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cystic echinococcosis of the liver: A primer for hepatologists

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World Journal of Hepatology 2014年第5期6卷 293-305页

作者： Francesca Rinaldi Enrico Brunetti Andreas Neumayr Marcello Maestri Samuel Goblirsch Francesca Tamarozzi Division of Infectious and Tropical Diseases San Matteo Hospital Foundation 27100 Pavia Italy Department of Clinical Surgical Diagnostic and Paediatric Sciences University of Pavia WHO Collaborating Centre for the Clinical Management of Cystic Echinococcosis 27100 Pavia Italy Swiss Tropical and Public Health Institute 4002 Basel Switzerland University of Basel 4003 Basel Switzerland Division of General Surgery University of Pavia San Matteo Hospital Foundation 27100 Pavia Italy Emergency Medicine Winona Health Services Winona MN 55987 United States

cystic echinococcosis(CE) is a complex, chronic and neglected disease with a worldwide distribution. The liver is the most frequent location of parasitic cysts. In humans, its clinical spectrum ranges from asymptom-atic infection to severe, potentially fatal disease. Four approaches exist in the clinical management of CE: surgery, percutaneous techniques and drug treatment for active cysts, and the "watch and wait" approach for inactive cysts. Allocation of patients to these treat-ments should be based on cyst stage, size and location, available clinical expertise, and comorbidities. However, clinical decision algorithms, efficacy, relapse rates, and costs have never been properly evaluated. This paper reviews recent advances in classification and diagnosisand the currently available evidence for clinical deci-sion-making in cystic echinococcosis of the liver.

关键词： cystic echinococcosis Hydatidosis Clinical management Diagnosis Treatment Surgery Albenda-zole Watch-and-wait Follow-up Percutaneous treatment

cystic duct cancer: Should it be deemed as a type of gallbladder cancer?

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World Journal of Gastroenterology 2019年第44期25卷 6541-6550页

作者： Tu-Nan Yu Ying-Ying Mao Fang-Qiang Wei Hui Liu Department of General Surgery Sir Run Run Shaw HospitalSchool of MedicineZhejiang UniversityHangzhou 310016Zhejiang ProvinceChina Department of Epidemiology and Biostatistics Zhejiang Chinese Medical UniversityHangzhou 310053Zhejiang ProvinceChina Department of Hepatobiliary and Pancreatic Surgery Zhejiang Provincial People’s HospitalHangzhou Medical CollegeHangzhou 310014Zhejiang ProvinceChina Zhejiang Provincial Key Laboratory of Laparoscopic Technology Sir Run Run Shaw HospitalSchool of MedicineZhejiang UniversityHangzhou 310016Zhejiang ProvinceChina

BACKGROUND According to the latest American Joint Committee on Cancer and Union for International Cancer Control manuals,cystic duct cancer(CC)is categorized as a type of gallbladder cancer(GC),which has the worst prognosis among all types of biliary cancers.We hypothesized that this categorization could be verified by using taxonomic methods.AIM To investigate the categorization of CC based on population-level data.METHODS Cases of biliary cancers were identified from the Surveillance,Epidemiology,and End Results 18 registries database.Together with routinely used statistical methods,three taxonomic methods,including Fisher’s discriminant,binary logistics and artificial neuron network(ANN)models,were used to clarify the categorizing problem of CC.RESULTS The T staging system of perihilar cholangiocarcinoma[a type of extrahepatic cholangiocarcinoma(EC)]better discriminated CC prognosis than that of GC.After adjusting other covariates,the hazard ratio of CC tended to be closer to that of EC,although not reaching statistical significance.To differentiate EC from GC,three taxonomic models were built and all showed good accuracies.The ANN model had an area under the receiver operating characteristic curve of 0.902.Using the three models,the majority(75.0%-77.8%)of CC cases were categorized as EC.CONCLUSION Our study suggested that CC should be categorized as a type of EC,not GC.Aggressive surgical attitude might be considered in CC cases,to see whether long-term prognosis could be immensely improved like the situation in EC.

