Effects of obesity on health-related quality of life in juvenile-onset systemic lupus erythematosus.

OBJECTIVE: This study evaluated the effects of obesity on health-related quality of life (HRQOL) measures in juvenile-onset systemic lupus erythematosus (jSLE). METHODS: Obesity was defined as a body mass index (BMI) ≥ 95 th percentile according to the Sex-specific Center for Disease Control BMI-For-Age Charts and determined in a multicenter cohort of jSLE patients. In this secondary analysis, the domain and summary scores of the Pediatric Quality of Life (PedsQL) Inventory and the Child Health Questionnaire (CHQ) of obese jSLE patients were compared to those of non-obese jSLE patients as well as historical obese and non-obese healthy controls. Mixed-effects modeling was performed to evaluate the relationship between obesity and HRQOL measures. RESULTS: Among the 202 jSLE patients, 25% (n = 51) were obese. Obesity had a significant negative impact on HRQOL in jSLE, even after adjusting for differences in current corticosteroid use, disease activity, disease damage, gender and race between groups. Obese jSLE patients had lower physical functioning compared to non-obese jSLE patients, and to non-obese and obese healthy controls. Compared to their non-obese counterparts, obese jSLE patients also had worse school functioning, more pain, worse social functioning and emotional functioning. Parents of obese jSLE patients worry more. The CHQ scores for obese jSLE patients were also worse compared to non-obese jSLE patients in several other domains. CONCLUSION: Our study demonstrates the detrimental effects of obesity on patient-reported outcomes in jSLE. This supports the importance of weight management for the therapeutic plan of jSLE.

Evaluation of the cytokine response in Kawasaki disease.

Intracellular cytokines interleukin 6, tumor necrosis factor (TNF) alpha and TNF-beta were studied in peripheral blood mononuclear cells of patients with Kawasaki disease during the acute, subacute and convalescent stages of the disease utilizing cytokine-specific monoclonal antibodies and indirect immunofluorescence. Intracellular cytokines TNF-alpha and -beta were present only during the acute stage before initiation of therapy and not in the subacute or convalescent phase. Intracellular interleukin 6 was seen in both the acute phase and, in small numbers of patients, in the subacute stage of the illness. Overall 15 of 25 (60%) patients produced at least one intracellular cytokine. In the acute stage both monocyte and lymphocyte cytokines were detected intracellularly, TNF-alpha and TNF-beta in 58% of patients whereas interleukin 6 was seen in only 16%. This study provides evidence to support the involvement of activated mononuclear cells, both T cells and monocytes and their secreted soluble products, cytokines, in the pathogenesis of Kawasaki disease.

A comparative study of orthoplast cock-up splints versus ready-made Droitwich work splints in juvenile chronic arthritis.

A comparative study of a purpose-made cock-up orthoplast splint with a ready-made Camp Droitwich splint, was undertaken in 12 children with juvenile chronic arthritis involving the wrists and carpi. The splints were randomly allocated so that in half, the dominant hand received the orthoplast and in half the non-dominant. Assessments of joint range, grip strength and functional activities were undertaken at 3 and 6 months after use of the splint; the durability of the splints was also assessed. Overall both splints performed their function adequately, the Camp Droitwich being preferred for comfort, but the orthoplast maintaining a slightly better joint position.

Perhaps vigintiphobia should only apply to infants with Rhesus erythroblastosis.

