Long-Standing Chronic Recurrent Pericarditis Managed With Advanced Diagnostic and Therapeutic Methods.

A 65-year-old woman with a history of idiopathic pericarditis presented with chronic recurrent pericarditis. Because of the inability to taper off anakinra without recurrent flares, she transitioned to rilonacept, which led to symptom abatement. Her positive response to rilonacept therapy correlated with an improvement in inflammatory changes noted on cardiac magnetic resonance imaging.

High flow variant postural orthostatic tachycardia syndrome amplifies the cardiac output response to exercise in adolescents.

Postural orthostatic tachycardia syndrome (POTS) is characterized by chronic fatigue and dizziness and affected individuals by definition have orthostatic intolerance and tachycardia. There is considerable overlap of symptoms in patients with POTS and chronic fatigue syndrome (CFS), prompting speculation that POTS is akin to a deconditioned state. We previously showed that adolescents with postural orthostatic tachycardia syndrome (POTS) have excessive heart rate (HR) during, and slower HR recovery after, exercise - hallmarks of deconditioning. We also noted exaggerated cardiac output during exercise which led us to hypothesize that tachycardia could be a manifestation of a high output state rather than a consequence of deconditioning. We audited records of adolescents presenting with long-standing history of any mix of fatigue, dizziness, nausea, who underwent both head-up tilt table test and maximal exercise testing with measurement of cardiac output at rest plus 2-3 levels of exercise, and determined the cardiac output () versus oxygen uptake () relationship. Subjects with chronic fatigue were diagnosed with POTS if their HR rose ≥40 beat·min(-1) with head-up tilt. Among 107 POTS patients the distribution of slopes for the , relationship was skewed toward higher slopes but showed two peaks with a split at ~7.0 L·min(-1) per L·min(-1), designated as normal (5.08 ± 1.17, N = 66) and hyperkinetic (8.99 ± 1.31, N = 41) subgroups. In contrast, cardiac output rose appropriately with in 141 patients with chronic fatigue but without POTS, exhibiting a normal distribution and an average slope of 6.10 ± 2.09 L·min(-1) per L·min(-1). Mean arterial blood pressure and pulse pressure from rest to exercise rose similarly in both groups. We conclude that 40% of POTS adolescents demonstrate a hyperkinetic circulation during exercise. We attribute this to failure of normal regional vasoconstriction during exercise, such that patients must increase flow through an inappropriately vasodilated systemic circulation to maintain perfusion pressure.

Predictors of resident satisfaction in caring for limited English proficient families: a multisite study.

OBJECTIVE: To assess residents' self-efficacy, satisfaction and frustration in the care of limited English proficient (LEP) families and to identify individual and programmatic factors associated with the above outcomes. METHODS: A multisite cross-sectional survey of pediatric residents currently in training and caring for LEP families was conducted. Resident self-efficacy scores in specific skill domains were assessed. Clustered multivariate logistic regression analyses were used to identify individual and programmatic factors associated with increased satisfaction and frustration. Qualitative analyses were also utilized to identify themes related to frustrating aspects of care. RESULTS: A total of 271 of 449 eligible residents representing 7 US pediatric residency programs participated in our study (60% response rate). A majority of residents (51%) rated their self-efficacy in the overall care of LEP families as low. Satisfaction was associated with a high self-efficacy score (odds ratio [OR] 4.7, 95% confidence interval [CI] 1.8-12.6), increasing year in training (OR 3.2, 95% CI 1.01-10.2), frequent non-English language use (OR 3.1, 95% CI 1.1-8.8), and instruction on the use of interpreters (OR 1.9, 95% CI 1.02-3.64). Satisfaction was inversely associated with increased LEP patient volumes (OR 0.40, 95% CI 0.18-0.91). Clinical inefficiency related to interpreter use and distrust of interpreter skills were frequently cited as aspects that contribute to overall frustration. CONCLUSIONS: A majority of residents reported lacking self-efficacy in their ability to deliver care to LEP patients, which may influence overall satisfaction with such encounters. Strategies that promote resident self-efficacy and assess non-English language proficiency should be included in future training curricula. Exposing trainees to best practices in interpreter-based encounters may further promote resident satisfaction.

