Organ Transplantation for Cystic Fibrosis

Abstract

Cystic fibrosis (CF) remains the most common indication for lung transplantation in children and the third most common in adults and has the highest median survival posttransplant for all pretransplant diagnoses. Criteria for transplant in patients with CF vary widely among transplant centers and early referral to multiple centers may be needed to maximize opportunities for lung transplantation. Comorbidities unique to CF such as resistant and atypical pathogens like Burkholderia and Mycobacterium abscessus, and cirrhosis require special consideration for lung transplantation but should not be considered as absolute contraindications. For those patients who are listed for lung transplantation, mechanical support with extracorporeal membrane oxygenation and mechanical ventilation can be efficacious as bridges to lung transplantation in experienced centers with adequate resources. Liver and pancreas transplantations are also acceptable options for end-organ disease related to CF and can provide improvements in both quantity and quality of life.

Keywords

cystic fibrosis - lung transplantation - Burkholderia - extracorporeal membrane oxygenation - multiorgan transplantation - nontuberculous mycobacteria

• References

• 1 Benden C, Goldfarb SB, Stehlik J. An aging population of patients with cystic fibrosis undergoes lung transplantation: an analysis of the ISHLT Thoracic Transplant Registry. J Heart Lung Transplant 2019; (e-pub ahead of print)
• 2 Goldfarb SB, Hayes Jr D, Levvey BJ. , et al; International Society for Heart and LungTransplantation. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Twenty-first Pediatric Lung and Heart-Lung Transplantation Report-2018; focus theme: multiorgan transplantation. J Heart Lung Transplant 2018; 37 (10) 1196-1206
• 3 Chambers DC, Cherikh WS, Goldfarb SB. , et al; International Society for Heart and Lung Transplantation. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: thirty-fifth adult lung and heart-lung transplant report-2018; focus theme: multiorgan transplantation. J Heart Lung Transplant 2018; 37 (10) 1169-1183
• 4 Adler FR, Aurora P, Barker DH. , et al. Lung transplantation for cystic fibrosis. Proc Am Thorac Soc 2009; 6 (08) 619-633
• 5 Corris PA. Lung transplantation for cystic fibrosis and bronchiectasis. Semin Respir Crit Care Med 2013; 34 (03) 297-304
• 6 Kerem E, Reisman J, Corey M, Canny GJ, Levison H. Prediction of mortality in patients with cystic fibrosis. N Engl J Med 1992; 326 (18) 1187-1191
• 7 Augarten A, Akons H, Aviram M. , et al. Prediction of mortality and timing of referral for lung transplantation in cystic fibrosis patients. Pediatr Transplant 2001; 5 (05) 339-342
• 8 Rosenbluth DB, Wilson K, Ferkol T, Schuster DP. Lung function decline in cystic fibrosis patients and timing for lung transplantation referral. Chest 2004; 126 (02) 412-419
• 9 Tuppin MP, Paratz JD, Chang AT. , et al. Predictive utility of the 6-minute walk distance on survival in patients awaiting lung transplantation. J Heart Lung Transplant 2008; 27 (07) 729-734
• 10 Hayes Jr D, Higgins RS, Black SM. , et al. Effect of pulmonary hypertension on survival in patients with idiopathic pulmonary fibrosis after lung transplantation: an analysis of the United Network of Organ Sharing registry. J Heart Lung Transplant 2015; 34 (03) 430-437
• 11 Tantisira KG, Systrom DM, Ginns LC. An elevated breathing reserve index at the lactate threshold is a predictor of mortality in patients with cystic fibrosis awaiting lung transplantation. Am J Respir Crit Care Med 2002; 165 (12) 1629-1633
• 12 Ramos KJ, Quon BS, Heltshe SL. , et al. Heterogeneity in survival in adult patients with cystic fibrosis with FEV1 < 30% of predicted in the united states. Chest 2017; 151 (06) 1320-1328
