bluuebunny/crossref_metadata_2025 huggingface dataset:
Changes in the tobacco leaf apoplast proteome in response to salt stress
Authors: Dani Valeria , Simon William J. , Duranti Marcello , Croy Ron R. D.
Published: January 2005

The apoplast of plant cells is a dynamic compartment involved in many processes, including maintenance of tissue shape, development, nutrition, signalling, detoxification and defence. In this work we usedNicotiana tabacumplants as a model to investigate changes in the soluble apoplast composition induced in response to salt stress. Apoplastic fluid was extracted from leaves of control plants and plants exposed to salt stress, using a vacuum infiltration procedure. Two‐dimension electrophoretic analyses revealed about 150 polypeptide spots in the pH range of 3.0 to 10.0, in independent protein extracts, with a high level of reproducibility between the two sample sets. Quantitative evaluation and statistical analyses of the resolved spots in treated and untreated samples revealed 20 polypeptides whose abundance changed in response to salt stress. Mass spectroscopic peptide separation and sequencing was used to identify polypeptides affected by salt stress. While the levels of some proteins were reduced by salt‐treatment, an enhanced accumulation of protein species known to be induced by biotic and abiotic stresses was observed. In particular, two chitinases and a germin‐like protein increased significantly and two lipid transfer proteins were expressed entirelyde novo. Some apoplastic polypeptides, involved in cell wall modifications during plant development, remained largely unchanged. The significance of these components is discussed in the context of stress responses in plants.
Pressure‐induced amorphization of Na2Al2Si3O10 · 2H2O and KAlSi2O6 zeolites
Authors: Goryainov Sergei
Published: January 2005

Pressure‐induced amorphization has been observed in two zeolites, natrolite Na2Al2Si3O10 · 2H2O and leucite KAlSi2O6. Using Raman microprobe, amorphized samples were analysed in situ at high pressures at compression in methanol–ethanol medium in diamond anvil cell and also after release of pressure. Before amorphization, one crystal‐to‐crystal phase transition was observed in each zeolite. It was shown for Na2Al2Si3O10 · 2H2O that the transition from crystalline to amorphous phase is partially reversible for compression to the peak pressure in the range of ∼7–10 GPa and practically irreversible above ∼10 GPa. Surprising, the dehydrated crystal KAlSi2O6 exhibited very high quasi‐stability, at compression up to 16 GPa for one day. Long time compression (for 15 days) led to almost full amorphization of KAlSi2O6. (© 2005 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
A simulation model for assessing aberration detection methods used in public health surveillance for systems with limited baselines
Authors: Hutwagner L. C. , Thompson W. W. , Seeman G. M. , Treadwell T.
Published: January 2005

Public health officials continue to develop and implement new types of ongoing surveillance systems in an attempt to detect aberrations in surveillance data as early as possible. In public health surveillance, aberrations are traditionally defined as an observed value being greater than an expected historical value for that same time period. To account for seasonality, traditional aberration detection methods use three or more years of baseline data across the same time period to calculate the expected historical value. Due to the recent implementation of short‐term bioterrorism surveillance systems, many of the new surveillance systems have limited historical data from which to calculate an expected baseline value.

Three limited baseline aberration detection methods, C1‐MILD, C2‐MEDIUM, and C3‐ULTRA, were developed based on a one‐sided positive CUSUM (cumulative sum) calculation, a commonly used quality control method used in the manufacturing industry. To evaluate the strengths and weakness of these methods, data were simulated to represent syndromic data collected through the recently developed hospital‐based enhanced syndromic surveillance systems. The three methods were applied to the simulated data and estimates of sensitivity, specificity, and false‐positive rates for the three methods were obtained. For the six syndromes, sensitivity for the C1‐MILD, C2‐MEDIUM, and C3‐ULTRA models averaged 48.2, 51.3, and 53.7 per cent, respectively. Similarly, the specificities averaged 97.7, 97.8, and 96.1 per cent, respectively. The average false‐positive rates for the three models were 31.8, 29.2, and 41.5 per cent, respectively. The results highlight the value and importance of developing and testing new aberration detection methods for public health surveillance data with limited baseline information. Copyright © 2005 John Wiley & Sons, Ltd.
Bayesian methods for health‐related decision making
Authors: Kadane Joseph B.
Published: January 2005

This article reviews the basics of Bayesian decision theory, and comments on its use in medical decision making. It emphasizes the subjectivity of the probability and utility inputs, and the desirability, in certain contexts, of representing several decision makers, each with his or her own probabilities and utilities. Applications and ethical considerations are also discussed. A brief bibliography gives pointers to the literature. Copyright © 2005 John Wiley & Sons, Ltd.
Computational methods in medical decision making: to screen or not to screen?
Authors: Kafadar Karen , Prorok Philip C.
Published: January 2005

