caa a22 4500 445825561 CHVBK 20180317145258.0 cr unu---uuuuu 170323e20111201xx s 000 0 eng 10.1007/s00246-011-0058-x doi (NATIONALLICENCE)springer-10.1007/s00246-011-0058-x Prospective, Longitudinal Study of Plastic Bronchitis Cast Pathology and Responsiveness to Tissue Plasminogen Activator [Elektronische Daten] [Lauren Heath, Shelley Ling, Jennifer Racz, Gerta Mane, Lindsay Schmidt, Jeffrey Myers, Wan Tsai, Regine Caruthers, Jennifer Hirsch, Kathleen Stringer] Plastic bronchitis (PB) is a rare disease that often occurs in patients with congenital heart disease (CHD) who have undergone staged single-ventricle palliation. It is characterized by the formation of rubbery "casts” in the airways. PB treatment frequently includes inhaled tissue plasminogen activator (tPA). However, the efficacy of tPA to reduce cast burden is unknown. This is further complicated by our lack of knowledge of cast composition. We obtained spontaneously expectorated PB casts from children (n=4) with CHD and one adult patient with idiopathic PB. Pathological assessment was made from paraffin-preserved samples. Casts were treated with phosphate-buffered saline (PBS) or tPA. Cast response to tPA was assessed by changes in cast weight and the production of fibrin d-dimer. Independent of dose, tPA reduced cast weight compared with PBS-treatment (P=0.001) and increased d-dimer levels. Histological staining showed that PB casts from all patients were composed of fibrin and contained notable numbers of lymphocytes. Cast composition did not change over time. Collectively, these data support that in our PB patients, casts are composed of fibrin and are responsive to tPA treatment. This makes inhaled tPA a potentially viable option for symptomatic relief of PB while we work to unravel the complexity of PB pathogenesis. Springer Science+Business Media, LLC, 2011 Congential heart disease nationallicence Fontan physiology nationallicence Pulmonary drug delivery nationallicence Heath Lauren Department of Clinical, Social and Administrative Sciences, College of Pharmacy, University of Michigan, 48109, Ann Arbor, MI, USA aut Ling Shelley Department of Clinical, Social and Administrative Sciences, College of Pharmacy, University of Michigan, 48109, Ann Arbor, MI, USA aut Racz Jennifer Department of Clinical, Social and Administrative Sciences, College of Pharmacy, University of Michigan, 48109, Ann Arbor, MI, USA aut Mane Gerta Department of Clinical, Social and Administrative Sciences, College of Pharmacy, University of Michigan, 48109, Ann Arbor, MI, USA aut Schmidt Lindsay Division of Anatomic Pathology, Department of Pathology, University of Michigan, 48109, Ann Arbor, MI, USA aut Myers Jeffrey Division of Anatomic Pathology, Department of Pathology, University of Michigan, 48109, Ann Arbor, MI, USA aut Tsai Wan Division of Pediatric Pulmonology, Department of Pediatrics and Communicable Diseases, University of Michigan, 48109, Ann Arbor, MI, USA aut Caruthers Regine Department of Pharmacy, University of Michigan Hospitals and Health Centers, University of Michigan, 48109, Ann Arbor, MI, USA aut Hirsch Jennifer Department of Surgery, Division of Pediatric Cardiac Surgery, University of Michigan Health System, 48109, Ann Arbor, MI, USA aut Stringer Kathleen Department of Clinical, Social and Administrative Sciences, College of Pharmacy, University of Michigan, 48109, Ann Arbor, MI, USA aut Pediatric Cardiology Springer-Verlag 32/8(2011-12-01), 1182-1189 0172-0643 32:8<1182 2011 32 246 https://doi.org/10.1007/s00246-011-0058-x text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00246-011-0058-x text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Heath Lauren Department of Clinical, Social and Administrative Sciences, College of Pharmacy, University of Michigan, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Ling Shelley Department of Clinical, Social and Administrative Sciences, College of Pharmacy, University of Michigan, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Racz Jennifer Department of Clinical, Social and Administrative Sciences, College of Pharmacy, University of Michigan, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Mane Gerta Department of Clinical, Social and Administrative Sciences, College of Pharmacy, University of Michigan, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Schmidt Lindsay Division of Anatomic Pathology, Department of Pathology, University of Michigan, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Myers Jeffrey Division of Anatomic Pathology, Department of Pathology, University of Michigan, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Tsai Wan Division of Pediatric Pulmonology, Department of Pediatrics and Communicable Diseases, University of Michigan, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Caruthers Regine Department of Pharmacy, University of Michigan Hospitals and Health Centers, University of Michigan, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Hirsch Jennifer Department of Surgery, Division of Pediatric Cardiac Surgery, University of Michigan Health System, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Stringer Kathleen Department of Clinical, Social and Administrative Sciences, College of Pharmacy, University of Michigan, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 773 0- Pediatric Cardiology Springer-Verlag 32/8(2011-12-01), 1182-1189 0172-0643 32:8<1182 2011 32 246 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer