Search

Intraoperative rerupture during open surgical clipping of cerebral aneurysms in subarachnoid hemorrhage (SAH) is a relatively frequent and potentially catastrophic occurrence. Patients who suffer rerupture have been shown to have worse outcomes at discharge compared with those who do not have rerupture. Perioperative injury likely plays a large part in the clinical worsening of these patients. However, due to the increased vessel manipulation and repeat exposure to acute hemorrhage, it is possible that secondary injury from increased incidence of vasospasm also contributes. Identifying an increased rate of vasospasm in these patients would justify early aggressive treatment with measures to prevent delayed cerebral ischemia. The authors investigated whether patients who suffer intraoperative rerupture during surgical treatment of ruptured cerebral aneurysms are at increased risk of developing vasospasm.Five hundred consecutive patients treated with open surgical clipping for SAH were reviewed, and clinical and imaging data were collected. Angiographic vasospasm was defined as vessel narrowing believed to be consistent with vasospasm on angiography. Symptomatic vasospasm was defined as angiographic vasospasm in the setting of a clinical change attributable to vasospasm. Rates of angiographic and symptomatic vasospasm among patients with and without intraoperative rerupture were compared.There were no significant differences between the groups with and without rupture with respect to age, sex, modified Fisher grade, history of hypertension, or smoking. The group with intraoperative rupture had more patients with Hunt and Hess Grade I. Angiographic vasospasm was noted in 279 (66%) of the 425 patients without rerupture compared with 49 (65%) of the 75 patients with rerupture (p = 1.0, Fisher's exact test). Symptomatic vasospasm was noted in 154 (36%) of the 425 patients without rerupture, compared with 31 (41%) of the 75 patients with rerupture (p = 0.44, Fisher's exact test). In multivariate analysis, higher modified Fisher grade was significantly predictive of vasospasm, whereas older age and male sex were protective.This study found no significant influence of intraoperative rerupture during open surgical clipping on the rate of angiographic or symptomatic vasospasm. Brief exposure to acute hemorrhage and vessel manipulation associated with rerupture events did not affect the rate of vasospasm. Risk of vasospasm was related to increased modified Fisher grade, and inversely related to age and male sex. These results do not justify early, targeted vasospasm therapy in patients with intraoperative rerupture.

Objective: A liver biopsy is necessary to grade and stage chronic hepatitis C virus (HCV) infection. In a previous study of patients with nonalcoholic liver disease, an aspartate aminotransferase (AST) to alanine aminotransferase (ALT) ratio >1 suggested cirrhosis. We sought to examine the value of the AST/ALT ratio in distinguishing cirrhotic patients with chronic HCV infection from noncirrhotic patients and to correlate the ratio with the grade and stage of hepatitis and other biochemical indices. Methods: We retrospectively studied 139 patients with chronic HCV infection. Routine biochemical indices were determined, and the histological grade of necroinflammatory activity and the stage of fibrosis of the liver biopsy specimens were scored. Results: The mean AST/ALT ratio in the cirrhotic patients (n = 47) was higher than in the noncirrhotic patients (n = 92) (1.06 ± 0.06 vs 0.60 ± 0.09; p < 0.001). A ratio ≥1 had 100% specificity and positive predictive value in distinguishing cirrhotic from noncirrhotic patients, with a 53.2% sensitivity and 80.7% negative predictive value. The ratio correlated positively with the stage of fibrosis but not with the grade of activity or other indices. Of the cirrhotic patients, 17% had no clinical or biochemical features suggestive of chronic liver disease except for an AST/ALT ratio ≥1. Conclusion: The AST/ALT ratio is a dependable marker of fibrosis stage and cirrhosis in patients with chronic HCV infection.

