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Contribution of pancreatic enzyme replacement therapy to survival and quality of life in patients with pancreatic exocrine insufficiency

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World Journal of Gastroenterology 2019年第20期25卷 2430-2441页

作者： Peter Layer Nataliya Kashirskaya Natalya Gubergrits Department of Internal Medicine Israelitic HospitalHamburg 22297Germany Department of Genetic Epidemiology (cystic fibrosis group) Federal State Budgetary Institution “Research Centre for Medical Genetics”Moscow 115522Russia Department of Internal Medicine Donetsk National Medical UniversityLyman 83001Ukraine

The objective of this study was to analyze the current evidence for the use of pancreatic enzyme replacement therapy (PERT) in affecting survival and quality of life in patients with pancreatic exocrine insufficiency (PEI). Systematic searches of the literature were performed using the PubMed database. Articles were selected for inclusion if they reported findings from trials assessing the effects of PERT on quality of life, survival, malabsorption, growth parameters (such as height, body weight and body mass index), or gastrointestinal symptoms (such as abdominal pain, stool consistency and flatulence). PERT improved PEI-related malabsorption and weight maintenance in patients with cystic fibrosis, chronic pancreatitis, pancreatic cancer, and post-surgical states. In patients with chronic pancreatitis, PERT improved PEI-related symptoms and quality of life measures. Several small retrospective studies have also suggested that PERT may have a positive impact on survival, but long-term studies assessing this effect were not identified. PERT is effective for treating malnutrition and supporting weight maintenance, and it is associated with improved quality of life and possibly with enhanced survival in patients with PEI. However, there is evidence that not all patients with PEI receive adequate PERT. Future work should aim to assess the long-term effects of PERT on the survival of patients with PEI.

关键词： Pancreatic exocrine insufficiency Pancreatic enzyme replacement therapy Survival Quality of life Malabsorption cystic fibrosis Chronic pancreatitis Pancreatic cancer Post-surgical states

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Risk factors for hepatic steatosis in adults with cystic fibrosis: Similarities to non-alcoholic fatty liver disease

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World Journal of Hepatology 2018年第1期10卷 34-40页

作者： Fares Ayoub Cesar Trillo-Alvarez Giuseppe Morelli Jorge Lascano Department of Medicine University of Florida Department of Medicine Division of Pulmonary Critical Care and Sleep Medicine University of Florida Department of Medicine Division of Gas-troenterology Hepatology and Nutrition University of Florida Department of Medicine Adult Cystic Fibrosis Center Division of Pulmonary Critical Care and Sleep Medicine University of Florida

AIM To investigate the clinical, biochemical and imaging characteristics of adult cystic fibrosis(CF) patients with hepatic steatosis as compared to normal CF *** We performed a retrospective review of adult CF patients in an academic outpatient setting during 2016. Baseline characteristics, genetic mutation analysis as well as laboratory values were collected. Abdominal imaging(ultrasound, computed tomography, magnetic resonance) was used to determine presence of hepatic steatosis. We compare patients with hepatic steatosis to normal *** Data was collected on 114 patients meeting inclusion criteria. Seventeen patients(14.9%) were found to have hepatic steatosis on imaging. Being overweight(BMI > 25)(P = 0.019) and having a higher pp FEV1(75 vs 53, P = 0.037) were significantly associated with hepatic steatosis. Patients with hepatic steatosis had a significantly higher median alanine aminotransferase level(27 vs 19, P = 0.048). None of the hepatic steatosis patients had frank CF liver disease, cirrhosis or portal hypertension. We found no significant association with pancreatic insufficiency or CF related *** Hepatic steatosis appears to be a clinically and phenotypically distinct entity from CF liver disease. The lack of association with malnourishment and the significant association with higher BMI and higher pp FEV1 demonstrate similarities with non-alcoholic fatty liver disease. Long term prospective studies are needed to ascertain whether CF hepatic steatosis progresses to fibrosis and cirrhosis.

