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ISHLT Monograph:
Pediatric Lung Transplantation

Christian Benden, Stuart C Sweet, Samuel Goldfarb and James K Kirklin

Why a monograph of Pediatric lung transplantation?
Rationale: Pediatric lung transplantation has been performed since the 1980s and has evolved as an accepted therapy in selected children with end-stage parenchymal and vascular lung diseases, as it offers prolongation of life and improved health-related quality of life. More than 1,500 pediatric lung and heart-lung transplant operations have been performed in children to date according to the recent Pediatric Registry Report of the International Society for Heart and Lung Transplantation (ISHLT). The total number of pediatric lung transplants undertaken is approximately 60 to 70 per year. There are less than 30 established pediatric lung transplant programs worldwide, only two of which perform 10-20 transplants annually. The vast majority of pediatric centers carry out less than five transplants each year. In addition, adolescent patients, in particular with cystic fibrosis, undergo lung transplantation in many of the adult lung transplant programs.

However, pediatric lung transplantation presents a specific challenge as children who undergo transplantation are not 'just small adults'. Pediatric candidates show distinct differences with regards to their underlying lung disease, a challenging surgical approach, the effects of immunosuppressant drugs, the impact of infections on the developing immune system and the child's somatic growth.

To date, the published literature in the field of pediatric lung transplantation consists mostly of single-center reports including small patient cohorts, mainly retrospective studies. The International Pediatric Lung Transplant Collaborative (IPLTC) has been established to increase the cumulative knowledge in the growing field of pediatric lung transplantation. In a first step, members of the IPLTC have recently agreed upon a unified protocol of post-transplant management for pediatric lung transplant recipients. The issue of a Monograph on Pediatric Lung Transplantation by the ISHLT would be an experience-based rather than an evidence-based document, offering reference to established pediatric lung transplant centers and those being newly set up. In addition, adult lung transplant programs performing lung transplants in adolescent patients receive additional information, particular important for that age group, including information about adherence and compliance to therapy.

Furthermore, a Monograph on Pediatric Lung Transplantation would help efforts to harmonize management protocols further, enabling more multi-center collaborations, necessary to adequately power prospective research studies in the future.

Outline of topics:

  1. Referral and transplant criteria (contraindications)
  2. Timing of listing and patient management on the waiting list
  3. Pediatric aspects of lung transplant surgery - including historic perspective
  4. Pediatric issues of donor selection -How to increase the donor pool?
  5. Early post-operative management on PICU (Pediatric ECMO support)
  6. Immunosuppression (induction therapy/maintenance immunosuppression)
  7. Infectious diseases issues including anti-infective prophylaxis and therapy
  8. Monitoring for acute allograft rejection including antibody-mediated rejection
  9. Monitoring for Bronchiolitis Obliterans Syndrome (BOS)
  10. Treatment of Bronchiolitis Obliterans Syndrome (BOS)
  11. Co-morbidities after pediatric lung transplantation including CKD, DM etc
  12. PTLD and cancer
  13. Survival and functional status after pediatric lung transplantation
  14. Living donor lung transplantation
  15. Pediatric aspect of lung re-transplantation
  16. Multi-organ transplantation
  17. Growth and development
  18. Developmental immunology
  19. Adherence and health-related quality of life/non-survival related outcome
  20. Cognitive function/education and school performance
  21. Transition from pediatric to adult care

The ISHLT Monograph on Pediatric Lung Transplantation is anticipated to be published in April 2013.