Pseudohypoaldosteronism presenting with thrombocytosis and bilateral pneumothoraces in an infant.

Pseudohypoaldosteronism type 1 (PHA-1) is a rare salt-wasting syndrome caused by a peripheral resistance to aldosterone. Here, we describe an unusual presentation of the autosomal dominant PHA-1 featuring bilateral pneumothoraces at birth, thrombocytosis in infancy, and hypercalcemia in addition to the well-described findings of hyponatremia, hyperkalemia, and failure to thrive. These findings contribute to the limited case descriptions of PHA-1 and may suggest additional diagnostic considerations in a neonate presenting with hyperkalemia, hyponatremia, and failure to thrive.

Complete self-sufficiency planning: designing and building disaster-ready hospitals.

OBJECTIVES: The need for healthcare systems and academic medical centers to be optimally prepared in the event of a disaster is well documented. Events such as Hurricane Katrina demonstrate a major gap in disaster preparedness for at-risk medical institutions. To address this gap, we outline the components of complete self-sufficiency planning in designing and building hospitals that will function at full operational capacity in the event of a disaster. We review the processes used and outcomes achieved in building a new critical access, freestanding children's hospital in Florida. METHODS: Given that hurricanes are the most frequently occurring natural disaster in Florida, the executive leadership of our hospital determined that we should be prepared for worst-case scenarios in the design and construction of a new hospital. A comprehensive vulnerability assessment was performed. A building planning process that engaged all of the stakeholders was used during the planning and design phases. Subsequent executive-level review and discussions determined that a disaster would require the services of a fully functional hospital. Lessons learned from our own institution's previous experiences and those of medical centers involved in the Hurricane Katrina disaster were informative and incorporated into an innovative set of hospital design elements used for construction of a new hospital with full operational capacity in a disaster. RESULTS: A freestanding children's hospital was constructed using a new framework for disaster planning and preparedness that we have termed complete self-sufficiency planning. CONCLUSIONS: We propose the use of complete self-sufficiency planning as a best practice for disaster preparedness in the design and construction of new hospital facilities.

Postural tachycardia in children and adolescents: what is abnormal?

OBJECTIVES: To evaluate whether the use of adult heart rate (HR) criteria is appropriate for diagnosing orthostatic intolerance (OI) and postural tachycardia syndrome (POTS) in children and adolescents, and to establish normative data and diagnostic criteria for pediatric OI and POTS. STUDY DESIGN: A total of 106 normal controls aged 8-19 years (mean age, 14.5±3.3 years) underwent standardized autonomic testing, including 5 minutes of 70-degree head-up tilt. The orthostatic HR increment and absolute orthostatic HR were assessed and retrospectively compared with values in 654 pediatric patients of similar age (mean age, 15.5±2.3 years) who were referred to our Clinical Autonomic Laboratory with symptoms of OI. RESULTS: The HR increment was mildly higher in patients referred for OI/POTS, but there was considerable overlap between the patient and control groups. Some 42% of the normal controls had an HR increment of ≥30 beats per minute. The 95th percentile for the orthostatic HR increment in the normal controls was 42.9 beats per minute. There was a greater and more consistent difference in absolute orthostatic HR between the 2 groups, although there was still considerable overlap. CONCLUSION: The diagnostic criteria for OI and POTS in adults are unsuitable for children and adolescents. Based on our normative data, we propose new criteria for the diagnosis of OI and POTS in children and adolescents.

Iron insufficiency and hypovitaminosis D in adolescents with chronic fatigue and orthostatic intolerance.