关键词： cystic fibrosis liver disease Hepatic steatosis Non-alcoholic fatty liver disease

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Role of cytokines and chemokines in non-alcoholic fatty liver disease

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World Journal of Gastroenterology 2012年第8期18卷 727-735页

作者： Vincent Braunersreuther Giorgio Luciano Viviani Franois Mach Fabrizio Montecucco Division of Cardiology Foundation for Medical Researches Faculty of Medicine Geneva University Hospital Department of Internal Medicine Adult Diabetes Centre University of Genoa

Non-alcoholic fatty liver disease (NAFLD) includes a variety of histological conditions (ranging from liver steatosis and steatohepatitis, to fibrosis and hepatocarcinoma) that are characterized by an increased fat content within the liver. The accumulation/deposition of fat within the liver is essential for diagnosis of NAFLD and might be associated with alterations in the hepatic and systemic inflammatory state. Although it is still unclear if each histological entity represents a different disease or rather steps of the same disease, inflammatory processes in NAFLD might influence its pathophysiology and prognosis. In particular, non-alcoholic steatohepatitis (the most inflamed condition in NAFLDs, which more frequently evolves towards chronic and serious liver diseases) is characterized by a marked activation of inflammatory cells and the upregulation of several soluble inflammatory mediators. Among several mediators, cytokines and chemokines might play a pivotal active role in NAFLD and are considered as potential therapeutic targets. In this review, we will update evidence from both basic research and clinical studies on the potential role of cytokines and chemokines in the pathophysiology of NAFLD.

关键词： Inflammation Non-alcoholic fatty liver disease Cytokine Chemokine

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Variability of anti-human transglutaminase testing in celiac disease across Mediterranean countries

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World Journal of Gastroenterology 2017年第24期23卷 4437-4443页

作者： Andrea Smarrazzo Giuseppe Magazzù Mongi Ben-Hariz Maria Legarda Tamara Virtut Velmishi Elefhteria Roma Aydan Kansu Dusanka Micetic-Turk Enzo Bravi Pio Stellato Carmela Arcidiaco Luigi Greco Department of Translational Medical Sciences School of MedicineSection of PediatricsUniversity of Naples“Federico II” European Laboratory for Food Induced Diseases Celiac Regional centre Pediatric Gastroenterology and Cystic Fibrosis UnitUniversity of Messina Pediatric Unit Mongi SLIM’s Hospital of Tunis Paediatric Gastroenterology Unit Cruces University Hospital Service of Pediatric Gastroenterology"Mother Teresa"Hospital First Department of Pediatrics University of Athens Department of Pediatric Gastroenterology School of MedicineAnkara University University Medical centre Paediatric Department Eurospital SpA

To verify the precision and accuracy of transglutaminase antibodies (TGA) assays across Mediterranean countries. METHODSThis study involved 8 referral centres for celiac disease (CD) in 7 Mediterranean countries. A central laboratory prepared 8 kits of 7 blinded and randomized serum samples, with a titrated amount of Human TGA IgA. Each sample was analysed three times on three different days, with each centre running a total of 21 tests. The results were included in a blindly coded report form, which was sent to the coordinator centre. The coordinator estimated the mean coefficient of Variation (CoVar = σ/μ), the mean accuracy (Accur = Vobserved - Vreal) and the mean percent variation (Var% = [(Vobserved - Vreal)/Vreal] × 100). RESULTSThe analysis showed that 79.17% of the mean variation fell between -25% and +25% of the expected value, with the accuracy and precision progressively increasing with higher titres of TGA. From values 1.25 times greater than the normal cut-off, the measurements were highly reliable. CONCLUSIONTGA estimation is a crucial step for the diagnosis of CD; given its accuracy and precision, clinicians could be confident in establishing a diagnosis.

