the five radiologists (kappa = 0.26 +/- 0.05) and among the five intensivists (kappa = 0.29 +/- 0.05). Thus, considerable inter-observer variability occurred in assessing the radiographic criterion for ALI/ARDS, as previously shown in adults. Given the low incidence of ALI/ARDS in children, this variability may have a large impact in studies of pediatric ALI/ARDS.
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http://dx.doi.org/10.1002/ppul.20890DOI Listing
October 2008

Can bronchodilator response predict bronchial response to methacholine in preschool coughers?

Pediatr Pulmonol 2008 Aug;43(8):815-21

AP-HP Robert Debré Hospital, Physiology Department, Paris, France.

The aim of the present study was to determine the relationship between bronchodilator response, assessed by interrupter resistance (Rint), and bronchial reactivity in preschool children with chronic cough. Thirty-eight children coughers (median age 5.0 years, range 2.8-6.4) were tested. Bronchodilator response was recorded within 4 months before methacholine challenge. Response to the latter was assessed using transcutaneous partial pressure of oxygen and Rint. Children were considered responders if a 20% fall in transcutaneous partial pressure of oxygen occurred during the bronchial challenge. Bronchodilator response was not different between responders (n = 24) and nonresponders (n = 14) [median (range) -0.11 (-0.44-0.09) vs. -0.08 (-0.21-0.10) kPa L(-1) sec; respectively]. However, none of the nonresponders had a bronchodilator response larger than -0.21 kPa L(-1) sec, this cutoff had a 100% positive and a 44% negative predictive value to predict a positive methacholine challenge. The relationship between bronchodilator response and bronchial methacholine responsiveness reached the limit of significance (P = 0.048). Furthermore, the magnitude of the bronchodilator response was correlated to the level of methacholine-induced level of bronchoconstriction (P = 0.01), and to the postchallenge bronchodilation (P = 0.04), all values expressed as % predicted. Moreover, the postbronchodilator Rint value obtained with preceding methacholine challenge was lower than the postbronchodilator value without preceding methacholine challenge in 71.4% (10/14) of the nonresponders and in only 33.3% (8/24) of the responders. Conclusions in preschool coughers bronchodilator response, assessed by the interrupter technique, was correlated to the bronchial responsiveness to methacholine. Non responders had a bronchodilator response not larger than -0.21 kPa L(-1) sec.
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http://dx.doi.org/10.1002/ppul.20877DOI Listing
August 2008

Variants of the mannose-binding lectin gene in the Benin population: heterozygosity for the p.G57E allele may confer a selective advantage.

Hum Biol 2007 Dec;79(6):687-97

'INSERM, U763, Hôpital Robert Debré, 48 Blvd. Sérurier, Paris, F-75019 France.

Human mannose-binding lectin (MBL) plays an important role in innate immunity. MBL deficiency is associated with mutations in the promoter region and in exon 1 of the MBL2 gene. Such deficiency has been correlated with elevated incidence of infections in infancy and in immunocompromised adults. We determined the distribution profile of the MBL2 gene variants in the general population of Benin (West Africa) and in a vulnerable subset of children with sickle cell disease (SCD) (SS homozygotes). Five hundred forty-two healthy individuals (274 newborns, 268 adults) and 128 patients with SCD (35 newborns, 93 children) were screened for the common variant alleles in the MBL2 secretor haplotype region (exon 1 and promoter). The p.G57E variant allele was the most frequent allele compared to p.G54D (27.5% vs. 1.6%, respectively). The p.R52C allele was not found in this population. There was no difference in allele or genotype frequencies between healthy newborns and newborns with SCD. Alleles associated with MBL deficiency were more frequent in adults than in newborns (69.8% vs. 57.3%, respectively; p = 0.002). This enrichment was exclusively due to an elevated proportion of heterozygotes for the p.G57E allele (47.0% vs. 35.3%, respectively; p = 0.004), supporting a potential selective advantage of this genotype. Our results, compared to those reported in other African countries, support the implication of the MBL2 gene in various major infections in Africa, such as meningitis and tuberculosis in HIV-positive patients.
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http://dx.doi.org/10.1353/hub.2008.0012DOI Listing
December 2007

High cyclin E staining index in blastemal, stromal or epithelial cells is correlated with tumor aggressiveness in patients with nephroblastoma.

