104 results on '"Frkovic, Marijan"'
Search Results
2. Clinical features, treatment and outcome of pediatric patients with severe cutaneous manifestations in IgA vasculitis: Multicenter international study
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Sestan, Mario, Kifer, Nastasia, Sozeri, Betul, Demir, Ferhat, Ulu, Kadir, Silva, Clovis A., Campos, Reinan T., Batu, Ezgi Deniz, Koker, Oya, Sapina, Matej, Srsen, Sasa, Held, Martina, Gagro, Alenka, Fonseca, Adriana Rodrigues, Rodrigues, Marta, Rigante, Donato, Filocamo, Giovanni, Baldo, Francesco, Heshin-Bekenstein, Merav, Giani, Teresa, Kataja, Janne, Frkovic, Marijan, Ruperto, Nicolino, Ozen, Seza, and Jelusic, Marija
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- 2023
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3. Whole exome sequencing in patients with childhood‐onset systemic lupus erythematosus: Results from a Croatian national study.
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Sestan, Mario, Arsov, Todor, Kifer, Nastasia, Frkovic, Marijan, Grguric, Danica, Ellyard, Julia, Cook, Matthew, Vinuesa, Carola G., and Jelusic, Marija
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SYSTEMIC lupus erythematosus ,MEDICAL genetics ,MEDICAL genomics ,GENETIC variation ,DRUG target - Abstract
The purpose of this study was to identify new and low‐frequency gene variants using whole exome sequencing (WES) in patients with childhood‐onset systemic lupus erythematosus (cSLE), that may be involved in the pathogenesis of SLE. We performed WES on selected 17 trios (in some cases including other informative family members) in which the proband presented with severe, atypical clinical features, resistance to conventional therapy, a family pattern of occurrence and/or syndromic characteristics. After performing WES and analysis of gene variants, 17 novel and/or low‐frequency variants were identified in 7 patients. One variant was classified as pathogenic (KMT2D, NM_003482.3:c.8626delC, predicted to truncate the protein p.(Gln2876Serfs*34)) and two as likely pathogenic according to the American College of Medical Genetics and Genomics classification guidelines (ADAR, NM_001111.3:c.2815A>G, predicted to encode p.(Ile939Val); BLK, NM_001715.2:c.211G>A, predicted to encode p.(Ala71Thr)). The other variants remain of uncertain significance at this point of time. WES is an important diagnostic and research instrument, producing a growing list of likely genes and gene variants that may be of relevance in the pathogenesis of cSLE and potentially point to novel therapeutic targets. [ABSTRACT FROM AUTHOR]
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- 2024
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4. Insight into the Interplay of Gd-IgA1, HMGB1, RAGE and PCDH1 in IgA Vasculitis (IgAV)
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Held, Martina, primary, Kozmar, Ana, additional, Sestan, Mario, additional, Turudic, Daniel, additional, Kifer, Nastasia, additional, Srsen, Sasa, additional, Gagro, Alenka, additional, Frkovic, Marijan, additional, and Jelusic, Marija, additional
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- 2024
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5. Comparison of EULAR/PRINTO/PReS Ankara 2008 and 2022 ACR/EULAR classification criteria for granulomatosis with polyangiitis in children.
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Akca, Ummusen Kaya, Batu, Ezgi Deniz, Jelusic, Marija, Calatroni, Marta, Bakry, Reima, Frkovic, Marijan, Vinšová, Nikol, Campos, Reinan T, Horne, AnnaCarin, Caglayan, Sengul, Vaglio, Augusto, Moroni, Gabriella, Emmi, Giacomo, Ghiggeri, Gian Marco, Koker, Oya, Sinico, Renato Alberto, Kim, Susan, Gagro, Alenka, Matucci-Cerinic, Caterina, and Çomak, Elif
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PREDICTIVE tests ,SCHOENLEIN-Henoch purpura ,MICROSCOPIC polyangiitis ,RETROSPECTIVE studies ,BEHCET'S disease ,COGAN syndrome ,GRANULOMATOSIS with polyangiitis ,PEDIATRICS ,MEDICAL records ,ACQUISITION of data ,TAKAYASU arteritis ,SENSITIVITY & specificity (Statistics) ,POLYARTERITIS nodosa ,CHILDREN - Abstract
Objective Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis. The 2022 ACR/EULAR-endorsed classification criteria for GPA was derived using data only from adult patients. We aimed to assess the performance of the ACR/EULAR classification criteria for GPA in paediatric patients and compare it with the EULAR/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 criteria for GPA. Methods Retrospective data of paediatric patients with GPA in 20 centres from 9 countries were evaluated. The diagnosis of GPA was made according to the expert opinion. The sensitivity, specificity, positive predictive value, and negative predictive value of the criteria sets were evaluated. Results The study included 77 patients with GPA and 108 controls [IgA vasculitis (n = 44), Takayasu's arteritis (n = 20), microscopic polyangiitis (n = 16), polyarteritis nodosa (n = 14), Behçet's disease (n = 12), eosinophilic granulomatosis with polyangiitis (n = 1) and Cogan's syndrome (n = 1)] with a median age of 17.8 and 15.2 years, respectively. Among patients with GPA, constitutional symptoms (85.7%) and ENT involvement (79.2%) were the most common presentations. In the GPA group, 73 patients fulfilled the Ankara 2008 criteria and 69 the ACR/EULAR classification criteria. Sensitivities of the Ankara 2008 criteria and the ACR/EULAR classification criteria were 94.8% and 89.6%, while specificities were 95.3% and 96.3%, respectively. No significant difference was found between sensitivities and specificities of both classification criteria (P = 0.229 and P = 0.733, respectively). Conclusion In children, both the ACR/EULAR and EULAR/PRINTO/PReS Ankara 2008 classification criteria for GPA perform well and similarly. [ABSTRACT FROM AUTHOR]
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- 2024
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6. Glutathione S-Transferase Gene Polymorphisms as Predictors of Methotrexate Efficacy in Juvenile Idiopathic Arthritis.
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Huljev Frkovic, Sanda, Jelusic, Marija, Crkvenac Gornik, Kristina, Rogic, Dunja, and Frkovic, Marijan
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JUVENILE idiopathic arthritis ,GENETIC polymorphisms ,DELETION mutation ,GLUTATHIONE ,METHOTREXATE - Abstract
Because of the unpredictable efficacy of methotrexate (MTX) in the treatment of juvenile idiopathic arthritis (JIA), the possibility of a favourable outcome is reduced in more than 30% of patients. To investigate the possible influence of glutathione S-transferase M1 (GSTM1) and T1 (GSTT1) gene deletion polymorphisms on MTX efficacy in patients with JIA, we determined these polymorphisms in 63 patients with JIA who did not achieve remission and 46 patients with JIA who achieved remission during MTX therapy. No significant differences were observed in the distribution of single GSTM1 or GSTT1 deletion polymorphisms or their combination between the two groups: 58.7% to 63.5%; p = 0.567, 17.4% to 22.2%; p = 0.502, and 13% to 12.7%; p = 0.966, respectively. Our results suggest that GSTM1 and GSTT1 deletion polymorphisms do not influence the efficacy of MTX in patients with JIA. Additional studies are required to determine the possible influence of GST deletion polymorphisms on MTX efficacy in patients with JIA. [ABSTRACT FROM AUTHOR]
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- 2024
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7. Association of Glutathione Transferase M1, T1, P1 and A1 Gene Polymorphism and Susceptibility to IgA Vasculitis.
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Juras, Ana, Crkvenac Gornik, Kristina, Held, Martina, Sestan, Mario, Turudic, Daniel, Sapina, Matej, Srsen, Sasa, Huljev Frkovic, Sanda, Frkovic, Marijan, Gagro, Alenka, and Jelusic, Marija
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GENETIC polymorphisms ,SINGLE nucleotide polymorphisms ,IMMUNOGLOBULIN A ,GLUTATHIONE transferase ,CHILD patients ,VASCULITIS - Abstract
Endothelial cell injury is a hallmark of IgA vasculitis (IgAV), possibly associated with various factors, including oxidative stress. Certain single nucleotide polymorphisms (SNPs) of glutathione S-transferases (GST) genes have been shown to increase susceptibility to oxidative stress. The objective of our study was to evaluate the gene polymorphisms of GSTM1, GSTT1, GSTP1, and GSTA1 in patients with IgAV. DNA was extracted from the blood of 124 children with IgAV and 168 age-matched healthy controls. A higher frequency of the GSTM1 null genotype was observed in patients with gastrointestinal (GI) system involvement compared to those without GI system involvement (51.5% vs. 28.6%, p = 0.011). Additionally, the GSTM1 null genotype was less prevalent (30.8% vs. 69.2%, p = 0.032), while the GSTP1 Val/Val genotype was significantly more prevalent in patients who developed urogenital complications (scrotal swelling) during the course of the disease (60% vs. 40%, p = 0.039). This study is the first to suggest an association between GSTM1 and GSTP1 polymorphisms and various phenotypes observed during the clinical course of IgAV in the pediatric population. However, it was performed on a national and likely single ethnic cohort, too small for definitive conclusions, so larger studies are needed to confirm this association. [ABSTRACT FROM AUTHOR]
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- 2024
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8. Serum Levels of S100A8/A9 as a Biomarker of Disease Activity in Patients with IgA Vasculitis
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Srsen, Sasa, primary, Held, Martina, additional, Sestan, Mario, additional, Kifer, Nastasia, additional, Kozmar, Ana, additional, Supe Domic, Daniela, additional, Benzon, Benjamin, additional, Gagro, Alenka, additional, Frkovic, Marijan, additional, and Jelusic, Marija, additional
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- 2024
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9. Erythrocyte Glutathione S-Transferase Activity as a Sensitive Marker of Kidney Function Impairment in Children with IgA Vasculitis
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Frkovic, Marijan, primary, Turcic, Ana, additional, Gagro, Alenka, additional, Srsen, Sasa, additional, Frkovic, Sanda Huljev, additional, Rogic, Dunja, additional, and Jelusic, Marija, additional
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- 2024
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10. Involvement of M1-Activated Macrophages and Perforin/Granulysin Expressing Lymphocytes in IgA Vasculitis Nephritis
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Laskarin, Gordana, primary, Babarovic, Emina, additional, Kifer, Nastasia, additional, Bulimbasic, Stela, additional, Sestan, Mario, additional, Held, Martina, additional, Frkovic, Marijan, additional, Gagro, Alenka, additional, Coric, Marijana, additional, and Jelusic, Marija, additional
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- 2024
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11. HLA Polymorphisms and Clinical Manifestations in IgA Vasculitis
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Held, Martina, primary, Stingl Jankovic, Katarina, additional, Sestan, Mario, additional, Sapina, Matej, additional, Kifer, Nastasia, additional, Srsen, Sasa, additional, Frkovic, Marijan, additional, Gagro, Alenka, additional, Grubic, Zorana, additional, and Jelusic, Marija, additional
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- 2024
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12. Quality of life in children suffering from juvenile idiopathic arthritis-associated uveitis
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Sestan, Mario, Grguric, Danica, Sedmak, Maja, Frkovic, Marijan, Kifer, Nastasia, Grubic, Marina, Peric, Sanja, Vukojevic, Nenad, Potocki, Kristina, and Jelusic, Marija
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- 2020
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13. Systemic lupus erythematosus: A new autoimmune disorder in Kabuki syndrome
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Arsov, Todor, Sestan, Mario, Cekada, Nastasia, Frkovic, Marijan, Andrews, Dan, He, Yuke, Shen, Nan, Vinuesa, Carola G., and Jelusic, Marija
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- 2019
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14. Comparison of EULAR/PRINTO/PReS Ankara 2008 and 2022 ACR/EULAR Classification Criteria for Granulomatosis with Polyangiitis in Children
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Kaya Akca, Ummusen, primary, Batu, Ezgi Deniz, additional, Jelusic, Marija, additional, Calatroni, Marta, additional, Bakry, Reima, additional, Frkovic, Marijan, additional, Vinšová, Nikol, additional, Campos, Reinan T, additional, Horne, AnnaCarin, additional, Caglayan, Sengul, additional, Vaglio, Augusto, additional, Moroni, Gabriella, additional, Emmi, Giacomo, additional, Ghiggeri, Gian Marco, additional, Koker, Oya, additional, Sinico, Renato Alberto, additional, Kim, Susan, additional, Gagro, Alenka, additional, Matucci-Cerinic, Caterina, additional, Çomak, Elif, additional, Ekici Tekin, Zahide, additional, Arslanoglu Aydin, Elif, additional, Heshin-Bekenstein, Merav, additional, Acar, Banu Celikel, additional, Gattorno, Marco, additional, Akman, Sema, additional, Sozeri, Betul, additional, Palmblad, Karin, additional, Al-Mayouf, Sulaiman M, additional, Silva, Clovis Artur, additional, Doležalová, Pavla, additional, Merkel, Peter A, additional, and Ozen, Seza, additional
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- 2023
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15. Il‐17 inhibitor in patient with juvenile psoriasis and primary immunodeficiency.
