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Problem: Competency-based training is a model of medical education program that uses an organized framework of competencies to develop entrustable professional activities (EPAs) for each specialty. Implementation of EPAs varies by medical specialty and country. Although national models for primary care pediatrics have been suggested in the past, standardized EPAs have not been established., Approach: The authors, all members of the curriculum working group of the European Confederation of Primary Care Paediatricians, describe the development and design of a training model based on EPAs specifically for pediatricians in primary care. The objective was to facilitate and disseminate a training model that standardizes and improves training in primary care pediatrics. They aimed to make the model flexible enough so that it can be adapted to different training models and different European health systems., Outcomes: Between May 2017 and December 2019, the authors developed a framework that includes 7 domains of competencies for the training in pediatric primary care and a set of 11 core EPAs to guide the learning process. They structured each EPA as follows: title, activity profile, domains of competence, competencies within each domain, required knowledge and skills, learning objectives described in terms of outcomes, and a 3-stage rating supervision scale to evaluate the level of performance., Next Steps: The next step is the progressive implementation of these EPAs into the postgraduate primary care pediatrics training programs of European health systems. To support this implementation, the authors intend to develop training videos to assist in daily teaching of the EPAs. The authors will also develop a pan-European online resident forum designed specifically to facilitate EPA learning by sharing and discussing clinical cases. They plan to launch a pilot study on the use of the EPAs in primary care health centers in 6 European countries., (Copyright © 2023 by the Association of American Medical Colleges.)

Background: Adults with transposition of the great arteries (TGA) after atrial switch repair have an increased risk for arrhythmia and sudden cardiac death. We analyzed whether a remote monitoring (RM) system as part of an implantable cardiac device contributes to timely recognition and improved treatment of critical arrhythmias in these patients., Methods and Results: All consecutive TGA patients (n=11) requiring a pacemaker or cardiac resynchronization therapy with or without implantable cardioverter defibrillator between 2008 and 2011 were included. RM-detected arrhythmia, abnormality of device integrity and reaction time from event transmission until acknowledgement via email and clinical decision making were analyzed and compared to a control group (n=21). In 10 patients (91%) 17 arrhythmias were detected, 8 patients (80%) indicated no symptoms. In the RM group time interval from transmission to acknowledgement was 2.4 days (range, 0-4.5 days). Clinical decision-making was advanced by a mean of 77.5 days (range, 10-197 days) compared with conventional follow-up and identified adaption of anti-arrhythmic medication in 8, electrical cardioversion in 2, overdrive pacing in 1 and radiofrequency ablation in 2 patients. A coronary sinus lead fracture was identified in 1 patient followed by successful replacement., Conclusions:  RM enables early detection of tachyarrhythmia followed by optimization of medical treatment and potentially life-saving anti-tachycardic intervention in adults after atrial repair of TGA.

Background: Thermal stress is a risk factor for sudden infant death syndrome (SIDS). Recently, baby sleeping bags have been recommended as a preventive measure against SIDS. The aim of this study was to describe in which way the use of baby sleeping bags might influence thermoregulation of sleeping infants and maybe the incidence of SIDS., Methods: Body surface temperature was recorded by use of infrared thermography in 15 infants (median age 49 days). Recordings were done twice: after sleeping for 60 min under a blanket and after sleeping for 60 min in a baby sleeping bag. Temperature was recorded and compared for defined sites of body surface., Results: Infants' mean body surface temperature as well as core temperature after sleeping in a baby sleeping bag did not show significant differences when compared to infants sleeping under a conventional blanket., Conclusion: Under controlled conditions, core temperature and mean body surface temperature are comparable, equally if using a baby sleeping bag or conventional bedding. However, under the more uncontrolled conditions of baby care at home, sleeping bags might provide a more constant temperature profile, while other bedding conditions may lead to significant variations of temperature pattern., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: Pacifier use has been postulated to decrease the risk of sudden infant death syndrome (SIDS). The responsible mechanisms are, however, unclear., Objectives: Since little is known about the non-nutritive sucking (NNS) habits of infants during sleep, we investigated NNS patterns and changes of physiological parameters during NNS in sleeping infants., Methods: Polygraphic recordings were performed in 12 infants with a median age of 55 days (range 7-82) who regularly used a pacifier during sleep. Episodes of active suckling (bursts) and quiescent periods were differentiated by video observations. We evaluated the time of suckling in relation to the total time of pacifier use, the median number of bursts per min, the median duration of single bursts and the median interval between 2 sequent bursts. In 48 randomly selected bursts, we additionally analyzed changes in heart rate, respiratory frequency and oxygen saturation compared to the 10-second period preceding the burst., Results: Median sleep time with a pacifier held in mouth was 31.3 min (13.0-117.6), of which 15.5% (6.4-36.7%) was spent with active suckling. The median number of bursts per min was 2.2 (1.2-4.5). The median duration of a burst was 3 s (1-22) and the median interval between 2 bursts was 10 s (1-1,434). Heart rate, respiratory frequency and oxygen saturation did not change significantly during suckling bursts., Conclusions: This pilot study presents important data for sucking habits in pacifier users which may provide a basis for further investigations concerning the efficacy of pacifiers in SIDS prophylaxis., (Copyright 2009 S. Karger AG, Basel.)