Screening for a disease such as cancer is often regarded as a beneficial and successful strategy for reducing mortality. However, as with any clinical treatment or intervention, benefit cannot be assumed, and screening can entail both costs and harms, so the screening as a ‘treatment’ must undergo evaluation. An evaluation requires a definition of the treatment ‘benefit’, design of studies to measure that benefit with as little bias and variance as possible, and the development of methods for estimating the potential benefit. In screening studies, the factors most central to the evaluation are unobservable (e.g. earliest point in time at which disease becomes detectable, or ‘preclinical’; time at which disease might have been detected in the absence of screening; test sensitivity). Thus, screening programs should be evaluated on scenarios in which these factors are varied, to ensure the robustness of the estimated benefit under a variety of circumstances. This article describes the importance of computational methods and simulations to assess the benefit of screening programs, particularly for cancer, based on randomized screening trials, with special attention to benefit time, lead time, and bias due to length‐biased sampling. Copyright © 2005 John Wiley & Sons, Ltd.
Intrinsic meta‐analysis of contingency tables
Authors: Casella George , Moreno Elías
Published: January 2005

Meta‐analysis has a natural formulation as a Bayesian hierarchical model. The main theoretical difficulty is the construction of a sensible relationship between the parameters of the individual statistical experiments and the meta‐parameter. Since that prior information on such a relationship is typically not available, we argue that this relationship should be dictated by the structure of the model at hand. We then propose a novel procedure based on intrinsic priors which we fully develop for the case of meta‐analysis of 2 × 2 contingency tables. Illustrations on real and artificial tables are given. Copyright © 2005 John Wiley & Sons, Ltd.
Evaluating current policy for detecting mosaicism in amniotic fluid cultures: implications for current cell counting practices
Authors: Caudill Samuel P. , Van Dyke Daniel L. , Chen Andrew T. L. , Reidy John A. , Ing Paul S. , Schwartz Stuart , Vance Gail H.
Published: January 2005

Chromosomal mosaicism is one of the most vexing problems for clinical cytogenetic laboratories and personnel time used for analysis at the microscope is one of the principle costs in cytogenetic laboratories. We use data collected from 26 cytogenetic laboratories to evaluate whether the American College of Medical Genetics guidelines for minimum number of cells to count to exclude mosaicism in amniotic fluid specimens is appropriate. An accurate estimate of the number of mosaics that are missed by current cell counting practices is an important step in this process. Thus, we present a new method for estimating the number of mosaics that are missed and we use computer simulation to evaluate this new method. Our results indicate that if the clinical significance of mosaicism is suspected to be minimal for certain cytogenetic anomalies when the percentage of abnormal cells is 15 per cent or less, then it may be sufficient to use a 15‐cell counting‐rule‐for‐detection along with a minimum total cell count of 30 regardless of whether abnormal cells or normal cells are in the minority. Published in 2005 by John Wiley & Sons, Ltd.
A mixed‐effects quintile‐stratified propensity adjustment for effectiveness analyses of ordered categorical doses
Authors: Leon Andrew C. , Hedeker Donald
Published: January 2005

Observational studies can be used to evaluate treatment effectiveness among patients with a broader range of illness severity than typically seen in randomized controlled clinical trials. However, there are several difficulties with observational evaluations including non‐equivalent comparison groups, treatment doses and durations that vary widely, and, in longitudinal studies, multiple courses of treatment per subject. A mixed‐effects approach to the propensity adjustment for non‐equivalent comparison groups is described that can account for each of these perturbations. The strategy involves two stages. First, characteristics that distinguish among subjects who receive various levels of treatment are examined in a model of propensity for treatment intensity using mixed‐effects ordinal logistic regression. Second, the propensity‐stratified effectiveness of ordered categorical doses is compared in a mixed‐effects grouped time survival model of time until recovery. The model is applied in a longitudinal, observational study of antidepressant effectiveness. Then a Monte Carlo simulation study indicates that the strategy has acceptable type I error rates and minimal bias in the estimates of treatment effectiveness. Statistical power exceeds 0.90 for an odds ratio of 1.5 withN= 250 and 500, and is acceptable for an odds ratio of 2.0 withN= 100. Nevertheless, withN= 100, the models that had high intraclass correlation coefficients had greater tendency towards non‐convergence. This approach is a useful strategy for observational studies of treatment effectiveness. It is capable of adjusting for selection bias, incorporating multiple observations per subject, and comparing effectiveness of ordinal doses. Copyright © 2005 John Wiley & Sons, Ltd.
Statistical collaboration to impact policy decisions
Authors: Morton Sally C.
Published: January 2005

The objective of this paper is to support the following thesis: In health services research, a statistician can, and often should, contribute beyond what is generally defined as the ‘classical’ statistician project role. I use projects from RAND Health, the health services research division of the RAND Corporation, to demonstrate the issues that arise for the statistician in this expanded collaborative role. These projects are the ‘HIV Cost and Services Utilization Study’ (HCSUS) and the ‘Ephedra: Clinical Efficacy and Side Effects Project’. HCSUS collected information on a nationally representative sample of people in care for HIV infection in order to determine what services were being delivered and their cost in order to guide policy decisions on the allocation of limited health care resources. The ephedra project was a systematic review conducted by the Southern California Evidence‐Based Practice Centre to provide information on the existing science so that the National Institutes of Health could guide an expanded research effort to better understand the safety of ephedrine alkaloids. In order to collaborate on more than just the data analysis of a typical project, the statistician must proactively contribute to all project phases. These phases include problem formulation, study design, data collection, analysis, and the communication of results. The statistician needs to effectively and efficiently balance methodological, substantive, practical, and sometimes even ethical demands. As a result of contributing beyond standard statistical tasks, the statistician may directly impact health policy decisions. Copyright © 2005 John Wiley & Sons, Ltd.
Recrystallization of Bacterial S‐Layers on Flat Polyelectrolyte Surfaces and Hollow Polyelectrolyte Capsules
Authors: Toca‐Herrera José Luis , Krastev Rumen , Bosio Vera , Küpcü Seta , Pum Dietmar , Fery Andreas , Sára Margit , Sleytr Uwe B.
Published: January 2005

