References of "SEGHAYE, Marie-Christine"
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See detailSalmonella infections in young children due to turtle contamination
MATHY, F.; CHIARADIA, F.; MASTOURI, M. et al

Conference (2013, March)

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See detailDelayed diagnosis of cardiomyopathy in a girl treated for asthma bronchiale
MASTOURI, M.; FUDVOYE, Julie ULg; ANTOLE, N. et al

Conference (2013, March)

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See detailCardiac malformations in neonates born from mothers with gestational diabetes
ANTOLE, N.; JACQUEMART, C.; GKIOUGKI, Evangelia ULg et al

Conference (2013, March)

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See detailNeonates show transient and incomplete anti-inflammatory response to cardiac surgery
Wiecker, Carola; Buding, Brigitte; Vazquez-Jimenez, Jaime et al

Conference (2013, January)

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See detailInterleukin-6 as a marker of inflammatory related post-operative myocardial dysfunction
von der Stück, Hannah; Buding, Brigitte; Vazquez-Jimenez, Jaime et al

Conference (2013, January)

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See detailReply to the Editor
SEGHAYE, Marie-Christine ULg

in Annals of Thoracic Surgery (2012), 94(5), 1787

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See detailQuel suivi pour les adultes presentant une cardiopathie congenitale?
Miltner, B.; Pierard, Luc ULg; SEGHAYE, Marie-Christine ULg

in Revue Médicale de Liège (2012), 67(7-8), 407-12

During the last decades, the care of children with congenital heart disease has markedly improved. In consequence, the number of those who reach adulthood is continuously growing. In 2010, the European ... [more ▼]

During the last decades, the care of children with congenital heart disease has markedly improved. In consequence, the number of those who reach adulthood is continuously growing. In 2010, the European Society of Cardiology published guidelines for the management of grown-up congenital heart disease (GUCH) and recommended that each GUCH patient should be examined at least once in a specialized center. Due to their specific needs, this population requires specific and organized care. Indeed, GUCH patients do not only have significant extra-cardiac problems, but also present specific cardiac complications such as right ventricular failure, incisionnal arrhythmias. They may require repeated surgical and/ or percutaneous interventions. Since this emerging population is ageing, they also may suffer from arterial hypertension, diabetes and coronary artery disease. Last but not least, young women with congenital heart disease need specialized advice and care for contraception, follow-up during pregnancy and peripartal management. In order to optimize the follow-up of this growing population, a shared care model where cardiologists, specialized GUCH centers and other specialized centers for the treatment of extra-cardiac problems work in tight collaboration is recommended. [less ▲]

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See detailDexamethasone pretreatment provides antiinflammatory and myocardial protection in neonatal arterial switch operation.
Heying, Ruth; Wehage, Edith; SCHUMACHER, Katharina ULg et al

in Annals of Thoracic Surgery (2012), 93(3), 869-76

BACKGROUND: This prospective double-blinded randomized study tested the hypothesis that preoperative treatment with dexamethasone would attenuate inflammatory priming of the myocardium, reduce the ... [more ▼]

BACKGROUND: This prospective double-blinded randomized study tested the hypothesis that preoperative treatment with dexamethasone would attenuate inflammatory priming of the myocardium, reduce the systemic inflammatory reaction upon cardiac operation, and provide organ protection in neonates. METHODS: Twenty neonates (age, 8 to 21 days) with transposition of the great arteries scheduled for arterial switch operation were included. Nine received dexamethasone (1 mg/kg body weight) 4 hours before cardiopulmonary bypass, and 11 received natrium chloride. We studied intramyocardial messenger RNA expression of interleukin (IL)-6, IL-8, IL-1beta, and tumor necrosis factor-alpha (TNF-alpha), as well as IL-10 and expression of TNF-alpha on protein level in right atrial tissue taken before institution of CPB. We measured plasma levels of IL-6, IL-10, lipopolysaccharide binding protein, and cardiac troponin T. Cytokine expression was related to postoperative outcome. RESULTS: Pretreatment with dexamethasone led to a significant decrease in myocardial expression of IL-6, IL-8, IL-1beta, and TNF-alpha messenger RNA and to a decrease in protein synthesis of TNF-alpha. Plasma concentrations of IL-6 were significantly lower and those of IL-10 significantly higher in pretreated patients. This was associated with lower cardiac troponin T values and lower dobutamine requirement. Levels of lipopolysaccharide binding protein were significantly higher postoperatively in pretreated neonates. CONCLUSIONS: Dexamethasone administration before arterial switch operation leads to a shift in the myocardial and systemic cytokine expression profile in neonates with transposition of the great arteries, with downregulation of proinflammatory and upregulation of antiinflammatory cytokines. Lower myocardial cell damage and lower catecholamine requirement suggest myocardial protection in treated patients. [less ▲]

