Intravenous supplementation type and volume are associated with 1-year outcome and major complications in patients with chronic intestinal failure.

BACKGROUND AND AIM: No marker to categorise the severity of chronic intestinal failure (CIF) has been developed. A 1-year international survey was carried out to investigate whether the European Society for Clinical Nutrition and Metabolism clinical classification of CIF, based on the type and volume of the intravenous supplementation (IVS), could be an indicator of CIF severity. METHODS: At baseline, participating home parenteral nutrition (HPN) centres enrolled all adults with ongoing CIF due to non-malignant disease; demographic data, body mass index, CIF mechanism, underlying disease, HPN duration and IVS category were recorded for each patient. The type of IVS was classified as fluid and electrolyte alone (FE) or parenteral nutrition admixture (PN). The mean daily IVS volume, calculated on a weekly basis, was categorised as <1, 1-2, 2-3 and >3 L/day. The severity of CIF was determined by patient outcome (still on HPN, weaned from HPN, deceased) and the occurrence of major HPN/CIF-related complications: intestinal failure-associated liver disease (IFALD), catheter-related venous thrombosis and catheter-related bloodstream infection (CRBSI). RESULTS: Fifty-one HPN centres included 2194 patients. The analysis showed that both IVS type and volume were independently associated with the odds of weaning from HPN (significantly higher for PN <1 L/day than for FE and all PN >1 L/day), patients' death (lower for FE, p=0.079), presence of IFALD cholestasis/liver failure and occurrence of CRBSI (significantly higher for PN 2-3 and PN >3 L/day). CONCLUSIONS: The type and volume of IVS required by patients with CIF could be indicators to categorise the severity of CIF in both clinical practice and research protocols.

[Clinical nutrition therapy in patients with short bowel syndrome in line with principles of personalized medicine]. / Klinikai táplálásterápia rövidbél-szindrómában a személyre szabott orvoslás elvei szerint.

Home parenteral nutrition administered in selected care centres has been financed in Hungary since January, 2013. The authors discuss diagnostic issues, treatment and nutrition therapy of short bowel syndrome patients in line with the principles of personalised medicine. The most severe form of short bowel syndrome occurs in patients having jejunostomy, whose treatment is discussed separately. The authors give a detailed overview of home parenteral feeding, its possible complications, outcomes and adaptation of the remaining bowel. They describe how their own care centre operates where they administer home parenteral nutrition to 12 patients with short bowel syndrome (5 females and 7 males aged 51.25±14.4 years). The body mass index was 19.07±5.08 kg/m2 and 20.87±3.3 kg/m2, skeletal muscle mass was 25.7±6.3 kg and 26.45±5.38 kg, and body fat mass was 14.25±8.55 kg and 11.77±2.71 kg at the start of home parenteral nutrition and presently, respectively. The underlying conditions of short bowel syndrome were tumours in 4 patients, bowel ischaemia in four patients, surgical complications in three patients, Crohn's disease in one patient, and Crohn's disease plus tumour in one patient.

SpyGlass cholangioscopy ­ first experiences / SpyGlass-kolangioszkópia - elso tapasztalatok.