关键词： Coeliac disease Gluten Transglutaminase Non-invasive testing Accuracy Precision

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Comparative Variability of Nasal Potential Difference Measurements in Human and Mice

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Open Journal of Respiratory Diseases 2012年第2期2卷 43-56页

作者： Anissa Leonard Bob Lubamba Barbara Dhooghe Sabrina Noel Pierre Wallemacq Patrick Lebecque Teresinha Leal Louvain centre for Toxicology and Applied Pharmacology Institut de Recherche Clinique et Expérimentale Université Catholique de Louvain Brussels Belgium Pediatric Pulmonology & cystic fibrosis Unit St Luc University Hospital Brussels Belgium

Background: Nasal potential difference (NPD) test has long been used to assist in the diagnosis of cystic fibrosis (CF) and more recently as an outcome measure in clinical trials of new CF therapies. This test has also been adapted to the mouse nose. Objectives: We aimed at evaluating variability of the NPD measurements in CF patients displaying two severe CFTR mutations and in sex-matched healthy controls. NPD recorded from F508del-CF and normal wild-type mice were also compared. Methods and results: In each setting, tests were performed by a single qualified operator. In the clinical setting, the latest standardized operation protocol of the CF foundation was followed. A total of 80 tracings were obtained from 10 patients (23.2 y;range 14 to 32) and 10 healthy subjects (34 y;range 24 to 53), each tested twice, in both nostrils. Two CF and two controls were excluded from the statistical data analysis due to the presence of a single non interpretable NPD tracing (4/80, 5%). To achieve equal sample size, tests were obtained from 8 CF mice and normal wild-type. Comprehensive multivariate analysis of paired data showed a good reproducibility of NPD parameters in the clinical and the preclinical setting;lower variability was observed in mice. However, 95% repeatability limits of NPD parameters were large indicating a large measurement error, poor precision and low within-subject repeatability. In both settings, chloride secretion was shown to be the most reproducible and repeatable parameter. Conclusion: In human as in mice, NPD showed good reproducibility but limited within-subject repeatability.

关键词： Animal Models CFTR cystic fibrosis Nasal Potential Difference

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Alterations of gene profiles in Leydig-cell-regenerating adult rat testis after ethane dimethane su Ifonate-treatment

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Asian Journal of Andrology 2015年第2期17卷 253-260,I0009页

作者： Yu-Fei Zhang Kai-Ming Yuan Yong Liang Yan-Hui Chu Qing-Quan Lian Yu-Fei Ge Wei Zhen Chantal M Sottas Zhi-Jian Su Ren-Shan Ge Heilongjiang Key Laboratory of Anti-fibrosis Biotherapy Mudanjiang Medical University Mudanjiang 157011 China The 2nd Affiliated Hospital Wenzhou Medical College Wenzhou 325000 China The Affiliated Taizhou Municipal Hospital Taizhou University Taizhou 318000 China The Population Council New York 10065 USA Department of Pathology Molecular Pathology Laboratory Montefiore Medical Centre Albert Einstein College of Medicine Bronx NY 10467 USA College of Life Science and Technology Jinan University Guangzhou 510632 China

成年睾丸间质细胞（ALC）是睾丸中产生雄激素的唯一内分泌细胞，只占有睾丸细胞的1％-5％。大鼠睾丸中的睾丸间质细胞被EDs去除后能够再生。本研究中，我们主要研究了大鼠睾丸经EDS处理后睾丸间质细胞再生及基因的变化图谱。EDS处理后7... 详细信息

