Extensive Small Bowel Resection Research Articles

Totally thoracoscopic atrial fibrillation surgery following massive small bowel resection due to superior mesenteric artery embolization: report of two cases

BackgroundThromboembolic occlusion of the superior mesenteric artery (SMA) is a grave complication in individuals diagnosed with atrial fibrillation (AF). This condition often necessitates extensive bowel resection, culminating in short bowel syndrome, which presents challenges for anticoagulant administration and/or antiarrhythmic therapy.Case presentationPresented here are findings of two patients, aged 78 and 72 years, respectively, who underwent comprehensive thoracoscopic AF surgery subsequent to extensive small bowel resection following SMA embolization. In each, onset of AF precipitated an embolic event, while the concurrent presence of short bowel syndrome complicated anticoagulation management. Total thoracoscopic AF surgery, comprised stapler-closure of the left atrial appendage (LAA) and bilateral epicardial clamp-isolation of the pulmonary veins, an operative modality aimed at addressing AF rhythm control and mitigating embolic events such as cerebral infarction, led to favorable outcomes in both cases. Additionally, computed tomography (CT) conducted one month post-surgery revealed the absence of residual tissue in the LAA, with the left atrium demonstrating a well-rounded, spherical shape. At the time of writing, the patients have remained asymptomatic following surgery regarding thromboembolic and arrhythmic manifestations for 29 and 10 months, respectively, notwithstanding the absence of anticoagulant or antiarrhythmic pharmacotherapy. Additionally, electrocardiographic surveillance has revealed persistent sinus rhythm.ConclusionsThe present findings underscore the feasibility and efficacy of a total thoracoscopic AF surgery procedure for patients presented with short bowel syndrome complicating SMA embolization, thus warranting consideration for its broader clinical application.

Very low risk of short bowel after Roux-en-Y gastric bypass – a large nationwide Swedish cohort study

BackgroundRoux-en-Y gastric bypass (RYGB) is one of the most common bariatric procedures. Internal herniation may lead to small bowel ischemia requiring small bowel resection, resulting in short bowel syndrome. ObjectiveTo determine the incidence of extensive small bowel resection in patients operated with RYGB. We also aimed to look for early clinical warning signs among patients requiring extensive small bowel resection. SettingCohort from national quality registers. MethodsAll patients having undergone RYGB between January 2007 to June 2019 were analyzed in the Scandinavian Obesity Surgery Registry (SOReg). We identified patients with small bowel obstruction (SBO) for whom small bowel resection was necessary. Additionally, we assessed clinical signs in these patients. ResultsThe study included 57,255 patients having undergone RYGB. Closure of the mesenteric openings was performed in 78%. Surgery for SBO was required in 3659 (6%) of patients, and small bowel resection in 188 (.3%). Extensive small bowel resection, resulting in less than 1.5 meters of remaining small bowel, was required in 7 patients (.01%). All patients with extensive small bowel resection presented with abdominal pain and had confirmed internal herniation as the cause of the small bowel resection, and 2 of 7 patients died. Closure of mesenteric defects was not associated with a reduction in overall small bowel resection rates (P = .89) ConclusionSurgery for SBO after RYGB was common (6%). The risk of extensive small bowel resection leading to short bowel was low (.01%). Patients with abdominal pain after RYGB should be assessed for internal hernia, as it can be devastating.

Potential for intestinal transplantation after acute mesenteric ischemia in patients aged less than 70 years: A population-based study.

Acute mesenteric ischemia (AMI) has a high mortality rate due to the development of bowel necrosis. Patients are often ruled outside active care if a large proportion of small bowel is necrotic. With the development of treatment for short bowel syndrome (SBS) and intestinal transplantation methods, long-term survival is possible even after extensive small bowel resections. This study aims to assess the incidence of SBS and potentially suitable candidates for intestinal transplantation among patients treated for AMI. This population-based retrospective study comprised patients aged less than 70 years and diagnosed with AMI between January 2006 and October 2020 in Helsinki and Uusimaa health care district, Finland. Altogether, AMI was diagnosed in 711 patients, of whom 133 (19%) were aged below 70. An intervention was performed in 110 (83%) patients. Of these 133 patients, 16 (12%) were ruled outside active treatment due to extensive small bowel necrosis at exploratory laparotomy, of whom 6 (5%) were potentially suitable for intestinal transplantation. Two patients were considered as potential candidates for intestinal transplantation at bowel resection but died of AMI. Nine (7%) patients needed parenteral nutrition after resection, and two of them (2%) developed SBS. Only one patient needed long-term parenteral nutrition after hospital discharge. This patient remained dependent on parenteral nutrition but died before evaluation of intestinal transplantation could be carried out while the other patient was able to return to enteral nutrition. A small number of patients with AMI below 70 years of age are potentially eligible for intestinal transplantation.