OBJECTIVES: More than 10% of adolescents suffer from severe fatigue and/or orthostatic intolerance. Adult studies show correlations between iron insufficiency and fatigue as well as between hypovitaminosis D and non-specific pain. We sought to determine whether there were correlations between nutritional factors (iron status, and serum vitamin D levels) and chronic ill health. METHODS: We reviewed records of 188 adolescents with symptoms of fatigue and/or orthostatic intolerance and who underwent autonomic reflex screening. RESULTS: Of the 188 patients, 130 patients (69%) had excessive postural tachycardia (PT) with a heart rate (HR) change of ≥30 bpm. 62 patients (47%, n = 131) had iron insufficiency with low iron stores, and 29 patients (22%, n = 131) were iron deficient. HR change did not correlate to ferritin level (P = 0.15). 21 patients (22%, n = 95) had hypovitaminosis D (25-hydroxyvitamin D ≤20 ng/mL). There was a significant association with hypovitaminosis D and orthostatic intolerance (P = 0.024). CONCLUSION: In patients presenting with chronic fatigue and/or orthostatic intolerance, low ferritin levels and hypovitaminosis D are common, especially in patients with PT.

Empiric treatment of protracted idiopathic purpura fulminans in an infant: a case report and review of the literature.

INTRODUCTION: Idiopathic purpura fulminans is a cutaneous thrombotic disorder usually caused by autoimmune-mediated protein C or S deficiency. This disorder typically presents with purpura and petechiae that eventually slowly or rapidly coalesce into extensive, necrotic eschars on the extremities. We present the first known case of idiopathic purpura fulminans consistent with prior clinical presentations in the setting of a prothrombotic genetic mutation, but without hallmark biochemical evidence of protein C or protein S deficiency. Another novel feature of our patient's presentation is that discontinuation of anti-coagulation has invariably led to recurrence and formation of new lesions, which is unexpected in idiopathic purpura fulminans because clearance of autoimmune factors should be followed by restoration of anti-coagulant function. Although this disease is rare, infants with suspected idiopathic purpura fulminans should be rapidly diagnosed and immediately anti-coagulated to prevent adverse catastrophic outcomes such as amputation and significant developmental delay. CASE PRESENTATION: A six-month-old Caucasian boy was brought to our pediatric hospital service with a low-grade fever and subacute, symmetric, serpiginous, stellate, necrotic eschars on his forearms, legs and feet that eventually spread non-contiguously to his toes, thighs and buttocks. In contrast to his impressive clinical presentation, his serologic evaluation was normal, and he was not responsive to corticosteroids and antibiotics. Full-thickness skin biopsies revealed dermal vessel thrombosis, leading to a diagnosis of idiopathic purpura fulminans and successful treatment with low-molecular-weight heparin, which was transitioned to warfarin. Long-term management has included chronic anti-coagulation because of recurrence of lesions with discontinuation of treatment. CONCLUSION: In infants with necrotic eschars, it is important to first consider infectious, inflammatory and hematologic etiologies. In the absence of etiology for protracted idiopathic purpura fulminans, management should include tissue biopsy, in which thrombotic findings warrant a trial of empiric anti-coagulation. Some infants, including our patient, may need long-term anti-coagulation, especially when the underlying etiology of coagulation remains unidentified and symptoms recur when treatment is halted. Given that our patient still requires anti-coagulation, he may have a yet to be identified autoimmune-mediated mechanism for his truly idiopathic case of protracted purpura fulminans.

Functional disability in adolescents with orthostatic intolerance and chronic pain.

A retrospective review identified 99 adolescents (79% female) referred to a tertiary care center to evaluate the relationship between symptoms of orthostatic intolerance and chronic pain. Regression analysis indicated that functional disability was strongly associated with pain intensity (P < .001) and depression (P = .024). The association between functional disability and number of symptoms of orthostatic intolerance trended toward significance (P = .057). Meeting a threshold heart rate increment of 30 beats per minute on head-up tilt was not associated with functional disability (P = .188). Separate regression analysis of female patients showed similar results to the full sample but with a stronger relationship between depression and functional disability and a weaker relationship between heart increment and functional disability. In this sample of adolescents with symptoms of orthostatic intolerance and chronic pain, pain intensity and depression were associated with functional status, but postural tachycardia was not. Further research is needed to clarify potential gender differences.

