{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T15:21:47Z","timestamp":1770823307948,"version":"3.50.1"},"reference-count":186,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2004,8,1]],"date-time":"2004-08-01T00:00:00Z","timestamp":1091318400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Seminars in Neonatology"],"published-print":{"date-parts":[[2004,8]]},"DOI":"10.1016\/j.siny.2003.12.001","type":"journal-article","created":{"date-parts":[[2004,1,27]],"date-time":"2004-01-27T10:23:12Z","timestamp":1075198992000},"page":"311-329","source":"Crossref","is-referenced-by-count":36,"title":["Pulmonary pathology"],"prefix":"10.1016","volume":"9","author":[{"given":"Daphne E.","family":"deMello","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.siny.2003.12.001_BIB1","doi-asserted-by":"crossref","first-page":"9029","DOI":"10.1016\/S0021-9258(19)84483-6","article-title":"Isolation and characterization of cDNA clones for the 35kDa pulmonary surfactant associated protein","volume":"261","author":"Floros","year":"1986","journal-title":"J Biol Chem"},{"key":"10.1016\/j.siny.2003.12.001_BIB2","doi-asserted-by":"crossref","first-page":"L162","DOI":"10.1152\/ajplung.1995.268.2.L162","article-title":"Human SP-A: then and now","volume":"268","author":"Floros","year":"1995","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.siny.2003.12.001_BIB3","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1165\/ajrcmb.19.2.140","article-title":"Collectins and pulmonary host defense","volume":"19","author":"Crouch","year":"1998","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB4","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1034\/j.1600-065X.2000.917311.x","article-title":"Collectins and pulmonary innate immunity","volume":"173","author":"Crouch","year":"2000","journal-title":"Immunol Rev"},{"key":"10.1016\/j.siny.2003.12.001_BIB5","first-page":"131","article-title":"Absence of tubular myelin in lungs of infants dying with hyaline membrane disease","volume":"127","author":"deMello","year":"1987","journal-title":"Am J Pathol"},{"key":"10.1016\/j.siny.2003.12.001_BIB6","first-page":"1631","article-title":"Immunogold localization of SP-A in lungs of infants dying from respiratory distress syndrome","volume":"142","author":"deMello","year":"1993","journal-title":"Am J Pathol"},{"key":"10.1016\/j.siny.2003.12.001_BIB7","first-page":"1285","article-title":"Expression of the 35kDa and low molecular weight surfactant-associated proteins in the lungs of infants dying with respiratory distress syndrome","volume":"134","author":"deMello","year":"1989","journal-title":"Am J Pathol"},{"key":"10.1016\/j.siny.2003.12.001_BIB8","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1056\/NEJM196702162760701","article-title":"Pulmonary disease following therapy of hyaline membrane disease","volume":"276","author":"Northway","year":"1967","journal-title":"N Engl J Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB9","doi-asserted-by":"crossref","first-page":"1376","DOI":"10.1164\/ajrccm\/144.6.1376","article-title":"Surfactant protein A, phosphatidylcholine, and surfactant inhibitors in epithelial lining fluid. Correlation with surface activity, severity of respiratory distress syndrome, and outcome in small premature infants","volume":"144","author":"Hallman","year":"1991","journal-title":"Am Rev Respir Dis"},{"key":"10.1016\/j.siny.2003.12.001_BIB10","doi-asserted-by":"crossref","first-page":"1989","DOI":"10.1164\/ajrccm.151.6.7767549","article-title":"Surfactant protein-A deficiency in a primate model of bronchopulmonary dysplasia","volume":"151","author":"King","year":"1995","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB11","doi-asserted-by":"crossref","first-page":"9594","DOI":"10.1073\/pnas.93.18.9594","article-title":"Altered surfactant function and structure in SP-A gene targeted mice","volume":"93","author":"Korfhagen","year":"1996","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.siny.2003.12.001_BIB12","doi-asserted-by":"crossref","first-page":"4336","DOI":"10.4049\/jimmunol.158.9.4336","article-title":"Surfactant protein A-deficient mice are susceptible to group B streptococcal infection","volume":"158","author":"LeVine","year":"1997","journal-title":"J Immunol"},{"key":"10.1016\/j.siny.2003.12.001_BIB13","doi-asserted-by":"crossref","first-page":"3934","DOI":"10.4049\/jimmunol.165.7.3934","article-title":"Distinct effects of surfactant protein A or D deficiency during bacterial infection on the lung","volume":"165","author":"LeVine","year":"2000","journal-title":"J Immunol"},{"key":"10.1016\/j.siny.2003.12.001_BIB14","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1165\/ajrcmb.26.3.4584","article-title":"Surfactant protein-A-deficient mice display an exaggerated early inflammatory response to a beta-resistant strain of influenza A virus","volume":"26","author":"Li","year":"2002","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB15","first-page":"462","article-title":"Pneumocystis carinii alters surfactant protein A concentrations in bronchoalveolar lavage fluid","volume":"125","author":"Sternberg","year":"1995","journal-title":"J Lab Clin Med"},{"issue":"Suppl 1","key":"10.1016\/j.siny.2003.12.001_BIB16","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1159\/000244202","article-title":"Potential role of surfactant proteins A and D in innate lung defense against pathogens","volume":"67","author":"Van Golde","year":"1995","journal-title":"Biol Neonate"},{"key":"10.1016\/j.siny.2003.12.001_BIB17","doi-asserted-by":"crossref","first-page":"931","DOI":"10.1152\/physrev.1997.77.4.931","article-title":"Immunomodulatory functions of surfactant","volume":"77","author":"Wright","year":"1997","journal-title":"Physiol Rev"},{"key":"10.1016\/j.siny.2003.12.001_BIB18","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1016\/S0952-7915(99)80006-5","article-title":"Lung surfactant proteins involved in innate immunity","volume":"11","author":"Eggleton","year":"1999","journal-title":"Curr Opin Immunol"},{"key":"10.1016\/j.siny.2003.12.001_BIB19","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1034\/j.1600-065X.2000.917306.x","article-title":"Innate immunity and pulmonary host defense","volume":"173","author":"Zhang","year":"2000","journal-title":"Immunol Rev"},{"key":"10.1016\/j.siny.2003.12.001_BIB20","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1034\/j.1600-065X.2000.917308.x","article-title":"The roles of surfactant protein A and D in innate immunity","volume":"173","author":"Lawson","year":"2000","journal-title":"Immunol Rev"},{"key":"10.1016\/j.siny.2003.12.001_BIB21","volume":"vol. 1","year":"1995"},{"key":"10.1016\/j.siny.2003.12.001_BIB22","doi-asserted-by":"crossref","first-page":"608","DOI":"10.1165\/ajrcmb.14.6.8652189","article-title":"Surfactant protein A accumulation in the alveoli of patients with pulmonary alveolar proteinosis: oligomeric structure and interaction with lipids","volume":"14","author":"Hattori","year":"1996","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB23","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1042\/bj2730249","article-title":"Function and regulation of expression of pulmonary surfactant proteins","volume":"273","author":"Weaver","year":"1991","journal-title":"Biochem J"},{"key":"10.1016\/j.siny.2003.12.001_BIB24","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1056\/NEJM199302113280606","article-title":"Deficiency of pulmonary surfactant protein B in