title: Endothelial, inducible and neuronal nitric oxide synthase in congenital pulmonary lymphangiectasis.
abstract: Abnormal growth and development of lymphatic pulmonary structures leads to severe hypoxia in congenital pulmonary lymphangiectasis (CPL). This case study aims to determine the cellular source and topographical distribution of the nitric oxide synthases in CPL. It studies the post mortem tissue of a term newborn with the clinical course and histological findings of CPL and three controls without pulmonary pathology. It was found that endothelial cells of pulmonary arteries and lymphatic structures stained significantly more for endothelial nitric oxide synthase protein in the CPL patient compared to the controls. The authors conclude that synthesis of endothelial nitric oxide synthase is upregulated in vascular and lymphatic endothelial cells in congenital pulmonary lymphangiectasis.
authors.affiliation: Neonatology and Paediatric Intensive Care Medicine, Dept of General Paediatrics, Heinrich-Heine-University, Moorenstr. 5, D-40225 Düsseldorf, Germany. thomas.hoehn@uni-duesseldorf.de
authors.forename: T
authors.initials: T
authors.lastname: Hoehn
authors.forename: M
authors.initials: M
authors.lastname: William
authors.forename: A R
authors.initials: AR
authors.lastname: McPhaden
authors.forename: H
authors.initials: H
authors.lastname: Stannigel
authors.forename: E
authors.initials: E
authors.lastname: Mayatepek
authors.forename: R M
authors.initials: RM
authors.lastname: Wadsworth
mesh_terms: D000328:Adult; D004729:Endothelium, Lymphatic / Q000473:pathology; D004730:Endothelium, Vascular / Q000473:pathology; D005260:Female; D005311:Fetal Hypoxia / Q000473:pathology*; D006801:Humans; D007124:Immunoenzyme Techniques; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities / Q000473:pathology; D008171:Lung Diseases / Q000151:congenital* / Q000473:pathology; D008200:Lymphangiectasis / Q000151:congenital* / Q000473:pathology; D052248:Nitric Oxide Synthase Type I / Q000032:analysis*; D052250:Nitric Oxide Synthase Type III / Q000032:analysis*; D011247:Pregnancy; D012016:Reference Values; D014443:Tyrosine / Q000031:analogs & derivatives / Q000032:analysis
chemical_list: C002744:3-nitrotyrosine; D014443:Tyrosine; C496320:NOS1 protein, human; C496314:NOS3 protein, human; D052248:Nitric Oxide Synthase Type I; D052250:Nitric Oxide Synthase Type III