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A new section on the approach to diagnostic histological interpretation, is the overture to this chapter on inherited and developmental disorders. This initial section is split chronologically into the early neonatal and infantile period and later childhood and adulthood; with the intention of reflecting clinical practice as closely and succinctly as possible. Disorders of the biliary tree, bile formation and secretion, and hepatocyte metabolism are the core of this chapter, a merger of chapters 3 and 4 of the previous edition. Considerations on the pathogenetic and/or clinical overlap between developmental, genetic and metabolic disorders were the rationale behind this change. The complexity of hepatocyte metabolism is reflected into the miriad of related pathological conditions. Recent technological advances, particularly in genomics in the last five years, have resulted in a plethora of new entities and changes in terminology, challenging the authors to balance detail and application to clinical practice. Tables and figures from previous editions have been largely kept due to their quality and contemporary relevance, whilst new ones have been added to accommodate new advances (e.g. classification of mitochondrial disorders), a compromise to bridge between the two editions for the accustomed reader. Liver involvement in immunodeficiency and miscellaneous disorders precede the final section on anatomical anomalies. Vascular anomalies are now included in the chapters on vascular disorders.

OBJECTIVE: To evaluate whether pathologic complete response (pCR) exclusively defines major pathologic response to treatment with improved survival. SUMMARY BACKGROUND DATA: pCR following trimodality therapy for esophagogastric adenocarcinoma is infrequent but associated with improved prognosis. Yet most clinical trials and correlative studies designate pCR as the primary endpoint. METHODS: We analyzed our prospectively maintained database for patients who underwent trimodality therapy for locally advanced esophageal adenocarcinoma between 1995 and 2017. Overall survival (OS) was examined by percentage treatment response (TR) in the primary tumor bed and pathologic nodal stage (ypN0) using Kaplan-Meier plots. Optimal thresholds of TR for differentiating patients in terms of OS were investigated with descriptive plots using restricted cubic spline functions; associations were quantified using Cox multivariable analysis. RESULTS: Among 788 patients, median follow-up was 37.5 months (range, 0.4–210.6); median OS was 48.3 months (95% CI, 42.2–58.8). Absence of residual nodal disease was independently associated with improved survival (P

ZusammenfassungIn jüngster Zeit wurden in der Therapie der eosinophilen Ösophagitis (EoE) und insbesondere im Bereich der topischen Corticosteroide erhebliche Fortschritte erreicht. Neue EoE-spezifische Darreichungsformen wurden entwickelt und haben in Form der orodispersiblen Budesonid-Tablette zu der ersten in Deutschland und anderen europäischen und außereuropäischen Ländern zugelassenen Therapie der EoE bei Erwachsenen geführt. In den USA steht eine EoE-spezifische orale Budesonid-Suspension kurz vor der Zulassung. Dagegen bleibt die wissenschaftliche Datenlage zur Wirksamkeit von Protonenpumpeninhibtoren weiterhin limitiert. Auch im Bereich der Biologika konnten nach langer Zeit Substanzen identifiziert werden, die erstmals in Phase 2 sehr vielversprechende Ergebnisse gezeigt haben und sich derzeit in klinischen Prüfungen der Phase 3 befinden. In diesem Artikel sollen die aktuellen Fortschritte und Perspektiven in der Therapie der EoE dargestellt und diskutiert werden.

Lysosomal acid lipase (LAL) deficiency is a rare, autosomal recessive disease caused by mutations in the LIPA gene, which produces cholesteryl ester and triglyceride accumulation predominantly in hepatocytes, adrenal glands, and gastrointestinal tract. We describe two new cases occurring in siblings, aged 5 and 7 years, who presented with hepatomegaly, dyslipidemia, and abnormal liver function. Percutaneous liver biopsy revealed portal inflammation, hypertrophic Kupffer cells with a foamy appearance and microvesicular steatosis with fibrosis. Immunostaining for lysosomal markers, cathepsin D and LAMP1 reflected the lysosomal nature of the lipid vacuoles. After enzymatic confirmation, enzyme replacement therapy was initiated for both siblings. Follow-up transaminase levels and lipid profiles showed a notable decrease in AST and ALT and a slight increase in HDL cholesterol. It is crucial to increase awareness of this rare condition among clinicians and pathologists. The expression of lysosomal markers around the lipid vacuoles might help diagnose LAL deficiency in pediatric patients.

Objective To assess the initial clinical experience with a novel endograft system (NEXUS Aortic Arch Stent Graft System) designed to treat aortic arch pathologies and address the morphology and hemodynamic challenges of the aortic arch. Summary background data The aortic arch remains the most challenging part of the aorta for both open and endovascular repair. Transcatheter aortic arch repair has the potential to significantly reduce surgical risks. Methods Patients underwent transcatheter aortic arch repair with a single branch, two stent graft system, implanted over a through-and-through guidewire from the brachiocephalic trunk, to the descending aorta with an ascending aorta stent graft. The ascending aorta stent graft is deployed into a designated docking sleeve to connect the two stent grafts and isolate the aortic arch pathology. Proximal landing zone in all cases was in Zone 0. Anatomical inclusion criteria included adequate landing zone in the ascending aorta, brachiocephalic trunk and descending thoracic aorta. Preparatory debranching procedure was performed in all patients with carotid - carotid crossover bypass and left carotid to left subclavian bypass, or parallel graft from descending aorta to left subclavian artery. Safety and performance were evaluated through one year. Survival analysis used the Kaplan-Meier method. Results Twenty-eight patients, 79% males, with a mean age of 72.2 ± 6.2 years were treated with 100% procedural success. Isolated aortic arch aneurysm was the principle pathology in 17 (60.7%) of patients while chronic aortic dissection was the principle pathology in 6 (21.4%) of patients. The remaining 5(17.8%) had combined or other pathologies. At one month, the vascular pathology was excluded in 25 of 26 alive patients (96.1%). The 30 days mortality rate was 7.1%, stroke rate was 3.6% (all non-disabling) and combined mortality/stroke rate was 10.7%. One year mortality was 10.7%, without device or aneurysm related death. Two patients (7.1%) reported stroke or transient ischemic attack at one year that recovered completely. One year combined mortality/stroke rate was 17.8%. There were three patients (10.7%) that had device related unplanned reinterventions through one year. Conclusions The NEXUS Aortic Arch Stent Graft System, a novel single branch, two stent graft system used for endovascular aortic arch repair that requires landing in the ascending aorta, demonstrates a high success rate with excellent one year safety and performance.

Malakoplakia is a rare chronic inflammatory condition, which primarily occurs in genitourinary tract, with prostatic malakoplakia being extremely rare. We present two cases of acute urinary retention, with clinically firm nodular prostate and a raised serum prostate-specific antigen. Transrectal ultrasound-guided prostatic biopsy showed features of malakoplakia. There was a significant reduction of size of prostate on transrectal ultrasonography after 4 weeks of antibiotics. However, one patient had failed trial without catheter and was subjected to transurethral resection of prostate. The biopsy of the prostatic chips also showed features of malakoplakia. Other patient improved symptomatically after antibiotics and was managed conservatively. Both the patients are on regular follow-up and are asymptomatic. Prostatic malakoplakia presenting as urinary retention is very uncommon with around 12 cases in the literature. Recognition of prostatic malakoplakia is important because clinically it can masquerade prostatic malignancy. Treatment with antibiotics is necessary before subjecting the patients for surgery in patients with obstructive symptoms.

