Assessing fatigue and related factors in adolescents with familial Mediterranean fever (FMF): psychometric properties of the PedsQL Multidimensional Fatigue Scale.

BACKGROUND: Fatigue is a common problem in pediatric rheumatic diseases and is associated with poor quality of life. However, no validated methods are available to measure fatigue in adolescents with familial Mediterranean fever (FMF). The aim of the study was to establish validity and reliability for the child self-report PedsQL Multidimensional Fatigue Scale (PedsQL-MFS) and to investigate the effects of physical characteristics, diseaserelated characteristics, sleep quality/duration, and the amount of physical activity on fatigue in adolescents with FMF. METHODS: Seventy-one adolescents with FMF (13-18 years) were included. Children were examined regarding physical- and disease-related characteristics and completed patient-reported outcome measures (PROMs) regarding sleep quality/duration, physical activity levels, and fatigue. PedsQL-MFS was re-completed within the following 7-14 days. RESULTS: PedsQL-MFS demonstrated excellent test-retest reliability (ICC in 95% CI: 0.877-0.958) and internal consistency (Cronbach`s α: 0.928). All items contributed to the total score (item-total correlation > 0.3). PedsQLMFS scores were significantly correlated to fatigue (r: -0.666, p < 0.001), physical activity (r: 0.373, p < 0.001), sleep quality (rs: 0.678, p < 0.001), and sleep duration (r: 0.473, p < 0.001). Being female, having attacks in the last six months, a sleep duration of less than seven hours, and engaging in less physical activity resulted in higher fatigue. CONCLUSIONS: PedsQL-MFS seems to be feasible for assessing fatigue in adolescents with FMF. Sex, recent attacks, sleep, and physical activity should be taken into consideration in the fatigue management of patients with FMF.

Role of fecal calprotectin in the assessment of intestinal inflammation in children with familial Mediterranean fever.

AIM: Familial Mediterranean fever (FMF) is the most common auto-inflammatory disease with recurrent fever and serositis episodes. In recent years, some cases with FMF were reported with gastrointestinal involvement without amyloidosis, vasculitis and inflammatory bowel disease (IBD). It is not yet known whether gastrointestinal involvement is a part of the disease or not. The aim of this study is to investigate the frequency of intestinal inflammation by using a noninvasive method, fecal calprotectin measurement, in pediatric FMF patients. METHOD: Sixty-five FMF patients, 30 healthy controls and 11 patients with acute ulcerative colitis were included in the study. A standard survey inquiring gastrointestinal and other clinical symptoms was completed. The medications, MEFV mutations, whole blood count and C-reactive protein levels were recorded. Fecal calprotectin was studied with the enzyme-linked immunosorbent assay method from the feces samples of the all subjects. RESULTS: None of the FMF patients had clinical signs of IBD. Fecal calprotectin levels of the FMF patients were found to be significantly higher than the healthy controls (174.8 ± 150.8 vs 52.9 ± 36.5, p < 0.001). Fecal calprotectin levels of the ulcerative colitis patients were significantly higher than the FMF patients (523.5 ± 183 vs 174.8 ± 150.8, p = 0.001). There was a correlation between fecal calprotectin levels and neutrophil/lymphocyte ratio (r = 0.324, p = 0.009). CONCLUSION: Our results supported subclinical intestinal inflammation in pediatric FMF patients. Further studies are needed to clarify the reason for intestinal inflammation in FMF patients.

The Publication Rates of Pediatric Rheumatology Abstracts Presented in European League Against Rheumatism 2009 Congress.

OBJECTIVES: This study aims to evaluate the publication rates and features of the abstracts related to pediatric rheumatology presented in European League against Rheumatism 2009 congress. PATIENTS AND METHODS: A systematic search was performed to find full-text publications of abstracts related to pediatric rheumatology in European League against Rheumatism 2009 congress. Full-text publication rate, the elapsed time between presentation and full-text publication, type of the disease in the studies, distribution of abstracts and full-texts according to countries, journals, and presentation types were investigated. RESULTS: Totally 220 abstracts were detected related to pediatric rheumatology. Twenty-two of them had only the title, authors' names, and institution of origin, but no abstract. Therefore, a total number of 198 abstracts were evaluated. Eighty-six (43.4%) abstracts were found to be accepted as full-text articles. The elapsed time between presentation and full text publication was median 19 months (range 0 to 64 months). While 12 orally presented abstracts (34%) became full-text articles, this rate was 45% (74 abstracts) for poster presentations. There was no significant difference in the elapsed time to reach full-text publication and impact factors between presentation types (p=0.832 and p=0.053, respectively). CONCLUSION: The full-text publication rates were within similar ranges when compared to other reports in rheumatology field. It seems that even though European League against Rheumatism is a general rheumatology congress, it takes an important place in pediatric rheumatology field as well.