• 13 Mayer-Hamblett N, Rosenfeld M, Emerson J, Goss CH, Aitken ML. Developing cystic fibrosis lung transplant referral criteria using predictors of 2-year mortality. Am J Respir Crit Care Med 2002; 166 (12, Pt 1): 1550-1555
• 14 Belkin RA, Henig NR, Singer LG. , et al. Risk factors for death of patients with cystic fibrosis awaiting lung transplantation. Am J Respir Crit Care Med 2006; 173 (06) 659-666
• 15 Ellaffi M, Vinsonneau C, Coste J. , et al. One-year outcome after severe pulmonary exacerbation in adults with cystic fibrosis. Am J Respir Crit Care Med 2005; 171 (02) 158-164
• 16 Ramos KJ, Smith PJ, McKone EF. , et al; CF Lung Transplant Referral Guidelines Committee. Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines. J Cyst Fibros 2019; 18 (03) 321-333
• 17 Lynch III JP, Sayah DM, Belperio JA, Weigt SS. Lung transplantation for cystic fibrosis: results, indications, complications, and controversies. Semin Respir Crit Care Med 2015; 36 (02) 299-320
• 18 Hadjiliadis D, Steele MP, Chaparro C. , et al. Survival of lung transplant patients with cystic fibrosis harboring panresistant bacteria other than Burkholderia cepacia, compared with patients harboring sensitive bacteria. J Heart Lung Transplant 2007; 26 (08) 834-838
• 19 Dobbin C, Maley M, Harkness J. , et al. The impact of pan-resistant bacterial pathogens on survival after lung transplantation in cystic fibrosis: results from a single large referral centre. J Hosp Infect 2004; 56 (04) 277-282
• 20 Liou TG, Adler FR, Huang D. Use of lung transplantation survival models to refine patient selection in cystic fibrosis. Am J Respir Crit Care Med 2005; 171 (09) 1053-1059
• 21 Vos R, Vanaudenaerde BM, Geudens N, Dupont LJ, Van Raemdonck DE, Verleden GM. Pseudomonal airway colonisation: risk factor for bronchiolitis obliterans syndrome after lung transplantation?. Eur Respir J 2008; 31 (05) 1037-1045
• 22 Aaron SD, Vandemheen KL, Ferris W. , et al. Combination antibiotic susceptibility testing to treat exacerbations of cystic fibrosis associated with multiresistant bacteria: a randomised, double-blind, controlled clinical trial. Lancet 2005; 366 (9484): 463-471
• 23 Haja Mydin H, Corris PA, Nicholson A. , et al. Targeted antibiotic prophylaxis for lung transplantation in cystic fibrosis patients colonised with Pseudomonas aeruginosa using multiple combination bactericidal testing. J Transplant 2012; 2012: 135738
• 24 Moore CA, Pilewski JM, Venkataramanan R. , et al. Effect of aerosolized antipseudomonals on Pseudomonas positivity and bronchiolitis obliterans syndrome after lung transplantation. Transpl Infect Dis 2017; 19 (03) e12688
• 25 LiPuma JJ. Burkholderia and emerging pathogens in cystic fibrosis. Semin Respir Crit Care Med 2003; 24 (06) 681-692
• 26 Lynch III JP. Burkholderia cepacia complex: impact on the cystic fibrosis lung lesion. Semin Respir Crit Care Med 2009; 30 (05) 596-610
• 27 Holden MTG, Seth-Smith HMB, Crossman LC. , et al. The genome of Burkholderia cenocepacia J2315, an epidemic pathogen of cystic fibrosis patients. J Bacteriol 2009; 191 (01) 261-277
• 28 Chaparro C, Maurer J, Gutierrez C. , et al. Infection with Burkholderia cepacia in cystic fibrosis: outcome following lung transplantation. Am J Respir Crit Care Med 2001; 163 (01) 43-48
• 29 de Perrot M, Chaparro C, McRae K. , et al. Twenty-year experience of lung transplantation at a single center: Influence of recipient diagnosis on long-term survival. J Thorac Cardiovasc Surg 2004; 127 (05) 1493-1501
• 30 Murray S, Charbeneau J, Marshall BC, LiPuma JJ. Impact of burkholderia infection on lung transplantation in cystic fibrosis. Am J Respir Crit Care Med 2008; 178 (04) 363-371
• 31 Alexander BD, Petzold EW, Reller LB. , et al. Survival after lung transplantation of cystic fibrosis patients infected with Burkholderia cepacia complex. Am J Transplant 2008; 8 (05) 1025-1030
