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To determine the prevalence of non-use and limited use of cochlear implants. To find out the reasons for the same. This study was carried out among the paediatric patients who underwent cochlear implantation under the SHP* scheme in Civil Hospital, Ahmedabad between 2013 and 2020. Study Design: Retrospective cross-sectional study. Study Setting: Civil Hospital Ahmedabad. Sample Size: 150 paediatric patients. Patients were selected based on a random number generator. The data was collected through a telephonic interview based upon a preformed questionnaire. The study indicates that out of a total 150 participants, 109 (72.7%) used their cochlear implant regularly while 41 (27.3%) did not. Major issue faced by the participants was in the external device -damage to the device and monetary issues for its repair. The participants who have dropped the usage of their implants faced issues mostly after 2 years of usage. The age group of less than 3 years showed 15.6% of non-usage, while the age group of 3–6 years showed an increase to 34.5% of non-users. The age group of more than 6 years showed an even higher proportion (41.3%) of non-users. It is recommended that the government ensures the follow-up of the patients undergoing cochlear implantation for at least 2 years after the surgery. The proportion of non-users in the consecutive increasing age groups saw a rise in number. Therefore, it is recommended to divert the resources towards lower age groups to facilitate a better outcome. [ABSTRACT FROM AUTHOR]

Background: The development of guidelines by gastroenterology societies increasingly stresses evidence-based endoscopic practice. Aims: We performed a systematic assessment to determine whether endoscopic video teaching platforms incorporate evidence-based educational strategies and methods in order to disseminate guideline-based endoscopic management strategies. Methods: Platforms with a video component were systematically identified using the Google search engine, Apple and Android application stores, and searching four major gastroenterology society websites and three known platforms, to identify all relevant platforms. Two video samples from each teaching platform were reviewed independently by two authors and assessed for use of a priori defined principles of evidence-based medicine, as determined by consensus agreement and for the use of simulation. Results: Fourteen platforms were included in the final analysis, and two videos from each were analyzed. One of the 14 platforms used simulation and incorporated evidence-based medicine principles consistently. Nine of the 14 platforms were not transparent in regard to citation. None of the platforms consistently cited the certainty of evidence or explained how evidence was selected. Conclusions: Education of guideline-based endoscopic management strategies using principles of evidence-based medicine is under-utilized in endoscopic videos. In addition, the use of cognitive simulation is absent in this arena. There is a paucity of evidence-based cognitive endoscopy simulators designed for fellows that incorporate systematic evaluation, and efforts should be made to create this platform. [ABSTRACT FROM AUTHOR]

Nanostructured iron–zirconium oxides (NIZOs), with Fe-to-Zr molar ratio of 1:1 and 1:2, have been found to show high adsorption efficiency toward Pb2+ from aqueous solution. The NIZO was synthesized by the co-precipitation method and characterized in terms of XRD, TEM, SEM–EDX, BET surface area analysis, TGA, FT-IR spectroscopy and zeta potential measurement. The data of adsorption of Pb2+ on the NIZO, under optimized condition of pH, Pb2+ concentration, adsorbent amount, time and temperature, were fitted in Langmuir, Freundlich and Temkin adsorption model, and the data showed the best agreement with Langmuir model with R2 = 0.990 and 0.994 for Fe/Zr molar ratio = 1:1 and 1:2, respectively. The thermodynamic studies showed that the adsorption of Pb2+ on the NIZO proceeds spontaneously and exothermically through the involvement of weak van der Waals forces. Further, the adsorption of Pb2+ on the NIZO was found to follow pseudo-second-order kinetics. [ABSTRACT FROM AUTHOR]

Pure, 3 mol% and 5 mol% Sr-doped cerium oxide nanoparticles were synthesized by facile aqueous co-precipitation method using cerium nitrate hexahydrate and strontium chloride hexahydrate as the precursors without using any capping agent. The synthesized material was characterized by XRD, SEM, EDX, TEM, Raman spectroscopy and UV–Vis spectroscopic techniques. SEM analysis showed agglomeration of the particles. The Debye–Scherrer analysis revealed fluorite structure of the synthesized material with crystallite size in 6–10 nm range. TEM confirmed the spherical morphology of the particles and particle size distribution in the range of 5–8 nm. UV–Vis spectroscopic study revealed that Sr-doping led to increase in the band gap from 3.2 to 3.7 eV and shifting of absorption edge to the lower wavelength. The blue shift in the band gap with the dopant concentration shows that the band gap of doped cerium oxide nanoparticles can be tuned with variation in the dopant concentration. [ABSTRACT FROM AUTHOR]

