Serum TNF-α levels and indicators of disease activity in children with oligoarticular juvenile idiopathic arthritis (oJIA) in the first year of the disease.

BACKGROUND: Juvenile idiopathic arthritis is the most common arthropathy in childhood. Clinical assessment in JIA patients is based on clinical examination and conventional parameters of inflammation. Regardless of the JIA form, a distinctive characteristic of JIA is joint inflammation, which is sustained by an imbalance between pro- and anti-inflammatory factors. A significant amount of research has confirmed elevated levels of TNF-alpha in the serum and synovial fluid of JIA patients. The aim of the study was to compare serum TNF-alpha levels and indicators of disease activity in children with newly diagnosed oJIA in the first year of the disease and to assess the diagnostic value of TNF-alpha. METHODS: In a 1-year prospective study, TNF-alpha levels were measured using ELISA in serum samples for 22 oJIA patients. The control group consisted of 16 healthy children. The data were correlated with disease activity indicators and CHAQ score. RESULTS: Concentrations of TNF-alpha were significantly higher in the study group than in the control group at time 0 [10.03 pg/mL (2.16 - 15.53) vs. 0.00 pg/mL (0.00 - 0.12); p < 0.001] and at time 2 [0.00 pg/mL (0.00 - 9.26) vs. 0.00 pg/mL (0.00 - 0.12); p = 0.014]. The analysis of changes in TNF-alpha concentrations in the study group over time showed no statistically significant differences. No correlation between concentrations of TNF-alpha and any of the analyzed indicators of disease activity and CHAQ was found. CONCLUSIONS: Assessment of TNF-alpha concentration in blood serum in children with oJIA has no diagnostic value in monitoring the severity of the disease and the effectiveness of treatment.

Proinflammatory cytokines in monitoring the course of disease and effectiveness of treatment with etanercept (ETN) of children with oligo- and polyarticular juvenile idiopathic arthritis (JIA).

BACKGROUND: The demonstration of the quantitative prevalence of specific cytokines in JIA formed the basis for the introduction of biological anticytokine drugs to treatment. Routine assessment of the concentration of these cytokines in blood serum may enable earlier decision making on the legitimacy of biological treatment (anti-TNF). The aim of the study was to assess the diagnostic value of TNFalpha, IL-6, and IL-1beta in monitoring the course of the disease and effectiveness of treatment with etanercept of children with oligo- and polyarticular JIA. METHODS: In a 1-year prospective study, cytokine levels were measured using ELISA in serum samples for 19 JIA patients in whom no clinical improvement was noted in spite of treatment with disease modifying antirheumatic drugs (DMARDs) and glucocorticosteroids (GCS). All the patients started treatment with ETN. The control group included healthy children (n = 18). RESULTS: Concentrations of TNF-a and IL-6 in blood serum at time 0 were significantly higher than in the control group. IL-6 concentration decreased during treatment with ETN in children in the inactive phase of the disease. Mean concentrations of TNF-a in serum increased several dozen times irrespective of clinical improvement. TNFalpha concentrations were statistically significantly higher in patients in the inactive phase of the disease in comparison with those in the active phase and correlated with the dose of ETN. Only the concentration of IL-6 correlated with the JADAS-27 value at all time points. CONCLUSIONS: We conclude that IL-6 may serve as a biomarker of activity of the disease in children with JIA treated with ETN.

Transverse testicular ectopia with abnormal karyotype - a case report.

OBJECTIVES: Growth disturbances and developmental malformations of external genitalia, such as hypospadias, bifid scrotum and micropenis, coexisting with non-palpable testes, may develop as a result of primary endocrinological dysfunctions as well as an effect secondary to chromosomal aberrations. Therefore, patients with these symptoms require specific diagnostic and therapeutic approaches. DESIGN AND METHODS: We present an example of TTE as a presentation of karyotype abnormalities. Clinical presentation - 9.5 year old boy presented with hypospadias, bilateral cryptorchidism and right inguinal hernia and short stature. RESULTS: Endocrine test showed low testosterone levels with adequate gonadal response. Laparoscopy was performed and revealed the presence of TTE. CONCLUSIONS: The presence of mosaic karyotype with abnormal Y chromosome does not exclude a possibility of testis migration disorders, including TTE, caused by other (possibly genetic) factors. Laparoscopy is a technique of choice for diagnosis and treatment in cases of cryptorchidism.