INTRODUCTION: Intraoperative re-rupture of cerebral aneurysms is a frequent complication of subarachnoid hemorrhage (SAH) treatment. Studies have demonstrated worsened outcomes after re-rupture, particularly in those treated with surgical clipping, for reasons that are unknown. We designed a study to test the hypothesis that worsened outcomes in these patients may be due to increased vasospasm. Detecting a higher incidence of vasospasm in this population would justify aggressive early treatment to prevent subsequent ischemic injury in those with intraoperative re-rupture after SAH. METHODS: All patients with aneurysmal SAH treated with open surgical clipping between 2006-2008 were examined at a single institution. Angiographic vasospasm was defined as narrowing of vessels seen on catheter angiography. Symptomatic vasospasm was defined as clinical deterioration after other causes had been excluded or radiographic evidence of infarction attributable to vasospasm. RESULTS: We identified 241 consecutive patients that included 41 with intraoperative re-rupture (17%). Average age was 54 ± 13 years and 69% of the patients were female. Hunt Hess scores between the group with intraoperative re-rupture and without were similar (2.1 ±1.3 vs. 2.4 ± 1.3, p = 0.09). Modified Fischer scores were also similar between the two groups (2.8 ±1.2 vs. 2.8 ± 1.2, p = 0.66). Time from rupture to definitive treatment was not significantly different (3.5 days ± 3.2 vs. 3.5 days ± 3.2, p=0.9). Patients in both groups had similar histories of smoking (58% vs. 50%, p=0.47) and HTN (49% vs. 53%, p=0.72). Angiographic vasospasm was seen in 61% of the group without re-rupture and 63% in the group with re-rupture (p=0.83). Symptomatic vasospasm was seen in 38% of patients without re-rupture and 49% of patients with re-rupture (p=0.26). In-hospital mortality was higher in the group with intraoperative re-rupture (20% vs. 9%, p DISCUSSION: Our results corroborated previous findings of worsened outcomes after intraoperative re-rupture in SAH patients treated with surgical clipping, but did not show a significant difference in rates of vasospasm. These findings indicate that the worsened outcome of these patients is likely secondary to other, as yet known, causes.

The diagnosis of chronic pancreatitis in patients with characteristic symptoms but normal pancreatic imaging is challenging. Assessment of pancreatic function through secretin pancreatic function testing (SPFT) has been advocated in this setting, but its diagnostic accuracy is not fully known.This was a retrospective review of patients who received SPFT at our tertiary care institution between January 1995 and December 2008 for suspected chronic pancreatitis. For all patients, medical records were reviewed for evidence of subsequent development of chronic pancreatitis by imaging and/or pathology. Patients were then categorized as "true positive" or "true negative" for chronic pancreatitis based on follow-up imaging or histologic evidence.In all, 116 patients underwent SPFT. Of the 27 patients who tested positive, 7 were lost to follow-up. Of the remaining 20 SPFT-positive patients, 9 (45%) developed radiologic or histologic evidence of chronic pancreatitis after a median of 4 years (1-11 years). Of the 89 patients who had negative SPFT testing, 19 were lost to follow-up. Of the remaining 70 patients, 2 were eventually diagnosed with chronic pancreatitis based on subsequent imaging/histology after a median follow-up period of 7 years (3-11 years). The sensitivity of the SPFT in diagnosing chronic pancreatitis was 82% with a specificity of 86%. The positive predictive value (PPV) of chronic pancreatitis was 45% with a negative predictive value (NPV) of 97%.In patients with suspected early chronic pancreatitis and normal pancreatic imaging, SPFT is highly accurate at ruling out early chronic pancreatitis with a NPV of 97%.

Defects in cellular energy metabolism represent an early feature in a variety of human neurodegenerative diseases. Recent studies have shown that targeting energy metabolism can protect against neuronal cell death in such diseases. Here, we show that meclizine, a clinically used drug that we have recently shown to silence oxidative metabolism, suppresses apoptotic cell death in a murine cellular model of polyglutamine (polyQ) toxicity. We further show that this protective effect extends to neuronal dystrophy and cell death in Caenorhabditis elegans and Drosophila melanogaster models of polyQ toxicity. Meclizine’s mechanism of action is not attributable to its anti-histaminergic or anti-muscarinic activity, but rather, strongly correlates with its ability to suppress mitochondrial respiration. Since meclizine is an approved drug that crosses the blood–brain barrier, it may hold therapeutic potential in the treatment of polyQ toxicity disorders, such as Huntington’s disease.