Vessel-Sparing Lymphadenectomy Should Be Performed in Small Intestine Neuroendocrine Neoplasms.

Simple SummaryPrimary tumor resection with lymphadenectomy in small intestine neuroendocrine neoplasms potentially requires extensive small bowel resections due to central lymph node metastases and mesenteric fibrosis. Retrograde vessel-sparing lymphadenectomy (VS-LA) might be a sufficient method for avoiding local recurrence and for sparing the small bowel at the same time. We retrospectively analyzed clinical, surgical and pathological data of 50 patients with SI-NENs who exclusively underwent small bowel resections; half of them received conventional lymphadenectomy and 25 underwent VS-LA. VS-LA resulted in shorter resected bowel segments (median 40 cm vs. 65 cm, p = 0.007) with similar rates of local R0 resections (72% vs. 84%) and number of resected lymph nodes (median 13 vs. 13). Postoperative complications occurred significantly less in the vessel-sparing group. VS-LA should be the preferred surgical method in small bowel resections for SI-NENs.Introduction: The goal of primary tumor resection with lymphadenectomy (PTR) in small intestine neuroendocrine neoplasms (SI-NENs) is to avoid local recurrence while sparing as much of the small bowel as possible, even in the case of extensive mesenteric fibrosis. The results of PTR with retrograde vessel-sparing lymphadenectomy (VS-LA) were compared to those of conventional lymphadenectomy (Con-LA). Methods: Prospectively collected clinical, surgical and pathological data of consecutive patients with SI-NENs who underwent small bowel resections were retrospectively analyzed regarding the resection technique performed. Results: In a 7-year period, 50 of 102 patients with SI-NENs had only small bowel resections; of those, 25 were VS-LA and 25 were Con-LA. Patients with VS-LA had tendentially more advanced diseases with slightly higher rates of abdominal pain, mesenteric shrinkage and more level III lymph node involvement compared to patients with Con-LA. VS-LA, however, resulted in shorter resected bowel segments (median 40 cm vs. 65 cm, p = 0.007) with similar rates of local R0 resections (72% vs. 84%) and resected lymph nodes (median 13 vs. 13). Postoperative clinically relevant complications occurred in 1 of 25 (4%) in the VS-LA and in 7 of 25 (28%) patients in the Con-LA group (p = 0.02). Three months after surgery, 1 of 25 (4%) patients of the VS-LA group and 10 of 25 (40%) patients in the Con-LA group (p = 0.002) complained about abdominal pain. One of eight patients in the VS-LA group and two of thirteen patients in the Con-LA group who had completely resected stage III disease complained about diarrhea (p = 0.31). Conclusion: VS-LA seems to be oncologically safe and should be considered in small bowel resections for SI-NENs.

Superior mesenteric arteriovenous fistula embolization with a vascular plug for upper gastrointestinal hemorrhage: a case report

BackgroundSuperior mesenteric arteriovenous fistula is a rare vascular anomaly often presenting with sequelae of portal hypertension, heart failure, or mesenteric ischemia. This report describes a patient with a previously unidentified superior mesenteric arteriovenous fistula who presented with variceal bleeding, thought to be the leading cause of mortality associated with this condition. Although this patient was initially referred for a transjugular intrahepatic portosystemic shunt procedure, following a thorough review of her clinical history and imaging, she instead underwent embolization of the arteriovenous fistula likely responsible for her symptoms.Case PresentationA 75-year-old woman with a past surgical history of extensive small bowel resection presented with active variceal bleeding requiring transfusions. She was referred to vascular and interventional radiology for transjugular intrahepatic portosystemic shunt procedure; however, her clinical presentation was inconsistent with cirrhosis. This prompted a further review of her imaging, which identified a superior mesenteric arteriovenous fistula as the probable etiology of her varices. This fistula was subsequently embolized with a vascular plug and follow-up upper endoscopy at 1-month demonstrated complete resolution of her varices.ConclusionsThis report highlights a potential etiology of variceal bleeding in the acutely ill patient. Through a thorough consultation, the patient described here was able to avoid a procedure with the potential to cause catastrophic consequences, and instead receive the appropriate treatment for an uncommon condition.Level of EvidenceLevel 4, Case Report.

Surgical Treatment of Short Bowel Syndrome-The Past, the Present and the Future, a Descriptive Review of the Literature.