• 32 Boussaud V, Guillemain R, Grenet D. , et al. Clinical outcome following lung transplantation in patients with cystic fibrosis colonised with Burkholderia cepacia complex: results from two French centres. Thorax 2008; 63 (08) 732-737
• 33 Aslam S, Courtwright AM, Koval C. , et al. Early clinical experience of bacteriophage therapy in 3 lung transplant recipients. Am J Transplant 2019; 19 (09) 2631-2639
• 34 Aguilar-Guisado M, Givaldá J, Ussetti P. , et al; RESITRA cohort. Pneumonia after lung transplantation in the RESITRA Cohort: a multicenter prospective study. Am J Transplant 2007; 7 (08) 1989-1996
• 35 Bar-On O, Mussaffi H, Mei-Zahav M. , et al. Increasing nontuberculous mycobacteria infection in cystic fibrosis. J Cyst Fibros 2015; 14 (01) 53-62
• 36 Olivier KN, Weber DJ, Wallace Jr RJ. , et al; Nontuberculous Mycobacteria in Cystic Fibrosis Study Group. Nontuberculous mycobacteria. I: multicenter prevalence study in cystic fibrosis. Am J Respir Crit Care Med 2003; 167 (06) 828-834
• 37 Malouf MA, Glanville AR. The spectrum of mycobacterial infection after lung transplantation. Am J Respir Crit Care Med 1999; 160 (5, Pt 1): 1611-1616
• 38 Chernenko SM, Humar A, Hutcheon M. , et al. Mycobacterium abscessus infections in lung transplant recipients: the international experience. J Heart Lung Transplant 2006; 25 (12) 1447-1455
• 39 Chalermskulrat W, Sood N, Neuringer IP. , et al. Non-tuberculous mycobacteria in end stage cystic fibrosis: implications for lung transplantation. Thorax 2006; 61 (06) 507-513
• 40 Lobo LJ, Chang LC, Esther Jr CR, Gilligan PH, Tulu Z, Noone PG. Lung transplant outcomes in cystic fibrosis patients with pre-operative Mycobacterium abscessus respiratory infections. Clin Transplant 2013; 27 (04) 523-529
• 41 Gilljam M, Scherstén H, Silverborn M, Jönsson B, Ericsson Hollsing A. Lung transplantation in patients with cystic fibrosis and Mycobacterium abscessus infection. J Cyst Fibros 2010; 9 (04) 272-276
• 42 Qvist T, Pressler T, Thomsen VO, Skov M, Iversen M, Katzenstein TL. Nontuberculous mycobacterial disease is not a contraindication to lung transplantation in patients with cystic fibrosis: a retrospective analysis in a Danish patient population. Transplant Proc 2013; 45 (01) 342-345
• 43 Perez AA, Singer JP, Schwartz BS. , et al. Management and clinical outcomes after lung transplantation in patients with pre-transplant Mycobacterium abscessus infection: a single center experience. Transpl Infect Dis 2019; 21 (03) e13084
• 44 Hamad Y, Pilewski JM, Morrell M, D'Cunha J, Kwak EJ. Outcomes in lung transplant recipients with mycobacterium abscessus infection: a 15-year experience from a large tertiary care center. Transplant Proc 2019; 51 (06) 2035-2042
• 45 Dedrick RM, Guerrero-Bustamante CA, Garlena RA. , et al. Engineered bacteriophages for treatment of a patient with a disseminated drug-resistant Mycobacterium abscessus. Nat Med 2019; 25 (05) 730-733
• 46 Flume PA, Egan TM, Paradowski LJ, Detterbeck FC, Thompson JT, Yankaskas JR. Infectious complications of lung transplantation. Impact of cystic fibrosis. Am J Respir Crit Care Med 1994; 149 (06) 1601-1607
• 47 Mortensen KL, Johansen HK, Fuursted K. , et al. A prospective survey of Aspergillus spp. in respiratory tract samples: prevalence, clinical impact and antifungal susceptibility. Eur J Clin Microbiol Infect Dis 2011; 30 (11) 1355-1363
• 48 Hong G, Psoter KJ, Jennings MT. , et al. Risk factors for persistent Aspergillus respiratory isolation in cystic fibrosis. J Cyst Fibros 2018; 17 (05) 624-630
• 49 Luong M-L, Chaparro C, Stephenson A. , et al. Pretransplant Aspergillus colonization of cystic fibrosis patients and the incidence of post-lung transplant invasive aspergillosis. Transplantation 2014; 97 (03) 351-357
