Congenital Heart Disease and General Practice

The treatment of patients with congenital heart disease (CHD) has progressed vastly over the last five decennia. In the Netherlands, around 200,000 children are born each year, around 1,800 of whom have a CHD. This incidence – 6 – 8 per thousand live births – is reported to be similar round the world, making CHD the world’s commonest congenital anomaly. In the Netherlands, this high incidence and the improved treatment options both mean that the number of people surviving CHD is increasing by approximately 5% per year. No longer are most patients with a CHD children: the paediatric population is now outnumbered by the adult population. Although accurate statistics on prevalence are lacking, as many as 70,000 children and adults with different types of CHD are estimated to be alive in the Netherlands today. Representing a prevalence of approximately 4 per 1000 patients, this approaches the prevalence of insulin-dependent diabetes mellitus (11 per 1000 patients) and rheumatoid ar

Spallation reactions. A successful interplay between modeling and applications

The spallation reactions are a type of nuclear reaction which occur in space by interaction of the cosmic rays with interstellar bodies. The first spallation reactions induced with an accelerator took place in 1947 at the Berkeley cyclotron (University of California) with 200 MeV deuterons and 400 MeV alpha beams. They highlighted the multiple emission of neutrons and charged particles and the production of a large number of residual nuclei far different from the target nuclei. The same year R. Serber describes the reaction in two steps: a first and fast one with high-energy particle emission leading to an excited remnant nucleus, and a second one, much slower, the de-excitation of the remnant. In 2010 IAEA organized a worskhop to present the results of the most widely used spallation codes within a benchmark of spallation models. If one of the goals was to understand the deficiencies, if any, in each code, one remarkable outcome points out the overall high-quality level of some models and so the great improvements achieved since Serber. Particle transport codes can then rely on such spallation models to treat the reactions between a light particle and an atomic nucleus with energies spanning from few tens of MeV up to some GeV. An overview of the spallation reactions modeling is presented in order to point out the incomparable contribution of models based on basic physics to numerous applications where such reactions occur. Validations or benchmarks, which are necessary steps in the improvement process, are also addressed, as well as the potential future domains of development. Spallation reactions modeling is a representative case of continuous studies aiming at understanding a reaction mechanism and which end up in a powerful tool.Comment: 59 pages, 54 figures, Revie

Imaging of the spleen in malaria

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Medical predictors for long-term behavioral and emotional outcomes in children and adolescents after invasive treatment of congenital heart disease

The aim of the study was to test the following: (1) the predictive value of medical variables for long-term parent-reported behavioral and emotional problems in children and adolescents who underwent invasive treatment of congenital heart disease in infancy and (2) the relationship between parental psychological distress and parental reports on problems in children. The Child Behavior Checklist was used to investigate to what extent behavioral and emotional problems in 7- to 17-year-old children with congenital heart disease can be predicted by the following: (1) medical history, (2) therapeutic intervention and direct postinterventional course, (3) long-term medical course, (4) present contact with physicians, and (5) present medical status. The General Health Questionnaire was used to assess parental distress (especially anxiety). Higher Child Behavior Checklist total problems scores were predicted by cardiac medication before therapeutic intervention. Palliative intervention (Rashkind procedure) before therapeutic intervention was associated with more favorable scores on total problems and externalizing. Long-term maternal distress was significantly related to parent-reported problems in children. Long-term behavioral and emotional outcomes are only marginally predicted by medical variables. In counseling of children with congenital heart disease and their parents, attention should be paid to long-term maternal distress that has an influence on parent-reported problems in childre

Enlarged right ventricular size at 11 years' follow-up after closure of secundum-type atrial septal defect in children

