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Role of CFTR mutation analysis in the diagnostic algorithm for cystic fibrosis

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World Journal of Pediatrics 2017年第2期13卷 129-135页

作者： Michelle Ratkiewicz Matthew Pastore Karen Sharrock McCoy Rohan Thompson don hayes Jr. Shahid Ijaz Sheikh Departments of Pediatrics the Ohio State University College of Medicine ColumbusOhioUSA Section of Pulmonary Medicine Nationwide Children's HospitalColumbusOhioUSA Departments of Pediatrics the Ohio State University College of Medicine ColumbusOhioUSA Section of Human and Molecular Genetics Nationwide Children's HospitalColumbusOhioUSA Departments of Pediatrics the Ohio State University College of Medicine ColumbusOhioUSA Internal Medicine the Ohio State University College of Medicine ColumbusOhioUSA Section of Pulmonary Medicine Nationwide Children's HospitalColumbusOhioUSA

Background:The cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation identification is being used with increased frequency to aid in the diagnosis of cystic fibrosis (CF) in those suspected with *** of this study was to identify diagnostic outcomes when CFTR mutational analysis was used in CF *** mutational analysis results were also compared with sweat chloride ***:This study was done on all patients at our institution who had CFTR mutation analysis over a sevenyear period since August ***:A total of 315 patients underwent CFTR mutational ***-one (16.2%) patients had two mutations *** them 32 had positive sweat chloride levels (≥60 mmol/L),while seven had borderline sweat chloride levels (40-59 mmol/L).An additional 70 patients (22.3%) had only one mutation *** them eight had positive sweat chloride levels,and 17 had borderline sweat chloride ***-five patients (17.5%) without CFTR mutations had either borderline (n=45) or positive (n=10) sweat chloride *** patients with a CF phenotype had negative CFTR analysis but elevated sweat chloride *** eighty-three patients (26.4%) CFTR mutational analysis was done without corresponding sweat chloride ***:Although CFTR mutation analysis has improved the diagnostic capability for CF,its use either as the first step or the only test to diagnose CFTR dysfunction should be discouraged and CF diagnostic guidelines need to be followed.

关键词： CFTR cystic fibrosis sweat chloride

Impact of transjugular intrahepatic porto-systemic shunt on post liver transplantation outcomes: Study based on the United Network for Organ Sharing database

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World Journal of Hepatology 2017年第2期9卷 99-105页

作者： Khalid Mumtaz Sherif Metwally Rohan M Modi Nishi Patel Dmitry Tumin Anthony J Michaels James Hanje Ashraf El-Hinnawi don hayes Jr Sylvester M Black Wexner Medical Center Gastroenterology Hepatology and Nutrition Division the Ohio State University Department of Internal Medicine the Ohio State University Department of Pediatrics the Ohio State University Department of Surgery the Ohio State University Section of Pulmonary Medicine the Ohio State University

AIM To determine the impact of transjugular intrahepatic porto-systemic shunt(TIPS) on post liver transplantation(LT) *** Utilizing the United Network for Organ Sharing(UNOS) database, we compared patients who underwent LT from 2002 to 2013 who had underwent TIPS to those without TIPS for the management of ascites while on the LT waitlist. The impact of TIPS on 30-d mortality, length of stay(LOS), and need for re-LT were studied. For evaluation of mean differences between baseline characteristics for patients with and without TIPS, we used unpaired t-tests for continuous measures and χ~2 tests for categorical measures. We estimated the impact of TIPS on each of the outcome measures. Multivariate analyses were conducted on the study population to explore the effect of TIPS on 30-d mortality post-LT, need for re-LT and LOS. All covariates were included in logistic regression *** We included adult patients(age ≥ 18 years) who underwent LT from May 2002 to September 2013. Only those undergoing TIPS after listing and before liver transplant were included in the TIPS group. We excluded patients with variceal bleeding within two weeks of listing for LT and those listed for acute liver failure or hepatocellular carcinoma. Of 114770 LT in the UNOS database, 32783(28.5%) met inclusion criteria. Of these 1366(4.2%) had TIPS between the time of listing and LT. We found that TIPS increased the days on waitlist(408 ± 553 d) as compared to those without TIPS(183 ± 330 d), P < 0.001. Multivariate analysis showed that TIPS had no effect on 30-d post LT mortality(OR = 1.26; 95%CI: 0.91-1.76) and re-LT(OR = 0.61; 95%CI: 0.36-1.05). Pre-transplant hepatic encephalopathy added 3.46 d(95%CI: 2.37-4.55, P < 0.001), followed by 2.16 d(95%CI: 0.92-3.38, P = 0.001) by TIPS to LOS. CONCLUSION TIPS did increase time on waitlist for LT. More importantly, TIPS was not associated with 30-d mortality and re-LT, but it did lengthen hospital LOS after transplantation.