PLoS One 2008 May 21;3(5):e2216. Epub 2008 May 21.

Assistance Publique-Hôpitaux de Paris, Hôpital Robert Debré, Service d'Anatomie et de Cytologie Pathologiques, Paris, France.

Purpose: Identifying among nephroblastoma those with a high propensity for distant metastases using cell cycle markers: cyclin E as a regulator of progression through the cell cycle and Ki-67 as a tumor proliferation marker, since both are often deregulated in many human malignancies.

Methodology/principal Findings: A staining index (SI) was obtained by immunohistochemistry using anti-cyclin E and anti-Ki-67 antibodies in paraffin sections of 54 postchemotherapy nephroblastoma including 42 nephroblastoma without metastasis and 12 with metastases. Median cyclin E and Ki-67 SI were 46% and 33% in blastemal cells, 30% and 10% in stromal cells, 37% and 29.5% in epithelial cells. The highest values were found for anaplastic nephroblastoma. A correlation between cyclin E and Ki-67 SI was found for the blastemal component and for the epithelial component. Univariate analysis showed prognostic significance for metastases with cyclin E SI in stromal cells, epithelial cells and blastemal cells (p = 0.03, p = 0.01 and p = 0.002, respectively) as well as with Ki-67 SI in blastema (p<10(-4)). The most striking data were that both cyclin E SI and blastemal Ki-67 SI discriminated between patients with metastases and patients without metastasis among intermediate-risk nephroblastoma.

Conclusions: Our findings show that a high cyclin E SI in all components of nephroblastoma is correlated with tumor aggressiveness and metastases, and that assessment of its expression may have prognostic value in the categorization of nephroblastoma.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0002216PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2373888PMC
May 2008

Independent effect of visceral adipose tissue on metabolic syndrome in obese adolescents.

Horm Res 2008 21;70(1):22-8. Epub 2008 May 21.

INSERM, U690, Hôpital Robert Debré, Paris, France.

Background/aims: Obesity is linked to metabolic complications, even in children, but the role of the distribution of adiposity is unclear. We aimed to assess which compartment of fat mass - total (TFM), visceral (VFM) or subcutaneous (SCFM)--is related to metabolic complications in overweight and obese children and adolescents.

Design: Analyses were conducted in 159 overweight or obese children and adolescents (median body mass index 4.0 SD). TFM was measured by dual-energy x-ray absorptiometry. Distribution of abdominal fat was assessed by MRI. Insulin resistance (IR) was determined using a homeostatic model assessment. The definition of metabolic syndrome (MS) was derived from National Cholesterol Education Program ATP III.

Results: A parental history of obesity was positively and significantly associated with fat content of the three compartments (TFM: beta = 2.22; VFM: beta = 0.17; SCFM: beta = 0.12, respectively). VFM was also associated with gender (beta = -0.29) and ethnicity (beta = -0.54). TFM was a significant and independent determinant of IR (beta = 0.02) whereas IR and VFM only were significantly related to MS (OR = 3.55 and 3.66 respectively).

Conclusion: Our data indicate that even in overweight children VFM was influenced by several factors such as sex and ethnicity and that a relationship was evidenced between the amount of VFM and MS.
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http://dx.doi.org/10.1159/000129674DOI Listing
August 2008

Long-term outcome of prenatally detected posterior urethral valves: single center study of 65 cases managed by primary valve ablation.

J Urol 2008 Jan 19;179(1):307-12; discussion 312-3. Epub 2007 Nov 19.