关键词： cystic duct cancer Gallbladder cancer Extrahepatic cholangiocarcinoma Surgical management Taxonomy Categorization

cystic fibrosis transmembrane conductance regulator functional evaluations in a G542X+/-IVS8Tn:T7/9 patient with acute recurrent pancreatitis

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World Journal of Clinical Cases 2019年第22期7卷 3757-3764页

作者： Sara Caldrer Gabriella Bergamini Angela Sandri Silvia Vercellone Luca Rodella Angelo Cerofolini Francesco Tomba Filippo Catalano Luca Frulloni Mario Buffelli Gloria Tridello Hugo de Jonge Baroukh Maurice Assael Claudio Sorio Paola Melotti Department of Medicine General Pathology DivisionCystic Fibrosis Translational Research Laboratory“D.Lissandrini”University of VeronaVerona 37134Italy cystic Fibrosis Center Azienda Ospedaliera Universitaria Integrata VeronaVerona 37126Italy Endoscopic Surgery Unit Azienda Ospedaliera Universitaria Integrata VeronaVerona 37126Italy Department of Medicine Pancreas CenterUniversity of VeronaVerona 37134Italy Department of Neurosciences Biomedicine and Movement SciencesPhysiology Section UniversityVerona 37126Italy Gastroenterology and Hepatology Erasmus University Medical CenterRotterdam 3015Netherlands

BACKGROUND Acute recurrent pancreatitis(ARP)is characterized by episodes of acute pancreatitis in an otherwise normal gland.When no cause of ARP is identifiable,the diagnosis of"idiopathic"ARP is given.Mutations in the cystic fibrosis transmembrane conductance regulator(CFTR)gene increase the risk of ARP by 3-to 4-times compared to the general population,while cystic fibrosis(CF)patients present with a 40-to 80-times higher risk of developing pancreatitis.CASE SUMMARY In non-classical CF or CFTR-related disorders,CFTR functional tests can help to ensure a proper diagnosis.We applied an individualized combination of standardized and new CFTR functional bioassays for a patient referred to the Verona CF Center for evaluation after several episodes of acute pancreatitis.The CFTR genotype was G542X+/-with IVS8Tn:T7/9 polymorphism.The sweat(Cl-)values were borderline.Intestinal current measurements were performed according to the European cystic Fibrosis Society Standardized Operating Procedure.Recent nasal surgery for deviated septum did not allow for nasal potential difference measurements.Lung function and sputum cultures were normal;azoospermia was excluded.Pancreas divisum was excluded by imaging but hypoplasia of the left hepatic lobe was detected.Innovative tests applied in this case include sweat rate measurement by image analysis,CFTR function in monocytes evaluated using a membrane potential-sensitive fluorescent probe,and the intestinal organoids forskolin-induced swelling assay.CONCLUSION Combination of innovative CFTR functional assays might support a controversial diagnosis when CFTR-related disorders and/or non-classical CF are suspected.

关键词： Recurrent acute pancreatitis cystic fibrosis cystic fibrosis transmembraneconductance regulator function Intestinal current Organoids Sweat test Controversialdiagnosis Case report

Molecular markers contribute to the clinical diagnosis for pancreatic cystic neoplasms

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Chinese Medical Journal 2020年第7期133卷 847-852页

作者： Zhen Liu Shu Zhang School of Medicine and Life Sciences University of JinanShandong Academy of Medical SciencesJinanShandong 250200China Department of Medical Oncology Shandong Cancer Hospital and InstituteShandong First Medical University and Shandong Academy of Medical SciencesJinanShandong 250117China