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Sestan, Mario, Frkovic, Marijan, Ceovic, Romana, Murat‐Susic, Slobodna, and Jelusic, Marija
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PRIMARY immunodeficiency diseases , *INTERLEUKIN-17 , *INTERSTITIAL lung diseases , *PSORIASIS , *SARCOIDOSIS , *DRUG side effects , *COMMON variable immunodeficiency - Abstract
This article discusses a case study of a 15-year-old patient with juvenile psoriasis and primary immunodeficiency. The patient presented with erythematosquamous skin lesions, nail abnormalities, ankle pain and swelling, cough, and fever. Extensive investigations revealed a diagnosis of psoriasis vulgaris, severe psoriasis, and arthritis of the left ankle. The patient also exhibited hypogammaglobulinemia and a decreased count of switched memory B-cells, leading to a diagnosis of common variable immunodeficiency (CVID). Treatment with a biological agent, secukinumab, targeting interleukin-17 (IL-17), resulted in significant improvement in skin lesions and other symptoms. The case highlights the complexities of managing treatment for patients with a combination of autoimmune and immunodeficiency disorders. [Extracted from the article]
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- 2024
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16. Successful use of adalimumab as a conservative treatment for bilateral knee lipoma arborescens in patient with psoriatic juvenile idiopathic arthritis – case report and review of literature
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Frkovic, Marijan, primary, Kujundzic, Magdalena, additional, Cavka, Mislav, additional, and Jelusic, Marija, additional
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- 2022
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17. 2019 ACR/EULAR classification criteria and therapy in predicting organ damage accrual in patients with childhood-onset systemic lupus erythematosus: A retrospective study over the last 29 years
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Kifer, Nastasia, primary, Sestan, Mario, additional, Frkovic, Marijan, additional, Kifer, Domagoj, additional, Kozmar, Ana, additional, Padjen, Ivan, additional, Potocki, Kristina, additional, Anic, Branimir, additional, Batinic, Drago, additional, Malcic, Ivan, additional, and Jelusic, Marija, additional
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- 2022
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18. The paediatric vasculitis activity score (PVAS) and proteinuria in IgAV nephritis: is there an association with different histologic findings?
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Kifer, Nastasia, Sestan, Mario, Held, Martina, Kifer, Domagoj, Srsen, Sasa, Gudelj Gracanin, Ana, Heshin-Bekenstein, Merav, Giani, Teresa, Cimaz, Rolando, Frkovic, Marijan, Bulimbasic, Stela, Gagro, Alenka, Coric, Marijana, and Jelusic, Marija
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IgA vasculitis ,IgA vasculitis nephritis ,PVAS - Abstract
Introduction: IgA vasculitis (IgAV) is usually self-limiting with a favorable prognosis. However, the development of nephritis (IgAVN) can lead to chronic kidney disease and kidney biopsy has been a continued standard in determining the severity of IgAVN. The association between the disease activity, as well as laboratory parameters descibing kidney function, and different histologic classifications used for IgAVN are still left unclear. Objectives: To determine whether there is an association between histologic variables, measures of disease activity (PVAS) and markers of kidney function. Methods: Patients included were diagnosed with IgAV and IgAVN based on EULAR/PRINTO/PRES criteria in the period from 2003 to 2021. Their renal biopsy findings were examined using light microscopy, immunofluorescence, and electron microscopy analyses. Four classifications were used: ISKDC, Haas classification, Oxford classification, and SQC classification. PVAS was determined at the time of diagnosis. Results: The study included 67 patients, with the median (range) age of 10.8 (3.1-28.5) years at the diagnosis. Fifty-eight percent of patients were male, with a male to female ratio of 1.4:1. The median time from IgAV diagnosis to IgAV nephritis was 5 (0-270) days. The median time from the onset of nephritis to kidney biopsy was 30 (2-2555) days. Laboratory parameters were tested for association with all four classifications. Twenty- four hours protein excretion has shown statistically significant correlation with higher grade in Oxford classification (b= 0.58 ± 0.12, p
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- 2022
19. Association between glutathione S-transferase (GST) M1,T1 and A1 polymorphisms and IgA vasculitis: a pilot study
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Juras, Ana, Held, Martina, Sestan, Mario, Batnozic Varga, Mateja, Kifer, Nastasia, Srsen, Sasa, Gagro, Alenka, Frkovic, Marijan, Huljev Frkovic, Sanda, Jelusic, Marija, and Crkvenac Gornik, Kristina
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IgA vasculitis ,GST ,polymorphism - Abstract
Introduction: IgA vasculitis (IgAV) is the most common childhood vasculitis. Considering the clinical heterogeneity, genetic factors might play a role in pathogenesis of IgAV. Glutathione S- transferase (GST) are members of a multigene family of metabolic enzymes divided into four major subfamilies designated as GSTα (GSTA1), GSTμ (GSTM1), GSTθ (GSTT1) and GST π (GSTP1), act as cell housekeepers protect cells against oxidative stressors in the environment by detoxifying a wide variety of potentially toxic and carcinogenic electrophiles. Deletions in GSTs lead to reduction in detoxification enzymatic activity. It was identified that detoxification effects modified by GSTs polymorphism possibly can aggravate the susceptibility to diseases. Objectives: To investigate the GSTA1, GSTM1 and GSTT1 genes polymorphism and their influence to susceptibility for IgAV. Methods: Clinical data were collected from four Croatian tertiary centers for pediatric rheumatology. GSTA1, GSTM1, and GSTT1 polymorphisms were detected in patients and controls. DNA was isolated from whole blood using the QIAGEN QIAamp kit. GSTA1 (-69C>T) was examined by the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method whereas the GSTM1 and GSTT1 were determined by the PCR method. Results: Pilot study included 107 patients diagnosed with IgAV, of whom 56 girls and 51 boys, with median age at the time of diagnosis 6.25 (4.5-8.0) years, as well as 75 sex and age- matched controls. All patients had purpuric rash, 75, 7% had arthralgia or arthritis, 36, 5% had gastrointestinal involvement, while 31, 7% patients developed IgA vasculitis nephritis (IgAVN). The frequencies of GSTM1 (−) null allele and GSTT1 null (−) allele in IgAV patients were 56, 1% and 26, 2% respectively. There was no statistically significant difference in the null genotype distribution of GSTM1 and GSTT1 between groups (CI 0.49-1.62, OR 0.89, p=0.714 ; CI 0.35- 1.44, OR 0.70, p=0.335). The frequency of GSTA1 C/C, GSTA1 C/T and GSTA1 T/T genotypes in IgAV patients were 36, 5%, 44, 8% and 18, 7% respectively. There was no statistically significant differences in genotype frequencies between patients and controls (CI 0.83-3.00, OR 1.38, p=0.167 ; CI 0.33-1.09, OR 0.60, p=0.09 ; CI 0.55-2.65, OR 1.20, p=0.639). Patients with gastrointestinal involvement had statistically significant difference in the null genotype distribution of GSTM1 compared with patients without gastrointestinal involvement (CI 0.15- 0.81, OR 0.35, p=0.014). Conclusion: Our pilot study provides evidence that the examinated polymorphisms were not associated with the increase individual susceptibility for IgAV, although GSTM1 genotype proved to have effect on gastrointestinal involvement in IgAV. For precise evaluation of results it is necessary to include larger study populations, however this study offers some essential information for further research. SUPPORT: Croatian Science Foundation IP-2019-04- 8822.
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- 2022
20. Software Quality Control (SQC) and Oxford classifications predict poor renal outcome better than The International Study of Kidney Disease in Children (ISKDC) and Haas in patients with IgAV nephritis: a multicenter study
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Kifer, Nastasia, Bulimbašić, Stela, Šestan, Mario, Held, Martina, Kifer, Domagoj, Sršen, Saša, Gudelj Gračanin, Ana, Heshin-Bekenstein, Merav, Giani, Teresa, Cimaz, Rolando, Gagro, Alenka, Frkovic, Marijan, Ćorić, Marijana, and Jelušić, Marija
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IgA vasculitis ,Henoch-Schonlein purpura ,nephritis ,pathology - Abstract
Introduction Several histologic classifications are used in the evaluation of IgA vasculitis nephritis (IgAVN), however, to date, no studies have determined which one has the strongest association with the severity of IgAVN and, as a consequence, its outcomes. Materials and methods Patients included in the study were diagnosed with IgAV and IgAVN in seven tertiary university medical centers in Croatia, Italy and Israel. The International Study of Kidney Disease in Children (ISKDC), Haas, Oxford, and Software Quality Control (SQC) classifications were used in the analysis and description of renal biopsy. Time from biopsy to outcome evaluation was a statistically significant factor in outcome prediction that was used to define the base model, and was a covariate in all the tested models. Results Sixty-seven patients were included in this study. The SQC classification proved to be the best one in outcome prediction, followed by the Oxford classification. The ISKDC and Haas classifications could not predict renal outcome. The Oxford parameters for mesangial hypercellularity and tubular atrophy, as well as the SQC parameters for cellular crescents showed an independent statistically significant contribution to outcome prediction. High level of twenty-four hour protein excretion was associated with a higher grade in the Oxford, SQC and ISKDC classifications. Endocapillary proliferation was positively associated with the Pediatric Vasculitis Activity Score (PVAS) at diagnosis, while tubular atrophy was negatively associated. Conclusion The SQC, followed by the Oxford classification were found to provide the best classifications of renal biopsy analysis in patients to predict the outcome in patients with IgAVN. Cellular crescents, mesangial hypercellularity and tubular atrophy showed significant contributions, indicating that active and chronic variables should be included in the estimation.