Acute oesophageal necrosis is a rare cause of haematemesis associated with high mortality and morbidity in elderly patients with multiple comorbidities. Acute oesophageal necrosis is thought to be caused by a combination of hypoperfusion, vascular disease and duodenal disease causing transient gastric outlet obstruction and therefore reflux of gastric contents. The subsequent necrosis is associated with significant morbidity and mortality. We present a case of an 83-year-old man presenting with sepsis secondary to gallstone cholangitis, who developed haematemesis 2 days post admission. Oesopho-gastro-duodenoscopy demonstrated necrosis to the oesophagus and duodenal ulceration. This was the first case of acute oesophageal necrosis observed within our hospital. We review the literature on the management of acute oesophageal necrosis and discuss the impact of acute oesophageal necrosis and its complications on the patient’s long-term outcome.

We present a case of a 41-year-old woman who visited the emergency department (ED) with acute abdomen. She was diagnosed with perforated appendicitis and abscess formation on CT. She was treated conservatively with antibiotics and discharged. On control CT 3 months later, the appendix had healed, but signs of thickening of the terminal ileum were noticed and colonoscopy was performed, which was uneventful and showed no signs of inflammation. Twelve hours later, she developed pain in the right lower quadrant, followed by fever, and visited the ED. Physical examination and blood work showed signs consistent with acute appendicitis, and appendectomy was performed laparoscopically 6 hours later. The patient recovered remarkably shortly afterwards. Whether colonoscopy resulted in de novo appendicitis or exacerbated an already existing inflammation remains unknown. However, endoscopists should be aware of this rare, yet serious complication and consider it in the workup of post-colonoscopy abdominal pain.

A male patient, aged over 75 years, was referred to the emergency room for pain in his right hypochondrium. This pain, which persisted for approximately 3 days, radiated to his right flank and right iliac quadrant and worsened after meals. The patient reported no nausea, vomiting, diarrhoea, fever or recent traumatic events. Blood laboratory tests, ultrasonography, an MRI and a CT scan were performed, leading to the diagnosis of a mass in the Morrison’s pouch with ultrasound features of adipose tissue compatible with a liposarcoma, infiltrating the liver and the right kidney. Subsequent CT-guided needle aspiration and a histological examination led to the diagnosis of actinomycosis. The patient was initially treated with a daily dose of 18 million IU of benzylpenicillin for 4 weeks and subsequently prescribed the oral administration of 3 g/day of amoxicillin for 11 months. A monitoring CT scan was performed after 1, 5, 8 and 12 months, and a CT scan re-evaluation confirmed that the mass had completely healed.

A term male infant was born to a healthy 24-year-old mother with antenatally diagnosed liver-up, left congenital diaphragmatic hernia (CDH) and gastroschisis. The infant was stabilised in the neonatal intensive care unit and then underwent primary repair of the CDH via left subcostal incision and silo placement for the gastroschisis. Serial silo reductions were started postoperatively and umbilical flap closure for the gastroschisis was performed on day of life 6. The patient was weaned from respiratory support, started on enteral feeds, and discharged home at 1 month of age. He was weaned from supplemental nasogastric feeds by 6 weeks of age and is currently well and thriving at 11 months of age.

Primary retroperitoneal neuroendocrine tumours (NETs) are extremely rare, and many a times, these are metastatic lesions with known or unknown primary location, most commonly arising from the stomach, duodenum, small intestine and rectum. NETs arising from the duodenum are more commonly seen in the first part of the duodenum followed by the second part. The incidence is increasing because of easy accessibility to endoscopies and cross-sectional imaging. In NETs, lymph node (LN) metastasis occurs commonly when the tumour size is more than 2 cm. In contrast, LN metastasis occurs even with subcentimetric lesions, especially the ampullary variant of NETs. A patient presented to us with mild abdominal pain and found to have retroduodenal mass. On evaluation found to be a metastatic LN deposit of NET with the primary arising from the first part and supra-ampullary part of duodenum.

A 57-year-old man was incidentally diagnosed with a 7 cm left-sided retroperitoneal mass. The mass was presumed to be arising from the left adrenal gland on a CT scan of the adrenal glands. Thus, a thorough workup ensued, looking into the possible functionality of the lesion. All investigations resulted in the negative. Subsequently, the patient was referred for surgery to fully characterise the tumour. Histology proved that the lesion was a benign retroperitoneal schwannoma with no relation to the adrenal gland.

Primary adenocarcinoma of the fourth portion of the duodenum (D-IV) is reported infrequently than other parts of the duodenum. Its diagnosis is often late because of non-specific symptoms and signs. We encountered a 48-year-old male patient who was diagnosed as duodeno-duodenal intussusception, underwent segmental duodenal resection with duodenojejunal anastomosis and confirmed as adenocarcinoma of D-IV. He received adjuvant chemotherapy and is doing well at 1 year of follow-up. This report describes about the rare case of isolated adenocarcinoma of the D-IV presented as intussusception which is never reported before and successfully treated by segmental resection of the duodenum and jejunum.

Boerhaave’s syndrome or spontaneous perforation of the oesophagus is a life-threatening condition that carries high mortality. Delayed diagnosis has a mortality rate of 20%–50%. While surgical intervention has been the mainstay of treatment, advancements in endoscopy and oesophageal stenting have allowed for alternative management. Our case involves a 33-year-old man with self-induced emesis and DKA. After 10 days in the ICU, he developed a large right pleural effusion, which was treated with chest tube placement. Upper GI study confirmed delayed Boerhaave’s syndrome. A self-expanding stent was inserted followed by percutaneous endoscopic gastrostomy (PEG) for decompression and jejunal extension for nutrition. He developed empyema and underwent right thoracotomy for washout and lung decortication. Stent was exchanged once due to recurrent leak following migration and removed after 40 days. Endoscopic stent placement with PEG with jejunal extension followed by thoracotomy is a viable alternative to primary repair of delayed oesophageal perforation.

Morgagni hernia is the rarest type of congenital diaphragmatic hernia, which can present late in adulthood. Here, we report a case of Morgagni hernia in an elderly woman who presented as an acute coronary syndrome with raised troponin level. X-ray of the chest (CXR) showed air–fluid level in the right lower hemithorax with loss of right diaphragmatic outline and subsequently confirmed strangulated Morgagni hernia with CT. She was treated with emergency laparotomy to reduce the hernia content and surgical repair with mesh done. In conclusion, Troponin can be falsely positive in Morgagni hernia patients, possibly due to strain on the heart by herniated bowel contents. Basic imaging such as a (CXR) is useful in the case of chest pain to rule out the non-cardiac causes. Although ‘time is the myocardium’ in the setting of all cases of chest pain with raised troponin, CXR should be done before treatment that poses bleeding risk and unnecessary delay in laparotomy.

