Autonomic neurophysiologic implications of disorders comorbid with bladder pain syndrome vs myofascial pelvic pain.

AIMS: The neuropathophysiology of a debilitating chronic urologic pain condition, bladder pain syndrome (BPS), remains unknown. Our recent data suggests withdrawal of cardiovagal modulation in subjects with BPS, in contrast to sympathetic nervous system dysfunction in another chronic pelvic pain syndrome, myofascial pelvic pain (MPP). We evaluated whether comorbid disorders differentially associated with BPS vs MPP shed additional light on these autonomic differences. METHODS: We compared the presence and relative time of onset of 27 other medical conditions in women with BPS, MPP, both syndromes, and healthy subjects. Analysis included an adjustment for multiple comparisons. RESULTS: Among 107 female subjects (BPS alone = 32; BPS with MPP = 36; MPP alone = 9; healthy controls = 30), comorbidities differentially associated with BPS included irritable bowel syndrome (IBS), dyspepsia, and chronic nausea, whereas those associated with MPP included migraine headache and dyspepsia, consistent with the distinct autonomic neurophysiologic signatures of the two disorders. PTSD (earliest), anxiety, depression, migraine headache, fibromyalgia, chronic fatigue, and IBS usually preceded BPS or MPP. PTSD and the presence of both pelvic pain disorders in the same subject correlated with significantly increased comorbid burden. CONCLUSIONS: Our study suggests a distinct pattern of comorbid conditions in women with BPS. These findings further support our hypothesis of primary vagal defect in BPS as compared with primary sympathetic defect in MPP, suggesting a new model for chronic these pelvic pain syndromes. Chronologically, PTSD, migraine, dysmenorrhea, and IBS occurred early, supporting a role for PTSD or its trigger in the pathophysiology of chronic pelvic pain.

North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome.

Cyclic vomiting syndrome (CVS) is a disorder noted for its unique intensity of vomiting, repeated emergency department visits and hospitalizations, and reduced quality of life. It is often misdiagnosed due to the unappreciated pattern of recurrence and lack of confirmatory testing. Because no accepted approach to management has been established, the task force was charged to develop a report on diagnosis and treatment of CVS based upon a review of the medical literature and expert opinion. The key issues addressed were the diagnostic criteria, the appropriate evaluation, the prophylactic therapy, and the therapy of acute attacks. The recommended diagnostic approach is to avoid "shotgun" testing and instead to use a strategy of targeted testing that varies with the presence of 4 red flags: abdominal signs (eg, bilious vomiting, tenderness), triggering events (eg, fasting, high protein meal), abnormal neurological examination (eg, altered mental status, papilledema), and progressive worsening or a changing pattern of vomiting episodes. Therapeutic recommendations include lifestyle changes, prophylactic therapy (eg, cyproheptadine in children 5 years or younger and amitriptyline for those older than 5), and acute therapy (eg, 5-hydroxytryptamine receptor agonists, termed triptans herein, as abortive therapy, and 10% dextrose and ondansetron for those requiring intravenous hydration). This document represents the official recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition for the diagnosis and treatment of CVS in children and adolescents.

Near-infrared spectroscopy muscle oximetry of patients with postural orthostatic tachycardia syndrome.

Postural orthostatic tachycardia syndrome (POTS) is a disabling condition characterized by orthostatic intolerance with tachycardia in the absence of drop-in blood pressure. A custom-built near-infrared spectroscopy device (NIRS) is applied to monitor the muscle oxygenation, noninvasively in patients undergoing incremental head-up tilt table (HUT). Subjects (6 POTS patients and 6 healthy controls) underwent 30 mins of 70°on a HUT. The results showed a significant difference in deoxyhemoglobin (Hb), change-in-oxygenation (ΔOxy) and blood volume (ΔBV) between patients and healthy controls. However, oxyhemoglobin (HbO2) showed a significantly faster rate of change in the healthy controls during the first 10 mins of the tilt and during the recovery. This NIRS muscle oximetry tool provides quantitative measurements of blood oxygenation monitoring in diseases such as POTS.

Autonomic abnormalities in cyclic vomiting syndrome.

BACKGROUND: Although cyclic vomiting syndrome (CVS) is associated with migraine, and migraine in turn is associated with orthostatic tachycardia, few studies have explored the association of CVS and autonomic dysfunction. We describe the results of autonomic testing in 6 children with characteristic CVS. PATIENTS AND METHODS: All patients fully met the established criteria for the diagnosis of CVS, were well hydrated, and were beyond their episode of vomiting. We performed 3 tests of cardiovascular function and 1 sudomotor test, using standard previously published methods. RESULTS: The findings were surprisingly uniform, with normal cardiovascular responses to deep breathing and to the Valsalva maneuver in all patients, a significant increase in heart rate (>30 beats per minute) with tilt testing, and a vasodepressor tendency in 2 patients. Interestingly, abdominal pain occurred at blood pressure nadir in both these patients and in a third patient without the vasodepressor findings but who described syncope clinically. Sudomotor test results were abnormal in all 6 patients, with reduced responses in 5 of 6 and exaggerated responses in the 6th. All 6 patients reported a personal or family history of migraine headaches. CONCLUSIONS: CVS is associated with remarkably uniform primarily sympathetic autonomic dysfunction, affecting mainly the vasomotor and sudomotor systems, and compatible with an underlying autonomic neuropathy. The occurrence of symptoms during tilt testing in half the patients suggests that these findings may play a true pathophysiologic role. A vagally modulated sympathetic effect is postulated as the best mechanistic model to account for all current physiologic data on cyclic vomiting and gastroparesis.

Pediatric autonomic disorders.

The scope of pediatric autonomic disorders is not well recognized. The goal of this review is to increase awareness of the expanding spectrum of pediatric autonomic disorders by providing an overview of the autonomic nervous system, including the roles of its various components and its pervasive influence, as well as its intimate relationship with sensory function. To illustrate further the breadth and complexities of autonomic dysfunction, some pediatric disorders are described, concentrating on those that present at birth or appear in early childhood.

Endoscopic retrograde cholangiopancreatography in children and adolescents.

OBJECTIVES: Endoscopic retrograde cholangiopancreatography (ERCP) is becoming a more frequently used diagnostic and therapeutic tool in children. We sought to determine the indications, feasibility, safety, and effect on patient management of ERCP in pediatric patients of varying age. METHODS: All ERCPs performed during a 4-year period in patients aged 18 years or less at an academic hospital were retrospectively reviewed. The indications, type of anesthesia administered, type of duodenoscope used, diagnostic findings, therapeutic interventions, complication rate, and effect on management were compared between children (age 0-12 years) and adolescents (age 13-18 years). RESULTS: A total of 53 procedures were performed in 43 patients whose median age was 13.5 years. ERCP was successful in 50 of 53 cases (94%) with a complication rate of 6%. Endoscopic therapy was provided in 24 of 53 cases (45%). Compared with adolescents (n = 28), children (n = 25) were more likely to receive general anesthesia (96% vs. 29%; P < 0.001) and undergo ERCP with a pediatric duodenoscope (0% vs. 40%). ERCP affected management in 73% of cases, equally in both groups. CONCLUSION: ERCP is a successful and safe diagnostic and therapeutic modality in a variety of pancreatobiliary disorders that directly affects management in children of all ages.