• 50 Bhaskaran A, Hosseini-Moghaddam SM, Rotstein C, Husain S. Mold infections in lung transplant recipients. Semin Respir Crit Care Med 2013; 34 (03) 371-379
• 51 Weigt SS, Copeland CAF, Derhovanessian A. , et al. Colonization with small conidia Aspergillus species is associated with bronchiolitis obliterans syndrome: a two-center validation study. Am J Transplant 2013; 13 (04) 919-927
• 52 Rammaert B, Puyade M, Cornely OA. , et al; SCEDO-LUNG collaborative group. Perspectives on Scedosporium species and Lomentospora prolificans in lung transplantation: results of an international practice survey from ESCMID fungal infection study group and study group for infections in compromised hosts, and European Confederation of Medical Mycology. Transpl Infect Dis 2019; 21 (05) e13141
• 53 Parize P, Boussaud V, Poinsignon V. , et al. Clinical outcome of cystic fibrosis patients colonized by Scedosporium species following lung transplantation: a single-center 15-year experience. Transpl Infect Dis 2017; 19 (05) e12738
• 54 Morlacchi LC, Greer M, Tudorache I. , et al. The burden of sinus disease in cystic fibrosis lung transplant recipients. Transpl Infect Dis 2018; 20 (05) e12924
• 55 Walter S, Gudowius P, Bosshammer J. , et al. Epidemiology of chronic Pseudomonas aeruginosa infections in the airways of lung transplant recipients with cystic fibrosis. Thorax 1997; 52 (04) 318-321
• 56 Mainz JG, Hentschel J, Schien C. , et al. Sinonasal persistence of Pseudomonas aeruginosa after lung transplantation. J Cyst Fibros 2012; 11 (02) 158-161
• 57 Vital D, Hofer M, Benden C, Holzmann D, Boehler A. Impact of sinus surgery on pseudomonal airway colonization, bronchiolitis obliterans syndrome and survival in cystic fibrosis lung transplant recipients. Respiration 2013; 86 (01) 25-31
• 58 Syed SA, Whelan FJ, Waddell B, Rabin HR, Parkins MD, Surette MG. Reemergence of lower-airway microbiota in lung transplant patients with cystic fibrosis. Ann Am Thorac Soc 2016; 13 (12) 2132-2142
• 59 Choi KJ, Cheng TZ, Honeybrook AL. , et al. Correlation between sinus and lung cultures in lung transplant patients with cystic fibrosis. Int Forum Allergy Rhinol 2018; 8 (03) 389-393
• 60 Habib A-RR, Buxton JA, Singer J, Wilcox PG, Javer AR, Quon BS. Association between chronic rhinosinusitis and health-related quality of life in adults with cystic fibrosis. Ann Am Thorac Soc 2015; 12 (08) 1163-1169
• 61 Kang SH, Meotti CD, Bombardelli K, Piltcher OB, de Tarso Roth Dalcin P. Sinonasal characteristics and quality of life by SNOT-22 in adult patients with cystic fibrosis. Eur Arch Otorhinolaryngol 2017; 274 (04) 1873-1882
• 62 Leung M-K, Rachakonda L, Weill D, Hwang PH. Effects of sinus surgery on lung transplantation outcomes in cystic fibrosis. Am J Rhinol 2008; 22 (02) 192-196
• 63 Holzmann D, Speich R, Kaufmann T. , et al. Effects of sinus surgery in patients with cystic fibrosis after lung transplantation: a 10-year experience. Transplantation 2004; 77 (01) 134-136
• 64 Flume PA, Strange C, Ye X, Ebeling M, Hulsey T, Clark LL. Pneumothorax in cystic fibrosis. Chest 2005; 128 (02) 720-728
• 65 Rolla M, D'Andrilli A, Rendina EA, Diso D, Venuta F. Cystic fibrosis and the thoracic surgeon. Eur J Cardiothorac Surg 2011; 39 (05) 716-725
• 66 Mooney JJ, Bhattacharya J, Dhillon GS. Effect of broader geographic sharing of donor lungs on lung transplant waitlist outcomes. J Heart Lung Transplant 2019; 38 (02) 136-144
• 67 Valapour M, Lehr CJ, Skeans MA. , et al. OPTN/SRTR 2017 annual data report: lung. Am J Transplant 2019; 19 (Suppl. 02) 404-484
• 68 Spahr JE, Love RB, Francois M, Radford K, Meyer KC. Lung transplantation for cystic fibrosis: current concepts and one center's experience. J Cyst Fibros 2007; 6 (05) 334-350