Short bowel syndrome (SBS) is a devastating disorder with both short- and long-term implications for patients. Unfortunately, the prevalence of SBS has doubled over the past 40 years. Broadly speaking, the etiology of SBS can be categorized as congenital or secondary, the latter typically due to extensive small bowel resection following diseases of the small intestine, e.g., necrotizing enterocolitis, Hirschsprung’s disease or intestinal atresia. As of yet, no cure exists, thus, conservative treatment, primarily parenteral nutrition (PN), is the first-line therapy. In some cases, weaning from PN is not possible and operative therapy is required. The invention of the longitudinal intestinal lengthening and tailoring (LILT or Bianchi) procedure in 1980 was a major step forward in patient care and spawned further techniques that continue to improve lives for patients with severe SBS (e.g., double barrel enteroplasty, serial transverse enteroplasty, etc.). With this review, we aim to provide an overview of the clinical implications of SBS, common conservative therapies and the development of operative techniques over the past six decades. We also provide a short outlook on the future of operative techniques, specifically with respect to regenerative medicine.

Dietary Management of Chronic Kidney Disease and Secondary Hyperoxaluria in Patients with Short Bowel Syndrome and Type 3 Intestinal Failure.

Short gut syndrome can lead to type 3 intestinal failure, and nutrition and hydration can only be achieved with parenteral nutrition (PN). While this is a lifesaving intervention, it carries short- and long-term complications leading to complex comorbidities, including chronic kidney disease. Through a patient with devastating inflammatory bowel disease’s journey, this review article illustrates the effect of short gut and PN on kidney function, focusing on secondary hyperoxaluria and acute precipitants of glomerular filtration. In extensive small bowel resections colon in continuity promotes fluid reabsorption and hydration but predisposes to hyperoxaluria and stone disease through the impaired gut permeability and fat absorption. It is fundamental, therefore, for dietary intervention to maintain nutrition and prevent clinical deterioration (i.e., sarcopenia) but also to limit the progression of renal stone disease. Adaptation of both enteral and parenteral nutrition needs to be individualised, keeping in consideration not only patient comorbidities (short gut and jejunostomy, cirrhosis secondary to PN) but also patients’ wishes and lifestyle. A balanced multidisciplinary team (renal physician, gastroenterologist, dietician, clinical biochemist, pharmacist, etc.) plays a core role in managing complex patients, such as the one described in this review, to improve care and overall outcomes.

Emerging of Fatal Colitis with Multidrug-Resistant Candida glabrata after Small Bowel Transplantation

Background Small bowel transplantation is a potential option for patients with intestinal-failure, and the incidences of infections caused by Candida species that are more resistant to antifungal drugs are increasing in these patients. In this manuscript, we reported a case of fatal colitis after small bowel transplantation induces by multidrug-resistant (MDR) Candida glabrata. Case Presentation. A 52-year-old man has undergone an extensive small bowel resection with the length of the remaining bowel which was less than 40 cm who became a candidate for transplantation. Four months after transplantation, the patient experienced severe bloody diarrhea with abdominal distension. Ileoscopy and colonoscopy did not show neither pathological change and rejection nor cytomegalovirus (CMV) infection posttransplantation. Abdomen computed tomography showed diffuse moderate small bowel wall thickening. After detection of budding yeast in the stool samples, stool culture was positive for Candida, DNA was extracted, and ITS1-5.8s-ITS2 region of the fungal agent was amplified. Sequencing analysis of PCR and antifungal susceptibility testing revealed that this isolate was multidrug-resistant C. glabrata. Besides, there was no evidence for other pathogens known to cause infection in various laboratory tests. Immediate antifungal treatments with caspofungin remained unsuccessful, and on the eighteenth day of admission, the patient expires with septic shock. Conclusion These findings highlight the challenging management of candidiasis in patients with small bowel transplantation. Infectious diseases due to MDR organisms have emerged as a vital clinical problem in this patient population.