Alzheimer's disease (AD) is a neurodegenerative condition associated with cognitive loss, behavioural changes, functional ability decline and caregiver burden. Given the worldwide public health impact of AD, novel interventions to reduce suffering experienced by AD patients need to be developed. Foods may offer a mechanism for intervention complementary to drugs, devices, biologicals and vaccines. Apart from foods with health claims (including dietary supplements), medical foods are also being explored as an intervention option. The purpose of this article is to describe how medical foods may complement other interventions for AD patients by: (i) defining what a medical food is; (ii) discussing whether AD is a condition amenable to medical food intervention; (iii) reviewing current clinical trial data on medical foods used in participants with AD; and (iv) highlighting steps needed to establish a more comprehensive framework for developing medical foods for AD. While medical foods may be defined differently in other countries, the US Orphan Drug Act of 1998 defined a medical food as a food formulated for enteral intake, taken under physician supervision, and intended to meet the distinctive nutritional requirements identified for a disease or condition. For AD to be amenable to medical food intervention, it must: (i) result in limited or impaired capacity to ingest, digest, absorb or metabolize ordinary food- stuff or certain nutrients; or (ii) have unique, medically determined nutrient requirements; and (iii) require dietary management that cannot be achieved by modification of the normal diet alone. While these criteria are most likely met in advanced AD, identifying unique nutritional requirements in early AD that cannot be met by normal diet modification requires a better under- standing of AD pathophysiology. A PubMed search using the terms 'medical food' and 'Alzheimer', limited to clinical trials published in English with human participants with AD aged >65 years and supplemented by other articles known to meet the inclusion criteria, revealed that only two medical foods, AC-1202 and Souvenaid® with Fortasyn Connect™, have clinical trial results available for discussion. As medical food development for AD is a relatively new endeavour, a window of opportunity exists for all stakeholders to develop a comprehensive framework for assuring that medical food interventions for AD achieve the highest possible scientific and ethical standards to warrant commercialization. [ABSTRACT FROM AUTHOR]

A 45-year-old man presented with vertebral collapse at L5 as an initial manifestation of multiple myeloma and underwent spinal fusion surgery using recombinant human bone morphogenetic protein-2 (rhBMP-2). Subsequent computed tomography (CT) scans and X-rays revealed heterotopic ossification of the left psoas muscle, pelvis, and anterior abdominal wall. While the occurrence of heterotopic ossification has previously been reported when rhBMP-2 has been used for spinal fusion surgery, this case demonstrates that it can occur to a much greater degree than previously seen. [ABSTRACT FROM AUTHOR]

Background: Endoscopic papillectomy (EP) offers a safe and effective method for resection of ampullary adenomas. Data regarding the long-term resolution of adenoma following EP are limited. The aim of this study therefore was to examine the timing of recurrence after EP of ampullary adenomas. Methods: This was a single-center retrospective study including patients who received EP for ampullary adenomas from 8/2000 to 1/2018. Patients with confirmed complete eradication of adenoma were included in the recurrence analysis with recurrence defined as finding adenomatous histology after 1 negative surveillance endoscopy. Kaplan–Meier estimates were calculated to determine recurrence rates. Results: Of the 165 patients who underwent EP, 136 patients (mean age 61.9, 51.5% female) had adenomatous histology with a mean lesion size of 21.2 mm. A total of 124 (91.2%) achieved complete eradication with a follow-up of 345.8 person-years. Recurrence occurred in 20 (16.1%) patients at a mean of 3.2 (± 3) years (range 0.5–9.75 years) for a recurrence rate of 5.8 (95% CI 3.6–8.8) per 100 person-years. Nine (45%) recurrences occurred after the 1st 2 years of surveillance. Recurrence rate did not differ by baseline pathology [low-grade dysplasia: 5.2 (95% CI 3.0–9.0), high-grade dysplasia: 6.9 (95% CI 2.3–15.5), adenocarcinoma: 7.7 (95% CI 0.9–25.1)]. Conclusion: Recurrence remains a significant concern after EP. Given the timing of recurrence, long surveillance periods may be necessary. Larger multicenter studies are needed, however, to determine appropriate surveillance intervals. [ABSTRACT FROM AUTHOR]