成年睾丸间质细胞（ALC）是睾丸中产生雄激素的唯一内分泌细胞，只占有睾丸细胞的1％-5％。大鼠睾丸中的睾丸间质细胞被EDs去除后能够再生。本研究中，我们主要研究了大鼠睾丸经EDS处理后睾丸间质细胞再生及基因的变化图谱。EDS处理后7、21、35、90天后分析血清中睾酮、睾丸基因图谱和一些类固醇生成相关的蛋白变化情况。EDS处理后第7天睾丸中睾酮水平几乎检测不到，然后开始恢复。处理后7天，81个基因下调2倍或者更多，其中48个基因检测不到，包括类固醇合成通路中的蛋白Star、Scarbl、Cypllal、Cypl7al、Hsd3bl、CyplblICyp2al。这些基因在21天开始表达增多，到90天已经完全恢复到正常水平。89个基因上调2倍或者更高，nPkib。这些上调的基因到90天恢复正常；其中Cyp2al直到35天才开始恢复，表明这个基因只在成年睾丸间质细胞中表达，不在前体细胞中表达。qPCR，Westernblot、半定量免疫组织染色检测了随机抽取的基因和蛋白确认其变化情况。

关键词： EDS 基因分析睾丸间质细胞类固醇生成

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Low Level of cystic fibrosis Transmembrane Conductance Regulator Is Associated with Human Sperm Autophagy and Vitality

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Advances in Reproductive Sciences 2024年第1期12卷 23-36页

作者： Jie Hu Han Liu Chaoyan Ou Liangzhao Liu Linfeng Mo Xuming Liang Yonghua He Department of Epidemiology and Statistics School of Public Health Guilin Medical University Guilin China School of Public Health and Laboratory Medicine Yiyang Medical College Yiyang China Medicine and Health Science College Guangzhou Huashang Vocational College Guangzhou China centre of Reproductive Medicine Affiliated Hospital of Guilin Medical University Guilin China

Low sperm motility is one of the main causes of male infertility. cystic fibrosis transmembrane conductance regulator (CFTR, an anion channel protein) is related to the progressive motility of sperm. CFTR disruptor CFTRinh-172 or forskolin (FSK) in this study were used to treat human sperm separately, and the rates of sperm autophagy and progressive motility, mitochondrial membrane potential (MMP) and ATP concentration, and the expression levels of related factors were detected to explore their relationship. It was showed that sperms treated with CFTRinh-172 or FSK reduced the levels of cAMP, CFTR and PKA, but increased sperm autophagy rate, expression levels of AMPK and LC3B. However, reactive oxygen species content had no significant difference. It was indicated that low level of CFTR performed with cAMP and its downstream effectors such as PKA and AMPK to regulate mitochondrial structure and function, leading to increased autophagy rate and reduced vitality of sperm.

关键词： Low Level of cystic fibrosis Transmembrane Conductance Regulator Is Associated with Human Sperm Autophagy and Vitality

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Comprehensive semen examination in patients with pancreatic-sufficient and pancreatic-insufficient cystic fibrosis

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Asian Journal of Andrology 2023年第5期25卷 591-597页

作者： Anna O Sedova Maria I Shtaut Elizaveta E Bragina Tatyana M Sorokina Galina V Shmarina Marina V Andreeva Lyubov E Kurilo Stanislav A Krasovskiy Aleksander V Polyakov Vyacheslav B Chernykh Research centre for Medical Genetics Russia Moscow 115522

We examined a cohort of 93 cystic fibrosis(CF)male patients who were pancreatic-sufficient(PS-CF;n=40)or pancreatic-insufficient(PI-CF;n=53).Complex semen examination was performed,including standard semen analysis,quantitative karyological analysis(QKA)of immature germ cells(IGCs),transmission electronic microscopy(TEM),biochemical analysis,and sperm DNA fragmentation by terminal deoxynucleotidyl transferase-mediated dUTP nickend labeling(TUNEL)*** was diagnosed in 83(89.2%)*** other 10(10.8%)patients were found to be nonazoospermic and showed various spermatological diagnoses(asthenozoospermia,n=2;asthenoteratozoospermia,n=3;oligoasthenozoospermia,n=1;oligoasthenoteratozoospermia,n=3;and normozoospermia,n=1)with no specific morphological *** was detected in 89.2%azoospermic and 30.0%nonazoospermic *** seminal pH(30%)of spermatozoa with noncondensed(“immature”)chromatin was revealed in 2 of 6 nonazoospermic semen samples analyzed by TEM.

