Osteosarkom (OS) je nejčastější zhoubný nádor skeletu, představuje asi 35 % primárních kostních nádorů. Úspěšná léčba osteosarkomu vyžaduje úzkou spolupráci odborníků multidisciplinárního týmu, kam patří onkolog, chirurg, patolog a radiolog. Nejlepší péči zajišťují specializovaná centra s odpovídajícími zkušenostmi a možností komplexní péče. Léčebný režim musí zahrnovat radikální odstranění primárního nádoru, případně metastáz v kombinaci se systémovou chemoterapií. Z cytostatik je to zejména doxorubicin, vysoko-dávkovaný metotrexát, cisplatina a ifosfamid. Cílem rozdělení chemoterapie na část předoperační a pooperační je redukce nádorové masy a lepší ohraničení tumoru vůči okolním strukturám. Tím je usnadněna chirurgická radikalita s možností náhrady postižené části skeletu bez mutilujícího výkonu. Důležitá je také znalost histopatologické odpovědi na předoperační chemoterapii, která ovlivňuje intenzitu pooperační léčby. Dosud není dostatečně vyřešena léčba metastatického onemocnění a případné recidivy. Zlepšení výsledků je možno očekávat až po zavedení nových léčebných přípravků a modalit., Osteosarcoma (OS) is the most common malignant tumour of the skeleton accounting for approximately 35 % of primary bone tumours. Successful treatment of osteosarcoma requires a close cooperation between the specialists of a multidisciplinary team consisting of an oncologist, surgeon, pathologist and radiologist. The best care is provided by specialized centres with appropriate experience in which comprehensive care is available. The therapeutic regimen must include radical removal of the primary tumour and/or metastases in combination with systemic chemotherapy. The cytostatic drugs primarily used include doxorubicin, high-dose methotrexate, cisplatin and ifosfamide. The goal of dividing chemotherapy into preoperative and postoperative parts is a reduction in the tumour mass and improved delineation of the tumour from the surrounding structures. This improves the surgical radicality with the affected part of the skeleton being replaced without the need for a mutilating procedure. Also of importance is the knowledge of the histopathological response to preoperative chemotherapy which affects the intensity of postoperative treatment. The management of metastatic disease and possible recurrence has not been addressed adequately as yet. Improved results can be expected with the introduction of novel therapeutic agents and modalities., Hubert Mottl, Jarmila Kruseová, Jiří Schovanec, and Lit.: 55
Cieľ: Cieľom prieskumu bolo zistiť prevalenciu a rizikové faktory hypertenzie bieleho plášťa (WCH) u detí. Metodika: Formou retrospektívnej štúdie sme analyzovali zdravotné záznamy 140 detí (36 dievčat, 104 chlapcov) s vysokým krvným tlakom nameraným v kardiologickej ambulancii, u ktorých bolo vykonané 24-hodinové ambulantné monitorovanie krvného tlaku (ABPM) a určený typ hypertenzie. Hypertenzia bola diagnostikovaná, keď bol systolický a/alebo diastolický krvný tlak meraný auskultačnou technikou ≥ 95. percentil pre pohlavie, vek a výšku. WCH bola definovaná ako priemerná hodnota krvného tlaku v ambulancii nachádzajúca sa v percentilovom pásme hypertenzie a hodnota tlaku krvi počas 24-hodinového monitorovania ambulantného tlaku nachádzajúca sa v pásme normálnych hodnôt krvného tlaku. Výsledky: V skupine 140 detí bola potvrdená prítomnosť WCH u 51 detí (36,4 %) – 13 dievčat a 38 chlapcov. Z celkového súboru sa WCH vyskytovala u 36,1 % zaradených dievčat a 36,5 % zaradených chlapcov. Systolický TK sa v kardiologickej ambulancii nachádzal nad 95. percentilom, diastolický TK pod 90. percentilom. Väčšina pacientov s WCH bola vo veku 15 – 19 rokov (80,4 %) a v pásme od 3. do 90. percentilu BMI (72,5 %). U pacientov prevládala negatívna rodinná anamnéza hypertenzie (54,9 %) a zriedkavé návštevy kardiologickej ambulancie (2-4x). Krvný tlak v ambulancii merala deťom výlučne sestra. Záver: Zistenie prevalencie WCH a jej rizikových faktorov je u detí významné, pretože prevažuje názor, že