A pancreatic cystic neoplasm(PCN)is a rare pancreatic disease.Malignant PCNs are usually identified incidentally while evaluating other lesions.However,PCNs are being identified more frequently owing to the increased use of abdominal imaging.Malignant PCNs have complicated and diverse biological behaviors,including various malignant risk factors,diverse molecular features,natural history,and complex pathological classifications.Although many diagnostic methods,such as cross-sectional imaging and endoscopic evaluation,have been developed,malignant PCNs are still difficult to differentiate from benign tumors.On searching for related articles in the recent decade,we found that some molecular biomarkers such as carcinoembryonic antigen could be useful for discriminating between malignant tumors and benign tumors.However,cytopathologic evaluation is the most useful method for differentiating between benign and malignant lesions.Although cytopathologic evaluation has a specificity of 100%for identifying malignancies,its accuracy is often hampered by the low cellularity of PCN cells in the cystic fluid.Herein,we review the progress in the use of cellular and molecular markers for the accurate identification of PCNs.

关键词： Pancreatic cystic neoplasm Biomarker Diagnosis

cystic lesion around the hip joint

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World Journal of Orthopedics 2015年第9期6卷 688-704页

作者： Kiminori Yukata Sho Nakai Tomohiro Goto Yuichi Ikeda Yasunori Shimaoka Issei Yamanaka Koichi Sairyo Jun-ichi Hamawaki Department of Orthopedic Surgery Hamawaki Orthopaedic Hospital Department of Orthopaedics Tokushima University Hospital

This article presents a narrative review of cystic lesions around the hip and primarily consists of 5 sections:Radiological examination, prevalence, pathogenesis, symptoms, and treatment. cystic lesions around the hip are usually asymptomatic but may be observed incidentally on imaging examinations, such as computed tomography and magnetic resonance imaging. Some cysts may enlarge because of various pathological factors, such as trauma, osteoarthritis, rheumatoid arthritis, or total hip arthroplasty(THA), and may become symptomatic because of compression of surrounding structures, including the femoral, obturator, or sciatic nerves, external iliac or common femoral artery, femoral or external iliac vein, sigmoid colon, cecum, small bowel, ureters, and bladder. Treatment for symptomatic cystic lesions around the hip joint includes rest, nonsteroidal anti-inflammatory drug administration, needle aspiration, and surgical excision. Furthermore, when these cysts are associated with osteoarthritis, rheumatoid arthritis, and THA, primary or revision THA surgery will be necessary concurrent with cyst excision. Knowledge of the characteristic clinical appearance of cystic masses around the hip will be useful for determining specific diagnoses and treatments.

关键词： Bursa cystic lesion Ganglion cyst Hip Synovial cyst

Meta-analysis Reveals Potential Influence of Oxidative Stress on the Airway Microbiomes of cystic Fibrosis Patients

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Genomics, Proteomics & Bioinformatics 2019年第6期17卷 590-602页

作者： Xing Shi Zhancheng Gao Qiang Lin Liping Zhao Qin Ma Yu Kang Jun Yu CAS Key Laboratory of Genome Sciences and Information Beijing Institute of GenomicsChinese Academy of SciencesBeijing 100101China University of Chinese Academy of Sciences Beijing 100190China Department of Respiratory&Critical Care Medicine Peking University People’s HospitalBeijing 100044China Department of Biochemistry and Microbiology School of Environmental and Biological SciencesRutgersThe State University of New JerseyNew BrunswickNJ 08901USA Bioinformatics and Mathematical Biosciences Lab Department of AgronomyHorticulture and Plant Science and Department of Mathematics and StatisticsSouth Dakota State UniversityBrookingsSD 57007USA