A 62-year-old woman presented with non-specific abdominal pain, elevated alkaline phosphatase levels and bilateral sequential visual loss. Visual acuity was counting fingers in right eye (RE) and 20/400 in left eye (LE). She was noted to have optic nerve pallor in RE and mildly elevated optic disc with signs of ocular inflammation in LE. After 2 weeks, vision deteriorated to light perception bilaterally with now extensive vitreal inflammation present in both eyes. Positive rapid plasma reagen and Treponema pallidum’s antibody tites confirmed syphilis infection. Unfortunately, as the diagnosis was delayed by many months, her visual acuity remained poor (hand motions in RE and 20/50 in LE) despite treatment with intravenous penicillin. This case reminds us of the re-emergence of this ‘great masquerader’ and highlights the importance of maintaining high suspicion for syphilis in patients with unexplained visual loss and systemic symptoms, even in older adults without identifiable risk factors.

We present an 87-year-old woman who presented to the emergency department with a 7-day history of severe abdominal pain at her ileostomy parastomal hernia site. Prior to presentation, her general practitioner had started her on a reducing steroid dose for suspected Crohn’s disease exacerbation. On examination, she had a distended abdomen with localised guarding and tenderness over her hernia site. A CT scan demonstrated an incarcerated perforated gallbladder within her parastomal hernia. Successful surgical management was performed involving an enterotomy, refashioning her ileostomy and an open cholecystectomy. The patient recovered well with a short postoperative stay. This report is intended as a guide for clinicians in the differential diagnoses for acute abdominal pain and an unusual presentation of a gallbladder perforation.

Gastroenteropancreatic neuroendocrine tumours (GEPNETs) are a heterogenous group of tumours which are rising in incidence. Morbidity and mortality related to these tumours is dependent on the location of metastatic spread. Hyperammonaemia and subsequent encephalopathy has previously been described in GEPNET and is typically associated with a poor prognosis. We describe a case of a 55-year-old woman with hyperammonaemic encephalopathy and a new diagnosis of GEPNET. Given the poor prognosis and the outcomes in this patient group we feel this case highlights the benefit of a multimodality treatment approach including peptide receptor radionucleotide therapy and transarterial chemoembolisation.

Background Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and coronavirus disease 2019 (COVID-19) continues to have a devastating impact across the globe. However, little is known about the disease course in patients with autoimmune hepatitis (AIH). Methods Data for patients with AIH and SARS-CoV-2 infection were combined from three international reporting registries and outcomes were compared to those with chronic liver disease of other aetiology (non-AIH CLD) and to patients without liver disease (non-CLD). Results Between 25th March and 24th October 2020, data were collected for 932 patients with CLD and SARS-CoV-2 infection including 70 with autoimmune hepatitis (AIH). Fifty-eight (83%) of AIH patients were taking one or more immunosuppressive drug. There were no differences in rates of major outcomes between AIH and non-AIH CLD including hospitalization (76% vs 85%; p= 0.06), ICU admission (29% vs. 23%; p=0.240), and death (23% vs. 20%; p=0.643). Factors associated with death within the AIH cohort included age (OR 2.16/10 years; 1.07–3.81), Child-Turcotte-Pugh (CTP) class B (OR 42.48; 4.40–409.53), and CTP-C cirrhosis (OR 69.30; 2.83–1694.50), but not use of immunosuppression. Propensity score matched (PSM) analysis comparing AIH with non-AIH CLD demonstrated no increased risk adverse outcomes including death (+3.2%; -9.2%–15.7%). PSM analysis of AIH versus non-CLD patients (n=769) demonstrated increased risk of hospitalization with AIH (+18.4%; 5.6–31.2%), but equivalent risk of all other outcomes including death (+3.2%; -9.1%–15.6%). Conclusion AIH patients were not at increased risk of adverse outcomes despite immunosuppressive treatment compared to other causes of CLD and to matched cases without liver disease., Graphical abstract, Highlights • This is the largest cohort of patients with autoimmune hepatitis and laboratory proven SARS-COV-2 infection reported to date. • There were no differences in rates of major adverse COVID-19 outcomes including hospitalization, intensive care unit (ICU) admission, and death between AIH patients and those with other aetiologies of liver disease. • When compared to patients without liver disease in propensity score matched analysis, patients with AIH had higher rates of hospitalization but no increased risk of ICU admission or death despite potential reporting of AIH cases with more severe baseline liver disease. • Independent risk factors for death in AIH patients were age and baseline liver disease severity, but not the use of immunosuppression., Lay summary: Little is known about the outcomes of COVID-19 in patients with autoimmune hepatitis (AIH), a rare chronic inflammatory liver disease. This study combines data from three large registries to describe the course of COVID-19 in this patient group. We show that AIH patients do not appear to have an increased risk of death from COVID-19 compared to patients with other forms of liver disease and compared to patients without liver disease, despite the use of medications which suppress the immune system.

Objective: : To develop an international Core Outcome Set (COS), a minimal collection of outcomes that should be measured and reported in all future clinical trials evaluating treatments of acute simple appendicitis in children.Summary Background Data: A previous systematic review identified 115 outcomes in 60 trials and systematic reviews evaluating treatments for children with appendicitis, suggesting the need for a COS.Methods: The development process consisted of four phases: (1) an updated systematic review identifying all previously reported outcomes, (2) a two-stage international Delphi study in which parents with their children and surgeons rated these outcomes for inclusion in the COS, (3) focus groups with young people to identify missing outcomes, and (4) international expert meetings to ratify the final COS.Results: The systematic review identified 129 outcomes which were mapped to 43 unique outcome terms for the Delphi survey. The first-round included 137 parents (eight countries) and 245 surgeons (10 countries), the second-round response rates were 61% and 85% respectively, with ten outcomes emerging with consensus. After two young peoples’ focus groups, two additional outcomes were added to the final COS (12): mortality, bowel obstruction, intra-abdominal abscess, recurrent appendicitis, complicated appendicitis, return to baseline health, readmission, reoperation, unplanned appendectomy, adverse events related to treatment, major and minor complications.Conclusion: An evidence-informed COS based on international consensus, including patients and parents has been developed. This COS is recommended for all future studies evaluating treatment of simple appendicitis in children, to reduce heterogeneity between studies and facilitate data synthesis and evidence-based decision-making.

Mucocele of the apendix is a rare clinical and pathological entity with a higher incidence in the female population, and non-specific symptomatology that includes more diagnoses including neoplastic and non-neoplastic causes of the apendix dilation and mucin proliferation. With obtained numerous variations in symptomatology, clinical picture, intraoperative presentation, surgical treatment and histopathological findings, the aim of this paper is an inspection into current knowledge of this disease, its histopathological characteristics, differential diagnosis and current treatment modalities. We present a patient with a clinical picture of the acute abdomen and numerous comorbidities. Ultrasound diagnostics indicated the presence of thin-walled dilatation of the small intestine, especially in the lower right quadrant, as well as the presence of free fluid in the peritoneal cavity. Apendectomy and removal of the mucocele and resection of the gangrenous sigmoid colon according to Hartmann were done. A definitive histopathological finding suggests ischemic colitis and retention mucocele of the apendix with normal mucosa. Histopathologically, four types of mucocele apendixes are distinguished, namely the retention mucinous cyst, mucosal hyperplasia, mucinous cystadenoma and mucinous cystadenocarcinoma. Surgical treatment is the method of choice in treating these tumors, either open or laparoscopic. Imperative is the preservation of the integrity of the entire mucocele, due to the possible dissemination of contents of the cyst and the subsequent development of pseudomyxoma peritonei.

