The Czech Home Parenteral Nutrition Registry REDNUP: Comprehensive Analysis of Adult Patients' Data.

INTRODUCTION: Home parenteral nutrition (HPN) is the primary treatment modality for patients with chronic intestinal failure, one of the least common organ failures. This article provides a retrospective analysis of the data collected on HPN patients in the Czech Republic over the past 30 years. METHODS: National registry data were collected using a standardised online form based on the OASIS registry (Oley - A.S.P.E.N. Information System) across all centres providing HPN in the Czech Republic. Data collected prospectively from adult patients in the HPN program were analysed in the following categories: epidemiology, demographics, underlying syndrome, diagnosis, complications, and teduglutide therapy prevalence. RESULTS: The registry identified a total of 1,838 adult patient records, reflecting almost 1.5 million individual catheter days. The prevalence of HPN has risen considerably over the last few decades, currently reaching 5.5 per 100,000 population. The majority of patients have short bowel syndrome and GI obstruction, with cancer being the most prevalent underlying disease. Catheter-related bloodstream infections have been the most prevalent acute complication. However, the incidence in 2022 was only 0.15 per 1,000 catheter days. The study also observed an increase in the prevalence of patients on palliative HPN over the last decade. CONCLUSION: This study presents a thorough analysis of data from the Czech REgistr Domaci NUtricni Podpory (REDNUP) registry. It shows an increasing prevalence of HPN, namely, in the palliative patient group. The sharing of national data can improve understanding of this rare condition and facilitate the development of international guidelines.

Long-term home parenteral nutrition and profile of amino acids in serum and their loss in urine.

Amino acids are an essential part of parenteral nutrition. This study aimed to determine the serum profile of amino acids and their loss in urine in patients with long-term home parenteral nutrition (HPN) during 12 h of infusion in comparison with similar parameters in the remaining 12 h as well as in healthy participants. We enrolled forty-five patients with long-term HPN for 6-75 (median, 33) months. The indication for HPN was short bowel syndrome secondary to radical resection of the small intestine following complications of Crohn's disease. HPN was administered via two-chamber all-in-one bags prepared in a hospital pharmacy overnight for 12 h each day. The average dose of amino acids, carbohydrates and fats administered was 1·5, 3·4 and 0·68 g/kg per d, respectively, at an infusion rate of 0·11, 0·28 and 0·06 g/kg per h, respectively. The levels of essential amino acids in the serum of the patients were not significantly different from those in healthy individuals; however, of the non-essential amino acids, cystine and glutamine levels were lower and glycine and ornithine levels were higher in the patients (P < 0·05). Excretion of amino acids in the urine during 12 h of infusion at an infusion rate of 0·11 g/kg per h was 301 mg, while it was 104 mg during the remaining 12 h (P < 0·0001). Our patients on long-term HPN had a normal serum profile of essential amino acids. The total urinary excretion of amino acids during 12 h of infusion accounted for only 0·34 % (0·23-0·46) of the administered dose.

A cirrhotic patient in an internal medicine ward.

Liver cirrhosis represents a common condition with substantial mortality. Manifestation and progression of ascites, hepatic encephalopathy or gastrointestinal bleeding are among main reasons for hospital admission. Infections represent another specific area in cirrhotic patients. Timely and correct diagnosis and therapy of these conditions are the mainstay of optimal outcome. Manifestation of complications of liver cirrhosis significantly deteriorates prognosis of the patient. Ascites in portal hypertension develops as a result of sodium and consequently water retention. Therapy comprises of restriction of sodium intake, diuretic therapy with combination of spironolactone and furosemide, alternatively large-volume paracentesis. Hepatic encephalopathy comprises a spectrum of neuropsychiatric abnormalities from subtle changes to overt desorientation and asterixis to hepatic coma. Treatment includes correcting of predisposing conditions, administering of non-absorbable disaccharides or rifaximin. The most common cause of bleeding in a cirrhotic patient is oesophageal bleeding. Therapy is complex including hemodynamic stabilisation, antibiotic prophylaxis, vasoactive and endoscopic treatment. Infections are common causes of decompensation and occurrence of complications of advanced chronic liver disease. Their unfavourable outcome is a result of a complex immune disorder in cirrhotic patients. Specific type of infection in cirrhosis is spontaneous bacterial peritonitis, which has to be always excluded with diagnostic paracentesis. The mainstay of successful therapy of infections is timely and vigorous broad spectrum antibiotic therapy which can significantly improve otherwise unfavourable outcome of these patients.

