OBJECTIVES: Despite increasing interest, prospective data on the use of degradable starch microsphere-transarterial chemoembolization (DSM-TACE) in the management of patients with unresectable HCC are still scarce. The objective of the HepaStar study was to collect prospective safety and effectiveness data in a prospective multicenter observational study. MATERIALS AND METHODS: Between January 2017 and December 2022, consecutive participants with unresectable or recurrent HCC treated with DSM-TACE as standard of care at 6 participating centers in Europe were enrolled. Tumor response was evaluated according to the mRECIST criteria. Overall survival (OS), progression-free survival (PFS), and adverse events (AEs) were assessed by using Kaplan-Meier analysis and Common Terminology Criteria for Adverse Events, version 5. Liver function deterioration was assessed by monitoring changes in liver blood tests during the follow-up. RESULTS: Seventy-nine participants (median age, 69 years (IQR, 51-87 years); 67 men (85%)) were enrolled and treated. The median follow-up time was 18 months (IQR 9.5-38.0 months). The estimated median OS and PFS for the entire cohort was 32 months (CI, 95% 21-NaN) and 9 months (CI, 95% 7-NaN), respectively. Eleven (13.9%) participants experienced at least one grade 3 or 4 AE. The most frequent grade 3-4 AE was elevated bilirubin (2.2%, 5 of 79). Deterioration of bilirubin, AST, ALT, and albumin were observed in 24.1%, 23.7%, 19%, and 24% of participants, respectively. CONCLUSION: DSM-TACE achieves promising survival in patients with unresectable or recurrent HCC. This technique shows a favorable safety profile both in terms of treatment-related AEs and liver function deterioration. KEY POINTS: Question Although degradable starch microspheres transarterial chemoembolization is widely used in clinical practice across Europe, prospective data on its application in hepatocellular carcinoma patients remains limited. Findings Degradable starch microspheres transarterial chemoembolization results in promising survival rates, good tumor response rates, and low rates of treatment-related adverse events. Clinical relevance In patients with unresectable hepatocellular carcinoma, degradable starch microspheres transarterial chemoembolization represents a safe and effective alternative to more well-established chemoembolization techniques like conventional transarterial chemoembolization and drug-eluting beads transarterial chemoembolization.
- MeSH
- Chemoembolization, Therapeutic * methods MeSH
- Carcinoma, Hepatocellular * therapy mortality MeSH
- Middle Aged MeSH
- Humans MeSH
- Microspheres MeSH
- Liver Neoplasms * therapy mortality MeSH
- Prospective Studies MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Starch * administration & dosage MeSH
- Treatment Outcome MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
- Observational Study MeSH
Problematika trombotických mikroangiopatií (TMA) představuje, nejen v porodnictví, velmi závažný patologický stav, který je spojen s tvorbou trombóz na úrovni kapilár i arteriol v důsledku poškození endotelu a aktivace komplementu. Je pro- vázen mikroangiopatickou hemolytickou anémií (MAHA), trombocytopenií a dysfunkcí různých orgánů. Relativně často je navíc spojen se sekundárními systémovými změnami srážlivosti. TMA zahrnují velmi nesourodou skupinu syndromů a sta- vů, kdy ke konečné diagnóze docházíme postupným vylučováním jednotlivých příčin („per exclusionem“). V porodnické praxi se nejčastěji setkáváme s tím, že těhotné/rodičky/nedělky se prezentují pod obrazem preeklampsie/HELLP syndromu (hemolysis, elevated liver enzymes, low platelets). Tento všem porodníkům jinak dobře známý stav zahrnuje obraz MAHA (dynamické snižování hladiny hemoglobinu, zvyšování hladiny bilirubinu, snížení haptoglobinu, přítomnost schistocytů v periferním nátěru krve), periportální ischémie jater (elevace transamináz) a trombocytopenie v důsledku vyšší agregace trombocytů v poškozené periferní mikrocirkulaci. HELLP syndrom se řadí mezi TMA také, měl by však spontánně odezní- vat přibližně do 48-72 hodin po porodu. Pakliže se tak nestane, je velmi důležité pomýšlet na jiné příčiny TMA, které často představují ještě vážnější ohrožení života než HELLP syndrom. Důkladná znalost diferenciální diagnostiky je proto velmi důležitá. Problematiku tedy musí dobře ovládat každý poskytovatel zdravotní péče těhotným ženám, a proto ji kolektiv autorů předkládá ve formě tohoto doporučeného postupu.