• 69 Singer JP, Blanc PD, Hoopes C. , et al. The impact of pretransplant mechanical ventilation on short- and long-term survival after lung transplantation. Am J Transplant 2011; 11 (10) 2197-2204
• 70 Bermudez CA, Rocha RV, Zaldonis D. , et al. Extracorporeal membrane oxygenation as a bridge to lung transplant: midterm outcomes. Ann Thorac Surg 2011; 92 (04) 1226-1231 , discussion 1231–1232
• 71 Toyoda Y, Bhama JK, Shigemura N. , et al. Efficacy of extracorporeal membrane oxygenation as a bridge to lung transplantation. J Thorac Cardiovasc Surg 2013; 145 (04) 1065-1071
• 72 Hayanga AJ, Du AL, Joubert K. , et al. Mechanical ventilation and extracorporeal membrane oxygenation as a bridging strategy to lung transplantation: significant gains in survival. Am J Transplant 2018; 18 (01) 125-135
• 73 Hayes Jr D, Kukreja J, Tobias JD, Ballard HO, Hoopes CW. Ambulatory venovenous extracorporeal respiratory support as a bridge for cystic fibrosis patients to emergent lung transplantation. J Cyst Fibros 2012; 11 (01) 40-45
• 74 Gelfond D, Borowitz D. Gastrointestinal complications of cystic fibrosis. Clin Gastroenterol Hepatol 2013; 11 (04) 333-342 , quiz e30–e31
• 75 Mendez BM, Davis CS, Weber C, Joehl RJ, Fisichella PM. Gastroesophageal reflux disease in lung transplant patients with cystic fibrosis. Am J Surg 2012; 204 (05) e21-e26
• 76 Leung DH, Narkewicz MR. Cystic fibrosis-related cirrhosis. J Cyst Fibros 2017; 16 (Suppl. 02) S50-S61
• 77 Nash EF, Volling C, Gutierrez CA. , et al. Outcomes of patients with cystic fibrosis undergoing lung transplantation with and without cystic fibrosis-associated liver cirrhosis. Clin Transplant 2012; 26 (01) 34-41
• 78 Freischlag KW, Messina J, Ezekian B. , et al. Single-center long-term analysis of combined liver-lung transplant outcomes. Transplant Direct 2018; 4 (05) e349
• 79 Stephenson AL, Sykes J, Berthiaume Y. , et al. Clinical and demographic factors associated with post-lung transplantation survival in individuals with cystic fibrosis. J Heart Lung Transplant 2015; 34 (09) 1139-1145
• 80 Tsang VT, Johnston A, Heritier F, Leaver N, Hodson ME, Yacoub M. Cyclosporin pharmacokinetics in heart-lung transplant recipients with cystic fibrosis. Effects of pancreatic enzymes and ranitidine. Eur J Clin Pharmacol 1994; 46 (03) 261-265
• 81 Weill D, Benden C, Corris PA. , et al. A consensus document for the selection of lung transplant candidates: 2014--an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2015; 34 (01) 1-15
• 82 Hollander FM, van Pierre DD, de Roos NM, van de Graaf EA, Iestra JA. Effects of nutritional status and dietetic interventions on survival in cystic fibrosis patients before and after lung transplantation. J Cyst Fibros 2014; 13 (02) 212-218
• 83 Koutsokera A, Varughese RA, Sykes J. , et al. Pre-transplant factors associated with mortality after lung transplantation in cystic fibrosis: a systematic review and meta-analysis. J Cyst Fibros 2019; 18 (03) 407-415
• 84 Ramos KJ, Kapnadak DG, Somayaji R, Morrell ED, Pilewski JM, Lease ED. , et al. Underweight patients with cystic fibrosis have acceptable survival after lung transplant: A UNOS registry study. Chest, in preparation
• 85 Singer LG, Brazelton TR, Doyle RL, Morris RE, Theodore J. ; International Lung Transplant Database Study Group. Weight gain after lung transplantation. J Heart Lung Transplant 2003; 22 (08) 894-902
• 86 Moran A, Dunitz J, Nathan B, Saeed A, Holme B, Thomas W. Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care 2009; 32 (09) 1626-1631
• 87 Moran A, Brunzell C, Cohen RC. , et al; CFRD Guidelines Committee. Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care 2010; 33 (12) 2697-2708
• 88 Brennan AL, Geddes DM, Gyi KM, Baker EH. Clinical importance of cystic fibrosis-related diabetes. J Cyst Fibros 2004; 3 (04) 209-222
