Onemocnění Bedřicha Smetany spojené s kompletní ztrátou sluchu je v dlouho probíhající diskusi dáváno do souvislosti s různými chorobami. Uvádí se senilní demence, ateroskleróza, progresivní paralýza při lues, a přitom je opomíjena ototoxicita rtuti. Její vysoká koncentrace v kosterních ostatcích skladatele byla prokázána autory tohoto článku imunohistochemicky, a především metodou neutronové aktivační analýzy (NAA) již před 23 roky. Význam tohoto nálezu byl stále opomíjen v souvislosti s dominující snahou prokázat venerické onemocnění skladatele, jehož diagnóza však nebyla zatím jednoznačně ověřena. Proto byly revidovány dosavadní výsledky osteologického vyšetření a v dostupném vzorku bylo doplněno imunohistochemické vyšetření za použití protilátky proti treponemám a stříbřicí metoda s negativním výsledkem. Na druhé straně odborná rešerše literatury zaměřená na ototoxicitu rtuti potvrdila náš předpoklad, že by se na ztrátě sluchu mohla podílet chronická intoxikace tímto kovem, který byl zřejmě součástí mastí aplikovaných nejen v dětství, ale i později v dospělosti. Tento kov donedávna používaný v dermatologii jako součást mastí se resorbuje, a jak bylo recentně prokázáno, vyvolává různé neuropsychické poruchy, včetně poruch až ztráty sluchu. Svým sdělením bychom chtěli iniciovat novou diskusi o všech aspektech skladatelova onemocnění.

Bedřich Smetana’s illness associated with complete hearing loss has been linked to various diseases in a long-running discussion. Senile dementia, atherosclerosis, progressive paralysis in lues are mentioned, while the ototoxicity of mercury is neglected. Its high concentration in the composer's skeletal remains was demonstrated by the authors of this article immunohistochemically and especially by the neutron activation analysis (NAA) method 23 years ago. The significance of this find has been neglected in connection with the dominant effort to prove the composer's venereal disease, the diagnosis of which, however, has not yet been unequivocally verified. Therefore, the existing results of the osteological examination were revised and the available sample was supplemented with an immunohistochemical examination using an antibody against treponemes and a silvering method with a negative result. On the other hand, an expert literature search focused on mercury ototoxicity confirmed our assumption that chronic intoxication with this metal, which was probably part of ointments applied not only in childhood but also later in adulthood, could contribute to hearing loss. This metal, until recently used in dermatology as a component of ointments, is resorbed and, as has recently been proven, causes various neuropsychic disorders, including disorders and even hearing loss. With this communication, we would like to initiate a new discussion on all aspects of the composer’s illness.

• MeSH
• diferenciální diagnóza MeSH
• hluchota * chemicky indukované   MeSH
• hudba MeSH
• lidé MeSH
• ototoxicita MeSH
• otrava rtutí diagnóza   komplikace   MeSH
• významné osobnosti * MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• práce podpořená grantem MeSH

AIM: To evaluate the most important factors of quality of life in patients after vestibular schwannoma surgery. MATERIALS AND METHODS: Patients with unilateral sporadic occurrence of vestibular schwannoma who underwent surgery via suboccipital-retrosigmoid approach were included in the prospective study (2018-2021). Patients after previous Leksell gamma knife irradiation (or other methods of stereotactic radiosurgery) were excluded. Quality of life was assessed using 10 validated questionnaires that were distributed preoperatively, 3 months and 1 year after the surgery. RESULTS: A total of 76 patients were included in the study, complete data were analysed in 43 of them (response rate 57%). Grade III and IV represented up to 70% of all tumors. Patients with larger tumors had a significantly higher risk of postoperative facial nerve paresis, liquorrhea and lower probability of hearing preservation. Patients with smaller tumors and those, who suffered from headaches before surgery had more frequent and severe headaches after surgery. Postoperative headaches were associated with higher incidence of anxiety and tinnitus. More frequent anxiety was also identified in patients with preoperative serviceable hearing who became deaf after surgery. Nevertheless, tinnitus and hearing impairment appeared to have less impact on overall quality of life compared to headaches and facial nerve function. CONCLUSION: According to our results, tumor size, postoperative function of the facial nerve and occurrence of postoperative headaches had the greatest influence on the overall postoperative quality of life in patients after vestibular schwannoma surgery.