v detskom veku je WCH predispozíciou k trvalej hypertenzii. Na základe zistení rizikových faktorov, môžeme vykonávať prevenciu WCH., Aim: The aim of this study was to find out the prevalence and risk factors of white coat hypertension (WCH) in children. Methods: We realized the retrospective study and analyzed records of 140 children (36 girls, 104 boys) with high blood pressure measured in cardiology outpatients' clinic, to whom a 24-hour ambulatory blood pressure monitoring (ABPM) was made and the type of hypertension was specified. Hypertension was diagnosed when systolic and/or diastolic blood pressure measurements with auscultatory technique were ≥ 95th percentile for sex, age and height. White coat hypertension (WCH) has been defined as mean blood pressure (BP) readings in outpatients' clinic being located in the hypertensive range while 24-hour ambulatory blood pressure monitoring is in the normal range. Results: In the group of 140 children the presence of WCH was confirmed in 51 children (36,4 %) - 13 girls and 38 boys. WCH occurred in 36,1 % of girls and 36,5 % of boys in the total sample of respondents. Systolic BP in cardiology outpatients' clinic reached the value above the 95th percentile, diastolic BP below the 90th percentile. The most patients with WCH were 15 – 19 years old (80,4 %) and in 3rd - 90th percentile of BMI (72,5 %). Patients prevailed negative family history of hypertension (54,9 %) and fewer visit (2-4 times) of cardiology outpatients' clinic. Blood pressure of children was measured exclusively by a nurse in this outpatients' clinic. Conclusion: Determination of the prevalence of WCH and its risk factors in children is important because of prevailing opinion that WCH in children is predisposition in permanent hypertension. Based on the detection of risk factors, we can implement prevention of WCH., Anna Ovšonková, Daniela Kubisová, and Literatura
The Material of the study has formed 78 sick, found on stationary treatment in Republican Scientific Centre Coloproktologii since 1992 on 2010. As it is seen, from table, from 78 sick mans was 58(74,3%), womans 20(25,7%). 19(24,3), sick were at age from 15 before 20 years, 49(60,2%) at age from 21 before 40 years and 13 (16,6%) sick from 41 before 60 years. The Main complaint sick at arrival were a stubborn constipations, which noted beside 70 (89,7%) sick, including absence of the independent chair existed beside 55(70,5%), but beside 54(69,2%) sick were noted periodic stomachache, growing on measure of the absence of the chair. The Ballooned belly existed beside all 78 (100%) sick moreover beside 20(25,6%) of them flatulence was constant. The Sickness and retching existed beside 24(30,7%), weakness, reduction to capacity to work beside 52(66,6%), increasing of the temperature of the body beside 10(12,8%), paradoxical diarrhoeas beside 6(7,6%) sick. Endoskopicheskiy method (rectoromonoscopy, colonoscopy) turned out to be else less informations 51,8% coincidences of the diagnosis. So we biopsy on Svensonu executed beside all sick, entered with suspicion on disease Girshprunga. In our observations from 78 sick beside 42(53,8,1%) were aboveanalni, beside 20(25,6%) sick rectalis, beside 13(16,6%)rectosigmoideys , beside 2(2,5%) leftside and beside 1(1,2%) sick subtotalis form hipoganglios. At biopsies on Svensonu on observations, from 78 sick, beside 44(56,4%) is revealled hipoganglios, but beside 35(44,8%) аganglios rectum. As can be seen from presented tables, from 78 sick beside 68(87,2%) us is executed onemoments radical operation, 10(12,8%) sick is as far as possible made resection hipoor aganglionarnaya of the zone, decompensate part of the large intestine and is formed colostomy. In all events at operations. The Remote results executed radical operation on cause disease Girshprunga traced from 1 before 10 years beside 57(73%) sick. The Results of the surgical treatment were valued on scale Vezika: good, satisfactory and unsatisfactory. In our observations beside 46(80,7%) sick results came in well, beside 10(17,5%) satisfactory and beside 1(1,8%) sick was an unsatisfactory result., Mirzahmedov M. M., Ahmedov M. A., Sapaev D. A., and Literatura
