Creating a data dictionary for pediatric autonomic disorders.

PURPOSE: Whether evaluating patients clinically, documenting care in the electronic health record, performing research, or communicating with administrative agencies, the use of a common set of terms and definitions is vital to ensure appropriate use of language. At a 2017 meeting of the Pediatric Section of the American Autonomic Society, it was determined that an autonomic data dictionary comprising aspects of evaluation and management of pediatric patients with autonomic disorders would be an important resource for multiple stakeholders. METHODS: Our group created the list of terms for the dictionary. Definitions were prioritized to be obtained from established sources with which to harmonize. Some definitions needed mild modification from original sources. The next tier of sources included published consensus statements, followed by Internet sources. In the absence of appropriate sources, we created a definition. RESULTS: A total of 589 terms were listed and defined in the dictionary. Terms were organized by Signs/Symptoms, Triggers, Co-morbid Disorders, Family History, Medications, Medical Devices, Physical Examination Findings, Testing, and Diagnoses. CONCLUSION: Creation of this data dictionary becomes the foundation of future clinical care and investigative research in pediatric autonomic disorders, and can be used as a building block for a subsequent adult autonomic data dictionary.

Cardiorespiratory function after operation for pectus excavatum.

OBJECTIVE: We performed a review of current data to determine the effect that operation has on pulmonary function, aerobic capacity, and stroke volume in patients with pectus excavatum. STUDY DESIGN: Two reviewers independently assessed clinical trials and collected data on interventions and outcomes. To qualify for inclusion, a study had to include preoperative and postoperative assessment, provide outcomes in either a published percentile or qualified matched control form to control for interval growth, and include only original patient groups. RESULTS: Postoperative total lung capacity for patients who had Ravitch repair was significantly lower (SMD, 0.71 [CI -1.06, -0.36]; I(2) = 19.6%) than preoperative. Based on 2 studies after removal of the Nuss bar, FEV(1) was significantly increased from preoperative values (SMD, 0.39 [CI, 0.03, 0.74]; I(2) = 0%). Stroke volume increased after surgery (SMD, 0.40 [CI, 0.10, 0.70]; I(2) = 0%) after Ravitch repair. There was a trend toward improved exercise tolerance, but it was not statistically significant. CONCLUSIONS: Total lung capacity was decreased after Ravitch repair, and FEV(1) was increased after Nuss bar removal. Stroke volume may be increased after Ravitch repair. Exercise tolerance was not improved after either type of surgical repair.

Dalhousie dyspnea scales: construct and content validity of pictorial scales for measuring dyspnea.

BACKGROUND: Because there are no child-friendly, validated, self-report measures of dyspnea or breathlessness, we developed, and provided initial validation, of three, 7-item, pictorial scales depicting three sub-constructs of dyspnea: throat closing, chest tightness, and effort. METHODS: We developed the three scales (Throat closing, Chest tightness, and Effort) using focus groups with 25 children. Subsequently, seventy-nine children (29 children with asthma, 30 children with cystic fibrosis. and 20 children who were healthy) aged 6 to 18 years rated each picture in each series, using a 0-10 scale. In addition, each child placed each picture in each series on a 100-cm long Visual Analogue Scale, with the anchors "not at all" and "a lot". RESULTS: Children aged eight years or older rated the scales in the correct order 75% to 98% correctly, but children less than 8 years of age performed unreliably. The mean distance between each consecutive item in each pictorial scale was equal. CONCLUSION: Preliminary results revealed that children aged 8 to 18 years understood and used these three scales measuring throat closing, chest tightness, and effort appropriately. The scales appear to accurately measure the construct of breathlessness, at least at an interval level. Additional research applying these scales to clinical situations is warranted.

Measurement of exercise cardiac output by thoracic impedance in healthy children.

The purpose of this study was to track changes in stroke volume during exercise by impedance cardiography in order to validate the method, and to obtain such data in a large number of healthy children for reference purposes. One hundred and fifteen healthy children (aged 7-19 years) performed progressive exercise to voluntary exhaustion with work increments every minute on a cycle ergometer. Oxygen uptake (VO(2)) was measured on a breath-by-breath system. Cardiac output was measured with an ICG-M501 impedance cardiograph. Stroke volume was normalized for body surface area and expressed as stroke volume index. Cardiac output was regressed against VO(2), and differences between stroke volume index at rest and exercise were assessed by repeated measures analysis of variance. Cardiac output increased linearly with VO(2) in all subjects: individual slopes and intercepts averaged 5.16 (1.56) l.min(-1) per l.min(-1) VO(2), and 4.25 (1.92) l.min(-1), respectively [mean (SD)]. Stroke volume index rose by an average of 29% from rest to exercise, reaching a maximum of 52 ml.m(-2) in boys and girls. Most subjects demonstrated a continuous, gentle rise in stroke volume index with increasing work rate, though a minority demonstrated a falling index as work increased above the anaerobic threshold, despite rising cardiac output. Impedance cardiography accurately tracks cardiac output and can be a useful clinical and research tool in pediatric cardiology and exercise physiology.

Determinants of physical fitness in children with asthma.

RATIONALE: This study was designed to examine the relationships among weight, asthma severity, physical activity, and aerobic fitness in children with asthma. SUBJECTS AND METHODS: Sixty-four asthmatic children 8 to 12 years old (53% female) were assessed while attending a summer asthma camp. Measures included height and weight, spirometry, histamine bronchial provocation challenge, maximal aerobic power, and questionnaires to quantify habitual activity, perceived activity limitations due to asthma, perceived competence in physical activity, and attitudes toward physical activity. Asthma severity was determined from spirometric indices (forced expiratory volume during the first second), degree of airway hyperresponsiveness, and amount of medication prescribed. RESULTS: There was no correlation between asthma severity and aerobic fitness. Only perceived competence at physical activity was found to have a significant correlation with aerobic fitness. Appropriate-weight, overweight, or obese (defined by body mass index) children all had similar results for maximum aerobic power and level of habitual activity. However, overweight or obese children reported greater limitation of physical activity. Their asthma-impairment scores were higher than the scores of appropriate-weight peers, although standard measures of pulmonary function were no different among groups. The higher asthma-severity scores were related to greater medication needs in the overweight or obese children with asthma. CONCLUSIONS: Lower maximum aerobic power in asthmatic children is related more to how capable they perceive themselves than to asthma severity. Overweight asthmatic children experience greater limitation of physical activity and thus are prescribed more medication, although by standard measures of asthma severity, they are very similar to normal-weight peers with asthma. Efforts should be directed at understanding the reasons responsible for reduced exercise tolerance before escalating pharmacologic treatment.