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Biomedical subjects

J Hess

Publications and source records attributed to J Hess.

At least 163 records · Page 9Linked to original sources

Smart Classification System (SmaCS): design, implementation, and application for congenital heart disease terminology.

The development of a generic Smart Classification System (SmaCS) for the design, construction, maintenance, and use of complex controlled terminologies is described. SmaCS is used to create a knowledge-base containing more than 2,500 items for the description of abnormalities, treatments, complications, etc. within the domain of congenital heart disease. The purpose of the combination of domain specific knowledge and terminology is to provide a tool useful for both data entry and the subsequent analysis of the data collected.

Artificial Intelligence↗

Long-term follow-up after surgical closure of ventricular septal defect in infancy and childhood.

OBJECTIVES: The purpose of this study was to assess the health-related quality of life of patients who underwent surgical closure of a ventricular septal defect at a young age between 1968 and 1980. BACKGROUND: Since the beginning of open heart surgery for congenital cardiac malformations, the surgical techniques have continually improved. As a result, even infants have become eligible for surgical repair. Long-term follow-up data are not available on the health-related quality of life of nonselected patients after surgical repair at a young age. We therefore conducted a follow-up study of 176 infants and children consecutively operated on in one institution between 1968 and 1980. METHODS: Patients who were alive and could be traced through the offices of local registrars received an invitation to participate in the follow-up study, consisting of an interview, physical examination, echocardiography, exercise testing and standard 12-lead and 24-h electrocardiography. RESULTS: One hundred nine patients (78% of those eligible for follow-up) participated. The mean interval after operation (+/- SD) was 14.5 +/- 2.6 years. Eighty-four percent of the patients assessed their health as good or very good, and 89% had been free of any medical or surgical intervention since the operation. At physical examination all patients were in good health. Their mean exercise capacity was 100 +/- 17% (range 56% to 141%) of predicted values; 84% of the patients had a normal exercise capacity. Echocardiography demonstrated a small residual ventricular septal defect in seven patients (6%). There were no signs of pulmonary hypertension. No patient had symptomatic arrhythmias. CONCLUSIONS: Long-term results of surgical closure of ventricular septal defect in infancy and childhood are good. Pulmonary hypertension is absent. Personal health assessment is comparable to that of the normal population, as is exercise capacity, even though many patients have anatomic, hemodynamic or electrophysiologic sequelae.

Adolescent↗

Presentation of Listeria monocytogenes antigens by major histocompatibility complex class I molecules to CD8 cytotoxic T lymphocytes independent of listeriolysin secretion and virulence.

Virulence and intracellular persistence of Listeria monocytogenes markedly depend on secretion of listeriolysin (Hly), which promotes invasion of the pathogen from the endosome into the cytosol. Recent studies have provided compelling evidence that Hly also facilitates recognition of listerial antigens, in association with major histocompatibility complex (MHC) class I molecules, by CD8 T lymphocytes. Data presented here confirm that the Hly-deficient strains, the prfA- mutant L. monocytogenes SLCC53 and the transposon mutants L. monocytogenes M3 and M20 are avirulent for mice, and unable to replicate inside bone marrow-derived macrophages (BMM phi). Furthermore, BMM phi infected with M3, M20 or SLCC53 were as efficiently lysed as BMM phi infected with the Hly-positive wild-type strain EGD by MHC class I-dependent CD8 cytotoxic T lymphocytes. Using the highly sensitive polymerase chain reaction method, hly mRNA was detectable in BMM phi infected with L. monocytogenes EGD or SLCC53, but totally absent in M3-infected BMM phi. In the case of M20, an excision of the transposon occurred, but the excision was not precise and the hly gene was approximately 400 base pairs shorter. These findings argue against a unique role for Hly in MHC class I presentation of listerial antigens, although Hly appears central to virulence and intracellular replication. Thus, virulence of L. monocytogenes is dissociable from MHC class I presentation of listerial antigens.

Animals↗

Psychosocial functioning of young adults after surgical correction for congenital heart disease in childhood: a follow-up study.

