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Pediatric lung transplantation is the most aggressive therapeutic option for children with end-stage pulmonary disease, but it remains a relatively rare operation. Major diagnoses necessitating transplant vary according to recipient age group. Cystic fibrosis is the most common indication in children ?6 years, while pulmonary hypertension and surfactant disorders account for the majority of cases in infants 1 year of age. Lungs are unique in that they remain directly exposed to the external environment with its infective and immunologic challenges and therefore high levels of immunosuppression are required. Acute rejection affects the majority of lung transplant recipients and typically is seen during the first 3 months post transplantation. It may be asymptomatic or present with nonspecific symptoms mimicking infection, including cough, fever, hypoxemia, tachypnea, or dyspnea. This chapter discusses the perioperative management of a patient post lung transplantation with symptomatology for diagnostic evaluation.
This chapter addresses aspects of lung transplantation (LT) that are unique to infants, children, and adolescents. The primary diagnoses leading to LT in the pediatric age group are cystic fibrosis (CF) and pulmonary hypertension, either idiopathic or related to congenital heart disease. The main difference in surgical technique relates to the increased use of bypass. The vast majority of pediatric LT recipients receive two lungs; for those with CF and other suppurative diseases, the decision is based on the infection risk. Transbronchial biopsies (TBBx) are also more challenging in pediatrics, particularly in infants and toddlers. Graft failure and infection are important causes of death in the first year after transplant. In pediatrics, as few centers perform enough transplants each year to adequately power outcome studies, uniform treatment strategies and multi-center collaborations helps to identify strategies for earlier diagnosis and allow assessment of treatment efficacy.
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