Publications by authors named "Tibboel D"

Objectives: Gastrointestinal hormones control gut functions in response to enteral nutrition. Diseases involving the gastrointestinal tract, such as necrotizing enterocolitis, may affect gut hormone secretion and therefore influence gut functions. Because bowel rest is an important part of the treatment, infants with this disease are especially at risk for an altered gut hormone secretion and thus for compromised gut functions.

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Although our knowledge of pain and its management in the perinatal period has increased, little is known about the first hours and days of life when major physiologic transition events occur. Prematurity and critical illnesses further complicate analgesic use during this time. Increased morbidity and mortality have been shown in infants receiving placebo infusions after surgery compared with infants with analgesia, highlighting the negative consequences of pain in infants.

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Based on the authors' review of the literature on pediatric postoperative pain assessment with special attention to groups of vulnerable infants, this article (1) reports on type of surgery and its relationship to postoperative pain intensity; (2) reviews the characteristics of existing postoperative pain instruments for neonates, infants, and toddlers; (3) discusses timing, duration, and who should assess postoperative pain; (4) reviews the specific literature on pain assessment in critically ill infants, including the extremely low birth weight and the cognitively and/or neurologically impaired infant, and (5) discusses the role of parents in postoperative pain assessment. Postoperative pain instruments are useful for specific groups of vulnerable infants, but it is important that in addition to the valuable scoring of pain, common sense is used and factors such as developmental stage, temperament and personality, number of previous painful experiences, anxiety, and environmental factors are taken into account.

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Design: The authors reviewed the available pediatric pain literature and selected those studies that reported quantitative information on the reliability and validity of, and the optimal cutoff points for, the visual analog scale (VAS) when used as an observational pediatric pain tool.

Results: Available psychometric findings concerning the observational VAS (VAS obs ) are limited. The estimated interrater reliability of the VAS (obs) from 9 studies ranged from 0.

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Background/purpose: Thyroid transcription factor-1 (TTF-1) was detected in human respiratory epithelial cells from 11 weeks of gestation. TTF-1 is involved in both lung morphogenesis and in the regulation of surfactant proteins. Recently, low expression of TTF-1 in the nitrofen rat model of congenital diaphragmatic hernia (CDH) was shown and restoration of this downregulation by antenatal glucocorticolds (CS) was reported.

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Background/purpose: The cost-effectiveness of medical interventions is becoming an important issue for decision makers. Until recently, evidence of the cost-effectiveness of neonatal surgery was largely lacking. The authors analyzed the cost-effectiveness of neonatal surgery and subsequent treatment for congenital diaphragmatic hernia (CDH).

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A randomized double-blind clinical trial compared the efficacy of 10 microg/kg/h morphine continuous intravenous infusion (CM) with that of 30 microg/kg morphine (IM) every 3h after major abdominal or thoracic surgery, in 181 infants aged 0-3 years. Efficacy was assessed by the caregiving nurses with the COMFORT 'behavior' and a visual analogue scale (VAS) for pain, every 3h in the first 24h after surgery. Random regression modeling was used to simultaneously estimate the effect of randomized group assignment, actual morphine dose (protocol dosage plus extra morphine when required), age category, surgical stress, and the time-varying covariate mechanical ventilation on COMFORT 'behavior' and the observational VAS rated pain, respectively.

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Hypothesis: Sepsis is an epiphenomenon of parenteral nutrition-associated cholestasis (PNAC) and not a causative factor, and the incidence of sepsis is not affected by the presence or absence of PNAC.

Design: Observational cohort study.

Setting: Pediatric surgery department in a tertiary referral children's hospital.

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A 21-month-old Caucasian female with the combination of craniofrontonasal syndrome and a posterolateral defect of the diaphragm (type Bochdalek) is described. This is thought to be a previously undescribed combination. Pedigree analysis is consistent with an X-linked mode of inheritance of the craniofrontonasal syndrome.

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Polyhalogenated aromatic hydrocarbons (PHAHs), such as polychlorinated dibenzo-p-dioxins and dibenzofurans, polybrominated diphenylethers, and bisphenol A derivatives are persistent environmental pollutants, which are capable of interfering with reproductive and endocrine function in birds, fish, reptiles, and mammals. PHAHs exert estrogenic effects that may be mediated in part by their hydroxylated metabolites (PHAH-OHs), the mechanisms of which remain to be identified. PHAH-OHs show low affinity for the ER.

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In conditions associated with high serum iodothyronine sulfate concentrations, e.g. during fetal development, desulfation of these conjugates may be important in the regulation of thyroid hormone homeostasis.

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Objectives: To determine the value of pulmonary artery Doppler velocimetry relative to fetal biometric indices and clinical correlates in the prenatal prediction of lethal lung hypoplasia (LH) in prolonged (>1 week) oligohydramnios.