Liver cirrhosis and pregnancy: a case report and review of literature.

Liver cirrhosis is a chronic liver disease in which the liver tissue and the vascular beds are remodeled leading to impaired hepatic function. Portal hypertension and subsequent esophageal varices are a frequent complication of liver cirrhosis and are a cause of mortality in patients with liver cirrhosis. Pregnancy in women with liver cirrhosis is uncommon, the incidence being about 1 in 5 950 pregnancies. Hepatocellular damage and the associated alteration in the metabolism of the sex hormones is thought to be responsible and leads to anovulation. In spite of all these factors, women with cirrhosis can and do become pregnant. Pregnancy is successful in most of the patients with chronic liver disease, but maternal and fetal complication rates are still high for decompensated liver cirrhosis. Portal hypertension associated with pregnancy is a high-risk situation as both pregnancy and portal hypertension share some of the hemodynamic changes. Risks of variceal bleeding and hepatic decompensation increases many fold during pregnancy. Despite the possible complications mentioned above, the maternal-fetal morbidity and mortality rates have been decreased by the current developments in hepatology, prevention of bleeding from varices with drugs and/or endoscopic variceal ligation, improvement in liver transplantation, and an increased experience in these issues. We present a case of a 31-year-old female patient with liver cirrhosis who successfully managed pregnancy and birth without complications after the insertion of transjugular intrahepatic portosystemic shunt (TIPS). Unfortunately, 2 years after delivery, the patient developed lymphoblastic lymphoma and, despite intensive therapy for this disease, the patient died at the age of 40. We did not find any link between liver cirrhosis and lymphoblastic lymphoma.

Small bowel adenocarcinoma diagnosed by video capsule endoscopy in a patient with celiac disease: a case report and review of literature.

Celiac disease is an immune mediated entheropathy triggered by gluten in genetically predisposed individuals. Patients with celiac disease are at a higher risk of gastrointestinal malignancies. Diagnosis at an advance stage is one of the factors of an unfavorable prognosis of these complications. Our patient is a woman who was diagnosed with celiac disease at 53 years of age. After two years on a gluten-free diet she developed sideropenic anemia. No source of bleeding was found on the esophagogastroduodenoscopy or colonoscopy. Video capsule endoscopy revealed exulcerated bleeding stenosis in the jejunum, in front of which the capsule lodged. There were no signs of infiltration on simultaneous CT enterography. The patient was operated on and the infiltration of the jejunum was resected. The specimen was evaluated by a histopathologist as a moderately differentiated adenocarcinoma. Due to the risk factors, the patient received adjuvant chemotherapy. The knowledge of the malignant complications of celiac disease, their risk factors and the possibilities of modern enteroscopic methods could help in the early diagnosis and improvement of the prognosis of these diseases. Due to a lack of data and an absence of guidelines, treatment of a small bowel adenocarcinoma is based on an expert agreement and guidelines for colon cancer. Surgical treatment is the only potentially curative option. For stage II with risk factors and stage III adjuvant chemotherapy should be considered.

[Splanchnic vein thrombosis]. / Splanchnická zilní trombóza.

Splanchnic vein thrombosis (SVT) represents an unusual manifestation of venous thromboembolism. The etiological factors for SVT can be divided into local and systemic, frequently found concurrently. SVT can be the first presenting symptom in myeloproliferative neoplasms. SVT puts the patients affected in jeopardy of developing the intestinal infarction, impairing the liver function and portal hypertension development with the risk of potentially life-threatening gastrointestinal bleeding. The current guidelines emphasise the role of anticoagulation in acute splanchnic thrombosis. Considering the potentially fatal complications it is necessary to tailor the anticoagulant treatment individually. The duration of anticoagulant therapy is strongly dependent upon the risk evaluation of thrombosis recurrence. The article deals with the causes, diagnostic methods and aspects influencing the therapeutic strategy.Key words: anticoagulation - liver cirrhosis - portal hypertension - prothrombotic state - splanchnic vein thrombosis.