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See detailAnesthésie pédiatrique et hôpital de jour: les grands problèmes des petits enfants
GROSJEAN, Valérie ULg; Veyckemans, F.; SEGHAYE, Marie-Christine ULg et al

in Revue Médicale de Liège (2011), 66(3), 135-139

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See detailHYPERINSULINISM-HYPERAMMONEMIA: AN UNUSUAL CAUSE OF HYPOKETOTIC HYPOGLYCEMIA
HARVENGT, Julie ULg; LEBRETHON, Marie-Christine ULg; leroy, patricia et al

Poster (2010, March)

BACKGROUND Etiological diagnosis of hypoglycaemia in infancy is a complex process, requiring careful integration of detailed history, clinical and laboratory data. The causes of recurrent infant ... [more ▼]

BACKGROUND Etiological diagnosis of hypoglycaemia in infancy is a complex process, requiring careful integration of detailed history, clinical and laboratory data. The causes of recurrent infant hypoglycaemia include excessive insulin secretion, surreptitious insulin administration, deficiency of counter-regulatory hormones and inborn errors of metabolism. CLINICAL CASE A 10 month old girl was admitted at our emergency unit for generalized seizures without fever. Routine laboratory investigations were normal but blood glucose level was at 31 mg/dl. No ketone bodies were found in the urine. Past medical history revealed failure to thrive. A first seizure episode at 8 months of age during family’s holiday is reported. Tests performed in a foreign hospital revealed glycaemia at 36mg/dl. During her stay in our paediatric unit, several hypoglycaemias (31-45 mg/dl) were documented related to irritability as initial symptom of neuroglucopaenia. Detailed medical history revealed that fast tolerance was shorten with hypoglycaemia documented between one to three hours after eating. Clinical examination showed absence of hepatomegaly and failure to thrive: weight, -3SD; height, -2SD, and cranial circumference -2SD. At the time of hypoglycaemia, urinary tests revealed absence of ketonuria, that basically evokes hyperinsulinism or fatty acid oxidation deficiencies but these deficiencies were rapidly excluded by the very short fast state. Blood acylcarnitine profile was normal. Hyperinsulinism is defined by a ratio glycaemia/insulin below 4 with insulin values not necessary high. Since hyperinsulinism can not be excluded with only one blood measure, series of taking were performed during 24 hours. One of these tests was clearly positive with ratio equal to 2.3 (glycaemia at 41 mg/dl, insulin at 18µU/ml). For this patient, ammonemia was also tested with values ranged from 242 to 275 µg/dl (normal < 125) and the diagnosis of hyperinsulinism/hyperammoniemia (hi/ha) was made and confirmed by molecular analysis (mutation c.965G>A (p.R269H) in the GLUD1 gene). The treatment consists in this case by diazoxide and reduction of leucine intakes (< 200 mg of leucine/meal). DISCUSSION Differential diagnosis of hypoglycaemia with absence of ketonuria and absence of hepatomegaly include fatty acids β-oxidation defects, ketogenesis defects and hyperinsulinisms. Short fasting and post-prandial induced hypoglycaemia pointed to hyperinsulinism in our patient. Congenital hyperinsulinism includes KATP, glucokinase or glutamate deshydrogenase mutations. Hi/ha syndrome is due to activating mutations in the GLUD1 gene, coding for the glutamate dehydrogenase (GDH). Such mutations reduce the sensitivity of the enzyme to allosteric inhibition by GTP and consequently increase its sensitivity to allosteric activation by L-leucine. Hyperactivity of the GDH is responsible for over-oxidation of glutamate in β-pancreatic cells, increase of the ATP/ADP ratio and insulin release. Hyperactivity of GDH in liver is also responsible for hyperammonemia, which is usually mild and considered harmless for the brain. Nevertheless, recent studies have shown an increased epilepsy risk in cohorts of patients with hi/ha. CONCLUSION This case points out the importance of necessity for first investigations of infant documented case of hypoglycaemia. Patient history must focus on symptoms such as shorten fast tolerance periods and neurological symptoms of glucose deprivation. Blood samples should be taken at the time of hypoglycaemia and urine samples as soon as possible after the episode of hypoglycaemia. Initial normal insulin values do not allow the exclusion of the diagnosis of hyperinsulinism. [less ▲]

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See detailPrevalence of and risk factors for perioperative arrhythmias in neonates and children after cardiopulmonary bypass: continuous holter monitoring before and for three days after surgery.
Grosse-Wortmann, Lars; Kreitz, Suzanna; Grabitz, Ralph G et al

in Journal of cardiothoracic surgery (2010), 5

BACKGROUND: A comprehensive evaluation of postoperative arrhythmias following surgery for congenital heart disease by continuous Holter monitoring has not been carried out. We aimed, firstly, to establish ... [more ▼]