HF02-07 IN TURP-ETUITY? HISTORICAL TREATMENTS FOR BPH AND CONSIDERATION OF THEIR MODERN-DAY ANALOGUES

You have accessJournal of UrologyHistory of Urology Forum II (HF02)1 Sep 2021HF02-07 IN TURP-ETUITY? HISTORICAL TREATMENTS FOR BPH AND CONSIDERATION OF THEIR MODERN-DAY ANALOGUES Dylan Buller, and Keith O'Brien Dylan BullerDylan Buller More articles by this author , and Keith O'BrienKeith O'Brien More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000001993.07AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: The enlarged prostate has long been a target of surgical intervention. While procedures such as simple prostatectomy and transurethral resection of the prostate have found durability over the past hundred years, attempts at novel approaches to treat benign prostatic hyperplasia (BPH) have been trialed for centuries. Similarities can be found between abandoned historical methods and modern-day interventions. METHODS: Primary and secondary sources of both historical and modern-day procedures for BPH surgery were reviewed. RESULTS: Obstructive urinary symptoms in the aging male have been recognized for millennia. In 1575, Paré’s transurethral punch device was employed to remove obstructing urethral tissue. Remarkably, this was 75 years before Jean Riolan the Younger identified the prostate as the culprit in urinary obstruction. Although largely forgotten over the next 300 years, the prostatic punch re-emerged at the hands of famed urologic pioneer Hugh Hampton Young in 1909. In the early 1800s, Syng devised a sound that included an inflatable bag at its tip; upon insertion, the bag was inflated in an attempt to dilate the prostatic urethra. This procedure was abandoned due to poor patient tolerability and lack of effect–that is, until the introduction and subsequent abandonment of ‘transurethral balloon dilation of the prostate’ at the end of the 20th century. The ‘transurethral implantable nitinol device,’ a newly introduced procedure, is predicated on similar foundational concepts. In the 1890s, Bier and Meyer reported on bilateral internal iliac artery ligation as a method for shrinking the enlarged prostate. Alas, Meyer’s first attempt necessitated extensive small bowel resection, and his patient ultimately died. Indeed, advocates of prostate artery embolization, which emerged at the turn of the 21st century, may feel heartened by the minimally invasive access employed by this modern analogue. Fortunately, not all historical techniques have been revisited. White introduced orchiectomy for BPH in 1893, and in 1896, Cabot reported that 84% of castrated BPH patients experienced symptomatic improvement. Literature was also published on the successful treatment of BPH with vasectomy; this, too, was abandoned in the early 1900s. CONCLUSIONS: Interventions to relieve urinary obstruction caused by the enlarged prostate have made great advancements in efficacy and safety. Nonetheless, many modern techniques have foundations in historical practices. Early reports of success do not necessarily confer durability of effects or longstanding usage in practice. Source of Funding: None © 2021 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 206Issue Supplement 3September 2021Page: e239-e239 Advertisement Copyright & Permissions© 2021 by American Urological Association Education and Research, Inc.MetricsAuthor Information Dylan Buller More articles by this author Keith O'Brien More articles by this author Expand All Advertisement Loading ...

A rare case of Polyarteritis Nodosa associated with autoimmune hepatitis: a case report

BackgroundPolyarteritis nodosa is a type of vasculitis affecting medium- and small-sized arteries that has been associated with hepatitis B but does not have an established relationship with autoimmune hepatitis. Here we report the case of an adult patient with autoimmune hepatitis who, shortly after diagnosis, developed life-threatening polyarteritis nodosa.Case presentationA 45-year-old woman was diagnosed with autoimmune hepatitis after initially presenting with a two-month history of fatigue, nausea, and anorexia and a three-week history of scleral icterus. Her liver biopsy showed mild portal fibrosis and her liver chemistries improved with prednisone and azathioprine. Three months later, she presented to the emergency department with fever, bilateral ankle pain, rash, oral ulcers, and poor vision. Physical examination was notable for erythema nodosum, anterior uveitis, retinal vasculitis, and frosted branch angiitis (frosted branch angiitis (a widespread florid translucent perivascular exudate). She subsequently developed repeated episodes of ischemic acute bowel necrosis that required multiple surgeries and extensive small bowel resections. Surgical pathology of the small bowel resection revealed ischemic necrosis, medium and small vessel vasculitis with microvascular thrombi consistent with polyarteritis nodosa. Azathioprine was discontinued and she was treated with pulse steroids followed by a prednisone taper, cyclophosphamide, and intravenous immune globulin with overall improvement in her symptomatology. Since her hospitalization, she has been maintained on low-dose prednisone and mycophenolate mofetil.ConclusionsIn patients with recent diagnosis of autoimmune hepatitis, there should be a modest suspicion for concomitant polyarteritis nodosa if symptoms and signs of multisystem vasculitis develop.

The Roles of Lactobacillus Acidophilus and Pectin in Preventing Postoperative Sepsis and Intestinal Adaptation in a Rat Model of Short Bowel Syndrome.