Manganese in Whole Blood and Hair in Patients with Long-Term Home Parenteral Nutrition.

BACKGROUND: Manganese is an essential trace element and indispensable component of nutrition mixtures in long-term home parenteral nutrition (HPN) of patients. On the other hand, neurotoxic effects of excess manganese in the organism have been known for a long time. The objective of the present study was to determine manganese concentration in whole blood and hair of patients with long-term home parenteral nutrition. METHODS: We examined 16 patients (7 men and 9 women) aged from 28 to 68 years on long-term HPN lasting from 4 to 96 months. The short bowel syndrome was an indication for HPN. The daily dose of manganese ranged between 80 and 470 microg/day (1.2 to 8.5 pg/kg/day). RESULTS: In the investigated patients we detected approximately a doubled value of manganese concentration in whole blood in comparison to the control group (16.2 microg/L; 12.9-20.4 microg/L and 7.4 microg/L; 6.4-8.4 microg/L). In five patients with symptoms of cholestatic hepatopathy, Mn concentration in whole blood exceeded the value of 20.0 microg/L. Magnetic resonance of the brain in four of these patients detected a hyperintense T1-signal in the globus pallidus without any clinical symptoms similar to the Parkinson's syndrome. The content of manganese in the patients' hair was also significantly increased (p < 0.04). CONCLUSIONS: The results of our study corroborate the necessity of careful monitoring of the manganese concentration in the organism during HPN, especially in patients with liver disorders. Individualized HPN with greater accessibility of variable mixtures of trace elements would certainly be greatly beneficial, at least with regard to problems associated with manganese substitution.

Trace Element Status (Zinc, Copper, Selenium, Iron, Manganese) in Patients with Long-Term Home Parenteral Nutrition.

BACKGROUND: The objective of the present study was to determine concentrations of zinc (Zn), copper (Cu), iron (Fe), selenium (Se) in blood plasma and manganese (Mn) in the whole blood in patients with long-term home parenteral nutrition (HPN) in comparison to the control group. PATIENTS AND METHODS: We examined 68 patients (16 men and 52 women) aged from 28 to 68 years on a long-term HPN lasting from 4 to 96 months. The short bowel syndrome was an indication for HPN. The daily doses of Zn, Cu, Fe, Se and Mn in the last 3 months were determined. RESULTS: No significant differences in blood plasma were found for Zn, Cu and Fe in patients with HPN and in the control group (p > 0.05). The concentration of Mn in whole blood was significantly increased in HPN patients (p < 0.0001), while Se concentration in these patients was significantly decreased (p < 0.005). The concentration of Mn in the whole blood of 16 patients with cholestasis was significantly increased compared to the patients without cholestasis (p < 0.001). The Cu concentration was increased with no statistical significance. CONCLUSION: In long-term HPN, the status of trace elements in the patients has to be continually monitored and the daily substitution doses of these elements have to be flexibly adjusted. Dosing schedule needs to be adjusted especially in cases of cholestatic hepatopathy. A discussion about the optimal daily dose of Mn in patients on HPN is appropriate. For clinical practice, the availability of a substitution mixture of trace elements lacking Mn would be advantageous.

[Home nutrition care in the Czech Republic]. / Domácí nutricní péce v Ceské republice.

Home artificial nutrition includes application of enteral and parenteral nutrition in home environment, in cases of natural nutrition access failure. Home enteral nutrition uses various modality and ways, when persist the functional bowel. Chronic intestinal failure indicates dependence of home parenteral nutrition and is aplicated with central venous accesses. Home artificial nutrition improves patient´s quality of life and is more economical compared with hospital care.

[Adherence to the guidelines for treatment of mild biliary acute pancreatitis in the setting of tertiary center in the Czech Republic]. / Casování chirurgické a endoskopické terapie akutní biliární pankreatitidy v podmínkách terciární nemocnice v Ceské republice.