The issue of thrombotic microangiopathy (TMA) represents, not only in obstetrics, a serious pathological condition that is associated with the formation of thromboses at the level of capillaries and arterioles due to endothelial damage and complement activation. It is accompanied by microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and dysfunction of various organs. In addition, it is relatively often associated with secondary systemic changes in coagulation. TMAs comprise a very heterogeneous group of syndromes and conditions, where the final diagnosis is reached by sequential exclusion of the individual causes (‚per exclusionem‘). In obstetric practice, we most often encounter pregnant/parturient/ pregnant women presenting with a picture of pre-eclampsia/HELLP syndrome (haemolysis, elevated liver enzymes, low platelets). This condition, otherwise well known to all obstetricians, includes the picture of MAHA (dynamic decrease in hemoglobin, increase in bilirubin, decrease in haptoglobin, presence of schistocytes in the peripheral blood smear), periportal hepatic ischemia (elevation of transaminases) and thrombocytopenia due to increased platelet aggregation in the damaged peripheral microcirculation. HELLP syndrome is also classified as a TMA, but should resolve spontaneously within approximately 48-72 hours after delivery. If this does not happen, it is very important to think about other causes of TMA, which often represent an even more serious threat to life than HELLP syndrome. A thorough knowledge of differential diagnosis is therefore very important. The issue therefore needs to be well understood by every provider of health care to pregnant women, which is why the team of authors presents it in the form of this recommended practice.
... hepatologii 302 -- 4.3.1.2 Ikterus 303 -- Obsah 1. díl XXIII -- 4.3.1.3 Vrozené poruchy metabolismu bilirubinu ...
2., zcela přepracované vydání 2 svazky : ilustrace ; 31 cm
Publikace se zaměřuje na vnitřní lékařství. Určeno odborníkům v praxi i studentům lékařství.; Toto vydání zahrnuje 1. a 2. díl. Kniha je rozdělena do dvou dílů a rozřazena do 20 kapitol, v nichž se kromě základních oborů vnitřního lékařství (kardiologie, pneumologie, gastroenterologie, nefrologie, onkologie, hematologie, revmatologie, endokrinologie a metabolismu, geriatrie) dotýká i oborů blízkých, jako jsou neurologie, psychiatrie, medicína akutních stavů, otravy, bolest, infekční nemoci, výživa, imunologie, genetika. Je zde zařazena i kapitola o zdraví a nemoci a nově kapitola zabývající se paliativní medicínou a multimorbiditou ve vnitřním lékařství, které stále více ovlivňují naši klinickou praxi. K napsání druhého vydání publikace vedl především obrovský pokrok v diagnostice a léčbě v jednotlivých podoborech vnitřního lékařství, nahromadění nových poznatků, ale také zájem lékařů o původní učebnici. První i druhý díl publikace poskytují informace nejen integrálním oborům interní medicíny, ale i oborům, které s ní úzce souvisejí.
- MeSH
- Internal Medicine MeSH
- Conspectus
- Patologie. Klinická medicína
- Učební osnovy. Vyučovací předměty. Učebnice
- NML Fields
- vnitřní lékařství
- NML Publication type
- kolektivní monografie
- učebnice vysokých škol
Background: A 69-year-old man with multiple myeloma and left-sided heart failure presented to the hospital with a two-month fever. Method: A transjugular liver biopsy was performed due to the rapid progression of liver failure. The procedure was complicated by an intraperitoneal hemorrhage. The bleeding was managed expectantly. Result: Significantly elevated serum bilirubin levels occurred on the 13th day after liver biopsy. Increasing serum bilirubin levels were observed until the patient's death due to a biliovenous fistula at the liver biopsy site. Simultaneously, his slightly elevated liver enzymes returned to normal. The patient died 23 days after liver biopsy due to acute respiratory distress syndrome. Fistulous communication between the biliary tree and the hepatic venous system with subsequent bile leakage into the venous system (bilhemia) can lead to bile deposition in the lungs. Bile deposition in the lungs may potentiate and accelerate the development of diffuse alveolar damage with hyaline membranes. Conclusions: Lambda and kappa light chain deposition in the pulmonary alveoli in patients with multiple myeloma can mimic typical hyaline membranes.
