Brown tumors are bony lesions caused by rapid osteoclastic activity, which rare involved jaws. Renal osteodystrophy (ROD) is associated with different pathogenetic mechanisms--disorder of calcium-phosphate metabolism, impaired metabolism of vitamin D, increased parathyroid activity that lead to extreme concentrations of parathormone. The authors report two cases of jaw enlargement in patients received haemodialysis with excessive increase values of alkaline phosphatase and parathormone in serum. The patients were treated surgically with corrective procedures in maxillo-facial area. ROD of the jaws could be severe complication in dialysis patients with end stage of CKD if no appropriate care aimed at correction or prevention of parathyroid hyperfunction was applied to them. and PF. Pechalova, EG. Poriazova
Metastatický melanom je agresivní onemocnění. Dosud využívané léčebné možnosti jako aplikace dakarbazinu, temozolomidu, high dose interleukinu-2 byly relativně omezené. Vemurafenib je malá molekula, která má schopnost inhibovat mutovanou BRAF proteinkinázu. Na základě provedených klinických studií BRIM-2 a BRIM-3, kdy vemurafenib dokázal prodloužit celkové přežití (OS) a dobu do progrese onemocnění (PFS), byl zaregistrována v EU k léčbě v monoterapii u dospělých pacientů s neresekovatelným nebo metastazujícím melanomem s pozitivní mutací V600 genu BRAF., Metastatic melanoma is an aggressive disease. Historical treatment options have been limited (e.g. dacarbazine, temozolomide, high dose interleukin-2) and associated with poor outcomes. Vemurafenib is a first-in-class, small molecule BRAFV600 inhibitor. Vemurafenib is approved in the EU as monotherapy in adult patients with BRAFV600 mutation-positive unresectable or metastatic melanoma. In the trial BRIM-2 a BRIM-3 vemurafenib significantly improved both overall survival (OS) and progression free survival (PFS) in patients with unresectable melanoma., Ivo Kocák, Ilona Kocáková, Stanislav Špelda, and Literatura
Cíl. Retrospektivně zhodnotit efekt a dlouhodobou průchodnost transjugulární intrahepatální portosystémové spojky (TIPS) u nemocných s trombózou jaterních žil. Metoda. V letech 1992-2011 jsme vytvořili TIPS celkem u 38 pacientů s trombózou jaterních žil nekontrolovatelnou medikamentózní léčbou. Léčeno bylo devět mužů a 29 žen ve věku 13-76 let (medián 33 let), v šesti případech se jednalo o děti. Predisponující trombofilní stav byl prokázán u 84,2 % osob. Klinická forma Buddova-Chiariho syndromu byla v šesti případech akutní, v 18 případech subakutní a ve 14 případech chronická. Indikací k výkonu byl u 31 nemocných ascites, u pěti pacientů jaterní selhání a u dvou krvácení do zažívacího traktu. Zákroky byly prováděny standardní technikou v analgosedaci nebo v celkové anestezii. U 17 pacientů jsme do kanálu TIPS implantovali nepotažený stent nebo kombinaci potaženého a nepotaženého stentu, u 21 nemocných jsme zkrat vyztužili pouze pro TIPS dedikovaným potaženým stentem (stentgraftem). Sledování průchodnosti zkratu po výkonu bylo založeno na pravidelných ultrasonografických, klinických a laboratorních kontrolách, při prokázané poruše průchodnosti byla provedena reintervence. Výsledky. Celková doba sledování souboru byla 8 dní - 207 měsíců (medián 52 měsíců). Technického úspěchu jsme dosáhli u 100% nemocných, hemodynamická úspěšnost výkonu byla 86,8 %, s poklesem mediánu portosystémového gradientu z 23 mm Hg na 8 mm Hg, klinicky bylo dosaženo ústupu obtíží u 89,5% nemocných. Významné komplikace jsme zaznamenali celkem u sedmi osob, žádný pacient nezemřel v přímé souvislosti se zákrokem. V průběhu sledování zemřelo celkem jedenáct nemocných (29 %), přežití souboru za 1 rok a za 5 let od TIPS bylo 85,3 % a 65,4 %. Během doby sledování jsme provedli celkem 50 reintervencí pro dysfunkci spojky, průměrný počet reintervencí přepočtený na jednoho nemocného za dobu 5 let byl 1,65 výkonu u pacientů se zkratem vytvořeným stentem a 0,67 výkonu u nemocných, u kterých byl primárně použit stentgraft. Ve skupině se zavedeným nekrytým stentem byla primární průchodnost 52,9% za 1 rok a 20 % za 5 let od výkonu, u skupiny s implantovaným potaženým stentem byla jednoletá primární průchodnost 80 % a pětiletá primární průchodnost 33,3%. V průběhu sledování jsme zároveň u osmi pacientů provedli angioplastiku dolní duté žíly pro její symptomatickou stenózu, u šesti z těchto nemocných byl do dolní