Objective: It has been reported that pacifiers might reduce the risk of SIDS by favouring infants' arousability from sleep. We evaluated the influence of a pacifier on the frequency and duration of spontaneous arousals in healthy infants., Methods: Polygraphic recordings were performed in 14 infants with an age of 51.7+/-19.9 days (means+/-SD) who regularly used a pacifier during sleep. Cortical and subcortical arousals were scored according to the recommendations of the "International Paediatric Work Group on Arousals." The number of arousals per 10-min-period and the duration of arousals were determined for periods of pacifier use as well as for periods after pacifier dislodgement and were compared with the data of 10 control infants (age 49.8+/-16.5 days) who never used a pacifier., Results: Altogether, 211 arousals in pacifier users and 225 arousals in non-users were scored. In pacifier users, 2.0+/-1.6 arousals per 10-min-period with a duration of 12.2+/-3.0 s occurred during pacifier use, and 1.7+/-1.6 arousals per 10-min-period with a duration of 12.2+/-3.1s occurred during periods without pacifier. In pacifier non-users, 2.3+/-1.2 arousals per 10-min-period (duration 13.9+/-2.9s) were scored. The results did not show a significant difference concerning frequency and duration of spontaneous arousals between pacifier users and non-users., Conclusions: Our findings suggest that factors other than arousal mechanisms might be responsible for the efficacy of pacifiers in SIDS prophylaxis.

Aim: To find out the reasons for mothers to either use or not use a pacifier and to find out the mother's reasons for changing their mind., Method: We analysed the data of 174 mother-infant pairs by means of a semistructured questionnaire performed shortly after birth, and at the age of 7 weeks and 5 months, respectively., Results: The main reason for mothers primarily refusing a pacifier was their concern about malformation of the teeth or jaw. Until the end of the fifth month, 31% of the mothers changed their mind about pacifier use. Mothers primarily refusing a pacifier introduced a pacifier due to the need of soothing the infant. Mothers, who initially intended to use a pacifier, changed their mind due to rejection by the infant. The prevalence of pacifier use at the age of 5 months was 78%. The majority of mothers (69%) introduced the pacifier during the first week of life., Conclusion: About one third of mothers had changed their mind about pacifier use, either because of rejection by the infant or the need of settling. Breastfeeding mothers require more and better information concerning the appropriate time of pacifier onset.

The incidence of gastrointestinal stromal tumors (GISTs) in children is exceptionally low. However, during the last decade these tumors attracted increasing attention, because they were found to express the cell surface transmembrane receptor kit (CD117) that has tyrosine kinase activity. This tyrosine kinase can be semi-selectively inhibited by signal transduction inhibitors such as imatinib mesylate (Glivec), which is a competitive inhibitor of c-kit, c-abl, platelet-derived growth factor receptor-alpha (PDGFR-alpha) and PDGFR-beta, and abl-related gene (arg). The authors present the clinical, radiographic, and pathological findings of 4 children who were diagnosed with gastric GIST. One of them had an incomplete Carney triad including GIST and mediastinal paraganglioma. All 4 patients presented with anemia and anemia-related symptoms and underwent total resection of the tumor. One patient received additional chemotherapy (in the pre-imatinib era) and 2 patients received a short course of imatinib mesylate. With a follow-up of 116, 55, 23, and 10 months all patients are alive in first complete continuous remission. In children and adolescents, particularly in female patients, GISTs should be included in the differential diagnosis of anemia secondary to gastrointestinal hemorrhage. Complete surgical resection is the mainstay of treatment for this tumor, with imatinib mesylate restricted to patients with advanced or metastatic tumors. Since late recurrences (up to 30 years following initial diagnosis) are reported, a life-long follow-up is mandatory in these patients.