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- 2022
21. 445 Determining the effectiveness of systemic immunomodulatory therapy in the treatment of patients with juvenile idiopathic arthritis associated uveitis depending on the chosen outcome measures
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Peric Sanja, Jelusic Marija, Vukojevic Nenad, Ivkic Petra Kristina, Frkovic Marijan, Held Martina, Sestan Mario, Galiot Delic Martina, Barisic Kutija Marija, Knezevic Josip, and Kifer Nastasia
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medicine.medical_specialty ,business.industry ,Internal medicine ,medicine ,Outcome measures ,Arthritis ,Juvenile ,medicine.disease ,business ,Uveitis - Published
- 2021
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22. 446 Single nucleotide polymorphisms of genes HMGB1 and AGER and its association with clinical features of IgA vasculitis
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Held, Martina, primary, Varga, Mateja Batnozic, additional, Sestan, Mario, additional, Sapina, Matej, additional, Kifer, Nastasia, additional, Grguric, Danica, additional, Gornik, Kristina Crkvenac, additional, Frkovic, Marijan, additional, Arvaj, Nena, additional, Wagner, Jasenka, additional, and Jelusic, Marija, additional
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- 2021
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23. 451 Association between gastrointestinal manifestations and the risk of renal disease in children with IgA vasculitis
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Sestan, Mario, primary, Kifer, Nastasia, additional, Srsen, Sasa, additional, Ovuka, Aleksandar, additional, Varga, Mateja Batnozic, additional, Sapina, Matej, additional, Held, Martina, additional, Ban, Maja, additional, Kozmar, Ana, additional, Coric, Marijana, additional, Bulimbasic, Stela, additional, Crkvenac, Kristina, additional, Milosevic, Danko, additional, Frkovic, Marijan, additional, Gagro, Alenka, additional, and Jelusic, Marija, additional
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- 2021
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24. 456 M2 macrophage infiltration and Henoch-Schönlein’s purpura nephritis
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Kifer, Nastasia, primary, Sestan, Mario, additional, Held, Martina, additional, Kifer, Domagoj, additional, Frkovic, Marijan, additional, Babarovic, Emina, additional, Bulimbasic, Stela, additional, Coric, Marijana, additional, Gagro, Alenka, additional, Laskarin, Gordana, additional, and Jelusic, Marija, additional
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- 2021
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25. 453 Determining the effectiveness of systemic immunomodulatory therapy in the treatment of patients with juvenile idiopathic arthritis associated uveitis depending on the chosen outcome measures
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Kutija, Marija Barisic, primary, Sestan, Mario, additional, Peric, Sanja, additional, Kifer, Nastasia, additional, Ivkic, Petra Kristina, additional, Delic, Martina Galiot, additional, Knezevic, Josip, additional, Held, Martina, additional, Frkovic, Marijan, additional, Jelusic, Marija, additional, and Vukojevic, Nenad, additional
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- 2021
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26. 452 The impact of systemic immunomodulatory therapy on the intraocular inflammation and the need for topical glucocorticoid therapy in patients with juvenile idiopathic arthritis-associated uveitis
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Kutija, Marija Barisic, primary, Sestan, Mario, additional, Peric, Sanja, additional, Kifer, Nastasia, additional, Ivkic, Petra Kristina, additional, Delic, Martina Galiot, additional, Jandrokovic, Sonja, additional, Held, Martina, additional, Frkovic, Marijan, additional, Jelusic, Marija, additional, and Vukojevic, Nenad, additional
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- 2021
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27. 442 The influence of HMGB1 gene (RS41369348) polymorphism on the susceptibility and clinical features of patients with IgAV
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Varga, Mateja Batnozic, primary, Sestan, Mario, additional, Wagner, Jasenka, additional, Crkvenac, Kristina, additional, Kifer, Nastasia, additional, Frkovic, Marijan, additional, Stefinovec, Laura, additional, Grguric, Danica, additional, Puseljic, Silvija, additional, and Jelusic, Marija, additional
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- 2021
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28. Application of computerized color telethermography in 225 children and adolescents with Raynaud's phenomenon
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Skreb, Nikola, Held, Martina, Sestan, Mario, Kifer, Nastasia, Turudic, Daniel, Frkovic, Marijan, Stipic, Jagoda, and Jelusic, Marija
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Raynaud's phenomenon ,thermography ,children ,adolescents - Abstract
Introduction: Computerized color telethermography (CCTT) is an established diagnostic procedure in clinical practice that is used for assessment and follow-up of patients with microcirculatory disorders in adult population, such as Raynaud's phenomenon (RP). However, CCTT is still not validated in pediatric patients with RP. Objectives: To analyze the CCTT findings in children and adolescents with clinically suspicious RP and its relationship with age, gender and season as well as with biochemical and immunological laboratory findings. Methods: Retrospective study included pediatric patients with suspected RP who underwented CCTT diagnostic procedure at the University Hospital Centre Zagreb from 2010 to 2019. Laboratory findings included inflammatory parameters, complete blood count, renal function tests and immunological tests (ANA, ENA screen, ANCA, RF, antiphospholipid antibodies, serum IgG and complement levels). Differences between categorical variables were examined using Chi- Square test and among numerical using t-test, followed by logistical regression analysis. Results: Out of 225 patients with suspected RP, 176 were females (78.2%) and 49 were males (21.7%) giving a female to male ratio bigger than 3:1. In 44 patients (19, 6%) CCTT was compatible with the diagnosis of primary RP, 68 patients (30, 2%) were classified as secondary RP, whereas 27 patients (12%) had CCTT results which where considered unspecific. In 86 patients (38, 2%) CCTT findings were normal. Among the patients classificated as secondary RP using CCTT, the most of them, 28 (41.2%), were diagnosed with juvenile idiopathic arthritis, while 26 (38.2%) had no evident other disease. The median (range) age at the time of performing CCTT was 15.16 (13.50-16.75) years. According to their age, patients were divided into 3 groups: childhood (3-10 years), early adolescence (11-14 years) and late adolescence (15-18 years). The most of them were in late adolescence group (53, 8%) and these patients were 2.4 times more likely to be diagnosed with primary RP (OR 0.41, CI 0.18-0.92, p=0.03 for early adolescence group). Seasonal influence was a statistically significant factor in CCTT confirmation of the primary RP, since the largest number of patients with primary RP were diagnosed during the winter. If CCTT was performed in the winter, there was 7.25 times higher chance to confirm the primary RP with CCTT compared to the spring time (OR 7.25, CI 2.25-23.25, p=0.03). However, such influence was not observed for secondary RP. Patients diagnosed as secondary RP on CCTT had statistically significantly lower leukocyte (p=0.03) and platelet count (p=0.04), as well as C3 levels (p=0.008), but higher creatinine levels (p=0.008) in comparison with patients with normal CCTT findings. Concerning the immunological findings, it was shown that females, regardless of age, with positive ENA screen had 4 times higher risk to be diagnosed with secondary RP (OR 0.25, CI 0.06-0.95, p=0.04 for females with negative ENA). Conclusion: Patients with secondary RP in whom the underlying systemic disease had not yet manifested had the greatest benefit from CCTT. In these patients, no other diagnostic method can replace CCTT. In patients with suspected primary RP, it is best to perform CCTT in the winter months. We observed that female adolescents were referred to CCTT more often and that regardless of age females with positive ENA screen were more likely to be diagnosed as secondary RP on CCTT.
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- 2021
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29. DiGeorge syndrome; Juvenile idiopathic arthritis; Tumor necrosis factor-alpha inhibitor; Adverse effects
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Sestan, Mario, Kifer, Nastasia, Frkovic, Marijan, Laskarin, Ana-Marija, and Jelusic, Marija
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musculoskeletal diseases ,DiGeorge syndrome ,Juvenile idiopathic arthritis ,Tumor necrosis factor-alpha inhibitor ,Adverse effects ,immune system diseases ,hemic and lymphatic diseases ,skin and connective tissue diseases - Abstract
Authors present a response to therapy followed by adverse effects in a 21-year-old patient with DGS and hypogammaglobulinemia suffering from polyarticular type of JIA, treated simultaneously with intravenous immunoglobulins (IVIGs) and tumor necrosis factor-alpha inhibitor (TNFi).
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- 2020
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30. Gastrointestinal involvement and its association with the risk for nephritis in IgA vasculitis
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Sestan, Mario, primary, Kifer, Nastasia, additional, Frkovic, Marijan, additional, Sapina, Matej, additional, Srsen, Sasa, additional, Batnozic Varga, Mateja, additional, Ovuka, Aleksandar, additional, Held, Martina, additional, Gudelj Gracanin, Ana, additional, Kozmar, Ana, additional, Bulimbasic, Stela, additional, Coric, Marijana, additional, Laskarin, Gordana, additional, Gagro, Alenka, additional, and Jelusic, Marija, additional
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- 2021
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31. P182 Organ damage in croatian cohort of patients with childhood onset systemic lupus erythematosus
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Kifer, Nastasia, primary, Sestan, Mario, additional, Hosticka, Emilija, additional, Novoselec, Maja, additional, Varga, Mateja Batnozic, additional, Padjen, Ivan, additional, Frkovic, Marijan, additional, Kifer, Domagoj, additional, Anic, Branimir, additional, Batinic, Drago, additional, Potocki, Kristina, additional, Malcic, Ivan, additional, and Jelusic, Marija, additional
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- 2020
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32. Adverse effects of TNF inhibitor in a patient with DiGeorge syndrome and juvenile idiopathic arthritis
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Sestan, Mario, primary, Kifer, Nastasia, additional, Frkovic, Marijan, additional, Laskarin, Ana-Marija, additional, and Jelusic, Marija, additional
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- 2020
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33. Persistence and Severity of Cutaneous Manifestations in IgA Vasculitis Is Associated with Development of IgA Vasculitis Nephritis in Children.
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Sestan, Mario, Srsen, Sasa, Kifer, Nastasia, Sapina, Matej, Batnozic Varga, Mateja, Ovuka, Aleksandar, Held, Martina, Kozmar, Ana, Frkovic, Marijan, Laskarin, Gordana, Gagro, Alenka, and Jelusic, Marija
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CUTANEOUS manifestations of general diseases ,IMMUNOGLOBULIN A ,PARTIAL thromboplastin time ,VASCULITIS ,NEPHRITIS ,SCHOENLEIN-Henoch purpura ,CHILDREN with cerebral palsy - Abstract
Background/Objective: The purpose of this study was to evaluate the prevalence and severity of skin involvement in children with IgA vasculitis (IgAV) and its relationship with clinical and biochemical parameters and the risk of developing IgA vasculitis nephritis (IgAVN), the only cause of long-term morbidity and the main prognostic factor in IgAV patients. Methods: This national multicenter retrospective study included 611 patients under the age of 18 years with IgAV referred to five Croatian tertiary hospitals between 2009 and 2019. Patient data were collected from a database with systematic analysis of IgAV patients in the Croatian population. Results: Among the 611 children, 205 (33.55%) had purpura on the lower extremities, in 207 (33.88%) the rash extended on the trunk, in 149 (24.39%) it extended to the upper extremities, in 32 (5.24%) the rash was generalized, while 15 (2.47%) had the most severe skin symptoms: bullae, ulcerations, and necroses. IgAVN developed in 130 (21.28%) and persistent IgAVN (present for >3 months) in 48 (7.86%) children. Multivariate logistic regression found that presence of ulcerations and necroses (OR 3.20 [95% CI 1.03–9.91]), persistent purpura (OR 2.89 [95% CI 1.71–4.88]), and higher age (OR 1.16 [95% CI 1.09–1.23]) were significant predictors of IgAVN, whereas persistent purpura (OR 20.11 [95% CI 1.09–372.52]), male sex (OR 3.32 [95% CI 1.13–9.80]), and higher age (OR 1.15 [95% CI 1.00–1.30]) were predictors of persistent IgAVN. Among the laboratory parameters, higher serum urea (OR 1.43 [95% CI 1.03–2.00]) and reduction in activated partial thromboplastin time (OR 0.83 [95% CI 0.74–0.93]) were shown to have a significant impact on increasing the risk of persistent IgAVN. Conclusion: With increasing severity and duration of cutaneous manifestations in IgAV, the risk of developing IgAVN increases, making the prognosis worse, with a greater likelihood to need more aggressive treatment. [ABSTRACT FROM AUTHOR]
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- 2022
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34. QUALITY OF LIFE IN JUVENILE IDIOPATHIC ARTHRITIS-ASSOCIATED UVEITIS: DO WE NEED A NEW ASSESSMENT QUESTIONNAIRE?