duté žíly zaveden stent. Závěr. Vytvoření TIPS je velmi efektivní metodou léčby pacientů s trombózou jaterních žil. Použití pro TIPS dedikovaných potažených stentů vede knižšímu počtu dysfunkcí zkratu s nižším počtem reintervencí nezbytných k obnově průchodnosti, nutností je však současná důsledná antikoagulační léčba a léčba základního hematologického onemocnění., Aim. To retrospectively evaluate the efficacy and long-term patency of transjugular intrahepatic portosystemic shunt (TIPS) in patients with hepatic vein thrombosis. Method. In years 1992-2011, totally 38 patients with hepatic vein thrombosis resistant to medical therapy underwent TIPS creation in our department. We treated 9 males and 29 females, age 13-76 years (median 33 years), 6 patients were children. The underlying thrombophilic state was confirmed in 84.2% of treated patients. The Budd-Chiari syndrome was acute in 6, subacute in 18 and chronic in 14 cases. Ascites was indication for TIPS in 31 patients, liver failure in 5 and gastrointestinal bleeding in 2 persons. All the procedures were performed in anal-gosedation or under general anestesia using standard technique. In 17 patients, we implanted non-covered stent or combination of non-covered and covered stents during TIPS creation, in the other 21 patients only the TIPS dedicated covered stents had been used. TIPS patency during follow-up after the procedure was based on regular ultra-sonographic, clinical and laboratory examinations. In case of shunt dysfunction, a rein-tervention was performed. Results. The total follow-up period was 8 days - 207 months (median 52 months). The technical success rate was 100%, hemo-dynamical success rate 86.8% with median portosystemic gradient decrease from 23 mm Hg to 8 mm Hg, and clinically we were succesfull in 89.5% of patients. In 7 patients, severe complications occurred during shunt creation, but none of the patients died in direct connection with the procedure. Totally 11 patients (29%) died during follow-up period, the 1-year and 5-years survival rates were 85.3% and 65.4%, respectively. Due to TIPS dysfunction, totally 50 reinterventions were needed, the average 5-year reintervention rate per patient was 1.65 procedures in the bare stent group and 0.67 porcedures in the covered stent group. In the non-covered stent group, we achieved primary patency rates 52.9% 1 year and 20% 5 years after TIPS creation, in the covered stent group the 1-year primary patency rate was 80% and 5-year primary patency rate 33.3%. Angioplasty of symptomatic stenosis of the inferior vena cava was performed in 8 patients during follow-up, in 6 of these patients also stent implantation was necessary. Conclusion. TIPS creation is a very effective treatment of patients with hepatic vein thrombosis. The use of TIPS dedicated covered stents leads to lower dysfunction rate with lower number of reinterventions needed to reestablish the shunt patency, but strict simultaneous anticoagulation treatment and treatment of the underlying hematologie disease are necessary., Renc O, Krajina A, Hůlek P, Lojík M, Raupach J, Chovanec V, Jirkovský V, Fejfar T, Šafka V, Pozler O, Dulíček P, Čermáková E, Machová V., and Literatura
In view of the high anti-oxidative potential oftocotrienol, the role of the tocotrienol-rich fraction (TRF) of palm oil in preventing pregnancy induced hypertension (PIH) was explored in a randomized double-blind placebo-controlled clinical trial in an urban teaching hospital. Healthy primigravidae were randomized to receive either oral TRF 100 mg daily or placebo, from early second trimester until delivery. Out of 299 women, 151 were randomized into the TRF arm and 148 into the placebo arm. A total of 15 (5.0%) developed PIH. Although there was no statistically significant difference in the incidence of PIH (4/151 or 2.6% in the TRF arm vs. 11/148 or 7.4% in the placebo arm, p = 0.058) between the two arms, there was a tendency towards a lower incidence of PIH in the TRF arm compared to the placebo arm. With TRF supplementation, the relative risk (RR) of PIH was 0.36 (95% CI 0.12-1.09). In conclusion, although TRF from palm oil does not statistically significantly reduce the risk of development of PIH in the population studied, the 64% reduction in incidence of PIH is substantial. The findings warrant further clinical trials, particularly in high risk populations. and ZA. Mahdy, HH. Siraj, H. Khaza'ai, MS. Mutalib, MH. Azwar, MA. Wahab, AZ. Dali, R. Jaafar, NA. Ismail, MA. Jamil, N. Adeeb