INTRODUCTION: Cholecystectomy (CCX) represents the crucial procedure in preventing relapses of biliary acute pancreatitis (BAP). Endoscopic papilosphincterotomy (EPST) represent an acceptable alternative in patients unsuitable for surgery. Current guidelines recommend patients with mild BAP to undergo CCX in 2 maximally 4 weeks following discharge from the hospital, ideally during the same hospital stay. Adherence to the guidelines differs significantly between particular countries and institutions. AIM: To evaluate adherence to the guidelines of BAP management in conditions of tertiary hospital in the Czech Republic. METHODS: Retrospective analysis of consecutive patients hospitalized in the Clinic of Gastroenterology, University Hospital Brno for acute pancreatitis in years 2007-2012. Cases with both sonographic findings of lithiasis/sludge and 3-fold AST/ALT elevation were considered of clearly biliary etiology. RESULTS: We identified 328 patients treated for acute pancreatitis. Clearly biliary etiology was identified in 116 cases (54 male, 62 female). From 114 analyzed patients with complete documentation 81 underwent CCX, 23 were not operated and 10 cases were patients with history of previous CCX. Total mortality of the group was 5.3%. Out of 81 patients who had CCX was 48 cases of mild BAP. CCX was done during the same hospital stay and/or within 4 weeks from dismissal in 20 patients, therefore, current guidelines were followed in 41.7% of our study group. Eighteen out of the remaining 28 patients underwent ERCP with EPST. Therefore, within 4 weeks from dismissal 75% of our patients underwent a procedure (CCX and/or EPST) with a potential to reduce the risk of BAP recurrence. CONCLUSION: When longer (4 weeks) interval between mild BAP and CCX is applied, the current guidelines are followed in 41.7% of patients treated at our institution, which is comparable with the literature data. As much as 75% of our patients underwent a procedure (CCX and/or EPST) with a potential to reduce the risk of BAP recurrence. However, only 12.5% of index CCX is not favorable outcome that needs improvement. Similar difficulties are being dealt with in the most countries in the World.

Indications for venous access in oncology - recommendations of national professional societies and current state in the Czech Republic.

BACKGROUND: The aim of the paper is to present the current recommendations and indications of venous access in oncology which reflect and recognize the opinions of national and international professional societies. It focuses exclusively on the indications of intravenous catheter placement for anticancer treatment, such as medium-term and long-term venous accesses. MATERIALS AND METHODS: The survey results obtained from a national questionnaire of 24 oncology centers identified the current situation in the Czech Republic. There were evaluated relevant data on the number of and the criteria for the introduction of venous accesses provided by physicians. Comparisons were made between current oncological practice and recommendations provided by evidence-based medicine. RESULTS: At each center surveyed in the Czech Republic, an average of 130 ports and 80 permanent implanted central catheters are introduced annually. The ports are increasingly indicated, with over a half of the centers surveyed introducing ports to more than 100 patients a year, with four centers introducing a total of 1,600 ports annually. In all centers, the decision for venous access is made by an oncologist. However, most procedures are performed by a doctor of another specialization, most often by a surgeon, a radiologist or an anesthesiologist. More than a half of the indications for venous access placement result from poor peripheral venous system or complications of parenteral therapy, not from comprehensive assessment prior to the initiation of the therapy. CONCLUSION: Based on our findings, we developed general indications and recommendations for venous access to cancer patients which represent the consensus of an interdisciplinary team of specialists, predominantly from the committee of professional societies - the Society for Ports and Permanent Catheters, the Working Group of Nutritional Care in Oncology of the Czech Oncological Society and the Society of Clinical Nutrition and Intensive Metabolic Care. The number of introduced venous access catheters remains insufficient to meet the needs in the Czech Republic, which necessitates increased awareness and possibilities for safe drug administration.

Microbiome and Metabolome Profiles Associated With Different Types of Short Bowel Syndrome: Implications for Treatment.

