Lethal course of meconium ileus in preterm twins revealing a novel cystic fibrosis mutation (p.Cys524Tyr).

Background: In term newborns meconium ileus is frequently associated with cystic fibrosis. Reports on meconium ileus in preterm infants being diagnosed with cystic fibrosis early after birth are very scarce. Associations between genotype and phenotype in cystic fibrosis and its particular comorbidities have been reported.

Plasma B-type natriuretic peptide levels in children with heart disease.

Aim: To determine plasma B-type natriuretic peptide (BNP) levels in children with heart diseases before medical or surgical treatment for monitoring therapeutic efficacy in an observational prospective clinical trial at tertiary care centre.

Methods: In 522 paediatric patients at an age of 6.4 ± 5.

Long-term remission after first-line single-agent treatment with arsenic trioxide of relapsed acute promyelocytic leukemia in an 8-year-old boy.

Arsenic trioxide (ATO) has been proven to be highly effective in adults with newly diagnosed or relapsed acute promyelocytic leukemia (APL). Only very limited data are published on the use of ATO as a single agent for first-line therapy of relapsed APL. The authors present a case of a 8-year-old boy with a bone marrow relapse of APL 7 years after first diagnosis, who achieved durable molecular remission with ATO as single agent: induction therapy for 12 weeks, consolidation for 4 weeks, then 6 cycles of 10 days over a period of 6 months.

Favourable long-term outcome after immediate treatment of neonatal hyperammonemia due to N-acetylglutamate synthase deficiency.

Introduction: N-Acetylglutamate synthase (NAGS) deficiency is a rare urea cycle disorder, which may present in the neonatal period with severe hyperammonemia and marked neurological impairment.

Case Report: We report on a Turkish family with a patient who died due to hyperammonemia in the neonatal period. Reduced activity of NAGS and carbamyl phosphate synthetase were found at autopsy.

Inflammatory response and neurodevelopmental outcome after open-heart surgery in children.

Long-term neurodevelopmental sequelae are commonly detectable in children after open-heart surgery with cardiopulmonary bypass (CPB). The objective of the study was to determine the neurodevelopmental outcome in these children in relation to postoperative inflammatory reaction. This is a prospective, observational study on children with congenital heart defects (n = 32) undergoing elective open-heart surgery in a tertiary pediatric cardiac center.

Pain relief in ventilated preterms during endotracheal suctioning: a randomized controlled trial.

Background: In Switzerland approximately 8% of infants are born prematurely. Some of them undergo mechanical ventilation including endotracheal suctioning (ETS). ETS is one of the most frequently performed interventions and is linked to stress and pain, but its treatment is controversial.

Prognostic value of plasma N-terminal pro-brain natriuretic peptide in children with congenital heart defects and open-heart surgery.

Objective: To assess whether preoperative and postoperative plasma levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) predict postoperative outcome of open-heart surgery in children.

Study Design: A prospective observational study was conducted with 40 children with congenital heart defects who were undergoing elective open-heart surgery. Plasma levels of NT-proBNP, troponin T, lactate, C-reactive protein, and total neutrophil cell counts were measured before, during, and 1 and 3 hours after the end of cardiopulmonary bypass grafting (CPB).

The efficacy of non-pharmacological interventions in the management of procedural pain in preterm and term neonates. A systematic literature review.

Background: Neonates in a neonatal intensive care unit are exposed to a high number of painful procedures. Since repeated and sustained pain can have consequences for the neurological and behaviour-oriented development of the newborn, the greatest attention needs to be paid to systematic pain management in neonatology. Non-pharmacological treatment methods are being increasingly discussed with regard to pain prevention and relief either alone or in combination with pharmacological treatment.

Electroencephalographic changes after pediatric cardiac surgery with cardiopulmonary bypass: is slow wave activity unfavorable?

Pediatric cardiac surgery with cardiopulmonary bypass (CPB) is frequently associated with neurologic deficits. We describe the postoperative EEG changes, assess their possible causes, and evaluate their relevance to neurologic outcome. Thirty-one children and five neonates with congenital heart disease were included.

Monocyte function-associated antigen expression during and after pediatric cardiac surgery.

Objective: Systemic inflammatory response syndrome and infectious complications are major causes of morbidity and mortality after cardiopulmonary bypass. Recent work in adult patients suggests that the balance between proinflammatory and anti-inflammatory mediators is important. We hypothesized that the expression of different function-related receptors on circulating monocytes might reflect the net response of the inflammatory reaction.