• MeSH
• bolesti hlavy MeSH
• kvalita života MeSH
• lidé MeSH
• prospektivní studie MeSH
• tinnitus * MeSH
• vestibulární schwannom * chirurgie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

Úvod: Pacienti s těžkým průběhem CRSwNP (chronic rhinosinustis with nasal polyps), kteří splňují dle EPOS 2020 indikační kritéria, jsou indikováni k biologické léčbě. V patogenezi onemocnění se významně uplatňuje imunopatologická odpověď II. typu. V léčbě se využívají monoklonální protilátky, které blokují signalizaci zánětlivých cytokinů. V České republice jsou schváleny tři preparáty – dupilumab, mepolizumab a omalizumab. Metodika: Retrospektivním sledováním jsme vytvořili soubor 26 pacientů léčených na Klinice otorinolaryngologie a chirurgie hlavy a krku ve Fakultní nemocnici v Motole v období od listopadu 2020 do prosince 2023, kteří byli léčeni dupilumabem. V souboru jsme sledovali vývoj hodnot NPS (nasal polyp score), SNOT-22 (22-item sinonasal outcome test), OMT (odourized markers test, test parfémovaných fixů), hodnotu IgE, hladinu eozinofilie a TKA (test kontroly astmatu) od počátku léčby a v 16. týdnu, v 1. roce a ve 2. roce léčby. Výsledky: Dle výsledků dochází ke statisticky signifikantnímu zlepšení nejen subjektivních symptomů, ale i objektivního nálezu většiny proměnných. Po 1. roce od léčby jsme zjistili statisticky signifikantní zlepšení skóre NPS (z 6,15 ± 1,35 na 1,69 ± 1,76), SNOT-22 (z 58,83 ± 17,37 na 15,33 ± 16,80), OMT (z 1,52 ± 1,83 na 5,88 ± 3,5) a IgE (z 245 ± 251 kIU/litr na 64 ± 68 kIU/litr). Ve studii byla na našem souboru pacientů prokázána účinnost biologik. Závěr: Cílem práce je informovat o prvních výsledcích biologické léčby pacientů s chronickou rinosinusitidou s nosní polypózou léčených na Klinice otorinolaryngologie a chirurgie hlavy a krku 1. LF Fakultní nemocnice v Motole. V naší studii došlo ke statisticky významnému zlepšení SNOT-22, OMT, NPS a sérové hladiny IgE. Výsledky byly statisticky signifikantní při porovnání vstupních hodnot s 16. týdnem kontroly, stejně tak při porovnání vstupních hodnot s kontrolou v 1. roce. V průběhu léčby jsme se nesetkali se žádnou závažnou komplikací.

Introduction: Patients with severe chronic rhinosinusitis with nasal polyps (CRSwNP), meeting the EPOS 2020 criteria, are indicated for biological treatment. The pathogenesis of the disease significantly involves a type II immunopathological response. Treatment involves monoclonal antibodies that block the signaling of inflammatory cytokines. In the Czech Republic, three medications are approved – dupilumab, mepolizumab, and omalizumab. Methodology: A retrospective study was conducted, comprising of 26 patients treated with dupilumab at the Department of Otorhinolaryngology and Head and Neck Surgery at Motol University Hospital from November 2020 to December 2023. In the study cohort, we monitored the progression of nasal polyp score (NPS) scores, 22-item Sinonasal Outcome Test (SNOT-22), Odourized Markers Test (OMT), IgE levels, eosinophil count, and Asthma Control Test from the beginning of treatment and at week 16, 1 year, and 2 years into treatment. Results: According to the results, there is a statistically significant improvement not only in subjective symptoms but also in the objective findings of most variables. After 1 year of treatment, we observed a statistically significant improvement in NPS (from 6.15 ± 1.35 to 1.69 ± 1.76), SNOT-22 (from 58.83 ± 17.37 to 15.33 ± 16.80), OMT (from 1.52 ± 1.83 to 5.88 ± 3.5), and IgE levels (from 245 ± 251 kIU/l to 64 ± 68 kIU/l). In the study, the effectiveness of biologics was demonstrated in our patient cohort. Conclusion: The aim of this study is to report the initial outcomes of biologic treatment in patients with chronic rhinosinusitis with nasal polyposis treated at the Department of Otorhinolaryngology and Head and Neck Surgery, 1st Faculty of Medicine, Motol University Hospital. Our study demonstrated a statistically significant improvement in SNOT-22, OMT, NPS, and serum IgE levels. The results were statistically significant when comparing baseline values with the 16-week follow-up, as well as when comparing baseline values with the 1-year follow up. No serious complications were encountered during the course of treatment.

