Massive infected pancreatic necrosis in an 8-year-old: Endoscopic management.

Pancreatitis is a condition much more commonly found in adults, but when diagnosed in the pediatric population, is often due to medications, congenital pathology, and critical illness. This patient had previously undergone treatment with 6-mercaptopurine and presented with pancreatitis that eventually worsened to a walled-off necrotic collection with paracolic extensions reaching the pelvis. Given clinical worsening with development of shock, procedural options for source control were weighed with gastroenterology, pediatric surgery, and interventional radiology, before pancreatic necrosectomy was determined to be the treatment of choice, given the adjacency of the collection to the stomach. A total of three separate endoscopic pancreatic necrosectomy procedures were performed and the patient s clinical status improved greatly, with vast improvement later seen on outpatient imaging. This successful treatment course argues for the efficacy of pancreatic necrosectomy even in very large walled off collections, and most importantly, lead to a positive outcome in this young patient.

Uretero-Jejunal Fistula: A Rare Cause of Acute Pyelonephritis.

Penetrating peptic ulcers often lead to severe complications. The development of uretero-enteric fistulas is rare and can be challenging to diagnose and treat. Here, we present the case of a 41-year-old patient who previously underwent gastrojejunostomy for superior mesenteric artery syndrome and developed a peptic jejunal ulcer, leading to a uretero-jejunal fistula and finally causing acute pyelonephritis. The patient was managed with a multidisciplinary approach including medical therapy and endoscopic and radiologic interventions.

Ileosigmoid Anastomotic Perforation Three Weeks After Placement of Lumen-Apposing Metal Stent (LAMS).

Benign anastomotic intestinal strictures are difficult to manage as there may be limited response to dilation. Fully covered self-expanding metal stents have been utilized in small case series; however, stent suturing is required due to the high risk of stent migration. Lumen-apposing metal stents (LAMS) are fully covered and have a novel dumbbell shape that prevents stent migration. Initial reports identify low migration rates and good clinical success rates. This is the first report of perforation following treatment of an ileosigmoid stricture in a 52-year-old female, three weeks after LAMS placement.

Sequential Crohn's Ileitis, Ileosigmoidal Fistula, Segmental Sigmoid Polyposis, and Sigmoid Stricture: The Natural History.

BACKGROUND: We have previously recognized segmental sigmoid polyps as an indicator of a fistula from Crohn's ileitis to the sigmoid or the proximal rectum. In the course of this study, we realized that many patients with this fistula had no sigmoid polyps, but the sigmoid was the site of marked inflammation and early or late stricture formation. Furthermore, in some patients with a stricture, the fistula was not recognized until the surgeon (or the pathologist) dissected an inflammatory peri-ileal and/or a perisigmoidal mass.In this study, we have sought to clarify the sequence of events by focusing on the segmental inflammation and the stricturing of the sigmoid so that its significance can be recognized as a local complication of the ileitis and the progression of its severity as opposed to arising sui generis. MATERIALS AND METHODS: From our database of >3000 patients with inflammatory bowel disease at Lenox Hill Hospital, we identified 45 patients with Crohn's ileitis and ileosigmoid fistula (ISF): 24 had segmental sigmoid polyps and 18 had segmental inflammatory sigmoid strictures. The fistula was first seen by imaging in 36 patients, but not until resection by the surgeon or dissection by the pathologist in 7 patients. RESULTS: The method of diagnosis for the initial recognition of the ISF and the sigmoid stricture is presented in Table 1. In 36 of the 45 cases, the ISF was recognized by radiologic imaging. In total, 31 of the 36 cases required surgical intervention, not because of the fistula, but because of small-bowel obstruction due to the ileitis. In 7 of the 31 (22%) cases, the fistula was recognized only by dissection of the inflammatory ileosigmoid mass by the surgeon or examination of the surgical specimen by the pathologist. The sequence of events from the originating ileitis to the ISF to the segmental sigmoid polyposis and stricture, with the resulting sigmoid obstruction, is shown in Figures 1A-E. CONCLUSIONS: We emphasize the natural history of the ISF so that its recognition will lead to earlier medical management of the originating ileitis. Furthermore, it adds evidence of the recognition that the causative agent of Crohn's disease is carried by the fecal stream.

Segmental sigmoid polyposis as a colonoscopic indicator of an ileosigmoid fistula in Crohn's ileitis.