One of the primary causes of morbidity and mortality in those with short bowel syndrome (SBS) is sepsis, caused by bacterial translocation (BT). Since synbiotics can cease gut-related bacterial overgrowth, they may serve as a supportive dietary supplement-based strategy after gastrointestinal surgery. This study was conducted to determine the effects of Lactobacillus acidophilus and pectin on BT and gut adaptation after extensive small bowel resection in the rat. Forty rats were distributed in four groups. Group A suffered laparotomy, group B suffered gut transection and reanastomosis, SBS rats (group C) suffered 75% small gut resection, and finally, Group D suffered gut resection and treated with a synbiotic cocktail from day 7 before the surgery to day 14 after it. Intestinal structural changes and BT to mesenteric lymph nodes, liver, portal blood, and peripheral blood were detected on day 15 post-surgery. Treatment with a synbiotic cocktail led to a considerable reduction in bacterial translocation to liver and portal vein (degree II) compared with SBS untreated rats. Also, synbiotic administration significantly increased jejunum and ileum villus height and crypt depth, ileum villus width, and percentage of goblet cells in jejunum and ileum compared with SBS rats. In the rat model of short bowel syndrome, L. acidophilus, and pectin, as a potential synbiotic compound, could decrease the BT from the gut and improve the bowel adaptation.

Non-occlusive Mesenteric Ischaemia and Acute Kidney Injury: A Case Report of Severe Idiopathic Systemic Capillary Leak Syndrome.

Systemic capillary leak syndrome is a rare, underdiagnosed and life-threatening disease characterized by periodic episodes of hypovolaemic shock due to leakage of plasma from the intravascular to the extravascular space. It is associated with haemoconcentration, hypoalbuminaemia and generalized oedema.We report the case of a patient with a history of emergent extensive small and large bowel resection and several episodes of hypovolaemic shock with acute renal injury, who presented with abdominal pain, headache and generalized oedema. Severe systemic capillary leak syndrome was diagnosed after a complex diagnostic approach. This case report describes the acute and prophylactic treatment administered to the patient and the 4-year follow-up. We highlight the importance of timely recognition and prompt treatment, as well as the need for new investigations to prevent the serious and unusual complications seen in this case.LEARNING POINTSIdiopathic systemic capillary leak syndrome (ISCLS) should be suspected in the presence of the triad of hypotension, haemoconcentration and hypoalbuminaemia; the diagnostic work-up is challenging and requires exclusion of several causes of hypotension and shock of uncertain aetiology.Acute mesenteric ischaemia leading to extensive and emergent bowel resection is an irreversible but atypical complication of ISCLS; other complications include myocardial oedema and deep vein thrombosis.ISCLS is characterized by three phases; supportive as well as prophylactic treatment adapted to each phase is crucial for prognosis and to avoid end-organ damage.

Villus Growth, Increased Intestinal Epithelial Sodium Selectivity, and Hyperaldosteronism Are Mechanisms of Adaptation in a Murine Model of Short Bowel Syndrome

BackgroundShort bowel syndrome results from extensive small bowel resection and induces adaptation of the remaining intestine. Ileocecal resection (ICR) is the most frequent situation in humans. Villus hypertrophy is one hallmark of mucosal adaptation, but the functional mechanisms of mucosal adaptation are incompletely understood.AimsThe aim of the study was to characterize a clinically relevant model of short bowel syndrome but not intestinal failure in mice and to identify outcome predictors and mechanisms of adaptation.MethodsMale C57BL6/J mice underwent 40% ICR and were followed for 7 or 14 days. Small bowel transection served as control. All mice underwent autopsy. Survival, body weight, wellness score, stool water content, plasma aldosterone concentrations, and paracellular permeability were recorded.ResultsUnlike controls, resected mice developed significant diarrhea with increased stool water. This was accompanied by sustained weight loss throughout follow-up. Villus length increased but did not correlate positively with adaptation. Plasma aldosterone concentrations correlated inversely with body weight at day 14. After ICR, intestinal epithelial (i.e., tight junctional) sodium permeability was increased.Conclusions40% ICR results in moderate to severe short bowel syndrome. Successful adaptation to the short bowel situation involves villus elongation but does not correlate with the degree of villus elongation alone. In addition, increased intestinal epithelial sodium permeability facilitates sodium-coupled solute transport. Hyperaldosteronism correlates with the severity of weight loss, indicates volume depletion, and counterregulates water loss.

Teduglutide for the treatment of short bowel syndrome – a safety evaluation

ABSTRACTIntroduction: Teduglutide is an analog of glucagon-like peptide 2 (GLP-2) which is approved for the treatment of patients with short bowel syndrome (SBS) who are dependent on parenteral support.Areas covered: Short bowel syndrome is a rare condition that can result from extensive resection of small bowel, congenital abnormalities, or inflammatory conditions that leads to poor nutrient processing capacity of the intestine. In this review, the safety reported in controlled clinical trials and real-world experience with teduglutide are presented in depth.Expert opinion: Trials and limited real-world experience demonstrated that teduglutide could decrease parenteral support requirements with the potential of achieving independence from parenteral support in some patients. This is an important treatment option for SBS patients given the well-established risks associated with parenteral support. The available data, albeit limited due to the small number of patients in the so-far performed studies, suggest that teduglutide appears to be safe to use in patients with intestinal failure who are dependent on parenteral support. However, additional long-term safety data are needed.