congenital pulmonary alveolar proteinosis","volume":"328","author":"Nogee","year":"1993","journal-title":"N Engl J Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB25","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/S0022-3476(94)70119-9","article-title":"Molecular and phenotypic variability in the congenital alveolar proteinosis syndrome associated with inherited surfactant protein B (SP-B) deficiency","volume":"125","author":"deMello","year":"1994","journal-title":"J Pediatr"},{"key":"10.1016\/j.siny.2003.12.001_BIB26","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1164\/ajrccm.159.2.9806033","article-title":"Normal lung function in subjects heterozygous for surfactant protein B deficiency","volume":"159","author":"Yusen","year":"1999","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB27","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1542\/peds.96.6.1046","article-title":"Partial deficiency of surfactant protein B in an infant with chronic lung disease","volume":"96","author":"Ballard","year":"1995","journal-title":"Pediatrics"},{"key":"10.1016\/j.siny.2003.12.001_BIB28","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1080\/107710497174877","article-title":"Congenital alveolar proteinosis in the Netherlands: a report of five cases with immunohistochemical and genetic studies on surfactant apoproteins","volume":"17","author":"de La Fuente","year":"1997","journal-title":"Pediatr Pathol Lab Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB29","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1007\/s004310051132","article-title":"Congenital alveolar proteinosis caused by a novel mutation of the surfactant protein B gene and misalignment of lung vessels in a kindred with multiple consanguineous marriages","volume":"158","author":"Wallot","year":"1999","journal-title":"Eur J Pediatr"},{"key":"10.1016\/j.siny.2003.12.001_BIB30","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1034\/j.1399-0004.2000.570506.x","article-title":"Aberrant SP-B mRNA in lung tissue of patients with congenital alveolar proteinosis (CAP)","volume":"57","author":"Lin","year":"2000","journal-title":"Clin Genet"},{"key":"10.1016\/j.siny.2003.12.001_BIB31","doi-asserted-by":"crossref","first-page":"1860","DOI":"10.1172\/JCI117173","article-title":"A mutation in the surfactant protein B gene responsible for fatal neonatal disease in multiple kindreds","volume":"93","author":"Nogee","year":"1994","journal-title":"J Clin Invest"},{"key":"10.1016\/j.siny.2003.12.001_BIB32","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1080\/152279501750399384","article-title":"Pulmonary alveolar proteinosis: a review","volume":"20","author":"deMello","year":"2001","journal-title":"Pediatr Pathol Mol Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB33","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1006\/mgme.1998.2702","article-title":"An SP-B gene mutation responsible for SP-B deficiency in fatal congenital alveolar proteinosis. Evidence for a mutation hotspot in exon 4","volume":"64","author":"Lin","year":"1998","journal-title":"Mol Genet Metab"},{"key":"10.1016\/j.siny.2003.12.001_BIB34","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1002\/(SICI)1098-1004(199912)14:6<502::AID-HUMU9>3.0.CO;2-C","article-title":"Compound SFTPB 1549C\u2192GAA (121ins2) and 457delC heterozygosity in severe congenital lung disease and surfactant protein B (SP-B) deficiency","volume":"14","author":"Tredano","year":"1999","journal-title":"Hum Mutat"},{"key":"10.1016\/j.siny.2003.12.001_BIB35","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1164\/ajrccm.161.3.9903153","article-title":"Allelic heterogeneity in hereditary surfactant protein B (SP-B) deficiency","volume":"161","author":"Nogee","year":"2000","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB36","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1016\/S0022-3476(98)70439-1","article-title":"Transient surfactant protein B deficiency in a term infant with severe respiratory failure","volume":"132","author":"Klein","year":"1998","journal-title":"Pediatrics"},{"key":"10.1016\/j.siny.2003.12.001_BIB37","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1542\/peds.105.3.538","article-title":"Brief report: population-based estimates of surfactant protein B deficiency","volume":"105","author":"Cole","year":"2000","journal-title":"Pediatrics"},{"key":"10.1016\/j.siny.2003.12.001_BIB38","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1042\/0264-6021:3430145","article-title":"An alternatively spliced surfactant protein B mRNA in normal human lung: disease implications","volume":"343","author":"Lin","year":"1999","journal-title":"Biochem J"},{"key":"10.1016\/j.siny.2003.12.001_BIB39","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/S0092-8674(00)80725-4","article-title":"Gene slicing: repeats that count","volume":"97","author":"Selker","year":"1999","journal-title":"Cell"},{"key":"10.1016\/j.siny.2003.12.001_BIB40","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1126\/science.286.5439.481","article-title":"Epigenetics: regulation through repression","volume":"286","author":"Wolffe","year":"1999","journal-title":"Science"},{"key":"10.1016\/j.siny.2003.12.001_BIB41","doi-asserted-by":"crossref","first-page":"7794","DOI":"10.1073\/pnas.92.17.7794","article-title":"Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice","volume":"92","author":"Clark","year":"1995","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.siny.2003.12.001_BIB42","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1165\/ajrcmb.21.4.3436","article-title":"Surfactant protein-B-deficient mice are susceptible to hyperoxic lung injury","volume":"21","author":"Tokieda","year":"1999","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB43","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1203\/00006450-199912000-00014","article-title":"Surfactant protein B corrects oxygen-induced pulmonary dysfunction in heterozygous surfactant protein B-deficient mice","volume":"46","author":"Tokieda","year":"1999","journal-title":"Pediatr Res"},{"key":"10.1016\/j.siny.2003.12.001_BIB44","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1165\/ajrcmb.16.1.8998078","article-title":"Decreased lung compliance and air trapping in heterozygous SP-B-deficient mice","volume":"16","author":"Clark","year":"1997","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB45","first-page":"L647","article-title":"Aberrant processing of surfactant protein C in hereditary SP-B deficiency","volume":"268","author":"Vorbroker","year":"1995","journal-title":"Am J Physiol"},{"key":"10.1016\/j.siny.2003.12.001_BIB46","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1165\/ajrcmb.11.2.8049084","article-title":"Ultrastructure of lung in surfactant protein B (SP-B) deficiency","volume":"11","author":"deMello","year":"1994","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB47","doi-asserted-by":"crossref","first-page":"3371","DOI":"10.1074\/jbc.275.5.3371","article-title":"Ablation of a critical surfactant protein B intramolecular disulfide bond in transgenic mice","volume":"275","author":"Beck","year":"2000","journal-title":"J Biol Chem"},{"key":"10.1016\/j.siny.2003.12.001_BIB48","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1016\/S0022-3476(05)83276-7","article-title":"Surfactant protein B deficiency: antenatal diagnosis and prospective treatment with surfactant replacement","volume":"125","author":"Hamvas","year":"1994","journal-title":"J