Összefoglaló. Bevezetés: A SpyGlass-kolangioszkópia újonnan kifejlesztett endoszkópos technika, mely az epeutak közvetlen vizualizációját teszi lehetové. A kolangioszkóp egy 10,8 Fr átméroju, a duodenoszkóp munkacsatornáján keresztül az epeútba vezetheto, a különbözo endoszkópos tartozékok számára saját munkacsatornával bíró endoszkóp. Fo indikációs területe a bizonytalan dignitású epeúti szukületek diagnosztikája, valamint a konvencionális endoszkópos technikával nem megoldható epeúti kövesség terápiája. Célkituzés: Célunk a SpyGlass berendezés hasznosságának és hatásosságának megítélése. Módszer: A Jahn Ferenc Dél-pesti Kórház Gasztroenterológia Osztályán 2018. január 1. és 2020. december 31. között a Spyglass DS I, míg 2021 januárjában a SpyGlass DS II rendszert használtuk. 14 diagnosztikus és 15 terápiás beavatkozást végeztünk. A diagnosztikus beavatkozások beválogatási kritériuma azon bizonytalan dignitású epeúti szukületeket fogalta magában, melyek esetén a végso diagnózis korábban elvégzett endoszkópos retrográd kolangiopankreatográfiával vagy endoszkópos, ultrahangvezérelt szövettani mintavétellel nem volt megállapítható. A terápiás beavatkozás indikációja a konvencionális endoszkópos technikával nem megoldható epeúti kövesség volt. Eredmények: A makroszkópos megítélés és a végso diagnózis egyezésének tekintetében a kolangioszkópia pontossága 85% volt. A szövettani diagnózis pontossága kolangioszkópvezérelt biopsziák esetén 62,5%. A makroszkópos diagnózis szenzitivitása 100%, specificitása 71% volt, a szövettani minták szenzitivitása 60%, specificitása 100% volt. Komplett clearence-t 4 esetben értünk el, ez összesen 57,14% sikerességi rátának felel meg. Következtetés: A SpyGlass-vizsgálat lehetové teszi a bizonytalan eredetu epeúti szukületek pontos értékelését, valamint megkönnyíti a szövettani mintavételezést. A diagnosztikus specificitás és szenzitivitás tekintetében a nemzetközi irodalmi adatok eléréséhez további fejlodés és az esetszámok növelése szükséges. A SpyGlass-vezérelt elektrohidraulikus lithotripsia a konvencionális endoszkópos technikával nem megoldható nehéz epeúti kövek kezelési alternatívája. Orv Hetil. 2022; 163(4): 150-156 Summary. INTRODUCTION: SpyGlass cholangioscopy is a recently developed endoscopic technique to the direct visualization of the biliary tract. The SpyGlass cholangioscop is a 10,8 Fr diameter endoscop which can be guided to the biliary tract through the work channel of the doudenoscope and has its own work channel for the different endoscopic accessories. The main indications of the examination are the diagnosis of the uncertain dignity biliary stenosis and the therapy of the biliary stones which failed conventional therapy. OBJECTIVE: Our aim was to assess the utility and efficacy of the SpyGlass system. METHOD: In Jahn Ferenc South Pest Hospital Gastroenterology Department, we used the SpyGlass™ DS I system between 2018 and 2020 and from 2021 the SpyGlass™ DS II. 14 diagnostic and 15 therapeutic Spyglass procedures have been performed. Inclusion criterion of diagnostic procedures was indeterminate bile duct stenosis where the final diagnosis could not be confirmed by endoscopic retrograde cholangiopancreatography or endoscopic ultrasound-guided biopsy. Inclusion criteria of the therapeutic examinations were difficult bile duct stones which failed conventional therapy. RESULTS: Concerning the correspondence of the macroscopic image and the final diagnosis, the accuracy of the cholangioscope was 85%. The accuracy of the histological diagnosis in the case of cholangioscopy-guided biopsies was 62.5%. The sensitivity of the macroscopic diagnosis was 100%, specificity was 71%, while the sensitivity of histologic samples was 60% and the specificity was 100%. Complete clearence was performed four times in the case of therapeutic procedures, which refers to 57.14% success rate. CONCLUSION: The use of SpyGlass enhances the precise evaluation of indeterminate bile duct lesions and tissue acquisition is easier to perform. However, to reach the international standards of diagnostic sensitivity and specificity, further improvement and examinations are necessary. Spyglass-guided electrohydraulic lithotripsy is an alternative for difficult stones which failed conventional therapy. Orv Hetil. 2022; 163(4): 150-156.

Autoimmune pancreatitis in Hungary: a multicenter nationwide study.