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Sestan, Mario, Grguric, Danica, Sedmak, Maja, Kifer, Nastasia, Frkovic, Marijan, Peric, Sanja, Potocki, Kristina, Vukojevic, Nenad, and Jelusic, Marija
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Juvenilni idiopatski artritis ,uveitis ,kvaliteta života ,JAMAR - Abstract
Background: Uveitis, the most common extra- articular manifestation in juvenile idiopathic arthritis (JIA), occurs in 10-20% of patients. Although prognosis of uveitis within JIA (JIA- U) improved, complications still cause severe impairment of visual function in 25-33% of children that affects their psychophysical and psychosocial development and quality of life (QoL). Objectives: To study the QoL and it’s dimensions in children suffering from JIA-U as well as to investigate is there any difference in childhood’s and parent’s perception of disease between the group of children with JIA- U and children with JIA without uveitis. Methods: The study included 42 children with JIA and their parents. Patients were divided into two groups. The first consisted of 21 children with JIA-U and the second of 21 children with JIA and no uveitis. Both groups of patients and their parents filled the Juvenile Arthritis Multidimensional Assessment Report questionnaire (JAMAR) for monitoring and assessing the health status of children with JIA. The variables used to test differences were: QoL, functional ability, pain level, disease activity estimation, and current emotional state of the child. The significance of differences between groups of children and parents was verified by the independent-samples t-test. The Pearson correlation coefficient was used for measurement of the strength of the linear relationship between variables. Results: There were no statistically significant differences in the JIA-U group and the control group in either of the examined variables. Although there is a tendency of higher scores in children with JIA- U, which indicates their worse functioning, higher pain intensity and worse current emotional state, these differences were not statistically significant. Two groups did not differ significantly in the assessment of their own overall functional ability, which was associated with experienced pain intensity. Stronger pain intensity was associated with dysfunction (r = 0.642, p
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- 2019
35. Periodic fever syndrome with novel TRNT1 variant - possible cause of TRNT1 deficiency or just an incidental finding?
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Sestan Mario, Arsov Todor, Kifer Nastasia, Frkovic Marijan, Vinuesa Carola, and Jelusic Marija
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TRNT1 deficijencija ,sindrom periodičnih vrućica ,sekvencioniranje cijelog egzoma - Abstract
Introduction: Biallelic pathogenic variants in TRNT1 gene, coding for the enzyme transfer RNA nucleotidyltransferase 1, cause TRNT1 deficiency with various clinical manifestations of autoinflammation, autoimmunity and immunodeficiency, including the syndrome of congenital sideroblastic anaemia with immunodeficiency, periodic fevers and developmental delay (SIFD). Objectives: Authors present a case of a child with recurrent fevers and epilepsy with de novo heterozygous mutation in TRNT1 gene of uncertain role in disease pathogenesis, successfully treated with TNFα inhibitor. Methods: Retrospective analysis of patient medical data and genomic testing using whole exome sequencing (WES). Results: Three months old female infant was referred to our Department with right-sided hemiconvulsions and dysrhythmic EEG changes treated with valproic acid. Subsequently, she had seizures on several occasions successfully treated with diazepam. Since 11 months of age, the child has had periodic protracted febrile episodes. Seven months after the onset of fever, she developed intermittent erythema accompanied with swelling of both hands, feet, knees and face. Subsequently, episodes of fever recurred in an irregular rhythm. The patient has been showing signs of progressive fatigue and severe facial erythema unrelated to febrility. Her physical exam was unremarkable apart from occasional facial erythema. Laboratory findings were normal, except for the long-lasting microcytic anemia (the lowest hemoglobin was 85 g/L) and elevated C-reactive protein and erythrocyte sedimentation rate during the febrile episodes. Comprehensive laboratory, microbiological and immunological examination failed to establish underlying diagnosis. Bone marrow examinations were normal. The frequent and prolonged febrile episodes were successfully treated with short- term methylprednisolone, however this treatment failed to prevent the occurrence of new episodes. To ameliorate fevers, TNFα inhibitor (etanercept) was consequently introduced in therapy at 5 years of age. Over the four month period after the initiation of TNFα inhibitor, the child had no further febrile episodes or seizures and there was an improvement in appetite and general condition. It is planned to reduce the dose of antiepileptics. Genomic testing using trio WES identified a de novo heterozygous TRNT1 variant, NM_182916.2 (TRNT1_v001): c.448C>T, p.(R150C), predicted to be pathogenic with high CADD score. Previous cases of TRNT1 deficiency have been described with bialellic TRNT1 pathogenic variants and analyses are underway to elucidate whether this finding could establish the underlying diagnosis. At present we hypothesize that this could be a case of unidentified second intronic TRNT1 variant or that this TRNT1 variant could have a dominant negative effect. In this respect, it should be noted that in our patient we have identified some but not other features of SIFD. Conclusion: Clinical manifestations associated with TRNT1 deficiency have a broad spectrum with significant clinical heterogeneity, which makes it difficult for rapid clinical recognition. Although the WES is as a powerful tool for the diagnosis of clinically unrecognized genetic conditions, there are still unresolved cases that pose a challenge in routine clinical practice.
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- 2019
36. Henoch-Schonlein purpura nephritis in Croatian children: a retrospective study in five tertiary care centers
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Batnozic Varga, Mateja, Cekada, Nastasia, Sestan, Mario, Srsen, Sasa, Ruzman, Lucija, Zaninovic, Maja, Ovuka, Aleksandar, Ozdanovac, Ivana, Pecnjak, Marija, Kifer, Domagoj, Frkovic, Marijan, Gagro, Alenka, and Jelusic, Marija
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Henoch-Schönlein purpura ,nephritis ,kidney biopsy - Abstract
Introduction: Henoch-Schönlein purpura nephritis (HSPN) is the most severe complication of Henoch- Schönlein purpura (HSP) that can occur at any time of the disease process and includes isolated microscopic or macroscopic hematuria, mild or heavy proteinuria with or without nephrotic syndrome, renal failure and hypertension. Objectives: To determine a possible prognostic factor for earlier HSPN onset, to explore indications for kidney biopsy according to urine analysis and the correlation between 24-h urinary protein levels and biopsy findings, as well as biopsy findings and patient outcome. Methods: The cross-sectional study included all children with HSPN diagnosed by EULAR/PRES/PRINTO criteria from 2009 to 2017 at 5 tertiary care centres in Croatia. Results: Out of 540 patients diagnosed with HSP, 91 children who developed HSPN (16.85%) were included. The patient population with HSPN included 52 boys (57.14%) and 39 girls (42.86%). Median (range) age of HSP diagnosis was 8 years (1.5-17.5) and from the HSP diagnosis to HSPN onset was 1.5 (0- 60) months. Median (range) follow-up time was 44 (4-167) months. Nephritis was present in 18.68% of cases at the HSP onset. No statistically significant difference or correlation was found between the time of HSP diagnosis to HSPN onset and gender, age, purpura distribution, joint and gastrointestinal involvement. Kidney biopsy was done in 26 patients (28.57%). Among them, 3 patients had isolated persistent hematuria, 3 had isolated proteinuria and 20 patients had both hematuria and proteinuria. Median (range) 24-h urinary protein levels in patients who underwent biopsy was 1.28 (0-7.46) g/dU. The leading indication for biopsy was simultaneous hematuria and proteinuria (p1g/dU. No significant difference was found in patient outcome for different biopsy findings (p=0.214). Conclusion: Simultaneous hematuria and proteinuria was a statistically significant factor for kidney biopsy. However, while isolated proteinuria was not the sole determining factor, excessive levels of 24-h urinary proteins should be taken in consideration. Due to the small number of patients and no uniform classification generally used in grading biopsy findings, a statistically significant difference in regard to outcome could not be confirmed, indicating the need for both in future research.
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- 2018
37. Disease activity and organ damage in patient with childhood-onset systemic lupus erythematosus, from childhood to adulthood: a retrospective study over the last 25 years
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Hosticka, Emilija, Novoselec, Maja, Sestan, Mario, Cekada, Nastasia, Frkovic, Marijan, Padjen, Ivan, Sentic, Mirna, Anic, Branimir, and Jelusic, Marija
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childhood-onset systemic lupus erythematosus ,SLEDAI 2K ,SDI - Abstract
Background Although clinical symptoms and immunological findings are common in both children and adults with systemic lupus erythematosus (SLE), children generally have a more severe clinical presentation at the time of diagnosis with a larger number of affected organs, a much more aggressive clinical course and greater chance of developing organ damage over time. Objectives To compare the SLEDAI-2K disease activity index in patients with SLE at the time of diagnosis with SLEDAI-2K in the same patients in adulthood and to compare of the SLICC/ACR damage index (SDI) in patients with cSLE at the last follow up in childhood with SLICC/ACR of the same patients in adulthood. Methods This retrospective study included children who were diagnosed with cSLE, according to the ACR 1997 and SLICC 2012 criteria, in the period from 1991–2016 at the Referral Centre for Paediatric and Adolescent Rheumatology Republic of Croatia, Department of Paediatrics, University Hospital Centre Zagreb and who by the end of March 2017 reached the age of majority at 18 and continued their treatment at the Department of Internal Medicine, University Hospital Centre Zagreb. Results Out of 95 children with cSLE, 48 patients (42 females and 6 males) who attained the age of majority, were included in the study. Mean age at the time of diagnosis was 13.5 years (range 6–18), and the mean disease duration was 11 years. Mean SLEDAI- 2K was 19, 25 (range 0–42) in childhood and 7125 (range 0– 30) in adulthood. In adulthood, thirty-two patients (66.67%) showed improvement, three (6.25%) disease progression, six (12.5%) had the same disease activity and seven patients (14.58%) were in remission. 13 children (27%) had organ damage at the last follow up with mean SDI 0.43 (0–6) and 20 patients in adulthood (41, 67%) had organ damage with SDI 0.75 (0–6). Cataract, erosive arthritis and avascular necrosis were the most common organ damage in both groups. The most common presenting symptoms in childhood were musculoskeletal (predominantly arthritis) occurring in 34 children (70.83%), mucocutaneous (rash) noted in 31 (64.58%) and fever in 21 patients (43.75%). Of different laboratory tests the most common were positive antinuclear antibodies (ANA) screen (95.83%) and hypocomplementaemia (75%). Proteinuria was noticed in 26 children (54.17%). Similarly, in adulthood the most common symptoms were arthritis in 10 (20.83%) and rash in 8 patients (16.67%). Alopecia, headaches and visual disturbances were represented with 12.5% each. ANA screen was positive in 27 patients (56.25%) and hypocomplementaemia present in 22 patients (45.83%). Conclusions At the time of diagnosis in childhood, disease activity is very high while in adulthood there is a significant decrease in disease activity. Higher disease activity in childhood is related to the development of the organ damage in adulthood.
