The Role of Patient and Parental Resilience in Adolescents with Chronic Musculoskeletal Pain.

OBJECTIVES: To assess the level of resilience among patients with chronic musculoskeletal pain and their parents and to determine factors associated with patient and parental resilience. STUDY DESIGN: Cross-sectional cohort study of children aged 13-17 years diagnosed with chronic musculoskeletal pain and their parents. Patient-parent pairs were seen for initial consultation in the pediatric rheumatology pain clinic at Children's Hospital of Philadelphia between March and May 2018 and were administered a series of questionnaires including measures of resilience (Connor-Davidson Resilience Scale 10 item, The 14-item Resilience Scale, and the 7Cs of Resilience Tool). We calculated Pearson correlation coefficients to examine the relationship between the variables of interest and resilience. RESULTS: According to all resilience measures, patients and parents had low to moderate levels of resilience. These levels were lower than those previously reported among healthy populations, as well as those with chronic medical conditions. According to the Connor-Davidson Resilience Scale 10 item, patient-level resilience was negatively correlated with pain level (r = -0.48), physical disability (r = -0.54), and symptom severity (r = -0.53). The level of resilience among patients was positively correlated with energy level (r = 0.57) and health-related quality of life (r = 0.64). Parental resilience was positively correlated with parental mental health (r = 0.61). CONCLUSIONS: Higher patient resilience was correlated with reduced disease severity among adolescents with chronic musculoskeletal pain. Future research should explore whether fostering resilience in adolescents with chronic musculoskeletal pain via the application of resilience-training interventions mitigates disease burden in this vulnerable patient population.

Use of Rituximab and Risk of Re-hospitalization for Children with Neuromyelitis Optica Spectrum Disorder.

BACKGROUND: Treatment algorithms for neuromyelitis optica spectrum disorder (NMOSD) vary, and sparse data exist regarding the impact of initial treatments on disease course. We aimed to determine whether administration of rituximab during first hospitalization reduces 1-year readmission rates. METHODS: We conducted a retrospective cohort study of subjects with NMOSD using the Pediatric Health Information System database from 2005-2015. Subjects were ages 1 to 21 years who received glucocorticoids and an ICD-9-CM code indicating neuromyelitis optica (NMO) during first hospitalization. All subjects had at least 12 months of continuous enrollment. The primary exposure was ≥1 rituximab dose during first hospitalization. We tested for the association of rituximab use with all-cause re-hospitalization, the primary outcome, using survival analysis. Re-hospitalization was considered if a hospital admission occurred > 30 days after initial discharge with exclusion of admissions with re-dosing of rituximab and data were censored at 12 months. Secondary outcomes included time to and median duration of re-hospitalization using 25th percentiles of survival time and the Wilcoxon-rank sum test, respectively. RESULTS: Of 180 subjects who met inclusion criteria, 71.7% were female and the median age was 13 years (IQR: 10, 15). 52 subjects (28.9%) received rituximab during first hospitalization, and there was an increasing trend in rituximab use over time (p<0.01). Overall, 36.7% of children were readmitted and time to readmission was a median of 365 days (IQR: 138, 365). Rituximab exposure was not associated with re-hospitalization (adjusted HR: 0.71: 95% CI: 0.38, 1.34) nor a reduced time to re-hospitalization. Median duration of re-hospitalization was 2 days shorter in the rituximab exposed group (p=0.02). Receipt of physical therapy, a surrogate marker for neurologic impairment, during first hospitalization was associated with re-admission within 12 months (adjusted HR: 4.81; 95% CI: 1.14, 20.29). CONCLUSIONS: Among children with NMOSD, first-line administration of rituximab was not associated with risk of or time to re-hospitalization. Rituximab use was found to be associated with a shorter duration of re-hospitalization. Need for physical therapy during first hospitalization was independently associated with an increased risk of re-admission.

BIRC4 Mutation: An Important Rare Cause of Uveitis.

We report a 6-year-old man with chronic severe recalcitrant bilateral anterior uveitis and a remote history of hemophagocytic lymphocytic histiocytosis secondary to Epstein-Barr virus infection. The patient was treated for idiopathic uveitis after an initial extensive evaluation failed to reveal a specific diagnosis. The patient failed to achieve sustained inactive disease with multiple monotherapies including topical glucocorticoid, methotrexate, infliximab, mycophenolate mofeti, and cyclosporine. Disease control was finally attained with a combination of cyclosporine and adalimumab. After more recent testing, he was found to have a novel deletion on the BIRC4 (baclovirus inhibitor of apoptosis repeat containing protein 4) gene, the causative gene for X-linked lymphoproliferative syndrome type 2. We conclude that male patients with chronic idiopathic uveitis should be questioned about a history of hemophagocytic lymphocytic histiocytosis during their workup and screened for BIRC4 mutation if appropriate.