Pediatr"},{"key":"10.1016\/j.siny.2003.12.001_BIB49","first-page":"L151","article-title":"Surfactant protein C: a review of its unique properties and metabolism","volume":"263","author":"Beers","year":"1992","journal-title":"Am J Physiol"},{"key":"10.1016\/j.siny.2003.12.001_BIB50","doi-asserted-by":"crossref","first-page":"10326","DOI":"10.1016\/S0021-9258(19)81519-3","article-title":"Two SP-C genes encoding human pulmonary surfactant proteolipid","volume":"263","author":"Glasser","year":"1988","journal-title":"J Biol Chem"},{"key":"10.1016\/j.siny.2003.12.001_BIB51","doi-asserted-by":"crossref","first-page":"L727","DOI":"10.1152\/ajplung.1995.269.6.L727","article-title":"Posttranslational processing of surfactant protein C in rat type II cells","volume":"269","author":"Vorbroker","year":"1995","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.siny.2003.12.001_BIB52","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1056\/NEJM200102223440805","article-title":"A mutation in the surfactant protein C gene associated with familial interstitial lung disease","volume":"344","author":"Nogee","year":"2002","journal-title":"N Engl J Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB53","doi-asserted-by":"crossref","first-page":"15287","DOI":"10.1074\/jbc.273.24.15287","article-title":"Synthetic processing of surfactant protein C by alveolar epithelial cells: the COOH terminus of proSP-C is required for post-translational targeting and proteolysis","volume":"273","author":"Beers","year":"1998","journal-title":"J Biol Chem"},{"key":"10.1016\/j.siny.2003.12.001_BIB54","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1165\/ajrcmb\/6.6.601","article-title":"The C-terminal domain of the pulmonary surfactant protein C precursor contains signals for intracellular targeting","volume":"6","author":"Keller","year":"1992","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB55","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1242\/jcs.00267","article-title":"Deletion of exon 4 from human surfactant protein C results in aggresome formation and generation of a dominant negative","volume":"116","author":"Wang","year":"2003","journal-title":"J Cell Sci"},{"key":"10.1016\/j.siny.2003.12.001_BIB56","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1067\/mpd.2001.114545","article-title":"Surfactant protein deficiency in familial interstitial lung disease","volume":"139","author":"Amin","year":"2001","journal-title":"J Pediatr"},{"key":"10.1016\/j.siny.2003.12.001_BIB57","doi-asserted-by":"crossref","first-page":"19402","DOI":"10.1074\/jbc.270.33.19402","article-title":"Tumor necrosis factor-alpha inhibits surfactant protein C gene transcription","volume":"270","author":"Bachurski","year":"1995","journal-title":"J Biol Chem"},{"key":"10.1016\/j.siny.2003.12.001_BIB58","first-page":"714","article-title":"Tumor necrosis factor-alpha decreases surfactant protein B mRNA in murine lung","volume":"270","author":"Pryhuber","year":"1996","journal-title":"Am J Physiol"},{"key":"10.1016\/j.siny.2003.12.001_BIB59","doi-asserted-by":"crossref","first-page":"1954","DOI":"10.1172\/JCI114929","article-title":"Tumor necrosis factor-alpha inhibits expression of pulmonary surfactant protein","volume":"86","author":"Wispe","year":"1990","journal-title":"J Clin Invest"},{"key":"10.1016\/j.siny.2003.12.001_BIB60","doi-asserted-by":"crossref","first-page":"1322","DOI":"10.1164\/rccm.200112-123OC","article-title":"Heterozygosity for a surfactant protein C gene mutation associated with usual interstitial pneumonitis and cellular nonspecific interstitial pneumonitis in one kindred","volume":"165","author":"Thomas","year":"2002","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB61","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1242\/jcs.114.2.293","article-title":"Biosynthesis of surfactant protein C: characterization of aggresome formation by EGFP chimeras containing propeptide mutants lacking conserved cysteine residues","volume":"114","author":"Kabore","year":"2001","journal-title":"J Cell Sci"},{"key":"10.1016\/j.siny.2003.12.001_BIB62","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1165\/ajrcmb.26.1.4686","article-title":"Overexpression of SP-C mature SP-C peptide causes neonatal lethality in transgenic mice","volume":"26","author":"Conkright","year":"2002","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB63","doi-asserted-by":"crossref","first-page":"20S","DOI":"10.1378\/chest.121.3_suppl.20S","article-title":"Mutation in the surfactant protein C gene associated with interstitial lung disease","volume":"121","author":"Nogee","year":"2002","journal-title":"Chest"},{"key":"10.1016\/j.siny.2003.12.001_BIB64","doi-asserted-by":"crossref","first-page":"1201","DOI":"10.1164\/rccm.2203017","article-title":"Genetic basis of familial interstitial lung disease: misfolding or function of surfactant protein C?","volume":"165","author":"Whitsett","year":"2002","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB65","doi-asserted-by":"crossref","first-page":"6366","DOI":"10.1073\/pnas.101500298","article-title":"Altered stability of pulmonary surfactant in SP-C deficient mice","volume":"98","author":"Glasser","year":"2001","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.siny.2003.12.001_BIB66","doi-asserted-by":"crossref","first-page":"14291","DOI":"10.1074\/jbc.M210909200","article-title":"Pneumonitis and emphysema in sp-C gene targeted mice","volume":"278","author":"Glasser","year":"2003","journal-title":"J Biol Chem"},{"key":"10.1016\/j.siny.2003.12.001_BIB67","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1042\/0264-6021:3510779","article-title":"Very low surfactant protein C contents in newborn Belgian white and blue calves with respiratory distress syndrome","volume":"351","author":"Danlois","year":"2000","journal-title":"Biochem J"},{"key":"10.1016\/j.siny.2003.12.001_BIB68","doi-asserted-by":"crossref","first-page":"1557","DOI":"10.1164\/rccm.2105139","article-title":"Pulmonary interstitial glycogenosis: a new variant of neonatal interstitial lung disease","volume":"165","author":"Canakis","year":"2002","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB69","doi-asserted-by":"crossref","first-page":"1983","DOI":"10.1183\/09031936.97.10091983","article-title":"Pulmonary surfactant in cystic fibrosis","volume":"10","author":"Griese","year":"1997","journal-title":"Eur Respir J"},{"key":"10.1016\/j.siny.2003.12.001_BIB70","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1165\/ajrcmb.20.1.3253","article-title":"Deficient hydrophilic lung surfactant proteins A and D with normal surfactant phospholipid molecular species in cystic fibrosis","volume":"20","author":"Postle","year":"1999","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB71","doi-asserted-by":"crossref","first-page":"11869","DOI":"10.1073\/pnas.95.20.11869","article-title":"Altered surfactant homeostasis and alveolar type II cell morphology in mice lacking surfactant protein D","volume":"95","author":"Botas","year":"1998","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.siny.2003.12.001_BIB72","doi-asserted-by":"crossref","first-page":"28438","DOI":"10.1074\/jbc.273.43.28438","article-title":"Surfactant protein-D regulates surfactant phospholipid homeostasis in vivo","volume":"273","author":"Korfhagen","year":"1998","journal-title":"J Biol Chem"},{"key":"10.1016\/j.siny.2003.12.001_BIB73","doi-asserted-by":"crossref","first-page":"5972","DOI":"10.1073\/pnas.100448997","article-title":"Increased