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- 2018
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38. Comparison of Computerized Color Telethermography and Nailfold Capillaroscopy in Diagnostics of Secondary Raynaud’s Phenomenon in Children
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Sestan, Mario, Cekada, Nastasia, Turudic, Daniel, Batnozic Varga, Mateja, Stipic, Jagoda, Baresic, Marko, Frkovic, Marijan, Kifer, Domagoj, and Jelusic, Marija
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Raynaudov sindrom ,kapilaroskopija ,kompjutorizirana kolor-teletermografija ,dijete - Abstract
Background: Raynaud’s phenomenon (RP) is a condition characterized by periodical vasospasm in response to cold temperatures or emotional stress exposure. To distinguish between primary and secondary RP, clinical examination, laboratory fi ndings, nailfold capillaroscopy (NC) and computerized color telethermography (CCTT) are necessary. Objectives: To analyze RP features in children in correlation with the most frequently associated laboratory tests, CCTT and NC. Methods: Th is study included children clinically recognized as RP in the period from 2011–2017 at the Referral Center for Pediatric and Adolescent Rheumatology Republic of Croatia. Laboratory data included serum level of IgG, C3, C4, CH50, RF, presence of ANA and ANCAs. Results: CCTT, performed in 188 patients, classifi cated 15 as primary RP, 57 as secondary RP, while in 47 no classifi cation could be made. Among patients classifi cated as secondary RP on CCTT, the most of them, 14 (24.6%), were diagnosed with juvenile idiopathic arthritis (JIA). Th ere were 5 patients (8.8%) with systemic sclerosis (SSc), 2 (3.5%) with mixed connective tissue disease (MCTD), 1 (1.7%) with systemic lupus erythematosus, 11 (19.3%) with undiff erentiated connective tissue disease (UCTD), whilst 24 (42.1%) had no evident other disease. Th e appearance of abnormal capillaroscopic pattern was found in 17 out of 89 patients and nonspecifi c capillaroscopic alterations were noticed in 27. Among patients with the appearance of abnormal capillaroscopic pattern, 5 (29.4%) were diagnosed with SSc, 3 (17.6%) with JIA, 2 (11.8%) with MCTD, 1 (5.9%) with dermatomyositis, 2 (11.8%) with UCTD, whilst 4 (23.5%) had no evident rheumatic disease. All patients with RP diagnosed with SSc and MCTD had both the appearance of abnormal capillaroscopic pattern and CCTT fi ndings consistent with secondary RP. No statistically signifi cant diff erence between NC and CCTT in predicting the diagnosis of secondary RP was determined (McNemar’s test, χ2 = 0.042, p = 0.838) nor was there signifi cant diff erence between NC and CCTT in regard to the results of laboratory fi ndings (χ2 = 1.042, p = 0.307). Conclusions: We found that nailfold capillaroscopy and CCTT were equally eff ective in the diagnosis of secondary RP in children. Th ere was no diff erence between them in regard to the results of immunological laboratory fi ndings distinctive with secondary RP.
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- 2018
39. Simultaneous treatment with TNF inhibitor and IVIG in a patient with primary immunodeficiency and autoimmunity: a treatment conundrum
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Cekada, Nastasia, Sestan, Mario, Frkovic, Marijan, Kelecic, Jadranka, and Jelusic, Marija
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primary immunodeficiency ,DiGeorge syndrome ,autoimmunity ,TNF inhibitor ,IVIG - Abstract
Background and aims: DiGeorge syndrome (DGS) is a primary immunodeficiency disease which can generate underdeveloped B lymphocytes, causing hypogammaglobulinemia in 6% of patients. Autoimmune diseases are recurrent among these patients, with juvenile idiopathic arthritis (JIA) being 50 times more common. Our aim is to present a patient suffering from DGS and JIA treated simultaneously with TNF inhibitor and IVIG, which is a rare occurrence in clinical practice. Methods: All medical information was obtained from patient’s medical records from 2009 to 2017 at the University Hospital Centre Zagreb, Croatia. Results: Male patient was diagnosed with rheumatoid factor negative polyarticular JIA at the age of 3. The following years he was continuously treated with NSAIDs, methotrexate and corticosteroids. Due to frequent infections, dysmorphic facial structures, agenesis of left kidney and ventricular septal defect, DGS was diagnosed at the age of 15. On the account of underdeveloped B lymphocytes, the patient received IVIG every 4-6 weeks. A year later, due to remitting relapses of polyarthritis, adalimumab was introduced. He was successfully treated for a two-year period after which he developed psoriasis, mediastinal lymphadenopathy and interstitial lung infiltrates, which subsided when adalimumab was revoked. Conclusion: The introduction of anti-TNF therapy was debatable due to the added risk of infections and malignancies, to which the patient was already susceptible on the account of the DGS. However, concurrent therapy with IVIG and TNF inhibitors can be beneficial but it is of great importance to carefully monitor the patient for possible side effects and interactions between drugs.
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- 2018
40. Predicting organ damage in patients with childhood-onset systemic lupus erythematosus: a retrospective study over the last 25 years
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Cekada, Nastasia, Sestan, Mario, Hosticka, Emilija, Novoselec, Maja, Batnozic Varga, Mateja, Padjen, Ivan, Frkovic, Marijan, Kifer, Domagoj, Anic, Branimir, Batinic, Drago, Potocki, Kristina, Malcic, Ivan, and Jelusic, Marija
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Sistemski eritemski lupus s početkom u dječjoj dobi ,SLEDAI 2K ,SDI - Abstract
Introduction: Childhood-onset systemic lupus erythematosus (cSLE) is a multisystemic, chronic autoimmune disease occurring in children and adolescents under 18, presenting heterogeneously, with the course of the disease often more severe than in adults, and the activity of the disease widely being evaluated by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI 2K) and damage by the SLICC/ACR damage index (SDI). Objectives: To compare and explore the correlation between the SLEDAI-2K disease activity index at the time of diagnosis and the SLICC/ACR damage index (SDI) of patients at their last follow up, to examine the quantity of organ damage in regard to patients’ characteristics and disease duration and to predict the risk of organ damage occurrence in time. Methods: The retrospective study included children treated for cSLE in the period from January 1991 to September 2017 at Department of Pediatrics, University Hospital Center Zagreb. The follow up data of patients after the age of majority at 18 and who were under care of the Department of Internal Medicine, University Hospital Center Zagreb was also included. All children were diagnosed according to the ACR 1997 and SLICC 2012 criteria. Data analysis was done using R environment for statistical computing. Results: The disease development of 93 children (74 females and 19 males) with cSLE was examined in this study. The median (range) follow up time was 7 (0.5-24) years and the median (range) age at diagnosis was 13 (5-19) years. Two patients have died during the examined period. Mean (SD) SLEDAI- 2K was 18.3 (9.0) at the disease onset. 35 children (38 %) had organ damage at the last follow up with the median (range) SDI 0 (0-7). The first organ systems damaged in affected patients were renal (28%), musculoskeletal (22%), ocular (19%), neuropsychiatric (17%), cardiovascular (11%) and peripheral vascular (2.8%), but with no significant statistical difference regarding the type of organ damage. Kendall rank correlation coefficient was used to evaluate the relationship between SLEDAI-2K at the disease onset and SDI, determining a positive correlation which was statistically significant (τb = 0.252, p = 0.003). However, no significant correlation was determined between the duration of the disease and SDI (τb = 0.042, p = 0.628) or follow up period and SDI (τb = 0.111, p = 0.191) nor was there significant difference of SDI in regard to gender (Asymptotic Wilcoxon- Mann-Whitney Test, p= 0.574). Using Kaplan-Meier method we estimated the decrease in ratio of patients without organ damage since diagnosis or the occurrence of the first symptoms. Since the estimated ratio of patients with organ damage at the endpoint was less than 50%, we could not estimate the time required for damage development in 50% of patients. However, we are 95% sure that the damage is not happening in the first 9 or 10 years after diagnosis or the occurrence of the first symptoms, respectively. Conclusion: The high correlation detected between SLEDAI-2K and SDI indicated that the presentation of the cSLE at onset can be prognostic of the course and long-term prognosis of lupus. Our findings suggest that it is unlikely that organ damage will occur in 50% of patients in the first nine years of the disease course.
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- 2018
41. Childhood-Onset Systemic Lupus Erythematosus over the Last 25 Years: Predicting Organ Damage
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Cekada Nastasia, Sestan Mario, Hosticka Emilija, Novoselec Maja, Batnozic Varga Mateja, Padjen ivan, Frkovic Marijan, Kifer Domagoj, Anic Branimir, Batinic Drago, Potocki Kristina, Malcic Ivan, and Jelusic Marija
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childhood-onset systemic lupus erythematosus ,organ damage ,sistemski eritemski lupus ,oštećenje ,aktivnost ,djeca - Abstract
Background: Childhood-onset systemic lupus erythematosus (cSLE) is a chronic autoimmune disease with the course often more severe than in adults, and the activity of the disease widely being evaluated by SLE Disease Activity Index (SLEDAI 2K) and damage by the SLICC/ACR damage index (SDI). Objectives: To explore the correlation between the SLEDAI-2K disease activity index at the time of diagnosis and the SLICC/ACR damage index of patients at their last follow up and to predict the risk of organ damage occurrence in time. Methods: The retrospective study included children treated for cSLE from January 1991 to September 2017 at Department of Pediatrics, UHC Zagreb. All children were diagnosed according to the ACR 1997 and SLICC 2012 criteria. Results: The disease development of 93 children (74 females) with cSLE was examined in this study. The median (range) follow up time was 7 (0.5–24) years and the median (range) age at diagnosis was 13 (5–19) years. Mean (SD) SLEDAI-2K was 18.3 (9.0) at the disease onset. 35 children (38 %) had organ damage at the last follow up with the median (range) SDI 0 (0–7). Th e first organ systems damaged in affected patients were renal (28%), musculoskeletal (22%), ocular (19%), neuropsychiatric (17%), cardiovascular (11%) and peripheral vascular (2.8%). A statistically signifi cant positive correlation was found between SLEDAI-2K at the disease onset and SDI (τb = 0.252, p = 0.003). No significant correlation was determined between the duration of the disease (τb = 0.042, p = 0.628) or follow up period (τb = 0.111, p = 0.191) and SDI, nor in SDI in regard to gender (p= 0.574). Using Kaplan-Meier method we estimated the decrease in ratio of patients without organ damage since diagnosis or the occurrence of the first symptoms. Since the estimated ratio of patients with organ damage at the endpoint was less than 50%, we could not estimate the time required for damage development in 50% of patients. However, we are 95% sure that the damage is not happening in the first 9 years aft er diagnosis. Conclusions: The high correlation detected between SLEDAI-2K and SDI indicated that the presentation of the cSLE at onset can be prognostic of the course and long-term prognosis of lupus. Our findings suggest that it is unlikely that organ damage will occur in 50% of patients in the first nine years of the disease course. References: 1. Gladman DD, Ibanez D, Urowitz MB. Systemic lupus erythematosus disease activity index 2000. J Rheumatol. 2002; 29:288–91.
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- 2018
42. SAT0484 QUALITY OF LIFE IN JUVENILE IDIOPATHIC ARTHRITIS-ASSOCIATED UVEITIS: DO WE NEED A NEW ASSESSMENT QUESTIONNAIRE?
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Sestan, Mario, primary, Grguric, Danica, additional, Sedmak, Maja, additional, Kifer, Nastasia, additional, Frkovic, Marijan, additional, Peric, Sanja, additional, Potocki, Kristina, additional, Vukojevic, Nenad, additional, and Jelusic, Marija, additional
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- 2019
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43. AB0995 EXPLORING UVEITIS IN EARLY ONSET ANA POSITIVE JIA
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Kifer, Nastasia, primary, Hrgovic, Mihovil, additional, Sestan, Mario, additional, Peric, Sanja, additional, Kifer, Domagoj, additional, Frkovic, Marijan, additional, Potocki, Kristina, additional, Vukojevic, Nenad, additional, and Jelusic, Marija, additional
- Published
- 2019
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44. Geospatial clustering of childhood IgA vasculitis and IgA vasculitis-associated nephritis.