Exercise performance in adolescents with autonomic dysfunction.

OBJECTIVE: To test the hypothesis that excessive postural tachycardia is associated with deconditioning rather than merely being an independent sign of autonomic dysfunction in patients with postural orthostatic tachycardia syndrome (POTS). STUDY DESIGN: We retrospectively analyzed records from 202 adolescents who underwent both head up-tilt and maximal exercise testing. Patients were classified as POTS if they had ≥ 30 min(-1) rise in heart rate (HR) after tilt-table test; and deconditioned if peak O(2) uptake was < 80% predicted. Changes in HR during exercise and recovery were compared between groups. RESULTS: Two-thirds of patients were deconditioned, irrespective of whether they fulfilled diagnostic criteria for POTS, but peak O(2) uptake among patients with POTS was similar to patients without POTS. HR was higher at rest and during exercise; whereas stroke volume was lower during exercise, and HR recovery was slower in patients with POTS compared with patients without POTS. CONCLUSIONS: Most patients who presented with chronic symptoms of dizziness, fatigue, or pre-syncope, were deconditioned, but, because the proportion of deconditioned patients was similar in POTS vs non-POTS groups, we conclude that HR changes in POTS are not solely because of inactivity resulting in deconditioning.

Orthostatic heart rate and blood pressure in adolescents: reference ranges.

This descriptive population study of 307 public high school students, ages 15 to 17 years, was performed to establish reference ranges for orthostatic changes in heart rate and blood pressure in adolescents, and to identify influential variables. Noninvasive measurements of blood pressure and heart rate were obtained. Reference ranges for orthostatic heart rate change in this population at 2 minutes were -2 to +41 beats per minute and at 5 minutes were -1 to +48 beats per minute. Orthostatic blood pressure changes were within the adult range for 98% of adolescents tested. One-third of participants experienced orthostatic symptoms during testing. In conclusion, this study shows that orthostatic symptoms and large orthostatic heart rate changes occur in adolescents. This suggests that the current orthostatic heart rate criterion aiding the diagnosis of adult orthostatic intolerance syndromes is likely not appropriate for adolescents and should be reevaluated.

Postural orthostatic tachycardia syndrome: a clinical review.

Postural orthostatic tachycardia syndrome was defined in adult patients as an increase >30 beats per minute in heart rate of a symptomatic patient when moving from supine to upright position. Clinical signs may include postural tachycardia, headache, abdominal discomfort, dizziness/presyncope, nausea, and fatigue. The most common adolescent presentation involves teenagers within 1-3 years of their growth spurt who, after a period of inactivity from illness or injury, cannot return to normal activity levels because of symptoms induced by upright posture. Postural orthostatic tachycardia syndrome is complex and likely has numerous, concurrent pathophysiologic etiologies, presenting along a wide spectrum of potential symptoms. Nonpharmacologic treatment includes (1) increasing aerobic exercise, (2) lower-extremity strengthening, (3) increasing fluid/salt intake, (4) psychophysiologic training for management of pain/anxiety, and (5) family education. Pharmacologic treatment is recommended on a case-by-case basis, and can include beta-blocking agents to blunt orthostatic increases in heart rate, alpha-adrenergic agents to increase peripheral vascular resistance, mineralocorticoid agents to increase blood volume, and serotonin reuptake inhibitors. An interdisciplinary research approach may determine mechanistic root causes of symptoms, and is investigating novel management plans for affected patients.

Management of knee arthropathy in patients with vascular malformations.