Service de Chirurgie et Urologie Pédiatrique, Université Paris 7, Hôpital Robert Debré, Assistance Publique Hôpitaux de Paris, Paris, France.

Purpose: Management of posterior urethral valves is significantly modified by the prenatal diagnosis. Our aim was to assess long-term outcome of children with prenatally detected posterior urethral valves treated at our institution by primary valve ablation without routine urinary drainage or diversion.

Materials And Methods: A total of 79 cases of posterior urethral valves were detected prenatally at our hospital between 1987 and 2004. Of these cases 65 were managed postnatally, while pregnancy was terminated in 14. We studied the prenatal parameters of gestational age at diagnosis, renal parenchyma on ultrasound and amniotic fluid volume. Fetal urine was analyzed when indicated. Long-term outcome was assessed.

Results: Primary valve ablation was done in all cases except 2. Median followup was 6.8 years (range 1 to 14.3). At the end of followup there were 11 cases of renal failure (17%) with 5 detected before 24 weeks of gestation, 6 cases of oligohydramnios and 9 cases of abnormal parenchyma. Gestational age at diagnosis and oligohydramnios were statistically significant predictors of final renal outcome (p = 0.003 and p = 0.02, respectively), while renal parenchymal changes were not (p = 0.23). When fetal urinalysis detected good prognosis (12 cases) renal failure developed in none, compared to 2 of the 3 cases with a bad prognosis. Continence was achieved in 42 of 55 toilet trained children (76%), 3 had nocturnal enuresis and 10 (18%) were incontinent.

Conclusions: Our long-term results of prenatally detected posterior urethral valves confirm that early valve ablation can be considered as the primary treatment in the majority of patients, without the need for preoperative drainage or diversion. Gestational age at diagnosis and volume of amniotic fluid are significant predictors of postnatal renal outcome.
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http://dx.doi.org/10.1016/j.juro.2007.08.160DOI Listing
January 2008

Postnatal clinical and imaging follow-up of infants with prenatal isolated mild ventriculomegaly: a series of 101 cases.

Pediatr Radiol 2007 Oct 28;37(10):981-9. Epub 2007 Aug 28.

Department of Paediatric Imaging, Hôpital Robert Debré, Paris, France.

Background: Postnatal imaging and clinical outcome of fetuses with isolated mild ventriculomegaly (IMV) have never been systematically analysed.

Objective: To evaluate the postnatal clinical outcomes of a large cohort of fetuses with IMV and to correlate them with pre- and postnatal imaging.

Materials And Methods: We report a prospective study of 101 fetuses with IMV (10-15 mm ventriculomegaly with otherwise normal US, MRI, karyotype and TORCH screening). IMV was divided into minor (10-11.9 mm) and moderate (12-15 mm) ventriculomegaly. Ventriculomegaly was considered uni- or bilateral, stable, progressive, regressive or resolved according to the prenatal US follow-up. Clinical follow-up was performed by a neuropaediatrician. Postnatal imaging included cranial US (n = 71) and MRI (n = 76).

Results: The outcome of minor and moderate IMV was excellent in 94% and 85% of infants, respectively. It was not different between uni- and bilateral IMV, and between stable, regressive and resolved IMV, and was independent of gestational age at diagnosis and gender. Fixed neurological abnormalities were observed in nine infants. Postnatal MRI showed white-matter abnormalities in 14 infants, including 6 of the 9 infants with a poor outcome.

Conclusion: The prognosis was slightly better in minor IMV than in moderate IMV. Postnatal MRI showed white-matter abnormalities in two-thirds of the infants with a poor outcome.
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http://dx.doi.org/10.1007/s00247-007-0582-2DOI Listing
October 2007

Interrupter resistance short-term repeatability and bronchodilator response in preschool children.

Respir Med 2007 Dec 27;101(12):2482-7. Epub 2007 Aug 27.