Polyelectrolyte multilayer (PE) deposition and S‐layer technology have been combined to make novel robust biomimetic surfaces and membranes. Isolated subunits of the bacterial cell surface layer from Bacillus sphaericus CCM2177 SbpA was self‐assembled on PE multilayer supports, with the composition of the multilayer playing a crucial role in determining the structure of the resulting supported protein layers. Flat substrates were studied using atomic force microscopy and neutron reflectometry; protein on suitable PE combinations showed a crystalline structure with lattice constants equal to those found in vivo on bacterial surfaces. The mechanical stability of the S‐layer is higher when recrystallized on PEs than directly on silicon supports. The recrystallization process was subsequently used to coat colloidal particles, permitting the determination of zeta potentials before and after coating. Hollow capsules could also be coated in the same way, as proven by various techniques. Our results suggest that electrostatic interactions via divalent cations are important for the assembly process. The results also demonstrate that the versatility of the PE multilayer membranes can be successfully combined with the well‐defined surface chemistry and structure of 2D protein crystals.
A General in situ Hydrothermal Rolling‐Up Formation of One‐Dimensional, Single‐Crystalline Lead Telluride Nanostructures
Authors: Zhang Lizhi , Yu Jimmy C. , Mo Maosong , Wu Ling , Kwong Kwan Wai , Li Quan
Published: January 2005

In this study we demonstrate that one‐dimensional (1D) nanostructured lead telluride (PbTe) can be synthesized in a hydrothermal reaction between lead foil and tellurium powder. The resulting materials were characterized by X‐ray diffraction, scanning electron microscopy, and transmission electron microscopy. The formation of the 1D structure can be explained by an in situ hydrothermal rolling‐up mechanism whereby PbTe is formed hydrothermally and deposited on the lead substrate. The lead underneath the PbTe layer is then selectively etched by a cetyltrimethylammonium bromide solution, thus allowing the PbTe to roll up into 1D structures. This method can be extended to prepare other 1D tellurides, including CdTe, Cu2Te, and Ag2Te.
Cover Picture: Recrystallization of Bacterial S‐Layers on Flat Polyelectrolyte Surfaces and Hollow Polyelectrolyte Capsules (Small 3/2005)
Authors: Toca‐Herrera José Luis , Krastev Rumen , Bosio Vera , Küpcü Seta , Pum Dietmar , Fery Andreas , Sára Margit , Sleytr Uwe B.
Published: January 2005

The cover picture shows a representation of an artificial virus envelope, which can be formed when crystalline bacterial‐cell surface layers (S‐layers) are coated onto polyelectrolyte nanocapsules. The schematic behind shows the layered structures of such materials. Recrystallization on flat substrates, as well as hollow capsules, can yield novel and robust biomimetic surfaces and membranes. For more information, please read the Full Paper “Recrystallization of Bacterial S‐Layers on Flat Polyelectrolyte Surfaces and Hollow Polyelectrolyte Capsules” by J. L. Toca‐Herrera, A. Fery, and co‐workers on page 339ff.
Perceiving Socially and Morally: The Question of Triangulation
Authors: Wright Edmond
Published: January 2005

One evolutionary advantage is that, because of sensory and perceptual relativity (acknowledged as an empirical fact), the tracking of portions of the real relevant to the living creature can be enhanced if updating from species-member to species-member can take place. In human perception, the structure is therefore in the form of a triangulation (Davidson's metaphor) in which continual mutual correction can be performed. Language, that which distinguishes human beings from other animals, capitalizes on that structure. The means by which updating of adaptiveness takes place in the human species is shown to involve a covert hypothesis of singularity in co-reference, a structure that brings the idea of mutual faith and its character to the fore.
Moral Justification and the Idea of an Ethical Position
Authors: Baz Avner
Published: January 2005

In this paper I develop a critique of Kantian ethics, and more precisely a critique of a particular conception of moral reasoning. The fundamental assumption that underlies the conception that I am targeting is that to justify (morally or otherwise) an action is (perhaps with an ‘all things being equal’ clause) to settle its value, in such a way that all rational participants would have to acknowledge that value. As an alternative to the Kantian conception, I propose a conception in which the basic unit of moral reasoning is not an action but rather what I call an ‘ethical position’—where an ethical position is where, at any given moment and with respect to the matter at hand, you stand, and where moral reasoning consists in the articulation of ethical positions.
Hume's Atomism about Events: a Response to Ulrich Meyer
Authors: Dummett Michael
Published: January 2005

Ulrich Meyer's objections to Dummett's arguments on the time continuum fail because he takes Dummett to endorse Hume's atomistic doctrine that events are ‘loose and separate’, In fact, Dummett rejects this doctrine. He used it in his original article only to indicate that certain implications which are conceptually possible fom the point of view of the classical model of time are not actually conceptually possible.
Regional right and left ventricular function after the Senning operation: an ultrasonic study of strain rate and strain
Authors: Eyskens Benedicte , Weidemann Frank , Kowalski Miroslaw , Bogaert Jan , Dymarkowski Steven , Bijnens Bart , Gewillig Marc , Sutherland George , Mertens Luc
Published: January 2005