关键词： congenital bilateral agenesia/aplasia of vas deferens cystic fibrosis cystic fibrosis transmembrane conductance regulator gene semen spermatozoa

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成年囊性纤维化气管软化发病率和严重度的动态电影CT评价

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国际医学放射学杂志 2009年第5期32卷 502-502页

作者： S.McDermott S.C.Barry E.E.Judge S.Collins P.A.Jong H.A.W.M.Tiddens 高莉 Department of Respiratory Medicine and National Referral centre for adult cystic fibrosis St Vincent’s University HospitalElm ParkDublin 4Ireland

目的应用动态电影多层CT,确定囊性纤维化（CF）成年病人气管软化的发生率和严重度，并将影像表现与肺功能测试（PFT）结果、肺实质病变严重程度相对照。方法本研究经伦理委员会批准并符合医疗健康保险要求，40例CF病人[男22例，女18例... 详细信息

目的应用动态电影多层CT,确定囊性纤维化（CF）成年病人气管软化的发生率和严重度，并将影像表现与肺功能测试（PFT）结果、肺实质病变严重程度相对照。方法本研究经伦理委员会批准并符合医疗健康保险要求，40例CF病人[男22例，女18例；年龄18～54岁。平均（22±8）岁]；前瞻性行PFT，标准薄层CT和两次动态电影多层CT扫描。

关键词：囊性纤维化气管软化成年病人严重度 CT评价电影发病率病变严重程度

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Seasonal variation and living alone are related to pulmonary rehabilitation non-completion

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World Journal of Respirology 2013年第2期3卷 29-37页

作者： James R Walsh Zoe J Mc Keough Norman R Morris Stephanie T Yerkovich Michelle E Wood Jenny D Paratz Queensland Lung Transplant Service Prince Charles Hospital Queensland 4032 Australia School of Medicine University of Queensland Queensland 4072 Australia Discipline of Physiotherapy University of Sydney Sydney NSW 2141 Australia School of Rehabilitation Sciences and Griffith Health Institute Griffith University Gold Coast 4215 Australia adult cystic fibrosis centre Prince Charles Hospital Queensland 4032 Australia

AIM: To identify baseline characteristics that independently predict pulmonary rehabilitation non-completion and compare these findings against the participant's reasons for non-completion. METHODS: Participants with chronic obstructive pulmonary disease(COPD) who attended a standardised twice weekly, eight week pulmonary rehabilitation program(located in the sub-tropics, latitude 27°29' South) between 2010 and 2012 were recruited. Thebaseline characteristics of program completers and non-completers were compared in a case-controlled design. Participants who attended 4 session of the program) were asked by one independent investigator to participate in a survey about their pulmonary rehabilitation experience. Baseline characteristics were assessed for differences between program completers and non-completers. The baseline characteristics included disease severity, exercise capacity, smoking history, participant's social support and the season when each participant commenced rehabilitation. Non-completers that agreed to participate in the survey were asked to indicate what personal factors or external factors contributed to their program non-completion. Comparisons of completers and non-completers baseline characteristics were performed using cross-tabulations and t-tests, with significant measures analysed in a multivariate binary logistic regression model. Non-completers survey responses were compared to the identified independent predictors using ***: Twenty-six participants(23.4%) of the 111 participants with COPD [(mean ± SD) age was 67.4 ± 9.2 years and FEV1 54.6% ± 22.3%)], were classified as non-completers. Forty-five participants(40.5%) commenced pulmonary rehabilitation during winter. Thirty-six participants(32.4%) were living alone at program commencement. In the multivariate analysis(n = 111), only programs that commenced in winter(Exp B: 0.255, 95

关键词： Chronic obstructive pulmonary disease Pulmonary rehabilitation Predictive factors Program completion Program non-completion

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