Trousseau University Hospital

Paris, France

Trousseau University Hospital

Paris, France
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Etard C.,Institute for Radiological Protection and Nuclear Safety | Bigand E.,French Society of Medical Physicists SFPM | Bigand E.,La Timone University Hospital | Salvat C.,French Society of Medical Physicists SFPM | And 8 more authors.
European Radiology | Year: 2017

Objectives: A national retrospective survey on patient doses was performed by the French Society of Medical physicists to assess reference levels (RLs) in interventional radiology as required by the European Directive 2013/59/Euratom. Methods: Fifteen interventional procedures in neuroradiology, vascular radiology and osteoarticular procedures were analysed. Kerma area product (KAP), fluoroscopy time (FT), reference air kerma and number of images were recorded for 10 to 30 patients per procedure. RLs were calculated as the 3rd quartiles of the distributions. Results: Results on 4600 procedures from 36 departments confirmed the large variability in patient dose for the same procedure. RLs were proposed for the four dosimetric estimators and the 15 procedures. RLs in terms of KAP and FT were 90 Gm.cm2 and 11 mins for cerebral angiography, 35 Gy.cm2 and 16 mins for biliary drainage, 75 Gy.cm2 and 6 mins for lower limbs arteriography and 70 Gy.cm2 and 11 mins for vertebroplasty. For these four procedures, RLs were defined according to the complexity of the procedure. For all the procedures, the results were lower than most of those already published. Conclusions: This study reports RLs in interventional radiology based on a national survey. Continual evolution of practices and technologies requires regular updates of RLs. Key Points: • Delivered dose in interventional radiology depends on procedure, practice and patient. • National RLs are proposed for 15 interventional procedures. • Reference levels (RLs) are useful to benchmark practices and optimize protocols. • RLs are proposed for kerma area product, air kerma, fluoroscopy time and number of images. • RLs should be adapted to the procedure complexity and updated regularly. © 2017 European Society of Radiology


Bichali S.,University of Nantes | Brault D.,Argenteuil Hospital | Masserot C.,Trousseau University Hospital | Boscher C.,University of Nantes | And 9 more authors.
European Journal of Pediatrics | Year: 2017

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzyme defect often presenting with neonatal jaundice and/or hemolytic anemia. G6PD hemolytic events are linked with exposure to a pro-oxidant agent. We here report three cases of initial G6PD crises in breastfed children secondary to maternal consumption of a tonic drink which contains quinine. Quinine was found in breast milk of one of the mothers after she consumed tonic water. Conclusion: The amount of quinine that is transmitted through breast milk appears to be sufficient to induce G6PD crises in breastfed children. We hence recommend that consumption of quinine-containing sodas during breastfeeding should be avoided in populations with a high prevalence of G6PD deficiency.(Table presented.) © 2017 Springer-Verlag GmbH Germany


Collongues N.,University of Strasbourg | Marignier R.,Lyons University Hospital | Zephir H.,Lille University Hospital Center | Papeix C.,Pitie Salpetriere Hospital | And 15 more authors.
Neurology | Year: 2010

Background: Neuromyelitis optica (NMO) is a rare inflammatory disease. Average age at onset is 35 years. Few data exist on patients with pediatric-onset NMO (p-NMO), with disease onset before age 18 years. We report the clinical and paraclinical features and long-term outcome of patients with p-NMO and compare them with a large adult-onset NMO (a-NMO) cohort. Methods: We performed a retrospective, multicenter study of patients with p-NMO in pediatric and adult medical centers. We identified 125 patients with NMO (12 p-NMO; 113 a-NMO) fulfilling the 2006 criteria. Data were collected using hospital files and standardized assessment forms for NMO. Results: Patients with p-NMO were followed up during a mean 19.3 years. Median age at onset was 14.5 years (4.1-17.9) with a female:male ratio of 3:1. Three patients (25%) fulfilled Paty criteria for multiple sclerosis on first brain MRI, including one patient with acute disseminated encephalomyelitis. Median interval between onset and residual Expanded Disability Status Scale (EDSS) score 4 was 20.7 years, score 6 was 26 years, and score 7 was 28.7 years. Median interval between onset and residual visual loss ≤1/10 was 1.3 years. Compared with a-NMO, p-NMO showed a longer time to EDSS scores 4 and 6, largely explained by the severity of the first myelitis in the a-NMO group. Time to first treatment was longer in the p-NMO group (13.1 vs 3.4 years). Conclusion: Patients with p-NMO can present a diffuse inflammatory process on first brain MRI and have a longer time to disability than patients with a-NMO. Copyright © 2010 by AAN Enterprises, Inc. All rights reserved.


