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.

[Diagnostic options in chronic recurrent multifocal osteomyelitis: case reports]. / Moznosti diagnostiky chronickej multifokálnej recidivujúcej osteomyelitídy: kazuistiky.

Chronic recurrent multifocal osteomyelitis (CRMO) is the most severe form of chronic non-bacterial osteomyelitis. In children and adolescents, the metaphyses of long bones are affected most frequently, but the lesions can be found at any site on the skeleton, as well as in other organs such as the skin, eyes, gastrointestinal tract or lungs. Since the clinical signs of CRMO and its course are not invariable and clear, it is often very difficult to make a clinical diagnosis. The authors present their experience with the CRMO diagnosis in three cases. In the first patient, CRMO was localised in the left proximal tibia, left proximal femur, left sacrum and left clavicle bone. In the second patient, it was found in the left distal tibia, tarsometatarsal joints of the left foot and left distal femur. The third patient showed lesions in the inferior pubic ramus and ischial bone on the left side.With interdisciplinary co-operation it was possible to make the exact diagnosis of CRMO in all three female patients, and the disease was successfully managed. A precise therapeutic procedure could be derived from the results of randomised controlled studies which, however, cannot be made because of an infrequent occurrence of this disease. A meta-analysis of the cases so far published in the literature would be a more likely option.

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

We report herein the results of the cross-cultural adaptation and validation into the Slovak language of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The Slovak CHAQ CHQ were fully validated with 3 forward and 3 backward translations. A total of 119 subjects were enrolled: 52 patients with JIA (15% systemic onset, 37% polyarticular onset, 10% extended oligoarticular subtype, and 38% persistent oligoarticular subtype) and 67 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA patients, with the systemic, polyarticular and extended oligoarticular subtypes having a higher degree of disability, pain, and a lower overall well-being when compared to their healthy peers. Also the CHQ clinically discriminated between healthy subjects and JIA patients, with the systemic onset, polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the Slovak version of the CHAQ-CHQ is a reliable, and valid tool for the functional, physical and psychosocial assessment of children with JIA.

[Endothelial dysfunction and lipid profiles: analysis of the EDO Study]. / Endotelová dysfunkcia a lipidový profil: analýzy stúdie EDO.

On the basis of experimental as well as clinical observations, endothelial dysfunction is (defined as impaired or absent endothelium-dependent relaxation) considered to be an important factor in the atherogenesis. Serum lipids abnormalities have been accepted as an epidemiological risk factor of atherosclerosis. In vitro, experimental as well as epidemiological studies revealed the fact, that lipoprotein oxidation plays an important role in atherogenesis. Recently invented non-invasive methods to test and measure the endothelial function in vivo opened the opportunity to study the influence of different serum lipids on the endothelial function directly. Therefore, we decided to employ this non-invasive method for studying the endothelial function and observe the influence of various levels of plasma lipids and lipoprotein oxidation on the endothelial function of arteries in middle-aged men, since they are the most endangered part of population. In our study we used a method of measuring the diameter of a. radialis by high-resolution ultrasound (Sonoline 450, Siemens, Japan) and further mathematical and statistical analysis of functional as well as relative vasodilation reserve followed these measurements. Blood samples were taken within 24 hours of ultrasonography to study serum lipids (total cholesterol, HDL, triglycerides) and parameters of oxidation/antioxidation (superoxid dismutase--SOD, malondialdehyde--MDA). Sixty men, 25-45 years old, from an area of basically the same level of pollution were examined. We found a negative correlation between FVDR and TCH (p = 0.01), FVDR and Tg (p = 0.002) and FVDR a TCH/HDL (p = 0.015). Positive correlation exists (p < 0.001) between TCH, Tg levels and TCH/HDL ratio and MDA level in all cases. Analysing further data from the EDO Study, we can conclude, that increased plasma lipids are more likely to be oxidized, which, in turn, is the probable reason of endothelium-dependent vasodilation impairment.

[Selected parameters of lipoprotein metabolism in cerebrovascular diseases]. / Vybrané parametre metabolizmu lipoproteínov pri cerebrovaskulárnych ochoreniach.