Forty-two children who sustained a serum bilirubin (SBR) level above 339 mumol/L as newborn infants were assessed at our Growth and Development Clinic to determine presence of sequelae. Only one child (2.3%) had mild sensorineural deafness and one child (2.3%) performed below age-matched standards on psychological testing. As the SBR level rose the psychological scores were lower. Three infants had sepsis associated with the hyperbilirubinaemia. Two (maximum SBR levels of 371 and 366 mumol/L) children were normal (General Cognitive Index (GCI) 117 and 119, respectively) and one child (maximum SBR level 556 mumol/L) was borderline abnormal (GCI 74) on psychological testing; he also suffered from Rhesus erythroblastosis. Premature infants recorded a mean GCI of 109.9 (+/- 33.4) and for term infants mean GCI was 110.3 (+/- 17.3; NS); however, the youngest premature infant was 32 weeks' gestation. When maximum SBR level was correlated with GCI and Motor Index (MI) the only significant correlation (r = -0.7445; P = 0.03) occurred in infants with Rhesus erythroblastosis and GCI. Since exchange transfusion has a mortality of between 0.3 and 5.3% and an associated morbidity incidence of 5.2% we suggest that the standard indication for its use (SBR level of 342 mumol/L) should only apply to infants with Rhesus erythroblastosis. The actual SBR level which places a newborn infant at significant risk of bilirubin encephalopathy, where the cause of jaundice is other than Rhesus erythroblastosis, cannot be determined by this study.(ABSTRACT TRUNCATED AT 250 WORDS)

Wegener's granulomatosis in childhood.

The authors reviewed the medical histories and radiological examinations of five pediatric patients with a histologic diagnosis of Wegener's granulomatosis (WG) seen over a six year period in whom a total of 22 thoracic CT scans were performed. Involvement of both the upper and lower respiratory tracts was seen in all patients at presentation. One patient had subglottic stenosis necessitating tracheotomy. Pulmonary hemorrhage occurred in three patients at initial diagnosis. Classic cavitary lung nodules were seen in two patients--one at initial presentation, the other at relapse. The plain radiographic lower respiratory tract manifestations of pediatric WG were protean both at initial presentation and during follow up. Similarly, disease expression was highly variable on thoracic CT examinations but, overall, multifocal parenchymal infiltrates with or without small peripheral nodules were the commonest thoracic CT manifestations. As a consequence of cytotoxic and corticosteroid therapy the long-term prognosis of WG has improved considerably in recent years. Knowledge of the varied patterns of the primary disease and potential for iatrogenic complications are necessary for successful radiologic assessment of pediatric patients with WG.

Splenic necrosis in Wegener's granulomatosis.

The CT appearance of asymptomatic splenic necrosis in a 11-year-old girl with Wegener's granulomatosis is presented. Sonography showed splenic inhomogeneity with patency of the splenic artery and vein. Follow-up CT showed lobulation and shrinkage of the spleen with return to a normal attenuation.

Local synthesis of both macrophage and T cell cytokines by synovial fluid cells from children with juvenile rheumatoid arthritis.

The production of tumour necrosis factor-alpha (TNF-alpha), TNF-beta and IL-6 in synovial fluid was studied in 50 samples of synovial fluid from 44 children with juvenile rheumatoid arthritis (JRA) by identifying cytokine production at a single-cell level. Post Ficoll-separated synovial fluid mononuclear cells were permeabilized and then intracellular TNF-alpha, TNF-beta and IL-6 protein production was examined using indirect immunofluorescence and murine anti-cytokine MoAbs. All three cytokines were measured in 37 of the 50 samples. In 25 of the 37 samples there was complete concordance; all three cytokines were present in six and absent in 19 samples. At least one cytokine was present in 27/50 (54%) of synovial fluid samples. Overall, TNF-alpha was detected in 22/49 (45%) samples, TNF-beta in 15/41 (37%) and IL-6 in 16/45 (36%) samples. Five patients had serial arthrocentesis, and in these samples there were two patients who had initially positive cytokine production, which on subsequent measurement was negative; in the other three patients there was no change from the previous cytokine production. We provide evidence that synovial fluid mononuclear cells produce monocyte and T cell cytokines in JRA. These findings suggest a role for both T cell and macrophage products in the pathogenesis of JRA, and the potential for modulation of cytokine production as a target for therapeutic intervention.

Psoriatic eruption in Kawasaki disease.

We describe 10 patients who developed a psoriatic skin eruption during either the acute or convalescent phase of Kawasaki disease. The skin eruption was pustular in 3 patients, but more typical psoriasiform skin lesions were seen in the remaining 7 patients. No patient has yet developed chronic psoriasis.