关键词： Transjugular intrahepatic porto 全身的分流分流肝移植腹水为结束阶段肝疾病当模特儿死亡 Transjugular

Animal models of ex vivo lung perfusion as a platform for transplantation research

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World Journal of Experimental Medicine 2014年第2期4卷 7-15页

作者： Kevin Nelson Christopher Bobba Samir Ghadiali don hayes Jr Sylvester M Black Bryan A Whitson Department Biomedical Engineering The Ohio State UniversityColumbusOH 43210United States Department of Internal Medicine Division of PulmonaryAllergyand Critical Care and Sleep MedicineThe Ohio State University Wexner Medical Center The Ohio State University Department of Pediatrics Nationwide Children’s HospitalColumbusOH 43210United States Department of Surgery Division of TransplantationColumbusOH 43210United States Department of Surgery Division of Cardiac SurgeryThe Collaboration for Organ PerfusionProtectionEngineering and Regeneration LaboratoryThe Ohio State University Wexner Medical CenterColumbusOH 43210United States

Ex vivo lung perfusion(EVLP) is a powerful experimental model for isolated lung research. EVLP allows for the lungs to be manipulated and characterized in an external environment so that the effect of specific ventilation/perfusion variables can be studied independent of other confounding physiologic contributions. At the same time,EVLP allows for normal organ level function and real-time monitoring of pulmonary physiology and mechanics. As a result,this technique provides uniqueadvantages over in vivo and in vitro models. Small and large animal models of EVLP have been developed and each of these models has their strengths and weaknesses. In this manuscript,we provide insight into the relative strengths of each model and describe how the development of advanced EVLP protocols is leading to a novel experimental platform that can be used to answer critical questions in pulmonary physiology and transplant medicine.

关键词： Ex vivo lung perfusion Transplantation Rat Porcine Small animal Large animal Model Ex vivo lung perfusion

Effect of transplant center volume on post-transplant survival in patients listed for simultaneous liver and kidney transplantation

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World Journal of Hepatology 2018年第1期10卷 134-141页

作者： Rohan M Modi Dmitry Tumin Andrew J Kruger Eliza W Beal don hayes Jr James Hanje Anthony J Michaels Kenneth Washburn Lanla F Conteh Sylvester M Black Khalid Mumtaz Department of Internal Medicine Ohio State University Wexner Medical CenterColumbusOH 43210United States Department of Anesthesiology and Pain Medicine Nationwide Children’s HospitalColumbusOH 43205United States Department of General Surgery Division of TransplantationOhio State University Wexner Medical CenterColumbusOH 43210United States Comprehensive Transplant Center Ohio State University Wexner Medical CenterColumbusOH 43210United States Section of Pulmonary Medicine Nationwide Children’s HospitalColumbusOH 43205United States Department of Internal Medicine Division of GastroenterologyHepatology and NutritionOhio State University Wexner Medical CenterColumbusOH 43210United States

AIM To examine the effect of center size on survival differences between simultaneous liver kidney transplantation(SLKT) and liver transplantation alone(LTA) in SLKT-listed *** The United Network of Organ Sharing database was queried for patients ≥ 18 years of age listed for SLKT between February 2002 and December 2015. Posttransplant survival was evaluated using stratified Cox regression with interaction between transplant type(LTA vs SLKT) and center *** During the study period, 393 of 4580 patients(9%) listed for SLKT underwent a LTA. Overall mortality was higher among LTA recipients(180/393, 46%) than SLKT recipients(1107/4187, 26%). The Cox model predicted a significant survival disadvantage for patients receiving LTA vs SLKT [hazard ratio, hazard ratio(HR) = 2.85; 95%CI: 2.21, 3.66; P < 0.001] in centers performing 30 SLKT over the study period. This disadvantage was modestly attenuated as center SLKT volume increased, with a 3% reduction(HR = 0.97; 95%CI: 0.95, 0.99; P = 0.010) for every 10 SLKs *** In conclusion, LTA is associated with increased mortality among patients listed for SLKT. This difference is modestly attenuated at more experienced centers and may explain inconsistencies between smaller-center and larger registry-wide studies comparing SLKT and LTA outcomes.

关键词： Kidney transplantation Center volume Mortality Liver transplantation United network for organ sharing

Mechanical circulatory support in lung transplantation: Cardiopulmonary bypass, extracorporeal life support, and ex-vivo lung perfusion

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World Journal of Respirology 2015年第2期5卷 78-92页

作者： Shaylyn C Bennett Eliza W Beal Curtis A Dumond Thomas Preston Jim Ralston Amy Pope-Harman Sylvester Black don hayes Jr Bryan A Whitson Department of Surgery the Ohio State University Wexner Medical Center Division of Cardiothoracic Surgery Department of Surgerythe Ohio State University Wexner Medical Center Department of Cardiovascular Perfusion and ECMO Nationwide Children’s Hospital Department of Perfusion the Ohio State University Wexner Medical Center Division of Pulmonary AllergyCritical Care and Sleep Medicinethe Ohio State University Wexner Medical Center Division of Transplantation Surgery Department of Surgerythe Ohio State University Wexner Medical Center Department of Pediatric Pulmonology Nationwide Children’s Hospital

Lung transplant is the standard of care for patients with end-stage lung disease refractory to medical management. There is currently a critical organ shortage for lung transplantation with only 17% of offered organs being transplanted. Of those patients receiving a lung transplant, up to 25% will develop primary graft dysfunction, which is associated with an 8-fold increase in 30-d mortality. There are numerous mechanical lung assistance modalities that may be employed to help combat these challenges. We will discuss the use of mechanical lung assistance during lung transplantation, as a bridge to transplant, as a treatment for primary graft dysfunction, and finally as a means to remodel and evaluate organs deemed unsuitable for transplant, thus increasing the donor pool, improving survival to transplant, and improving overall patient survival.

关键词： Lung transplant Cardiopulmonary bypass Extracorporeal membrane oxygenation Extracorporeal life support Extracorporeal lung assist Interventional lung assist Ex-vivo lung perfusion