Background and Study Aim: The incidence of cholangiocarcinoma (CCA) in primary sclerosing cholangitis (PSC) ranges between 7 and 14 %. Despite using multiple tissue sampling modalities, detection of CCA remains a challenge. Probe-based confocal laser endomicroscopy (pCLE) has been utilized to visualize subepithelial biliary mucosa in patients with indeterminate strictures. We assessed the technical feasibility and operating characteristics of pCLE in a cohort of PSC patients with dominant biliary strictures (DS). Patients and Methods: This was a chart review of a prospectively maintained database at a single tertiary referral center of 15 PSC patients with 21 dominant stenoses undergoing pCLE. A data collection sheet included demographics, ERCP, cholangioscopy, pCLE (Miami criteria), tissue sampling results, and follow-up to 12 months or liver transplantation. Operating characteristics for pCLE and ERCP tissue sampling were calculated. Results: Sufficient visualization of DS by pCLE was achieved in 20/21 (95 %). pCLE sensitivity, specificity, PPV, and NPV were 100 % (95 % CI 19.3-100 %), 61.1 % (95 % CI 35.8-82.6 %), 22.2 % (95 % CI 3.5-59.9 %), and 100 % (95 % CI 71.3-100 %), respectively, in detecting neoplasia. In comparison, concomitant tissue sampling yielded sensitivity, specificity, PPV, and NPV of 0 % (95 % CI 0-80.7 %), 94.4 % (95 % CI 72.6-99.1 %), 0 % (95 % CI 0-83.5 %), and 89.5 % (95 % CI 66.8-98.4 %), respectively. Conclusions: pCLE achieves a high technical success rate in patients with PSC and DS. This single center, small series, suggests that pCLE may have a high sensitivity and negative predictive value to exclude neoplasia. If verified in larger prospective studies, the technology may be utilized to risk stratify dominant strictures in patients with PSC. [ABSTRACT FROM AUTHOR]

OBJECTIVES:Commercially available 10F cholangioscope systems have limitations in examination of the biliary tree. Further, they are not readily available in all endoscopy units. Direct cholangioscopy (DC) using slim and ultraslim gastroscopes have been utilized to detect and treat bile duct (BD) diseases. It often requires initial duodenoscope introduction, followed by over-the-wire exchange to a gastroscope for BD intubation. We report a novel single-step DC technique using forward-viewing endoscopes without requiring tandem-scope exchange.METHODS:In patients with native papilla, a 'J' maneuver, accomplished by retroflexing the endoscope in the second portion of the duodenum and withdrawing the retroflexed scope into the BD, was used to achieve free intubation. A variety of readily available standard endoscopes were used. For biliary-enteric anastomoses, balloon dilation, if necessary followed by enteroscope advancement, was utilized for BD visualization.RESULTS:A total of 18 patients underwent 22 DC procedures (8 male, 10 female, mean age 69 years). Direct intubation was successful in all procedures. Indications included BD stone (n=10), BD stricture or tumor (n=8). A native papilla was present in 13 patients and biliary-enteric anastomoses in 5; 8 patients had altered gastrointestinal anatomy. Sphincterotomy was required in 12/13 native papilla patients to facilitate DC. Free intubation of the BD was accomplished in 19 of 22 exams, and 3 facilitated by guidewire (over-the-wire in 2 and alongside a guidewire in 1). Overtube-assisted enteroscopy was used to reach the BD in four patients. The findings were stones (n=6), strictures or tumors (n=7), abnormal mucosa (n=5), a dilated duct confirming stone clearance without stricture (n=3), and retained stent (n=1). Interventions were biopsy (n=8), stone extraction (n=5), electrohydraulic lithotripsy (n=3), chromoendoscopy (n=2), narrow-band imaging (n=2), through the scope stricture dilation (n=2), and argon-plasma coagulation of biliary tumor (n=1). Complications were cholangitis managed with intavenous antibiotics (n=1).CONCLUSIONS:Single-step DC can be achieved without using tandem-scope exchange, and may be clinically useful for diagnostic and therapeutic purposes in managing select biliary tract diseases. [ABSTRACT FROM AUTHOR]