Item does not contain fulltextBACKGROUND: The fate of right ventricular dimensions after surgical closure of secundum-type atrial septal defects remains unclear. The objectives of this study were to assess ventricular dimensions, exercise capability, and arrhythmias of patients operated for secundum-type atrial septal defect and compare the results with those in healthy references. METHODS: A total of 78 consecutive patients underwent surgical closure for a secundum-type atrial septal defect between 1990 and 1995. In all, 42 patients were included and underwent a cross-sectional evaluation including echocardiography, magnetic resonance imaging, exercise testing, and 24-hour ambulatory electrocardiography. Patients were matched with healthy controls for gender, body surface area, and age. RESULTS: The mean age at surgery was 4.6 plus or minus 2.8 years, and the mean age at follow-up was 16 plus or minus 3 years. There were no residual intracardiac lesions. The mean right ventricular endsystolic volume was significantly larger in patients (142 +/- 26 millilitres) than in references (137 +/- 28 millilitres; p = 0.04). In 25% of the patients, right ventricular end-systolic volume was larger than the 95th percentile for references. No relevant arrhythmias were detected. Exercise testing did not reveal differences with healthy references: maximal power (169 +/- 43 Watt patients versus 172 +/- 53 controls; p = 0.8), maximal oxygen uptake (38 +/- 8 versus 41 +/- 13 millilitres per minute per kilogram; p = 0.1). COMMENT: After surgical closure of secundum-type atrial septal defect, right ventricular end-systolic volume is increased. These findings have no impact on rhythm status or exercise capacity at this stage of follow-up, but may have implications for the timing of surgery or the technique of closure if confirmed in longer follow-up

Exercise capacity and ventricular function in patients treated for isolated pulmonary valve stenosis or tetralogy of Fallot

BACKGROUND We hypothesized 1) that long-term ventricular outcome and exercise capacity would be better in patients with isolated pulmonary valve stenosis (PS) treated with balloon pulmonary valvuloplasty (BPV) than in patients operated for tetralogy of Fallot (TOF), and 2) that ventricular outcome and exercise capacity would not be different in PS patients and healthy controls. METHODS We included 21 PS patients after BPV (16.2 ± 5.2 years) and 21 patients operated for TOF (16.6 ± 5.6 years), matching them for gender, age at treatment, and age at study. Patients underwent cardiovascular magnetic resonance (CMR) imaging, exercise testing, 12-lead ECG and 24-hour Holter monitoring for assessment of right ventricular (RV) size and function, pulmonary regurgitation (PR), exercise capacity and electrocardiographic status. Healthy controls for CMR imaging and exercise testing were matched for gender and age at study. RESULTS RV volumes and PR percentage were significantly larger in TOF patients than in PS patients; biventricular ejection fraction (EF) was not different. PR was mild in most PS patients. RV end-systolic volume was significantly larger in PS patients than in healthy controls; RVEF was significantly lower. Both patient groups had similar exercise test results. Peak workload and VO(2) max. were significantly lower in PS patients than in healthy controls. CONCLUSIONS Longstanding mild PR in PS patients can lead to an enlarged RV, reduced RV function and reduced exercise capacity. Despite more PR and larger RV volumes in TOF patients, exercise capacity and biventricular function are similar in both patient groups

Polyhydroxyalkanoate synthesis in transgenic plants as a new tool to study carbon flow through beta-oxidation.

Transgenic plants producing peroxisomal polyhydroxy- alkanoate (PHA) from intermediates of fatty acid degradation were used to study carbon flow through the beta-oxidation cycle. Growth of transgenic plants in media containing fatty acids conjugated to Tween detergents resulted in an increased accumulation of PHA and incorporation into the polyester of monomers derived from the beta-oxidation of these fatty acids. Tween-laurate was a stronger inducer of beta-oxidation, as measured by acyl-CoA oxidase activity, and a more potent modulator of PHA quantity and monomer composition than Tween-oleate. Plants co-expressing a peroxisomal PHA synthase with a capryl-acyl carrier protein thioesterase from Cuphea lanceolata produced eightfold more PHA compared to plants expressing only the PHA synthase. PHA produced in double transgenic plants contained mainly saturated monomers ranging from 6 to 10 carbons, indicating an enhanced flow of capric acid towards beta-oxidation. Together, these results support the hypothesis that plant cells have mechanisms which sense levels of free or esterified unusual fatty acids, resulting in changes in the activity of the beta-oxidation cycle as well as removal and degradation of these unusual fatty acids through beta-oxidation. Such enhanced flow of fatty acids through beta-oxidation can be utilized to modulate the amount and composition of PHA produced in transgenic plants. Furthermore, synthesis of PHAs in plants can be used as a new tool to study the quality and relative quantity of the carbon flow through beta-oxidation as well as to analyse the degradation pathway of unusual fatty acids