INTRODUCTION: Cystic fibrosis transmembrane conductance regulator modulator therapy improves nutritional status and quality of life. Clinical trials have shown pancreatic insufficiency conversion, mostly in pediatric patients treated with ivacaftor. Studies with elexacaftor/tezacaftor/ivacaftor (ETI) in older patients have not suggested restoration of exocrine pancreas function, but quality data in adults are lacking. Our aim was to show the effect of ETI in adults with cystic fibrosis (CF) on nutritional status and digestive function. We hypothesized improvement of nutritional parameters and gastrointestinal symptoms, reduction of pancreatic enzyme replacement therapy, but uncertain improvement in exocrine pancreatic function. METHODS: We prospectively enrolled adults with CF treated with ETI from August 2021 to June 2022. We measured anthropometric parameters, laboratory nutritional markers, change of fecal elastase, pancreatic enzymes replacement therapy needs, and gastrointestinal symptoms. RESULTS: In the cohort of 29 patients (mean age 29.1 years), 82.8% suffered exocrine pancreatic insufficiency. After ETI, mean BMI increased by 1.20 kg/m2 (p < 0.001), mean body weight by 3.51 kg (p < 0.001), albumin by 2.81 g/L, and prealbumin by 0.06 (both p < 0.001). Only 1 patient, initially pancreatic insufficient (4.5%, p < 0.001), developed pancreatic sufficiency, indicated by increased fecal elastase from 45 μg/g to 442.1 μg/g. Mean change in lipase substitution decreased by 1,969 units/kg/day (p < 0.001) and stools frequency by 1.18 per day (p < 0.001). CONCLUSION: Our data suggest increased nutritional parameters, lower pancreatic substitution requirements, and improved defecation in adult CF patients on ETI. Improvement in exocrine pancreatic function might be mutation-specific and needs further study.
- MeSH
- aminofenoly terapeutické užití MeSH
- benzodioxoly terapeutické užití MeSH
- chinoliny MeSH
- chinolony terapeutické užití MeSH
- cystická fibróza * farmakoterapie komplikace MeSH
- dospělí MeSH
- exokrinní pankreatická insuficience * farmakoterapie etiologie komplikace MeSH
- fixní kombinace léků * MeSH
- gastrointestinální nemoci farmakoterapie MeSH
- indoly * terapeutické užití MeSH
- lidé MeSH
- mladý dospělý MeSH
- nutriční stav * MeSH
- pankreatická elastasa metabolismus MeSH
- prospektivní studie MeSH
- pyrazoly terapeutické užití MeSH
- pyridiny terapeutické užití MeSH
- pyrrolidiny terapeutické užití MeSH
- výsledek terapie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
BACKGROUND: Inflammatory bowel disease (IBD) care and education might differ around Europe. Therefore, we conducted this European Variation In IBD PracticE suRvey (VIPER) to investigate potential differences between countries. METHODS: This trainee-initiated survey, run through SurveyMonkey®, consisted of 47 questions inquiring basic demographics, IBD training, and clinical care. Results were compared according to gross domestic product (GDP) per capita, for which countries were divided into 2 groups (low/high income, according to the World Bank). RESULTS: The online survey was completed by 1,285 participants from 40 European countries, with a majority of specialists (65.3%) working in academic institutions (50.4%). Significant differences in IBD-specific training (55.9% vs. 38.4%), as well as availability of IBD units (58.4% vs. 39.7%) and multidisciplinary meetings (73.2% vs. 40.1%), were observed between respondees from high and low GDP countries (p < 0.0001). In high GDP countries, IBD nurses are more common (85.9% vs. 36.0%), also mirrored by more nurse-led IBD clinics (40.6% vs. 13.7%; p < 0.0001). IBD dieticians (33.4% vs. 16.5%) and psychologists (16.8% vs. 7.5%) are mainly present in high GDP countries (p < 0.0001). In the current COVID era, telemedicine is available in 73.2% versus 54.1% of the high/low GDP countries, respectively (p < 0.0001). Treat-to-target approaches are implemented everywhere (85.0%), though access to biologicals and small molecules differs significantly. CONCLUSION: Much variability in IBD practice exists across Europe, with marked differences between high and low GDP countries. Further work is required to help address some of these inequalities, aiming to improve and standardize IBD care and training across Europe.