Background:Systemic right ventricular dysfunction is a major concern in the follow-up of patients who underwent an atrial redirection procedure for transposition (concordant atrio-ventricular and discordant ventriculo-arterial connections). No good non-invasive method is currently available for quantifying right ventricular function.Aims:We have used ultrasonically based imaging of strain rate and strain to quantify regional deformation in the right ventricle after the Senning operation, comparing properties of regional deformation of the right ventricle with right ventricular ejection fraction as measured using magnetic resonance imaging.Methods:In 20 asymptomatic patients who had undergone the Senning procedure, we measured peak systolic strain rate and systolic strain values in the right ventricular free wall, the septum and the left ventricular lateral wall using colour Doppler myocardial imaging, comparing the data with findings obtained in 30 healthy subjects. Global right ventricular ejection fraction was assessed using magnetic resonance imaging.Results:Properties of deformation of the right ventricular free wall were reduced and homogeneous after the Senning procedure compared to normals, with significantly lower values for peak systolic strain rate and systolic strain (−1.1 ± 0.4 vs. −2.5 ± 0.9 s−1; p < 0.05 and −16 ± 7% vs. −38 ± 13%; p < 0.05, respectively). There was a significant correlation between regional longitudinal right ventricular systolic strain values and right ventricular ejection fraction (r = −0.87, p < 0.001). In the septum, peak systolic strain rate was again reduced and homogeneous (−1.2 ± 0.4 vs. −1.8 ± 0.5 s−1; p < 0.05 vs. normals). Also in the left ventricle, the lateral wall peak systolic strain rate and systolic strain values were reduced (−1.5 ± 0.5 vs. −2.1 ± 0.9 s−1; p < 0.05 and −20 ± 6% vs.−25 ± 9%; p < 0.05, vs. normals, respectively).Conclusions:Properties of regional longitudinal deformation of the systemic right ventricle are reduced after the Senning procedure compared to normal controls, and correlate well with global right ventricular performance. These findings suggest that ultrasonic strain rate and strain imaging could be used in the non-invasive follow-up of ventricular function in these patients.
The hemi-Mustard, bi-directional Glenn, and Rastelli operations used for correction of congenitally corrected transposition, achieving a “ventricle and a half ” repair
Authors: DiBardino Daniel J. , Heinle Jeffrey S. , Fraser Charles D.
Published: January 2005

Based on experience in several centers, the double switch operation has reportedly become the standard surgical therapy for congenitally corrected transposition. We report and discuss here the use of a “ventricle and a half” double switch operation performed due to the concerns raised intraoperatively because of the size of the morphologically right ventricle. Although the long-term course of such a procedure in this setting remains unknown, we submit that the proposed benefits of the double switch operation, even when used in the “ventricle and a half” configuration, may be superior to the alternatives.
Proposals for future training in interventional paediatric cardiology
Authors: Duke Christopher , Qureshi Shakeel A.
Published: January 2005

Training in all medical disciplines is currently undergoing a major overhaul. There is a move away from the old concept of training as an apprentice, towards more structured programmes of training. Proposals have recently been made to shorten higher specialist training to 3–4 years, thus producing “generalist” consultants to meet the growing need for service. Advanced subspeciality training can then be undertaken by those with the ability, and desire, to do so following the core training. In the near future, therefore, subspeciality training will need to meet the needs of those undertaking core training, and those wishing to develop a higher degree of expertise. It will have to be focused, to allow skills appropriate to the future practice to be undertaken as a consultant to be developed efficiently. We propose a new scheme for training in interventional paediatric cardiology as a template for subspeciality training. The scheme proposes training at three levels. The first level, basic training, is required of all trainees. Training for the intermediate level will be required for those proposing to carry out diagnostic cardiac catheterisation and basic intervention. Training at the advanced level will be needed by those who wish to carry out a wide range of interventional procedures. A curriculum is proposed for each level, detailing skills that must be attained. Attendance at procedures needs to be driven by the educational needs of the trainee, rather than the requirements of the service. Objective methods are suggested to allow assessment of competence. These should supersede log-books, which document only procedural numbers. Training may be needed for the trainers to ensure that teaching and assessment achieve agreed standards of excellence.
Time course of appearance of markers of arrhythmia in patients with tetralogy of Fallot before and after surgery
Authors: Balkhi Rim Al , Beghetti Maurice , Friedli Beat
Published: January 2005

Sudden death and ventricular tachycardia are known to occur late after correction of tetralogy of Fallot. Abnormal dispersion of the QT interval, ventricular late potentials, and prolongation of the QRS complex, alone or in combination, are useful markers of the risk for such complications. Our present prospective study investigates the time course of appearance of two markers, dispersion of QT and JT, and ventricular late potentials, before and after corrective surgery. Dispersion of QT and JT, and signal averaged electrocardiographic parameters, were determined sequentially in 20 patients before, a mean of 9 ± 3 days after, and again 35 ± 11 days post-operatively. Dispersion of QT was already abnormal before surgery in two-fifths of the patients, but increased markedly in the later post-operative period. Ventricular late potentials were absent before surgery and in the immediate post-operative period, but were found in one-fifth of patients 1 month later. We conclude that abnormal dispersion of QT is, to some extent, a fact of the natural history of tetralogy of Fallot, but is significantly amplified by surgery. Ventricular late potentials, on the other hand, are absent before surgery, but appear with some delay after the operation, probably as a result of scarring rather than the surgical incision itself. Long-term follow-up is needed to assess the significance of these findings.
Diverse experience with the CardioSEAL®and STARFlex®septal occluders
Authors: Apostolopoulou Sotiria C. , Laskari Cleo V. , Kiaffas Maria , Papagiannis John , Rammos Spyridon
Published: January 2005