The Treatment of Juvenile Fibromyalgia with an Intensive Physical and Psychosocial Program.

OBJECTIVE: To assess the short-term and 1-year outcomes of children with fibromyalgia treated with intensive physical and occupational therapy (PT/OT) and psychotherapy. STUDY DESIGN: Children with fibromyalgia seen at a tertiary care hospital were treated with 5-6 hours of intensive PT/OT daily and at least 4 hours of psychosocial services weekly. All medications used for fibromyalgia were discontinued. Children underwent standardized testing, including a visual analog scale for pain; the Bruininks-Oseretsky Test of Motor Performance, Second Edition; the Bruce treadmill protocol; the Functional Disability Inventory; the Pain Stages of Change Questionnaire, adolescent version; and the Pediatric Quality of Life Inventory, Teen Report, at 3 time points: at program entry, at the end of the intensive program, and 1 year after the end of the program. RESULTS: Sixty-four children (median age, 16 years; 95% Caucasian; 94% female; median duration of symptoms, 21 months) were studied. The mean pain score decreased significantly from program entry to the end of the program (from 66 of 100 to 25 of 100; P = .001). At the 1-year follow-up, 33% reported no pain. All measures of function on the Bruininks-Oseretsky Test of Motor Performance, Second Edition improved significantly and remained at that level or continued to improve over the subsequent year. The mean Bruce treadmill protocol time first increased from 588 seconds to 801 seconds (P < .001) and then dropped to 750 seconds (P = .005), which is at the 90th percentile for age and sex. All Pain Stages of Change Questionnaire, adolescent version subset scores improved significantly initially and were stable or improved at 1 year, as did the Pediatric Quality of Life Inventory, Teen Report total score. CONCLUSION: Children with fibromyalgia can be successfully treated without medications with a very intensive PT/OT and psychotherapy program. They have significantly improved pain and function by subject report and objective measures of function.

Diagnosis and treatment of low back pain in the pediatric population.

Back pain in the pediatric population is a common complaint presenting to sports medicine clinics. There is a wide differential that should be considered, including mechanical, infectious, neoplastic, inflammatory, and amplified musculoskeletal pain. The history, pain quality, and examination are key components to help distinguish the etiologies of the pain and direct further evaluation. Laboratory investigations, including blood counts and inflammatory markers, can provide insight into the diagnosis. The HLA-B27 antigen can be helpful if a spondyloarthropathy is suspected. Imaging as clinically indicated typically begins with radiographs, and the use of MRI, CT, or bone scan can provide additional information. Proper diagnosis of back pain is important because prognosis and treatments are significantly different. We review the pertinent evaluation, differential diagnoses, and treatment of low back pain in the pediatric population.

Variation in inpatient therapy and diagnostic evaluation of children with Henoch Schönlein purpura.

OBJECTIVE: To describe variation regarding inpatient therapy and evaluation of children with Henoch Schönlein purpura (HSP) admitted to children's hospitals across the United States. STUDY DESIGN: We conducted a retrospective cohort study of children discharged with a diagnosis of HSP between 2000 and 2007 by use of inpatient administrative data from 36 children's hospitals. We examined variation among hospitals in the use of medications, diagnostic tests, and intensive care services with multivariate mixed effects logistic regression models. RESULTS: During the initial HSP hospitalization (n = 1988), corticosteroids were the most common medication (56% of cases), followed by opioids (36%), nonsteroidal antiinflammatory drugs (35%), and antihypertensive drugs (11%). After adjustment for patient characteristics, hospitals varied significantly in their use of corticosteroids, opioids, and nonsteroidal antiinflammatory drugs; the use of diagnostic abdominal imaging, endoscopy, laboratory testing, and renal biopsy; and the use of intensive care services. By contrast, hospitals did not differ significantly regarding administration of antihypertensive drugs or performance of skin biopsy. CONCLUSIONS: The significant variation identified may contribute to varying HSP clinical outcomes between hospitals, warrants further investigation, and represents a potentially important opportunity to improve quality of care.