OBJECTIVE: To describe our experience with surgical intervention for symptomatic intraarticular vascular malformations of the knee in patients with peripheral vascular malformations including Klippel-Trénaunay syndrome (KTS). METHODS: Eleven patients underwent surgical intervention for symptomatic intraarticular vascular malformations of the knee between 1987 and 2008. Seven patients had KTS, and 4 patients had venous malformations. Surgical indications, imaging studies, clinical course, surgical procedures, complications, and follow-up visits were reviewed and recorded. RESULTS: A total of 11 patients (8 males; 3 females; mean age, 11.7 years; range, 2.5-23 years) underwent 12 surgical procedures. Five patients had an amputation, and 6 patients had knee synovectomies. One patient had bilateral knee synovectomies. Surgical indications included pain, swelling, limited mobility, and/or loss of knee motion. The average time of follow-up was 54 months (range, 7-109 months). Patient-reported pain scores decreased significantly from a mean of 2.9 +/- 1.4 preoperatively to 1.3 +/- 0.9 postoperatively (P = 0.01). CONCLUSIONS: When necessary, surgical intervention for intraarticular vascular malformations of the knee (amputation or synovectomy) may be effective in decreasing pain and improving mobility in patients with peripheral vascular malformations. It is possible that early synovectomy may slow or prevent the rapid destructive arthritis that occurs in these knees. Surgeons and patients should anticipate complications related to bleeding from vascular malformations. We recommend a multidisciplinary approach to the patient with KTS, particularly when surgical intervention is indicated.

Outcomes in adolescents with postural orthostatic tachycardia syndrome treated with midodrine and beta-blockers.

BACKGROUND: Postural orthostatic tachycardia syndrome (POTS) is associated with debilitating fatigue, dizziness, and discomfort in previously healthy adolescents. The effects of medical therapy have not been well studied in this patient population. This study assessed the relative efficacy and impact of drug therapy on the functioning and quality of life in adolescents with POTS. METHODS: A retrospective, single center, chart review analysis with a follow-up written survey was conducted on a group of 121 adolescents who had undergone autonomic reflex screening at the Mayo Clinic from 2002 to 2005 as part of an evaluation for possible POTS. RESULTS: Of 121 surveys sent, 47 adolescents returned a completed survey. In this cohort of patients, the two most commonly prescribed drug therapies were midodrine (n = 13) and beta-blockers (n = 14). Patients in the midodrine group were comparable to patients in the beta-blocker group in gender, age, pretreatment postural heart rate changes, and months from initial evaluation to survey completion. More patients treated with a beta-blocker reported improvement after visiting Mayo Clinic (100% vs 62%, P = 0.016) and more attributed their progress to medication (63.6% vs 36.4%, P = 0.011) than did those treated with midodrine. CONCLUSION: Treatment with both midodrine and beta-blockers was associated with overall improvement in POTS patients' general health; however, adolescents taking beta-blockers were more likely than those taking midodrine to credit the role of medications in their improvement.

Orthostatic intolerance and gastrointestinal motility in adolescents with nausea and abdominal pain.

OBJECTIVE: To describe the relationships between gastric emptying, autonomic function, and postural tachycardia in adolescent patients with nausea and/or abdominal discomfort. It was hypothesized that patients with both gastrointestinal symptoms and symptoms of orthostatic intolerance are more likely to show abnormal tilt table results and delayed gastric emptying. PATIENTS AND METHODS: A retrospective review was conducted of adolescent patients who came to a pediatric referral center because of nausea and dyspepsia and who subsequently underwent both autonomic reflex screening and gastric emptying testing. Patients with a heart rate change of 30 or more beats per minute on the heads-up tilt table test were assigned to the postural orthostatic tachycardia syndrome (POTS) group (n = 21), and those with a heart rate change of fewer than 30 beats per minute on the heads-up tilt table test were assigned to the non-POTS group (n = 10). RESULTS: There was no significant difference between the POTS and non-POTS groups with regard to presenting symptoms (P > 0.05). Overall, 13 (42%) individuals had abnormal gastric emptying results (delayed in 6, accelerated in 7), but gastric emptying scores were similar between the POTS and non-POTS groups. Furthermore, there was no correlation between an individual's gastric emptying results at 1, 2, and 4 hours and that person's heart rate change on HUT (r = -0.05, -0.15, and -0.19). CONCLUSIONS: Although altered gastric emptying and postural tachycardia are common in a referral population of adolescents with nausea and/or abdominal discomfort, the clinical presentation was not predictive of test results. Furthermore, delayed gastric emptying was not correlated with the current definition of postural tachycardia.