Physiology Department, Robert Debré Hospital, 48 Bd Sérurier, 75019 Paris, France.

Unlabelled: Interrupter resistance (Rint) technique can be easily and successfully performed in preschool children. The establishment of Rint short-term repeatability is essential to interpret any Rint change after a pharmacological intervention.

Aims Of The Study: In preschool children with asthma or chronic cough: (1) to assess two indices of short-term repeatability: (a) intra-measurement and (b) within-occasion between-test repeatability; (2) to study the relationship between short-term repeatability and bronchodilator response (BDR).

Results: Rint intra-measurement repeatability assessed by the coefficient of variation was similar at baseline and after bronchodilator in asthmatics and in coughers (median 10% and 12%, respectively). There was no significant difference between asthmatics and coughers for both coefficient of repeatability (CR) (0.25 kPa L(-1)s and 32% of predicted vs 0.16 kPa L(-1) s and 21% of predicted, respectively) and BDR (median -14.7% vs -21.1% of predicted, respectively). However, in 20% of the study children, baseline variability of Rint modified the significance of the BDR.

Conclusion: In the present study, Rint short-term repeatability was similar to that of previous studies. Similar Rint repeatability in coughers and in asthmatic children favored the use of asthmatic CR for both populations, and a -35% cut-off as a positive BDR. In 20% of study children, baseline Rint variability could influence the significance of the BDR. In order to improve assessment of BDR using Rint, further studies are needed (1) to compare the variability of Rint to other resistance measurement techniques and (2) to define the best method for Rint calculation and for expression of BDR.
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http://dx.doi.org/10.1016/j.rmed.2007.07.005DOI Listing
December 2007

Prevalence of CARD15/NOD2 mutations in Caucasian healthy people.

Am J Gastroenterol 2007 Jun 23;102(6):1259-67. Epub 2007 Feb 23.

INSERM Avenir U763; AP-HP; Université Paris 7, Hôpital Robert Debré, Paris, France.

Background: Crohn's disease (CD) has been associated with CARD15/NOD2 mutations in Caucasians. The R702W, G908R, and 1007fs mutations represent 82% of the mutated chromosomes. The relative risk of developing CD in homozygous or compound heterozygous people has been estimated as between 10 and 40 times that of the general population. This high risk may support the opinion that CARD15/NOD2 variants are strong CD risk factors at the individual and population levels.

Subjects And Methods: The allele and genotype frequencies were calculated for the R702W, G908R, and 1007fs mutations in 3,575 Caucasian healthy controls recruited by 15 groups distributed on three continents. Geographic homogeneity was tested and the observed proportion of double mutants was compared with the expected value using chi2 tests.

Results: The allele frequencies of the R702W, G908R, and 1007fs mutations were 4.3% (3.6-4.9), 1.2% (0.8-1.6), and 2.3% (1.8-2.8), respectively, with large geographic fluctuations of the G908R, 1007fs, and wild-type alleles (P<0.0001). At the population level, no simple relationship was observed between mutation frequencies and the disease incidences in the studied populations. At the individual level, no significant deficit of double-dose mutation carriers among healthy controls was found, providing strong evidence that the penetrances of the most at-risk genotypes are low.

Conclusion: Altogether, these data confirm that CARD15/NOD2 acts in interaction with other unknown risk cofactors.
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http://dx.doi.org/10.1111/j.1572-0241.2007.01149.xDOI Listing
June 2007

Tracheostomy does not improve the outcome of patients requiring prolonged mechanical ventilation: a propensity analysis.

Crit Care Med 2007 Jan;35(1):132-8

Intensive Care Unit, Avicenne Teaching Hospital, 125 route de Stalingrad, 93000 Bobigny, France.

Objective: To examine the association between the performance of a tracheostomy and intensive care unit and postintensive care unit mortality, controlling for treatment selection bias and confounding variables.

Design: Prospective, observational, cohort study.