Mental health and adjustment to juvenile idiopathic arthritis: Level of agreement between parent and adolescent reports according to Strengths and Difficulties Questionnaire and Adolescent Outcomes Questionnaire.

The aims of this study were threefold. Firstly, to analyze the psychometric properties of the Polish-language Pediatric Outcomes Data Collection Instrument (PODCI) questionnaire in the self-report Adolescent Outcomes Questionnaire (adolescents, 11-18 years of age) and in the parent-report Adolescent Outcomes Questionnaire (completed by a parent or guardian of an adolescent aged 11-18 years). Secondly, to determine the level of agreement between parents and adolescents in rating dysfunction in juvenile idiopathic arthritis (JIA) and thirdly, to examine associations between psychological adjustments of patients to JIA and disease as well as their socio-demographic characteristics. The study sample consisted of 52 participants. 26 adolescents between the ages of 11 and 18 years with a diagnosis of JIA and 26 parents were considered for inclusion. Disease course was classified as pauciarticular (n = 12, 46.2%) and polyarticular (n = 14, 53.8%). Participants completed the PODCI (self- and parent- report) twice and the Strengths and Difficulties Questionnaire-25 (SDQ-25). Considering the distribution of results regarding PODCI normative scores, 73.1% of parents and 69.2% of patients scored below 50 on the Global Functioning Scale; that is lower than the average for the general healthy population. Regarding the parent report, the total score of the SDQ-25 equaled 11.86 (SD 2.66), whereas the patient report equaled 11.23 (SD 2.78). The study groups do not differ significantly in regards to either the PODCI or the SDQ-25 results. Parents and adolescents with JIA appear to hold very similar perceptions of patients' health. Greater differences emerge as disease severity and age of patients increase. Excellent internal consistency, intrarater and test-retest reliability of the Global Functioning Scale have been confirmed in the Polish version of the PODCI, the questionnaire may therefore aid identification of patients reporting significant problems in this group.

The Pediatric Outcomes Data Collection Instrument for a Polish sample with juvenile idiopathic arthritis: psychometric properties of proxy version.

AIM: The aims of the study were to investigate the psychometric properties of the Pediatric Outcomes Data Collection Instrument (PODCI) for parents of children aged 2-10 years, in a Polish sample of juvenile idiopathic arthritis and to compare the results from children with chronic arthritis to the results from a normative sample. METHODS: The sample consisted of the parents of 29 (72.5%) girls and 11 (27.5%) boys. The ages of the children ranged from 2 to 10 years (mean 6.50, standard deviation [SD] 2.63). Disease course was classified as oligoarticular (n = 26, 65%) and polyarticular (n = 14, 35%). Parents who took part in the intrarater reliability study completed the Polish version of PODCI twice. The PODCI consists of the following subscales: Upper Extremity and Physical Function, Transfer and Mobility Tasks, Sports/Physical Functioning, Pain/Comfort, and Happiness. A Global Functioning Scale consists of the mean of the 'mean of items' values for the first four scales. RESULTS: The mean standarized Global Functioning Scale was 81.79 (SD 13.45), whereas the mean normative Global Functioning Scale score equaled 33.65 (SD 18.19). Cronbach's alpha value of the Global Functioning Scale was excellent and equaled 0.81. The effect size concerning the Global Functioning Scale equaled 0.123. Test-retest reliability equaled 0.98, whereas intrarater reliability (intraclass correlation coefficient) equaled 0.99. CONCLUSIONS: The translation and cross-cultural adaptation of the PODCI questionnaire provides a Polish equivalent to assess physical function in children with chronic arthritis.