Renal lithiasis and inflammatory bowel diseases, an update on pediatric population.

Background and aim of the work: Historical studies have demonstrated that the prevalence of symptomatic nephrolithiasis is higher in patients with inflammatory bowel disease (IBD), compared to general population. The aim of the review was to analyze literature data in order to identify the main risk conditions described in literature and the proposed treatment. Methods: A research on the databases PubMed, Medline, Embase and Google Scholar was performed by using the keywords “renal calculi/lithiasis/stones” and “inflammatory bowel diseases”. A research on textbooks of reference for Pediatric Nephrology was also performed, with focus on secondary forms of nephrolithiasis. Results: Historical studies have demonstrated that the prevalence of symptomatic nephrolithiasis is higher in patients with inflammatory bowel disease (IBD), compared to general population, typically in patients who underwent extensive small bowel resection or in those with persistent severe small bowel inflammation. In IBD, kidney stones may arise from chronic inflammation, changes in intestinal absorption due to inflammation, surgery or intestinal malabsorption. Kidney stones are more closely associated with Crohn’s Disease (CD) than Ulcerative Colitis (UC) in adult patients for multiple reasons: mainly for malabsorption, but in UC intestinal resection may be an additional risk. Nephrolithiasis is often under-diagnosed and might be a rare but noticeable extra-intestinal presentation of pediatric IBD. Secondary enteric hyperoxaluria the main risk factor of UL in IBD, this has been mainly studied in CD, whether in UC has not been completely explained. In the long course of CD recurrent urolithiasis and calcium-oxalate deposition may cause severe chronic interstitial nephritis and, as a consequence, chronic kidney disease. ESRD and systemic oxalosis often develop early, especially in those patients with multiple bowel resections. Even if we consider that many additional factors are present in IBD as hypomagnesuria, acidosis, hypocitraturia, and others, the secondary hyperoxaluria seems to finally have a central role. Some medications as parenteral vitamin D, long-term and high dose steroid treatment, sulfasalazine are reported as additional risk factors. Hydration status may also play an important role in this process. Intestinal surgery is a widely described independent risk factor. Patients with ileostomy post bowel resection may have relative dehydration from liquid stool, which, added to the acidic pH from bicarbonate loss, is responsible for this process. In this acidic pH, the urinary citrate level excretion reduces. The stones most commonly seen in these patients contain uric acid or are mixed. In addition, the risk of calcium containing stones also increases with ileostomy. The treatment of UL in IBD involves correction of the basic gastrointestinal tract inflammation, restricted dietary oxalate intake, and, at times, increased calcium intake. Citrate therapy that increases both urine pH and urinary citrate could also provide an additional therapeutic benefit. Finally, patients with IBD in a pediatric study had less urologic intervention for their calculosis compared with pediatric patients without IBD. (www.actabiomedica.it)

Role of the JAK2 V617F Mutation in Inflammatory Bowel Disease-associated Thromboembolism: Case Report and Review of the Literature

Mutations in the Janus kinase-2 (JAK2) pathway are implicated in myeloproliferative neoplasia (MPN), as well as IBD. In patients without overt MPN, JAK2 mutation is implicated in intra-abdominal thrombosis. Despite this shared genetic predisposition, the role of JAK2 mutation in IBD-associated thromboembolism is not clear. We describe a case of catastrophic arterial thrombosis in an ulcerative colitis (UC) patient with JAK2 V617F mutation. A 62 year old woman presented with 3 months of bloody diarrhea and 15kg weight loss. Inflammatory markers were elevated but her hemogram was normal. Colonoscopy revealed pancolonic edema and erythema but no ulceration or spontaneous bleeding, consistent with moderate UC, Mayo score 2. The patient was started on prednisone and 5-ASA. Her symptoms improved and she was discharged home. She returned in 2 weeks with severe abdominal pain and shock. CT of the abdomen revealed splenic and renal infarcts and extensive thrombus in the common hepatic the superior mesenteric arteries. She underwent an emergent SMA embolectomy and extensive small bowel resection. A thrombophilia work-up revealed a positive JAK2 V617F mutation and warfarin was started. No evidence of MPN was found on bone marrow examination. A comprehensive search of Medline, PubMed, and EMBASE up to November 2016 was performed. We identified three articles related to JAK2 V617F mutations in IBD, comprising 171 patients. In the first study, no JAK2 V617F mutations were identified in 48 IBD with prior thrombotic events. In the second study, 100 IBD patients with no previous thrombotic event or MPN were tested, and no JAK2 V617F mutations were identified. The third study included 23 IBD patients with erythrocytosis or thrombocytosis but no previous thrombotic events. Three cases of JAK2 V617F mutations were identified, exceeding the expected thresholds for statistical significance. In summary, JAK2 V617F mutation is implicated in MPN but its role in IBD-related thrombosis remains unclear. Studies to date do not establish a pathophysiologic link between JAK2 mutations and IBD-related thrombosis. Given the known association between JAK2 V617F mutation and MPN, IBD patients presenting with erythrocytosis or thrombocytosis may benefit from JAK2 mutation testing. IBD patients with large intra-abdominal thrombosis may also benefit from testing for JAK2 mutations as this may alter the duration of anticoagulation treatment.Figure: Kaplan-Meier curves for time to first dose of anti-TNF therapy stratified type of insurance drug coverage. Publicly funded subjects (solid line) experienced longer times to first anti-TNF dose than privately funded subjects (dashed line).Figure: Rates of hospitalizations and emergency department visits stratified by public versus private drug insurance coverage. Additional sensitivity analyses are performed in which the following groups were excluded (excl): those who received first anti-TNF dose as inpatients (Inpt) or through a compassionate use program (Comp); those with private drug insurance coverage who received supplemental public funding (Copay). All rate differences between private and public drug coverage were statistically significant (* p<0.001).