Anatomical variations of the median nerve and the persistent median artery (PMA) in the carpal tunnel (CT) are important to understand for their clinical and surgical significance. The aim of this cohort retrospective study was to investigate the prevalence of aberrant median nerve branches and persistent median artery in the CT in a selected population using magnetic resonance imaging (MRI). MR wrist images of 194 patients, 77 males and 117 females, aged 12–80 years were randomly selected and retrieved from our clinical and radiology data base. The MR examinations were performed using either 1.5-T or 3.0-T magnet using a cylindrical receive–transmit wrist coil for all cases. The course of the bifurcation of the median nerve was followed on axial T2-weighted and axial proton density fat saturated images and classified as either proximal, within, or distal to the CT. The flexor retinaculum proximally and the metacarpal bases were used as anatomic landmarks to subdivide these three categories. In addition, the median artery was searched in order to assess the prevalence of its presence inside the CT. A total of 194 wrists were analyzed by two musculoskeletal-trained radiologists. They were blinded on the population age, gender, and the sides of the wrists. Agreement was reached by consensus. Among the 194 wrists, there was bifurcation of the nerve proximal to the CT in 12 (6.1%) wrists. There was nerve bifurcation within the CT in 36 (18%) wrists. Nerve bifurcation distal to the CT was more frequently observed, occurring in 147 (75%) wrists. Only one nerve trifurcation was seen within the CT. There was no gender predominance for the nerve bifurcation within the tunnel. There were 107 right wrists and 87 left wrists. On the right side, bifurcation of the nerve within the CT was seen in 21 (19.6%) wrists; and on the left side bifurcation of the nerve was present in 15 (17.2%) wrists. Statistically, we found no significant difference in the prevalence of the bifid nerve within the tunnel in the subgroups based on age, gender, or side of the wrists. A persistent median artery (PMA) within the tunnel was observed in 21 (11%) wrists—10 males and 11 females. Of these, four (19%) cases were presented with coexistent PMA and bifid median nerve within the tunnel. Statistically, we found that the two variations are not independent traits, and their covariance is not null. There was a high prevalence of bifid median nerve (19%) and PMA (11%) within the tunnel regardless of gender or age. The PMA was more frequent on the left side. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Symptomatic cholelithiasis is a common disease in the general population with an increased prevalence in patients with cirrhosis. While cholecystectomy is the procedure of choice for the treatment of symptomatic cholelithiasis, cirrhotics have an increased risk of complications associated with this therapy. We have found that placement of an endoscopic gallbladder stent is an alternative, less invasive treatment for cirrhotic patients with symptomatic gallbladder disease and describe our experience here. METHODS: A retrospective medical record review of 23 patients with cirrhosis who underwent endoscopic retrograde cholangiography with gallbladder stent placement for symptomatic gallbladder disease from July 1994 to August 2004. RESULTS: Indications for stent placement included recurrent biliary colic (56.5%), acute calculous cholecystitis (39%), acalculous cholecystitis (8.6%), and gallstone pancreatitis (8.6%). All patients experienced resolution of their symptoms following stent placement. Twenty patients (87%) were asymptomatic from 5 days to 3 years post-procedure until transplantation, death, or end of study period. Nine patients (39%) underwent liver transplantation, 5 days to 34 months after the procedure. Eleven patients are well, with ten patients awaiting liver transplantation. Three patients developed late complications and were treated successfully with antibiotics. CONCLUSION: Endoscopic stenting of the gallbladder may be a potential treatment for symptomatic gallbladder disease in patients with cirrhosis awaiting liver transplantation, considered to be high-risk for cholecystectomy. [ABSTRACT FROM AUTHOR]

