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Le Touquet – Paris-Plage, France

Colomb V.,Hopital Necker Enfants Malades
Journal of Nutrition | Year: 2013

Hospitalized children are vulnerable to malnutrition during serious illness or recovery from injury and are at subsequent risk of increased morbidity and growth retardation. In cases in which enteral nutrition is not possible, parenteral nutrition (PN)can be used to ensure that patients at nutritional risk receive appropriate amounts of macro- and micronutrients. Nutritional needs cannot be met by 1 standard PN formulation in pediatric patients (term to 18 y) because of the wide range of needs according to age, weight, degree of maturity, and disease state. Preparation of individualized PN is associated with several limitations, including prescribing errors, stability issues, and risk of infection. These risks may be avoided by the availability of a range of pediatric PN formulations provided as commercial premixed 3-chamber bags (3-CBs). These 3-CBs were developed in conjunction with experienced neonatologists and pediatricians in accordance with international guidelines. A prospective study has previously shown the practical handling and ease of use of 2 formulations of these 3-CBs,1 designed for term infants and toddlers up to 2 y of age and 1 for children and adolescents aged 2-18 y. The majority of pharmacists and nurses described the 3-CB as easy to use and favored it over individual bottles, bags compounded on the ward, ready-to-use compounded bags, and premixes prepared by the pharmacy and tailored to patient needs. These formulations offer a means of improving the quality of care in hospital pediatric units, particularly in the absence of a nutrition support team. © 2013 American Society for Nutrition. Source

Bahi-buisson N.,University of Paris Descartes | Dulac O.,Hopital Necker Enfants Malades
Handbook of Clinical Neurology | Year: 2013

Epilepsies associated with inborn errors of metabolism (IEM) represent a major challenge. Seizures rarely dominate the clinical presentation, which is more frequently associated with other neurological symptoms, such as hypotonia and/or cognitive disturbances. Although epilepsy in IEM can be classified in various ways according to pathogenesis, age of onset, or electroclinical presentation, the most pragmatic approach is determined by whether they are accessible to specific treatment or not.The main potentially treatable causes comprise vitamin B6 (pyridoxine deficiency), biotine, and GLUT1 deficiency (GLUT1DS) syndromes. Folinic acid-dependent seizures are allelic with pyridoxine dependency.Incompletely treatable IEMs include pyridoxal phosphate, serine, and creatine deficiencies.The main IEMs that present with epilepsy but offer no specific treatment are nonketotic hyperglycinemia, mitochondrial disorders, sulfite oxidase deficiency, ceroid-lipofuscinosis, Menkes disease, and peroxisomal disorders. © 2013 Elsevier B.V. Source

Sarnacki S.,Hopital Necker Enfants Malades | Brisse H.,University Pierre and Marie Curie
Hormone Research in Paediatrics | Year: 2011

Surgery of ovarian tumors in children requires a good knowledge of these lesions. Complete resection is mandatory for malignant lesions, and in the case of benign tumors preservation of healthy ovarian tissue is crucial. Diagnosis is based on clinical features (age and hormonal status), imaging and tumor marker levels. Laparoscopy is of great help in making a diagnosis and staging when the lesion is malignant. Laparotomy by a supra-pubic approach is, however, the only way to ensure a safe treatment of the lesion by avoiding any risk of tumor spillage, which constitutes a chance loss. Surgical treatment consists of complete ovariectomy for a malignant tumor and partial ovariectomy when the lesion is surely benign. Preservation of fertility is based on conservative surgery for uni- or bilateral benign lesions, and may be discussed in some selected cases of bilateral malignant tumors. When the remaining ovarian tissue predicts precocious ovarian failure, ovarian tissue or oocyte cryopreservation may be proposed to patients and their families. Copyright © 2010 S. Karger AG. Source

Blanc J.,M3C Necker | Stos B.,M3C Necker | De Montalembert M.,Hopital Necker Enfants Malades | Bonnet D.,M3C Necker | Boudjemline Y.,M3C Necker
Journal of the American Society of Echocardiography | Year: 2012

Background: Several adult studies have shown that sickle cell disease is associated with cardiac abnormalities and premature death. The aim of this study was to use speckle-tracking strain, a relatively load independent parameter, to evaluate systolic left ventricular (LV) and right ventricular (RV) function in a pediatric sickle cell disease population. Methods: Twenty-eight patients with sickle cell disease (mean age, 10.0 ± 3.6 years; mean body surface area, 1.14 ± 0.27 m 2) and 29 controls matched for age and body surface area were compared. Cardiac output, LV dimension, wall thickness and circumferential strain, LV and RV longitudinal systolic strain, conventional and tissue Doppler parameters, and pulmonary pressure were assessed. Results: LV cardiac output was significantly higher in patients, as were indexed LV systolic diameter, indexed LV mass, and E/E′ septal ratio. Indexed LV diastolic diameter, wall thickness, LV shortening fraction, and global LV longitudinal and circumferential strains were similar in patients and controls. However, their global RV longitudinal strain was significantly lower, although tricuspid annular plane systolic excursion and color-coded tricuspid S-wave velocity were similar. Among patients, 21% had tricuspid regurgitation velocities > 2.5 m/sec, but none had tricuspid regurgitation velocities > 3 m/sec. Indexed LV diastolic dimension and systolic pulmonary artery pressure were significantly higher in patients whose hemoglobin was <80 g/L, but parameters of systolic and diastolic LV function were similar. Conclusions: In children with sickle cell disease, LV diastolic function is significantly altered, although LV systolic function, evaluated by global longitudinal strain, is normal. In addition, cardiac output is increased, and elevated tricuspid regurgitation velocity is common, whereas it is never found in controls. Most importantly, global RV longitudinal systolic strain is significantly altered. Copyright 2012 by the American Society of Echocardiography. Source

Le Douarin N.M.,College de France | Couly G.,Hopital Necker Enfants Malades | Creuzet S.E.,French National Center for Scientific Research
Developmental Biology | Year: 2012

The role of the neural crest (NC) in the construction of the vertebrate head was demonstrated when cell tracing techniques became available to follow the cells exiting from the cephalic neural folds in embryos of various vertebrate species. Experiments carried out in the avian embryo, using the quail/chick chimera system, were critical in showing that the entire facial skeleton and most of the skull (except for he occipital region) were derived from the NC domain of the posterior diencephalon, mesencephalon and rhombomeres 1 and 2 (r1, r2). This region of the NC was designated FSNC (for Facial Skeletogenic NC). One characteristic of this part of the head including the neural anlage is that it remains free of expression of the homeotic genes of the Hox-clusters. In an attempt to see whether this rostral Hox-negative domain of the NC has a specific role in the development of the skeleton, we have surgically removed it in chick embryos at 5-6 somite stages (5-6 ss). The operated embryos showed a complete absence of facial and skull cartilages and bones showing that the Hox expressing domain of the NC caudally located to the excision did not regenerate to replace the anterior NC. In addition to the deficit in skeletal structures, the operated embryos exhibited severe brain defects resulting in anencephaly. Experiments described here have shown that the neural crest cells regulate the amount of Fgf8 produced by the two brain organizers, the Anterior Neural Ridge (ANR) and the isthmus. This regulation is exerted via the secretion of anti-BMP signaling molecules (e.g. Gremlin and Noggin), which decrease BMP production hence enhancing the amount of Fgf8 synthesized in the ANR (also called "Prosencephalic organizer") and the isthmus. In addition to its role in building up the face and skull, the NC is therefore an important signaling center for brain development. © 2012 Elsevier Inc. Source

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