Apart from closure of atrial septal defects, there is little information concerning the use of the CardioSEAL family of occluders in congenitally malformed hearts. We review here our initial experience using the CardioSEAL and STARFlex occluders in 12 patients aged 17.3 ± 11.2 years, with a range from 4 to 34 years. Of the patients, 5 had fenestrated extracardiac Fontan procedures, 5 had persistent patency of the arterial duct, 1 had a leak across a Mustard baffle, and the final patient had a huge pulmonary arteriovenous malformation. We implanted successfully 9 CardioSEAL, and 3 STARFlex occluders, with sizes from 17 to 40 mm. In one patient, the occluder embolized to the right pulmonary artery, from where it was retrieved through the catheter. In two patients, there was a trivial residual leak immediately after implantation, but no patient had a residual leak after 6 months of follow-up. We noted improved ventricular dimensions, without any fractures of the arms of the occluders, perforations, or disturbances of flow after 2.4 ± 0.9 years of follow-up. We have demonstrated, therefore, the versatility of the CardioSEAL and STARFlex occluders, which have been used safely and effectively to close a variety of intra and extracardiac communications other than atrial septal defects.
Follow-up in patients with congenital cardiac disease more complex than haemodynamic assessment
Authors: Fredriksen Per Morten , Mengshoel Anne Marit , Frydenlund Aina , Sørbye Øystein , Thaulow Erik
Published: January 2005

The objective of the study was to assess behavioural and emotional problems, as well as physical capacity, in children and adolescents with congenital cardiac disease. From the database of Paediatric Heart Section, Children's Clinic, Rikshospitalet University Hospital, Oslo, Norway, we identified 430 patients whose parents received questionnaires using the Child Behaviour Check-List. The response rate was 75.8%. In addition, the parents received a questionnaire focusing on special issues with regard to physical activity. Parents of children and adolescents with congenital cardiac diseases reported significantly more behavioural problems than did a reference population and boys were scored higher compared to girls. Analysis showed a significant impact of physical capacity on the score representing total problems, as well as scores for externalising and internalising behaviour. Compared to a reference population, parents of children and adolescents with congenital cardiac disease score their children higher on most scales when rated using the Child Behaviour Check-List. The type of diagnosis did not affect the scores reflecting the total problem. The main factor of impact on behavioural problems was, as evaluated by the parents, the physical capacity of the children.
Congenital cardiac malformations in Iceland from 1990 through 1999
Authors: Stephensen Sigurdur Sverrir , Sigfusson Gunnlaugur , Eiriksson Herbert , Sverrisson Jon Thor , Torfason Bjarni , Haraldsson Asgeir , Helgason Hrodmar
Published: January 2005

Introduction and Background:About 1% of live-born children have congenital malformations of the heart. The aim of our study was to investigate the incidence of such defects in children born in Iceland during a period of 10 years, extending from 1990 through 1999.Materials and Methods:Information about the patients was obtained from medical records from two hospitals that cover the whole country, a private clinic of pediatric cardiologists, an echocardiography database, autopsy reports, and death certificates. We investigated the distribution of specific malformations, the age at diagnosis, the symptoms leading to the diagnosis, the source of referral, and treatment and quality of life.Results:Between 1990 and 1999, there were 44,013 live births in Iceland, of which 740 patients were diagnosed with congenital cardiac malformations, accounting for 1.7% of the live-born children. The distribution was made up of 338 patients with ventricular septal defect (45.7%), 90 with atrial septal defect (12.2%), 85 with patency of the arterial duct (11.5%), 48 with pulmonary valvar stenosis (6.5%), 38 with a bicuspid aortic valve (5.1%), 28 with aortic coarctation (3.8%), 22 with tetralogy of Fallot (3.0%), 14 with transposed great arteries (1.9%), 11 with aortic stenosis (1.5%), 10 with atrioventricular septal defect and common atrioventricular orifice (1.4%), 9 with mitral valvar regurgitation (1.2%), 7 with sub-aortic stenosis (0.9%), and 5 with hypoplasia of the left heart (0.7%). Extracardiac anomalies were seen in 89 patients (12.0%). Chromosomal defects were seen in 36 patients, of whom 28 had Down's syndrome.Discussion:The annual incidence of diagnosis of patients with congenital cardiac malformations increased during the period of study. This was noted for minor defects, but the incidence of the major anomalies did not alter. Our observed yearly incidence, at 1.7%, was higher than noted in a previous study covering the years 1985 through 1989, and is also higher than in other population-based studies. The most likely explanation is the fact that access to pediatric cardiologists in Iceland is very good. Diagnosis, registration, and follow-up are conducted by only a few cardiologists, and take place at a single center for pediatric cardiology.
Repair in infancy of right aortic arch with aberrant left brachiocephalic artery in the setting of a variant of the hypoplastic left heart syndrome
Authors: Sett Suvro S. , Mawson John B. , Sandor George G. S.
Published: January 2005