• 89 Bismuth E, Laborde K, Taupin P. , et al. Glucose tolerance and insulin secretion, morbidity, and death in patients with cystic fibrosis. J Pediatr 2008; 152 (04) 540-545 , 545.e1
• 90 Hadjiliadis D, Madill J, Chaparro C. , et al. Incidence and prevalence of diabetes mellitus in patients with cystic fibrosis undergoing lung transplantation before and after lung transplantation. Clin Transplant 2005; 19 (06) 773-778
• 91 Heisel O, Heisel R, Balshaw R, Keown P. New onset diabetes mellitus in patients receiving calcineurin inhibitors: a systematic review and meta-analysis. Am J Transplant 2004; 4 (04) 583-595
• 92 Weiss ES, Allen JG, Modi MN, Merlo CA, Conte JV, Shah AS. Lung transplantation in older patients with cystic fibrosis: analysis of UNOS data. J Heart Lung Transplant 2009; 28 (02) 135-140
• 93 Hackman KL, Snell GI, Bach LA. Poor glycemic control is associated with decreased survival in lung transplant recipients. Transplantation 2017; 101 (09) 2200-2206
• 94 Aris RM, Renner JB, Winders AD. , et al. Increased rate of fractures and severe kyphosis: sequelae of living into adulthood with cystic fibrosis. Ann Intern Med 1998; 128 (03) 186-193
• 95 Paccou J, Zeboulon N, Combescure C, Gossec L, Cortet B. The prevalence of osteoporosis, osteopenia, and fractures among adults with cystic fibrosis: a systematic literature review with meta-analysis. Calcif Tissue Int 2010; 86 (01) 1-7
• 96 Sheikh S, Gemma S, Patel A. Factors associated with low bone mineral density in patients with cystic fibrosis. J Bone Miner Metab 2015; 33 (02) 180-185
• 97 Spira A, Gutierrez C, Chaparro C, Hutcheon MA, Chan CK. Osteoporosis and lung transplantation: a prospective study. Chest 2000; 117 (02) 476-481
• 98 Conwell LS, Chang AB. Bisphosphonates for osteoporosis in people with cystic fibrosis. Cochrane Database Syst Rev 2014; (03) CD002010
• 99 Egan TM, Murray S, Bustami RT. , et al. Development of the new lung allocation system in the United States. Am J Transplant 2006; 6 (5, Pt 2): 1212-1227
• 100 Egan TM, Edwards LB. Effect of the lung allocation score on lung transplantation in the United States. J Heart Lung Transplant 2016; 35 (04) 433-439
• 101 Glazier A. The lung lawsuit: a case study in organ allocation policy and administrative law. J Health Biomed Law 2018; XIV: 139-148
• 102 Valapour M, Lehr CJ, Skeans MA. , et al. OPTN/SRTR 2016 annual data report: lung. Am J Transplant 2018; 18 (Suppl. 01) 363-433
• 103 Vock DM, Durheim MT, Tsuang WM. , et al. Survival benefit of lung transplantation in the modern era of lung allocation. Ann Am Thorac Soc 2017; 14 (02) 172-181
• 104 Lehr CJ, Skeans M, Dasenbrook EC. , et al. Effect of including important clinical variables on accuracy of the lung allocation score for cystic fibrosis and chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2019; 200 (08) 1013-1021
• 105 Snell GI, Yusen RD, Weill D. , et al. Report of the ISHLT Working Group on Primary Lung Graft Dysfunction, part I: definition and grading-a 2016 consensus group statement of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2017; 36 (10) 1097-1103
• 106 Prekker ME, Nath DS, Walker AR. , et al. Validation of the proposed International Society for Heart and Lung Transplantation grading system for primary graft dysfunction after lung transplantation. J Heart Lung Transplant 2006; 25 (04) 371-378
• 107 Crespo MM, McCarthy DP, Hopkins PM. , et al. ISHLT consensus statement on adult and pediatric airway complications after lung transplantation: definitions, grading system, and therapeutics. J Heart Lung Transplant 2018; 37 (05) 548-563
• 108 Cantu E, Diamond JM, Suzuki Y. , et al; Lung Transplant Outcomes Group. Quantitative evidence for revising the definition of primary graft dysfunction after lung transplant. Am J Respir Crit Care Med 2018; 197 (02) 235-243
