Hopital University des Enfants

Brussels, Belgium

Hopital University des Enfants

Brussels, Belgium
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PubMed | Northern General Hospital, Alderhey Childrens Hospital, Semmelweis University, Klinikum am Steinenberg and 56 more.
Type: Journal Article | Journal: Molecular genetics and metabolism | Year: 2015

There appears little consensus concerning protein requirements in phenylketonuria (PKU).A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics.The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n=24 centres) (infants <1 year, >2-3g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n=10 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n=4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n=25 centres) giving the least (infants <1 year, >2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day).The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population.


PubMed | Radboud UMC, UniversitatsSpital Zurich, Alderhey Childrens Hospital, Childrens Hospital and 38 more.
Type: Comparative Study | Journal: Molecular genetics and metabolism | Year: 2015

Dietary management of 418 adult patients with galactosaemia (from 39 centres/12 countries) was compared. All centres advised lactose restriction, 6 restricted galactose from galactosides fruits and vegetables and 12 offal. 38% (n=15) relaxed diet by: 1) allowing traces of lactose in manufactured foods (n=13) or 2) giving fruits, vegetables and galactosides (n=2). Only 15% (n=6) calculated dietary galactose. 32% of patients were lost to dietetic follow-up. In adult galactosaemia, there is limited diet relaxation.


Reguerre Y.,University of Angers | Martelli H.,University Paris - Sud | Rey A.,CNRS Gustave Roussy Institute | Rogers T.,Institute of Child Life and Health | And 6 more authors.
European Journal of Cancer | Year: 2012

Background: Localised pelvic rhabdomyosarcomas (pRMS) are rare tumours with a poorer prognosis than the majority of RMS. This study analysed patient outcome according to the type of local therapy delivered and the effect of disease-related factors on prognosis. Patients and methods: 97 children with localised pRMS were enrolled in the SIOP-MMT84, 89 and 95 studies. After primary surgery or biopsy, all children received ifosfamide/actinomycin/vincristine- based chemotherapy. Radiotherapy and surgery were planned in patients failing to achieve complete remission. Results: Median age at diagnosis was 52 months [5 months-18 years]. IRS staging was I for five patients, II for 15 and III for 77. Patients had embryonal RMS (N = 41), alveolar RMS (N = 29), botryoid RMS (N = 3), or not otherwise specified RMS (N = 24). Outcome: 87 patients achieved local control (90%), 37 relapsed (43%), mainly locally (84%). With a median follow-up of more than 10 years [4-22 years], 5-year OS was 66% (95% CI: 56-75%) and EFS was 52% (95% CI: 42-61%). Among the 18 IRS-I/II patients treated without radiotherapy, 15 survived. Seven out of the 20 IRS-III patients treated without local therapy died. In multivariate analysis, IRS staging, age greater than 10 years and lymph node involvement had a negative impact on OS. Perineal/perianal locations had a trend towards a worse prognosis. Conclusion: pRMS still have a relatively poor prognosis. Radiotherapy or brachytherapy is necessary for all IRS-III patients including those with radiological complete remission after neoadjuvant chemotherapy with or without surgery. Radiotherapy may be withheld in IRS-I patients and children under 3 years with IRS-II pRMS. © 2012 Elsevier B.V. All rights reserved.


PubMed | University Hospital of Tuebingen, Hannover Medical School, Copenhagen University, Hopital University des Enfants and 7 more.
Type: Journal Article | Journal: Pediatric diabetes | Year: 2015

Modern insulin regimens for the treatment of type 1 diabetes are highly individualized. The concept of an individually tailored medicine accounts for a broad variety of different insulin regimens applied. Despite clear recommendations for insulin management in children and adolescents with type 1 diabetes there is little distinctiveness about concepts and the nomenclature is confusing. Even among experts similar terms are used for different strategies. The aim of our review--based on the experiences of the Hvidoere Study Group (HSG)--is to propose comprehensive definitions for current insulin regimens reflecting current diabetes management in childhood and adolescence. The HSG--founded in 1994--is an international group representing 24 highly experienced pediatric diabetes centers, from Europe, Japan, North America and Australia. Different benchmarking studies of the HSG revealed a broad variety of insulin regimens applied in each center, respectively. Furthermore, the understanding of insulin regimens has been persistently different between the centers since more than 20yr. Not even the terms conventional and intensified therapy were used consistently among all members. Besides the concepts conventional and intensified, several other terms for the characterization of insulin regimens are in use: Basal Bolus Concept (BBC), multiple daily injections (MDI), and flexible insulin therapy (FIT) are most frequently used, although none of these expressions is clearly or consistently defined. The proposed new classification for insulin management will be comprehensive, simple, and catchy. Currently available terms were included. This classification may offer the opportunity to compare therapeutic strategies without the currently existing confusion on the insulin regimen.