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Sapina, Matej, Frkovic, Marijan, Sestan, Mario, Srsen, Sasa, Ovuka, Aleksandar, Varga, Mateja Batnozic, Kramaric, Karolina, Brdaric, Dario, Milas, Kresimir, Gagro, Alenka, Jelusic, Marija, and Batnozic Varga, Mateja
- Abstract
Objectives: Research on spatial variability of the incidence of IgA vasculitis (IgAV) in children and its potential implications for elucidation of the multifactorial aetiology and pathogenesis is limited. We intended to observe spatial variability of the incidence of IgAV and IgA vasculitis-associated nephritis (IgAVN) using modern geostatistical methods, and hypothesised that their spatial distribution may be spatially clustered.Methods: Patients' data were retrospectively collected from 2009 to 2019 in five Croatian University Hospital Centres for paediatric rheumatology, and census data were used to calculate the incidence of IgAV. Using spatial empirical Bayesian smoothing, local Morans' I and local indicator of spatial autocorrelation (LISA), we performed spatial statistical analysis.Results: 596 children diagnosed with IgAV were included in this study, of which 313 (52.52%) were male. The average annual incidence proportion was estimated to be 6.79 per 100 000 children, and the prevalence of IgAVN was 19.6%. Existence of spatial autocorrelation was observed in both IgAV and IgAVN; however, clustering distribution differed. While IgAV showed clustering in Mediterranean and west continental part around cities, IgAVN was clustered in the northern Mediterranean and eastern continental part, where a linear cluster following the Drava and Danube river was observed.Conclusion: IgAV incidence in Croatia is similar to other European countries. Spatial statistical analysis showed a non-random distribution of IgAV and IgAVN. Although aetiological associations cannot be inferred, spatial analytical techniques may help in investigating and generating new hypotheses in non-communicable diseases considering possible environmental risk factors and identification of potential genetic or epigenetic diversity. [ABSTRACT FROM AUTHOR]- Published
- 2021
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45. Effects of anti-TNF therapy on ophtalmological complications in children with rheumatic diseases
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Frkovic, Marijan, primary, Vukojevic, Nenad, additional, and Jelusic, Marija, additional
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- 2014
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46. Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part two: Genoa, Italy. 28 September – 01 October 2016
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Lomakina, Olga, Alekseeva, Ekaterina, Valieva, Sania, Bzarova, Tatiana, Nikishina, Irina, Zholobova, Elena, Rodionovskaya, Svetlana, Kaleda, Maria, Nakagishi, Yasuo, Shimizu, Masaki, Mizuta, Mao, Yachie, Akihiro, Sugita, Yuko, Okamoto, Nami, Shabana, Kousuke, Murata, Takuji, Tamai, Hiroshi, Smith, Eve M., Yin, Peng, Jorgensen, Andrea L., Beresford, Michael W., Eleuteri, Antonio, Goilav, Beatrice, Lewandowski, Laura, Phuti, Angel, Wahezi, Dawn, Rubinstein, Tamar, Jones, Caroline, Newland, Paul, Marks, Stephen, Corkhill, Rachel, Ekdawy, Diana, Pilkington, Clarissa, Tullus, Kjell, Putterman, Chaim, Scott, Chris, Fisher, Antony C., Jorgensen, Andrea, Batu, Ezgi Deniz, Kosukcu, Can, Taskiran, Ekim, Akman, Sema, Ozturk, Kubra, Sozeri, Betul, Unsal, Erbil, Ekinci, Zelal, Bilginer, Yelda, Alikasifoglu, Mehmet, Ozen, Seza, Lythgoe, Hanna, Brunner, Hermine I., Gulati, Gaurav, Jones, Jordan T., Altaye, Mekibib, Eaton, Jamie, Difrancesco, Mark, Yeo, Joo Guan, Leong, Jingyao, Bathi, Loshinidevi D/O Thana, Arkachaisri, Thaschawee, Albani, Salvatore, Abdelrahman, Nagla, Beresford, Michael W, Leone, Valentina, Groot, Noortje, Shaikhani, D., Bultink, I. E. M., Bijl, M., Dolhain, R. J. E. M., Teng, Y. K. O., Zirkzee, E., de Leeuw, K., Fritsch-Stork, R., Kamphuis, S. S. M., Wright, Rachael D., Abdawani, Reem, Al Shaqshi, Laila, Al Zakwani, Ibrahim, Gormezano, Natali W., Kern, David, Pereira, Oriany L., Esteves, Gladys C. C., Sallum, Adriana M., Aikawa, Nadia E., Pereira, Rosa M., Silva, Clovis A., Bonfa, Eloisa, Beckmann, Jessica, Bartholomä, Nora, Venhoff, Nils, Henneke, Philipp, Salzer, Ulrich, Janda, Ales, Boteanu, Alina Lucica, Corral, Sandra Garrote, Giraldo, Alberto Sifuentes, Gámir, Mariluz Gámir, Mendoza, Antonio Zea, Adrovic, Amra, Dedeoglu, Reyhan, Sahin, Sezgin, Barut, Kenan, Koka, Aida, Oztunc, Funda, Kasapcopur, Ozgur, Rodriguez-Lozano, Ana Luisa, Rivas-Larrauri, Francisco, de la Puente, Silvestre García, Alves, Andressa G. F., Giacomin, Maria F. D. A., Farhat, Juliana, Braga, Alfésio L. F., Sallum, Adriana M. E., Campos, Lúcia M. D. A., Pereira, Luiz A. A., Lichtenfels, Ana J. D. F. C., Silva, Clóvis A., Farhat, Sylvia C. L., Acar, Banu, Ozcakar, Z. Birsin, Çakar, Nilgün, Uncu, Nermin, Gür, Gökçe, Özdel, Semanur, Yalçınkaya, Fatoş, Scott, Christiaan, Brice, Nicky, Nourse, Peter, Arango, Christine, Mosquera, Angela C., Malagon, Clara, Sakamoto, Ana P., Silva, Marco F. C. D., Lopes, Ananadreia S., Russo, Gleice C. S., Sallum, Adriana E. M., Kozu, Katia, Bonfá, Eloisa, Saad-Magalhães, Claudia, Pereira, Rosa M. R., Len, Claudio A., Terreri, Maria T., Suri, Deepti, Didel, Siyaram, Rawat, Amit, Singh, Surjit, Maritsi, Despoina, Onoufriou, MArgarita, Vougiouka, Olga, Tsolia, Maria, Bosak, Edi Paleka, Vidović, Mandica, Lamot, Mirta, Lamot, Lovro, Harjaček, Miroslav, Van Nieuwenhove, Erika, Liston, Adrian, Wouters, Carine, Tahghighi, Fatemeh, Ziaee, Vahid, Raeeskarami, Seid-Reza, Aguiar, Francisca, Pereira, Sandra, Rodrigues, Mariana, Moura, Cláudia, Rocha, Gustavo, Guimarães, Hercília, Brito, Iva, Fonseca, Rita, Horneff, Gerd, Klein, Ariane, Minden, Kirsten, Huppertz, Hans-Iko, Weller-Heinemann, Frank, Kuemmerle-Deschner, Jasmin, Haas, J-Peter, Hospach, Anton, Menendez-Castro, Ricardo, Huegle, Boris, Haas, Johannes-Peter, Swart, Joost, Giancane, Gabriella, Bovis, Francesca, Castagnola, Elio, Groll, Andreas, Lovell, Daniel J., Wolfs, Tom, Hofer, Michael, Panaviene, Violeta, Nielsen, Susan, Anton, Jordi, Uettwiller, Florence, Stanevicha, Valda, Trachana, Maria, Marafon, Denise Pires, Ailioaie, Constantin, Tsitsami, Elena, Kamphuis, Sylvia, Herlin, Troels, Doležalová, Pavla, Susic, Gordana, Flatø, Berit, Sztajnbok, Flavio, Pistorio, Angela, Martini, Alberto, Wulffraat, Nico, Ruperto, Nicolino, Gattorno, Marco, Brucato, Antonio, Finetti, Martina, Lazaros, George, Maestroni, Silvia, Carraro, Mara, Cumetti, Davide, Carobbio, Alessandra, Lorini, Monia, Rimini, Alessandro, Marcolongo, Renzo, Valenti, Anna, Erre, Gian Luca, Belli, Riccardo, Gaita, Fiorenzo, Sormani, Maria Pia, Imazio, Massimo, Abinun, Mario, Smith, Nicola, Rapley, Tim, McErlane, Flora, Kearsley-Fleet, Lianne, Hyrich, Kimme L., Foster, Helen, Tzaribachev, Nikolay, Zeft, Andrew, Cimaz, Rolando, Bohnsack, John, Griffin, Thomas, Carrasco, Ruy, Dare, Jason, Foeldvari, Ivan, Vehe, Richard, Simon, Teresa, Brunner, Hermine, Verazza, S., Davì, S., Consolaro, A., Insalaco, A., Gerloni, V., Cimaz, R., Zulian, F., Pastore, S., Corona, F., Conti, G., Barone, P., Cattalini, M., Cortis, E., Breda, L., Olivieri, A. N., Civino, A., Podda, R., Rigante, D., La Torre, F., D’Angelo, G., Jorini, M., Gallizzi, R., Maggio, M. C., Consolini, R., De Fanti, A., Alpigiani, M. G., Martini, A., Ravelli, A., Kısaarslan, Aysenur Pac, Gunduz, Zubeyde, Dusunsel, Ruhan, Dursun, Ismail, Poyrazoglu, Hakan, Kuchinskaya, Ekaterina, Abduragimova, Farida, Kostik, Mikhail, Sundberg, Erik, Omarsdottir, Soley, Klevenvall, Lena, Erlandsson-Harris, Helena, Basbozkurt, Gokalp, Erdemli, Ozge, Simsek, Dogan, Yazici, Fatih, Karsioglu, Yildirim, Tezcaner, Aysen, Keskin, Dilek, Ozkan, Huseyin, Acikel, Cengizhan, Demirkaya, Erkan, Orbán, Ilonka, Sevcic, Krisztina, Brodszky, Valentin, Kiss, Emese, Tekko, Ismaiel A., Rooney, Madeleine, McElnay, James, Taggart, Cliff, McCarthy, Helen, Donnelly, Ryan F., Slatter, Mary, Nademi, Zohreh, Friswell, Mark, Jandial, Sharmila, Flood, Terence, Hambleton, Sophie, Gennery, Andrew, Cant, Andrew, Duong, Phoi-Ngoc, Koné-Paut, Isabelle, Filocamo, Giovanni, Gamir, María Luz, Sanner, Helga, Carenini, Laura, Topdemir, Mesut, Karslioglu, Yildirim, Gok, Faysal, Tsurikova, Nadezhda, Ligostaeva, Elena, Ramchurn, Navdha R., Kostareva, O., Nikishina, I., Arsenyeva, S., Rodionovskaya, S., Kaleda, M., Alexeev, D., Dursun, Ismail Dursun, Murias, Sara, Barral, Estefania, Alcobendas, Rosa, Enriquez, Eugenia, Remesal, Agustin, de Inocencio, Jaime, Castro, Tania M., Lotufo, Simone