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The Dutch Child Health Care guideline on the early detection of congenital heart disease was developed according to the principles for evidence-based guideline development and contains recommendations for the conduct of the examination during routine check-ups and for referral criteria. Most congenital heart diseases can be detected in the first year of life if physicians and nurses work according to this guideline. Important early times for screening are the home visit to infants at the age of two weeks by the child health nurse and the routine check-up of infants at the age of four weeks by the child health care physician. A routine cardiac screening is carried out in every child by the nurse and the physician at times specified in the basic list of professional responsibilities. A more extensive examination is carried out when indicated. Routine cardiac check-ups are recommended until the age of four, after which they should be done only when indicated on medical grounds. A feasibility study showed that physicians and nurses can apply the guideline effectively, provided that they take part in prior specific training.

Background: Adherent cells undergo apoptosis when detached from their home ground, a process called anoikis (homelessness)., Methods: We developed a new and sensitive method to analyse apoptosis and anoikis of adherent cell types using a time resolved fluorometric assay with Europium-labelled Annexin V. Anoikis was induced with tumor necrosis factor-alpha/cycloheximide and three cell fractions of the cell cultures were prepared and analysed. Fraction 1 consisted of adherent cells, analysed while growing on their support (without detachment by trypsinisation). Fraction 2 contained detached cells due to anoikis (floating cells) and fraction 3 contained apoptotic bodies. Both fractions 2 and 3 were present in the culture medium and were isolated by differential centrifugation., Results: TNF-alpha treatment of three different types of adherent cell cultures induced a significant increase of the amount of floating cells (anoikis) and apoptotic bodies compared to control cell cultures. Also in the adherent cell fractions a small amount of apoptosis was observed., Conclusions: The novel time resolved assay provides the ability to analyse the cell death cascade in adherent cell cultures of the same sample at the same time in a sensitive and reproducible way.

This study represents the development and validation of a cardiac-specific module of the generic health-related quality of life (HRQoL) instrument, the TAAQOL (TNO/AZL Adult Quality Of Life), for young adults with congenital heart disease (CHD). Items were selected based on literature, an explorative previous study in CHD patients, interviews with patients, and the advice of experts. The newly developed Congenital Heart Disease-TNO/AZL Adult Quality of Life (CHD-TAAQOL) was tested in 156 patients with mild or complex CHD and consisted of three hypothesised subject scales: 'Symptoms' (9 items), 'Impact Cardiac Surveillance' (7 items), and 'Worries' (10 items). Cronbach's alpha for the three scales were 0.77, 0.78, and 0.82, respectively. Scale structure was confirmed by Principal Component Analysis, corrected item-scale and interscale correlations. Overall, 55% of reported health status problems were associated with negative emotions, which is an argument for assessing HRQoL as a concept distinct from health status. Convergent validity with validated generic instruments (TAAQOL and Short Form-36, SF-36) showed satisfactory coefficients. Discriminant validity was proven by significantly higher scores for mild CHD patients compared with those with complex CHD. In conclusion, the CHD-TAAQOL module together with the generic TAAQOL can be used to assess group differences for cardiac-specific HRQoL in young adults with CHD. Testing psychometric properties of the CHD-TAAQOL shows satisfactory results. However, to detect changes in HRQoL over time, further research is needed.

Objective: To evaluate job participation, career-related problems, and actual job problems in adults with complex congenital heart disease (CHD) compared with adults with mild CHD and reference groups., Design: Cross-sectional study., Setting: Patients were randomly selected from the archives of the Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, the Netherlands., Patients and Main Outcome Measures: In total, 76 patients with complex CHD and 80 with mild CHD (age range, 17-32 years) completed a self-reported questionnaire on employment and handicaps, with reference data available (response rate, 70%)., Results: In the study groups, 45 (59%) of 76 patients with complex CHD had a paid job compared with 61 (76%) of 80 patients with mild CHD. Patients older than 25 years with complex CHD had significantly lower job participation (64%) than the general population (83%). Multiple logistic regression showed that type of CHD and level of education were significantly and independently related to job participation (odds ratio, 4.8; 99% confidence interval, 1.2-19.6; and odds ratio, 4.7; 99% confidence interval, 1.3-17.2, respectively). Of the 76 patients with complex CHD, 42 (55%) experienced disease-related career problems, in contrast to only 1 patient with mild CHD. Both CHD groups had more job-related mobility handicaps than did the reference group. However, in the mild CHD group, handicaps could be attributed to additional noncardiac diseases., Conclusions: Patients with complex CHD have reduced job participation compared with patients with mild CHD and the general population. Many receive disability benefits or experience career problems or job handicaps. Career counseling focusing on physical abilities and level of education may help prevent or reduce these job-related problems.

Objective: To discuss the need for continuing cardiac surveillance in adults with hemodynamically insignificant congenital cardiac disease., Methods: In 82 patients with mild congenital cardiac malformations, aged from 17 to 32 years, we investigated the subjective health status, the health-related quality of life, any difficulties encountered in daily life, the regularity of follow-up, current diagnosis, and antibiotic prophylaxis., Results: The subjective health status, and the health-related quality of life, did not differ from those of the general population. Nevertheless, patients experienced unnecessary difficulties with choice of sport, obtaining insurance cover, and education. After clinical re-evaluation, diagnosis and antibiotic regimes had to be changed in 9 patients (11%)., Conclusions: Patients with a mild congenital cardiac malformation consider themselves to be in good health. So as to fine tune the diagnosis, and update the information supplied to the patients, we suggest that at least a cardiological assessment be carried out at the age of 16 to 18 years. In this way, patients might be protected from unnecessary difficulties, such as restrictions for sport or the charging of unjustifiably high rates for insurance.