• 109 Morrell MR, Pilewski JM, Gries CJ. , et al. De novo donor-specific HLA antibodies are associated with early and high-grade bronchiolitis obliterans syndrome and death after lung transplantation. J Heart Lung Transplant 2014; 33 (12) 1288-1294
• 110 Verleden SE, Vanaudenaerde BM, Emonds MP. , et al. Donor-specific and -nonspecific HLA antibodies and outcome post lung transplantation. Eur Respir J 2017 50(05). doi: 10.1183/13993003.01248-2017
• 111 Safavi S, Robinson DR, Soresi S, Carby M, Smith JD. De novo donor HLA-specific antibodies predict development of bronchiolitis obliterans syndrome after lung transplantation. J Heart Lung Transplant 2014; 33 (12) 1273-1281
• 112 Verleden GM, Glanville AR, Lease ED. , et al. Chronic lung allograft dysfunction: definition, diagnostic criteria, and approaches to treatment-a consensus report from the Pulmonary Council of the ISHLT. J Heart Lung Transplant 2019; 38 (05) 493-503
• 113 Glanville AR, Verleden GM, Todd JL. , et al. Chronic lung allograft dysfunction: definition and update of restrictive allograft syndrome-a consensus report from the Pulmonary Council of the ISHLT. J Heart Lung Transplant 2019; 38 (05) 483-492
• 114 Belloli EA, Degtiar I, Wang X. , et al. Parametric response mapping as an imaging biomarker in lung transplant recipients. Am J Respir Crit Care Med 2017; 195 (07) 942-952
• 115 Ruttens D, Verleden SE, Vandermeulen E. , et al. Prophylactic azithromycin therapy after lung transplantation: post hoc analysis of a randomized controlled trial. Am J Transplant 2016; 16 (01) 254-261
• 116 Van Herck A, Frick AE, Schaevers V. , et al. Azithromycin and early allograft function after lung transplantation: a randomized, controlled trial. J Heart Lung Transplant 2019; 38 (03) 252-259
• 117 Vos R, Eynde RV, Ruttens D. , et al; Leuven Lung Transplant Group. Montelukast in chronic lung allograft dysfunction after lung transplantation. J Heart Lung Transplant 2019; 38 (05) 516-527
• 118 Kulkarni HS, Cherikh WS, Chambers DC. , et al. Bronchiolitis obliterans syndrome-free survival after lung transplantation: an International Society for Heart and Lung Transplantation Thoracic Transplant Registry analysis. J Heart Lung Transplant 2019; 38 (01) 5-16
• 119 Martin C, Hamard C, Kanaan R. , et al. Causes of death in French cystic fibrosis patients: the need for improvement in transplantation referral strategies!. J Cyst Fibros 2016; 15 (02) 204-212
• 120 Sweet SC, Aurora P, Benden C. , et al; International Pediatric Lung Transplant Collaborative. Lung transplantation and survival in children with cystic fibrosis: solid statistics--flawed interpretation. Pediatr Transplant 2008; 12 (02) 129-136
• 121 Hayes Jr D, Glanville AR, McGiffin D, Tobias JD, Tumin D. Age-related survival disparity associated with lung transplantation in cystic fibrosis: an analysis of the registry of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2016; 35 (09) 1108-1115
• 122 Hofer M, Benden C, Inci I. , et al. True survival benefit of lung transplantation for cystic fibrosis patients: the Zurich experience. J Heart Lung Transplant 2009; 28 (04) 334-339
• 123 Gruber S, Eiwegger T, Nachbaur E. , et al. Lung transplantation in children and young adults: a 20-year single-centre experience. Eur Respir J 2012; 40 (02) 462-469
• 124 Singer LG, Chowdhury NA, Faughnan ME. , et al. Effects of recipient age and diagnosis on health-related quality-of-life benefit of lung transplantation. Am J Respir Crit Care Med 2015; 192 (08) 965-973
• 125 Flass T, Narkewicz MR. Cirrhosis and other liver disease in cystic fibrosis. J Cyst Fibros 2013; 12 (02) 116-124
• 126 Mendizabal M, Reddy KR, Cassuto J. , et al. Liver transplantation in patients with cystic fibrosis: analysis of United Network for Organ Sharing data. Liver Transpl 2011; 17 (03) 243-250
