Paediatric Rheumatology Unit

Ankara, Turkey

Paediatric Rheumatology Unit

Ankara, Turkey
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Demirkaya E.,Paediatric Rheumatology Unit | Acikel C.,Gulhane Military Medical Faculty | Fidanci K.,Gulhane Military Medical Faculty | Karaman D.,Gulhane Military Medical Faculty | And 6 more authors.
Clinical and Experimental Rheumatology | Year: 2014

Objective: The aim of this study was to develop a multidimensional assessment instrument named "Juvenile Vasculitis Multidimensional Assessment Report" (J-VAMAR) to measure all the domains of the vasculitis. In this qualitative study, it is primarily aimed to enrich the item generation for the J-VAMAR. Methods: Twelve children with vasculitis and their mothers (n=12) were enrolled in this study. The data were collected using both a demographic data form and a semi-structured interview form. The study was performed on individual patient face-to face interview. Data were analysed by grounded theory and the N Vivo 9 software program. Results: Four categories were obtained. These categories were (i) physical effects of the illness, (ii) emotional effects of the illness, (iii) social effects of the illness and (iv) experienced challenges related to treatment process. In the physical effect category severe pain, physical limitations, weakness and fatigue; in emotional effect category thought of death, hopelessness and dissatisfaction about body image; in the social effects category decrease in academic performance, absenteeism to school and concealing the sickness from friends were the most common features. In the fourth category, subjects complained of lifelong drug use and frequency of daily drug consumptions. Conclusion: These results provide evidence- based data for the assessment of children with vasculitis by several domains including physical, emotional and social aspects as well as treatment protocols. The study provides the basis and/or justification for selecting the domains that the developing multidimensional instrument should include. © Clinical and Experimental Rheumatology 2014.


Aikawa N.E.,Paediatric Rheumatology Unit | Silva C.A.A.,Paediatric Rheumatology Unit | Ishida M.A.,University of Sao Paulo | Precioso A.R.,Butantan Institute
Rheumatology (United Kingdom) | Year: 2012

Objective: To assess the efficacy and safety of pandemic 2009 influenza A (H1N1) in SLE under different therapeutic regimens. Methods: A total of 555 SLE patients and 170 healthy controls were vaccinated with a single dose of a non-adjuvanted preparation. According to current therapy, patients were initially classified as SLE No Therapy (n = 75) and SLE with Therapy (n = 480). Subsequent evaluations included groups under monotherapy: chloroquine (CQ) (n = 105), prednisone (PRED) ≥20mg (n = 76), immunosuppressor (IS) (n = 95) and those with a combination of these drugs. Anti-H1N1 titres and seroconversion (SC) rate were evaluated at entry and 21 days post-vaccination. Results: The SLE with Therapy group had lower SC compared with healthy controls (59.0 vs 80.0%; P<0.0001), whereas the SLE No Therapy group had equivalent SC (72 vs 80.0%; P = 0.18) compared with healthy controls. Further comparison revealed that the SC of SLE No Therapy (72%) was similar to the CQ group (69.5%; P = 0.75), but it was significantly reduced in PRED ≥20mg (53.9%; P = 0.028), IS (55.7%; P = 0.035) and PRED ≥20mg+ IS (45.4%; P = 0.038). The concomitant use of CQ in each of these later regimens was associated with SC responses comparable with SLE No Therapy group (72%): PRED ≥20mg+CQ (71.4%; P = 1.00), IS +CQ (65.2%; P = 0.54) and PRED ≥20mg+ IS +CQ (57.4%; P = 0.09). Conclusion: Pandemic influenza A H1N1/2009 vaccine response is diminished in SLE under immunosuppressive therapy and antimalarials seems to restore this immunogenicity. © The Author 2012. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.