BACKGROUND: The gut microbiome and metabolome may significantly influence clinical outcomes in patients with short bowel syndrome (SBS). The study aimed to describe specific metagenomic/metabolomics profiles of different SBS types and to identify possible therapeutic targets. METHODS: Fecal microbiome (FM), volatile organic compounds (VOCs), and bile acid (BA) spectrum were analyzed in parenteral nutrition (PN)-dependent SBS I, SBS II, and PN-independent (non-PN) SBS patients. RESULTS: FM in SBS I, SBS II, and non-PN SBS shared characteristic features (depletion of beneficial anaerobes, high abundance of Lactobacilaceae and Enterobacteriaceae). SBS I patients were characterized by the abundance of oxygen-tolerant microrganisms and depletion of strict anaerobes. Non-PN SBS subjects showed markers of partial FM normalization. FM dysbiosis was translated into VOC and BA profiles characteristic for each SBS cohort. A typical signature of all SBS patients comprised high saturated aldehydes and medium-chain fatty acids and reduced short-chain fatty acid (SCFA) content. Particularly, SBS I and II exhibited low protein metabolism intermediate (indole, p-cresol) content despite the hypothetical presence of relevant metabolism pathways. Distinctive non-PN SBS marker was high phenol content. SBS patients' BA fecal spectrum was enriched by chenodeoxycholic and deoxycholic acids and depleted of lithocholic acid. CONCLUSIONS: Environmental conditions in SBS gut significantly affect FM composition and metabolic activity. The common feature of diverse SBS subjects is the altered VOC/BA profile and the lack of important products of microbial metabolism. Strategies oriented on the microbiome/metabolome reconstitution and targeted delivery of key compounds may represent a promising therapeutic strategy in SBS patients.

Efficiency of acidemia correction on intermittent versus continuous hemodialysis in acute methanol poisoning.

CONTEXT: Acidemia is a marker of prognosis in methanol poisoning, as well as compounding formate-induced cytotoxicity. Prompt correction of acidemia is a key treatment of methanol toxicity and methods to optimize this are poorly defined. OBJECTIVE: We studied the efficiency of acidemia correction by intermittent hemodialysis (IHD) and continuous renal replacement therapy (CRRT) in a mass outbreak of methanol poisoning. METHODS: The study was designed as observational cohort study. The mean time for an increase of 1 mmol/L HCO3-, 0.01 unit arterial blood pH, and the total time for correction of HCO3- were determined in IHD- and CRRT-treated patients. RESULTS: Data were obtained from 18 patients treated with IHD and 13 patients treated with CRRT. At baseline, CRRT group was more acidemic than IHD group (mean arterial pH 6.79 ± 0.10 versus 7.05 ± 0.10; p = 0.001). No association was found between the rate of acidemia correction and age, weight, serum methanol, lactate, formate, and glucose on admission. The time to HCO3- correction correlated with arterial blood pH (r= -0.511; p = 0.003) and creatinine (r = 0.415; p = 0.020). There was association between the time to HCO3- correction and dialysate/effluent and blood flow rates (r= -0.738; p < 0.001 and r= -0.602; p < 0.001, correspondingly). The mean time for HCO3- to increase by 1 mmol/L was 12 ± 2 min for IHD versus 34 ± 8 min for CRRT (p < 0.001), and the mean time for arterial blood pH to increase 0.01 was 7 ± 1 mins for IHD versus 11 ± 4 min for CRRT (p = 0.024). The mean increase in HCO3- was 5.67 ± 0.90 mmol/L/h for IHD versus 2.17 ± 0.74 mmol/L/h for CRRT (p < 0.001). CONCLUSIONS: Our study supports the superiority of IHD over CRRT in terms of the rate of acidemia correction.

Factors participating in the development and mortality of variceal bleeding in portal hypertension--possible effects of the kidney damage and malnutrition.

BACKGROUND/AIMS: Acute bleeding from esophageal varices due to portal hypertension is a frequent and severe complication of liver cirrhosis. The development of esophageal varices as well as their rupture depends on the level of portal pressure; however, a number of other factors may play a negative role in the rise of bleeding and its prognosis. METHODOLOGY: The report presented has compared a set of 46 patients admitted to hospital for acute bleeding with 48 cirrhotics hospitalized for other reasons. RESULTS: Bleeding patients had significantly higher level of nitrogenous substances (urea 14.1 mmol/L vs. 7.78 mmol/L, p < 0.01, creatinine 129.8 micromol/L vs. 106.04 micromol/L; p = 0.09). The disturbed renal function in itself probably does not increase the risk of bleeding, it may be rather considered a certain prognostic index of the portal hypertension degree. Bleeding patients had a lower level of total protein (60.7 g/L vs. 69.9 g/L; p < 0.01) with only slight insignificant decrease of albumin (26.64 g/L vs. 28.51 g/L). Cirrhotic patients are known to suffer from malnutrition and it is possible that malnutrition shares negatively and directly in the rise of bleeding. CONCLUSIONS: A prognostic index of mortality was a more conspicuous disorder of hepatic function (bilirubin 97.4 micromol/L vs. 57.4 micromol/L; p = 0.1; prolonged prothrombin time 1.99 INR vs. 1.56 INR; p = 0.01) and again the disorder of renal function (creatinine 166.7 micromol/L vs. 114.9 micromol/L; p = 0.09). Therefore, the maintenance of good renal function must be a component of complex therapy given to bleeding patients.