Purpose To compare diagnostic accuracy and patient tolerance of MR colonography with intravenous contrast and luminal air (MRC) to conventional colonoscopy (CC). Materials and methods IRB approval and written informed consent were obtained. Forty-six patients, both screening and symptomatic, underwent MRC followed by CC. The MRC technique employed 3D T1W spoiled gradient echo sequences performed after the administration of gadopenetate dimeglumine, with parallel imaging. The diagnostic accuracy and tolerance of patients for MRC was compared to CC. Results Twenty-four polyps were detected in eighteen patients with CC (5 polyps ≥10 mm, 4 polyps 6–9 mm, 15 polyps ≤5 mm). MRC was 66.7% (12/18) sensitive and 96.4% (27/28) specific for polyp detection on a per-patient basis. When analyzed by polyp size, sensitivity and specificity of MRC was 100% (5/5) and 100% (19/19), respectively, for lesions greater than 10 mm, 100% (4/4) and 100% (20/20) for lesions 6–9 mm, and sensitivity of 20% (3/15) lesions less than 5 mm. The sensitivity and specificity of MRC for detecting significant lesions (>6 mm) was 100% (9/9) and 100% (15/15), respectively. Regarding tolerance of the exams, there were no significant differences between MRC and CC. Thirty-five percent ( n = 16) of patients preferred MRC as a future screening test compared to 33% ( n = 15) for CC. Conclusion MRC using air as an intraluminal contrast agent is a feasible and well-tolerated technique for detecting colonic polyps ≥6 mm in size. Further studies are warranted.

BACKGROUND & AIMS: Celiac crisis is a life-threatening syndrome in which patients with celiac disease have profuse diarrhea and severe metabolic disturbances. Celiac crisis is rare among adults and not well documented. To improve awareness of this condition and to facilitate diagnosis, we reviewed cases of celiac crisis to identify presenting features, formulate diagnostic criteria, and develop treatment strategies. METHODS: Cases of biopsy-proven celiac disease were reviewed. Celiac crisis was defined as acute onset or rapid progression of gastrointestinal symptoms that could be attributed to celiac disease and required hospitalization and/or parenteral nutrition, along with signs or symptoms of dehydration or malnutrition. RESULTS: Twelve patients met preset criteria for celiac crisis; 11 developed celiac crisis before they were diagnosed with celiac disease. Eleven patients had increased titres of transglutaminase antibodies and 1 had immunoglobulin A deficiency. Results of biopsy analyses of duodenum samples from all patients were consistent with a Marsh 3 score (33% with total villous atrophy). Patients presented with severe dehydration, renal dysfunction, and electrolyte disturbances. All patients required hospitalization and intravenous fluids, 6 required corticosteroids, and 5 required parenteral nutrition. All patients eventually had a full response to a gluten-free diet. CONCLUSIONS: Celiac crisis has a high morbidity and, although rarely described, occurs in adults and often has a clear precipitating factor. Patients who present with severe unexplained diarrhea and malabsorption should be tested for celiac disease; treatment with systemic steroids or oral budesonide should be considered. Nutritional support often is required in the short term but most patients ultimately respond to gluten avoidance.

SummaryMitochondria are complex organelles whose dysfunction underlies a broad spectrum of human diseases. Identifying all of the proteins resident in this organelle and understanding how they integrate into pathways represent major challenges in cell biology. Toward this goal, we performed mass spectrometry, GFP tagging, and machine learning to create a mitochondrial compendium of 1098 genes and their protein expression across 14 mouse tissues. We link poorly characterized proteins in this inventory to known mitochondrial pathways by virtue of shared evolutionary history. Using this approach, we predict 19 proteins to be important for the function of complex I (CI) of the electron transport chain. We validate a subset of these predictions using RNAi, including C8orf38, which we further show harbors an inherited mutation in a lethal, infantile CI deficiency. Our results have important implications for understanding CI function and pathogenesis and, more generally, illustrate how our compendium can serve as a foundation for systematic investigations of mitochondria.

Endovascular treatment of broad-necked bifurcation aneurysms remains challenging. Stent-assisted coiling has been successful but requires catheterization of the branches off the parent vessel. We present the case of a patient who failed primary and stent-assisted coiling of a large basilar tip aneurysm because the morphology of the aneurysm precluded successful distal catheterization of the posterior cerebral artery (PCA) branches. Using the PulseRider device, which does not require catheterization of bifurcation branches, we were able to treat the aneurysm successfully.

Endovascular treatment of broad-necked bifurcation aneurysms remains challenging. Stent-assisted coiling has been successful but requires catheterization of the branches off the parent vessel. We present the case of a patient who failed primary and stent-assisted coiling of a large basilar tip aneurysm because the morphology of the aneurysm precluded successful distal catheterization of the posterior cerebral artery (PCA) branches. Using the PulseRider device, which does not require catheterization of bifurcation branches, we were able to treat the aneurysm successfully.