metalloproteinase activity, oxidant production, and emphysema in surfactant protein D gene-inactivated mice","volume":"97","author":"Wert","year":"2000","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.siny.2003.12.001_BIB74","doi-asserted-by":"crossref","first-page":"L1002","DOI":"10.1152\/ajplung.00118.2002","article-title":"Sequential targeted deficiency of SP-A and -D leads to progressive alveolar lipoproteinosis and emphysema","volume":"283","author":"Hawgood","year":"2002","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.siny.2003.12.001_BIB75","doi-asserted-by":"crossref","first-page":"L365","DOI":"10.1152\/ajplung.2000.278.2.L365","article-title":"Pulmonary-specific expression of SP-D corrects pulmonary lipid accumulation in SP-D gene-targeted mice","volume":"278","author":"Fisher","year":"2000","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.siny.2003.12.001_BIB76","doi-asserted-by":"crossref","first-page":"1282","DOI":"10.1126\/science.2999978","article-title":"The human gene encoding GM-CSF is at 5q21\u2013q32, the chromosome region detected in the 5q\u2212 anomaly","volume":"230","author":"Huebner","year":"1985","journal-title":"Science"},{"key":"10.1016\/j.siny.2003.12.001_BIB77","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1182\/blood.V82.1.22.bloodjournal82122","article-title":"A cytokine receptor gene cluster in the X\u2013Y pseudoautosomal region?","volume":"82","author":"Kremer","year":"1993","journal-title":"Blood"},{"key":"10.1016\/j.siny.2003.12.001_BIB78","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1016\/S1357-2725(99)00077-1","article-title":"The beta common chain (\u03b2c) of the granulocyte macrophage-colony stimulating factor, interleukin-3 and interleukin-5 receptors","volume":"31","author":"Scott","year":"1999","journal-title":"Int J Biochem Cell Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB79","doi-asserted-by":"crossref","first-page":"2211","DOI":"10.1172\/JCI119758","article-title":"Human pulmonary alveolar proteinosis associated with a defect in GM-CSF\/IL-3\/IL5 receptor common beta chain expression","volume":"100","author":"Dirksen","year":"1997","journal-title":"J Clin Invest"},{"key":"10.1016\/j.siny.2003.12.001_BIB80","first-page":"1097","article-title":"Defective expression of granulocyte-macrophage colony-stimulating factor\/interleukin-3\/interleukin-5 receptor common beta chain in children with acute myeloid leukemia associated with respiratory failure","volume":"92","author":"Dirksen","year":"1998","journal-title":"Blood"},{"key":"10.1016\/j.siny.2003.12.001_BIB81","doi-asserted-by":"crossref","first-page":"1924","DOI":"10.1056\/NEJM199612193352513","article-title":"Efficacy of granulocyte-macrophage colony-stimulating factor in acquired alveolar proteinosis","volume":"335","author":"Seymour","year":"1996","journal-title":"N Engl J Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB82","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1126\/science.8171324","article-title":"Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis","volume":"264","author":"Dranoff","year":"1994","journal-title":"Science"},{"key":"10.1016\/j.siny.2003.12.001_BIB83","doi-asserted-by":"crossref","first-page":"5592","DOI":"10.1073\/pnas.91.12.5592","article-title":"Granulocute\/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology","volume":"91","author":"Stanley","year":"1994","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.siny.2003.12.001_BIB84","doi-asserted-by":"crossref","first-page":"1131","DOI":"10.1182\/blood.V77.6.1131.1131","article-title":"Molecular physiology of granulocyte-macrophage colony-stimulating factor","volume":"77","author":"Gasson","year":"1991","journal-title":"Blood"},{"key":"10.1016\/j.siny.2003.12.001_BIB85","doi-asserted-by":"crossref","first-page":"L650","DOI":"10.1152\/ajplung.1996.270.4.L650","article-title":"Surfactant metabolism in transgenic mice after granulocyte macrophage-colony stimulating factor ablation","volume":"270","author":"Ikegami","year":"1996","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.siny.2003.12.001_BIB86","doi-asserted-by":"crossref","first-page":"9565","DOI":"10.1073\/pnas.92.21.9565","article-title":"Hematopoietic and lung abnormalities in mice with a null mutation of the common beta subunit of the receptors for granulocyte-macrophage colony-stimulating factor and interleukins 3 and 5","volume":"92","author":"Robb","year":"1995","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.siny.2003.12.001_BIB87","doi-asserted-by":"crossref","first-page":"2657","DOI":"10.1084\/jem.183.6.2657","article-title":"The pulmonary alveolar proteinosis in granulocyte macrophage colony-stimulating factor\/interleukins 3\/5 beta c receptor-deficient mice is reversed by bone marrow transplantation","volume":"183","author":"Nishinakamura","year":"1996","journal-title":"J Exp Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB88","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1038\/sj.bmt.1700958","article-title":"Persistence of pulmonary pathology and abnormal lung function in IL-3\/GM-CSF\/IL-5 \u03b2c receptor-deficient mice despite correction of alveolar proteinosis after bone marrow transplantation","volume":"20","author":"Cook","year":"1997","journal-title":"Bone Marrow Transplant"},{"key":"10.1016\/j.siny.2003.12.001_BIB89","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/1074-7613(95)90046-2","article-title":"Mice deficient for the IL-3\/GM-CSF\/IL-5 beta c receptor exhibit lung pathology and impaired immune response, while beta IL-3 receptor-deficient mice are normal","volume":"2","author":"Nishinakamura","year":"1995","journal-title":"Immunity"},{"key":"10.1016\/j.siny.2003.12.001_BIB90","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1172\/JCI118461","article-title":"Pulmonary epithelial cell expression of GM-CSF corrects the alveolar proteinosis in GM-CSF-deficient mice","volume":"97","author":"Huffman","year":"1996","journal-title":"J Clin Invest"},{"key":"10.1016\/j.siny.2003.12.001_BIB91","first-page":"L556","article-title":"Aerosolized GM-CSF ameliorates pulmonary alveolar proteinosis in GM-CSF-deficient mice","volume":"276","author":"Reed","year":"1999","journal-title":"Am J Physiol"},{"key":"10.1016\/j.siny.2003.12.001_BIB92","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1165\/rcmb.2003-0148OC","article-title":"Peroxisome proliferation-activated receptor-{gamma} is deficient in alveolar macrophages from patients with alveolar proteinosis","volume":"29","author":"Bonfield","year":"2003","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB93","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1165\/rcmb.2002-0023OC","article-title":"Autoantibodies against granulocyte macrophage colony-stimulating factor are diagnostic for pulmonary alveolar proteinosis","volume":"27","author":"Bonfield","year":"2002","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB94","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1084\/jem.190.6.875","article-title":"Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte\/macrophage colony-stimulating factor","volume":"190","author":"Kitamura","year":"1999","journal-title":"J Exp Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB95","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1016\/S0014-5793(98)01668-8","article-title":"Lungs of patients with idiopathic pulmonary alveolar proteinosis express a factor which