Assessment of comorbid symptoms in pediatric autonomic dysfunction.

PURPOSE: Pediatric patients with autonomic dysfunction and orthostatic intolerance (OI) often present with co-existing symptoms and signs that might or might not directly relate to the autonomic nervous system. Our objective was to identify validated screening instruments to characterize these comorbidities and their impact on youth functioning. METHODS: The Pediatric Assembly of the American Autonomic Society reviewed the current state of practice for identifying symptom comorbidities in youth with OI. The assembly includes physicians, physician-scientists, scientists, advanced practice providers, psychologists, and a statistician with expertise in pediatric disorders of OI. A total of 26 representatives from the various specialties engaged in iterative meetings to: (1) identify and then develop consensus on the symptoms to be assessed, (2) establish committees to review the literature for screening measures by member expertise, and (3) delineate the specific criteria for systematically evaluating the measures and for making measure recommendations by symptom domains. RESULTS: We review the measures evaluated and recommend one measure per system/concern so that assessment results from unrelated clinical centers are comparable. We have created a repository to apprise investigators of validated, vetted assessment tools to enhance comparisons across cohorts of youth with autonomic dysfunction and OI. CONCLUSION: This effort can facilitate collaboration among clinical settings to advance the science and clinical treatment of these youth. This effort is essential to improving management of these vulnerable patients as well as to comparing research findings from different centers.

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.

Outcomes of Adolescent-Onset Postural Orthostatic Tachycardia Syndrome.

OBJECTIVES: To determine the clinical course of adolescent-onset postural orthostatic tachycardia syndrome (POTS) and to assess health-related quality of life, 2-10 years after diagnosis. STUDY DESIGN: Pediatric patients, 13-18 years of age, diagnosed with POTS at Mayo Clinic, Rochester, from 2003 to 2010 were mailed a questionnaire if they were at least 18 years of age at the time of the mailing. The primary outcome measures were norm-based, age- and sex-adjusted, 36-Item Short Form Health Survey physical composite score and mental composite score. RESULTS: The survey was mailed to 502 patients with a response rate of 34% (n = 172). The mean duration from diagnosis to survey completion was 5.4 (SD, 1.9) years; the mean age of the respondents at the time of the survey was 21.8 (2.2) years. The responders were predominantly females (84% vs 68% of nonresponders; P < .001). Only 33 (19%) respondents reported complete resolution of symptoms, and an additional 51% reported persistent but improved symptoms, and 28 (16%) had only intermittent symptoms. The majority (71%) consider their health at least "good." The mean physical composite score was significantly lower than the population norm (mean [SD], 36.6 [15.8] vs 50; P < .001), however, the corresponding mean mental composite score was normal (50.1 [11.2]). CONCLUSIONS: Overall, 86% of adolescents with POTS report resolved, improved, or just intermittent symptoms, when assessed via questionnaire at an average of 5 years after initial treatment. Patients with persistent symptoms have more physical than mental health concerns.

Adolescent fatigue, POTS, and recovery: a guide for clinicians.

Many teenagers who struggle with chronic fatigue have symptoms suggestive of autonomic dysfunction that may include lightheadedness, headaches, palpitations, nausea, and abdominal pain. Inadequate sleep habits and psychological conditions can contribute to fatigue, as can concurrent medical conditions. One type of autonomic dysfunction, postural orthostatic tachycardia syndrome, is increasingly being identified in adolescents with its constellation of fatigue, orthostatic intolerance, and excessive postural tachycardia (more than 40 beats/min). A family-based approach to care with support from a multidisciplinary team can diagnose, treat, educate, and encourage patients. Full recovery is possible with multi-faceted treatment. The daily treatment plan should consist of increased fluid and salt intake, aerobic exercise, and regular sleep and meal schedules; some medications can be helpful. Psychological support is critical and often includes biobehavioral strategies and cognitive-behavioral therapy to help with symptom management. More intensive recovery plans can be implemented when necessary.