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.

[Central serous choroidopathy as rare complication of the corticosteroid treatment]. / Centrálna serózna chorioretinopatia ako zriedkavá komplikácia kortikosteroidnej liecby.

The secondary glaucoma and complicated cataract are known and frequent complications of the corticosteroid treatment. The central serous choroidopathy (CSCH) is rare complication of corticotherapy in which occurs the focal serous ablation of the neurosensoric retina in the macular region caused by means of the leakage of the fluid trough the damaged retinal pigment epithelium. The authors present 5 cases of the CSCH during the corticosteroid treatment period, or the worsening of the already existing disease after the corticosteroid treatment. Despite the generally recognized fact the corticosteroids may precipitate the CSCH and they are contraindicated in its treatment, we repeatedly meet patients with this diagnosis, who are treated by means of systemic or parabulbar corticosteroids.

[Vasculitis of retinal vessels in lung tuberculosis--a case report]. / Vaskulitída sietnicových ciev pri pl'úcnej tuberkulóze--kazuistika.

The authors examined during the period 2003-2004 in an eye practice 652 patients who were hospitalized due to active lung tuberculosis. All patients were treated with combination of 3-4 antituberculotic drugs and the examination was performed to observe the possible side effects of ethambutol to the optic nerve. The most interesting was the ocular finding in a young man treated due to acute lung tuberculosis in whom during the antituberculotic therapy with combination of 4 antituberculotics, the acute retinal vasculitis developed. This type of vasculitis is rare demonstration of ocular tuberculosis. The ocular finding resolved after starting the systemic corticoid therapy.

[Sarcoidosis--a case report]. / Sarkoidóza--kazuistika.

We refer about a 47 years old woman patient with polymorphous complaints, in which the ophthalmologic examination with the findings of bilateral choroiditis and papilitis in the right eye helped to establish the diagnosis of sarcoidosis. According to the ophthalmic finding only, the treatment with Prednisone was started. With this treatment, the ocular findings improved, and polymorphous symptoms subsided. We stress the importance of good interdisciplinary cooperation.

[Changing clinical image of celiac sprue in childhood]. / Menící se klinický obraz celiakální sprue v detském veku.

BACKGROUND: Celiac sprue is considered to be the second most common chronic disease in childhood after allergic diseases. At present, the prevalence of this disease is stated as high as approximately 1% in inhabitants of the North America and Western Europe. Aetiology of celiac sprue is multifactorial as it is in other chronic diseases. Pathogenetically, it is an autoimmune disease whose main autoantigene is the tissue transglutaminase. It affects those individuals carrying HLA-DO2 or HLA-DO8 gene and those who were exposed to wheat gliadine or similar amino acids (prolamines) in rye and barley. The purpose of the present study was to find out whether clinical manifestation of celiac sprue changed in our group of patients in course of 23 years. METHODS AND RESULTS: In forty-eight children celiac sprue was diagnosed according to histopathological and histochemical findings in the small intestine mucosa. In the children examined within 5 years in 1982-1987, main clinical symptom of celiac sprue was diarrhoea. In the control group of children examined within 7 months in 2004-2005, intestinal and extraintestinal symptoms in celiac sprue were equally distributed; the so-called sleeping forms occurred too. CONCLUSIONS: It is discussed what are the trigger mechanisms and the possible danger of celiac sprue manifestations and why celiac sprue is diagnosed in the older children at present time compared with the past years.

[Central serous chorioretinopathy--treatment with beta blockers]. / Centrálna serózna chorioretinopatia--liecba betablokátormi.

The authors present an account on their first experience with treatment of central serous chorioretinopathy (CSCHR) by beta-blocking agents. In 21 patients with CSCHR Trimepranol (metipranololum) a beta non-selective blocker, 2 x 10 mg/day for two to three months was used. In 30 patients with CSCHR Vasocardin (Metoprololi tartas) was used i.e. a beta-1 selective blocking agent, 2 x 50 mg/day for two to three months. In all patients remission of the disease occurred, on average 4.5 to 4.8 weeks after the onset of treatment. During treatment of CSCHR by beta-blocking agents no significant difference was found in the action of beta selective and non-selective blockers regardless of the duration of the disease before onset of treatment and the number of relapses.