The majority of inherited mitochondrial disorders are due to mutations not in the mitochondrial genome (mtDNA) but rather in the nuclear genes encoding proteins targeted to this organelle. Elucidation of the molecular basis for these disorders is limited because only half of the estimated 1,500 mitochondrial proteins have been identified. To systematically expand this catalog, we experimentally and computationally generated eight genome-scale data sets, each designed to provide clues as to mitochondrial localization: targeting sequence prediction, protein domain enrichment, presence of cis-regulatory motifs, yeast homology, ancestry, tandem-mass spectrometry, coexpression and transcriptional induction during mitochondrial biogenesis. Through an integrated analysis we expand the collection to 1,080 genes, which includes 368 novel predictions with a 10% estimated false prediction rate. By combining this expanded inventory with genetic intervals linked to disease, we have identified candidate genes for eight mitochondrial disorders, leading to the discovery of mutations in MPV17 that result in hepatic mtDNA depletion syndrome. The integrative approach promises to better define the role of mitochondria in both rare and common human diseases.

A 46-year-old man with psoriasis and morbid obesity developed acute aphasia and right hemiplegia. As a result of his weakness, he had fallen and struck his right eye on a desk. His presenting examination demonstrated right periorbital ecchymosis without accompanying ptosis, expressive aphasia, left gaze deviation with corresponding hemianopsia, and dense right hemiplegia, summating an NIH Stroke Scale score of 13.

The mechanism by which pancreas divisum may lead to recurrent episodes of acute pancreatitis in a subset of individuals is unknown. Abnormalities of the cystic fibrosis gene product (CFTR) have been implicated in the genesis of idiopathic chronic pancreatitis. The aim of this study was to determine if CFTR function is abnormal in patients with pancreas divisum and recurrent acute pancreatitis (PD/RAP).A total of 69 healthy control subjects, 12 patients with PD/RAP, 16 obligate heterozygotes with a single CFTR mutation, and 95 patients with cystic fibrosis were enrolled. CFTR function was analyzed by nasal transepithelial potential difference testing in vivo. The outcomes of the PD/RAP patients following endoscopic and surgical treatments were concomitantly analyzed.Direct measurement of CFTR function in nasal epithelium in response to isoproterenol demonstrated that the values for PD/RAP were intermediate between those observed for healthy controls and cystic fibrosis patients. The median value was 13 mV for PD/RAP subjects, which was statistically different from healthy controls (22 mV, p= 0.001) and cystic fibrosis pancreatic sufficient (-1 mV, p0.0001) and pancreatic insufficient (-3 mV, p0.0001) patients.These results suggest a link between CFTR dysfunction and recurrent acute pancreatitis in patients with pancreas divisum and may explain why a subset of patients with pancreas divisum develops recurrent acute pancreatitis.

Background: The industry standard since 1990 for self-expanding biliary metallic stents has been the Wallstent. In 1998 the Spiral Z-stent was released. This randomized trial compared the Z-stent with the Wallstent in the treatment of malignant biliary obstruction. Methods: Patients with unresectable malignant biliary obstruction distal to the bile duct bifurcation were randomized to receive a 10-mm diameter Wallstent or a 10-mm diameter Z-stent. Results: A total of 145 patients were randomized; 13 were excluded. Sixty-four patients who received a Z-stent and 68 who had a Wallstent are included in the analysis. Tumors responsible for bile duct obstruction were pancreatic cancer (108), cholangiocarcinoma (15), metastatic cancer (6), and papillary cancer (3). Metallic stents were successfully placed in all patients. Seven technical problems were encountered during placement of the Z-stent and 5 with the Wallstent. There were 21 occlusions requiring reintervention (8 Z-stent, 13 Wallstent; p = 0.30). Median time to reintervention was the following: Z-stent, 162 days; Wallstent, 150 days (p = 0.22). A total of 104 patients died of progressive disease or other cause; 7 patients remain alive with patent stents. The overall calculated median patency rates were: Z-stent, 152 days; Wallstent, 154 days (p = 0.90). Conclusions: The Spiral Z-stent is comparable with the Wallstent in terms of placement, occlusion rates, and overall patency. Occasional early occlusion of both stents suggests tumor characteristics instead of the size of the mesh openings in the stents as important factors. (Gastrointest Endosc 2003;57:830-6.)