The lethal chronic airway infection of the cystic fibrosis(CF) patients is predisposed by colonization of specific CF-philic pathogens or the CF microbiomes, but key processes and reasons of the microbiome settlement in the patients are yet to be fully understood, especially their survival and metabolic dynamics from normal to diseased status under treatment. Here, we report our meta-analysis results on CF airway microbiomes based on metabolic networks reconstructed from genome information at species level. The microbiomes of CF patients appear to engage much more redox-related activities than those of controls, and by constructing a large dataset of anti-oxidative stress(anti-OS) genes, our quantitative evaluation of the anti-OS capacity of each bacterial species in the CF microbiomes confirms strong conservation of the anti-OS responses within genera and also shows that the CF pathogens have significantly higher anti-OS capacity than commensals and other typical respiratory pathogens. In addition, the anti-OS capacity of a relevant species correlates with its relative fitness for the airways of CF patients over that for the airways of controls.Moreover, the total anti-OS capacity of the respiratory microbiome of CF patients is collectively higher than that of controls, which increases with disease progression, especially after episodes of acute exacerbation and antibiotic treatment. According to these results, we propose that the increased OS in the airways of CF patients may play an important role in reshaping airway microbiomes to a more resistant status that favors the pre-infection colonization of the CF pathogens for a higher anti-OS capacity.

关键词： cystic fibrosis Airway microbiome Meta-analysis ROS Respiratory pathogens

c.753＿754delAG, a novel CFTR mutation found in a Chinese patient with cystic fibrosis: A case report and review of the literature

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World Journal of Clinical Cases 2019年第15期7卷 2110-2119页

作者： Yu-Qing Wang Chuang-Li Hao Wu-Jun Jiang Yan-Hong Lu Hui-Quan Sun Chun-Yan Gao Min Wu Department of Respiratory Medicine Children’s Hospital of Soochow University

BACKGROUND cystic fibrosis(CF)is rare in Asian populations relative to the Caucasian population.In this paper,we report the cystic fibrosis transmembrane conductance regulator(CFTR)variation in a family of Chinese CF patients,and systematically review the previous literature.CASE SUMMARY Here we report a 30-month-old Chinese girl who was diagnosed with CF based on her history and symptoms such as recurrent productive cough,wheezing with repeated infection of Pseudomonas aeruginosa,and parasinusitis.Chest computed tomography(CT)scanning revealed obvious exudative lesions and bilateral bronchiectasis.Liver CT scanning revealed a low-density lesion in the left lobe of the liver.A diagnosis of CF was made based upon CFTR gene tests.The CFTR gene was sequenced using the blood samples of her and her parents and showed a heterozygous novel missense mutation of c.753_754delAG in exon 7.In addition,a heterozygous c.1240 C>T mutation was found in exon 10 of the CFTR.The mutation c.753_754delAG was verified to have been inherited from her mother,and the c.1240 C>T mutation was from her father who was diagnosed with congenital absence of vas deferens.CONCLUSION A novel mutation of CFTR,c.753_754delAG,was found in a Chinese CF child.c.2909G>A is the most common mutation among Chinese CF patients.

关键词： cystic fibrosis cystic fibrosis transmembrane conductance regulator Mutation Chinese children Case report

Role of abdominal ultrasound for the surveillance follow-up of pancreatic cystic neoplasms: a cost-effective safe alternative to the routine use of magnetic resonance imaging

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World Journal of Gastroenterology 2019年第18期25卷 2217-2228页

作者： Luca Morelli Simone Guadagni Valerio Borrelli Roberta Pisano Gregorio Di Franco Matteo Palmeri Niccolò Furbetta Dario Gambaccini Santino Marchi Piero Boraschi Luca Bastiani Alessandro Campatelli Franco Mosca Giulio Di Candio General Surgery Unit Department of Surgery Translational and New Technologies in Medicine University of Pisa EndoCAS (Center for Computer Assisted Surgery) University of Pisa Diagnostic and Interventional Ultrasound in Transplants Unit Azienda Ospedaliero-Universitaria Pisana Gastroenterology Unit University of Pisa 2~(nd) Radiology Unit Department of Diagnostic Imaging Azienda Ospedaliero-Universitaria Pisana Institute of Clinical Physiology National Council of Research