NOD2 Deficiency Impairs the Intestinal Tight Junction Barrier in a Murine Short Bowel Model.

Introduction: Short bowel syndrome with chronic intestinal failure occurs after extensive small bowel resection. Despite intestinal adaptation, protein, energy, fluid and micronutrient balance cannot be maintained by a conventional diet. NOD2 mutations are a risk factor for chronic intestinal failure independent of the underlying disease. The mechanisms are not well understood. In a murine short bowel model the epithelial barrier function in the adapted jejunum was studied in the presence and absence of NOD2. Methods: Male C57BL6/J NOD2 knockout (−/−) and wild type (+/+) mice underwent 40% ileocecal resection (ICR). Sham control mice received transsection of the ileum. Mice were followed up for 14 days. At baseline (d0) 1cm of the most proximal resected ileum was studied in a vertical Ussing chamber and histologically. After 14d 1cm of the most distal remaining small intestine was studied and compared to baseline. In order to study the paracellular leak pathway, flux of 4kDa FITC-dextran was measured. The pore pathway was characterized by transepithelial resistance (TER) and NaCl diultion potentials. Results: Both +/+ and −/− animals showed similar morphologic adaptation 7 days after ICR such as significantly increased villus height (+/+ 229% P=0.028 vs. d0 and −/− 191% P=0.028 vs. d0, n=6-7 each) and crypt depth (+/+ 153% P=0.028 vs. d0 and −/− 129% P=0.028 vs. d0, n=6-7 each). The barrier to uncharged macromolecules (4 kDa FITC-dextran flux) was largely unaffected by NOD2 status. TER was not different in jejunal segments of +/+ and −/− mice at baseline levels (d0) and increased in the adapted jejunum. TER increase at d14 was significantly higher in −/− mice (74.5±6.0 Ω*cm2 +/+ ICR vs. 105.0±10.6 Ω*cm2 −/− ICR, n=5). In line with this, relative permeability to sodium was more severely impaired in the adapted tissue of −/− mice (+/+ ICR d0 7.7±0.4 vs. 14d 6.9±0.3 P=0.35, −/− ICR d0 6.3±0.1 vs. 14d 5.7±0.1 P=0.0157, n=5 each). Conclusion: Profound morphologic adaptation occurs after extensive ileocecal resection. The paracellular leak flux across the adapted epithelium is unaffected by NOD2 status. In contrast, TER is increased after adaptation and more so in absence of NOD2. This may in part be explained by an altered internal electric field in the structurally altered mucosa. However, adaptation of the tight junction barrier occurs as well. Our studies indicate that NOD2 function specifically contributes to the regulation of tight junction mediated charge selective ion flux in the adapted jejunum.