Inactive Disease in Enthesitis-related Arthritis: Association of Increased Body Mass Index.

OBJECTIVE: Patients with enthesitis-related arthritis (ERA) were less likely to achieve and sustain inactive disease than children with other subtypes of juvenile idiopathic arthritis. The aim of this study was to evaluate the effect of increased body mass index (BMI) on clinical features of the disease and to investigate whether being overweight or obese limits the possibility of achieving clinically inactive disease in patients with ERA. METHODS: The hospital charts of 72 patients with ERA were reviewed. Demographic and clinical findings were recorded. Patients were divided into 2 groups according to whether they had "healthy weight" (BMI < 85th percentile) or "increased weight" (BMI ≥ 85th percentile) at baseline. The primary outcome of this study was to achieve inactive disease at 1 year after the initiation of therapy. The inactive disease criterion of Wallace, et al was used to define inactive disease status. RESULTS: Twenty patients had increased BMI. The frequency of tarsitis and ankle involvement was higher in patients with increased weight. Thirty-seven patients were inactive at the end of 1 year. In univariate analyses, male sex, increased BMI, ankle involvement, and tarsitis were found to be associated with failure to achieve inactive disease. Multivariate backward stepwise regression analyses revealed that failure to achieve clinically inactive disease was associated with increased BMI and ankle involvement. CONCLUSION: Being overweight or obese was associated with failure to achieve inactive disease in patients with ERA. Because body weight is a modifiable factor, individualized interventions may have clinical implications for better therapeutic outcome.

The relationship of neutrophil-to-lymphocyte ratio with gastrointestinal bleeding in Henoch-Schonlein purpura.

Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis of childhood. Gastrointestinal (GI) bleeding is one of the major complications of HSP. The blood neutrophil-to-lymphocyte ratio (NLR) is identified as a potentially useful marker of clinical outcome in inflammatory diseases. NLR may be a useful biomarker of GI bleeding in children with HSP, which has a neutrophil-dominated inflammation. The aim of this study was to evaluate NLR in patients with HSP and to investigate the relationship with GI bleeding. The study consisted of 63 HSP patients and 38 age- and sex-matched healthy children. C-reactive protein, white blood cell count, platelet count, mean platelet volume (MPV), hemoglobin level, and NLR were evaluated. Logistic regression analysis and receiver operating characteristic (ROC) analysis were used to determine the variables associated with GI bleeding. NLR and MPV were the only two indicators associated with GI bleeding in HSP in logistic regression analysis. The area under the ROC curve analysis indicated that NLR could be a more efficient potential predictor of GI bleeding in HSP when compared to MPV. This study suggested that higher NLR might predict GI bleeding in HSP.

Potential predictors of relapse after treatment of asthma exacerbations in children.

BACKGROUND: Knowledge of factors that affect relapse will allow close monitoring of patients at risk, resulting in a decreased rate of readmission to the emergency department. OBJECTIVE: To determine risk factors associated with relapse within 7 days after treatment of asthma exacerbations in children. METHODS: This was a multicenter, prospective study of children with asthma attacks. Patients between the ages of 6 months and 17 years who met the criteria between June 2009 and September 2012 were considered. RESULTS: The study included 1177 patients (775 males [65.8%]) with a mean (SD) age of 70.72 (48.24) months. Of them, 199 (16.9%) had a relapse within 1 week after being discharged from the hospital. Factors independently associated with relapse identified by a logistic regression model for the 1,177 study visits were having taken a short-acting inhaled ß2-agonist within 6 hours before admission (odds ratio [OR], 2.43; 95% confidence interval [CI], 1.728-3.426; P = .001), presence of retraction on physical examination (OR, 1.76; 95% CI, 1.123-2.774; P = .01), no prescription for high-dose inhaled steroids on release (OR, 2.02; 95% CI, 1.370-3.002; P < .001), and not being given a written instructional plan (OR, 1.55; 95% CI, 1.080-2.226; P = .02). CONCLUSION: Whereas having taken short-acting ß2-agonists within 6 hours before admission and the presence of retractions on physical examination increased the risk of relapse after treatment of the acute attack, being given high-dose inhaled steroids and a written instructional plan when being sent home reduced the risk.