International trends in health science librarianship: Part 2--Northern Europe.

This is the third in a series of articles exploring international trends in health science librarianship in the first decade of the 21st century. The invited authors were asked to reflect on developments in their country--viz. Austria, Belgium, France and the Netherlands.

Dilated cardiomyopathy caused by a novel TNNT2 mutation-added value of genetic testing in the correct identification of affected subjects.

Diagnosing familial dilated cardiomyopathy requires careful family history taking and clinical evaluation in first degree relatives. Based on the results of these findings the diagnosis may be established in the proband. However, due to the age-dependent expression of the disease, doubt may persist regarding the exact status of other family members, especially in young individuals.

Speech and language in Simpson-Golabi-Behmel syndrome: a case report.

Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked disorder caused by a mutation of the glypican-3 gene. The physical characteristics associated with SGBS have been documented in several papers, but information on the behavioral phenotype is scarce. We report on the speech and language characteristics in an 8 year-old-boy with SGBS.

Survey of attitudes towards curriculum reforms among medical teachers in different socio-economic and cultural environments.

Background: Curriculum reforms in medical schools require cultural and conceptual changes from the faculty.

Aims And Methods: We assessed attitudes towards curriculum reforms in different academic, economic, and social environments among 776 teachers from 2 Western European medical schools (Belgium and Denmark) and 7 medical schools in 3 countries in post-communist transition (Croatia, Slovenia, Bosnia and Herzegovina). The survey included a 5-point Likert-type scale on attitudes towards reforms in general and towards reforms of medical curriculum (10 items each).

A comprehensive assessment of medical schools in Bosnia and Herzegovina.

Objectives: To perform internal and external evaluations of all 5 medical schools in Bosnia and Herzegovina against international standards.

Methods: We carried out a 2-stage survey study using the same 5-point Likert scale for internal and external evaluations of 5 medical schools in Bosnia and Herzegovina (Banja Luka, Foca/East Sarajevo, Mostar, Sarajevo and Tuzla). Participants consisted of managerial staff, teaching staff and students of medical schools, and external expert assessors.

Is morbidity influenced by staging in the fontan palliation? A single center review.

In a retrospective study of 32 consecutive patients undergoing a total cavopulmonary connection (TCPC), we tried to determine if the trend for decreasing age at Fontan completion and reducing the intervals between the staged procedures during the past decade was associated with a change in morbidity and outcome. In 8 patients the Fontan circulation was completed in one stage and in 24 patients an intermediate step by hemi-Fontan or bidirectional cavopulmonary anastomosis was performed before Fontan completion. Mean age at TCPC and mean interval since the previous palliation have decreased significantly during the past decade.

Percutaneous closure by a vascular plug of a fistula between the superior caval vein and the left atrium.

We report the case of a 7-year-old boy with a history of pulmonary atresia and intact septum who developed a fistula between the remnant of the ligated superior caval vein and the left atrium after bidirectional superior cavopulmonary (Glenn) anastomosis. The close proximity to the right pulmonary veins made closure by a standard occluder impossible. An Amplatzer vascular plug without rim enabled us to close the connection percutaneously without obstructing the pulmonary venous flow.

Temptation of academic medicine: second alma mater and "shared employment' concepts as possible way out?

Apparently, in developing and in well-developed societies we are confronted with a crisis of academic medicine in all aspects: health care, teaching, and research. Health care providers in teaching hospitals are under pressure to generate revenues, academic research is pressed to keep pace with institutions devoted solely to research, and teaching is often understood not as privilege and honor but as burden and nuisance. The key problem and the principal cause of the crisis are low interest of the best young graduates to follow an academic career in a world where the benefits and values of the private sector are prevailing.

Outcome analysis of major cardiac operations in low weight neonates.

Background: From June 1995 to January 2003, 49 consecutive neonates of less than 2,500 g underwent early surgery for congenital heart disease. A retrospective analysis was performed to evaluate the early to medium term outcome.

Methods: Major cardiac surgery for congenital heart defects included a complete correction in 31 patients (group I) and a palliative procedure in 18 patients (group II).

Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium.

Objective: To assess long term outcome of patients who underwent Mustard or Senning repair for transposition of the great arteries up to 30 years earlier.