BACKGROUND: To date, most cases of autoimmune pancreatitis (AIP) have been reported from Japan. The aim of the present study was to assess the clinical features and management of AIP cases in Hungary. METHODS: The demographics, clinical presentation, laboratory and imaging findings, extrapancreatic involvement, treatment response and recurrence were evaluated in the first 17 patients diagnosed with AIP in Hungary. RESULTS: The mean age at presentation was 42.7 years (range: 16-74); 47% of the patients were women. New-onset mild abdominal pain (76%), weight loss (41%) and jaundice (41%) were the most common symptoms, with inflammatory bowel disease being the most frequent (36%) extrapancreatic manifestation. Diffuse pancreatic swelling was seen in 7 patients (41%) and a focal pancreatic mass in 8 (47%). Endoscopic retrograde cholangiopancreatography revealed pancreatic duct strictures in all study patients. The serum IgG4 level at presentation was elevated in 62% of the 8 patients in whom it was measured. All the percutaneous core biopsies (5 patients) and surgical specimens (2 patients), and 2 of the 4 biopsies of the papilla of Vater revealed the typical characteristic findings of AIP: a diffuse lymphoplasmacytic infiltration, marked interstitial fibrosis and obliterative phlebitis. Immunostaining indicated IgG4-positive plasma cells in 62% of the 8 patients in whom it was performed. Granulocytic epithelial lesions (GEL) were present in 3 patients. The patients without GELs were older (mean age 59 years), while those with GEL were younger (mean age 34 years), and 2 of 3 were female and had ulcerative colitis. A complete response to steroid treatment was achieved in all 15 patients. Because of the suspicion of a pancreatic tumor, 2 patients with focal AIP underwent partial pancreatectomy. One patient relapsed, but responded to azathioprine. CONCLUSIONS: This first Hungarian series has confirmed several previously reported findings on AIP. AIP with GEL was relatively frequent among our patients: these patients tended to be younger than in earlier studies and displayed a female preponderance with a high coincidence of ulcerative colitis. Performance of a percutaneous biopsy is strongly recommended. The response to immunosuppressive therapy was excellent. and IAP.

Changes in the management of variceal gastrointestinal haemorrhage in our department / [A varixeredetu gastrointestinalis vérzés ellátásának változása osztályunkon]

Introduction: Variceal bleeding is a life-threatening complication of portal hypertension with a six-week mortality rate of approximately 20%. Aim: To analyse whether the changes introduced in the treatment of variceal gastrointestinal haemorrhage in our department affected the mortality rate of these patients. Method: A retrospective method was used to compare the data of patients treated with variceal bleeding in 2014 and 2015. In 2015, two changes were made in the treatment of patients with variceal bleeding: all patients were treated in the subintensive care unit and terlipressin was administered to all patients susceptible to variceal haemorrhage. Bleeding was mitigated by means of sclerotherapy and/or ligation. Significance was calculated using Student's t-test, then we performed logistic regression to find out what treatment factors affect mortality rate. Patients: 2014 vs. 2015 figures ­ number of patients: 24 vs. 30, average age: 59.8 vs. 57.6 years, male (%): 70.8 vs. 66.7. There were no significant differences between the Child­Pugh stages of the two years, p = 0.53. For the analysis we also grouped patients based on whether irrespective of the year of treatment they were administered terlipressin or not. Number of patients: 22 vs. 32, average age: 60.4 vs. 57.4, male (%): 63.6 vs. 70.6. Results: Mortality in 2015 and 2014: 23% and 33%, respectively. Mortality of patients treated with terlipressin: 18.2 vs. 34.4, p = 0.09. Child­Pugh stages had the strongest influence on mortality (stage A vs. B p = 0.05, stage A vs. C p = 0.02). Terlipressin administered in Child­Pugh stage C reduced mortality at a rate bordering on significance (p = 0.055). Conclusion: Despite the comparatively small number of cases, the changes introduced in our department in 2015 in the treatment of variceal gastrointestinal haemorrhages resulted in a significant reduction of hospital mortality rates. Orv Hetil. 2020; 161(15): 583­587.

Home parenteral nutrition provision modalities for chronic intestinal failure in adult patients: An international survey.

