Abstract
Between June 1990 and September 1995, 8 of 24 children with cystic fibrosis (CF) who were accepted either for combined transplantation or isolated liver transplantation died while waiting for a graft; 11 underwent transplantation and 5 are currently on the waiting list. Of the 11 children who had surgery, 7 (group 1) underwent one of the following procedures: heartlung-liver (n = 4), sequential double lung-liver (n = 2), or bilateral lobar lung from a split left lung and reduced liver (n = 1). During the same period, the four other children (group 2) underwent isolated liver transplantation (three full-size livers, one partial liver). There was one perioperative death in each group. Pulmonary infection was the most common cause of morbidity in group 1. Other complications in group 1 included tracheobronchial stenosis (n = 2), biliary stricture (n = 2), and severe ascites (n = 2). All were successfully treated. Obliterative bronchiolitis developed in three patients. This was treated with FK 506. In group 2, pulmonary function tests improved or remained stable after liver transplantation. Surgical complications in group 2 included severe ascites (n =1), biliary stricture (n = 1), and abscess of the liver (n = 1). Actuarial survival was 85.7 % ± 2 % in group 1 at 1 year; it remained unchanged at 3 years and was 64.2 % at 5 years.
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Bismuth H, Houssin D (1984) Reducedsized orthotopic liver graft in hepatic transplantation in children. Surgery 95: 367–370
Broelsch CE, Emond JC, Whitington PF, Thistlethwaite JR, Baker AL, Lichtor JL (1990) Application of reduced-size liver transplants as split grafts, auxiliary orthotopic grafts, and living related segmental transplants. Ann Surg 212: 368–377
Calne RY, Sells RA, Pena JR, Davis DR, Millard PR, Herbertson BM, Binns RM, Davies DAL (1969) Induction of immunological tolerance by porcine liver allografts. Nature 223: 472–476
Colonna JO II, Shaked A, Gomes AS, Colquhoun SD, Jurim O, Mc Diarmid SV, Millis JM, Goldstein LI, Busuittil RW (1992) Biliary strictures complicat ing liver transplantation. Ann Surg 216: 344–352
Cooper JD, Bilingham M, Egan T, et al. (1993) A working formulation for the standardization of nomenclature and for clinical staging of chronic dysfunction in lung allografts. J Heart Lung Transplant 12: 713–716
Couetil JP, Grousset A, Benaim D, Sapoval M, Hernigou A, Coppens P, Fayolle P, Carpentier A (1994-95) Misc au point experimentale de la bipartition pulmonaire avec transplantation lobaire bilaterale chez le chien et application en clinique humaine. Chirurgie 120: 512–517
Couetil JP, Houssin D, Soubrane O, Chevalier P, Dousset B, Loulmet D, Achkar A, Tolan M, Amrein C, Guinvarch A, Guillemain R, Birnbaum P, Carpentier A (1995) Combined lung and liver transplantation in cystic fibrosis. A 4.5 year experience. J Thorac Cardiovasc Surg 110: 1415–1423
Cox KL (1990) The role of liver transplantation in cystic fibrosis patients. Cystic Fibrosis Conference S1.4: 78–79
de Leval MR, Smyth R, Whitehead B, et al. (1991) Heart and lung transplantation for terminal cystic fibrosis: a 4 1/2-year experience. J Thorac Cardiovasc Surg 101: 633–642
Dennis C, Caine N, Sharples L, et al. (1993) Heart lung transplantation for end stage respiratory disease in a cystic fibrosis patient at Papworth Hospital. J Heart Lung Transplant 12: 893–902
Egan TM, Detterbeck FC, Mill RM, Paradowski LJ, Lackner RP, Ogden WD, Yankaskas JR, Westerman JH, Thompson JT, Weiner MA, Cairns EL, Wilcox BR (1995) Improved results of lung transplantation for patients with cystic fibrosis. J Thorac Cardiovasc Surg 109:224–235