A 21-year-old woman visited out hospital for lower abdominal pain and bloody diarrhoea at 19 weeks of pregnancy. Endoscopic findings revealed longitudinal ulcerations with hyperaemia and oedema in the sigmoid colon. These findings and clinical presentation confirmed the diagnosis of ischaemic colitis. Conservative treatment, including fasting and intravenous hydration, was administered, and the patient made a good recovery. After discharge, there was no recurrence during pregnancy and postpartum period. It is important to make early diagnosis and treatment, and multidisciplinary teamwork between obstetricians, gastroenterologist and endoscopist is required.

Rectal duplication cysts are rare congenital anomalies of the gastrointestinal tract. They can present with pain, gastrointestinal bleeding, infection or compressive symptoms on the rectum and urinary bladder. A 79-year-old man presented with a 8×5 cm swelling in the sacral region above the natal cleft with a positive cough impulse. During surgical exploration, there were two cysts with the posterior one presenting as the hernia. The cyst was excised and histopathological examination confirmed a diagnosis of rectal duplication cyst. Rectal duplications can lie anterior or more commonly posterior to the rectum. Differential diagnoses include dermoid cyst, diverticular cyst, sacrococcygeal teratoma or meningocele and endoscopic ultrasound is imaging of choice for diagnosis. Surgical excision is the treatment of choice. This is a rare presentation of a rectal duplication cyst as a perineal hernia with only a handful of cases in literature.

A 77-year-old woman presented with a 2-week history of malaise, prostration, anorexia, abdominal pain, vomiting and diarrhoea. She had been taking systemic corticosteroids for the past year. During hospitalisation, renal insufficiency, ionic changes and liver function abnormalities were detected and corrected. However, the patient developed total dysphagia. UGE revealed multiple shallow ulcers below the cricopharyngeal level and in the distal oesophagus, with normal-appearing intervening mucosa. Histological examination allowed the diagnosis of herpes simplex virus esophagitis. Treatment with intravenous acyclovir was instituted for 14 days. In the elderly, herpetic esophagitis may present with non-specific complains, such as prostration or anorexia. In the reported case, dysphagia was only detected as a late symptom, addressing the importance of maintaining a high degree of suspicion for the diagnosis of herpes simplex virus esophagitis.

A young male in his early 30s presented to us with increasing swelling at the umbilicus, and an umbilical hernia was diagnosed. At laparoscopic intraperitoneal onlay mesh (IPOM) repair, an unexpected finding of a thin innocuous-looking fibrous film over the small bowel was noted. This finding presented a dilemma as to the probable pathology of this material, and a decision had to be made on whether laparoscopic IPOM could be continued. It was prudently decided to abandon the plan of placing a mesh intraperitoneally and an open repair of the umbilical hernia was done. In retrospect this was a wise decision, as, after 7 months he had to have a laparotomy for intestinal obstruction, when the classic thick fibrous encapsulating abdominal cocoon was seen. Hence here we have followed the evolution of the abdominal cocoon from its original asymptomatic phase to the classic encapsulating sclerosing peritonitis with probably laparoscopic gas insufflation being the precipitating factor.

Double filtration plasmapheresis (DFPP) is an apheretic technique that selectively removes high molecular weight substances using a plasma component filter. DFPP has been used to treat positive-sense RNA virus infections, mainly chronic hepatitis C virus (HCV) infection, because of its ability to directly eliminate viral particles from blood plasma from 2008 to about 2015, before direct-acting antiviral agents was marketed. This effect has been termed virus removal and eradication by DFPP. HCV is a positive-sense RNA virus similar to West Nile virus, dengue virus and the SARS and Middle East respiratory syndrome coronaviruses. SARS-CoV-2 is classified same viral species. These viruses are all classified in Family Flaviviridae which are family of single-stranded plus-stranded RNA viruses. Viral particles are 40–60 nm in diameter, enveloped and spherical in shape. We present a rare case of HCV removal where an RNA virus infection that copresented with virus-associated autoimmune hepatitis was eliminated using DFPP. Our results indicate that DFPP may facilitate prompt viraemia reduction and may have novel treatment applications for SARS-CoV-2, that is, use of therapeutic plasma exchange for fulminant COVID-19.

In addition to the legs, restless legs syndrome (RLS) affects various other parts of the body, including the arms, abdomen, face, head-neck, oral cavity, genital area and bladder. RLS is also associated with several comorbid conditions, including irritable bowel syndrome (IBS). We are reporting two cases of RLS who also had IBS, fulfilling the Rome IV criteria. The administration of levodopa and dopamine agonists provided a complete improvement in both IBS and RLS. Review of the literature suggest that the clinical semiology and clinical pattern of IBS (urge to defaecate, abdominal pain, abdominal distension, bloating, disturbed sleep and circadian rhythm) simulate the semiology and pattern of RLS. Similarities are also noted in the associated comorbid conditions, effective drugs and proposed hypotheses for both clinical syndromes. We hypothesise that RLS may affect intestine, and IBS-like symptoms in a subset of patients with RLS may be the part of RLS symptoms complex.

Granulomatosis with polyangiitis (GPA) is a rare necrotising small vessel vasculitis typically associated with oronasal, pulmonary and renal manifestations. Pancreatic disease is an exceedingly rare initial presentation and is associated with delayed diagnosis and rapid progression. We discuss a 66-year-old woman presenting with epigastric pain, elevated lipase and radiographic evidence of focal pancreatitis. She had no relevant medical history and no lithiasis seen on imaging. Pertinent findings include strawberry gingivitis, positive proteinase-antineutrophil cytoplasm antibody (98% specificity) and focal nodular parenchymal lung lesions on CT chest—all of which are consistent with a diagnosis of GPA. She was promptly started on high-dose steroids which resulted in significant clinical and biochemical improvement. Cyclophosphamide was added once biopsy confirmed the absence of malignancy. In order to optimise the clinical outcomes of GPA, physicians must keep a wide differential and high index of suspicion in the setting of unexplained pancreatitis with systemic features.