Background: Anticholinergic medications may increase risk of dementia and stroke, but prospective studies in healthy older people are lacking.Objective: Compare risk of incident dementia and stroke by anticholinergic burden among initially healthy older people.Design: Prospective cohort study.Setting: Primary care (Australia and USA).Participants: 19,114 community-dwelling participants recruited for the ASPREE trial, aged 70+ years (65+ if US minorities) without major cardiovascular disease, dementia diagnosis, or Modified Mini-Mental State Examination score below 78/100.Measurements: Baseline anticholinergic exposure was calculated using the Anticholinergic Cognitive Burden (ACB) score. Dementia was adjudicated using Diagnostic and Statistical Manual of Mental Disorders volume IV criteria, and stroke using the World Health Organization definition.Results: At baseline, 15,000 participants (79%) had an ACB score of zero, 2930 (15%) a score of 1-2, and 1184 (6%) a score of ≥ 3 (indicating higher burden). After a median follow-up of 4.7 years and adjusting for baseline covariates, a baseline ACB score of ≥ 3 was associated with increased risk of ischemic stroke (adjusted HR 1.58, 95% CI 1.06, 2.35), or dementia (adjusted HR 1.36, 95% CI 1.01, 1.82), especially of mixed etiology (adjusted HR 1.53, 95% CI 1.06, 2.21). Results were similar for those exposed to moderate/highly anticholinergic medications.Limitations: Residual confounding and reverse causality are possible. Assessment of dose or duration was not possible.Conclusions: High anticholinergic burden in initially healthy older people was associated with increased risk of incident dementia and ischemic stroke. A vascular effect may underlie this association. These findings highlight the importance of minimizing anticholinergic exposure in healthy older people. [ABSTRACT FROM AUTHOR]

Background: Biliary stricture evaluation with brush cytology and intraductal forceps biopsy carries a low sensitivity, but the combination of newer modalities may improve sensitivity. Aim: To determine whether the addition of advanced modalities increases diagnostic yield of ERCP-based sampling. Methods: This single-center retrospective study evaluates patients with biliary strictures sampled using brush cytology. Operating characteristics were calculated for individual and combinations of modalities including cholangioscopy, fluoroscopy- and cholangioscopy-directed intraductal biopsy, fluorescence in situ hybridization (FISH), and confocal laser endomicroscopy. Analyses under Standard Criteria (SC) included malignant results as "positive" and Expanded Criteria (EC) included "suspicious" and "high-grade dysplasia" results as "positive." Results: A total of 614 patients were included, and 354 (57.8%) received brush cytology alone, which had a sensitivity of 38.5% (SC) to 40.3% (EC) and a specificity of 97.8% (EC) to 99.3% (SC). Combining brush cytology with fluoroscopy-guided biopsy (n = 259, 42.2%) had a sensitivity of 62.5% (SC) to 67.9% (EC) and specificity of 90.2% (EC) to 96.7% (SC). Adding FISH to brush cytology had a sensitivity of 84.2% (SC) to 87.5% (EC) and specificity of 54.1% (SC and EC), while cholangioscopy visualization addition resulted in a sensitivity of 80.4% (SC) to 92.2% (EC) and specificity of 67.3% (EC) to 89.1% (SC). There were no significant differences in sensitivity and specificity using SC and EC. Conclusions: Brush cytology has a low sensitivity, but the addition of other modalities increases sensitivity. There was no difference in specificity between the SC and the EC, supporting the inclusion of "suspicious" impressions with malignant results at our center. [ABSTRACT FROM AUTHOR]

