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BACKGROUND Congenital pulmonary vein stenosis (PVS) is associated with high mortality because surgical repair is usually not feasible or is ineffective. In addition, acquired PVS after repair of congenital heart disease is a potential complication that occurs in 5% to 10% of patients and carries a poor prognosis. Lung transplantation has been proposed as a(More)
BACKGROUND Dyskeratosis congenita (DC) is a progressive, multi-system, inherited disorder of telomere biology with high risks of morbidity and mortality from bone marrow failure, hematologic malignancy, solid tumors and pulmonary fibrosis. Hematopoietic stem cell transplantation (HSCT) can cure the bone marrow failure, but it does not eliminate the risks of(More)
EBV-transformation induces B lymphocytes to secrete high levels of human IL-10 [9]. Additionally, EBV contains a gene, BCRF1, that encodes for a protein that shares activity with human IL-10 in vitro. Thus, infection by EBV seems to promote a Th2 environment in the infected host. One may even hypothesize that EBV-derived IL-10 initiates a cascade of events(More)
More than 1300 lung or heart-lung transplants have been performed in children to date, resulting in many years of improved quality of life. Increasing experience has demonstrated that this therapy is unique and differs from adult lung transplantation in terms of indications, complications, pharmacokinetics, and monitoring. Unlike adult lung transplant(More)
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(More)
OBJECTIVE Effectiveness of preoperative and postoperative extracorporeal membrane oxygenation support in pediatric lung transplantation was studied. METHODS Institutional database of pediatric lung transplants from 1990 to 2008 was reviewed. RESULTS Three hundred forty-four patients underwent lung transplants in the study period. Thirty-three of 344(More)
Flexible bronchoscopy with transbronchial biopsy (TBB) is routinely performed in adult and pediatric lung transplant recipients. The clinical signs and symptoms of acute cellular rejection (ACR) are often identical to those of infection. TBB is a fairly sensitive and specific tool in which to diagnose ACR and can be performed safely in children of all ages.(More)
In a retrospective, single-center cohort study, outcomes of infants and toddlers undergoing lung transplant at St. Louis Children's Hospital between 1990 and 2004 were compared to older children. Patients with cystic fibrosis (exclusively older children) and those who underwent heart-lung, liver-lung, single lung or a second transplantation were excluded(More)