- MeSH
- biologické přípravky * MeSH
- COVID-19 * MeSH
- idiopatické střevní záněty * epidemiologie terapie MeSH
- lidé MeSH
- průzkumy a dotazníky MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Evropa MeSH
We present a case of a fish bone impacted in the papilla of Vater resulting in dyspepsia and mild elevation in liver function tests, which was subsequently treated endoscopically. Fish bones are one of the most commonly encountered swallowed foreign bodies. However, involvement of the biliary tract, such as the one described by us, represents an extremely rare complication of fish bone ingestion. The diagnosis of a foreign body in the biliary tract can be difficult, and early endoscopic or surgical extraction may be required to avoid complications such as biliary stone formation, obstructive jaundice, cholangitis or cholecystitis, and/or biliary sepsis. Prompt endoscopic treatment can avoid severe biliary complications or surgical therapy.
- MeSH
- cholangiopankreatografie endoskopická retrográdní MeSH
- cholangitida * etiologie MeSH
- jídla MeSH
- lidé MeSH
- žlučové kameny * MeSH
- žlučové ústrojí * MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- kazuistiky MeSH
Metabolic syndrome and its components such as obesity, hypertriglyceridemia, type-2 diabetes mellitus (DM-T2), and arterial hypertension are unequivocally serious problems for every society. This is especially true in economically developed countries where the imbalance in lifestyle between caloric intake and caloric output still gets greater and greater. This fact is not only a concern for the adult population but for children as well. However, metabolic syndrome does not only affect society and health in regards to cardiovascular diseases, it significantly concerns gastroenterology where it is classified as nonalcoholic fatty pancreas disease (NAFPD). The data gained from several trials show that the prevalence of NAFDP is 33% (95% CI 24-41%). When it comes to the diagnostic procedures concerning the presence of pancreatic fat, a whole spectrum of suitable methods are recommended. Probably, the most exact method is the use of magnetic resonance imaging. However, for common clinical practice, the abdominal sonographic examination based on the comparison of the pancreatic parenchymatous echogenity versus renal or hepatic echogenity is used. The clinical consequences of pancreatic steatosis and steatopancreatitis are significant. These diseases are connected with DM-T2 and insulin resistance. In recent years, changes of exocrine pancreatic function, particularly its decrease, have also been described. It is known that there is a close correlation between NAFPD and nonalcoholic hepatic steatosis and also with the increased thickness of aortic intima-media. There is also an important relationship between NAFPD and pancreatic carcinoma. Pancreatic steatosis, and especially its NAFPD form, is a serious state which can be treatable by the possible effective management of metabolic syndrome parameters, including obesity.
- MeSH
- inzulinová rezistence MeSH
- lidé MeSH
- mikrobiota MeSH
- nealkoholová steatóza jater komplikace diagnóza mikrobiologie patologie MeSH
- nemoci slinivky břišní diagnóza epidemiologie mikrobiologie patologie MeSH
- obezita komplikace MeSH
- rizikové faktory MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
- MeSH
- diabetes mellitus 2. typu * MeSH
- lidé MeSH
- nádory tračníku * MeSH
- rizikové faktory MeSH
- studie případů a kontrol MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- dopisy MeSH
- komentáře MeSH
BACKGROUND: Diagnosis of pancreatic cancer (PC) in early stages is still challenging for gastroenterologists. The early detection of cancer is one of the utmost importance for the successful therapy of this malignancy. An accurate differential diagnosis of focal pancreatic lesions plays also an important role, whether it is differential diagnosis of chronic pancreatitis from PC or autoimmune pancreatitis (AIP) from PC. Raised serum immunoglobulin G4 (IgG4) levels to twice the normal value are considered one of significant diagnostic features of type 1 AIP. However, IgG4 can be increased also in patients with PC, but levels usually do not exceed twice the normal value. METHODS: In years 2012-2017, IgG4 serum levels were examined in 115 patients with histologically confirmed PC. Patients with PC and elevated IgG4 level (above 135 mg/dL) had tested their histological resection specimens or bioptic specimens from pancreatic lesion, with targeted detection of the presence of IgG4 and plasmocytes in the pancreatic tissue and changes characteristic for type 1 AIP. RESULTS: A plasmatic IgG4 level in 115 patients with diagnosed PC was higher than 135 mg/dL in 14 patients (12.2%). Out of them, 2 patients (1.7%) revealed a serum IgG4 level higher than double the normal value, that is, higher than 270.0 mg/dL (suggestive of AIP). One patient met histological criteria for diagnosis of AIP in the simultaneous presence of PC. CONCLUSION: Diagnosis of early cancer stages, particularly differentiating AIP from PC can be sometimes problematic. IgG4 levels can be slightly elevated also in case of PC. A targeted biopsy of the pancreas is the method of choice in cases suspected from a focal form of AIP and we recommend to prefer it over other modalities, such as, for example, response to steroid therapy.