neutralizes granulocyte-macrophage colony stimulating factor","volume":"442","author":"Tanaka","year":"1999","journal-title":"FEBS Lett"},{"key":"10.1016\/j.siny.2003.12.001_BIB96","doi-asserted-by":"crossref","first-page":"L1164","DOI":"10.1152\/ajplung.2000.278.6.L1164","article-title":"Distinct changes in pulmonary surfactant homeostasis in common beta-chain and GM-CSF-deficient mice","volume":"278","author":"Reed","year":"2000","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.siny.2003.12.001_BIB97","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1007\/s100249910048","article-title":"Absence of lamellar bodies with accumulation of dense bodies characterizes a novel form of congenital surfactant defect","volume":"3","author":"Tryka","year":"2000","journal-title":"Pediatr Dev Pathol"},{"key":"10.1016\/j.siny.2003.12.001_BIB98","doi-asserted-by":"crossref","first-page":"608","DOI":"10.1164\/ajrccm.161.2.9905062","article-title":"Deficiency of lamellar bodies in alveolar type II cells associated with fatal respiratory disease in a full-term infant","volume":"161","author":"Cutz","year":"2000","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB99","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1002\/aja.1001290307","article-title":"The developing bronchial arteries in a fetus of the 12th week","volume":"129","author":"Boyden","year":"1970","journal-title":"Am J Anat"},{"key":"10.1016\/j.siny.2003.12.001_BIB100","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1111\/j.1651-2227.1967.tb15375.x","article-title":"The normal intrapulmonary arterial pattern of the human late fetal and neonatal lung. A microangiographic and histologic study","volume":"56","author":"Robertson","year":"1967","journal-title":"Acta Paediatr Scand"},{"key":"10.1016\/j.siny.2003.12.001_BIB101","first-page":"35","article-title":"Intra-pulmonary arterial development during fetal life\u2014branching pattern and structure","volume":"113","author":"Hislop","year":"1972","journal-title":"J Anat"},{"key":"10.1016\/j.siny.2003.12.001_BIB102","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1136\/thx.28.2.129","article-title":"Pulmonary arterial development during childhood: branching pattern and structure","volume":"28","author":"Hislop","year":"1973","journal-title":"Thorax"},{"key":"10.1016\/j.siny.2003.12.001_BIB103","first-page":"117","article-title":"Arteries and veins","volume":"vol. 79","author":"deMello","year":"1997"},{"key":"10.1016\/j.siny.2003.12.001_BIB104","first-page":"211","article-title":"Vascular development of lung","volume":"vol. 2","author":"deMello","year":"2002"},{"key":"10.1016\/j.siny.2003.12.001_BIB105","series-title":"The pulmonary circulation and gas exchange","first-page":"77","article-title":"Structural remodeling of the pulmonary vasculature by environmental change and disease","author":"Reid","year":"1994"},{"key":"10.1016\/j.siny.2003.12.001_BIB106","doi-asserted-by":"crossref","first-page":"1097","DOI":"10.1164\/ajrccm\/140.4.1097","article-title":"Embryonic origins and assembly of blood vessels","volume":"140","author":"Noden","year":"1989","journal-title":"Am Rev Respir Dis"},{"key":"10.1016\/j.siny.2003.12.001_BIB107","doi-asserted-by":"crossref","first-page":"568","DOI":"10.1165\/ajrcmb.16.5.9160839","article-title":"Early fetal development of lung vasculature","volume":"16","author":"deMello","year":"1997","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB108","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1007\/s100240010090","article-title":"Embryonic and early fetal development of human lung vasculature and its functional implications","volume":"3","author":"deMello","year":"2000","journal-title":"Pediatr Dev Pathol"},{"key":"10.1016\/j.siny.2003.12.001_BIB109","series-title":"The fetal and neonatal pulmonary circulation","first-page":"37","article-title":"Structural elements of human fetal and neonatal lung vascular development","author":"deMello","year":"1999"},{"key":"10.1016\/j.siny.2003.12.001_BIB110","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1203\/00006450-198908000-00002","article-title":"Structural maturation of the human fetal lung: a morphometric study of the development of air\u2013blood barriers","volume":"26","author":"Dimaio","year":"1989","journal-title":"Pediatr Res"},{"key":"10.1016\/j.siny.2003.12.001_BIB111","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1093\/ajcp\/76.5.722","article-title":"Congenital alveolar capillary dysplasia\u2014an unusual cause of respiratory distress in the newborn","volume":"76","author":"Janney","year":"1981","journal-title":"Am J Clin Pathol"},{"key":"10.1016\/j.siny.2003.12.001_BIB112","doi-asserted-by":"crossref","first-page":"499","DOI":"10.3109\/15513819209024200","article-title":"Persistent pulmonary hypertension of the newborn due to alveolar capillary dysplasia","volume":"12","author":"Cullinane","year":"1992","journal-title":"Pediatr Pathol"},{"key":"10.1016\/j.siny.2003.12.001_BIB113","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1097\/00003246-199304000-00026","article-title":"Late presentation of misalignment of lung vessels with alveolar capillary dysplasia","volume":"21","author":"Abdallah","year":"1993","journal-title":"Crit Care Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB114","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1007\/s00431-002-0927-7","article-title":"Alveolar capillary dysplasia: a cause of persistent pulmonary hypertension of the newborn","volume":"161","author":"Alameh","year":"2002","journal-title":"Eur J Pediatr"},{"key":"10.1016\/j.siny.2003.12.001_BIB115","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1016\/S0378-3782(99)00065-1","article-title":"Alveolar capillary dysplasia. Report of a case of prolonged life without extracorporeal membrane oxygenation (ECMO) and review of the literature","volume":"57","author":"Al-Hathlol","year":"2000","journal-title":"Early Hum Dev"},{"key":"10.1016\/j.siny.2003.12.001_BIB116","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/S0022-3476(94)70267-5","article-title":"Misalignment of pulmonary veins with alveolar capillary dysplasia: affected siblings and variable phenotypic expression","volume":"124","author":"Boggs","year":"1994","journal-title":"J Pediatr"},{"key":"10.1016\/j.siny.2003.12.001_BIB117","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1016\/S0022-3476(89)80800-5","article-title":"Misalignment of lung vessels and alveolar capillary dysplasia: a cause of persistent pulmonary hypertension","volume":"114","author":"Cater","year":"1989","journal-title":"J Pediatr"},{"key":"10.1016\/j.siny.2003.12.001_BIB118","doi-asserted-by":"crossref","first-page":"1159","DOI":"10.1542\/peds.96.6.1159","article-title":"Alveolar capillary dysplasia\u2014a cause of persistent pulmonary hypertension unresponsive to a second course of extracorporeal membrane oxygenation","volume":"96","author":"Chelliah","year":"1995","journal-title":"Pediatrics"},{"key":"10.1016\/j.siny.2003.12.001_BIB119","first-page":"249","article-title":"Pulmonary hypertension of the newborn and urogenital anomalies in two male siblings: a new family with misalignment of pulmonary vessels","volume":"7","author":"Manouvrier-Hanu","year":"1996","journal-title":"Genet Couns"},{"key":"10.1016\/j.siny.2003.12.001_BIB120","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1055\/s-2007-993907","article-title":"Alveolar capillary dysplasia, with and without misalignment of pulmonary