Variceal bleeding in portal hypertension: bacterial infection and comparison of efficacy of intravenous and per-oral application of antibiotics--a randomized trial.

OBJECTIVE: To determine the prevalence of bacterial infection in patients admitted to hospital with variceal bleeding in comparison with patients with liver cirrhosis admitted because of another reason. To compare the effect of orally administered antibiotics vs. intravenous antibiotics. METHODS: Bacteriological investigation of blood culture, urine, throat smear, perianal smear and ascites (polymorphonuclear count as well in ascites) was made in 46 cirrhotic patients admitted to hospital with variceal bleeding and 48 cirrhotic patients admitted because of another reason. Bleeders were treated endoscopically (sclerotization) and pharmacologically (terlipressin 1 mg every 4 h for 5 days), and were randomly allocated to the treatment with oral norfloxacin (25 patients) or intravenous ampicillin/sulbactam (21 patients). Early and late mortalities were evaluated. RESULTS: The incidence of infection was high in both groups (63.0% bleeders vs. 54.2% controls), but bleeding patients more often had positive blood culture (17.3% vs. 8.6%) and statistically significantly more positive findings in the throat smears (36.9% vs. 17.3%, P=0.04), which gives the evidence of increased pathological colonization in these patients. No difference in survival was seen in patients with per-oral or intravenous administration of antibiotics. CONCLUSION: Bacterial infection was demonstrated in high percentage in patients with liver cirrhosis admitted to hospital. The administration of antibiotics is indicated in these patients. Intravenous application is probably of the same efficacy as per-oral one.

Spontaneous bacterial peritonitis in the Czech Republic: prevalence and aetiology.

The aim of the study was to determine the prevalence and detailed data concerning the incidence of spontaneous bacterial peritonitis in the Czech Republic. Ninety-nine patients with liver cirrhosis and ascites were examined. Spontaneous bacterial peritonitis was diagnosed in 35 patients (35.4%). It was revealed more often in patients with alcoholic aetiology of cirrhosis whose anamnesis involved sub-febrile or febrile states and the deterioration of ascites. Elevated serum leucocyte counts and increased levels of C-reactive protein can contribute to the diagnosis. A low level of total protein and albumin in ascites predisposes to the increase of this infection. The reduction of the platelet count in a set of patients with spontaneous bacterial peritonitis indicates the influence of portal hypertension in the aetiology of the disease.

Different clinical utility of oropharyngeal bacterial screening prior to percutaneous endoscopic gastrostomy in oncological and neurological patients.

BACKGROUND: The aim of this study was to monitor oropharyngeal bacterial colonization in patients indicated for percutaneous endoscopic gastronomy (PEG). METHODS: Oropharyngeal swabs were obtained from patients prior to PEG placement. A development of peristomal infection was evaluated. The analysis of oropharyngeal and peristomal site pathogens was done. RESULTS: Consecutive 274 patients referred for PEG due to neurological disorder or cancer completed the study. Oropharyngeal colonization with pathogens was observed in 69% (190/274), dominantly in the neurologic subgroup of patients (P < 0.001). Peristomal infection occurred in 30 (10.9%) of patients and in 57% of them the correlation between oropharyngeal and peristomal agents was present. The presence of oropharyngeal pathogens was assessed as an important risk factor for the development of peristomal infection only in oncological patients (OR = 8.33, 95% CI: 1.66-41.76). Despite a high prevalence of pathogens in neurological patients, it did not influence the risk of peristomal infection with the exception for methicillin resistant Staphylococcus aureus (MRSA) carriers (OR 4.5, 95% CI: 1.08-18.76). CONCLUSION: During oropharyngeal microbial screening prior to the PEG insertion, the detection of pathogens may be a marker of the increased risk of peristomal infection in cancer patients only. In neurological patients the benefit of the screening is limited to the detection of MRSA carriers.