Interstitial lung diseases (ILD) are a heterogeneous group of rare diffuse diseases affecting the lung parenchyma in children and adults. Childhood interstitial lung diseases (chILD) are often diagnosed at very young age, affect the developing lung, and can have different presentations and prognosis compared to adult forms of these diseases. In addition, chILD in many cases may apparently remit, and have a better response to therapy and better prognosis than adult ILD. Many affected children will reach adulthood with minimal activity or clinical remission of the disease. They need continuing care and follow-up from childhood to adulthood if the disease persists and progresses over time, but also if they are asymptomatic and in full remission. Therefore, for every chILD patient an active transition process from paediatric to adult care should be guaranteed. This European Respiratory Society (ERS) statement provides a review of the literature and current practice concerning transition of care in chILD. It draws on work in existing transition care programmes in other chronic respiratory diseases, disease-overarching transition-of-care programmes, evidence on the impact of these programmes on clinical outcomes, current evidence regarding long-term remission of chILD as well as the lack of harmonisation between the current adult ILD and chILD classifications impacting on transition of care. While the transition system is well established in several chronic diseases, such as cystic fibrosis or diabetes mellitus, we could not find sufficient published evidence on transition systems in chILD. This statement summarises current knowledge, but cannot yet provide evidence-based recommendations for clinical practice.
- MeSH
- dítě MeSH
- dospělí MeSH
- intersticiální plicní nemoci * terapie diagnóza MeSH
- lidé MeSH
- mladiství MeSH
- pneumologie normy MeSH
- přechod k lékaři pro dospělé * normy organizace a řízení MeSH
- prognóza MeSH
- společnosti lékařské MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé MeSH
- mladiství MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
- Geografické názvy
- Evropa MeSH
Čtvrté, rozšířené vydání 175 stran : ilustrace (převážně barevné) ; 31 cm
Příručka, která se zaměřuje na metody akrální koaktivační diagnostiky a terapie různých muskuloskeletálních nemocí. Určeno odborné veřejnosti.; Publikace je určená odborníkům, kteří pracují s metodami na neurofyziologických principech a jejich vědecky podložených základech.
- Klíčová slova
- akrální koaktivační terapie,
- MeSH
- kineziologie aplikovaná MeSH
- muskuloskeletální nemoci rehabilitace MeSH
- terapie cvičením MeSH
- vývoj člověka MeSH
- Publikační typ
- příručky MeSH
BACKGROUND AND OBJECTIVES: Intratumoral hemorrhage (ITH) in vestibular schwannoma (VS) after stereotactic radiosurgery (SRS) is exceedingly rare. The aim of this study was to define its incidence and describe its management and outcomes in this subset of patients. METHODS: A retrospective multi-institutional study was conducted, screening 9565 patients with VS managed with SRS at 10 centers affiliated with the International Radiosurgery Research Foundation. RESULTS: A total of 25 patients developed ITH (cumulative incidence of 0.26%) after SRS management, with a median ITH size of 1.2 cm 3 . Most of the patients had Koos grade II-IV VS, and the median age was 62 years. After ITH development, 21 patients were observed, 2 had urgent surgical intervention, and 2 were initially observed and had late resection because of delayed hemorrhagic expansion and/or clinical deterioration. The histopathology of the resected tumors showed typical, benign VS histology without sclerosis, along with chronic inflammatory cells and multiple fragments of hemorrhage. At the last follow-up, 17 patients improved and 8 remained clinically stable. CONCLUSION: ITH after SRS for VS is extremely rare but has various clinical manifestations and severity. The management paradigm should be individualized based on patient-specific factors, rapidity of clinical and/or radiographic progression, ITH expansion, and overall patient condition.
- MeSH
- krvácení chirurgie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mikrochirurgie MeSH
- následné studie MeSH
- radiochirurgie * škodlivé účinky MeSH
- retrospektivní studie MeSH
- vestibulární schwannom * chirurgie patologie MeSH
- výsledek terapie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
Research shows that male body odor plays an important role in women's mate choice and that olfactory abilities are associated with women's sexual functioning. What remains unclear is what types of partner's odor actually shape women's experience during intimate activities. This study therefore explored women's experience associated with the partner's various odors and investigated how they affect women's intimate and sexual encounters. We performed semi-structured individual interviews with 20 single women and 20 women in a long-term relationship. Thematic analysis revealed four key natural odor types of the partner: body odor, sweat, genital odor, and semen odor. Further, we have identified three main types of fragrance odor (cologne, shower gel, and laundry agents) and investigated their perception in both intimate (hugging, kissing, cuddling, lying side by side) and sexual (intercourse, oral sex, ejaculation) contexts. Both partner's natural odor and fragrance affected women's emotional state (ranging from pleasant to unpleasant) and behavioral response (ranging from approach to avoidance of partner). Women's odor perception was frequently context-dependent, so that even mostly negatively perceived odors (e.g., semen, genital odor) were often accepted as part of sexual encounter. Finally, women's perception was negatively modified by partner's specific sweat (after workday, workout, or when the partner is ill) during intimate encounters. Our results highlight the complexity and interindividual variability of partner's odor perception.
