title: Results of heart-lung transplantation in children with cystic fibrosis.
abstract: Children with cystic fibrosis represent the largest group referred for, and undergoing, heart-lung transplantation at our institute. Between June 1988 and July 1993, 76 patients were accepted for transplantation, of whom 25 were transplanted, while a further 36 died waiting. Those transplanted ranged from 5-18 years of age and included 13 males and 12 females. Organs were used from donors matched by ABO blood group, size and cytomegalovirus (CMV) status. Post-transplant maintenance immunosuppression comprised cyclosporin A, azathioprine and prednisolone. Anti-thymocyte globulin and high dose methylprednisolone were given peri-operatively and for acute rejection episodes. Actuarial survival was 67% at 1 year, 61% at 2 years and 54% at 3 years. Obliterative bronchiolitis (OB) has occurred in 13 patients (52%) and was the major cause of mortality and morbidity. In three patients, OB was associated with the development of tracheal anastomotic stenosis. Other complications included diabetes mellitus (n = 9), pancreatitis (n = 1) and hypertension (n = 8). Despite these problems, those surviving the first year post-transplant showed a mean FEV1 of 71% (compared to 29% pre-transplant) and enjoyed an overall improved quality of life.
authors.affiliation: Cardiothoracic Unit, Hospital for Sick Children, London, UK.
authors.forename: B F
authors.initials: BF
authors.lastname: Whitehead
authors.forename: P G
authors.initials: PG
authors.lastname: Rees
authors.forename: K
authors.initials: K
authors.lastname: Sorensen
authors.forename: C
authors.initials: C
authors.lastname: Bull
authors.forename: J
authors.initials: J
authors.lastname: Fabre
authors.forename: M R
authors.initials: MR
authors.lastname: de Leval
authors.forename: M J
authors.initials: MJ
authors.lastname: Elliott
mesh_terms: D000206:Actuarial Analysis; D000293:Adolescent; D001989:Bronchiolitis Obliterans / Q000453:epidemiology* / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume; D006084:Graft Rejection / Q000453:epidemiology* / Q000209:etiology; D016041:Heart-Lung Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D008297:Male; D011182:Postoperative Care; D011183:Postoperative Complications / Q000453:epidemiology* / Q000209:etiology; D012141:Respiratory Tract Infections / Q000453:epidemiology* / Q000382:microbiology; D015996:Survival Rate; D013997:Time Factors; D014019:Tissue Donors
chemical_list: D007166:Immunosuppressive Agents