- Publication type
- Journal Article MeSH
- Case Reports MeSH
Biliary drainage is then one of the necessary procedures to help patients suffering from icterus to reduce serum bilirubin levels and relieve symptoms. The aim of this study was identifying risk factors for survival in patients with cholangiocarcinoma (CCA) treated with percutaneous transhepatic biliary drainage (PTBD) and to develop a simple scoring system predicting survival from PTBD insertion. This single-centre retrospective study included 175 consecutive patients undergoing PTBD for extrahepatic CCA (perihilar and distal). Prognostic factors affecting survival of patients with CCA treated with PTBD were analysed. A multivariate analysis showed that mass forming tumor with mass larger than 5 cm and presence of metastasis at the time of PTBD served as a negative prognostic factor (p = 0.002), better survival was associated with lower preprocedural bilirubin and lower CRP (p = 0.003). Multivariate analysis identified two significant risk factors for 3-month mortality: mass-forming tumors and bilirubin levels exceeding 185 μmol/L. A simple scoring system was developed to predict 3-month mortality after PTBD in patients with advanced CCA, demonstrating 86.3% negative predictive value and 43.2% positive predictive value.
- MeSH
- Bilirubin blood MeSH
- Cholangiocarcinoma * mortality therapy pathology MeSH
- Adult MeSH
- Drainage * methods MeSH
- Middle Aged MeSH
- Humans MeSH
- Bile Duct Neoplasms * mortality therapy pathology MeSH
- Prognosis MeSH
- Retrospective Studies MeSH
- Risk Factors MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
INTRODUCTION: Pulsed electric field (PEF) has emerged as a promising energy source for catheter ablation of atrial fibrillation (AF). However, data regarding the in-vivo effect of PEF energy on erythrocytes during AF ablation procedures are scarce. This study aimed to quantify the impact of PEF energy on erythrocyte damage during AF ablation by assessing specific hemolytic biomarkers. METHODS: A total of 60 patients (age: 68 years, males: 72%, serum creatinine: 91 μmol/L) with AF underwent catheter ablation of AF using PEF energy delivered by a multipolar pentaspline Farawave catheter (Farapulse, Boston Scientific, Inc.). Ablation beyond pulmonary vein isolation was performed at the operator's discretion. Peripheral venous blood was sampled for assessing the plasma levels of free hemoglobin (fHb), direct (conjugated) bilirubin, lactate dehydrogenase (LDH), and creatinine before, immediately after the ablation, and on the next day. RESULTS: Following the PEF ablation with duration of [median (interquartile range)] 75 (58, 95) min, with 74 (52, 92) applications and PVI only in 27% of patients, fHb, LDH, and direct bilirubin significantly increased, from 40 (18, 65) to 493 (327, 848) mg/L, from 3.1 (2.6, 3.6) to 6.8 (5.0, 7.9) μkat/L, and from 12 (9, 17) to 28 (16, 44) μmol/L, respectively (all p < .0001). A strong linear correlation was found between the peak fHb and the number of PEF applications (R = 0.81, p < .001). The major hemolysis (defined as fHb >500 mg/L) was predicted by the number of PEF applications with the corresponding area under the receiver operating characteristic curve of 0.934. The optimum cut-off value of >74 PEF applications predicted the major hemolysis with 89% sensitivity and 87% specificity. CONCLUSION: Catheter ablation of AF using PEF energy delivered from a pentaspline catheter is associated with significant intravascular hemolysis. More than 74 PEF applications frequently resulted in major hemolysis. However, the critical amount of PEF energy that may cause kidney injury in susceptible patients remains to be investigated.
- MeSH
- Bilirubin blood MeSH
- Biomarkers * blood MeSH
- Time Factors MeSH
- Equipment Design MeSH
- Erythrocytes MeSH
- Atrial Fibrillation * surgery diagnosis physiopathology blood MeSH
- Hemoglobins metabolism MeSH
- Hemolysis * MeSH
- Catheter Ablation * adverse effects instrumentation MeSH
- Creatinine blood MeSH
- L-Lactate Dehydrogenase blood MeSH
- Middle Aged MeSH
- Humans MeSH
- Predictive Value of Tests MeSH
- Risk Factors MeSH
- Aged MeSH
- Cardiac Catheters MeSH
- Treatment Outcome MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
... 57.9 Cos pacientem dál 483 -- 58 Žloutenka (Václav Vobruba) 485 -- Úvod 485 -- 58.1 Metabolismus bilirubinu ...