Introduction Differing biases may affect enrollment patterns and result in trial participants that are not representative of clinical practice nor ideal candidates for therapy. We sought to evaluate the stringency of inclusion/exclusion criteria for five recent endovascular stroke treatment (EST) trials and then measure any bias in the decision making process for EST trial enrollment at our institution. Materials and methods From a prospectively maintained registry, all patients treated in clinical practice with EST from April 2004 to June 2013 were identified. The decision to perform EST at our institution is based upon the clinical history, examination, and favorable imaging pattern of proximal vessel occlusion without evidence of significant ischemic injury. Trial eligibility criteria from EST trials active at our institution were used to determine the proportion and characteristics of patients eligible for each trial. Results Among 299 patients treated with EST, average age was 68, 57% were female, NIHSS was 17, and 45% received IV tPA. 125/269 (47%) would have been eligible for MR RESCUE, 33/208 (16%) for IMS III, 10/20 (50%) for SWIFT, 32/41 (78%) for MultiMERCI, 6/11 (55%) for TREVO2. Patients enrolled in trials numbered 57, 21% of all patients in the study time period and 17–50% of eligible patients. Using the trial with the most enrollments as an example (22 patients, MR RESCUE), reasons for inability to enrol included concurrent enrollment in another trial (43%), referring physician refusal for randomization (35%), inability to complete imaging (8%) and inability to obtain consent (7%). Compared to patients enrolled in the trial, those eligible but treated outside of a trial presented with similar distributions of age (68 vs. 69, p = 0.73) and gender (female 52% vs. 55%, p = 1.0). Eligible patients treated outside of trials presented with lower NIHSS (17 vs. 20, p Conclusions Endovascular trials excluded the majority of patients deemed suitable for ERT in routine practice. Competing interests from other trials as well as reluctance to randomise against EST limited enrollment. The population of patients enrolled in trials tended to present later, have more severe strokes, and suffer worsened outcomes. Conviction in the benefit of EST led to the treatment of patients most likely to benefit from EST outside of trials. As a result, recent EST trials may have not reflected the treatment population and outcomes of routine practice. Disclosures S. Sheth: None. J. Saver: None. R. Jahan: None. S. Starkman: None. G. Duckwiler: None. S. Tateshima: None. N. Gonzalez: None. D. Liebeskind: None.. . UCLA Stroke Investigators: None.

Background: Management of chronic pancreatitis remains a challenge. Current strategies reserve surgery as a last-available option after medical therapy, lifestyle modifications, and endoscopy have failed. Some clinicians advocate early consideration of surgical treatment to prevent disease progression and reduce the risk of opioid dependency. Aims: The aim of this study was to describe the use of elective surgery for chronic pancreatitis in a high-volume, teaching hospital. Patients & methods: Retrospective analysis of administrative patient data for in-patients with chronic pancreatitis 2003 2013 inclusively. Demographic and clinical information, including number of admissions and costs, was obtained through hospital decision support. Surgery of interest: Frey procedure, pancreaticoduodenectomy, total pancreatectomy, distal pancreatectomy, and Duodenum-Preserving Pancreatic Head Resection. Debridements and emergent pancreatic operations excluded. Results: Of 3,480 patients, 406 (11.7%) received surgery of interest. Both newchronicpancreatitisdiagnosesandproceduresperformedremainedstable throughout this time period. Whipple and other pancreaticoduodenectomies comprised the largestproportionofoperationswith48.8% (n1⁄4198). 22.7%were distal pancreatectomy, and 18.7%were pancreatojejunostomy including Frey’s procedure. There was a non-significant decrease in use of pancreaticoduodenectomy over time. The group receiving surgery was older (mean: 57.8 vs. 54.2 years

Although they occur less frequently than cardiopulmonary events, gastrointestinal complications now are recognized increasingly in chronically critically ill patients. These are usually the consequence of systemic processes and multiorgan dysfunction that are prevalent in this patient population. Recognition of gastrointestinal complications in critically ill patients is extremely challenging because classic signs and symptoms are often absent or masked by the frequent use of sedative and narcotic medications in intubated patients. Moreover, laboratory and radiologic studies are not always helpful. This situation often results in delayed diagnosis which, in turn, leads to increased mortality. In this article, the authors review the epidemiology, clinical features, diagnosis, prevention, and management of the various gastrointestinal complications that occur in a chronically ventilated patient. These critically ill patients receive their care both in long-term acute care facilities and in intensive care units (ICUs).