BACKGROUND Patients with pancreatic cystic neoplasms(PCN), without surgical indication at the time of diagnosis according to current guidelines, require lifetime imagebased surveillance follow-up. In these patients, the current European evidencedbased guidelines advise magnetic resonance imaging(MRI) scanning every 6 mo in the first year, then annually for the next five years, without reference to any role for trans-abdominal ultrasound(US). In this study, we report on our clinical experience of a follow-up strategy of image-based surveillance with US, and restricted use of MRI every two years and for urgent evaluation whenever suspicious changes are detected by US.AIM To report the results and cost-efficacy of a US-based surveillance follow-up for known PCNs, with restricted use of MRI.METHODS We retrospectively evaluated the records of all the patients treated in our institution with non-surgical PCN who received follow-up abdominal US and restricted MRI from the time of diagnosis, between January 2012 and January 2017. After US diagnosis and MRI confirmation, all patients underwent US surveillance every 6 mo for the first year, and then annually. A MRI scan was routinely performed every 2 years, or at any stage for all suspicious US findings.In this communication, we reported the clinical results of this alternative followup, and the results of a comparative cost-analysis between our surveillance protocol(abdominal US and restricted MRI) and the same patient cohort that has been followed-up in strict accordance with the European guidelines recommended for an exclusive MRI-based surveillance protocol.RESULTS In the 5-year period, 200 patients entered the prescribed US-restricted MRI surveillance follow-up. Mean follow-up period was 25.1 ± 18.2 mo. Surgery was required in two patients(1%) because of the appearance of suspicious features at imaging(with complete concordance between the US scan and the on-demand MRI). During the follow-up, US revealed changes in PCN appearance in 28 patients(14%). These comprised

关键词： Ultrasound Pancreatic cystic neoplasms Magnetic resonance imaging Surveillance

Management of mucinous cystic neoplasms of the pancreas

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World Journal of Gastroenterology 2010年第45期16卷 5682-5692页

作者： Mario Testini Angela Gurrado Germana Lissidini Pietro Venezia Luigi Greco Giuseppe Piccinni Unit of Endocrine Digestive and Emergency SurgeryDepartment of Biomedical Sciences and Human OncologySection of General and Oncologic SurgeryUniversity Medical School of Bari70124 BariItaly Department of Emergency and Organ Transplantation University Medical School of Bari70124 BariItaly

The purpose of this study was to investigate the actual management of mucinous cystic neoplasm (MCN) of the pancreas. A systematic review was performed in December 2009 by consulting PubMed MEDLINE for publications and matching the "pancreatic mucinous cystic neoplasm", "pancreatic mucinous cystic tumour", "pancreatic mucinous cystic mass", "pancreatic cyst", and "pancreatic cystic neoplasm" to identify English language articles describing the diagnosis and treatment of the mucinous cystic neoplasm of the pancreas. In total, 16 322 references ranging from January 1969 to December 2009 were analysed and 77 articles were identified. No articles published before 1996 were selected because MCNs were not previously considered to be a completely autonomous disease. Definition, epidemiology, anatomopathological findings, clinical presentation, preoperative evaluation, treatment and prognosis were reviewed. MCNs are pancreatic mucinproducing cysts with a distinctive ovarian-type stroma localized in the body-tail of the gland and occurring in middle-aged females. The majority of MCNs are slow growing and asymptomatic. The prevalence of invasive carcinoma varies between 6% and 55%. Preoperative diagnosis depends on a combination of clinical features, tumor markers, computed tomography (CT), magnetic resonance imaging, endoscopic ultrasound with cyst fluid analysis, and positron emission tomography-CT. Surgery is indicated for all MCNs.

关键词： Pancreatic cystic lesion Pancreatic mucinous cystic neoplasm Pancreatic mucin-producing cysts Pancreatic cystic neoplasm Pancreatic ovarian-type stroma