Background: Biliary-enteric anastomotic strictures (AS) in long-limb surgical biliary bypass (LLBB) require percutaneous transhepatic biliary drains (PTBD), enteroscopy-assisted ERCP (E-ERCP), or surgical revision.Aim: To compare E-ERCP and PTBD for AS treatment.Methods: E-ERCP stricturoplasty included dilation, cautery, and stent; PTBD included balloon dilation and serial drain upsizing events.Results: From May 2008 to October 2015, 71 patients (37 M, median age 52) had E-ERCP (n = 45) or PTBD (n = 26) for AS in Roux-en-Y hepaticojejunostomy: liver transplant (n = 28), cholecystectomy injury revision (n = 21), other (n = 13) or Whipple's resection (n = 9). Median follow-up is 11 months (range 1-56) in 67 (94%) patients. Technical success, clinical improvement, and adverse events between E-ERCP and PTBD were similar (76% vs. 77%, p = 0.89; 82% vs. 85%, p = 0.80, and 6% vs. 5%, p = 0.60, respectively). However, E-ERCP had fewer post-procedural hospitalization days (0.2 ± 0.65 vs. 4.5±10, p = 0.0001), mean procedures (4.4 ± 6.3 vs. 9.5 ± 8, p = 0.006), and median months of treatment to resolve AS (1, range 1-22 vs. 7, range 3-23; p = 0.003). Two patients in PTBD group required surgery.Conclusions: (1) Technical success and clinical improvement are seen in the majority of LLBB patients with biliary-enteric AS undergoing E-ERCP or PTBD. (2) E-ERCP is associated with fewer procedures, post-procedure hospitalization days, and months to resolve AS. When expertise is available, E-ERCP to identify and treat AS should be considered as an alternative to PTBD. [ABSTRACT FROM AUTHOR]

Background: Statin use has been frequently associated with depressive symptoms in an older population. However, the nature of this association is uncertain in the literature. In this study, we aimed to investigate the association of statin intake and the prevalence of depressive symptoms in healthy community-dwelling older adults living in Australia and the USA.Methods: We analysed baseline data from 19,114 participants, over 70 years of age (over 65 years of age, if from an ethnic minority). The association of self-reported statin use and prevalence of depressive symptoms, as measured by a validated depression scale [Center for Epidemiological Studies Depression Scale (CES-D 10)], was determined using logistic regression models. Multivariable logistic models were implemented to account for important demographics and other lifestyle and socioeconomic factors, such as sex, age, living status, education and smoking history.Results: A total of 5987 individuals were statin users. Of those, 633 (10.6%) had depressive symptoms (CES-D 10 cut-off ≥ 8), compared with 1246 (9.5%) of the non-statin users. In the unadjusted model, statin use was associated with an increase in prevalence of depressive symptoms (odds ratio 1.13, confidence interval 1.02-1.25, p = 0.02). However, after adjusting for important demographic and socioeconomic factors, the use of statins was not significantly associated with depressive symptoms (odds ratio 1.09, confidence interval 0.98-1.20, p = 0.11). In secondary analyses, only simvastatin was marginally associated with an increased prevalence of depressive symptoms. Statins were associated with a decreased prevalence of depressive symptoms in individuals with severe obesity (body mass index > 35 kg/m2) and an increased prevalence in participants between 75 and 84 years of age.Conclusion: This study in a large community-dwelling older population did not show any association of statins with late-life depressive symptoms, after accounting for important socioeconomic and demographic factors. Confounding by indication is an important issue to be addressed in future pharmacoepidemiologic studies of statins. [ABSTRACT FROM AUTHOR]

Purpose: To explore the relationship between sociodemographic and lifestyle variables with health-related quality of life (HRQoL) of a large cohort of 'healthy' older individuals.Methods: The sample included individuals aged 65+ years from Australia (N = 16,703) and the USA (N = 2411) enrolled in the ASPirin in Reducing Events in the Elderly (ASPREE) multicentre placebo-controlled trial study and free of cardiovascular disease, dementia, serious physical disabilities or 'fatal' illnesses. The associations with the physical (PCS) and mental component scores (MCS) of HRQoL (SF-12 questionnaire) were explored using multiple linear regression models from data collected at baseline (2010-2014).Results: The adjusted PCS mean was slightly higher in the USA (49.5 ± 9.1) than Australia (48.2 ± 11.6; p < 0.001), but MCS was similar in both samples (55.7 ± 7.5 and 55.7 ± 9.6, respectively; p = 0.603). Males, younger participants, better educated, more active individuals, or those currently drinking 1-2 alcoholic drinks/day showed a better HRQoL (results more evident for PCS than MCS), while current heavy smokers had the lowest physical HRQoL in both countries. Neither age, walking time, nor alcohol intake was associated with MCS in either cohort.Conclusions: Baseline HRQoL of ASPREE participants was higher than that reported in population-based studies of older individuals, but the associations between sociodemographic and lifestyle variables were consistent with the published literature. As the cohort ages and develops chronic diseases, ASPREE will be able to document HRQoL changes. [ABSTRACT FROM AUTHOR]