1. elektronické vydání 1 online zdroj (712 stran)
Třetí vydání kolektivní monografie o dětské přednemocniční a urgentní péči. V obecných kapitolách jsou popsány elementární principy fungování přednemocniční péče a základní údaje z dětské fyziologie a farmakologie, speciální sekce shromažďuje údaje o konkrétních urgentních stavech interní i chirurgické povahy vyskytujících se v dětském věku.
- Keywords
- Lékařské obory, Anesteziologie, resuscitace, emergency, Chirurgie, ortopedie, traumatologie, Interna, Pediatrie,
- MeSH
- Ambulatory Care MeSH
- Pediatric Emergency Medicine MeSH
- Child MeSH
- Accidents MeSH
- Emergency Medical Services MeSH
- Check Tag
- Child MeSH
- NML Fields
- urgentní lékařství
- pediatrie
BACKGROUND & AIMS: α1-Antitrypsin (AAT) is a major protease inhibitor produced by hepatocytes. The most relevant AAT mutation giving rise to AAT deficiency (AATD), the 'Pi∗Z' variant, causes harmful AAT protein accumulation in the liver, shortage of AAT in the systemic circulation, and thereby predisposes to liver and lung injury. Although intravenous AAT augmentation constitutes an established treatment of AATD-associated lung disease, its impact on the liver is unknown. METHODS: Liver-related parameters were assessed in a multinational cohort of 760 adults with severe AATD (Pi∗ZZ genotype) and available liver phenotyping, of whom 344 received augmentation therapy and 416 did not. Liver fibrosis was evaluated noninvasively via the serum test AST-to-platelet ratio index and via transient elastography-based liver stiffness measurement. Histologic parameters were compared in 15 Pi∗ZZ adults with and 35 without augmentation. RESULTS: Compared with nonaugmented subjects, augmented Pi∗ZZ individuals displayed lower serum liver enzyme levels (AST 71% vs 75% upper limit of normal, P < .001; bilirubin 49% vs 58% upper limit of normal, P = .019) and lower surrogate markers of fibrosis (AST-to-platelet ratio index 0.34 vs 0.38, P < .001; liver stiffness measurement 6.5 vs 7.2 kPa, P = .005). Among biopsied participants, augmented individuals had less pronounced liver fibrosis and less inflammatory foci but no differences in AAT accumulation were noted. CONCLUSIONS: The first evaluation of AAT augmentation on the Pi∗ZZ-related liver disease indicates liver safety of a widely used treatment for AATD-associated lung disease. Prospective studies are needed to confirm the beneficial effects and to demonstrate the potential efficacy of exogenous AAT in patients with Pi∗ZZ-associated liver disease.
- MeSH
- alpha 1-Antitrypsin Deficiency * complications drug therapy MeSH
- Adult MeSH
- Phenotype MeSH
- Genotype MeSH
- Liver Cirrhosis etiology MeSH
- Humans MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
In the last decade, geopolitical instability across the globe has increased the risk of a large-scale radiological event, when radiation biomarkers would be needed for an effective triage of an irradiated population. Ionizing radiation elicits a complex response in the proteome, genome, and metabolome and hence can be leveraged as rapid and sensitive indicators of irradiation-induced damage. We analyzed the plasma of total-body irradiated (TBI) leukemia patients (n = 24) and nonhuman primates (NHPs; n = 10) before and 24 h after irradiation, and we performed a global metabolomic study aiming to provide plasma metabolites as candidate radiation biomarkers for biological dosimetry. Peripheral blood samples were collected according to the appropriate ethical approvals, and metabolites were extracted and analyzed by liquid chromatography mass spectrometry. We identified an array of metabolites significantly altered by irradiation, including bilirubin, cholesterol, and 18-hydroxycorticosterone, which were detected in leukemia patients and NHPs. Pathway analysis showed overlapping perturbations in steroidogenesis, porphyrin metabolism, and steroid hormone biosynthesis and metabolism. Additionally, we observed dysregulation in bile acid biosynthesis and tyrosine metabolism in the TBI patient cohort. This investigation is, to our best knowledge, among the first to provide valuable insights into a comparison between human and NHP irradiation models. The findings from this study could be leveraged for translational biological dosimetry.