Background and purpose The natural history of perimedullary arteriovenous fistulae (PMAVFs), also known as type IV spinal cord AV fistulae, is incompletely understood. Both open surgical and endovascular approaches have been described as treatment modalities for this disease. The goal of this study was to evaluate the long term outcome of patients with PMAVFs treated at a single tertiary care institution. Materials and methods We conducted a retrospective study on 32 patients with PMAVFs, evaluated between 1983 and 2009. Data were gathered by reviewing outpatient clinic notes, operative and radiological reports, and spinal angiograms. The PMAVFs were categorized into one of three types based on the angiographic imaging criteria. Pretreatment and post-treatment ambulation and micturition symptoms were quantified using the Aminoff and Logue scale (ALS). Results 30 patients underwent corrective procedures, four by embolization alone, 11 by surgery alone and 15 patients received a combination of the two. 28 patients underwent follow-up spinal angiography, with residual shunting noted in six patients. Mean follow-up period was 54 months (range 1–228). Analysis of the ALS scores revealed that treatment of PMAVFs, independent of modality, resulted in significant improvement in ambulation, but inconsistent changes in micturition. In addition, residual fistula at the time of follow-up angiogram was associated with worsened neurologic status or lack of improvement. Outcome analysis based on fistula type showed dramatic improvement in ALS ambulation scores (62%) for type 3 fistulae compared with type 1 and 2 (26%, 27%, respectively). Conclusion Significant improvement in ambulation, but not micturition, was observed following treatment. Residual fistula on follow-up angiography was associated with progressive worsening or lack of improvement in neurologic function. Patients with type 3 fistulae were shown to benefit most from treatment, with marked improvement in post-treatment ambulation scores. As endovascular and surgical techniques continue to evolve, further studies are warranted.

The objective of this article is to review the available literature on the epidemiology, predisposing factors, and conditions associated with primary gallbladder cancer, and to discuss the role of prophylactic cholecystectomy in high-risk patient populations. Gallbladder cancer is a highly malignant tumor with a poor 5-yr-survival rate. It is a tumor of the elderly and has striking genetic, racial, and geographic characteristics, with an extremely high prevalence in Native Americans and Chileans. Cholelithiasis is a well-established risk factor for gallbladder cancer and the risk seems to correlate with stone size. Polyps that are >1 cm, single, sessile, and echopenic are associated with a higher risk of malignancy. Anomalous junction of pancreaticobiliary ducts (AJPBD), especially without choledochal cyst, and porcelain gallbladder are additional factors that predispose to gallbladder cancer. Lesser associations include chronic bacterial infections of the gallbladder, typhoid carrier state, certain occupational and environmental carcinogens, hormonal changes in women, and certain social, dietary, and familial factors. It is important to identify high-risk groups for gallbladder cancer because of the dismal nature of this tumor. In patients with porcelain gallbladder and anomalous junction of the pancreatic and biliary ducts, cholecystectomy is recommended provided that the patient is a good operative candidate. Patients with large solitary polyps or gallstones require close ultrasonic follow-up. With the advent of endoscopic ultrasound it is expected that early changes of malignancy in polyps will be reliably detected, and more patients will potentially be cured with a simple cholecystectomy. Through a MEDLINE/PAPERCHASE search we identified and reviewed articles regarding gallbladder cancer published in English-language journals between 1966 and 1999, using the key words biliary tract and gallbladder diseases, cancer, neoplasms, surgery, cholelithiasis, gallstones, cholecystitis, gallbladder polyps, risk factors, chemical industry, occupational diseases, typhoid, porcelain gallbladder, bacteremia, and precancerous conditions. We also used the bibliography of relevant articles to increase our search. A total of 122 publications were selected using the mentioned data source.