• MeSH
• biologická terapie metody   MeSH
• dospělí MeSH
• humanizované monoklonální protilátky aplikace a dávkování   MeSH
• kvalita života MeSH
• lidé středního věku MeSH
• lidé MeSH
• nosní polypy * farmakoterapie   imunologie   komplikace   patologie   MeSH
• olfaktometrie metody   statistika a číselné údaje   MeSH
• rinosinusitida * farmakoterapie   imunologie   komplikace   patologie   MeSH
• senioři MeSH
• statistika jako téma MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• klinická studie MeSH
• práce podpořená grantem MeSH

Mikrochirurgická exstirpace je jednou ze základních modalit léčby pacientů s vestibulárním schwannomem. S ohledem na významný pokles mortality a morbidity pacientů s vestibulárním schwannomem v uplynulých dekádách, se kvalita života stala nejvýznamnějším kritériem současného managementu léčby. I přes velký vědecko-technologický pokrok se v chirurgické léčbě vestibulárních schwannomů můžeme setkat s řadou závažných komplikací, které mohou mít na kvalitu života zcela zásadní vliv. Řada dotazníkových studií potvrzuje, že se jedná zejména o pooperační lézi lícního nervu, poruchu rovnováhy a dlouhodobou bolest hlavy. Naproti tomu tinnitus nebo jednostranná hluchota může z pohledu celkové kvality života představovat méně závažný handicap.

Microsurgical extirpation is one of the basic modalities of treatment for patients with vestibular schwannoma. Considering the significant decrease in mortality and morbidity of patients with vestibular schwannoma in the past decades, the quality of life has become the most important criterion of current treatment management. Despite the great scientific and technological progress in the surgical treatment of vestibular schwannoma, we can encounter a number of serious complications that can have a major impact on the quality of life. A number of questionnaire studies confirm that these are mainly postoperative facial nerve lesion, dizziness and long-term headache. In contrast, tinnitus or unilateral hearing loss may be a less serious handicap in terms of overall quality of life.

• MeSH
• bolesti hlavy etiologie   prevence a kontrola   terapie   MeSH
• kvalita života MeSH
• lidé MeSH
• nemoci vestibulokochleárního nervu chirurgie   komplikace   MeSH
• neurochirurgické výkony metody   škodlivé účinky   MeSH
• pooperační komplikace * etiologie   patofyziologie   terapie   MeSH
• poruchy sluchu etiologie   patofyziologie   MeSH
• rizikové faktory MeSH
• tinnitus etiologie   patofyziologie   MeSH
• únik mozkomíšního moku etiologie   MeSH
• vestibulární nemoci etiologie   MeSH
• vestibulární schwannom * chirurgie   komplikace   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• práce podpořená grantem MeSH
• přehledy MeSH

INTRODUCTION: Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors, which are mostly benign in nature. Amongst all genes, Succinate Dehydrogenase Subunit D (SDHD) is the most commonly mutated in familial HNPGLs. In about 30% of HNPGLs, germline mutations in SDHD can also occur in the absence of positive family history, thus giving rise to "occult familial" cases. Our aim was to evaluate the pattern of SDHD germline mutations in Czech patients with HNPGLs. MATERIALS AND METHODS: We analyzed a total of 105 patients with HNPGLs from the Otorhinolaryngology departments of 2 tertiary centers between 2006 - 2021. All underwent complex diagnostic work-up and were also consented for genetic analysis. RESULTS: Eighty patients aged 13-76 years were included; around 60% with multiple PGLs were males. Carotid body tumor was the most frequently diagnosed tumor. Germline SDHD mutation was found in only 12% of the Czech patients; approximately 78% of those harboring the mutation had negative family history. The mutation traits had higher affiliation for multiple tumors with nearly 70% patients of ≤ 40 years of age. CONCLUSION: An SDHD mutation variant was shared amongst unrelated patients but no founder-effect was established. Our findings confirmed that the pattern of SDHD mutation distribution amongst HNPGLs in Czech Republic differs from most studies worldwide.

