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Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis 被引量:10
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作者 Moran Lavie Tzipora Manovitz +7 位作者 Daphna Vilozni Sarina Levy-Mendelovich Ifat Sarouk Ilana Weintraubv David Shoseyov Malena Cohen-Cymberknoh Joseph Rivlin Ori Efrati 《World Journal of Gastroenterology》 SCIE CAS 2015年第1期318-325,共8页
AIM: To investigate the long-term follow-up of distal intestinal obstruction syndrome(DIOS) in Israeli cystic fibrosis(CF) patients.METHODS: This is a multi-center,comparative,retrospective study in which we reviewed ... AIM: To investigate the long-term follow-up of distal intestinal obstruction syndrome(DIOS) in Israeli cystic fibrosis(CF) patients.METHODS: This is a multi-center,comparative,retrospective study in which we reviewed the medical records of all CF patients from three major CF centers in Israel who were treated in the period from 1980 to 2012.Patients diagnosed with DIOS were defined as the study group.The patients were diagnosed with DIOS based on their clinical presentation and typical findings on either abdominal X-ray or computerized tomography scan.For the control group,CF patients with no DIOS were matched to the patients in the study group for age,sex,and cystic fibrosis transmembrane conductance regulator(CFTR) mutations.For both groups,the collected data included age,sex,CFTR genotype,weight,height,and body mass index.Clinical data included respiratory function tests in the last five years prior to the study,respiratory function test immediately before and after the DIOS event,number of hospitalizations,sputum culture results,and CFrelated conditions diagnosed according to the CF clinical practice guidelines.In the study group,data on the DIOS treatment and tendency for DIOS recurrence were also analyzed.RESULTS: The medical charts for a total of 350 CF patients were reviewed.Of the 350 CF patients,26(7.4%) were diagnosed with DIOS.The control group included 31 CF patients with no DIOS diagnosis.The mean follow-up period was 21.6 ± 8.2 years.The total of DIOS episodes in the follow-up period was 60.The distribution of DIOS episodes was as follows: 6/26(23.1%) study patients had one episode of DIOS intheir lifetime,7/26(26.9%) had two episodes,7/26(26.9%) had three episodes,and 6/26(23.1%) had four or more episodes.Compared to the control group,DIOS patients had a significantly higher incidence of meconium ileus in the past(65.4% vs 0%,respectively,P < 0.02),more Aspergillus spp.colonization(34.6% vs 3.2%,respectively,P < 0.02),and a higher number of hospitalizations due to respiratory exacerbations(8.6 vs 6.2 mean total hospitalizations per follow-up period,respectively,P < 0.02).No other significant differences were found between the control and study groups.The conservative treatment of DIOS,which mainly includes hydration and stool softeners,was successful in 82% of the episodes.The survival rate was similar for both groups.CONCLUSION: CF patients with DIOS suffer from recurrent hospitalizations and airway pathogen acquisition.Although recurrence of DIOS is common,conservative treatment is successful in most patients. 展开更多
关键词 DISTAL INTESTINAL OBSTRUCTION SYNDROME CYSTIC fibr
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Risk factors for hepatic steatosis in adults with cystic fibrosis: Similarities to non-alcoholic fatty liver disease 被引量:2
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作者 Fares Ayoub Cesar Trillo-Alvarez +1 位作者 Giuseppe Morelli Jorge Lascano 《World Journal of Hepatology》 CAS 2018年第1期34-40,共7页
AIM To investigate the clinical, biochemical and imaging characteristics of adult cystic fibrosis(CF) patients with hepatic steatosis as compared to normal CF controls.METHODS We performed a retrospective review of ad... AIM To investigate the clinical, biochemical and imaging characteristics of adult cystic fibrosis(CF) patients with hepatic steatosis as compared to normal CF controls.METHODS 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 controls.RESULTS 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 diabetes.CONCLUSION 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. 展开更多
关键词 CYSTIC fibrosis LIVER DISEASE Hepatic STEATOSIS Non-alcoholic fatty LIVER DISEASE
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Cystic fibrosis transmembrane conductance regulator functional evaluations in a G542X+/-IVS8Tn:T7/9 patient with acute recurrent pancreatitis 被引量:2
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作者 Sara Caldrer Gabriella Bergamini +12 位作者 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 《World Journal of Clinical Cases》 SCIE 2019年第22期3757-3764,共8页