A right aortic arch, with a retroesophageal transverse arch and an aberrant left brachiocephalic artery, is a rare anomaly by itself. When combined with severe mitral stenosis and hypoplasia of the left ventricle, it is rarer still. We describe the diagnosis and treatment of this rare anomaly in an infant using a modification of the Norwood operation.
Successful repair of critical tricuspid regurgitation secondary to ruptured papillary muscle in a newborn
Authors: Lim Kyoung Ah , Huh June , Jun Tae-Gook
Published: January 2005

We report a rare case of an isolated critical tricuspid regurgitation due to rupture of a papillary muscle. This patient presented with a cyanosis immediately after birth. Despite mechanical ventilation and medical management to decrease the pulmonary vascular resistance, the low cardiac output persisted, along with the cyanosis. Repair of the tricuspid valve was performed, using an artificial tendinous cord, on the 4th day of life. The short-term result of the surgery is satisfactory, but the patient requires long-term follow-up.
Preoperative diagnosis of co-existing divided left atrium and tetralogy of Fallot
Authors: Carroll Sheila J. , Solowiejczyk David , Gersony Welton M.
Published: January 2005

We present the eighth published case of divided left atrium co-existing with tetralogy of Fallot. This is the first report of preoperative echocardiographic diagnosis of this unusual combination of defects. Demonstration of a partition within the left atrium is imperative for successful repair of the combined lesions. We draw attention to the need for careful echocardiography in patients where an obstruction to pulmonary venous drainage would dramatically affect the outcome subsequent to surgical correction.
Persistent electrical and morphological atrial abnormalities after early closure of atrial septal defect
Authors: Cua Clifford L. , Sparks Elizabeth E. , Chan David P. , Daniels Curt J.
Published: January 2005

Atrial arrhythmias are associated with enlarged atrial chambers and an increased duration of the P wave. Repair of atrial defects within the oval fossa is expected to normalize atrial size. Few studies, however, have evaluated electrical and morphological atrial features after repair. Our study was performed to determine if atrial abnormalities exist after surgical closure of such atrial septal defects, and whether early closure improves outcome. We recruited patients who had undergone surgical closure of a defect within the oval fossa, so-called “secundum” atrial septal defects. Electrocardiograms, signal averaged electrocardiograms, and echocardiograms were performed. Two-tailed test and Pearson correlation was utilized for statistical analysis. The population consisted of 20 patients and 27 controls, with the mean age of the patient being 11.25 ± 5.10 years, their age at surgery 6.55 ± 5.10 years, and the time since surgery 4.70 ± 2.61 years. The size of the right (23.88 ± 6.35 ml/m2 versus 18.84 ± 4.43 ml/m2) and left (21.91 ± 12.47 ml/m2 versus 17.72 ± 4.83 ml/m2) atrium were significantly larger in the patients. The duration of the P wave (108 ± 16 ms versus 96 ± 8 ms) and the duration of the PR interval (155 ± 18 ms versus 138 ± 23 ms) were longer. No correlation existed between age or interval since surgery with atrial sizes or measurements of the signal averaged electrocardiogram. We conclude that, despite surgical repair, abnormalities exist in patients with an atrial septal defect. Early surgery does not appear to prevent the atrial abnormalities.
Outcomes for children with acute myocarditis
Authors: English Robert F. , Janosky Janine E. , Ettedgui Jose A. , Webber Steven A.
Published: January 2005

The optimum treatment for myocarditis in children is unknown. We present outcomes for this disease as seen in a large series of children. Thus, we identified all children seen with myocarditis at Children’s Hospital of Pittsburgh since 1985, including only those with biopsy-proven myocarditis, or cardiac dysfunction and proof of concomitant cardiotropic viral infection. Outcomes were defined as complete recovery, incomplete recovery, and death or transplantation.

We identified 41 patients, 37 proven by histology, and 4 patients who were too unstable for biopsy but had proof of viral infection. Of the group, 27 (66%) made a complete recovery, 4 (10%) had incomplete recovery, and 10 (24%) either died (5) or underwent transplantation (5). The median time to death or transplantation was 8.4 months, with a range from 1 day to 49 months. Steroids had been administered to 16 patients, of whom 10 made a complete recovery, 2 an incomplete recovery, 2 died, and 2 were transplanted. Intravenous immune globulin was given in isolation to one patient, who made a complete recovery, and to 18 in combination with steroids, of whom 12 made a complete recovery, 2 an incomplete recovery, 2 died, and 2 were transplanted. The remaining 6 patients received neither steroids nor intravenous immune globulin, and of these, 4 made a complete recovery, 1 was transplanted, and 1 died. Freedom from death or transplantation was 81% at 1 year, and 74% at 5 years, with no difference between the modes of treatments. The median time to recovery of function was also comparable between the groups. Thus, in our patients, treatment with intravenous immune globulin appeared to confer no advantage to steroid therapy alone. These data emphasise the need for randomised trials to assess the efficacy of current treatments, as well as that of new therapies.
Quantitative analysis of right atrial performance after surgical repair of tetralogy of Fallot
Authors: Hui Wei , Abd El Rahman Mohamed Y. , Dsebissowa Fatima , Rentzsch Axel , Gutberlet Matthias , Alexi-Meskishvili Vladimir , Hetzer Roland , Lange Peter E. , Abdul-Khaliq Hashim
Published: January 2005

We aimed to assess the right atrial performance in patients after surgical correction of tetralogy of Fallot, and to clarify the relationship between the pump function of the right atrium and right ventricular systolic function.