Modesto C.,University of Barcelona | Anton J.,Hospital Sant Joan Of Deu | Rodriguez B.,University of Barcelona | Bou R.,Paediatric Rheumatology Unit | And 6 more authors.
Scandinavian Journal of Rheumatology | Year: 2010

Objective: To ascertain the incidence and prevalence of juvenile idiopathic arthritis (JIA) in Catalonia (autonomous region in northeast Spain), examined according to the currently established disease subtypes. Methods: Before initiating the study, we conducted an educational programme on paediatric rheumatology, addressed to all general paediatricians in Catalonia. A 2-year (2004-2006), prospective, population-based study was then carried out to determine the incidence of JIA. Prospective and retrospective data retrieval was performed to calculate prevalence. The International League of Associations for Rheumatology (ILAR, Edmonton revision) classification criteria were applied. Results: Over the study period, 145 new cases of JIA were diagnosed. The mean annual incidence was 6.9/105 children aged less than 16 years (range 5.8-8.1 years; 9.0 years for girls and 4.8 years for boys). On separate analysis of patients ≤ 6 and > 6 years, the distribution in younger children was found to be similar for both girls and boys, whereas in older children, most girls belonged to the oligoarthritis and polyarthritis subgroups, and boys to the enthesitis-related arthritis and undifferentiated subgroups. The calculated prevalence of JIA (31 October 2006) was 39.7 (36.1-43.7)/105 children younger than 16. The relative risk of girls having JIA was 2.1 [95% confidence interval (CI) 1.7-2.7, p < 0.001]. In 70% of patients, the diagnosis was established before the age of 7. Subgroup distribution of prevalent cases mirrored that of incident cases. Conclusion: This is the first population-based study on the epidemiology of JIA in Catalonia. Incidence and prevalence rates are lower than those reported for several areas in Nordic countries of Europe. Oligoarthritis was the most common subtype. © 2010 Taylor & Francis on license from Scandinavian Rheumatology Research Foundation.


PubMed | Selcuk University, University Utrecht, Gulhane Military Medical Faculty, Istanbul Kanuni Sultan Suleyman Educational and Research Hospital and 10 more.
Type: Journal Article | Journal: Clinical and experimental rheumatology | Year: 2016

To develop and test a new multidimensional questionnaire for assessment of children with auto-inflammatory disease (AID) such as FMF, PFAPA, HIDS, TRAPS in standard clinical care.The juvenile auto-inflammatory disease multidimensional assessment report (JAIMAR) includes 16 parent or patient-centered measures and four dimensions that assess functional status, pain, therapeutic compliance and health-related quality of life (physical, social, school, emotional status) with disease outcome. It is proposed for use as both a proxy-report and a patient self-report, with the suggested age range of 8-18 years for use as a self-report.250 children with FMF were included in the study. Total of 179 forms were filled up by parents and patients, and 71 forms were filled up by parents having children less than 8 years. Completing and scoring the JAIMAR can be done in 15 minutes. For the JAIMARs dimensions, the Cronbachs alpha coefficient for internal consistency was between 0.507-0.998. There was a significant and a positive correlation between the test-retest scale scores (ICC=0.607-0.966). Concerning construct validity, all factors loadings were above 0.30. For the criterion validity, the correlation level between each dimension and the related scale ranged from medium (r=0.329, p<0.0001) to large (r=0.894, p<0.0001). The parents proxy-reported and childrens self-reported data were outstandingly concordant (r=0.770-0.989).The development of the JAIMAR introduces a new and multi-dimensional approach in paediatric rheumatology practice. It is a new tool for children with auto-inflammatory dis-ease and it may help enhance their quality of care.


Magni-Manzoni S.,Paediatric Rheumatology Unit | Collado P.,Hospital Universitario Severo Ochoa | Jousse-Joulin S.,Brest University Hospital Center | Naredo E.,Hospital General Universitario Gregorio Maranon | And 4 more authors.
Rheumatology (United Kingdom) | Year: 2014

Objective: The aim of this study was to investigate the current use of musculoskeletal US (MSUS) and the most relevant areas of interest for this imaging modality in paediatric rheumatology. Methods: A questionnaire was developed by the paediatric subgroup of the OMERACT US task force and e-mailed to the members of the main international paediatric rheumatology networks and societies. Responses were entered in an electronic database. Results were analysed quantitatively or summarized qualitatively in the case of open questions. Results: The overall response rate was 36% (262/719). The use of MSUS varied among members of the various networks/societies. MSUS was considered of high relevance for improvement of diagnostic skills, for the guidance of joint injections and for the assessment of specific joints, namely the hip, ankle, midfoot and wrist. It was considered useful for early detection of synovitis and in determining disease activity and disease remission. Conclusion: Although at present MSUS is not widely used by paediatric rheumatologists, there is considerable interest in this imaging technology among members of the international networks. The results of this survey suggest that the next objective in the research agenda should be the standardization of the assessment of joints in healthy children. This will then help differentiate pathological (i.e. synovitic) joints from normal joints. The initial target joints should be the hip, ankle, midfoot and wrist. MSUS training focused on the assessment of paediatric patients might be very important in implementing the use of this technique in clinical practice and research. © The Author 2013. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.