- MeSH
- autoimunitní nemoci krev diagnóza diagnostické zobrazování patologie MeSH
- chronická nemoc MeSH
- chronická pankreatitida krev diagnóza diagnostické zobrazování patologie MeSH
- diferenciální diagnóza MeSH
- endosonografie MeSH
- imunoglobulin G krev MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory slinivky břišní krev diagnóza diagnostické zobrazování patologie MeSH
- senioři MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
BACKGROUND: In adults, infliximab (IFX) levels correlate with disease activity, and antibodies to IFX (ATIs) predict treatment failure. We aimed to determine the association of IFX levels and ATIs with disease activity in a paediatric population. We prospectively collected blood, stool, and clinical data from 65 patients (age 10.5-15.1 years) with Crohn's disease (CD) before IFX administration, and measured IFX trough levels, ATIs, and faecal calprotectin levels (CPT). Samples were collected during maintenance therapy. We used multivariate analysis to identify the predictors of IFX levels. SUMMARY: Lower levels of IFX were associated with ATIs positivity (OR 0.027, 95% CI 0.009-0.077). Higher C-reactive protein (CRP) level, erythrocyte sedimentation rate, and CPT levels were found in patients with lower IFX levels. The optimal combination of sensitivity (0.5) and specificity (0.74) for disease activity was calculated for IFX levels ≥1.1 µg/mL using CRP level <5 mg/L as a marker of laboratory remission. In a model that used CPT ≤100 µg/g as the definition of remission, the optimal IFX trough level was 3.5 µg/mL. No independent association between remission and ATIs was found in our study population. However, we found an independentz association between IFX levels and serum albumin levels (OR 1.364, 95% CI 1.169-1.593), p < 0.001. Key Messages: The paediatric population was similar to adult populations in terms of the association between IFX and ATIs as well as between IFX and disease activity.
- MeSH
- biologické markery metabolismus MeSH
- C-reaktivní protein metabolismus MeSH
- Crohnova nemoc krev farmakoterapie MeSH
- dítě MeSH
- feces chemie MeSH
- indukce remise MeSH
- infliximab aplikace a dávkování terapeutické užití MeSH
- krevní sedimentace MeSH
- leukocytární L1-antigenní komplex metabolismus MeSH
- lidé MeSH
- mladiství MeSH
- plocha pod křivkou MeSH
- ROC křivka MeSH
- terapie neúspěšná MeSH
- zánět patologie MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- pozorovací studie MeSH
BACKGROUND: Most patients with Crohn's disease (CD) require one or more operations during their lifetime. Repeated resections and surgical complications may result in short gut in a subset of patients, typically those with extensive small bowel disease or a penetrating CD phenotype. The effects of short bowel syndrome (SBS) can range in seriousness from mild to life-threatening advanced intestinal failure. Worldwide, CD is the second leading indication for intestinal transplantation (ITx) in SBS, but the overall incidence of ITx is quite low. Key Messages: Currently, total parenteral nutrition (TPN) is the preferred treatment option for patients with SBS because of its superior survival outcome. However, TPN can fail from loss of venous access due to catheter-associated thromboses, recurrent catheter-related blood stream infections, or intestinal-failure-associated liver dysfunction. Three types of transplantations are available for CD patients - small bowel alone, liver plus small bowel and multivisceral, which includes other intra-abdominal organs. An abdominal wall transplant is required in case of abdominal wall defects or lack of free intra-abdominal space. The current 5-year survival rate of 54% following ITx of the isolated small bowel appears worse than that associated with TPN. However, outcomes are substantially improving because of surgical and technical advances and progress in medical therapy. On the other hand, ITx carries the risk of both complications (e.g., rejection, infections, and post transplant lymphoproliferative disorders) and adverse events associated with immunosuppression. CD recurrence has been reported in a few patients, but this primarily histologic recurrence might not be of great clinical importance. CONCLUSIONS: ITx has become a well-established treatment for those who fail on TPN and who have life-threatening complications. Fortunately, it concerns only a small proportion of CD patients, but it does offer reasonable survival and quality of life. Primary management of patients with small bowel failure should be provided by a center experienced in medical intestinal rehabilitation, nutrition, and transplantation of other solid organs.