Barber C.,Starship Hospital | Ille S.,GfK SE | Vergison A.,Hopital University Des Enfants | Coates H.,University of Western Australia
International Journal of Pediatric Otorhinolaryngology | Year: 2014

Objectives: The Ear infections Attitudes Research study investigated parental attitudes and awareness towards acute otitis media (AOM) and evaluated the burden of AOM for affected children, their families, and parental work capabilities. Methods: This study, conducted via online interviews in October-November 2010, included parents (N=2867) from 12 countries, whose children aged ≤3.5 years had experienced ≥1 professionally diagnosed AOM episode in the last 6 months (AOM-experienced group; N=1438) or had never experienced any professionally diagnosed AOM episode (non AOM-experienced group; N=1429). The interviews consisted of questions with multiple-choice, five-point scaled or free-text answers. Answers to multiple-choice questions were presented as frequencies of particular responses and those to scaled questions as mean values or percentages of parents considering each aspect as applicable. Results: Parents considered that the main AOM burdens for affected children were pain (mean values on five-point scales: 4.4 and 4.5), disturbed sleep (4.3 and 4.3) and irritability (4.2 and 4.0) and for their families, sleepless nights (4.2 and 3.8) and worries about the child's recovery (4.1 and 4.3) and about potential long-term implications (4.0 and 4.3) in the AOM-experienced and non AOM-experienced groups, respectively. During their child's most recent AOM episode, 95% of parents in the AOM-experienced group used antibiotics, 76% reported that their doctors prescribed antibiotics for immediate use, 13% were advised to return for antibiotic prescription if symptoms did not abate and 9% received a prescription for antibiotics to use if symptoms did not improve. Both reported prescription and usage rates for antibiotics were higher than expected. When their child had AOM, 73% of parents had to be absent from work or rearrange their working hours. Among those who took leave from work, 67% stayed at home for 2-7 days. Conclusions: Parents perceive AOM to be a burden for their child and families, particularly the pain and disturbed sleep due to AOM, and this disease had a significant effect on parents' ability to attend work. Given how common AOM is, this loss of workdays may lead to substantial financial burden for families and the society. Antibiotics were almost invariably used in all countries despite current guidelines. © 2013 Elsevier Ireland Ltd.


Cheron G.,University of Paris Descartes | Jais J.P.,Service de Sante Publique | Cojocaru B.,University of Paris Descartes | Parez N.,Hopital Louis Mourier | Biarent D.,Hopital University des Enfants
European Journal of Pediatrics | Year: 2011

We tested the hypothesis that application of the principles learned from the European Paediatric Life Support (EPLS) course improves child health assessment and care. In a retrospective study, residents from five paediatric emergency departments were included. For each of them, we analysed five medical records of infants and children suffering from diarrhoea; three were in ambulatory care and two were inhospital care with IV hydration. Two independent observers analysed the records using a standardized checklist of 14 clinical points, as well as three items to evaluate the adequacy of treatment according to hydration status. Agreement between readers was evaluated the kappa coefficient of concordance. Statistical associations between each item and the EPLS course status was assessed by logistic regression taking into account the clustered data structure. Fifty residents and 240 medical records were included. Twenty-six residentswere EPLS trained (intervention group) and 24 residents were not (control group). The results of the analyses of the medical records by the observers were concordant (kappa >0.91). Medical records in the intervention group contained moreclinical information on circulatory status (P<0.0001). Residents in the intervention group prescribed goal-directedtherapy more often (P=0.006). For children with shock,they administered volume resuscitation (P=0.01) with goaldirectedtherapy more often (P=0.003). This is the firstevaluation of an educational program focusing on the actionsof learners in the clinical environment. Our findings highlight that the EPLS course is associated with a betterclinical analysis of hydration and circulation status as well as with goal-directed therapy. © Springer-Verlag 2011.


Peerboccus M.,Hopital University des Enfants | Damry N.,Hopital University des Enfants | Pather S.,Hopital University des Enfants | Devriendt A.,Hopital University des Enfants | Avni F.,Hopital University des Enfants
Pediatric Radiology | Year: 2013

Background: Kidney ultrasonography is frequently performed in children; to date there are no specific guidelines regarding hydration before the examination. Objective: Because certain renal measurements can be indicative of pathology, we used sequential US images to investigate the effect of standardised hydration in children relative to renal size, echogenicity and pelvic expansion. Materials and methods: Thirty-one children (7 years to 14 years old) underwent US examinations before hydration (T0) and at 30 min (T30) and 60 min (T60) after ingesting water. We measured bladder volume, inter-polar kidney size, renal volume and anterior-posterior diameter of the pelvis. Cortical echogenicity was compared to that of the liver and spleen. Results: On the right side the increase in average inter-polar renal size was 2.5% at T30 and 2.6% at T60 (P < 0.05) compared to T0. On the left the increase was 3.8% at T30 and 4.3% at T60 (P < 0.05). Volume expansion for the right kidney was 16.8% at T30 and 14.0% at T60 (P < 0.05). On the left it was 12.9% at T30 and 10.2% at T60 (P < 0.05). Cortical echogenicity progressed from hypoechogenicity to isoechogenicity (P < 0.05). The percentage of expanded pelves increased with hydration (T0: 3.2%, T30: 9.7%, T60: 22.6%). Conclusion: Oral hydration influences renal length, volume and echogenicity in children. Hydration results in expansion of the pelvis. We recommend standardisation of hydration before US examination. © 2013 Springer-Verlag Berlin Heidelberg.


Biban P.,Neonatal And Paediatric Intensive Care Unit Major City Hospital | Filipovic-Grcic B.,Clinical Hospital Center | Biarent D.,Hopital University des Enfants | Manzoni P.,Neonatology and Hospital NICU
Early Human Development | Year: 2011

Most newborns are born vigorous and do not require neonatal resuscitation. However, about 10% of newborns require some type of resuscitative assistance at birth. Although the vast majority will require just assisted lung aeration, about 1% requires major interventions such as intubation, chest compressions, or medications.Recently, new evidence has prompted modifications in the international cardiopulmonary resuscitation (CPR) guidelines for both neonatal, paediatric and adult patients. Perinatal and neonatal health care providers must be aware of these changes in order to provide the most appropriate and evidence-based emergency interventions for newborns in the delivery room. The aim of this article is to provide an overview of the main recommended changes in neonatal resuscitation at birth, according to the publication of the international Liaison Committee on Resuscitation (ILCOR) in the CoSTR document (based on evidence of sciences) and the new 2010 guidelines released by the European Resuscitation Council (ERC), the American Heart Association (AHA), and the American Academy of Pediatrics (AAP). © 2010 Elsevier Ltd.


PubMed | GfK SE, Hopital University Des Enfants, University of Western Australia and Starship Hospital
Type: Journal Article | Journal: International journal of pediatric otorhinolaryngology | Year: 2014

The Ear infections Attitudes Research study investigated parental attitudes and awareness towards acute otitis media (AOM) and evaluated the burden of AOM for affected children, their families, and parental work capabilities.This study, conducted via online interviews in October-November 2010, included parents (N=2867) from 12 countries, whose children aged 3.5 years had experienced 1 professionally diagnosed AOM episode in the last 6 months (AOM-experienced group; N=1438) or had never experienced any professionally diagnosed AOM episode (non AOM-experienced group; N=1429). The interviews consisted of questions with multiple-choice, five-point scaled or free-text answers. Answers to multiple-choice questions were presented as frequencies of particular responses and those to scaled questions as mean values or percentages of parents considering each aspect as applicable.Parents considered that the main AOM burdens for affected children were pain (mean values on five-point scales: 4.4 and 4.5), disturbed sleep (4.3 and 4.3) and irritability (4.2 and 4.0) and for their families, sleepless nights (4.2 and 3.8) and worries about the childs recovery (4.1 and 4.3) and about potential long-term implications (4.0 and 4.3) in the AOM-experienced and non AOM-experienced groups, respectively. During their childs most recent AOM episode, 95% of parents in the AOM-experienced group used antibiotics, 76% reported that their doctors prescribed antibiotics for immediate use, 13% were advised to return for antibiotic prescription if symptoms did not abate and 9% received a prescription for antibiotics to use if symptoms did not improve. Both reported prescription and usage rates for antibiotics were higher than expected. When their child had AOM, 73% of parents had to be absent from work or rearrange their working hours. Among those who took leave from work, 67% stayed at home for 2-7 days.Parents perceive AOM to be a burden for their child and families, particularly the pain and disturbed sleep due to AOM, and this disease had a significant effect on parents ability to attend work. Given how common AOM is, this loss of workdays may lead to substantial financial burden for families and the society. Antibiotics were almost invariably used in all countries despite current guidelines.


PubMed | Hopital University des Enfants
Type: Journal Article | Journal: Pediatric radiology | Year: 2013

Kidney ultrasonography is frequently performed in children; to date there are no specific guidelines regarding hydration before the examination.Because certain renal measurements can be indicative of pathology, we used sequential US images to investigate the effect of standardised hydration in children relative to renal size, echogenicity and pelvic expansion.Thirty-one children (7years to 14years old) underwent US examinations before hydration (T0) and at 30min (T30) and 60min (T60) after ingesting water. We measured bladder volume, inter-polar kidney size, renal volume and anterior-posterior diameter of the pelvis. Cortical echogenicity was compared to that of the liver and spleen.On the right side the increase in average inter-polar renal size was 2.5% at T30 and 2.6% at T60 (P<0.05) compared to T0. On the left the increase was 3.8% at T30 and 4.3% at T60 (P<0.05). Volume expansion for the right kidney was 16.8% at T30 and 14.0% at T60 (P<0.05). On the left it was 12.9% at T30 and 10.2% at T60 (P<0.05). Cortical echogenicity progressed from hypoechogenicity to isoechogenicity (P<0.05). The percentage of expanded pelves increased with hydration (T0: 3.2%, T30: 9.7%, T60: 22.6%).Oral hydration influences renal length, volume and echogenicity in children. Hydration results in expansion of the pelvis. We recommend standardisation of hydration before US examination.

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