Background Adenoid cystic carcinoma of the trachea (ACCT) is a rare cancer; ACCT with thyroid invasion is particularly rare. We first suspected anaplastic thyroid carcinoma (ATC) but diagnosed ACC after performing fine needle aspiration cytology (FNAC). A tracheal origin was confirmed after operation. Case description We report the case of a 77-year-old female presenting to our hospital with acute inspiratory dyspnea requiring emergency tracheotomy. Physical examination revealed a right anterior neck swelling with a hard and unmovable mass. Computed tomography (CT) and ultrasonography (US) showed tumor extension to the right thyroid lobe, and between the first and third tracheal rings, which caused severe stenosis of the lumen. Next, we performed FNAC. Clinical findings were highly suspicious for ACCT with thyroid invasion. Thirty-five days after the first visit to our department, the patient underwent total laryngectomy, cervical esophagectomy, and thyroidectomy with bilateral selective neck dissections at another hospital. The tumor was located in the right posterior wall of the trachea, with extension into the right thyroid gland. Pathological examination showed an infiltrative carcinomatous proliferation with tubular and cribriform patterns. The tumor was classified as pT4N1. A definite diagnosis was made after histopathological analyis of the surgical specimen confirmed ACCT. The tumor was found to be positive for FABP7, a putative prognostic marker of ACC, and metastasized to the lungs 3 years after the surgery. Conclusions ACCT with thyroid invasion is an extremely rare malignant neoplasm. FNAC was useful for differentiating ACCT from other diagnoses and enabled appropriate surgical treatment.

Background & Aims Efforts to assess and improve the effectiveness of Barrett’s esophagus (BE) screening and surveillance are ongoing in the United States. Currently, there are limited population-based data in the United States to guide these efforts. Methods We performed a retrospective cohort study using data from large commercial and Medicare Advantage health plans in the United States from 2004 – 2019. We identified individuals with BE and analyzed the proportion who developed EAC. EACs were classified as prevalent EAC (diagnosed within 30 days of index endoscopy), post-endoscopy esophageal adenocarcinoma (PEEC, diagnosed 30 – 365 days after index endoscopy), and incident EAC (diagnosed 365 days or more after index endoscopy). Using this cohort, we performed a nested case-control study to identify factors associated with prevalent EAC at BE diagnosis and study healthcare utilization prior to BE diagnosis. Results We identified 50,817 individuals with incident BE. Of the 366 who developed EAC, 67.2%, 13.7%, and 19.1% were diagnosed with prevalent EAC, PEEC, and incident EAC respectively. Factors positively associated with prevalent EAC versus BE without prevalent EAC included male sex, dysphagia, weight loss, and Charlson-Deyo comorbidity score. In those with prevalent EAC, most patients with dysphagia or weight loss had their symptoms first recorded within three months of EAC diagnosis. Healthcare utilization rates were similar between those with and without prevalent EAC. Conclusions Two-thirds of EACs among individuals with BE are diagnosed at the time of BE diagnosis. Additionally, PEEC accounts for 14% of these EACs. These results may guide future research studies that investigate novel BE diagnostic strategies that reduce the morbidity and mortality of EAC.

A lumen-apposing metal stent (LAMS) is a saddle-shaped stent with large flanges at both ends, thereby preventing stent migration and helping with approximation of the adjacent structures. We report the case of a 25-year-old female with remnant choledochal cyst which was successfully treated with LAMS after initial treatment failure with a plastic stent. Although complete excision of the cyst is the definite treatment of choledochal cysts, endoscopic ultrasonography-guided cystoduodenostomy can be considered in cases wherein surgery is not feasible and dysplasia is not present. LAMS may be preferred to plastic stents for effective resolution of remnant choledochal cyst and prevention of ascending infection.

Background/Aims: Proinflammatory cytokines released from adipocytes can influence the development, progression, and treatment of inflammatory bowel disease (IBD), and may be associated with worse clinical outcomes. Methods: For 2016–2018, we analyzed data from the Nationwide Inpatient Sample to identify adult (≥18 years) hospitalizations with a primary discharge diagnosis of IBD. The study sample was divided based on the presence or absence of obesity. The primary outcomes included inpatient mortality, while the secondary outcomes consisted of system-based complications and disease implications on the United States healthcare system.Results: We identified 282,005 hospitalizations of IBD from 2016 to 2018. Of these hospitalizations, 26,465 (9.4%) had a secondary diagnosis of obesity while 255,540 (90.6%) served as controls. IBD hospitalizations with obesity had a higher mean age (47.9 years vs. 45.2 years, PPPPP

Patients with postoperative Crohn's disease are difficult to manage because of their risk of experiencing a more severe course, multiple symptom confounders, and poor sensitivity of symptomatic remission to rule out intestinal inflammation. In this group, data are lacking on biologic therapeutic efficacy, and recommendations are lacking for those with multiple medication failures. Novel noninvasive testing can simultaneously exclude alternate causes of symptoms (serum C4, fecal fat, small intestinal bowel overgrowth breath testing) and assess intestinal inflammation (fecal calprotectin, endoscopic healing index). In addition, endoscopy-based disease activity assessment and management are required. Endoscopy should be performed within 6 months of surgery, and aggressive disease activity monitoring can be considered with colonoscopy every 1-2 years subsequently to ensure late recurrence is detected. Patients with multiple resections should be screened for short bowel syndrome. Predictive biomarkers are needed to guide medication selection in this high-risk population. Postoperative prophylactic biologic therapy is prudent for patients with preoperative biologic failure. However, there are no high-quality data to guide which agent should be selected. Selecting biologics with an alternative mechanism of action in those who had failed a biologic with adequate drug concentrations and selection of different agents in those with previous intolerance are reasonable. Significantly more study is required to assess the efficacy of therapies in this setting.

Background To learn from the crisis caused by the coronavirus disease (COVID-19) pandemic and be prepared for future pandemics, it is important to investigate the impact of this period on the wellbeing of patients with inflammatory bowel disease (IBD). Aims To describe the health-related quality of life (HRQoL) and disease control of IBD patients during the first wave of the COVID-19 pandemic in The Netherlands. Methods Between March 17 and July 1, 2020, patients aged 18 years and older with IBD from the Erasmus MC (Rotterdam, The Netherlands) were invited to complete online questionnaires at week 0, 2, 6 and 12. The Inflammatory Bowel Disease Questionnaire (IBDQ), the Inflammatory Bowel Disease Control-8 (IBD-control-8) and the numeric rating scale on fatigue were used. The evolution of the different outcomes over time was measured using mixed models. Results Of 1151 invited patients, 851 patients (67% CD and 33% UC or IBD-U) participated in the study (response rate 74%). No relevant changes in total scores were found over time for the IBDQ (effect estimate 0.006, 95% CI [− 0.003 to 0.015]) and IBD-control-8 (effect estimate 0.004, 95% CI [0.998–1.011]). There was a slight, increasing trend in fatigue scores over time (effect estimate 0.011, 95% CI [0.004, 0.019]). Conclusions This first lock down due to the COVID-19 pandemic in The Netherlands did not impact on the HRQoL and disease control of patients with IBD. Up to date information may have contributed to a stable HRQoL in IBD patients even in an extreme period with restrictions and insecurities. Supplementary Information The online version contains supplementary material available at 10.1007/s10620-021-07118-8.

