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Biomedical subjects

J Hoza

Publications and source records attributed to J Hoza.

81 records · Page 5Linked to original sources

Immunological investigation in children with juvenile chronic arthritis.

BACKGROUND: Immunological investigation is a part of the complex view on a child with juvenile chronic arthritis (JCA). We analyzed the data of a cohort of children with JCA in order to determine the real contribution of this investigation to their diagnosis and therapy. MATERIAL AND METHODS: We included the investigation of humoral immunity and autoantibodies of 78 children with JCA. 18 children completed investigation of both humoral and cellular immunity of paired peripheral blood (PB) and synovial fluid (SF). Humoral immunity consisted from immunoglobulins, complement, circulating immune complexes, rheumatoid factors, soluble HLA I. molecules and antinuclear and antineutrophil cytoplasmic antibodies. Cellular immunity included cytometric studies of CD3, CD4, CD8, CD16/CD56, CD19, CD20, 23, CD3 HLA DR+, CD45 RA, CD45 RO, alpha/beta and gamma/delta T cells. To observe the status of Th1/Th2 balance in children with JCA, the cytokines IL-4, IFN gamma, TNF alpha and IL-6 were measured in the tissue culture of the synovial cells. RESULTS: The parameters of humoral immunity in serum showed wide variability. We could not confirm particular changes specific for the forms or stage of the disease. ANCA were positive in 21 out of 78 children with JCA, 3 times both in PB and SF. More typical pattern could be followed in the comparison of PB and SF, with immunoglobulins and complement always found lower in SF than in PB. The cellular immunity was represented by the activation of lymphocytes mainly in SF, reverse ratio of CD45 RA and RO cells in PB and SF with marked predominance of memory T cells in the joint. High levels of sHLA in SF are the nonspecific marker of activation, the same is true for high levels of TNF alpha and IL 6 in SF cell culture supernatant. CONCLUSION: The described changes in immunological parameters of humoral and cellular immunity are not specific for JCA. In the individual cases they can contribute to the diagnosis and monitoring of the disease. The investigation of sHLA molecules and cytokine profile should be restricted only for research.

Adolescent↗

The Czech version of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ).

We report herein the results of the cross-cultural adaptation and validation into the Czech language of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The Czech CHAQ-CHQ were fully validated with 3 forward and 3 backward translations. A total of 150 subjects were enrolled: 81 patients with JIA (14% systemic onset, 44% polyarticular onset, 10% extended oligoarticular subtype, and 32% persistent oligoarticular subtype) and 69 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA patients, with the systemic, polyarticular and extended oligoarticular subtypes having a higher degree of disability, pain, and a lower overall well-being when compared to their healthy peers. Also the CHQ clinically discriminated between healthy subjects and JIA patients, with the systemic onset, polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the Czech version of the CHAQ-CHQ is a reliable, and valid tool for the functional, physical and psychosocial assessment of children with JIA.

Adolescent↗

Soluble adhesion molecules ICAM-1 and E-selectin in juvenile arthritis: clinical and laboratory correlations.