BACKGROUND: A comprehensive evaluation of postoperative arrhythmias following surgery for congenital heart disease by continuous Holter monitoring has not been carried out. We aimed, firstly, to establish the time course of pre- and early postoperative arrhythmias by beat-to-beat analysis following cardiopulmonary bypass and, secondly, to examine which surgical procedures present risk factors for specific arrhythmias. METHODS: 494 consecutive patients, including 96 neonates, were studied with serial 24-hour Holter electrocardiograms before as well as uninterruptedly during the first 72 hours after surgery and prior to discharge. RESULTS: Within 24 hours of surgery 59% of the neonates and 79% of the older children developed arrhythmias. Junctional ectopic tachycardia occurred in 9% of neonates and 5% of non-neonates and ventricular tachycardia in 3% and 15%, respectively.For neonates, male sex and longer cross-clamping time independently increased the risk for arrhythmias (odds ratios 2.83 and 1.96/minute, respectively). Ventricular septal defect repair was a strong risk factor for junctional ectopic tachycardia in neonates and in older children (odds ratios 18.8 and 3.69, respectively). For infants and children, older age (odds ratio 1.01/month) and closure of atrial septal defects (odds ratio 2.68) predisposed to arrhythmias of any type. CONCLUSIONS: We present the largest cohort of neonates, infants and children that has been prospectively studied for the occurrence of arrhythmias after cardiac surgery. Postoperative arrhythmias are a frequent and transient phenomenon after cardiopulmonary bypass, provoked both by mechanical irritation of the conduction system and by humoral factors. [less ▲]

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See detailLong-term outcome of speech and language in children after corrective surgery for cyanotic or acyanotic cardiac defects in infancy.
Hovels-Gurich, Hedwig H; Bauer, Sebastian B; Schnitker, Ralph et al

in European Journal of Paediatric Neurology : Official Journal of the European Paediatric Neurology Society (2008), 12(5), 378-386

The purpose of this prospective study was to assess whether outcome of speech and language in children 5-10 years after corrective surgery for tetralogy of Fallot (TOF) or ventricular septal defect (VSD ... [more ▼]

The purpose of this prospective study was to assess whether outcome of speech and language in children 5-10 years after corrective surgery for tetralogy of Fallot (TOF) or ventricular septal defect (VSD) in infancy was influenced by the preoperative condition of hypoxemia or cardiac insufficiency and whether it was associated with perioperative risk factors and neurodevelopmental outcome. A total of 35 unselected children, 19 with TOF and hypoxemia and 16 with VSD and cardiac insufficiency, operated with combined deep hypothermic circulatory arrest and low-flow cardiopulmonary bypass at mean age 0.7+/-0.3 (mean+/-standard deviation) years, underwent, at mean age 7.4+/-1.6 years, standardized evaluation of speech and language functions. Results were compared between subgroups and related to perioperative factors, sociodemographic and neurodevelopmental status. Age at testing, socioeconomic status and history of speech and language development were not different between the subgroups. In contrast, total scores on oral and speech motor control functions (TFS) as well as on oral and speech apraxia (Mayo Test) were significantly reduced (p<0.02 to <0.05), and scores on anatomical oral structures tended to be lower (p<0.09) in the TOF group as compared to the VSD group. No differences were found for auditory word recognition and phonological awareness as assessed by the Auditory Closure subtest of the Illinois Test of Psycholinguistic Abilities and the test of auditory analysis skills, respectively. In all children, higher age at testing and better socioeconomic status were associated with better results in all domains of assessment (p<0.001 to <0.04). Consistent impairments of all oral and speech motor control functions (TFS and Mayo Test) were present in 29% of all children with a mean age of 6.5 years in contrast to 43% with normal performance and a mean age of 8.3 years. On the receptive speech tasks, only 6% scored below the normal range of their age group. TFS subscores were significantly correlated with age, bypass duration and motor function, but not correlated with socioeconomic status, duration of cardiac arrest, intelligence and academic achievement. Children with preoperative hypoxemia due to cyanotic cardiac defects in infancy are at higher risk for dysfunction in speech and language than those with cardiac insufficiency due to acyanotic heart defects. Age at testing, socioeconomic status, and duration of cardiopulmonary bypass influenced test results. Long-term outcome in speech and language functions can be considered as a sensitive indicator of overall child development after cardiac surgery. [less ▲]

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See detailCongenital cystic adenomatoid malformation type 0-a rare cause of neonatal death.
Stuhrmann, Stefan; Sachweh, Jorg; Bindl, Lutz et al

in Pediatric Critical Care Medicine (2007), 8(6), 580-581

OBJECTIVE: We give the first account of failure of extracorporeal membrane oxygenation therapy secondary to congenital cystic malformation of the lung (CCAM) type 0. DESIGN: Case report. SETTING ... [more ▼]

OBJECTIVE: We give the first account of failure of extracorporeal membrane oxygenation therapy secondary to congenital cystic malformation of the lung (CCAM) type 0. DESIGN: Case report. SETTING: Pediatric intensive care unit. PATIENT: A female neonate, appropriate for gestational age, with respiratory failure immediately after delivery. INTERVENTIONS: : Cardiopulmonary support with venoarterial extracorporeal membrane oxygenation. RESULTS: There was no improvement of pulmonary function, and the patient died. CCAM type 0 was diagnosed postmortem. CONCLUSIONS: CCAM type 0 should be considered as a rare differential diagnosis of irreversible lung pathologies leading to failure of extracorporeal membrane oxygenation therapy for neonatal respiratory failure. [less ▲]

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