- MeSH
- Biomarkers blood MeSH
- Whole-Body Irradiation * MeSH
- Adult MeSH
- Radiation, Ionizing MeSH
- Leukemia blood metabolism MeSH
- Middle Aged MeSH
- Humans MeSH
- Macaca mulatta MeSH
- Metabolome * MeSH
- Metabolomics methods MeSH
- Animals MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Female MeSH
- Animals MeSH
- Publication type
- Journal Article MeSH
Cieľ práce: Analyzovať klinické, laboratórne a epidemiologické údaje v súbore pacientov s akútnou hepatitídou E na Klinike infektológie a cestovnej medicíny v Košiciach (KICM). Materiál a metodiky: Retrospektívna analýza údajov z nemocničného informačného systému u pacientov s diagnózou akútnej hepatitídy E, ktorí boli vyšetrení alebo hospitalizovaní na KICM v rokoch 2015–2023. Štatistické vyhodnotenie sledovaných údajov so zameraním na epidemiológiu, priebeh, komplikácie. Výsledky: Súbor tvorilo 62 pacientov. Mužov bolo 58 %. Priemerný vek bol 56 rokov. Bolo hospitalizovaných 74 % pacientov s priemernou dĺžkou hospitalizácie 10 dní. Najčastejším klinickým prejavom bol ikterus (40 % pacientov). Šiesti pacienti mali vyšetrené HEV RNA v stolici, u všetkých sa potvrdil genotyp 3. U 5 % pacientov sa jednalo o importovanú nákazu (HEV RNA vyšetrené nemali), 95 % prípadov bolo autochtónnych. Kontakt s nakazeným hepatitídou E v anamnéze udávalo 26 % pacientov. V anamnéze malo preexistujúce ochorenie pečene 13 % pacientov, u týchto sa potvrdili vyššie hodnoty bilirubínu, GMT a amoniaku. U pacientov s poruchou imunity v anamnéze neboli zaznamenané štatisticky významné odchýlky. U jedného pacienta s preexistujúcim ochorením pečene bol priebeh ochorenia fulminantný s úmrtím. U štyroch pacientov, ktorí mali pri hepatitíde E aj neurologické symptómy boli zistené nižšie hodnoty bilirubínu. Závery: V súbore dominovali starší muži. U všetkých pacientov, u ktorých bolo realizované vyšetrenie HEV RNA, bol potvrdený genotyp 3. Potvrdili sa vyššie hodnoty bilirubínu, amoniaku a GMT u pacientov s preexistujúcim ochorením pečene. Pacienti s neurologickými komplikáciami mali nižšie hodnoty bilirubínu. U jedného pacienta s preexistujúcim ochorením pečene došlo k úmrtiu.
Aim: To analyse clinical, laboratory, and epidemiological data of a cohort of patients with acute hepatitis E treated at the Clinic of Infectology and Travel Medicine (CITM) in Košice. Material and methods: Retrospective analysis of hospital information system data on patients diagnosed with acute hepatitis E who were examined or hospitalized at CITM in 2015-2023. Statistical evaluation of the available data with a focus on epidemiology, course, and complications. Results: The cohort consisted of 62 patients. Fifty-eight percent were male. The mean age was 56 years. Seventy-four percent of patients were hospitalized, with a mean length of hospital stay of 10 days. The most common clinical manifestation was jaundice (in 40% of patients). Six patients had stool HEV RNA testing and all were confirmed to have genotype 3. In 5% of patients, the infection was classified as imported (they did not have HEV RNA tested), and 95% of cases were autochthonous. A history of contact with an HEV infected person was reported by 26% of patients. A history of preexisting liver disease was noted in 13% of patients who were confirmed with higher bilirubin, GMT, and ammonia levels. No statistically significant differences were found for patients with a history of immune deficiency. One patient with preexisting liver disease developed fulminant infection resulting in death. Four hepatitis E patients with neurological symptoms had lower bilirubin levels. Conclusions: The study cohort included predominantly older men. Genotype 3 was confirmed in all patients who underwent HEV RNA testing. Higher bilirubin, ammonia, and GMT levels were confirmed in patients with preexisting liver disease. Patients with neurological complications had lower bilirubin levels. One patient with preexisting liver disease died.
- MeSH
- Acute Disease MeSH
- Hepatitis E * diagnosis epidemiology transmission MeSH
- Humans MeSH
- Disease Transmission, Infectious MeSH
- Retrospective Studies MeSH
- Hepatitis E virus MeSH
- Check Tag
- Humans MeSH
- Geographicals
- Slovakia MeSH