To investigate the long-term psychosocial outcome of congenital heart disease, the emotional, intellectual and social functioning of 288 (young) adult patients was assessed with standardized assessment procedures 9-23 years (mean follow-up interval: 16 years) after surgical correction for congenital heart disease in childhood, and compared with that of reference groups. With respect to emotional functioning, the patients reported significantly fewer feelings of hostility, fewer neurotic complaints and a better self-esteem than reference subjects. Overall, the results concerning social functioning showed favourable outcomes on daily activities (school, employment) and leisure-time activities for (young) adults with congenital heart disease. No significant differences were found between scores of different cardiac diagnostic groups on hostility, neuroticism, self-esteem and leisure-time activities. The possibility whether the 'denial'-mechanism might have contributed to the positive outcomes is discussed.

Activities of Daily Living↗

Calcium signalling in individual T-cells measured by confocal microscopy.

Laser-scanning confocal microscopy was used in conjunction with a highly fluorescent Ca2+ indicator fluo-3 to visualize real-time alterations in the intracellular Ca2+ concentration ([Ca2+]i) in individual living Jurkat T-cells during the first minutes of activation by phytohaemagglutinin (PHA) at the physiological temperature (37 degrees C). With a delay of 30-120 s, PHA induced a strong [Ca2+]i peak in the micromolar range (1-3 microM). The rise in [Ca2+]i lasted for 1-2 minutes, and was followed by a sustained plateau of elevated [Ca2+]i in the 0.2-0.5 microM range. Some cells (10-20%) responded with additional asynchronous 0.5-1.5 microM peaks during the plateau phase. These oscillations continued for 10-20 minutes. The spans of the peaks ranged from 30 to 100 s, intervals between peaks varied from 60 to 300 s. It was shown that the initial [Ca2+]i peak was associated with Ca2+ mobilisation from internal sources, whereas the plateau was maintained by an influx of Ca2+ from external medium. In K(+)-rich medium or in the presence of quinine, a K+ channel blocker, no secondary response to PHA-activation characterised by an elevated plateau was observed. The data suggest that the Ca2+ influx was dependent on the membrane potential and/or the extracellular K(+)-concentration. Optical sectioning showed that the intracellular Ca2+ distributed almost homogeneously throughout the cell volume both in control and in PHA-stimulated cells including those exhibiting Ca2+ oscillations. This suggests that Ca2+ signals are localized not only in cytoplasm at the cell plasma membrane but can be also transferred directly into the nucleus.

Aniline Compounds↗

Clinical significance of maternal anti-Ro/SS-A antibodies in children with isolated heart block.

OBJECTIVES: We studied 30 consecutive children with isolated heart block to assess the clinical impact of the presence of maternal anti-Ro/SS-A antibodies for isolated heart block. BACKGROUND: Isolated heart block in children, often associated with maternal autoimmune disease leading to anti-Ro/SS-A auto-antibody production, is an infrequent but potentially lethal disorder. METHODS: Thirty children with isolated heart block were studied with respect to medical history and electrocardiographic (ECG) analysis. The presence of anti-Ro/SS-A antibodies was determined in the maternal serum. We also examined the ECGs of all brothers and sisters of the patients for conduction abnormalities. RESULTS: Twenty-one of the 30 children had an anti-Ro/SS-A-positive mother (group A); the other 9 children had an anti-Ro/SS-A-negative mother (group B). Comparison of the clinical data from both mothers and children revealed that these two groups differed significantly with respect to the following: Prenatal diagnosis and obstetric complications occurred more often in group A, whereas progression to complete block, QRS width > 0.08 s, premature ventricular contractions and ventricular standstills > 4.5 s occurred more often in group B. In addition, mothers of children in group A reported more spontaneous abortions. All siblings of children in groups A and B had normal ECGs, excluding a subclinical form of heart block. CONCLUSIONS: Two types of heart block can be recognized: Congenital heart block is associated with maternal anti-Ro/SS-A antibodies and numerous obstetric and neonatal complications. It is diagnosed prenatally or at birth and is usually complete at onset and probably has a substantial recurrence risk. Heart block that is acquired later in life is not associated with maternal autoimmunity and has no risk for recurrence. It often presents as a partial block but progresses to complete block in time.

Adolescent↗

Temporary tricuspid valve detachment in closure of congenital ventricular septal defect.