BACKGROUND & AIMS: The safety and effectiveness of a home parenteral nutrition (HPN) program depends both on the expertise and the management approach of the HPN center. We aimed to evaluate both the approaches of different international HPN-centers in their provision of HPN and the types of intravenous supplementation (IVS)-admixtures prescribed to patients with chronic intestinal failure (CIF). METHODS: In March 2015, 65 centers from 22 countries enrolled 3239 patients (benign disease 90.1%, malignant disease 9.9%), recording the patient, CIF and HPN characteristics in a structured database. The HPN-provider was categorized as health care system local pharmacy (LP) or independent home care company (HCC). The IVS-admixture was categorized as fluids and electrolytes alone (FE) or parenteral nutrition, either commercially premixed (PA) or customized to the individual patient (CA), alone or plus extra FE (PAFE or CAFE). Doctors of HPN centers were responsible for the IVS prescriptions. RESULTS: HCC (66%) was the most common HPN provider, with no difference noted between benign-CIF and malignant-CIF. LP was the main modality in 11 countries; HCC prevailed in 4 European countries: Israel, USA, South America and Oceania (p < 0.001). IVS-admixture comprised: FE 10%, PA 17%, PAFE 17%, CA 38%, CAFE 18%. PA and PAFE prevailed in malignant-CIF while CA and CAFE use was greater in benign-CIF (p < 0.001). PA + PAFE prevailed in those countries where LP was the main HPN-provider and CA + CAFE prevailed where the main HPN-provider was HCC (p < 0.001). CONCLUSIONS: This is the first study to demonstrate that HPN provision and the IVS-admixture differ greatly among countries, among HPN centers and between benign-CIF and cancer-CIF. As both HPN provider and IVS-admixture types may play a role in the safety and effectiveness of HPN therapy, criteria to homogenize HPN programs are needed so that patients can have equal access to optimal CIF care.

[Home parenteral nutrition. May it be performed in Hungary, too?]. / Otthoni parenteralis táplálás. Lehetséges Magyarországon is?

Patients suffering from intestinal failure can receive home parenteral nutrition in several countries of the world since the end of the 1960s. In Hungary, intestinal failure patients can receive parenteral nutrition only in hospital, even if they can live in family or they are capable to work under certain circumstances because the present health financial system and legal environment do not allow home parenteral nutrition. This system is extremely uneconomical, reserves hospital beds and is opposing the European practice. Based on literature of recent years, the authors reviewed the life expectancy, the quality of life of patients receiving home parenteral nutrition and the frequency of complications and home parenteral nutrition systems applied worldwide. The specialized knowledge needed for home parenteral nutrition is entirely available, the solutions and technical supports are ready in the Hungarian market. Professionals committed to nutrition therapy have initiated academic collaboration among specialties to arrange the poorly handled questions of home parenteral nutrition and to establish the home parenteral nutrition system in Hungary.

From eosinophilic esophagitis to esophagus perforation: clinical management strategies.

INTRODUCTION: Eosinophilic esophagitis is a chronic, antigen-mediated inflammation of the esophagus. The disease is most common at young ages, with a male to female ratio of 3:1. Eosinophilic granulocyte infiltration induced by oral/aeroantigens in the esophagus, mucosal hyperplasia, and fibrosis of the subepithelial layers can lead to constriction, dysphagia, blockage and esophageal perforation. CASE REPORT: A 36-year-old male patient presented in June 2016 with dysphagia as the main complaint. Workup with plain chest radiography with a water soluble contrast swallow did not reveal any pathological lesions. The patient's swallowing difficulties persisted and one year later he was treated by esophageal food bolus impaction (EFBI) in another institution. A new plain chest radiography with a water soluble contrast swallow confirmed a 9 cm long stricture in the middle third with an EFBI. During gastroscopy, a clinical picture of eosinophilic esophagitis was noted, with partially destroyed foreign body at 25cm and iatrogenic perforation at the upper half of the esophagus. After preoperative intensive care unit valuation and preparation, transhiatal esophagectomy without thoracotomy and cervical esophagostomy was performed with pyloromyotomy and feeding jejunostomy. The postoperative period was uneventful. Histological examination confirmed the presence of strictures and perforation on the background of eosinophilic esophagitis. Elective esophageal reconstruction with cervical esophagogastric anastomosis was performed on January 2018. Control blood tests revealed persistent eosinophilia, while the plain chest radiography with a water soluble contrast swallow showed no contrast leakage. Per os nutrition was resumed and the patient was discharged in good general condition. CONCLUSIONS: Eosinophilic esophagitis is a rare and difficult to diagnose entity due to its non-specific clinical presentation. In order to avoid complications and undesired delay in diagnosis, one should take into consideration this entity in every clinical situation of a young male patient with swallowing complaints.

