European journal of pediatrics
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The association of conotruncal heart defects with 22q11.2 chromosomal microdeletions is well established. However, it is not clear whether particular types of conotruncal malformations or additional cardiovascular anomalies are associated with microdeletions. In addition, cardiac surgery outcome in children with conotruncal defects and del22q11.2 is not well described. ⋯ A complicated postoperative course was more frequent and mortality was higher in patients with conotruncal defects and with/without microdeletion. We conclude that additional cardiovascular anomalies are significantly more frequent in children with 22q11.2 microdeletion and TOF/PA-VSD. Children with conotruncal heart defects and 22q11.2 microdeletion more frequently experienced complicated postoperative course after cardiac surgery.
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Chronic relentless lung infection and pancreatic insufficiency are the cardinal features of cystic fibrosis (CF), a life-shortening autosomal recessive disease. Mutations in the 'cystic fibrosis transmembrane conductance regulator' (CFTR) are currently classified into five groups according to their repercussion on CFTR protein synthesis and its chloride channel function. Stop codon mutations (class I) result in a truncated nonfunctional CFTR, class II mutations consist of aberrantly folded CFTR protein that is degraded by the cell quality control system, while class III mutations lead to defective regulation of the CFTR protein and, consequently, the absence of CFTR function. ⋯ The positive effect of nebulised hypertonic saline on mucociliary clearance is based on the restoration of ASL height. Recent advances in the current treatment of lung infection and inflammation are highlighted in this review. Lung transplantation should be considered in terminally ill patients, but the timing of the transplantation is crucial: transplanting too early shortens survival, while transplanting too late results in patients dying on the waiting list.
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Piritramide is a synthetic opioid commonly used in Germany and Austria for the analgesia of pediatric patients. Little pharmacokinetic and pharmacodynamic data for the pediatric population is available. The aim of this investigation was to gain pharmacodynamic data on postsurgical analgesia and the side effects of piritramide. ⋯ We conclude that dosages of more than 50-70 microg/kg are needed for sufficient analgesia in ventilated postsurgical infants. In spontaneously breathing patients, 50-70 microg/kg provides a 120-min period of analgesia for more than 50% of patients. Cardiovascular stability of the patients was good and, with one exception, there was no respiratory depression.
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Our aim was to compare intrapleural streptokinase (SK) treatment and simple tube drainage in the treatment of children with complicated parapneumonic pleural effusion. A retrospective review of medical records included patient demographics, clinical presentation, biochemical and microbial studies of pleural effusion, radiographic evaluation of chest tube drainage, use of fibrinolytic agents and type of surgical intervention. During the 2.5-year period (1999-2002), 53 children (29 M, 24 F) with complicated parapneumonic effusions or empyema were identified. ⋯ The average length of hospital stay was 19.1 +/- 5.5 and 21.9 +/- 11.2 days for the drainage and streptokinase groups, respectively; the time to afebrile state after admission was 5.8 +/- 4.1 and 7.6 +/- 7.5 days. The percentage of patients who eventually required surgical intervention was 8.3% for the drainage group and 20.6% for the streptokinase group. In conclusion, in the treatment of complicated parapneumonic effusions or empyema, the adjunctive treatment with intrapleural SK does not significantly reduce durations of fever, chest tube drainage and hospital stay, and the need for surgery, regardless of the stage of the disease, compared to simple closed tube drainage.
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The aim of this study was to establish the reference values of preductal oxygen saturation (SpO2) in healthy infants immediately after birth. SpO2 recordings of 200 term neonates (vaginal group;n=150 and cesarean group;n=50) with regular respiratory pattern were evaluated. The median SpO2 values in the first, fifth and tenth minutes were 71, 92, and 98% in vaginal deliveries and 70, 79, and 96% in cesarean deliveries, respectively. ⋯ The time needed to reach a SpO2>90% was three times longer in cesarean deliveries. Healthy neonates are poorly saturated immediately after birth. The duration to reach a SpO2>90% was longer in infants born by cesarean deliveries.