• MeSH
• dospělí MeSH
• incidence MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mutace MeSH
• nechromafinní paragangliom * MeSH
• paragangliom * epidemiologie   genetika   diagnóza   MeSH
• senioři MeSH
• sukcinátdehydrogenasa genetika   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika MeSH

BACKGROUND AND AIM: Minimally invasive endoscopic techniques are the treatment of choice for Zenker's diverticulum (ZD). Our objective was to evaluate the results and efficacy of endoscopic and open approaches, to determine whether postoperative C-reactive protein (CRP) serum levels can be used as a marker to exclude the presence of esophageal leakage, and to identify risk factors for development of postoperative leakage and complications. MATERIALS AND METHODS: Patients who underwent transcervical myotomy and diverticulotomy (TMD) or endoscopic cricopharyngeal myotomy (ECM) with CO2 laser for ZD in years 2008-2021 at our department were included in this retrospective study. RESULTS: Of the 101 patients enrolled, 83 patients underwent ECM and 18 TMD. The procedure time of the endoscopic technique was significantly shorter compared to that of transcervical method (p < 0.001). The median time to oral intake was 6 days for ECM and 10.5 days in the TMD group (p < 0.001). There was a 13% (n = 11) and 16.7% (n = 3) symptomatic recurrence rate and 3.6% (n = 3) and 16.7% (n = 3) major complications rate for ECM and TMD groups, respectively. The incidence of contrast leak (CL) evaluated by postoperative swallow study was 8.9% (9 from 83 patients in the ECM group only). In patients with CL, a significant increase in CRP levels on postoperative day (POD) 2 and 3 was detected when compared to patients without CL. CONCLUSIONS: Endoscopic diverticulotomy with CO2 laser represents a safe and efficient treatment of ZD. Elevation of postoperative CRP serum levels over 123.8 mg/L on POD2 and 98.8 mg/L on POD3 may indicate presence of an esophageal leakage.

• MeSH
• C-reaktivní protein MeSH
• ezofágoskopie metody   MeSH
• lasery plynové * terapeutické užití   MeSH
• lidé MeSH
• oxid uhličitý MeSH
• retrospektivní studie MeSH
• výsledek terapie MeSH
• Zenkerův divertikl * chirurgie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

Nano-sized carriers are widely studied as suitable candidates for the advanced delivery of various bioactive molecules such as drugs and diagnostics. Herein, the development of long-circulating stimuli-responsive polymer nanoprobes tailored for the fluorescently-guided surgery of solid tumors is reported. Nanoprobes are designed as long-circulating nanosystems preferably accumulated in solid tumors due to the Enhanced permeability and retention effect, so they act as a tumor microenvironment-sensitive activatable diagnostic. This study designs polymer probes differing in the structure of the spacer between the polymer carrier and Cy7 by employing pH-sensitive spacers, oligopeptide spacers susceptible to cathepsin B-catalyzed enzymatic hydrolysis, and non-degradable control spacer. Increased accumulation of the nanoprobes in the tumor tissue coupled with stimuli-sensitive release behavior and subsequent activation of the fluorescent signal upon dye release facilitated favorable tumor-to-background ratio, a key feature for fluorescence-guided surgery. The probes show excellent diagnostic potential for the surgical removal of intraperitoneal metastasis and orthotopic head and neck tumors with very high efficacy and accuracy. In addition, the combination of macroscopic resection followed by fluorescence-guided surgery using developed probes enable the identification and resection of most of the CAL33 intraperitoneal metastases with total tumor burden reduced to 97.2%.

• MeSH
• chytré polymery * MeSH
• fluorescence MeSH
• fluorescenční barviva chemie   MeSH
• lidé MeSH
• nádorové buněčné linie MeSH
• nádorové mikroprostředí MeSH
• nádory hlavy a krku * diagnostické zobrazování   chirurgie   MeSH
• polymery MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH

This is the first histological and molecular analysis of two chondrosarcomas with target-like chondrocytes that were compared with a group of conventional chondrosarcomas and enchondromas. The unique histological feature of target-like chondrocytes is the presence of unusual hypertrophic eosinophilic APAS-positive perichondrocytic rings (baskets). In the sections stained with Safranin O/Fast green, the outer part of the ring was blue and the material in the lacunar space stained orange, similarly to intercellular regions. Immunohistochemical examination showed strong positivity for vimentin, factor XIIIa, cyclin D1, osteonectin, B-cell lymphoma 2 apoptosis regulator (Bcl-2), p53 and p16. The S-100 protein was positive in 25 % of neoplastic cells. Antibodies against GFAP, D2-40 (podoplanin), CD99, CKAE1.3 and CD10 exhibited weak focal positivity. Pericellular rings/baskets contained type VI collagen in their peripheral part, in contrast to the type II collagen in intercellular interterritorial spaces. Ultrastructural examination revealed that pericellular rings contained an intralacunar component composed of microfibrils with abundant admixture of aggregates of dense amorphous non-fibrillar material. The outer extralacunar zone was made up of a layer of condensed thin collagen fibrils with admixture of non-fibrillar dense material. NGS sequencing identified a fusion transcript involving fibronectin 1 (FN1) and fibroblast growth factor receptor 2 (FGFR2) at the RNA level. At the DNA level, no significant variant was revealed except for the presumably germline variant in the SPTA1 gene.

• MeSH
• chondrocyty chemie   patologie   ultrastruktura   MeSH
• chondrosarkom * chemie   diagnóza   patologie   MeSH
• extracelulární matrix chemie   metabolismus   ultrastruktura   MeSH
• imunohistochemie MeSH
• lidé MeSH
• nádory kostí * diagnóza   metabolismus   MeSH
• proteiny S100 metabolismus   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Here we present our experience with the occurrence of neoplastic chondrocytes with target-like appearance surrounded with unusual hypertrophic thick eosinophilic perichondrocytic rings (baskets), sometimes containing two or several layers. METHODS AND RESULTS: Pericellular rings (baskets) were positive in APAS and Masson's staining method and showed immunoreactivity with antibody against type IV collagen. Such single cells or small groups of such cells were observed rarely in 3 osteochondromas, 2 skeletal chondromas, 2 extraskeletal chondromas and 2 skeletal and 1 laryngeal chondrosarcomas. Moreover, 1 unusual soft tissue chondrosarcoma was composed entirely of target-like chondrocytes with hypertrophic extremely thick perichondrocytic rings. Such cartilage-forming tumour with target-like cells, which, to the best of our knowledge, is the first such chondrosarcoma reported in the literature. Ultrastructural evidence is presented that perichondrocytic rings have complicated structure.They contained microfibrillar component with abundant admixture of irregular aggregates of dense amorphous non-fibrillar material localised in lacunar spaces. In outer part of the rings predominated microfibrillar structures corresponding to type VI collagen that produced a rather dense capsule-like demarcation line against surrounding intercellular spaces. CONCLUSIONS: The described unusual changes are probably the result of hyperproduction and remodelation of perichondrocytic matrix by abnormal neoplastic chondrocytes in response to unknown factors. Local vascular and molecular signals, may be supposed as probable causes of this phenomenon.

• MeSH
• chondrocyty MeSH
• chondrom * MeSH
• chondrosarkom * MeSH
• chrupavka MeSH
• lidé MeSH
• nádory kostí * MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

The study describes the synthesis, physicochemical properties, and biological evaluation of polymer therapeutics based on N-(2-hydroxypropyl)methacrylamide (HPMA) copolymers intended for a tumor-targeted immuno-oncotherapy. Water-soluble linear and cholesterol-containing HPMA precursors were synthesized using controlled reversible addition-fragmentation chain transfer polymerization to reach molecular weight Mn about 2 × 104 g·mol-1 and low dispersity. These linear or self-assembled micellar conjugates, containing immunomodulatory agent cucurbitacin-D (CuD) or the anticancer drug doxorubicin (Dox) covalently bound by the hydrolytically degradable hydrazone bond, showed a hydrodynamic size of 10-30 nm in aqueous solutions. The CuD-containing conjugates were stable in conditions mimicking blood. Importantly, a massive release of active CuD in buffer mimicking the acidic tumor environment was observed. In vitro, both the linear (LP-CuD) and the micellar (MP-CuD) conjugates carrying CuD showed cytostatic/cytotoxic activity against several cancer cell lines. In a murine metastatic and difficult-to-treat 4T1 mammary carcinoma, only LP-CuD showed an anticancer effect. Indeed, the co-treatment with Dox-containing micellar polymer conjugate and LP-CuD showed potentiation of the anticancer effect. The results indicate that the binding of CuD, characterized by prominent hydrophobic nature and low bioavailability, to the polymer carrier allows a safe and effective delivery. Therefore, the conjugate could serve as a potential component of immuno-oncotherapy schemes within the next preclinical evaluation.

• Publikační typ
• časopisecké články MeSH