Delayed Presentation of Short Bowel Syndrome Complicated with Severe Degree of Nutritional Deficiencies, Nephrocalcinosis and Distal Renal Tubular Acidosis

Background: Short bowel syndrome (SBS) is a malabsorptive state due to functional or anatomic loss of extensive segments of small intestine which can lead to nutritional deficiencies and metabolic disarrangements. Here we describe a young patient with short bowel presenting with severe nutritional deficiencies, nephrocalcinosis and distal renal tubular acidosis. Nephrocalcinosis and distal renal tubular acidosis are closely associated and each can lead to the other. There are only rare case reports of short bowel syndrome complicated with nephrocalcinosis and distal RTA and severe degree of metabolic derangement with nutritional deficiencies. And this case highlights the importance of early management of short bowel syndrome in order to prevent long-term complications. Case presentation: A forty two-year-old Sri Lankan male patient who had undergone appendectomy at the age of nineteen years, which was complicated by bowel gangrene and about 240 cm of small bowel was resected. Later he presented with recurrent urolithiasis and urinary tract infections and ten years after the surgery imaging revealed bilateral nephrocalcinosis and obstructive uropathy. Distal Renal tubular acidosis (RTA) was diagnosed at the same time. He also had osteomalacia, proximal myopathy, multiple vitamin deficiencies, mixed deficiency anemia. He was started on calcium, iron supplements, parenteral B12, vitamin D injections and alkali therapy to achieve a normal serum bicarbonate concentration. His calcium and Vitamin D supplementation is currently being monitored with serum calcium, 25-hydroxyvitamin D, serum alkaline phosphatase, serum PTH and urinary calcium/creatinine ratio. Conclusion: Patients with extensive small bowel resection are at risk for nutrient, mineral, and vitamin deficiencies because of the loss of absorptive surface. It can also be complicated with nephrocalcinosis and distal RTA which is rare in short bowel syndrome. It is therefore important to identify patients at risk of short bowel syndrome and institute an early management, follow up plan to prevent complications.

Impact of Care in a Multidisciplinary Clinic on a Child With Ultrashort Gut

Abstract Short bowel syndrome (SBS) is a condition characterized by dependence on total parenteral nutrition due to the inability of the intestines to absorb adequate nutrients, electrolytes, and water. SBS is most commonly seen in pediatric patients after extensive small bowel resection due to necrotizing enteroclolitis, intestinal atresias, gastroschisis, or volvulus (Cummings &amp; Mercurio, 2012). The management of children with SBS focuses on maximizing enteral nutrition while mitigating the potential life-threatening complications of central venous line infections and liver failure. The process of slowly advancing enteral feedings and promoting enterocyte proliferation and villus height is termed adaptation (Warner, 2013). These changes provide an increased surface area for nutrient absorption. The promotion of adaptation is the cornerstone of pediatric intestinal rehabilitation. In the following paragraphs, a case study is presented describing the experiences of a child with ultrashort gut and her journey to independence from total parenteral nutrition at 3 years old. Key interventions that may have played a role in this child's success will be discussed.

The Long and Short of It: A Case of Long-term Teduglutide for the Treatment of Intestinal Rejection in Small Bowel Transplant: 2016 ACG Presidential Poster Award

Small bowel transplantation (SBT) is a therapeutic option for those patients who suffer from short bowel syndrome, intestinal failure, and life-threatening complications related to total parental nutrition (TPN). Post-small bowel transplant rejection continues to be a major cause of morbidity and mortality. Endoscopically, graft rejection is characterized by hemorrhage, blunting, and sloughing of the intestinal villi while histologically, it is characterized by increased apoptotic bodies and decreased mitotic bodies per crypt. Teduglutide, a glucagon-like peptide 2 analogue, has been shown to improve intestinal absorption by promoting villous hypertrophy and crypt proliferation. We hypothesized that teduglutide 5 mg subcutaneously daily can promote small bowel villous growth and attenuate small bowel transplant rejection by increasing crypt division while decreasing the apoptotic rate. This novel application of teduglutide may facilitate recovery from rejection and improve nutritional absorption. We present an interesting case of a 22-year-old woman with a myofibroblastic tumor of the mesentery who underwent extensive small bowel resection and subsequent living related small bowel transplantation. She presented with fever and abdominal pain post-operative day 14 after SBT. She underwent ileoscopy revealing ulcerated and extensively denuded intestinal mucosal villi. Histology demonstrated inflammatory changes extending through the muscularis mucosa that was consistent with severe acute graft rejection. She was subsequently placed on teduglutide while continuing immunosuppressive therapy and underwent serial endoscopic evaluations with random small bowel biopsies. Endoscopically, there was serial improvement in the health of the small bowel graft, as histologically demonstrated by an increase in villous growth, overall decrease in the apoptotic rate, and overall increase mitotic rate over 9 months. Furthermore, we conducted novel fluorescent in-situ hybridization (FISH) analysis which confirmed an XY karyotype in the regenerating stem cells of the small bowel graft, indicating that her father's stem cells from the donated small bowel were indeed regenerating. In conclusion, we present a novel application of teduglutide to mitigate small bowel transplant rejection and promote villous growth. Teduglutide should be considered in these patients to help improve nutritional status, allow them to wean off TPN, and ultimately improve quality of life.