OBJECTIVE: To determine serum and synovial fluid (SF) concentrations of soluble intercellular adhesion molecule-1 (ICAM-1) and E-selectin (E-sel) in patients with active juvenile idiopathic arthritis (JIA) and in paediatric controls and correlate them with clinical and laboratory variables. METHODS: Total of'30 JIA patients were evaluated: 15 with polyarticular disease course (JIA-poly) and 15 with oligoarthritis (JIA-oligo). Paediatric age-matched control groups consisted of 11 Henoch-Schönlein purpura (HSP) and 10 febrile patients (FC) and 28 healthy children (HC). Current medication, the erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and full blood count (FBC) were recorded. Soluble ICAM-1 and E-sel in serum and SF were measured by a sandwich ELISA kit. RESULTS: In the JIA-poly group the concentration of ICAM-1 was significantly higher than in healthy (p < 0.01), but notfebrile controls. Both ICAM-1 and E-selectin correlated with the active joint count (p < 0.01). In 13 JIA patients no correlanon was found between SF ICAM-1 and E-sel levels and the SF leucocyte counts. No significant differences were seen in the disease control and JIA-oligo groups compared to HC. A significant negative correlation with age was observed for the group as a whole (ICAM-1: p < 0.05, E-sel: p < 0.01); E-sel correlated with the leucocyte and thrombocyte counts (p < 0.01), and both molecules with CRP (p < 0.05) and with each other (p < 0.01). CONCLUSION: A high concentration of soluble ICAM-1 in JIA patients with polyarthritis is reported here for the first time. None of the patients showed signs of injection or vasculitis, where generalised endothelial activation could be its main source. Our finding of correlations between both ICAM-1 and E-sel levels and joint counts supports the hypothesis of their synovial origin. ICAM- I and E-sel could serve as a marker of aggressive disease, but their predictive value needs to be further studied.

Adolescent↗

Subtypes of HLA-DRB1*03, *08, *11, *12, *13 and *14 in early onset pauciarticular juvenile chronic arthritis (EOPA) with and without iridocyclitis.

A set of 200 patients with early onset pauciarticular juvenile chronic arthritis (EOPA-JCA) from Munich (165) and Prague (35) was investigated for the subtypes of HLA-DRB1*03, *08, *11, *12, *13 and *14. In addition, the relationship of DRB1, DQA1, DQB1 and DPB1 alleles with iridocyclitis in patients with EOPA-JCA was investigated. Subtyping for DRB1*03 was not informative, as all DR3 positive patients and all except one of the controls possessed DRB1*0301. Thus, the role of DRB1*0302 could not be assessed. The subtypes for DRB1*12, *13, and *14 did not reveal any statistically significant difference between patients and controls. In contrast, the subtype DRB1*1104 was the one most strongly associated with EOPA-JCA (chi 2 31.2, p value < 10(-6)). It appears that the subtype DRB1*1103 may also be associated with EOPA-JCA. The association of EOPA-JCA with DR8 is almost exclusively due to the subtype *0801. For the other alleles *0802, *0803, and *0804 there is no evidence for or against involvement in JCA. The analysis of iridocyclitis in EOPA-JCA revealed that DRB1*1104 is not more frequent in patients with eye disease than in patients without eye disease. The presence of DRB1*01 appears to convey some protective effect against the occurrence of iridiocylitis in EOPA-JCA, as had been previously observed by Melin-Aldana et al.

Age of Onset↗

Eosinophilic fasciitis in an adolescent girl with lymphadenopathy and vitiligo-like and linear scleroderma-like changes. A case report.

The patient, a 14-year-old girl, suffered from arthralgias which occurred after tonsillitis. Two months later she developed edema of the left lower extremity, finger flexion contractures and induration of the skin of the left leg, associated with hypergammaglobulinemia, peripheral hypereosinophilia, elevated ESR and a positivity of ANA and anti ds-DNA antibodies. A biopsy of the inguinal lymph node, performed because of left inguinal and retroperitoneal lymphadenopathy, showed only slight inflammatory activation and a granulomatous reaction after lymphography. A few days after the lymphography linear erythema evolving later into hyperpigmentation and corresponding to the superficial lymphatics developed on the left side of the body, very probably as a reaction to the patent-blue dye. Deep en-block skin biopsy confirmed the diagnosis of eosinophilic fasciitis (EF). After two years of therapy with prednisone and d-penicillamine the patient felt well, and her flexion contractures resolved, ANA were positive, while anti ds-DNA were negative. Linear hyperpigmentation persisted, and linear scleroderma-like changes developed on the left lower limb. A vitiligo-like lesion on the right foot which occurred after one year of therapy persisted. The possible risk of developing systemic connective tissue disease necessitates the long term follow up of this patient.

Adolescent↗