In a consecutive series of 149 patients with congenital ventricular septal defect (VSD), temporary tricuspid valve detachment was applied in 39 (detached group) to facilitate the transatrial approach for closure of the defect. Baseline characteristics showed that, preoperatively, the detached group were younger (1.3 +/- 2.3 vs. 3.5 +/- 4.1 years, P = 0.002), shorter (0.67 +/- 0.20 vs 0.87 +/- 0.34 m, P = 0.001), lighter (6.9 +/- 5.4 vs 13.5 +/- 12.0 kg, P < 0.002), and had a higher mean right atrial pressure (6 +/- 2 vs 4 +/- 3 mm Hg, P < 0.003), mean end-diastolic right ventricular pressure (10 +/- 3 vs 8 +/- 3 mm Hg, P < 0.01) and mean pulmonary vascular resistance (267 +/- 202 vs 170 +/- 131 dyn s cm-5, P < 0.02) on cardiac catheterization. At surgery the aortic cross-clamp time was longer (48 +/- 17 vs 39 +/- 15 min, P = 0.003). Seven patients died (2 detached, 5 not-detached), from causes not related to either tricuspid detachment or VSD closure. Follow-up was complete with a mean duration of 2.0 years (range 0.1-5.5). All 142 survivors were investigated by echocardiography, which showed normal tricuspid valve function in all but 29 patients who had trivial regurgitation (6 detached, 23 not-detached). There was no tricuspid stenosis. In 30 patients (8 detached, 22 not-detached) a trivial residual VSD could be detected. One reoperation (not-detached) was performed 12.5 months after the initial surgery for recurrent VSD.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Acute effects of balloon valvuloplasty and pacing on left ventricular performance in children with moderate pulmonary valve stenosis, analysed by systolic and diastolic pressure-volume relationships.

Right ventricular overload of volume and/or pressure type may affect left ventricular systolic and diastolic function. This has been shown in animal studies and has been suggested in non-invasive studies in man. Altered geometry of the left ventricle, myocardial hypertrophy and changes in contractile state may be responsible for the change in function. Balloon valvuloplasty is an effective treatment for isolated valvular pulmonary stenosis in children, and results in an immediate decrease of right ventricular systolic pressure. Whether this results in immediate changes in left ventricular performance is unknown. Eight children (age 5.2 to 13.9 years) with moderate pulmonary valve stenosis underwent pulmonary balloon valvuloplasty under general anaesthesia. Left ventricular function measurements before and after valvuloplasty were performed using a combined micromanometer-conductance catheter to obtain end-systolic (ESPVR) and end-diastolic (EDPVR) pressure-volume relationships employing inferior vena cava occlusion both at normal and pacing-induced increased heart rates. Pulmonary valvuloplasty resulted in a decrease in peak systolic right ventricular pressure from 62.8 +/- 13.5 to 34.4 +/- 7.3 mmHg (P < 0.001), without significant changes in left ventricular systolic and end-diastolic pressure, or in cardiac index. The ESPVR was fitted to a linear function to obtain the slope (Ees) and the volume intercept at 75 mmHg (V75). The EDPVR was fitted to an exponential function. At baseline, Ees was 1.68 +/- 0.99 mmHg.ml-1 and V75 was 33.6 +/- 21.8 ml. Neither valvuloplasty nor pacing, which increased mean heart rate from 81 to 112 beats.min-1 (P < 0.001), resulted in significant changes of the parameters Ees or V75.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Catheterization↗

Synthesis and secretion of bacterial antigens by attenuated Salmonella via the Escherichia coli hemolysin secretion system.

We describe a plasmid system which allows the secretion of foreign antigens in attenuated Salmonella aroA strains by the secretion apparatus of E. coli hemolysin. The gene (or gene fragment) encoding the antigen is inserted in frame into a residual position of the hlyA gene, encoding the HlyA secretion signal (HlyAs). Generally, the fused gene is efficiently expressed and the synthesized antigen is in part secreted into the culture supernatant and in part exposed on the surface of the producing Salmonella strain. The successful use of this approach is demonstrated with two antigens of Salmonella typhimurium, PagC and SlyA, both of which are potent virulence factors but produced only in small amounts under in vitro culture conditions and two virulence proteins of Listeria monocytogenes, p60 and listeriolysin. Interestingly the listeriolysin fusion protein proved to be cytolytically active and allowed, when expressed in Salmonella, the escape of these bacteria into the cytoplasm of infected macrophages.