Clinical classification of adult patients with chronic intestinal failure due to benign disease: An international multicenter cross-sectional survey.

BACKGROUND & AIMS: The aim of the study was to evaluate the applicability of the ESPEN 16-category clinical classification of chronic intestinal failure, based on patients' intravenous supplementation (IVS) requirements for energy and fluids, and to evaluate factors associated with those requirements. METHODS: ESPEN members were invited to participate through ESPEN Council representatives. Participating centers enrolled adult patients requiring home parenteral nutrition for chronic intestinal failure on March 1st 2015. The following patient data were recorded though a structured database: sex, age, body weight and height, intestinal failure mechanism, underlying disease, IVS volume and energy need. RESULTS: Sixty-five centers from 22 countries enrolled 2919 patients with benign disease. One half of the patients were distributed in 3 categories of the ESPEN clinical classification. 9% of patients required only fluid and electrolyte supplementation. IVS requirement varied considerably according to the pathophysiological mechanism of intestinal failure. Notably, IVS volume requirement represented loss of intestinal function better than IVS energy requirement. A simplified 8 category classification of chronic intestinal failure was devised, based on two types of IVS (either fluid and electrolyte alone or parenteral nutrition admixture containing energy) and four categories of volume. CONCLUSIONS: Patients' IVS requirements varied widely, supporting the need for a tool to homogenize patient categorization. This study has devised a novel, simplified eight category IVS classification for chronic intestinal failure that will prove useful in both the clinical and research setting when applied together with the underlying pathophysiological mechanism of the patient's intestinal failure.

Analysis of Circulating MicroRNAs In Vivo following Administration of Dexamethasone and Adrenocorticotropin.

Purpose. The interaction of hormones of the pituitary-adrenal axis and adrenal cortex-associated circulating microRNAs is mostly unknown. We have studied the effects of dexamethasone and adrenocorticotropin on the expression of five circulating microRNAs (hsa-miR-27a, hsa-miR-200b, hsa-miR-214, hsa-miR-483-5p, and hsa-miR-503) reported to be related to the adrenal cortex in plasma samples. Methods. Expression of microRNAs was studied in plasma samples of 10 individuals examined by 1 mg dexamethasone suppression test and another 10 individuals stimulated by 250 µg tetracosactide (adrenocorticotropin). Total RNA was isolated and microRNA expression was analyzed by real-time reverse transcription quantitative polymerase chain reaction normalized to cel-miR-39 as reference. Results. Only circulating hsa-miR-27a proved to be significantly modulated in vivo by hormonal treatments: its expression was upregulated by dexamethasone whereas it was suppressed by adrenocorticotropin. Secreted hsa-miR-27a was significantly induced by dexamethasone in vitro in NCI-H295R cells, as well. The expression of hsa-miR-483-5p proposed as diagnostic marker for adrenocortical malignancy was not affected by dexamethasone or tetracosactide administration. Conclusions. hsa-miR-27a expression is modulated by hormones of the hypothalamic-pituitary-adrenal axis both in vitro and in vivo. The biological relevance of hsa-miR-27a modulation by hormones is unclear, but the responsiveness of circulating microRNAs to hormones of the pituitary-adrenal axis is noteworthy.

Endoscopic treatment of sphincterotomy-associated distal common bile duct strictures by using sequential insertion of multiple plastic stents.