veins: an association of congenital anomalies","volume":"15","author":"Garola","year":"1998","journal-title":"Am J Perinatol"},{"key":"10.1016\/j.siny.2003.12.001_BIB121","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1007\/s100249910035","article-title":"Congenital misalignment of pulmonary veins with alveolar capillary dysplasia causing persistent neonatal pulmonary hypertension: report of two affected siblings","volume":"3","author":"Gutierrez","year":"2000","journal-title":"Pediatr Dev Pathol"},{"key":"10.1016\/j.siny.2003.12.001_BIB122","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1080\/107710497174417","article-title":"Congenital alveolar capillary dysplasia: rare cause of persistent pulmonary hypertension","volume":"17","author":"Haraida","year":"1997","journal-title":"Pediatr Pathol Lab Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB123","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1007\/s003830050445","article-title":"Alveolar capillary dysplasia with misalignment of the pulmonary veins: a rare but fatal cause of neonatal respiratory failure","volume":"14","author":"Kane","year":"1998","journal-title":"Pediatr Surg Int"},{"key":"10.1016\/j.siny.2003.12.001_BIB124","doi-asserted-by":"crossref","first-page":"299","DOI":"10.3109\/15513818509078790","article-title":"Congenital alveolar capillary dysplasia: a developmental vascular anomaly causing persistent pulmonary hypertension of the newborn","volume":"3","author":"Khorsand","year":"1985","journal-title":"Pediatr Pathol"},{"key":"10.1016\/j.siny.2003.12.001_BIB125","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/S0022-3468(97)90105-6","article-title":"Nitric oxide inhalation therapy for an infant with persistent pulmonary hypertension caused by misalignment of pulmonary veins with alveolar capillary dysplasia","volume":"32","author":"Kitayama","year":"1997","journal-title":"J Pediatr Surg"},{"key":"10.1016\/j.siny.2003.12.001_BIB126","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1016\/S0002-9149(99)00395-1","article-title":"Prospective diagnosis of alveolar capillary dysplasia in infants with congenital heart disease","volume":"84","author":"Lane","year":"1999","journal-title":"Am J Cardiol"},{"key":"10.1016\/j.siny.2003.12.001_BIB127","doi-asserted-by":"crossref","first-page":"163","DOI":"10.3109\/15513819109064753","article-title":"Misalignment of pulmonary veins and alveolar capillary dysplasia","volume":"11","author":"Langston","year":"1991","journal-title":"Pediatr Pathol"},{"key":"10.1016\/j.siny.2003.12.001_BIB128","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1164\/ajrccm.155.2.9032222","article-title":"Combined therapy with inhaled nitric oxide and intravenous prostacyclin in an infant with alveolar\u2013capillary dysplasia","volume":"155","author":"Parker","year":"1997","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB129","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/S0929-693X(97)83463-X","article-title":"Neonatal hypoxemia due to misaligned pulmonary vessels with alveolar capillary dysplasia","volume":"5","author":"Liet","year":"1998","journal-title":"Arch Pediatr"},{"key":"10.1016\/j.siny.2003.12.001_BIB130","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1111\/j.1651-2227.1995.tb13644.x","article-title":"Congenital misalignment of pulmonary vessels: an unusual syndrome associated with PPHN","volume":"84","author":"Ng","year":"1995","journal-title":"Acta Paediatr"},{"key":"10.1016\/j.siny.2003.12.001_BIB131","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1111\/j.1365-2559.1995.tb00030.x","article-title":"Misalignment of lung vessels and alveolar capillary dysplasia","volume":"27","author":"Oldenburg","year":"1995","journal-title":"Histopathology"},{"key":"10.1016\/j.siny.2003.12.001_BIB132","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1007\/s00428-003-0814-2","article-title":"Misalignment of lung vessels: diagnostic role of conventional histology and immunohistochemistry","volume":"442","author":"Pucci","year":"2003","journal-title":"Virchows Arch"},{"key":"10.1016\/j.siny.2003.12.001_BIB133","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1007\/s100240010125","article-title":"Congenital alveolar capillary dysplasia with misalignment of pulmonary veins associated with hypoplastic left heart syndrome","volume":"4","author":"Rabah","year":"2001","journal-title":"Pediatr Dev Pathol"},{"key":"10.1016\/j.siny.2003.12.001_BIB134","doi-asserted-by":"crossref","first-page":"1163","DOI":"10.1001\/archpedi.1997.02170480093016","article-title":"Pathological case of the month. Congenital alveolar capillary dysplasia and misalignment of lung vessels","volume":"151","author":"Rehan","year":"1997","journal-title":"Arch Pediatr Adolesc Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB135","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1038\/sj.jp.7200335","article-title":"Congenital misalignment of pulmonary vessels and alveolar capillary dysplasia: how to manage a neonatal irreversible lung disease?","volume":"20","author":"Somaschini","year":"2000","journal-title":"J Perinatol"},{"key":"10.1016\/j.siny.2003.12.001_BIB136","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1016\/S0022-3476(97)70203-8","article-title":"Inhaled nitric oxide enhances oxygenation but not survival in infants with alveolar capillary dysplasia","volume":"130","author":"Steinhorn","year":"1997","journal-title":"J Pediatr"},{"key":"10.1016\/j.siny.2003.12.001_BIB137","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1016\/S0022-3476(99)70264-7","article-title":"Alveolar capillary dysplasia: an emerging syndrome","volume":"134","author":"Thibeault","year":"1999","journal-title":"J Pediatr"},{"key":"10.1016\/j.siny.2003.12.001_BIB138","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1046\/j.1440-1754.2002.00014.x","article-title":"Incidence of alveolar capillary dysplasia in severe idiopathic persistent pulmonary hypertension of the newborn","volume":"38","author":"Tibballs","year":"2002","journal-title":"J Paediatr Child Health"},{"key":"10.1016\/j.siny.2003.12.001_BIB139","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1136\/jmg.35.1.58","article-title":"Familial persistent pulmonary hypertension of the newborn resulting from misalignment of the pulmonary vessels (congenital alveolar capillary dysplasia)","volume":"35","author":"Vassal","year":"1998","journal-title":"J Med Genet"},{"key":"10.1016\/j.siny.2003.12.001_BIB140","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1016\/S0046-8177(86)80182-4","article-title":"Misalignment of lung vessels: a syndrome causing persistent neonatal pulmonary hypertension","volume":"17","author":"Wagenvoort","year":"1986","journal-title":"Hum Pathol"},{"key":"10.1016\/j.siny.2003.12.001_BIB141","doi-asserted-by":"crossref","first-page":"2077","DOI":"10.1084\/jem.178.6.2077","article-title":"The related flt4, flt1, and kdr receptor tyrosine kinases show distinct expression patterns in human fetal endothelial cells","volume":"178","author":"Kaipainen","year":"1993","journal-title":"J Exp Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB142","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1210\/jc.79.1.316","article-title":"In the human fetus, vascular endothelial growth factor is expressed in epithelial cells and myocytes, but not vascular endothelium: implications for mode of action","volume":"79","author":"Shifren","year":"1994","journal-title":"J Clin Endocrinol