We included in the study 50 asymptomatic patients following corrective surgery of tetralogy of Fallot, comparing them to 30 normal subjects. Right atrial areas were measured by echocardiography, and the active fractional area of emptying was expressed, in percentages, as the area measured at the onset of atrial contraction, minus the minimal area, divided by the area at the onset of atrial contraction. We used this value to assess the atrial pump function. Right atrial peak strain rates were measured by tissue Doppler imaging. Compared to controls, patients with tetralogy of Fallot had a significantly reduced right atrial active fractional area of emptying (p = 0.005), along with a reduced peak late diastolic strain rate (p = 0.002). Among 20 patients who underwent magnetic resonance tomographic examination, a relatively higher right atrial peak late diastolic strain rate was shown in patients with a right ventricular ejection fraction of less than 50% (p = 0.021).

Right atrial performance is reduced in patients after surgical correction of tetralogy of Fallot. When facing right ventricular systolic dysfunction, nonetheless, the right atrial pump function may be relatively enhanced. Tissue Doppler derived strain rate can provide quantitative analysis of regional right atrial performance.
Unguarded tricuspid valvar orifice in the fetus
Authors: Wong Kenny K. , Farquharson Duncan I. , Duncan Walter J.
Published: January 2005

The unguarded tricuspid valve is uncommon. We describe herein a fetus with a grossly dilated right ventricle and atrium, with severe tricuspid and pulmonary valvar regurgitation. The right ventricle was akinetic, and no tricuspid tissue or valvar apparatus was identified. Colour Doppler showed a highly unusual retrograde flow of blood through the right heart. The pregnancy was terminated, and necropsy examination confirmed the gross dilation of the right heart chambers, with severely dysplastic valvar tissue at the right atrioventricular junction effectively giving an unguarded orifice. There was no valvar displacement, and the left heart was normal. The fetus had a normal karyotype, albeit with absent kidneys.
Spontaneous echoes due to hypoalbuminemia
Authors: Hadash Amir , Braver Yulia , Lorber Avraham
Published: January 2005

We found spontaneous echoes in two teenagers with nephrotic syndrome and profound hypoalbuminemia, both having normal cardiac structure, function and output. The phenomenon disappeared after the level of albumin normalized. In one patient, all spontaneous echoes disappeared following convalescence, the level of albumin in the serum then being documented at normal levels. The second patient, who presented with profound hypoalbuminemia, was infused with human albumin because of oliguria, following which the spontaneous echoes disappeared.
Multiple fistulas from the coronary arteries to the left ventricle in tricuspid atresia
Authors: Ikeda Hiroyuki , Aotsuka Hiroyuki , Nakajima Hiromichi
Published: January 2005

It is rare to find multiple fistulas arising from all three coronary arteries and draining into the left ventricle. Coronary angiography revealed this anomaly in a one-year-old girl with tricuspid atresia after conversion to the Fontan circulation. To the best of our knowledge, this is the first report of such multiple fistulas in the setting of tricuspid atresia, and also the first report in childhood.
A study of congenital cardiac disease in a neonatal population – the validity of echocardiography undertaken by a neonatologist
Authors: Samson Gregory R. , Kumar Suresh R.
Published: January 2005

Objectives:To estimate the incidence of, and profile the spread of, congenital cardiac defects, and to assess the accuracy of the echocardiographic diagnosis as performed by a neonatologist.Design:Hospital-based study.Methods:All neonates meeting our criterions, specifically those with a persistent murmur 48 hours after birth, underwent an echocardiographic examination to exclude an underlying congenital cardiac defect. All scans performed by the neonatologist were videotaped and reviewed by a paediatric cardiologist. We assessed concordance according to inter-rater agreement.Results:Out of 11,085 live births, there were 83 infants with a congenital cardiac defect, giving an incidence of 7.49 per 1000 live births, with 95% confidence interval from 5.88 to 9.09. Of the infants with a murmur persisting at or greater than 48 hours after birth, who had a median age of 4.5 days, 75% had a congenital heart defect, with ventricular septal defect being the commonest, encountered in 56.7% of cases. Concordance between the neonatologist and the cardiologist was good, with Cohen's Kappa coefficient being calculated at 0.68, and 95% confidence interval from 0.51 to 0.85.Conclusion:The incidence of congenital cardiac malformations as determined in our hospital-based study in the United Arab Emirates is similar to that described in the Gulf region and worldwide. A persistent murmur at or greater than 48 hours after birth is strongly suggestive of an underlying congenital cardiac malformation. Our experience shows that a neonatologist appropriately trained in echocardiography can perform as well as a paediatric cardiologist. Where specialist resources are limited, this allows for early diagnosis, earlier referral if necessary, and early institution of appropriate therapy.
A population-based study of extra-cardiac anomalies in children with congenital cardiac malformations
Authors: Eskedal Leif , Hagemo Petter , Eskild Anne , Aamodt Geir , Seiler Karry Stephen , Thaulow Erik
Published: January 2005

We describe the prevalence of extra-cardiac anomalies in children with congenital cardiac malformations, and their impact on survival, compared to the outcome in children with the congenital cardiac lesions as the only recognised anomaly.

