Recommendations for the Management of Uveitis Associated With Juvenile Idiopathic Arthritis: The Czech and Slovak adaptation of SHARE Initiative.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children and uveitis is its most important extra-articular manifestation. Evidence-based recommendations are available only to a limited extent and therefore JIA associated uveitis management is mostly based on physicians experience. Consequently, treatment practices differ widely, both nationally and internationally. Therefore, an effort to optimize and publish recommendations for the care of children and young adults with rheumatic diseases was launched in 2012 as part of the international project SHARE (Single Hub and Access Point for Pediatric Rheumatology in Europe) to facilitate clinical practice for paediatricians and (paediatric) rheumatologists. The aim of this work was to translate published international SHARE recommendations for the diagnosis and treatment of JIA associated uveitis and to adapt them for use in the Czech and Slovak Republics. International recommendations were developed according to the standard methodology of the European League against Rheumatism (EULAR) by a group of nine experienced paediatric rheumatologists and three experts in ophthalmology. It was based on a systematic literature review and evaluated in the form of an online survey and subsequently discussed using a nominal group technique. Recommendations were accepted if > 80% agreement was reached (including all three ophthalmologists). A total of 22 SHARE recommendations were accepted: 3 on diagnosis, 5 on disease activity assessment, 12 on treatment and 2 on future recommendations. Translation of the original text was updated and modified with data specific to the czech and slovak health care systems and supplemented with a proposal for a protocol of ophthalmological dispensarization of paediatric JIA patients and a treatment algorithm for JIA associated uveitis. Conclusion: The aim of the SHARE initiative is to improve and standardize care for paediatric patients with rheumatic diseases across Europe. Therefore, recommendations for the diagnosis and treatment of JIA-associated uveitis have been formulated based on the evidence and agreement of leading European experts in this field.

[Type I diabetes mellitus and associated autoimmune diseases in the first-degree relatives of diabetic children: questionnaire based study]. / Diabetes mellitus 1. typu a sdruzená autoimunitní onemocnení u prvostupnových príbuzných diabetických deti: výsledky dotazníkové studie.

BACKGROUND: Type 1 diabetes mellitus (T1DM) is associated with increased incidence of other autoimmune diseases. The shared genetic background may play a role in the disease pathogenesis. The aim of our study was to assess the prevalence of T1DM and other autoimmune disorders in the first-degree relatives of diabetic children. METHODS AND RESULTS: Data were retrospectively obtained using structured questionnaires from 868 diabetic children younger than 18 years (434 girls and 434 boys, age 12.5 +/- 4.0, mean +/- SD) and their 2704 relatives. The control group included 1466 non-diabetic schoolmates and friends (796 girls, 670 boys, age 11.9 +/- 4.5) and their 4510 first-degree relatives. In the questionnaire we asked about occurrence of thyroid and celiac disease in cases and controls, and about occurrence of T1DM, thyroid and celiac disease in their first-degree relatives. We observed significantly higher prevalence of T1DM in fathers (4.4% vs. 0.8%), mothers (2.0% vs. 0.5%) and siblings (2.5% vs. 0%) of diabetic children compared to controls. Thyroid disease was found significantly more in diabetic children (10.0% vs. 1.9%) and their siblings (3.1% vs. 1.7%). Prevalence of celiac disease was also higher in diabetic children than in controls (3.2% vs. 0.5%), but it does not differ in their first-degree relatives. CONCLUSIONS: We found significantly higher prevalence of thyroid and celiac disease in T1DM children than in controls. Targeted screening and early detection of thyroid and celiac diseases in T1DM patients are likely to be necessary. We observed an increased prevalence of T1DM and thyroid disease in first-degree relatives of diabetic children, however screening of autoimmune diseases associated with T1DM in the first-degree relatives remain controversial.

Higher maternal age at delivery, and lower birth orders are associated with increased risk of childhood type 1 diabetes mellitus.

In several populations, maternal age at delivery and birth order have been demonstrated to variously affect the risk of Type 1 diabetes mellitus in the offspring. The aim of the present study was to investigate this relation in the Czech population. Questionnaire data on 640 children with childhood-onset Type 1 DM and data on 50 random controls to each case, obtained from the national Birth Registry and matched for the calendar year of birth, were analysed using multivariate logistic regression. The risk of Type 1 DM increases with higher maternal age at birth of the child (OR = 1.07, CI 95 % 1.05 - 1.09 per one year increment), and decreases with higher birth order (OR = 0.70, CI 95 % 0.62 - 0.79 per increment in birth order). There was no significant difference in these effects across the five-years bands of age at diabetes onset. We detected no independent effect of maternal education, either. Our study provides further evidence that the risk of Type 1 diabetes in the offspring increases with higher maternal age at delivery and lower birth order.

[Prediction of type 1 diabetes mellitus in first degree Czech relatives of diabetic patients]. / Predikce diabetes mellitus 1. typu u ceských prvostupnových príbuzných diabetických pacientu.

Diagnosis of autoimmune beta cell destruction by genetic risk analysis, autoantibody evaluation and the test of stimulated insulin secretion performance in first-degree relatives of diabetic patients. 208 Czech children and adults (101 boys and 107 girls, 186 siblings, 22 offspring of diabetic parents, aged 1-22 years, mean age 11.5 +/- 5.4 years) were enrolled in the study. Complete DQB1, DQA1 typing and DRB1*04 subtyping were performed by the PCR in 202 subjects. Sera of all children were investigated for anti-GAD65, anti-IA2 and insulin antibodies using RIA methods. The cut-off normal levels were determined as the 99th percentile of 105 non-diabetic children. IVGTT was performed in children with significant titre of one or more autoantibodies. Total level of stimulated insulin secretion < 48 mU/l was assessed as defect of FPIR. Risk genotype DQA1*05-DQB1*0201/DQA1*03-DQB1*0302 (OR = 100, CI 95% 13-730) was found in 24 of 202 first-degree relatives (12%). 22 children (11%) carried strong protective allele DQB1*0602 (OR = 0.03, CI 95% 0.01-0.12). Autoantibody positivity was recognised in 9 of 208 children (2.9%) and IVGTT was performed. Positivity of anti-GAD65, anti-IA2 or IAA was identified in 5 of 24 children with the highest risk genotype (21%) and in 4 children of 113 with lower risk or neutral genotypes (3.5%). Borderline positivity of one autoantibody was found in 1 boy with the highest risk genotype and in 2 children with lower risk genotypes. Only temporary anti-GAD65 positivity was found in girl with protective genotype. Type 1 diabetes mellitus was diagnosed in boy during IVGTT and disease manifested 6 months after IVGTT in girl with defect of FPIR. Standardised methods for prediction of Type 1 diabetes were introduced in first-degree relatives of diabetic patients. These methods are used for Czech registry of diabetic children.