The present article describes two unrelated cases of progressive myoclonic epilepsy (PME) of the Lafora's disease and Unverricht-Lundborg types who were treated with topiramate (TPM) as add-on therapy for their myoclonus. After the initiation of topiramate therapy both cases responded with marked decrease in myoclonic seizure frequency and improvement of quality of life. Topiramate appears to be a useful alternative agent in cases of PME and could be consider for adjunctive therapy. and CF. Demir, HH. Ozdemir, B. Müngen
Cílem našeho sdělení je demonstrace pacienta, který si intramuskulární aplikací preparátu obsahujícího steroidy s příměsí vitaminu D navodil intoxikaci vitaminem D (1583 nmol/l) s hyperkalcémií (4,04 mmol/l) a iritací pankreatu (sérová amyláza 8,43 μkat/l)., The aim of this paper is to demonstrate a patient who induced vitamin D intoxication (1583 nmol/l) with hypercalcemia (4.04 mmol/l) and pancreatic irritation (serum amylase 8.43 μkat/l) by intramuscular application of steroids with the addition of vitamin D., Bučková D., Tůmová J., Beňovská M., Vlašínová J., and Literatura
We assessed the intensive care unit (ICU) patients for Invasive aspergillosis (IA) with culture and non-culture based diagnostic methods from Iran. Thirty-six ICU patients with underlying predisposing conditions for IA were enrolled in the study. Sixty eight Bronchoalveolar lavage (BAL) samples were collected by bronchoscope twice a weekly. BAL samples were analyzed by microscopic examination, fungal culture and galactomannan (GM) detection. The Platelia Aspergillus GM EIA was used to quantify GM indices. Samples with a BAL GM index > or = 1 were considered as positive for GM. Patients were classified as having probable or possible IA. Out of 36 suspected patients to IA, 36.1% of cases showed IA which were categorized as: 4 cases of possible IA and 9 of probable IA. 76.2% of BAL samples were positive for GM. From 13 patients with IA, 11 (84.6%) had at least one positive BAL GM index. Of these patients, 9 (81.8%) showed probable IA. The main underlying predisposing conditions were neutropenia (53.8%) and COPD (30.8%). Our study has indicated that COPD must be considered as one of the main predisposing condition for occurrence of aspergillosis in ICU patients. Our data have also revealed that GM detection in BAL samples play a significant role to IA diagnosis. and MT. Hedayati, S. Khodavaisy, M. Alialy, SM. Omran, MR. Habibi
UNLABELLED: Aim of our study was to evaluate the importance of atopy patch testing with aeroallergens as a diagnostic method in patients suffering from atopic dermatitis. METHOD: The complet dermatological and allergological examinations were performed in 29 patients; 10 men, 19 women with the average age of 27.8 years, min. 17, max. 57 years; with the median SCORAD 24.2 points, s.d. 13.3 points. Wormwood, grass, dog dander, cat dander, dermatophagoides pharinae, dermatophagoides pteronyssinus and birch pollen were examined in diagnostic procedures. Skin prick tests, specific IgE were examined; the atopy patch tests were performed with aeroallergens for skin prick tests in concentration 1 x skin prick tests. RESULTS: Specific IgE and skin prick tests to one or more tested aeroallergens were positive altogether in 27 patients; atopy patch tests were positive only in one of these patients. CONCLUSION: For atopy patch testing with aeroallergens the concentration of 1 x skin prick tests is low to confirme the eczematic reaction in patients suffering from allergy to inhallant allergens. and J. Celakovská, K. Ettlerová, K. Ettler, J. Vanecková
Benzén má hematotoxické účinky, poškodzuje centrálnu nervovú sústavu a imunitný systém, je karcinogénom kategórie 1 a mutagénom kategórie 2. Expozícia benzénu je limitovaná hygienickými predpismi. V súbore 99 pracovníkov exponovaných benzénu a 19 neexponovaných (kontrolná skupina) boli v moči stanovené dva biomarkery benzénovej expozície rôznymi analytickými metódami. Kyselina trans,trans-mukonová bola stanovená chromatograficky a fenol spektrofotometricky. Cieľom štúdie bolo porovnať mieru ich vylučovania v oboch sledovaných skupinách a dokázať vhodnosť chromatografického stanovenia kyseliny trans,trans-mukonovej v moči ako rýchly a spoľahlivý biomonitoring pracovného