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.

[Eye manifestation of extrarenal malignant rhabdoid tumour]. / Ocní manifestace extrarenálního maligního rhabdoidního tumoru.

Extrarenal malignant rhabdoid tumour (EMRT) is very rare and aggresive childhood neoplasm with a rapid progression. The prognosis is still very poor with 80 % mortality rate. We report a case of a newborn baby with extrarenal malignant rhabdoid tumour of an upper eyelid. An EMRT was diagnosed based on the histological examination. This case report highlights the clinical presentation, radiological features and difficulty in diagnosis. The purpose is to underline the importance of its inclusion in the differential diagnosis of any aggresive lesion in a child. Key words: malignant rhabdoid tumour, childhood, diagnostic process.

Clinical analysis of systemic and adipose tissue levels of selected hormones/adipokines and stromal-derived factor-1.

Recent studies demonstrated that selected hormones/adipokines may be involved into the regulation of bone metabolism and bone marrow-derived hematopoietic stem/progenitor cells (HSPCs) mobilization in humans. Interestingly, in obese individuals significantly higher numbers of spontaneously circulating stem cells are also observed. Therefore in this study we comprehensively examined plasma and AT (subcutaneous and visceral/omental) levels of hormones/adipokines involved in HSPCs mobilization in lean, overweight and obese individuals as well as verified their potential associations with concentrations of HSPCs chemoattractant, stromal-derived factor-1 (SDF-1). Blood and AT samples (35 subcutaneous and 35 omental) were obtained from individuals undergoing elective surgery. Plasma and AT-derived interstitial fluid levels of resistin, visfatin, osteocalcin and SDF-1 were measured using ELISA. In our study obese patients had almost significantly (P<0.06) higher plasma visfatin and resistin levels as well as lower osteocalcin concentrations (P<0.04) than lean individuals. Osteocalcin and resistin concentrations were strongly associated with levels of SDF-1 and metalloproteinases (MMP 2 and 9). AT levels of all examined substances were significantly lower than the corresponding levels in the plasma (in all cases at least P<0.05), and depot-specific differences in the concentrations of these factors were found only in terms of osteocalcin and SDF-1. In addition, subcutaneous and visceral/omental concentrations of osteocalcin and visfatin, but not of resistin, were associated with values of such parameters as age, body mass or adiposity indexes (BMI and BAI, respectively) and/or waist-to-hip ratio (WHR). In summary, our study showed that in obese individuals the biochemical constellation of adipokines/hormones involved in the process of HSPCs mobilization resembles this observed during pharmacological HSPCs mobilization. Moreover, our study offers further indirect translational evidence for existence of a biochemical cross-talk between bone and AT metabolism (so called - bone-fat- axis) in humans.