Hogan J.,REIN Registry | Savoye E.,REIN Registry | Macher M.-A.,France Robert Debre University Hospital | Bachetta J.,University of Lyon | And 5 more authors.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association | Year: 2014

BACKGROUND: Major inequalities in access to renal transplant waiting lists have been demonstrated among adult patients both in the USA and Europe. In this French nationwide study, we sought to ascertain the influence of patient and centre characteristics.METHODS: We included all children (<18 years) in the French End-Stage Renal Disease National Registry, who started renal replacement therapy (RRT) between 1 January 2002 and 31 December 2011. The primary outcome was the probability of being listed within 6 months after starting RRT. Hierarchical logistic regression models were used to study the association between the patient or the centre characteristics and the outcome. Centre effects were assessed by studying the centre-level residual variance.RESULTS: A total of 614 incident patients treated in 54 centres were included; 421 (68.6%) were listed within 6 months after starting RRT. A higher risk of not being listed was found in patients younger than 2 years or with a renal disease with a high risk of recurrence after transplantation [odds ratio (OR): 2.61; 95% confidence interval (CI): 1.37-4.97]. We found a significant vintage effect: the probability of not being listed decreased over time (OR per 1 year +0.83, 95% CI: 0.74-0.94). Although we found no significant gender effect, a trend towards disfavouring girls persisted over the study period. We found a significant centre effect that remained after adjusting for patient characteristics. However, none of the centre characteristics that we studied (centre size, pre-emptive transplantation program, paediatric versus adult centres and the proportion of patients on the waiting list placed on inactive status during the first month after listing) explained this variability.CONCLUSIONS: Our study confirms inequalities among children in rapid access to the renal transplant waiting list and shows that patient and centre characteristics play a role in these inequalities. Further studies focusing on the organization and practices of the centres are needed to explain the remaining variability. © The Author 2014. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.


Quelin C.,Rennes Sud University Hospital | Loget P.,Rennes Pontchaillou University Hospital | Verloes A.,French Institute of Health and Medical Research | Bazin A.,Cerba Laboratory | And 22 more authors.
European Journal of Medical Genetics | Year: 2012

The Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive multiple congenital malformation syndrome caused by dehydrocholesterol reductase deficiency. The diagnosis is confirmed by high 7- and secondarily 8-dehydrocholesterol levels in plasma and tissues and/or by detection of biallelic mutations in the DHCR7 gene. The phenotypic spectrum of SLOS is broad, ranging from a mild phenotype combining subtle physical anomalies with behavioral and learning problems, to a perinatally lethal multiple malformations syndrome. The fetal phenotype of SLOS has been poorly described in the literature. We report a series of 10 fetuses with molecularly proven SLOS. Even in young fetuses, the facial dysmorphism appears characteristic. Genital abnormalities are rare in 46,XX subjects. Gonadal differentiation appears histologically normal and in agreement with the chromosomal sex, contrary to what has been previously stated. We observed some previously unreported anomalies: ulnar hypoplasia, vertebral segmentation anomalies, congenital pulmonary adenomatoid malformation, fused lungs, gastroschisis, holomyelia and hypothalamic hamartoma. This latter malformation proves that SLOS phenotypically overlaps with Pallister-Hall syndrome which remains clinically a major differential diagnosis of SLOS. © 2011 Elsevier Masson SAS.


PubMed | France Bordeaux University Hospital, University of Lyon, Lille University Hospital Center, REIN Registry and 3 more.
Type: Journal Article | Journal: Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association | Year: 2014

Major inequalities in access to renal transplant waiting lists have been demonstrated among adult patients both in the USA and Europe. In this French nationwide study, we sought to ascertain the influence of patient and centre characteristics.We included all children (<18 years) in the French End-Stage Renal Disease National Registry, who started renal replacement therapy (RRT) between 1 January 2002 and 31 December 2011. The primary outcome was the probability of being listed within 6 months after starting RRT. Hierarchical logistic regression models were used to study the association between the patient or the centre characteristics and the outcome. Centre effects were assessed by studying the centre-level residual variance.A total of 614 incident patients treated in 54 centres were included; 421 (68.6%) were listed within 6 months after starting RRT. A higher risk of not being listed was found in patients younger than 2 years or with a renal disease with a high risk of recurrence after transplantation [odds ratio (OR): 2.61; 95% confidence interval (CI): 1.37-4.97]. We found a significant vintage effect: the probability of not being listed decreased over time (OR per 1 year +0.83, 95% CI: 0.74-0.94). Although we found no significant gender effect, a trend towards disfavouring girls persisted over the study period. We found a significant centre effect that remained after adjusting for patient characteristics. However, none of the centre characteristics that we studied (centre size, pre-emptive transplantation program, paediatric versus adult centres and the proportion of patients on the waiting list placed on inactive status during the first month after listing) explained this variability.Our study confirms inequalities among children in rapid access to the renal transplant waiting list and shows that patient and centre characteristics play a role in these inequalities. Further studies focusing on the organization and practices of the centres are needed to explain the remaining variability.


