Interstitial lung disease as an indication for pediatric lung transplant.

Interstitial lung disease can be an indication for lung transplant at any age, but it is a particularly common indication for lung transplant in infants. Nevertheless, not all interstitial lung diseases will lead to lung transplant in childhood. Genetic testing has aided the identification of these diseases in children. In severely affected patients, however, definitive diagnosis is not always necessary to consider referral to a transplant center. At experienced transplant centers, a multidisciplinary team educates patient families and aids in the transplant evaluation of children with interstitial lung disease. Children who have undergone transplant require lifetime immunosuppression and close surveillance, but can enjoy good quality of life for years following surgery.

American Thoracic Society 2019 Pediatric Core Curriculum.

The American Thoracic Society Pediatric Core Curriculum updates clinicians annually in pediatric pulmonary disease in a 3 to 4 year recurring cycle of topics. The 2019 course was presented in May during the Annual International Conference. An American Board of Pediatrics Maintenance of Certification module and a continuing medical education exercise covering the contents of the Core Curriculum can be accessed online at www.thoracic.org.

Pre-transplant mechanical ventilation increases short-term morbidity and mortality in pediatric patients with cystic fibrosis.

BACKGROUND: Patients with cystic fibrosis (CF) who are listed for lung transplantation may require mechanical ventilatory support before transplant. Although CF is a risk factor for poor outcomes in adults, no data currently exist pertaining to pre-transplant ventilatory support in children with CF. METHODS: In a retrospective cohort study, we reviewed the medical records of 18 consecutive CF patients transplanted at St. Louis Children's Hospital (SLCH) who required mechanical ventilation before lung transplantation. Controls included patients transplanted at SLCH who were not mechanically ventilated before transplant and were matched for underlying diagnosis, gender, age, type of transplant (cadaveric vs living donor) and year of transplant. RESULTS: Ventilated and non-ventilated patients were similar in their pre-transplant characteristics (weight, height and body mass index) and ischemic and bypass times. However, patients ventilated before transplantation had significantly worse immediate post-transplant outcomes, including early graft dysfunction (p = 0.012), prolonged mechanical ventilation (34.1 vs 5 days, p = 0.009), prolonged stay in the pediatric intensive care unit (35.4 vs 8.1 days, p = 0.01), longer time to hospital discharge (38.4 vs 21.3 days, p = 0.033), and worse 1-year mortality after transplant (221.6 vs 335.2 days, p = 0.021). Among ventilated patients, length of pre-transplant ventilation did not affect post-transplant outcomes (length of ventilation, p = 0.92; length of stay in the pediatric intensive care unit, p = 0.68; time to hospital discharge, p = 0.46; and 1-year mortality rate, p = 0.25). CONCLUSIONS: This is the first report in pediatric patients with CF demonstrating that mechanical ventilation before lung transplant is a predictor of poor short-term outcomes, including 1-year-survival, after transplant. Length of pre-transplant ventilatory support does not appear to impact outcomes.