Antigens, Bacterial↗

Aortic root replacement with pulmonary autograft in children.

Between September 1988 and February 1993, 14 patients whose ages ranged from 3 months to 16 years (mean 11.1 +/- 4.3 years) underwent replacement of the aortic root with the autologous pulmonary root for aortic valve disease. The follow-up was 4 years (cumulative total of 25.2 patient-years). There was no early mortality. Late mortality (one patient) was 7.1% (95% confidence limits 0% to 21%). This patient had juvenile rheumatoid arthritis and died of consequent congestive heart failure with autograft failure 6 months after operation. Event-free survival after 4 years was 78.6% (95% confidence limits 50% to 95%). One patient was reoperated on because of autograft failure caused by a relapse of rheumatic fever. One patient operated on for critical neonatal aortic stenosis has subnormal exercise tolerance because of restrictive cardiomyopathy and pulmonary homograft regurgitation. The other 12 patients were in New York Heart Association functional class I at the end of follow-up. There was no prevalence of bacterial endocarditis. There were no signs of primary structural degeneration of the pulmonary autograft. During follow-up, in eight patients, increased anulus diameter of the pulmonary autograft could be demonstrated by precordial two-dimensional echocardiography, suggesting growth of the autograft. Our experience shows that aortic root replacement with the pulmonary autograft can be done with low mortality and morbidity in children with aortic valve disease. The operation seems to be contraindicated in children with juvenile rheumatoid arthritis because of the risk of recurrence of rheumatic disease in the autograft. The pulmonary autograft has also been shown to be susceptible to recurrence of rheumatic inflammation in children with a history of acute rheumatic fever. Despite pulmonary autograft replacement of the aortic valve in infants with critical valvular aortic stenosis and endocardial fibroelastosis, clinical results may be poor. Growth of the autograft is suggested by echocardiographic follow-up. We consider aortic root replacement with the pulmonary autograft the procedure of choice in children who require aortic valve replacement.

Adolescent↗

Long-term follow-up (9 to 20 years) after surgical closure of atrial septal defect at a young age.

To assess the long-term cardiac status after surgical closure of an atrial septal defect (ASD) at a young age, 104 of 135 children who consecutively underwent surgery (aged 0 to 14 years) at 1 institution between 1968 and 1980 participated in a follow-up study and underwent a complete cardiologic examination. Mean follow-up was 14.5 +/- 2.8 years. Most patients (87%) believed their health to be good or very good. At physical examination, all patients were in good health. Ninety-three patients (89%) were in sinus rhythm. Echocardiography showed that right ventricular dilatation was present in 27 patients (26%), 2 of whom had a residual ASD. Bicycle ergometry revealed that 88 patients (88%) had a normal exercise capacity. Both supraventricular and ventricular arrhythmias were observed in 67% of patients by 24-hour ambulatory electrocardiography, but only 3 (3%) had received antiarrhythmic medication, and 4 (4%) had needed a pacemaker. In the group of patients with right ventricular dilatation, the exercise capacity and prevalence of arrhythmias did not differ significantly from those in the group with a normal sized right ventricule. The outcome in patients with a secundum-type ASD was not different from that of those with a sinus venosus-type ASD. The finding of anatomic, functional or electrophysiologic abnormalities was not associated with a longer duration of follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Total scalp replantation based on one artery and one vein.

A successful replantation of a totally avulsed scalp, including both eyebrows, with only one arterial and one venous anastomosis to the superficial temporal vessels is described. Apart from a small partial skin necrosis of the right eyebrow, the entire transplant survived. Subsequently, the patient required only minor additional skin grafting, correction of scars with a skin expander, and reconstruction of the eyebrow with hair micrografts. Replantation of the total scalp based on two or more vessels has previously been recommended and reported elsewhere. In the present case, complete survival of the scalp on only one artery and one vein was demonstrated, indicating that replantation should be considered even if available vessels for anastomosis are minimal.

Adult↗