Aim: To evaluate whether bladder voiding in healthy infants is accompanied by body movements or any changes in heart rate (HR), respiratory frequency (RF) or electroencephalogram (EEG) frequency during sleep and during wakefulness., Methods: Polygraphic recordings were performed on 33 healthy infants (17 female) born at term. The infants' age at study entry was 41+/-10 d, and actual body weight was 4876+/-403 g (mean+/-SD). Bladder voiding was recorded by an adapted enuresis detector connected to the polygraphic computer unit., Results: Awakening was observed in 12 (36%) infants 77+/-9 s before bladder voiding. Twenty-one infants (64%) continued sleeping during bladder voiding. In sleeping infants, bladder voiding occurred during non-REM sleep only, and was accompanied by a cortical arousal. During wakefulness, RF was lower, and HR and EEG frequency were higher, but stayed constant during bladder voiding., Conclusion: Our observations demonstrate that bladder voiding in healthy infants during sleep is accompanied by body movements and changes in HR and EEG frequency, indicating cortical arousals, whereas during wakefulness these changes cannot be observed.

The aim of the study was to find out whether bladder voiding in healthy sleeping infants was accompanied by any arousal reaction. Polygraphic recordings were performed in 21 healthy infants (11 female) born at term. The infants' age at study entry was 42 +/- 4 days and actual body weight was 4852 +/- 689 g (mean +/- SD). Bladder voiding was recorded by an adapted enuresis detector which was connected to the polygraphic computer unit. Arousals were defined as suggested by the 'International Paediatric Work Group on Arousals'. Awakenings were excluded from the study. Bladder voiding was recorded at a mean time of 68 +/- 7 min after the infant had fallen asleep and occurred during quiet sleep (QS). Electroencephalogram frequency (P < 0.01) and heart rate (P < 0.05) were higher during the 5-s period before and after bladder voiding when compared with a 30-s interval before voiding. Furthermore, bladder voiding was accompanied by body movements in all infants. Respiratory frequency did not change significantly. We could demonstrate for the first time in sleeping infants, that bladder voiding during QS was accompanied by a cortical arousal.

The aim of this study was to investigate the effect of thyrotropin-releasing hormone (TRH) administration (standard TRH stimulation test) on plasma leptin levels in infants, children and adolescents. Plasma leptin levels were analyzed with regard to age, body mass index (BMI) and results in standard TRH stimulation test. The study population consisted of 79 infants, children and adolescents (age: 4.50 [0.04-20.49] years; BMI: 16.47 [12.46-38.32] kg/m2; BMI SDS: 0.21 [-2.97 to 3.26]) (median [range]). Plasma leptin levels significantly decreased 30 minutes after TRH administration (5 microg/kg; maximum 200 microg i.v.) (p <0.0001). No correlation was found in leptin decrease when comparing the different groups with regard to age, BMI and results in the TRH stimulation test. Positive correlation was found when comparing BMI SDS to plasma leptin levels before (r = 0.74; p <0.001) and 30 minutes after TRH injection (r = 0.73; p <0.001). There was poor correlation when age of patients was compared to plasma leptin levels before (r = 0.46; p <0.05) and 30 minutes after TRH injection (r = 0.47; p <0.05). In summary, we found that TRH administration decreases plasma leptin levels in infants and adolescents independent of age, BMI and thyroid function.

Objective: Sudden infant death syndrome (SIDS) remains a challenge for health professionals despite decreasing rates in recent years. The figures for different areas and time periods are hardly comparable, because of differences in postmortem investigations and classification criteria. In 1992, the European Society for the Study and Prevention of Infant Deaths (ESPID) proposed a classification for any sudden and unexpected death in infancy. This proposal has been used in our study since 1993 to better classify sudden infant death (SID) cases., Method: 56 consecutive SID cases observed between 1993 and 2002 in Styria, the south-eastern province of Austria, were analysed by a multidisciplinary team of health professionals. The study group consisted of pediatricians, forensic pathologists, pathologists, psychologists, nurses, members of the parents' association and health authorities. SID cases were analysed with regard to potential risk factors during pregnancy and early life, the circumstances of death (death scene) and post-mortem findings. From the latter, every SID was classified as either 1) classic SIDS, 2) borderline SIDS, 3) non-autopsied SID or 4) explained death., Results: Of the 56 SID cases, 22 were assigned to category 1, 19 to category 2, four to category 3, and in 11 cases death could be explained by major post-mortem findings. For 17/22 cases in category 1 and 11/19 cases in category 2, the death scene investigation showed the typical risk profile of manner of bedding and/or environmental conditions. In three cases, child abuse or infanticide was considered possible but could not be proven despite careful autopsy. In recent years, SIDS incidence in Styria has decreased to approximately 0.18/1,000 live-born infants, and the few deaths still occurring mainly present with the typical risk profile., Conclusion: An extensive analysis of SID events is a prerequisite for reliable and comparable SIDS statistics. Our data show that in several SID cases careful post-mortem examinations led to an explanation of death. In other cases, minor alterations may have contributed to the lethal event. These findings should therefore be considered in the classification of SIDs. The ESPID classification of 1992 appears to be very useful for this purpose and its use may therefore be recommended.