- MeSH
- Crohnova nemoc komplikace chirurgie MeSH
- dospělí MeSH
- imunosupresivní léčba škodlivé účinky MeSH
- kvalita života MeSH
- lidé MeSH
- míra přežití MeSH
- mladý dospělý MeSH
- nemoci střev etiologie chirurgie MeSH
- parenterální výživa úplná MeSH
- syndrom krátkého střeva etiologie chirurgie MeSH
- tenké střevo transplantace MeSH
- výsledek terapie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mladý dospělý MeSH
- Publikační typ
- časopisecké články MeSH
In humans, the gut microbiota forms a complex ecosystem consisting of a vast number of bacteria, Archaea, fungi and viruses. It represents a major stimulus to the development of the immune system and many other physiological functions, so that it may shape the individual's susceptibility to infectious and immune-mediated diseases. The emergence of new '-omics' methods recently revolutionized the way we study the host-microbe interactions, but they also raised new questions and issues. In this review, we discuss the impact of these new data on the current and future therapies for chronic inflammatory diseases. We also outline the major conceptual, technical and interpretational issues that recently led to some misleading conclusions and discuss in brief the current research directions in the field.
- MeSH
- idiopatické střevní záněty mikrobiologie MeSH
- lidé MeSH
- nemoci imunitního systému mikrobiologie MeSH
- střevní mikroflóra * MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
BACKGROUND: Population-based studies represent the whole spectrum of patient population and should represent the mainstay when evaluating patients' prognosis. A high number of CD patients need surgical intervention during the disease course. The disease course of inflammatory bowel diseases, including ulcerative colitis (UC) and Crohn's disease (CD), is quite varied and still quite unpredictable. Key Messages: According to earlier studies, up to 60% of patients undergo at least one operation after 10 years of CD duration. Newer cohorts report lower cumulative probability of surgery of approximately 40% after 10 years. The colectomy rate in UC is approximately 10% after 10 years from diagnosis with a geographic difference. Similarly to CD, the colectomy rate seems to decrease over time. There is some evidence that the increasing use of immunosuppressive and/or biological therapy might have been responsible for this favourable trend. However, other factors may have an impact on decreasing surgical trend over time. The relative risk (RR) of colorectal cancer (CRC) in UC is approximately doubled compared to background population. However, the absolute risk in general is relatively low between 1.1 and 5.3% after 20 years of disease duration. Furthermore, a decreasing trend in the incidence of CRC has been reported in recent studies. Importantly, several factors such as disease extent, activity, age at UC onset, and so on may increase/modify an individual risk. Similar to UC, CD patients have approximately 2 times higher RR of cancer compared to background population. The risk is higher for colon than for rectum cancer and present only in CD patients with colonic involvement. CONCLUSIONS: The surgery rate in CD has decreased over the time period. The evidence on colectomy rate in UC is less conclusive. The RR of CRC in UC and CD is approximately doubled compared to that of the background population, but it seems to be decreasing in more recent cohorts.
- MeSH
- biologická terapie statistika a číselné údaje MeSH
- Crohnova nemoc * komplikace terapie MeSH
- dospělí MeSH
- epidemiologické studie * MeSH
- imunosupresiva terapeutické užití MeSH
- kolektomie statistika a číselné údaje MeSH
- kolorektální nádory * etiologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- riziko MeSH
- senioři MeSH
- ulcerózní kolitida * komplikace terapie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH