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Wilson's disease (WD) can present with liver disease, neurological deficits, and psychiatric disorders. Results of genetic prevalence studies suggest that WD might be much more common than previously estimated. Early recognition of WD remains challenging because it is a great imitator and requires a high index of suspicion for correct and timely diagnosis. Early diagnosis of WD is crucial to ensure that patients can be started on adequate treatment. In association with other clinical and biochemical tests, liver biopsy results and molecular genetic testing can also be used for diagnosing WD. Medical therapy is effective for most patients; liver transplant can rescue those with acute liver failure or those with advanced liver disease who fail to respond to or discontinue medical therapy. Although novel therapies, such as gene therapy, are on the horizon, screening and prevention of delayed diagnosis remains paramount.
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Degeneração Hepatolenticular , Transtornos Mentais , Humanos , Degeneração Hepatolenticular/diagnóstico , Degeneração Hepatolenticular/genética , Degeneração Hepatolenticular/terapia , Biópsia , Terapia GenéticaRESUMO
DESCRIPTION: Endoscopic gallbladder drainage is a feasible and efficacious alternative to percutaneous drainage in the management of acute cholecystitis for high-risk surgical candidates. Endoscopic ultrasound-guided gallbladder drainage and per-oral cholecystoscopy is facilitated by the use of lumen-apposing metal stents. Endoscopic ultrasound-guided gallbladder drainage should be performed by those expert in advanced therapeutic endoscopic ultrasound. Multidisciplinary collaboration between interventional radiology and surgery is paramount in the care of these patients. Choosing the optimal drainage method is dependent on individual patient characteristics. METHODS: This commentary was drawn from a review of the literature to provide practical advice. Because this was not a systematic review, we did not perform any formal rating of the quality of evidence or strength of the presented considerations. This expert commentary was commissioned and approved by the American Gastroenterological Association (AGA) Institute Clinical Practice Updates Committee and the AGA Governing Board to provide timely guidance on a topic of high clinical importance to the AGA membership, and underwent internal peer-review by the Clinical Practice Updates Committee and external peer-review through standard procedures of Clinical Gastroenterology and Hepatology.
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Colecistite Aguda , Vesícula Biliar , Humanos , Vesícula Biliar/diagnóstico por imagem , Vesícula Biliar/cirurgia , Colecistite Aguda/diagnóstico por imagem , Colecistite Aguda/cirurgia , Endossonografia/métodos , Drenagem/métodos , Endoscopia/métodos , Stents , Resultado do TratamentoRESUMO
Cholangiocarcinoma is a lethal malignancy of the biliary epithelium that can arise anywhere along the biliary tract. Surgical resection confers the greatest likelihood of long-term survivability. However, its insidious onset, difficult diagnostics, and resultant advanced presentation render the majority of patients unresectable, highlighting the importance of early detection with novel biomarkers. Developing liver-directed therapies and emerging targeted therapeutics may offer improved survivability for patients with unresectable or advanced disease. In this article, the authors review the current multidisciplinary standards of care in resectable and unresectable cholangiocarcinoma, with an emphasis on novel biomarkers for early detection and nonsurgical locoregional therapy options.
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Neoplasias dos Ductos Biliares , Colangiocarcinoma , Humanos , Ductos Biliares Intra-Hepáticos/patologia , Neoplasias dos Ductos Biliares/terapia , Neoplasias dos Ductos Biliares/patologia , Colangiocarcinoma/terapia , Colangiocarcinoma/patologiaRESUMO
BEST PRACTICE ADVICE STATEMENTS: BEST PRACTICE ADVICE 1: Forceps bite-on-bite or deep-well biopsies or tunnel biopsies can sometimes establish a pathologic diagnosis of SEL. BEST PRACTICE ADVICE 2: EUS is the modality of choice to evaluate indeterminate SEL of the GI track and/or if non-diagnostic tissue by forceps biopsies. BEST PRACTICE ADVICE 3: SEL arising from the submucosa can be sampled using tunnel biopsies (or deep-well biopsies), EUS guided fine-needle aspiration (FNA), EUS guided fine-needle biopsy (FNB), or advanced endoscopic techniques (unroofing or endoscopic submucosal resection). BEST PRACTICE ADVICE 4: SEL arising from muscularis propria should be sampled (preferably using FNB or FNA) to determine whether the lesion is a GIST or leiomyoma. Structural assessment and staining will allow differentiation of mesenchymal tumors and assessing their malignant potential. BEST PRACTICE ADVICE 5: Endoscopic resection techniques have been described for removal of SEL and should be limited to endoscopists skilled in advanced tissue resection techniques. BEST PRACTICE ADVICE 6: Management of each SEL depends on the size of the lesion, histopathology, their malignant potential, and presence of symptoms. BEST PRACTICE ADVICE 7: SEL that have an endoscopic appearance consistent with a lipoma or pancreatic rest and normal mucosal biopsies do not need further evaluation or surveillance. BEST PRACTICE ADVICE 8: For SEL arising from muscularis propria that are less than 2 cm in size, surveillance using EUS should be considered. BEST PRACTICE ADVICE 9: Gastric GIST larger than 2 cm should be considered for resection. BEST PRACTICE ADVICE 10: Subepithelial lesions that are ulcerated, bleeding, or causing symptoms should be considered for resection.
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Tumores do Estroma Gastrointestinal , Neoplasias Gástricas , Humanos , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico/métodos , Endoscopia Gastrointestinal/métodos , Tumores do Estroma Gastrointestinal/patologia , Pâncreas/patologia , Neoplasias Gástricas/patologiaAssuntos
Neoplasias Colorretais/fisiopatologia , Endoscopia/métodos , Linfoma de Célula do Manto/diagnóstico por imagem , Rituximab/uso terapêutico , Ultrassonografia de Intervenção/métodos , Humanos , Linfoma de Célula do Manto/complicações , Linfoma de Célula do Manto/patologia , Masculino , Pessoa de Meia-Idade , Rituximab/farmacologiaRESUMO
PURPOSE OF REVIEW: Pancreatic duct stones are sequela of chronic pancreatitis. They can cause pancreatic duct obstruction which is the most important cause of pain in chronic pancreatitis. Stone resolution has shown to improve pain. The goal of this review is to highlight recent endoscopic and surgical advancements in treatment of pancreatic duct stones. RECENT FINDINGS: Stone fragmentation by extracorporeal shock wave lithotripsy has become first line and the mainstay of treatment for majority of patients with pancreatic duct stones. Introduction of digital video pancreatoscopy in the last few years with the capability of guided lithotripsy has provided a robust therapeutic option where extracorporeal shock wave lithotripsy is unsuccessful or unavailable. Historically, surgery has been considered a more reliable and durable option when feasible. However, it had not been compared with more effective endoscopic therapy. Lithotripsy (extracorporeal and pancreatoscopy guided) is evolving as a strong treatment modality for pancreatic stones.
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Cálculos/terapia , Pancreatopatias/terapia , Ductos Pancreáticos , Cálculos/etiologia , Colangiopancreatografia Retrógrada Endoscópica/métodos , Humanos , Terapia a Laser/métodos , Litotripsia/efeitos adversos , Litotripsia/métodos , Pancreatite Crônica/complicações , PrognósticoRESUMO
PURPOSE OF REVIEW: The emphasis of this review article is to bring to the forefront the spectrum of pancreatobiliary disorders and their association with diabetes. We hope to systematically discuss the link between diabetes and disorders of the pancreas including acute and chronic pancreatitis, as well as pancreatic cancer. We also hope to review and highlight the literature with respect to other cancers of the biliary tree including gallbladder and cholangiocarcinoma. Lastly, we discuss diabetes and biliary dyskinesia. RECENT FINDINGS: Newer cohort studies suggest the presence of diabetes to increase the severity and worsen outcomes in acute pancreatitis. Post pancreatitis diabetes is a novel disease entity being increasingly recognized. The use of pancreatic enzyme replacement therapy (PERT) in chronic pancreatitis may attenuate the risk of endocrine dysfunction. Pancreatic cancer may either be the consequence of diabetes or a presenting manifestation. The use of anti-diabetic medications may confer protection against development of pancreatic cancer. Gallbladder adenocarcinoma and cholangiocarcinoma risks are elevated in those with underlying diabetes. Diabetes can directly impact or deteriorate outcome of acute and chronic pancreatitis and well as pancreatobiliary malignancies. It could also occur as a result of loss of endocrine function after pancreatitis. Like many areas of the digestive tract, biliary motility could be impacted by diabetes.
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Automatic diagnosis tool helps physicians to evaluate capsule endoscopic examinations faster and more accurate. The purpose of this study was to evaluate the validity and reliability of an automatic post-processing method for identifying and classifying wireless capsule endoscopic images, and investigate statistical measures to differentiate normal and abnormal images. The proposed technique consists of two main stages, namely, feature extraction and classification. Primarily, 32 features incorporating four statistical measures (contrast, correlation, homogeneity and energy) calculated from co-occurrence metrics were computed. Then, mutual information was used to select features with maximal dependence on the target class and with minimal redundancy between features. Finally, a trained classifier, adaptive neuro-fuzzy interface system was implemented to classify endoscopic images into tumor, healthy and unhealthy classes. Classification accuracy of 94.2% was obtained using the proposed pipeline. Such techniques are valuable for accurate detection characterization and interpretation of endoscopic images.
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BACKGROUND AND AIMS: The ability to safely and effectively obtain sufficient tissue for pathologic evaluation by using endoscopic ultrasound (EUS) guidance remains a challenge. Novel designs in EUS needles may provide for improved ability to obtain such core biopsies. The aim of this study was to evaluate the diagnostic yield of core biopsy specimens obtained using a novel EUS needle specifically designed to obtain core biopsies. PATIENTS AND METHODS: Multicenter retrospective review of all EUS-guided fine-needle biopsies obtained using a novel biopsy needle (SharkCore FNB needle, Medtronic, Dublin, Ireland). Data regarding patient demographics, lesion type/location, technical parameters, and diagnostic yield was obtained. RESULTS: A total of 250 lesions were biopsied in 226 patients (Median age 66 years; 113 (50â%) male). Median size of all lesions (mm): 26 (2â-â150). Overall, a cytologic diagnosis was rendered in 81â% specimens with a median number of 3 passes. When rapid onsite cytologic evaluation (ROSE) was used, cytologic diagnostic yield was 126/149 (85â%) with a median number of 3 passes; without ROSE, cytologic diagnostic yield was 31/45 (69â%, Pâ=â0.03) with a median number of 3 passes. Overall, a pathologic diagnosis was rendered in 130/147 (88â%) specimens with a median number of 2 passes. Pathologic diagnostic yield for specific lesion types: pancreas 70/81 (86â%), subepithelial lesion 13/15 (87â%), lymph node 26/28 (93â%). Ten patients (10/226, 4â%) experienced adverse events: 4 acute pancreatitis, 5 pain, 1 fever/cholangitis. CONCLUSIONS: Initial experience with a novel EUS core biopsy needle demonstrates excellent pathologic diagnostic yield with a minimum number of passes.
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Introduction. Large volume paracentesis is considered a safe procedure carrying minimal risk of complications and rarely causing morbidity or mortality. The most common complications of the procedure are ascitic fluid leakage, hemorrhage, infection, and perforation. The purpose of this study was to evaluate all hemorrhagic complications and their outcomes and to identify any common variables. Methods. A literature search for all reported hemorrhagic complications following paracentesis was conducted. A total of 61 patients were identified. Data of interest were extracted and analyzed. The primary outcome of the study was 30-day mortality, with secondary endpoints being achievement of hemostasis after intervention and mortality based on type of intervention. Results. 90% of the patients undergoing paracentesis had underlying cirrhosis. Three types of hemorrhagic complications were identified: abdominal wall hematomas (52%), hemoperitoneum (41%), and pseudoaneurysm (7%). Forty percent of the patients underwent either a surgical (35%) or an IR guided intervention (65%). Patients undergoing a surgical intervention had a significantly higher rate of mortality at day 30 compared to those undergoing IR intervention. Conclusion. Abdominal wall hematomas and hemoperitoneum are the most common hemorrhagic complications of paracentesis. Transcatheter coiling and embolization appear to be superior to both open and laparoscopic surgery in treatment of these complications.
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BACKGROUND AND AIM: The double layer stent (DLS) has a unique design and has been used for palliation of malignant biliary obstruction, but literature on this stent is limited. Our aim was to compare plastic (PS), DLS and metal stents (MS) in terms of complication rates, time to occlusion, and patency rate in patients with malignant biliary obstruction (MBO). METHODS: A retrospective review of stents placed for MBO at our institution in the period between January 2009 and April 2011 was conducted. A total of 114 stents were identified, of which 44 were MS (39 %), 37 DLS (32 %), and 33 PS (29 %). A stent was considered occluded when an unplanned stent removal or intervention occurred due to clinical suspicion of biliary obstruction. RESULTS: Stents remained patent for 95 days (range 7-359 days) in the DLS group and 59 days (range 7-228 days) in the PS group (P = 0.014) and 128.7 days (range 4-602 days) in the metal stent group. Twenty-seven percent (n = 9) of PS occluded after a mean of 60 days while 16 % (n = 7) of MS occluded after a mean of 87 days and 5 % (n = 2) of DLS occluded after a mean of 85 days (DLS vs. PS P = 0.012, DLS vs. MS P = 0.13, MS vs. PS P = 0.22). CONCLUSIONS: DLS are superior to PS in patients with MBO and appear to be comparable to MS. MS had a longer patency rate but were comparable to DLS in early and late complications. We speculate that the less expensive DLS may be a cost effective alternative in the palliation of MBO.