Progressive bilateral lipoma arborescens of the knee complicated by juvenile spondyloarthropathy: a case report and review of the literature.

OBJECTIVES: To report an unusual case of lipoma aborescens (LA) presented in a patient with treatment-responsive juvenile spondyloarthropathy (JSPA) and to summarize the clinical manifestations, therapy and prognosis of LA by literature review. METHODS: We report an atypical case of a 17-year-old patient with an initial presentation of juvenile spondyloarthropathy, whose inflammatory condition was improved successfully by traditional anti-rheumatic drugs and an anti-TNF alpha agent but developed progressive swelling of bilateral knees. Lipoma arborescens were diagnosed in each knee by synovial biopsy obtained by arthroscopic surgery. Fifty-one cases of LA have been reported and are reviewed in detail. RESULTS: Clinically, LA could present as monoarthritis or oligoarthritis. The lateral compartment of the knee is the most common site of involvement. Several cases were reported as a comorbidity of inflammatory diseases, but were not improved by anti-inflammatory therapy. Most patients were diagnosed by classic MRI and biopsy findings. The lesions can be managed by open or arthroscopic surgery, but a minority of the cases may have reoccurrence in the same or opposite joint. CONCLUSIONS: LA is a very rare lesion of the synovial and bursal tissue with an unknown etiology. It is considered to be a benign proliferation of the synovial fat associated with trauma, degenerative or inflammatory conditions. LA should be considered as a secondary or comorbid condition in inflammatory arthropathies if other joints respond well to intensive therapy and one or more do not.

Health care utilization of refugee children after resettlement.

Refugee children can have significant health problems. Our objective was to describe health status and health care utilization of refugee children after resettlement. A retrospective chart review of refugee children was performed. Initial laboratory data was extracted. Primary care visits, emergency room visits, and subspecialty referrals in the first 15 months from arrival were recorded. The sample included 198 refugees, many with positive initial screening tests. After arrival, 21% had an emergency department visit, 40% had a primary care sick visit, and 71% had a primary care follow-up. Mean number of visits ranged from 0.3 for emergency department to 1.9 for follow-up. Fifty-seven percent were referred to at least one subspecialist. Refugee children had substantial disease burden at arrival. Most had primary care follow-up visits and subspecialty referral after resettlement. These visits were largely for problems identified on initial screening and for general pediatric illnesses.

Intra-articular corticosteroid therapy for juvenile idiopathic arthritis: report of an experiential cohort and literature review.

The objective of the study to review an experiential cohort of patients receiving IACS and review the associated literature. Review of 121 IACS in 61 patients with JIA. At 3-month intervals, injected joints were evaluated for swelling and range of motion, and the patient and parent were questioned regarding associated pain and morning stiffness. Data were analyzed by log-rank analysis according to injected corticosteroid preparation and its dosage. Adverse events were also recorded. A thorough literature search was done for the literature review. Mean duration of response was 12.5 months (52% of joints in remission at 1 year, 20% after 2 years, and 7% after 3 years). Response was longer with at least 1 mg/kg of corticosteroid, with the longest responses seen with triamcinolone hexacetonide (THA)>triamcinolone acetonide>methylprednisolone. Adverse events were cutaneous atrophy at three injections sites (2.5%), and transient Cushingoid habitus and increased appetite in two patients (3%). Review of the literature generated similar responses to those included herein. Thus, there have been several recommendations for IACS to be a major JIA treatment, and surveys now demonstrate a high level of usage by pediatric rheumatologists. In conclusion the use of IACS in JIA substantiated. THA at a dose of 1-1.5 mg/kg is ideal.

Persistent elevation of fibrin D-dimer predicts longterm outcome in systemic juvenile idiopathic arthritis.