PubMed | Selcuk University, Gulhane Military Medical Faculty, Uludag University, Kanuni Sultan Suleyman Research and Training Hospital and 14 more.
Type: Journal Article | Journal: Clinical and experimental rheumatology | Year: 2015

To develop and assess the validity and reliability of an adherence scale concerning medical treatment in paediatric FMF patients.The Medication Adherence Scale in FMF Patients (MASIF) is a 18-item questionnaire that evaluates adherence to medication in four domains. Validation of the instrument was accomplished in paediatric FMF patients (aged 2-18 years) under medication at least for 6 months. The first step was to build up the scale through qualitative approach (with interviews using semi-structured questions). Validation analyses included assessment of feasibility, face and content validity; construct validity, internal consistency and test-retest reliability.One hundred and fifty patients with FMF were enrolled in the study. The mean age of the patients was 11.114.02 years and 48.7% of them were male. The MASIF was found to be feasible and valid for both face and content. It correlated with the Morisky Medication Adherence Scale as a gold standard thereby demonstrating good construct validity (r=0.515, p<0.001). Assessment of content validity identified four subscales. The internal consistency, Cronbachs alpha was 0.728. There was a positive and significant correlation between test and retest scores (r=0.843; p<0.001). Also, a significant correlation between parents and childrens reports (r=0.781, p<0.001).Based on these results, the use of this scale to assess and follow up the adherence to treatment in paediatric FMF patients under medical treatment is recommended.


Falcini F.,University of Florence | Ozen S.,Hacettepe University | Magni-Manzoni S.,Paediatric Rheumatology Unit | Candelli M.,University Cattolica Sacro Cuore | And 9 more authors.
Clinical and Experimental Rheumatology | Year: 2012

Objective: Kawasaki syndrome (KS) is an acute systemic vasculitis of unknown origin predominantly affecting young children. Early diagnosis is crucial to prevent cardiac complications. However, the differential diagnosis of patients with the incomplete or atypical form of the disease poses a heavy challenge for the paediatrician. Our aim was to evaluate the prevalence of incomplete and atypical cases among children with KS and to identify clinical and laboratory variables that may help differentiate incomplete and atypical KS from other febrile diseases at this age. Methods: We established an international registry to recruit patients with KS, including those with incomplete and atypical forms. The control group included age-matched febrile children admitted to the hospital with a variety of diseases mimicking KS. The aim was to define clinical or laboratory clues to help in the discrimination of incomplete and atypical KS patients from others. Results: Two hundred and twenty-eight patients with incomplete KS (78%) and atypical KS (22%) were compared to 71 children with other febrile diseases. Patients with incomplete and atypical KS presented a statistically significant higher frequency of mucosal changes, conjunctivitis, extremity abnormalities and perineal desquamation compared to the group of other febrile diseases. In addition, C-reactive protein and platelet counts were significantly higher in incomplete and atypical KS compared to the other group. Conclusion: This is the largest series of incomplete and atypical KS patients of non East-Asian ancestry: we suggest that in patients with the aforementioned clinical features and laboratory evidence of systemic inflammation in terms of increased C-reactive protein and platelet counts an echocardiogram should be performed and diagnosis of KS considered. © Copyright CLINICAL AND EXPERIMENTAL RHEUMATOLOGY 2012.