A., Freye, Tatjana, Carlomagno, Raffaella, Zumbrunn, Thomas, Bonhoeffer, Jan, Schneider, Elvira Cannizzaro, Kaiser, Daniela, Hofer, Michaël, Hentgen, Véronique, Woerner, Andreas, Schwarz, Tobias, Klotsche, Jens, Niewerth, Martina, Ganser, Gerd, Jeyaratnam, Jerold, ter Haar, Nienke, Rigante, Donato, Dedeoglu, Fatma, Baris, Ezgi, Vastert, Sebastiaan, Frenkel, Joost, Hausmann, Jonathan S., Lomax, Kathleen G., Shapiro, Ari, Durrant, Karen L., Brogan, P. A., Hofer, M., Kuemmerle-Deschner, J. B., Lauwerys, B., Speziale, A., Leon, K., Wei, X., Laxer, R. M., Signa, Sara, Rusmini, Marta, Campione, Elena, Chiesa, Sabrina, Grossi, Alice, Omenetti, Alessia, Caorsi, Roberta, Viglizzo, Gianmaria, Ceccherini, Isabella, Federici, Silvia, Lachmann, Helen, Ruperto, Nicola, Vanoni, Federica, Gomes, Sonia Melo, Omoyinmi, Ebun, Arostegui, Juan I., Gonzalez-Roca, Eva, Eleftheriou, Despina, Klein, Nigel, Brogan, Paul, Volpi, Stefano, Santori, Elettra, Picco, Paolo, Pastorino, Claudia, Rice, Gillian, Tesser, Alessandra, Crow, Yanick, Candotti, Fabio, Sinoplu, Ada B., Yucel, Gozde, Pamuk, Gizem, Damian, Laura O., Lazea, Cecilia, Sparchez, Mihaela, Vele, Paulina, Muntean, Laura, Albu, Adriana, Rednic, Simona, Lazar, Calin, Mendonça, Leonardo O., Pontillo, Alessandra, Kalil, Jorge, Castro, Fabio M., Barros, Myrthes T., Pardeo, Manuela, Messia, Virginia, De Benedetti, Fabrizio, Insalaco, Antonella, Malighetti, Giorgia, Gorio, Chiara, Ricci, Francesca, Parissenti, Ilaria, Montesano, Paola, Bonafini, Barbara, Medeghini, Veronica, Cattalini, Marco, Giordano, Lucio, Zani, Giulia, Ferraro, Rosalba, Vairo, Donatella, Giliani, Silvia, Maggio, Maria Cristina, Luppino, Girolamo, Corsello, Giovanni, Fernandez, Maria Isabel Gonzalez, Montesinos, Berta Lopez, Vidal, Adriana Rodriguez, Gorospe, Juan I. Arostegui, Penades, Inmaculada Calvo, Rafiq, Nadia K., Wynne, Karen, Hussain, Khalid, Brogan, Paul A., Ang, Elizabeth, Ng, Nicholas, Kacar, Ayla, Gucenmez, Ozge Altug, Makay, Balahan, Unsal, Sevket Erbil, Sahin, Yasin, Kutlu, Tufan, Cullu-Cokugras, Fugen, Ayyildiz-Civan, Hasret, Erkan, Tulay, Al Zuhbi, Sana, Abdalla, Eiman, Russo, Ricardo A., Katsicas, María M., Minoia, Francesca, Ravelli, Angelo, Bhattad, Sagar, Gupta, Anju, Pandiarajan, Vignesh, Nada, Ritambhra, Tiewsoh, Kaara, Hawkins, Philip, Rowczenio, Dorota, Fingerhutova, Sarka, Franova, Jana, Prochazkova, Leona, Hlavackova, Eva, Dolezalova, Pavla, Evrengül, Havva, Yüksel, Selçuk, Doğan, Mustafa, Gürses, Dolunay, Evrengül, Harun, De Pauli, Silvia, Pastore, Serena, Bianco, Anna Monica, Severini, Giovanni Maria, Taddio, Andrea, Tommasini, Alberto, Salugina, Svetlana O., Fedorov, Evgeny, Kamenets, Elena, Zaharova, Ekaterina, Sleptsova, Tatiana, Alexeeva, Ekaterina, Savostyanov, Kirill, Pushkov, Alexander, Bzarova, Tatyana, Valieva, Saniya, Denisova, Rina, Isayeva, Kseniya, Chistyakova, Evgeniya, Soloshenko, Margarita, Kaschenko, Elena, Kaneko, Utako, Imai, Chihaya, Saitoh, Akihiko, Teixeira, Vitor A., Ramos, Filipa O., Costa, Manuela, Aviel, Yonatan Butbul, Fahoum, Shafe, Brik, Riva, Özçakar, Zeynep Birsin, Celikel, Banu Acar, Yalcinkaya, Fatos, Schiappapietra, Benedetta, Davi’, Sergio, Mongini, Federica, Giannone, Luisa, Bava, Cecilia, Alpigiani, Maria Giannina, Consolaro, Alessandro, Lazarevic, Dragana S., Vojinovic, Jelena, Basic, Jelena, Muratore, Valentina, Marzetti, Valentina, Quilis, Neus, Benavente, Belen Serrano, Alongi, Alessandra, Civino, Adele, Quartulli, Lorenzo, Januskeviciute, Giedre, van Dijkhuizen, Pieter, Groot, N., van Dijk, W., Kardolus, A., Suárez, Raul Gutiérrez, Nordal, Ellen B., Rypdal, Veronika G., Berntson, Lillemor, Ekelund, Maria, Aalto, Kristiina, Peltoniemi, Suvi, Zak, Marek, Glerup, Mia, Arnstad, Ellen D., Fasth, Anders, Rygg, Marite, Duarte, Ana Catarina, Sousa, Sandra, Teixeira, Lídia, Cordeiro, Ana, Santos, Mª José, Mourão, Ana Filipa, Santos, Maria José, Eusébio, Mónica, Lopes, Ana, Oliveira-Ramos, Filipa, Salgado, Manuel, Estanqueiro, Paula, Melo-Gomes, José, Martins, Fernando, Costa, José, Furtado, Carolina, Figueira, Ricardo, Branco, Jaime C., Fonseca, João E., Canhão, Helena, Mourão, Ana F., Santos, Maria Jose, Coda, Andrea, Cassidy, Samuel, West, Kerry, Hendry, Gordon, Grech, Debra, Jones, Julie, Hawke, Fiona, Grewal, Davinder Singh, Foley, Charlene, Killeen, Orla, MacDermott, Emma, Veale, Douglas, Fearon, Ursula, Konukbay, Dilek, Tarakci, Ela, Arman, Nilay, Şahin, Sezgin, Munro, Jane, Morgan, Esi, Riebschleger, Meredith, Horonjeff, Jennifer, Strand, Vibeke, Bingham, Clifton, Collante, Ma. Theresa M., Ganeva, Margarita, Stefanov, Stefan, Telcharova, Albena, Mihaylova, Dimitrina, Saraeva, Radoslava, Tzveova, Reni, Kaneva, Radka, Tsakova, Adelina, Temelkova, Katya, Picarelli, Maria Mercedes C., Danzmann, Luiz C., Barbé-Tuana, Florencia, Grun, Lucas K., Jones, Marcus H., Frković, Marijan, Ištuk, Karla, Birkić, Ika, Sršen, Saša, Jelušić, Marija, Easton, Alan, Quarmby, Rachael, Khubchandani, Raju, Chan, Mercedes, Srp, Radoslav, Kobrova, Katerina, Nemcova, Dana, Hoza, Jozef, Uher, Michal, Saifridova, Melania, Linkova, Lenka, Charuvanij, Sirirat, Leelayuwattanakul, Isree, Pacharapakornpong, Thita, Vallipakorn, Sakda A.-O., Lerkvaleekul, Butsabong, Vilaiyuk, Soamarat, Lanni, Stefano, Davì, Sergio, Cron, Randy Q., Passarelli, Chiara, Pisaneschi, Elisa, Novelli, Antonio, Bracaglia, Claudia, Caiello, Ivan, de Graaf, Kathy, Guilhot, Florence, Ferlin, Walter, Schulert, Grant, Grom, Alexi A., Nelson, Robert, de Min, Cristina, Holzinger, Dirk, Kessel, Christoph, Fall, Ndate, Grom, Alexei, de Jager, Wilco, Strippoli, Raffaele, Horne, Anna, Ehl, Stephan, Ammann, Sandra, Lehmberg, Kai, Beutel, Karin, Foell, Dirk, Horne, AnnaCarin, Pagani, Laura, Espada, Graciela, Gao, Yi-jin, Shenoi, Susan, Weitzman, Sheila, Prencipe, Giusi, Pascarella, Antonia, Ferlin, Walter G., Chatel, Laurence, Jacqmin, Philippe, De Graaf, Kathy, Ballabio, Maria, Johnson, Zoë, Lapeyre, Geneviève, de Benedetti, Fabrizio, Cristina, de Min, Wakiguchi, Hiroyuki, Hasegawa, Shunji, Hirano, Reiji, Okazaki, Fumiko, Nakamura, Tamaki, Kaneyasu, Hidenobu, Ohga, Shouichi, Yamazaki, Kazuko, Nozawa, Tomo, Kanetaka, Taichi, Ito, Shuichi, Yokota, Shumpei, McLellan, Kirsty, MacGregor, Ishbel, Martin, Neil, Davidson, Joyce, Hansmann, Sandra, Eikelberg, Andreas, Haug, Iris, Schuller, Sabrina, Benseler, Susanne M., Nazarova, Liliia S., Danilko, Kseniia V., Malievsky, Viktor A., Viktorova, Tatiana V., Mauro, Angela, Barnicoat, Angela, Hurst, Jane, Canham, Nathalie, Lacassagne, Sandrine, Wiener, Anastasia, Hügle, Boris, Denecke, Bernd, Costa-Filho, Ivan, Haas, Johannes Peter, Tenbrock, Klaus, Popp, David, Boltjes, Arjan, Rühle, Frank, Herresthal, Stefanie, van Wijk, Femke, Schultze, Joachim, Stoll, Monika, Klotz, Luisa, Vogl, Thomas, Roth, Johannes, Quesada-Masachs, Estefania, de la Sierra, Daniel Álvarez, Prat, Marina Garcia, Sánchez, Ana M. Marín, Borrell, Ricardo Pujol, Barril, Sara Marsal, Gallo, Mónica Martínez, Caballero, Consuelo Modesto, Chyzheuskaya, Iryna, Byelyaeva, Lyudmyla M., Filonovich, Rostislav M., Khrustaleva, Helena K., Zajtseva, Larisa I., Yuraga, Tamara M., Giner, Thomas, Hackl, Lukas, Albrecht, Julia, Würzner, Reinhard, Brunner, Juergen, Minute, Marta, Parentin, Fulvio, Nocerino, Agostino, Nørgaard, Mette, Alberdi-Saugstrup, Mikel, Zak, Marek S., Nielsen, Susan M., Nordal, Ellen, Müller, Klaus G., Avramovič, Mojca Zajc, Dolžan, Vita, Toplak, Nataša, Avčin, Tadej, Ruperto, N., Lovell, D. J., Wallace, C., Toth, M., Foeldvari, I., Bohnsack, J., Milojevic, D., Rabinovich, C., Kingsbury, D., Marzan, K., Quartier, P., Minden, K., Chalom, E., Horneff, G., Kuester, R. M., Dare, J., Heinrich, M., Kupper, H., Kalabic, J., Brunner, H. I., Burgos-Vargas, Ruben, Constantin, Tamas, Dehoorne, Joke, Stanevica, Valda, Kobusinska, Katarzyna, Zuber, Zbigniew, Mouy, Richard, Rumba-Rozenfelde, Ingrida, Job-Deslandre, Chantal, Pederson, Ronald, Bukowski, Jack, Hinnershitz, Tina, Vlahos, Bonnie, Keskitalo, Paula, Kangas, Salla, Vähäsalo, Paula, Valencia, Raul A. Chavez, Martino, David, Ponsonby, Anne-Louise, Chiaroni-Clarke, Rachel, Meyer, Braydon, Allen, Roger C., Akikusa, Jonathan D., Craig, Jeffrey M., Saffrey, Richard, Ellis, Justine A., Wallace, Carol, Uziel, Yosef, Sterba, Gary, Schneider, Rayfel, Russo, Ricardo, Ramanan, Athimalaipet V., Schmid, Jana Pachlopnik, Nichols, Kim E, Miettunen, Paivi, Kitoh, Toshiyuki, Ilowite, Norman T., Henter, Jan-Inge, Grom, Alexei A, Behrens, Edward M., Avcin, Tadej, Aricò, Maurizio, Grevich, Sriharsha, Lee, Peggy, Ringold, Sarah, Leroux, Brian, Leahey, Hannah, Yuasa, Megan, Foster, Jessica, Sokolove, Jeremy, Lahey, Lauren, Robinson, William, Newson, Joshua, Stevens, Anne, Shoop, Stephanie