Paediatric on-call consultants' learning within and beyond the objectives of a coherent CPD program.

Background: Evaluations, using questionnaires, of a two-year long CPD program for on-call consultant paediatricians, showed that the overall objective of the program was largely met. We stipulate that the coherency of the CPD program contributed to the learning. To gains a deeper understanding of the participants learning within and beyond the overall objectives of the program, we decided to conduct an interview study enrolling participants from the first two CPD courses.

Strengthening paediatric competencies and educational skills among general paediatricians.

Aim: To develop a coherent programme that addresses the need for continuing professional development in general paediatrics and educational skills for senior paediatricians at outpatient clinics in Sweden today.

Methods: Educational needs in the target group were investigated using a questionnaire. Themes and sub-themes extracted from responses informed the curriculum formulation of the continuing professional development (CPD) programme, which was completed using a variety of learning, assessment and evaluation methods.

Integrating a pedagogic course in a CPD programme for paediatricians at out-patient clinics.

Equipping paediatricians for the challenges of the explosive development of knowledge and specialised health care calls for a well-planned continuing professional development (CPD) strategy which updates paediatric competencies and the pedagogic skills among paediatricians. The purpose of the study was to evaluate the effects of a pedagogic course, integrated into a CPD programme for paediatricians at out-patient clinics. The pedagogic course comprised three learning components, participation in a CPD programme, during two and a half years, a pedagogic learning module and a pedagogic assignment.

An overview of how on-call consultant paediatricians can recognise and manage severe primary immunodeficiencies.

Severe primary paediatric immunodeficiency syndromes are rare and potentially fatal unless suspected, diagnosed and treated early. We provide clinical guidance and support for on-call consultant paediatricians working in secondary level hospitals on how to recognise and manage children with these conditions. Our paper addresses four conditions that risk the most severe outcomes if they are not adequately cared for during on-call periods, such as weekends: severe combined immunodeficiency, haemophagocytic lymphohistiocytosis, severe congenital neutropaenia and chronic granulomatous disease.

Fifteen-minute consultation: Recognising primary immune deficiencies in children.

Children with primary immunodeficiency syndromes present with broad variation of clinical features and the consequences are often severe if not promptly recognised. Here, support is provided for the general paediatrician to recognise primary immunodeficiencies among the many children they meet in their clinical practice.

Combining learning for educators and participants in a paediatric CPD programme.

Background: Most continuing professional development (CPD) programmes do not include an educational training module. In our country, educational practice in the areas of CPD and continuing medical education relies traditionally on conventional lectures. This is in sharp contrast to the educational research that clearly demonstrates that educational programmes emphasising adult learning methods have greater potential to change physicians' clinical practice.

Using scenario-based training to promote information literacy among on-call consultant pediatricians.

Background: Traditionally, teaching hospital staff to search for medical information relies heavily on educator-defined search methods. In contrast, the authors describe our experiences using real-time scenarios to teach on-call consultant pediatricians information literacy skills as part of a two-year continuing professional development program.

Case Presentation: Two information-searching workshops were held at Sahlgrenska University Hospital in Gothenburg, Sweden.

Blood pressure and anthropometry in children treated with stimulants: a longitudinal cohort study with an individual approach.

Background: Knowledge about the long-term effects on blood pressure (BP) and body mass index (BMI) when treating young patients for attention-deficit/hyperactivity disorder (AD/HD) with stimulants is limited. Most of the studies have reported mean and not individual values for anthropometrics and BP in treatment with stimulants. This seems to be the first study of changes based on the analyses of individual data measured over time.

[On call education for paediatricians may improve patient safety. Continuing professional development project in western Sweden evaluated].

As part of a mandate to provide continuing professional development (CPD) for paediatricians in western Sweden and to support patient safety, we have addressed the professional demands which are imposed on today's consultant paediatricians on call, by designing and implementing a CPD programme for this group. Learning objectives for specific learning modules were developed from a pre-course needs assessment among consultants. The implemented programme included a variety of learning, assessment and evaluation methods.

Devising a competency-based continuing professional development programme to meet the needs of on-call consultant paediatricians.

Aim: To define the clinical competencies currently required by on-call consultant paediatricians in Sweden and to describe how these competencies can be acquired by using an outcome-based continuing professional development (CPD) programme.

Methods: The overall aims and objectives of the programme were formulated to meet the current responsibilities of an on-call consultant. It was delineated during a precourse needs assessment of 26 consultants, before being implemented with 16 participants, using a variety of learning, assessment and evaluation methods.

Clinical manifestation and a new ISCU mutation in iron-sulphur cluster deficiency myopathy.

Myopathy with deficiency of succinate dehydrogenase and aconitase is a recessively inherited disorder characterized by childhood-onset early fatigue, dyspnoea and palpitations on trivial exercise. The disease is non-progressive, but life-threatening episodes of widespread weakness, severe metabolic acidosis and rhabdomyolysis may occur. The disease has so far only been identified in northern Sweden.

Plasma levels of natriuretic peptide type B and A in children with heart disease with different types of cardiac load or systolic dysfunction.