Setting: Twelve French medical or surgical intensive care units.

Patients: Unselected patients requiring mechanical ventilation for > or =48 hrs enrolled between 1997 and 2004.

Interventions: None.

Measurements And Main Results: Two models of propensity scores for tracheostomy were built using multivariate logistic regression. After matching on these propensity scores, the association of tracheostomy with outcomes was assessed using multivariate conditional logistic regression. Results obtained with the two models were compared. Of the 2,186 patients included, 177 (8.1%) received a tracheostomy. Both models led to similar results. Tracheostomy did not improve intensive care unit survival (model 1: odds ratio, 0.94; 95% confidence interval, 0.63-1.39; p = .74; model 2: odds ratio, 1.12; 95% confidence interval, 0.75-1.67; p = .59). There was no difference whether tracheostomy was performed early (within 7 days of ventilation) or late (after 7 days of ventilation). In patients discharged free from mechanical ventilation, tracheostomy was associated with increased postintensive care unit mortality when the tracheostomy tube was left in place (model 1: odds ratio, 3.73; 95% confidence interval, 1.41-9.83; p = .008; model 2: odds ratio, 4.63; 95% confidence interval, 1.68-12.72, p = .003).

Conclusions: Tracheostomy does not seem to reduce intensive care unit mortality when performed in unselected patients but may represent a burden after intensive care unit discharge.
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http://dx.doi.org/10.1097/01.CCM.0000251134.96055.A6DOI Listing
January 2007

Microstructural development of human brain assessed in utero by diffusion tensor imaging.

Pediatr Radiol 2006 Nov 8;36(11):1133-40. Epub 2006 Sep 8.

Department of Paediatric Imaging, Hôpital Robert Debré, AP-HP, 48 boulevard Serurier, Paris 75019, France.

Background: Diffusion-weighted MR imaging (DWI) has been shown to be a great tool to assess white matter development in normal infants. Comparison of cerebral diffusion properties between preterm infants and fetuses of corresponding ages should assist in determining the impact of premature ex utero life on brain maturation.

Objective: To assess in utero maturation-dependent microstructural changes of fetal cerebral white matter using diffusion tensor MR imaging.

Materials And Methods: An echoplanar sequence with diffusion gradient (b=700 s/mm(2)) applied in six non-colinear directions was performed between 31 and 37(+3) weeks of gestation in 24 fetuses without cerebral abnormality on T1- and T2-weighted images. Apparent diffusion coefficient (ADC) and fractional anisotropy (FA) were measured in the white matter.

Results: Mean ADC values were 1.8 microm(2)/ms in the centrum semiovale, 1.2 microm(2)/ms in the splenium of the corpus callosum and 1.1 microm(2)/ms in the pyramidal tract. The paired Wilcoxon rank test showed significant differences in ADC between these three white matter regions. Mean FA values were 1.1%, 3.8% and 4.7%, respectively, in the centrum semiovale, corpus callosum and pyramidal tract. A significant age-related decrease in ADC and an increase in FA towards term were demonstrated in the pyramidal tract and corpus callosum.

Conclusion: Diffusion tensor imaging in utero can provide a quantitative assessment of the microstructural development of fetal white matter. Anisotropic parameters of the diffusion tensor should improve with technical advances.
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http://dx.doi.org/10.1007/s00247-006-0266-3DOI Listing
November 2006

Time course of catch-up in adiposity influences adult anthropometry in individuals who were born small for gestational age.

Pediatr Res 2005 Aug 31;58(2):243-7. Epub 2005 Jul 31.

INSERM U457, Robert Debré Hospital, 75019 Paris, France.