J. W., Verstappen, Suzanne M. M., Thomson, Wendy, McDonagh, Janet E., Beukelman, Timothy, Kimura, Yuki, Natter, Marc, Ilowite, Norm, Mieszkalski, Kelly, Burrell, Grendel, Best, Brian, Bristow, Helen, Carr, Shannon, Dennos, Anne, Kaufmann, Rachel, Schanberg, Laura, Simonini, Gabriele, Lancini, Francesca, Gerbaux, Margaux, Lê, Phu-Quoc, Goffin, Laurence, Badot, Valérie, La, Céline, Caspers, Laure, Willermain, François, Ferster, Alina, Ceci, Maria, Licciardi, Francesco, Turco, Marco, Santarelli, Francesca, Montin, Davide, Toppino, Claudia, Alizzi, Clotilde, Papia, Bruno, Vergara, Beatrice, Corpora, Umberto, Messina, Luca, Tsinti, Maria, Dermentzoglou, Vasiliko, Tziavas, Panagiotis, Perica, Marija, Bukovac, Lana Tambić, Çakan, Mustafa, Ayaz, Nuray Aktay, Keskindemirci, Gonca, Lang, Michael, Laing, Catherine, Benseler, Susanne, Gerschman, Tommy, Luca, Nadia, Schmeling, Heinrike, Dropol, Anastasia, Taiani, Jaymi, Johnson, Nicole, Rusted, Brian, Nalbanti, Panagiota, Pratsidou, Polyxeni, Pardalos, Grigoris, Tzimouli, Vasiliki, Taparkou, Anna, Stavrakidou, Maria, Papachristou, Fotios, Kanakoudi-Tsakalidou, Florence, Bale, Peter, Robinson, Emily, Palman, Jason, Ralph, Elizabeth, Gilmour, Kimberly, Heard, Clare, Wedderburn, Lucy R., Barrense-Dias, Yara, Gregory, Antonarakis, Amira, Dhouib, Paolo, Scolozzi, Sylviane, Hanquinet, Michaël, Hofer, Panko, Nataliya, Shokry, Salah, Rakovska, Liudmila, Pino, Sally, Diaz-Maldonado, Adriana, Guarnizo, Pilar, Torreggiani, Sofia, Cressoni, Paolo, Garagiola, Umberto, Di Landro, Giancarla, Farronato, Giampietro, Corona, Fabrizia, Bell, Samantha, Bhatti, Parveen, Nelson, Lee, Mueller, Beth A., Simon, T. A., Baheti, A., Ray, N., Guo, Z., Hazra, Anasuya, Stock, Thomas, Wang, Ronnie, Mebus, Charles, Alvey, Christine, Lamba, Manisha, Krishnaswami, Sriram, Conte, Umberto, Wang, Min, Kingsbury, Daniel, Koskova, Elena, Smolewska, Elzbieta, Vehe, Richard K., Lovell, Daniel, Kubota, Tomohiro, Yasumura, Junko, Kizawa, Toshitaka, Yashiro, Masato, Yamatou, Tsuyoshi, Yamasaki, Yuichi, Takei, Syuji, Kawano, Yoshifumi, Nykvist, Ulrika Järpemo, Magnusson, Bo, Wicksell, Rikard, Palmblad, Karin, Olsson, Gunnar L., Modaressi, Mohammadreza, Moradinejad, Mohammad-Hassan, Seraya, Valentina, Vitebskaya, Alisa, Moshe, Veronica, Amarilyo, Gil, Harel, Liora, Hashkes, Phillip J, Mendelson, Amir, Rabinowicz, Noa, Reis, Yonit, Dāvidsone, Zane, Lazareva, Arina, Šantere, Ruta, Bērziņa, Dace, Staņēviča, Valda, Varnier, Giulia Camilla, Maillard, Susan, Ferrari, Cristina, Zaffarano, Silvia, Wienke, Judith, Enders, Felicitas Bellutti, van den Hoogen, Lucas L., Mertens, Jorre S., Radstake, Timothy R., Hotten, Henny G., Fritsch, Ruth, Wedderburn, Lucy, Nistala, Kiran, Prakken, Berent, van Royen-Kerkhof, Annet, Alhemairi, Mohammad, Muzaffer, Mohammed, Van Dijkhuizen, Pieter, Deakin, Claire T., Simou, Stefania, De Iorio, Maria, Wu, Qiong, Amin, Tania, Dossetter, Lee, Campanilho-Marques, Raquel, Deakin, Claire, Pilkington, Clarissa A., Rosina, Silvia, Soponkanaporn, Sirisucha, Arıcı, Zehra S., Tuğcu, Gökçen D., Batu, Ezgi D., Sönmez, Hafize E., Doğru-Ersöz, Deniz, Talim, Beril, Kiper, Nural, Özen, Seza, Solyom, Alexander, Batu, Ezgi, Mitchell, John, Kariminejad, Ariana, Hadipour, Fatemeh, Hadipour, Zahra, Torcoletti, Marta, Agostoni, Carlo, Di Rocco, Maja, Tanpaiboon, Pranoot, Superti-Furga, Andrea, Bonafé, Luisa, Arslan, Nur, Guelbert, Norberto, Ehlert, Karoline, Grigelioniene, Giedre, Puri, Ratna, Schuchman, Edward, Gomez, Pilar, Gonzalez, Tatiana, Yepez, Ricardo, Vargas, Camilo, Fernanda, Falcini, Lepri, Gemma, Ferrari, Alessandra, Matucci-Cerinic, Marco, Meini, Antonella, Moneta, Gian Marco, Marasco, Emiliano, Nicolai, Rebecca, Bracci-Laudiero, Luisa, Kopchak, Olga, Mushkin, Alexander, Maletin, Alexey, Mosquera, Catalina, Amorim, Rita A., Molina, Juliana, Moreira, Gustavo, Santos, Flávia H., Fraga, Melissa, Keppeke, Livia, Silva, Vanessa M., Hirotsu, Camila, Tufik, Sergio, Terreri, Maria Teresa, Braga, Vinícius L., Fonseca, Maria Beatriz, Schinzel, Vania, Terreri, Maria Teresa R., Jorge, Liliana, Guerra, Liana, Junior, Edson Amaro, Castiglione, Maria Cristina, Tricarico, Alessandra, Boulter, Emily, Schultz, Andre, Murray, Kevin, Falcini, Fernanda, Stagi, Stefano, Bellucci, Eleonora, Grein, Ingrid H. R., Pileggi, Gecilmara, Pinto, Natália B. F., de Oliveira, Aline L., Belyaeva, Lyudmila, Filonovich, Rostislav, Khrustaleva, Helena, Zajtseva, Larisa, Ilisson, Jaanika, Pruunsild, Chris, Gilliaux, Olivier, Corazza, Francis, Lelubre, Christophe, Morel, Zoilo, C, Claudia Saad-Magalhães, Lira, Luis, Ladino, Mabel, Eraso, Ruth, Arroyo, Ivonne, Silva, Clovis, and Rose, Carlos
- Published
- 2017
- Full Text
- View/download PDF
47. Rituximab in Treatment of Children with Refractory Vasculitis and Systemic Lupus Erythematosus - Single Center Experience in Croatia
- Author
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Srsen, Sasa, Frkovic, Marijan, Malcic, Ivan, and Marija Jelusic
- Subjects
Male ,Adolescent ,Croatia ,Remission Induction ,Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ,Lupus Nephritis ,immune system diseases ,Adrenal Cortex Hormones ,Antirheumatic Agents ,systemic lupus erythematosus, vasculitis, child, adolescent ,Humans ,Lupus Erythematosus, Systemic ,Female ,skin and connective tissue diseases ,Child ,Rituximab - Abstract
The aim of this study was to present our experience in rituximab therapy in patients with childhood-onset systemic lupus erythematosus, lupus nephritis, and ANCA-associated vasculitis. We conducted a retrospective clinical chart review of all patients treated with rituximab in the time period from January 2009 to December 2015. Eight patients (3 boys and 5 girls) aged 8 to 15 at the onset of disease were treated with rituximab. Remission of disease was accomplished in 4 patients with childhood- onset systemic lupus erythematosus and lupus nephritis, a partial improvement was achieved in 1 patient with childhoodonset systemic lupus erythematosus and lupus nephritis as well as in 2 patients with vasculitis, while in one patient with vasculitis treatment with rituximab showed no effect and the patient died due to Candida sepsis. Reduction of corticosteroid doses was enabled by rituximab treatment. Rituximab appeared to be a safe and efficient therapeutic option in severe cases of childhood- onset systemic lupus erythematosus or ANCA-associated vasculitis that failed to respond to conventional therapy or as a rescue therapy in life-threatening conditions.
48. Applied Geostatistics in Pediatric Rheumatology - Spatial Clustering of IgA Vasculitis
- Author
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Sapina, Matej, Frkovic, Marijan, Mario Sestan, Srsen, Sasa, Ovuka, Aleksandar, Varga, Mateja Batnozic, Cekada, Nastasia, Kramaric, Karolina, Brdaric, Dario, Milas, Kresimir, Gagro, Alenka, and Jelusic, Marija
49. Henoch-Schonlein Purpura Nephritis: Different Histological Classifications, but Which One Is Most Strongly Associated to the Outcome of the Disease? Pilot Study of the Paediatric Rheumatology European Society Vasculitis Working Party
- Author
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Held, Martina, Sestan, Mario, Coric, Marijana, Bulimbasic, Stela, Giani, Teresa, Martin, Neil, Cekada, Nastasia, Srsen, Sasa, Gracanin, Ana Gudelj, Domagoj Kifer, Heshin, Merav, Ravelli, Angelo, Cimaz, Rolando, Ozen, Seza, Gagro, Alenka, Frkovic, Marijan, and Jelusic, Marija
50. sj-docx-1-tab-10.1177_1759720X211024828 – Supplemental material for Gastrointestinal involvement and its association with the risk for nephritis in IgA vasculitis
- Author
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Sestan, Mario, Kifer, Nastasia, Frkovic, Marijan, Sapina, Matej, Srsen, Sasa, Batnozic Varga, Mateja, Ovuka, Aleksandar, Held, Martina, Gudelj Gracanin, Ana, Kozmar, Ana, Bulimbasic, Stela, Coric, Marijana, Laskarin, Gordana, Gagro, Alenka, and Jelusic, Marija
- Subjects
FOS: Clinical medicine ,110604 Sports Medicine ,FOS: Health sciences ,111599 Pharmacology and Pharmaceutical Sciences not elsewhere classified ,3. Good health ,110314 Orthopaedics - Abstract
Supplemental material, sj-docx-1-tab-10.1177_1759720X211024828 for Gastrointestinal involvement and its association with the risk for nephritis in IgA vasculitis by Mario Sestan, Nastasia Kifer, Marijan Frkovic, Matej Sapina, Sasa Srsen, Mateja Batnozic Varga, Aleksandar Ovuka, Martina Held, Ana Gudelj Gracanin, Ana Kozmar, Stela Bulimbasic, Marijana Coric, Gordana Laskarin, Alenka Gagro and Marija Jelusic in Therapeutic Advances in Musculoskeletal Disease
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