BACKGROUND: A rare, late complication of endoscopic biliary sphincterotomy is the occurrence of short strictures extending from the papillary orifice to the distal parts of the extraduodenal common bile duct. METHODS: We evaluated the efficacy of the sequential insertion of multiple stents in the treatment of endoscopic biliary sphincterotomy associated common bile duct strictures. The design of the study is a prospective, single-arm observational study at a university-affiliated teaching hospital of 20 patients with distal common bile duct strictures because of choledocholithiasis-related endoscopic biliary sphincterotomy. Endoscopic treatment consisted of the sequential insertion of an increasing number of plastic stents with ever-larger diameters in 3-month follow-up intervals until stricture resolution. The primary outcome of the study was the rate of resolution of the stricture. The parameters measured were the duration of placement of stents, the maximum diameter, the total number of stents, and the total number of endoscopic sessions required for dilation of the strictures. RESULTS: After a median of 9.0 months of stent placement (range 3-22 months) and a median of 20F maximum stent diameter (range 10F-30F), 18 patients (90%) remained stent-free for a median of 14.5 months (range 6-38 months). Two patients (10%) had stricture recurrences at 10 and 24 months. Multivariate regression analysis demonstrated that the time elapsed after endoscopic biliary sphincterotomy was significantly associated with the stent-placement time (however, significance was removed by correction for multiple testing) and the number of ERCPs required for dilation. The initial common bile duct size was significantly associated with the total stent number and diameter needed for stricture resolution (however, significance was removed by correction for multiple testing). Limitations are the low case number and the single-arm, noncontrolled study design. CONCLUSIONS: Sequential insertion of an increasing number of biliary stents affords effective treatment of the distal biliary strictures that develop as a late complication of endoscopic biliary sphincterotomy.

Medium-term results of endoscopic treatment of common bile duct strictures in chronic calcifying pancreatitis with increasing numbers of stents.

OBJECTIVES: The goal of this study was to evaluate our medium-term results on common bile duct stenting with increasing numbers of stents on strictures due to chronic calcifying pancreatitis. BACKGROUND: Common bile duct strictures frequently complicate the course of chronic calcifying pancreatitis. The effectiveness of endoscopic stenting to resolve definitely these strictures is still debated. STUDY: Twenty-nine patients with common bile duct stricture due to chronic calcifying pancreatitis were stented and followed up. Biliary sphincterotomy, dilation of the stricture, and insertion of plastic biliary stents (7.5-10 F) were performed. Patients were scheduled for elective stent changing/restenting at 3-month intervals or any time when it was urgently indicated. Our basic intention was to insert the maximum possible number of stents to reach as large diameter as the stricture allowed. All stents were removed after the disappearance of common bile duct dilatation or left in place in cases of persisting strictures. RESULTS: Eighteen patients (60%) had complete radiologic and serologic recovery after a mean of 21.1 months overall stenting time and had a stent free follow-up period for a mean of 12.1 months without recurrence of stricture. Five patients (16%) still have stents in place after 26 months. Three patients (13%) required surgery. There were 3 deaths (10%): 1 for unrelated cause and 2 with septic shock of biliary origin. CONCLUSIONS: Most chronic calcifying pancreatitis patients with common bile duct strictures respond to the increasing numbers of endoscopic stents, and remain stent free for medium term periods. Less patients (30%) does not benefit of biliary stenting, who are candidates for surgery.

Autoimmune pancreatitis associated with immune-mediated inflammation of the papilla of Vater: report on two cases.

Autoimmune pancreatitis (AIP) is defined histologically by periductal and interacinar lymphocytic infiltration. Immunohistochemically, the majority of these lymphocytes are identified as T cells. Epithelial HLA-DR antigen expression was also described as a marker of autoimmunity in this type of chronic pancreatitis. We report 2 cases, a 56-year-old man and a 29-year-old woman, with AIP associated with immune-mediated inflammation of the main duodenal papilla (MDP). Serologically, antinuclear antibody positivity was detected in the male patient. The female patient, treated medically for ulcerative proctitis, had no serological evidence of autoimmune disease. Macroscopic papillitis was present only in the male patient, and endoscopic biopsy samples were taken from this swollen MDP. Since we could not exclude malignancy, a pancreatic head resection was performed in both patients. The histologic and immunohistochemical studies of the resected specimens showed periductal T-lymphocytic infiltration in the pancreatic and papillary tissues. Furthermore, HLA-DR-antigen expression was also demonstrated in epithelial cells of the pancreas and MDP. The immunohistological features of endoscopic biopsy samples from the swollen MDP were identical as in the surgically resected specimens. Immune-mediated inflammation of the MDP may be associated with AIP.