Natriuretic peptide levels B (BNP) and A (ANP) have been described in children with different diagnose of congenital heart defects (CHD). However, the impact of the type of cardiac load per se on natriuretic peptide levels, irrespective of diagnosis, has not been reported. The aim of the present study was to evaluate the levels of BNP and ANP in children with congenital and acquired heart disease according to different types of cardiac load.

Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy.

Aims: Hypertrophic cardiomyopathy (HCM) is the commonest inherited cause of sudden cardiac death in children; current guidelines suggest HCM screening after 12-15 years of age. The study aims to establish the age range at highest risk.

Methods And Results: Cohort study from six regional centres of paediatric cardiology, including children presenting with sudden death; n = 150 (59% = male; 39% familial HCM).

Increased natriuretic peptide type B level after the second palliative step in children with univentricular hearts with right ventricular morphology but not left ventricular morphology.

We analyzed the natriuretic peptide type B (BNP) and A (ANP) levels in the plasma of 38 children with stable functionally univentricular heart defects, 10 of whom had undergone the first palliative step (Shunt), 13 of whom had undergone the second palliative step (Glenn), and 15 of whom had completed total cavopulmonary connection (TCPC). The levels of BNP and ANP were significantly higher after the first palliative step, 31.6 ng/l (8.

Brain natriuretic peptide assessed at long-term follow-up before and after maximal exercise in surgically palliated patients with functionally univentricular hearts.

We evaluated the concentrations of brain natriuretic peptide in the plasma as a marker of systolic ventricular function before and after maximal exercise in 15 surgically palliated patients with functionally univentricular hearts, with apparently good ventricular function. Of the patients, 6 with median age of 14.6 years, and a range from 12.

3-methylcrotonyl-CoA carboxylase deficiency and severe multiple sclerosis.

This report describes a female with isolated 3-methylcrotonyl-CoA carboxylase deficiency. She had a mild Reye-like episode, loss of scalp hair, psychomotor retardation, and an attention-deficit hyperactivity disorder. The diagnosis was made at 13 years of age when she developed relapsing remitting multiple sclerosis with a malignant course.

Echocardiographic and electrocardiographic identification of those children with hypertrophic cardiomyopathy who should be considered at high-risk of dying suddenly.

Background: Hypertrophic cardiomyopathy is a common cause of sudden death in children. In this study, we aimed to identify clinical measures for stratification of this risk in childhood.

Patients And Methods: By means of a retrospective cohort study from six regional centres of paediatric cardiology, we identified 128 patients with hypertrophic cardiomyopathy presenting below 19 years of age, with a mean follow-up of 10.

Increased plasma levels of natriuretic peptide type B and A in children with congenital heart defects with left compared with right ventricular volume overload or pressure overload.

Aim: Natriuretic peptide levels B (BNP) and A (ANP) have been described in children with congenital heart defects (CHD) with pressure and volume overload. However, the impact of ventricular morphology per se on natriuretic peptide levels has not been reported. The aim of the present study was to evaluate plasma BNP and ANP in children with CHD with left or right ventricular volume or pressure overload.

Pulmonary gas exchange during exercise in Fontan patients at a long-term follow-up.

Unlabelled: Desaturation during exercise in the Fontan type of circulation may be due to known right-to-left shunts in some patients, while in others there are no obvious signs of shunts.

Aim: To evaluate the gas exchange and exercise capacity in patients with Fontan circulation.

Material And Methods: Twenty patients with the Fontan type of circulation, median age at operation 7.

Increased levels of brain and atrial natriuretic peptides after the first palliative operation, but not after a bidirectional glenn anastomosis, in children with functionally univentricular hearts.

We evaluated the concentrations of the brain and atrial natriuretic peptides in the plasma as markers of ventricular function and volume load in children with functionally univentricular hearts. We studied 7 children aged from 0.5 to 0.

Syncope--an unusual presentation of ventricle dysfunction in a patient with Fontan circulation.

We report a case of recurrent syncope in association with moderate ventricular dysfunction and mild AV-valve regurgitation in an 18-year-old girl, 4 years after she underwent total cavopulmonary connection surgery. Cardiac catheterization revealed a transpulmonary gradient of 1-2 mmHg. During exercise, a dramatic fall in blood pressure and blood oxygenation was observed, paralleled by an increase in heart rate and central venous pressure.

Repeated blood gas monitoring in healthy children and adolescents by the transcutaneous route.

The aim of the study was to establish transcutaneous oxygen tension (tcPO(2)), carbon dioxide tension (tcPCO(2)), and oxygen saturation (tcSO(2)) values and to investigate the intra- and interindividual variation in these values in healthy children and adolescents. Forty-one healthy children/adolescents (21 girls; mean age, 12.1 years; SD, 3.

Sustained elevated concentrations of cardiac troponin T during acute allograft rejection after heart transplantation in children.

Background: The diagnosis of acute rejection after heart transplantation is made on the basis of endomyocardial biopsy. In children, where the method may be associated with complications, a noninvasive alternative would be desirable. We evaluated the myocardial damage marker cardiac troponin T (cTnT) as a marker of rejection in children who have undergone heart transplantation.