Metab"},{"key":"10.1016\/j.siny.2003.12.001_BIB143","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1002\/aja.1002040303","article-title":"Coordinate expression of vascular endothelial growth factor receptor-1 (flt-1) and its ligand suggests a paracrine regulation of murine vascular development","volume":"204","author":"Breier","year":"1995","journal-title":"Dev Dyn"},{"key":"10.1016\/j.siny.2003.12.001_BIB144","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1096\/fasebj.11.5.9141503","article-title":"Vascular development: cellular and molecular regulation","volume":"11","author":"Beck","year":"1997","journal-title":"FASEB J"},{"key":"10.1016\/j.siny.2003.12.001_BIB145","doi-asserted-by":"crossref","first-page":"7657","DOI":"10.1073\/pnas.92.17.7657","article-title":"Exogenous vascular endothelial growth factor induces malformed and hyperfused vessels during embryonic neovascularization","volume":"92","author":"Drake","year":"1995","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.siny.2003.12.001_BIB146","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1038\/380435a0","article-title":"Abnormal blood vessel development and lethality in embryos lacking a single VEGF allele","volume":"380","author":"Carmeliet","year":"1996","journal-title":"Nature"},{"key":"10.1016\/j.siny.2003.12.001_BIB147","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1038\/380439a0","article-title":"Heterozygous embryonic lethality induced by targeted inactivation of the VEGF gene","volume":"380","author":"Ferrara","year":"1996","journal-title":"Nature"},{"key":"10.1016\/j.siny.2003.12.001_BIB148","doi-asserted-by":"crossref","first-page":"11947","DOI":"10.1016\/S0021-9258(18)99049-6","article-title":"The human gene for vascular endothelial growth factor. Multiple protein forms are encoded through alternative exon splicing","volume":"266","author":"Tischer","year":"1991","journal-title":"J Biol Chem"},{"key":"10.1016\/j.siny.2003.12.001_BIB149","doi-asserted-by":"crossref","first-page":"794","DOI":"10.1046\/j.1523-1755.1999.00610.x","article-title":"Role of vascular endothelial growth factor in the regulation of angiogenesis","volume":"56","author":"Ferrara","year":"1999","journal-title":"Kidney Int"},{"key":"10.1016\/j.siny.2003.12.001_BIB150","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1007\/BF00437467","article-title":"Similarities and differences between the vascular endothelial growth factor (VEGF) splice variants","volume":"15","author":"Neufeld","year":"1996","journal-title":"Cancer Metastasis Rev"},{"key":"10.1016\/j.siny.2003.12.001_BIB151","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1002\/1097-0177(2000)9999:9999<::AID-DVDY1093>3.0.CO;2-D","article-title":"Differential expression of VEGF isoforms in mouse during development and in the adult","volume":"220","author":"Ng","year":"2001","journal-title":"Dev Dyn"},{"key":"10.1016\/j.siny.2003.12.001_BIB152","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1165\/ajrcmb.27.2.4703","article-title":"Defective pulmonary development in the absence of heparin-binding vascular endothelial growth factor isoforms","volume":"27","author":"Galambos","year":"2002","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB153","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1038\/376066a0","article-title":"Role of the flt-1 receptor tyrosine kinase in regulating the assembly of vascular endothelium","volume":"376","author":"Fong","year":"1995","journal-title":"Nature"},{"key":"10.1016\/j.siny.2003.12.001_BIB154","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1038\/376062a0","article-title":"Failure of blood-island formation and vasculogenesis in Flk-1 deficient mice","volume":"376","author":"Shalaby","year":"1995","journal-title":"Nature"},{"key":"10.1016\/j.siny.2003.12.001_BIB155","doi-asserted-by":"crossref","first-page":"1078","DOI":"10.1126\/science.3461562","article-title":"Inhibition of endothelial regeneration by type-\u03b2 transforming growth factor from platelets","volume":"233","author":"Heimark","year":"1986","journal-title":"Science"},{"key":"10.1016\/j.siny.2003.12.001_BIB156","doi-asserted-by":"crossref","first-page":"4544","DOI":"10.1073\/pnas.86.12.4544","article-title":"An activated form of transforming growth factor \u03b2 is produced by cocultures of endothelial cells and pericytes","volume":"86","author":"Antonelli-Orlidge","year":"1989","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.siny.2003.12.001_BIB157","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1016\/0092-8674(90)90448-N","article-title":"TGF-\u03b2 induces bimodal proliferation of connective tissue cells via complex control of an autocrine PDGF loop","volume":"63","author":"Battegay","year":"1990","journal-title":"Cell"},{"key":"10.1016\/j.siny.2003.12.001_BIB158","doi-asserted-by":"crossref","first-page":"1845","DOI":"10.1242\/dev.121.6.1845","article-title":"Defective hematopoiesis and vasculogenesis in transforming growth factor-\u03b2 1 knock-out mice","volume":"121","author":"Dickson","year":"1995","journal-title":"Development"},{"key":"10.1016\/j.siny.2003.12.001_BIB159","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1083\/jcb.109.1.309","article-title":"Inhibition of endothelial cell movement by pericytes and smooth muscle cell: activation of a latent transforming growth factor-beta 1-like molecule by plasmin during co-culture","volume":"109","author":"Sato","year":"1989","journal-title":"J Cell Biol"},{"key":"10.1016\/j.siny.2003.12.001_BIB160","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1038\/ng1294-345","article-title":"Endoglin, a TGF-beta binding protein of endothelial cells, is the gene for hereditary hemorrhagic telangiectasia type I","volume":"8","author":"McAllister","year":"1994","journal-title":"Nat Genet"},{"key":"10.1016\/j.siny.2003.12.001_BIB161","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1038\/ng0696-189","article-title":"Mutations in the activin receptor-like kinase 1 gene in hereditary hemorrhagic telangiectasis type 2","volume":"13","author":"Johnson","year":"1996","journal-title":"Nat Genet"},{"key":"10.1016\/j.siny.2003.12.001_BIB162","first-page":"395","article-title":"The mouse tie receptor tyrosine kinase gene: expression during embryonic angiogenesis","volume":"9","author":"Korhonen","year":"1994","journal-title":"Oncogene"},{"key":"10.1016\/j.siny.2003.12.001_BIB163","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1038\/sj.jp.7200243","article-title":"Alveolar capillary dysplasia: diagnostic potential for cardiac catheterization","volume":"19","author":"Hintz","year":"1999","journal-title":"J Perinatol"},{"key":"10.1016\/j.siny.2003.12.001_BIB164","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1080\/107710497175083","article-title":"Alveolar capillary dysplasia: lung biopsy diagnosis, nitric oxide responsiveness, and bronchial generation count","volume":"17","author":"Sirkin","year":"1997","journal-title":"Pediatr Pathol Lab Med"},{"key":"10.1016\/j.siny.2003.12.001_BIB165","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1002\/ajmg.10056","article-title":"Bilateral tibial agenesis with ectrodactyly (OMIM 119100): further evidence for autosomal recessive inheritance","volume":"104","author":"Witters","year":"2001","journal-title":"Am J Med Genet"},{"key":"10.1016\/j.siny.2003.12.001_BIB166","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1046\/j.1440-1754.2001.00554.x","article-title":"Alveolar capillary