Considerable research has focused on patients with trinucleotide (CGG) repeat expansions in the fragile X mental retardation 1 ( FMR1) gene that fall within either the full mutation (>200 repeats) or premutation range (55-200 repeats). Recent interest in individuals with gray zone expansions (41-54 CGG repeats) has grown due to reported phenotypes that are similar to those observed in premutation carriers, including neurological, molecular, and cognitive signs. The purpose of this manuscript is to describe a series of adults with FMR1 alleles in the gray zone presenting with movement disorders or memory loss. Gray zone carriers ascertained in large FMR1 screening studies were identified and their clinical phenotypes studied. Thirty-one gray zone allele carriers were included, with mean age of symptom onset of 53 years in patients with movement disorders and 57 years in those with memory loss. Four patients were chosen for illustrative case reports and had the following diagnoses: early-onset Parkinson disease (PD), atypical parkinsonism, dementia, and atypical essential tremor. Some gray zone carriers presenting with parkinsonism had typical features, including bradykinesia, rigidity, and a positive response to dopaminergic medication. These patients had a higher prevalence of peripheral neuropathy and psychiatric complaints than would be expected. The patients seen in memory clinics had standard presentations of cognitive impairment with no apparent differences. Further studies are necessary to determine the associations between FMR1 expansions in the gray zone and various phenotypes of neurological dysfunction. [ABSTRACT FROM AUTHOR]

Background: Radiofrequency ablation of malignant biliary strictures has been offered for the last 3 years, but only limited data have been published. Aim: To assess the safety, efficacy, and survival outcomes of patients receiving endoscopic radiofrequency ablation. Methods: Between April 2010 and December 2013, 69 patients with unresectable neoplastic lesions and malignant biliary obstruction underwent 98 radiofrequency ablation sessions with stenting. Results: A total of 69 patients (22 male, aged 66.1 ± 13.3) were included in the registry. The etiology of malignant biliary stricture included unresectable cholangiocarcinoma ( n = 45), pancreatic cancer ( n = 19), gallbladder cancer ( n = 2), gastric cancer ( n = 1), and liver metastasis from colon cancer ( n = 3). Seventy-eight percentage of patients had prior chemotherapy. All strictures were stented post-radiofrequency ablation with either plastic stents or metal stents. The mean stricture length treated was 14.3 mm. There was a statistically significant improvement in stricture diameter post-ablation ( p < 0.0001). The likelihood of stricture improvement was significantly greater in pancreatic cancer-associated strictures [RR 1.8 (95 % 1.03-5.38)]. Seven patients (10 %) had adverse events, not linked directly to radiofrequency ablation. Median survival was 11.46 months (6.2-25 months). Conclusion: Radiofrequency ablation is effective and safe in malignant biliary obstruction and seems to be associated with improved survival. [ABSTRACT FROM AUTHOR]

Background: Techniques to confirm suspected pancreaticobiliary (PB) malignancy when index sampling is non-diagnostic include cholangiopancreatoscopy (CP) and endoscopic ultrasound (EUS). However, comparative data are lacking. Aim: The purpose of this study was to compare the yield of EUS and CP for the diagnosis of PB pathology. Methods: Consecutive patients with indeterminate PB pathology who underwent both CP and EUS within 3 months of each other were retrospectively identified. For CP, tissue sampling included biopsy under direct inspection (cholangioscopy-directed biopsy), biopsy following CP with fluoroscopic guidance (cholangioscopy-assisted biopsy), or brush cytology. For EUS-FNA, lesions included ductal strictures or hypoechoic masses. A comparison of operating characteristics between CP and EUS utilizing tissue confirmation or 12-month clinical course consistent with either benign or malignant disease was performed. Results: Between February 2000 and June 2007, 66 (33 males, 33 females, median age 64.5) patients with indeterminate PB pathology who had undergone both CP and EUS within 3 months of each other were included. Lesions amenable to sampling were noted in 59 CP and 50 EUS patients. On follow-up, 39 patients had neoplasia and 27 were benign. The sensitivity/specificity for the diagnosis of neoplasia for CP and EUS was 48.7/96.3 % and 33.3/96.3 %, respectively (comparison of sensitivities, P = 0.183). The combined (CP and EUS) sensitivity/specificity was 66.7/96.3 % ( P = 0.0064 and P = 0.0001 comparing combined sensitivity vs. sensitivity of either CP alone or EUS alone, respectively). Conclusions: In patients who undergo both EUS and CP for indeterminate PB pathology, the combined yield of EUS and CP to detect neoplasia appears to be higher than either examination alone. [ABSTRACT FROM AUTHOR]

BACKGROUND: Argon plasma coagulation (APC) has been used to trim uncovered Elgiloy® stents, but the extent of thermal damage and bile duct injury is not known. The goal of this study was to evaluate the safety and efficacy of APC for this application. METHODS: Eight Elgiloy® (covered and uncovered) and four nitinol stents were deployed in the bile duct at ERCP in 12 anesthetized pigs. In nine pigs, the excess distal ends were trimmed in vivo using short bursts of APC. Three pigs served as controls. Bile ducts and stent specimens were then harvested for gross and histological examinations by a single-blinded pathologist. RESULTS: APC effectively trimmed all the stents. Seven APC-treated bile ducts and three controls showed epithelial distortion consistent with pressure injury from stent expansion. Two APC-treated bile ducts showed mild thermal injury. The damage was superficial, extending to a maximum depth of 0.1 mm with rare foci involving subepithelial connective tissue. CONCLUSION: APC at indicated settings effectively cuts covered and uncovered Elgiloy® as well as nitinol stents, but can cause biliary epithelial injury secondary to conduction of heat and electrical energy. Proper technique and settings should be followed and short bursts of energy judiciously applied in order to minimize this danger. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Assess the role of cholangioscopy in primary sclerosing cholangitis for 1) detection of cholangiocarcinoma using cholangioscopy-assisted biopsy 2) detection of stones not seen on cholangiography 3) stone removal with cholangioscopy-directed lithotripsy. METHODS: Prospective cohort of consecutive patients referred for cholangioscopy to evaluate dominant strictures or stones. A data collection sheet was employed. Follow-up was by chart review/phone contact. Clinical improvement was defined as resolution of jaundice or ≥50% reduction in pain or cholangitis episodes requiring hospitalization. RESULTS: 41 patients (30M, 11F) had 60 cholangioscopy procedures (55 per oral, 5 percutaneous). 33/41 (80%) patients underwent 44 tissue sampling events. Histology: positive for extrahepatic cholangiocarcinoma (N = 1), negative/atypical (N = 31), and inadequate (N = 1). Stones were found in 23/41 (56%) patients, of which 7/23 (30%) were missed on cholangiography and detected only by cholangioscopy. 9/23 (39%) underwent cholangioscopy-directed lithotripsy. Stone clearance: complete (N = 10, 7 by cholangioscopy-directed lithotripsy after failed conventional stone extraction); partial (N = 7); and not attempted (N = 6). Median follow-up was 17.0 months (range 1–56). Clinical improvement was achieved in 25/40 (63%). Eight patients have undergone transplant and cholangiocarcinoma was present in the explant of two at 1 and 12 months post-cholangioscopy, respectively. CONCLUSIONS: This is the first series of patients with primary sclerosing cholangitis undergoing cholangioscopy for the evaluation of dominant strictures and cholangioscopy-directed stone therapy with demonstrable clinical benefits. Stones detected by cholangioscopy were missed by cholangiography in nearly one of three patients. Cholangioscopy-directed lithotripsy may be superior to conventional ERCP for achieving complete stone clearance. Despite the use of cholangioscopy, diagnosis of cholangiocarcinoma remains technically challenging. [ABSTRACT FROM AUTHOR]