BACKGROUND AND AIMS: Ileosigmoid fistulas (ISFs) are frequently undiagnosed prior to surgery. This study was designed to describe a polyp or cluster of polyps limited to the sigmoid colon as a marker of ISF in patients with ileitis. This novel finding will increase a gastroenterologist's opportunity to detect them preoperatively and their prognostic implication of worsening ileitis. METHODS: The medical records of patients with Crohn's disease and ISF were reviewed to determine whether colonoscopy had revealed polyposis limited to the sigmoid colon and its frequency. RESULTS: Thirty-seven patients with Crohn's ileitis complicated by ISF were identified from our database. Twenty had one or more sigmoid polyps without polyps elsewhere in the colon suggesting the site of fistula exit. Fifteen of the patients had ISF and five had ileorectal fistula (IRF). The fistula was detected by various means, including colonoscopy, sigmoidoscopy, small bowel X-ray series, barium enema, computed tomography, and magnetic resonance enterography. The ISF was generally diagnosed prior to the recognition and significance of the segmental polyps. These polyps were inflammatory or hyperplastic on pathologic review. CONCLUSION: Most ISFs and IRFs are now found preoperatively by imaging and some are incidental surgical findings. The segmental sigmoid polyps that we describe should help the gastroenterologist to be suspicious of ISF. The polyps are a surrogate marker for the progression of the fistula and the underlying ileitis as they tend to appear after the fistula has matured and lead to increased intensity of medical therapy well before surgical intervention is required.

Telaprevir-induced DRESS.

Telaprevir, a protease inhibitor, was recently approved for management of Chronic Hepatits C (CHC) due to HCV genotype 1. Various RCTs have demonstarted increased incidence of cutaneous adverse effects with use of Telaprevir. Herein, we report two cases of drug rash with eosinophilia and systemic symptoms (DRESS) secondary to Telaprevir use.

Toll-like receptors and liver disease.

Toll-like receptors (TLRs) are pattern recognition receptors that play an important role in host defence by recognizing pathogen-associated molecular patterns (PAMP). Recent studies indicate that TLR signalling plays an important role in progression of chronic liver diseases. Ongoing clinical trials suggest that therapeutic manipulation of TLR pathways may offer novel means of reversing chronic liver diseases. Upon activation by their respective ligands, TLRs initiate an intracellular pro-inflammatory/anti-inflammatory signalling cascade via recruitment of various adaptor proteins. TLR associated signalling pathways are tightly regulated to keep a check on inappropriate production of pro-inflammatory cytokines and interferons thereby preventing various autoimmune and inflammatory processes. Herein, we review the current state of knowledge of hepatic distribution, signalling pathways and therapeutic modulation of TLRs in chronic liver diseases.

Neurofibromatosis type 1-associated hypertension secondary to coarctation of the thoracic aorta.

Neurofibromatosis type 1 (NF-1), also known as von Recklinghausen's disease, is an autosomal dominant genetic disorder. NF-I vasculopathy has been used to describe various vascular malformations associated with NF-1. Secondary hypertension related to NF-1 vasculopathy has been reported because of renal artery stenosis, coarctation of the abdominal aorta and other vascular lesions; however, coarctation of the thoracic aorta has seldom been reported. We report the first case, to our knowledge, of isolated coarctation of thoracic aorta in a pregnant female with NF-1. Healthcare providers caring for patients with NF-1 should be aware of associated vascular complications.

Catalase and estradiol inhibit mitochondrial protein S-glutathionylation.

Regulation and downstream effects of mitochondrial protein S-glutathionylation in response to oxidative stress are poorly understood. The study aim was to determine whether anti-oxidants such as catalase and estradiol alter mitochondrial protein S-glutathionylation and in turn affect apoptosis following ultraviolet B (UV-B) light irradiation. HeLa cells were transduced with increasing amounts of adenovirus encoding catalase (Ad-Cat) and ß-galactosidase (Ad-Lac Z) or pre-incubated with estradiol before induction of apoptosis by UV-B light exposure. Inhibition of mitochondrial protein S-glutathionylation was assessed using autoantibodies specific for the non-S-glutathionylated form of PDC-E2. The percentage of apoptotic cells following UV-B irradiation were not significantly different between mock cells (cells with no virus infection) and Ad-Cat and Ad-Lac Z infected cells at all viral doses (all p > 0.050). Autoantibody staining of non-S-glutathionylated PDC-E2 in apoptotic cells was three times greater in only Ad-Cat infected cells compared to only Ad-Lac Z infected cells (81.3 ± 16.7 vs 26 ± 7.2 %, respectively, p = 0.030). Similarly estradiol treatment (33 and 100 nM) also significantly increased PDC-E2 staining in apoptotic cells compared to non-treated cells (both p < 0.010). The percentage of apoptotic cells was not significantly different with any of the estradiol concentrations (all p > 0.100). The observed procaspase 12 cleavage following UV-B irradiation suggests that a mitochondrial-independent apoptotic pathway was activated. In conclusion, following an apoptotic stimulus, estradiol may inhibit mitochondrial protein S-glutathionylation without inhibiting apoptosis. This effect may play a role in ninefold greater prevalence of autoantibodies against PDC-E2 in women with primary biliary cirrhosis.