Our population comprises the 3527 children born with congenital cardiac anomalies between 1990 and 1999, and registered at the largest tertiary centre for Paediatric Cardiology in Norway. Extra-cardiac anomalies were found in one-fifth of the population, with Down's syndrome accounting for nearly one-third. Survival improved for children born between 1995 and 1999 compared with those born in the period from 1990 to 1994 for all groups, except for children with additional extra-cardiac anomalies in the absence of Down's syndrome. The results were the same for children undergoing surgical treatment of their cardiac malformation. The survival in children with Down's syndrome improved in comparable fashion to those without extra-cardiac anomalies. Children with extra-cardiac anomalies in the absence of Down's syndrome represent a heterogeneous group, with varying patterns of survival. Survival did not improve in these latter patients during the period of our study.
New trends in chromosomal investigation in children with cardiovascular malformations
Authors: Schellberg Ruth , Schwanitz Gesa , Grävinghoff Lutz , Kallenberg Rolf , Trost Detlef , Raff Ruth , Wiebe Walter
Published: January 2005

We investigated a group of 376 children, seen over a period of 7 years with different types of congenital cardiovascular defects, to assess the presence of chromosomal aberrations. The diagnostic approach, achieved in 3 consecutive steps, revealed conventional chromosomal aberrations in 30 of the patients (8%) excluding trisomies 13, 18, 21. Fluorescence in situ hybridisation for microdeletions showed 51 microdeletions (15%), with 43 patients having deletions of 22q11.2, 7 patients with deletion of 7q11.23, and 1 patient with deletion of 4p16.3. In 23 patients with additional clinical abnormalities, we carried out a subtelomeric screening. This revealed, in two cases (9%), different subtelomeric aberrations, namely deletions of 1p and of 1q. Thus, subtelomeric screening proved to be a very valuable as a new diagnostic approach. Our approach to genetic investigation in three phases makes it possible to detect a high rate of pathologic karyotypes in patients with congenital cardiovascular malformations, thus guaranteeing more effective genetic counselling of the families, and a more precise prognosis for the patient.
Vasculopathy of the large arteries in children infected by the human immune deficiency virus
Authors: Obor Nancy Anyango , Cilliers Antoinette Myrna
Published: January 2005

We report on two children with advanced acquired immune deficiency syndrome presenting with vasculopathy involving the large vessels. Both patients had extensive involvement of the aorta and its branches. One patient presented with heart failure, and mild systemic hypertension secondary to renal arterial stenosis, while the other patient manifested with gangrene of both arms.
The signalling pathway which causes contraction via P2‐purinoceptors in rat urinary bladder smooth muscle
Authors: Naramatsu Masahiro , Yamashita Toshikazu , Kokubun Shinichiro
Published: January 2005

The signalling pathway which causes contractions to adenosine 5′‐O‐2‐thiodiphosphate (ADPβS) and α,β‐methylene adenosine 5′‐diphosphate (α,β‐Me ADP) was investigated in rat urinary bladder smooth muscle by measuring isotonic tension.

The responses to 10 μM α,β‐methylene adenosine 5′‐triphosphate (α,β‐Me ATP) in 0 and 3.6 mM Ca2+ were 5.9±1.3 (n=10) and 122.2±6.4 (n=8) % respectively of those obtained in 1.8 mM Ca2+, whereas those to 100 μM ADPβS were 34.6±3.3 (n=8) and 96.8±7.2 (n=8) %, in 0 and 3.6 mM Ca2+, respectively. In both experimental conditions, the responses to the two agonists expressed as % of the control responses were significantly different (P<0.01).

Indomethacin at high concentrations (>1 μM) decreased the responses to α,β‐Me ATP (10 μM), ADPβS (100 μM) and α,β‐Me ADP (100 μM). However, no significant difference was obtained between the responses to all the agonists at 30 μM indomethacin.

2‐Nitro‐4‐carboxphenyl n,n‐diphenylcarbamate (NCDC) at concentrations between 1 μM and 100 μM concentration‐dependently decreased the responses to ADPβS (100 μM) and α,β‐Me ADP (100 μM) and almost completely inhibited them at 100 μM. Although the responses to α,β‐Me ATP (10 μM) were also inhibited by the drug, at 50 and 100 μM NCDC the responses to α,β‐Me ATP were significantly larger than those to ADPβS and α,β‐Me ADP (P<0.01).

NCDC 100 μM significantly inhibited the KCl‐induced contraction to 65.9±4.9% (n=6) of the control (P<0.01).

It is suggested that the contraction via ADPβS‐sensitive receptors in the rat urinary bladder smooth muscle mainly depends on Ca2+ ions liberated from intracellular Ca2+ stores, though the contribution of Ca2+ ions from the extracellular space cannot be neglected. The release of Ca2+ ions from stores is mainly mediated by the production of inositol trisphosphate (IP3) via the activation of phospholipase C.

British Journal of Pharmacology (1997) 122, 558–562; doi:10.1038/sj.bjp.0700157