Few studies concerning the importance of wheat allergy affecting the course of atopic eczema in adolescents and adult patients exist. Aim: The evaluation if wheat allergy can deteriorate the course of atopic eczema. Follow-up of patients with confirmed food allergy to wheat. Method: Altogether 179 persons suffering from atopic eczema were included in the study: 51 men and 128 women entered the study with an average age of 26.2 (s.d. 9.5 years) Dermatological and allergological examinations were performed, including skin prick tests, atopy patch tests, and specific serum IgE for wheat, open exposure test and double-blind, placebo-controlled food challenge test with wheat flour. Results: Wheat allergy affecting the coures of atopic eczema was confirmed in eight patients (4.5%) out of 179 patients enrolled in this study by double-blind, placebo controlled food challenge test. The course of atopic eczema showed a positive trend in patients with confirmed food allergy at 3, 6, 9, 12 month follow-up (statistical evaluation with paired t-test) after the elimination of wheat flour. Conclusion: Wheat allergy may play an important role in the worsening of atopic eczema (acting as a triggering exacerbating factor) only in a minority of adolescents and adult patients (4.5% in our study). The diagnostic methods (skin prick test, specific IgE, atopy patch test, history) cannot be used as separated tests for the determination of food allergy to wheat in patients with atopic eczema.Open exposure tests and double-blind, placebo-controlled food challenge should be used for the confirmation of wheat allergy affecting the course of atopic eczema., Jarmila Čelakovská, Květuše Ettlerová, Karel Ettler, Jaroslava Vaněčková, Josef Bukač, and Literatura 34
Cieľ: V práci sme sa zamerali na vedomosti pacientov s astmou v oblastiach self-manažmentu tohto ochorenia a najmä na vplyv faktoru dĺžky trvania tohto ochorenia na úroveň vedomostí o self-manažmente. Zároveň sme zisťovali vzťah medzi úrovňou vedomostí o self-manažmente ochorenia a kontrolou astmy. Metodika: V tejto prierezovej štúdii bolo do súboru zaradených 297 respondentov. Na testovanie kontroly astmy a úrovne vedomostí pacientov sme použili valídne nástroje a to Dotazník vedomostí o self-manažmente astmy pre dospelých (Measuring asthma self-management knowledge in adults) a Test kontroly astmy (Astma Control Test ACTTM). Na štatistické spracovanie dát boli využité: Pearsonov Chi-kvadrat test nezávislosti a Spearmanov korelačný koeficient. Výsledky: Až 220 z 297 respondentov (74,07 %) preukázalo nedostatočné vedomosti, a to konkrétne v doméne Vedomosti o liečbe astmy. V oblasti Manažment bronchiálnej astmy malo nedostatočné vedomosti 191 respondentov (64,31 %). Početnosť správnych odpovedí zároveň naznačuje, že úroveň vedomostí v doménach self-manažmentu astmy, významných pre kontrolu ochorenia, bola vyššia v skupine pacientov diagnostikovaných v priemere jeden rok ako v skupine, ktorá trpí ochorením dlhšie (6-10 rokov), i keď rozdiel nie je štatisticky významný. Záver: Nedostatky vo vedomostiach astmatikov, vyššia frekvencia chýb v oblastiach self-manažmentu, vrátane self-monitoringu a horšia kontrola astmy pri ochorení diagnostikovanom viac ako 6 rokov naznačujú, že je potrebné v klinickej praxi venovať čas a pozornosť reedukácii, opakovanému posúdeniu vedomostí a praktických zručností (aplikácia metód self-manažmentu, inhalačnej techniky) v skupine už skôr diagnostikovaných astmatikov., Aim: This project is aimed to find out the patient knowledge with asthma in the area of self-management of this disease and particularly the influence of the length of the disease on the knowledge level about self-management. At the same time we tried to find out the relation between the knowledge level of the asthma self-management and control of asthma disease. Methods: The total number of 297 adult asthma patients were included in the cross sectional study. The valid instruments Asthma Control Test and Measuring asthma self management knowledge in adults were used for testing asthma control and the degree of patient knowledge in self management of asthma. The Chi-squared test and the Spearman´s rank correlation coefficient were used for statistical processing of data. Results: A total of 220 out of 279 respondents (74%) showed poor knowledge of selfmanagement of asthma, particularly in “Knowledge of asthma treatment” domain and 191 patients in “Management of bronchial asthma” domain (64%). Patients who had been diagnosed one year ago had better level of knowledge in selfmanagement of asthma than those who had been treated for asthma longer than six years (6-10 years). This difference was not significant, but it was related to those domains which has significant influence on asthma control (domains 2-5). Conclusion: Poor knowledge of asthma patients, higher frequency of errors in self management and worse control of asthma in patients with asthma longer than six years imply the need for better and repeated education, check of patient knowledge and skills for self-management, use of peak flow meter included in this group of patients., Michaela Dingová, Petronela Osacká, and Literatura
Cíl studie: Zavedení metody stanovení hladin neurotransmiterových metabolitů v mozkomíšním moku do rutinní klinické praxe je podmínkou včasné diagnostiky a léčby neurotransmiterových onemocnění v dětském věku. Materiál a metoda: Metodou vysoce účinné kapalinové chromatografie ve spojení s elektrochemickou detekcí bylo analyzováno 82 vzorků mozkomíšního moku dětských pacientů s podezřením na neurometabolické onemocnění. Výsledky: Byla stanovena věkově vázaná referenční rozmezí. Ve třech vzorcích byla prokázána patologicky snížená koncentrace kyseliny homovanilové. U dvou pacientů s významnou mikrocefalií a závažným organickým poškozením mozku se pravděpodobně jedná o sekundární deficit. Neurologický nález u třetího pacienta svědčil pro primární poruchu metabolismu neurotransmiterů na úrovni tyrosinhydroxylázy (TH). Deficit TH byl následně potvrzen na molekulárně-genetické úrovni. Závěr: Diagnostika poruch metabolismu biogenních aminů je obtížná a vyžaduje provedení lumbální punkce se speciálním odběrem mozkomíšního moku. Na našem pracovišti jsme vyšetření hladin neurotransmiterových metabolitů zařadili do rutinní diagnostiky všech dětí s podezřením na neurometabolické onemocnění. Diagnostikovali jsme prvního pacienta v České republice s primární poruchou metabolismu neurotransmiterů a zahájili léčbu., Objective: The implementation of cerebrospinal fluid neurotransmitter metabolites analysis in clinical routine is necessary for early diagnosis and treatment of pediatric neurotransmitter disorders. Material and methods: We analysed 82 cerebrospinal fluid samples from children suspected of neurometabolic disorder using high performance liquid chromatography coupled with electrochemical detection. Results: We established age-related reference values. In three samples the level of homovanillic acid was found to be markedly decreased. In two patients with severe microcephaly and extensive organic brain damage secondary deficit is concerned. Neurological findings in third patient led us to the suspicion of primary neurotransmitter metabolism disorder at the level of tyrosine hydroxylase (TH). Subsequently, TH deficiency was confirmed by molecular genetic analysis. Conclusion: Diagnosis of biogenic amines metabolism disorders is difficult and needs lumbar puncture to be performed. In our hospital we implemented cerebrospinal fluid neurotransmitter metabolites analysis into routine diagnostic workup for every patient suspected with neurometabolic disorder. We diagnosed first Czech patient with primary neurotransmitter metabolism disorder and commenced the treatment., Szentiványi K., Hansíková H., Krijt J., Zeman J., Honzík T., and Literatura 19