Oxidative stress precedes peak systemic inflammatory response in pediatric patients undergoing cardiopulmonary bypass operation.

Oxidative stress seems to contribute to cardiopulmonary bypass (CPB)-related postoperative complications. Pediatric patients are particularly prone to these complications. With this in mind, we measured oxidative stress markers in blood plasma of 20 children undergoing elective heart surgery before, during, and up to 48 h after cessation of CPB, along with inflammatory parameters and full analysis of iron status.

CXC-chemokine stimulation of neutrophils correlates with plasma levels of myeloperoxidase and lactoferrin and contributes to clinical outcome after pediatric cardiac surgery.

Several CXC-chemokines, of which interleukin (IL)-8 is the prototype, are potent neutrophil chemotactic and activating cytokines, inducing the secretion of granule proteins and the generation of reactive oxygen intermediates that may cause tissue damage and amplify inflammatory responses. Here, we investigated whether chemokines play a key role in the inflammatory process following cardiac surgery with cardiopulmonary bypass (CPB) in children. We performed an observational prospective clinical study of 40 pediatric patients before, during, and after open heart surgery with CPB.

Pain assessment in the neonate using the Bernese Pain Scale for Neonates.

Background: Neonates who require treatment in the neonatal intensive care unit (NICU) are subjected to many invasive painful procedures.

Aims: Assessment of pain in preterm and term neonates with or without ventilation on continuous positive airway pressure using the Bernese Pain-Scale for Neonates (BPSN). The validity and the reliability of the BPSN was established.

Cytomegalovirus-associated necrotizing enterocolitis in a preterm twin after breastfeeding.

Cytomegalovirus (CMV) in breast milk is transmitted to infants and may be associated with disease especially in preterm infants. We present a preterm twin with postnatally acquired CMV infection and evidence of CMV-associated necrotizing enterocolitis.

Plasma levels of interleukin-8 and expression of interleukin-8 receptors on circulating neutrophils and monocytes after cardiopulmonary bypass in children.

Objective: Cardiopulmonary bypass induces a systemic inflammatory response that causes substantial clinical morbidity. This study sought to determine cellular and humoral variables of inflammation. We hypothesized that chemokines are a major source of stimulation of neutrophils and monocytes in pediatric cardiac surgery.

Age-related low expression of HLA-DR molecules on monocytes of term and preterm newborns with and without signs of infection.

Objectives: Presentation of human leucocyte antigen (HLA) molecules is an important part of an efficient immune response. Since bacterial infections are more common in newborns, we hypothesized that the level of HLA-DR expression may influence the host defense system.

Study Design: HLA-DR expression on monocytes was examined by flow cytometry during the first week of life of term and preterm neonates with and without signs of infection and of adults.

Increased respiratory burst and increased expression of complement receptor-3 (CD11b/CD18) and of IL-8 receptor-A in neutrophil granulocytes from newborns after vaginal delivery.

To study neutrophil activation in cord blood as a function of the mode of delivery, we performed analysis of the function of neutrophil granulocytes by assessing their ability to produce reactive oxygen products (ROP) as well as neutrophil cell surface expression of CD11b/CD18 and interleukin (IL)-8 receptors quantified with flow cytometry. Plasma levels of granulocyte colony-stimulating factor (G-CSF) were measured using an immunoassay. Neutrophil granulocytes were derived from cord blood of term newborns delivered vaginally (n = 20) and by cesarean section (n = 10), and, for comparison, from adult peripheral blood (n = 15).

Inflammatory response of neutrophil granulocytes and monocytes after cardiopulmonary bypass in pediatric cardiac surgery.

Objective: To determine whether the activation state of polymorphonuclear neutrophils (PMNs) and monocytes contributes to the inflammatory response after cardiopulmonary bypass (CPB) in pediatric cardiac surgery.

Design: Observational prospective clinical study.

Setting: Pediatric intensive care unit of a university hospital.

Does critical incident reporting contribute to medication error prevention?

Unlabelled: Medication-related critical incidents (CIs) comprise harmful and potentially harmful events. The aim of CI monitoring is quality improvement through system changes. In a prospective survey, we analysed our drug-related CIs of the year 2001 with an emphasis on how they contributed to system changes.