Background There are different methods to repair a perforated peptic ulcer, the two most frequently used are the Graham patch omentopexy and the primary closure. Currently there is no high-level evidence to provide guidance of the optimal method of repair. The aim of this study is to compare the outcomes of the two methods so as to provide improved guidance for surgeons undertaking this repair. Methods A systematic review and meta-analysis was conducted including any study that compared Graham patch omentopexy with primary closure in adults. Embase, Medline, Cochrane and Google's search engine were searched. The primary outcome was breakdown of the repair resulting in bile leak and the secondary outcomes were mortality, operation time, wound infection and time to start oral intake. The meta-analysis was conducted using Review Manager Software version 5:4. Outcome data were reported as odd ratios and weighted mean differences with their 95% confidence intervals. Results Of the 229 studies identified, 6 were suitable for analysis, 4 were retrospective, one was a prospective cohort study and one was a randomized controlled trial. Meta-analysis showed no difference in occurrence of bile leak or mortality between primary closure and Graham patch omentopexy (OR 0.64; 95% (0.26–1.54) & 0.66; 95% (0.25–1.76) respectively). There was no difference in the rates of wound infection OR 0.65; 95% (0.4–1.05). The duration of the operation was shorter in the primary closure group by 5.6 min; 95% (−21 + 10.4). Conclusion There was no difference in the clinical outcomes between the two modes of repair.

Background and aim Liver fibrosis resulting from persistent liver injury represents a major healthcare problem globally. Traditional Chinese medicine has played an essential role in the treatment of liver fibrosis in recent years. Thus, this study aims to assess the effect of Longhu Rendan (LHRD), a Chinese traditional patent medicine, on liver fibrosis and its potential mechanism. Methods The liver fibrosis in mice was induced via the intraperitoneal injection of carbon tetrachloride (CCl4) for 6 weeks or bile duct ligation for 15 days. Various methods were used to judge the therapeutic effect of LHRD. Results LHRD significantly suppressed the activity of serum index of abnormal liver function, liver cell apoptosis, and necrosis, attenuating liver injury. Moreover, LHRD treatment alleviated liver fibrotic features, such as the reduction of collagen deposition and hepatic stellate cell activation as well as profibrotic gene expression. Mechanistically, LHRD treatment inhibited nuclear transcription factor-kappa B signaling and inflammatory gene expression and diminished the production of reactive oxygen species and 4-hydroxynonenal, along with the downregulation of NADPH oxidase 4. Conclusions Overall, the present study demonstrates that LHRD ameliorates liver injury and fibrosis via the inhibition of inflammation and oxidative stress in mice, indicating that LHRD is a potential medicine for the treatment of liver fibrosis.

Esophageal bleeding is a common complication in patients with liver cirrhosis. In thee present study, our aim was to divulge major factors predicting esophageal bleeding in Iranian children with liver cirrhosis. This was a cross-sectional study including 101 children < 18 years old referred to the Pediatric Endoscopy Unit of Nemazee Teaching Hospital of Shiraz from 2014 until 2016. Children with esophageal varices were included. The patients were divided into two groups including those with and without history of esophageal bleeding. Statistical methods were performed in SPSS 16 software. There were 49 boys and 52 girls. The mean age was 7.74±5.26 years old. A history of esophageal bleeding was observed in 53 (52.4%). In univariate analyses, significant relationships were found between esophageal bleeding and varices size (P=0.001), Child-Pugh score (P=0.01), age of bleeding initiation (P

Background Serum hepatitis B surface antigen (HBsAg) levels correlate with the duration of chronic hepatitis B virus (HBV) infection and may predict the extent of hepatic fibrosis. Methods We analyzed data from the SONIC-B database, which contains data from 8 global randomized trials and 2 large hepatology centers. Relationship between HBsAg levels and presence of significant fibrosis (Ishak 3–4) or cirrhosis (Ishak 5–6) were explored, and clinically relevant cutoffs were identified to rule out cirrhosis. Results The dataset included 2779 patients: 1866 hepatitis B e antigen (HBeAg)-positive; 322 with cirrhosis. Among HBeAg-positive patients, lower HBsAg levels were associated with higher rates of significant fibrosis (odds ratio [OR], 0.419; P < .001) and cirrhosis (OR, 0.435; P < .001). No relationship was observed among HBeAg-negative patients. Among HBeAg-positive patients, genotype-specific HBsAg cutoffs had excellent negative predictive values (>97%) and low misclassification rates (≤7.1%) and may therefore have utility in ruling out cirrhosis. Diagnostic performance of the HBsAg cutoffs was comparable among patients in whom cirrhosis could not be ruled out with fibrosis 4 (FIB-4). Conclusions Hepatitis B virus genotype-specific HBsAg cutoffs may have utility in ruling out presence of cirrhosis in HBeAg-positive patients with genotypes B, C, and D and can be an adjunct to FIB-4 to reduce the need for further testing.

Summary Background The management of recurrent anal fistulas after previous surgery is usually challenging. The present study aimed to review the characteristics and treatment outcomes of recurrent anal fistulas as compared to primary anal fistulas. Methods The records of patients with anal fistula who underwent surgery were reviewed. Characteristics and treatment outcomes of patients with recurrent anal fistulas were compared to those of patients with primary anal fistula without a history of surgery. Results The study included 138 patients with recurrent anal fistulas, 76.8% of which were complex. Failure of healing was recorded in 25 (18.1%) patients and fecal incontinence (FI) in 9 (6.5%). Patients with recurrent anal fistulas had significantly higher percentage of anterior, complex, and horseshoe fistulas than patients with primary fistulas. Surgery for recurrent anal fistulas was followed by a significantly higher rate of failure of healing than primary fistulas (18.1% vs. 9.8%, P = 0.011), whereas the rates of FI were comparable amongst the two groups (6.5% vs. 2.8%, P = 0.07). Patients who had more than two previous operations for anal fistula had a significantly higher rate of FI than patients who underwent one or two previous surgeries (20% vs. 3.7% vs. 14.3%, P = 0.04), yet healing rates were comparable. Conclusion Recurrent anal fistulas were more complex than primary fistulas. Surgical treatment of recurrent anal fistula was followed by a significantly higher rate of failure of healing and similar rate of FI as compared to primary anal fistulas. The number of previous fistula surgeries had a significant effect on postoperative continence state.

Patients with symptomatic gastrointestinal stromal tumor (GIST) typically present with gastrointestinal bleeding and abdominal pain. This report presents an unusual case of fundic GIST complicated by gastroduodenal intussusception, manifesting as acute pancreatitis. The patient presented with epigastric pain and pancreatic enzyme elevation; thus, he was diagnosed with acute pancreatitis. Computed tomography showed evidence of pancreatitis and a 4×4.7 cm well-defined hyperdense lesion in the 2nd part of the duodenum, compressing the pancreatic head and pancreatic duct. Esophagogastroduodenoscopy revealed invagination of the gastric folds into the duodenum, causing pyloric canal blockage consistent with gastroduodenal intussusception. Spontaneous reduction of the lesion during endoscopy revealed a 4 cm pedunculated subepithelial mass with central ulceration originating from the gastric fundus. Endoscopic ultrasound demonstrated a heterogeneous hypoechoic lesion originating from the 4th layer of the gastric wall. Laparoscopic-endoscopic intragastric wedge resection of the fundic lesion was subsequently performed, and surgical histology confirmed GIST.

Colonic diffuse ganglioneuromatosis is an extremely rare disease in which multiple tumors derived from the ganglion cells, nerve fibers, and supporting cells are distributed in the colon. It is generally considered to be a benign neoplastic condition and is occasionally associated with rare hereditary conditions such as neurofibromatosis type I or multiple endocrine neoplasia type 2B. Here, we report a case of a patient in whom colon cancer developed 12 years after the initial diagnosis of colonic diffuse ganglioneuromatosis, which suggests a possible association between colonic diffuse ganglioneuromatosis and colorectal cancer.