dysplasia with antenatal anomalies mimicking trisomy 21","volume":"37","author":"McGaughran","year":"2001","journal-title":"J Paediatr Child Health"},{"key":"10.1016\/j.siny.2003.12.001_BIB167","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1038\/sj.jp.7200494","article-title":"Alveolar capillary dysplasia with misalignment of pulmonary veins and anterior segment dysgenesis of the eye: a report of a new association and review of the literature","volume":"21","author":"Merchak","year":"2001","journal-title":"J Perinatol"},{"key":"10.1016\/j.siny.2003.12.001_BIB168","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1016\/S0090-4295(00)00496-9","article-title":"Urethral atresia in a neonate with alveolar capillary dysplasia and pulmonary venous misalignment","volume":"55","author":"Vick","year":"2000","journal-title":"Urology"},{"key":"10.1016\/j.siny.2003.12.001_BIB169","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/S1084-2756(02)00196-3","article-title":"Genetic influences and neonatal lung disease","volume":"8","author":"Hallman","year":"2003","journal-title":"Semin Neonatol"},{"key":"10.1016\/j.siny.2003.12.001_BIB170","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1007\/s00431-002-1046-1","article-title":"Polymorphisms of surfactant protein B encoding gene: modifiers of the course of neonatal respiratory distress syndrome?","volume":"161","author":"Makri","year":"2002","journal-title":"Eur J Pediatr"},{"key":"10.1016\/j.siny.2003.12.001_BIB171","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1186\/rr163","article-title":"Surfactant gene polymorphisms and interstitial lung diseases","volume":"3","author":"Pantelidis","year":"2002","journal-title":"Respir Res"},{"key":"10.1016\/j.siny.2003.12.001_BIB172","doi-asserted-by":"crossref","first-page":"2751","DOI":"10.1093\/hmg\/9.18.2751","article-title":"Surfactant proteins A and B as interactive genetic determinants of neonatal respiratory distress syndrome","volume":"9","author":"Haataja","year":"2000","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.siny.2003.12.001_BIB173","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1034\/j.1399-0004.2001.600303.x","article-title":"Family-based transmission disequilibrium test (TDT) and case-control association studies reveal surfactant protein A (SP-A) susceptibility alleles for respiratory distress syndrome (RDS) and possible race differences","volume":"60","author":"Floros","year":"2001","journal-title":"Clin Genet"},{"key":"10.1016\/j.siny.2003.12.001_BIB174","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1183\/09031936.01.00043401","article-title":"Surfactant protein gene A, B, and D marker alleles in chronic obstructive pulmonary disease of a Mexican population","volume":"18","author":"Guo","year":"2001","journal-title":"Eur Respir J"},{"issue":"Suppl 1","key":"10.1016\/j.siny.2003.12.001_BIB175","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1159\/000047173","article-title":"Surfactant protein A and B genetic variants and respiratory distress syndrome: allele interactions","volume":"80","author":"Floros","year":"2001","journal-title":"Biol Neonate"},{"key":"10.1016\/j.siny.2003.12.001_BIB176","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1007\/s004390100574","article-title":"Respiratory distress syndrome: evaluation of genetic susceptibility and protection by transmission disequilibrium test","volume":"109","author":"Haataja","year":"2001","journal-title":"Hum Genet"},{"key":"10.1016\/j.siny.2003.12.001_BIB177","doi-asserted-by":"crossref","first-page":"1569","DOI":"10.1086\/302906","article-title":"Association between the surfactant protein A (SP-A) gene locus and respiratory-distress syndrome in the Finnish population","volume":"66","author":"Ramet","year":"2000","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.siny.2003.12.001_BIB178","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1016\/S0925-4439(98)00077-5","article-title":"Genetics of the hydrophilic surfactant proteins A and D","volume":"1408","author":"Floros","year":"1998","journal-title":"Biochim Biophys Acta"},{"key":"10.1016\/j.siny.2003.12.001_BIB179","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1203\/00006450-199802000-00003","article-title":"Association of pulmonary surfactant protein A (SP-A) gene and respiratory distress syndrome: interaction with SP-B","volume":"43","author":"Kala","year":"1998","journal-title":"Pediatr Res"},{"key":"10.1016\/j.siny.2003.12.001_BIB180","doi-asserted-by":"crossref","first-page":"583","DOI":"10.1042\/bj3050583","article-title":"Dinucleotide repeats in the human surfactant protein-B gene and respiratory-distress syndrome","volume":"305","author":"Floros","year":"1995","journal-title":"Biochem J"},{"key":"10.1016\/j.siny.2003.12.001_BIB181","doi-asserted-by":"crossref","first-page":"324","DOI":"10.1002\/ajmg.a.20058","article-title":"Analysis of 40 sporadic or familial neonatal and pediatric cases with severe unexplained respiratory distress: relationship to SFTPB","volume":"119A","author":"Tredano","year":"2003","journal-title":"Am J Med Genet"},{"key":"10.1016\/j.siny.2003.12.001_BIB182","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1155\/1999\/961430","article-title":"Novel, non-radioactive, simple and multiplex PCR-cRFLP methods for genotyping human SP-A and SP-D marker alleles","volume":"15","author":"DiAngelo","year":"1999","journal-title":"Dis Markers"},{"key":"10.1016\/j.siny.2003.12.001_BIB183","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1155\/2002\/194075","article-title":"Surfactant protein B intron 4 variation in German patients with COPD and acute respiratory failure","volume":"18","author":"Seifart","year":"2002","journal-title":"Dis Markers"},{"key":"10.1016\/j.siny.2003.12.001_BIB184","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1038\/sj.bjc.6600353","article-title":"Surfactant protein B gene variations enhance susceptibility to squamous cell carcinoma of the lung in German patients","volume":"87","author":"Seifart","year":"2002","journal-title":"Br J Cancer"},{"key":"10.1016\/j.siny.2003.12.001_BIB185","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1203\/00006450-200206000-00006","article-title":"Surfactant protein D gene polymorphism associated with severe respiratory syncytial virus infection","volume":"51","author":"Lahti","year":"2002","journal-title":"Pediatr Res"},{"key":"10.1016\/j.siny.2003.12.001_BIB186","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1164\/ajrccm.165.4.2102080","article-title":"An ELISA technique for quantification of surfactant apoprotein (SP)-C in bronchoalveolar lavage fluid","volume":"165","author":"Schmidt","year":"2002","journal-title":"Am J Respir Crit Care Med"}],"container-title":["Seminars in Neonatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S108427560300174X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S108427560300174X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2023,4,27]],"date-time":"2023-04-27T20:01:24Z","timestamp":1682625684000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S108427560300174X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,8]]},"references-count":186,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2004,8]]}},"alternative-id":["S108427560300174X"],"URL":"https:\/\/doi.org\/10.1016\/j.siny.2003.12.001","relation":{},"ISSN":["1084-2756"],"issn-type":[{"value":"1084-2756","type":"print"}],"subject":[],"published":{"date-parts":[[2004,8]]}}}