Objectives: To evaluate difficulties in daily life, and satisfaction with level of knowledge about their disease, in patients with congenital cardiac disease in order to improve counselling., Methods: A self-administered questionnaire was completed by 80 patients with mild, and 76 with complex, congenital cardiac disease. They were aged from 17 to 32 years., Results: Even patients with only mild malformations experienced difficulties related to their disease, but being found in only 11%, these were significantly less than those uncovered in 87% of those with complex disease (p < 0.001). Those patients with complex malformations frequently felt restricted in choices because of their disease in areas such as sport (59%), employment (51%), and education (34%). Other difficulties reported were: paying a higher premium for life insurance (29%), having to give up on a sport (28%), and being excluded from a job (18%). Depending on the item, between one-fifth and two-thirds of participants reported gaps in knowledge, most frequently for "causes of congenital cardiac disease", "future consequences", and "family planning". For 53% of those with mild anomalies, and 93% of severely affected patients, the cardiologist is the most important source of information., Conclusions: A minority of adults with mild, and a majority of those with complex congenital cardiac disease report difficulties in daily life. A substantial number of these patients feel that they have an inadequate level of knowledge about their disease. Our results suggest the need for a specific programme of counselling.

Objective: To examine the impact of previously operated complex congenital heart disease on health related quality of life and subjective health status and to determine the relation between these parameters and physical status., Design: Cross sectional; information on medical follow up was sought retrospectively., Setting: Patients were randomly selected from the archives of the paediatric cardiology department, Leiden University Medical Centre, Leiden, The Netherlands, and approached irrespective of current cardiac care or hospital of follow up., Patients: Seventy eight patients with previously operated complex congenital heart disease (now aged 18-32 years) were compared with the general population., Main Outcome Measures: Health related quality of life was determined with a specifically developed questionnaire (Netherlands Organisation for Applied Scientific Research Academic Medical Centre (TNO-AZL) adult quality of life (TAAQOL)) and subjective health status was assessed with the 36 item short form health survey (SF-36). Physical status was determined with the objective physical index, Somerville index, and New York Heart Association functional class., Results: Health related quality of life of the patients was significantly worse than that of the general population in the dimensions gross motor functioning and vitality (p < 0.01). Correlations between health related quality of life and physical status were poor. Patients had significantly worse subjective health status than the general population in the dimensions physical functioning, role functioning physical, vitality, and general health perceptions (p < 0.01). Correlations between subjective health status and physical indices were weak., Conclusion: Adult survivors with previously operated complex congenital heart disease experienced limitations only in the physical dimensions of health related quality of life and subjective health status. Objectively measured medical variables were only weakly related to health related quality of life. These results indicate that, when evaluating health related quality of life, dedicated questionnaires such as the TAAQOL should be used.

CCAAT/enhancer binding proteins (C/EBP) have been demonstrated to impact directly the development of multiple hematopoietic lineages. However, the role of C/EBPbeta in the differentiation of various hematopoietic lineages has not been thoroughly examined. We used primary bone marrow cultures to assess directly the ability of C/EBPbeta to influence myelopoiesis. Retroviral expression vectors were used to express C/EBPbeta ectopically in murine primary long-term bone marrow cultures. The differentiation potential of these cells was determined using hematopoietic colony assays and differential staining of cells within the cultures. Bone marrow cultures that overexpressed C/EBPbeta had fewer myeloid progenitors and a significant increase in the number of granulocytes. The ability of C/EBPbeta to alter hematopoiesis in vitro was dependent on the presence of the transcriptional activation domain because LIP, which lacks this functional domain, did not decrease the ability of bone marrow cultures to support myeloid progenitors. These data also show that C/EBPbeta influences hematopoiesis by altering stromal cell function rather than the intrinsic developmental potential of myeloid progenitor cells.

Objective: To assess and compare the quality of life of patients treated with haemodialysis and chronic ambulatory peritoneal dialysis (CAPD) in Utrecht and Willemstad, Curaçao., Design: Transverse multicentre study., Methods: All haemodialysis and CAPD patients in Utrecht and all haemodialysis patients in Curaçao under treatment for over 6 months were studied. The objective tests applied were the 'Nottingham health profile', the 'affect balance scale', the 'index of well-being' and the 'Amsterdam complaint profile'. Possible correlations between individual patient-related and treatment-related factors and biochemical variables were also investigated., Results: The objective and subjective tests revealed only slight differences in quality of life in the three groups. In a few respects, the CAPD patients rated the quality of life slightly better. In the Utrecht group a positive relationship was seen between haematocrit (higher owing to treatment with erythropoietin) and plasma bicarbonate concentration, and the quality of life.

The molar lineic absorbance of haemiglobin cyanide was determined by direct titration of haemiglobin with cyanide: 540 epsilon HiCN = 1101 m2 X mol(-1). The stability constant (equilibrium association constant): KHiCN = 1.5 X 10(6) 1/mol, was calculated from the titration curve.