According to the current concepts, pathogenesis of acne includes signifcant morphological and functional changes and hypercolonization of C. acnes of the sebaceous hair follicle. But most important input into pathogenesis of acne gives permanent inflammation, previously considered as a secondary pathophysiological reaction. Recently, much attention has been given to pruritus associated with acne lesions. This literature review analyzes the data concerning the pathogenetic role of neurogenic inflammation in acne; the clinical importance of itch in the overall presentation of the disease is underlined, and its influence on psychosomatic correlations is presented.

Background: Acromegaly is a disorder associated with hypersecretion of growth hormone, most usually caused by a pituitary adenoma. Dysmotility of the gastrointestinal tract has been reported in acromegalic patients. Achalasia is a disorder characterized by aperistalsis of the oesophagus with incomplete lower oesophageal sphincter relaxation and whose aetiology remains unknown. Mutations in some genes have previously been associated with the development of acromegaly or achalasia. The study aims were to analyse mutations in selected genes in a woman having both of these diseases, to identify their aetiological factors, and to suggest explanations for the co-incidence of acromegaly and achalasia. Methods and Results: A female patient with acromegaly, achalasia, and a multinodular thyroid gland with hyperplastic colloid nodules underwent successful treatment of achalasia via laparoscopic Heller myotomy, a thyroidectomy was performed, and the pituitary macroadenoma was surgically excised via transnasal endoscopic extirpation. Germline DNA from the leukocytes was analysed by sequencing methods for a panel of genes. No pathogenic mutation in AAAS, AIP, MEN1, CDKN1B, PRKAR1A, SDHB, GPR101, and GNAS genes was found in germline DNA. The somatic mutation c.601C>T/p.R201C in the GNAS gene was identified in DNA extracted from a tissue sample of the pituitary macroadenoma. Conclusions: We here describe the first case report to our knowledge of a patient with both acromegaly and achalasia. Association of acromegaly and soft muscle tissue hypertrophy may contribute to achalasia's development. If one of these diagnoses is determined, the other also should be considered along with increased risk of oesophageal and colorectal malignancy.

OBJECTIVES: To investigate the accrual proportion and patients' reasons for not participating in the PREOPANC trial on neoadjuvant chemoradiotherapy vs. immediate surgery in resectable and borderline resectable pancreatic cancer, and to compare these patients' outcomes with those of patients who had been randomized in the trial.SUMMARY OF BACKGROUND DATA: The external validity of multicenter randomized trials in cancer treatment has been criticized for suboptimal non-representative inclusion. In trials, it is unclear how outcomes compare between randomized and non-randomized patients.METHODS: At eight of 16 participant centers, this multicenter observational study identified validation patients, who had been eligible but not randomized during recruitment for the PREOPANC trial. We assessed the accrual proportion, investigated their most common reasons for not participating in the trial, and compared resection rates, radical (R0) resection rates and overall survival (OS) between the validation patients and PREOPANC patients, who had been randomized in the trial to immediate surgery.RESULTS: In total, 455 patients had been eligible during the recruitment period, 151 of whom (33%) had been randomized. Fifty-five percent of the 304 validation patients had refused to participate. Median OS in the validation group was 15.2 months, against 15.5 months in the PREOPANC group (p = 1.00). The respective resection rates (76% vs. 73%) and R0 resection rates (51% vs. 46%) did not differ between the groups.CONCLUSIONS: The PREOPANC trial included a reasonable percentage of 33% of eligible patients. In terms of the outcomes survival, resection rate, and R0 resection rate, this appeared to be a representative group.

Patients with primary sclerosing cholangitis (PSC) commonly undergo ileal pouch-anal anastomosis (IPAA) for medically-refractory ulcerative colitis (UC) or colorectal dysplasia. Pouchitis develops more frequently in patients with PSC, potentially leading to increased morbidity. We aimed to assess clinical characteristics and treatment outcomes for pouchitis in patients with PSC compared to a matched, non-PSC cohort.All patients with PSC who underwent IPAA and were diagnosed with pouchitis (PSC-pouchitis) were identified. A matched cohort composed of non-PSC patients who underwent IPAA for UC and subsequently developed pouchitis (UC-pouchitis) was developed. Relevant demographic, clinical, endoscopic, histologic, and treatment data were collected and compared between groups.Of those with PSC-pouchitis (n=182), 53.9% and 46.1% underwent IPAA for medically-refractory disease and dysplasia, respectively, compared to 88.7% and 11.3% in the UC-pouchitis group (P.001). Patients with PSC-pouchitis were more likely to develop chronic pouchitis (68.1% vs 34.1%; P.001), have moderate-to-severe pouch inflammation (54.9% vs 32.4%; P.001), and prepouch ileitis (34.1% vs 11.5%; P.001) compared to UC-pouchitis. Of those with PSC-pouchitis, 50.6% and 17.6% developed chronic antibiotic-dependent or antibiotic-refractory pouchitis, respectively, compared to 25.8% and 7.7% with UC-pouchitis. There was no difference in treatment response between the two groups with use of thiopurines, anti-tumor necrosis factor agents, and newer biologics.PSC-associated pouchitis presents with a unique clinical phenotype, characterized by increased risk of chronic pouchitis, moderate-to-severe pouch inflammation, prepouch ileitis, and less response to conventional antimicrobial therapy.

Background & Aims Coronavirus disease 2019 (COVID-19) pandemic lockdown and restrictions had significant disruption to patient care. We aimed to evaluate the impact of COVID-19 restrictions on hospitalizations of patients with alcoholic and nonalcoholic cirrhosis as well as alcoholic hepatitis (AH) in Alberta, Canada. Methods We used validated International Classification of Diseases (ICD-9 and ICD-10) coding algorithms to identify liver-related hospitalizations for nonalcoholic cirrhosis, alcoholic cirrhosis, and AH in the province of Alberta between March 2018 and September 2020. We used the provincial inpatient discharge and laboratory databases to identify our cohorts. We used elevated alanine aminotransferase or aspartate aminotransferase, elevated international normalized ratio, or bilirubin to identify AH patients. We compared COVID-19 restrictions (April–September 2020) with prior study periods. Joinpoint regression was used to evaluate the temporal trends among the 3 cohorts. Results We identified 2916 hospitalizations for nonalcoholic cirrhosis, 2318 hospitalizations for alcoholic cirrhosis, and 1408 AH hospitalizations during our study time. The in-hospital mortality rate was stable in relation to the pandemic for alcoholic cirrhosis and AH. However, nonalcoholic cirrhosis patients had lower in-hospital mortality rate after March 2020 (8.5% vs 11.5%; P = .033). There was a significant increase in average monthly admissions in the AH cohort (22.1/10,000 admissions during the pandemic vs 11.6/10,000 admissions before March 2020; P Conclusions Before and during COVID-19 monthly admission rates were stable for nonalcoholic and alcoholic cirrhosis; however, there was a significant increase in AH admissions. Because alcohol sales surged during the pandemic, future impact on alcoholic liver disease could be detrimental.