OBJECTIVE: We previously demonstrated that levels of fibrin d-dimer correlate with disease activity and response to therapies in systemic juvenile idiopathic arthritis (sJIA). We hypothesized that persistence of D-dimer elevation in the patterns previously described, but over a longer followup period, would signal poor outcome. METHODS: We studied 31 children identified from 2 centers. Subjects were assigned a risk category based on their first obtained D-dimer concentration. Risk categories were based on results of our initial study, where normalization of D-dimer in patients no longer taking immunosuppressive therapy predicted good short-term outcome, and persistent D-dimer elevation while taking immunosuppressives predicted bad outcome (radiographic abnormalities, joint replacement surgery, or poor functional class) or a severe systemic manifestation. Outcome was determined at the last followup visit, a minimum of 2 years after measurement of the initial d-dimer level. RESULTS: The 31 children were a mean 16.4 years old at an average of 8.8 years after their initial diagnosis. Ten children had a severe outcome during this period; all 10 had a study baseline risk category of "high." Of the 14 subjects who had a high risk category at study baseline, none had a mild outcome. CONCLUSION: Our study indicated that a paradigm of risk of severe disease based upon persistent elevation of fibrin d-dimer on first measurements (greater than a mean of 29 months in our initial study and at least 24 months in the additional subjects) is promising to predict poor longer-term outcome in sJIA. A larger prospective study is warranted to substantiate the preliminary data and assess the relative comparative value to other biomarkers and clinical endpoints.

A statewide assessment of tuberculin skin testing of preschool children enrolled in medicaid managed care.

OBJECTIVES: This study examined tuberculosis screening among preschool children enrolled in a statewide Medicaid managed care program. METHODS: A random sample of 2,000 was selected from 19 to 35 month old children who were continuously enrolled in Rhode Island's Medicaid managed care program for 1 year. Sociodemographic data were obtained from computerized administrative databases. Medical record audits were performed to obtain the dates and results of tuberculosis tests. RESULTS: Data from the medical record audits were available for 1,988 of the study children. For 1,215 of the study children (1,215/1,988 = 61%) a tuberculin skin test had been performed, but a reading was only documented for 736 children (60% of children who received a tuberculin skin test) and only one child tested positive (0.1%). CONCLUSIONS: A majority of preschool children in this population in which the prevalence of risk factors for tuberculosis is likely to be relatively high did have a tuberculosis test performed. However, in many cases the tuberculin skin test was either not read or the results not documented. The low rate of positivity is consistent with current AAP guidelines for selective tuberculin skin testing.

Soluble intercellular adhesion molecule-1 and E-selectin as markers of disease activity and endothelial activation in juvenile idiopathic arthritis.

OBJECTIVE: To determine whether soluble forms of the adhesion molecules intercellular adhesion molecule-1 (ICAM-1) and E-selectin correlate with clinical measures or other markers of endothelial activation in children with juvenile idiopathic arthritis (JIA) over time. METHODS: A total of 28 children with JIA were studied every 3 months over 2 years. At each interval, serum was tested for soluble (s)ICAM-1 and sE-selectin, plasma for fibrin d-dimer and von Willebrand factor (vWF), and the following clinical variables were recorded: erythrocyte sedimentation rate (ESR), physician and parent global assessments, swollen and limited joint counts, and functional assessment by Childhood Health Assessment Questionnaire. Concentrations of the adhesion molecules were also determined once in 30 age matched healthy children. RESULTS: Among all JIA subtypes, baseline sICAM-1 was elevated compared to controls; sE-selectin was higher in patients with systemic disease compared to other subtypes and controls. sE-selectin correlated with ESR, but there were no other correlations between concentrations of either adhesion molecule or any other clinical variables or vWF antigen. sICAM-1 was higher in those with elevated compared to normal d-dimer. There were no differences between mean sICAM-1 and sE-selectin before or during disease flare or improvement periods, except for an increase in sICAM-1 with flares in patients with systemic disease. CONCLUSION: sICAM-1 is elevated in children with active JIA. sE-selectin is only elevated in children with active systemic disease. Although some relationships were found between the adhesion molecules and other variables, they did not correlate with most variables, and did not parallel the disease course. Thus, we cannot recommend the routine use of these molecules as clinical biomarkers of disease activity. This study confirms that endothelial activation is key to the pathogenesis of JIA, especially in the systemic subtype.

Child death review. The state of the nation.