Design: Retrospective review of medical records.

Setting: The six university hospitals in Belgium with paediatric cardiology departments.

Left ventricular outflow obstruction in rhabdomyoma of infancy: meta-analysis of the literature.

Primary heart tumors are exceptional in infants and children. Most common is the rhabdomyoma, often associated with tuberous sclerosis (Bourneville's disease). This tumor is generally believed to have no hemodynamic effects in the majority of cases.

The midwall stress-velocity relationship during manipulation of afterload.

Despite providing a physiological correction, measurements of contractility using the midwall stress-velocity relationship still show evidence of an unexplained hypercontractile state in some children. We investigated if by using midwall shortening indexes, the known overestimation of contractility at low afterload could be prevented. In 12 piglets (5 or 6 weeks old), afterload was manipulated by balloon occlusion of the descending aorta and infusion of sodium nitroprusside up to 5 mg/kg/min, and left ventricular function was measured using multiple variables.

Use of an event recorder in the decision for pacemaker implantation in a child with syncope.

Unlabelled: The use of a patient-triggered and automatic event recorder is documented in a 17-month-old girl presenting with paroxysmal episodes of loss of consciousness. After pacemaker implantation, the paroxysmal attacks disappeared.

Conclusion: we recommend a more frequent use of the event recorder in the investigation of syncope, especially in small children.

Limitations of Doppler echocardiography for the post-operative evaluation of aortic coarctation.

Doppler blood flow measurements and derived pressure differences, through the Bernoulli equation, are used in the diagnosis of aortic coarctation, a congenital stenosis distal to the left subclavian artery. Doppler velocities remain elevated at the coarctation site after successful repair of coarctation, leading to high Doppler derived pressure differences without significant arm-leg pressure differences. We studied this apparent contradiction of two diagnostic methods, in vivo using patient and control data, and in vitro using a hydraulic model.

Fixed region of nondistensibility after coarctation repair: in vitro validation of its influence on Doppler peak velocities.

After coarctectomy, local loss of distensibility is noted in addition to mild anatomic narrowing. We hypothesize that the increased Doppler peak velocities measured at the aortic isthmus in these patients partly reflect obstruction secondary to the stiff surgical scar. The hypothesis was studied in a pulsatile hydraulic model.

The influence of low afterload on the nature of the stress-velocity relationship.

Objectives: Correct assessment of contractility by conventional methods during manipulation of afterload is often disappointing. To this purpose, the stress-velocity relationship offers assessment of contractility at different levels of afterload. We decided to study the influence of afterload on the nature of the stress-velocity relation.

Entrapment of a guide wire during percutaneous occlusion of a coronary artery fistula.

Coronary artery fistulas are extremely rare and coil occlusion by intervention techniques seems to be the therapy of choice. We describe the case of a 3-month-old infant with a coronary artery fistula. During occlusion of the fistula a coronary guide wire got entrapped in a small coronary branch, but could be successfully retrieved with a microsnare without damage to the heart.

Influence of age and low afterload on the stress-velocity relation of the left ventricle.

The relation between systolic meridional wall stress (WS) and velocity of circumferential fiber shortening (VcFc) is widely accepted as a preload-independent index of contractility, with a linear relation in most subjects older than 2 y. However, this relation seems to become different in infants and after administration of inotropic agents. We decided to study the nature of the stress-velocity relation by a cross-sectional assessment of the influence of age, low afterload, and increased contractility.

Low-dose dobutamine stress echocardiography in children and young adults.

Dobutamine stress echocardiography has become widely accepted in the evaluation of adult patients with coronary heart disease. We wanted to assess the feasibility and the physiologic responses of stress echocardiography at low doses of dobutamine in a population of normal children and adults. Once achieved, we submitted a group of post-anthracycline patients to the test to assess the sensitivity of low-dose dobutamine stress echocardiography in the detection of cardiac dysfunction.

Simultaneous delivery of two patent arterial duct coils via one venous sheath.

A female child, 10 months of age, weighing 7.2 kg, was catheterised for closure of a patent arterial duct. Aortography was performed in the lateral projection and the minimum diameter of the arterial duct was assessed by comparing it to the size of the catheter.