[Variations in lipid metabolism in long-term monitoring of children treated for diabetes mellitus]. / Odchylky látkové premeny lipidu pri dlouhodobém sledování detí lécených pro diabetes mellitus.

The authors investigated selected indicators of the lipid metabolism in 31 children with insulin-dependent diabetes for a period of 3 to 14 years. They divided the patients into two groups those with a glycosylated haemoglobin below or above 9 mumol/1 g haemoglobin. They compared the assembled results of the two groups and also with the results obtained in a control group. The total cholesterol was in both diabetic groups higher than in healthy subjects and was higher than the value which is considered from the aspect of the genesis and development of atherosclerosis as a risk value. Children with poorly compensated diabetes, i. e. with a glycosylated Hb level above 9 mumol had a higher cholesterol level as compared with well compensated diabetic children. The pre-beta fraction of lipoprotein increased in both groups of patients, however, more in those where the disease was not well compensated. There was a parallel decrease of the alpha fraction and the lipoprotein profile had a markedly atherogenic character. The apolipoprotein B concentration was in patients with well compensated diabetes lower, as compared with controls but in patients with poorly compensated diabetes after 4-5 years treatment is was significantly higher. Poor compensation of diabetes led after 4-5 years duration to a significant rise of the serum triglyceride level. As the blood lipid levels are influenced in a significantly way by diet, compensation of the disease and a low-fat diet are essential with regard to the results assembled in this investigation for prevention of ischaemic heart disease in diabetic subjects.(ABSTRACT TRUNCATED AT 250 WORDS)

Screening of congenital hypothyroidism in newborns in Bohemia and Moravia.

Regular examinations of thyroid function in newborn infants were started in the district of Praha 10 in 1975. Later the incidence of congenital hypothyroidism in Czechoslovakia was assessed in a pilot study including several centers in 1981. As a screening method we used the estimation of total thyroxine (T4) in a dried spot of blood obtained from a heel prick on the fifth day after birth. A total of 45,535 newborns was screened by such method and the incidence was 1:5059 of live born infants. Since 1985 the screening of congenital hypothyroidism has been extended to cover the whole territory of Czechoslovakia. Three screening centers (in Praha, Brno and Banská Bystrica) are responsible for carrying out this screening program. The first screening method employed was the determination of total thyroxine in a dried spot of blood on filter paper as mentioned above. By the end of December 1987, 313,618 samples from newborns have been assayed in Bohemia and Moravia. The incidence of congenital hypothyroidism was found to be 1:5700 of live newborn infants. The treatment of detected cases was started before the end of the first month after birth which ensured normal development of children thus afflicted. When T4 was the first method employed, a relatively high incidence of altered TBG (thyroxine binding globulin) production (1:6400) was encountered, especially in boys.

Inhibition of lipolysis by oxytocin and vasopressin.

The administration of 5 I. U. oxytocin (by quick infusion) or of 5 I. U. vasopressin-lysine (intramuscularly) to healthy subjects was followed by a significant decrease in the plasma non-esterified fatty acid level. We regard this as evidence of inhibition of basal lipolysis in the adipose tissue. Vasopressin also completely blocked an increase induced in the plasma non-esterified fatty acid level by activating hormone-sensitive lipase in the adipose tissue by the infusion of 0.5 mg noradrenaline.

Trehalase activity in diabetes mellitus and in cirrhosis of the liver.

Trehalase (an enzyme decomposing the disaccharide trehalose) activity was studied in 29 healthy subjects, 25 patients with cirrhosis and 112 diabetics. Mean trehalase activity was 176 +/- 11 units in the control group, 647 +/- 421 units in the patients with cirrhosis and 467 +/- 239 units in diabetics. The differences between the control group on the one hand and the groups with cirrhosis and diabetes on the other were statistically significant. The results show that the organism, under pathological conditions, makes far greater use of its enzymatic apparatus to assure its basic requirements, but the scatter of the values is so great that the determination of trehalase has no discriminative value in individual cases.