Feigelson J, Anagnostopoulos C, Poquet M, Pecan Y, Munck A, Navarro J (1993) Liver cirrhosis in cystic fibrosis — therapeutic implications and long term follow up. Arch Dis Child 68: 653–657
Geddes DM (1988) Cystic fibrosis Future trends in care. Thorax 43: 115–130
Gonwa TA, Nery JR, Husberg BO, Klintmalm GB (1988) Simultaneous liver and renal transplantation in man. Transplantation 46: 690–693
Hobeika J, Houssin D, Bernard O, Devictor D, Grimon G, Chapuis Y (1994) Orthotopic liver transplantation in children with chronic liver disease and severe hypoxemia. Transplantation 57:224–228
Hodson ME, Madden BP, Steven MH, Tsang VT, Yacoub MH (1991) Non-invasive mechanical ventilation for cystic fibrosis patients — a potential bridge to transplantation. Eur Respir J 4: 524–527
Kalayoglu M, Sollinger HW, Stratta RJ, et al (1988) Extended preservation of the liver for clinical transplantation. Lancet 1:617–619
Kamada N, Davies HS, Roser B (1981) Reversal of transplantation immunity by liver grafting. Nature 292: 840–842
Lange PA, Stoller JK (1995) The hepatopulmonary syndrome. Ann Intern Med 122: 521–529
Madden BP, Hodson ME, Tsang V, Radley-Smith R, Khaghani A, Yacoub MH (1992) Intermediate-term results of heart-lung transplantation for cystic fibrosis. Lancet I: 1583–1587
Mieles LA, Orenstein D, Teperman L, Podesta L, Koneru B, Starzl TE (1989) Liver transplantation in cystic fibrosis. Lancet 1: 1073
Noble-Jamieson G, Valente J, Barnes ND, Friend PJ, Jamieson NV, Rasmussen A, Calne RY (1994) Liver transplantation for hepatic cirrhosis in cystic fibrosis. Arch Dis Child 71: 349–352
Park RM, Grand RJ (1981) Gastrointestinal manifestations of cystic fibrosis: a review. Gastroenterology 81: 1143–1161
Pasque MK, Cooper JD, Kaiser LR, Haydock DA, Triantafillou A, Trulock EP (1990) Improved technique for bilateral lung transplantation: rationale and initial clinical experience. Ann Thorac Surg 49: 785–791
Penketh ARL, Wise A, Mearns MB, Hodson ME, Batten JC (1987) Cystic fibrosis in adolescents and adults. Thorax 42: 526–532
Ramirez JC, Patterson GA, Winton TL, de Hoyos AL, Miller JD, Maurer JR (1992) Bilateral lung transplantation for cystic fibrosis. J Thorac Cardiovasc Surg 103:287–294
Scott-Jupp R, Lama M, Tanner MS (1991) Prevalence of liver disease in cystic fibrosis. Arch Dis Child 66: 698–701
Smith J, Wallwork J (1995) Retransplantation in heart-lung recipient with obliterative bronchiolitis (letter). J Thorac Cardiovasc Surg 109: 818
Starnes VA, Lewiston N, Theodore J, Stoehr C, Stinson E, Shumway NE, Oyer PE (1992) Cystic fibrosis. Target population for lung transplantation in North America in the 1990 s. J Thorac Cardiovasc Surg 103: 1008–1014
Starnes VA, Barr ML, Cohen RG (1994) Lobar transplantation. Indications, techniques and outcome. J Thorac Cardiovasc Surg 108: 403–411
Stern RC, Stevens DP, Boat TF, Doershuk CF, Izant RJ Jr, Matthews LW (1976) Symptomatic hepatic disease in cystic fibrosis: incidence, course, and outcome of portal systemic shunting. Gastroenterology 70: 645–649
Van Obbergh L, Carlier M, de Clety SE, Sokal E, Rennotte MT, Veyckemans F, Dekock M, Frans A, Otte JB (1993) Liver transplantation and pulmonary gas exchanges in hypoxemic children. Am Rev Respir Dis 148:1408–1410
Wood A, Higgenbottam T, Jackson M, Scott J, Stewart S, Wallwork J (1989) Airway mucosal bielectric potential difference in cystic fibrosis after lung transplantation. Am Rev Respir Dis 140:1645–1649
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Couetil, J.P.A., Soubrane, O., Houssin, D.P. et al. Combined heart-lung-liver, double lung-liver, and isolated liver transplantation for cystic fibrosis in children. Transplant Int 10, 33–39 (1996). https://doi.org/10.1007/BF02044339
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DOI: https://doi.org/10.1007/BF02044339