Background: The Institute of Medicine defines guidelines as statements to assist physician and patient decisions regarding appropriate health care in clinical situations. Guidelines are meant to create a succinct roadmap for diagnosis and treatment by analyzing and summarizing the abundance of medical studies. This is predicated on guidelines being unbiased and based on strong quality of evidence. Currently, though, no studies have reviewed the quality of the interventional medical subspecialty guidelines Methods: We reviewed the interventional medicine subspecialty society's websites for interventional guidelines. The American Society of Gastroenterology Endoscopy (ASGE), American Association of Diagnostic and Interventional Nephrology (ASDIN), American Association for Bronchology and Interventional Pulmonology, American Thoracic Society, CHEST, and Society for Cardiovascular Angiography and Interventions (SCAI) were reviewed for guidelines that graded the level of evidence, methods used to grade the evidence, and disclosures of conflicts of interest (COI). The data were evaluated in aggregate and by society. Results: 153 interventional guidelines were evaluated of which two were duplicates. 46% (n=70/151) of the interventional guidelines graded the quality of evidence. ASGE graded 72% (n=48/67) compared to 28% (n=22/78) by SCAI, and 0 (n=4) by ASDIN and 0 (n=2) by the pulmonary societies. Of the 3460 recommendations reviewed 11% (n=374) were supported by the highest quality of evidence, level A (multiple randomized controlled trials), 41% (n=1414) were level B (single or nonrandomized controlled trials), and 48% ((n=1645) were level C (expert opinion). See table 1 for breakdown by society. While SCAI used two systems to grade the level of evidence, ASGE used 5 different systems to grade its evidence. Furthermore, 62% (n=94/ 151) of the guidelines failed to comment on COI. Of the 57 articles that disclosed COI, 91% (n=52) reported potential conflicts. In total 1855 COI were reported of which 967 were non-research based COI. Authors with conflicts (n=320/705) averaged 5.8 conflicts/ authors. While the SCAI disclosed potential COI in 52% of their guidelines, ASGE did so in only 24% of their guidelines, and the ASDIN and pulmonary societies did not disclose COI. Conclusions: Majority of the medicine subspecialty interventional guidelines fail to grade the quality of evidence, and when present, most used lower quality evidence. Furthermore, most guidelines fail to comment on potential COI. When assessed, numerous COI were present. Interventional procedures carry unique risks to the patients and guidelines should ideally be based on strong quality of evidence. Future interventional guidelines should clearly state the quality of evidence for their recommendations, utilize a standard grading system, and be transparent regarding all potential biases. Table 1. Total number of practice guidelines with graded evidence and quality of evidence for recommendations

Background Gamma Knife radiosurgery (GKRS) is an option in the treatment of pediatric arteriovenous malformations (AVMs), especially for AVMs in eloquent locations or patients with good functional status. Prior studies have indicated that lower target volumes and higher prescription doses are associated with improved obliteration rates. Certain angiographic features including maximal diameter, diffuse border, and venous drainage patterns have also been shown to have n influence on rate of obliteration after GKRS.1 2 3 Methods We retrospectively reviewed all pediatric patients age ≤18 treated with GKRS for an AVM between 2000 and 2010 at our institution. Angiographic features based on pre-treatment angiography were prospectively recorded in a standardized manner and scored by experienced neuroradiologists. Results Between 2000 and 2010, 41 children (23 boys and 18 girls) underwent GKRS for an AVM. Follow-up information was available for 39 patients with 3-year angiographic follow-up in 22. Mean age at time of treatment was 13.5±3.5 years. Two patients had a Spetzler-Martin grade (SMG) II, 23 had grade III, 12 had grade IV, and 4 had grade V. The mean target volume for GKRS was 9.2±10.2 cm3 while the median prescription dose of radiation was 18Gy (range 16–20 Gy). 13 patients underwent staged GKRS. Among the 22 patients with 3-year angiographic follow-up, complete obliteration was observed in 9 (40.9%), while 12 (54.5%) showed a partial response. Only one patient had no response. Prescription dose was significantly correlated to angiographic response (p=0.046, logistic regression), with a dose ≥18 Gy being associated with a 75% complete obliteration rate compared to 27.2% in patients treated with 1), and fewer draining veins reaching a sinus (1 vs >1). Factors that did not have an influence on response rate included diffuse borders of AVM, having only deep venous drainage, venous ectasia, venous reflux, presence of any aneurysm, embolization prior to GKRS, and arterial inflow through anterior vs. posterior circulation. Conclusion There is a growing literature that GKRS is an effective treatment for pediatric AVMs. Our study finds that in addition to AVM volume and prescription dose, certain angioarchitectural features play a role in the rate of obliteration after treatment with GKRS. Smaller AVMs with fewer draining veins and fewer draining veins reaching a sinus had favorable outcomes. Competing interests None. References 1. Chang et al. J Neurosurg 2000;93(Suppl 3):96–101. 2. Kano et al. J Neurosurg Pediatrics 2012;9:1–10. 3. Yeon et al. Childs Nerv Syst 2011;27:1109–19.