- MeSH
- čich fyziologie MeSH
- čichová percepce fyziologie MeSH
- dospělí MeSH
- interpersonální vztahy MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- odoranty * MeSH
- pot MeSH
- sexuální chování * psychologie MeSH
- sexuální partneři * psychologie MeSH
- sperma MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Chronická myeloidní leukemie (CML) je myeloprolifera- tivní onemocnění charakterizované přítomností Philadelphského chromozomu – translokací BCR::ABL1 – spojenou se vznikem fúzního proteinu Bcr-Abl s vlastní tyrosinkinázovou aktivitou. V terapii CML jsou již mnoho let využívány tyrosinkinázové inhibitory (TKI) Bcr-Abl, které blokují nadměrnou aktivitu tohoto proteinu. Postupně byly do klinického užití zavedeny TKI 1. generace imatinib, TKI 2. generace dasatinib, nilotinib a bosutinib a TKI 3. generace ponatinib. Nejmodernějším lékem, který lze využít u pacien tů s CML, je asciminib, první zástupce skupiny inhibitorů STAMP, který působí jako alosterický inhibitor kinázy Bcr-Abl a je účinný i v přítomnosti mutací BCR::ABL1 spojených s rezistencí vůči TKI. U mladších pa cien tů bez významných komorbidit je při selhání TKI 2. generace a u pokročilejších fází onemocnění vhodnou léčebnou strategií také alognní transplantace krvetvorných buněk.
Chronic myeloid leukaemia (CML) is a myeloproliferative disorder characterized by the presence of the Philadelphia chromosome, i.e. BCR::ABL1 translocation, associated with the formation of the Bcr-Abl fusion protein with intrinsic tyrosine kinase activity. Tyrosine kinase inhibitors (TKIs) of Bcr-Abl have been used in CML therapy for many years to block the excessive activity of this protein. The first- generation TKI imatinib, the second-generation TKIs dasatinib, nilotinib and bosutinib, and the third-generation TKI ponatinib have gradually been introduced into clinical use. The most recent drug that can be used in CML patients is asciminib, the first representative of the STAMP inhibitor group, which acts as an allosteric inhibitor of the Bcr-Abl kinase and is effective even in the presence of BCR::ABL1 mutations associated with TKI resistance. In younger patients without significant comorbidities, allogeneic hematopoietic cell transplantation is also an appropriate treatment strategy in the case of failure of the 2nd generation TKIs and in more advanced stages of the disease.
Paroxysmální noční hemoglobinurie (PNH) je vzácné hematologické onemocnění, jehož dominujícími projevy jsou anemie a trombotické komplikace v důsledku aktivace komplementu a intravaskulární hemolýzy. V minulosti se léčba PNH opírala zejména o podpůrnou terapii (transfuze, prevence trombóz), v posledním desetiletí však začaly být využívány monoklonální protilátky (inhibitory komplementu), které zcela změnily terapii PNH. Inhibitory C5 složky komplementu (ekulizumab, ravulizumab) výrazně snížily trombotické riziko i klinické obtíže pacien tů, u mnoha takto léčených nemocných však nadále dochází k hemolýze – příčinou je suboptimální inhibice C5 složky komplementu a absence účinku na extravaskulární hemolýzu, která je dána působením C3b složky. V terapii těchto pacien tů se nyní uplatňují blokátory alternativní dráhy komplementu, a to především pegcetakoplan, inhibitor C3 složky komplementu. Nadějným lékem je rovněž inhibitor faktoru B iptakopan, testovány jsou také kombinace inhibitoru faktoru D s ekulizumabem nebo ravulizumabem.
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare haematological disorder with its predominant mani festations being anaemia and thrombotic complications due to complement activation and intravascular haemolysis. In the past, the treatment of PNH relied mainly on supportive therapy (transfusions, thrombosis prevention), in the last decade, however, monoclonal antibodies (complement inhibitors) have been introduced and have completely changed the treatment of PNH. Complement C5 inhibitors of (eculizumab, ravulizumab) have significantly reduced the thrombotic risk and clinical symptoms of patients, but many patients treated with C5 inhibitors continue to experience haemolysis – the cause is suboptimal inhibition of the complement C5 and the lack of effect on extravascular haemolysis, which is mediated through the complement C3b. Alternative complement pathway blockers are now used in the treatment of these patients, most notably pegcetacoplan, an inhibitor of the complement C3. The factor B inhibitor iptacopan is also a promising drug, and combinations of a factor D inhibitor with eculizumab or ravulizumab are also being tested.