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.Mutat... 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. 展开更多
关键词 Recurrent acute PANCREATITIS CYSTIC FIBROSIS CYSTIC FIBROSIS transmembraneconductance regulator function Intestinal current ORGANOIDS SWEAT test Controversialdiagnosis Case report
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Acquired cystic fibrosis transmembrane conductance regulator dysfunction 被引量:1
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作者 Catherine Banks Laura Freeman +1 位作者 Do Yeon Cho Bradford A.Woodworth 《World Journal of Otorhinolaryngology-Head and Neck Surgery》 2018年第3期193-199,共7页
Sinonasal respiratory epithelium is a highly regulated barrier that employs mucociliary clearance(MCC)as the airways first line of defense.The biological properties of the airway surface liquid(ASL),combined with coor... Sinonasal respiratory epithelium is a highly regulated barrier that employs mucociliary clearance(MCC)as the airways first line of defense.The biological properties of the airway surface liquid(ASL),combined with coordinated ciliary beating,are critical components of the mucociliary apparatus.The ASL volume and viscosity is modulated,in part,by the cystic fibrosis transmembrane conductance regulator(CFTR).The CFTR is an anion transporter of chloride(Cl-)and bicarbonate(HCO 3-)that is located on the apical surface of respiratory epithelium and exocrine glandular epithelium.Improved understanding of how dysfunction or deficiency of CFTR influences the disease process in both genetically defined cystic fibrosis(CF)and acquired conditions has provided further insight into potential avenues of treatment.This review discusses the latest data regarding acquired CFTR deficiency and use of CFTR specific treatment strategies for CRS and other chronic airway diseases. 展开更多
关键词 CFTR Cystic fibrosis CFTR deficiency CFTR dysfunction Chronic sinusitis Chronic rhinosinusitis Chronic obstructive pulmonary disease Tobacco Hypoxia RESVERATROL L-ascorbate
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Protective and antifungal properties of Nanodisk-Amphotericin B over commercially available Amphotericin B 被引量:1
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作者 Do-Yeon Cho Kyle J.Hoffman +5 位作者 Gobind S.Gill Dong-Jin Lim Daniel Skinner Calvin Mackey Steven M.Rowe Bradford A.Woodworth 《World Journal of Otorhinolaryngology-Head and Neck Surgery》 2017年第1期-,共7页
Objective:Amphotericin B (AMB),a potent antifungal agent,has been employed as topical and systemic therapy for sinonasal fungal infections.A novel formulation of nanodisc (ND) containing super aggregated AMB (ND-AMB) ... Objective:Amphotericin B (AMB),a potent antifungal agent,has been employed as topical and systemic therapy for sinonasal fungal infections.A novel formulation of nanodisc (ND) containing super aggregated AMB (ND-AMB) for the treatment of fungal infections has been recently developed to provide greater protection from AMB toxicity than current,clinically approved lipid-based formulations.The objective of the current study was to evaluate the safety and potency of ND-AMB for sinonasal delivery using an in vitro model.Methods:Human sinonasal tissue was harvested during endoscopic sinus surgery and grown at air-liquid interface until well-differentiated.Cultures were exposed to ND-AMB vs AMB and changes in K+ permeability and resistance were measured and recorded via Ussing chamber assay.Ciliary beat frequency (CBF) was analyzed in parallel as well as cytotoxic assay.Potency was assessed using real-time PCR measurement of the Aspergillus fumigatus 18S rRNA.Results:Ussing chamber studies revealed K+ currents that increased rapidly within 30 s of adding AMB (10 μg/mL) to the apical side,indicating apical membranes had become permeable to K+ ions.In contrast,negligible induction of K+ current was obtained following addition of NDAMB [AMB =(107.7 ± 15.9) μA/cm2 AMB vs ND-AMB =(2.3 ± 0.7) μA/cm2 ND-AMB;P =0.005].ND-AMB also protected nasal epithelial cells from cytotoxicity of AMB (P < 0.05).There was no difference in ciliary beat frequency between the two groups (P =0.96).The expression of A.fumigatus 18S rRNA with exposure of lower dose of ND-AMB was significantly lower compared to that with AMB (P < 0.05).Conclusions:Data from the present study suggests ND-AMB protects human nasal epithelia membranes from AMB toxicity by protecting against apical cell K+ permeability while maintaining uncompromised antifungal property compared to AMB.ND-AMB could provide a novel topical therapy for sinonasal fungal diseases. 展开更多
关键词 Chronic rhinosinusitis AMPHOTERICIN Drug delivery Transepithelial ion transport SINUSITIS Sinus epithelium Mucociliary clearance Ciliary beat frequency
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