Methods: Forty-two singleton pregnancies with oligohydramnios associated with premature rupture of membranes ([PROM]; n = 31) or bilateral renal pathology (n = 11) were examined using color-coded Doppler ultrasound in a cross-sectional study design. Mean gestational age was 28.

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Background: The treatment of pediatric patients with drugs in hospitals is being impeded by a shortage in the availability of licensed drugs in an appropriate formulation. We have studied the extent of use of drugs that are not licensed for use in children (unlicensed) and drugs that are used outside the terms of the product license (off-label). We conducted this study in a Dutch academic children's hospital.

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Advancements in medical technology over the last decades have greatly benefited perioperative care of newborns undergoing major surgical interventions. Yet, a proportion of these babies will not survive, and doctors are forced to face the difficult ethical question of whether, in cases of severe congenital malformations or acquired diseases, the expected quality of life justifies the decision to continue, withhold, or withdraw treatment. In a tripartite approach, the authors present their relevant experiences with these newborns in the pediatric surgical department of the Sophia Children's Hospital (SCH).

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Congenital diaphragmatic hernia (CDH) is a major cause of refractory respiratory failure in the newborn. Besides pulmonary hypoplasia, the pathophysiology of CDH also includes surfactant deficiency. Vitamin A (vit A) is important for various aspects of lung development.

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Purpose: The aim of this study was to compare the effects of enteral and total parenteral feeding on septic complications in neonates on extracorporeal membrane oxygenation (ECMO).

Methods: Ninety-six neonates were on ECMO between January 1992 and February 1998. Matching for diagnosis and exclusion of neonates with sepsis before ECMO or undergoing surgery on ECMO left 16 enterally fed neonates (cases) and 35 parenterally fed neonates (controls) for analysis.

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Children aged 0-3 yr were stratified for age and randomized to receive either continuous morphine (CM, 10 microg x kg(-1) x h(-1)) with three-hourly placebo boluses or intermittent morphine (IM, 30 microg x kg(-1) every 3 h) with a placebo infusion for postoperative analgesia. Plasma concentrations of epinephrine, norepinephrine, insulin, glucose and lactate were measured before and at the end of surgery and 6, 12 and 24 h after surgery. Pain was assessed with validated pain scales [the COMFORT scale and a visual analogue scale (VAS)] with the availability of additional morphine doses.

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To estimate the association between behavioral and physiological pain measures and to identify determinants predicting the level of association, the COMFORT 'behavior' scale, heart rate (HR), mean arterial pressure (MAP), and the variability of HR and MAP (HRV and MAPV) were assessed every 3 hours after major abdominal or thoracic surgery. Subjects were 204 infants aged 0-3 years. The within-subject correlations, using the repeated measures, were 0.

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Background: Analgesic acetaminophen (INN, paracetamol) plasma concentrations after major surgery in neonates and infants have not yet been established in the literature. We therefore conducted a study in our intensive care unit.

Methods: Forty children, mean (standard deviation) age, 10.

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Background: Episodes of hypoxia and reperfusion play an important role in the development of intestinal damage during perinatal development. The aim of this study was to investigate the histopathology of the intestine in the developing chick embryo after exposure to repetitive hypoxia and recovery under two different conditions: normoxic and hyperoxic (60% O2).

Methods: Chick embryos were exposed to 5 minutes of hypoxia.

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The transcription factor GATA6 is expressed in the fetal pulmonary epithelium of the developing mouse lung and loss of function studies strongly suggested that it is required for proper branching morphogenesis and epithelial differentiation. We have further investigated the role of GATA6 in this process by utilizing a pulmonary epithelium specific promoter to maintain high levels of GATA6 protein during fetal lung development. Transgenic mice expressing Gata6 cDNA under the control of the human Surfactant Protein-C (SP-C) promoter were generated and their lungs were analyzed during fetal stages.

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Serratia species are known for the infections they cause in adult and neonatal intensive care patients. Little is known about colonization and infection in paediatric intensive care patients. This study aims to describe the type of infections in critically ill newborns, infants and children, caused by Serratia spp.

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Background: In necrotizing enterocolitis (NEC), (sub)mucosal edema, hemorrhage, ulceration, or necrosis will disturb intestinal integrity, as reflected by an increased intestinal permeability. Enteral substrate is therefore withheld for a variable period up to 3 weeks (in many clinics). The authors used the sugar absorption test to measure intestinal permeability changes in surgically treated necrotizing enterocolitis patients and surgical controls to evaluate the usefulness of this test in timing the (re-)introduction of enteral feeding in NEC patients as intestinal integrity recovers.

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Recent loss-of-function studies in mice show that the transcription factor GATA6 is important for visceral endoderm differentiation. It is also expressed in early bronchial epithelium and the observation that this tissue does not receive any contribution from Gata6 double mutant embryonic stem (ES) cells in chimeric mice suggests that GATA6 may play a crucial role in lung development. The aim of this study was to determine the role of GATA6 in fetal pulmonary development.

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