Journy N.,Institute for Radiological Protection and Nuclear Safety | Rehel J.-L.,Institute for Radiological Protection and Nuclear Safety | Ducou Le Pointe H.,Trousseau University Hospital | Lee C.,U.S. National Cancer Institute | And 5 more authors.
British Journal of Cancer | Year: 2015

Background: Recent epidemiological results suggested an increase of cancer risk after receiving computed tomography (CT) scans in childhood or adolescence. Their interpretation is questioned due to the lack of information about the reasons for examination. Our objective was to estimate the cancer risk related to childhood CT scans, and examine how cancer-predisposing factors (PFs) affect assessment of the radiation-related risk. Methods: The cohort included 67 274 children who had a first scan before the age of 10 years from 2000 to 2010 in 23 French departments. Cumulative X-rays doses were estimated from radiology protocols. Cancer incidence was retrieved through the national registry of childhood cancers; PF from discharge diagnoses. Results: During a mean follow-up of 4 years, 27 cases of tumours of the central nervous system, 25 of leukaemia and 21 of lymphoma were diagnosed; 32% of them among children with PF. Specific patterns of CT exposures were observed according to PFs. Adjustment for PF reduced the excess risk estimates related to cumulative doses from CT scans. No significant excess risk was observed in relation to CT exposures. Conclusions: This study suggests that the indication for examinations, whether suspected cancer or PF management, should be considered to avoid overestimation of the cancer risks associated with CT scans. © 2015 Cancer Research UK.


PubMed | Institute for Radiological Protection and Nuclear Safety, University Pierre and Marie Curie, Trousseau University Hospital, Pellegrin University Hospital and U.S. National Cancer Institute
Type: Journal Article | Journal: British journal of cancer | Year: 2015

Recent epidemiological results suggested an increase of cancer risk after receiving computed tomography (CT) scans in childhood or adolescence. Their interpretation is questioned due to the lack of information about the reasons for examination. Our objective was to estimate the cancer risk related to childhood CT scans, and examine how cancer-predisposing factors (PFs) affect assessment of the radiation-related risk.The cohort included 67,274 children who had a first scan before the age of 10 years from 2000 to 2010 in 23 French departments. Cumulative X-rays doses were estimated from radiology protocols. Cancer incidence was retrieved through the national registry of childhood cancers; PF from discharge diagnoses.During a mean follow-up of 4 years, 27 cases of tumours of the central nervous system, 25 of leukaemia and 21 of lymphoma were diagnosed; 32% of them among children with PF. Specific patterns of CT exposures were observed according to PFs. Adjustment for PF reduced the excess risk estimates related to cumulative doses from CT scans. No significant excess risk was observed in relation to CT exposures.This study suggests that the indication for examinations, whether suspected cancer or PF management, should be considered to avoid overestimation of the cancer risks associated with CT scans.


Pirot N.,Pavillon Jacques Delarue | Crahes M.,Pathology Laboratory | Adle-Biassette H.,University of Rouen | Soares A.,University of Rouen | And 9 more authors.
Journal of Neuropathology and Experimental Neurology | Year: 2016

To distinguish pyruvate dehydrogenase deficiency (PDH) from other antenatal neurometabolic disorders thereby improving prenatal diagnosis, we describe imaging findings, clinical phenotype, and brain lesions in fetuses from 3 families with molecular characterization of this condition. Neuropathological analysis was performed in 4 autopsy cases from 3 unrelated families with subsequent biochemical and molecular confirmation of PDH complex deficiency. In 2 families there were mutations in the PDHA1 gene; in the third family there was a mutation in the PDHB gene. All fetuses displayed characteristic craniofacial dysmorphism of varying severity, absence of visceral lesions, and associated encephaloclastic and developmental supra-and infratentorial lesions. Neurodevelopmental abnormalities included microcephaly, migration abnormalities (pachygyria, polymicrogyria, periventricular nodular heterotopias), and cerebellar and brainstem hypoplasia with hypoplastic dentate nuclei and pyramidal tracts. Associated clastic lesions included asymmetric leukomalacia, reactive gliosis, large pseudocysts of germinolysis, and basal ganglia calcifications. The diagnosis of PDH deficiency should be suspected antenatally with the presence of clastic and neurodevelopmental lesions and a relatively characteristic craniofacial dysmorphism. Postmortem examination is essential for excluding other closely related entities, thereby allowing for biochemical and molecular confirmation. © 2016 American Association of Neuropathologists, Inc. All rights reserved.

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