BACKGROUND AND AIMS: Conditions such as irritable bowel syndrome (IBS), functional dyspepsia, and functional constipation are among the prevalent gastrointestinal (GI) disorders classified as disorders of gut-brain interaction (DGBI), which can adversely affect the lives of sufferers. This study aimed to assess the degree and consequences of overlapping DGBI in a large population-based global scale. METHODS: Internet survey data from 54,127 adults (49.1% women) in 26 countries were analyzed by 4 GI anatomic regions (esophageal, gastroduodenal, bowel, and anorectal). The number of DGBI-affected GI regions was assessed, including associations with sex, age, disease severity, quality of life, psychosocial variables, and health care utilization. RESULTS: A total of 40.3% of surveyed individuals met Rome IV criteria for a DGBI. The percentages with 1-4 DGBI-affected GI regions were 68.3%, 22.3%, 7.1%, and 2.3%, respectively. The IBS symptom severity score increased significantly from 1 (207.6) to 4 (291.6) regions, as did non-GI symptom reporting (somatization), anxiety and depression, concerns and embarrassment about bowel function, doctor visits, medications, and abdominal surgeries (all P < .0001). Quality of life decreased with increasing number of DGBI regions (P < .0001). In a logistic mixed model, non-GI symptoms (odds ratio [OR], 1.09; 95% confidence interval [CI], 1.08-1.10), being very vs not concerned (OR, 2.55; 95% CI, 2.27-2.90), being very vs not embarrassed about bowel function (OR, 1.20; 95% CI, 1.08-1.33), and mean number of doctor visits (OR, 1.23; 95% CI, 1.115-1.32) were most strongly associated with number of DGBI regions. CONCLUSIONS: DGBI in multiple anatomic GI regions is associated with increased psychological comorbidity, health care utilization, and IBS severity. Physician awareness of overlap could improve quality of care, prevent unnecessary interventions, and yield more positive health outcomes. ispartof: CLINICAL GASTROENTEROLOGY AND HEPATOLOGY vol:20 issue:5 pages:E945-E956 ispartof: location:United States status: published

ObjectiveFollowing an episode of acute biliary pancreatitis, cholecystectomy is advised to prevent recurrent biliary events. There is limited evidence regarding the optimal timing and safety of cholecystectomy in patients with necrotising biliary pancreatitis.DesignA post hoc analysis of a multicentre prospective cohort. Patients with biliary pancreatitis and a CT severity score of three or more were included in 27 Dutch hospitals between 2005 and 2014. Primary outcome was the optimal timing of cholecystectomy in patients with necrotising biliary pancreatitis, defined as: the optimal point in time with the lowest risk of recurrent biliary events and the lowest risk of complications of cholecystectomy. Secondary outcomes were the number of recurrent biliary events, periprocedural complications of cholecystectomy and the protective value of endoscopic sphincterotomy for the recurrence of biliary events.ResultsOverall, 248 patients were included in the analysis. Cholecystectomy was performed in 191 patients (77%) at a median of 103 days (P25–P75: 46–222) after discharge. Infected necrosis after cholecystectomy occurred in four (2%) patients with persistent peripancreatic collections. Before cholecystectomy, 66 patients (27%) developed biliary events. The risk of overall recurrent biliary events prior to cholecystectomy was significantly lower before 10 weeks after discharge (risk ratio 0.49 (95% CI 0.27 to 0.90); p=0.02). The risk of recurrent pancreatitis before cholecystectomy was significantly lower before 8 weeks after discharge (risk ratio 0.14 (95% CI 0.02 to 1.0); p=0.02). The complication rate of cholecystectomy did not decrease over time. Endoscopic sphincterotomy did not reduce the risk of recurrent biliary events (OR 1.40 (95% CI 0.74 to 2.83)).ConclusionThe optimal timing of cholecystectomy after necrotising biliary pancreatitis, in the absence of peripancreatic collections, is within 8 weeks after discharge.

Background/Aims: The preventive role of hydroxychloroquine (HCQ) on coronavirus disease 2019 (COVID-19) remains unclear. The aim of this study was to examine the effects of HCQ and other immunosuppressive drugs on the incidence of COVID-19.Methods: The data were collected from the South Korea National Health Insurance Sharing-COVID-19 database. All individuals who underwent nasopharyngeal and oropharyngeal swab tests for COVID-19 from January 2020 to May 2020 are included. The association between COVID-19 risk and HCQ use was examined in a propensity score-matched population. Factors associated with COVID-19 were identified using multiple logistic regression analysis.Results: Total 8,070 patients with COVID-19 and 121,050 negative controls were included from the database. Among all participants, 381 were HCQ users. In a propensity score-matched population, the incidence of COVID-19 was 7.1% in HCQ users and 6.8% in non-users. The odds ratio (OR) for HCQ use was 1.05 with a 95% confidence interval (CI) of 0.58 to 1.89. Among the subpopulation of patients with rheumatoid arthritis (RA), 33 were diagnosed with COVID-19 and 478 were not. Use of HCQ, glucocorticoids, or other immunosuppressive drugs was not associated with COVID-19 risk, whereas abatacept use was. Chronic lung disease was an independent risk factor for COVID-19 diagnosis in patients with RA (adjusted OR, 6.07; 95% CI, 1.10 to 33.59).Conclusions: The risk of COVID-19 did not differ between HCQ users and non-users. Glucocorticoids, conventional disease-modifying antirheumatic drugs (DMARDs), and biological DMARDs other than abatacept did not increase the risk of COVID-19.

50% to 80% Crohn's disease (CD) and 10% to 30% ulcerative colitis (UC) patients require surgery over their lifetime. Biologic therapies may alter this natural history, but data on the effect of biologics on surgery rates in this patient population are mixed. We sought to investigate the influence of biologics on surgery prevalence in CD and UC.We used a commercial database (Explorys Inc, Cleveland, OH), which includes electronic health record data from 26 major integrated US healthcare systems. We identified all patients who were diagnosed with CD or UC that were treated with any biologics between 2015 and 2020. The primary outcome was to examine the association between biologics therapy and the prevalence of bowel resection. Also, we identified the factors associated with surgery in IBD patients on biologics.Of 32,904,480 patients in the database, we identified 140,540 patients with CD and 115,260 patients with UC, of whom 25,840 (18%) and 9,050 (7.8%) patients received biologics, respectively. The prevalence of intestinal resection was significantly lower in biologics-treated CD patients (9.3%) compared to those who did not receive biologics (12.1%) (p.001). Similarly, biologic-treated UC patients were significantly less likely to undergo colectomy (7.3%) compared to UC patients who did not receive biologic therapy (11.0%) (p.001). Tobacco use, Clostridium difficile infection, and perianal disease were associated with intestinal resection in CD. Colon neoplasm and Clostridium difficile infection were associated with colectomy in UC.In this study of a large healthcare administrative database, inflammatory bowel disease (IBD) patients treated with biologics were significantly less likely to undergo bowel resection when compared to those who never received biologics. This data suggests that biologics may impact surgical rates in IBD.