prostredia. Priemerná koncentrácia fenolu v oboch skupinách sa pohybovala na fyziologickej úrovni. V skupine exponovaných benzénu bola priemerná koncentrácia fenolu vyjadrená ako Priemer (SD) 11,07 (8,51) mg.g-1 kreatinínu, v kontrolnej skupine bola 12,16 (11,42) mg.g-1 kreatinínu. Priemerná koncentrácia kyseliny trans,trans-mukonovej exponovaných pracovníkov bola 0,64 (0,95) mg.g-1 kreatinínu, v porovnaní s kontrolnou skupinou 0,29 (0,24) mg.g-1 kreatinínu, jej obsah v moči exponovaných pracovníkov nepresahoval doporučenú hodnotu koncentračného limitu pre úroveň technickej smernej hodnoty benzénu v zmysle legislatívy platnej v SR, ale prekračoval doporučenú biologickú medznú hodnotu podľa Americkej rady vládnych hygienikov pre priemysel. Štatisticky t-testom bol potvrdený signifikantný rozdiel v nameraných hodnotách vylučovanej kyseliny trans,trans-mukonovej medzi kontrolnou skupinou pacientov a pracovníkov exponovaných benzénu, dokazujúci vhodnosť jej stanovenia ako rýchly a spoľahlivý biomonitoring pracovného prostredia., Benzene has hematoxic effects, causes damage to the central nervous system and immune system, is classified as a Category 1 carcinogen, and a Category 2 mutagen. Benzene exposure is limited by public health legislation. A total of 99 biological samples of benzene-exposed workers and 19 biological samples of non-exposed patients in a control group were examined. Concentration determination of two selected biomarkers of benzene exposure was performed using two different analytical methods. Trans,transmuconic acid was determined by chromatography and phenol was determined by spectrophotometry. The purpose of the study was to compare their excretion rate in both monitored groups, and to prove appropriateness of chromatographic determination of trans,trans-muconic acid in urine for quick and reliable biomonitoring of the working environment. The average phenol concentration in both groups varied at physiological levels: mean (SD) was 11.07 (8.51) mg.g-1 creatinine for a benzene-exposed group, and 12.16 (11.42) mg.g-1 creatinine for a control group. As for trans,trans-muconic acid, the average concentration was 0.64 (0.95) mg.g-1 creatinine in case of benzene-exposed workers compared to 0.29 (0.24) mg.g-1 creatinine for a control group. Concentration of trans,trans-muconic acid in urine from exposed workers did not exceed a recommended concentration limit for Threshold Limit Value level for benzene, but exceeded a recommended Biological Exposure Indices value set by the American Conference of Governmental Industrial Hygienists. A statistic t-test revealed a significant difference in measured values of excreted trans,transmuconic acid between a control group of patients and benzene-exposed workers proving appropriateness of determination for quick and reliable bio-monitoring of the working environment., Ivica Bajusová, Peter Kolarčik, Michal Ihnatko, Tatiana Kimáková, Ľubomír Legáth, and Literatura Ivica Bajusová, Peter Kolarčik, Michal Ihnatko, Tatiana Kimáková, Ľubomír Legáth: Porovnanie miery vylučovania vybraných biomarkerov expozície benzénu. Hygiena, 2013, roč. 58, č. 2, str. 70-74.
Primární nádory mozku jsou heterogenním onemocněním. Nejzávažnější diagnózu představuje glioblastoma multiforme, který tvoří 75 % z high grade gliomů. Jedná se o agresivní nádory s omezenými terapeutickými možnostmi a špatnou prognózou. Medián přežití od stanovení diagnózy je bez léčby 4,6 měsíce, s léčbou kolem 12 měsíců. Zlatým standardem terapie je v dnešní době radikální operace, chemoradioterapie a následně adjuvantní chemoterapie temozolomidem. V naší práci jsme se zaměřili na přínos přidání temozolomidu ke standardní radioterapii v porovnání s jeho toxicitou., Primary brain tumors are a heterogeneous disease. The most important is glioblastoma multiforme, which constitutes 75 % of the high grade gliomas. These are aggressive tumors with limited therapeutic options and poor prognosis. Median survival from diagnosis is 4,6 months without treatment in the management of around 12 months. The gold standard therapy today is radical surgery, chemoradiotherapy followed by adjuvant chemotherapy with temozolomide. In our work we focus on the benefit of adding temozolomide to standard radiotherapy compared with its toxicity., Kateřina Zycháčková, Markéta Pospíšková, Milan Kohoutek, Michal Filip, Alena Jakšičková, Michal Zycháček, and Literatura