Although necessary for a normal final height in individuals who were born small for gestational age (SGA), catch-up growth is associated with drastic changes in body composition that have been suspected to favor the later development of the long-term metabolic complications by promoting central adiposity; however, the specific contribution of catch-up itself on these later complications remains unclear. Therefore, the aim of the study was to characterize the dynamic changes in adiposity during childhood in individuals who were born SGA and to investigate their consequences on adulthood. The magnitude and the time course of postnatal changes in body mass index (BMI) relative to birth and their consequences on adult adiposity were investigated in 127 adults who were born SGA and had available serial anthropometric data in childhood (0-6 y) and adulthood. Catch-up in BMI, observed in 91% of individuals who were born SGA, was mostly completed within the first or second year of age. Overall, adult BMI was correlated with the magnitude of gain in BMI during childhood. However, this effect was significant only when this gain persisted after the first year of life. Similarly, the influence of the magnitude in gain in BMI on the risk for adult BMI >25 kg/m(2) was significantly influenced by the age at which the gain in BMI occurred. In summary, although the extent of catch-up in BMI affects adiposity in adulthood, this effect is mostly deleterious when occurring after 1 y of age, suggesting that a rapid catch-up process should be more suitable than a delayed one. Whether this observation holds through regarding the metabolic syndrome remains to be elucidated.
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http://dx.doi.org/10.1203/01.PDR.0000169980.35179.89DOI Listing
August 2005

Alternate-site testing is reliable in children and adolescents with type 1 diabetes, except at the forearm for hypoglycemia detection.

Diabetes Care 2005 Mar;28(3):710-1

Department of Endocrinology, Robert Debre Teaching Hospital, 75019 Paris, France.

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http://dx.doi.org/10.2337/diacare.28.3.710DOI Listing
March 2005

The French Congenital Central Hypoventilation Syndrome Registry: general data, phenotype, and genotype.

Chest 2005 Jan;127(1):72-9

Service de Physiologie, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris, Université Paris 7, Paris, France.

Objective: To analyze the main clinical features, genetic mutations, and outcomes of patients of the French Congenital Central Hypoventilation Syndrome (CCHS) Registry.

Design: A country-wide cohort established throughout a long-term multicenter effort.

Patients: Seventy French patients with CCHS (29 male patients and 41 female patients).

Methods: The following items were analyzed: the most important moments of the disease course; the main clinical characteristics; associated pathologic conditions; management; clinical outcome; and genetic mutations.

Results: An average of four new cases of CCHS per year was observed in the last 5 years. Thus, the incidence may be estimated to be 1 per 200,000 live births in France. The median age at diagnosis was 3.5 months (range, 0.5 to 15 months) before 1995 and < 2 weeks in the last 5 years (p = 0.01). CCHS occurred in isolation in 58 of 70 patients. In the remainder, it was associated with Hirschsprung disease (HSCR) [nine patients], Hirschsprung and neural crest tumor (two patients), and growth hormone deficiency (one patient). Among the 50 patients who lived beyond 1 year of age, all but one received nighttime ventilation, with 10 of them (20%) receiving it noninvasively. Three patients (6%) required daytime ventilatory support in addition to nighttime ventilation. The overall mortality rate was 38% (95% confidence interval [CI], 27 to 49%). The median age at death was 3 months (range, 0.4 months to 21 years). The 2-year mortality rate was greater in male patients than in female patients (p = 0.02; relative risk [RR], 2.71; 95% CI, 1.14 to 6.47) but was not affected by HSCR (p = 0.93; RR, 0.95; 95% CI, 0.28 to 3.2). The 43 patients who are currently alive (11 men; sex ratio, 0.4) have a mean age of 9 years (range, 2 months to 27 years). Among the 34 patients tested thus far, heterozygous mutations of the paired-like homeobox gene 2B (PHOX2B) gene were found in 31 patients (91%).

Conclusion: Our four major findings are the extreme rarity of CCHS, the improved recognition over time, the lack of effect of HSCR on the mortality rate, and the high frequency of PHOX2B mutations.
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http://dx.doi.org/10.1378/chest.127.1.72DOI Listing
January 2005