Background: The diagnosis of chronic pancreatitis in patients with characteristic symptoms but normal pancreatic imaging is challenging. Assessment of pancreatic function through collection of duodenal fluid following secretin stimulation testing (SST) has been advocated in this setting. The diagnostic accuracy of SST in detecting early chronic pancreatitis is not fully known. Aim: To determine if subjects with suspected chronic pancreatitis and abnormal SST subsequently develop histologic or radiographic changes of chronic pancreatitis compared with those with normal SST. Methods: We performed a retrospective review of subjects who received SST at our institution between January 1995 and December 2007 for suspected chronic pancreatitis. All had normal cross sectional and endoscopic imaging of the pancreas prior to SST. A positive test was defined as a peak bicarbonate level of

Background Adenoma detection rate is an important measure of colonoscopy quality; however, factors including procedure order that contribute to adenoma detection are incompletely understood. Objective The aim of this study was to prospectively evaluate factors associated with adenoma detection rate. Design Prospective cohort study. Data were collected on patient and physician characteristics, trainee participation, time of day, and case rank. Setting Outpatient tertiary-care center. Patients This study involved consecutive patients presenting for first screening colonoscopies. Main Outcome Measurements Adenoma and polyp detection rates (proportion of cases with one or more lesion detected) and ratios (mean number of lesions detected per case). Results A total of 2139 colonoscopies were performed by 32 gastroenterologists. Detection rates were 42.7% for all polyps, 25.4% for adenomas, and 5.0% for advanced adenomas. Adenoma detection was associated with male sex and increasing age on multivariate analysis. In the overall study cohort, time of day and case rank were not significantly associated with detection rates. In post hoc analysis, polyp and adenoma detection rates appeared lower after the fifth case of the day for endoscopists with low volumes of cases and after the tenth case of the day for endoscopists with high volumes of cases. Limitation Single center. Conclusion Overall, time of day and case rank did not influence adenoma detection rate. We observed a small but significant decrease in detection rates in later procedures, which was dependent on physician typical procedure volume. These findings imply that colonoscopy quality in general is stable throughout the day; however, there may be a novel "stamina effect" for some endoscopists, and interventions aimed at improving colonoscopy quality need to take individual physician practice styles into consideration.

Purpose: Celiac crisis is a life-threatening syndrome where celiac disease presents with profuse diarrhea and severe metabolic disturbances. Celiac crisis in adults is believed to be uncommon and is not well documented. However, it is likely that many patients with celiac crisis are not definitively diagnosed. In order to improve awareness and to facilitate diagnosis, we reviewed cases of celiac crisis seen at Beth Israel Deaconess Medical Center in Boston, MA and Mayo Clinic in Rochester, MN. These data were used to identify presenting features, formulate diagnostic criteria for celiac crisis in adults and develop treatment strategies. Methods: We reviewed cases of biopsy proven celiac disease in the last 5 years. Celiac crisis was defined as acute onset or rapid progression of gastrointestinal symptoms attributable to celiac disease requiring hospitalization and/or parenteral nutrition along with at least 2 of the 7 factors listed below: 1.Signs of severe dehydration including hemodynamic instability or orthostasis 2.Neurologic dysfunction 3.Renal dysfunction: creatinine >2.0 4.Metabolic acidosis: pH 10 lbs Results: Twelve patients met the above criteria. Of these, 8 were women and 4 were men. 11 developed celiac crisis prior to diagnosis of celiac disease. IgA tissue transglutaminase titer was available for 11 patients. Of these, one had IgA deficiency, and all of the others had elevated tTG, eight with levels greater than 4 times the normal limits. Biopsies of the duodenum in all patients were consistent with a Marsh 3 score. HLA type was available for 10, of which 9 were positive for DQ2 and 1 for DQ8. Patients presented with severe dehydration, renal dysfunction, and electrolyte disturbances, of which hypocalemia was most common. All patients required hospitalization and intravenous fluids and five required parenteral nutrition. Six patients required corticosteroids. All patients had a rapid clinical response to gluten free diet. Conclusion: Celiac crisis is associated with high morbidity and although rarely described, does occur in adults, often without a clear precipitating factor. Patients presentingwith severe unexplained diarrhea and malabsorption should be tested for celiac disease and treatment with systemic steroids or oral budesonide considered. Nutritional support is often required in the short term but most patients ultimately respond to gluten avoidance. We are hopeful that our delineation of formal diagnostic criteria for celiac crisis will aid in identification and management of these patients.