Martini G.,Paediatric Rheumatology Unit | Biscaro F.,Paediatric Rheumatology Unit | Boscaro E.,University of Padua | Calabrese F.,University of Padua | And 5 more authors.
BMC Musculoskeletal Disorders | Year: 2015

Background: Endothelial progenitor cells (EPC) promote angiogenesis and vascular repair. Though reduced EPC levels have been shown in rheumatoid arthritis, no study has so far evaluated EPCs in children with juvenile idiopathic arthritis (JIA). We aimed to study circulating EPCs in children with JIA, their relation to disease activity, and effects of anti TNF-α treatment. Methods: Circulating EPCs were quantified by flow cytometry based on CD34, CD133 and KDR expression in peripheral blood of 22 patients with oligoarticular JIA and 29 age-matched controls. EPCs were re-assessed in children with methotrexate-resistant oligo-extended JIA before and up to 12 month after initiation of anti-TNF-alpha therapy. Plasma concentrations of inflammatory and EPC-regulating factors were measured using a multiplex array. Confocal immunofluorescence was used to demonstrate EPCs in synovial tissues. Results: Children with active JIA showed a significant reduction of relative and absolute counts of circulating progenitor cells and EPCs compared to age-matched healthy controls. CD34+ cell levels were modestly and inversely correlated to disease activity. A strong inverse correlation was found between serum TNF-α and EPC levels. In 8 patients treated with anti TNF-α agents, the number of EPCs rose to values similar to healthy controls. CD34+KDR+ EPCs were found in the synovial tissue of JIA children, but not in control. Conclusions: Children with JIA have reduced levels of the vasculoprotective and proangiogenic EPCs. While EPCs may contribute to synovial tissue remodelling, EPC pauperization may indicate an excess cardiovascular risk if projected later in life. © 2015 Martini et al.; licensee BioMed Central.


Uziel Y.,Meir Medical Center | Uziel Y.,Tel Aviv University | Chapnick G.,Meir Medical Center | Chapnick G.,Tel Aviv University | And 7 more authors.
Clinical and Experimental Rheumatology | Year: 2012

Objective: To examine the changes in bone strength in a cohort of children with "growing pains" (GP) after 5 years follow-up and the correlation with pain outcome. Methods: Bone strength was measured by quantitative ultrasound. Subjects were 39 children with GP previously studied. Controls were normograms based on the measurement of bone speed of sound in 1085 healthy children. Current GP status was assessed by parental questionnaires. Bone strength was compared with pain outcome. Results: We examined 30/39 (77%) patients after 5 years. Bone strength was significantly increased when compared to the first study (Z score 0.65±1.77 vs. -0.62±0.90, p≥0.001). While overall there was no significant difference in the bone strength between the 16 (53%) patients whose GP resolved and the 14 (47%) who continued to have GP episodes (p=0.71), all 6 (20%) patients with a speed of sound Z-score ≥-1 continued to have GP (p=0.003). Conclusion: Our findings that pain improves in most patients parallel to the increase in bone strength may support the hypothesis of GP representing in some patients a local overuse syndrome. © Clinical and Experimental Rheumatology 2012.


PubMed | University of Padua and Paediatric Rheumatology Unit
Type: | Journal: BMC musculoskeletal disorders | Year: 2015

Endothelial progenitor cells (EPC) promote angiogenesis and vascular repair. Though reduced EPC levels have been shown in rheumatoid arthritis, no study has so far evaluated EPCs in children with juvenile idiopathic arthritis (JIA). We aimed to study circulating EPCs in children with JIA, their relation to disease activity, and effects of anti TNF- treatment.Circulating EPCs were quantified by flow cytometry based on CD34, CD133 and KDR expression in peripheral blood of 22 patients with oligoarticular JIA and 29 age-matched controls. EPCs were re-assessed in children with methotrexate-resistant oligo-extended JIA before and up to 12 month after initiation of anti-TNF-alpha therapy. Plasma concentrations of inflammatory and EPC-regulating factors were measured using a multiplex array. Confocal immunofluorescence was used to demonstrate EPCs in synovial tissues.Children with active JIA showed a significant reduction of relative and absolute counts of circulating progenitor cells and EPCs compared to age-matched healthy controls. CD34(+) cell levels were modestly and inversely correlated to disease activity. A strong inverse correlation was found between serum TNF- and EPC levels. In 8 patients treated with anti TNF- agents, the number of EPCs rose to values similar to healthy controls. CD34(+)KDR(+) EPCs were found in the synovial tissue of JIA children, but not in control.Children with JIA have reduced levels of the vasculoprotective and proangiogenic EPCs. While EPCs may contribute to synovial tissue remodelling, EPC pauperization may indicate an excess cardiovascular risk if projected later in life.

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