In series of patients with stroke, selected by random (n = 68), mean age 62.44 +/- 9.12 years (range 39-82 yrs), there were 23 females (33.8%), mean age 65.43 +/- 10.11 yrs and 45 males (66.2%) mean age 60.8 +/- 8.3 yrs. Lp(a) reference values have been obtained from a group of 283 healthy individuals (age ranging from 15 to 65 years). The cholesterol, triacyglycerol, Apo B reference values come from the database of the Department of Clinical Biochemistry. There were 52 hypoxemic stroke patients in the whole observed group. Triacylglycerol serum level TAG < or = 2.89 mmol/l was observed in 47 cases (90.3%), the serum level TAG > 2.89 mmol/l was present in 5 cases (9.7%). The occurrence of TAG normal serum level was significantly more frequent than its pathologic increase (p < 0.001). Apolipoprotein Apo B < or = 1.67 g/l serum level was present in 41 (78.8%) and Apo B > 1.67 g/l in 11 (21.2%) cases (p < 0.001). Apo B < or = 1.67 g/l serum levels in 23 cases (82.1%) and Apo B > 1.67 g/l in 5 cases (18%) were observed among the stroke diabetes mellitus patients (n = 28)--statistic difference in 1/1000 level. In the total hypoxemic stroke group (n = 52), Lp(a) < or = 0.278 g/l was observed in 44 cases (84.6%), Lp(a) > 0.278 g/l serum level was present in 8 cases (15.4%)/ - p < 0.001. According to EASD consensus the serum level of Lp(a) = 0.278 g/l has been considered as "cut-off limit". Similar distribution of Lp(a) serum levels was observed in the diabetes mellitus stroke group (n = 28), the ischemic heart group (n = 54), the group with aortosclerosis (n = 16) and in the group with arterial hypertension (n = 50). Elevated TAG serum levels were not in correlation with the number of sites where atherosclerotic changes were proved by arteriography, ultrasound investigation e.g. in the extracranial brain supplying arteries. Elevated Lp(a) serum levels did not correlate with the stage of ischemic heart disease and they correlated with the stage of functional CNS defect in arterial hypertension and atherosclerosis. Metabolic disorders of lipoprotein and apolipoprotein, namely genomic transcription of lipoprotein seem to be more significant risk stroke factors, but, if they are present, they contribute to the occurrence of arteriosclerosis of some larger arteries. Elevated Lp(a) serum levels did not correlate with the stage of the heart ischemic disease and aortosclerosis, but they correlate with the stage of functional CNS defect due to arteriosclerosis and arterial hypertension, hence the increase in Lp(a) serum level as an indicator of arteriosclerotic evolution of cerebral arteries is significant. Our results, hence, do confirm a common supposition for Lp(a) serum level as an independent arteriosclerotic risk factor of the brain arteries. (Fig. 7, Tab. 1, Ref. 22.)

[Short-term administration of increasing doses of thyroxine has a different effect on serum lipids in male rats and in female rats]. / Krátkodobá aplikácia stúpajúcich dávok tyroxínu má rozdielny úcinok na lipidy séra u samcov a samíc potkanov.

The effects of short-term administration of thyroxine (T4) on serum levels of cholesterol, HDL-cholesterol and triacylglycerols were studied on young adult rats. T4 was injected in three different doses, i.e. 125, 250 and 500 micrograms for four days. The results show significant sexual differences. T4 caused a significant decrease in HDL-cholesterol levels in both sexes. The concentration of cholesterol and triacylglycerol decreased only in males, namely after injections of the highest dose of T4. In females the T4 injections caused no effect in the investigated parameters. It is concluded, that also in short-term applications of hormones, the sex may significantly change the results. Presented results support the latest views on specific interrelationship between thyroid hormone and HDL in comparison with other types of LP. (Tab. 2, Ref. 16.).

[Changes in cholesterol and triacylglycerol serum levels in rats during postnatal ontogenesis]. / Zmeny v koncentrácii cholesterolu a triacylglycerolov v sére potkanov pocas postnatálnej ontogenézy.

In the serum of young normal rats during the first two months of life the concentrations of triacylglycerols (TG) and of cholesterol (CH) were determined. Low values in both studied parameters found in newborn animals were followed by a marked increase during the first week of life. In comparison with adult animals high values remained during the first five weeks of life. The results are discussed with regard to the high intake of lipids by milk during the period of sucking, as well as from the point of possible mobilization of tissue lipid stores because of the similar trend in the ontogenetic expression of the gene coding the hormone-sensitive lipase (HSL). On the basis of evaluation of the analytical as well as biological variance it was found, that for the level of 50% of confidentiality the serum concentrations of cholesterol should be in the range X +/- 0.26 mmol/l. For the level of 95% the range of values is X +/- 0.67 mmol/l. It is suggested that these results could be used as reference values for serum lipids in the rat during ontogenesis. (Fig. 1, Ref. 21.)