• 127 Noble-Jamieson G, Barnes ND. Liver transplantation for cirrhosis in cystic fibrosis. J Pediatr 1996; 129 (02) 314
• 128 Milkiewicz P, Skiba G, Kelly D. , et al. Transplantation for cystic fibrosis: outcome following early liver transplantation. J Gastroenterol Hepatol 2002; 17 (02) 208-213
• 129 Martin P, DiMartini A, Feng S, Brown Jr R, Fallon M. Evaluation for liver transplantation in adults: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology 2014; 59 (03) 1144-1165
• 130 Squires RH, Ng V, Romero R. , et al; American Association for the Study of Liver Diseases; American Society of Transplantation; North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline by the American Association for the Study of Liver Diseases, American Society of Transplantation and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr 2014; 59 (01) 112-131
• 131 Freeman AJ, Sellers ZM, Mazariegos G. , et al. A multidisciplinary approach to pretransplant and posttransplant management of cystic fibrosis-associated liver disease. Liver Transpl 2019; 25 (04) 640-657
• 132 Dowman JK, Watson D, Loganathan S. , et al. Long-term impact of liver transplantation on respiratory function and nutritional status in children and adults with cystic fibrosis. Am J Transplant 2012; 12 (04) 954-964
• 133 Polineni D, Piccorelli AV, Hannah WB. , et al. Analysis of a large cohort of cystic fibrosis patients with severe liver disease indicates lung function decline does not significantly differ from that of the general cystic fibrosis population. PLoS One 2018; 13 (10) e0205257
• 134 Miller MR, Sokol RJ, Narkewicz MR, Sontag MK. Pulmonary function in individuals who underwent liver transplantation: from the US Cystic Fibrosis Foundation registry. Liver Transpl 2012; 18 (05) 585-593
• 135 Neofytos D, Chatzis O, Nasioudis D. , et al; Swiss Transplant Cohort Study. Epidemiology, risk factors and outcomes of invasive aspergillosis in solid organ transplant recipients in the Swiss Transplant Cohort Study. Transpl Infect Dis 2018; 20 (04) e12898
• 136 Husain S, Silveira FP, Azie N, Franks B, Horn D. Epidemiological features of invasive mold infections among solid organ transplant recipients: PATH Alliance® registry analysis. Med Mycol 2017; 55 (03) 269-277
• 137 Desai CS, Gruessner A, Habib S, Gruessner R, Khan KM. Survival of cystic fibrosis patients undergoing liver and liver-lung transplantations. Transplant Proc 2013; 45 (01) 290-292
• 138 Black SM, Woodley FW, Tumin D. , et al. Cystic fibrosis associated with worse survival after liver transplantation. Dig Dis Sci 2016; 61 (04) 1178-1185
• 139 Bandsma RHJ, Bozic MA, Fridell JA. , et al. Simultaneous liver-pancreas transplantation for cystic fibrosis-related liver disease: a multicenter experience. J Cyst Fibros 2014; 13 (04) 471-477
• 140 Usatin DJ, Perito ER, Posselt AM, Rosenthal P. Under utilization of pancreas transplants in cystic fibrosis recipients in the United Network Organ Sharing (UNOS) data 1987–2014. Am J Transplant 2016; 16 (05) 1620-1625
• 141 Kimura N, Khan MS, Schecter M. , et al. Changing demographics and outcomes of lung transplantation recipients with cystic fibrosis. J Heart Lung Transplant 2016; 35 (10) 1237-1244
• 142 Arnon R, Annunziato RA, Miloh T. , et al. Liver and combined lung and liver transplantation for cystic fibrosis: analysis of the UNOS database. Pediatr Transplant 2011; 15 (03) 254-264
• 143 Wolf JH, Sulewski ME, Cassuto JR. , et al. Simultaneous thoracic and abdominal transplantation: can we justify two organs for one recipient?. Am J Transplant 2013; 13 (07) 1806-1816
• 144 Pinderski LJ, Kirklin JK, McGiffin D. , et al. Multi-organ transplantation: is there a protective effect against acute and chronic rejection?. J Heart Lung Transplant 2005; 24 (11) 1828-1833