BACKGROUND: Child death review (CDR) is a mechanism to more accurately describe the causes and circumstances of death among children. The number of states performing CDR has more than doubled since 1992, but little is known about the characteristics of these programs. The purpose of this study was to describe the current status of CDR in the United States and to document variability in program purpose, scope, organization, and process. METHODS: Investigators administered a written survey to CDR program representatives from 50 states and the District of Columbia (DC), followed by a telephone interview. RESULTS: All 50 states and DC participated; 48 states and DC have an active CDR program. A total of 94% of programs agreed that identifying the cause of and preventing future deaths are important purposes of CDR. Assistance with child maltreatment prosecution was cited as an important purpose by only 13 states (27%). Twenty-two states (45%) review deaths from all causes, while six states (12%) review only deaths due to child maltreatment. CDR legislation exists in 33 states. Fifty-three percent of the CDR programs were implemented since 1996, and 59% report no or inadequate funding. CDR contributes to the death investigation process in seven states (14%), but the majority (59%) of reviews are retrospective, occurring months to years after the child's death. CONCLUSIONS: CDR programs in the United States share commonalities in purpose and scope. Without national leadership, however, the wide variation in organization and process threatens to limit CDR effectiveness.

Synovial fluid levels of E-selectin and intercellular adhesion molecule-1: relationship to joint inflammation in children with chronic arthritis.

E-selectin and intercellular adhesion molecule (ICAM)-1 are crucial to the inflammatory response in chronic inflammatory arthritis. Soluble (s) levels of these molecules in sera and synovial fluid (SF) correlate with some clinical parameters and synovial tissue expression of the same molecules in rheumatoid arthritis. Studies of sera from children with chronic inflammatory arthritis corroborate this information; corresponding SF data are relatively lacking. We thus studied SF sE-selectin and sICAM-1 in 28 children with active juvenile rheumatoid arthritis or a spondyloarthropathy. Levels were correlated with erythrocyte sedimentation rate (ESR), SF leukocyte counts, duration of disease, and duration of response to concomitant intra-articular corticosteroid injection. Levels were compared according to use of methotrexate and/or sulfasalazine. Synovial fluid sE-selectin correlated with ESR and SF leukocyte counts. There was a trend toward lower sICAM-1 in patients treated with sulfasalazine and/or methotrexate. We conclude that SF levels of sE-selectin accurately reflect intra-synovial inflammation. Soluble ICAM-1 levels may reflect the effects of disease-modifying agents.

Sleep and its relationship to pain, dysfunction, and disease activity in juvenile rheumatoid arthritis.

OBJECTIVE: To determine what sleep abnormalities may exist in children with juvenile rheumatoid arthritis (JRA). and their relationship to pain, dysfunction. and disease activity. METHODS: Twenty-five children with active JRA (11 pauciarticular, 9 polyarticular, 5 systemic) had their sleep assessed by parallel, validated patient and parent questionnaires (Sleep Self-Report, SSR, and Children's Sleep Habits Questionnaire, CSHQ). Disease activity was assessed by parent and physician global assessments (on a 5 point scale: 0 = no disease activity to 4 = very severe disease), erythrocyte sedimentation rate (ESR), and numbers of swollen and limited joints. Functional assessment was based on parental completion of the Juvenile Arthritis Functional Assessment Report (JAFAR). Pain was assessed by the average pain visual analog scale of the Varni Pediatric Pain Questionnaire. Results were compared to those from 45 healthy age and sex matched controls by Mann-Whitney U tests, and correlated with variables of JRA disease activity, function, and pain using Spearman correlations. RESULTS: Patients with JRA had higher total score on the CSHQ (p < 0.0001), as well as subscales assessing night wakings, parasomnias. sleep anxiety, sleep-disordered breathing, and morning wakening/daytime sleepiness (p < 0.0001-0.05). There were no correlations between CSHQ scores and JRA disease activity or pain variables, but the total score on the SSR did correlate with pain (r = 0.56, p = 0.005). CONCLUSION: We conclude that sleep abnormalities are common in children with JRA, and are multi-dimensional.